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Year 5: Pathology > Histopathology > Flashcards

Histopathology Flashcards

1
Q

Blood supply of the liver

A

Dual blood supply: portal vein, hepatic artery

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2
Q

What are the cells of the liver?

A

Hepatocytes

Bile ducts

BVs

Endothelial cells

Kupffer cells

Stellate cells

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3
Q

What is the basic structure of the liver?

A

Hepatic lobule

At the centre are the terminal branches of the hepatic vein. The angles of the hexagon are formed by the portal tracts that contain 3 structures: BD, hepatic artery and portal vein

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4
Q

Where are centrilobular hepatocytes found?

Where are periportal hepatocytes found?

A

Located near the terminal hepatic vein i.e. zone 3: more metabolically active.

Located near the portal tract, receive blood rich in nutrients and O2.

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5
Q

What organ is this?

A

Liver

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6
Q

What are the functions of the liver?

A

Metabolic: involved in glycolysis, glycogen storage, glucose synthesis, amino acid synthesis, FA synthesis, lipoprotein metabolism. Drug metabolism.

Protein synthesis: make all circulating proteins except gamma globulins, including albumin, fibrinogen, and coag factors

Storage: glycogen, viamins A, D and B12 in large amounts. Small amounts of vitamin K, folate, Fe, Cu

Hormone metabolism: activates vit D. Conjugation and excretion of steroid hormones (oestrogens, GCs)< peptide hormone metabolism (insulin, GH< PTH)

Bile synthesis: 600-1000ml daily.

Immune function: antigens from gut reach liver via portal criculation and phagocytosed by kuppfer cells.

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7
Q

Which protein is not synthesised in the liver?

A

Gamma globulins

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8
Q

Which vitamins are stored in the liver?

A

A, D , B12 (large amount)

K (small amount)

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9
Q

What is this structure

A

Portal tract

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10
Q

What cellular changes occur following injury to the liver?

A

Loss of hepatocyte microvilli

Activation of stellate cells

Deposition of scar matrix

Loss of fenestrae

Kuppfer cell activation

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11
Q

Definition of cirrhosis

A

Involves whole liver

Fibrosis

Nodules of regenerating heaptocytes

Distortion of liver vascular architecture: intra and extra hepatic shunting of blood

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12
Q

How can cirrhosis be classified?

A

According to nodule size: micro or macronodular

According to aetiology: ETOH/insulin resistance, viral heaptitis etc

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13
Q

What are the complications of cirrhosis?

A

Portal HTN

Hepatic encephalopathy

HCC

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14
Q

What are the causes of acute hepatitis

A

Viruses

Drugs

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15
Q

What is the process shown here?

What is this known as?*

A

Acute hepatitis

“Spotty necrosis”

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16
Q

What are the causes of chronic hepatitis?

What does the grade of chronic hepatitis refer to?

Stage?

A

Viral

Drugs

Autoimmune

Grade= severity of inflammation

Stage= severity of fibrosis

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17
Q

What does this show?

A

Portal hepatitis

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18
Q

What does this show?

Also known as?

A

Interface hepatitis

“piecemeal necrosis”

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19
Q

What does this show?

A

Liver fibrosis

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20
Q

What is the pathological course of hep C infection

A

Acute: asymptomatic, 15-30% clear the infection

Fibrosis: (F0-F3), virus unlikely to clear without treatment, people may still be asymptomatic. Fatigue, URQ discomfort, transient appetite loss. End stage symptoms: itching, depression, impaired memory.

Scarring can be mild to severe. Extrahepatic Cxs: cryoglobulinaemia, glomerulonephritis, kerratoconjunctivitis sicca.

Cirrhosis (F4): Symptoms due to hepatic insufficiency and portal HTN: ascites, oesophageal varices, hepatic encephalopathy. Consider OLT

HCC: early stage: OLT, Sx, percutaenous ablation. Intermediate: TACE, Terminal

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21
Q

Spectrum of ETOHic liver disease?

A

Fatty liver

Alcoholic hepatitis

Cirrhosis

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22
Q

What does this show?

A

Fatty liver disease

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23
Q

What does this show?

What are the arrows pointing to?

A

Alcoholic hepatitis

Mallory bodies

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24
Q

What does this show?

What are the features?

A

Alcoholic liver cirrhosis

Micronodular

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25
Q

What does NAFLD look like?

What causes it?

A

Histologically looks like alcoholic liver disease

Due to insulin resistance associated with raised BMI and DM

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26
Q

What is PBC?

Features

Abs?

A

Primary biliary cirrhosis

Bile duct loss associated with chronic inflammation: granulomas

AMAs (anti-mitochondrial)

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27
Q

What does this show?

A

Primary biliary cirrhosis

Bile duct loss with granulomas

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28
Q

What is PSC?

What are the features?

With what is it associated?

A

Primary sclerosing cholangitis

Periductal bile duct fibrosis leading to loss

Associated with UC

Increased risk of cholangiocarcinoma

ERCP Dxic

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29
Q

What does this show?

A

Primary sclerosing cholangitis

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30
Q

What is haemochromatosis?

Cause?

Cxs?

A

Genetically determined increased gut iron absorption

Gene on chromosome 6

Parenchymal damage to organs secondary to Fe deposition-> “bronzed diabetes”

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31
Q

What does this show?

A

Haemochromatosis

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32
Q

What is haemosiderosis?

A

Accumulation of Fe in macrophages

Seen following blood transfusion

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33
Q

What is Wilson’s

By what is it caused

Cxs?

A

Accumlation of Cu due to feailure of excretion by hepatocytes

Genes on chromosome 13

Accumulates in the liver and CNS-> hepatolenticular degenration

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34
Q

What is a histopathological stain for Wilson’s?

A

Rhodanine

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35
Q

What disease is this?

A

Wilson’s wth a rhodanine stain.

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36
Q

What is a clinical sign in Wilson’s?

A

Keyser-Fleischer rings

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37
Q

What are the features of autoimmune hepatitis?

A

Interface hepatitis with plasma cells. Anti-SMA Abs

Responds to steroids.

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38
Q

What is Alpha-one antitrypsin deficiency?

A

Failure to secrete alpha-one antitrypsin

Leads to intra-cytoplasmic inclusions, hepatitis and cirrhosis

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39
Q
A

Alpha-1 antitrypsin

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40
Q

What are the causes of hepatic granulomas?

A

Specific: PBC, drugs

General: TB, sarcoid

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41
Q

What are the benign liver tumours?

A

Liver cell adenoma

bile duct adenoma

haemangioma

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42
Q

What is the most common type of liver tumour?

A

Secondary > primary

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43
Q

What are the primary liver malignancies?

A

HCC

Hepatoblastoma

Cholangiocarcinoma

Haemangiosarcoma

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44
Q

What does this show?

A

HCC

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45
Q

With what is cholangiocarcinoma associated?

Whence can it arise?

How is the prognosis?

A

PSC, helminth infection, cirrhosis, congenital liver abnormalities, Lynch syndrome Type II

Intrahepatic ducts, extrahepatic ducts (including GB)

Poor

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46
Q

What does this show?

A

Cholangiocarcinoma

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47
Q

What are the clinical features of hepatic adenoma?

A

Associated with OCP

Presents with abdo pain/ intraperitoneal bleeding

Resection if symptomatic, >5cm or if no shirnkage when stopping OCP

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48
Q

What are the features of haemangioma:

A

Most common benign lesion

No Rx

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49
Q

What are causes of HCC?

Ix?

A

Hepattis B + C, ETOHic cirrhosis. Haemocrhomatosis, NAFLD, aflatoxin, androgenic steroids.

Ix: alpha fetoprotein, USS

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50
Q

What is Lynch Syndrome caused by?

What are the classifications?

A

MMR defect

Type 1: HNPCC

Type 2: Extracolonic

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51
Q

What are the common sites to metastasise to the liver?

A

GI, breast or bronchus

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52
Q

What are the major causes of cirrhosis

A

ALD

NAFLD

Chronic viral hepatitis (B+/-D, C)

Autoimmune

Biliary causes:PBC and PSC

Genetic: haemochromatosis (HFE gene), Wilsons (ATP7B gene), A1AT, galactosaemia, glycogen storage disease

Drugs e.g. methotrexate

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53
Q

What are the causes of micronodular cirrhosis?

A

Uniform liver involvement (<3mm)

Alcoholic hepatitis, biliary tract disease

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54
Q

What are the causes of macronodular cirrhosis?

A

Variable nodule size (>3mm)

Viral hepatitis, Wilson’s, A1AT

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55
Q

What is the pathological process in cirrhosis?

A

Chronic inflammation causes stellate cell activation in the space of Disse

They become myofibroblasts that initiate fibrosis by deposition of collagen in space of Disse

Myofibroblasts contract, constricting sinusoids and increasing vascular resistance.

Undamaged hepatocytes regenerate in nodules between fibrous septa

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56
Q

What is the name of the score used to indicate Px in liver cirrhosis?

On what is it based?

A

Modified Child’s Pugh

Ascites

Encephalopathy

Bilirubin

Albumin

Prothrombin time

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57
Q

What are the thresholds for the Child pugh score?

A

<7: 45% 5 year survival

7-9: 20%

>10: <20%

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58
Q
A
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59
Q

What is portal HTN secondary to?

What happens?

A

Increased vascular resistance in liver

Hyperdynamic circulation

Sodium retention and plasma volume expansion

When portal pressure >10-12 mmHG, venous system dilates and collateral vessels form

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60
Q

Where are the collateral vessels found in portal HTN?

GORLDRA

A

GO junction

Rectum

L renal vein

Diaphragm

Retroperitoneum

Anterior abdominal:umbilical vein

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61
Q

What are the causes of portal HTN?

A

Pre-hepatic: Portal vein thrombosis: Factor V leiden

Hepatic

Pre-sinusoidal: Schistosomiasis, PBC, sarcoid

Sinusoidal: cirrhosis

Post-sinusoidal: veno-occlusive disease

Post-hepatic: Budd-Chiari syndrome

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62
Q

Causes of Budd-Chiari Syndrome

Mx of Budd-Chiari

A

30% idiopathic

Thrombophilia

OCP

Leukaemias

Compression by renal tumours, HCC

RTx

Thrombolyse, treat underlying cause. TIPS

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63
Q

What is a TIPS?

A

Transjugular Intrahepatic portosystemic shunt

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64
Q

What are the macroscopic and microscopic features of

Hepatic steatosis

A

Large, pale, yellow, greasy

Accumulation of fat droplets in hepatocytes

Fully reversible if ETOH avoided

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65
Q

What are the macroscopic and microscopic features of

Alcoholic hepstitis

A

Large, fibrotic liver

Hepatocyte ballooning and necrosis due to accumulation of fat, water and proteins

Mallory bodies

Fibrosis

Seen acutely after heavy night of drinking. Can range from asymptomatic to fulminant liver failure

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66
Q

What are the macroscopic and microscopic features of

Alcoholic cirrhosis

A

Yellow-tan, fatty, enlarged, transforms into shrunken, non-fatty brown organ

Micronodular cirrhosis (<3mm)

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67
Q

What are the features of NAFLD?

A

Hepatic steatosis in non-ETOHs

Most common cause of chronic liver disease in West

NAFLD: simple steatosis

NASH: steatosis and hepatitis, can progresss to cirrhosis

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68
Q

What are the features of autoimmune hepatitis?

A

Common with other autoimmune disease e.g. coeliac, SLE< RA, thyroiditis, Sjogrens, UC

78% female, young and postmenopausal

HLA-DR3

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69
Q

What is Type 1 autoimmune hepatitis?

A

ANA, anti-SMA< anti-actin, anti-soluble liver Ag

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70
Q

What is Type 2 autoimmune hepatitis?

A

Anti-LKM (liver, kidney, microsomal)

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71
Q

Mx of autoimmune hepatitis

A

Immunosuppression until transplant.

Disease recurs in 40%

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72
Q

Features of PBC

Epidemiology

LFTs

Abs

Hx

Mx

A

Auotimmune inflammatory destruction of medium sized intra-hepatic bile ducts-> cholesstasis->slow development of crrhosis

F>M 10:1

Peak incidence 40-50y/o

raised ALP, cholesterol, IgM, hyperbilirubinaemia (late)

AMA in >90%

US scan shows no bile duct dilatation

Histology: bile duct loss with granulomas

Fatigue, pruritus and abdo discomfort. Skin pigementation, anthelasma, steatorrhoea, Vit D malabsorption, inflammatory arthropathy

Ursodeoxycholic acid in early phase

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73
Q

Features of PSC

Epidemiology

Bloods

Dx

Cx

A

Inflammation and obliterative fibrosis of extrahepatic and intrahepatic bile ducts-> multi-focal stricture formation with dilation of preserved semgnets

M>F

Peak incidence at 40-50y/o

IBD associated (UC)

Raised ALP< several associated auto-Ig (p-ANCA)

US: bile duct dilatation

ERCP: shows beding of the bile ducts due to multifocal strictures

Increased incidence of cholangiocarcinoma

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74
Q

Haemochromatosis

Epidemiology

Pathophysiology

Histology

Signs/Symptoms

Ix

Treatment

A

Genetic

40-50y/o

Autosomal recessive: mutated HFE gene 6p21.3-> fe absorption which deposits in liver, heart, pancreas, adrenals, pituitarry, joints, skin-> fibrosis

Fe deposits in liver stains with Prussian blue

Skin bronzing (melanin deposition), DM, hepatomegaly, cardiomyopathy, hypogonadism, pseudogout

Ix: raised Fe, ferritin, transferrin sa >45%, decreased TIBC

Venesection

Desferrioxamine

30% with cirrhosis -> HCC

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75
Q

Wilson’s disease

Epidemiology

Pathophysiology

Histology

Signs/Symptoms

Ix

Treatment

A

v. rare

11-14y

Autosomal recessive: mutated ATP7B (Chr13) encodes Cu transporting ATPase-> decreased biliary Cu excretion and deposition in liver, CNS, iris.

Cu stains with Rhodanine stain, Mallory bodies and fibrosis on microscopy.

Liver disease: acute hepatitis, fulminant liver failure or cirrhosis.

Neuro disease: parkinsonism, psychosis, dementia

Decreased serum caeruloplasmin, decreased serum Cu, increased urinary Cu

Lifelong penicillinamine

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76
Q

A1AT

Epidemiology

Pathophysiology

Histology

Signs/Symptoms

Ix

Treatment

A

Autosomal dominant: A1AT accumulates in hepatocytes -> intracytoplasmic inclusions -> hepatitis. lack of A1AT in lungs -> emphysema

Intracytoplasmic inclusions of A1AT which stain with periodic acid Schiff

Kids: neonatal jaundice

Adults: emphysema and liver disease

Ix: redcued A1AT absent alpha globulin band on electrophoresis.

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77
Q

What are the functions of bone?

A

Mechanical: support and site of muscle attachment

Protective: vital organs and bone marrow

Metabolic: Ca reserve

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78
Q

What is the composition of bone?

Inorganic

Organic

A

Inorganic: (65%)

Ca hydroxyapatite (10Ca 6PO4 OH2)

body store for 99% of body Ca

85% of P, 65% Na and Mg

Organic: bone cells and protein matrix

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79
Q

What is the structure of bone medial to lateral?

A

Medulla, Cortex, Periosteum

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80
Q

What is the structure of bone proximal to distal?

A

Diaphysis

Metaphysis

Epihpyseal line

Epiphysis

Subchondral bone

Articular cartilage

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81
Q

What are the features of cortical bones?

A

Long bones

80% of bony skeleton

Appendicular

80-90% calcified

Mainly mechanical and protective

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82
Q

What are the features of cancellous bone?

A

Vertebrae and pelvis

20% of skelton

Axial

15-25% calcified

Mainly metabolic

Large surface

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83
Q

What type of bone is this?

A

Cortical

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84
Q

What type of bone is this?

A

Cancellous

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85
Q

What are the types/classifications of bone?

A

Woven/lamellour

Anatomically: flat/long bones

  • intramembranous and anedochondral ossification

Trabecular (cancellous)/compact (cortical)

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86
Q

What are the arrows pointing at?

A
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87
Q

What is the function of osteoBlasts?

A

Build bone by laying down osteoid

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88
Q

What is the function of osteoclasts?

A

Multinucleate cells of macrophage family

Resorb bone

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89
Q

What are osteocytes?

A

Osteoblast like cells which sit in lacunae in bone

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90
Q

What is a lacuna?

A

In histology, a lacuna is a small space containing an osteocyte in bone or chondrocyte in cartilage.

The Lacunae are situated between the lamellae, and consist of a number of oblong spaces.

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91
Q
A
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92
Q

Draw a diagram showing the modulation of osteoclastogenesis

A

RANK is expressed on the surface of osteoclast lineage cells

RANKL expressed on MSCs of ostebolast lineage and on B and T Ls

When RANKL binds to RANK this causes osteoclast precursor cell to differentiate, increasing bone resorption

OPG competed with RANK for RANKL

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93
Q

How do tumour cells mediate local growth of tumour in bone?

A

Oncogene products produced by tumour cells metastasising to bone influence the bone cells to resorb bone and promote local growth of the tumour. This is mediated by the RANK /OPG signalling pathway.

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94
Q

What type of malignancy often causes more bone growth than destruction?

A

Prostate carcinoma

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95
Q

What is metabolic bone disease?

A

Disordered bone turnover due to imblanace of chemicals in body: vitamins, hormones, minerals

Net effect: reduced bone mass-> pathological #

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96
Q

What are the 3 main categroies of metabolic bone disease?

A

Non-endocrine e.g. age-related

Endocrine: e.g. Vit D, PTH

Disuse osteopenia

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97
Q

What are the histological characteristics in metabolic bone disease

Where must the biopsy be taken?

A

Static parameters: cortical thickness and porosity, trabecular bone volume, thickness, number & separation of tranbeculae

Biopsy from iliac crest

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98
Q

What is this showing?

What is the pink?

A

Trabecular (cancellous) bone

The trabecular bone, the grey is the marrow

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99
Q

What can be used to study the hisodynamic parameters of bone?

A

Fluorescent tetracycline labelling

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100
Q

What is the aetiology of osteoporosis?

A

1o: age, post-menopause
2o: drugs, systemic disease

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101
Q

What is the pathogenesis of osteoporosis?

High turnover?

Low turnover?

A

Pathogenesis: low intiial bone mass or accelerated bone loss can reduce bone mass below # threshold

90% of cases due to insufficient Ca intake and post-menopausal oestrogen deficiecny

High turnover: increased bone resorption

Low turnover: decreased bone formation

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102
Q

What are the influencing factors for osteoporosis?

A

Nutrition and social practices: ETOH, smoking, malabsorption, Vit C & DD

Endocrine

Immobilisation

Iatrogenic: corticosteroids, long term heparin or phenyotin therapy, casstration, XS thyroid therapy

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103
Q

What are the risk factors for osteoporosis?

A

Advanced age

Female

Smoking

ETOH

Early menopause

LT immobility

Low BMI

Poor Diet/malabsorption

Thyroid disease

Low testosterone

Chronic renal disease

Steroids

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104
Q

What is the UK societal impact of osteoporotic #s?

A

50% of patients cannot live independently post #

20% die

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105
Q

What is the presentation of osteoporosis?

A

Back pain and #

Classic #s:

wrist (Colle’s)

hip: NOF and intertrochanteric

pelvis

>60% vertebral #s are asymptomatic with compresion # usually in T11-L2

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106
Q

Ix in osteoporosis

A

Lab:

Serum Ca, P and ALP (usually N)

Urinary Ca

Collagen breakdown products

Imaging

Bone densitometry

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107
Q

What are the cut offs for bone densitometry?

A

T score: 1-2.5SD below normal peak bone mass= osteopenia

>2.5SD= osteoporosis

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108
Q

What is osteomalacia?

A

Defective bone mineralisation:

either Vit DD or PO4 deficiency

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109
Q

What are the sequelae of osteomalacia?

A

Bone pain/tenderness

#

Proximal weakness

Deformity

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110
Q

What does this show?

A

Osteomalacia (Rickets)

There is a widening of the growth plate which is also irregular and the femur and tibia become bowed as the child starts to walk and the legs have to weight bear.

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111
Q

What is the abnormality

What disease?

A

Horizontal # in Looser’s zone

Osteomalacia

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112
Q

Features of hyperPTHism

A
  • Excess PTH
  • increased Ca + PO4 excretion in urine
  • hypercalcaemia
  • hypophosphataemia
  • skeletal changes of osteitis fibrosa cystica
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113
Q

What is the differnec between 1o and 2o hyperparathyroidism?

A

1o: parathyroid adenoma (85-90%) or chief cell hyperplasia
2o: chronic renal deficiency, Vit DD, malabsorption

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114
Q

Hypercalcaemic mnemonic

A
  • Symptoms Mnemonic
  • Stones (Ca oxalate renal stones)
  • Bones (osteitis fibrosa cystica, bone resorption)
  • Abdominal groans (acute pancreatitis)
  • Psychic moans (psychosis & depression)
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115
Q

What is renal osteodystrophy?

A

Comprises all the skeletal changes of chronic renal disease

Increased bone resorption: osteitis fibrosa cystica

Osteomalacia

Osteosclerosis

Growth retardation

Osteoporosis

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116
Q

What does this XR show?

A

Osteitis fibrosa cystica: increase bone resorption

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117
Q

Lab features of renal osteodystrophy

A
  • PO4 retention – hyperphosphataemia
  • Hypocalcaemia as a result of decreased vit D
  • 2o hyperparathyroidism
  • Metabolic acidosis
  • Aluminium deposition
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118
Q

What is Paget’s?

What are the 3 phases?

A

Disorder of bone turnover

Osteolytic

Osteolytic-osteosclerotic

Quiescent osteosclerotic

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119
Q

What is this?

A

Paget’s disease of bone

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120
Q

Features of Paget’s?

A

Onset >40y

M=F

Rare in asians/africans

Mono-ostotic in 15%, remained polyostotic

Aetiology is unknown

Familial pattern shows autosomal pattern with incomplete penetrance

Parvomyxovirus type particles have been seen in Pagetic bone

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121
Q

What are the 5 most commonly affected sites in Paget’s?

A

Vertebrae

Skull

Pelvis

Femur

Tibia

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122
Q

Clinical features of Paget’s?

A

Pain

micro#

Nerve compression

Skull changes may put medulla at risk

+/- haemodynamic chanes, HF

Development of sarcoma in area of involvement in 1%

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123
Q

What does this show?

A

Paget’s disease affecting tibia

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124
Q

What are the indications for bone biopsy?

A

Suspected osteomalacia

Diagnostic classification of renal osteodystrophy

Osteopaenia: in young patients (<50) or associated with abnormal Ca metabolism

Classification of hereditary childhood bone disease

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125
Q

XR. histological and biochemical findings for Osteoporosis

A

No XR

Loss of cancellous bone

N Ca, N PO4, N ALP

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126
Q

XR and histological findings for Osteomalacia

A

Looser’s zones: pseudo#s, splaying of metaphysis

Excess of unmineralised bone: osteoid

N/L Ca, L PO4, H ALP

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127
Q

XR and histological findings for 1o hyperparathyroidism

A

Brown’s tumours, Salt and pepper skull, Subperiosteoal bone resorption in phalanges

Osteitis fibrosa cystica: marrow fibrosis and cysts aka Brown tumour

Raised Ca,, Low/N PO4, Raised/N ALP

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128
Q

What are Brown’s tumours?

A

The brown tumor is a bone lesion that arises in settings of excess osteoclast activity, such as hyperparathyroidism. It is not a true neoplasm, as the term “tumor” suggests; however, it may mimic a true neoplasm.[1]Brown tumours are radiolucenton x-ray.

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129
Q
A

Hyperparathyroidism: Brown’s tuour of the hands

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130
Q
A

Salt and pepper sign of the calvaria refers to multiple tiny hyperlucent areas in the skull vault caused by resorption of trabecular bone in hyperparathyroidism.

There is loss of definition between the inner and outer tables of the skull and a ground-glass appearance as well as spotty deossification.

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131
Q

XR and histological findings for 1o Paget’s?

A

Mixed lytic and sclertoic. Skull: osteoprorosis circumscripta, cotton wool.

Vertebrae: picture frame, ivory vertebrae

Pelvis: sclerosis and lucency

Huge osteclosts w >100 nuclei, mosaic (like jigsaw) pattern of lamellar bone

N Ca + PO4, +++ALP

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132
Q
A

Osteoporosis circumscripta cranii (also known as osteolysis circumscripta) refers to discrete radiolucent regions of the skull on plain radiographs. They are often seen in context of the lytic (incipient-active) phase of Paget’s disease of the skull, but may be observed in other circumstances as well, e.g. hyperparathyroidism, leontiasis ossea 9.

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133
Q
A

Cotton wool skull- Pagets

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134
Q
A

Picture frame vertebra

Paget’s

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135
Q
A

Ivory vertebra

Paget’s

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136
Q

What are the features of astrocytes?

A

Most abundant

Anchor neurones by numerous projections, regulate environment e.g. ions, neurotransmitters and form BBB

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137
Q

Features of oligodendrocytes?

A

Coat axons with their cell membranes forming myelin

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138
Q

Features of ependymal cells?

A

Line the cavities of the CNS, make up walls of the ventricles, create and secrete CSF and beat cilia to help move the CSF.

Act as neuronal stem cells

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139
Q

Radial glia features

A

Arise from the neuroepithelial cells in embrogenes, act as the scaffold for new neurones

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140
Q

Features of Schwann cells?

A

Provide myelination to PNS

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141
Q

Features of satellite cells?

A

Small cells that surround the neurones in sensory sympathetic and parasympathetic ganglia, helping to regulate the environment

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142
Q

Features of enteric glial cells

A

Intrinsic ganglia of the GIT

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143
Q

Function of microglia

A

Act as macrophages

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144
Q

What are the glial cells?

A

Astrocytes

Oligodendrocytes

Microglia

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145
Q

Which cells interface with the CSF?

A

Ependyma

Choroid plexus epithelium

Meninges

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146
Q

Which CNS cells interface with blood?

A

Endothelium and pericytes

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147
Q

What is the most common CNS tumour?

A

Metastatic neoplasm

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148
Q

What are the principle malignancies causing neurometastases?

A

Leukaemias and lymphomas, more so in young.

Lung, breast and malignant menaloma

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149
Q

What are the features of brain mest?

Pathology?

Symptoms?

A

May involve the meninges as well as the parenchyma

Well demarcated solitary or multiple lesions with surrounding oedema

Neurological efffects and raised ICP

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150
Q

What are the 5 most common malignancies leading to CNS tumours in adults?

BLCPB

A

Breast

Lung

Large Bowel

Prostate

Bladder

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151
Q

What are the most common group of CNS primary tumours?

A

Astrocytomas

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152
Q

Astrocytomas

Age of onset

Pathology

Grading

Symptoms

Px

A

Any age, elderly usually worse

An infiltrative growth pattern in the cerebral hemispheres

Grading is based on the degree of dy/dx with histological grade an important predictor of behaviour

Raised ICP and focal neuro signs

Depends on site, grade and age of the patient

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153
Q

What are the types of astrocytoma?

A

Pilocytic astrocytomas: more common in children but can occur any age.

Anaplastic astrocytoma

Glioblastoma multiforme: necrotic, poorly differentiated tumour

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154
Q

Features of oligodendroglioma

Px

A

Most common in adulthood

Usually in the cerebral hemisphere and are soft and gelatinous

Better demarcated than infiltrating astroctomas

Calcifcaition common

Px less predictable, dependant on grade site, patient age, cytogenetics etc

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155
Q

Features of ependyomas?

Symptoms?

NB AA?

A

Occur at any age

Most offen within the ventricular cavities or within the canal of the SC

Usually well demarcated

Symptoms depend on site: intracranial: hydrocephalus or raised ICP

Anaplastic astrocytomas/variants often also disseminate through the sub arachnoid space

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156
Q

Where are ependyomas found in <20y/o?

In adults?

A

In te ventricular cavities

In the SC

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157
Q

Features of primitive neuroepithelial neoplasms

A

Composed of embryonal primitive cells

Most common in children

Undifferentiated lesions

Px: most survive 5y or more

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158
Q

Medulloblastoma features

A

Lesion of the cerebellum in the first 2 decades of life

Leads to raised ICP and cerebellar signs

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159
Q

Features of primary CNS lymphoma

A

Increased since AIDs

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160
Q

Features of meningiomas

A

Derive from meningioethlial cells

Most occur in the brain parenchyma but can occur in the cranial vault and cord.

Usually adults

Increased in NF2

Usually lobulated lesions, sharp interface between tumour and parenchyma

Overlying skull may be thickened or invaded by the tumour

Symptoms: raised ICP with focal neurological signs

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161
Q

NF2 in brain tumours?

A

Meningioma

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162
Q

Ventricular tumour/hydrocephlaus in brain tumours?

A

Ependyoma

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163
Q

Indolent/childhood in brain tumours?

A

Pilocytic astrocytoma

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164
Q

Soft gelatinous calcified brain tumour

A

Oligodendroma

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165
Q

What genetic conditions predispose to CNS malignancy?

A

VHL

NF 1 + 2

Li-Fraumeni

Gorlin

Turcot syndrome

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166
Q

Sings of supratentorial tumours

A

Focal neurological deficit

Seizure

Altered mental status

Headache

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167
Q

Signs of subtenotrial tumours

A

Cerebellar ataxia

Long tract signs

CN palsy

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168
Q

What are the neurosurgical approaches to a CNS malignancy

A

Stereotacic biopsy

Open biopsy

Craniotomy for debulking

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169
Q

CNS tumours:

Grade 1

2

3

4

A

LT survival

Causes death in >5y

Death in <5y

Deaith within 6m-1y

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170
Q

Features of infiltrative gliomas

A

Account for 80% of gliomas.

Astrocytomas- Oligodendrogliomas and mixed

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171
Q

What is the most aggressive infiltrative glioma?

A

De novo glioblastoma

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172
Q

What mutation is responsible for 80% of diffuse astrocytomas?

A

IDH1

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173
Q

What is secondary glioblastoma?

A

From progression of a lower grade astrocytoma

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174
Q

What is the proportion of diffuse astrocytomas found in the crebellum?

A

10%

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175
Q

Which has the better Px, astrocytomas or oligodendroglioma?

What is important?

A

Oligodendroglioma

Resection

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176
Q
  • Usually 1st and 2nd decade - 20% of CNS tumours below 14 years and 15% between 14-18 years
  • Often cerebellar, optic-hypothalamic, brain stem
  • Often cystic. Always contrast enhancement
  • They can disseminate in the subarachnoid space (es: follow nerve roots)
  • Compressive margins (never diffuse infiltration)
  • Variable histological features
  • Very often Rosenthal fibres and granular bodies
  • Hallmark: Piloid “hairy” cell
A

Pilocytic astrocytoma

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177
Q
  • Rare (0.5 per 100,000 year, in children)
  • 75% arise in the vermis in children and hemispheric in adults
  • Present with cerebellar signs, cranial hypertension
A

Medulloblastoma

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178
Q
  • 24-30% primary intracranial tumours
  • Incidental in up to 10% of post-mortem
  • Usually adults – rare in patients younger than 40 (more aggressive)
  • Focal symptoms (seizure, compression)
  • Any site of craniospinal axis
A

Meningioma

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179
Q

What is crucial in the assesmnet of grade of CNS primaries?

A

Mitotic activity

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180
Q

What is occuring in this image?

A

Pseudoinvasion along Virchow-Robin’s space

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181
Q

What is the commonest cause of CNS disease?

What is the most common cause of this?

A

Infarction

Cerebral atherosclerosis

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182
Q

What proportion of strokes are infarctive?

A

70-80%

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183
Q

Where do thrombosis occur in the CNS?

A

Atherosclerosis affects the larger extracerebral vessels worse, often near the carotid bifurcation or in the basilar artery

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184
Q

Which part of the CNS do emboli affect?

A

Intracerebral atery, usually from the heart or atherosclerotic plaques

Usually occurs in the MCA branches

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185
Q

What is a watershed infarction?

A

Occurs on border zone, hypoperfusion of the most distal edges of the blood supply. Not necessarily occlusive.

ACA and MCA most at risk

Site: distally is affected more than proximal

Dependant on the anastamoses around the zone

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186
Q

Cortical border zone

A

Between ACA and MCA

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187
Q

Internal border zone?

A

Between LCA and MCA

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188
Q

Cortical border zone

A

Between MCA and PCA

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189
Q

Def: TIA

A

Under 25hrs, self-limiting vascular obstruction, emboli and or platelet-fibrin aggregates

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190
Q

Px of TIA

A

1/3rd will get a significant infarct within 5 years

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191
Q

Def: intraparenchymal haemorrhage

Presentation

Site

A

Haemorrhage into the brain substance, usually rupture of one of the small intraparenchymal vessels

Raised ICP, rapid LOC

Basal ganglia, abrupt onset

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192
Q

Cause of ICH

A

50% HTN

Weakening of the walls, accelerated atheroscleroris in LV, hyaline atherosclerosis in smaller vesseles.

Clotting disorders, neoplasms, amyloid, vasculitis and vascular malformations also contribute

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193
Q

SAH most common cause

Most cmmon sites

A

Rupture of Berry aneurysm (present in 1% of the population)

80% ICA bifurcation, 20% vertebrobasilar circulation

30% of patients they are multiple

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194
Q

Berry aneurysm

>6-10mm

>25mm

A

Greatest risk of rupture

Mass lesion

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195
Q

Presentation and Px of SAH

A

Sudden onset, severe headache, vomiting, LOC

50% die within a few days, worse Px if there were warning bleeds

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196
Q

Types of vascular malformations

Significance

A

AVM

Capillary telangectasias

Venous Angiomas

Cavernous angiomas

Important cause of ICH and developmental abnormalities

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197
Q

EDH features

A

Meningeal artery rupture which lies between the dura mater. Associated with skull fractures

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198
Q

Presentation of EDH

A

Lucid interval followed by progressive LOC. Bleeding is arterial and there is subsequent mass effect

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199
Q

SDH features

A

Between the dura and the arachnoid mater.

Disruption of the bridging veins.

Associated with rapid change of head velocity which leads to tearing of the veins.

Usually clear Hx of tumour caused by raised ICP. Venous bleeding therefore slower development than EDH.

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200
Q

Presentation of SDH

A

There is a less good history of trauma and it is associated with brain atrophy. Often causes vague alteration in mental state rather than classical features of raised ICP

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201
Q

Features of Parenchymal injury

A

Occurs due to sudden acceleration or deceleration with sufficient force to tear nerve cell process in the cerebral white matter

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202
Q

Presentation of parenchymal injury

Sequelae?

A

Concussion, transient LOC and neurological deficit. Sometimes with seizrues with recovery over hours or days.

Diffuse axonal injury: causes most post-traumatic dementia and with hypoxic ischaemic injury is the leading cause of persistent vegetative state

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203
Q

Coup vs contracoup

A

Contustion at site vs away from site of impact

Need to know the MOI

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204
Q

What is Duret’s Haemorrhage

A

Bleeding in the ventral and paramedian part of the upper brainstaim (pons and midbrain)

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205
Q

Features of cerebral oedema

A

Gyral flat and te sulci are obliterated

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206
Q

Causes of brain oedema

A

Anything that can damage the BBB

Can be classified as

Vasiogenic: where the integrity of the BBB is disrupted which is aggravated by the lack of lymphatic CNS drainage

Cytotoxic: secondary to cellular injury e.g. due to general hypoxic ischaemic injury.

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207
Q

Features of herniation

A

Due to raised ICp, can force the brain against unyielding bony wall

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208
Q

Subfalcine herniation

A

Cingulated gyrus displaced under falx cerebri

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209
Q

Transtentorial hernia

A

Uncal gyral, medial temporal lobe compressed against the free margin of the tentorium cerebelli

Results in compression of the PCA and oculomotor nerve

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210
Q

Tonsilar herniation

A

Cerebellar tonsils herniate through the foramen magnum causing brainstem compression

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211
Q

Stroke

Symptoms

Vascular territories

Ix

Mx

A

Sudden onset, FAST, numbness, loss of vision, dysphagia

MCA most common

CT/MRI (infarct vs haemorrhage)

Ix for vascular risk: BP, FBC, ESR, U&E, glucose, lipids, CXR, ECG, carotid doppler

Aspirin +/- dipyridamole

Thrombolytics (<3h)

+/’- caroit dendarterectomy

LT: treat HTN, reduce lipids, anticoagulate

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212
Q

TIA

Symptoms

Vascular territories

Ix

Mx

A

<24hrs, amaurosis fugax, carotid bruit

Any however characteristically embolic atherogenic debris from the carotid artery travels to the opthalmic branch of the internal caroitd

Cartoid USS

IX for vascular risk as in stroke

As for stroke except no thrombolysis

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213
Q

What proportion of SAH are from ruptured Berry Aneurysm

What conditions increase risk of Berry Aneurysm

A

85%

PKD, Ehler’s Danlos and patients with coarctation

Also associated with AVMs, capillary telangectasias, venous and cavrnous angiomas

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214
Q

Haemorrhage brain injury classification

A

Non traumatic:

ICH

SAH

Traumatic:

EDH

SDH

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215
Q

6 types of brain herniation

A

Uncal

Central (transtentorial)

Cingulate (subfalcine)

Transcalvarial

Upward

Tonsilar

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216
Q
A

Cerebral oedema

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217
Q

What are the two types of hydrocephalus?

A

Communicating/non-obstructive

Non-communicating/obstructive

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218
Q

Causes of non-obstructive hydrocephalus

A

Impaired reabsorption of CSF:

Normal pressure hydrocephalus

Hydrocephalus ex-vacuo

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219
Q

Features of normal pressure hydrocephalus

A

Associated with elevated CSF causing increased ICP and increased ventricular size

Thought to be scondary to an increase in fluid levels.

Infection, tumours, trauma and haemorrhage

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220
Q

Features of hydrocephalus ex-vacuo

A

Atrophy causing secondary enlargement of the ventricle and subarachnoid space

Not the result of increased CSF production pressure but a compensatory enlargement of ventricle due to loss of brain parenchyma

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221
Q

Causes of obstructive hydrocephalus

A

Actual obstruction to CSF flow

Adhesion, external compression, interventricular cysts and tumours

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222
Q

Def of stroke

This excludes

A

A stroke is a clinical syndrome characterised by rapidly developing clinical symptoms and / or signs of focal, and at times global loss of cerebral function, with symptoms lasting more than 24 hours or leading to death, with no apparent cause other than that of vascular origin (Hatano, 1976).

This definition includes stroke due to cerebral infarction, primary intracerebral haemorrhage, intraventricular haemorrhage, and most cases of subarachnoid haemorrhage

It excludes subdural haemorrhage, epidural haemorrhage, or intracerebral haemorrhage (ICH) or infarction caused by infection or tumour.

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223
Q

Two types of cerebral ischaemia

A

Focal: defined bascular territory

Global: failure of systemic circulation

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224
Q

NB re atypical brain infarct

A

Atypical refers to odd anatomy – not classical vascular territory

Clinical features and neuroimaging can be misleading.

“Atypical brain infarct may mimic tumors”

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225
Q

Mx of AVM

A

Surgery, embolisation, Radiosurgery

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226
Q

Features of AVM

A
  • Occur anywhere in the CNS
  • Becomes symptomatic between 2nd and 5th decade (mean age 31.2 years)
  • Present with haemorrhage, seizures, headache, focal neurological deficits
  • High pressure – MASSIVE BLEEDING!!!
  • Seen at angiography
  • Risk of bleeding 1.3-3.9% yearly
  • Risk of re-bleeding 6.0-6.9% during the first year
  • Morbidity after rupture 53-81% - high in eloquent areas
  • Mortality 10-17.6%
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227
Q

Features of cavernous angioma

A

“Well-defined malformative lesion composed of closely packed vessels with no parenchyma interposed between vascular spaces”

Anywhere in the CNS

Usually symptomatic after age 50

Pathogenesis unknown

Rarely multiple – familial (linkage chrom. 7p, 7q, 3q)

Present with headache, seizures, focal deficits, haemorrhage

A few feeding vessels

Low pressure – RECURRENT BLEEDINGS

Angiographically usually negative

Therapy: surgery

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228
Q

Histopathological differentiation between infarct and haemoorhage

A

INFARCT

  1. Tissue necrosis (stains)
  2. Rarely haemorrhagic
  3. Permanent damage in the affected area (except for penumbra)
  4. No recovery

HAEMORRHAGE

  1. Bleeding
  2. Dissection of parenchyma
  3. Less macrophages
  4. Limited tissue damage (periphery)
  5. Partial recovery
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229
Q

Lesions in fatal non-missile head injury

A

Primary

Skull fracture 75%

Surface contusions 95%

Diffuse axonal injury (DAI) 30%

Intracranial haematoma 60%

Secondary

Brain swelling 53%

Ischaemic brain damage 55%

Infection 4%

Due to raised ICP 75%

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230
Q

Intracranial haematoma

A

66% of fatal non-missile head injury

10% extradural

56% subdural, subarachnoid, intracerebral or burst lobe

surgical evacuation

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231
Q
A
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232
Q
A

SDH

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233
Q
A

EDH

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234
Q
A
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235
Q

What are connectomes?

A

Functional conective areas in the brain

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236
Q

What are the central neurotransmitters

A

GABA: inhibitory

Glutamate: excitatory

Ach, dopamine, serotonin

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237
Q

What are microglial cells?

A

Resident macrophage population constituting 20% CNS cells. Have motile processes. Involved in immune surveillance and tissue reomdelling (synaptic stripping)

Cytotoxic in neurodegeneration and neuroinflammation

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238
Q

Def: neurodegenerative disease

A

Progressive, irreversible condition leading to neuronal loss

Often caused by intra or extracellular accumulation of a misfolded protein. Usually sporadic resulting in dementia.

Common in the ageing population

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239
Q

Def: neuroinflammatory disease

A

Conditions characterised by an innate and/or adaptive inflammatory response. Can be reveersible, progressive irreversible

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240
Q

Def: dementia

A

A serious loss of global cognitive ability in a previously unimpaired person beyond what might be expected from normal ageing. Can be a static event e.g. from TBI or due to neurodegeneration.

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241
Q

Dx of dementia

A

Difficult to diagnose on symptoms alone, some have a distinct clinical phenotype which gives a high probability of a dx e.g. PD.

Based on neuroimaging, brain biopsy or PM examniation

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242
Q

Exmaples of misfolded proteins implicated in dementias?

A

Tau, beta-amyloid, alpha-synuclein, Huntingtin, PrP, Fus

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243
Q

Features of AD

A

Dense deposits around neurones (neuritic plaques)

Twisted bands of fibre (NFTs)

Begins after 5th-6th decade, pathogenesis unclear.

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244
Q

Pathological findings in AD

A

Senile plaques: complex spherical structures involving the grey matter.

Diffuse plaques are amorphous and seen in ageing brains.

Neuritic plaques consist of clusters of radially orientated abnormal axons and dendrites= dystrophic dendrites

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245
Q

Features of NFTs

A

Ubiquitin abnormally accumulates inside cells, indicates a disease process. These protein accumulations= inclusion bodies

NFTs are aggregates of hyperphosphorylated Tau proteins.Intracellular structures composed of two filaments wound in a double helix

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246
Q

What conditions features Tau?

A

AD, progressive SNP, some other uncommon conditions

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247
Q

Features of Tau

A

Normal component of the neuronal cytoskeleton, stabilises MTs. 6 isoforms.

Function is regulated by kinases.

Unphosphorylated or hyperphosphorylated Tau fails to bind to MTs, accumulating in NFTs and neuritic plaques

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248
Q
A

Senile plaque

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249
Q
A

NFT

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250
Q

Risk factors for AD

A

Age

FHx

T21

Head trauma

PD and depression

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251
Q

Dx of AD

A

Clinical suspicion

MRI and PET scans helpful

Role of biopsy unclear

Dx and PM is definitive

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252
Q

Features of Beta amyloid

A

Derived from APP which is a ubiquitously expressed membrane molecule.

Cleaved by secretases which are regulated by Presenilin 1

Amyloid accumulation results from defective APP cleavage

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253
Q
A

Beta amyloid

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254
Q

What fragment of amyloid accumulates in AD?

A

Fragment 39-42

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255
Q

What is the role of ApoE in AD?

A

Produced by astrocytes and mediates phospholipid and cholesterol mobilisation

ApoE4 has a high affinity to beta amyloid and less activity.

ApoE4 is a risk factor for late onset sporadic AD

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256
Q

Rx in AD

Classes, e.g.

A

Acetylcholinesterases: Tacrine (Cognex(), Donepezil, Rivastigmine. Produce mild benefits without influencing progression

nAChRs: galantamine

Glutamate antagonist: memantine (also used in vascular dementia)

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257
Q

DDx in Parkinson

A

Parkinsonian syndromes:

Primary: PD

Secondary

Parkinson-plus syndrome

Familial Neurodegenerative diseases

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258
Q

Primary parkinsonism=

A

PD (sporadic, familial)

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259
Q

Causes of secondary parkinsonism

A

Drug induced: dopamine antags and depletor

Hemiatryophy-hemiparkinsonism

Normal pressure hydrocephalus

Hypoxia

Infectious: postencephalitic

Toxin: e.g. MPTP

Trauma

Tumour

Vascular: multiinfarct state

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260
Q

Causes of parkinson plus syndromes

A

Cortical-basal ganglionic degeneration

Dementia sydnromes: AD, LBD, frontotemporal dementia

Lytico-Bodig (ALS)

Multiple system atrophy syndromes: Shy Drager, OPCA, MND parkinsonism

Progressive pallidal atrophy

PSN Palsy

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261
Q

Familial Neurodegenerative diseases causing parkinsonism

A

Hallervoden-Spatz

Huntingoton

Lubag

Mitochondrial cytopathies with Striatal Necrosis

Neuroacanthocytosis.

WIlsons

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262
Q

Features of PD

A

Usually sporadic, develops after 50y

Pathogenesis unclear

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263
Q

Symptoms of PD

A

Attributed to cell death of the dopamine producing neurones in the SN
Need to lose 80-85% of the dopaminergic neurones and deplete dopamine levels by 70% before they appear.

Cell death in substantita nigra results in depigmentation

Lewy Bodies found in cell bodies and Lewy neurites in neuronal projections

Alpha-synuclein and ubiquitin positive

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264
Q

Clinical features of PD

A

Resting tremor, rigidity, bradykinesia, autonomic dysfunction, dysphagia

Psychiatric: hallucinations, anxiety and dementia

L-DOPA responsiveness

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265
Q

What is alpha-synuclein

A

Small protin associated with synaptic membrane, accumulates and has toxic effect.

Mutations reported in instances of familial parkinson’s

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266
Q
A

Lewy Bodies

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267
Q

Causes of demyelinating lesions

A

Viral infections: PML (progressive multifocal leukoencephalopathy caused by JC virus in immune deficiencies

Genetic: leukodystrophies

Autoimmune: MS, acute haemorrhagic encephalomyeltisi, acute disseminated encephalomyelitis

Nutritional: central pontine myleniosis

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268
Q

Function of myelin

A

Produced by oligodendrocytes, fundamental for axon conduction, highly susceptible to damage.

Lipid rich insulating membrane

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269
Q

Features of MS

A

Peak age: 20-40y

Assocaited with some HLAs

Usually presents with focal symptoms, optic neuritis and poor coordination

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270
Q

What are the types of MS

A

Relapsing remitting: recovery less severe, evolves into secondary progressive agter years

Primary progressive (10%): no recovery after episodes of demyelination: severe

Progressive relapsing

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271
Q

What is neuromyeltitis optica

A

Rare variant of MS

Aka Devic disease

Autoimmune disease against the SC and optic nerves with auqaporin 4 attacked

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272
Q

What is Balo disease?

A

Rare variant of MS

Rapidly progressive with concentric scoliosis

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273
Q

Pathology of MS

A

Loss of myelin, lesions centred by veins with sharpy margins

Axonal preservation in early plaques

Active plaques- glial scar

Remyleinating shadow plaques

Infiltration of macrohpages.

Cortical involvement is the leading cause of disability, CI leading to dementia, related to inflamm rather than demyelination

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274
Q

Patholocial protein in:

AD

A

Tau, beta-amyloid

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275
Q

Patholocial protein in:

LBD

A

Alpha synuclein, ubiquitin

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276
Q

Patholocial protein in:

Corticobasal degeneration

A

Tau

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277
Q

Patholocial protein in:

Frontotemporal dementia

A

Tau

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278
Q

Patholocial protein in:

Pick’s disease

A

Tau

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279
Q

Imaging findings in AD

A

Generalised atrophy of the brain

Widened sulci

Narrowed Gyri

Enlarged ventricles (most marked in the temporal and frontal lobes with loss of cholinergic neurones)

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280
Q

Features of LBD

A

Psychological disturbances occur early, day to fluctuations in cogntiive performance

Vsiual hallucinations

Spontaneous motor signs of Parkinsonism

Recurrent falls and syncope

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281
Q

Myelin basic protein and proteo-lipid protein=

A

MS

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282
Q

Features of multiple system atrophy

Types

A

Degenerative neurological disorder characterised by features that can present in a similar manner to Parkinson’s but show a poort response to parkinson’s medication

Shy Drager

Stratonigral

Olivopontocerebellar

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283
Q

Shy Drager

A

MSA: autonomic dysfunction

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284
Q

Striatonigral MSA

A

Difficulty with movement

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285
Q

Olivopontocerebellar MSA

A

Difficulty with balance and coordination

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286
Q

Features of vCJD

A

vSporadic neuropsychiatric disorder

vPatients <45 yrs old

vCerebellar ataxia

vDementia

vLonger duration than CJD

vLinked to BSE

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287
Q

Neuropathology of AD

A
  • Extracellular plaques
  • Neurofibrillary tangles
  • Cerebral amyloid angiopathy (CAA)
  • Neuronal loss (cerebral atrophy)
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288
Q
A

Cortical atrophy- AD

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289
Q

Diagnotics gold standard in PD

A

alpha-synuclein immunostaining

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290
Q

Functions of the kidney

A

Excretion of metabolic waste products and foreing chemicals

Regulation of fluid and electrolyte balance, acid base balance

Hormone secretion: EPO, Renin and 1,25 cholecaliferol

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291
Q

What are the two types of glomerulaur disease

A

Failure to:

Filter an adequate amount of blood resulting in a lack of waste product excretion

Failure to maintain a barrier function leading to the loss of protein and/or blood cells in the urine

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292
Q

What must be distinguished in renal disease

A

Syndrome: e.g. ARF, nephrotic etc.

Morphological changes: glomerulonephritis, thrombotic microangiopathy

Aetiologies: SLE, amyloidosis, drugs and infetions

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293
Q

What are immune complexes in the context of renal disease?

Antigens

Rate

Stie

A

Composed of a lattice work of Ab and Ag, may become deposited in te glomerulus and lead to an inflammatory response-> complement activation and stimulation of inflammatory cells through Fc receptors

May be endogenous e.g. SLE or exogenous e.g. derived from an infective organism

Rate: occur at different rates: rapidly progressive glomerulonephritis vs slow onset

Site may bary but glomerular injury is determined by the immune complex location

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294
Q

Congenital diseases of the kidney

A

Bilateral/unilateral agenesis

Ectopic e.g. pelvic

Horseshoe (usually fused at the lower pole)

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295
Q

Features of Adult PKD

A

Causes 10% of ESRF

Cysts arise from all portions of the nephron

Renal failure develops from 40-70y

Genes: PKD1 and PKD2

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296
Q

Features of acquired cystic disease

A

Cysts develop in the kidneys of ESRF on dialysis

Carcinoma can develop in 7% in 10y

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297
Q

What is acute renal failure?

What does it result in?

Biochemically?

A

Rapid loss in golmerular filtration and tubular function

Results in an abnormal water and electrolyte balance

Reduced GFR manifestated as rasied serum creatinine (Normal= <1.3) and urea (10-20mg/dL)

May r3esult in acidosis, hyperkalaemia and fluid overload

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298
Q

How can disease of the kidney be classified?

e.g.?

A

According to the site of the nephron it affects

  1. Glomerulus

Nephrotic syndrome

Nephritic syndrome

  1. Tubules and interstitium

ATN

Tubulointerstitial nephritis: acute phyelonephritis, chronic pyelonephritis & refulx nephropathy, interstitial nephritis

  1. BVs:

Thombotic microangiopathies (HUS, TTP)

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299
Q

Features of nephrotic sydnrome

A

Proteinuria (>3g/24h)

Hypoalbuminaemia

Oedema

(+hyperlipidaemia)

“Swelling” facial in children, peripheral in adults

“frothy urine”

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300
Q

What are three primary causes of nephrotic syndrome

A

Minimal change disease

Membranous glomerular disease

Gocal segmental glomerluosclerosis

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301
Q

Features of minimal change disease

Light microscopy

Electron microscopy

Immunofluorescnece

Px

Response to steroids

A

Primary cause of nephrotic syndrome

Most common in children (75%) with second peak in elderly

No changes

Loss of podocyte foot processes

No immune deposits

<5% ESRF

90% respond

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302
Q

Features of membranous glomerular disease

Light microscopy

Electron microscopy

Immunofluorescnece

Px

Response to steroids

A

Primary cause of nephrotic syndrome

Common in adults (30%)

Diffuse glomerular BM thickening

Loss of podocyte foot processes. Subepithelial deposits spikey

Ig and complements in granular deposits along entire GBM

40% ESRF at 2-20y

Can be 1o or 2o to SLE, infections, drugs and malignancy

Poor response to steroids

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303
Q

Features of Focal Segmental Glomerulosclerosis

Light microscopy

Electron microscopy

Immunofluorescnece

Px

Response to steroids

A

Common in adults (30%), Afrocarribean

Focal and segmental glomerular consolidation and scarring. Hyalinosis

Loss of podocyte foot processes

Ig and complement in scarred areas

50% ESRF in 10y

1o but can 2o to obesity and HIV nephropathy

50% respond

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304
Q

What are 2 secondary causes of nephrotic syndrome

A

DM

Amyloidosis

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305
Q

Histology of DM nephrotic syndrome

Hints in question

A

Diffuse GBM thickening

Messangial matric nodules aka Kimmelstiel Wilson nodules

Asian

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306
Q

Histology of amyloidosis nephrotic syndreom

Hints

A

Apple green birefringence with congo Red

May have chronic inflammation:

RA, chronic infection.

Causes AA protien depositions

May have Ig light chain deposition most commonly from MM

Clinical clues of amyloidosis: Macroglossia, HF, hepatomegaly

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307
Q

Features of nephritic syndrome

A

Manifestation of glomerular inflammation i.e. glomerulonephritis

Haematuria

Dysmorphic RBCs and RBC casts in urine

May also have:

Oliguria

Raised urea and creatinine

HTN

Proteinuria (not in nephrotic range)

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308
Q

Causes of nephritic syndrome

A

Acute postinfectious

IgA Nephropathy (Berger Disease)

Rapidly progressive (crescenteric) GN

Hereditary nephritis (Alport’s)

Thin BM Disease (Bening familal haematuria)

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309
Q

What are the features of Acute Postinfectious GN

A

Occurs 1-3w post streptococcal throat infection or impetigo (usually Group A S= Strep pyogenes)

Glomerular damage due to immune complex deposition

Haematuria (red cell casts), proteinuria, oedema, HTN

Bloods: raised ASOT titre, decreased C3

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310
Q

Light microscopy: Increased cellularity of glomeruli

FM: granular deposits of Ig Ga and C3 in GMB

EM: subendothelial humps

A

Findings in Acute Postinfectous GN

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311
Q

Features of Berger disease

A

IgA Nephropathy

Most common GN worldwide

IgA complex deposition in glomeruli

Presents 1-2d after URTI with Frank haematuria

Main symptoms are persistent or recurrent frank haematuria or asymptomatic macroscopic haematuria

Can rapidly prgoress to ESRF

Biopsy: granular deposition of IgA and complement in mesangium

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312
Q

GN 1-2d after URTI with haematuria

A

IgA Nephropathy (Berger)

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313
Q

Nephropathy 1-3w post streptotoccal infection

A

Acute Postinfectious GN

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314
Q

Feature of Rapidly progresswive (Crescenteric GN)

A

Most aggressive GN causing ESRF within weeks

Presents as nephritic syndrome but oliguria and renal failure are more pronounced

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315
Q

How is cresenteric GN calssified?

A

Based on immunological findings:

Type 1: Anti-GBM Ab

Type 2: Immune complex

Type-3: pauci-immune/ANCA associated

Regardless of cause all are characterised by crescenets in glomeruli on light micrscopy

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316
Q

Features of T1 crescenteric GN

Cause

Light micrscopy

Fluorescnece microscopy

Additional organ involvement

A

Anti-GBM Ab

Goodpasture’s. HLA-DRB1 association

Crescents

Linear deposition of IgG in GBM

Lungs: pulmonary haemorrhage

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317
Q

Features of T2 crescenteric GN

Cause

Light micrscopy

Fluorescnece microscopy

Additional organ involvement

A

Immune complex mediated

SLE, IgA nephropathy, postinfectious GN

Crescents

Granular IgG immune complex deposition on GBM/mesangium

Often limited, except in SLE

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318
Q

Features of T3 crescenteric GN

Cause

Light micrscopy

Fluorescnece microscopy

Additional organ involvement

A

Pauci-immune i.e. lacking anti-GBM or immune complex

c-ANCA: Wenger’s granulomatosis

p-ANCA: microscopic polyangitis

Cresecents

Lack/scanty immune complex deposition

Vasculitis: skin rashes or pulmonary haemorrhage

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319
Q

Features of Alport’s

A

Hereditary Nephritis

Caused by mutation in Type IV collagen alpha 5 chain

X-linked

Nephritic syndrome + sensorineural deafness and eye disorders

Presents at 5-20y with nephritic syndrome progressing to ESRF

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320
Q

Features of Benign Familial Haematuria

A

Thin BM disease nephirits

Rarely causes nephritic syndrome, normally exclusively asymptomatic haematuria

Diffuse thinning of the GBM caused by autosomal dominant mutation in type 4 collagen alpha 4 chain.

Usually asymptomatic and diagnosed incidentally

Usually normal renal function

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321
Q

Differentials for asymptoamtic haematuria

A

Benign Familal Haematuria

Berger

Alport

NB: IgA and Thin BM are more common causes of asymptomatic haematuria than of nephritic syndrome. IgA more likely to cause frank haematuria and change in renal funciton and slighlty more common in Asian pop

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322
Q

Features of ATN

Histologically

A

Damage to tubular epithelial cells-> blockage of tubules by casts-> reduced flow and haemodynamic changes-> acute renal failure

Necrosis of short segments of tubules

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323
Q

What is the most common cause of acute renal failure?

A

ATN

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324
Q

What are the common causes of ATN?

A

Ischaemia: burns, septicaemia

Nephrotoxins: drugs (gentamicin, NSAIDs, radiographic contrast agents, mygolobin, heavy metals)

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325
Q

What is tubulointerstitial nephritis?

Causes?

A

A group of renal inflammatory disorders involving the tubules and interstitium

Acute pyelonephritis

Chronic pyelonephritis and reflux nephropathy

Acute interstitial nephritis

Chronic interstitial nephritis/analgesic nephropathy

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326
Q

What is acute pyelonephritis?

A

Bacterial infection of the kidney, usually as a result of ascending infection, most commonly by E. Coli

Presents with fever, chills, sweats, flank pain, renal angle tenderness and leukocytosis +/- frequency, dysuria, haematuria

Leukocytic casts seen in the urine

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327
Q

What is chronic pyelonephritis and reflux nephropathy?

A

Chronic inflammation and scarring of the renal parenchyma caused by recurrent and persistent bacterial infection

Can be due to:

Chronic obstruciton: posterior urethral valves, renal calculi

Urine reflux

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328
Q

What is acute interstitital nephritis

A

A hypersensitivity reaction usually to a drug (NSAID, Abx, diuretics)

Usually begins days after drug exposure

Presents with fever, skin rash, haematuria, proteinuria, eosinophilia

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329
Q

What is chornic interstitial nephirits?

A

Seen in elderly with long term analgesic consumption e.g. NSAIDs, paracetamol

Symptoms occur late in disease: HTN, anaemia, proteinuria and haematuria

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330
Q

What are the thrombotic microangiopathies?

A

HUS and TTP

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331
Q

What characterises the thrombotic microangiopathies?

A

Thrombosis generally renal in HUS and widespread in TTP

Triad of:

Microangiopathic haemolytic anaemia

Thrombocytopenia

Sometimes renal failure (HUS)

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332
Q

Pathogenesis of the thrombotic microangiopathies

A

Widespread fibrin deposition in vessels forming platelet fbirin thrombi, damages passing platelets and RBCs

Leads to platetel and RBC destruction

Resulting in thrombocytopenia and MAHA

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333
Q

HUS

Epidemiology

Pathophysiology

Sign/symptoms

Renal involvement

Dx

A

Usually affects children

Associated with dairrhoea caused by E. Coli 0157:H7

Can be non-diarrhoea associated due to abnromal protines in the complement pathway

Thrombi confined to kidney

Decreased platelet count-> bleeding, haematemesis, melena

MAHA-> pallor and jaundice

Usually involves renal failure

Dx:

Anaemia, thrombocytopaenia

Signs of haemolyiss: raised bilirubin, LDH and reticulocytes

Fragmented RBCs on blood smear

Coomb’s negative as not AIHA

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334
Q

TTP

Epidemiology

Pathophysiology

Sign/symptoms

Renal involvement

Dx

A

Usually affects adults

Thrombi occurs throughout circulation with CNS involvement particularlr

Reduced platelets-> bleeding

MAHA-> pallor and jaundice

Usually no renal involvement, neuro symptoms predominate (headache, altered consciousness, seizures, coma)

Dx:

As for HUS

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335
Q

What is ARF?

A

A rapid loss of renal funciton manifesting as increased serum creatinine and urea

Complications include metabolic acidosis hyperkalaemia, fluid overload, HTN, hypocalcaemia, uraemia

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336
Q

Causes of acute renal failure

A

Pre-renal (Most cmmon): renal hypoperfusion

e.g. hypovolaemia, sepsis, burns, acute pancreatitis and RAS

Renal:

ATN commonest renal cause of ARF

Acute GN

Thrombotic microangiopathy

Post-renal: obstruction to urine flow

Stones, tumour, prostatic hypertrophy and retroperitoneal fibrosis

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337
Q

Def: Chronic renal failure

A

Progressive irreversible loss of renal function characterised by prolonged symptoms and signs of uraemia

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338
Q

What are the signs of uraemia?

A

Fatigue, itching, anorexia, eventually confusion

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339
Q

What are the most common causes of CRF in the UK?

A

DM (20%)

GN (15%)

HTN and vascular disease (15%)

Reflux nephropathy (10%)

PKD (10%)

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340
Q

With what is HUS associated

A

E Coli O157: H1

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341
Q

How is CRF classified?

A

5 stages based on GFR:

Stage 1: kidney damage with normal renal function (proteinuria)

>90

Stage 2: mildly impaired

60-89

Stage 3: moderately impaired

30-59

Stage 4: severely impaired

15-29

Stage 5: renal failure (RRT required)

<15 or if being treated with RRT

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342
Q

Features of Adult PKD

A

Autosomal dominant inheritance, 85% due to mutations in PKD1 remained in PKD2, both encode polycystin

Pathological features: large multicystic kidneys with destroyed renal parenchyma, liver cysts (PKD1) and Berry aneurysms.

Clinical features: haematuria, flank pain, UTI. Clinical features are often due to cysts cxs e.g. rupture, infection, haemorrhage

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343
Q

Features of lupus nephritis

A

Depends on site and intensity of immune complex deposition

Presentation may be with

Acture renal failure

Urinary abnromalities

Nephrootic syndrome

or progressive CRF

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344
Q

What is Class 1 Lupus Nephritis

A

Minimal mesangial lupus nephirits

Immune complexes with no structural alteration

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345
Q

What is Class 2 LN

A

Mesangial proliferative LN: immune complexes and mild/moderate increase in mesangial matrix and cellularity

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346
Q

What is Class 3 LN?

A

Focal lupus nephritis: active swelling and proliferation in less than half the glomeruli

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347
Q

What is Class 4 LN?

A

Diffuse LN involving more than half of the glomeruli

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348
Q

What is Class V LN?

A

Membarnous LN with subiepithalil complex deposition?

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349
Q

What is Class VI LN?

A

Advanced sclerosing: complete sclerosis of >90% of the glomeruli

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350
Q

How do NSAIDs predispose to acute tubular injury?

A

Through inhibition of prostaglandins

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351
Q

Struture of the normal oesophagus

A

Proximal: squamous epithelium

Distal: columnar (about 1.5-2cm below the diaphragm)

LOS: 2-cm segment proximal to the OGJ

OGJ: point where tubular oesophagus joins saccular stomach

Squamo-columnar junction/ Z line: irregular serrated margin usually at +/- 40cm from incisors but may lie anywhere within distal 2cm

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352
Q

Where is teh squamocolumnar junction?

A

+/- 40cm from the incisors in distal 2cm of oesoophagus

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353
Q

What is the most common cause of oesophagitis?

What is this?

What is the pathology?

A

GORD

Reflux of acidic gastric contents into the oesophagus

Ulceration, necrotic slough, inflammatory exudates, granulation tissue, fibrosis

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354
Q

What are the Cxs of GORD?

A

Haemorrhage, perforation, stricture, Barret’s oesophagus

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355
Q

How is GORD classified?

What does this consitute?

A

Los Angeles Classificaiton

Grade A: mucosal breaks confined to the mucosal fold, each no longer than 5mm

Grade B: at least one mucosal break longer than 5mm coninfed to the mucosal fold but not continuous between two folds

Grade C: mucosal breaks that are continuous between the tops of the mucosal folds but not circumferential

Grade D: extensive mucosal breaks engaging at least 75% of the oesophageal circumference

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356
Q

What is Barret’s oesophagus?

A

Re-epithelialisation by metaplastic olumner epithelium with goblet cells

Barret’s: columnar lined oesophagus

Goblet cell/intestinal metaplasia

Precancerous form: metaplastic galndular epithelium-> dysplasia-> Adenocarcinoma

Surveillance: repeat endoscopy and biopsy to detect early neoplastic changes

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357
Q

Adenocarcinoma of the oesophagus

A

30-40% of primary oesophageal carcinoma

Associated with Barrett’s

Other Cas: squamous or combined form

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358
Q

Features of squamous cell carcinoma of the oesophagus

A

Dysphagia, anorexia, weight loss

90% in the mid and lower oesophagus

Invasion into the muscularis propria

30-40% have invasion of the mediastinum, 50% have LN mets

Cytology + biopsy

Can spread directly, by LNs or liver mets

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359
Q

Features of oesophageal varices

A

Extremely dilated submucosal veins in the lower third othe oesophagus.

A consequence of portal HTN commonly due to cirrhosis

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360
Q

Featuires of the normal stomach

A

Made of the cardia, body and antrum

Lined by gastric mucosa: columnar epithelium (mucin secreting) and glands, then lamina propria and muscularis mucosa

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361
Q

Features of acute gastritis

A

Acute insult

Neutrophil infiltration

Caused by chemicals e.g. Aspirin/NSAIDs, ETOH or corrosives or infection (H. Pylori)

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362
Q

Features of chronic gastritis

A

Persistent insult

H. pylori associated

Lymphocytic infiltration

May also by neutrophils and MALT induction. Also associated with chemical, autoimmune and others

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363
Q

Features of chemical gastritis

A

Reactive or reflux, caused by NSAIDs.

Pattern: foveolar hyperplasia (mucus producing cells), smp and sparse chronic inflammation +/- neutrophils

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364
Q

What differentiates between actue/chronic gastritis

A

Acute: neutrophil infiltration

Chronic: lymphocyte

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365
Q

Features of helicobacter associated gastritis

A

H. pylori or Heilmanni

Chronic gastritis +/- activity, severity varias

Outcome variable:

Persistence

Intestinal metaplasia

Dysplasia

Ca and lymphoma

Eradication willl reduce the risk of Ca but will not eliminate cancer due to [redetermined pathways

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366
Q

Causes of pernicious anaemia

A

Parietal cell Abs (90%)

IF Abs (60%)

Chronic gastritis and body atrophy

Outcome variable: Vit B12, atrophy and Ca

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367
Q

What are some other causes of gastritis

A

Infection: CMV, Herpes, Strongyloides (immunosuppresion)

IBD: Crohn’s

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368
Q

Why should we treat gastritis?

A

Chronic gastritis/ulcer

Intestinal metaplasia

Dysplasia

Cancer

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369
Q

Why should alll ulcers be biopsied?

Cxs?

A

To exclude malignancy

Bleeding (anaemia), perforation, peritonitis

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370
Q

What is intestinal metaplasia a response to?

What is a consideration?

A

IM in gastric mucosa is a response to LT damage e.g. H. Pylori and Bile

There is a Ca risk

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371
Q

What is gastric dysplasia?

A

An abnormal pattern of growth in which some of the histological features of malignancy are present but a non or pre-invasive stage

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372
Q

Features of Gastric cancer

A

High incidence in Japan, Chile, Italy, CHina, Portual and Russia

M>F

90% are carcinomas

Environmental factors: smoking, diet

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373
Q

Mx of duodenitis?

A

Do a gastric Bx to assess H. pylori status, the principal cause is acid in the presence of gastric metaplasia

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374
Q

Normal architecture of the duodenum?

A

Villus:crypt 2:1

No increase in lamina propria cellularity

No evdience of epithelial damage, no neutrophils

Brunner’s glands in 1st part

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375
Q

Pathogens in the duodenum?

A

Immunosuppressed

CMV: microsporidois, crytposporidiosis in immunosuppression

Giarda Lambila Infection

Whipples disease: Tropheryma Whippeli

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376
Q

Endoscopic findings for partial villous atrophy

A

Show scalloping with a smooth shiny mucosa (or normal)

Bx: early changes are hard to see on biopsy.

There is normal variation in villous height

May be crypt hyperplasia or intraepithelial lymphocytes

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377
Q

Cause of gastric lymphoma

Small intestine?

A

H. pylori

Coeliacs: 10% will get primary lymphoma (less often carcinoma of the gut) if not adeuqately treated.

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378
Q

Mx of GORD

A

lifestyle changes (smoking, weight loss), PPI/H2R antag

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379
Q

Prevalence of Barrett’s?

Pathogenesis

A

Seen in 10% of those with symptomatic GORD

Upwards migration of the SCJ

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380
Q

Where is oesophageaul adenocarcinoma seen?

A

Lower 1/3rd of oesophagus due to association with Barrett’s

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381
Q

Risk factors for SCC oesophageal carcinoma?

A

ETOH, smoking

Achalasia of cardia

Plummer-Vinson

Nutritional deficiencies

Nitrosamines

HPV

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382
Q

Presentation of SCC oesophagus

A

Progressive dysphagia, odynopgagia, anorexia, severe weight loss

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383
Q

Mx of varices

A

Emergency endoscopy-> sclerotherapy/banding

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384
Q

Def: gastric ulcer

A

Breach through muscularis mucosa into submucosa

Epigastric pain +/- weight loss

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385
Q

What differentiates between gastric ulcer vs dudodenal ulcer?

A

Gastric ulcer is WORSE with food

Duodenal ulcer is RELIEVED by food

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386
Q

Ix of gastric ulcer

A

Biopsy for H. pylori status: punched outm lesion with rolled margins

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387
Q

Rx for H. pylori

A

Triple therapy

PPI

Clarithromycin

Amoxicillin or metronidazole

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388
Q

Features of coeliac

A

T-cell mediated autoimmune disease: DQ2, DQ8 HLA status

Villous atrophy and malabsorption

Presents in young children and Irish women (EMQs)

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389
Q

Symptoms of coeliac

A

Malabsorption: steatorrhoea, abdo pain, N+V, weight loss, fatigue, IDA, failure to thrive, rash (dermatitis herpetiformis)

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390
Q

Serological tests for Coeliac

A

Anti-endomysial Ab (best sensitivity and specificity)

Anti-TTG

Anti-gliadin (poor marker of disease control)

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391
Q

Gold standard Ix in coeliac?

A

Upper GI endoscopy and duodenal biopsy

(villous atrophy, crypt hyperplasia, lymphocyte infiltrate

10% progress to duodenal T-cell lymphoma if not adequately treated

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392
Q
A

Stomach (body)

lined by gastric mucosa columnar epithelium (foveolar, mucin secreting)

specialised glands in the lamina propria

muscularis mucosa

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393
Q
A

Stomach (antrum)

lined by gastric mucosa columnar epithelium (fovelolar, mucin secreting)

Non-specialised glands in the lamina propria

(gastric pits)

mucularis mucosa

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394
Q
A

Duodenum

Glandular epithelium

with goblet cells

(intestinal type epithelium)

Villous architecture

villous:crypt ratio of >2:1

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395
Q

Differences between metaplasia, dysplasia, cancer

A

Metaplastic glandular epithelium

(intestinal type)

Dysplasia changes showing some of the cytological and histological features of malignancy but no invasion through the basement membrane

Adenocarcinoma invasion through the basement membrane

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396
Q

What are the two morphological classifications of gastric cancer?

A

Intestinal: well differentaited

Diffuse: poorly differentiated Linitis plastica, includes signet ring cell caricnoma

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397
Q
A

VIllous atrophy

Coeliac disease

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398
Q
A

Duodenal MALToma

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399
Q

Layers of the skin

A

Epidermis:

tratum corneum

Stratum lucidum

Stratum granulosum

Stratum spinosum

Stratum Basale

DEF

Dermis:

Paipllary Dermis

Reticular dermis

Subutis

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400
Q

What are these structures?

A
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401
Q

What is the organisation of the epidermis

A

Corneum

Lucidum

Granulosum

Basale

DEJ

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402
Q

What are the layers of the skin from superficial to deep?

A

Horny layer

Granular layer

Squamous cell layer

Basal layer

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403
Q

Epidermis cell activitiy from dep to superfifical

A

Basalae: mitosis, cells bound to BM by hemidesomosomes

Spinosum (prickle layer): cells linked by desmosomes

S granulosum: nuceli disintegrate

Corneum: non-nucleated, contains keratin

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404
Q
A
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405
Q

What does hyperkeratosis mean?

A

Increase in S. corneum/ kertain

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406
Q

What is parakeratosis?

A

Nuclei in S corneum

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407
Q

What is acanthosis?

A

Increases in spinosum

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408
Q

What is acantholysis?

A

Decreased cohesions between keratinocytes

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409
Q

What is spongiosis?

A

Intercellular oedema

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410
Q

What is legtiginious epidermis?

A

Linear pattern of melanocyte proliferation within epidermal basa cell layer

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411
Q

Features of dermatitis

A

All have same histpoapthology

Spongiosis of the epidermis, perivascular chronic inflammatory infiltrate in the dermis

Dilated dermal capillaries

Chronic: acanthosis, crusting/scaling

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412
Q

What are the different types of dermetitis

A

Atopic (eczma)

Contact

Seborrhoeic

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413
Q

Features of atopic dermatitis

A

Infants: face, scalp

Older: flexural areas

If chronic may lead to lichenification, persists into adulthood in those with FHx of atopy

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414
Q

Features of contact dermatitis

A

Type IV hypersensitivity e.g. to Nickel, rubber

Erythema, swelling, pruritus

Commonly affects ear lobes and neck (from jewellery), wrst (leather watch straps), feet (from shoes)

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415
Q

Features of seborrhoeic dermatitis

A

Inflammatory reaction to a yeast- Malassezia

Infants: cradle cap

Young adults: mild erythema, fine scaling, mildly pruritic affecting face, eyebrow, eyelid, anterior chest, external ear

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416
Q
A
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417
Q

Features of Lichen Planus

A

Lesions are pruritic, purple, polygonal, papules and plaques

Mother of pearl sheen and fine network on surface called Wickam’s striae

Usually on inner surfaces of wrists, can also affect oral mucous membranes.

Aetiology unknown

Hyperkeratosis with saw toothing of rete ridges and basal cell degeneration with chornic inflammatory infiltrate

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418
Q

Hyperkeratosis with saw toothing of rete ridges and basal cell degeneration with chornic inflammatory infiltrate

A

Lichen planus

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419
Q

Wickam’s striae seen in?

A

Lichen planus

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420
Q

Features of Psoriasis

A

2%

Commonest form is plaque is chronic plaque psoriasis with salmon pink plaques and a silver scale

Rubbing them causing pin-point bleeding

Koebner phenomenon: lesions form at sites of trauma

Cells have increased proliferation rate

Parakeratosis, loss of granular layer, clubbing of rete ridges giving test tubes in a rack appearance. Munro’s microabscesses

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421
Q

Parakeratosis, loss of granular layer, clubbing of rete ridges giving test tubes in a rack appearance. Munro’s microabscesses

A

Plaque psoriasis

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422
Q

What is Auspitz’s sign

A

Rubbing of psoriatic plaques causing pin-point bleeding

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423
Q

What is Koebner phenomenon

A

Psoriatic plaques forming at trauma sites

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424
Q

What are the types of psoriasis

A

Chronic plaque psoriasis

Flexural psoriasis: seen in later lift, usually groin, natal cleft and sub mammary areas

Guttate psoriasis: rain drop plaque distribution seen 2 week post Strep-throat

Erthrodermic/pustular psoriasis: severe widespread disease often systemic symptoms

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425
Q

With what is psoriasis associated?

A

Pitting

Onycholysis

Subungual hyperkeratosis

Arthritis

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426
Q
A
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427
Q
A
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428
Q

Features of pityrisasis Rosea

A

Salmon pink scaly eruption on the trunk extending outwards

Herald patch

May be assocaited with a virus

Pathology: non specific dermatitis

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429
Q

Features of erythema multiforme

A

Causes annular target lesions on hands and feet.

Pleomorphic lesions that can be a combination of macules, papules, urticarial weals, vesicles, bullae and petechiae

Subepidermal bulllae on histology

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430
Q

Causes of erythema multiforme

A

Infections e.g. HsV, mycoplasma or drug reactions e.g. penicllin, salicylates, anti-malarials

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431
Q

Spectrum of erythema multiforme

A

SJS, TEN

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432
Q

Bullous disesease

Def:

Site

A

Vesicles <0,5cm

Bullae >0.5cm

Can be sub-epidermal, intra-epidermal and subcorneal

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433
Q

What are the three types of bullous disease

A

Dermatitis herpetiformis

Pemphigoid

Pemphigus

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434
Q

Features of dermatitis herpetiformis

A

Itchy vesicles on extensor surfaces of elbows, buttocks.

Associated with coeliac

IgA Abs bind to the BM leading to subepidermal bulla

Microabscesses which coalesce to form subepidermal bullae. Neutrophil and IgA deposts at the tips of the dermal papillae

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435
Q

Microabscesses which coalesce to form subepidermal bullae. Neutrophil and IgA deposts at the tips of the dermal papillae

A

Dermatitis herpetiformis

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436
Q
A

Dermatitis herpetiformis

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437
Q

Features of pemphiogid

A

Large tense bullae on erythematous ase. Often on forearms, groin and axillae, seen in the elderly

Bullae do not rupture as early as pemphigus

IgG Abs bind to demidesomosomes of BM-> subepidermal bullae

PemphigoiD: Bullae are deep

Subepidermal bullae with eosinophils.

Linear deposition of IgG along BM

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438
Q

Subepidermal bullae with eosinophils.

Linear deposition of IgG along BM

A

Pemphiogid

Deep: subepidermal bulla

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439
Q

Features of Pemphigus

A

Bullae are easily rupturd and found on skin AND mucous membranes

IgG Abs bind to desmosomal protines-> intraepidermal bulla

PemphiguS- superficial

Intraepidermal bulla. Netlike pattern of IgG deposits

Acantholysis

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440
Q

Intraepidermal bulla. Netlike pattern of IgG deposits

Acantholysis

A

Pemphigus

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441
Q
A
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442
Q

Features of lupus rash

A

Chronic, discoid LE, buttterfly rash, alopecia

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443
Q
A
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444
Q

What are the types of cutaenous tumours

A

Benign:

Seborrhoeic keratosis

Premalignant:

Actinic Keratosis

Keratoachthoma

Bowen’s Disease

Malignant:

SCC

BSS

Melanocytic:

Melanocytic naevia

Lentigines

MM

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445
Q

Features of seborrhoeic keratitis

A

Rough, waxy plaques, stuck on appear in middle age/elderly

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446
Q

Features of Actinic keratosis

A

(Solar keratosis)

Rough, sandpiper like lesions on sun-exposed areas

SPAIN:

Solar elastosis

Parakeratosis

Atypia/dysplasia

Inflammation

Not full thickness

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447
Q

SPAIN:

Solar elastosis

Parakeratosis

Atypia/dysplasia

Inflammation

Not full thickness

A

Actinic keratosis

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448
Q

Features of keratoacanthoma

A

Rpaidly growing dome shaped nodule which may develop a necrotic crusted centre.

Grows over 2-3w and clears spontaenously

May be difficult to differentiate from SCC hsitologically

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449
Q

Features of Bowen’s disease

A

Intra-epidermal SCC in situ

Flat, red, scaly patches on sun-exposed areas

Full thickness atypia/dysplasia with BM intact

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450
Q

Full thickness atypia/dysplasia with BM intact

A

Bowen’s disease

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451
Q

Features of SCC

A

When Bowen’s has spread to involve dermis. Similar clinical features to Bowen’s but may ulcerate

Atypia/dysplasia throughout epdiermis, nuclear crowding and spreading through BM into dermis

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452
Q

Atypia/dysplasia throughout epdiermis, nuclear crowding and spreading through BM into dermis

A

SCC

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453
Q

Features of BCC

A

Aka: rodent ulcer

Slowly growing tumour, rarley metastatic but locally destructive

Pearly surfact often with telangectasia

Mass of basal cells pushing down into dermis. Nuclei align in outermost layer (pallisading)

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454
Q

Mass of basal cells pushing down into dermis. Nuclei align in outermost layer (pallisading)

A

BCC

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455
Q

Features of melanocytic naevi

A

Proliferation in the basal layer, nesting into dermis

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456
Q

What are lentigines?

A

Melanocyte proliferation limited to the epidermis

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457
Q

Features of MM

A

Atypical melanocytes which intiially grow horizontally in the epidermis (radial growth phase), then grow vertically into dermis (vertical growth phase). Vertical growth produces Buckshot appearance (=pagetoid cells)

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458
Q

What is the most important prognostic factor for MM?

A

Breslow Thickness

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459
Q

ABCD of MM

A

Asymmetry

Border irregularity

Colour

Diamete >6mm

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460
Q

What are the different classifications of MM

A

Lentigo MM

Superficial spreading MM

Nodular MM

Acral lentiginous MM

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461
Q

Features of lentigo MM

A

Sun exposed areas of elderly caucasians: flat, flowly growing black lesions. MM can develop in a lentigo MM

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462
Q

Features of superficial spreding MM

A

Irregular borders with variation in colour. Not associated with any special site

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463
Q

Features of nodular MM

A

Can occur at all sites and seen in younger age group

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464
Q

Features of acral lentiginous MM

A

Palms, soles and subungual areas.

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465
Q
A

Erythema multiforme

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466
Q
A
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467
Q
A
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468
Q
A
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469
Q
A
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470
Q

SJS/TEN features

A

Dermatological emergency: sheets of skin detachment

Nikolsky sign positive: mucosal involvement prominent

Commonly caused by drug e.g. sulfonamide antibiotics

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471
Q

SJS vs TEN

A

<10% body surface vs >30%

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472
Q

Features of skin appenage tumours

A

Hair follicles and seat glands can produce specialised tumours of skin

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473
Q

What are the different types of cutaneous vascular tumours

A

Capillary haemangioma- strawberry mark

Flat haemangioma- port wine stain

Kaposi’s sarcoma

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474
Q

What is mycosis fungiodes?

A

Most common form of cutaenous T-cell lymphoma generally affects skin.

Is a NHL

Has a muschroom like appearance

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475
Q

Hisotlogical features of herpes infection of skin

A

Cluster of inflamed pustules and vesicles

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476
Q
A
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477
Q
A

Viral infection

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478
Q
A
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479
Q
A

Pemphigoid

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480
Q
A

Pemphigus

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481
Q

Function of cortical bone

A

Aka Compact bone

Facilitaties mane bone functions: suppors, protection and elverage

Forms cortex, dense hard bone to which muscles are attached

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482
Q

Function of trabecula bone

A

Also called cancellous bone

Higher SA, less dense, softer, weaker and less stiff,

High vascular and contains red bone marrow where haematopoesis.

Vertebrae

Has role in homeostasis of Ca

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483
Q

What are the types of non-neiplastic bone disease

A

Trauma

Infeciton

Degeneration

Inflammation

Metabolic

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484
Q

What are the classifications of fractures

A

Complete or incomplete

Closed

Comminuted

Compound

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485
Q
A
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486
Q

What are the stages of # repair

A

Organisation of a haematoma at # site (pro-callus)

Formation of fibrocartilaginous callus

Mineralisation of fiberocartilaginous callus

Remodelling of bone along weight bearing lines

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487
Q

What are the factors influencing #] repair

A

Type

Presence of infection

Pre-existing conditions e.g. neoplasm, metabolic, drugs (steroids), Vit deficiency

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488
Q

What are the features of osteomyelitis in adults

A

Sites: direct spread, secondary to diabetic skin ulcer, dental abscess etc.

S. aureus (90%)

E coli

Kleb

Salmonella (associated with SCD)

Pseudomonas IVDU

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489
Q

What are the features of osteomyelitis in children

A

Haematgoenous spread to long bones, usually metaphytic

Neonates: Haemophils influenza, Group B strep

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490
Q

Clinical features of osteomyelitis

A

Fever, malaise, chills, leucocytosis

Local: pain, swelling and tenderness

Blood test +ve in 60%

XR: mixed picture eventually lytic, 10d post onset

Periosteal reaction

Mottled rarefaction (increased osseus vascularity and lifting up of the periosteum)

Involcrum: after 1w irregular sub-periosteal bone formation

Lytic destruction (10-14d)

Seqeustra: after 3-6w, some areas of cortex may become detached

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491
Q

XR features of osteomyelitis

A

Periosteal reaction

Involcrum (after 1w: sub-periosteal bonefrmation)

Irregular lytic destruction

Sequestra: 3-6w, necrotic areas of cortex may become detached.

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492
Q

What is the clinical staging of osteomyelitis

A

Cierny-Mader Staging

Anatomical Type: 1- medullary, 2- superficial, 3- localised, 4- diffuse

Physiologic: Host A-normal, B- local or systemic compromise, C- treatment worse than disease

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493
Q

Mx of osteomyelitis

A

Biopsy: culture

6w IV antibiotics

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494
Q

Features of TB osteomyelitis

A

Rare cause mainly affecting immunocompromised

More destructive and resistant to contral

Spinal disease may result in psoas abscess: inguinal lump and severe skeletal deformity: Pott’s

Systemic amyloidosis may result in protracted cases as an inflammatory response to chornic TB infection

Marrow replaced by inflammatory response and a granuloma forms. There are Langerhan’s type giant cells which have a peripheral rim of nuceli

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495
Q

Marrow replaced by inflammatory response and a granuloma forms. There are Langerhan’s type giant cells which have a peripheral rim of nuceli

A

TB osteomyelitis

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496
Q

With what are Langerhan’s type giant cells associated

A

TB

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497
Q

Features of Syphillitic OM

A

Caused by Traponema pallidum

Can be congenital or acquired

Congenital: skeletal lesions: osteochondritis, osteoperiostitis, diaphyseal ostomyelitis

Acquired: sekeltal lesions are late onset, require lack of Rx:

non gummatous periosteitis

gummatous inflammation of bone and joints

neuropathic joints (tabes dorsalis)

neuropathic shaft fractures

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498
Q

non gummatous periosteitis

gummatous inflammation of bone and joints

neuropathic joints (tabes dorsalis)

neuropathic shaft fractures

A

Syphillitic OM

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499
Q

Features of Lyme disease

A

Inflammatory arthropathy as part of a complex multisystem illness resulting from tick bite

Caused by Borrelia burgdorferi: ixodes dammini (tick spp.)

Can be mistaken for OM

Associated with erythema chronicam migrans

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500
Q

What are the stages of Lyme disease?

A

1: early localisation, selling + rash (90%) usually within 7-10d and in groin, axilla, earlobe or on thigh

Stage 2: early dissemination, affects multiple organ systems

Stage 3: low grade, late and persistent, dominated by arthritis, mimics RA

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501
Q

Mx of Lyme disease

A

Prevention: long trousers, vaccines

Antibiotics for proven disease

Dx is purely clinical

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502
Q
A

OM

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503
Q

What is a Brodie abscess?

A

Subacute OM

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504
Q

Features of OA

A

Degenerative joint disease:

Disease of cartilage, cartialge is lost and osteophytes formed, pain due to friction

Eboination of underlying bone

Avascular necrosis of the femoral head can occur

Cartilage degernation

Fissuring

Abnormal matrix calcification

Osteophytes

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505
Q

What is the difference between primary and secondary OM

A

Primary is age related

Secondary is to a prveiously damaged joint or congenitally abnormal joint

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506
Q

XR changes in osteoarthritis

A

Loss of joint space

Osteophytes

Subchondral sclerosis

Subchondral cysts

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507
Q

Clinical features of OA

A

Big weight bearing joints, vertebrae and kness

PIPJ: Bouchards’

DIPJ: Herbeden

May also affect MCP

Synovial disease: synovium Bx is non-specific with increased vessels and chronic inflammation

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508
Q

XR features of RA

A

Loss of joint space

Erosions

Soft Bones

Soft tissue swelling

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509
Q

Features of RA

A

Disease of the synovium/severe chronic relapsing synovitis

Slow and progressive.

F>M

RF +ve in 80%, immunocomplexes are more associated with extra-articular disease

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510
Q

What factors are involved in genetic predisposition to RA

A

HLA-DR4 and DR1

TNF, STAT

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511
Q

Clinical features of RA

A

Hand: radial deviation of wrist and ulnar deviation of fingers

Swan neck deformity: extension of POPJ and flexion of DIPJ

Boutonniere: flexion PIPJ

Z shaped thumb

Symmetrical pain and swelling

DIPJ swelling

Small joints of hands and feet, wrists, elbows, ankles and knees

Mild anaemia, raised ESR, RF +ve, rheumatoid nodules?

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512
Q

Swan neck deformity

A

Extension of PIPJ and flexion of DIPJ

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513
Q

Boutonniere deformity

A

Flexion PIPJ, extension of DIPJ

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514
Q

Histopathology of RA

A

Proliferative synovitis with thickening of the synovial membranes, hyperplasia of surface synoviocytes, intense inflammatory cell infiltrate, fibrin deposition on the joint and encrosis

Pannus: exuberant inflamed synovium of the articular surface, hyperplastic synovium.

Proliferative synovitis

Grimley-Sokoloff cells

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515
Q

What are Grimley-Sokoloff cells

A

Hyperplastic synovial cells

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516
Q

What are the stages of RA

A

Unknown antigen reaches the synovial membrane

T-cell proliferation associated with increased B cells and angiogenesis

Chronic inflammation with inflammatory cytokines

Pannus

Cartilage and bone destruction

517
Q

What differentiates between RA and OA in hands

A

RA spares DIPJ

520
Q

Features of Gout

A

Male, middle aged

Increased dietary purin intake, ETOH, diuretics, inherited metabolic abnormalities

Great toe: MTP (podagara), lower extremities

Urate crystals: needle shaped

Negatively birefringent crystals

521
Q

Clinical features of gout

A

Hot, swollen, red, painful joint.

Tophus is the pathognomic lesion (on pinna and hands)

522
Q

Negatively birefringent crystals=

A

Urate

GOUT

523
Q

Featurs of pseduogout

A

>50y

Idiopathic, hyperPTH, DM, hypothyroid, Wilsons

Knee and shoulder

Hot, swollen joint with effucion

Calcium pyrophosphate crystals, rhomboid shaped

Positively birefringent

524
Q

Positively birefringent crystals=

A

Pseudogout

525
Q

Mx gout

A

Acute attack: colchicine

LT: allopurinol

Conservative: ETOH and purine intake (sardines/liver) reduction

526
Q

Mx pseudogout

A

NSAIDs or intra-articular steroids

527
Q

Subtypes of pseudogout

A

Sporadic

Metabolic: haemachromatosis, primary HPT, mypo Mg, low PO4

Hydroxylapatite crystal deposition

Hereditary: AD, ANKH mutation, transmembrane protein

Traumatic: e.g. bad OA of the knee

528
Q

Crystals in pseudogout

A

Calcium pyrophosphate (mainly knees) or calcium hydroxyapatite (knees and shoulders)

Crystals survive processing, whereas crystals of gout dissolve in aqueous solution and need to be fixed in alcohol or sent unfixed.

Crystals are not needle shaped but found in polyhonal or rhomboid islands

529
Q
530
Q
531
Q
A

AVN of femoral head with characteristic wedge shaped infarct

532
Q
A

Swan neck deformity

533
Q
A

Boutonniere

534
Q
A

RA vs OA synovium

RA= proliferative

535
Q

What is the most common site for bone malignancy?

536
Q

What is the clinical presentation of bone tumours?

A

Pain, swelling, deformity and fracture

537
Q

Diagnosis of bone tumours

A

XR: evaluate site, margin and size. Number of lesions and soft tisse extension. Any associated disease or fratcure

Biopsy: performed by radiologist with a Jamshidi needle to produce a core lesion. Can be CT or USS duiged. Open Bx for sclerot or inaccessible lesions

538
Q

What are the different types of malignant bone tumour?

A

Osteosarcoma

Chondrosarcoma

Eqing’s sarcoma

Giant cell (borderline malignancy)

539
Q

What are the most common malignant bone tumours?

A

Secondary metastatic

540
Q

What adult malignancies metastasise to bone?

A

Breast

Prostate

Lung

Kidney Thyroid

541
Q

What childhood malignanices commonly metastasise to bone?

A

Neuroblastoma, Wilm’s, osteosarcoma, Ewings, rhabdomyosarcoma

542
Q

Features of osteosarcoma

A

Adolescents. M>F. 75% 10-30y.

Site: knee>tibia>humerus>skull>femur

Originates from cells of mesenchymal origin, osteoblastic differentiation differentiation and produce malignant osteoid

Commonest primary bone tumour

Malignant mesenchymal cells +/- bone and cartilage formation. H&E stain of osteosarcoma will show mixed malignant bone in a backgrown of less cellular chondroid tumour. ALP +VE

543
Q

Malignant mesenchymal cells +/- bone and cartilage formation. H&E stain of osteosarcoma will show mixed malignant bone in a backgrown of less cellular chondroid tumour. ALP +VE`

A

Osteosarcoma

544
Q
A

osteosarcoma

545
Q
A

Osteosarcoma

ALP +ve

546
Q

XR appearance of osteosarcoma

A

Usually metaphyseal

Lytic

Permeative

Elevated periosetum (Codman’s triangle)

547
Q

Classification of osteosarcoma

A

Site within bone: intramedullary, cortical or surface

Degree of differentiation: grade

Multicentricity: synchronous or metachronous

Primary or secondary

548
Q

Mx of osteosarcoma

A

CTx and limb salvage surgery

549
Q

Features of chondrosarcoma

A

Malignant cartilage producing sarcoma

>40

M>F

Pelvis, axial skeleton, proximul femur, proximal tibia

XR: ltyic lesions with fluffy calcification involving axial skelton

Malignant chondrocytes +/- chondroid matrix may dedifferentiate to high grade sarcoma

Px: 70% 5y but depends on grade and size

550
Q

XR: ltyic lesions with fluffy calcification involving axial skelton

A

Chondrosarcoma

551
Q

Malignant chondrocytes +/- chondroid matrix may dedifferentiate to high grade sarcoma

A

Chondrosarcoma

552
Q

Classiicaiton of chondrosarcoma

A

Site: intramedullary or juxtacortical

Histologically: conventional (myxoid or hyaline), clear cell, dedifferentiated or mesenchymal

553
Q

Features of Ewing’s Sarcoma

A

AKA PNET (primitive peripheral neuroectodermal tumour)

Highly malignant small round cell tumour found in bone and soft tissue

<20y/0

Long bones and pelvis: diaphysis or metaphsis

XR: Onion skinning of periosteum

Sheets of small round cells. CD99+ve, T 11.22 translocation

Px: 75% 5y

554
Q

XR: Onion skinning of periosteum

A

Ewing’s Sarcoma

555
Q

Sheets of small round cells. CD99+ve, T 11.22 translocation

A

Ewing’s Sarcoma

556
Q
A

Ewing’s Sarcoma

Onion skinning of the periosteum

557
Q

With what is chromosomal translocation of 11:22 associated?

A

Ewing Sarcoma

558
Q

What are the different types of Round cell tumours?

A

Ewing

Small cell osteosarcoma

Rhabdomyosarcoma

Neuroblastoma

Lymphoma

SCC

559
Q

What are the features of Giant Cell Tumours

A

Borderline malignancy

20-40y

F>M

Epiphysis with metaphyseal extension

Knee epiphysis, either femur or tibia, wrist, pelvis, sarcum

XR: lytic lesions right up to articular surface

Osteoclast-type multinucleate giant cells on background of spindle/ovoid cells

Usually benign with unpredicatble behaviour

Locally aggreswsive, may recur and can metastasise

560
Q

XR: lytic lesions right up to articular surface

Osteoclast-type multinucleate giant cells on background of spindle/ovoid cells

A

Giant Cell Tumour

561
Q
A

Giant cell tumour

Lytic lesion right up to articular surface

562
Q

Features of malignant bone tumours

A

60 times rarer than lung Ca

Primary malignant bone tumours are most cmmon in children and young adults

On H&E appear red and destroy local bone

563
Q

What are the types of benign bone tumours

A

Osteid osteoma

Osteoma

Enchondroma

Osteochondroma

Fibrous dysplasia

Simple bone cysts

Osteoblastoma

564
Q

What are the features of fibrous dysplasia

A

Normal bone replaced with fibrous bone tissue causing abnromal sweeling and growth of bone.

F>M, after 3rd decade

Any bone: ribs and proximal femur commonest. Monosototic>polyostotic

XR: soap bubble osteolysis. Shepher’d crook deformity

Irregular trabeculae of woven bone in bland spindle cell stroma. Bone trabeculae shapes= chinese letters

565
Q

XR: soap bubble osteolysis. Shepher’d crook deformity

Irregular trabeculae of woven bone in bland spindle cell stroma. Bone trabeculae shapes= chinese letters

A

Fibrous dysplasia

566
Q
A

Shepherds Crook

Fibrous dysplasia

567
Q
A

Chinese letters

Fibrous Dysplasia

568
Q

Albright syndrome

A

Polyostotic fibrous dysplasia

Cafe au lait spots

Precocious puberty (endocrine problems)

569
Q

What are the tumour like coniditions of bone?

A

Fibrous dysplasia

Metaphyseal fibrous cortical defect/non-ossifying fibroma

Reparative giant cell granuloma

Ossifying fibrome

Simple bone cyst

570
Q

What are the features of simple bone cysts?

A

Found in humer or femur

Fluid filled and unilocular, lystic well defined margins

571
Q

How can the benign tumours of bone be classified?

A

Cartilaginous differentiation:

Osteochondroma

Endochrondroma

Chondroblastoma

Bone forming tumours:

Osteoid osteoma

Osteoblastoma

Osteoma

572
Q

What are the features of osteoid osteomas

A

Adolescent

M>F

Arise from osteoblasts, tend to be <1.5cm, most common in long bones (tibia and femur)

Night pain relieved by aspirin

Radiolucent nidus with sclerotic rim

Normal bone

573
Q

Radiolucent nidus with sclerotic rim

Normal bone

A

Osteoid osteoma

574
Q

What are the features of osteoma

A

Middle age

A new piece of bone usually growing on another piece of bone, tpically inthe head and neck

Normal bone

Assocaited with Gardner syndrome

575
Q

What is Gardner Syndrome?

A

GI polyps + multiple osteomas + epidermoid cysts

576
Q

What are the features of osteoblastomas?

A

Similar to osteoid osteomas. Osteoblastoma is a giant osteoid osteoma

XR: speckled mineralisation

577
Q

XR: speckled mineralisation

A

Osteoblastoma

578
Q
A

Osteoid osteoma

Radiolucent nidus with sclerotic rim

579
Q
A

Osteoblastoma

Speckled mineralisation

580
Q

What are the features of enchondromas?

A

Benign tumours of cartilage.

55% of pts 10-40y

Hand>humerus>knee>foot

XR: lytic lesion. Cotton wool calcifcation. Expansile O-ring sign

581
Q

XR: lytic lesion. Cotton wool calcifcation. Expansile O-ring sign

A

Enchondroma

582
Q

Features of osteochondroma

A

50%pts 10-20y. M:F

Knee, humerus, tibia and femur. Growth plates of long bones

A benign cartilage capped bony outgrowth.

XR: well defined bony protruberance from bone

Cartilage capped

Most common neoplsam of the skeletonl.

If there is irritation it can be removed by surgery

583
Q

XR: well defined bony protruberance from bone

Cartilage capped

A

Osteochondroma

584
Q

Features of chondroblastomas

A

Rare, originating from chondroblasts, most common epiphyseal tumour in children

585
Q

Ollier’s Syndrome

A

Multiple endochondromas

586
Q

Maffuci’s syndrome

A

Multiple endochondromas with haemangioma

587
Q

Diaphyseal aclasis/ hereditary multiple exostoses

A

Multiple exostoses (osteochondromas) + short stature + bone deformity

588
Q

What is the definition of soft tissue tumour

A

Mesenchymal proliferation which occur in the extraskeletal non-epithelial tissues of the body excluding the meninges and the lymphoreticular system

Anywhere but the majority occur in the large muscles of extremities, chest wall and retroperitoneum.

Mostly older. M>F but varies by histological type

589
Q

What are the types of soft tissue sarcoma?

A

Myxoid liposarcoma

Spindle cell sarcoma

Pleomorphic tumours

Synovial sarcoma: biphasic patter with epithelial and spindle cell areas.

590
Q

What are the congenital lower GI diseases?

A

Atresia and stenosis

Duplication

imperforate anus

Hirschprung’s

591
Q

What are the features of Hirschprungs?

A

Absence of ganlgion cells in myenteric plexus

Distal colon fails to dilate

80% male

Constipation, abdominal distension, vomiting and overlow diarrhoea in young babies. Associated with DS

Genetics: proto-oncogene Cr10+

592
Q

Dx and Mx of Hirschprung’s

A

Clinical impression, biopsy of affected segment: hypertrophied nerve fibres but no ganglia

Mx: resection of affected: constricted segment

593
Q

How can the acquired diseases of the lower GI be classified?

A

Mechanical

Inflammatory

Ischaemic

Malignant

594
Q

What are the causes of GI obstruction

A

Constipation

Diverticular disease

Adhesions

Herniation

External mass

Volvulus

Intussuception

595
Q

What is volvulus?

A

Complete twisting of bowel loop at mesenteric base around a vascular pedicle.

Intestinal obstruction +/- infarciton.

Infants: commonly small bowel

Elderly: sigmoid>caecal

596
Q

Features of diverticular disease

A

High incidence in West due to low fibre diet and high intraluminal pressure

Herniation occurs at weak points in the wall of the bowel, 90% in left colon.

Seen as outpouchings on endoscopy.

Cxs: diverticulitis, gross perforation, fistula to bowel, bladder or vagina, or obstruction

597
Q

What are the inflammatory causes of bowel disease?

A

Acute:

Infection

Toxins (abx)

CTx/RTx

Chronic:

IBD

TB

598
Q

Causes of acute colitis

A

Infection, durgs and toxins, CTx

599
Q

Features of infective colitis

A

Secretory diarrhoea (toxin)

Exudative diarrhoea (invasion and muscoal damage)

Severe damage and perforation

Systemic illness

600
Q

What is pseudomembranous colitis

A

Abx associated colitis.

Acute colitis with pseudomembrane formation. Caused by protein exotoxins of C. diff

Dx; histology, biopsy, C diff toxin or stool assay

Rx: metronidazole or vancomycin

601
Q

Features of iscahemic colitis/ infarction

A

Can be acute or chronic

Most common vascular disorder of the GIT

Usually occurs in “watershed” zones e.g. splenic flexure (SMA/IMA) and the rectosigmoid (IMA/IIA)

Can cause damage in tissue layers leading to perfroation: muscoal, mural, transmural

602
Q

Causes of iscahemic colitis

A

Arterial: atheroma, thrombosis and embolism

Venous: thrombus, hypercoagulable states

Small vessel disease: DM, cholesterol emboli, vasculitis

Low flow state: CCF, haemorrhage, shock

Obstruction: hernia, intussuception, volvuluis and adhesions

603
Q

Clinical features of IBD

A

Diarrhoea +/- blood

Fever

Abdo pain

Acute abdomen

Weight loss

Extra-intestinal manifestations

604
Q

Crohn’s epidemiology

A

Western populations

F>M

White>non-white

Smoking aggravates

605
Q

UC epidemiology

A

More common than Crohn’s

Whites>non-whites

20-25y

606
Q

Aetiology of IBD

A

Genetic predispotiion (familial aggregation, twin studies, HLA0

Infection (MTB, measles)

Abnormal host immunoreactivity

607
Q

Pathophysiology of Crohn’s

A

Affects whole GIT

Most common in terminal ileum and caecam

Skip lesions: patchy distribution

Cobblestone appearance: areas of health mucose lie above diseased mucosa

Apthous ulcer.

Rosethorn ulcers can join together to form serpentine granulomas

Transmural inflammation

Non-caseasting granulomas

Fistula/fissure formation more common

608
Q

Skip lesions

609
Q

Apthous ulcer

A

First lesion in Crohn’s

610
Q

Non-caseating granulomas with transmural inflammation

611
Q

Pathophysiology of UC

A

Extends proximally from rectum

Continuous

Small bowel not affected unless v. severe colitis casuses backwash ileitis

Extensive superficial broad ulcers confined to the mucosa

No granulomas/fissures/fistulae/strictures

Islands of regenerating muscoa bulge into lumen to cause pseudopolyps

612
Q

Mucosal continous ulceration with no granulomas

613
Q

Backwash ileitis

A

Severe pancolitic UC

614
Q

Thick rubber hose like bowel wall with narrow lumen

Fat wrapping

615
Q

Bowel wall normal thickness in IBD

616
Q

IBD:

Intermittent diarrhoea, pain and fever

617
Q

IBD:

Bloody diarrhoea, mucus, crampy abdominal pain relieved by defacation

618
Q

Extra GI manifestation of IBD

A

Malabsorption and Fe def: stomatitis

Eyes: uveitis, conjuncitvitis

Skin: erythena nodosum, pyoderma gangrenosum, erythema multiforme, digital clubbing

Joints: migratory asymmetrical polyarthropathy of large joints, sacroiliitis, myositis, ankylosing spondylitis

Pericholangitis, primary sclerosing cholangitis, steatosis

619
Q

PSC

A

UC

Pancolitis= increased

620
Q

Extra-intestinal manifestation of IBD:

Eyes

A

Uveitis, conjunctivitis

621
Q

Extra-intestinal manifestation of IBD:

Skin

A

Eythema nodosum

Pyoderma gangrenosum

Erythema multiforme

Clubbing

622
Q

Extra-intestinal manifestation of IBD:

Joints

A

Migratory asymmetrical polyarthritis

Sacroilitis

Anky spond

Myositis

623
Q

Extra-intestinal manifestation of IBD:

Liver

A

Pericholangitis

PSC

Steatosis

624
Q

Cxs of Crohn’s

A

Strictures (recurrent)

Fistulae

Abscess fromation

Perforation

625
Q

Cxs of UC

A

Severe haemorrhage

Toxic megacolon

30% require colectomy within 3y for uncontrollable symptoms

Adenocarcinoma (20-30x risk)

626
Q

Ix in Crohn’s

A

Markers of inflammation: ESR, CRP, Ba contrast, endoscopy

627
Q

Ix in UC

A

Rectal biopsy

Flexible sig/colonoscopy

AXR

Stool culture

628
Q

Mx Crohn’s

A

Mild: prednisolone

Severe: IV hydrocortisone, metronidazole

Additional therapies:

Azathioprine, methotrexate, infliximab

629
Q

Mx UC

A

Mild: prednisolone and 5 ASA (meslalzine)

Moderate: prednisolone, 5ASA and steroid eneme BD

Severe: admit, nbm IV fluids and IV hydrocortisone, rectal steroids

For remission: 5ASA 1st line, azathioprine 2nd line

630
Q

Features of carcinoid syndrome

A

Diverse group of tumours of enterochromaffin cell origin

Produce 5-HT

Commonly found in the bowel but also lung, ovaries, testes, usually slow growing

631
Q

Features of carcinoid syndorme

A

Bronchoconstriction

Flushing

Diarrhoea

632
Q

Features of carcinoid crisis

A

Life threatening vasodilation, hypotension, tachycardia.

Bronchoconstriction

Hyperglycaemia

633
Q

Ix of carcinoid syndrome?

Rx

A

24h urine 5-HIAA- main 5HT metabolite

Octreotide: somatostatin analgoue

634
Q

What are the non-neoplastic polyps of the large bowel?

A

Hyperplastic

Inflammatory: pseudopolyps

Hamartomotous (juvenile, Peutz Jeghers)

635
Q

What are the features of adenoma

A

Benign dysplastic lesions that are the precursor to most adenocarcinomas

Mostly asymptomatci so need surveillance if >3.4cm

Classified based on architecture as tubular, tubulovillous or villous adenoma

Size is most important risk factor for malignancy, in addition to degree of dysplasia and increased villous component

636
Q

What can villous adenoma lead to and why?

A

Hypoproteinaemic hypokalaemia because they leak large amounts of protein and K

637
Q

What is the sequence of metaplasia in adenocarcinoma

A

Normal colon-> at risk mutation after first hit mutation in APC

At risk_> adenoma after second hit to APC

Progression to APC follows activation of KRAS, LOF in p53

638
Q

Features of hamartomatous polyps

A

Found sporadically in some genetically acquired syndromes

639
Q

Features of Juvenile polyposis

A

Focal malformations of mucosa and lamina propria

Generally <5y/o.

Mostly in rectum leading to bleeding

Usually solitary but >100= juvenile polyposis which may require colectomy to stop haemorrhage

640
Q

Features of Peutz-Jeghers

A

Multiple polyps, mucocutaneous hyperpigmentation, freckles around mouth, palms and soles

Increased risk of intussucception and malignancy.

Require regular surveillance of GIT, pelvis and gonds

641
Q

Features of hyperplastic polyps?

A

Seen at 50-60y and though to be caused by shedding of epithelium-> cell build up

642
Q

What are the mesenchymal lesions of the GIT?

A

Stromal lesions

Lipoma

Sarcoma

643
Q
A

Villous adenoma

644
Q
645
Q
A

Hyperplastic polyps

646
Q

Epidemiology of CRC

A

2nd commonest cause of cancer death in UK

60-79y/o

<50y= ?familial syndrome

98% are adenocarcinomas, 45% in rectum

647
Q

Aetiology of CRC

A

Diet low in fibre high in fat

Lack of exercise

Obesity

Familaly syndromes

Chronic IBD

NSAID protective (COX2 overexpressed in 90%)

648
Q

Clinical features of right sided CRC

A

Fe def, anaemia. Weight loss

649
Q

Clinical features of left sided CRC

A

Change in bowel habit, crampy LLQ pain

650
Q

Ix in CRC

A

Sigmoidoscopy, solonoscopy, proctoscopy

Barium enem

Bloods: FBC

CT/MRI

CEA to moniotr disease

651
Q

How is CRC graded and staged?

A

Grade: level of differentiation

Staging: Duke’s

652
Q

Outline Duke’s staging

A

A: confined to the wall of the bowel >95%

B: Through wall of bowel

1: extending into muscularis propria 67%
2: transmural invasion without LN involvement 54%

C: LN metastases

1: Extending into muscularis propria with LN involvement 43%
2: Transmural with involved LNs 23%

D: Distant mets <10%

653
Q

Mx of CRC

A

Sx:

Rectal cancer/ low sigmoid cancer:

<1-2cm above anal sphincter (lower 1/3rd of rectum): abdomino-perineal resection

>1-2cm above anal sphincter: anterior resection

Sigmoid cancer: sigmoid colectomy

Descending colon and distal transverse: left hemicolectomy

Caecum, ascending colon and proximal transverse: right hemicolectomy

Transverse colon: extended right hemicolectomy

RTx to reduce local recurrence post-Sx

CTx in pallliation: 5 fluorouracil

654
Q

Features of FAP

A

70% AD mutations in APC gene with 30% AR mutation in MMR genes

Presents 10-15y with >100 adenomatous polyps required for Dx

All will progress to adenoarcinoma if left untreated thereefore most have prophylactic colectomy

Increased risk of neoplasia elsewheree.g. ampulla of Vater and stomach

At birth hypertrophy of retinal pigment epithelium

655
Q

Gardner Syndrome

A

Same clinical, pathological and aetiological features as FAP

Distinctive extra-intestinal manifestations:

Multiple osteomas of skull and mandible

Epidermoid cysts

Desmoid tumours

Dental caries

Post-surgical mesenteric fibromatoses

656
Q

Turcot’s syndrome

A

Malignant tumours of the CNS associated with FAP

657
Q

HNPCC

A

AD mutation in MMR gene

3-5% of colorectal cancer at early age of onset

Cacrinomas usually in right colon, few polyps but fast preogression

Also assocaited with endometrial, ovarian, small bowel, transitional cell and stomach carcinoma

658
Q

Lynch syndrome

659
Q

Lynch syndrome I

A

Familial colon cancer

660
Q

Lynch syndrome 2

A

Associated with other cancers of the GIT or reproductive system

661
Q

What are the Amsterdam criteria

A

Used to determine testing of individuals at risk of HNPCC

Amsterdam Criteria:

Three or more family members with a confirmed diagnosis of colorectal cancer, one of whom is a first degree (parent, child, sibling) relative of the other two

Two successive affected generations

One or more colon cancers diagnosed under age 50 years

Familial adenomatous polyposis (FAP) has been excluded

Amsterdam Criteria II:

Three or more family members with HNPCC-related cancers, one of whom is a first degree relative of the other two

Two successive affected generations

One or more of the HNPCC-related cancers diagnosed under age 50 years

Familial adenomatous polyposis (FAP) has been excluded

662
Q

What are the samples in cytopathology?

A

Gynae: NHSCSP

Non gynae: exfoliative samples

663
Q

Features of the NHS CP

A

Women invited from 25-65y

Samples taken are liquid based cytology

Viewed microscopically

Also test for HPV and STDs

Reduces incidence of invasive squamous cell carcinoma

664
Q

What are the high risk types of HPV

What account for 70% of cervical cancers

665
Q

What are the significant mutations in lung adenocarcinoma?

A

EGFR, ALK-1

666
Q

What are the significant mutations in melanoma

667
Q

What are the significant mutations in breast Ca

A

BRAC1/2

CERB-B2

668
Q

What are the significant mutations in CRC

669
Q

Def: DM

A

Metabolic disorder characterised by chronic hyperglycaemia due to lack of insulin.

670
Q

Aetiology T1DM

T2DM

A

Autoimmune destruction of insulin producing beta cells by CD4 and CD8 T lymphocytes

Strongly linked to insulin resistance and obesity

671
Q

Presentation of DM

A

Polyuria (osmotic diuresis)

Polydipsia (raised plasma osmolality)

Hyperglycaemia predisposes to recurrent UTI and skin infections

T1 can present as DKA

672
Q

Dx of DM

A

Fasting glucose >7mmol/l

Random >11.1mmol/l

673
Q

Cxs of DM

A

IHD

CKD

Blindness

PVD

Foot ulceration

674
Q

Ectopic pancreas

A

Pancreatic tissue located in the duodenum , stomach or Meckel’s

25-50% asymptomatic in adulthood

675
Q

Pancreas divisum

A

Failure of fusion of the ventral and dorsal panceatic duct system

Higher risk of pancreatitis

676
Q

Annular pancreas

A

Partial or complete obstructionof the duodenum by the pancres.

Duodenum surrounded by a ring of pancreatic tissue which is continuous with the head of the pancreas

677
Q

Exocrine functions of the pancreas

A

Produces 2l/d of enzymic HCO3- rich fluid, stimulated by secretin and CCL

678
Q

Secretin features

A

Produced by s-cells of the duodenum

Controls gastric acid secretion and buffering with HCO3

679
Q

CCK features

A

Responsible for stimulating digestion of fat and protein

Made by I-cells in the duodenum

Causes release of digestive enzymes

680
Q

Alpha cells of pancreas

A

Glucagon-> increases blood glucose

681
Q

Delta cells of pancreas

A

Somatostatin to regulate alpha and beta cells

682
Q

D1 in pancreas

A

Vasoactive peptide stimulating the secretion of H2O into the pancreatic system

683
Q

PP in pancreas

A

Pancreatic polypeptide

Self-regulates secretion activities

684
Q

Macrovascular cxs of DM

A

Cardiac- MI

Renal- GN, pyelonephritis

Cerebral- CVA

685
Q

Microvascular Cxs of DM

A

Diabetic retinopathy

Claudidcation

686
Q

Causes of acute pancreatitis

I GET SMASHED

A

Idiopathic

Gallstones

Ethanol

Trauma

Steroids

Mumps

Autoimmune

Scorpion Venom

Hyperlipidaemia

ERCP

Drugs e.g. thiazides

687
Q

Presentation of acute pancreatitis

A

Severe epigastric pain radiating to back, relived by sitting forward.

Vomiting prominent

Hypoetnsive shock in severe cases

A signficantly raised plasma amylase in clinical setting is virutally diagnostic

Can result in the formation of a pseudocyst, associated with alcoholic pancreatitis

688
Q

What cells perform the exocrine function in the pancreas?

A

Acinar cells

689
Q

Amylase vs lipase in pancreatitis

A

Amylase is only transiently raised

Lipase is more sensitive

690
Q

Cxs of acute pancreatitis

A

Severe cases may lead to shock and multiorgan failure

Infection of necrosed pancreas worsens prognosis

691
Q

Histology in acute pancreatitis

A

Coagulative necrosis

692
Q

Causes of chronic pancreatitis

A

Alcoholism

CF

Hereditary

Pancreatic duct obstruction e.g. stone/tumour

Autoimmune IgG4 sclerosing

693
Q

IgG4 sclerosing disease

A

Autoimmune chronic pancreatitis

694
Q

NB re chronic pancreatitis

A

Can very closely mimic pancreatic cancer clinically, radiologically and pathologically

695
Q

What is this tissue?

696
Q

Presentation of chronic pancreatitis

A

Chornic upper abdominal pain

Weight loss

Steatorrhoea and diarrhoea occur when most of the gland has been destroyed.

697
Q

Pathology of Chronic pancreatitis

A

Very similar to pancreatic carcinoma:

Grossly replaced with fibrous tissue, loss of exocrine tissue, duct dilatation with thick secretions, calcification

698
Q

What are the features of Acinar cell carcinoma

A

Rare (1%) malingnant epithelial neiplasm of the pancreas demonstrating evidence of enzyme production by the neoplastic cells

Older adults.

699
Q

Presentation of acinar cell carcinoma

A

Non specific symptoms: abdominal pain, weight loss, N+D

10% get multifocal fat necrosis and polyarthralgia due to lipase secretion

700
Q

Histopathology of pancreatic acinar carcinoma

A

Neoplastic epithelial cells gowing in sheets

Some have abundnant eosinophilic granular cytoplasm

Positive immunoreactivity for lipase, trypsin, chymotrypsin.

701
Q

Px of acinar cell carcinoma?

A

5YSR= <10%

702
Q

What type of pancreatic malignancy is this?

A

Acinar cell carcinoma

neoplastic epithelial cells growing in sheets, trabeculae and acini, some have abundant eosinophilic
granular cytoplasm, positive immunoreactivity for lipase, trypsin and chymotrypsin.

703
Q

What is the most common pancreatic malignancy?

Epidemiology?

A

Ductal cell adenocarcinoma of the pancreas

M>F

>60y/o

704
Q

What are the risk factors for pancreatic carcinoma?

A

Smoking is the main

Diet

Genetic e.g. FAP, HNPCC

705
Q

What are the clinical features of ductal cell carcinoma

A

Cachexia and anorexia

Upper abdominal and back pain that is persistent and severe

Painless jaundice, pruritis, steatorrhorea

DM

Trousseau’s syndrome

Ascites

Abdominal mass

Vrichow’s node

Corvosier’s sign

706
Q

Troussau’s syndrome

A

The Trousseau sign of malignancy or Trousseau’s Syndrome is a medical sign involving episodes of vessel inflammation due to blood clot (thrombophlebitis) which are recurrent or appearing in different locations over time (thrombophlebitis migrans or migratory thrombophlebitis). The location of the clot is tender and the clot can be felt as a nodule under the skin.[1] Trousseau’s syndrome is a rare variant of venous thromboembolism (VTE) that is characterized by recurrent, migratory thrombosis in superficial veins and in uncommon sites, such as the chest wall and arms. This syndrome is particularly associated with pancreatic and lung cancer. [2] Trousseau’s Syndrome can be an early sign of gastric or pancreatic cancer,[3] typically appearing months to years before the tumor would be otherwise detected.[4] Heparin therapy is recommended to prevent future clots.[5] The Trousseau sign of malignancy should not be confused with the Trousseau sign of latent tetany caused by hypocalcemia.

707
Q

Ix in ?pancreatic ductal cell carcinoma?

A

Bloods: decreased Hb, raised bilirbuin, hypercalcaemia

CT/MRI/ERCP

CA19.9 >70IU

708
Q

Tumour marker in pancreatic acrinoma?

709
Q

Mx of pancreatic ductal carcinom

A

CTx palliative (5FU)

Whipple’s procedure

Px v poor <5% 5YSR

710
Q

What type of pancreatic malignancy is this?

A

Ductal cell carcinoma

711
Q

What are the features of pancreatic endocrine tumours?

A

Group of epithelial tumours of the pancreas showing endocrine differentiation

May be functioning or non-functioning

2% of all pancreatic tumours

30-60y/o

Associated with MEN1

712
Q

Symptoms of functional neuroendocrine tumours of the pancreas

A

Present with symptoms related to hormone excess e.g.

Insulinoma: hypoglycaemic attacks

Gastrinoma: Zollinger-Ellison syndrome: recurrent ulceration

VIPoma: diarrhoea

Glucagonoma: necrolytic migrating erythemia

713
Q

Ix in neuroendocrine tumour of pancreas

Mx

714
Q

MEN1

PPP

A

Parathyroid hyperplasia/adenoma

Pancreatic endocrine tumour

Pituitary adenoma

715
Q

MEN 2A

A

Parathyroi

Thyroid

Phaeo

716
Q

MEN2B

A

Medullary thyroid

Phaeo

Neuroma

Marfanoid phenotype

717
Q

What are the different types of pancreatic cystic tumours?

A

Intraductal papillary mucinous neoplasm

Mucinous cystic neoplasm

Serous cystic neoplasms

Solid pseudopapillary neoplasm

718
Q

What type of pancreatic malignancy is this?

A

Pancreatic neuroendocrine tumour

719
Q

What is atherosclerosis?

A

A stereotypic arterial response to injury caused by an excessive and harmful inflammatory and fibroproliferative response

Chronic inflammation in the intima of large arteries

720
Q

What are the sites of predilection for atherosclerosis?

A

Branch points of arteries, bifurcations and curvatures

721
Q

What is shear stress?

A

Sideways force on the endothelium representing the tendency to be dragged along with blood flow.

Turbulence is atherogenic

722
Q

Pathophysiology of atherosclerosis?

A

Damage to smooth endothelium

LDL enters hte endothelium, becomes trapped and undergoes oxidative modification

Monocyte recruitment via chemokines (MCP-1)

Adhere to endothelium via ICAM-1 and VCAM-1

Transendothelial migration

Oxidised lipoproteins are taken up by monocytes, recognised by macrophage scavenger receptors and PRRs (can stain for macrophages with CD68)

These form foam cells which excrete inflammatory mediators e.g. IL-1

Collagenolysis via MMP occurs

Foam cells die by apoptosis releasing cholesterol into a lipid core.

Fatty streaks are hte earliest lesions seen in atherosclerosis

Platelets stick to the damaged tissue

Endothelium proliferates: fibrous cap forms on top of the endothelium and more cholesterol is deposited in the core

The plaque enlarges blocking arteries

723
Q

What is the earliest lesion seen in atherosclerosis

A

Fatty streaks

724
Q

What is the function of LDL

A

Cholesterol rich, carries cholesterol from liver to tissues

Oxidised LDL causes inflammation, endothelial damage and VSMC death

725
Q

What are the functions of VSMCs?

A

Two phenotypes: contractile and synthetic:

Synthesise collagen and strengthen the fibrous cap

726
Q

Function of endothelial cells

A

Biologically active lining of BVs

Portal of LDL emigration

Modualted by shear stress

Express adhesion molecules

727
Q

Features of familial hypercholesterolaemia?

A

AR

Extreme cholesterol levels >20mmol/L which is resistant to lipid lowering agents

Xanthomas in skin and tendons

Rapid atherosclerosis

728
Q

Timescale for atherosclerosis

A

Intermediate lesions: 20y

Advanced lesions: 40y

Cx >40y

729
Q

What are the Cxs of atherosclerosis?

A

Stenosis: carotids

Rupture: leading to sudden thrombosis and or emoblism

Erosion: can lead to thrombosis

Intraplaque haemorrhage

Aneurysm

730
Q

What are the structures of unstable plaques

A

Large lipid core, fibrous cap with a prominent inflammatory infiltrate

Inflammation has a lytic effect and there are fewer synthetic VSMCs

There is a structural breakdown of the superficial cap which tears causing intraplaque haemorrhage and superficial endothelial erosion

731
Q

Symptoms of atherosclerosis

A

Insufficient blood supply leads to cellular dysfunction which is often experienced as pain on exertion: angina pectoris, intermitten claudication

Critical iscahemia results in cell death: MI, cerebral infarction, gangrene

Cell death occurs, then micrsscopic changes, then gross cahnges

732
Q

What is usually the cause of death in MI?

733
Q

MI Histology: <6h

734
Q

MI Histology: 6-24h

A

Loss of nuclei, homogenous cytoplasm and necrotic cell death

Neutrophil infiltration occurs first

735
Q

MI Histology: 1-4d

A

Infiltration of polymorphs

Then macrophages- clear debris

736
Q

MI Histology: 5-10d

A

Removal of debris

737
Q

MI Histology: 1-2w

A

Granulation tissue, new blood vessels, myofibroblasts and collagen synthesis

Weeks-months-> strengthening and decellularising scar

738
Q

What are the complications of acute MI

A

Arrythmias

Congestive heart failure

Fibrionous pericarditis

Cardiac rupture

Papillary muscle dysfunction or rupture

Aneurysm

PE

Dressler syndrome

Cardiogenic schock

M

739
Q

What are the mechanical complications of MI?

A

Rupture of the LV, rupture of the interventricular septum and MR

740
Q

What are the pericardial complications of MI

A

3 types:

Early infarct associated pericarditis

Pericardial effusion (+/- tamponade)

Dressler’s

741
Q

Why does MI lead to increased risk of arrythmia?

A

The damaged myocardium acts as a substrate for re-entrant circuits due to changes in tissue refractoriness:

Generalised autonomic dysfunction resulting in enhanced automaticity of the myocardium and conduction system

There is an electrolyte imbalance: hypokalaemia and hypomagnesia

Transmural infarction can interrupt afferent and efferent limbs of the SNS

742
Q

How does the risk of VF change after MI

A

Greatest immediately after, declines afterwards

743
Q

What proportion of patients with AMI are susceptible to arrythmia?

744
Q

Which type of MI has an increased risk of VF?

745
Q

What is the consequence of papillary muscle dysfunction or rupture

A

Can cause mitral valve incompetence

Usually occurs in the first few days

746
Q

When does thromboembolism follow MI?

A

>1w due to mural thrombi overlying infarcted tissue

747
Q

How do aneurysms arise in AMI

A

End-stage scars can form large transmural infarcts which result in large bulging aneurysms

>4w

748
Q

What is the triad in Dresslers?

A

Triad of fever, pleuritic pain and pericardial effusion

749
Q

What are the causes of HF?

A

IHD

Valve disease

Myocarditis

HTN

Cardiomyopathy

750
Q

Pathology seen in HF?

A

Dilated heart

Scarring and thinning of the walls

Pleural effusion

Pulmonary oedema

Hepatomegaly

Ascites

Peripheral oedema

751
Q

Why does ventricular remodelling occur?

A

Neurohumoral imbalance, increased cytokine expression, immune and inflammatory changes and altered fibrinolysis. Leads to multiple effects

752
Q

Hypertrophic heart leads to?

A

Diastolic heart failure

753
Q

Dilated heart leads to?

A

Systolic heart failure

754
Q

Features of liver in HF

A

nutmeg liver

Venous congestion and fatty changes

Hepatic cirrhosis with firosis

755
Q

Def: cardiomyopathy

A

Intrinsic disease of heart muscle

756
Q

Def: dilated cardiomyopathy

A

Progressive loss of myocytes, characterised by a dilated heart, HF, arrythmias and sudden death

757
Q

Pathology of dilated cardiomyopathy

A

Myocytes replaced with fine interstitial fibrosis

758
Q

Causes of dilated cardiomyopathy

A

Idiopathic

Infective

Toxic: ETOH, CTx, cobalt Fe

Hormonal: hyper, hypothyroid, DM, peri-partum

Genetics: haemochromatosis, Fabry’s, Mc ARdle’s

Immunological: myocarditis

759
Q

Def: hypertrophic cardiomyopathy

A

LV hypertrophy, characteristic fibre dissaray

Familial in 50% (AD with variable penetrance)

Thickening of the septum narrows the LV outflow tract

760
Q

Clinical findings in hypertrophic cardiomyopathy

A

Systolic murmur

Arrhythmias

Hx of sudden death

761
Q

Cause of HOCM

A

Sarcomere mutations (myosin heavy chain)

762
Q

Def: restrictive cardiomyopathy

A

Walls are rigid, heart is prevented from stretching and filling with blood

763
Q

Causes of restrictive cardiomyopathy

A

Priamry:

Loffler’s endocarditis

Endocardial fibroelastosis

Secondary:

Infiltrative: amyloidosis, sarcoidosis, haemochromatosis

Interstitial: postradiation fibrosis

764
Q

Loeffler endocarditis

A

Loeffler endocarditis is a form of restrictive cardiomyopathy which affects the endocardium and occurs with white blood cell proliferation, specifically of eosinophils.[1] Restrictive cardiomyopathy is defined as a disease of the heart muscle which results in impaired filling of the heart ventricles during diastole.[1][2]

765
Q

Features of chronic rheumatic valve disease?

A

Sequaelae of earlier rheumatic fever, predominantly left sided

Mitral>aortic>tricuspid>pulmonic

766
Q

Which valve is most commonly affected in rheumatic fever?

767
Q

What are the pathological chagnes occuring to the valve in chronic rheumatic valvular disease?

A

Leaflets thicken

Fusion of the commissures

Chordae tendinae also thicken, shorten and fuse

768
Q

What are Aschoff bodies?

A

Nodules found in hearts of individuals with rheumatic fever which resulted from inflammation of the heart muscle

769
Q

What are the causes of AR?

A

Rigid: rheumatic and degenerative

Destructive: microbial endocarditis

Collapse: Prolapse through a VSD or due to myxomatous degeneration

Disease of the arotic valve ring

Mecahnical: if the heart dilates the valve may become insufficient to cover the dilated area, can occur in Marfan’s dissecting aneurysms, ank spond, syphillitic aortitis and cystic medial degeneration of the aortic media

770
Q

Presentation of MR

A

Middle aged woman with SOC, chest pain, mid systolic click, late systolic murmur

771
Q

Histology of MR

A

Increased glycosaminoglycans which are prominent on Alvian blue stain

772
Q

Types of pericarditis?

A

Fibrinous: MI and uraemia

Purulent: staphylococcus

Granulomatous: TB

Haemorrhagic: tumour, TB, uraemia

Fibours aka constrictive

773
Q

What is usually the cause of pericardial effusion?

A

Chronic heart failure

774
Q

Features of LV failure

A

Damming of blood within pulmonary circulation_> dyspnoea, orthopnoea, PND, wheeze, fatigue

Eventually leads to reduced peripheral BP and flow

775
Q

Ix in HF

A

BNP, CXR, ECG, Echo

776
Q

What is the most common mutation in HOCM?

777
Q

Features of acute rheumatic fever?

A

Heart: pancraditis

Joints: arthritis and synovitis

Skin: erythema marginatum, subcut nodules

Neurological: Encephalopathy, Sydenham’s chorea

778
Q

Clinical presentaiton of rheumatic fever

A

Clinical features develop 2-4w post strep throat

779
Q

What is the main group causing acute rheumatic fever?

A

Lancefield GAS

780
Q

Anitschkov myocytes?

A

Regenerating myocytes seen in acute rheumatic fever

781
Q

Verrucae

Aschoff bodies

Anitschkov myocytes

A

Rheumatic fever

782
Q

Treatment of acute rheumatic fever

A

Benpen

Erythromycin if penallergic

783
Q

What are the causes of vegetative endocarditis?

A

Rheumatic heart disease

Infective endocarditis

Non-bacterial thrombotic endocarditis (marantic)

Libman Sacks endocarditis

784
Q

Large irregular masses on valve cusps extending into the chordea

A

Infective endocarditis

785
Q

Small, bland vegetations attached to lines of closure, formed of thrombi

786
Q

<2mm vegetations that are sterile and platelet rich

A

Libman=Sacks Endocarditis

787
Q

With what is Libman Sacks endocarditis associated?

A

SLE and anti-phsopholipid

788
Q

What are the predisposing factors leading to infective endocarditis?

A

Rheumatic heart disease

Mitral valve prolapse

Calcified valves

Bicuspid aortic valve

Prosthetic valve

Congential defects

789
Q

What is infective endocarditis usually secondary to?

A

Bacteraemia as a consequence of

poor dentalo hygiene

IVDU

Soft tissue infection

Dental treatments

Cannulae

Cardiac Sx/pacemeakers

790
Q

What are the casuative organisms in acute infective endocarditis?

A

Staph aureus

Strep pyogenes

791
Q

Pathology of acute infective endocarditis

A

Larger and more localised vegetations spreading into the aorta

792
Q

What are the causative organisms in subacute infective endocarditis?

A

Strep viridans

Staph epidermis

HACEK

Coxiella

Mycoplasma

Candida

793
Q

Pathology in subacute infective endocarditis?

A

Friable, soft thrombi, small

Extending into chordae

794
Q

Clinical features of infective endocarditis

A

Fever, malaise, anaemia, rigors, splenomegaly, new murmur

Roth spots

Splinter haemorrhages

Janeway lesions

Osler’s nodes

Usually mitral/aortic unless IVDU

795
Q

Dx of infective endocarditis

A

Duke’s criteria

796
Q

Rx in infective endocarditis?

A

Benpen + gentamicin

797
Q

Site, pathology, aetiology:

Chronic bronchitis

A

Bronchus

Dilatation nof the airways and excess mucus production

Tobacco smoke, air pollution

798
Q

Site, pathology, aetiology:

Bronchiectasis

A

Bronchus

Airway dilatation and scarring

799
Q

Site, pathology, aetiology:

Asthma

A

Bronchus

SM cell hyperplasia, excess mucus, inflammation

Immunologic

800
Q

Site, pathology, aetiology:

Emphysema

A

Acinus

Airspace enlargement, wall destruction

Tobacco smoke, alpha-1 anti-trypsin deficiency

801
Q

Site, pathology, aetiology:

Bronchiolitis

A

Bronchiole

Inflammatory scarring, obliteration

Tobacco smoke, air pollutants

802
Q

Clinical features of chronic bronchitis

A

Cough and sputum on most days for 3m over 2 years

803
Q

Dilatation of the airways

Goblet cell hyperplasia

Hypertrophy of mucuous glands

A

Chronic bonchitis

804
Q

Cx of chronic bronchitis

A

Reuccrent infections

Chronic hypoxia

Pulmonary HTN

805
Q

Permanent dilatation of the bronchi

A

Bronchiectasis

806
Q

Curschmann spirals

Charcot-Leyden crystals

807
Q

Whorls of shed epithelium

A

Curschmann spirals

808
Q

Whorls of shed epithelium

Eosinophils

Charcot-Leyden crystals

809
Q

Loss of the alveolar parnehcyma distal to the terminal bronchiole

810
Q

Cx of emphysema

A

Pneumothorax

Respiratory failure

Pulmonary HTN

811
Q

Cx of bronchiectasis

A

Recurrent infections

Haemoptysis

Pulmonary HTN

Amyloidosis

812
Q

Symptoms of bronchiectasis

A

Cough

Purulent sputum

Fever

813
Q

Causes of bronchiectasis

A

Inflammatory:

Post-inefctious

Abnromal host defence: hypogammaglobinaemia, CTx

Ciliary dyskinesia: Kartageners and 2o

Obstruction

Post-inflammatory (aspiration)

Secondary to bronchiolar disease and interstitial fibrosis

Systemic disease

Asthma

Congenital:

CF

Primary ciliary dyskinesia

Hypogammaglobulinaemia

814
Q

Def: interstitial lung disease

A

Group of >200 disease characterised by inflammation and fibrosis of the pulmonary connective tissue, particulary the most peripheral and delivate interstitium of the alveolar wall

815
Q

What are the features of interstital lung disease

A

Restrictive features on spirometry:

Decreased CO diffusion capacity

Decreased lung volume

Decreased compliance

816
Q

Presentation of interstitial lung disease

A

SOB

End-inspiratory crackles

Cyanosis, pulmonary HTN and cor pulmonale

All have honey-comb lung in end stage

817
Q

Honey-comb lung

A

Interstitial lung disease

818
Q

How can interstitial lung disease be characterised?

A

Fibrosing:

Idiopathic pulmoanry fibrosis

Pneumoconiosis

Crpyotgenic organising pneumonia

Associated with CTD

Drug-induced

Radiation pneumonitis

Granulomatous:

Sarcoid

EAA

Associated with vasculitides e.g. Wegener’s, Churg-Staruss, microscopic polyangitis

Eosinophilic

Smoking related

819
Q

Features of cryptogenic fibrosin alveolitis/idiopathic pulmonary fibrosis

A

M>F

Unknown causative agents

Usual intersitital pneumonia required for Dx

(also seen in CTD, asbestosis and EAA)

820
Q

Progressive patchy, interstitial fibrosis with loss of normal lung architecture and honeycomb change, beginning at the periphery of the lobule, usualy sub-pleural

Hyperplasia of type 2 pneumocytes causing cyst formation= honeycomb

A

Usual Interstitial fibrosis

821
Q

Clinical presentation of Idiopathic interstitial fibrosis

A

Increasing exertional dyspnoea and non-productive cough

40-70y/o

Hypoxaemia-> cyanosis and pulmonary HTN

+/- cor pulmonale and clubbing

822
Q

Rx of interstitial fibrosis

A

Steroids

Cyclophosphamide

Azathioprine

(Limited impact on survival)

823
Q

Which CTDs can have prominent lung fibrosis?

A

RA, systemic sclerosis, SLE

824
Q

Def: pneumoconiosis

A

Typically and occupational lung disease: non-neoplastic reaction to inhalation of mineral dusts or inorganic particles

Majority affect the upper lobe e.g. coal workers penumoconiosis, silicosis, asbsestosis (tends to affect the lower lobe)

825
Q

Granulomatous infections

A

TB, fungal (histoplasma, cryptococcus, coccidioides, aspergillus, pneumoystitsi) parasites

826
Q

Non-infectious granulomatous disease

A

Sarcoid

Foreign body

Drugs or occupational lung disease

827
Q

What are the group of immune-mediated lung disorders characterised by intense/prolonged exposure to inhaled organic antigens leading to widespread alveolar inflammation

A

EEA

Hypersensitivity pneumonitiis

Crypotgenic organisng pneumonia

Bronchiolitis obliterans organising pneumonia

828
Q

Polypoid plugs of loose connective tissue within alevoli/bronchioes?

A

Feature of granuloma formation and organisn pneumonia

829
Q

Acute presentation of EEA etc

A

Inhalation of antigenic dust in sensitised individual leading to systemic symptoms (fever, chills, chest pain, SOB, cough) within hours of exposure

Usually settles by following day

Progresses to EEA

830
Q

Progressive, persistent productive cough and SOB

Finger clubbing and severe weight loss

831
Q

Different types of EEA

A

Farmers lung: moudly hay
- Saccharopolyspora rectivirgula

Pigeon fanciers lung

  • Proteins in excreta/feathers

Humidifiers lung

  • heated reservoirs

Malt-workers lung:

  • germinating barley

Cheese washers loung:

  • fungi
832
Q

What are the different categhroies of penumonia?

A

Bronchopneumonia: patchy bronchial/peri-bronchial distribution. Low virulence organisms

Lobar pneumonis: fibrinosuppurative consolidation

Atypical: interstitial pneumonitiis without intra-alveolar inflammation

833
Q

Stages of lobar pneumonia

A

Consolidation

Red hepatisation (neutrophilia)

Grey hepatisation (fibrosis)

Resolution

834
Q

What are the diseases oif the pulmonary vasculature

A

PE

Pulmonary HTN

835
Q

What are the tumours of the lung

A

Squamous CC

Adenocarcinoma

Small CC

Large CC

Mesothelioma

836
Q

What is the most common tumour of the lung?

A

Squamous CC

837
Q

Which lung cancers are more common in smokers?

A

Squamous cell

Small cell

838
Q

Features of squamous cell carcinoma

A

Closely correlated with smoking

M>F

Usually proximal bronchi, local spread with late metastasis

Less responsive to chemo

Variety of subtypyes: papillary, basaloid,

Associated with caviation and hypercalcaemia

839
Q

Progression of squamous cell carcinoma

A

Epithelium

Hyperplasia

Squamous metaplasia

Angiosquamous dysplasia

Carcinoma in situ

Invasive carcinoma

840
Q

Keratinisation, intercellular prickles

Sqamous cells on cytology

A

Squamous cell lung carcinoma

841
Q

Features of lung adenocarcinoma

A

Most common in womena dn non smokers

Malignant epithelial tumour with glandular differntiation or mucin production

Occurs peripherally and metastasises early

842
Q

Histo: glandular differentiation

Cyto: cells containing mucin vaculose

EGFR mutations

A

Adenocarcinoma

843
Q

Progression of adenocarcinoma

A

Atytpical adenomatous hyperplasia-> non mucinous BAC-> mixed pattern adenocarcinoma

844
Q

Features of small cell carcinoma

A

Usually occurs centrally in the proximal bronchi

Arises from neuroendocrine cellls

Associated with ectopic ACTH secretion, Lambert-Eaton, cerebellar degeneration

Highly malignant with early metastasis usually by diagnosis

Poor prognosis

p53 and RB1 mutations common

845
Q

Lambert-Eaton

A

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease — a disease in which the immune system attacks the body’s own tissues. The attack occurs at the connection between nerve and muscle (the neuromuscular junction) and interferes with the ability of nerve cells to send signals to muscle cells.

846
Q

Paraneoplastic cerebellar degeneration syndrome

A

Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to an underlying (usually undetected) malignant tumor. Patients with paraneoplastic neurological syndrome (PNS) most often present with neurologic symptoms before an underlying tumor is detected.

Paraneoplastic neurologic syndromes include many neurologic disorders including paraneoplastic cerebellar degeneration (PCD) caused by an immune-mediated mechanism other than a metastatic complication in patients with an underlying malignancy. Any part of the nervous system can be involved depending on the type of primary malignancy. These syndromes affect 1-3% of all cancer patients.[1] These syndromes are difficult to diagnose and respond poorly to treatment. However, the oncologic outcome of patients with antibody-associated paraneoplastic syndromes does not significantly differ from that of patients who do not have the antibodies or a paraneoplastic syndrome.

847
Q

Anti-Yo

A

Paraneoplastic cerebellar degeneration

848
Q

Features of large cell carcinoma

A

Poorly differentiated malignant epithelial tumour: large cells, large nuclei with prominent nuceloli

No evidence of glandular or squamous differentiation

Associated with paraneoplastic syndromes:

ADH, ACTH, PTH, PTHrP, Calcitonin, serotonin, bradykinin

849
Q

NSCLC: ERCC1

A

Poorer response to cisplatin

850
Q

EGFR in adenocarcinoma

A

Target for anti-EGFR TKI therapy

851
Q

Kras mut in adeno/squamous cell carcinoma

A

Poor prognosis- no response to TKI

852
Q

EML4-ALK

A

Usualy adenocarcinoma= no benefit from TKI

853
Q

Staging of lung malignancy

A

T1-4 based on size, invasion of pleura and pericardium

LN metas: N0-2 (N0- no LNs, N1-N2 LNs involved, dependant on extent)

M0-1: mets or na

854
Q

Features of mesothelioma

A

Arises from either parietal or visceral pleura

Spreads within pleural space

Associated with pleural effusion, chest pain and dyspnoea

Latent period of 25-45 following asbestos exposure

855
Q

How do large PEs affect pulmonary vasculature

A

Impact in the main pulmonary arteries leading to acute cor pulmonale, cardiogenic shock, and death if >60% of pulmonary bed occluded

856
Q

Saddle embolus=

A

Occluding the pulmonary trunk

857
Q

How do small emboli impact pulmonary vasculature

A

May be silent or cause peripheral wedge infarctions

Repeated infarctions can lead to pulmonary HTN

858
Q

What are non-thrombotic emboli

A

BM, amniotic fluid, tumour, air, foreign body

859
Q

Def: pulmonary HTN

A

Mean pulmonary arterial pressure >25mmHg at rest

860
Q

Causes of pulmonary HTN

A

Can be primary

More often secondary due to:

Chronic lung disease

Heart diseases

Recurrent thrombo-emboli

Autoimmune disorders

861
Q

What are the group most commonly affected by primary pulmonary HTN?

A

Women aged 20-40

862
Q

How can secondary causes of pulmonary HTN be classified?

A

Pre-capillary:

Chronic hypoxia/embolus

Capillary:

Fibrosis

Post-capillary:

Left heart disease, veno-occlusive disease

863
Q

Def: pulmonary oedema

A

Intra-alvolar fluid accumulation leading to poor gas exchange, main aetiology is left heart failure

864
Q

Iron-laden macrophages=

A

Heart failure cells

Seen in pulmonary oedema due to heart failure

865
Q

Causes of diffuse alveolar damage

A

ARDS in adults

Neonates: hyaline membrane disease of newborn, insufficient surfactant production

866
Q

Common congenital disorders of the lung?

A

Lung agenesis or hypoplasia

Tracheal and bronchial stenosis

Congeital cysts

867
Q

Causes of ARDS

A

Infection

Aspiration

Trauma

Inhaled irritant gases

Shock

Blood transfusion

DIC

Drug overdose

Pancreatitis

Idiopathic

869
Q

Macroscopic features of asthma?

A

Mucus plug

Overinflated lungs

870
Q

What are the two types of emphysema and their causes?

A

Paracinar: caused by anti-trypsin deficiency

Centrilobular: caused by smoking, loss of the centre of the bronchiole

871
Q

What are the organs affected by CF?

A

Lung

GIT: meconium ileus and malabsroption

Pancreas: pancreatitis and malabsorption

Liver: cirrhosis

Male reproductive system: infertility

872
Q

How does chronic hypoxia cause pulmonary HTN

A

Normal response of lung is to reduce blood flow to hypoxic areas

Results in chronic vasoconstriction of the pulmonary arterioles which leads to morphological changes e.g. eccentric intimal fibrosis, thickening of the muscle wall

873
Q

What is pulmonary veno-occulsive disease

A

Fibrotic occlusion of the pulmonary veins can be caused by:

Idiopathic

Herbal teas and diet pills

CTx

RTx

BM transplant

Renal transplant

HIV

Systemic sclerosis

874
Q

Dx of idiopathic pulmonary fibrosis

A

HRCT +- biopsy

875
Q

What is an issue with bevacizumab in squamous cell carcinoma

A

Some patients develop fatal haemorrhages with some durgs as it is an angiogenesis inhibitor

876
Q

Stain for adenocarcinoma

877
Q

Stain for SqCC

A

CK5/6, P63, TTF-1

878
Q

Cx of lung cancer

A

Bronchial obstruction

Invasion of local structures

Extension through pleura or pericardium

Diffuse lymphatic spread within lungs

Metabolic spread

Paraneoplastic ssyndromes

879
Q

What are the paranetoplastic syndromes seen in lung cancer and their consequences?

A

ADH: SIADH causing hyponatraemia

ACTH: Cushings

PTHrP: hypercalcaemia

Calcitonin; hypocalcaemia

Gonadotrophin: gynaecomastia

Serotonin: carcinoid syndrome

Nonednocrine: haematological/coagulation defects

880
Q

Consequences of lung cancer invading local structures

A

SVCO

Oesophagus: dysphagia

Horner’s syndrome

881
Q

Pathology in chronic meningitis?

A

Leptomeninges and sometimes dura thickened

Arachnoid adhesions may be present

Lymphocyte, plasma cell and epitheloid macrophages seen in the infiltrate

Caseous necrosis and granulomatous inflammation may be present in TB

Adhesions can cause a non-communicating hydrocephalus

882
Q

What are the leptomeninges?

A

Pia and arachnoid mater and the subarachnoid space

Inflammation usually due to infection.

883
Q

Features of cystitis

A

Can be acute or chronic

85% caused by gram negative bacilli that are normally resident in GIT e.g. E Coli etc

884
Q

What drug is associated with cystitis?

A

Cyclophosphamide with haemorrhagic cystitis

885
Q

What are the risk factors for cystitis

A

Female

Calculi

Urinary obstruction

DM

Sexually active

Instrumentation

(Cyclophosphamide)

886
Q

What are the types of bladder tumours?

A

Transitional cell

Squamous cell

Adenocarcinoma

887
Q

What is the most common bladder malignancy?

A

Transitional cell

888
Q

Epidemiology of TCC

A

90% of all bladder tumours

Male>Female

50-80y/o

889
Q

Risk factors for bladder TCC

A

Cigarette smoking

Aromatic amines

890
Q

Clinical features of TCC

A

Painless haematuria, freuqency, urgency, pyelonephritis or hydronephrosis if ureteral orifices involved

891
Q

Dx of TCC

A

Cystoscopy + biopsy

892
Q

With what is squamous CC of the bladder associated?

A

Schistosomiasis

893
Q

What is the aetiology of bladder adenocarcinoma?

A

Rare, arising from extensive intestinal metaplasia or from urachal remnants

894
Q

What is the urachus?

A

The urachus is a remnant of a channel between the bladder and the umbilicus (belly button) where urine initially drains in the fetus during the 1st trimester of pregnancy. The channel of the urachus usually seals off and obliterates around the 12th week of gestation and all that is left is a small fibrous cord between the bladder and umbilicus called the median umbilical ligament.

895
Q

What is BPH

A

DHT-mediated hyperplasia of prostatic stromal and epithelial cells resulting in the formation of large nodules

896
Q

Symptoms of BPH

A

Difficulty urinating

Retention

Frequency

Nocturia

Overflow dribbling

897
Q

Features of chronic pyelonephritis and reflux nephropathy

A

Tubulo-interstitial inflammation causing scars, leads to blunting of the renal calcyes and deformation,

Xanthogranulomatous PN can result from Gram -ve infections

898
Q

Mx of BPH

A

TURP

5 alpha reductase inhibitors

899
Q

What is the most common form of prostate Ca

A

Adenocarcinoma

Commonly seen in older gentlemen

900
Q

What is the scoring system for prostate carcinoma?

A

Gleason scoring:

Pattern 1- well differentiated tumours, uniform round glands packed into circumscribed nodules

Pattern 5: no glandular differentiation

Final score= predominant pattern plus worst pattern

901
Q

Gleason grading

A

Grade 1 <6

Grade 2 6+7

Grade 3 >8

902
Q

Dx of prostate adenocarcinoma

A

Hx

Ex

PSA (.4ng/ml)

903
Q

Mx of prostate adenocarcinoma

A

Stage T1/T2: RTx and Sx

T3: Extraprostatic spread: external beam radiation

904
Q

Causes of acute prostatitis?

A

E Coli and other gram -ve rods

Fever, chills and dysuria

905
Q

Features of chronic abacterial prostatitis

A

Manifestation similar to chronic bacterial but without hx of recurrent UTI

Culture negative

Dysuria or mild suprapubic discomfort

906
Q

Chronic bacterial prostatitis?

A

Symptoms of recurrent UTI

907
Q

What is the most common cause of granulomatous prostatitis?

A

Bladder BCG for treatment of carcinoma in situ

Non-specific granulomatous prostatitis

Symptoms of urinary incontinence and pelvic discomfort

908
Q

What are the most common types of testicular tumours?

A

Germ cells

909
Q

Draw the types of testicular tumours

910
Q

What is the most common type of germinal tumour?

911
Q

What is the impact of undescended testes on cancer risk?

912
Q

What is PIN

A

Prostatic intraepithelial neoplasia

Common in young men

33% risk of carcinoma

Doesn’t cause an elevated PSA but examine for carcinoma

913
Q

What are the markers for germ cell tumours?

A

AFp

beta HCG

LDH

914
Q

Seminoma: age group

915
Q

Seminoma marker?

A

Beta HCG

(AFP and ALP not raised)

916
Q

What is the most common testicular carcinoma in <3y/o?

A

Embryonal carcinoma and yolk sac tumour

Aggressive and malignant

AFP

917
Q

Marker of endodermal sinus tumour?

918
Q

Marker of choriocarcinoma?

919
Q

What is a condyloma acuminatum?

A

Genital wart

920
Q

What is the malignant penile lesion?

A

Squamous cell carcinoma

HPV-related

921
Q

Clinical features of renal cell carcinoma?

A

Costovertebral pain

Palpable mass

Haematuria

Paraneoplastic syndromes: Polycythaemia, hypercalcaemia, HTN, Cushing’s, amyloidosis

922
Q

Risk factors for testicular tumours?

A

Cryptorchidism

Testicular dysgenesis

Klinfelters

Testicular feminisation

923
Q

What are the types of renal carcioma

A

Clear cell

Papillary: commonest in dialysis-associated cystic disease

Chromophobe renal carcinoma: pale eosinophilic cells

924
Q

What are the most common organisms causing PID

A

Chlamydia

Gonorrhoea

925
Q

Symptoms of PID

A

Lower abdo pain

Dyspareunia

Vaginal bleeding/discharge

Fever

Adnexal tenderness

Cervical excitation

926
Q

Fitz-Hugh Curtis

A

RUQ from perihepatitis

Violin string peri-hepatic adhesions

927
Q

Cxs of PID

A

FHC syndrome

Infertility

Increased risk of ectopic

Intestinal obstruciton-> bacteraemia

Tubo-ovarian abscess

Peritonitis

Plical fusion

928
Q

Def: endometriosis

A

Presence of endometrial glands or stroma outside of uterus

10% of women

929
Q

What are the 3 theories around the aetiology of endometriosis?

A

Metaplasia of pelvic peritnoum

Implanatation of endometrium through retrograde menstruation

Induction of metaplasia

930
Q

Pelvic pain

Dysmenorrhoea

Deep dyspareunia

Reduced fertility

Nodules/tenderness in posterior fornix or uterus

Immobile uterus which is anteverted in severe disease

A

Endometriosis

931
Q

Red-blue to brown nodules “powder burns”

Choclate cysts in ovaries

A

Endometriosis

Endometriomas

932
Q

Def: adenomysosis

A

Ectopic endometrial tissue deep within the myometrium causing dysmenorrhoea

May cause menorrhagia and deep dyspareunia

Globular uterus

933
Q

What is the most common tumour of the female genital tract?

934
Q

Draw the location of fibroids

935
Q

What is important re fibroids

A

Oestrogen stimulation:

Enlarge during pregnancy

Regress post-menopausally

936
Q

Macroscopic and microscopic features of leiomyomas?

A

Sharp, circumscribed, discrete, round, firm, grey-white tumours of variable size

Bundles of smooth muscle cells

937
Q

Clinical features of fibroids

A

Menorrhagia

Dysmenorrhoea

Pressure effects: urinary frequency, tenesmus

Subfertility

Red degeneration in pregnancy

May rarely transform to leiomyosarcoma

938
Q

Leiomyosarcoma aetiology

A

May be due to transformation of leiomyoma

More common post-menopausally

939
Q

How is endometrial carcinoma staged?

A

FIGO

Stage 1: confined to uterus

Stage 2: involves cervix

Stage 3: spread to adenezae, serosa, positive peritoneal cytlogy, LNs (pelvic or para-aortic)

Stage 4: pelvic organs and distal spread

940
Q

Draw the division of endometrial cancer types

941
Q

What are the features of endometroid carcinoma

A

Look similar to normal endometrial glands

Associated with oestrogen excess, uusally in peri-menopausal women

942
Q

Risk factors for endometroid endometrial carcinoma

A

E2 excess:

OBESITY

Anovulatory amenorrhoea (PCOS)

Nulliparity

Early menarche

Late menopause

Tamoxifen

DM

HTN

943
Q

What is the more aggressive type of endometrial carcinoma?

A

Non-endometroid

944
Q

Features of VIN

A

Dysplasia of epithelium associated with HPV

I, II and III

Progression to invasive disease is lower than for CIN

945
Q

Significanece of Paget’s disease of vulva?

A

Adenocarcinoma in situ, rarely associated with invasive adenocarcinoma

946
Q

Tissue type in VIN

A

Generally squamous cell

947
Q

Draw the organisaiton of ovarian tumours

948
Q

Psammoma bodies, columnar epithelium=

A

Serous epithelial tumour

949
Q

No psammoma bodies

Associated with pseudomyxoma peritonei

A

Mucinous tumour of ovary

950
Q

Clear cytoplasm with hobnail appearance

A

Clear cell epithelial tumour of ovary

951
Q

What is the female counterpary of the seminoma

A

Dysgerminoma

952
Q

What is a mature teratome?

A

Dermoid cyst

Contains skin, hair, teeth, etc.

953
Q

What are immature teratomas

A

Germ cell tumours of the ovary

Malignant and usually solid, contain immature, embryonal tissues

954
Q

Meig’s syndrome associated with?

A

Fibroma of ovary

955
Q

Which ovarian malignancy produces E2?

A

Granulosa-Theca cell tumour

956
Q

Which ovarian malignancy produces androgens?

A

Sertoli-Leydig tumour

957
Q

What are the secondary ovarian tumours?

A

Endometroid, serousus, clear cell carcinomas: may be uterine primary

Krukenbergy tumours: metastatic mucin producing adenocarcinomas from stomach or breast

958
Q

Eponymous name for metastatic mucin-producing adenocarcinoma from stomach/breast

A

Krukenberg tumour

959
Q

What HPV strains associated with CIN

A

Commonly 16 and 18

960
Q

What is the transitional zone

A

Where the squamous cell transforms into columnar epithelium

961
Q

CIN I-III

A

I: dysplasia confined to lower 1/3

II: lower 2/3

III: full thickness but with bm intact

962
Q

Px of CIN grades

A

I: reverts to normal

30% of 3 progresses to cervical cancer over 10 yeras

963
Q

What is CGIN

A

Less common, treatment requires excision of entire endocervix

964
Q

What is the most common form of cervical carcinoma

A

Squamous cell although 20% can be adenocarcinomas etc.

965
Q

What marks the change from CIN to carcinoma

A

Invasion through basement membrane

966
Q

Clinical features of cervical carcinoma

A

PCB

IMB

Post MB

Pain

967
Q

Features of SLE

A

Autoimmune multi-system disorder

Type III hypersensitivity reaction

Increased in classical complement deficiciencies.

Can be drug-induced

Higher prevalence in Afrocarribean and women

968
Q

What HLA is associated with SLE

A

HLA DR3 (or 2)

969
Q

Autoantibodies in Lupus

A

ANA (95%)

Anti dsDNA

Anti-Sm

Drug-induced

Anti-histone

970
Q

LE bodies

Small vessel angiopathy

Onion skin lesions in the spleen

Libman-Sack endocarditis

971
Q

Signs and symptoms of Lupus

SOAP

BRAIN

MD

A

Serositis

Oral ulcers

Arthritis

Photosensitivity

Blood disorders (AIHA, ITP, leucopenia)

Renal involvement

ANA +ve

Immune phenomena (dsDNA, anti-SM, antiphospholipid Ab)

Neuro symptoms

Malar rash

Discoid rash

972
Q
973
Q

What are the two types of scleroderma?

A

Limited cutaneous (CREST)

Diffuse scleroderma

974
Q

Features of scleroderma

A

Autoimmune multi-system disorders

SKin fibrosis

975
Q

HLA associations in scleroderma

A

HLA DR5 and DRw8

976
Q

Autoantibody in CREST (limited cutaenous)

A

Anti-centromere

977
Q

Anti-centromere

A

CREST (limited cutaenous)

978
Q

Increased collagen in skin and organs

Onion skin thickening of arterioles

979
Q

Signs and symptoms of CREST

A

Skin changes on face and distal to elbows and knees

Calcinosis

Raynauds

Esophageal dysmotility

Sclerodactyly

Telangiectasia

Associated with pulmonary HTN

980
Q
A

Sclerodactyl

CREST

981
Q

Lung disease in scleroderma

A

CREST= pulmonary HTN

Diffuse= pulmoanry fibrosis

982
Q

Autoantibodies in diffuse scleroderma

A

Anti Scl-70

Fibrillarin

RNA pol I, II, III

PM-SCl

983
Q

Anti Scl-70

Fibrillarin

RNA pol I, II, III

PM-SCl

A

Diffuse scleroderma

984
Q

Inflammation within or around muscle fibres

A

Diffuse scleroderma

985
Q

Signs and symptoms of diffuse scleroderma

A

Skin changes can occur anywhere

Widespread organ involvement

Associated with pulmonary fibrosis

986
Q

Features of polymyositis/dermatomyositis

A

Autoimmune inflammatory disorder of muscle +/- skin

Associated with underlying malignancy

987
Q

Autoantibody in dermatomyositis?

A

Anti-Jo1

Anti-Mi2

988
Q

Anti-Jo1

A

Dermatomyositis

989
Q

Autoantibody in polymyositis

A

Anti-signal recognition peptide Ab

(anti-Jo1/ anti-Mi2 (D>P))

990
Q

Endomysial inflammatory infiltrate

A

Dermatomyositis

991
Q

Drop out of acpillaires and myofibre damage

A

Dermatomyositis

992
Q

Symtpoms and signs of polymyositis

A

Proximal limb, anterior neck weakness and oesophageal and respiratoy muscle involvement.

Elevated skeletal muscle enzymes

Abnormal EMG

Positive muscle biopsy

Associated with pulmonary fibrosis

993
Q

Signs and symptoms of dermatomyositis

A

Proximal limb, anterior neck weakness and oesophageal and respiratoy muscle involvement.

Elevated skeletal muscle enzymes

Abnormal EMG

Positive muscle biopsy

+ skin changes:

Heliotrope rash

Gottron Papules

Assoc c. pulmonary fibrosis

994
Q
A

Gottron papules

Dermatomyositis

995
Q
A

Heliotrope rash

Dermatomyositis

996
Q

What complement defects associated with lupus?

A

Defects in the classical pathway

997
Q

What drugs can induce lupus?

A

Hydralazine

Pocainamide

998
Q

How is lupus diagnosed

A

>4 of the ACR criteria

999
Q

Ix used in Lupus

A

CRp

ESR

Test for ANA

Complement

1000
Q

Complement in active lupus

A

C3 (common): Normal

C4 (classical): low i.e. undetectable

1001
Q

Complement in severe active SLE

A

C3 (common): Low

C4 (classical): low

1002
Q

Complement in inactive SLE

A

C3: normal

C4: normal

1003
Q

CRP in Lupus

A

Generally normal

1004
Q

ESR in lupus

1005
Q

Anti-dsDNA in Lupus

A

High

Can be used for disease monitoring

1006
Q

Anti-cardiolipin

Anti-Ro, La, Sm or RNP

in SLE

A

Sometimes high

1007
Q

Anti-topoisomerase=

A

Anti-SCl70

1008
Q

What are the large vessel vasculitides?

A

Takayasu’s

Temporal arteritis

1009
Q

What are the medium vessel vasculitides?

A

Polyarteritis Nodosa

Kawasaki’s

Buerger’s disease

1010
Q

What are the small vessel vasculitides?

A

Wegener’s

Churg Strauss

Microscopic polyangitis

HSP

1011
Q

Features of Takayasu’s arteritis

A

Pulseless disease

Increased in Japanese women

Vascular symptoms: absent pulse, bruits, claudication

Granulomatous vasculitis with massive intimal fibrosis

1012
Q

Pulseless disease

Increased in Japanese women

Vascular symptoms: absent pulse, bruits, claudication

Granulomatous vasculitis with massive intimal fibrosis

A

Takayasu’s

1013
Q

Features of temporal arteritis

A

Elderly

Scalp tenderness, temporal headache

Jaw claudication, blurred vision

ESR raised

Overlap with polymyalgia rheumatica

1014
Q

Elderly

Scalp tenderness, temporal headache

Jaw claudication, blurred vision

ESR raised

Overlap with polymyalgia rheumatica

A

Temporal arteritis

1015
Q

Granulomatous transmural inflammation and giant cells and skip lesions

A

Temporal arteritis

1016
Q

What is the most common form of arteritis?

1017
Q

Features of PAN

A

Renal involvement is main feature

Can involve other ograns

Spares lungs

30% have underlying Hep B

1018
Q

Renal involvement is main feature

Can involve other ograns

Spares lungs

30% have underlying Hep B

1019
Q

Microaneurysms on angiography

1020
Q

Necortising arteritis with inflammation, infiltration of polymorphs, lymphocytes and eosinophils

Arteritis is focal and sharply demarcated

Healed by fibrosis

1021
Q

Features of Buerger’s disease

A

Heavy smokers, usually men <35

Inflammation of arteries of extremities: usually tibial and radial

Pain, ulceration of toes, feet, fingers

Corkscrew appearance on angiogram

1022
Q

Heavy smokers, usually men <35

Inflammation of arteries of extremities: usually tibial and radial

Pain, ulceration of toes, feet, fingers

Corkscrew appearance on angiogram

A

Buerger’s disease

1023
Q

Features of Wegener’s granulomatosis

A

Upper resp tract: sinusitis, epistaxis, saddle nose

Lower resp tract: cavitation, pulmonary haemorrhage

Kidneys: cresecenteric glomerulonephritis

cANCA +ve

1024
Q

Triad in Wegeners

A

Upper RT

Lower RT

Kidneys

1025
Q

Saddle nose

Pulmonary haemorrhage

Crescenteric GN

A

Wegener’s

1026
Q

cANCA +ve

A

Wegener’s

1027
Q

Churg Strauss

A

Asthma, allergic rhinitis, eosinophilia

Later systemic involvement

pANCA (anti-PR3) +ve

1028
Q

Asthma, allergic rhinitis, eosinophilia

Later systemic involvement

A

Churg-Strauss

1029
Q

pANCA (anti-PR3) +ve

A

Churg Strauss

Microscopic polyarteritis

1030
Q

Granulomatosis with polyangitis

A

Wegener’s

1031
Q

Eosinophilic granulomatosis with polyangitis

A

Churg Strauss

1032
Q

What are the 3 types of ANCA-associated vasculitis

A

Microscopic polyangitis

Wegener’s

Churg Strauss

1033
Q

Features of microscopic polyangitis

A

Pulmonary renal syndrome:

Pulmonary haemorrhage

Glomerulonephritis

1034
Q

Features of HSP

A

IgA mediated vasculitis

Affects children

URTI precedes

Palpable purpuric rash, abdo pain, GN, arthritis, Orchitis

1035
Q

IgA mediated vasculitis

Affects children

URTI precedes

Palpable purpuric rash, abdo pain, GN, arthritis, Orchitis

1036
Q

Features of Cryoglobulinaemia

A

Presence of abnormal proteins in the blood which becomes thick or gel-like in cold temperatures

Cryoglobulins are Abs

Cause a range of Sx from skin rashes to kidney failure

1037
Q

What is amyloidosis?

A

Multisystem disorder caused by abnormal protein folding that are subsequently deposited as amyloid fibrils in tissues disrupting their function

1038
Q

How can amyloidosis be classified?

A

Primary and Secondary

1039
Q

What protein involved in primary amyloidosis?

A

AL

Derived from light chains therefore more common in patients with myeloma

1040
Q

What protein involved in secondary amyloidosis?

A

AA

Derived from serum AA which is an acute phase protein

Therefore secondary to chronic infection/inflammation

1041
Q

Associations of primary amyloidosis

A

Most common form

Most associated with plasma cell dyscrasias

Most have Bence Jones protein in urine and increased plasma cells

1042
Q

Causes of secondary amyloidosis

A

Autoimmune disease: RA, Ank Spond, IBD

Chronic infections: TB, osteomyelitis, IVDU

Non-imune: RCC, HL

1043
Q

What is seen in haemodialysis associated amyloidosis?

A

Deposition of beta2-microglobulin

1044
Q

What is the most common form of familial amyloidosis?

A

Familial Mediterranean Fever

1045
Q

Features of Familal Mediterranean Fever?

A

++++IL-1 production leading to attacks of fever and inflammation of serosal surfaces

AA amyloid, with predominant renal deposition

1046
Q

Clinical features of amyloidosis?

A

Caused by amyloid deposits in various organs:

Kidney: nephrotic syndrome

Heart: conduction defects, heart failure

Liver/spleen: hepatosplenomegaly

Tongue: macroglossia

Neuropathies: carpal tunnel

1047
Q

Apple green birefringence with Congo red stain under polarised light?

A

Amyloidosis

If not polarised light appears pink/red

1048
Q

Def: sarcoidosis

A

Multisystemic disease of unknown cause

Commonly affects young adults

Characterised by non-caseating granulomas in many tissues

1049
Q

Non-caseating granulomas, Schaumann and asteroid bodies

A

Sarcoidosis

1050
Q

Features of sarcoidosis

A

Most severe disease in Afro-Carribeans

Lungs most commonly involved

Often detected at routine CXR

Most seek help with pulmonary symptoms

1051
Q
A

Bilateral hilar lymphadenopathy

May also see fine nodular shadowing in mid zones

Sarcoidosis

1052
Q

What are the extrapulmonary manifestations of sarcoidosis

A

Skin: erythema nodosum, lupus pernio, skin nodules

LNs: lymphadenopathy, painless and rubbery

Joints: arthritis, bone cysts

Eyes: anterior uveitis-> misting of vision and painful red eye

Posterior uveitis-> progressive visual loss

Hepatosplenomegaly

Leukopaenia/anaemia

Hypercalcaemia/hpercalcuria

Myocardial involvement

CNS involvement

Keratoconjuncitvits sicca and lacrimal gland enlargement

1053
Q

Symptoms of anterior uveitis

A

Misting of vision and painful red eye

1054
Q

Symptoms of posterior uveitits

A

Progressive visual loss

1055
Q

Dx of sarcoidosis?

A

Dx of exclusion

Raised Ca

Raised ESR

Raised ACE

Transcbronchial biopsy

1056
Q
A

Lupus pernio

1057
Q

ACE in sarcoid

A

ngiotensin converting enzyme (ACE) participates in the renin cascade in response to hypovolemia. Its peptidase action on the decapeptide angiotensinogen I results in the hydrolysis of a terminal histidyl leucine dipeptide and the formation of the octapeptide angiotensin II, a potent vasoconstrictor that increases blood pressure.

The primary source of ACE is the endothelium of the lung. ACE activity is increased in sarcoidosis, a systemic granulomatous disease that commonly affects the lungs. In sarcoidosis, ACE is thought to be produced by epithelioid cells and macrophages of the granuloma.

Currently, it appears that ACE activity reflects the severity of sarcoidosis: 68% positivity in those with stage I sarcoidosis, 86% in stage II sarcoidosis, and 91% in stage III sarcoidosis. Serum ACE also appears to reflect the activity of the disease; there is a dramatic decrease in enzyme activity in some patients receiving prednisone.

Other conditions such as Gaucher disease, leprosy, untreated hyperthyroidism, psoriasis, premature infants with respiratory distress syndrome, adults with amyloidosis, and histoplasmosis have been associated with increased levels of ACE.

1059
Q

http://www.lab.anhb.uwa.edu.au/mb140/addons/mcqquiz.htm

1060
Q

What are the features of acute mastitis

A

Painful red breast, fever

Almost always occur during lactation and are due to staphylococcal infection via cracks in the nipples

Involved breast tissue is necrotic and infiltratred by neutrophils

1061
Q

Mx of acute mastitis?

A

Continued expression of milk

Abx

+/- surgical drainage

1062
Q

Features of periductal mastitis

A

Mostly in smokers and is not associated with lactation

1063
Q

Keritanising squamous epithelium extending deep into nipple duct orificies

A

Periductal mastitis

1064
Q

Features of duct ectasia

A

Inflammation and dilatation of the large breast ducts

Poorly defined palpable periarolar mass with thick white nipple secretions

Caused by granulomatous inflammation and dilation of large breast ducts

Mimics mammographic appearance of cancer

1065
Q

Features of fat necrosis

A

Inflammatory reaction to damaged adipose tissue

Presents as painless breast mass/skin thickening/mammographic lesion

Caused by trauma, surgery, radiotherapy

1066
Q

What are the benign proliferative breast conditions

A

Fibrocystic disease/ fibroadenosis

Gynaecomastia

1067
Q

What are the changes in breast tissue seen in fibrocystic disease?

A

Cystic change: small cysts form by dilation of lobules, contain fluid and often calcified

Fibrosis: inflammation and fibrosis secondary to cyst rupture

Adenosis: increased number of acini seen per lobule

Exagerrated response to hormonal influence

1068
Q

Breast lumpiness

A

Fibrocystic disease

1069
Q

Features of gynaecomastia

A

Unilateral or bilateral enlargement of the male breast

Indicative of hyperestrinism: ETOH, age, liver cirrhosis, functioning testicular tumour

Epithelial hyperplasia with finger-like projections into ducts

1070
Q

What are the benign proliferative conditions of the breast

A

Fibroadenoma

Duct papilloma

Radial scar

(Phyllodes tumour)

1071
Q

Features of fibroadenoma

A

Most common benign tumour, from stroma, often multiple and bilateral

Occuring at any age within the reporudcitve period

Epithelial response to size therefore increase during pregnancy and calcify after menopause

1072
Q

Mobile, well circumscribed, spherical, rubbery breast lump in young woman

A

Fibroadenoma

1073
Q

Features of duct papilloma

A

Benign papillary tumour arising within the duct system of the breast

Can be peripheral (within small terminal ductules) or central (larger lactiferous ducts)

Causes bloody discharge, not seen on mammogram, Ix with galactogram

1074
Q

Mx of intraductal papilloma

A

Excision of the affected duct is curative

1075
Q

Features of Radial scar

A

Benign sclerosing lesion of the breast

Central scarring surrounded by proliferating glandular tissue in stellate pattern

Resembles cancer on mammogram

1076
Q

Complex sclerosing lesion of the breast

A

Radiat scar

1077
Q

What are the proliferative breast diseases?

A

A diverse group of intraductal proliferative lesions of the breast associated with an increased risk of the development of subsequent invasvie breast carcinoma

Usually microscopic asymptomatic lesions

Usual epithelail hyperplasia

Fat epithelial atypia: low grade precursor to DCIS

In situ lobular neoplasia

1078
Q

What are the risk factors for breast carcinoma

A

Susceptiblity genes

Hormone exposure

Age

FHx

Race: caucasian>Afrocarribean>asian>hispanic

Obesity, tobacco, alcohol

1079
Q

What are the susceptibility genes associated with breast cancer

A

BRCA1/BRCA2

Increased risk of ovarian, prostate and pancreatic malignancy

BRCA mutations cause a lifetime risk of breast carcinoma of up to 85%

1080
Q

How does hormone exposure impact on breast cancer

A

Early menarche

Late menopause

Late 1st live birth (pregnancy leads to terminal differentiation of molk-producing luminal cells removing these from the potential cancer pool)

OCP/HRT

1081
Q

Presentation of breast cancer

A

Hard fixed lump

Paget’s disease of breast

Peau d’orange

Nipple retraction

1082
Q
A

Paget’s disease of breast

1083
Q

Breast cancer screening programme

A

47-73 y/o women invited every 3 years for mammography

1084
Q

Features of DCIS

A

Neoplastic intraductal epithelial proliferation with an inherent but not inevitable risk of progression to breast cancer

Limited to ducts/lobules by basement membranes

Increased incidence since mammography

Appear as areas of microcalficiation

1085
Q

Features of LCIS

A

Always incidental finding on biopsy as no microcalcifications or stromal reactions

20-40% bilateral

Cells lack adhesion protein

1086
Q

Features of invasive breast carcinoma

A

Malignant epithelial tumours which infiltrate hte breast with capacity to metastasise

1087
Q

Low grade BCA

A

Arises from low grade DICS or in situ lobular neoplasia and show 16q loss

1088
Q

What are the different histological classifications of invasive breast cancer

A

Ductal

Lobular

Tubular

Mucinous

1089
Q

Features of invasive ductal carcinoma

A

Carcinoma that cannot be classified into another group, most common

1090
Q

Features of invasive lobular carcinoma

A

Cells aligned in single file chains/strands

1091
Q

Features of tubular carcinomas

A

Well-formed tubules with low grade nuclei, rarely palpable as <1cm

1092
Q

Features of mucinous breast carcinoma

A

Cells produce abundant quantities of extracellular mucin which dissects into surrounding stroma

1093
Q

What are the components of the triple assessment?

A

Examination

Radiological examination (mammography, FNA, USS)

FNA and cytology

1094
Q

What are the components examined in histological grading of breast cancer?

A

Nuclear pleomorphism, tubule formation, mitotic activity

1095
Q

What happens with all breast neoplasms

A

Investigated for receptor status:

ER/PR: good prognosis- tamoxifen

Her-2: bad prognosis

1096
Q

What is the single most important factor in px of breast cancer

A

Axillary LN status

1097
Q

Tamoxifen=

A

Mixed agonist/antagonist of ER

1098
Q

Trastuzumab

A

Herceptin

Monoclonal Ab vs Her2

1099
Q

What is an important consideration for Herceptin?

A

Has a direct toxic effect on myocardium

Must monitor LVEF

1100
Q

Features of basal-like carcinoma of breast

A

Sheets ot atypical cells with lymphocytic infilrtate

Stains positive for CK5/6/14

1101
Q

Features of Phllodes tumour

A

Arise from interlobular stroma with increased cellularity and mitosies

>50y with a palpable mass

Low or high grade lesions.

Most benign but can be aggressive

Therefore excised with wide margins to limit local recurrence

1102
Q

Anterior pituitary secretions

1103
Q

What are the symptoms of pituitary disease?

A

•Hyperpituitarism

–Excess secretion of trophic hormones

–Usually due to functional adenoma

•Hypopituitarism

–Deficiency of trophic hormones

•Local mass effects

1104
Q

What is the most common type of pituitary adenoma?

A

Prolactinoma

1105
Q

Pituitary microadenoma=

1106
Q

What are the clinical effects of prolacitnomas

A

Amenorrhea, galactorrhoea, loss of libido, infertility

1107
Q

Clinical effects of growth hormone adenomas

A

Prepubertal children- gigantism

Adults- acromegaly

DM, muscle weakness, HTN, congesitve cardiac failure

1108
Q

Clinical effects of corticotroph cell adenomas

1109
Q

What are the significant causes of hypopituitarism

A

Nonsecretory pituitary adenomas

Ischaemic necrosis:

Sheehan’s

DIC, SCA, elevated ICP, Shock

Pituitary ablation by sx or irradiation

1110
Q

What are the clinical manifestations of anterior pituitary hypofunction

A
  • Children - growth failure (pituitary dwarfism)
  • Gonadotrophin deficiency - amenorrhea and infertility in women. Decreased libido and impotence in men
  • TSH and ACTH deficiency - hypothyroidism and hypoadrenalism
  • Prolactin deficiency - failure of post-partum lactation
1111
Q

What are the peptides released by the posterior pituitary

A

ADH

Oxytocin

1112
Q

What are the local mass effects of pituitary tumours

A

Compression of optic chiasm leading to bitemporal hemianopia

Signs and symptoms of raised ICP

Obstructive hydrocephalus

1113
Q
A

Anterior pituitary

1114
Q
1115
Q
1116
Q

Physiology of the thyroid

A

Responds to TSH from ant pit

Follicular cells convert thyroglobulin into T3 and T3

Effect is to increase BMR

Also contain a population of C -cells (parafollicular) that promote Ca absorption by the skeletal system

1117
Q

Features of non-toxic goitre

A

Enlargement of the thyroid

Common if there is impaired synthesis of thyroid hormone, most often due to I deficiency

Can be:

Seen at puberty in females

Due to ingestion of substances that interfere with thyroid hormone synthesis

Due to hereditary enzyme defects

1118
Q

Features of multinodular goitre

A
  • With time simple thyroid enlargement may be transformed to a multinodular pattern
  • May reach massive size
  • May lead to mechanical effects including dysphagia and airways obstruction
  • A hyperfunctioning nodule may develop leading to hyperthyroidism
1119
Q

Def: thyrotoxicosis

A

•Hypermetabolic state caused by elevated circulating levels of free T3 and T4

1120
Q

How can thyrotoxicosis be classified?

A

Primary:

Grave’s

Hyperfunctioning multinodular goitre

Hyperfunctioning adenoma

Thyroidits

Secondary:

TSH secreting pituitary adenoma (rare)

Not associated with thyroid disease:

Struma ovarii (ovarian teratoma with ectopic thyroid)

Factitious thyrotoxicosis (exogenous thyroid intake)

1121
Q

What is the most common cause of endogenous hyperthyroidism

1122
Q

Triad in Grave’s?

A

Thyrotoxicosis

Infiltrative opthalmoapthy with exopthalmos

Infiltrative dermopathy (myxoedema)

F>M

1123
Q
A

Exopthalmos

1124
Q
A

Pretibial myxoedema

1125
Q

Pathogenesis of Grave’s disease

A

Autoimmune disorder caused by autoantibodies including those vs TSHR and thyroglobulin

Abs vs TSHR most important-> stimulate release of thyorid hormones

Associated with other autoimmune diseases (hunt in packs)

1126
Q

How can hypothyroidism be classified

A

Primary:

Postablative

Autoimmune- Hashimoto’s

Iodine deficiency

Congenital biosynthetic defect

Secondary:

Pituitary or hypothalamic failure

1127
Q

What is the most common cause of hypothyroidism

A

Hashimotos’

1128
Q
A

Hashimoto’s thyroiditis

1129
Q

What is suggestive of thyroid maligiance in terms of the nature of thyroid nodules

A

Number- solitary

Consistency- solid

Age of patient- younger

Sex- Male

Iodine uptake- Cold

1130
Q

Features of thyroid adenomas

A
  • Usually solitary
  • Well circumscribed lesion that compresses the surrounding parenchyma
  • Well formed capsule
  • Small proportion cause thyrotoxicosis
  • Important to examine the capsule for invasion to exclude follicular carcinoma
1131
Q

Features of papillary carcinoma

A

Nonfunctional

Painless mass in neck

Cervical mets

10y survival up to 90%

  • May occur at any age
  • May have papillary architecture
  • BUT diagnosis is based on nuclear features

–Optically clear nuclei

–Intranuclear inclusions

•May be psammoma bodies

1132
Q
A

Papillary carcinoma

1133
Q

Features of follicular carcinoma

A
  • Peak incidence in middle age
  • Follicular morphology
  • May be well demarcated with minimal invasion or clearly infiltrative
  • Usually metastasise via bloodstream to lungs bone and liver
1134
Q

Features of medullary carcinoma

A
  • Neuroendocrine neoplasm derived from parafollicular C cells
  • 80% sporadic - adults 5-6th decade
  • 20% familial - MEN - younger patients
1135
Q

Features of anaplastic carcinoma

A
  • Occur in elderly patients
  • Very aggressive
  • Metastases common
  • Most cases death within one year due to local invasion
1136
Q

Functions of parathyroids

A
  • Activity controlled by level of free calcium in blood
  • Decreased calcium stimulates release of PTH
  • PTH

–Activates osteoclasts

–Increases renal tubular reabsorption of calcium

–Increases conversion of vitamin D to its active form

–Increases urinary phosphate excretion

–Increases intestinal calcium absorption

1137
Q

Causes of hyperparathyroidism

A
  • 80-90% - solitary adenoma
  • 10-20% hyperplasia of all 4 glands

–Sporadic or component of MEN type 1

•<1% carcinoma

1138
Q

Causes of hypoparathyroidism

A
  • Surgical ablation
  • Congenital absence
  • Autoimmune
1139
Q

Clinical manifestations of hypoparathyroidism

A

–Neuromuscular irritability - tingling, muscle spasms, tetany

–Cardiac arrhythmias

–Fits

–Cataracts

1140
Q
A

Adrenal gland

1141
Q

Zona glomerulosa secretes?

A

Aldosterone

1142
Q

Zona fasciculata secretes?

1143
Q

Zona reticularis secretes?

A

Androgens and GCs

1144
Q

Adrenal medulla secretes

A

Nadr + Adr

1145
Q

Manifestations of adrenocortical hyperfunction

A
  • Cushing’s syndrome – excess glucocorticoids
  • Hyperaldosteronism
  • Virilising syndromes – excess androgens
1146
Q

Clinical features of Cushing’s syndrome

A
  • Hypertension and weight gain
  • Truncal obesity
  • ‘moon’ facies
  • ‘buffalo hump’
  • Cutaneous striae
1147
Q

What is the most common cause of Cushing’s

A

Administration of exogenous GCs

Atrophic adrenal glands

1148
Q

What are the endogenous causes of Cushing’s syndrome

A
  • >50% due to primary hypothalamic-pituitary disease with increased ACTH – Cushing’s disease
  • Most associated with ACTH-producing adenoma in the pituitary
  • Some have hyperplasia of ACTH secreting cells in pituitary
  • Adrenal glands show nodular cortical hyperplasia
  • 30% of cases – primary adrenal
  • Most cases are due to a solitary neoplasm

–Adenoma

–Carcinoma

•Less commonly due to bilateral hyperplasia

1149
Q

What lung tumour associated with ectopic ACTH secretion

A

Small Cell Carcinoma

Adrenals show bilateral hyperplasia

1151
Q

Features of hyperaldosteronism

A

•Primary

–35 % aldosterone secreting adenoma – Conn’s syndrome

–60 % bilateral adrenal hyperplasia

–Clinical manifestations are hypertension and hypokalaemia

–Accounts for <1% of causes of hypertension but important to recognise as surgically correctable

1152
Q

Conn’s syndrome=

A

Primary hyperaldosteronism

1153
Q

Adrenogenital syndromes and neoplasms?

A

More commonly associated with carcinoma than adenoma

1154
Q

•Congenital adrenal hyperplasias

A

–Group of autosomal recessive disorders

–Hereditary defects in enzymes involved in cortisol biosynthesis

–Decreased cortisol results in increased ACTH, adrenal stimulation and increased androgen synthesis

–May present in childhood or less commonly in adults

1155
Q

Causes of adrenal insufficiency

A

Primary

Secondary due to reduced ACTH

1156
Q

Causes of acute primary adrenal insufficiency

A

–Sudden withdrawal of corticosteroid therapy

–Haemorrhage (neonates)

–Sepsis with DIC (Waterhouse-Friderichson syndrome)

1157
Q

Waterhouse-Friderichson syndrome

A

Waterhouse–Friderichsen syndrome (WFS), hemorrhagic adrenalitis or fulminant meningococcemia is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection: Typically the pathogen is the meningococcus Neisseria meningitidis.

1158
Q

Addison’s disease

A

Chronic primary adrenal insufficiency

1159
Q

Causes of chronic primary adrenal insufficiency

A

–Autoimmune (75-90%)

–TB

–HIV

–Metastatic tumour (lung and breast particularly)

–Rarely amyloid, fungal infections, haemochromatosis, sarcoidosis

1160
Q

What are the adrenocortical neoplasms

A

•Adenomas

–most non-functional

–May be associated with Cushing’s syndrome or Conn’s syndrome

•Carcinomas

–Rare

–Usually large

–More commonly associated with virilizing syndrome than adenoma

1161
Q

What are the neoplasms of the adrenal medulla

A

Phaeo

Neuroblastoma

1162
Q

Rule of 10s in phaeo?

A

–10% arise in association with a familial syndrome inc. MEN 2A and 2B, von Hippel-Lindau disease and Sturge-Weber syndrome

–10% are bilateral

–10% are malignant

–In addition 10% of catecholamine-secreting tumours arise outside the adrenal (paragangliomas)

1163
Q

A 65 year old man is in hospital after suffering an acute myocardial infarction. The house officer hears a pansystolic murmur on auscultation.

A.

Dilated cardiomyopathy

B.

Hypertrophic cardiomyopathy

C.

Myomalacia cordis

D.

Rheumatic fever

E.

Congenital septal defect

F.

Pericardial effusion

G.

Infective endocarditis

H.

Aortic stenosis

I.

Myxomatous mitral valve

J.

Pericarditis

K.

Mitral regurgitation

L.

Aortic regurgitation

A

Myomalacia cordis

1164
Q

A 46 year old women presents to A&E out of breath and with severe chest pain. On examination a mid systolic click late systolic murmur is revealed.

A.

Dilated cardiomyopathy

B.

Hypertrophic cardiomyopathy

C.

Myomalacia cordis

D.

Rheumatic fever

E.

Congenital septal defect

F.

Pericardial effusion

G.

Infective endocarditis

H.

Aortic stenosis

I.

Myxomatous mitral valve

J.

Pericarditis

K.

Mitral regurgitation

L.

Aortic regurgitation

A

Myxomatous mitral valve

1165
Q

Myomalacia cordis

A

Myomalacia cordis (weakness of cardiac muscle due to disintegration of dead myocytes):

Rupture of weakened cardiac muscle may result in:

· Mitral regurgitation:

Apical thrill, systolic murmur and pulmonary oedema

· Ventricular septal defect:

Left sternal edge thrill, murmur, hypotension

· External rupture:

Cardiac tamponade

1166
Q

A 68 year old smoker presents with jaundice and worsening abdominal and back pain. Scratch marks are seen on his arms and legs. He has lost 5kg in 2 months. Ultrasound shows dilated intrahepatic bile ducts.

A.

Carcinoma head of the pancreas

B.

Gallstones

C.

VIPoma (Werner Morrison syndrome)

D.

Haemochromatosis

E.

Vibrio cholerae infection

F.

Iatrogenic pancreatitis

G.

Insulinoma

H.

Renal tubular acidosis

I.

Gallstone pancreatitis

J.

Pseudocysts

K.

Hypercalcaemia

L.

Chronic alcoholic pancreatitis

M.

Cystic fibrosis

N.

Carcinoma tail of the pancreas

A

Carcinoma head of pancreas

1167
Q

What differentiates between carcinoma of the head of pancreas and tail of pancreas?

A

Carcinoma of the head causes jaundice, tail does not

1168
Q

A 59 year old widow complains of persistent back pain, loss of appetite and that she has dropped from dress size 18 to a size 14 in just 2 months. She was recently diagnosed with diabetes. A large central mass is palpable as well hepatosplenomegaly.

A.

Carcinoma head of the pancreas

B.

Gallstones

C.

VIPoma (Werner Morrison syndrome)

D.

Haemochromatosis

E.

Vibrio cholerae infection

F.

Iatrogenic pancreatitis

G.

Insulinoma

H.

Renal tubular acidosis

I.

Gallstone pancreatitis

J.

Pseudocysts

K.

Hypercalcaemia

L.

Chronic alcoholic pancreatitis

M.

Cystic fibrosis

N.

Carcinoma tail of the pancreas

A

Carcinoma tail of the pancreas

1169
Q

A 39 year old Nepalese man presents with severe watery diarrhoea. He is found to have hypokalaemia and, surprisingly, a metabolic acidosis. A RUQ mass is detected by contrast-enhanced spiral CT scanning. Stool bicarb is high and urine anion gap is negative.

A.

Carcinoma head of the pancreas

B.

Gallstones

C.

VIPoma (Werner Morrison syndrome)

D.

Haemochromatosis

E.

Vibrio cholerae infection

F.

Iatrogenic pancreatitis

G.

Insulinoma

H.

Renal tubular acidosis

I.

Gallstone pancreatitis

J.

Pseudocysts

K.

Hypercalcaemia

L.

Chronic alcoholic pancreatitis

M.

Cystic fibrosis

N.

Carcinoma tail of the pancreas

A

VIPoma

(Werner Morrison syndrome)

1170
Q

profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria, acidosis, vasodilation (flushing and hypotension), hypercalcemia and hyperglycemia.[3]

A

VIPoma

Werner Morrison Syndrome

1171
Q

Werner Morrison Syndrome

A

A VIPoma (also known as Verner–Morrison syndrome, after the physicians who first described it)[1] is a rare (1 per 10,000,000 per year) endocrine tumor,[2] usually (about 90%) originating from non-β islet cell of the pancreas, that produce vasoactive intestinal peptide (VIP). It may be associated with multiple endocrine neoplasia type 1.

The massive amounts of VIP in turn cause profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria (hence WDHA-syndrome, or pancreatic cholera syndrome), acidosis, vasodilation (flushing and hypotension), hypercalcemia and hyperglycemia.[3]

1172
Q

65 year old female with a large, cystic mass on tail of pancreas imaged using computed tomography. Further cytology reported the presence of epithelium

A.

Cystadenoma

B.

Carcinoma of the Pancreas

C.

Whipples’ resection

D.

Cystic Fibrosis

E.

Gall Bladder

F.

Agenesis

G.

Alcoholism

H.

Jaundice

I.

Pancreatitis

J.

Thrombophlebitis

K.

Trousseau’s Syndrome

L.

Scorpion Sting

M.

Hyperlipidaemia

N.

Type 1 Diabetes

O.

Pancreas Divisum

P.

Pseudocyst

A

Cystadenoma

1173
Q

55 year old, diabetic, afro-Caribbean male presents with weight loss, poor diet and a gnawing pain in his back, which is sometimes felt ‘under his chest’

A.

Cystadenoma

B.

Carcinoma of the Pancreas

C.

Whipples’ resection

D.

Cystic Fibrosis

E.

Gall Bladder

F.

Agenesis

G.

Alcoholism

H.

Jaundice

I.

Pancreatitis

J.

Thrombophlebitis

K.

Trousseau’s Syndrome

L.

Scorpion Sting

M.

Hyperlipidaemia

N.

Type 1 Diabetes

O.

Pancreas Divisum

P.

Pseudocyst

A

Carcinoma of the Pancreas

1174
Q

What differentiates between a cystadenoma and a pancreatic pseduocyst?

A

Pancreatic pseudocyst is a fluid filled collection contained within a well-defined capsule of fibrous or granulation tissue or a combination of both. It does not possess an epithelial lining. A cystadenoma has an epithelial wall or capsule that contains a fluid collection.

1175
Q

Caused by the action of acid and pepsin on the duodenal mucosa. Associated with increased output of stomach acid. Symptoms include pain in the upper abdomen, especially when the stomach is empty.

A.

Oesophageal varices

B.

Duodenal ulceration

C.

Pernicious anaemia

D.

Intestinal metaplasia

E.

Reflux oesophagitis

F.

Campylobacter jejuni

G.

Gastric cancer

H.

Helicobacter pylori

I.

Gastric ulcer

J.

Squamous carcinoma

K.

Barrett’s oesophagus

L.

Adenocarcinoma

A

Duodenal ulceration

1176
Q

Around 10 % eventually get primary lymphoma (less often, carcinoma) of the gut if not properly treated. HLA B8 is linked with this

A.

Pernicious anaemia

B.

H. pylori infection

C.

Coeliac disease

D.

Intestinal metaplasia

E.

Normal oesophagus

F.

GORD

G.

Chronic gastritis

H.

Partial villus atrophy

I.

Normal stomach

J.

Peptic ulcer

K.

Barrett’s oesophagus

1177
Q

A 30 year old female complaining of diarhorrea and weight loss. Biopsy of duodenum shows increased intraepithelial cytotoxic T cells.

A.

Acute Gastritis

B.

Pernicious Anaemia

C.

Barretts Oesophagus

D.

Oesophageal Adenocarcinoma

E.

Gastric Carcinoma

F.

GORD

G.

Coeliac Disease

H.

Oesophageal Varices

I.

Active Chronic Gastritis

J.

Duodenal Ulcer

1178
Q

A 26 year old man presents with watery diarrhoea, abdominal cramps, nausea, vomiting and a low grade fever. It started 3 days after eating some undercooked meat at a barbecue.

.

Carcinoma of the oesophagus

B.

Whipple’s disease

C.

Coeliac disease

D.

Cryptosporidiosis

E.

Partial villous atrophy

F.

Hiatus hernia

G.

Helicobacter pylori

H.

Gastric ulcer

I.

Gastro-oesophageal disease

J.

Barrett’s oesophagus

K.

Microsporidiosis

L.

Mucosal associated lymphoid tumour

M.

Tropical sprue

N.

Pernicious anaemia

O.

Lymphoma

P.

Duodenal ulcer

A

Cryptosporidios

1179
Q

A 58 year old female presents with malnutrition. She complains of abdominal pain, weight loss and arthritis. She has steatorrhoea. A jejunal biopsy showed periodic acid-Schiff (PAS)-positive macrophages

A.

Carcinoma of the oesophagus

B.

Whipple’s disease

C.

Coeliac disease

D.

Cryptosporidiosis

E.

Partial villous atrophy

F.

Hiatus hernia

G.

Helicobacter pylori

H.

Gastric ulcer

I.

Gastro-oesophageal disease

J.

Barrett’s oesophagus

K.

Microsporidiosis

L.

Mucosal associated lymphoid tumour

M.

Tropical sprue

N.

Pernicious anaemia

O.

Lymphoma

P.

Duodenal ulcer

A

Whipple’s disease

1180
Q

A 20-year-old student gives an 8 hour history of very frequent vomiting and epigastric cramping. O/E she is pale and shivering. Her serum WBC is normal.

A.

Gastroenteritis (Staphylococcus aureus)

B.

Gastric ulcer

C.

Mucosal-associated lymphoid tumour

D.

Gastro-oesophageal reflux disease

E.

Bulbar palsy

F.

Zollinger-Ellison syndrome

G.

Duodenal ulcer

H.

Adenocarcinoma

I.

Barrett’s oesophagus

J.

Mallory-Weiss tear

K.

Gastroenteritis (Salmonella)

L.

Haemorrhagic gastritis

M.

Pyloric stenosis

N.

Achalasia

A

Gastroenteritis- staph

1181
Q

How to classify causes of splenomegaly?

A

Size:

Massive, moderate, mild

1182
Q

Causes of massive splenomegaly

A

CML, myelofibrosis (UK)

Malaria, Kala-Azar (worldwide)

1183
Q

Causes of moderate splenomegaly

A

Portal HTN

Lymphoma

CLL

Thalassaemia

Metabolic diseases e.g. Gaucher’s

1184
Q

Causes of mild splenomegaly

A

Infection e.g. EBV, hepatitis, bacterial

CTD e.g. RA, PAN, SLE, Felty’s

Infiltrative disorders: amyloidosis, sarcoidosis

1185
Q

AA amyloidosis

A

Secondary to inflammatory conditions e.g. RA, Crohn’s etc

1186
Q

Pancreatic pseudocysts usually develop

A

Post surgery

1187
Q

Intestinal metaplasia

A

Intestinal metaplasia describes the transformation of an epithelium to the columnar epithelium found in the intestines. The distinguishing feature of intestinal epithelium is the presence of goblet cells. (NB. the stomach epithelium is also columnar, but it doesn’t contain the distinctive goblet cells. It contains foveolar cells which form glands and secrete mucin) Intestinal metaplasia is a response to chronic inflammation. It is commonly associated with chronic gastritis - H. pylori-induced or autoimmune. The presence of intestinal metaplasia is a strong risk-factor for the development of adenocarcinoma.

1188
Q

Barret’s oesophagus

A

Barrett’s oesophagus is another example of intestinal metaplasia due to chronic inflammation. This time chronic GORD is the cause. Again, the intestinal metaplasia is strongly associated with the development of adenocarcinoma. GORD and Barrett’s is the reason that oesophageal adenocarinoma tends to affect the distal third of the oesophagus while squamus cell oesophageal cancer affects the upper two thirds.

1189
Q

Which HLA alleles are assocaited with Coeliac disease?

A

DQ2 most strongly

DQ8

1190
Q

What differentiates between acute and chronic gastritis

A

Chronic gastritis is usually caused by H. pylori which would result in an antral gastritis. This patient had erosions throughout the gastric mucosa. Chronic gastritis would be have lymphocytes and plasma cells on biopsy - this patient had a neutrophilic infiltrate which is typical of an acute gastritis. Focal, acute mucosal defects are a well-known complication of NSAIDs which this patient was probably taking.

1191
Q

Oesophageal carcinoma tissue type lower

A

Adenocarcinoma

1192
Q

Oesphageal carcinoma tissue type middle

A

Squamous cell carcinoma

1193
Q

A 20-year-old student gives an 8 hour history of very frequent vomiting and epigastric cramping. O/E she is pale and shivering. Her serum WBC is normal.

Why is the WBC normal?

A

Staphylococcus aureus is a leading cause of gastroenteritis resulting from the consumption of contaminated food. Staphylococcal food poisoning is due to the absorption of staphylococcal enterotoxins preformed in the food. The onset of symptoms is rapid (from 30 min to 8 h) and usually spontaneous remission is observed after 24 h. As the presentation is so acute, the WCC often have not had a chance to increase (lag-time). The symptoms of staphylococcal food poisoning are abdominal cramps, nausea, vomiting, sometimes followed by diarrhea (never diarrhea alone).

1194
Q

A 26 year old gentleman presents to A and E with acute abdominal pain. He has lost 5 kg of weight in the last 2 weeks, and has been passing bloody diarrhoea with mucus. He is pyrexial and on examination is noted to have angular stomatitis.

A.

Diverticulitis

B.

Diverticular disease

C.

Angiodysplasia

D.

Crohn’s disease

E.

Intestinal TB

F.

Ulcerative colitis

G.

Gastroenteritis

H.

Colorectal carcinoma

I.

Pseudomembranous colitis

J.

Tubular adenoma

K.

Ischaemic colitis

L.

Sigmoid volvulus

A

Crohn’s disease

1195
Q

A 62 year old housewife returns to your outpatient clinic following another incidence of the passing of blood. Previous sigmoidoscopy, DRE and barium enema’s have failed to identify any lesion and she denies weight loss and diarrhoea. However, blood tests show a microcytic anaemia.

A.

Appendicitis

B.

Anorectal abscess

C.

Angiodysplasia

D.

Anterior rectocoele

E.

Diverticular disease

F.

Adenocarcinoma Duke’s stage B

G.

Diverticulitis

H.

Perianal haematoma

I.

Benign adenoma

J.

Haemorrhoids

K.

Villous adenoma

L.

Crohn’s disease

M.

Ischaemic colitis

N.

Irritable bowel syndrome

O.

Adenocarcinoma Duke’s stage C1

P.

Ulcerative colitis

A

olonic angiodysplasia is a common cause of acute or chronic rectal bleeding and iron deficiency anaemia. Angiodysplasias are tiny - 1-5 mm in diameter - hamartomatous capillary lesions in the colonic wall which produce bleeding out of proportion to their size. They are believed to be acquired, possibly as a result of tension on the veins where they pass through the muscularis. Diagnosis: subtraction mesenteric arteriography may demonstrate bleeding if rapid colonscopy: may visualise lesion Treatment: electrical coagulation via the colonoscope resection of segment of colon if the above is unsuccessful

1196
Q

A 73 year old woman attends complaining of recent onset of tiredness. She is pale and has hepatosplenomegaly and generalised lymphadenopathy in the neck, axillae and groins.

A.

Sarcoidosis

B.

Severe emphysema

C.

Chronic lymphocytic leukaemia

D.

Cirrhosis with hepatoma

E.

Hameochromatosis

F.

Liver abscess

G.

Gaucher’s disease

H.

Portal vein thrombosis

I.

Congestive heart failure

J.

Polycythaemia rubra vera

K.

Chronic myeloid leukaemia

A

Chronic lymphocytic leukaemia

1197
Q

A 45 year old male was admitted to hospital after having a fall. Investigations reveal a leucocytosis with elevated bilirubin and transferases. Levels were albumin, folate and vitamin B12 were low. Clotting studies showed a prolonged prothrombin time.

.

Hereditary haemochromatosis

B.

Alcoholic liver disease

C.

Hepatitis A

D.

Hepatitis B

E.

Hepatocellular carcinoma

F.

Wilson’s disease

G.

Autoimmune hepatitis

H.

Primary biliary cirrhosis

A

Alcoholic liver disease

1198
Q

A 35 year old patient with known Hepatitis B visits the doctor complaining of recent weight loss, loss of appetite, fevers and an ache in the right hypochondrium. On examination an enlarged, irregular, tender liver can be palpated.

A.

Primary sclerosing cholangitis

B.

Alpha1 antitrypsin deficiency

C.

Wilson’s disease

D.

Hereditary haemochromatosis

E.

Hepatocellular carcinoma

F.

Hepatitis B

G.

Primary biliary cirrhosis

H.

Ascending cholangitis

I.

Budd-Chiari syndrome

J.

Hepatitis C

K.

Alcoholic hepatitis

A

Hepatocellular carcinoma

1199
Q

A 65 year old retired factory worker presents with dysuria and frequency. He has also noticed that his urine is red. PSA is normal.

A.

Renal cell carcinoma

B.

Pyelonephritis

C.

Cystitis

D.

Urothelial carcinoma

E.

Adult PCKD

F.

Bladder cancer

G.

Prostatitis

H.

Prostate cancer

A

Bladder cancer

1200
Q

54 year old man presents to A&E with fever and weakness. Blood tests show hypercalcaemia . On eaxamination a palpable mass is found in the right loin area

A.

Renal cell carcinoma

B.

Pyelonephritis

C.

Cystitis

D.

Urothelial carcinoma

E.

Adult PCKD

F.

Bladder cancer

G.

Prostatitis

H.

Prostate cancer

A

.

Renal cell carcinoma

1201
Q

A 30 year old woman presents with lower abdominal pain accompanied by fever. She has been using an intra uterine contraceptive device since her wedding three years ago.

A.

Herpes virus infection

B.

Endometriosis

C.

Fibroids

D.

Endometritis

E.

Ectopic pregnancy

F.

Cervical polyps

G.

Arias-Stella phenomenon

H.

Polycystic ovary syndrome

I.

Candidiasis

J.

Salpingitis

A

Endometritis

1202
Q

A 30 year old woman attends a fertility clinic as her and her husband are unable to conceive. For the last few years her periods have been fairly heavy and prolonged. She has stopped wearing skirts because she thinks her ankles look fat.

A.

Herpes virus infection

B.

Endometriosis

C.

Fibroids

D.

Endometritis

E.

Ectopic pregnancy

F.

Cervical polyps

G.

Arias-Stella phenomenon

H.

Polycystic ovary syndrome

I.

Candidiasis

J.

Salpingitis

1203
Q

What is Charcot’s triad

A

Fever

RUQ pain

Jaundice

with rigors etc

Bacterial cholangitis

1204
Q

Features of PSC

A

Primary sclerosing cholangitis is a rare disease of unknown aetiology characterised by chronic inflammation and fibrosis of the bile duct. - usually men - associated with IBD, particularly ulcerative colitis - may be complicated by strictures, cholangitis, cholangiocarcinoma - autoantibodies are less frequent than in autoimmune chronic active hepatitis and primary biliary cirrhosis: in an exam, ANCA positivity might be a clue as P-ANCA positive in more than 50% of cases. Antimitochondrial antibodies almost never occur. - Liver Bx: fibrous obliterating cholangitis loss of interlobular and adjacent septal bile ducts - ERCP shows multiple annular strictures, separated by round or slightly dilated duct segments and the intrahepatic and extrahepatic bile ducts have a beaded appearance

1205
Q

fibrous obliterating cholangitis loss of interlobular and adjacent septal bile ducts - ERCP shows multiple annular strictures, separated by round or slightly dilated duct segments and the intrahepatic and extrahepatic bile ducts have a beaded appearance

1206
Q

Features of PBC

A

Primary biliary cirrhosis is a chronic, progressive, cholestatic liver disease which classically affects middle aged women (rare under 30 yrs). 95% of patients have anti-mitochondrial autoantibodies, especially E2 component of the pyruvate dehydrogenase complex. Pruritus and fatigue common, but also remember xanthelasma, hyperlipidaemia, feats of chronic liver disease.

1207
Q

A 40 year old woman has always known cramping pain associated with her periods – which have usually been heavy. Recently this pain has become constant throughout the month, and her periods have become more frequent. She claims never to have used oral contraception and has no children. She is abstaining from sexual intercourse as it is too painful.

A

Although you may classically think of the pain of endometriosis as cyclical - responding to the hormonal influence of menstruation, the history is very variable and may be progressively worse to even continuous pelvic pain. Constant pain is often due to adhesions (which this patient may have now developed). In the history - nulliparity, due to subfertility secondary to the endometriosis itself or abstinence due to deep dyspareunia is common. Rare, but possible…are symptoms such as cyclical haematuria or haemopytsis….(which is important to keep in your differential diagnosis, albeit at the bottom of your list…) or painful, cyclical, expanding masses in a pelvic scar.

1208
Q

This type of bone is 80-90% calcified and its function is mainly mechanical and protective.

1209
Q

This type of bone is immature and usually pathological.

A.

Lamellar

B.

Woven

C.

Osteoblast

D.

Osteoclast

E.

Metaphysis

F.

Osteocyte

G.

Periosteum

H.

Diaphysis

I.

Cortical

J.

Endosteum

K.

Epiphysis

L.

Cancellous

1210
Q

A 15-year-old male presents with a 2-month history of increasing pain in his right upper arm. Bone biopsy reveals sheets of cells with small, primitive nuclei and scanty cytoplasm. A positive immunoreactivity is seen with the MIC2 (CD99) antibody.

A.

Primary osteosarcoma

B.

Osteogenesis imperfecta

C.

Osteopetrosis

D.

Osteomalacia

E.

Osteomyelitis

F.

Osteoporosis

G.

Osteoarthritis

H.

Chondrosarcoma

I.

Ewing’s sarcoma

A

Ewing’s sarcoma

1211
Q

A 20-year-old male complains of a progressively enlarging painful mass on his right upper arm. Radiology demonstrates a lytic lesion of the proximal humerus with an accompanying Codman triangle. Microscopically, pleomorphic mesenchymal cells producing dark-staining osteoid are seen.

A.

Primary osteosarcoma

B.

Osteogenesis imperfecta

C.

Osteopetrosis

D.

Osteomalacia

E.

Osteomyelitis

F.

Osteoporosis

G.

Osteoarthritis

H.

Chondrosarcoma

I.

Ewing’s sarcoma

A

Primary osteosarcoma

1212
Q

A 24 year old police woman attends the clinic as her GP suspects she may have a parathyroid tumour. She has raised PTH and serum calcium. After a 24hr urinary collection it is noted the patient has a low urine calcium output

A.

Osteitis fibrosa

B.

Secondary hyperparathyroidism with chronic renal osteodystrophy

C.

Paget’s disease

D.

Cushing’s syndrome

E.

Cushing’s disease

F.

Familial hypocalcuric hypercalcaemia

G.

Primary hyperparathyroidism

H.

Tertiary hyperparathyroidism

I.

Osteomalacia

J.

Osteoporosis

K.

Primary hypthyroidism (myxoedema)

A

Familial hypocalcuric hypercalcaemia

1213
Q

ou see a new patient for the first time at the surgery, a 14 year old boy, complaining of pain in his right leg for 9 months and contracture of the right knee which has developed over this period. The child looks stunted and you can see bowing of the lower extremities in ambulators. His notes have not arrived yet from his previous doctor but his mother tells you ‘he was born 3 weeks premature with two small kidneys and three failed transplants means he has dialysis four times a day’. His blood results later show a raised PTH and phostate, low calcium and 1,25(OH)vitamin D. He is also acidotic.

A.

Osteitis fibrosa

B.

Secondary hyperparathyroidism with chronic renal osteodystrophy

C.

Paget’s disease

D.

Cushing’s syndrome

E.

Cushing’s disease

F.

Familial hypocalcuric hypercalcaemia

G.

Primary hyperparathyroidism

H.

Tertiary hyperparathyroidism

I.

Osteomalacia

J.

Osteoporosis

K.

Primary hypthyroidism (myxoedema)

A

Secondary hyperparathyroidism with chronic renal osteodystrophy

1214
Q

An 18 year old student presents to his GP with focal pain in his left fore-arm which is tender to touch and worsens at night. The pain is relieved with aspirin. An X-ray shows a 1cm are of radio-lucency in the tibia surrounded by dense bone.

A.

Osteoclastoma

B.

Osteosarcoma

C.

Osteochondroma

D.

Chondrosarcoma

E.

Ewing’s tumour

F.

Osteoid osteoma

G.

Osteoporosis

H.

Simple bone cyst

I.

Osteitis

J.

Trauma

K.

Rheumatoid arthritis

L.

Osteoarthritis

M.

Metastases

N.

Fibrous dysplasia

O.

Echondroma

A

Osteoid osteoma

1215
Q

A 14 year old boy complains to you of a painless lump on his left thigh, just above the knee which is slowly growing. His past medical history reveals that he fractured his femur in the same location several years before.

A.

Osteoclastoma

B.

Osteosarcoma

C.

Osteochondroma

D.

Chondrosarcoma

E.

Ewing’s tumour

F.

Osteoid osteoma

G.

Osteoporosis

H.

Simple bone cyst

I.

Osteitis

J.

Trauma

K.

Rheumatoid arthritis

L.

Osteoarthritis

M.

Metastases

N.

Fibrous dysplasia

O.

Echondroma

A

Osteochondroma

1216
Q

A 15 year old girl shows you a small lump on her upper arm on routine examination. She says the lump has been present for a couple of years and has slowly moved down, away from her shoulder.

A.

Osteoclastoma

B.

Osteosarcoma

C.

Osteochondroma

D.

Chondrosarcoma

E.

Ewing’s tumour

F.

Osteoid osteoma

G.

Osteoporosis

H.

Simple bone cyst

I.

Osteitis

J.

Trauma

K.

Rheumatoid arthritis

L.

Osteoarthritis

M.

Metastases

N.

Fibrous dysplasia

O.

Echondroma

A

Simple bone cyst

1217
Q

A 50 year old lady presents with pain in her jaw. She suffers from Paget’s disease. A ‘sunburst’ appearance is seen on X-ray along with a lifted periosteum (Codman’s triangle).

A.

Osteoclastoma

B.

Osteosarcoma

C.

Osteochondroma

D.

Chondrosarcoma

E.

Ewing’s tumour

F.

Osteoid osteoma

G.

Osteoporosis

H.

Simple bone cyst

I.

Osteitis

J.

Trauma

K.

Rheumatoid arthritis

L.

Osteoarthritis

M.

Metastases

N.

Fibrous dysplasia

O.

Echondroma

A

Osteosarcoma

1218
Q

An 8 year old boy is brought to his GP by his parents with pain in his hips and a fever. Blood results demonstrate a raised ESR and biopsy histology shows droplets of glycogen in the cytoplasm of small round cells in the pelvic bones.

A.

Osteoclastoma

B.

Osteosarcoma

C.

Osteochondroma

D.

Chondrosarcoma

E.

Ewing’s tumour

F.

Osteoid osteoma

G.

Osteoporosis

H.

Simple bone cyst

I.

Osteitis

J.

Trauma

K.

Rheumatoid arthritis

L.

Osteoarthritis

M.

Metastases

N.

Fibrous dysplasia

O.

Echondroma

A

Ewing’s tumour

1219
Q

70 year old man presents to his GP with a four day history of haemoptysis. He has noticed he has been loosing weight over the last 4 months and has felt tired and unwell. On examination he has bilateral ptosis and proximal weakness in the limbs which improves on repeated testing

A.

Cystic fibrosis

B.

Acute asthma

C.

Cryptogenic fibrosing alveolitis

D.

Tuberculosis

E.

Emphysema

F.

Pulmonary oedema

G.

Pulmonary embolism

H.

Squamous cell carcinoma

I.

Pneumococcal pneumonia

J.

Small cell carcinoma

K.

Thymoma

L.

Chronic bronchitis

M.

Mycoplasma pneumonia

A

Small cell carcinoma

1220
Q

A 35 year old woman presents with weight loss and tiredness. Her GP examines her and finds that she also has a fine tremor and is sweaty. Investigations: TSH <0.01, Free T4 36.0. There is low uptake on a technetium scan. The aetiology could be viral or autoimmune.

A.

Grave’s disease

B.

De Quervain’s

C.

Hashimoto’s thyroiditis

D.

Euthyroid state

E.

Medullary carcinoma

F.

Thyroid storm

G.

Subacute thyroiditis

H.

Postpartum thyroiditis

I.

Papillary carcinoma

A

Subacute thyroiditis

1221
Q

An 18 year old man notices a lump on his neck and goes to his GP. As well as the lump, the GP discovers cervical lymphadenopathy. There is no family history of any endocrine disorder, nor is he suffering from any other illness. Thyroglobulin is 140.

A.

Grave’s disease

B.

De Quervain’s

C.

Hashimoto’s thyroiditis

D.

Euthyroid state

E.

Medullary carcinoma

F.

Thyroid storm

G.

Subacute thyroiditis

H.

Postpartum thyroiditis

I.

Papillary carcinoma

A

Papillary carcinoma

1222
Q

eoplasms found in women aged between 30 and 40 as ovarian masses, usually unilateral. They are usually benign (90%) and are often the largest ovarian neoplasm.

A.

Cervical Squamous Cell Carcinoma

B.

Cervical Intraepithelial Neoplasia (CIN)

C.

Endometrial Adenocarcinoma

D.

Endometrial Polyps

E.

Vaginal Squamous Cell Carcinoma

F.

Mucinous Tumour

G.

Serous Tumour

H.

Teratoma (Mature)

I.

Endometrioid Tumour

J.

Teratoma (Immature)

A

Mucinous ovarian neoplasms account for 10-15% of all ovarian tumours and are rarely malignant (~10-15%). They are rarely bilateral, but can be very large. In contrast, whilst serous tumours are also usually benign (30% malignant) and common - accounting for 30% of all ovarian tumours - they have a strong tendency to be bilateral.

1223
Q

Squamous epithelium mixed with intestinal epithelium

A.

Serous cystadenocarcinoma

B.

Mature cystic teratoma

C.

Thecoma

D.

Dysgerminoma

E.

Fibroma

F.

Krukenberg tumour

G.

Mucinous cystadenocarcinoma

H.

Clear cell tumour

I.

Sertoli-Leydig tumour

J.

Immature teratoma

K.

Granulosa cell tumours

A

Mature cystic teratoma

1224
Q

Fibrous tissue containing spindle cells and lipid

A.

Serous cystadenocarcinoma

B.

Mature cystic teratoma

C.

Thecoma

D.

Dysgerminoma

E.

Fibroma

F.

Krukenberg tumour

G.

Mucinous cystadenocarcinoma

H.

Clear cell tumour

I.

Sertoli-Leydig tumour

J.

Immature teratoma

K.

Granulosa cell tumours

A

.Thecoma

Feedback:

In a fibroma, there are intersecting bundles of spindle cells. Thecomas in addition contain lipid.

1225
Q

Malignant cells surrounded by serous fluid and Psammoma bodies

A.

Serous cystadenocarcinoma

B.

Mature cystic teratoma

C.

Thecoma

D.

Dysgerminoma

E.

Fibroma

F.

Krukenberg tumour

G.

Mucinous cystadenocarcinoma

H.

Clear cell tumour

I.

Sertoli-Leydig tumour

J.

Immature teratoma

K.

Granulosa cell tumours

A

Serous cystadenocarcinoma

1226
Q

Germ cells mixed with lymphocytes

A.

Serous cystadenocarcinoma

B.

Mature cystic teratoma

C.

Thecoma

D.

Dysgerminoma

E.

Fibroma

F.

Krukenberg tumour

G.

Mucinous cystadenocarcinoma

H.

Clear cell tumour

I.

Sertoli-Leydig tumour

J.

Immature teratoma

K.

Granulosa cell tumours

A

Dysgerminoma

1227
Q

Seminomatous germ cell tumours are

A

radioSensitive, present in the 30s ; 15% secrete HCG ; 0% secrete AFP.

1228
Q

radioSensitive, present in the 30s ; 15% secrete HCG ; 0% secrete AFP.

A

Seminomatous germ cell tumours

1229
Q

Nonseminatous GCTs are

A

present in the 20s, and may secrete AFP and/or HCG.

1230
Q

Germ cell tumour originating in testis that is radiosensitive and classically presents in the 4th decade.

A.

Diffuse large B cell lymphoma (DLBCL)

B.

Seminoma

C.

Embryonal carcinoma

D.

Acute lymphoblastic leukaemia/lymphoma (ALL)

E.

Choriocarcinoma

F.

Teratoma

G.

Leydig cell tumour

H.

Acute myeloid leukaemia (AML)

I.

Sertoli cell tumour

J.

Yolk sac (endodermal sinus) tumour

1231
Q

Very aggressive tumour producing HCG and AFP; neoplastic cells are anaplastic.

A.

Diffuse large B cell lymphoma (DLBCL)

B.

Seminoma

C.

Embryonal carcinoma

D.

Acute lymphoblastic leukaemia/lymphoma (ALL)

E.

Choriocarcinoma

F.

Teratoma

G.

Leydig cell tumour

H.

Acute myeloid leukaemia (AML)

I.

Sertoli cell tumour

J.

Yolk sac (endodermal sinus) tumour

A

Embryonal carcinoma

1232
Q

Very aggressive HCG-producing tumour composed of cytotrophoblast and syncytiotrophoblast cells that metastasizes early.

A.

Diffuse large B cell lymphoma (DLBCL)

B.

Seminoma

C.

Embryonal carcinoma

D.

Acute lymphoblastic leukaemia/lymphoma (ALL)

E.

Choriocarcinoma

F.

Teratoma

G.

Leydig cell tumour

H.

Acute myeloid leukaemia (AML)

I.

Sertoli cell tumour

J.

Yolk sac (endodermal sinus) tumour

A

Choriocarcinoma

1233
Q

Commonest malignant cause of testicular mass in those aged under 5..

A.

Diffuse large B cell lymphoma (DLBCL)

B.

Seminoma

C.

Embryonal carcinoma

D.

Acute lymphoblastic leukaemia/lymphoma (ALL)

E.

Choriocarcinoma

F.

Teratoma

G.

Leydig cell tumour

H.

Acute myeloid leukaemia (AML)

I.

Sertoli cell tumour

J.

Yolk sac (endodermal sinus) tumour

A

Acute lymphoblastic leukaemia/lymphoma (ALL)

1234
Q

Commonest malignant cause of testicular mass in those aged 60.

A.

Diffuse large B cell lymphoma (DLBCL)

B.

Seminoma

C.

Embryonal carcinoma

D.

Acute lymphoblastic leukaemia/lymphoma (ALL)

E.

Choriocarcinoma

F.

Teratoma

G.

Leydig cell tumour

H.

Acute myeloid leukaemia (AML)

I.

Sertoli cell tumour

J.

Yolk sac (endodermal sinus) tumour

A

Diffuse large B cell lymphoma (DLBCL)

1235
Q

A 22-year-old lady presented with a vaginal discharge. Gram staining revealed “Clue cells” surrounded by rods, that were “Gram variable”.

A.

Neisseria gonorrheae

B.

Trichomonas vaginalis

C.

Herpes zoster

D.

Cervical Intraepithelial Neoplasia I

E.

Herpes simplex

F.

Cervical Intraepithelial Neoplasia III

G.

Cervical Intraepithelial Neoplasia II

H.

Granuloma Inguinale

I.

Candida albicans

J.

Group B Streptococcus

K.

Chlamydia

L.

Lymphogranuloma venereum

M.

Treponema pallidum (syphilis)

N.

Cervical polyps

O.

Gardnerella vaginalis

P.

Cervical Microglandular Hyperplasia

A

Gardnerella vaginalis

1236
Q

A pap smear taken from a chronic granulomatous ulcer shows a necrotic centre, periarteritis and endarteritis obliterans and an intense peripheral cellular infiltrate consisting mainly of mononuclear cells and giant cells.

A.

Neisseria gonorrheae

B.

Trichomonas vaginalis

C.

Herpes zoster

D.

Cervical Intraepithelial Neoplasia I

E.

Herpes simplex

F.

Cervical Intraepithelial Neoplasia III

G.

Cervical Intraepithelial Neoplasia II

H.

Granuloma Inguinale

I.

Candida albicans

J.

Group B Streptococcus

K.

Chlamydia

L.

Lymphogranuloma venereum

M.

Treponema pallidum (syphilis)

N.

Cervical polyps

O.

Gardnerella vaginalis

P.

Cervical Microglandular Hyperplasia

A

Treponema pallidum (syphilis)

1237
Q

A young lady is found to have chronic irritation and inflammation of the vulva. A pap smear and use of a silver stain reveals fungi within the keratin layer and superficial epithelium.

A.

Neisseria gonorrheae

B.

Trichomonas vaginalis

C.

Herpes zoster

D.

Cervical Intraepithelial Neoplasia I

E.

Herpes simplex

F.

Cervical Intraepithelial Neoplasia III

G.

Cervical Intraepithelial Neoplasia II

H.

Granuloma Inguinale

I.

Candida albicans

J.

Group B Streptococcus

K.

Chlamydia

L.

Lymphogranuloma venereum

M.

Treponema pallidum (syphilis)

N.

Cervical polyps

O.

Gardnerella vaginalis

P.

Cervical Microglandular Hyperplasia

A

Candida albicans

1238
Q

The metaplasia that occurs in the transformation zone involves which cell-types?

A

Glandular to squamous epithelium

1239
Q

A 27 year old woman undergoes a routine Pap test. Histology shows increased squamous epithelium with atypical cells showing koilocytosis (nuclear enlargement with perinuclear halo = clear area around nucleus). Follow-up colposcopy shows hyperchromatic nuclei present in the lower 1/3 of the epithelial layer from the basement membrane.

A.

Invasive cervical cancer

B.

CIN I

C.

Squamous to glandular epitheliem

D.

50-60 years

E.

CIN III

F.

45 years

G.

Glandular to squamous epithelium

H.

Ovarian cancer

I.

65 years

J.

30 - 40 years

K.

CIN II

1240
Q

22 year old lady presented for her routine smear test. The test showed a slight increase in DNA staining, and also a slightly larger variation in size of nuclei. Further questioning revealed she was a single mother living with her boyfriend, and had had multiple sexual partners since her first sexual contact at age 16.

A.

Group B Streptococcus

B.

Cervical Microglandular Hyperplasia

C.

Lymphogranuloma venereum

D.

Cervical Intraepithelial Neoplasia III

E.

Trichomonas vaginalis

F.

Neisseria gonorrheae

G.

Cervical Intraepithelial Neoplasia I

H.

Mild dyskaryosis

I.

Herpes simplex

J.

Granuloma Inguinale

K.

Gardnerella vaginalis

L.

Treponema pallidum (syphilis)

M.

Severe dyskaryosis

N.

Chlamydia

O.

Candida albicans

A

Mild dyskaryosis

1241
Q

Single most useful first-line cytological investigation for the confirmation of the benign status of an ovarian cyst.

A.

Exfoliative (brush) cytology

B.

Cystoscopy

C.

Cone biopsy

D.

Endometrial tissue sampling

E.

Percutaneous FNA biopsy

F.

Stereotactic radiographic cytological sampling method

G.

Fine needle aspirate

H.

Core biopsy

I.

Fluid cytology of alveolar washings

A

Core biopsies give HISTOLOGY Fine needle aspirates give CYTOLOGY - cannot assess basement membrane penetration (ie invasion)

FNA

1242
Q

Has a 100% positive predictive value for malignant cytopathological diagnosis.

1243
Q

What is the importance of measuriny urinary calcium in ?hyperPTHism

A

Familial hypocalciuric hypercalcaemia (FHH) is a dominantly inherited condition caused by a mutation of the calcium sensing receptor. The result is that the kidney reabsorbes calcium very avidly as a primary problem. This results in hypocalciuria and hypercalcaemia. Because the parathyroid gland calcium receptor also does not work, the parathyroids continue to secrete excess PTH despite the high calcium, so it looks just like primary hyperparathyroidism. However removing the parathyroids does not help the calcium, as the primary cause of trouble is in the kidney. In true primary hyperparathyroidism however, the calcium that is filtered hugely exceeds the tubular maximum for calcium, so there is no chance of reabsorption, so the urinary calcium is high, with resulting renal stones (which NEVER happens in FHH). There are many surgeons who have operated wrongly on FHH patients, removing the parathyroid only to find the calcium does not get better. As a consequence, we have written guidelines to surgeons, reminding them of the importance of measuring urine calcium in such a patient before removing the parathyroid gland.

1244
Q

70 year old man presents to his GP with a four day history of haemoptysis. He has noticed he has been loosing weight over the last 4 months and has felt tired and unwell. On examination he has bilateral ptosis and proximal weakness in the limbs which improves on repeated testing — Correct answer: Small cell carcinoma why is this Small Cell Carcinoma?

A

This guy has Myasthenic Syndrome Eaton-Lambert syndrome which occurs in ~3% of people with small cell l ung cancer. In contrast with myasthenia gravis, the fatigue gets BETTER with repeated use of the muscle.

1245
Q

What differentiates between autoimmune and De Quervain’s thyroiditis

A

Autoimmune tend to be painless

1246
Q

endarteritis obliterans in the context of STIs=

A

Treponema pallidum

1247
Q

An 86 year old man is admitted to A&E having collapsed at his home. He is unconscious and a couple of days later he is still deeply unconscious. His pupils are pin-point and their reaction to light is difficult to see clearly

A.

Vascular dementia

B.

Cerebellar stroke

C.

Opiate overdose

D.

Transient Ischaemic attack

E.

Cerebral embolus

F.

Subarachnoid haemorrhage

G.

Panic attack

H.

Pontine haemorrhage

I.

Subdural haemorrhage

J.

Temporal arteritis

K.

Transient hypotension

L.

Brain stem infarction

M.

Extradural haemorrhage

N.

Raised intracranial pressure

O.

Alzheimer’s disease

P.

Meningitis

A

Brain stem infarction

1248
Q

Occlusion of this cerebral vessel can cause weakness and numbness in the contralateral lower limb and similar but milder symptoms in the contralateral upper limb.

A.

Anterior cerebral artery

B.

Lacunar infarcts

C.

Middle cerebral artery

D.

Berry’s aneurysm

E.

Vertebrobasilar circulation

F.

Extradural haemorrhage

G.

Bacterial meningitis

H.

Hydrocephalus

I.

Amyloidosis

J.

Viral meningitis

K.

Subarachnoid haemorrhage

L.

Posterior cerebral artery

M.

Stroke

N.

Transient ischaemic attack (TIA)

1249
Q

Transtentorial herniations can potentially compromise the sufficiency this particular part of the cerebral circulation and cause occipital lobe infarction.

A.

Anterior cerebral artery

B.

Lacunar infarcts

C.

Middle cerebral artery

D.

Berry’s aneurysm

E.

Vertebrobasilar circulation

F.

Extradural haemorrhage

G.

Bacterial meningitis

H.

Hydrocephalus

I.

Amyloidosis

J.

Viral meningitis

K.

Subarachnoid haemorrhage

L.

Posterior cerebral artery

M.

Stroke

N.

Transient ischaemic attack (TIA)

1250
Q

5 year old obese woman goes to GP about worsening headaches. They are especially bad in the mornings and she also feels nauseous with some visual disturbances.

A.

Subdural haemorrhage

B.

Trigeminal neuralgia

C.

Glaucoma

D.

Migraine

E.

Berry aneurysm

F.

Subarachnoid haemorrhage

G.

Tension headache

H.

Sinusitis

I.

Benign intracranial hypertension

1251
Q

An elderly diabetic lady with an acute headache and associated blurred vision and vomiting. Pain is localised to the upper anterior region of her head.

A.

Subdural haemorrhage

B.

Trigeminal neuralgia

C.

Glaucoma

D.

Migraine

E.

Berry aneurysm

F.

Subarachnoid haemorrhage

G.

Tension headache

H.

Sinusitis

I.

Benign intracranial hypertension

1252
Q

A 60 year old lady presents with a hard palpable mass in the right breast. The mammography shows that the lump is calcified. FNA results show that the cells in the lump have pleomorphic nuclei and that the lump has a necrotic centre

A.

Invasive Carcinoma

B.

Gyaecomastia

C.

Lobular Carcinoma in situ

D.

Pagets Disease of the nipple

E.

Duct Papilloma

F.

Nipple adenoma

G.

Lymphoma

H.

Fibroadenoma

I.

Ductal Carcinoma in situ

A

Ductal Carcinoma in situ

1253
Q

A 23 year old lady comes to the breast clinic because she is worried of having breast cancer as her mother and grandmother both have had the disease. The mammogram finds no abnormality but you think you feel a number of multifocal lumps

A.

Invasive Carcinoma

B.

Gyaecomastia

C.

Lobular Carcinoma in situ

D.

Pagets Disease of the nipple

E.

Duct Papilloma

F.

Nipple adenoma

G.

Lymphoma

H.

Fibroadenoma

I.

Ductal Carcinoma in situ

A

Lobular Carcinoma in situ

1254
Q

A 45 year old lady is referred to the breast clinic with nipple retraction

A.

Invasive Carcinoma

B.

Gyaecomastia

C.

Lobular Carcinoma in situ

D.

Pagets Disease of the nipple

E.

Duct Papilloma

F.

Nipple adenoma

G.

Lymphoma

H.

Fibroadenoma

I.

Ductal Carcinoma in situ

A

Invasive Carcinoma

1255
Q

A 30 year old woman is referred to the breast clinic with a red, roughened and ulcerated nipple.

A.

Invasive Carcinoma

B.

Gyaecomastia

C.

Lobular Carcinoma in situ

D.

Pagets Disease of the nipple

E.

Duct Papilloma

F.

Nipple adenoma

G.

Lymphoma

H.

Fibroadenoma

I.

Ductal Carcinoma in situ

A

Pagets Disease of the nipple

1256
Q

A 56-year old female presents with a palpable mass in her left breast. Radiology shows microcalcification.

A.

Fine needle aspirate

B.

BRCA1 gene

C.

Hormone replacement therapy

D.

Paget’s disease of the nipple

E.

Carcinoma-in-situ (lobular)

F.

Nulliparity

G.

Axillary lymph node metastases

H.

Fibroadenoma

I.

Carcinoma-in-situ (ductal)

J.

Invasive breast carcinoma

K.

Oestrogen-receptor positivity

A

Fibroadenoma

1257
Q

involves focal calcification of the
media of small medium-sized arteries. It usually presents in patients
over 50 years of age and unlike atherosclerosis, there is no associated
inflammation in the pathogenesis.

A

Monckeberg arteriosclerosis

1258
Q

What differentiates dermatomyositis from MG

A

Sparing of the ocular muscles

1259
Q

45-year-old man is referred to the gastroenterology outpatient clinic due to
severe epigastric pain and an episode of haematemesis. Further testing reveals
he is Helicobacter pylori positive and has a 20 pack–year history of smoking.

A

Peptic ulcer disease (I) can either be duodenal or gastric. Ulcers differ from
erosions as they extend to the submucosa (sometimes to the muscularis
mucosa) and take weeks to heal, whereas erosions breach the mucosa only
and take days to heal. The main causes of peptic ulcers are Helicobacter
pylori, NSAIDs, Zollinger–Ellison syndrome and smoking. These factors
disrupt the balance between protective (mucus layer and bicarbonate secretion)
and damaging (acid and enzymes) elements leading to ulceration.

1260
Q

A 56-year-old woman is investigated by the hepatology team for decompensated
liver disease. A liver biopsy sample stains blue with Perl’s Prussian blue stain.

A Cholangiocarcinoma
B Cirrhosis
C α1-Antitrypsin deficiency
D Haemosiderosis
E Primary biliary cirrhosis
F Haemochromatosis
G Hepatocellular carcinoma
H Primary sclerosing cholangitis
I Wilson’s disease

A

Haemochromatosis (F) is an autosomal recessive condition that is due to
a mutation in the HFE gene. The HFE protein regulates iron absorption
that is stored as haemosiderin. Histological features of haemochromatosis
include a golden-brown haemosiderin deposition in the parenchyma
of many organs. Haemosiderin eventually leads to inflammation and subsequent fibrosis. Histological samples of affected tissue will stain blue
with Perl’s Prussian blue. Organs affected include the liver (cirrhosis),
pancreas (diabetes), skin (bronzed pigmentation), heart (cardiomyopathy)
and gonads (atrophy and impotence).

1261
Q

With what is PBC strongly associated?

A

Sjogren’s

1262
Q

Histological features include periductal fibrosis that eventually
invades the lumen causing concentric onion-ring fibrosis.

1263
Q

A 54-year-old man is referred to the dermatologist with a brown warty lesion
on his nose which has a rough consistency. Biopsy of the lesion reveals solar
elastosis.

A Pemphigoid
B Bowen’s disease
C Pityriasis rosea
D Lichen planus
E Actinic keratosis
F Psoriasis
G Basal cell carcinoma
H Erythema multiforme
I Malignant melanoma
J Pemphigus

A

Actinic keratosis

1264
Q

begins as a single scaly macule that is salmon-pink,
<5 cm and known as a ‘herald patch’. After several days multiple small
pink rashes appear posteriorly creating a fir-tree pattern.

A

Pityriasis rosea

1265
Q

involves the following: haematuria, red cell and
white cell casts, dysmorphic red cells, oliguria and hypertension.

A

Nephritic syndrome

1266
Q

form as a result of Tamm–Horsefall secretions
in the distal collecting duct and collecting duct that ‘glue’ cells
together,

A

Cellular casts

1267
Q

it mimics breast cancer; the presentation
may be nipple retraction due to fibrosis and bloody discharge secondary
to rupture of the ducts.

A

Duct ectasia

1268
Q

usually
occurs mainly in pre-menopausal women and is bilateral and multifocal.
No calcification occurs and hence there is no detection on mammogram.
Histologically there is no necrosis and uniform nuclei are present.

A

Lobular carcinoma in situ

1269
Q

occur in pre- or post-menopausal
women. They are usually unilateral and unifocal. On mammogram,
microcalcification may be visible secondary to central necrosis.

1270
Q

Histological investigation reveals an ‘artichoke-like’
appearance as the stroma pushes up on the epithelium to form clubs.

A

Phylloides tumour

1271
Q

has a scirrhous look
whereby the centre is very fibrous giving a dense white appearance.

has the worst prognosis compared to all other invasive carcinomas (medullary,
mucinous, tubular and papillary carcinoma).

A

Infiltrating ductal carcinoma

1272
Q

A 35-year-old man with pain and difficulty bending his left knee. X-ray reveals
many lytic lesions in the epiphysis of the patient’s left knee.

A Osteoporosis
B Fibrous dysplasia
C Paget’s disease
D Osteomalacia
E Osteochondroma
F Osteoid osteoma
G Renal osteodytrophy
H Enchondroma
I Giant cell tumour

A

Giant cell tumour (GCT; I) is a borderline malignant tumour of giant osteoclast
cells. The cells are similar to those found in Paget’s disease as they
have multiple nuclei (>20). The osteoclastic cells cause lytic lesions in the
epiphyses (especially around the knee) that are visible on X-ray and may
give a characteristic ‘soap bubble’ appearance. Histological features include
multinucleated giant osteoclasts with surrounding ovoid and spindle cells.

1273
Q

Histological features include
multinucleated giant osteoclasts with surrounding ovoid and spindle cells.

visible on X-ray and may
give a characteristic ‘soap bubble’ appearance

A

Giant cell tumour

1274
Q

An 8-year-old boy has been diagnosed with precocious puberty. A routine
examination by the paediatrician reveals café-au-lait spots on the child’s back.
The boy has had numerous fractures of his femur and tibia bilaterally after falls.

A Osteoporosis
B Fibrous dysplasia
C Paget’s disease
D Osteomalacia
E Osteochondroma
F Osteoid osteoma
G Renal osteodytrophy
H Enchondroma
I Giant cell tumour

A

Fibrous dysplasia (B) occurs due to the developmental arrest of normal
bone structures secondary to an osteoblast maturation defect. The most
common sites affected are the proximal femur and ribs. On X-ray,
fibrous dysplasia may cause a ground-glass or soap bubble appearance.
Histological investigation reveals trabeculae that lack osteoblastic
rimming. Two possible syndromes can arise: 1) Mono-ostotic (70
per cent) affecting femurs more than ribs occurring in patients under
30 years of age, and 2) McCune–Albright syndrome (30 per cent) that
is poly-ostotic and causes café-au-lait spots and precocious puberty.

1275
Q

A 57-year-old overweight patient suffers an acute myocardial infarction and
subsequently dies. A post-morterm examination of the infarcted area shows
extensive cell infiltration including polymorphs and macrophages. There is also
extensive debris post necrosis and the cytoplasm is homogeneous making it
difficult to see the outlines of the myocardial fibres. There is no evidence of
collagenization or a scar. How long after the initial attack did the patient die?
A At the time of the attack (0–6 hours)
B Hours after the attack (6–24 hours)
C Days after the attack (1–4 days)
D Within the first 2 weeks of the attack (4–14 days)
E Weeks and months after (14 days +)

A

C In the first 6 hours (A) in the evolution of an acute myocardial infarction
(MI), the histology is normal. Necrotic cell death takes place between
6 and 24 hours (B). Pathologically, there is contraction band necrosis
(dark red/pink wavy lines extending across the myocardial fibres), loss of
nuclei in the myocardial cells and the start of a homogeneous appearing
cytoplasm. At 1–4 days (C) following an acute MI, the start of an extensive
acute inflammatory response takes place with cell infiltration. Debris
is left by the necrosis in the previous stage and the cytoplasm is homogeneous
so that it is difficult to see the outlines of the myocardial fibres.
Infiltration of polymorphs, and later macrophages, takes place. Removal
of the debris takes place at about 5–10 days. At approximately 2 weeks
(D), the area undergoes repair and a classic young scar with new capillaries
is seen with early collagenization. Macrophages and myofibroblasts
are present in large numbers. Some granulation tissue and collagen synthesis
will continue on to the next stage. In the months (E) following an
acute MI, the area starts to strengthen with the formation of a decellularizing
scar. This established scar will be seen as a pale white collagenized
area within the interstitium between myocardial fibres.

1276
Q

A 41-year-old man presents with severe central chest pain which he describes as
‘tearing’ in nature and radiating to the back. He is tall, with long limbs and long
thin fingers. He also has an aortic regurgitation murmur. Histologically there is
cystic medial necrosis in the aortic wall. In which syndrome are these findings
most likely?
A Ortner’s syndrome
B Ehlers–Danlos syndrome
C Down syndrome
D Turner syndrome
E Marfan syndrome

A

Cystic medial necrosis is a disorder particularly affecting the aorta,
causing focal degeneration of the elastic tissue and muscle fibres in the
media, with accumulation of basophilic ground substance. This leads
to cyst-like pools between the fibres disrupting the normally parallel
arrays. Clinically, aneurysm formation becomes more likely. It is
more frequent after 40 years of age and is twice as common in males.
There is evidence that links cystic medial necrosis to aortic dissection
in patients with a variety of syndromes, the most common of which is
Marfan’s syndrome (E). These patients are characteristically tall with
long limbs and long thin fingers. All other syndromes are inconsistent
with this patient. The most common murmur in Ortner’s syndrome (A)
is mitral stenosis, associated with an enlarged left atrium and recurrent
laryngeal nerve palsy. Turner’s syndrome (D) is only present in females.
Congenital heart defects include: aortic coarctation, aortic stenosis,
ventricular septal defect and atrial septal defect, but aortic dissection is
uncommon. In Ehlers–Danlos syndrome (B), cystic medial necrosis can
cause fragile blood vessels but aortic regurgitation is not commonly

present. Down syndrome (C) patients may have congenital atrioventricular
septal canal defects. Other causes of cystic medial necrosis in patients
without Marfan’s syndrome are advanced age and chronic hypertension.

1277
Q

The activity of the plaques in a 25-year-old multiple sclerosis patient is described
with the presence of oedema and macrophages, and some myelin breakdown.
Which ICDNS (International Classification of Diseases of the Nervous System)
plaque type classification best fits the description?
A Acute plaque
B Early chronic active plaque
C Late chronic active plaque
D Chronic inactive plaque
E Shadow plaque

A

Multiple sclerosis (MS) is the leading cause of disability in Western
countries among young individuals between 20 and 40 years of age.
The pathological hallmark of MS is the presence of demyelinating
plaques on MRI scanning of the central nerves (spinal cord and brain).
Particular things to look for are myelin loss, destruction of oligodendrocytes,
and reactive astrogliosis, often with relative sparing of the axon
cylinder until later stages. The number of plaques, which range from a
few to several hundreds, is indicative of the severity of the disease.
According to the activity, the International Classification of Diseases
classifies plaques as follows;
Acute plaque (A): Minor changes (e.g. oedema) and often difficult to
recognize
Early chronic active plaque (B): Oedema and macrophages, indicative of
an inflammatory disorder of the central nervous system, with some myelin
breakdown. Reactive astrocytosis is present
Late chronic active plaque (C): Complete loss of myelin. Some macrophages
will contain myelin debris and there will be often very mild perivascular
inflammation at this stage with enlarged perivascular spaces
Chronic inactive plaque (D): Complete loss of myelin with the absence of
macrophages
Shadow plaque (E): Nearly complete remyelination as a thin myelin with some
scattered macrophages and a mild microglial up-regulation.

1278
Q

s a degenerative disease with
cell death of the dopamine-producing neurons of the substantia nigra,
resulting in depigmentation. Furthermore, patients with this condition
are alpha-synuclein and ubiquitin positive.

A

Parkinson’s disease

1279
Q

characteristically
shows cerebral atrophy in the caudate nucleus and putamen and
several changes in neurotransmitters.

A

Huntingtons

1280
Q

is associated
with glial cytoplasmic inclusion bodies (i.e. Papp–Lantos bodies).

1281
Q

is associated with
pernicious anaemia in the elderly and typically affects the body of the
stomach.

A

Autoimmune chronic gastritis

1282
Q

the dominant feature is the epithelial
change with minimal inflammation that can be idiopathic or due to reflux of bile-containing duodenal fluid or drugs

A

Reactive gastritis

1283
Q

is the most common form of
chronic gastritis, accounting for 90 per cent of cases, and it is known
that the pyloric antrum is the most severely affected area.

A

H pylori associated gastritis

1284
Q

is a rare disease characterized by gross
hyperplasia of gastric pits and a marked increase in mucosal thickness
and typically affects the fundus and body of the stomach.

A

Menetrier’s disease

1285
Q

Ninety to ninety-five per cent
of patients are HLA DR3 and HLA DR4 positive.

1286
Q

A 39-year-old man is diagnosed with a colon cancer proximal to the splenic
flexure that is poorly differentiated and highly aggressive. There are no associated
adenomata. It is an autosomal dominant condition that involves gene mutations
of DNA mismatch repair genes. What is the most likely diagnosis?
A Familial adenomatous polyposis
B Gardner’s syndrome
C Colorectal carcinoma
D Hereditary non-polyposis colorectal cancer
E Hamartomatous polyps

A

18 D Hereditary non-polyposis colorectal cancer (HNPCC) (D) is an uncommon
autosomal dominant disease but the cancers are poorly differentiated and
highly aggressive, therefore screening for identification of carriers for surveillance
is necessary. Familial adenomatous polyposis (FAP) (A) is also
a rare autosomal dominant condition that is caused by a mutation in the
FAP gene on chromosome 5. It is characterized by the presence of adenomata
in the large bowel, which this patient did not have, yet also carries a
90 per cent risk of developing into carcinoma by the age of 45. Gardner’s
syndrome (B) is similar clinically, pathologically and aetiologically to FAP
and also carries a high carcinoma risk. However, there are very distinct
extra-intestinal manifestations of Gardner’s syndrome, including multiple
osteomas of the skull and mandible, epidermoid cysts and desmoid
tumours. Colorectal carcinoma (C) of the sporadic type is inconsistent
with the history and age of the patient, as it is more commonly present
in patients over the age of 60. Hamartomatous polyps (E) often present
in childhood or adolescence as part of Peutz–Jeghers syndrome and these
carry a small risk of carcinomas. Patients also tend to have pigmented
lesions around the mouth that are characteristic of this condition.

1287
Q

A 32-year-old woman presents with generalized fatigue. Full blood count shows
a reduced haemoglobin level and reduced mean corpuscular volume. A peripheral
blood film has revealed iron deficiency anaemia. What features are most likely to
be seen on her peripheral blood film?
A Hypochromic and microcytic red blood cells with anisopoikilocytosis and
acanthocytes
B Hypochromic and microcytic red blood cells with hypersegmented
neutrophils
C Hypochromic and microcytic red blood cells with anisopoikilocytosis and
no evidence of basophilic stippling
D Hypochromic and microcytic red blood cells with Howell–Jolly bodies and
basophilic stippling
E Hypochromic and macrocytic red blood cells with target cells, acanthocytes
and Howell–Jolly bodies

A

C Features of iron deficiency anaemia are hypochromic (pale) and
microcytic (small) red blood cells. Poikilocytes are red blood cells
that are abnormally shaped. When there are variations in shape and
size, it is known as anisopoikilocytosis. Basophilic stippling (aggregation
of ribosomal
material) is absent in iron deficiency and present in
b-
thalassaemia trait and lead poisoning.
In megaloblastic anaemia, there is impaired DNA synthesis and this can
be caused by B12 deficiency, folate deficiency and drugs. Here the features
are the characteristic hypersegmented neutrophils and macrocytic
red blood cells.
In hyposplenism, there is presence of target cells known as codocytes
(red blood cells that have a high surface area:volume ratio).
Acanthocytes (spiculated blood cells/spur cells) and Howell–Jolly bodies
(nuclear remnants visible in red cells) are also present in the hyposplenism
picture.

1288
Q

A 27-year-old woman has developed pain in her right proximal femur. She has
a history of intermittent hip pain since childhood. An X-ray has demonstrated a
‘soap bubble’ appearance indicative of osteolysis and a characteristic shepherd’s
crook deformity. The biopsy would show irregular trabeculae of woven bone said
to resemble Chinese letters. What is the most likely diagnosis?
A Non-ossifying fibroma
B Fibrous dysplasia
C Giant cell reparative granuloma
D Ossifying fibroma
E Simple bone cyst

A

24 B Fibrous dysplasia (B) is a benign disorder of children and young adults,
whereby lesions composed of fibrous and bony tissue develop usually
in the ribs, femur, tibia or skull. It mostly presents as bone pain
and weakness in female patients under 30 years of age and results
from congenital dysplasia of bone, consistent with the patient’s history.
There are three forms: the more common monostotic form
(lesions localized to only one bone), polyostotic (multiple lesions) and
McCune–Albright syndrome, which also has endocrine manifestations.
Shepherd’s crook deformity refers to a varus angulation of the proximal
femur commonly seen in the femoral involvement of polyostotic
fibrous dysplasia. Histologically, it is characterized by loose fibrous tissue
with metaplastic immature or woven bone trabeculae arranged in a
‘Chinese letters’ formation.
Non-ossifying fibroma (A) is often asymptomatic and merely an incidental
finding on X-ray and occurs in an even younger group of
patients. Ossifying fibroma (D) is a benign, fibrous tumour with reactive
bone formation that shows local aggressive behaviour. Lesions are
found in the mandible for adults, and the tibia for children. Giant-cell
reparative granuloma (C) is an uncommon benign reactive intraosseous
lesion and can occur in the skull, jaw, hand and foot. The histology
resembles giant cell tumour of bone (see below). A simple bone cyst (E)
is common in the proximal metaphysis of the humerus and asymptomatic
unless there has been a fracture.

1289
Q

A 36-year-old man presents with swelling of his middle finger and subsequently
a fracture. His X-ray shows cotton wool calcification and histopathology shows
evidence of a tumour composed of benign hyaline cartilage. It is believed that he
has only a very slight risk of malignant transformation. What is the most likely
diagnosis?
A Osteochondroma
B Multiple myeloma
C Osteoid osteoma
D Giant cell tumour
E Enchondroma

A

25 E Enchondroma (E) is a benign intramedullary cartilage tumour usually
found in the central mature hyaline cartilage of the short tubular bones
of the hands and feet. It may present at any age (average is 40 years)
and is often asymptomatic, but some patients present with pain, fracture
or swelling of the affected area. There are lytic lesions on X-rays that
usually contain variably calcified chondriod matrix and histopathological
findings showing bluish-grey lobules of hyaline cartilege. There may
be a thin layer of lamellar bone surrounding the cartilage nodules but
no permeation of pre-existing host bone which is a positive sign that the
lesion is benign. Each potential enchondroma needs to be evaluated on
imaging and histology to distinguish it from low-grade chondrosarcoma.
Osteochondroma (A) is the most common skeletal neoplasm, often affecting
boys in their teens and often found in the long bones. When the
growth plates close in late adolescence, there is usually no further growth
of the osteochondroma. There is a low risk of malignancy in osteochondromas
associated with syndromes that involve multiple lesions such as
Ollier’s disease or Maffucci’s syndrome. Osteoid osteoma (C) is a small,
benign lesion surrounded by a zone of reactive bone. It mostly occurs in
the long bones, with a very distinct clinical picture in that pain is dull,
worse at night and relieved by aspirin. Similar radiology and histology
are seen in osteoblastomatomours that are larger than 1 cm diameter and
that commonly affect the vertebrae. Pathological fracture is a common
presentation of giant cell tumour (D), which is more common in females,
is locally aggressive and can metastasize. It does not occur in the immature
skeleton. It is more common in the metaphysics of the long bones,
especially the distal femur and proximal tibia. There is no calcification
on X-ray and, histologically, there would be numerous multinucleated
giant cells. The mono-nuclear cell population is the neoplastic cells.
Multiple myeloma (B) is highly unlikely as it is a different classification
of bone tumours altogether and is considered as a blood cell malignancy
rather than a primary bone tumour. It is a malignant tumour of plasma
cells that causes widespread osteolytic bone damage. It may be in are
bone only when it is known as plasmacytoma rather than myeloma.

Year 5: Pathology (6 decks)

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