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PACES 2024 > Station 4 Abdomen > Flashcards

Station 4 Abdomen Flashcards

1
Q

How to complete your examination in abdo exam with gross ascites?

A
  • cardio: raised JVP with steep x and y descent, early S3 suggestive of constrictive pericarditis
  • Urine dipstick for proteinuria
  • Temperature chart for fever (TB)
  • Rectal examination for a rectal mass
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2
Q

What is ascites

A

Pathologically accumulation of fluid in the peritoneal cavity

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3
Q

How much fluid must be present before there is flank dullness?

A

1.5 L of ascitic fluid

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4
Q

what are the causes of abdominal distension?

A

Fat, fluid, flatus, faeces, fetus and organ enlargement

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5
Q

How would you approach a patient with ascites clinically?

A

Abdominal exam

Liver
• Look for jaundice, spleen and stigmata of CLD - cirrhosis of liver
Liver palpable and smooth - think of Budd-chiari
Liver palpable and hard and nodular - think of malignancy

Kidneys
Look for evidence of kidney failure and anasarca

heart
Look for congestive cardiac failure or constrictive pericarditis

thyroid
Look for features of hypothyroidism

If all above absent, think of
• TB peritonitis
• Intra-abdominal malignancy
——-Carcinomatosis peritonei
—— Secondaries: Liver, Colon, ovaries, pancreas

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6
Q

Causes of ascites

A

Serum ascites albumin gradient >1.1g/dl = portal hypertension (97% accuracy)
- cirrhosis of liver
- Budd-Chiari
- CCF
- Constrictive pericarditis
- Malabsorption
- Meig’s syndrome
- Hypothyroidism

Serum ascites albumin gradient< 1.1g/dl
- Intra-abdominal malignancy
- TB
- Nephrotic syndrome
- Protein losing enteropathy

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7
Q

Causes of ascites with Serum ascites albumin gradient >1.1g/dl
Ie. portal hypertension (97% accuracy)

A
  • cirrhosis of liver
  • Budd-Chiari
  • CCF
  • Constrictive pericarditis
  • Malabsorption
  • Meig’s syndrome
  • Hypothyroidism
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8
Q

Causes of ascites with serum ascites albumin gradient <1.1g/dl

A
  • Intra-abdominal malignancy
  • TB
  • Nephrotic syndrome
  • Protein losing enteropathy
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9
Q

Pathophysiology of ascites in cirrhosis of the liver?

A
  • The chief factor is splanchnic vasodilatation
  • Cirrhosis leads to increased resistance to portal flow
  • Leading to portal hypertension
  • Portal hypertension results in local production of vasodilators, with splanchnic arterial vasodilatation

(1) Arterial underfilling
• Early stage - minimal effect on effective arterial volume as can be compensated by increase in plasma volume and cardiac output
• Later stage
- splanchnic vasodilation so marked that effectve arterial pressure falls and results in activation of vasoconstrictors and atrial natriuretic factors
- Sodium and fluid retention and expansion of plasma volume contributing to ascites
- Impaired free water execretion leading to dilutional hyponatraemia
- Renal vasoconstriction with hepatorenal syndrome

(2) Increase in splanchnic capillary pressure with lymph formation exceeding return therefore ascites

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10
Q

Ix of ascites

A

(liver, renal, heart, thyroid, TB)

labs: LFT, renal panel, TFT, FBC

ascitic tap:
- cell count, albumin, serum albumin ascitic gradient, send for microbiology and cytology

imaging:
US abdo/ CT to evaluate liver (Cirrhosis, budd chiari), kidneys
Echo and ECG
CXR (TB, pleural effusion)

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11
Q

ascitic tap to evaluate for ascites: what to send for

A

cell count, albumin, total protein concentration
cultures, AFB cultures
cytology

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12
Q

complications of ascitic tap?

A

<0.1%: haemoperitoneum, bowel perforation
1%: abdominal wall haematoma

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13
Q

management of patient with ascites secondary to liver cirrhosis?

A

treat the underlying cause
avoid alcohol or hepatotoxic medications

management of ascites:
general measures:
- salt restriction < 2g / day
- fluid restriction < 1L/day (for ascites, oedema with Na < 130)

  • diuretics (spironolactone and furosemide)
  • paracentesis (with albumin cover if >5L removed)
  • TIPSS (Transjugular intrahepatic portosystemic shunt; high rate of shunt stenosis up to 75% at 1 year)

Liver transplant

Manage other complications of cirrhosis

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14
Q

when to consider liver transplant in liver cirrhosis?

A

refractory ascites, hepatorenal syndrome, SBP

use MELD score to assign priority for liver tranplant (includes bilirubin, Cr, INR)

5 year survival rate for cirrhosis with ascites is 30-40% vs 70-80% post liver transplant

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15
Q

diagnosis of spontaneous bacterial peritonitis?

A

> 250 polymorphs / ml of ascitic fluid

  • commonly E coli, Klebsiella, pneumococci
  • occurs due to translocation of bacteria from intestinal lumen to LNs then bacteraemia
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16
Q

what features are more likely to suggest secondary peritonitis vs SBP?

A

such as peritoneal infections secondary to intraabdominal lesions, such as perforation of the hollow viscus, bowel necrosis, nonbacterial peritonitis, or penetrating infectious processes.

  • loculated infection or perforated viscus

fluid findings:
- > 1000 polymorphs
- LDH > upper limit of serum
- low glucose
- high protein > 1g/L
- CEA > 5ng/ml
- ALP >240u/L

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17
Q

treatment of spontaneous bacterial peritonitis?

A

3rd generation cephalosporin: ceftriaxone

IV Albumin to prevent hepatorenal syndrome

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18
Q

prevention of spontaneous bacterial peritonitis?

A

ciprofloxacin, norfloxacin

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19
Q

indications for SBP prophylaxis?

A

after 1 episode of SBP as recurrence as high as 70%/ year

in patients with acute variceal bleed

ascitic fluid protein concentration <1g/dl (controversial)

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20
Q

what does development of ascites in a patient with liver cirrhosis mean?

A

decompensation
- occurs in 50% of patients within 10 yrs of diagnosing compensated cirrhosis

poor prognosis
- only 50% survive beyond 2 years
- poor quality of life
- increased risk of infection and renal failure

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21
Q

features of ascites on abdominal examination

A
  • abdominal distension +/- everted umbilicus
  • positive fluid thrill with shifting dullness
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22
Q

important negatives to report on detecting ascites in abdominal examination?

A
  • no abdominal tenderness (SBP)
  • signs of chronic liver disease and decompensation (hepatic flap, jaundice)
  • signs of renal disease
  • signs of hypothyroidism
  • signs of malignancy
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23
Q

differentials to report as potential causes of ascites?

A

cirrhosis of liver, budd chiari syndrome
nephrotic syndrome
protein losing enteropathy
congestive cardiac failure
intra-abdominal malignancy or TB

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24
Q

what is cirrhosis of the liver?

A

defined pathologically
diffuse liver abnormality
fibrosis and abnormal regenerating nodules

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25
Q

causes of liver cirrhosis?

A

chronic ETOH ingestion
Non alcoholic fatty liver disease
Viral hepatitis - B and C
Cardiac failure

less common:
autominnue: autoimmune chronic active hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis

congenital: wilsons disease, hereditary haemochromatosis, alpha 1 AT deficiency, galatosemia, type 4 glycogen storage disease

haematological: haemolytic disease

vascular: budd-chiari

drugs: methotrexate, amiodarone, isoniazid

cryptogenic

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26
Q

drug causes of liver cirrhosis?

A

methotrexate, amiodarone, isoniazid

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27
Q

autoimmune conditions leading to liver cirrhosis?

A

autoimmune chronic active hepatitis,
primary biliary cirrhosis,
primary sclerosing cholangitis

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28
Q

vascular causes of liver cirrhosis?

A

budd chiari syndrome

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29
Q

congenital causes of liver cirrhosis

A

Wilsons disease,
hereditary haemochromatosis,
alpha 1 AT deficiency,
galatosemia,
type 4 glycogen storage disease

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30
Q

haemolytic causes of liver cirrhosis?

A

haemolytic disease

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31
Q

complications of cirrhosis?

A

portal hypertension: ascites, splenomegaly, varices

hepatorenal syndrome

hepatic encephalopathy

coagulopathy

HCC

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32
Q

features of coagulopathy in liver cirrhosis

A

low platelets, reduced clotting factors

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33
Q

stages of hepatic encephalopathy?

A

stage 1- depression, euphoria, sleep disturbance, normal eeg, may have asterixis

stage 2- lethargy, moderate confusion, asterixis present, abnormal eeg

stage 3- marked confusion, arousable, asterixis present, abnormal eeg

stage 4- coma, abnormal eeg

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34
Q

diagnosis of hepatorenal syndrome?

A

crcl<40
absence of other causes for renal impairment
absence of Cr improvement despite IV fluids, absence of proteinuria (0.5g/d), absence of haematuria (<50/hpf) and urinary Na < 10

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35
Q

types of hepatorenal syndrome?

A

type 1:
rapid deterioration in renal fn
ie. doubling of serum Cr in <2 wks to >221 umol/l

type 2: stable or slowly progressive that does not meet criteria for type 1

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36
Q

features of portal hypertension in liver cirrhosis?

A

ascites- tense ascites, SBP
splenomegaly-thrombocytopenia
varices- GI bleed

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37
Q

how do you stage cirrhosis of the liver?

A

child-pugh staging
- 5 parameters, score ranges 5 to 15
- prognostication

bilirubin (<34, 34-to 50, > 50 umol/l)
INR (<1.7, 1.7-2.3, > 2.3)
Albumin (>35, 28-35, <28)
ascites (mild, mod, severe)
encephalopathy (absent, I and II, III and IV)

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38
Q

child pugh scoring?

A

bilirubin (<34, 34-to 50, > 50 umol/l)
INR (<1.7, 1.7-2.3, > 2.3)
Albumin (>35, 28-35, <28)
ascites (mild, mod, severe)
encephalopathy (absent, I and II, III and IV)

A- 5 to 6 points (1 year 100%, 2 year 85%)
B: 7 to 9 (1 year 80%, 2 year 60%)
C: 10 to 15 (1 year 45%, 2 year 35%)

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39
Q

how to investigate liver cirrhosis?

A

confirming diagnosis: US or CT

establish aetiology: hepatitis screen, CAGE questionnaire (alcohol), liver biopsy in selected cases

prognosticate:
LFT - albumin, bilirubin
INR

complications:
endoscopy of GIT
mitotic change - US and AFP
evaluation of renal fn- urea, electrolytes, Cr
evaluation of ascitic fluid- cell count, SAAG, microbiology, cytology, AFB studies

evaluation for liver transplant

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40
Q

when should an abdominal paracentesis be done for a patient with ascites and cirrhosis?

A

newly diagnosed TRO SBP
symptomatic- fever, abdo pain, encephalopathy, GI bleed

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41
Q

management of liver cirrhosis?

A

education and counselling: regular f/u, stop ETOH drinking

manage underlying cause e.g. hep B/ C, alcoholic liver disease, NAFLD

manage complications

definitive treatment: ie. liver transplant

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42
Q

management of Hep C liver cirrhosis?

A
  • those at risk are IVDU and blood transfusions in the past

general measures
surveillance (HCC, screen for HIV)

treatment: direct acting antivirals (sofosbuvir and daclatasvir)

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43
Q

indications of treatment for hep C

A

HCV RNA levels (>50 IU/ml)
Raised ALT
Bx showing fibrosis and inflammation

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44
Q

management of hepatitis B

A

general measures (stop alcohol, hep A vaccination)

lifelong surveillance for HCC with US and AFP

antiviral for: (entecavir, tenofovir)
- immune clearance phase (HBeAg + ALT raised)
- reactivation phase (HBeAg -, ALT raised, HBV DNA raised)
- IFN alpha
- lamivudine

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45
Q

treatment of Alcoholic liver disease?

A

Maddrey’s discriminant function: for evaluating the severity and prognosis in alcoholic hepatitis and evaluates the efficacy of using alcoholic hepatitis steroid treatment.
- Treat with corticosteroids or total enteral nutrition (20-30kcal/kg/day)

**Alcohol cessation **

those with alcohol excess:
- >21u/w in males, >14u/w in females
- 100% of normal liver develops fatty liver
- 35% develop alcoholic hepatitis
- 20% develop cirrhosis
- 40% alcoholic hepatitis develop cirrhosis

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46
Q

using maddreys discrimination function to determine whether to treat with steroids in alcoholic liver disease?

A

PT x Bil x 4.6

> 32 = severe, so. treat with steroids

If less than 32, the patient is considered to have mild to moderate alcoholic cirrhosis and would likely not benefit from the use of steroids.

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47
Q

management of complications in liver cirrhosis?

A

hepatic encephalopathy: treat precipitants, prevent with low protein diet, lactulose, rifaximin

hepatorenal syndrome: treat with noradrenaline infusion, terlipressin or midodrine with octreotide plus albumin infusion for 5-15 days

ascites: furosemide, spironolactone

Upper GI bleed from varices: urgent endoscopy with ligation, banding, sclerotherapy, surgery
prevention with propranolol, variceal banding

hepatocellular carcinoma: surgery, transarterial chemoembolization, radiofrequency ablation

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48
Q

management of hepatic encephalopathy?

A

treat the acute precipitants

prevention:
low protein diet
lactulose - works by decreasing ammonia levels in blood
rifaximin - reduces ammonia production by eliminating ammonia-producing colonic bacteria

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49
Q

management of hepatorenal syndrome

A

vasoconstrictors - noradrenaline, terlipressin or midodrine in combination with octreotide

+ IV albumin infusion (1g/kg on D1 then 20-40g/day)

for 5-15 days

prevention (in patients with cirrhosis and ascites):
IV albumin

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50
Q

management of upper GI bleed

A

ABC approach

urgent endoscopy for banding/ ligation/ sclerotherapy

surgical management if above fails

prevention: propranolol to reduce HR by 25% or to 55-60bpm
variceal banding

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51
Q

management of HCC?

A

MDT approach
surgical mx
transarterial chemoembolization, radiofrequency ablation
palliative

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52
Q

definitive treatment for liver cirrhosis?

A

liver transplant
- MARS (molecular adsorbent recirculating system) dialysis as an interim measure before liver transplant

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53
Q

what are the factors precipitating decompensation?

A

infection - SBP, pneumonia, UTI
GI bleed
constipation
diuretics and electrolyte imbalance
diarrhoea and vomiting
sedatives
surgery

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54
Q

what are the nail changes of hypoalbuminaemia?

A

leukonychia (indicating albumin < 30g/dL)
muehrcke’s lines - transverse white lines

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55
Q

what are the causes of palmar erythema?

A

chronic liver disease
RA, thyrotoxicosis, polycythaemia
pregnancy, normal finding

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56
Q

what are the causes of anaemia in cirrhotic patients?

A

anaemia of chronic disease
Fe deficiency from GI bleed
haemolysis from hypersplenism
folate and B12 deficiency from poor nutrition

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57
Q

how many spider naevi should be present to be considered significant?

A

> 5

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58
Q

features/ signs of patient with primary biliary cholangitis as cause of chronic liver disease?

A

female middle age
chronic liver disease with:
pruritus
xanthelasma
generalised pigmentation
hepatosplenomegaly

assoc with sicca syndrome, arthralgia, raynauds, sclerodactyly and thyroid disease

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59
Q

stages of primary biliary cholangitis?

A

asymptomatic with normal LFTs (positive Abs)
asymptomatic with abnormal LFTs
symptomatic - lethargy and pruritus
decompensated

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60
Q

ix of primary biliary cholangitis?

A

cholestatic LFT: raised ALP
anti-mitochondrial antibodies - M2 Ab, IgM
lipid panel

other tests for chronic liver disease -LFT, INR
US abdomen

liver biopsy if diagnosis uncertain or to exclude concomitant autoimmune hepatitis/ NASH:
histology- granulomatous cholangitis

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61
Q

management of primary biliary cholangitis?

A

to prevent end stage liver disease and manage associated symptoms
offer patient support group

1st line: ursodeoxycholic acid
cholestyramine
fat soluble vitamins

if develops cirrhosis, manage accordingly
liver transplant

manage other co-existent autoimmune disease

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62
Q

clinical features of haemochromatosis as underlying cause of chronic liver disease?

A

male
slate-grey appearance, hepatomegaly

liver -cirrhosis, cancer
pancreas- DM
heart failure - CMP
pituitary dysfunction
pseudogout

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63
Q

suspecting haemochromatosis as cause of chronic liver disease, what to request for after clinical examination

A

urine dipstick - glucose (DM)
cardiovascular examination - may have cardiomyopathy
testicular examination - testicular atrophy (hypothalamic-pituitary dysfunction resulting in impaired gonadotropin secretion)

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64
Q

pathophysiology of hereditary haemochromatosis?

A

autosomal recessive
chromosome 6
HFE gene
increased Fe absorption with tissue deposition

  • classically seen in those of north european ancestry
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65
Q

ix of haemochromatosis?

A

raised ferritin and transferrin saturation
gene testing
liver biopsy

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66
Q

management of haemochromatosis?

A

counselling: avoid high iron diet,
avoid alcohol
avoid shellfish as they are susceptible to Vibrio vulnificus

genetic counselling

venesection: aim ferritin 20-30, Tsat < 50%

chelation therapy if phlebotomy intolerant or contraindication e.g. severe anaemia or heart failure

Liver transplant

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67
Q

differentials of generalised pigmentation?

A

liver - haemochromatosis (usu males), PBC (usu females)
addison’s
uraemia
chronic debilitating conditions e.g. malignancy
chronic haemolytic anaemia

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68
Q

clinical features of ulcerative colitis as cause of chronic liver disease?

A

skin - erythema nodosum, pyoderma gangrenosum
joint arthropathy - LL arthritis, AS, sacroiliitis
aphthous ulcers
ocular- iritis, uveitis, episcleritis

CLD; cirrhosis, chronic active hepatitis, primary sclerosing cholangitis, cholangiocarcinoma, metastatic colorectal cancer, amyloid

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69
Q

clinical features of Wilson’s disease as cause of chronic liver disease?

A

short stature

eyes:
- KF rings (due to deposition of Copper in descemet’s membrane; greenish yellow to golden brown pigmentation of limbus of cornea)
- sunflower cataract (copper deposits in the anterior and posterior capsule)

Extrapyramidal:
- tremor and chorea
- presents with difficulty writing and speaking in school

pseudogout

penicillamine complications:
- myasthenic: ptosis
- lupus: malar rash, small hand arthritis

request for urinalysis for glycosuria from proximal renal tubular acidosis

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70
Q

pathophysiology of wilsons disease?

A

autosomal recessive
chr 13
disorder of copper metabolism, defective copper excretion -> pathologic accumulation of cu in tissue

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71
Q

ix of wilson’s disease?

A

low serum caeruloplasmin
increased 24h urinary copper
liver biopsy - increased Copper deposition
genetic testing (can direct whether family screening is required)
MRI brain if neurological features

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72
Q

management of wilsons disease?

A

counselling: low Copper diet

Cu chelation agents:
penicillamine, trientine
zinc

liver transplant may be required

SE: myasthenia, drug induced lupus

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73
Q

clinical findings in chronic liver disease?

A

stigmata of chronic liver disease:
leukonychia, clubbing, palmar erythema, spider naevi, gynaecomastia, loss of axillary hair

decompensation:
ascites, portal hypertension, splenmegaly

complications:
asterixis to suggest hepatic encephalopathy
cachexia, enlarged Cx LNs (HCC)
conjunctival pallor (GI bleed with anaemia)

aetiology:
- alcohol: parotidomegaly, dupuytrens contracture
- hep b: tattoos,
- hep c: surgical scar with possibility of transfusion in past

treatment:
abdominal tap marks
sinus bradycardia indicating use of beta blockers

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74
Q

causes of massive splenomegaly (> 8 cm)?

A

CML
Myelofibrosis
Polycythaemia rubra vera
chronic malaria
kala azar (visceral leishmaniasis)
Others (Gauchers, rapidly progressive lymphoma)

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75
Q

causes of moderately enlarged spleen (4 to 8 cm/ 2-4 FB)?

A

myeloproliferative disorders
lymphoproliferative disorders
haematological - AI, ITP, thalassaemia, hereditary spherocytosis
chronic malaria
cirrhosis

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76
Q

causes of mildly enlarged spleen (4cm, < 2 FB)?

A

myeloproliferative/ lymphoproliferative disorders

infection:
viral - CMV, EBV
SBE, splenic abscesses, leptospirosis, meliodosis, TB, typhoid, brucellosis (farmer)
acute malaria

infiltrative - amyloidosis, sarcoidosis
endocrine: acromegaly, thyrotoxicosis
connective tissue - SLE, Feltys (RA + neutropenia + splenomegaly)
chronic haemolytic: thalassaemia, autoimmune HA, Hereditary spherocytosis, idiopathic thrombocytopenic purpura

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77
Q

causes of tender spleen?

A

infective causes: viral, bacterial, malaria
acute myeloproliferative and lymphoproliferative

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78
Q

causes of splenomegaly with pallor?

A

essentially same as moderately enlarged spleen causes:

myeloproliferative disorders
lymphoproliferative disorders
haematological - AIHA, thalassaemia
malaria
AI- Felty’s SLE
cirrhosis of the liver with portal hypertension

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79
Q

causes of splenomegaly with lymph nodes?

A

lymphoproliferative (CLL/ lymphoma)
infective (infectious mononucleosis, meliodosis, CMV, TB, HIV)

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80
Q

causes of massive liver?

A

HCC / secondaries/ myeloproliferative disorders
RV failure
alcoholic liver disease

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81
Q

causes of mild-moderate liver enlargement?

A

HCC / secondaries/ myeloproliferative disorders
RV failure
alcoholic liver disease

PLUS

infection:
viruses- EBV, CMV, hep A/B
bacteria- leptospirosis, meliodosis, abscesses, TB, brucellosis, syphilitic gumma
protozoal- hydatid cysts, amoebic abscess

malignancy- lymphoproliferative, myeloprolfierative, primary, secondary, adenoma from OCP

infiltrative- sarcoid (erythema nodosum, lupus pernio), amyloid, fatty liver

endocrine- amyloid, hyperthyroid

connective tissue disease

chronic haemolytic anaemia: AIHA, thalassaemia, hereditary spherocytosis

reidel’s lobe

possibility of minimal CLD signs with just hepatomegaly:
PBC
haemochromatosis

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82
Q

causes of tender liver?

A

liver abscess/ infective (viral, bacterial, parasitic)
HCC/ secondaries
right heart failure/ budd chiari

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83
Q

causes of pulsatile liver?

A

tricuspid regurgitation
HCC
AVM

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84
Q

causes of hard/irregular liver?

A

mitotic (primary/ secondary)
macronodular cirrhosis (post hep B/C, wilsons and A1AT deficiency)
amyloidosis, hydatid cyst, granulomatous disease, gummatous disease, APCKD

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85
Q

description of hepatosplenomegaly in abdo examination?

A

enlarged masses in the right and left hypochondrial regions of which I am unable to get above the masses

liver enlarged:
- size, edge, surface, consistency, tender, bruit, pulsatile

spleen enlarged:
size, edge, surface, consistency, tender

kidneys not enlarged and no associated ascites

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86
Q

what peripheral examination is important in hepatosplenomegaly?

A

CLD stigmata, jaundice, bruises
hepatic encephalopathy

causes:
- pallor, cachexia, Cx LNs, PRV
- toxic, rashes, tonsils (Infectious mono)
- chronic ETOH ingestion
- CCF
- SBE, SLE, RA, Haemolytic anaemia

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87
Q

ix of hepatomegaly?

A

according to most likely aetiologies

blood ix - diagnosis
imaging
liver biopsy

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88
Q

how to describe hepatomegaly?

A

size- cm from right costal margin
edge- regular/ irregular
surface- smooth/ nodular
consistency- soft, film, hard
tender
pulsatile
bruit

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89
Q

examination of peripheries if there is hepatomegaly?

A

jaundice, bruises, stigmata of CLD, oedema of LL
hepatic flap

causes:
- cachexia, cx LNs, conjunctival pallor
- dupuytrens contracture, parotidomegaly
- toxic looking, rashes, injected throat or enlarged tonsils
- CCD- presence of raised JVP

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90
Q

hepatomegaly:
how to complete examination?

A

check temperature chart for fever (if infective cause is a differential)
PR exam for masses (if secondaries are a differential)

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91
Q

what are the features of an enlarged spleen in contrast to an enlarged left kidney?

A

palpation
- unable to get above it
- notch border
- not bimanually palpable or ballotable

percussion note over the mass is dull from left 9th rub in mid axillary line extending inferior medially in the axis of the 10th rib

inspection shows that the mass moves inferior-medially with inspiration rather than inferiorly

auscultation may reveal splenic rub

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92
Q

is a normal spleen palpable or percussible?

A

palpable spleen implies that it is at least twice enlarged

a normal spleen can be percussed along the 9th, 10th, 11th rib but is not percussible beyond the anterior axillary line

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93
Q

splenomegaly on examination: what aetiology to examine for

A
  • cachexia, conjunctival pallor, cx LNs
  • polycythaemia - plethoric facies, conjunctival suffusion, bone marrow biopsy scar
  • toxic looking, rashes, enlarged tonsils
  • splinter haemorrhages, stigmata of IE
  • features of SLE, RA, chronic haemolytic anaemia
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94
Q

description of splenomegaly on examination

A

size: cm from left costal margin
palpable notch
edge- regular/ irregular
surface- smooth
consistency -firm
non tender
splenic rub

mention if any pallor/ lymphadenopathy

any associated hepatomegaly/ ascites/ ballotable kidneys

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95
Q

what is leukaemia?

A

accumulation of abnormal and immature WBCs in the bone marrow that spill into the blood resulting in bone marrow failure with pancytopenia, raised WCC and sometimes infiltration of other organs

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96
Q

what are myeloproliferative disorders?

A

group of clonal diseases arising from a defect in haemotopoietic stem cells
-classified according to the predominant cell lineage involved:
chronic myeloid leukaemia, polycythaemia, essential thrombocytosis, myelofibrosis

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97
Q

what is lymphoma?

A

replacement of normal LNs and lymphoid tissue with malignant proliferation of B or T lymphocytes

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98
Q

Reed sternberg cells

A

Hodgkin lymphoma

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99
Q

Tumour cells are plasma cells

A

multiple myeloma

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100
Q

Tumour cells have phenotype of B or T lymphocytes or their precursors?

A

Non Hodgkin lymphoma

-> tumour cells are mature: low-intermediate grade lymphoma
-> tumour cells are immature: high grade lymphoma

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101
Q

pathophysiology of acute leukaemia?

A

cells are dysplastic and immature
acquired somatic cell genetic event involving activation of oncogenes and loss of tumour suppressor gene

induced by radiation, viral infection, family hx, alkylating agents, down syndrome, wiskott-aldrich/fanconi anaemia

102
Q

types of acute leukaemia?

A

acute lymphoblastic leukaemia - precursor B ALL, B cell ALL (Burkitt), T Cell ALL (usu children)

acute myeloblastic/myeloid leukaemia - several subtypes based on genetics/ phenotype (Can be any age)

103
Q

presentation of acute leukaemia (signs/ symptoms)

A

bone marrow failure: anaemia, neutropenia with fever, thrombocytopenia with bruising/ bleeding

organ infiltration: tender bones, lymphadenopathy in ALL, hepatosplenomegaly, gum hypertrophy

104
Q

ix in acute leukaemia?

A

FBC: normochromic anaemia, high WBC with blasts, thombocytopenia
DIC screen, renal panel, LFT including LDH, uric acid

bone marrow aspirate: hypercellular with leukaemic blasts

cytochemistry, immunophenotyping, cytogenetics, DNA analysis, morphology for further subtyping

CXR for mediastinal mass (T-ALL)
LP and CSF

pre-chemo work up:
Hep B/C/HIV
G6PD
TTE
Dental clearance
HLA typing before starting chemotherapy

105
Q

treatment of acute leukaemia?

A

supportive: transfusion support, infection treatment and prophylaxis, managing complications

chemotherapy with induction, consolidation followed by allogenic bone marrow transplant

may need cranial prophylaxis for ALL

Hydrate well usually 2L/day

start allopurinol 300mg OM if no CI

106
Q

what is hodgkins lymphoma?

A

characterized by reed sternberg cells which are dysplastic B cells that have not undergone apoptosis
- related to EBV infection

107
Q

hodgkins lymphoma: clinical features?

A

lymphadenopathy
B symptoms: Fever, LOA/LOW, night sweats

108
Q

management of hodgkins lymphoma?

A

stage 1 and 2: RT
stage 3 and 4: chemotherapy e.g. ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)

109
Q

prognosis of hodgkin lymphoma?

A

5 year survival 70%

110
Q

what is non hodgkin lymphoma?

A

older age group, more common
proliferation of B/ T lymphocytes or their precursors largely confined to lymph nodes or bone marrow

risk factors: high fat diet, HIV, EBV

111
Q

non hodgkins lymphoma: clinical features?

A

lymphadenopathy, hepatosplenomegaly
involvement of GI/ CNS/ skin and BM failure

112
Q

management of non hodgkin lymphoma?

A

RT for local disease
single agent chemotherapy for low grade lymhoma e.g. chlorambucil
multiagent chemotherapy for aggressive disease

113
Q

prognosis of non hodgkin lymphoma?

A

aggressive disease
survival in 5 years - 50%

114
Q

what is chronic lymphocytic leukaemia?

A

commonest, mostly in males
due to accumulation of mature B lymphocytes in the bone marrow, liver, spleen, peripheral blood

disease of impaired apoptosis

115
Q

CLL; clinical features?

A

Lymphocytosis on FBC
symmetric lymphadenopathy
anaemia
bleeding/bruising
infections
hepatomegaly

116
Q

mx of CLL?

A

if asymptomatic, no need to treat. monitor

if symptomatic: corticosteroids, chlorambucil, cyclophosphamide, local RT

117
Q

ix of lymphoma?

A

blood ix
Lymph node or bone marrow biopsy
imaging: PET CT scan for staging, CXR

118
Q

what are the types of bone marrow transplant?

A

BM transplant, stem cell or cord blood transplantation

autologous or allogenic

119
Q

what is myelodysplasia?

A

preleukaemic condition occurring mainly in the elderly
stem cell disorder with dysplastic growth and development in all cell lineages

  • may be asymptomatic or present with unexplained anaemia/ later pancytopenia
  • blasts increased but usu < 20%
  • management supportive, in younger pts may consider allogenic BMT as they usually transform into acute laukaemia after 1-5 years
120
Q

what are B symptoms

A

fever >38
weight loss >10% over 6 months
drenching night sweats

121
Q

causes of hypersplenism?

A

vascular: splenic infarction from sickle cell anaemia, vasculitis, splenic artery thrombosis
infiltrative: amyloid, sarcoidosis
coeliac disease
autoimmune diseases

122
Q

what is polycythaemia rubra vera?

A

increase in Hb and Hct due to bone marrow proliferation
- erythropoiesis is autonomous and not dependent on erythropoietin

123
Q

polycythaemia rubra vera: clinical features?

A

hyperviscosity or hypermetabolism with headache, blurred vision, plethoric complexion
pruritus, sweats, splenmegaly, bleeding/ thrombosis

124
Q

ix of polycythaemia rubra vera?

A

diagnosis:
- increased red cell mass (Hb, Hct), normal SaO2
- EPO raised/ subnormal
- JAK2 V617F or Exon 12 positive
- bone marrow biopsy showing hypercellularity for age with panmyelosis, inclduing erythroid, granulocytic and megakaryocytic proliferation

may have raised plt, ALP, leukocytes, high B12

exclude secondary polycythaemia with high EPO e.g. COPD, altitude, cyanotic heart disease, smoking, RCC, renal cysts, uterine fibromyomas, HCC

If splenomegaly present: higher risk of transformation to myelofibrosis. exclude transformation to MF

125
Q

management of polycythaemia rubra vera?

A

maintain Hct <45% with venesection
if high risk: add hydroxyurea (myelosuppression) -> 2nd line: pegylated IFN-alpha, busulfan
Aspirin 100mg OM

if there is a hx of venous thrombosis, add systemic anticoagulation

126
Q

prognosis of polycythaemia rubra vera?

A

tendency to evolve:
30% to myelofibrosis
10-15% to acute leukaemia

median survival 10-15 years

main goal of therapy is to prevent thrombohaemorrhagic complications

127
Q

what is essential thrombocytosis?

A

proliferation of platelets leading to hyperviscosity
dysfunctional platelets leading to bleeding diathesis

128
Q

essential thrombocytosis: clinical features?

A

splenomegaly

129
Q

ix of essential thrombocytosis?

A

high plt (>450)
BM shows increased megakaryocytes

  • should not meet WHO criteria for CML, PV, PMF, MDS
  • presence of JAK2, CALR or MPL mutation (part of major diagnostic criteria)
  • presence of clonal marker or absence of evidence of reactive thrombocytosis (minor criterion)
130
Q

management of essential thrombocytosis?

A

hydroxyurea for cytoreduction
aspirin 100mg OM (start when plt<1000, to avoid in presence of extreme thrombocytosis)

if hx of venous thromboembolism, add anticoagulation

131
Q

prognosis of essential thrombocytosis?

A

very low risk of leukaemic transformation (<1% at 10 years)

0.8-4.9% risk of MF transformation at 10 years

risk of thrombosis is higher in JAK2 mutated ET cases
main goal of therapy is to prevent thrombohaemorrhagic complications

132
Q

what is myelofibrosis?

A

ineffective myeloid differentiation characterised by reactive bone marrow fibrosis that is de novo or 2’ ET/ PRV

133
Q

myelofibrosis; clinical features

A

elderly
presents with features of anaemia and massive splenomegaly due to extramedullary haematopoiesis, bleeding, gout

134
Q

ix of myelofibrosis?

A

leukoerythroblastic blood film
dry BM aspirate
fibrosis on trephine
- proliferation and atypia of megakaryocytes accompanied by either reticulin and/or collagen fibrosis

135
Q

management of myelofibrosis?

A

supportive with transfusion, splenectomy

136
Q

what is chronic myeloid leukaemia?

A

characterized by splenomegaly, leukocytosis, philadelphia chr (translocation chr 9;22 -> hybrid gene that codes for an active tyrosine kinase that mediates clonal proliferation)

137
Q

CML; clinical features

A

middle aged
presents with anaemia, splenomegaly, hypermetabolism e.g. gout

138
Q

ix of CML?

A

FBC, BMA, cytogenetics, low NAP score (neutrophil alk phosphatase)
PBF: high WBC, myeloid cells, basophils
hypercellular bone marrow

philadelphia chr +ve

139
Q

management of CML?

A

molecular targeted therapy
hydroxyurea as immunosuppressant
allogenic BMT

140
Q

prognosis of CML

A

3 phases: chronic, accelerated, blast crisis (acute leukaemia)
mean survival 3 years

141
Q

indications for splenectomy?

A
  • traumatic injury with splenic rupture
  • chronic haemolytic anaemia with severe anaemia, hypersplenism, abdo discomfort
  • ITP with severe thrombocytopenia not controlled by immunosuppression, abdominal discomfort
  • myeloproliferative disease with abdominal discomfort, severe hypersplenism
142
Q

management of patients post splenectomy?

A
  • vaccinations against encapsulated bacteria (meningococcus, streptococcus, haemophilus influenzae)
  • risk of thrombocytosis and thrombosis therefore usually post op start sc LMWH
  • prophylactic abx such as penicillin or erythromycin for > 2 yrs or lifelong
  • advice to report to hospital if ill, malaria prophylaxis, travel advice, medical alert bracelet
143
Q

what is thalassaemia?

A

characterised by reduced or absent production of one or more globin chains (alpha or beta, predominantly), resulting in a disruption of the ratio between alpha and non-alpha chains

144
Q

what is thalassaemia major?

A

almost all thal major patients have beta thal (not alpha thal)
defined by early age of onset, transfusion dependence

age of onset:
- beta thal major presents at 3-6 mos, some later
- beta thal intermedia usually presents later than 18 mos
- alpha thal intermedia usually presents at birth as alpha chains needed for both foetal and adult Hb

transfusion dependence:
- require regular blood transfusions usu once every 3-4 w
- thal intermedia patients receive transfusions when they are symptomatic or when Hb is low
- thal minor pts do not require transfusions

145
Q

what is thalassaemia intermedia

A

symptomatic thalassaemia but not requiring transfusion at least during first few years of life, and pts are able to survive into 2nd decade of life without chronic hypertransfusion therapy

146
Q

what is thalassaemia minor?

A

usually asymptomatic, mild MCHC anaemia
may have slight splenomegaly
not transfusion dependent

147
Q

what is alpha thalassaemia?

A

4 possible genotpes
2 genes encoding alpha globin chain on chr 16, so 4 alleles for alpha globin chain
-> deletion of one or more alpha globin genes

148
Q

features of alpha thal 2?

A

one mutant allele (aa/a-)
no anaemia
FBC, peripheral blood smear, Hb electrophoresis largely normal
may have slight hypochromia and/or microcytosis

149
Q

features of alpha thal 1 aka alpha thal minor?

A

2 mutant alleles: aa/– or a-/a-

PBF shows few coarse HbH inclusion bodies on BCB stain
mild MCHC anaemia
Hb electrophoresis normal - no HbH band seen
phenotypic features slightly worse in patients with aa/– configuration compared to a-/a- config

150
Q

features of HbH disease?

A

3 mutant alleles a-/–
clinically thal intermedia phenotype

haemolytic anaemia starts in the foetus,
newborns will be anaemic and jaundiced, occ with hydrops

PBF shows many HbH inclusion bodies on BCB stain (brilliant cresyl blue)- “golf ball” appearance
Hb electrophoresis shows a fast moving HbH band

151
Q

features of barts hydrops?

A

4 mutant alleles –/–

tetramer of gamma globin chains in the foetus
hydrops foetalis occurs due to high output cardiac failure,
foetus usually dies in late 2nd - mid 3rd trimester, or soon after birth

more common in asia

152
Q

features of beta thal minor or beta thal trait?

A

BB^0- absence of production of beta globin in one or BB^+ - decreased production of beta globin

clinically thal minor trait with mild MCHC anaemia

PBF may show target cells
Hb electrophoresis: elevated HbA2 of 3.5 to 7%, but normal HbA2 does not rule out beta thal trait
10-15% may have asymptomatic slight hepatomegaly

153
Q
A
154
Q

haemolytic anaemia: how to complete your examination?

A
  • look at BP chart, temperature
  • blood glucose level
  • CVS examination
  • checking testicular size
  • PR examination
155
Q

features of chronic haemolytic anaemia on abdo examination?

A

hyperpigmented skin due to haemosiderosis

peripheries: jaundice (may not be present due to extramedullary haemolysis), pallor, extramedullary erythropoiesis with prominent maxilla, supraorbital ridges, dental malocclusion, gum hypertrophy

abdo: liver/spleen/kidneys, ascites, scars, iron chelation therapy marks

signs to suggest complications:
short stature for failure to thrive
loss of axillary hair for hypopituitarism
cholecystectomy scar for previous gallstones
splenectomy scar
finger prick marks for DM
CCF/ Arrhythmias

signs of chronic liver disease: if present, could be due to hep b/ c from prev transfusions, or iron overload from recurrent transfusions (DDx haemachromatosis)

156
Q

ddx of chronic haemolytic anaemia

A

thalassaemia major/intermedia - likely transfusion dependent
hereditary spherocytosis or elliptocytosis

157
Q

ix of chronic haemolytic anaemia 2’ thalassaemia

A

for diagnosis, to establish complications

diagnosis: haemoglobin electrophoresis
- beta thal major/ intermedia (increased HbA2 and HbF)

genotyping - gene mutations in thalassaemia

assess severity
FBC: MCHC anaemia, (Hb) may have low Plt from hypersplenism
PBF: MCHC, anisocytosis, poikilocytosis, target cells, fragments from haemolysis, HbH inclusion bodies in alpha thal

osmotic fragility: decreased fragility

haemolysis markers: high retic count/ bilirubin, LDH, low haptoglobin

**complications of iron overload **
deranged LFT in cirrhosis 2’ iron overload, tests of synthetic function

serum ferritin: start iron chelation therapy when >1000mcg/L

TTE or cardiac MRI to monitor iron deposition

Fasting glucose, HbA1c

Anterior pituitary hormone screen:
FSH, LH, testosterone/oestrogen
TSH free T4
ACTH, 8am cortisol
Somatotropin, IGF1
Prolactin

Bone Mineral density studies to screen for osteoporosis

158
Q

What are HbH inclusion bodies in alpha thalassaemia

A

HbH is a tetramer of beta globin chains due to excess of beta globin in the RBC

159
Q

Hb Electrophoresis for alpha thal

A

decreased HbA, HbA2 and HbF
HbH band seen in HbH thal

160
Q

Hb electrophoresis for beta thal

A

elevated HbA2, increased HbF %
decreased HbA %

161
Q

osmotic fragility in hereditary spherocytosis

A

increased

as surface area to volume ratio very low- vol cannot expand any more

162
Q

genotyping in beta thal

A

point mutation in chr 11

163
Q

genotyping in alpha thal

A

most commonly in SEA
deletion mutation in chr 16

164
Q

when to determine when to start iron chelation therapy in transfusion dependent patients and how to monitor for complications

A

serum ferritin: start iron chelation therapy when >1000 mcg/L

MRI heart/liver once a year to monitor iron deposition

for endocrine complications
hypoPTH: Calcium, Phosphate
Thyroid function tests
DM: Blood sugar level, OGTT, urine glucose

165
Q

management of thalassaemia major

A

blood transfusions for patients with thal major or intermedia with anaemia

chelation therapy with sc desferrioxamine

splenectomy if haemolytic anaemia is severe or splenomegaly is symptomatic: decreases requirements for transfusions though effect is transient

haemotopoietic cell transplantation

166
Q

management of complications of thalassaemia major/ intermedia

A

from disease:
delayed puberty, gall stones, osteoporosis

from iron overload:
hypothalamus/ pituitary defect (anterior pituitary hormone replacement), hypothyroidism, DM, hypoparathyroidism, CLD, CCF

from blood transfusions, hep B, C, HIV

from iron chelation: vision and hearing loss from desferrioxamine toxicity

genetic counselling and screening

167
Q

side effects of desferrioxamine iron chelation therapy

A

anaphylaxis, ototoxicity and ophthalmotoxicity

168
Q

management of gallstones 2’ thalassaemia

A

cholecystectomy

169
Q

management to reduce risk of SE from desferrioxamine toxicity

A

monitor hearing and vision

170
Q

management of endocrine complications due to iron overload from multiple transfusions in thalassaemia major

A

monitor pubertal development
screen for DM, hypothyroidism
monitor for pituitary dysfunction

171
Q

DDx of right iliac fossa mass?

A
  • transplanted kidneys
  • carcinoma of caecum (hard mass, LNs)
  • abscess: appendicular, ileocaecal
  • Crohn’s disease (mouth ulcers, PR for fistulas)
  • ovarian tumours (in females)
  • others:
    • infection: amoebiasis, TB lymphadenitis, actinomycosis
    • carcinoid
    • ectopic kidney
172
Q

DDx left iliac fossa mass?

A
  • transplant kidney
  • colonic carcinoma (hard mass, hepatomegaly, LNs)
  • diverticular abscess
  • faecal mass
  • ovarian tumours
  • others: lymphadenitis
173
Q

common kidney diseases leading to transplant?

A

DM
HTN
Glomerulonephritis

174
Q

how does renal transplant compare with dialysis

A
  • higher patient survival rates
  • better QoL with lower hospitalization rates
175
Q

what are the causes of transplant loss?

A
  • patient death
  • allograft failure: immunological, non-immunological, recurrence of primary disease (GN, DM), chronic allograft nephropathy
176
Q
A
177
Q

what are the immunological causes of allograft failure in transplant kidney?

A

acute rejection
- single most important event determining graft survival
- can result in rapid loss of graft or progression to chronic rejection or chronic allograft nephropathy
- treated with pulse steroids or anti-lymphocyte antibody therapy

chronic rejection

178
Q

what are the non-immunological causes of allograft failure in transplant kidney?

A

renovascular thrombosis
ischaemia reperfusion injury
nephrotoxicity from calcineurin inhibitors
CMV, polyoma virus
DM, HTN, HLD

179
Q

what is delayed graft function in kidney transplant?

A

kidney being slow to ‘wake up’

defined as AKI requirement of dialysis in the first week post transplant
- main concern would be of acute rejection

180
Q

what strategies to reduce graft loss secondary to immune mediated rejection?

A
  • live donor better than cadaveric
  • HLA matched at A, B and DR loci
  • absence of pre-sensitisation: previous transplant, pregnancies, transfusions, idiopathic
  • immunosuppressive therapy to reduce acute rejection: traditionally use of steroids, ciclosporin. others: calcineurin inhibitors (eg. Cyclosporin, tacrolimus), MMF, sirolimus
181
Q

what factors increase risk of graft loss secondary to non- immune mediated rejection?
(pre-transplant)

A
  • donor factors: old age, CVA, HTN
  • Recipient factors: older, male, obese, diabetic, hypertension
182
Q

what factors increase risk of graft loss secondary to non- immune mediated rejection? and what strategies can reduce this?
(technical factors)

A

increased cold ischaemia time- strategy to reduce: Living donor transplant, renoprotective preservative solutions

hyperfiltration from inadequate nephron dose: strategy would be to match size and better if male to female; use of ACEi

183
Q

what post transplant factors increase risk of graft loss secondary to non- immune mediated rejection? and what strategies can reduce this?

A

calcineurin inhibitor induced nephrotoxicity:
- monitor levels
- use others e.g. sirolimus or MMF

CMV infections and polyoma virus
- prophylaxis with ganciclovir for CMV
- no Rx for polyoma virus

HTN, HLD, DM
- treat these, aim BP <130/80

184
Q

what are the complications of cyclosporin?

A

hirsutism/ hypertrichosis
gum hypertrophy
HTN
Nephrotoxicity
Hepatotoxicity
Electrolyte abnormalities: hyperK, hyperUricaemia, hypercholesterolaemia, hypoMg

185
Q

what are the complications of chronic steroid use?

A

skin -thin skin, telangiectasia, steroid purpura
cushingoid habitus
osteoporosis, AVN femoral head
peptic ulcer disease
HTN
DM
cataracts
steroid psychosis

186
Q

signs to mention in a patient with renal impairment?

A
  • uraemic flap
  • uraemic rub
  • no kussmaul’s breathing (acidosis)
  • no evidence of fluid overload: able to lie flat, no pedal oedema
  • conjunctival pallor to suggest anaemia

evidence of RRT:
- AVF: functioning?
- transplant kidney
- permcath
- PD catheter

187
Q

renal transplant patient: how to complete your examination?

A
  • temperature chart for fever
  • BP for hypertension
  • fundoscopy for hypertensive changes
  • urinalysis for haematuria, proteinuria, pyuria
188
Q

management of renal transplant patient

A

education and counselling, regular follow up, compliance to medications
treat underlying cause
preparation prior to transplant
post transplant management to reduce graft loss: immunosuppressive therapy, CMV prophylaxis with ganciclovir

189
Q

causes of a unilaterally enlarged kidney?

A

RCC
acute renal vein thrombosis
hypertrophy of single functioning kidney
pyonephrosis

190
Q

causes of bilaterally enlarged kidneys with asymmetrical enlargement (one may be bigger than the other)?

A

APCKD
Acromegaly
DM
bilateral hydronephrosis
tuberous sclerosis, Von Hippel Lindau, Amyloidosis

191
Q

unilateral enlarged kidney, how would you like to complete your examination

A

temperature chart for fever
BP for hypertension
fundoscopy for hypertensive changes
urinalysis -> but dont mention if ESRF on RRT
cardiovascular exam- for MVP and AR (assoc APCKD)
Neuro exam for stroke (III nerve palsy 2’ berry aneurysm)
fhx of stroke/ aneurysm

192
Q

what are the causes of bilateral enlarged kidneys?

A

APCKD

commoner:
- acromegaly (hepatosplenomegaly)
- early diabetic nephropathy
- bilateral hydronephrosis

rare:
- tuberous sclerosis
- amyloidosis
- von hippel lindau disease

193
Q

what is von hippel lindau disease?

A

autosomal dominant
multiple angiomata in the retina, CNS
cysts in liver, kidneys, pancreas
assoc RCC, phaeochromocytoma

194
Q

what are the conditions that can result in bilateral renal cysts?

A
  • polycystic kidneys
  • simple cyst
  • von hippel lindau
  • tuberous sclerosis
195
Q

renal complications of APCKD?

A
  • acute renal failure: malignant hypertension, UTI, nephrolithiasis
  • chronic renal failure
  • fever: UTI, pyelonephritis, pyocyst
  • hypertension
  • pain: chronic or acute from UTI, stones, cyst rupture, haemorrhage into cyst, upper tract obstruction
  • anaemia: CRF, persistent gross haematuria
  • polycythaemia: increased EPO
  • malnutrition: CRF, bilateral renal enlargement with early satiety
  • renal cell carcinoma (rare)
196
Q

extra renal complications of APCKD?

A

cysts in liver, spleen, pancreas, ovaries; colonic diverticular disease
Cardiac: MVP (25%), AR, TR
intracranial aneurysm (III nerve palsy), SAH 3%

197
Q

causes of hypertension in APCKD?

A

activation of RAA from intra renal ischaemia from architectural distortion

malignant hypertension may arise from:
renal artery stenosis from compression
renin producing cyst

198
Q

complications of chronic renal failure?

A

fluid overload
electrolytes - hyperK, hyperPO4
metabolic acidosis
uraemia and its complications
hypertension
anaemia - NCNC
secondary and tertiary hyperparathyroidism
renal bone disease

199
Q

why are patients with chronic renal failure sallow?

A

impaired excretion of urinary pigments combined with anaemia

200
Q

what are the types of signs in the nails that you can detect in patients with chronic renal failure

A

hypoalbuminaemia: leukonychia, muehrcke’s nails (paired white transverse line near distal end of nails)

renal failure:
terry’s nail (distal brown arc 1mm or >)
Mee’s line (single white line, also seen in arsenic poisoning)
Beau’s line (non pigmented indented band = catabolic state)

201
Q

causes of anaemia in patients with chronic renal failure?

A

EPO deficiency
Anaemia of chronic disease
Fe deficiency anaemia- blood loss, nutrition
Folate deficiency- nutrition

202
Q

what is Adult polycystic kidney disease?

A

multisystemic, progressive disease
characterised by cyst formation and enlargement in kidneys and other organs
auto dominant with almost 100% penetrance
focal cystic dilatation of renal tubules

2 predominant types: APCKD 1 on ch 16, APCKD 2 on ch 4

203
Q

clinical symptoms of patients with APCKD

A

present clinically in 3-4th decade
with
haematuria, HTN, recurrent UTI, pain and uraemia
stroke

by age 60 years, 50% will require RRT

204
Q

causes of mortality in APCKD?

A

ESRF (1/3)
Stroke and other hypertensive complication (1/3)
others

205
Q

ix of APCKD?

A

labs: FBC, RP, Ca, PO4, iPTH, uric acid
urinalysis

imaging:
USS abdomen
MRA for patients with high risk of aneurysm, barium enema and echocardiogram

genetic testing

206
Q

Ravine’s criteria for diagnosis of APCKD?

A

15-29: at least 2 cysts in 1 kidney or 1 cyst in each
30-59: at least 2 cysts in each kidney
> 60 years old: at least 4 cysts in each kidney

207
Q

management of APCKD?

A

education and counselling
regular follow up
screen first degree relatives

avoid medications that can precipitate renal impairment e.g. NSAIDs, tetracycline antibiotics

medical:
hypertension- ACEi, ARB
UTI, cyst infection - usually gram neg bacteria, can use bactrim/ fluoroquinolones
pain treatment
renal failure - medical treatment or RRT
antibiotic prophylaxis

surgical management

208
Q

surgical treatments for patients with APCKD?

A

pyocyst -drainage
cystectomy
nephrectomy
kidney transplant
anuerysm clipping
MVP with MR - valve replacement

209
Q

splenomegaly in essential thrombocytosis?

A
  • exclude transformation to myelofibrosis
  • higher risk of thrombosis
210
Q

why need to refer gastro in the case of pernicious anaemia?

A

increased risk of gastric cancer with autoimmune gastritis

can refer for scopes

211
Q

causes of pancytopenia?

A

reduced production:
nutritional deficiencies: B12, folate, Cu
infection: HIV, viral hepatitis, parvovirus B19
myelodysplastic syndrome
aplastic anaemia: Fanconi’s, Acquired
Drugs

infiltration of BM:
metastatic cancers
haematological malignancies
granulomatous disorders
myelofibrosis
storage disorders

peripheral destruction or sequestration:
DIC
TTP, HUS
AIHA
hypersplenism: 2’ cirrhosis, storage diseases, lymphoma, autoimmune disorders

both: impaired production and peripheral destruction
- PNH
- HLH
- Haematological malignancies
- Transfusion assoc GvH
- SLE

212
Q

diagnosis of myelodysplasia?

A

examination of blood and bone marrow showing blood cytopenias and hypercellular marrow with dysplasia, with or without excess of blasts

  • prognosis depends largely on marrow blast %, number and extent of cytopenias, and cytogenetic abnormalities
213
Q

treamtent of myelodysplasia?

A

low risk: growth factors, lenalidomide, transfusions

higher risk: hypomethylating agents, and whenever possible, allogeneic stem cell transplantation

214
Q

diagnosis of HLH?

A

fever
splenomegaly
cytopenias (affecting >=2 lineages in peripheral blood)
hypertriglyceridaemia +/- low fibrinogen <= 1.5g/L
haemophagocytosis in BM, spleen or LN
low or absent NK cell activity
Ferritin >/=500 ug/L
soluble CD25 >/=2400 U/mL

215
Q

Triggers of 2’ HLH?

A

Infection e.g. EBV, CMV
Malignancy!
Rheumatologic/ autoimmune disease
Immunosuppression
Pregnancy/HELLP syndrome (rare)

in children - need to rule out primary/ familial HLH
in adults - HLH should prompt investigation for underlying malignancy

216
Q

management of Hodgkin’s lymphoma?

A

depending on stage of disease

  • chemo/ RT or both
  • PET guided approach
217
Q

management of DLBCL, Non hodgkins lymphoma?

A

R CHOP
rituximab
cyclophosphamide
doxorubicin
vincristin
prednisolone

218
Q

complications of acute leukaemia?

A

infection
hyperviscosity: esp if WCC High >100 x 10^9
bleeding: including fundoscopy to look for retinal bleeding
DIC
Tumour lysis syndrome

219
Q

management of acute promyelocytic leukaemia?

A

All-Trans Retinoic Acid (ATRA) in 2 divided doses ASAP

correct coagulaopathy: Plt (>30 if asympto, >50 if bleeding), fibrinogen (>1.0g/L if asympto, >1.5g/L if bleeding)

for patients with high WBC (>10 x 10^9 and rising) while awaiting pre-chemo work up:
start hydroxyurea or IV cytarabine for cytoreduction

220
Q

patients at high risk of tumour lysis syndrome?

A
  • newly diagnosed acute leukaemia pts with TW > 100 x 10^9/L
  • usually ALL > AML
  • burkitt lymphoma/ leukaemia
  • other aggressive lymphomas especially if LDH high at presentation
221
Q

features of tumour lysis syndrome

A

LDH high
Uric acid high
K/ PO4 high
Ca low

222
Q

management of Tumour lysis syndrome?

A

hydration 3-3.5L/day if no cardiac issues
good urine output (1ml/kg/h), IV furosemide if necessary

allopurinol 300mg OM OR IV rasburicase
correction of electrolytes

may require dialysis in even of severe electrolyte imbalances or acidosis

223
Q

management of SVCO?

A

involve ICU team and thoracic surgeons early
IV access in lower limbs

steroids to reduce peritumoral oedema (though avoid if lymphoma suspected)

treat underlying tumour

if severe symptoms, can consider endovascular stenting

palliative RT can be considered in those not candidates for systemic treatment

224
Q

Haemolytic anaemia + PBF showing spherocytes

A

DCT +ve -> AIHA

225
Q

Haemolytic Anaemia + PBF showing agglutination?

A

key test: direct coombs test, cold agglutination titre
-> cold AIHA

226
Q

Haemolytic anaemia + PBF showing shistocytes?

A

DIC screen, plt count
-> think of DIC/TTP/HUS

227
Q

haemolytic anaemia + bite cells on PBF

A

heinz body, G6PD testing
review drug list!

consider oxidate haemolysis as cause

228
Q

signs of extramedullary haematopoiesis?

A

frontal bossing
maxillary overgrowth
scoliosis
hepatosplenomegaly

229
Q

signs and complications of chronic haemolysis?

A
  • conjunctival pallor
  • scleral icterus
  • kocher’s scar (previous cholecystectomy)
  • right ventricular heave, loud P2 (pulmonary hypertension)
230
Q

signs of iron overload?

A
  • slate grey skin
  • displaced apex beat (congestive cardiac failure)
  • implantable cardiac device
  • finger brick blood sugar marks (DM)
  • diabetic dermopathy
  • signs of chronic liver disease
231
Q

how to complete your abdominal examination if suspecting thalassaemia?

A
  • examine testes: hypogonadotrophic hypogonadism from pituitary iron loading
  • urine dip for glucose
  • examine CVS (iron overload)
  • examine musculoskeletal system (arthritis)
232
Q

ddx of thalassaemia?

A

thalassaemia major/ intermedia
hereditary spherocytosis/ elliptocytosis
autoimmune haemolytic anaemia

233
Q

peripheral stigmata of chronic liver disease?

A

hyperoestrogenism: gynaecomastia, paucity of axillary hair, testicular atrophy, palmar erythema, spider naevi

alcohol: parotidomegaly, dupuytrens

hypoalbuminaemia: leuconychia

234
Q

features of portal hypertension on examination?

A

ascites, caput medusae, splenomegaly

235
Q

features of decompensation in chronic liver disease?

A

ascites
jaundice
encephalopathy
purpura and bruising ?coagulopathy

236
Q

medications for IBD?

A

aminosalicylates for mild disease (oral or rectal sulfasalazine)
steroids + DMARDs: sulfasalazine, azathioprine, methotrexate
Biologics: anti-TNF (infliximab, adalimumab), anti IL12/23: ustekinumab
surgery

237
Q

criteria to assess severity in ulcerative colitis?

A

truelove and witts

looks at no of bloody stools,
HR, temperature
Hb, ESR/CRP

238
Q

Hereditary causes of GI polyposis?

A

adenomatous polyposis syndromes:
- familial adenomatous polyposis
- Gardner’s (FAP variant)

hamartomatous polyposis syndrome:
- peutz jegher
- juvenile polyposis

239
Q

evaluation of GI polyposis?

A

ask for personal or family history of GI polyps, cancer (colonic, extra colonic), prev scopes/ surgery

look for mucocutaneous pigmented macules (peutz jeghers)

refer GE, surgery, genetic counsellor

240
Q

features of peutz jeghers

A

characteristic mucocutaneous pigmentation
- 1-5mm diameter, most commonly in perioral region and buccal mucosa
- darker and more densely clustered than common freckles
- can occur on face/ arms/ palms, soles

hamartomatous GI polyps (in 88-100%)
- stomach, small bowe, colon, rectum
- polyp infarct/ulceration/bleeding/ intestinal obstruction, intussusception
- risk of adenoma and cancer

241
Q

management of peutz jeghers?

A

genetic counselling: autosomal dominant (10-20% de novo mutations)
- can offer genetic testing of patient and 1st degree relatives

**polyp management: **
endoscsopic polypectomy
surgery for larger polyps or if complications arise e.g. intussusception

cancer surveillance:
- risk of colorectal cancer
- extra colonic as well: stomach, small bowel, pancreas, breast, ovarian, uterus, cervix, testicular, lung

242
Q

what are the scoring criteria for wilson’s disease?

A

Leipzig criteria for diagnosis
Nazer score for prognosis

243
Q

clinical features of haemochromatosis important to ask for?

A
  • lethargy
  • joint pains: pseudogout
  • liver: cirrhosis
  • pancreas: DM
  • heart: CCF
  • pituitary: hypogonadism
244
Q

phlebotomy in haemochromatosis: what are the target values of ferritin/ Tsat?

A

Tsat < 50%
Ferritin 20-30

245
Q

examination findings of patient with primary biliary cholangitis

A

skin: xanthelasma, xanthoma (palms, soles, extensor surfaces of knees/ elbows, ankle/wrist tendons, buttocks), excoriations

eyes: KF rings rarely (Cu retention from prolonged cholestasis)

abdomen: hepatosplenomegaly

CLD stigmata

246
Q

pathophysiology of primary biliary cholangitis?

A

immune and cellular injury to biliary epithelial cells, resulting in cholestasis and progressive liver fibrosis

247
Q

symptoms of primary biliary cholangitis?

A

fatigue, pruritus
right sided abdo discomfort
sicca complex ?sjogrens
jaundice

ask for other autoimmune conditions e.g. coeliac, T1DM, RA

248
Q

Differentials for Gynaecomastia?

A
  • possible persistent (physiological) pubertal
  • drug causes (spironolactone)
  • rule out breast cancer (bloody discharge, rapid growth of lump, family history of breast ca, any lumps in armpit or neck)
  • pituitary cause (any head injury/ visual field defect/ headache/ panhypopit symptoms)
  • gonadal cause (ask if pubertal history is normal or not?, previous mumps orchitis? Any testicular injury?)
249
Q

examination of a patient with gynaecomastia?

A
  • examination of chest
  • axillary and cervical LN to show you are thinking of breast cancer
  • Offer to check for visual field defects
  • offer to examine testicles
250
Q

what medication is indicated to slow kidney function decline in adults at risk of rapidly progressing ADPKD?

A

Tolvaptan

(vasopressin 2 receptor antagonist)

PACES 2024 (14 decks)

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