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PACES Revision Cards > Abdominal Flashcards > Flashcards

Abdominal Flashcards

1
Q

Autosomal Dominant Conditions

A

APKD type 1 (chromo 16) and type 2 (chromo 4)
Hereditary spherocytosis
HHT

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2
Q

Autosomal Recessive Conditions

A

Haemochromatosis (chromo 6)
Wilson’s disease (chromo 13)
Alpha 1 antitrypsin (chromo 14)

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3
Q

How can you differentiate the spleen from a kidney on palpation?

A

1) Dull to percussion

2) Not ballotable

3) Palpable splenic notch

4) Cannot get above splenic mass

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4
Q

Outline some causes of Spider Naevi?

A

= vascular lesion in distribution of SVC

> 5 = pathological

1) Normal in childhood

2) Pregnancy

3) Oestrogen

4) Chronic liver disease

5) Thyrotoxicosis

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5
Q

Outline causes of Acanthosis Nigricans

A

Insulin-resistant diabetes mellitus
Paraneoplastic
Hypo/hyperthyroidism
Acromegaly
Cushing’s disease
Obesity

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6
Q

Outline causes of gynaecomastia

A

Idiopathic

Chronic liver disease
Chronic kidney disease
Thyrotoxicosis
Congenital e.g. Klinefelter’s

Drugs e.g.
- Haloperidol
- Spironolactone
- Omeprazole
- Alcohol
- Ketoconazole
- Digoxin

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7
Q

Outline causes of Hepatomegaly

A

Cs & Is of Hepatomegaly

C - Cirrhosis
C - Carcinoma (HCC)
C - CCF

I - Infectious e.g. Hepatitis, EBV, CMV
I - Immune(Auto) e.g. PBC, PSC
I - Infiltrative e.g. Amyloidosis
I - Iron (Haemochromatosis)

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8
Q

What is pre-hepatic jaundice?

A

Jaundice = elevated serum bilirubin

Pre-hepatic = disruption in bilirubin pathway occurs prior to hepatic bilirubin conjugation
Increased RBC haemolysis = increase in levels of UNCONJUGATED bilirubin

Causes:
- Haemolytic anaemias
- Gilbert’s (autosomal recessive)

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9
Q

What is post-hepatic jaundice?

A

Jaundice = elevated serum bilirubin

Post-hepatic = disruption in bilirubin pathway occurs after hepatic bilirubin conjugation, and results in reduced excretion of bilirubin from body

Blockage prevents excretion of CONJUGATED bilirubin

Causes:
- Gallstones
- Pancreatic cancer
- Cholangitis
- Cholangiocarcinoma

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10
Q

Which blood tests can you do to differentiate between types of jaundice?

A

Liver function tests (ALT>ALP indicates hepatic damage, raised yGT and ALP indicates post hepatic “cholestatic”)

Split bilirubin (conj & unconj bilirubin)

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11
Q

Outline the blood tests involved in a liver screen

A

AUTOIMMUNE
- ANA, anti-mitochondrial antibody, anti-smooth muscle antibody, liver-kidney microsomal antibody
- Anti-TTG

INFECTIOUS
- Hep B surface antigen, Hep C antibody
- Hep B core antibody
- CMV/EBV (if acute)

Immunoglobulins

METABOLIC
- HbA1c
- Ferritin & iron studies
- Caeruloplasmin

Alpha feto-protein

Paracetamol (if acute)

?alpha 1 AT level

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12
Q

Outline the pathophysiology of Liver Cirrhosis

A

1) Hepatocellular injury

2) Stimulates stellate cells to become myofibroblasts

3) Myofibroblasts secrete pro-inflammatory cytokines and produce collagen

4) = Tissue fibrosis

5) = Distorts hepatic architechture and vasculature

6) = Portal hypertension and only hepatocellular injury due to reduced perfusion

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13
Q

What are the main causes of liver cirrhosis?

A

TOP 3
Alcohol
MASLD
Hepatitis C

Other important causes
- Autoimmune e.g. AIH, PBC
- PSC
- Haemochromatosis
- Wilson’s
- CF
- Alpha 1 AT

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14
Q

What is decompensated CLD? How could this present?

A

= acute deterioration in liver function in patients with cirrhosis

Jaundice
Asterixis
Confusion
Ascites
Peripheral oedema

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15
Q

Outline some risk factors for developing decompensated liver disease

A

Alcohol
Infection
AKI/Dehydration
Constipation
Medications e.g. sedating agents
Thrombosis e.g. hepatic vein thrombus
Fluid overload

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16
Q

What is Child Pugh grading?

A

Severity of cirrhosis score

A to C
- A = 100% 1 year survival
- C = 50% 1 year survival

5 components to score
1) Serum albumin
2) Ascites
3) Bilirubin
4) INR
5) Hepatic encephalopathy

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17
Q

Outline how you would investigate for ?chronic liver disease

A

Full history
Medication history
Alcohol history

Routine bloods, including FBC, U&Es, coagulation and LFTs
Non-invasive liver screen blood tests
- See other flashcard

USS Liver

?CTAP/MRCP (depends of type of jaundice)

FIB-4 score (predicts likelihood of advanced fibrosis)

Transient Elastography (fibroscan)

Liver biopsy?

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18
Q

Who are offered Transient Elastography in order to screen for liver cirrhosis?

A

Patients with Hepatitis C
Men who drink > 50 units of alcohol/week
Women who drink > 35 units of alcohol/week
Patients with known Alcoholic Liver Disease

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19
Q

Which criteria can help you decide which patients with cirrhosis should have OGD surveillance for oesophageal varices?

A

Baveno Criteria

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20
Q

How often should patients with liver cirrhosis be offered HCC screening? What does this entail?

A

Liver USS every 6 months +/- aFP levels

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21
Q

Outline the management of ascites

A

Reduce dietary intake of sodium

Aldosterone antagonist (spironolactone)

Monitor weight

Therapeutic abdominal paracentesis

Transjugular intrahepatic portosystemic shunts

?Prophylactic antibiotics e.g. ciprofloxacin
- If previous SBP or cirrhotic with ascitic protein < 15g/L

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22
Q

Risk of TIPS procedure

A

Increased risk of hepatic encephalopathy

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23
Q

What would be the main indication for performing a transjugular liver biopsy as opposed to percutaneous?

A

Ascites

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24
Q

How can you delineate between transudate and exudate causes of ascites?

A

Serum:Ascites Albumin Gradient

SAAG>11g/L = Transudate

SAAG< 11g/L = Exudate

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25
Q

Causes of Transudate Ascites

A

Cirrhosis with portal HTN
CCF
Nephrotic syndrome

Budd-Chiari
Portal Vein thrombosis
Malabsorption
Myxoedema

(Failures and Thrombuses)

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26
Q

Causes of Exudate Ascites

A

Malignancy e.g. metastatic GI cancers, HCC, ovarian, peritoneal
TB peritonitis
Pancreatic ascites
Meig’s Syndrome
= ovarian fibroma, pleural effusion & ascites
Bowel Obstruction
Serositis in CTD

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27
Q

What is hepatorenal syndrome?

A

Renal hypoperfusion and failure, secondary to severe liver disease

Type 1 = Rapidly progressive, poor prognosis

Type 2 = Slowly progressive

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28
Q

How would you manage a patient with hepatorenal syndrome?

A

MDT approach - liver and renal specialists

Terlipressin (splanchnic vasoconstrictor)

Cautious monitoring of fluid balance - + HAS 20%

TIPSS

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29
Q

Grading of Hepatic Encephalopathy

A

West Craven Criteria

0 = clinically normal

1 = mild confusion, irritability, short attention span, disordered sleep

2 = drowsy, disorientated, inappropriate

3 = somnolent but rousable to voice, v confused, amnesia

4 = comatose

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30
Q

Alcoholic Hepatitis

A

= inflammatory condition related to ongoing alcohol intake

Severe Alcoholic Hepatitis = 30-50% 4 week mortality

Transaminases usually >500
AST:ALT > 2

MANAGEMENT
- Alcohol abstinence + withdrawal support
- MDT approach (nutrition, ASNs)
- Maddrey’s discrimnant function can help determine if will benefit from steroids (>32)

MELD score (Model for End Stage LD) - prognostic predictor

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31
Q

Autoimmune Hepatitis

A

3 main types
1) Type 1
= positive ANA and anti-smooth muscle antibodies
- Adults & children
2) Type 2
= Anti-liver/kidney microsomal type 1 antibodies (LKM1)
- Mainly children
3) Type 3
= Soluble liver-kidney antigen
- Middle aged adults

Usually young women, with associated autoimmune conditions e.g. coeliac

Raised IgG!!!!

Autoantibodies & liver biopsy

MANAGEMENT
- Steroids or steroid-sparing immunosuppression e.g. azathioprine
- Liver transplant

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32
Q

Primary Biliary Cholangitis

A

Autoimmune condition - destruction of small intrahepatic ducts

F:M = 9:1, middle aged women

Anti-mitochondrial antibodies & IgM

Associations = RA, Sjogren’s, Systemic sclerosis, coeliac

Classically asymptomatic with isolated raised ALP
Itch/fatigue/RUQ pain/jaundice

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33
Q

Management of Primary Biliary Cholangitis

A

MDT approach

Alcohol abstinence

Pruitus = cholestyramine

Vitamins ADEK (d2 reduced fat soluble vitamin absorption)

Ursodeoxycholic acid (reduces cholestasis and can improve prognosis)

Liver transplant (esp if bili > 100)

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34
Q

Possible complications of PBC

A

Liver cirrhosis
HCC (20x increased risk)
Osteoporosis/osteomalacia
Vitamin ADEK deficiency

If have liver transplant, there is a risk of recurrence!

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35
Q

Primary Sclerosing Cholangitis

A

= Inflammatory condition of intra and extrahepatic ducts = progressive scarring and obstruction

Associations = UC (80% of PSC pts have UC), Crohn’s, HIV

Male > Female

pANCA positive
ANA and anti-smooth muscle antibodies
MCRP –> ERCP (beaded appearance)
?Liver biopsy

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36
Q

Management of Primary Sclerosing Cholangitis

A

MDT approach

Alcohol abstinence

Vitamins (ADEK)

Ursodeoxycholic acid

?Liver transplant

SCREENING
- If have IBD = annual colonoscopies
- Screening for cholangiocarcinoma

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37
Q

Possible complications of Primary Sclerosing Cholangitis

A

Chronic liver disease & cirrhosis
Cholangiocarcinoma (10% increased risk!)
Increased risk of colorectal cancer

38
Q

Haemochromatosis

A

Autosomal recessive disorder of iron metabolism

HFE gene on chromosome 6 (variable penetrance)

Fatigue, arthralgia, sexual dysfunction (anterior pituitary function impaired in 2/3)

Multisystem
- Bronze skin
- Hypopituitarism = hypothyroid, sexual dysfunction, amenorrhoea
- Dilated cardiomyopathy
- T1DM
- CLD/cirrhosis
- Arthritis, pseudogout

+++ increased risk of HCC

39
Q

How would you investigate for haemochromatosis?

A

Full history & examination etc

Ferritin & Iron studies
- Raised ferritin
- Raised transferrin saturation (>55% in men, > 50% in women)
- Raised iron levels
- Low TIBC

HbA1c

Liver biopsy (Perl’s Stain)

Joint xrays (chondrocalcinosis)
Echocardiogram

Genetic testing (HFE gene most common) and offer family screening

40
Q

Management of Haemochromatosis

A

MDT approach - liver, endocrine, cardiology

Alcohol abstinence (increases iron accumulation)

Genetic counselling

Venesection (weekly to start) - according to transferrin saturation & ferritin levels

Desferrioxamine = Iron chelator

?Liver transplant

MONITORING
- Regular iron studies
- 6 monthly USS & aFP
- Monitor HbA1c

41
Q

Wilson’s Disease

A

Autosomal recessive condition resulting in excess copper deposition in tissues

ATP7B gene on chromosome 13

Onset = 10-25 years old

Multisystem
- Asterixis, chorea, dementia/neuropsych
- Kayser-Fleischer rings
- Haemolytic anaemia
- Hepatitis –> cirrhosis
- Renal tubular acidosis

Low levels of caeruloplasmin (= copper transport in serum) = less copper in serum, more deposited in tissues
Increased urinary copper excretion

Rx = pencillamine (chelator)

42
Q

Alpha 1 Antitrypsin Deficiency

A

Autosomal Recessive
SERPINA1 gene on chromo 14

COPD like respiratory disease (young pts with no smoking history)
Liver cirrhosis

MANAGEMENT
- Alcohol abstinence
- Stop smoking
- MDT approach
- ?recombinant alpha 1 AT

43
Q

Hepatocellular Carcinoma

A

3rd most common cancer worldwide

CAUSES
- Chronic Hepatitis B
- Chronic Hepatitis C
- Alcohol
- Haemochromatosis
- PBC

SCREENING = 6 monthly USS +/- aFP
Offered for high risk groups e.g.
- Cirrhosis secondary to Hep B/C
- Cirrhosis secondary to haemochromatosis
- Male pt with alcoholic cirrhosis

44
Q

Which cancer commonly metastasize to the liver?

A

Colorectal (adenocarcinoma)

Lung

Breast

Pancreatic

Gastric

Oesophageal

45
Q

Most common conditions for Liver Transplant

A

Commonest in UK = ALD

ALD
Paracetamol OD
MASLD
Autoimmune disease e.g. PBC
Haemochromatosis
HCC

46
Q

Criteria for Liver Transplant (Paracetamol)

A

= King’s Criteria

ABG pH < 7.3
Encephalopathy grade 3-4
PT > 100 seconds

47
Q

Criteria for Liver Transplant (Non paracetamol)

A

All 3 of :
1) PT>100 seconds
2) Grade 3-4 encephalopathy
3) Cr >300

OR any 3 of …
- PT > 50 seconds
- Jaundice/hepatic encephalopathy for >7 days
- >10 or <40 years old
- Bilirubin >300
- Unfavorable aetiology

48
Q

Complications of Liver Transplant

A

Surgical Complications
- Bleeding
- Post op infection
- Biliary leak
- Hepatic/portal vein thrombosis

Transplant Dysfunction
- Rejection/graft failure
- Recurrence of previous disease

Complications of immunosuppression
- Infections
- Malignancy
- CVD

49
Q

Complications of Immunosuppression

A

Infections - Bacterial, viral, fungal
Malignancy, esp skin
Nephrotoxicity
HTN
Increased CVD risk

50
Q

Causes of Pancreatitis

A

GET SMASHED

Gallstones
Ethanol
Trauma

Steroids
Mumps
Autoimmune
Smoking
Hypercalcaemia/Hypertriglyceridaemia
ERCP
Drugs e.g. azathioprine

Genetic causes
- Cystic fibrosis

51
Q

Complications of Pancreatitis

A

ACUTE
- Multisystem SIRs
- Resp failure/ARDS

CHRONIC
- Pain
- T3DM
- Portal/splenic vein thrombosis
- Biliary/duodenal obstruction
- Malignancy
- Pseudocysts

52
Q

Management of Chronic Pancreatitis

A

CONSERVATIVE
- Alcohol abstinence
- Stop smoking
- Nutrition input

MEDICAL
- Creon if evidence of malabsorption (faecal elastase) + PPI
- Analgesia, pain team

SURGICAL
- Endoscopic USS drainage of pseudocysts or AXIOS stent 6 week after initial presentation

53
Q

Indication for Combined Pancreas Kidney Transplant

A

T1DM +/- ESRF

54
Q

Pros & Cons of Combined Pancreas Kidney Transplant

A

PROs
+ Potentially curative of T1DM
+ Free from haemodialysis and insulin therapy
+ Reduced risk of vascular complications of diabetes (unclear if affects retinopathy yet)

CONS
- Lifelong immunosuppression (with risks of toxicity/infection/cancer)
- Risk of rejection/failure (5-7%)
- Risk of graft thrombosis

55
Q

Why are transplanted pancreas now attached to the duodenum?

A

Previously, transplant pancreas was anastomosed to bladder
- Could monitor pancreatic activity/risk of pancreatitis through urinary amylase

However - risk of urological complications and reflux pancreatitis!

Now, most transplanted pancreas are attached to the duodenum

56
Q

Complications of Chronic Kidney Disease

A

1) HTN & IHD

2) Anaemia (due to reduced EPO production)

3) Bone Mineral Disease
- May require parathyroidectomy for secondary hyperparathyroidism

4) Fluid retention

5) Uraemic complications e.g. pericarditis, encephalopathy

6) Calciphylaxis- painful necrotic lesions

57
Q

Causes of Enlarged Kidneys

A

Autosomal Dominant PKD
Hydronephrosis
Renal tumour(s)
Congenital renal anomalies e.g. horseshoe kidney
Amyloidosis

58
Q

Commonest causes of ESRF

A

Diabetes nephropathy
Hypertensive nephropathy
ADPKD
Chronic Glomerulonephritis
Obstructive uropathy

59
Q

Indications for Renal Replacement Therapy

A

RRT should be considered once eGFR <15

1) Hyperkalaemia (if refractory to Rx)
2) Metabolic acidosis (if refractory to Rx)
3) Complications of uraemia e.g. pericarditis
4) Severe pulmonary oedema/intractable fluid overload

60
Q

Contraindications to Living Donor Kidney Transplant

A

Uncontrolled HTN
Active Malignancy
Chronic infection
Bilateral renal artery atherosclerosis
Sickle Cell Disease

61
Q

Why are Kidney Transplants placed in the pelvis?

A

Good blood supply

Sufficient space

Proximity to bladder for ureteric anastomosis

Easy access for biopsies/nephrostomies

62
Q

Complications of AV Fistulae

A

Infection
- of AVF site
- Endocarditis

Thrombosis

Stenosis
- Often present with acute limb pain

Steal Syndrome
- Reduction/reversal of arterial blood flow distal to AVF = hand ischaemia

63
Q

Complications of Haemodialysis

A

DURING USE
- Hypotension
- Disequilibrium (blood urea reduced too rapidly = cerebral oedema)
- First-Use Syndrome = reaction to dialyser membrane (mild to anaphylactic)
- Haemolysis

LONG TERM
- CVD (20X more likely to die from CVD)
- Malnutrition
- Cardiomyopathy

Burden on quality of life - hospital appointments, restricted travel

64
Q

Contraindications to Peritoneal Dialysis

A

IBD

End-stage liver disease with ascites (SBP!)

Unrepaired abdominal hernias

Previous complex abdo surgeries

Polycystic Kidney Disease

65
Q

Complications of Peritoneal Dialysis

A

Peritonitis
- PD fluid WBC >100
- Can be recurrent/refractory
- Staph aureus most common cause

Hyperglycaemia (glucose used as dialysate)
Catheter blockage

Fluid retention
Constipation
Hernias

Sclerosing Encapsulating Peritonitis –> risk of bowel obstruction

66
Q

Outline example regime of immunosuppression for renal transplant

A

INITIAL
- Ciclosporin/tacrolimus with monoclonal antibody

MAINTENANCE
- Ciclosporin/tacrolimus with mycophenolate mofetil
+ regular steroids if >1 steroid responsive acute rejection episode

67
Q

Side Effects of Calcineurin Inhibitors

A

= Ciclosporin and Tacrolimus

Nephrotoxicity (C>T)
Tremor
Hyperkalaemia
HTN & hyperlipidaemia (CVD!!)

CICLOSPORIN
- Gingival hyperplasia
- Hypertrichosis

TACROLIMUS
- PRES
- New Onset Diabetes After Transplant (NODAT)
- Alopecia

68
Q

Examples of renal disease which can recur post renal transplant

A

IgA Nephropathy

Membranoproliferative GN
- lipodystrophy

Focal Segmental Glomerulonephritis

Membranous GN

Amyloidosis

69
Q

Opportunistic Infections in Renal Transplant pts

A

CMV
EBV
Pneumocystis jiroveic
BK virus
- usually dormant in renal tract
- causes ureteric stenosis and interstitial nephritis
JC virus
- PML!

70
Q

Autosomal Dominant Polycystic Kidney Disease

A

Most common cause of inherited kidney disease

Autosomal dominant

2 main types
- Type 1 = PKD1 - polycystin 1 on chromo 16
- Type 2 = PKD 2 - polycystin 2 on chromo 4

EXTRA-RENAL FEATURES
- Berry aneurysms (SAH)
- Mitral valve prolapse/regurg
- Aortic root dilatation / dissection
- Liver cysts (70%)!!!
- Diverticulitis –> perforation
- Abdominal herniae

71
Q

Management of ADPKD

A

BP control - ACEIs

Tolvaptan = vasopressin receptor antagonist
- Can slow rate of renal function decline & cyst progression
- Offered if CKD 2 or 3 or rapidly progressive

?Nephrectomy

Plan for RRT (monitor renal function)

72
Q

Indications for Nephrectomy in patients with ADPKD

A

1) Make room for kidney transplant

2) Chronic pain

3) Chronic renal cyst infections

4) Progression to RCC (rare)

73
Q

Alport’s Syndrome

A

X-linked dominant condition (COL45 gene)
- abnormal type IV collagen

More severe in males

Progressive renal failure
Bilateral sensorineural deafness
Lenticonus
Retinitis pigmentosa

Rx = ACEIs and renal transplant

HOWEVER - can develop anti-GBM antibodies to transplanted kidney = Goodpasture’s syndrome

74
Q

Ulcerative Colitis

A

Limited to colon

Continuous but superficial mucosal inflammation

Crypt abscesses on histology

Smoking improves symptoms/stopping smoking can unmask condition

80% of PSC pts have UC

Colectomy = curative

75
Q

Crohn’s Disease

A

Mouth to anus. Perianal disease particularly key feature of crohn’s disease

Skip lesions with transmural inflammation

Granulomas on histology

Smoking exacerbates symptoms

76
Q

Diagnostic criteria for Acute Colitis

A

= Truelove & Witts Criteria

MILD
<4 stools/day
No systemic features
Normal ESR

MODERATE
>4 stools/day
Minimal systemic features

SEVERE
>6 stools/day with blood
Systemic features e.g. fever, tachycardia
ESR >30

77
Q

Management of Acute Colitis

A

IV fluids
IV steroids (100mg hydrocortisone QDS)
VTE prophylaxis
Avoid antispasmodics/antimotility drugs
Discuss with gastro +/- surgical team

eCXR and AXR important but if acutely unwell, consider CT AP

78
Q

Indications for urgent surgical intervention in patients with severe UC

A

Toxic megacolon (caecum>9cm, colon > 5.5cm)

Still having >8 stools/day or CRP>45 after 3 days of IV steroids

Failure to respond to treatment after 10 days

79
Q

Management of UC (mild-moderate)

A

Inducing Remission
- Topical +/- oral aminosalicylate
- PO corticosteroid if not responding

Maintaining Remission
- Topical and/or oral aminosalicylate
- Azathioprine if
1) Severe relapse
2) >2 exacs in past year

80
Q

Factors which increase UC patient’s risk of colorectal cancer

A

Have had UC > 10 years

Pancolitis

Onset before age of 15

Unremitting

Poor compliance to treatment

81
Q

Inducing and maintaining remission in Crohn’s Disease

A

Inducing Remission
- Aminosalicylates and/or steroids
- Isolated perianal disease = metronidazole
- Refractory/fistulating/severe = Infliximab

Maintaining Remission
- STOP SMOKING!
- 1st line = Azathioprine (test TMPT level first - risk of bone marrow toxicity)
- 2nd line = MTX

82
Q

Hereditary Haemorrhagic Telangiectasia

A

A.k.a Osler-Weber-Rendu syndrome

Autosomal dominant

Diagnostic Criteria (4)
- 2 criteria = possible
- 3 criteria = definite
1) Spontaneous/Recurrent Epistaxis
2) Telangiectasias
- Typical sites = lips, oral cavity, fingers & nose
3) Visceral lesions e.g. AVMS
4) FHx (1st degree relative)

83
Q

Peutz Jeghers Syndrome

A

= numerous benign hamartomas in GI tract and hyperpigmented macules over hands/lips/feet

Autosomal dominant

Increased risk of malignancy
- Breast
- Ovarian
- Cervical
- Pancreas
- Testicular

50% mortality from GI tract cancer by age of 50

Rx = Pan-intestinal colonoscopy every 2-3 years

84
Q

Causes of Splenomegaly

A

1) INFILTRATIVE
- MPD, lymphoma
- Sarcoidosis, amyloidosis
- Gaucher type 1

2) INCREASE IN FUNCTION
= removal of defective of RBCs
- Hereditary Spherocytosis
- Sickle Cell disease
- Thalassaemia
OR
= immune system disordered/overactive
- Malaria
- EBV, CMV
- Visceral leishmaniasis
- Endocarditis
- Felty’s syndrome
- SLE

3) PORTAL HYPERTENSION

85
Q

Causes of MASSIVE Splenomegaly

A

cML

Myelofibrosis

Malaria

Visceral leishManiasis

86
Q

Outline the investigations you would request for work up of a patient with splenomegaly

A

Abdominal exam +/- rheum/thyroid exam

Bloods
- FBC, U&Es, LFTs
- TFTs
- Blood film
- Haemolysis screen e.g. LDH, haptoglobin, reticulocytes
- HIV screen

Thin and thick films x 3 if suspect malaria

USS abdomen

CT TAP (?malignancy)

Bone marrow aspirate& trephine

87
Q

Hereditary Spherocytosis

A

Most common hereditary haemolytic anaemia in pts of Northern European descent

Autosomal dominant
- Defect in RBC cytoskeleton = spherical RBCs = destroyed by spleen sooner

Failure to thrive
Jaundice (prehepatic)
Splenomegaly
Aplastic Crisis (parvovirus infection)

88
Q

Investigations for Herediary Spherocytosis

A

Bloods
- FBC, B12, folate, MCV, MCHC
- Blood film (spherocytes!)
- If diagnosis unclear = Cryohaemolysis test & EMA binding

(Used to do osmotic fragility test)

USS abdomen (size of spleen)

89
Q

Management of Hereditary Spherocytosis

A

MDT approach

?Genetic counselling

Folate replacement

Splenectomy
- Indicated if:
1) Recurrent anaemia
2) Massive splenomegaly

If get splenectomy =
Need vaccinations against encapsulated organisms (pneum & meningo) prior to surgery and then prophy abx after

90
Q

Chronic Myeloid Leukaemia

A

CML = 15% of all leukaemias
= uncontrolled myeloid proliferation

Philadelphia chromosome in 95% = translocation between 9 & 22 (BCR-ABL gene)

Anaemia
B symptoms - fever, weight loss, night sweats
Massive splenomegaly

Rx = Imatinib (inhibits BCR-ABL tyrosine kinase)
?Bone marrow transplant

91
Q

Chronic Lymphoblastic Leukaemia

A

Commonest haematological malignancy in adults

Accumulation of mature B cells
“MATURE B cells in MATURE adults”

B symptoms
Enlarged rubbery non-tender LNs
Hepato/splenomegaly

RX
= monitoring of FBC if asymptomatic/no BM failure/splenomegaly <10cm
otherwise
= FCR (fludarabine/cyclophosphamide/rituximab)

92
Q

Lymphoma

A

Hodgkins (presence of Reed Sternburg Cells) or Non Hodgkins

Painless lymphadenopathy
- Alcohol induced pain in NHL
B symptoms
Hepato-splenomegaly

Ann-Arbor Staging

Risk of
- Bone marrow failure (anaemia/ neutropaenia/thrombocytopaenia)
- AML transformation
- Compressive effects e.g. SVCO

PACES Revision Cards (9 decks)

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