Abdominal Flashcards

1
Q

Extra renal manifestations of polycystic kidney disease

A
  1. Renal angle/loin pain
  2. Cyst infections
  3. Renal calculi
  4. Hypertension - excess renin release
  5. CKD
  6. Hepatic cysts
  7. Berry aneurysms
  8. Mitral valve prolapse
  9. Aortic regurgitation
  10. Diverticular disease
  11. Inguinal hernias
  12. Polycythaemia - excess Epo production
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2
Q

Inheritance of polycystic kidney disease

A

Autosomal dominent

Type 1: 80% - mutation Chr 16
- PKD 1 gene

Type 2: 20% mutation Chr 4

  • PKD 2 gene
  • less severe
  • later onset
  • fewer cysts
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3
Q

Indications for nephrectomy in PKD

A
  1. Recurrent infections
  2. Intractable pain
  3. Making space for transplant
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4
Q

Barriers to renal transplantation

A
  1. Unable to match
  2. Current or recent malignancy
  3. Ongoing deep seated infections
  4. Obesity
  5. Ongoing vasculitis
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5
Q

Causes of chronic liver disease

A
  1. Alcohol
  2. Infection
    - Hep B, Hep C, CMV
  3. Malignancy
    - HCC
    - mets
  4. Auto-immune
    - autoimmune hepatitis
    - primary sclerosing cholangiitis
    - primary biliary cirrhosis
  5. Metabolic
    - Haematomachrosis
    - Wilsons disease
    - Non alcoholic fatty liver disease (NASH)
  6. Vascular
    - Budd Chiari
    - CCF
  7. Alpha 1 anti-trypsin deficiency
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6
Q

Chronic liver disease Ix

A
  1. FBC/U&E/LFT
  2. Coag!
  3. Albumin
  4. USS liver
  5. AFP
  6. Primary biliary cirrhosis = anti-mitochondrial antibody (M2 antibody)
  7. Iron studies - ferritin, iron, TIBC (decrease)
  8. Wilsons = decreased serum copper, decreased serum caeruloplasmin
  9. Primary sclerosing cholangiitis = ANA/AMA/ANCA may be +ve
  10. Autoimmune hepatitis = ANA + anti-smooth muscle antibody
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7
Q

Functions of spleen

A
  1. Sequestration + phagocytosis of old and abnormal RBC
  2. Extra-medullary haematopoeisis
  3. Immunological
  4. Blood pooling
    - 1/3 of platelets are held in spleen
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8
Q

Causes of splenomegaly

A

Infiltration:

  1. Malignant:
    - myeloproliferative
    - lymphproliferative
    - lymphoma
  2. Benign
    - amyloidosis
    - sarcoidosis
    - thyrotoxicosis
    - Gaucher’s disease (fat deposition disease)

Increased function

  1. Increased removal defective red blood cells
    - spherocytosis
    - thalassaemia
    - early sickle cell
    - nutritional anaemia
  2. Hyperplasia secondary to infection
    - malaria
    - visceral leishmaniasis
    - EBV (Glandular fever)
    - subacute infective endocarditis
    - TB
    - schistomiasis
    -
  3. Disordered immunoregulation
    - RA (Felty’s syndrome)
    - SLE
    - Amyloidosis

Defect in flow

  1. Portal hypertension
  2. Splenic vein thrombosis
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9
Q

Causes of massive splenomegaly

A
  1. Myelofibrosis
  2. CML
  3. Chronic malaria
  4. Leishmaliasis
  5. Gaucher’s disease
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10
Q

Genetic basis of Wilson’s disease

A

Mutation of ATP 7B gene

  • key to transport of copper into secretory pathway of the cell for incorporation into copper-containing enzymes and excretion of excess copper in the bile
  • mutation of gene leads to accumulation of free copper in hepatocytes
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11
Q

Hereditary haemorrhagic telangiectasia/Osler-Weber-Rendu syndrome
- presentations

A
  1. GI bleed
  2. Epistaxis
  3. Telangiectasia
  4. Complications of arteriovenous malformation
    - lung
    - brain
    - liver
    - bowel
    - spine
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12
Q

Haemochromatosis clinical features

A
  1. Hepatomegaly/cirrhosis
  2. Triad of fatigue/arthralgia/gonadal dysfunction (pituitary infiltration)
  3. Type 1 diabetes (bronzed diabetes)
  4. Skin discolouration
  5. Cardiomyopathy
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13
Q

Haemochromatosis genetics

A
  1. Autosomal recessive
  2. Prevalence 1 in 200
  3. 1/10 people carry the gene
  4. HFE gene: most common allele C282Y
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14
Q

Contra-indications for liver transplant

A
  1. IVDU
  2. Current EtOH XS
  3. Previous/current malignancy
  4. Significant medical or psychiatric co-morbidities
    5.
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15
Q

Varient syndromes for liver transplant

A
  1. Diuretic resistant ascites
  2. Intractable pruritus
  3. Recurrent cholangitis
    4.
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16
Q

Kings College Hospital liver transplant criteria for acute liver failure

A

Arterial lactate >3.5 4 hours after resus OR pH <7.3 or lactate >3 after 12 hours
INR >6.5
Creatinine >300
Encephalopathy grade III or IV

17
Q

Indications for splenectomy

A
  1. Trauma
  2. Hypersplenism
  3. ITP
  4. Haemolytic anaemia
  5. Vascular - aneurysm/clot of splenic artery
18
Q

Post splenectomy complications

A

Acute = bleeding

Infection

  • encapsulated bacteria: neisseria, strep pneumonia, haemophilus
  • pre-op vaccination against the above: pneumococcal, Hib vaccine, Men C vaccine
  • annual influenza vaccine
  • life long penicillin
  • anti-malarial if travelling abroad
19
Q

Hereditary sphrerocytosis

  • genetics
  • cause of haemolysis
  • clinical signs
  • Ix
  • Treatment
A

Genetics:

  • autosomal dominent or recessive
  • mutation in gene that codes for RBC membrane protein resulting in biconcave shape now being spherical

Cause of haemolysis
- extra-vascular haemolysis in spleen

Clinical signs

  • triad: fatigue, anaemia, jaundice
  • gallstones

Investigations

  • Hb, MCV, bilirubin, LDH, direct coombs (negative - to check for autoimmune), haptoglobin (reduced)
  • Blood film
  • Osmotic fragility test
  • EMA test - flow cytometry

Treatment

  • folic acid
  • splenectomy
  • cholecystectomy