Gastroenterology Flashcards

1
Q

What investigations would you order to assess progress of chronic liver disease?

A

Liver

  • LFT’s
  • Synthetic function (albumin, coagulation studies)

Nutrition

  • Fat soluble vitamins (retinol level, vitamin E level)
  • Calcium, magnesium, phophate

Portal hypertension

  • Abdominal USS with Doppler studies and portal pressures.
  • Endoscopy for varices.

Ascites

  • Ascitic tap

Encephalopathy

  • Clinical assessment
  • Ammonia, glucose and electrolytes.
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2
Q

What are complications of liver transplantation?

A

Rejection (60%)

  • Main cause of graft dysfunction
  • Most often in first 3 months
  • Increased transaminases, fever, malaise or jaundice.
  • Responds to pulse steroids, increased tacrolimus and addition of another immunosuppressant like mycophenylate.
  • If unresponsive treat with anti-T-cell antibody preperations (ATG, alemtuzumab).
  • Chronic rejection loss of >50% of bile ducts - optimise immunosuppression and dilate strictures.

Infection (50%)

  • Early (<1 month) - bacterial/fungal.
    • Intrabdominal and lines most common.
    • Fungal particularly with long ICU stays, reoperation or bowel perforation - prophylaxis with itraconazole, fluconazole or caspofungen.
  • Later (2-6 months) - viral (EBV, CMV, HZVZ)
    • Reduced with prophylactic ganciclovir or aciclovir.

Biliary obstruction (20%)

  • 6 weeks - 6 years post.
  • Percutaneous balloon dilatation, revision or retransplantation.

Hepatic vascular compromise (10%)

Post-transplant lymphoproliferative disorder (5-25%)

  • Risk factors: primary EBV infection following transplant, young age, EBV negative resipient of EBV positive donor, CMV infection, significant immunocompromise.

Recurrence of disease

Medication toxicity

  • Calcineuran inhibitors: tacrolimus and cyclosporine - renal impairment, hypertension, neurotoxicity and hyperlipidaemia
  • Steroids - Cushingoid features, high BSLs, cataracts, immunosuppression, osteopenia, adrenal crisis, hypertension.
  • Captopril - cough
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3
Q

Causes of chronic liver disease in child <5 years?

A

Infection

  • TORCH, echovirus, adenovirus, parvovirus B19, E. coli UTI

Structural

  • Extra hepatic biliary atresia, choledochal cyst, Alagille

Metabolic

  • Cystic fibrosis, AAT deficiency, hereditary fructose intolerance
  • Galactosaemia, tyrosinaemia, Niemann-Pick
  • Glycogen storage diseases - type IA & IV (Von Gierke & Anderson)

Endocrine

  • Hypothyroidism, hypopituitarism

TPN

Idiopathic neonatal hepatitis (25%)

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4
Q

Causes of chronic liver disease in children >5 years?

A

Infectious

  • EBV, CMV, toxoplasmosis, hepatitis A, B, C

Metabolic

  • Wilson disease, hereditary fructose intolerance, cystic fibrosis, AAT deficiency

Autoimmune chronic active hepatitis

  • Associated with thyroiditis, glomerulonephritis, erythema nodosum

Primary sclerosis cholangitis from IBD (>10 years)

  • Associated with arthritis, erythema nodosum, uveitis
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5
Q

What are the causes of portal hypertension?

A

Pre-hepatic

  • Portal vein thrombosis

Hepatic

  • Pre-sinusoidal - neoplasia, hepatic cyst, schistosomiasis.
  • Sinusoidal - A1AT deficiency, biliary atresia, cirrhosis, neonatal hepatitis.
  • Post- sinusoidal - veno-occlusive disease.

Post-hepatic

  • Budd-Chiari syndrome
  • Constrictive pericarditis
  • Right ventricular failure
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6
Q

What are the signs and causes of portal hypertension?

A

Cardinal sign is splenomegaly.

  • No hepatomegaly –> portal vein obstruction most likely.
  • Hepatomegaly but no signs of CLD –> congenital hepatic fibrosis.

Clinical features:

  • Splenomegaly
  • Cutaneous porto-systemic shunts (caput medusae, haemorrhoids).
  • Ascites, hypoalbuminaemia.
  • Thrombocytopenia.
  • Small, normal or enlarged liver.
  • FTT, reduced muscle bulk
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7
Q

What are treatment options for portal hypertension?

A

Medical:

  • B blockers
  • IV vasopressin

Endoscopy (varices):

  • Sclerotherapy
  • Banding
  • Balloon tamponade

Shunts:

  • Splenorenal or portocaval shunts - potentially limits future transplant, may precipitate encephalopathy.
  • Transjugular intrahepatic portosystemic shunt (TIPS) - temporising measure, used only in kids awaiting transplant.

Splenectomy

  • Avoid if possible, risk of infection.
  • Increased risk of bleeding

Liver transplant

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8
Q

What are common causes of need for liver transplant?

A

Structural

  • Extrahepatic biliary atresia (>50%)
  • Other (<5%)

Metabolic (15%)

  • A1AT, CF, galactosaemia, glycogen storage disorder (IA and IV), mitochondrial, Wilsons, organic acidaemia.

Acute hepatic necrosis (10%)

Infective (10%)

  • Chronic active hepatitis
  • Primary biliary cirrhosis

Primary malignancy (2%)

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9
Q

What are indications for liver transplant?

A

Poor quality of life

  • Intractable pruritis, lethargy, anorexia or recurrent infections.

Complications

  • Malnutrition (catabolic state)
  • Life-threatening variceal haemorrhage
  • Poor synthetic function (alb <20, INR >3.5)
  • Refractory hepatic encephalopathy
  • Refractory ascites
  • Hepatorenal syndrome

Underlying disease

  • Refractory primary sclerosing cholangitis
  • Hyperammonaemia - inborn error of metabolism
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10
Q

What substances can be checked for on stool sample and what do they indicate?

A

Fat globules

  • Fat malabsorption
  • Coeliac disease, pancreatic insufficiency, cholestasis, bacterial overgrowth

Fatty acid crystals

  • Fat malabsorption (partial hydrolysis)
  • Coeliac disease, cholestasis, bacterial overgrowth

White cells

  • Inflammation/infection
  • IBD, infection

Red cells

  • Inflammation/infection
  • Bleeding
  • IBD, infection

pH and reducing substances

  • CHO malabsorption
  • Lactose intolerance, sucrase isomaltase deficiency

A1 antitrypsin

  • Protein malabsorption
  • Intestinal lymphangiectasia, inflammatory bowel disease.
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11
Q

What are causes of hepatomegaly?

A

SHIRT

Structural

  • Extrahepatic biliary atresia
  • Choledocal cyst
  • Intahepatic biliary hypoplasia (Alagille)
  • Polycystic disease
  • Congenital hepatic fibrosis

Storage/Metabolic

  • Fat - CF, obesity, malnutrition
  • Lipid - Gaucher, Niemann Pick
  • Carbohdrate - DM, glycogen storage disorder (1 3 4 6), hereditary fructose intolerance, galactosaemia, Cushings, muccopolysccharidosis.
  • Protein - tyrosinaemia 1, urea cycle disorders.
  • Wilsons disease
  • A1AT
  • TPN cholestasis

Haematological

  • Thalassaemia
  • Sickle cell disease
  • ALL, AML, CML
  • Langerhan cell histiocytosis

Heart

  • Congestive cardiac failure
  • Constrictive pericarditis
  • Obstructed IVC

Infection - congenital or acquired

  • Viral - congenital rubella, CMV, EBV, coxsackie, echovirus, hepatitis A, B, C, D, E
  • Bacteria - E. coli UTI, Tb
  • Parasitic - hydatid, malaria, schistosomiasis, toxoplasmosis, visceral lava migrans

Infammatory

  • Chronic active hepatitis
  • Inflammatory bowel disease

Infiltrative

  • Langerhans cell histiocytosis
  • Sarcoiosis

Reticuloendothelial sytem

  • Non-Hodgkins
  • Hodgkins

Rheumatological

  • Systemic JIA
  • SLE
  • Sarcoidosis

Tumour/hamartoma/cysts

  • Primary - hepatoblastoma, hepatocellular carcinoma
  • Secondary - neuroblastoma, Wilms, gonadal
  • Haemangioma
  • Liver transplant

Trauma

  • Hepatic haematoma
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12
Q

What are causes of splenomegaly?

A

Cardiac - SBE

Connective tissue - sJIA, SLE

Haematological - chronic haemolytic anaemias

Infection

  • Viral - EBV, CMV
  • Bacterial - SBE, typhoid
  • Protozoal - malaria, toxoplasma

Injury - haematoma

Malignancy - leukaemia, lymphoma

Portal hypertension

  • Extrahepatic: post-neonatal umbilical vessel catheterisation/sepsis
  • Hepatic: cirrhosis, congenital heptic fibrosis
  • Suprahepatic: Budd-Chiari syndrome

Storage diseases - Gaucher, Neimann-Pick

Splenic cyst or hamartoma

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