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Tuesday, May 4, 2010

Zieve's syndrome

A 50-year-old man presents with shortness of breath. He is a known alcoholic and has a past history of ischaemic heart disease. On examination he looks jaundiced but his abdomen is soft and non-tender and his chest sounds clear with good breath sounds bilaterally.
His serum blood tests show the following results:

Hb7 g/dl
Bilirubin35 μmol/l
GGT400 iu/l
Cholesterol10 mmol/l
Which of the following diagnosis do you think he has?
  Zieve’s syndrome
Your answer
  Whipple’s disease

  Crigler–Najjar syndrome

  Gilbert’s syndrome

  Non-alcoholic steatohepatitis syndrome (NASH)
 

Zieve’s syndrome occurs in patients with excessive alcohol consumption with haemolysis and severe hyperlipidaemia, abdominal pain, transient mildly raised bilirubin. It usually occurs in males and resolves once alcohol consumption is stopped. Whipple’s disease is a cause of gastrointestinal (GI) malabsorption usually in middle-aged men, and is caused by the Gram-positive bacteria Tropheryma whippelii. Crigler–Najjar syndrome is a rare congenital disorder of bilirubin conjugation. It has two distinct forms, type 1 and type 2: type 2 patients can survive to adulthood without any neurological impairment, whereas this impairment is seen in neonates in type 1 cases. Gilbert’s syndrome is a benign cause of isolated hyperbilirubinaemia and can be affected by illness, surgery or dehydration. It is inherited in an autosomal recessive pattern. Non-alcoholic steatohepatitis (NASH) is the syndrome of fatty liver not caused by alcohol related to raised cholesterol, obesity and type 2 diabetes.

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