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69 years old woman without antecedents of hepatic disease who was admitted to Intensive Care Unit by severe pneumonia. Clinical exploration with nodular hepatomegaly without ascitis or splenomegaly. Liver function test with total proteins 7 g/dL, albumin 3.6 g/dL, Globulins 3.4 g/dL, total bilirrubins 0.62 mg/dL, Direct bilirrubin 0.15 mg/dL, Indirect bilirrubin 0.47 mg/dL, ALT 31 U/L, AST 40 U/L, AP 85 U/L, GGT 34U/L, LDH 188 U/L. Creatinine 0.8 mg/dL, BUN 13.3 mg/dL. Computed tomography shows multiple hepatic and renal cysts. Hepatic Ultrasound shows multiple hepatic cysts and a normal portal system, anterograde portal flow, suprahepatic veins and biliary tract without abnormalities or obstruction. Autosomal dominant polycyst liver disease is a rare disorder usually associated with autosomal dominant kidney polycyst disease with an increasing prevalence associated with age and female gender. Patients with autosomal-dominant polycystic disease present a wide spectrum of hepatic and renal involvement. Some patients have renal cysts only; others have liver cysts only; while others show variable degrees of both kidney and liver involvement. Although usually asymptomatic, patients with Adult Policyst Liver Disease (ALDP) may present with abdominal pain or swelling. Liver function is not usually compromised and computed tomography or abdominal ultrasonography is the most useful investigations. The complications are cyst rupture, infection, portal hypertension, obstructive jaundice, liver failure, cholangiocarcinoma and compression of surrounding structures.

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2010-09-28   |   1,033 visitas   |   1 valoraciones

Vol. 17 Núm.3. Julio-Septiembre 2010 Pags. 144-145 Rev Invest Med Sur Mex 2010; 17(3)