Laboratory studies include specific tests for diagnosis of the underlying illness and testing to monitor the status of the patient. Children presenting for the first time with evidence of chronic liver disease should have a standard investigation (Table 130-5). Monitoring should include coagulation tests, electrolytes and renal function testing, complete blood count with platelet count, transaminases, alkaline phosphatase, and γ-glutamyltransferase at appropriate intervals. Frequency of testing should be tailored to the pace of the patient’s illness. Ascites fluid can be tested for infection by culture and cell count and generally is found to have an albumin concentration lower than that of serum.
Serum albumin AST, ALT, GGT, alkaline phosphatase Total and direct bilirubin Serum cholylglycine or bile acids Serum cholesterol Ultrasound examination of liver and bile ducts Doppler ultrasound of hepatic vessels* Magnetic resonance cholangiography* Magnetic resonance angiography of hepatic vessels* Percutaneous or endoscopic cholangiography* Liver biopsy*
Ultrasound of liver, pancreas, and biliary tree
Consider magnetic resonance cholangiography or ERCP if evidence of biliary process Liver biopsy—as required for diagnosis or prognosis
Height, weight, skinfold thickness 25-Hydroxyvitamin D level Vitamin A level Vitamin E level Prothrombin time and partial thromboplastin time before and after
Treatment of chronic liver disease is complex. Supportive carefor each of the many problems encountered in these patientsis outlined in Table 130-6. Ultimately, survival depends on theavailability of a donor liver and the patient’s candidacy for transplantation. When transplantation is not possible or is delayed,palliative procedures, such as portosystemic shunts, can beconsidered. The transjugular intrahepatic portosystemic shuntis an expandable stent placed between the hepatic vein and abranch of the portal vein within the hepatic parenchyma. Thisprocedure is performed using catheters inserted via the jugularvein and is entirely nonsurgical. All portosystemic shunts carryincreased risk of hepatic encephalopathy.
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Table 130-6 Management of Chronic Liver Disease PROBLEM CLINICAL MANIFESTATIONS DIAGNOSTIC TESTING TREATMENT Gastrointestinal variceal bleeding* Hematemesis, rectal bleeding, melena, anemia CBC, coagulation tests, Doppler ultrasound, magnetic resonance angiography, endoscopy Somatostatin (octreotide) infusion, variceal band ligation or sclerotherapy, propranolol to reduce portal pressure, acid-blocker therapy, TIPSS or surgical portosystemic shunt if transplant not possible
CBC, Complete blood count; TIPSS, transjugular intrahepatic portosystemic shunt; WBC, white blood cell.
*May also have peptic ulcerations.