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Congenital hepatic fibrosis (CHF) is a recognized cause of portal hypertension with oesophageal varices, gastro-intestinal haemorrhage and cholangitis in children without significant impairment of hepatic or renal function. This report describes the varied clinical presentation of CHF as seen at King Faisal Specialist Hospital and Research Centre (KFSH & RC) and emphasizes the clinical patterns that should enable a pediatrician to consider the diagnosis. Fourteen children with CHF were diagnosed between 1981 and 1988. The age at presentation ranged from 1.8-14 years (mean: 7.5 years); clinical manifestations at diagnosis were splenomegaly (12), hepatomegaly (11), failure to thrive (10), marked abdominal distention (4), and fever (4). Liver function tests were normal except for high alkaline phosphatase. Eight patients had polycystic kidneys confirmed on ultrasound examination. Upper gastro-intestinal endoscopy showed oesophageal varices of variable severity in all eight patients examined. Splenoportography revealed splenic vein occlusion in one patient. One patient died within days of admission with convulsions, coma, and aspiration pneumonia. One patient was lost to follow-up. The remaining 12 patients are all alive and receive regular follow-up. Two patients required splenorenal shunt. In view of the prevalence of consanguinity in Saudi Arabia, the diagnosis of CHF should be considered in children with hepatomegaly despite normal liver function tests, and particularly in those with renal abnormalities and/or evidence of portal hypertension.

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We reviewed 100 consecutive cases of chronic active hepatitis, 50 of cirrhosis and 25 of chronic persistent hepatitis. Hepatitis B surface antigen was positive in 51 of the chronic active hepatitis cases and antinuclear factor, smooth muscle antibody, and antimitochondrial antibody were negative in all except one test. In contrast to the findings in chronic autoimmune hepatitis, 69% of our patients were male; symptoms were usually nonspecific; fever, arthritis, and autoimmune manifestations were absent; and anemia occurred in only six cases. The diagnosis of chronic hepatitis was often first suggested by routine biochemical profiles. Serum albumin, bilirubin, alkaline phosphatase, and lactic dehydrogenase were abnormal in less than 50%. Serum alanine aminotransferase (SGPT) was abnormal in 93% at time of biopsy and exceeded the aspartate aminotransferase (SGOT) in all cases of chronic persistent hepatitis, 95% of mild and moderate chronic active hepatitis, 77% of severe chronic active hepatitis, and 21% of cirrhosis. If the alanine aminotransferase/aspartate aminotransferase (SGPT/SGOT) ratio is less than 1, cirrhosis, hepatoma, or alcoholic liver disease should be suspected. The serum transaminase levels did not correlate with histological severity of chronic active hepatitis, showed marked fluctuation, and became normal in 39% of untreated patients during a mean follow-up period of 11 months. The serum transaminase levels therefore should not be used to monitor disease progression.

The screening value of the one-hour blood xylose test, corrected for body surface area, was prospectively studied in Saudi Arabian adults and children under investigation for suspected intestinal malabsorption. Sensitivity of discrimination between patients with and without upper small bowel disease was 91%, compared to 85% for the five-hour urine xylose test. Primary small bowel disorder was rare. In a three-year review, no cases of adult coeliac disease or tropical sprue were found. The most common causes of malabsorption were intestinal tuberculosis, abdominal lymphoma and immunoproliferative small intestinal disease. Despite its acceptability as an index of proximal small bowel function, the blood xylose test alone is an inadequate screening test for any of these conditions.