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Brief Reports Indian Pediatrics 2001; 38: 1035-1038 |
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Non-Wilsonian Cirrhosis in Turkish Children |
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Bahri Yurt, Nurten Koçak, Hasan Özen, Aysel Yüce, Figen Gürakan, Gülsev Kale
Many factors such as infections, anatomic malformations, metabolic disturbances, toxic substances, autoimmune hepatitis and cardio-vascular problems may cause chronic liver injury(1). In our country, a high incidence of parental consanguinity, hereditary metabolic disease and moderate endemicity of viral hepatitis B are important factors for develop-ment of cirrhosis(2). Wilson disease is the most frequent cause of cirrhosis in Turkey and we have previously published our experience on it(3). In this study we wanted to document the causes of nonWilsonian cirrhosis and their clinical, laboratory, histopathologic findings and prognosis. Subjects and Methods One hundred and fifty-two children with non-Wilsonian cirrhosis, who were diagnosed and followed between 1989 and 1999, were enrolled in the study. Hospital files of the patients were reviewed and information recorded. Etiological investigation of cirrhosis included urine and blood amino acids, serum tyrosine level, sweat chloride test, serum alpha-1 antitrypsin value, hepatitis B, C and TORCH serology. Patients were labelled as cryptogenic after all these tests were negative. Familial cirrhosis was defined in patients when there was another cirrhotic patient without a detectable cause in the family. Complete blood count, urine analysis and liver function tests including prothrombin (PT) and partial thromboplastin time (PTT) values had been performed at the beginning and later. Doppler ultrasonography was done on 8 and spleno-portography on 22 patients to see the portal circulation. Propranolol had been administered to those patients with a history of hematemesis and/or melena and to those with portal hypertension. One hundred and forty-five patients had histopathologic diagnosis (two of them post mortem). The diagnosis was established by clinical findings and laboratory investigation in seven patients because of bleeding risk. Results Seventy-eight patients (5l.3%) were male. The patients were diagnosed between 2 months and 15 years of age (median 5 years). Twenty-two (14.5%) patients had diagnosis before one year of age and 80(52.6%) had before 5 years of age. Only 27 (17.7%) had their diagnosis after 10 years of age. Parental consanguinity rate was 42.8%. Familial cirrhosis, chronic hepatitis B and biliary atresia were the frequent etiologic factors (Table I). No etiologic factor could be found in 93 (61.1%) cases. The most frequent complaint was jaundice (36.2%), and the most frequent physical find-ings were splenomegaly and/or hepatomegaly (Table II). Prolonged PT and elevated ALT and AST were the most frequently detected laboratory abnormalities. Splenoportography revealed signs of portal hypertension in 19 (86.4%) out of 22, and Doppler ultra-sonography in 7 out of 8 patients tested. Micronodular cirrhosis was diagnosed in 85 (60.3%) patients, mixed cirrhosis in 41 (29.1%) and macronodular cirrhosis in 4 (2.8%). Biopsy sample was insufficient for the diagnosis in 4 patients, and no morphologic classification could be done in 11 patients. Propranolol was administered to 42 patients. Nine (21.4%) of them experienced gastrointestinal bleeding during follow up. Sclerotherapy was performed on 4 patients. Splenectomy was done in 4 patients because of hypersplenism. Transjugular intrahepatic portosystemic shunt was performed on one patient. Three patients (tyrosinemia, Byler disease and cryptogenic) had liver trans-plantation and all died because of postoperative complications. Spontaneous bacterial perito-nitis developed in 2 (1.3%) patients. The patients were followed up to 15 years (range 2 days-15 years). Eighteen patients died because of hepatic failure (4 patients), postoperative complications (4 patients), liver transplantation complications (3 patients), and spontaneous bacterial peritonitis (1 patient). Three patients died at home.
Table I - Causes of Cirrhosis
TABLE II Clinical Features (n = 152)
Discussion In Turkey, a high incidence of parental consanguinity, hereditary metabolic disease and moderate endemicity of viral hepatitis B are important factors for development of cirrhosis(2). More than half of our patients were diagnosed before 5 years of age. This rate demonstrates that the majority of the patients were exposed to the etiologic factors at young ages. Although cirrhosis has more frequently been reported in males(2,4), there was no sex difference in this study. The rate of marriages among relatives is 21.1% in Turkey(5), lower than that in this study. This situation may explain the high frequency of familial/metabolic cirrhosis(6). An etiologic factor could not be detected in 61.1% of the patients. Familial/metabolic cirrhosis was the most frequent type followed by billary atresia and chronic hepatitis B. The rate of HBsAg carriage is 5% to 14% in Turkey, and chronic hepatitis B was responsible for 11.5-l3% of cirrhosis(2,4). Our findings show that the relative frequency of chronic hepatitis B in cirrhotic patients is decreasing. Focal biliary cirrhosis was detected in 4 cystic fibrosis (CF) patients. In one study it was shown that, until 4 years of age, 4% of CF patients had cirrhosis(7). Similar to the previous report (2), jaundice and protuberant abdomen were the most frequent complaints, and splenomegaly and hepatomegaly were the most frequent physical findings. At the time of diagnosis, 25.6% of the patients were decompansated. Spontaneous bacterial peritonitis is less frequently seen in children(8) and only 2 of our patients had spontaneous bacterial peritonitis during follow up. It may be due to frequent use of antibiotics for other infections. Decreased liver synthetic functions characterized by prolonged PT, hypoalbuminemia, reversed albumin/globulin ratio, and low cholesterol concentration was seen in 83.5%, 36.2%, 17.1%, and 14.5% of the patients, respectively. Thirty-two (21.0%) of our patients had iron deficiency anemia and 13 had hypersplenism. Propranolol is a widely used drug to pre-vent initial and recurrent variceal bleeding(9). Propranolol was administrated to 42 of our patients and only 9 (21.4%) of them had gastrointestinal bleeding during follow up. Micronodular cirrhosis was seen more frequently in this study and the majority of the patients were younger than 5 years of age. Macronodular cirrhosis was seen in older patients, supporting the contention that micronodular appearance may change to macronodular cirrhosis. It is concluded that cirrhosis is not uncommon in Turkish children. Although no etiologic factor could be detected in 61.1% of the patients, preventable causes were detected in half of the remaining patients. Contributors: BY participated in the data collection and drafting the paper. NK coordinated the study and will act as the guarantor. HO, AY, and FG helped in drafting the paper and took care of the patients. GL reevaluated the histological specimens for the study. Funding:
None.
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