Prognostic indicators in Indian childhood cirrhosis

Nineteen cases of Indian childhood cirrhosis (ICC) were studied for determining prognostic indicators. Lower serum bilirubin and high serum alkaline phosphatase were associated with good prognosis. Mere presence of hyaline did not influence the clinical and biochemical parameters, however excess hyaline formation was associated with rapidly deteriorating course. Extent of reticulin condensation had no specific significance, but extensive fibrosis was associated with low serum alkaline phosphatase level and shorter survival period. There were six survivors in the study with a follow up upto 26 months and repeat liver biopsies in most of them showed very little changes.     

Zinc therapy in Indian childhood cirrhosis

Fifty five histopathologically proved cases of Indian childhood cirrhosis (ICC) were studied for the effect of oral zinc oxide/zinc sulphate therapy both in doses of 120 mg/day. Twenty six patients were given zinc oxide, 19 were given zinc sulphate and 10 cases served as control. Serum zinc was estimated by atomic absorption spectrophotometer (ASS). Serum zinc was low in all the stages of ICC and was directly related to the severity of the disease. Serum zinc was 72±11μg/dl in stage I; 65±14μg/dl in stage II; 57±18 μg/dl in stage III compared with 123±23μg/dl in normal children. Inspite of oral zinc therapy clinical condition and liver histopathology advanced in all patients and they expired due to hepatic coma. It is concluded that hypozincemia in ICC is a nonspecific finding and zinc therapy has no role in its management. This study was carried out by research grant from Medical Research Center, Bombay Hospital Trust, Bombay—20.     

Abnormal lactic dehydrogenase isoenzymes in patients of Indian childhood cirrhosis and their siblings

In fifteen patients with Indian childhood cirrhosis (I.C.C.) total lactic dehydrogenase (LDH) activity was increased as compared with controls (P<0.001). The isoenzyme pattern in I.C.C. patients was quite distinctive from that observed in normal controls and in patients with acute viral hepatitis. In I.C.C. there was a two fold increase in LDH isoenzyme-1, and a decrease in LDH-2 and LDH-4. Sixty per cent patients with I.C.C. had abnormal bands between LDH-1 and 2 and LDH-2 and 3. Siblings of the patients with I.C.C. had normal total LDH activity, and showed double ringed bands in the region of LDH 1–3. The abnormal bands in patients of I.C.C. are explained on the basis of hybridization of LDH with other serum components.     

Enzymatic studies and isoenzyme pattern of gammaglutamyl transpeptidase (GGTP) in Indian childhood cirrhosis (CC)

Histopathological studies of liver cell showing necrosis, parenchymal cell injury and microsomal cell damage are known to affect membrane bound enzymes such as gamma-glutamyl transpeptidase (GGTP), alkaline phosphatase (AP) and 5′-nucleotidase (5′-NT). The significant rise in GGTP, AP and 5′-NT indicates the damage of microsomes of parenchymal cell in Indian Childhood Cirrhosis (ICC) patients. SGPT/AP ratio may be an index for distinguishing ICC with other liver diseases. The isoenzyme pattern of GGTP in sera of control and ICC subjects have one band prominent activity. Similarly biopsy samples of control and ICC subjects show one prominent band of GGTP activity. The appearance of isoenzyme of GGTP in blood circulation is an index of microsome damage, cell necrosis and parenchymal cell injury. The isoenzyme pattern of GGTP could be a diagnostic test for ICC.     

Late manifestation of Indian childhood cirrhosis in a 3-year-old German girl

We report on a 3.8-year-old German girl who presented with signs of subacute liver failure based on a 4-month history. Investigations done before admission excluded infectious, metabolic and immunological diseases. Laboratory findings were increased values of aminotransferases, hyperbilirubinaemia, hyperammonaemia and deteriorated plasmatic coagulation. Caeruloplasmin and serum copper concentrations were normal; however, urinary copper excretion was elevated (80 μg/l). Liver biopsy showed a micronodular liver cirrhosis and an extremely high liver copper concentration (1400 μg/g dry weight). Epidemiological investigations revealed an elevated copper concentration (8645 μg/l, normal: <2000) and a low pH value (6.3) of the drinking water supplied by copper pipes. The girl had been exposed to copper-contaminated drinking water since the age of 2 years. Conclusion Laboratory, histopathological findings and a proven chronic copper intoxication lead to the diagnosis of Indian childhood cirrhosis in a German girl. Whereas this disease is mostly described in patients with increased copper intake in infancy, our patient developed toxic liver cirrhosis with relatively late copper exposure. Indian childhood cirrhosis should be considered in the differential diagnosis of early childhood liver cirrhosis. Received: 4 February 1998 / Accepted in revised form: 16 July 1998     

Levamisole therapy in Indian childhood cirrhosis

Nine histopathologically proved cases of ICC were treated with the immunopotentiating agent levamisole. Equal number of ICC patients were on steroid treatment and served as controls. Fatal outcome was invariable in both groups. The duration of treatment i.e. from beginning of treatment to death did not differ in the two groups. It is concluded that levamisole in the doses given has no therapeutic value in ICC.     

Hepatic elastosis in Indian childhood cirrhosis

Summary and Conclusions Elastic tissue changes in the liver biopsy material of 49 cases of I.C.C. wsre studied using various staining reactions. The changes in the hepatic elastica included thickening and coarsening of the elastic fibres in the walls of the blood vassels. New elastic fibre formation was observed in an occasional and sometimes fibrosed and thickened bile duct. The significance of these findings is briefiy discussed. From the Department of Pathology and Bacteriology, S.P. Medical College, Bikaner (Rajasthan).