Dr. Ashish Bavdekar *
Consultant Pediatric Gastroenterologist,
Liver & Gastroenterology Unit KEM Hospital, Pune. *
|Up until the 1970s, |
Indian Childhood Cirrhosis (ICC) accounted for 50% of all chronic liver disease (CLD), 5% of all pediatric admissions and 10% of ward mortality at the KEM Hospital in Pune. Today, ICC is a rarity in India with only an occasional case reported annually even from large medical centers. On the other hand, disorders like, Wilson's disease, neonatal cholestasis, chronic hepatitis, etc have become relatively more important as treatable forms of pediatric liver disease.
Obstructive jaundice in early infancy now appears to be the commonest presentation of CLD in our country. Differentiation into biliary atresia, neonatal hepatitis, biliary hypoplasia though desirable early is difficult unless an investigation protocol is followed. As against the better prognosis in neonatal hepatitis, the surgical outcome in biliary atresia is poor primarily due to late surgery. Continued attacks of cholangitis and progressive cirrhosis inspite of adequate biliary drainage is especially frustrating. Liver transplantation, though available in India today, is far too expensive to be considered a realistic technology at least for the present.
Wilson's Disease (WD)
WD is now increasingly identified probably because of:
- Increased awareness of the disease and
- Improvement in investigatory facilities. The most striking feature of WD is the extremely varied clinical presentation, ranging from purely neurological symptoms to behaviour disturbances, scholastic regression, hemolysis and resistant rickets. The predominant presentation in children is however hepatic and almost any form of liver disorder can be mimicked. Rate of progression and response to therapy are also unpredictable but generally inversely related to duration of symptoms. Effective therapy arrests further liver damage, reduces neurological and osseous abnormalities and occasionally reverses cirrhosis but is lifelong and expensive.
Autoimmune chronic active hepatitis needs a high index of suspicion for diagnosis. The presentation like WD can be extremely varied ranging from acute hepatitis to acute liver failure to typical CLD. Auto-antibodies are now available at selected laboratories. Response to treatment is usually good but prolonged. Chronic hepatitis B is not very common at our center, while chronic hepatitis C will only rarely be seen in early childhood.
Metabolic liver disease (MLD)
Glycogen Storage Disease (GSD), Gaucher's Disease, Niemann Pick disease, galactosemia, tyrosinemia, etc are some of the MLDs seen often. GSD frequently manifested with massive hepatomegaly usually without splenomegaly and short stature with adiposity particularly of cheeks ("moon faces"). Hypoglycemia, convulsions, acidosis may not be present. Diagnosis of GSD is usually easy on biopsy but differentiation into various types is cumbersome, beyond the realms of our labs and often irrelevant for management.
Venous outflow obstruction disorders in the form of Budd Chiari syndrome or VOD through rare are important in the differential diagnosis of CLD. Histology in the form of marked central venous congestion with necrosis of hepatocytes and increased fibrosis leading to conclusion is characteristic. Hepatic neoplasms, congenital hepatic fibrosis, etc are other CLDs seen.
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|Bavdekar A D.. Available From : http://www.pediatriconcall.com/fordoctor/ Conference_abstracts/report.aspx?reportid=195|