Introduction: Written off in most books as a vanishing disease from the world with the advent of steel vessels, yet ICC still haunts North West India causing significant morbidity and mortality.

Aims: To study the varied clinical presentations, investigations and outcome of ICC.

Materials and Methods: All consecutive suspected cases of ICC coming to Pediatric Gastroenterology unit at PGIMER from Jan 95 to Dec 2004 were studied. All of them underwent detailed history and physical examination. The clinical diagnosis of ICC was based on large hepatomegaly, with a firm to hard liver having sharp leafy margins. Detailed dietary history and exposure to copper vessels was explored. The investigations carried out included hemogram, LFT, RFT, coagulogram, plasma hemoglobin, urine hemoglobin and ultrasound abdomen. All the patients having ascites underwent paracentesis. Urine copper estimation was done before and after D pencillamine in selected cases. Liver biopsy was done in patients who had normal coagulogram and platelet counts. Liver biopsy was sent for histopathological study and liver copper estimation. Upper gastrointestinal endoscopy (UGIE) was carried out wherever feasible. All patients were put on oral D pencillamine. (10 mg/kg/day).

Results: Total of 80 patients in the age group of 8 months to 3 years (mean 18.4 17.69 months) were studied. M:F was 5.2:1. Muslims and Christians were spared from the disease. On an average around 8 patients were seen annually which amounted to 1.6% of the total admissions in Pediatric Gastroenterology unit. History of exposure to copper vessels was found in 77 (96.25%). Majority of the children were top fed. Acute presentation (<2 weeks) was seen in 22 (27.5%) while others had an insidious onset (72.5%). All the patients seen had a history of irritability. Clinically majority had a decompensated (70%) liver disease where as 30% presented with compensated liver disease. The LFT revealed the mean bilirubin levels to be 8.98 10.52 mg/dl and the mean SGOT/SGPT levels were 174.89 and 110.78 IU. Hypoalbuminemia was seen in 53 (66.25%) and coagulopathy in 51 (63.75%). Ultrasound revealed hepatomegaly with altered echotechture alongwith splenomegaly and ascites in a few cases. Gall bladder was enlarged and palpable in 5 cases. Ascites was seen in 56.25% of the cases and 26.66% of them had SBP. Liver biopsy was possible in 36 patients and 7 patients underwent autopsy, thus a total of 41 patients had changes of ICC histologically. UGIE was possible in 51 patients and 66.66% of these had esophageal varices indicating portal hypertension. Mean serum copper was 191.5 44.40. Urinary copper pre D Pencillamine was 365.69 108.124 and post D Pencillamine was 983.75208.73. Liver copper studied in 18 patients was 571.72 microgram/g of wet weight of liver. 17 patients (21.29%) died during hospital stay and 16 (94.11%) had liver failure preterminally. Autopsy was done in 7 cases. Most of the children in stage three succumbed to the disease and 14 (17.5%) patients are still on follow up in PLC.

Conclusions: ICC continues to be a significant health problem with high morbidity and mortality in North West India. Around 70% children present in very late stage of the disease and have the evidence of portal hypertension. There is a need to create awareness among people regarding the adverse effects of the usage of copper vessels and top feeding. D Pencillamine is effective in the early stage of the disease.

Pedgastro 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 March 2006(Supplement 3)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
Indian_childhood_cirrhosis.asp