Current spectrum of hepatobiliary disorders in northern India.

OBJECTIVE: To evaluate the current spectrum of hepatobiliary disorders in children in Northern India. SETTING: Tertiary level referral hospital. METHODS: All children with hepatobiliary disorders presenting between January 1992 through July 1995 were evaluated by clinical assessment, liver function tests, viral and autoimmune markers, liver biopsy, copper studies and other relevant investigations. RESULTS: Two hundred and thirty five children with hepatobiliary disorders were seen over three and a half years period (67 cases per year). Acute hepatitis (28%), chronic liver disease (36%) and neonatal cholestasis syndrome (NCS) (26%) were the most common patterns of liver diseases. Chronic liver diseases were constituted by ICC (2%), post-hepatitic etiology (13%), Wilson's disease (21%), autoimmune (4%), non-Wilsonian metabolic diseases (16%), hepatic venous outflow obstruction (2%) and non-cirrhotic portal fibrosis (1%). Cirrhosis was documented in 71% and chronic hepatitis in 12% of cases with chronic liver disease. Fulminant hepatic failure was the presentation in 4% of children with liver diseases. CONCLUSION: Chronic liver diseases in Northern India are mainly constituted by post hepatitic, metabolic and cryptogenic etiology and ICC is rarely encountered. NCS is also one of the major subgroups of liver diseases in children.

Role of biliary scintigraphy in management of bile leak.

BACKGROUND: Endoscopic biliary drainage is effective in the management of patients with postoperative bile leak. Evidently, it cannot be used in patients with completely tied-off common bile duct (CBD). AIM: To ascertain whether biliary excretion scintigraphy could reliably detect patients with tied-off CBD so that endoscopic retrograde cholangiography (ERC), an invasive test, could be avoided. METHODS: Twelve patients with active bile leak (11 postcholecystectomy, one posttraumatic), in whom the biliary system was imaged by both 99m technetium mebrofenin biliary scintigraphy and ERC, were evaluated. RESULTS: Bile leak was demonstrated in all the 12 patients by scintigraphy. In six of these, there was no visualization of radionuclide activity in the intestines; in three of these six patients, ERC showed a patent CBD potentially amenable to endoscopic drainage, whereas in the remaining three, there was complete obstruction presumably due to CBD tie-off during surgery. CONCLUSION: Biliary excretion scintigraphy is unreliable for detecting complete biliary obstruction in patients with bile leak.

Endoscopic, histologic and microbiologic evaluation of upper abdominal pain with special reference to Helicobacter pylori infection.

OBJECTIVE: To study children with significant upper abdominal pain of unidentifiable etiology and evaluate: (a) the relationship of pain to inflammatory esophago-gastro-duodenal lesions and Helicobacter pylori (HP) infection, and (b) the response to specific therapy. DESIGN: Prospective study. SETTING: Pediatric section of a tertiary referral gastroenterology center. SUBJECTS: Thirty three consecutive children with significant upper abdominal pain [mean age 9.9 +/- 2.7, range 4-15 years; 20 males] were subjected to upper gastrointestinal tract endoscopy and antral mucosal biopsies obtained for rapid urease test (RUT), Gram's staining of impression/crush smears and culture for HP and histologic examination. Patients with HP gastritis were treated with triple therapy, colloidal bismuth subcitrate, amoxycillin and metronidazole, for two weeks. At 8 weeks from the initiation of therapy, patients were re-evaluated for symptoms and HP eradication by repeat endoscopy and antral biopsies. Patients with esophagitis, gastritis and duodenitis without HP infection were treated with ranitidine for 6 weeks. All the patients were followed up for 6 months. RESULTS: Histology revealed antral gastritis in 28/33 (85%) patients. HP infection was present in 12/28 (43%) patients with antral gastritis. Symptomatic improvement with triple therapy was observed in 10/12 (83%) patients with HP gastritis and eradication of HP in 5/7. Improvement on ranitidine therapy was observed in 12/16 (75%) patients with HP negative gastritis. On follow-up, no patient with initial improvement with therapy had relapse of symptoms. CONCLUSION: Symptomatic children with HP related gastritis should be treated with triple therapy and HP negative gastritis with H2-receptor antagonist.

Neonatal cholestasis syndrome: an appraisal at a tertiary center.

OBJECTIVE: To know the magnitude, etiology and clinical profile, the efficacy of various investigations and the outcome in patients with neonatal cholestasis syndrome (NCS). DESIGN: Prospective evaluation of 60 consecutive infants with NCS (mean age 3.9 +/- 1.9 months; 49 males) over a period of 3.5 years. SETTING: Tertiary level referral gastroenterology center in North India. METHODS: Liver function tests, urine examination, serum antibodies against Cytomegalovirus (CMV), Rubella and Toxoplasma; abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy were done. In appropriate setting, laparotomy and surgical corrections were done for biliary tract disorders. RESULTS: NCS constituted 19% of pediatric liver diseases. Considerable delay in presentation was observed [mean delay, extrahepatic biliary atresia (EHBA) = 4 +/- 2.0 months, neonatal hepatitis (NH) = 2.2 +/- 1.3 months]. Thirty three (55%) infants had EHBA, 14 (23%) NH (4 CMV, 2 galactosemia, 1 urinary tract infection and 7 idiopathic), 2 (3%) paucity of intralobular bile ducts and 11 (18%) were of indeterminate etiology. Liver biopsy was the most accurate (96.4%) investigation in discriminating between EHBA and NH. Of the 18 operated infants with EHBA (portoenterostomy-15 and hepatico-jejunostomy-3), 10 were alive (mean follow up = 22.8 +/- 8.6 months) of which 4 were completely asymptomatic. CONCLUSIONS: (i) NCS is an important cause of liver disease in Indian children. (ii) It requires prompt referral, quick investigative approach and targeted management. (iii) Liver biopsy is highly accurate in differentiating EHBA and NH. (iv) infants with EHBA and compensated status of liver should undergo corrective surgery irrespective of age at presentation.

Therapeutic gastrointestinal endoscopy.

The safety, efficacy and utility of various therapeutic gastrointestinal (GI) endoscopic procedures performed on children (January 1992 to July 1995) at a tertiary referral centre in India were studied. A total of 1,030 sessions (upper GI 972 and lower GI 58) of therapeutic GI endoscopy were performed in 162 children (mean age 7.4 +/- 4 years; upper GI 115, lower GI 47). Various upper GI endoscopic procedures done were injection sclerotherapy (EIS), endoscopic variceal ligation (EVL), bougie dilatation of oesophageal strictures, balloon dilatations of oesophageal stricture/pyloric obstruction and retrieval of foreign bodies in 75%, 6%, 9%, 4% and 12% of children respectively. Therapy for bleeding from oesophageal varices constituted the major group (75%). Repeated EIS (sessions total--876, mean 8, range 5-15) performed on 86 children resulted in control of bleeding in all and eradication of oesophageal varices in 85% of cases. Minor complications (oesophageal ulcers and oesophageal strictures) due to EIS were observed in 9% of children. EVL (10 sessions in 7 children) was effective in controlling bleeding and substantial decrease of varices in all without any complication. Oesophageal dilation either by bougie (61 sessions in 10 children) or balloon (6 sessions in 3 children) were performed for benign strictures. Balloon dilatation of pyloric obstruction was successfully done in 2 children. Foreign bodies (retained or sharp) were retrieved from upper GI tract in 14 children. No complications were observed with stricture dilatation/foreign body retrieval. Therapeutic lower GI endoscopies were performed in 47 children (colonoscopic polypectomy in 92%, anal dilatation and piles banding in 4% each). One child with juvenile polyposis coli developed sigmoid colon performation following colonoscopic polypectomy which required surgical correction. We conclude that upper and lower GI endoscopic therapeutic procedures in children are highly effective and safe. The risk of major complication is very small in experienced hands and occasional minor complications, can be managed conservatively.

Falciparum malaria with acute liver failure.

INTRODUCTION: Falciparum malaria occasionally presents with encephalopathy, jaundice and fever mimicking fulminant hepatic failure. PATIENTS: We recently managed seven cases (mean age 34 years, range 20-45; all men) of acute falciparum malaria presenting with a short history [mean duration 8.1 (4-15) days] of fever, jaundice, altered sensorium and oliguria. Only one patient had splenomegaly. Investigations revealed jaundice (bilirubin 1.9-30.7 mg/dl), moderate to severe anaemia (Hb 4-8 gm/dl), increased liver enzymes (2-4 times normal) and azotaemia (serum creatinine 1.6-7.4 mg/dl). Coagulation parameters were deranged in 3 with clinical bleeding in two cases. One patient without a past history of diabetes had increased blood glucose values with ketonuria. HBsAg was negative in all cases. Patients received supportive therapy along with intravenous quinine. Peritoneal dialysis was done in one patient. Three patients showed rapid recovery and four succumbed to the disease. Post-mortem liver biopsy showed Kupffer cell hyperplasia, pigment deposition, foci of steatosis and necrosis along with submassive necrosis in one case. CONCLUSIONS: In areas endemic for malaria, awareness of this entity is a must. In a patient with jaundice and altered sensorium, disproportionate anaemia, azotaemia and only mild elevation of liver enzymes should help differentiate these patients from cases of fulminant hepatic failure. The diagnosis can be confirmed by peripheral blood examination. Early institution of specific therapy may be the only life saving measure in these patients.

Biliary ascariasis associated with periampullary carcinoma.

A 65 year old man presented with obstructive jaundice, biliary colics and recurrent cholangitis. Sonography revealed dilated intrahepatic biliary radicles and common bile duct. Bile duct also showed linear parallel intraluminal structures suggesting biliary ascariasis. The lower end of common bile duct and pancreatic region showed a mass which proved to be a coexistent periampullary carcinoma.