Copyright © 2006 by Indian Society of Gastroenterology A n eleven-year-old boy presented with high-grade fe ver with chills and cough for 15 days. Cough was productive with yellowish sputum and streaked with blood. There was no history of vomiting, loose stools, abdomi- nal distension, jaundice or hematemesis. He was a full- term normal-delivery baby with normal milestones. In January 2004, he was found to be jaundiced and was diagnosed to have autoimmune hepatitis (AIH). At that time he had negative HBsAg, anti-HCV, anti-nuclear antibody (ANA) and liver-kidney microsome (LKM) anti- bodies; anti-smooth muscle antibody (SMA) was posi- tive (++), serum ceruloplasmin was 44.7 mg/dL, Kayser- Fleischer ring was negative. Liver biopsy showed diffuse lobular disarray, interface hepatitis, porto-portal bridging fibrosis, and moderate activity. Hepatocytes showed fea- tures of regeneration with prominent pseudo-rosette for- mation. Gastroscopy revealed grade II esophageal varices. He was put on prednisolone and azathioprine. In May 2005, he presented with esophageal variceal bleed. He was treated with endosclerotherapy. On inves- tigation, he was found to have pancytopenia; azathioprine was stopped and prednisolone restarted. In November 2005, he presented with ascites. Biliru- bin was 1 mg/dL, AST 39 IU/L, ALT 127 IU/L, alkaline phosphatase 139 U/L, albumin 2.6 g/dL. The dose of pred- nisolone was increased to 60 mg/day, and frusemide and spironolactone were started. On examination, his height was 148 cm and weight 43 Kg. He had cushingoid facies. Pulse was 120 per minute, body temperature was raised and he was pale. He was mildly icteric, with no cyanosis or clubbing. He had a pal- pable liver just beneath the right costal margin, firm. Spleen was palpable 5 cm below the left costal margin; clinical signs of ascites were doubtful. Cardiovascular system examination was normal. He had reduced breath sounds on the right inter- and infra-scapular regions with bron- chial breathing and crepitations. Investigations (Table) ESR was normal, reticulocytes 4.9%, and malarial para- sites were absent on peripheral smear examination. Serum bilirubin and alkaline phosphatase were normal. Sputum culture on two occasions was sterile; sputum smear for acid-fast bacilli was negative. Blood culture, including fungal, was sterile. Urine routine examination was normal. Arterial blood gases showed pH 7.55, pO 2 64, PCO 2 31, HCO 3 – 26, SPO 2 89% (January 26) and pH 7.57, PO 2 57, PCO 2 33, HCO 3 - 29.8, SPO 2 93% (February 6). Ultrasonography showed liver span of 13 cm with enlarged caudate lobe, coarse echotexture, irregular sur- face; portal vein measured 12 mm in diameter at the porta. Gall bladder and common bile duct were normal. Splenic vein was of normal caliber. Spleen was enlarged, measur- ing 15 cm. Both kidneys and pancreas were normal. Chest X-ray January 18 showed thin-walled large cyst in the left lower lobe with adjacent consolidation (Fig 1). Five days later, it showed increasing size of the cyst and opacity in the left lung, with multiple small nodular consolidations in the right lung. A week later X-ray showed increasing size of cavity as well as surrounding consolidation, with extensive nodular consolidation of the right middle and lower lobes (Fig 1). CT scan showed bilateral consolida- tion (nodular infiltrates) with cavitations. Radiological impression: infective condition or vasculitis. The infec- tive causes would include bacterial, nocardial, fungal and tuberculosis, as the child was immune-compromised. Management and course in hospital Prednisolone (60 mg) and frusemide-spironolactone (40- Macronodular cirrhosis and fever in a boy Kim Vaiphei, Uma Nahar Saikia, Babu Ram Thapa,* Ranjana Walker, @ Kushaljit Singh Sodhi, + Radha Krishan Dhiman # Departments of Histopathology, *Pediatric Gastroenterology, @ Immunopathology, + Radiodiagnosis and # Hepatology, Post graduate Institute of Medical Education and Research, Chandigarh 160 012 Clinico-pathologic conference Table: Laboratory investigations January 19, 2006 January 25 January 26 February 2 February 6 February 7 February 8 Hb (g/dL) 11.6 11.8 12.6 11.6 TLC (/cmm) 8100 7600 13000 16400 DLC (P, L, M, E) 74, 20, 2, 4 65, 30, 4, 1 80, 16, 3, 1 Platelets (/cmm) 48 54 47 AST (IU/L) 44 57 ALT (IU/L) 93 43 Proteins (g/dL) 6.0 5.7 Albumin (g/dL) 2.2 2.0 Prothrombin index (%) 50 43 50 Creatinine (mg/dL) 0.6 1.6 2.2 2.3 1.9 Urea (mg/dL) 41 99 141 117 99 Na/K (mEq/L) 115/4.6 125/3.8 115/3.1 110/3.7 110/3.4 Indian Journal of Gastroenterology 2006 Vol 25 September - October 251