Necrotizing Sarcoid Granulomatosis: Possibly Veiled Disease in Endemic Area of Mycobacterial Infection

No abstract available.

Alagille Syndrome: A Case Report

Alagille syndrome is a rare autosomal dominant disorder showing complicated systemic manifestations, although the hepatic manifestations are predominant in many patients. We report a case of Alagille syndrome in a male baby who presented with a heart murmur at 2 days old and his echocardiography showed aortic stenosis. He presented with acholic stool and jaundice at 3 months old and a liver biopsy revealed paucity of the interlobular bile duct in the portal tract. This progressed to cirrhosis, for which a liver transplantation was performed at 10 months old. The explanted liver showed biliary-type cirrhosis with severe cholestasis. There was an absence of the interlobular bile ducts on microscopic examination. Bile duct paucity, associated with cholestasis, a peculiar face (prominent forehead, deep-set eyes, pointed mandible and bulbous nasal tip), and cardiac anomaly were observed, which were consistent with Alagille syndrome. He died of heart failure.

Pseudofungi Associated with a Granulomatous Response in a Lymph Node: A Case Report

. We present herein a case of pseudofungi incidentally found in the mediastinal lymph nodes of a 31-year-old woman who had a left pneumonectomy for a pulmonary blastoma. The pseudofungi were located in the subcapsular sinuses of the lymph nodes with an associated granulomatous reaction. They revealed yellowish-brown hyphae-like structures with pseudosepta and irregular branching at various angles intermingled with round yeast-like forms. These structures stained positively with periodic acid-Schiff and Gomori methenamine silver, but also stained strongly positive for Prussian blue suggesting that they contain iron. The characteristic morphological features of pseudofungi are discussed with emphasis on the features that distinguish them from true fungal organisms.