Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5-15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) post-cholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)-(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.
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PMID:Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature. 1807 16

Intestinal lymphangiectasia may occur as a primary congenital disorder or a secondary disorder. Secondary lymphangiectasia could be associated with diseases such as abdominal carcinoma, retroperitoneal fibrosis or chronic pancreatitis. This is the first reported case of intestinal lymphangiectasia associated with recurrent histiocytosis X. This case report illustrates the need for more prospective, well-designed studies to determine the natural history and outcome of intestinal lymphangiectasia in the duodenum. Hopefully, these studies will also help clinicians identify which group of patients with intestinal lymphangiectasia in the duodenum is more likely to have a secondary cause.
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PMID:Intestinal lymphangiectasia associated with recurrence of histiocytosis X. 2194 59