Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Splenectomy is frequently required in children with various hematologic disorders. The reported advantages of laparoscopic splenectomy (LS) include less pain, shorter hospital stay, and improved cosmesis. This report evaluates the outcome of children undergoing LS at a single children's facility. One hundred twelve children underwent LS by the lateral approach between August 1995 and February 2001. Indications for LS were hereditary spherocytosis in 58, idiopathic thrombocytopenic purpura in 21, sickle cell disease in 19, and other conditions in 14. LS alone was completed in 89 children and LS and cholecystectomy (LSC) in 20. Three required conversion to open splenectomy. Accessory spleens were identified in 19. Complications included ileus (four), acute chest syndrome (four), bleeding (two), pneumonia (one), and diaphragm perforation (one). There was no mortality. An accessory spleen was missed in one child with recurrent anemia. Average operative time for LS was 106 minutes and for LSC 135 minutes. Operative time for LS decreased with experience but the difference was not significant. Average length of stay was 1.51 days (range 1-11) and was longer in sickle cell disease (2.47 days) versus hereditary spherocytosis (1.29 days) and idiopathic thrombocytopenic purpura (1.16 days). We conclude that LS is safe and effective in children with hematologic disorders and is associated with minimal morbidity, zero mortality, and a short length of stay.
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PMID:Laparoscopic splenectomy has become the gold standard in children. 1189 57

Gallstone ileus is a diagnosis of rarity, and a proximal site of obstruction in a young patient is even rare. Of the three cases in our experience, we found two cases of gallstone ileus (GSI) with typical epidemiology and presentation, one had combination of multiple rare associations. We report such a case, suspected to have gallstone ileus on ultrasound and confirmed diagnosis on computed tomography. Presence of biliary-enteric fistula, old age, and obstructive features, as in typical cases, was a bigger asset for diagnosis, but it was difficult to entertain diagnosis of GSI in young girl in absence of a demonstrable biliary-enteric fistula, with uncommon association of choledochal cyst and sickle cell disease. A very surprising finding, dilated major papilla, could however explain the pathogenesis which has also been reported in the past. Although differential opinions regarding management exist, we decided to follow two-stage surgery as our institute protocol. A minimal access approach has been immensely helpful in accurate diagnosis, and expedative management with early recovery has been proven in the past studies which we agreed with our experience.
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PMID:Laparoscopic Management of a Proximal Jejunal Gallstone Ileus with Patulous Ampulla and Choledochal Cyst-a Report of Unusual Presentation and a Review. 2833 Dec 67