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)

Of 62 patients with homozygous sickle cell disease subjected to open cholecystectomy for symptomatic gallstones over a 12-year period at the University Hospital of the West Indies, 25 were males and 37 were females. Of these, 27 were paediatric patients aged 18 years or less, and 35 were adults. Preoperative transfusion was selectively administered. All cases presented with right upper quadrant pain and 15 of 62 with obstructive jaundice. Mucocoele of the gallbladder, empyema of the gallbladder and common bile duct stones were detected in 2, 2, and 23 patients, respectively. Exploration of the common bile duct was necessary in 31 cases and a T-tube sited in 15 cases. Twelve of the 62 patients developed acute chest syndrome post operatively (20%). There were 2 deaths, both occurring in patients who had developed acute chest syndrome; in a 34 year old and a 10 year old patient. Common bile duct related morbidity was proportionately more common in paediatric patients than adults, represented by ductal dilation (48% vs 37%), ductal calculi (44% vs 31%) and retained stones (7% vs 3%).
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PMID:Biliary surgery in sickle cell disease: the Jamaican experience. 1187 1

Eighty cases (63 Males and 17 Females) of sickle cell disease were searched for gall stone by ultrasonography, 8 (10%) cases had gall stone. Gall stones were more common in males (11.1%) than females (5.8%). It was not found below 13 years of age. Three cases had single and five cases had multiple gall stones. Three of them had thickened gall bladder wall. Typical biliary colic was uncommon (1 case). There was no case of obstructive jaundice. HbF% level was significantly lower in cases with gall stone (12.31 +/- 3.95) than without gall stones (16.73 +/- 6.30). Episodes of aggravated anaemia and total serum bilirubin was significantly higher (1.27/pt and 4.12 +/- 1.34 mg%) in cases with gall stone than without gall stone (0.31/pt and 2.74 +/- 1.47 mg%). The above findings suggest association of greater degree of haemolysis with formation of gall stone in sickle cell disease cases.
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PMID:Cholelithiasis in sickle cell disease in India. 1252 Oct 86

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5-18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.
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PMID:Diagnostic and therapeutic ERCP in the pediatric age group. 1714 28