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)

Since June 1990, five girls and one boy have been evaluated for biliary colic. Gallstones were documented by sonography. Two girls, ages 8 and 14 years, had hereditary spherocytosis, and a 9-year-old boy had sickle cell disease. The other three girls, ages 13, 13, and 15 years, developed cholelithiasis and biliary colic without a history of hematological disease. Three children weighed less than 90 lb, with the smallest weighing 45 lb. All patients underwent laparoscopic cholecystectomy without complications. Operative cholangiography was performed in five of the six children. The KTP-532 laser was used for dissection of the gallbladder from the liver bed in two patients, and electrocautery was used in the remaining four. The average operating time was 1 hour 45 minutes. This is a report of the use of laparoscopic cholecystectomy in pediatric patients. The advantages of its use include a shorter hospitalization, decreased postoperative discomfort, and a much shorter interval between the surgical procedure and return to normal activities such as school and play. At this time, it is recommended for those children without complications from their cholelithiasis such as common duct obstruction and gallstone pancreatitis.
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PMID:Laparoscopic cholecystectomy in the pediatric patient. 183 14

The prevalence of gall stones was studied prospectively by abdominal ultrasound examination in 131 patients with sickle cell disease aged 10-65 years. Of 95 patients with homozygous sickle cell disease, 55 (58%) had gall stones or had had a cholecystectomy. Gall stones were present in four out of 24 (17%) patients with haemoglobin S + C disease and two out of 12 (17%) with haemoglobin S beta thalassaemia. The presence of gall stones was not related to sex, geographical origin, or haematological variables and was not associated with abnormal results of liver function tests. Symptoms typical of biliary colic were reported by 32 out of 47 adult patients with gall stones, and cholecystitis or cholestasis was diagnosed in 18. Cholecystectomy was performed in 29 patients with good relief of symptoms in most cases. Postoperative complications were common, occurring in 10 of the 28 patients who could be evaluated, but not generally serious; they were considerably lessened by a preoperative exchange transfusion that reduced the haemoglobin S concentration to below 40%. It is suggested that all patients with sickle cell disease should be screened for gall stones and that elective cholecystectomy should be performed in those with symptoms or complications.
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PMID:Gall stones in sickle cell disease in the United Kingdom. 311 90

Elective cholecystectomy was performed on 12 children (eight male and four female; age range, 4 to 19 years; and mean age, 11.2 years) with abdominal pain that was related to gallstones. Seven patients had jaundice, six had nausea, five had fat intolerance, and three had biliary colic. Two simple transfusions (10 mL/kg of packed red blood cells), designed to decrease the hemoglobin S content to less than 30% and to increase the total hemoglobin level to greater than 100 g/L, were given preoperatively two to three weeks apart. A third transfusion was given on the day before surgery if the total hemoglobin level was less than 100 g/L. The preoperative mean hemoglobin S content was decreased from 88% to 31%, and the mean total hemoglobin level was raised to 122 g/L. There were no preoperative or intraoperative complications. Post-operatively, no patients developed complications that were related to sickle cell anemia. Hospitalization averaged 6.3 days. Recurrent abdominal pain resolved shortly after surgery in all patients. With proper preoperative transfusions, elective cholecystectomy is safe in children with sickle cell anemia. Elective cholecystectomy should be recommended at the time of diagnosis of cholelithiasis.
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PMID:Cholecystectomy and cholelithiasis in sickle cell anemia. 338 86

Over a year (October 1992-September 1993), 30 patients (23 males and 7 females) with sickle cell anaemia and symptomatic gallstones underwent laparoscopic cholecystectomy (LC). The mean age was 26 years (range 15-44 years). The indications were: acute cholecystitis in four patients and long-standing biliary colic in the other 26. Twenty-four patients (80%) had a previous history of abdominal sickle cell crises. The preoperative HbF and HbS levels ranged from 18 to 33% and from 66 to 77.2%, respectively. The haemoglobin level ranged from 8.6 to 12 g% (mean 9.7 g%). Blood transfusion was given preoperatively to 19 patients (63%) and three patients needed perioperative transfusion. The mean operative time was 75 min (range 60-100 min). One of the four emergency cases was converted to open cholecystectomy due to difficult anatomy and inability to grasp a thick-walled, distended gallbladder. Two patients developed minor chest infections and one had an acute vaso-occlusive crisis on the fifth postoperative day and died despite the appropriate treatment (morbidity 6.6%, mortality 3.3%). The median hospital stay was 2 days (range 1-5 days). We believe that LC can be conducted safely in sickle cell patients with gallstones with minimal morbidity and mortality.
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PMID:Laparoscopic cholecystectomy in patients with sickle cell disease. 858 41

Gall stones in children have been commonly described in hereditary spherocytosis and sickle cell anaemia. In North India thalassemia is the commonest haemolytic anaemia. We studied the incidence of gall stones sonographically in children with thalassemia major. Of the 64 multitransfused children with thalassemia major studied (age range 5-20 years) none had symptoms of biliary colic or evidence of gall stones on sonography. The study has demonstrated that patients with thalassemia major are not prone to develop gall stones.
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PMID:Multitransfused children with thalassemia major do not have gallstones. 938 51

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