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

Gall bladder ultrasonography was performed on 157 fasting patients (mainly adults) with sickle cell disease (133 SS and 24 SC) with a view to establishing the prevalence of cholelithiasis in Nigerian 'sicklers'. There were 82 females and 75 males whose ages ranged from 9 to 60 years (mean 34.5). Gallstones were demonstrated in 38 patients, giving an overall prevalence of 24.2% (22.6% in SS and 33.3% in SC). Their ages ranged from 18 to 56 years (mean 37.0) in HbS + C patients and 10-34 (mean 22.0) in SS patients. All the patients except one were asymptomatic. Autopsy confirmed gallstones in two SS patients who died of unrelated problems. The higher prevalence obtained in this study (24.2%) compared with the prevalence (less than 10%) in earlier studies from Africa could be due to the predominantly adult age group screened and the greater sensitivity of cholecystosonography.
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PMID:Prevalence of cholelithiasis in Nigerians with sickle cell disease. 255 Nov 64

Nigeria has a population of 112 million with an annual growth rate of 3.2%. About 25% of adults throughout the country have the sickle cell trait, AS, while the Hb C trait is largely confined to the Yoruba people of southwestern Nigeria in whom it occurs in about 6%. Other variant hemoglobins including beta thalassemia are rare, but alpha thalassemia occurs in 39% (32% with 3 alpha-globin genes; 7% with 2 alpha-globin genes). Of a total of 5.4 million expected live births in 1988, about 90,000 will have SCD and 1.1 million the trait, AS. The clinical phenotype of sickle cell anemia is severe with manifestations occurring very early in childhood and mean Hb level 7.6 g/dl with HbF 5.9%. A very high infant mortality due to infections occurs especially in rural areas. Gallstones, leg ulcerations, and stroke appear less common than in American sicklers, and aplastic crises have not been described. Poor availability of resources to the public health and welfare sectors and economic inflation are severely curtailing access to appropriate medical and social services. This situation is frustrating to the families of a growing number of surviving patients in urban or middle to upper income groups. Efforts to create more awareness of SCD are paradoxically increasing frustration and stigmatization in the absence of a commensurate improvement of services. Any measures aimed at enhancing the sensitization of health professionals, policy makers, and resource allocators to the pertinent issues in the control of SCD would seem to be at this stage an important step in the right direction.
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PMID:A profile of sickle cell disease in Nigeria. 267 62

Homozygous SS sickle cell anemia affects 0.25% of the population of the West Indies. Gallstones are frequently found in this blood disease and are the cause of recurrent abdominal pain, cholecystitis and dangerous infectious complications in these patients. These complications are difficult to distinguish from very frequent episodes of vaso-occlusive abdominal pain. Three cases in childhood sickle cell disease are reported. The authors believe that elective cholecystectomy is to be recommended (emergency cholecystectomy is associated with a high morbidity) as the children operated were improved by surgery, with resolution of abdominal pain.
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PMID:[Value of elective cholecystectomy in children with homozygote sickle cell anemia. Apropos of 3 recent cases]. 275 43

Twenty-eight patients with sickle cell disease who presented with acute abdominal pain were evaluated with clinical laboratory, ultrasound, and biliary scintigraphic studies to determine their predictive capabilities for differentiating veno-occlusive disease (sickle cell crisis) from acute biliary disease. Eleven patients were treated surgically and 17 medically. Gallstones were demonstrated in 25 (90%) of the patients studied by ultrasound, and biliary scans were abnormal in nine patients (32%). Abnormal laboratory and radiological studies (ultrasound, biliary scintigraphy) were not significantly different in the two groups and had a low positive predictive value for detection of acute biliary disease. Nevertheless, normal biliary scintigraphy had high negative predictive value in that, if normal, it gave assurance that the cystic duct was patent and unnecessary surgery could be obviated in this high-risk category of patients.
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PMID:Diagnostic studies in patients with sickle cell anemia and acute abdominal pain. 329 81

The effect of hemolysis and infection/stasis on pigment gallstones was assessed by comparing the composition of stones from (1) U.S. patients without hemolysis or cirrhosis, (2) U.S. patients with sickle cell disease, and (3) Japanese patients with biliary infections. Gallstone composition was quantitated by infrared spectroscopy and chemical analyses. Gallstones from patients with sickle cell anemia contained more pigment, carbonate, calcium, and measured components than stones from U.S. patients without hemolysis (P less than 0.05). However, the similar types of calcium salts in black stones from patients with and without sickle cell anemia suggested that intermittent hemolysis may be a potential mechanism in the formation of black stones found in the general population. In Japanese patients with brown pigment stones, there was an absence of calcium carbonate, low levels of calcium phosphate, and the presence of calcium salts of fatty acids (P less than 0.05). Thus, the accompanying stasis and/or infection in this latter group was associated with the formation of a distinctive stone type and was not involved in the formation of the black stones. The similarly small proportion of cholesterol in each of these groups suggested that it was present due to coprecipitation rather than to cholesterol supersaturation.
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PMID:Pigment gallstone composition in patients with hemolysis or infection/stasis. 369 61

A prospective study designed to establish the incidence of gallstones among 48 Nigerian homozygous sickle cell disease patients was made using oral cholecystography and grey-scale ultrasonography. The age range was 2 to 35 years with a mean of 16 years. The youngest patient with gallstones was a 51/2-year-old boy. Gallstones were demonstrated on cholecystosonography in 12 of 48 patients (25%). The incidence of cholelithiasis was 7.7% in children 10 years or younger and 31.4% in patients above this age. There was no correlation between the incidence of cholelithiasis and serum bilirubin on one hand and positive history of abdominal pain on the other. Screening of patients over 15 years for gallstones is suggested with a view to performing elective cholecystectomy in patients who subsequently become symptomatic.
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PMID:Cholelithiasis in sickle cell disease: a cholecystographic and ultrasonographic evaluation in Nigerians. 391 Apr 31

Gallstones are frequently found in patients with sickle cell anemia. The differentiation between acute calculous biliary tract disease and sickle cell crisis can be difficult and should be based on the clinical presentation, comparison with previous episodes of abdominal pain, and judicious use of hepatobiliary radionuclide scanning. Emergency cholecystectomy is associated with a high morbidity and should be avoided if possible. Elective cholecystectomy is associated with a lower but still significant risk of complications. We believe patients with sickle cell anemia and symptomatic cholelithiasis should have elective cholecystectomy. Careful management is essential to minimize the danger of postoperative complications.
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PMID:Cholelithiasis in sickle cell anemia: surgical considerations. 394 32

The prevalence of cholelithiasis was studied prospectively by abdominal ultrasound (US) examination in 305 children with sickle cell disease aged 1-18 years (mean 10.45). Gallstones were present in 60 children (19.7%); an additional 50 had biliary sludge only (16.4%). On follow-up of 35 of the 50 children with sludge, 23 (65.7%) had developed gallstones and 5 had already had a cholecystectomy. Five continued to have sludge on follow-up while 7 were reported to have no sludge. Children with US evidence of sludge should be followed up regularly by US, and those who develop gallstones should undergo elective cholecystectomy. For those with biliary sludge only, we recommend elective cholecystectomy if there are hepatobiliary symptoms.
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PMID:The significance of biliary sludge in children with sickle cell disease. 939 Nov 96

The prevalence, incidence, risk factors, clinical associations, and morbidity of gallstones were studied in 311 patients with homozygous sickle cell disease and 167 patients with sickle cell-hemoglobin C disease in a cohort study from birth. Gallstones developed in 96 patients with homozygous sickle cell disease and 18 patients with sickle cell-hemoglobin C disease; specific symptoms necessitating cholecystectomy occurred in only 7 patients with homozygous sickle cell disease.
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PMID:Gallstones in sickle cell disease: observations from The Jamaican Cohort study. 1063 79


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