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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten patients with sickle cell anemia over the age of 10 who had cholecystectomy are reported. All had symptomatic cholecystitis; three had biliary tract obstruction. Transfusions were given preoperatively and with attention to oxygenation and fluid and electrolyte balance. There were few complications, none serious. Based upon the frequency of gallstones over the age of 10, the likelihood of symptoms or serious complications and the apparent ease with which the properly prepared sickle cell patient comes through surgery, we recommend routine cholecystograms in all adolescent and adult sickle cell anemia patients with elective cholecystectomy for those who have stones.
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PMID:Cholelithiasis in sickle cell anemia: a case for elective cholecystectomy. 45 36

The gallbladder is frequently diseased in sickle cell anemia. The distinction between cholecystitis and the abdominal crisis of sickle cell disease is difficult to make. Ultrasonography permits a rapid evaluation of the gallbladder in sickle cell disease.
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PMID:Ultrasonic evaluation of the gallbladder in sickle cell disease. 53 14

The most important side effects of oral contraceptives (OCs) and their incidence, together with advice and monitoring of the patient at risk, are pointed out. There is a mild increase in blood pressure in longterm contraceptive use caused by increased angiotensinogen production by the liver. It is significant only for women with a history of familial hypertension, diabetes mellitus, or pre-eclampsia. Smoking increases this risk. Urinary tract infections are 25-50% more frequent in pill users. Glucose tolerance is slightly decreased. Contraceptives' diabetogenic effect is higher in women with hereditary tendency for diabetes, latent diabetes, and/or obesity. They are contraindicated in latent diabetes. Findings are contradictory in their effects on cholesterol and triglyceride serum level, but the pill is contraindicated in lipid metabolism disorders. There is an increased incidence in cholecystitis and cholelithiasis in pill-users (70-80 additional cases/100,000 user years). Liver diseases, intrahepatic cholestasis, occur rarely and benign liver tumors have not conclusively been proved to be caused by the pill. A variety of laboratory findings have been related to contraceptive use and drug interactions occur with barbiturates, rifampicin, hydantoin, and phenylbutazone. Blood coagulation is increased, partially by increased production of various blood coagulation factors; but more importantly, by a decreased synthesis of antithrombin III, a natural protective mechanism against intravascular coagulation. This increases thrombosis risk. Risk doubles with simultaneous cigarette smoking. Various epidemiological studies indicate a 5-10 fold increase in thromboembolism and thrombophlebitis, deep vein thrombosis, and pulmonary embolism. There is a correlation between contraceptive use and cerebrovascular disorders and myocardial infarction. This risk increases with age and years of pill use. The pill is contraindicated with symptoms of thrombophlebitis and thromboembolism, sickle cell anemia, proposed surgery, and longterm immobilization. Overall risk factors are not too high. Recommendations for rational pill use related to age are given and further contraindications are mentioned.
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PMID:[Adverse effects of oral contraceptives]. 55 52

Hepatic dysfunction is a common finding in patients with sickle cell disease but viral hepatitis appears to be an unusual complication in the adult SS patient. Only five cases of viral hepatitis were recorded in 378 admissions for SS crisis. In contrast, hepatic crisis occurred as a distinct event in 9% of 88 patients with sickle cell anemia. This entity must be differentiated from acute cholecystitis or viral hepatitis. Transiently abnormal results of hepatitic function tests were observed in another 26 patients with extrahepatic crisis. Cirrhosis is relatively common and often the terminal event in SS disease. Choledocholithiasis and cholecystitis are infrequent complications despite the prevalence of gallstones in SS anemia.
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PMID:Sickle cell hepatopathy. 87 Sep 77

A case of sickle cell (HbSS) disease is presented in a patient with a history of recurrent admissions for abdominal pain, jaundice, and abnormal liver function tests. Although he was believed to have a sickle cell abdominal crisis, his abdominal X-ray films revealed three calcified stones. Each of these stones progressively passed through the common duct and into the duodenum while awaiting surgery. He has been followed for two years since his cholecystectomy without further hospitalizations. This case led to the investigation of cholelithiasis in sickle cell disease to dispel the following misconceptions. Some physicians and pediatricians believe that (1) cholelithiasis and cholecystitis are uncommon in sickle cell disease; (2) the complications of gallstones are not significant; (3) the operative risk in patients with sickle cell disease is high; (4) these patients with HbSS disease do not live long enough to get into trouble with gallstones. A review of the literature on cholelithiasis and HbSS disease presents adequate evidence to cause us to urge investigation of the gallbladder in all patients with HbSS disease and abdominal crises, and cholecystectomy as an elective procedure should stones be present.
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PMID:Cholecystitis and cholelithiasis masking as abdominal crises in sickle cell disease. 95 Nov 41

Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. Patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.
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PMID:Surgical management of children with hemoglobinopathies. 144 Jan 54

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

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

Five cases of biliary complications in childhood sickle cell disease are reported. In four cases, the pathology was gall stones causing recurrent abdominal pain in a 10 year old boy, a 13 year old girl and a 2 year old infant, and responsible for a "Salmonella septicaemia" in a 17 year old adolescent. In one case, a biliary cyst was diagnosed at 3 years of age. Four children underwent successful surgery. The complications of gall stones are difficult to distinguish from episodes of vasoocclusive abdominal pain. Ultrasonography is an easy method of detecting gall stones and may be repeated regularly in children over 10 years of age. All the children operated in this series were improved by surgery. Patients with sickle cell disease must be carefully prepared for general anaesthesia with a strict protocol of blood transfusion which is only possible in well equipped centers. Elective surgery is by far the best management as postoperative complications are much less common than after emergency surgery. A review of the literature shows that the general tendency is for surgical intervention as gall stones are a cause of recurrent abdominal pain, cholecystitis and dangerous infective complications in those patients.
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PMID:[Pathology of the bile ducts in children with homozygous sickle-cell anemia. Apropos of 5 recent cases]. 371 16

The authors analyze the experience with the association of the gall-bladder disease and sickle cell anemia in ten cases. The incidence of this association in these cases was higher, about 80% (eight patients). Because of the increased frequency of cholecystitis and cholelithiasis in this disease, cholecystography (oral or endovenous) is indicated in every patient with sickle cell anemia or another hereditary (Hemolytic jaundice).
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PMID:[Cholecystopathy in sickle cell anemia]. 677 66


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