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)

A study of the effect of tonsillectomy on the frequency of sickle cell pain crises was carried out on 15 patients with sickle cell disease (Hb SS) who presented with complaints of frequent pain crises and were found to have chronic tonsillitis. They comprised 9 females and 6 males and ranged in age from 6 to 35 years, with a mean of 15 years. Tonsillectomy was performed under general anaesthesia. The mean number of pain crises in the one-year period after tonsillectomy was 1.5 and was significantly less than the mean number 4.7 in the one year preceding the operation (p less than 0.001). Four patients failed to show a reduced number of crises and these were those whose tonsils at operation showed no pus in their tonsillar crypts. It is thus observed that chronic tonsillitis, symptoms of which may not be volunteered at examination, is a potent inductor of sickle cell pain crisis and that tonsillectomy is an effective mode of treatment, especially when the tonsillar crypts contain pus.
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PMID:Chronic tonsillitis, tonsillectomy and sickle cell crises. 358 61

Adenotonsillar hypertrophy and chronic tonsillitis are common findings in patients with sickle cell disease (SCD). Various preoperative transfusion regimens have been suggested to reduce the population of sickle erythrocytes and correct the anemia, ranging from conservative (correcting the anemia) to aggressive (lowering the level of HbS to less than 30%). A total of 39 patients with SCD were included in the study. They were divided into 2 groups. Fourteen patients in group 1 were assigned aggressive exchange transfusion and 25 patients in group 2 were assigned a conservative (simple) transfusion. The 2 groups were compared for possible operative and postoperative complications. Thirty percent of patients in both groups had postoperative complications. They ranged from mild local infection to acute chest syndrome. Simple transfusion was not associated with higher incidence of complications and resulted in only one-third as many transfusion requirements.
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PMID:A comparison of two transfusion regimens in the perioperative management of children with sickle cell disease undergoing adenotonsillectomy. 1268 48