Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Infection is the main factor of morbidity and mortality in children with sickle cell disease (SCD). The objective of this study is to determine it's epidemiologic outline in senegalese children and adolescents with SCD. We retrospectively studied infection data in all the charts of a cohort of 323 patients with SCD (307 SS, 13 SC and 3 s beta + thalassemia) followed at Albert Royer children hospital from january 1991 to december 1997. Serum sampling was systematically made for HIV and antigen HBs serology in all patients we received in the last 3 months (october to december 1997). Patients were aged from 5 months to 22 years (medium age = 8 years). 813 infection episodes were diagnosed, concerning 184 patients (56 per cent). SS patients were more affected (59 per cent) than the others (23 per cent, p = 0.04). ENT and broncho-pulmonary onsets were more frequent but had a generally benign course. Menigitidis, septicemia and osteomyelitis were exclusively diagnosed in SS patients. Their prevalences in this group were respectively: 1.0 per cent, 4.9 per cent and 9.8 per cent. HIV serology was determined in 155 patients, including 41 per cent with blood transfusion antecedents. All tests were negative. HBs antigen was determined in 104 patients and seroprevalence was 7.7 per cent in the whole group and 6.0 per cent in patients with transfusion antecedents and 7.7 per cent for the others. Plasmodium falciparum malaria onset was observed in 9.6 per cent of our patients and there was no case of cerebral malaria. Infection was involved in 9 of the 11 cases of death. Then infection constitute the major problem in children and adolescents with SCD in Dakar. However prevalences of severe onsets are comparable to data in Europe despite our poor follow up conditions. Senegal haplotype may lead to a good tolerance of SCD. Negative HIV serology and low HBs antigen seroprevalence in transfused patients are attributed to a relatively low level of HIV prevalence in the general population and a good transfusion security in Senegal.
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PMID:[Infections in Senegalese children and adolescents with sickle cell anemia: epidemiological aspects]. 1466 92

Splenomegaly is common in sickle cell disease (SCD) and can lead to complications. In order to evaluate its prevalence and progression in Senegalese children ad adolescents with SCD, we analyzed the records of all patients followed-up at Albert-Royer Children's Hospital in Dakar, Senegal, from January 1991 to December 2005. Age, clinical course specifying size of the spleen beyond the costal margin, and disease progression were the main data recorded. We included 698 patients (94.6% SS, 4.4% SC and 1% S-beta-thalassemia). The subjects' mean age when included in the cohort was 6 years and 11 months. Splenomegaly was observed in 122 patients (17.5 %), measuring 1-17 cm beyond the costal margin (mean, 4.7 cm). Splenomegaly was more frequent in SC patients (32.3 %) compared to SS patients (16.5 %, p=0.025). The frequency was greater in infants aged 0-12 months (25.6 %) and increased from the 1- to 5-year-old age group to the 6- to 10-year-old, before it decreased further. The course of splenomegaly was marked by subsequent reduction in 17.3 % of cases, total reversal in 43.4 %, and no size variation in 39.3 %. Acute splenic sequestration occurred in 3 SS cases aged 27, 29, and 32 months and was managed with blood transfusion. Seven patients (6 SS and 1 SC) aged 4-13 years presented hypersplenism. Splenectomy was performed in 4 of these cases, while the complication reversed after 2 to 3 blood transfusions in the 3 remaining cases. The prevalence of splenomegaly is relatively low in Senegalese children with SCD. The risk of complications requires monitoring the spleen and teaching parents palpation of the baby's abdomen for early management of splenic sequestration.
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PMID:[Epidemiology and course of splenomegaly in children and adolescents with sickle cell disease in Senegal]. 2048 83