Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024530 (malaria)
 44,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

About 120,000 infants are born each year with sickle cell disease (SCD) in Africa. The majority have Hb SS, but Hb SC and Hb S/beta+ thalassaemia are common in west Africa. The development of Plasmodium falciparum and P. malariae is partially inhibited in the Hb SS red cells, but malaria precipitates both haemolytic and infarctive crises, and is the commonest and most important cause of morbidity and mortality. The pneumococcus is likely to be the second major infectious cause of sickness and death. In one rural community, there were less than 2% of the expected number of subjects with SCD surviving beyond 5 years of age. Genetic factors improving prognosis include (1) the Senegal beta chain haplotype, which is linked to a high level of Hb F, and (2) alpha+ thalassaemia. Of environmental factors improving prognosis, the family is of first importance. The commonest age of presentation is 1-3 years. Children present with anaemic crises (malaria, splenic sequestration, folate deficiency, and possibly aplastic), infarctive crises (hand-foot syndrome, bone-pain, pulmonary and abdominal) or acute infections (malaria, pneumonia, septicaemia, meningitis, osteomyelitis). Tragically, many patients in central Africa have been infected by the human immunodeficiency virus (HIV) through blood transfusions; they present with generalised lymphadenopathy and other features of the acquired immunodeficiency syndrome (AIDS). The principles of management are (1) to ensure freedom from malaria, (2) to continue folic acid supplements, (3) to give blood transfusions only when anaemia endangers life, (4) to control pain, (5) to restore hydration, and (6) to prescribe broad spectrum antibiotics in large dosage and without delay, but only when there are definite indications, such as fever (greater than 39 degrees C), acute pulmonary disease, meningitis, and acute osteomyelitis. The advent of HIV and AIDS makes the control of SCD of even greater importance. Principles of control are (1) early diagnosis through appropriate laboratory techniques and selective screening, (2) education of parents, patients, health professionals and public, and (3) the maintenance of health at sickle cell clinics; measures must include antimalarial prophylaxis. SCD programmes should be integrated with primary health care and AIDS control programmes.
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PMID:The presentation, management and prevention of crisis in sickle cell disease in Africa. 265 Jul 73

Using files related to children admitted at the Brazzaville teaching hospital (Congo) between May 1995 and May 2002, the authors have studied the part of sickle cell crisis (SCC) in the sickle cell disease, have assessed the epidemiological particularities, the relation between some clinical, biological factors as well as the severity of the disease. On the whole, 587 SCC have been observed in homozygous SS children aged 6 to 17. The distribution was as follows: painful osteo-articular crisis (58.6%), abdominal crisis (23.5%), acute chest syndrome (14%), neurological strokes (3.2%) and priapism (0.7%). The neurological strokes (75%) and the hand-foot syndrome (77.8%) were predominant in male. As regards the interaction age-localization of the crisis, the hand-foot syndrome mainly concerned children under five, long bones and rachis impairment those aged 11 to 15; abdominal and neurological crisis were observed especially before the age of 10, the acute chest syndrome after 10 (68.3%). Malaria (48.9%) and bacterial or viral infections (24.5%) proved to be the triggering factors when these are identified (188 cases). SCC occurred in 67.5% of the cases during the hot and rainy seasons. Moreover the haemoglobin F rate above 10% was correlated with a low prevalence of SCC, in particular the potentially severe crisis, revealing at the same time its protective value. These results show that SCC, by their frequency and/or their severity constitute a major handicap when the vital prognosis is not involved. Rigorous health habits, appropriate vaccination programme, adequate malarial prophylaxis, optimal transfusional strategy and especially the use of hydroxyurea, prove to be urgent preventive measures to put into practice. Their efficient implementation will provide a better quality of life to the sickle cell patient and will decrease the risks of severe crisis.
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PMID:[Sickle-cell crisis in the child and teenager in Brazzaville, Congo. A retrospective study of 587 cases]. 1642 15