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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The study of 23 cases of hemoglobinopathies in Nigeria using electrophoresis, showed that 16 cases were HB-SS, 5 cases HB-SC, and 2 cases HB-S Th. There were infarcts of long bones, which were usually multiple in the diaphyses in 17 cases, septic arthritis of big joints in 4 patients and aseptic necrosis of the hip in one, and in the hip and knee in one. Infection was proved by culture in only the 4 joints and 7 of the affected long bones, 8 of the causative organisms were Salmonella and 3 were Staphylococci. Only 2 of the infected cases needed sequestrectomy and the other 9 responded to simpler methods like aspiration and incision of abscess U.L.A. without resulting in a sinus formation. The aseptic necrosis in the hip may simulate Perthe's disease and dysbaric osteonecrosis, the massive periosteal reaction may simulate Caffey's disease and hypervitaminosis A, and give rise to difficulties in diagnosis when hemoglobinopathies are not suspected.
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PMID:Orthopedic aspects of sickle cell anemia and allied hemoglobinopathies. 63 87

The authors have followed 140 patients with sickle cell disease anemia (101 cases) or related hemoglobinopathies (39 cases). Among them hip involvement was noted in 55 (104 hips). Forty three times the hip involvement occurred in childhood and twelve times in adult life. When the necrosis appeared in childhood (84 hips) the average age was twelve. The deformity involved the femoral head (coxa plana, coxa magna) as well as the neck (short neck, coxa vara). After an average follow-up of nineteen years, clinical and radiological examinations evidenced 64 functional impairments and 25 arthrosis, 10 of which have already been operated on. The necrosis appearing during adult life (20 hips) had the same outcome as idiopathic necroses, leading rapidly to arthrosis after collapse of the sequestrum. It seems that the etiology of the necroses is linked to rheologic disorders, the deformity of the red cells causing arteriolar thromboses. In this series the hip disease was correlated with sickle cell retinopathy as defined after angiography. On the contrary there was no correlation with the severity of anemia, its treatment, the ethnical origin of the patient.
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PMID:[Natural history of hip necrosis in sickle cell disease. Apropos of 104 necroses]. 269 46