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)

The course and management of avascular necrosis of the femoral head (AVNFH) in six hips of 5 sickle cell syndrome patients (3 with Hb SS, 1 with Hb SC and 1 with Hb S/beta+-thalassaemia) are described. Two patients (aged 13 and 17 years) presented with Perthes- and osteochondritis dessicans-type lesions. These hips progressed to roller-bearing-type joints with good function and no pain following conservative management of weight restriction and rest. Three patients (aged 14, 22 and 30 years at original presentation) suffered whole-head necrosis. Initially, these 3 patients had four hip joints replaced, two cemented-stemmed types, one cemented double-cup and one uncemented hemi-arthroplasty. All four joints failed and were revised 21-61 months after the original operation. One of the revision hips has now failed and is awaiting further surgery. These results demonstrate that it is very difficult to achieve a successful hip arthroplasty in the sickle cell syndrome patient.
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PMID:Avascular necrosis of the femoral head in sickle cell syndrome: a report of 5 cases. 312 Apr 70