Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The DF given weekly by the RBIT in 14 thalassemia/hemoglobinopathy patients decreased the frequency of URIs and febrile illnesses in 5/10 patients. Five of the six patients had absence of frequent enteritis. There were no instances of pneumonia and otitis media in two patients with frequent infections before DF. Skin color was improved within 3 months. Most of the patients became more active at the same hematocrit level. The height and weight in eight patients was normal. The total number of blood transfusions in all patients was significantly decreased. It is advisable to use weekly DF infusion by RBIT in the patient who cannot afford to use the infusion pump and the full program of iron chelation.
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PMID:Rubber band technique for slow subcutaneous infusion of desferrioxamine (Desferal). 339 May 34

Extramedullary hematopoiesis occurs in children with hemoglobinopathy and chronic anemia. The liver and spleen are often affected first, but other foci can develop to support erythrocyte demand. We report a case of a nine-year-old with beta thalassemia and temporal bone extramedullary hematopoiesis causing ossicular fixation and bilateral conductive hearing loss. There is only one case in the literature describing this phenomenon in pediatric patients, and this is the first case report of bilateral hearing loss from this physiologic phenomenon. Otolaryngologists should consider this etiology in patients with chronic anemia and conductive hearing loss in the absence of otitis media.
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PMID:Temporal bone extramedullary hematopoiesis as a causeof pediatric bilateral conductive hearing loss:Case report and review of the literature. 2848 23