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J Pak Med Assoc ; 61(11): 1137-9, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-22125999

RESUMO

Thalassaemia intermedia includes thalassaemias with clinical severity intermediate between asymptomatic thalassaemia minor and transfusion dependent thalassaemia major. By definition patients of thalassaemia intermedia maintain a haemoglobin level of 7-10 g/dl and do not, or only occasionally, require blood transfusion. An eight-year-old girl who was a known case of thalassaemia intermedia and had been occasionally transfused presented with fever, pain and swelling over the wrists, ankles and above the right knee joint. Radiographs showed medullary widening, cortical thinning and; multiple, recent and old, partially healed fractures of metadiaphseal regions of long bones. Her fractures have been immobilized by means of back slabs. In view of her recurrent fractures and growth retardation we advised a regular transfusion-chelation regimen to our patient to suppress her ineffective dyserythropoiesis. The treatment is expected to prevent further bone fragility and fractures, as well as improve her life quality.


Assuntos
Transfusão de Sangue , Terapia por Quelação , Fraturas Ósseas/complicações , Quelantes de Ferro/uso terapêutico , Talassemia/terapia , Antibacterianos/administração & dosagem , Antipiréticos/administração & dosagem , Criança , Feminino , Fraturas Ósseas/terapia , Humanos , Índice de Gravidade de Doença , Talassemia/sangue , Resultado do Tratamento , Vitaminas/administração & dosagem
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