[Clemence, age 8 months, presents with hand swelling].
Sickle cell disease is a congenital haemolytic anaemia which is frequent in people of African origin. It is characterised by the production of hemoglobin S and has a recessive autosomal transmission. We present the case of a 8-month old girl presenting a swelling of the hands without fever or history of traumatism. She had 69.3% Hb S The swelling of the hands was a manifestation of the hand-foot syndrome, caused by infarction in the small long bones of hands and feet. It is associated with osteonecrosis, induration, swelling and erythema and generally occurs at the age of three to six months. It is one of the numerous complications of sickle cell disease which must be recognised without delay in order to implement preventive and curative treatment and reduce its morbidity and lethality.
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Clinique Adjoint, Département Universitaire de Pédiatrie Ulg, CHR Citadelle, Liège.
D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004487:Edema / Q000209:etiology*; D005260:Female; D006225:Hand / Q000473:pathology*; D006801:Humans; D007223:Infant; D013577:Syndrome