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[Clemence, age 8 months, presents with hand swelling]
1Clinique Adjoint, Département Universitaire de Pédiatrie Ulg, CHR Citadelle, Liège.
Insights
Sickle cell disease, a genetic blood disorder, can manifest as hand-foot syndrome in infants. Early recognition of this painful swelling is crucial for timely treatment and improved outcomes.
Area of Science:
- Genetics and Hematology
- Pediatric Medicine
Background:
- Sickle cell disease (SCD) is an inherited blood disorder common in individuals of African descent.
- It is characterized by the presence of hemoglobin S and follows an autosomal recessive inheritance pattern.
Observation:
- A case report details an 8-month-old girl with significant hand swelling (69.3% Hb S).
- The swelling, without fever or trauma, was identified as hand-foot syndrome.
Findings:
- Hand-foot syndrome, a common early manifestation of SCD, results from bone infarction in the hands and feet.
- Symptoms include swelling, pain, erythema, and potential osteonecrosis, typically appearing between 3-6 months of age.
Implications:
- Prompt diagnosis of hand-foot syndrome in infants is vital for initiating preventive and therapeutic interventions.
- Early management can significantly reduce the morbidity and mortality associated with sickle cell disease complications.
Abstract:
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.