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October 3, 1977

The Hand-Foot Syndrome in Sickle Cell β-Thalassemia Disease

Author Affiliations

From the 1st Department of Pediatrics, Athens University at Aghia Sophia Children's Hospital, Athens 608, Greece.

JAMA. 1977;238(14):1540-1541. doi:10.1001/jama.1977.03280150110043

THE HAND-FOOT syndrome is a well-documented manifestation of sickle cell anemia; whether it is equally common in sickle cell β-thalassemia (S/thal) disease seems unlikely, since published reports are scarce.1,2

We report an infant with S/thal whose first clinical manifestations were pallor, fever, and painful, red swelling of both hands.

Procedures  An 11-month-old boy was admitted to the First Department of Pediatrics of Athens University. He had a high fever and edema of both hands of five days' duration, which did not regress during oral antibiotic therapy. A sister aged 6 years has S/thal disease. The family comes from Trikala of Thessaly, a region of Greece with a high frequency of sickle cell and β-thalassemia traits. Physical examination showed a pale, sick-looking infant. Temperature was 39 °C. Weight was 7,800 g, ie, below the third percentile, and height 77 cm, which corresponds to the 50th percentile. There was a spectacular