Resumo

GARCIA RODRIGUEZ, George N.; LEON JORGE, Maricel; PALACIOS CASTILLO, Tania  e  LOPEZ CABELLO, Jorge Luis. Ehlers Danlos syndrome: a clinical subregister in pediatric orthopedics?. Rev Cubana Pediatr [online]. 2008, vol.80, n.2, pp. 0-0. ISSN 1561-3119.

INTRODUCTION. The behavior of Ehlers Danlos syndrome caused it to be interpreted as a clinical subregister, little known, and with a few references. This genodermatosis is generally condemned not to have a specific treatment. The objective of this study was mainly to describe the morbidity of this syndrome from the orthopedic point of view. METHODS. A 5-year prospective and descriptive study was conducted among all the patients with Ehlers Danlos syndrome that were seen at the Orthopedics and Traumatology department of "José Martí" Teaching Pediatric Hospital (Sancti Spiritus, Cuba) from July 2001 to July 2006. A minimum follow-up time of 6 months was considered for the validation of the results. RESULTS. 41 patients affected with 72 diseases of orthopedic origin were studied. The frequency was approximately 1.7 diseases per patient, with a non significant predominance of females (n = 24). One of the most important perinatal antecedents was the presence of diverse hip dysplasia degrees. The presence of other non-orthopedic affections was not remarkable. The main orthopedic findings were the flexible flat foot (37), the genus recurvatum (11) and kyphoscoliosis (9). Aesthetic surgery and the corrective orthopedic surgery were the most used. CONCLUSIONS. The categorization of this disease and its timely treatment is an efficient method of control that would help to prevent the articular degeneration that generally precedes osteoarthritis.

Palavras-chave : Ehlers Danlos syndrome; articular hyperlaxity; vitamin C; Beighton's scale; Brighton's criteria; Human Genoma Project; genodermatosis.

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