ABSTRACT
This study was based on a prospective and a retrospective analysis of 35 patients who met Bohan and Peter criteria for juvenile dermatomyositis diagnosis.The mean follow-up time was three years ten months. Calcinosis was present in five (14.28 percent) patients, cutaneous ulcers in four (11.42 percent), and systemic involvement in nine (27.71 percent) patients. All patients presented alterations in the serum levels of muscle enzymes, and all of them were submitted to muscle biopsy as a diagnostic procedure. Nine (25.71 percent) patients received corticotherapy prior to and 26 (74.28 percent) after the muscle biopsy. Chloroquine, methotrexate, cyclosporine, cyclophosphamide and intravenous immunoglobulin were used in patients with poor response to corticotherapy. Continuation of cutaneous manifestations was observed in 4 (11.43 percent) patients, laboratorial activity in 1 (2.85 percent), cutaneous and laboratorial activities in 3 (8.57 percent). Ten (28.57 percent) patients were out of activity, and 17 (48.57 percent) in remission at study end-point, on March 2002. Two (5.71 percent) patients died
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Dermatomyositis , Adrenal Cortex Hormones , Dermatomyositis , Follow-Up Studies , Immunosuppressive Agents , Muscle, Skeletal , Prospective Studies , Retrospective Studies , Treatment OutcomeABSTRACT
Os autores apresentam 13 casos de síndrome oculofaríngea entre os quais encontraram casos de distrofia muscular progressiva, casos de lesäo periférica e um caso de miopatia com depósito de gorduras. É discutida a literatura a respeito dessa síndrome relacionando os achados de outros autores com os observados neste trabalho