RESUMO
El síndrome de Poland es una rara anomalía congénita que incluye la ausencia total o parcial de la musculatura pectoral mayor, habitualmente con otras malformaciones asociadas en la extremidad superior homolateral. Su incidencia se estima en 1/30.000 recién nacidos vivos, con una ratio varón: mujer de 3:1; es más frecuente la afectación del lado derecho (60-75%).Se presenta un caso esporádico en una recién nacida con una afectación leve del lado izquierdo y una hipoplasia del tejido mamario y la areola, en la que se detectó una dextrocardia aislada. Destacamos la necesidad de realizar estudios complementarios (radiografía torácica, serie ósea y ecografía abdominal) en todos los casos (AU)
Poland syndrome is a rare congenital anomaly consisting of unilateral partial or total absence of the greater pectoral muscle, associated usually to other malformations in the superioripsi lateral extremities. Its incidence has been estimated to be1/30,000 live births with a male: female ratio of 3:1, and is mostly right-sided (60-75%). We report the case of a female newborn with a mild defect in the left pectoral muscle, a smaller left areola and hypoplasia of subcutaneous tissue, in association with isolated dextrocardia with the apex pointing to the left. We emphasize the need for additional studies (chest x-ray, bone series, and abdominal ultrasound) in each case (AU)
Assuntos
Humanos , Feminino , Criança , Síndrome de Poland/diagnóstico , Dextrocardia/complicações , Tórax/anormalidades , Anormalidades Musculoesqueléticas/diagnósticoRESUMO
OBJECTIVE: We present 50 cases of Kawasaki's disease (KD), diagnosed and controlled in our hospital between January 1984 and December 1997. PATIENTS AND METHODS: Fifty patients were reviewed. In forty-six of these cases the onset was complete, according to the Research Committee of MLNS diagnosis criteria and four cases had incomplete onset. RESULTS: The ration male/female was 1.9/1. Both fever and oral cavity lesions were present in all cases. In 93.4% desquamation of fingers was observed. The most frequent skin lesion was maculopapular rash. Fourteen patients had atypical onset: adenophlegmon, aseptic meningitis, symptomatic hepatitis, parotiditis, queratopathia punctata and arthritis. Blood analysis showed alterations of high ESR (88% of cases), C-reactive protein (62%), leukocytosis (82%) and thrombocytosis (96%). Fifteen patients were treated with gamma globulin (IGIV), five received a single 2 g/Kg dose and ten received 400 mg/Kg per day during four days. Eight percent of our cases produced cardiovascular complications, none that included coronary aneurysms. CONCLUSIONS: After IGIV treatment we observed a shortening of the febrile period and amelioration of clinical symptoms. No deaths were reported.
