Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus

Made available in DSpace on 2018-12-11T16:59:38Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-10-01. Added 1 bitstream(s) on 2019-10-09T18:30:05Z : No. of bitstreams: 1 S1677-54492015000400346.pdf: 1096369 bytes, checksum: e7aa7d5fde0921748c91b8a930800c14 (MD5) Proteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life. Universidade Estadual Paulista – UNESP Department of Surgery and Orthopedics Universidade Estadual Paulista – UNESP Department of Pediatrics Universidade Estadual Paulista – UNESP Department of Surgery and Orthopedics Universidade Estadual Paulista – UNESP Department of Pediatrics