[Ectodermal dysplasias--a general view].
Ectodermal dysplasias comprise a group of about 150 diseases, in general of a genetic nature. The most common form--Christ, Siemens, Touraine's Syndrome--is characterized by high intermittent fever in infancy and, when the patient is not properly cared for, the hyperthermia may cause death. In Brazil, there are at least 400 men severely affected by this syndrome (such a number is not higher because approximately 50% of the patients die early) and 1,000 women. In women, however, the syndrome occurs in a mild form, and many go unnoticed. Disinformation renders difficult the diagnosis and treatment of patients. This was the reason that led us to create, in 1982, the Center for the Study of Ectodermal Dysplasias, the only specialized institution of such diseases in the world.
Pinheiro
M
M
Departamento de Genética, Universidade Federal do Paraná, Curitiba.
Freire-Maia
N
N
D002648:Child; D004476:Ectodermal Dysplasia / Q000235:genetics* / Q000473:pathology; D005190:Family; D005260:Female; D006801:Humans; D008297:Male; D010641:Phenotype
Displasias ectodérmicas--uma visão geral.