Expanding the spectrum of the Perlman syndrome

1988; Wiley; Volume: 29; Issue: 4 Linguagem: Inglês

10.1002/ajmg.1320290406

ISSN

1096-8628

Autores

Frank Greenberg, Karen Copeland, Mary V. Gresik, John M. Optiz, James F. Reynolds,

Tópico(s)

Genetic Syndromes and Imprinting

Resumo

Abstract We report on an infant with manifestations of Perlman syndrome including polyhydramnios, macrosomia, bilateral nephromegaly with nephroblastomatosis, visceromegaly and cryptorchidism. Other findings in this infant not seen in previous patients were diaphragmatic hernia, interrupted aortic arch, hypospadias and polysplenia. This infant meets the diagnostic criteria for Perlman syndrome, suggesting that diaphragmatic hernia and cardiac defects may be additional findings in this disorder.

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