A new Seckel-like syndrome of primordial dwarfism

1996; Wiley; Volume: 64; Issue: 3 Linguagem: Inglês

10.1002/(sici)1096-8628(19960823)64

ISSN

1096-8628

Autores

Michael S. Buebel, Carlos F. Salinas, G. Shashidhar Pai, R I Macpherson, Margaret K. Greer, Adolfo Perez‐Comas,

Tópico(s)

Otitis Media and Relapsing Polychondritis

Resumo

Seckel syndrome is a rare, recessively inherited disorder of severe growth retardation and distinct craniofacial, orodental, and skeletal anomalies. Even though there are well-established minimum diagnostic criteria for this syndrome, controversy exists about its boundaries and criteria for exclusion. We studied 2 remarkably similar, unrelated children with most of the clinical and radiographic manifestations of Seckel's original patient. Although their craniofacial and orodental anomalies are typical of Seckel syndrome, 1 child has unusual appearance of the hands and feet that have not been previously associated with it. This patient appears to define a new Seckel-like syndrome and suggests heterogeneity in this type of primordial dwarfism. © 1996 Wiley-Liss, Inc.

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