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从皮特 - 罗杰斯 - 丹克斯综合征到沃尔夫 - 赫希霍恩综合征,再回归?

From Pitt-Rogers-Danks syndrome to Wolf-Hirschhorn syndrome and back?

作者信息

Zollino M, Bova R, Neri G

机构信息

Istituto di Genetica Medica, Facoltà di Medicina A. Gemelli, Università Cattolica, Rome, Italy.

出版信息

Am J Med Genet. 1996 Dec 2;66(1):113-5. doi: 10.1002/(SICI)1096-8628(19961202)66:1<113::AID-AJMG29>3.0.CO;2-U.

Abstract

Apparently normal chromosomes without a molecular 4p16.3 deletion were found in a patient with a Wolf-Hirschhorn syndrome (WHS) phenotype. During a 10-year-period of observation he consistently presented with typical facial appearance, moderate to severe mental retardation, normal physical development with normal head circumference. Genetic results and the relatively mild clinical manifestations suggest that a diagnosis of Pitt-Rogers-Danks syndrome (PRDS) may be more likely in this patient. If WHS and PRDS will ultimately prove to be caused by haploinsufficiency of the same gene in 4p16, non-deleted patients such as the present one will be good candidates for the search of point mutations in such putative gene.

摘要

在一名具有Wolf-Hirschhorn综合征(WHS)表型的患者中发现了无分子4p16.3缺失的看似正常的染色体。在10年的观察期内,他始终表现出典型的面容、中度至重度智力发育迟缓、身体发育正常且头围正常。基因检测结果和相对较轻的临床表现表明,该患者更可能被诊断为皮特-罗杰斯-丹克斯综合征(PRDS)。如果最终证明WHS和PRDS是由4p16中同一基因的单倍剂量不足引起的,那么像本例这样的非缺失患者将是寻找该假定基因点突变的良好候选对象。

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