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短指畸形伴特殊言语障碍:两例新病例报告

SED-brachydactyly and distinctive speech: report of two new cases.

作者信息

García-Cruz D, Zafra de la Rosa G F, Sánchez-Corona J, Nazará Z, López-Cardona M G, García-Ortiz J E, Corona-Rivera J R, Cantú J M

机构信息

Instituto de Genética Humana "Dr. Enrique Corona Rivera", Departamento de Biología Molecular y Genómica, CUCS, Universidad de Guadalajara, Guadalajara, Jalisco, México.

出版信息

Genet Couns. 2007;18(1):85-97.

Abstract

We describe two unrelated patients and the mother of one of them showing clinical and radiological features as those previously described in the spondyloepiphyseal dysplasia-brachydactyly and distinctive speech (SED-BDS, also named Fantasy Island syndrome or Tattoo dysplasia) clinically characterized by short stature with acral shortness, distinctive face, mild blepharophimosis, upslanted palpebral fissures, abundant eyebrows and eyelashes, thick and abundant hair and coarse voice; and radiologically by brachymetacarpalia, brachymetatarsalia and brachyphalangia of all fingers and toes, short and broad long bones with normal morphology and small pelvis. The clinical and radiological features present in mother and son suggest a probable autosomal dominant mode of inheritance and variable expressivity.

摘要

我们描述了两名无血缘关系的患者以及其中一名患者的母亲,他们表现出的临床和放射学特征与先前在脊椎骨骺发育不良-短指畸形及独特语音(SED-BDS,也称为梦幻岛综合征或纹身发育不良)中所描述的一致。该病症临床特征为身材矮小伴肢端短小、面容独特、轻度睑裂狭小、睑裂向上倾斜、眉毛和睫毛浓密、头发浓密且粗糙以及声音粗哑;放射学特征为所有手指和脚趾均有掌骨短缩、跖骨短缩和指(趾)骨短缩,长骨短而宽但形态正常,骨盆较小。母亲和儿子所呈现的临床和放射学特征提示可能为常染色体显性遗传模式且存在可变表达。

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