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2号染色体2q24-q31缺失导致特征性手指异常:病例报告及文献复习

Deletion of chromosome 2q24-q31 causes characteristic digital anomalies: case report and review.

作者信息

Boles R G, Pober B R, Gibson L H, Willis C R, McGrath J, Roberts D J, Yang-Feng T L

机构信息

Childrens Hospital Los Angeles, CA 90027, USA.

出版信息

Am J Med Genet. 1995 Jan 16;55(2):155-60. doi: 10.1002/ajmg.1320550204.

Abstract

We describe a newborn boy with multiple anomalies, including bilateral split foot and an interstitial deletion of chromosome 2 (q24.2-q31.1). Four additional cases in 2 families involving similar deletions have been reported. Bilateral digital anomalies of hands and feet were seen in all 5 cases, including a wide cleft between the first and second toes, wide halluces, brachysyndactyly of the toes, and camptodactyly of the fingers. Other common manifestations have included postnatal growth and mental retardation, microcephaly, down-slanting palpebral fissures, micrognathia, and apparently low-set ears. Bilateral digital anomalies were reported in 22 of 24 cases with deletions including at least part of region 2q24-q31. Digital anomalies were not prevalent in 18 patients with deletions of chromosome 2q not overlapping 2q24-q31. 2q31.1 appears to be the common deleted segment in all cases with significant digital anomalies, which implies the existence of one or more genes involved in distal limb morphogenesis in this region. HOXD13 and EVX2, located in the proximity of 2q31, were not deleted in our patient by Southern analysis. Bilateral digital malformations of the hands and feet associated with other anomalies should be evaluated by chromosome analysis focused at the 2q24-q31 region.

摘要

我们描述了一名患有多种异常的男婴,包括双侧裂足和2号染色体(q24.2-q31.1)的间质性缺失。已有报道称,另外两个家族中有4例涉及类似缺失的病例。所有5例均出现手足双侧指(趾)异常,包括第一和第二趾之间的宽裂、宽拇趾、趾短指畸形以及手指屈曲畸形。其他常见表现还包括出生后生长发育和智力迟缓、小头畸形、睑裂向下倾斜、小颌畸形以及耳部明显低位。在24例至少包含2q24-q31部分区域缺失的病例中,有22例报告了双侧指(趾)异常。在18例2号染色体q区缺失但不与2q24-q31重叠的患者中,指(趾)异常并不常见。2q31.1似乎是所有有明显指(趾)异常病例中的共同缺失片段,这意味着该区域存在一个或多个参与远端肢体形态发生的基因。通过Southern分析,位于2q31附近的HOXD13和EVX2在我们的患者中未被缺失。对于伴有其他异常的手足双侧指(趾)畸形,应通过聚焦于2q24-q31区域的染色体分析进行评估。

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