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CTNNB1 基因突变与神经发育障碍、小头畸形和双侧永存原始玻璃体增生相关:病例报告及文献复习。

CTNNB1 gene mutation associated with neurodevelopmental disorder, microcephaly, and persistence of bilateral hyperplastic primary vitreous: A case report and literature review.

机构信息

Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.

Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.

出版信息

Arch Soc Esp Oftalmol (Engl Ed). 2022 Jan;97(1):44-47. doi: 10.1016/j.oftale.2020.11.018. Epub 2021 Nov 14.

Abstract

The most cases of persistence hyperplastic primary vitreous (PHPV) are unilateral and sporadic, however, bilateral presentation could be present in a small number of patients, in whom other genetic diseases must be ruled out. We describe a case of a 2 months child with bilateral persistence hyperplastic primary vitreous confirmed by ultrasound. In addition, with neurodevelopmental defects, microcephaly, facial dimorphism, axial hypotonia, and without brain abnormalities on MRI, in whom a de novo mutation of the CTNNB1 gene was found during the genetic study, which explains the findings.

摘要

大多数永存原始玻璃体增生症 (PHPV) 为单侧和散发性,但少数患者也可能为双侧,在此情况下必须排除其他遗传疾病。我们描述了一例 2 个月大的双侧永存原始玻璃体增生症患儿,该病例经超声检查确诊。此外,患儿还存在神经发育缺陷、小头畸形、面部畸形、轴向张力减退,且 MRI 未见脑部异常,遗传学研究发现 CTNNB1 基因存在新生突变,这可解释患儿的所有临床表现。

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