一例罕见的戈德堡-施普雷岑综合征病例报告

Priyanka J. Rabadia, Bhavi M. Makwana, Sonal Shah
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引用次数: 0

摘要

戈德堡-施普雷岑综合征是一种常染色体显性遗传疾病,由编码驱动蛋白家族结合蛋白的 KIFBP 基因突变引起。该蛋白与微管和肌动蛋白丝相互作用,对细胞骨架的形成和神经元的生长至关重要。起初,患者被怀疑患有伴有肠梗阻的胎粪性回肠梗阻或囊性纤维化,但进一步检查发现其患有戈德堡-史普雷岑综合征。迄今为止,医学文献中报道的病例不到 50 例。本病例综述旨在提高人们对这种罕见结缔组织疾病的认识,由于记录在案的病例很少,而且与其他综合征有一些重叠,因此这种疾病的诊断率仍然很低。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A rare case report of Goldberg-Shprintzen syndrome
Goldberg-Shprintzen syndrome is an autosomal dominant disorder caused by mutations in the KIFBP gene, which encodes the kinesin family binding protein. This protein is essential for cytoskeleton formation and neurite growth, interacting with microtubules and actin filaments. Initially, the patient was suspected to have meconium ileus with intestinal obstruction or cystic fibrosis, but further investigations revealed Goldberg-Shprintzen syndrome. To date, fewer than 50 cases have been reported in the medical literature. This case review aims to increase awareness of this rare connective tissue disorder, which remains underdiagnosed due to the scarcity of documented cases and some overlap with other syndromes.
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