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无关墨西哥患者考斯特洛综合征的临床和分子特征。

Clinical and molecular characterization of Costello syndrome in unrelated Mexican patients.

机构信息

Familial Medicine Unit 92, IMSS.

Western Biomedical Research Center, IMSS.

出版信息

Clin Dysmorphol. 2022 Apr 1;31(2):55-58. doi: 10.1097/MCD.0000000000000405.

Abstract

This study intends to describe for the first time a cohort of Mexican patients with Costello syndrome. The five exons of the HRAS gene were amplified in DNA samples from 13 patients with a clinical suspicion of Costello syndrome. PCR products were sequenced using the Ready Reaction Big Dye Terminator v.3.0 Kit and an ABI PRISM 310 sequencer. Only five patients (38%) showed causal variant in codon 12 of the HRAS gene (four with the p.Gly12Ser and one with the p.Gly12Ala variant). Three patients showed silent polymorphic variants (p.His27His and p.Leu159Leu). Clinical features in patients carrying the causal variant were variable. The alternative diagnosis of cardio-facio-cutaneous syndrome was considered in patients who did not have a causative variant in HRAS.

摘要

本研究旨在首次描述一组墨西哥 Costello 综合征患者。对 13 名临床疑似 Costello 综合征患者的 DNA 样本进行 HRAS 基因的五个外显子扩增。使用 Ready Reaction Big Dye Terminator v.3.0 试剂盒和 ABI PRISM 310 测序仪对 PCR 产物进行测序。只有五名患者(38%)在 HRAS 基因的密码子 12 显示出致病变异(四个 p.Gly12Ser,一个 p.Gly12Ala 变异)。三名患者显示出沉默多态性变异(p.His27His 和 p.Leu159Leu)。携带致病变异的患者的临床特征各不相同。在没有 HRAS 致病变异的患者中,考虑了心面皮肤综合征的替代诊断。

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