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骨骼发育不良的表型和基因型分析:中心的发展演变和印度十年的经验。

Phenotyping and genotyping of skeletal dysplasias: Evolution of a center and a decade of experience in India.

机构信息

Department of Medical Genetics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India.

Pediatric Orthopedics Services, Department of Orthopedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India.

出版信息

Bone. 2019 Mar;120:204-211. doi: 10.1016/j.bone.2018.10.026. Epub 2018 Nov 6.

Abstract

Genetic heterogeneity, high burden and the paucity of genetic testing for rare diseases challenge genomic healthcare for these disorders in India. Here we report our experience over the past decade, of establishing the genomic evaluation of skeletal dysplasia at a tertiary university hospital in India. Research or clinical genomic testing was carried out by Sanger sequencing and next-generation sequencing. Close national and international collaborations aided phenotyping and genotyping. We report 508 families (557 affected individuals) with the definitive molecular diagnosis of skeletal dysplasia. Dysostoses multiplex (n = 196), genetic inflammatory/rheumatoid-like osteoarthropathies (n = 114) and osteogenesis imperfecta and decreased bone density (n = 58) were the most common diagnoses. We enumerate the processes, clinical diagnoses and causal variants in the cohort with 48 novel variants in 21 genes. We summarize scientific contributions of the center to the description of clinical and mutation profiles and discovery of new phenotypes and genetic etiology. Our study illustrates the establishment and application of genomic testing tools for genetic disorders of skeleton in a large cohort. We believe this could be a model to emulate for other developing genetic centers.

摘要

遗传异质性、负担沉重以及罕见病基因检测的缺乏,给印度这些疾病的基因组医疗带来了挑战。在此,我们报告了过去十年在印度一所三级大学医院建立骨骼发育不良基因组评估的经验。研究或临床基因组检测采用 Sanger 测序和下一代测序进行。密切的国内外合作有助于表型和基因型分析。我们报告了 508 个家庭(557 名受影响个体)的骨骼发育不良的明确分子诊断。多发性发育障碍(n=196)、遗传炎症/类风湿性关节炎性骨关节炎(n=114)和成骨不全症及骨密度降低(n=58)是最常见的诊断。我们列举了队列中 48 个新变异在 21 个基因中的过程、临床诊断和因果变异。我们总结了该中心在描述临床和突变谱以及发现新表型和遗传病因方面的科学贡献。我们的研究说明了在一个大的队列中为骨骼遗传疾病建立和应用基因组检测工具的情况。我们相信,这可能为其他发展中的遗传中心提供一个借鉴的模式。

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