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PMID: 41095672 已发表 · epublish 英语

Intersecting Pathologies: COL1A1-Related Syndrome in the Setting of Childhood-Onset Hypopituitarism: Case Report and Literature Review.

Diagnostics (Basel, Switzerland) ·第 15 卷 ·第 19 期 ·2025-09-25

Pelineagră OE, Golu I, Chiriţă-Emandi A, Balaş M, Andreescu NI, Munteanu CV, Amzăr DG, Plotuna I, Aruncutean D, Vlad M

摘要

Background: Type I collagen is the most abundant protein of the extracellular matrix. Pathogenic variants in COL1A1 or COL1A2 are classically associated with osteogenesis imperfecta (OI) and Ehlers-Danlos syndrome (EDS). An emerging clinical entity-COL1-related overlap disorder-encompasses individuals exhibiting phenotypic features of both conditions. Methods: We report a 55-year-old male presenting with disproportionate short stature, grayish-blue sclerae, multiple fractures, long bone deformities, joint hypermobility, and atrophic surgical scarring. The patient also had long-standing, untreated childhood-onset hypopituitarism. Imaging studies revealed numerous prior fractures, bowing of forearm bones, and multiple Wormian bones. Results: Genetic testing confirmed a novel heterozygous COL1A1 exon 14 variant (c.940G > A, p.Gly314Arg), presenting with a phenotype consistent with a COL1-related overlap syndrome. Conclusions: This case expands the phenotypic spectrum of COL1A1 mutations and supports the concept of COL1-related phenotypic overlap.

关键词
C1ROD COL1A1 Ehlers–Danlos syndrome connective tissue disorder fractures hypopituitarism
文献信息
期刊
Diagnostics (Basel, Switzerland)
期刊简称
Diagnostics (Basel)
ISSN
2075-4418
发表日期
2025-09-25
语言
英语
国家/地区
Switzerland
NLM ID
101658402
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