Sobaihi Mrouge, Habiballah Abdullah K, Habib Abdulrahman M
Department of Pediatric, King Faisal Specialist Hospital and Research Centre, Jeddah, SAU.
Cureus. 2024 Sep 9;16(9):e69021. doi: 10.7759/cureus.69021. eCollection 2024 Sep.
Osteogenesis imperfecta is a genetic disorder characterized by decreased bone density, bone deformities, and fractures. It results from mutations in different genes, including all steps of collagen 1 synthesis and modifications. In addition, the gene is involved in the homeostasis of intracellular calcium. TMEM38B is a gene involved in the formation of a cation channel responsible for calcium entry intracellularly. Mutations in this gene are associated with osteogenesis imperfecta. However, this mutation has not been frequently discussed in the literature. In our study, we report a case of TMEM38B-associated autosomal recessive osteogenesis imperfecta in a child of a consanguineous family presented with a history of multiple prenatal and postnatal fractures. No other associated complications are present in our case.
成骨不全症是一种遗传性疾病,其特征为骨密度降低、骨骼畸形和骨折。它由不同基因的突变引起,包括I型胶原蛋白合成和修饰的所有步骤。此外,该基因还参与细胞内钙的稳态。跨膜蛋白38B(TMEM38B)基因参与形成负责钙进入细胞内的阳离子通道。该基因的突变与成骨不全症相关。然而,这种突变在文献中并未得到频繁讨论。在我们的研究中,我们报告了一例与TMEM38B相关的常染色体隐性遗传成骨不全症病例,该病例来自一个近亲家庭的儿童,有多次产前和产后骨折史。我们的病例中不存在其他相关并发症。