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两例同胞全血细胞减少伴遗传性叶酸吸收不良:病例报告

Hereditary Folate Malabsorption Presenting With Pancytopenia in Two Siblings: A Case Report.

作者信息

N'joumi Chaimae, Ghanam Ayad, Tkak Hassnae, Babakhouya Abdeladim, Rkain Maria

机构信息

Pediatrics, Centre Hospitalier Universitaire Mohammed VI, Oujda, MAR.

Pediatric Medicine, Faculty of Medicine and Pharmacy, Mohammed I University, Centre Hospitalier Universitaire Mohammed VI, Oujda, MAR.

出版信息

Cureus. 2025 Aug 11;17(8):e89809. doi: 10.7759/cureus.89809. eCollection 2025 Aug.

Abstract

Hereditary folate malabsorption (HFM) is a rare autosomal recessive disorder caused by mutations in the SLC46A1 gene, leading to impaired intestinal and central nervous system folate transport. We present two male siblings with clinical features suggestive of HFM. The first infant exhibited pancytopenia, diarrhea, hypogammaglobulinemia, and neurological regression due to delayed diagnosis and treatment discontinuation, resulting in a fatal outcome. The second sibling was diagnosed early based on clinical suspicion and family history and showed favorable progress after timely parenteral folinic acid therapy. This report underscores the importance of early recognition, the limitations of genetic access in low-resource settings, and the critical role of parenteral folinic acid in preventing irreversible complications.

摘要

遗传性叶酸吸收不良(HFM)是一种罕见的常染色体隐性疾病,由SLC46A1基因突变引起,导致肠道和中枢神经系统叶酸转运受损。我们报告了两名具有提示HFM临床特征的男性同胞。第一名婴儿因诊断延迟和治疗中断出现全血细胞减少、腹泻、低丙种球蛋白血症和神经功能衰退,最终导致死亡。第二名同胞基于临床怀疑和家族史早期确诊,并在及时接受肠外亚叶酸治疗后病情好转。本报告强调了早期识别的重要性、资源匮乏地区基因检测的局限性以及肠外亚叶酸在预防不可逆并发症方面的关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56f6/12422168/a35214bc663b/cureus-0017-00000089809-i01.jpg

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