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诊断范可尼贫血:来自印度农村地区的罕见病例报告。

Diagnosing Fanconi Anemia: A Rare Case Report From Rural India.

作者信息

Malik Aashita

机构信息

Pediatrics, Datta Meghe Institute of Higher Education and Research, Wardha, IND.

出版信息

Cureus. 2024 Jun 28;16(6):e63381. doi: 10.7759/cureus.63381. eCollection 2024 Jun.

DOI:10.7759/cureus.63381
PMID:39077270
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11283913/
Abstract

Fanconi anemia is a rare but most prevalent form of inherited aplastic anemia, predominantly transmitted in an autosomal recessive manner, except for one X-linked variant. It arises from mutations in the genes across 16 different complementation groups that are crucial for DNA stability. It is marked by a wide range of congenital malformations, progressive pancytopenia, and an increased risk of both hematological malignancies and solid tumors. The congenital abnormalities associated with it can affect various organ systems, including the skeletal system, with significant variability among patients. One similar case has been reported here, which had the typical clinical features of FA. Due to varied phenotypic presentation, diagnosing FA can be challenging. A Chromosomal Breakage Study using mitomycin C (MMC) or diepoxybutane (DEB) is a distinctive cellular marker that aids in the diagnosis.

摘要

范可尼贫血是一种罕见但最常见的遗传性再生障碍性贫血,除一种X连锁变异型外,主要以常染色体隐性方式遗传。它源于16个不同互补组中的基因突变,这些基因对DNA稳定性至关重要。其特征是广泛的先天性畸形、进行性全血细胞减少,以及血液系统恶性肿瘤和实体瘤的风险增加。与之相关的先天性异常可影响包括骨骼系统在内的各种器官系统,患者之间存在显著差异。本文报告了一例具有典型范可尼贫血临床特征的类似病例。由于表型表现多样,诊断范可尼贫血具有挑战性。使用丝裂霉素C(MMC)或环氧丁烷(DEB)进行染色体断裂研究是一种有助于诊断的独特细胞标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab4/11283913/d3387919631b/cureus-0016-00000063381-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab4/11283913/f3966117c0f8/cureus-0016-00000063381-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab4/11283913/d3387919631b/cureus-0016-00000063381-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab4/11283913/f3966117c0f8/cureus-0016-00000063381-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab4/11283913/d3387919631b/cureus-0016-00000063381-i02.jpg

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本文引用的文献

1
Patterns and frequency of renal abnormalities in Fanconi anaemia: implications for long-term management.范可尼贫血症患者肾脏异常的模式和频率:对长期管理的影响。
Pediatr Nephrol. 2018 Sep;33(9):1547-1551. doi: 10.1007/s00467-018-3952-0. Epub 2018 Apr 12.
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FANCA Gene Mutations with 8 Novel Molecular Changes in Indian Fanconi Anemia Patients.印度范可尼贫血患者中具有8种新分子变化的FANCA基因突变
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Prenatal detection of Fanconi anemia.范可尼贫血的产前检测
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Chromosomal instability and molecular mutations in multi spectrum disease of Fanconi anemia.范可尼贫血多谱疾病中的染色体不稳定与分子突变
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Guido Fanconi (1892-1979): a jack of all trades.圭多·范科尼(1892 - 1979):一位多才多艺的人。
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6
Quantitative PCR analysis reveals a high incidence of large intragenic deletions in the FANCA gene in Spanish Fanconi anemia patients.定量PCR分析显示,西班牙范可尼贫血患者中FANCA基因的大片段基因内缺失发生率很高。
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Fanconi anemia in Tunisia: high prevalence of group A and identification of new FANCA mutations.突尼斯的范可尼贫血:A型的高患病率及新FANCA突变的鉴定
J Hum Genet. 2003;48(7):352-61. doi: 10.1007/s10038-003-0037-z. Epub 2003 Jun 24.
8
A multiprotein nuclear complex connects Fanconi anemia and Bloom syndrome.一种多蛋白核复合物将范可尼贫血和布卢姆综合征联系起来。
Mol Cell Biol. 2003 May;23(10):3417-26. doi: 10.1128/MCB.23.10.3417-3426.2003.
9
The Fanconi anaemia/BRCA pathway.范可尼贫血/乳腺癌易感基因通路。
Nat Rev Cancer. 2003 Jan;3(1):23-34. doi: 10.1038/nrc970.
10
Interaction of the Fanconi anemia proteins and BRCA1 in a common pathway.范可尼贫血蛋白与BRCA1在共同通路中的相互作用。
Mol Cell. 2001 Feb;7(2):249-62. doi: 10.1016/s1097-2765(01)00173-3.