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范可尼贫血:一种罕见的遗传性疾病。

Fanconi Anemia: A Rare Genetic Disorder.

作者信息

Thakur Bharati, Hiwale K M

机构信息

Pathology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.

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

出版信息

Cureus. 2023 May 11;15(5):e38899. doi: 10.7759/cureus.38899. eCollection 2023 May.

Abstract

Fanconi anemia is a rare genetic disorder affecting various body systems. Congenital abnormalities, poor hematopoiesis, a higher incidence of acute myeloid leukemia, myelodysplastic syndrome, and malignancies are the hallmarks of this autosomal recessive condition. In certain instances, the clinical signs and highly diverse phenotypic presentation make a diagnosis challenging. In this case report, an eight-year-old boy presented with recurrent episodes of fever, generalized weakness and physical deformities. He had left thumb deformity, triangular face, short stature, and hyperpigmentation with café au lait spots. Bone marrow biopsy revealed hypoplastic marrow, peripheral blood smear revealed pancytopenia, and chromosomal breakage testing was also positive.

摘要

范可尼贫血是一种影响身体多个系统的罕见遗传性疾病。先天性异常、造血功能不良、急性髓系白血病、骨髓增生异常综合征和恶性肿瘤的发病率较高是这种常染色体隐性疾病的特征。在某些情况下,临床症状和高度多样的表型表现使诊断具有挑战性。在本病例报告中,一名8岁男孩出现反复发热、全身无力和身体畸形。他有左手拇指畸形、三角脸、身材矮小以及伴有咖啡牛奶斑的色素沉着。骨髓活检显示骨髓增生低下,外周血涂片显示全血细胞减少,染色体断裂检测也呈阳性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c7a/10257508/5b6a7eace807/cureus-0015-00000038899-i01.jpg

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