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儿童魏斯-克鲁斯卡综合征伴急性淋巴细胞白血病:偶然关联还是因果关联?

Acute lymphoblastic leukemia in a child with Weiss-Kruszka syndrome: Casual or causal association?

机构信息

Pediatric Unit, Azienda USL Ferrara - Sant'Anna University Hospital of Ferrara, Ferrara, Italy.

IRCCS, Istituto delle Scienze Neurologiche di Bologna, UOC Neuropsichiatria dell'età pediatrica, Bologna, Italy.

出版信息

Eur J Med Genet. 2022 Apr;65(4):104457. doi: 10.1016/j.ejmg.2022.104457. Epub 2022 Feb 16.

DOI:10.1016/j.ejmg.2022.104457
PMID:35182807
Abstract

Weiss-Kruszka syndrome is a recently described genetic disorder characterized by craniofacial features, ptosis, dysgenesis of the corpus callosum, and neurodevelopmental impairment. It is caused by heterozygous loss-of-function variantsin ZNF462 gene. During the time, the original phenotype was expanded, including several complications, sensorineural hearing loss, congenital hypogonadotropic hypogonadism with anosmia and complete growth hormone deficiency associated with empty sella syndrome. Here we report the first case of Weiss-Kruszka syndrome, associated to a de novo 9q31.1q31.3 microdeletion showing an acute lymphoblastic leukemia. A speculation on the contribution of our case to the phenotypic expansion of WSKA is here discussed. More clinical and functional studies are needed to elucidate this association. A possible expansion of the WSKA phenotype is discussed.

摘要

Weiss-Kruszka 综合征是一种最近描述的遗传疾病,其特征为颅面特征、上睑下垂、胼胝体发育不良和神经发育障碍。它是由 ZNF462 基因的杂合功能丧失变异引起的。在此期间,原始表型被扩展,包括几个并发症,感觉神经性听力损失、先天性促性腺激素低下性性腺功能减退症伴嗅觉缺失和完全生长激素缺乏症与空泡蝶鞍综合征相关。在这里,我们报告了首例 Weiss-Kruszka 综合征病例,与新发的 9q31.1q31.3 微缺失相关,该缺失表现为急性淋巴细胞白血病。在此讨论了我们的病例对 WSKA 表型扩展的贡献的推测。需要更多的临床和功能研究来阐明这种关联。讨论了 WSKA 表型的可能扩展。

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First Report of a Novel Variant Linked to Weiss-Kruszka Syndrome and Congenital Diaphragmatic Hernia: Insights into Potential Additional Malformations.与魏斯-克鲁什卡综合征和先天性膈疝相关的新型变异的首次报告:对潜在其他畸形的见解
Mol Syndromol. 2025 May 2:1-6. doi: 10.1159/000546167.