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一例罕见的染色体异常病例:先天性皮肤发育不全和生殖器模糊患者的19q13.11缺失

A Rare Case of Chromosomal Abnormality: 19q13.11 Deletion in a Patient With Aplasia Cutis Congenita and Ambiguous Genitalia.

作者信息

Srivastava Anshuman, Sharma Rishika, Kadam Sandhya, Bongurala Archana Reddy

机构信息

Family Medicine, Family HealthCare Network, Visalia, USA.

Pediatric Medicine, Kaweah Delta Health Care District, Visalia, USA.

出版信息

Cureus. 2025 Apr 23;17(4):e82871. doi: 10.7759/cureus.82871. eCollection 2025 Apr.

DOI:10.7759/cureus.82871
PMID:40290553
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12023630/
Abstract

Aplasia cutis congenita (ACC) is described as the local or generalized absence of the skin, the most common site being the scalp. Approximately one-third to one-sixth of the cases are associated with underlying bone and scalp dura mater defects. ACC may have other congenital defects, some of which are known as cleft deformities, abdominal wall defects, limb anomalies, and fetus papyraceus. Some of the ACC cases involve lesser-known defects. Different treatment options are available without clear, definitive guidelines. This case involves a patient who was born without an antenatal diagnosis of ACC and was found to have ambiguous genitalia and a chromosomal abnormality, a 19q13.11 deletion. Based on available treatment options in the literature, this patient was treated with a multidisciplinary specialty approach with conservative options and is waiting for future surgical repair. Limited cases of ACC are documented in the literature, which are considered great sources for obtaining knowledge about ACC. This case report aims to add to this great source with a discussion of the advantages and disadvantages of available treatment options and possible etiopathological factors. This case underscores the importance of a multidisciplinary approach to managing ACC, given its potential for diverse clinical presentations and the need for individualized treatment plans. ACC can be associated with various anomalies, including skeletal, neurological, and other congenital malformations.

摘要

先天性皮肤发育不全(ACC)被描述为局部或全身性皮肤缺失,最常见的部位是头皮。约三分之一至六分之一的病例伴有潜在的骨骼和头皮硬脑膜缺损。ACC可能伴有其他先天性缺陷,其中一些被称为腭裂畸形、腹壁缺损、肢体畸形和纸样胎儿。部分ACC病例涉及鲜为人知的缺陷。目前有不同的治疗选择,但缺乏明确、确切的指导方针。本病例中的患者出生时未进行产前ACC诊断,被发现存在生殖器模糊和染色体异常,即19q13.11缺失。根据文献中现有的治疗选择,该患者采用了多学科专业方法进行保守治疗,目前正在等待未来的手术修复。文献中记录的ACC病例有限,这些病例被视为获取ACC相关知识的重要来源。本病例报告旨在通过讨论现有治疗选择的优缺点以及可能的病因病理因素,为这一重要来源增添内容。鉴于ACC可能出现多种临床表现且需要个性化治疗方案,本病例强调了多学科方法管理ACC的重要性。ACC可能与各种异常有关,包括骨骼、神经和其他先天性畸形。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a12/12023630/6301db189339/cureus-0017-00000082871-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a12/12023630/6301db189339/cureus-0017-00000082871-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a12/12023630/6301db189339/cureus-0017-00000082871-i01.jpg

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本文引用的文献

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An Extensive Case of Aplasia Cutis Congenita.先天性皮肤发育不全广泛病例
Cureus. 2024 Jun 26;16(6):e63215. doi: 10.7759/cureus.63215. eCollection 2024 Jun.
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UBA2 variants underlie a recognizable syndrome with variable aplasia cutis congenita and ectrodactyly.UBA2 变异是一种具有可变先天皮肤发育不全和并指畸形的可识别综合征的基础。
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19q13.11 cryptic deletion: description of two new cases and indication for a role of WTIP haploinsufficiency in hypospadias.19q13.11 缺失:两例新病例的描述及 WTIP 杂合不足在尿道下裂中的作用提示。
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Aplasia cutis congenita of the scalp: is there a better treatment strategy?先天性头皮皮肤发育不全:是否存在更好的治疗策略?
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