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分析导致胚胎学经典型膀胱外翻发生发展的因素。

Analyzing the factors that contribute to the development of embryological classical type of bladder exstrophy.

作者信息

Margiana Ria, Juwita Widya, Ima Khoirul, Faizah Zakiyatul, Supardi Supardi

机构信息

Andrology Program, Faculty of Medicine, Airlangga University, Surabaya, Indonesia.

Master's Program in Biomedical Sciences, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia.

出版信息

Anat Cell Biol. 2023 Dec 31;56(4):421-427. doi: 10.5115/acb.23.056. Epub 2023 Aug 31.

Abstract

Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth, short penis, upward curvature during erection, wide penis, and undescended testes. Exstrophy affects 1/30,000 newborns. The bladder opens against the abdominal wall in bladder exstrophy, a rare genitourinary condition. This study is vital to provide appropriate therapy choices as a basis to improve patient outcomes. This study may explain bladder exstrophy and provide treatment. Epispadias, secretory placenta, cloacal exstrophy, and other embryonic abnormalities comprise the exstrophy-spades complex. The mesenchymal layer does not migrate from the ectoderm and endoderm layers in the first trimester, affecting the cloacal membrane. Embryological problems define the exstrophy-aspidistra complex, which resembles epimedium, classic bladder, cloacal exstrophy, and other diseases. Urogenital ventral body wall anomalies expose the bladder mucosa, causing bladder exstrophy. Genetic mutations in the Hedgehog cascade pathway, Wnt signal, FGF, BMP4, Alx4, Gli3, and ISL1 cause ventral body wall closure and urinary bladder failure. External factors such as high maternal age, smoking moms, and high maternal body mass index have also been associated to bladder exstrophy. Valproic acid increases bladder exstrophy risk; chemicals and pollutants during pregnancy may increase bladder exstrophy risk. Bladder exstrophy has no identified cause despite these risk factors. Exstrophy reconstruction seals the bladder, improves bowel function, reconstructs the vaginal region, and restores urination.

摘要

膀胱外翻是一种罕见的骨盆、膀胱和下腹部先天性疾病,膀胱外翻至腹壁,会导致异常生长、阴茎短小、勃起时向上弯曲、阴茎宽大以及睾丸未降。膀胱外翻影响1/30000的新生儿。膀胱外翻是一种罕见的泌尿生殖系统疾病,膀胱外翻至腹壁。本研究对于提供合适的治疗选择至关重要,可为改善患者预后提供依据。本研究或许能解释膀胱外翻并提供治疗方法。尿道上裂、分泌型胎盘、泄殖腔外翻及其他胚胎异常构成了膀胱外翻-尿道上裂复合体。在孕早期,间充质层未从外胚层和内胚层迁移,影响泄殖腔膜。胚胎学问题界定了膀胱外翻-蜘蛛抱蛋复合体,其类似于淫羊藿、典型膀胱、泄殖腔外翻及其他疾病。泌尿生殖腹侧体壁异常使膀胱黏膜外露,导致膀胱外翻。刺猬信号通路、Wnt信号、成纤维细胞生长因子、骨形态发生蛋白4、Alx4、Gliomb3和ISL1中的基因突变会导致腹侧体壁闭合及膀胱发育不全。高龄产妇、吸烟母亲及高体质指数等外部因素也与膀胱外翻有关。丙戊酸会增加膀胱外翻风险;孕期接触化学物质和污染物可能增加膀胱外翻风险。尽管存在这些风险因素,但膀胱外翻的病因仍未明确。膀胱外翻重建术可封闭膀胱、改善肠道功能、重建阴道区域并恢复排尿。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/456f/10714095/bb4bff53faf8/acb-56-4-421-f1.jpg

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