Talluri Sriharsha, Goedde Michael A, Rosenberg Eran, Canalichio Katie L, Peppas Dennis, White Jeffrey T
Department of Urology, University of Louisville, Louisville, KY, United States.
Department of Pediatric Urology, Norton Healthcare, Louisville, KY, United States.
Front Pediatr. 2021 Sep 23;9:729932. doi: 10.3389/fped.2021.729932. eCollection 2021.
Prune belly syndrome (PBS) is a rare congenital disease that predominantly occurs in males and is identified by its classic triad of abdominal wall musculature deficiencies, cryptorchidism, and urinary tract abnormalities. However, numerous anomalies involving the kidneys, heart, lungs, and muscles have also been reported. A multitude of chromosomal abnormalities have been implicated in its pathogenesis. PBS can occur in association with trisomy 18 and 21. Gene duplications and deletions have also been reported; however, a definite cause of PBS is still unknown. We report the first PBS patient with a copy number variant in 16p11.2.
梅干腹综合征(PBS)是一种罕见的先天性疾病,主要发生于男性,其典型三联征为腹壁肌肉组织缺陷、隐睾症和泌尿系统异常。然而,也有许多涉及肾脏、心脏、肺和肌肉的异常情况被报道。多种染色体异常与该病的发病机制有关。PBS可与18三体和21三体相关。基因重复和缺失也有报道;然而,PBS的确切病因仍不清楚。我们报告了首例16p11.2存在拷贝数变异的PBS患者。