Secuencia de Prune Belly: a propósito de un caso

Pediatría Pub Date : 2020-03-01 DOI:10.14295/RP.V53I1.139
W. Vidal
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Abstract

Background: The Prune-Belly sequence is a genetic disorder characterized by a triad that includes: weak abdominal wall musculature, urogenital anomalies, and bilateral cryptorchidism. Associated malformations usually accompany it. The surgical approach should be reserved for those patients with severe complications.Clinical case: We present a male neonate with agenesis of the abdominal wall musculature, multicystic renal dysplasia, and bilateral cryptorchidism, which is transferred from a primary hospital in eastern Ecuador and attended in conservative management during his hospitalization.Conclusions: The approach to SPB must be multidisciplinary due to its frequent association with other malformations. Prenatal diagnosis plays a fundamental role in the early detection of severe anomalies.
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李子肚序列:关于一个案例
背景:Prune-Belly序列是一种以三联征为特征的遗传性疾病,包括:腹壁肌肉组织薄弱、泌尿生殖系统异常和双侧隐睾。通常伴有相关畸形。手术入路应保留给有严重并发症的患者。临床病例:我们报告一男新生儿腹壁肌肉发育不全,多囊肾发育不良,双侧隐睾,从厄瓜多尔东部的一家初级医院转来,住院期间接受保守治疗。结论:由于SPB常与其他畸形合并,因此必须多学科联合治疗。产前诊断在早期发现严重异常方面起着根本性的作用。
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