Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients

Abstract

RUBACE is a practical, organ/system level, phenotyping tool designed to grade PBS severity and categorise patients into isolated PBS, syndromic PBS, and PBS-plus groups. This standardised system will facilitate genotype-phenotype correlations and future prospective multicentre studies assessing medical and surgical treatment outcomes.