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We report on a neonatal case of PHA caused by recurrent hydrometrocolpos presenting with vomiting and poor weight gain. There was significant hyponatraemia, hyperkalaemia, and anaemia requiring medical stabilisation prior to surgery. The mechanism by which PHA occurs in obstruction involves renal tubular dysfunction due to pressure from hydronephrosis and the release of intrarenal cytokines. In addition, there is an immature or resistant renal tubular responsiveness to aldosterone during infancy. Clinicians should be aware of this uncommon but serious presentation.<\/jats:p>","DOI":"10.1136\/bcr-2020-234813","type":"journal-article","created":{"date-parts":[[2020,6,29]],"date-time":"2020-06-29T02:25:28Z","timestamp":1593397528000},"page":"e234813","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["Rare case of pseudohypoaldosteronism in a neonate secondary to congenital hydrometrocolpos"],"prefix":"10.1136","volume":"13","author":[{"given":"Shruti","family":"Kumar","sequence":"first","affiliation":[{"name":"Health Education England West Midlands, Edgbaston, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7814-4520","authenticated-orcid":false,"given":"Helen","family":"McDermott","sequence":"additional","affiliation":[{"name":"Health Education England West Midlands, Edgbaston, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheilah","family":"Kamupira","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Neonatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juliana Chizo","family":"Agwu","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Neonatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2020,6,28]]},"reference":[{"key":"2025082712402565000_13.6.e234813.1","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/s40477-013-0009-x","article-title":"Imperforate hymen causing congenital hydrometrocolpos","volume":"16","author":"Vitale","year":"2013","journal-title":"J Ultrasound"},{"key":"2025082712402565000_13.6.e234813.2","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.jpurol.2007.10.009","article-title":"Hydrometrocolpos and acute renal failure: a rare neonatal presentation of Bardet-Biedl syndrome","volume":"4","author":"Cherian","year":"2008","journal-title":"J Pediatr Urol"},{"key":"2025082712402565000_13.6.e234813.3","unstructured":"Manchester university NHS Foundation trust paediatric biochemistry user guide. 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