Karacan Küçükali, GülçinÇetinkaya, SemaTunç, GaffariOğuz, M. MelekÇelik, NurullahAkkaş, Kardelen YağmurŞenel, SalihNaz, GülerayErdeve Savaş, Şenay2022-05-122022-05-122021Karacan Küçükali G, Çetinkaya S, Tunç G, Oğuz MM, Çelik N, Akkaş KY, Şenel S, Güleray Lafcı N, Savaş Erdeve Ş. Clinical Management in Systemic Type Pseudohypoaldosteronism Due to SCNN1B Variant and Literature Review. J Clin Res Pediatr Endocrinol. 2021 Nov 25;13(4):446-451. doi: 10.4274/jcrpe.galenos.2020.2020.0107.https://cms.galenos.com.tr/Uploads/Article_40066/JCRPE-13-446-En.pdfhttps://hdl.handle.net/20.500.12418/12863Systemic pseudohypoaldosteronism (PHA) is a rare, salt-wasting syndrome that is caused by inactivating variants in genes encoding epithelial sodium channel subunits. Hyponatremia, hyperkalemia, metabolic acidosis, increased aldosterone and renin levels are expected findings in PHA. Clinical management is challenging due to high dose oral replacement therapy. Furthermore, patients with systemic PHA require life-long therapy. Here we report a patient with systemic PHA due to SCNN1B variant whose hyponatremia and hyperkalemia was detected at the 24th hour of life. Hyperkalemia did not improve with conventional treatments and dialysis was required. He also developed myocarditis and hypertension in follow-up. Challenges for diagnosis and treatment in this patient are discussed herein. In addition, published evidence concerning common features of patients with SCNN1B variant are reviewed.en10.4274/jcrpe.galenos.2020.2020.0107.info:eu-repo/semantics/openAccess: Systemic pseudohypoaldosteronism, hyponatremia, hyperkalemia, metabolic acidosis, epithelial sodium channel, SCNN1BArticle134451446328400962-s2.0-85122222138N/AWOS:000734457400011Q3