{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T09:25:20Z","timestamp":1780565120978,"version":"3.54.1"},"reference-count":33,"publisher":"The Endocrine Society","issue":"12","license":[{"start":{"date-parts":[[2022,9,27]],"date-time":"2022-09-27T00:00:00Z","timestamp":1664236800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,10,26]]},"abstract":"Abstract<\/jats:title>\n \n Context<\/jats:title>\n Pseudohypoaldosteronism type 1 (PHA1) has been treated as a genetic variant of type IV renal tubular acidosis (RTA), leading to the conception that PHA1 develops hyperchloremic acidosis with a normal anion gap (AG).<\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n To delineate the acid\u2013base imbalance in PHA1A (dominant type) and PHA1B (recessive type).<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n We conducted the following: (1) a retrospective chart review of our patient with PHA1B, and (2) a literature search of PHA1 cases focusing on acid\u2013base balance. The main outcome measures were the incidence and nature of acidosis, including chloride levels and AG.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n In our patient with PHA1B, 7 salt-wasting episodes were analyzed. Acidosis was ascertained each time, and it was accompanied by hypochloremia except in 1 episode. AG was elevated in 5 episodes, while hyperlacticaemia was present in 3. In the literature, 41 cases of PHA1A and 65 cases of PHA1B have been identified. During salt-wasting crises, acidosis developed in 85% of PHA1A cases and 87% of PHA1B cases. Hypochloremia was present in 69% of PHA1A cases with available data (n = 13) and 54% of eligible PHA1B cases (n = 13), with mean chloride levels of 96\u2005mEq\/L and 95\u2005mEq\/L, respectively. Increased AG was less frequently reported (14% in PHA1A and 44% in PHA1B).<\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n Patients with PHA1 frequently presented with metabolic acidosis. However, hyperchloremia may not be a universal finding, whereas hypochloremia and increased AG may occur in a substantial proportion of the patients.<\/jats:p>\n <\/jats:sec>","DOI":"10.1210\/jendso\/bvac147","type":"journal-article","created":{"date-parts":[[2022,9,27]],"date-time":"2022-09-27T16:51:42Z","timestamp":1664297502000},"source":"Crossref","is-referenced-by-count":4,"title":["Acid\u2013Base Imbalance in Pseudohypoaldosteronism Type 1 in Comparison With Type IV Renal Tubular Acidosis"],"prefix":"10.1210","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4747-3983","authenticated-orcid":false,"given":"Masanori","family":"Adachi","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Showa University School of Medicine , Shinagawa-ku, Tokyo, 142-8555 , 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