{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T09:25:36Z","timestamp":1794043536502,"version":"4.8.0"},"reference-count":32,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T00:00:00Z","timestamp":1773273600000},"content-version":"vor","delay-in-days":11,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hypochloremic hypokalemic metabolic alkalosis (HHMA) may occur in unscreened, undiagnosed, and unsupplemented children with cystic fibrosis (cwCF) or in diagnosed people with CF (pwCF) who are inadequately supplemented with salt. It may also present as a clinical manifestation in children with cystic fibrosis transmembrane conductance regulator\u2010related disorder (cwCFTR\u2010RD) who have borderline or normal sweat chloride test (SCT) levels.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this single\u2010center retrospective study, we included cwCF and cwCFTR\u2010RD who experienced at least one HHMA episode between January 2002 and August 2024. We compared the demographic, genetic, clinical, and laboratory characteristics of both groups within the context of the\n                      <jats:italic>CFTR<\/jats:italic>\n                      variant diversity observed in T\u00fcrkiye.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 103 children (91 cwCF, 12 cwCFTR\u2010RD) were evaluated, comprising 150 HHMA episodes. Median SCT levels were significantly lower in cwCFTR\u2010RD than in cwCF (43 [IQR, 34\u201353] vs. 90 [IQR, 75\u201399]\u2009mmol\/L,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Variants such as F1052V, D1152H, and F200I were observed in 6 of 24 variants (25%) in cwCFTR\u2010RD. CF\u2010related complications including diabetes (15.3%), chronic\n                      <jats:italic>Pseudomonas aeruginosa<\/jats:italic>\n                      infection (18%), and pancreatic insufficiency (83%) occurred only in cwCF.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>HHMA represents an early and clinically significant manifestation in both cwCF and cwCFTR\u2010RD. Clearer acknowledgment of HHMA as a manifestation of CFTR\u2010RD within guidelines is crucial for improving diagnostic clarity and guiding appropriate management decisions. Salt supplementation, family education, and long\u2010term follow\u2010up are essential components of care for both groups. Furthermore, limited access to CFTR functional assays and the ineligibility of one\u2010third of cwCF for current modulators together show the ongoing diagnostic and therapeutic gaps.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71555","type":"journal-article","created":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T10:30:41Z","timestamp":1773311441000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Hypochloremic Hypokalemic Metabolic Alkalosis as a Manifestation of CFTR\u2010Related Disorder"],"prefix":"10.1002","volume":"61","author":[{"given":"Burcu","family":"Capraz Yavuz","sequence":"first","affiliation":[{"name":"Department of Pediatric Pulmonology Hacettepe University Faculty of Medicine, Ihsan Dogramaci Children's Hospital Ankara 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