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However, false positives have been reported in patients with different diseases. We describe and discuss 4 cases due to different clinical conditions in which we recorded false positive ST, and the test remained altered for a period of varying length.<\/jats:p><\/jats:sec><jats:sec><jats:title>Cases presentation<\/jats:title><jats:p>Case 1: Eight months old female child suffering from constipation, recurrent vomiting and failure to thrive, family history of recurrent pancreatitis without mutations in the<jats:italic>PRSS1<\/jats:italic>and<jats:italic>SPINK1<\/jats:italic>genes. Both ST and fecal elastase were altered although no<jats:italic>CFTR<\/jats:italic>gene mutations were found. Due to rapid clinical deterioration, celiac disease was suspected and diagnosed by laboratory tests and intestinal biopsy. After 2\u2009weeks of gluten-free diet ST and fecal elastase normalized.<\/jats:p><jats:p>Case 2: 14\u2009months old male suffering from bilateral renal dysplasia, episodes of metabolic alkalosis, recurrent respiratory infections and recurrent vomiting. The child had more ST positives, but no<jats:italic>CFTR<\/jats:italic>mutations were found. During follow-up, he developed sensorineural hearing loss and an atrial septic defect was found. Finally, a diagnosis of Klinefelter was made, but the ST normalized several years later.<\/jats:p><jats:p>Case 3 and 4: Two boys with stubborn constipation and fecal occlusion treated with Poly Ethylene Glycol (PEG) with salts showed pathological ST. The test returned normal a few days after stopping treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We hypotesized the possible causes of ST alteration in these conditions: in celiac disease it could be due to a transient dysregulation of the aquaporins, rapidly reversed by the diet; in Klinefelter, it may be due to stable pubertal hypoandrogenism; while, the PEG formulation itself contains salts that can temporarily alter ST.<\/jats:p><\/jats:sec>","DOI":"10.1186\/s13052-021-01060-1","type":"journal-article","created":{"date-parts":[[2021,5,14]],"date-time":"2021-05-14T09:02:26Z","timestamp":1620982946000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Elevated sweat chloride test: is it always cystic fibrosis?"],"prefix":"10.1186","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3078-4662","authenticated-orcid":false,"given":"C.","family":"Cimbalo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Tosco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Terlizzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Sepe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Castaldo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Salvadori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Raia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,5,14]]},"reference":[{"issue":"1","key":"1060_CR1","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1016\/S2213-2600(19)30337-6","volume":"8","author":"SC Bell","year":"2020","unstructured":"Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, et al. 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