{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T13:53:25Z","timestamp":1753883605076,"version":"3.41.2"},"reference-count":12,"publisher":"The Endocrine Society","issue":"10","license":[{"start":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T00:00:00Z","timestamp":1727654400000},"content-version":"vor","delay-in-days":12,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Neuroendocrine neoplasms (NENs) encompass a heterogeneous spectrum of tumors originating from the diffuse neuroendocrine cell system. Approximately 30% of NEN exhibit functional activity with clinical syndromes through hormone-mediated effects. Synchronous and metachronous functioning syndromes, resulting from the simultaneous release of distinct hormones, are exceptionally rare. Of note, hormonal excess syndromes can have a greater effect on patients\u2019 morbidity and mortality than the tumor mass itself. We present the case of a 49-year-old male patient affected by an oligo-metastatic ileal NEN, concurrently demonstrating vasointestinal peptide (VIP) and serotonin excretion, complicated by pulmonary tuberculosis. After the first cycle of Lutetium-177-DOTATATE peptide-radio-receptor therapy, the patient developed a severe watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome, despite receiving high-dose somatostatin analogues, everolimus, and telotristat ethyl, without any surgical options. The WDHA syndrome necessitated intensive-care-unit (ICU) admission with continual intravenous administration of electrolytes and fluids. With limited alternatives, an off-label intervention using the enkephalinase inhibitor racecadotril was initiated. After 5 days of treatment, the WDHA syndrome exhibited sufficient control, facilitating the patient's discharge from the ICU. This case report underscores racecadotril as an individualized, off-label treatment strategy for patients with severe VIPoma and serotonin-driven WDHA syndrome, where conventional therapeutic avenues have been exhausted.<\/jats:p>","DOI":"10.1210\/jcemcr\/luae177","type":"journal-article","created":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T02:49:26Z","timestamp":1727664566000},"source":"Crossref","is-referenced-by-count":0,"title":["Efficacy of Racecadotril in a Patient Affected by a Therapy-Refractory VIPoma and Carcinoid Syndrome"],"prefix":"10.1210","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-1306-894X","authenticated-orcid":false,"given":"Jannes","family":"Boesenkoetter","sequence":"first","affiliation":[{"name":"Department of Interdisciplinary Endoscopy, Medical Department 1, University Hospital Schleswig-Holstein, Campus Kiel , 24105 Kiel ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ina","family":"Ellrichmann","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel , 24105 Kiel ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bj\u00f6rn","family":"Konukiewitz","sequence":"additional","affiliation":[{"name":"Department of Pathology, University Hospital Schleswig-Holstein, Campus Kiel , 24105 Kiel ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5817-5589","authenticated-orcid":false,"given":"Mark","family":"Ellrichmann","sequence":"additional","affiliation":[{"name":"Department of Interdisciplinary Endoscopy, Medical Department 1, University Hospital Schleswig-Holstein, Campus Kiel , 24105 Kiel ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dominik M","family":"Schulte","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Diabetes and Clinical Nutrition, Department of Internal Medicine I, University Hospital Schleswig-Holstein, Campus Kiel , 24105 Kiel ,","place":["Germany"]},{"name":"Institute of Diabetes and Clinical Metabolic Research, University Hospital Schleswig-Holstein, Campus Kiel , 24105 Kiel ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2024,9,30]]},"reference":[{"issue":"12","key":"2024101805240405900_luae177-B1","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1016\/j.neo.2017.09.002","article-title":"Nothing but NET: a review of neuroendocrine tumors and carcinomas","volume":"19","author":"Oronsky","year":"2017","journal-title":"Neoplasia"},{"issue":"8","key":"2024101805240405900_luae177-B2","doi-asserted-by":"crossref","first-page":"e13318","DOI":"10.1111\/jne.13318","article-title":"European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes","volume":"35","author":"Hofland","year":"2023","journal-title":"J Neuroendocrinol"},{"issue":"7","key":"2024101805240405900_luae177-B3","doi-asserted-by":"crossref","first-page":"e13146","DOI":"10.1111\/jne.13146","article-title":"European neuroendocrine tumor society (ENETS) 2022 guidance paper for carcinoid syndrome and carcinoid heart disease","volume":"34","author":"Grozinsky-Glasberg","year":"2022","journal-title":"J Neuroendocrinol"},{"issue":"19","key":"2024101805240405900_luae177-B4","doi-asserted-by":"crossref","first-page":"4769","DOI":"10.3390\/cancers14194769","article-title":"Therapy resistant gastroenteropancreatic neuroendocrine tumors","volume":"14","author":"McClellan","year":"2022","journal-title":"Cancers (Basel)"},{"key":"2024101805240405900_luae177-B5","doi-asserted-by":"crossref","first-page":"93","DOI":"10.3389\/fphar.2012.00093","article-title":"A comprehensive review of the pharmacodynamics, pharmacokinetics, and clinical effects of the neutral endopeptidase inhibitor racecadotril","volume":"3","author":"Eberlin","year":"2012","journal-title":"Front Pharmacol"},{"issue":"5","key":"2024101805240405900_luae177-B6","first-page":"485","article-title":"Treatment of refractory diarrhoea in AIDS with acetorphan and octreotide: a randomized crossover study","volume":"8","author":"Beaugerie","year":"1996","journal-title":"Eur J Gastroenterol Hepatol"},{"issue":"12","key":"2024101805240405900_luae177-B7","first-page":"CD009359","article-title":"Racecadotril for acute diarrhoea in children","volume":"12","author":"Liang","year":"2019","journal-title":"Cochrane Database Syst 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