5444ca7f23f2be0a75bc14e17438704e pmc_34441851.pdf 8a94a5a58f372aab748acc3e7df386156f802cfc pmc_34441851.pdf a3b2876e6eafd382c9d3a2d2aa2c2e66f6972f5ce1aeda7e23f4d8f7e338819b pmc_34441851.pdf Title: Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries Subject: In Prader–Willi syndrome (PWS), conditions that are associated with hyponatremia are common, such as excessive fluid intake (EFI), desmopressin use and syndrome of inappropriate antidiuretic hormone (SIADH) caused by psychotropic medication. However, the prevalence of hyponatremia in PWS has rarely been reported. Our aim was to describe the prevalence and severity of hyponatremia in PWS. In October 2020, we performed a retrospective study based on the medical records of a large cohort of children and adults with PWS from seven countries. Among 1326 patients (68% adults), 34 (2.6%) had at least one episode of mild or moderate hyponatremia (125 Na < 135 mmol/L). The causes of non-severe hyponatremia were often multi-factorial, including psychotropic medication in 32%, EFI in 24% and hyperglycemia in 12%. No obvious cause was found in 29%. Seven (0.5%) adults experienced severe hyponatremia (Na < 125 mmol/L). Among these, five recovered completely, but two died. The causes of severe hyponatremia were desmopressin treatment for nocturnal enuresis (n = 2), EFI (n = 2), adrenal insufficiency (n = 1), diuretic treatment (n = 1) and unknown (n = 1). In conclusion, severe hyponatremia was very rare but potentially fatal in PWS. Desmopressin treatment for nocturnal enuresis should be avoided. Enquiring about EFI and monitoring serum sodium should be included in the routine follow-ups of patients with PWS. Keywords: Prader–Willi syndrome; hyponatremia; excessive fluid intake; syndrome of inappropriate antidiuretic hormone; desmopressin Author: Muriel Coupaye, Karlijn Pellikaan, Anthony P. Goldstone, Antonino Crinò, Graziano Grugni, Tania P. Markovic, Charlotte Høybye, Assumpta Caixàs, Helena Mosbah, Laura C. G. De Graaff, Maithé Tauber and Christine Poitou Creator: LaTeX with hyperref Producer: pdfTeX-1.40.21 CreationDate: Tue Aug 17 04:23:32 2021 CEST ModDate: Tue Aug 17 04:57:11 2021 CEST Tagged: no UserProperties: no Suspects: no Form: none JavaScript: no Pages: 8 Encrypted: no Page size: 595.276 x 841.89 pts (A4) Page rot: 0 File size: 264719 bytes Optimized: no PDF version: 1.7 name type encoding emb sub uni object ID ------------------------------------ ----------------- ---------------- --- --- --- --------- FTAXPX+VnURWPalladioL Type 1 Custom yes yes no 10 0 YDFURM+URWPalladioL-Ital Type 1 Custom yes yes no 15 0 IDECPB+URWPalladioL-Bold Type 1 Custom yes yes no 20 0 MRTDBV+URWPalladioL-Roma Type 1 Custom yes yes no 24 0 TNBSFT+CMSY10 Type 1 Builtin yes yes no 28 0 JYSWDU+VnURWPalladioL-Bold Type 1 Custom yes yes no 32 0 XPUSBJ+TimesNewRomanPS-BoldItalicMT Type 1C WinAnsi yes yes no 40 0 BSDWLC+TimesNewRomanPSMT Type 1C WinAnsi yes yes no 43 0 MCBCCI+SourceSansRoman-Regular Type 1C Custom yes yes no 65 0 MCBCCM+SourceSansRoman.613wght Type 1C Custom yes yes no 69 0 MCBCCO+SourceSansRoman-Bold Type 1C Custom yes yes no 73 0 OVNEXW+EURM10 Type 1 Builtin yes yes no 88 0 EUQOAW+EURM7 Type 1 Builtin yes yes no 98 0 Jhove (Rel. 1.22.1, 2019-04-17) Date: 2021-09-11 02:52:01 CEST RepresentationInformation: pmc_34441851.pdf ReportingModule: PDF-hul, Rel. 1.12.1 (2019-04-17) LastModified: 2021-08-30 03:36:27 CEST Size: 264719 Format: PDF Version: 1.7 Status: Well-Formed and valid SignatureMatches: PDF-hul MIMEtype: application/pdf PDFMetadata: Objects: 212 FreeObjects: 1 IncrementalUpdates: 0 DocumentCatalog: PageLayout: SinglePage PageMode: UseNone Outlines: Item: Title: Introduction Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@13c27452 Item: Title: Methods Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@262b2c86 Children: Item: Title: Study Rationale and Design Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@371a67ec Item: Title: Statistical Analysis Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@5ed828d Item: Title: Results Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@50d0686 Children: Item: Title: Individual Clinical Presentation of the Four Most Recent Cases with Severe Hyponatremia Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@7a3d45bd Item: Title: Severe Cases of Hyponatremia in PWS Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@1e7c7811 Item: Title: Mild or Moderate Cases of Hyponatremia in PWS Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@77ec78b9 Item: Title: Discussion Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@1a3869f4 Item: Title: Conclusions Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@a38d7a3 Item: Title: References Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@77f99a05 Info: Title: Hyponatremia in Children and Adults with Prader Willi Syndrome: A Survey Involving Seven Countries Author: Muriel Coupaye, Karlijn Pellikaan, Anthony P. Goldstone, Antonino Crinò, Graziano Grugni, Tania P. Markovic, Charlotte Høybye, Assumpta Caixàs, Helena Mosbah, Laura C. G. De Graaff, Maithé Tauber and Christine Poitou Subject: In Prader Willi syndrome (PWS), conditions that are associated with hyponatremia are common, such as excessive fluid intake (EFI), desmopressin use and syndrome of inappropriate antidiuretic hormone (SIADH) caused by psychotropic medication. However, the prevalence of hyponatremia in PWS has rarely been reported. Our aim was to describe the prevalence and severity of hyponatremia in PWS. In October 2020, we performed a retrospective study based on the medical records of a large cohort of children and adults with PWS from seven countries. Among 1326 patients (68% adults), 34 (2.6%) had at least one episode of mild or moderate hyponatremia (125 Na < 135 mmol/L). The causes of non-severe hyponatremia were often multi-factorial, including psychotropic medication in 32%, EFI in 24% and hyperglycemia in 12%. No obvious cause was found in 29%. Seven (0.5%) adults experienced severe hyponatremia (Na < 125 mmol/L). Among these, five recovered completely, but two died. The causes of severe hyponatremia were desmopressin treatment for nocturnal enuresis (n = 2), EFI (n = 2), adrenal insufficiency (n = 1), diuretic treatment (n = 1) and unknown (n = 1). In conclusion, severe hyponatremia was very rare but potentially fatal in PWS. Desmopressin treatment for nocturnal enuresis should be avoided. Enquiring about EFI and monitoring serum sodium should be included in the routine follow-ups of patients with PWS. 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