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Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries

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...

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Autores principales: Coupaye, Muriel, Pellikaan, Karlijn, Goldstone, Anthony P., Crinò, Antonino, Grugni, Graziano, Markovic, Tania P., Høybye, Charlotte, Caixàs, Assumpta, Mosbah, Helena, De Graaff, Laura C. G., Tauber, Maithé, Poitou, Christine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8396837/
https://www.ncbi.nlm.nih.gov/pubmed/34441851
http://dx.doi.org/10.3390/jcm10163555
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author Coupaye, Muriel
Pellikaan, Karlijn
Goldstone, Anthony P.
Crinò, Antonino
Grugni, Graziano
Markovic, Tania P.
Høybye, Charlotte
Caixàs, Assumpta
Mosbah, Helena
De Graaff, Laura C. G.
Tauber, Maithé
Poitou, Christine
author_facet Coupaye, Muriel
Pellikaan, Karlijn
Goldstone, Anthony P.
Crinò, Antonino
Grugni, Graziano
Markovic, Tania P.
Høybye, Charlotte
Caixàs, Assumpta
Mosbah, Helena
De Graaff, Laura C. G.
Tauber, Maithé
Poitou, Christine
author_sort Coupaye, Muriel
collection PubMed
description 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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spelling pubmed-83968372021-08-28 Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries Coupaye, Muriel Pellikaan, Karlijn Goldstone, Anthony P. Crinò, Antonino Grugni, Graziano Markovic, Tania P. Høybye, Charlotte Caixàs, Assumpta Mosbah, Helena De Graaff, Laura C. G. Tauber, Maithé Poitou, Christine J Clin Med Article 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. MDPI 2021-08-12 /pmc/articles/PMC8396837/ /pubmed/34441851 http://dx.doi.org/10.3390/jcm10163555 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Coupaye, Muriel
Pellikaan, Karlijn
Goldstone, Anthony P.
Crinò, Antonino
Grugni, Graziano
Markovic, Tania P.
Høybye, Charlotte
Caixàs, Assumpta
Mosbah, Helena
De Graaff, Laura C. G.
Tauber, Maithé
Poitou, Christine
Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries
title Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries
title_full Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries
title_fullStr Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries
title_full_unstemmed Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries
title_short Hyponatremia in Children and Adults with Prader–Willi Syndrome: A Survey Involving Seven Countries
title_sort hyponatremia in children and adults with prader–willi syndrome: a survey involving seven countries
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8396837/
https://www.ncbi.nlm.nih.gov/pubmed/34441851
http://dx.doi.org/10.3390/jcm10163555
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