Hyponatremia in Children and Adults with Prader-Willi Syndrome

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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Detalles Bibliográficos
Autores: Coupaye, Muriel|||0000-0002-8988-6260, Pellikaan, Karlijn|||0000-0002-0738-0275, Goldstone, Anthony|||0000-0001-8179-7071, Crinò, Antonino|||0000-0003-3548-7114, Grugni, Graziano|||0000-0003-4708-2544, Markovic, Tania|||0000-0003-2521-0357, Høybye, Charlotte|||0000-0002-3980-1927, Caixàs i Pedragós, Assumpta|||0000-0001-8472-9189, Mosbah, Helena|||0000-0001-7360-4209, De Graaff, Laura C. G., Tauber, Maithé, Poitou, Christine|||0000-0001-7769-6331
Tipo de recurso: artículo
Fecha de publicación:2021
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:290160
Acceso en línea:https://ddd.uab.cat/record/290160
https://dx.doi.org/urn:doi:10.3390/jcm10163555
Access Level:acceso abierto
Palabra clave:Desmopressin
Excessive fluid intake
Hyponatremia
Prader-Willi syndrome
Syndrome of inappropriate antidiuretic hormone
Descripción
Sumario: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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