Title |
Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome
|
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Published in |
BMC Pediatrics, February 2016
|
DOI | 10.1186/s12887-016-0563-4 |
Pubmed ID | |
Authors |
Daniel Landau, Harry J. Hirsch, Varda Gross-Tsur |
Abstract |
Prader-Willi syndrome is a complex neurogenetic, multisystem disorder. Despite the variable endocrine abnormalities and hypothalamic-pituitary axis dysfunction, hyponatremia has been reported in only a few PWS patients. In previously reported PWS individuals, hyponatremia was associated with abnormal fluid intake or during desmopressin treatment. We describe an infant with Prader-Willi syndrome who had severe, prolonged asymptomatic hyponatremia without a history of excessive fluid intake or desmopressin treatment. We compare the findings with those of the few other reported cases and describe, for the first time, results of a hypertonic saline infusion test and studies of adrenal cortical function. Hyponatremia should be suspected in children with Prader-Willi syndrome, especially in infants with severe failure to thrive. Further studies are needed to determine the pathophysiology of hyponatremia in this syndrome. |
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