Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome

Background 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 w...
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Autor*in:	Landau, Daniel [verfasserIn] Hirsch, Harry J. Gross-Tsur, Varda

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2016

Schlagwörter:	Prader-Willi syndrome (PWS) Hyponatremia Anti-diuretic hormone (ADH) Adrenocortioctrophic hormone (ACTH) test

Anmerkung:	© Landau et al. 2016

Übergeordnetes Werk:	Enthalten in: BMC pediatrics - London : BioMed Central, 2001, 16(2016), 1 vom: 18. Feb.
Übergeordnetes Werk:	volume:16 ; year:2016 ; number:1 ; day:18 ; month:02

Links:	Volltext

DOI / URN:	10.1186/s12887-016-0563-4

Katalog-ID:	SPR027761495

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520			\|a Background 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. Case presentation 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. Conclusion 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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allfields	10.1186/s12887-016-0563-4 doi (DE-627)SPR027761495 (SPR)s12887-016-0563-4-e DE-627 ger DE-627 rakwb eng Landau, Daniel verfasserin aut Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Landau et al. 2016 Background 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. Case presentation 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. Conclusion 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. Prader-Willi syndrome (PWS) (dpeaa)DE-He213 Hyponatremia (dpeaa)DE-He213 Anti-diuretic hormone (ADH) (dpeaa)DE-He213 Adrenocortioctrophic hormone (ACTH) test (dpeaa)DE-He213 Hirsch, Harry J. aut Gross-Tsur, Varda aut Enthalten in BMC pediatrics London : BioMed Central, 2001 16(2016), 1 vom: 18. Feb. (DE-627)326643621 (DE-600)2041342-7 1471-2431 nnns volume:16 year:2016 number:1 day:18 month:02 https://dx.doi.org/10.1186/s12887-016-0563-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2016 1 18 02
spelling	10.1186/s12887-016-0563-4 doi (DE-627)SPR027761495 (SPR)s12887-016-0563-4-e DE-627 ger DE-627 rakwb eng Landau, Daniel verfasserin aut Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Landau et al. 2016 Background 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. Case presentation 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. Conclusion 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. Prader-Willi syndrome (PWS) (dpeaa)DE-He213 Hyponatremia (dpeaa)DE-He213 Anti-diuretic hormone (ADH) (dpeaa)DE-He213 Adrenocortioctrophic hormone (ACTH) test (dpeaa)DE-He213 Hirsch, Harry J. aut Gross-Tsur, Varda aut Enthalten in BMC pediatrics London : BioMed Central, 2001 16(2016), 1 vom: 18. Feb. (DE-627)326643621 (DE-600)2041342-7 1471-2431 nnns volume:16 year:2016 number:1 day:18 month:02 https://dx.doi.org/10.1186/s12887-016-0563-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2016 1 18 02
allfields_unstemmed	10.1186/s12887-016-0563-4 doi (DE-627)SPR027761495 (SPR)s12887-016-0563-4-e DE-627 ger DE-627 rakwb eng Landau, Daniel verfasserin aut Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Landau et al. 2016 Background 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. Case presentation 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. Conclusion 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. Prader-Willi syndrome (PWS) (dpeaa)DE-He213 Hyponatremia (dpeaa)DE-He213 Anti-diuretic hormone (ADH) (dpeaa)DE-He213 Adrenocortioctrophic hormone (ACTH) test (dpeaa)DE-He213 Hirsch, Harry J. aut Gross-Tsur, Varda aut Enthalten in BMC pediatrics London : BioMed Central, 2001 16(2016), 1 vom: 18. Feb. (DE-627)326643621 (DE-600)2041342-7 1471-2431 nnns volume:16 year:2016 number:1 day:18 month:02 https://dx.doi.org/10.1186/s12887-016-0563-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2016 1 18 02
allfieldsGer	10.1186/s12887-016-0563-4 doi (DE-627)SPR027761495 (SPR)s12887-016-0563-4-e DE-627 ger DE-627 rakwb eng Landau, Daniel verfasserin aut Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Landau et al. 2016 Background 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. Case presentation 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. Conclusion 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. Prader-Willi syndrome (PWS) (dpeaa)DE-He213 Hyponatremia (dpeaa)DE-He213 Anti-diuretic hormone (ADH) (dpeaa)DE-He213 Adrenocortioctrophic hormone (ACTH) test (dpeaa)DE-He213 Hirsch, Harry J. aut Gross-Tsur, Varda aut Enthalten in BMC pediatrics London : BioMed Central, 2001 16(2016), 1 vom: 18. Feb. (DE-627)326643621 (DE-600)2041342-7 1471-2431 nnns volume:16 year:2016 number:1 day:18 month:02 https://dx.doi.org/10.1186/s12887-016-0563-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2016 1 18 02
allfieldsSound	10.1186/s12887-016-0563-4 doi (DE-627)SPR027761495 (SPR)s12887-016-0563-4-e DE-627 ger DE-627 rakwb eng Landau, Daniel verfasserin aut Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Landau et al. 2016 Background 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. Case presentation 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. Conclusion 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. Prader-Willi syndrome (PWS) (dpeaa)DE-He213 Hyponatremia (dpeaa)DE-He213 Anti-diuretic hormone (ADH) (dpeaa)DE-He213 Adrenocortioctrophic hormone (ACTH) test (dpeaa)DE-He213 Hirsch, Harry J. aut Gross-Tsur, Varda aut Enthalten in BMC pediatrics London : BioMed Central, 2001 16(2016), 1 vom: 18. Feb. (DE-627)326643621 (DE-600)2041342-7 1471-2431 nnns volume:16 year:2016 number:1 day:18 month:02 https://dx.doi.org/10.1186/s12887-016-0563-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 16 2016 1 18 02
language	English
source	Enthalten in BMC pediatrics 16(2016), 1 vom: 18. Feb. volume:16 year:2016 number:1 day:18 month:02
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topic_facet	Prader-Willi syndrome (PWS) Hyponatremia Anti-diuretic hormone (ADH) Adrenocortioctrophic hormone (ACTH) test
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author	Landau, Daniel
spellingShingle	Landau, Daniel misc Prader-Willi syndrome (PWS) misc Hyponatremia misc Anti-diuretic hormone (ADH) misc Adrenocortioctrophic hormone (ACTH) test Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome
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topic_title	Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome Prader-Willi syndrome (PWS) (dpeaa)DE-He213 Hyponatremia (dpeaa)DE-He213 Anti-diuretic hormone (ADH) (dpeaa)DE-He213 Adrenocortioctrophic hormone (ACTH) test (dpeaa)DE-He213
topic	misc Prader-Willi syndrome (PWS) misc Hyponatremia misc Anti-diuretic hormone (ADH) misc Adrenocortioctrophic hormone (ACTH) test
topic_unstemmed	misc Prader-Willi syndrome (PWS) misc Hyponatremia misc Anti-diuretic hormone (ADH) misc Adrenocortioctrophic hormone (ACTH) test
topic_browse	misc Prader-Willi syndrome (PWS) misc Hyponatremia misc Anti-diuretic hormone (ADH) misc Adrenocortioctrophic hormone (ACTH) test
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title	Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome
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title_full	Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome
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author_browse	Landau, Daniel Hirsch, Harry J. Gross-Tsur, Varda
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title_sort	case report: severe asymptomatic hyponatremia in prader-willi syndrome
title_auth	Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome
abstract	Background 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. Case presentation 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. Conclusion 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. © Landau et al. 2016
abstractGer	Background 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. Case presentation 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. Conclusion 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. © Landau et al. 2016
abstract_unstemmed	Background 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. Case presentation 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. Conclusion 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. © Landau et al. 2016
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title_short	Case report: severe asymptomatic hyponatremia in Prader-Willi Syndrome
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up_date	2024-07-03T14:59:52.602Z
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