Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center

Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients...
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Autor*in:	Pacoricona Alfaro, Dibia Liz [verfasserIn] Lemoine, Perrine Ehlinger, Virginie Molinas, Catherine Diene, Gwénaëlle Valette, Marion Pinto, Graziella Coupaye, Muriel Poitou-Bernert, Christine Thuilleaux, Denise Arnaud, Catherine Tauber, Maithé

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2019

Schlagwörter:	Prader-Willi syndrome Epidemiology Mortality Respiratory complications Sudden death

Anmerkung:	© The Author(s). 2019

Übergeordnetes Werk:	Enthalten in: Orphanet journal of rare diseases - London : BioMed Central, 2006, 14(2019), 1 vom: 04. Nov.
Übergeordnetes Werk:	volume:14 ; year:2019 ; number:1 ; day:04 ; month:11

Links:	Volltext

DOI / URN:	10.1186/s13023-019-1214-2

Katalog-ID:	SPR029504007

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520			\|a Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese.
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allfields	10.1186/s13023-019-1214-2 doi (DE-627)SPR029504007 (SPR)s13023-019-1214-2-e DE-627 ger DE-627 rakwb eng Pacoricona Alfaro, Dibia Liz verfasserin aut Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s). 2019 Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. Prader-Willi syndrome (dpeaa)DE-He213 Epidemiology (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Respiratory complications (dpeaa)DE-He213 Sudden death (dpeaa)DE-He213 Lemoine, Perrine aut Ehlinger, Virginie aut Molinas, Catherine aut Diene, Gwénaëlle aut Valette, Marion aut Pinto, Graziella aut Coupaye, Muriel aut Poitou-Bernert, Christine aut Thuilleaux, Denise aut Arnaud, Catherine aut Tauber, Maithé (orcid)0000-0002-0363-7862 aut Enthalten in Orphanet journal of rare diseases London : BioMed Central, 2006 14(2019), 1 vom: 04. Nov. (DE-627)50900637X (DE-600)2225857-7 1750-1172 nnns volume:14 year:2019 number:1 day:04 month:11 https://dx.doi.org/10.1186/s13023-019-1214-2 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 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_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 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 14 2019 1 04 11
spelling	10.1186/s13023-019-1214-2 doi (DE-627)SPR029504007 (SPR)s13023-019-1214-2-e DE-627 ger DE-627 rakwb eng Pacoricona Alfaro, Dibia Liz verfasserin aut Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s). 2019 Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. Prader-Willi syndrome (dpeaa)DE-He213 Epidemiology (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Respiratory complications (dpeaa)DE-He213 Sudden death (dpeaa)DE-He213 Lemoine, Perrine aut Ehlinger, Virginie aut Molinas, Catherine aut Diene, Gwénaëlle aut Valette, Marion aut Pinto, Graziella aut Coupaye, Muriel aut Poitou-Bernert, Christine aut Thuilleaux, Denise aut Arnaud, Catherine aut Tauber, Maithé (orcid)0000-0002-0363-7862 aut Enthalten in Orphanet journal of rare diseases London : BioMed Central, 2006 14(2019), 1 vom: 04. Nov. (DE-627)50900637X (DE-600)2225857-7 1750-1172 nnns volume:14 year:2019 number:1 day:04 month:11 https://dx.doi.org/10.1186/s13023-019-1214-2 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 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_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 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 14 2019 1 04 11
allfields_unstemmed	10.1186/s13023-019-1214-2 doi (DE-627)SPR029504007 (SPR)s13023-019-1214-2-e DE-627 ger DE-627 rakwb eng Pacoricona Alfaro, Dibia Liz verfasserin aut Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s). 2019 Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. Prader-Willi syndrome (dpeaa)DE-He213 Epidemiology (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Respiratory complications (dpeaa)DE-He213 Sudden death (dpeaa)DE-He213 Lemoine, Perrine aut Ehlinger, Virginie aut Molinas, Catherine aut Diene, Gwénaëlle aut Valette, Marion aut Pinto, Graziella aut Coupaye, Muriel aut Poitou-Bernert, Christine aut Thuilleaux, Denise aut Arnaud, Catherine aut Tauber, Maithé (orcid)0000-0002-0363-7862 aut Enthalten in Orphanet journal of rare diseases London : BioMed Central, 2006 14(2019), 1 vom: 04. Nov. (DE-627)50900637X (DE-600)2225857-7 1750-1172 nnns volume:14 year:2019 number:1 day:04 month:11 https://dx.doi.org/10.1186/s13023-019-1214-2 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 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_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 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 14 2019 1 04 11
allfieldsGer	10.1186/s13023-019-1214-2 doi (DE-627)SPR029504007 (SPR)s13023-019-1214-2-e DE-627 ger DE-627 rakwb eng Pacoricona Alfaro, Dibia Liz verfasserin aut Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s). 2019 Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. Prader-Willi syndrome (dpeaa)DE-He213 Epidemiology (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Respiratory complications (dpeaa)DE-He213 Sudden death (dpeaa)DE-He213 Lemoine, Perrine aut Ehlinger, Virginie aut Molinas, Catherine aut Diene, Gwénaëlle aut Valette, Marion aut Pinto, Graziella aut Coupaye, Muriel aut Poitou-Bernert, Christine aut Thuilleaux, Denise aut Arnaud, Catherine aut Tauber, Maithé (orcid)0000-0002-0363-7862 aut Enthalten in Orphanet journal of rare diseases London : BioMed Central, 2006 14(2019), 1 vom: 04. Nov. (DE-627)50900637X (DE-600)2225857-7 1750-1172 nnns volume:14 year:2019 number:1 day:04 month:11 https://dx.doi.org/10.1186/s13023-019-1214-2 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 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_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 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 14 2019 1 04 11
allfieldsSound	10.1186/s13023-019-1214-2 doi (DE-627)SPR029504007 (SPR)s13023-019-1214-2-e DE-627 ger DE-627 rakwb eng Pacoricona Alfaro, Dibia Liz verfasserin aut Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s). 2019 Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. Prader-Willi syndrome (dpeaa)DE-He213 Epidemiology (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Respiratory complications (dpeaa)DE-He213 Sudden death (dpeaa)DE-He213 Lemoine, Perrine aut Ehlinger, Virginie aut Molinas, Catherine aut Diene, Gwénaëlle aut Valette, Marion aut Pinto, Graziella aut Coupaye, Muriel aut Poitou-Bernert, Christine aut Thuilleaux, Denise aut Arnaud, Catherine aut Tauber, Maithé (orcid)0000-0002-0363-7862 aut Enthalten in Orphanet journal of rare diseases London : BioMed Central, 2006 14(2019), 1 vom: 04. Nov. (DE-627)50900637X (DE-600)2225857-7 1750-1172 nnns volume:14 year:2019 number:1 day:04 month:11 https://dx.doi.org/10.1186/s13023-019-1214-2 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 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_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 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 14 2019 1 04 11
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source	Enthalten in Orphanet journal of rare diseases 14(2019), 1 vom: 04. Nov. volume:14 year:2019 number:1 day:04 month:11
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author	Pacoricona Alfaro, Dibia Liz
spellingShingle	Pacoricona Alfaro, Dibia Liz misc Prader-Willi syndrome misc Epidemiology misc Mortality misc Respiratory complications misc Sudden death Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center
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topic_title	Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center Prader-Willi syndrome (dpeaa)DE-He213 Epidemiology (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Respiratory complications (dpeaa)DE-He213 Sudden death (dpeaa)DE-He213
topic	misc Prader-Willi syndrome misc Epidemiology misc Mortality misc Respiratory complications misc Sudden death
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title_full	Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center
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author_browse	Pacoricona Alfaro, Dibia Liz Lemoine, Perrine Ehlinger, Virginie Molinas, Catherine Diene, Gwénaëlle Valette, Marion Pinto, Graziella Coupaye, Muriel Poitou-Bernert, Christine Thuilleaux, Denise Arnaud, Catherine Tauber, Maithé
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title_sort	causes of death in prader-willi syndrome: lessons from 11 years’ experience of a national reference center
title_auth	Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center
abstract	Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. © The Author(s). 2019
abstractGer	Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. © The Author(s). 2019
abstract_unstemmed	Background In the last 20 years, substantial improvements have been made in the diagnosis, treatment and management of patients with Prader-Willi syndrome (PWS). Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. In those adult patients with data on obesity, 98% were reported to be obese. © The Author(s). 2019
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title_short	Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center
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Few data on causes of death are available since those improvements were made. Our study assessed the causes of death among French patients with PWS over the first 11 years of experience of the nationwide French Reference Center for PWS (FRC-PWS). Methods Our study relied on two sources of mortality information at national level between 2004 and 2014: The French Epidemiological Centre for the Medical Causes of Death (CépiDc) Registry and the FRC-PWS database. Causes of death were classified into seven categories: respiratory, cardiovascular, gastrointestinal, severe infection, sudden death, other causes, and unknown. Descriptive statistics were calculated separately for children (< 18 years-old) and adults (≥18 years-old). Results One hundred and four deaths were identified in France from 2004 to 2014. The median age at death was 30 years, ranging from less than 1 month to 58 years. Seventeen deaths occurred in patients under 18 years, with 70% of them in children under 2 years. Respiratory causes accounted for more than 50% of the deaths in patients with PWS in both children and adults. Both cause and age of death did not significantly differ according to gender or genetic subtype. Conclusions Patients with PWS die prematurely due to a respiratory cause in most cases at all ages. 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Causes of death in Prader-Willi syndrome: lessons from 11 years’ experience of a national reference center

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