Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study
Background Prader–Willi syndrome (PWS) is a congenital alteration of chromosome pair 15. It is characterized by short stature, muscular hypotonia, hyperphagia, obesity, behavioural and emotional disturbances, hypogonadism and partial Growth Hormone (GH) deficiency. The aim of this study was to asse...
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| Veröffentlicht in: | Journal of intellectual disability research 2007-04, Vol.51 (4), p.302-311 |
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| creator | Bertella, L. Mori, I. Grugni, G. Pignatti, R. Ceriani, F. Molinari, E. Ceccarelli, A. Sartorio, A. Vettor, R. Semenza, C. |
| description | Background Prader–Willi syndrome (PWS) is a congenital alteration of chromosome pair 15. It is characterized by short stature, muscular hypotonia, hyperphagia, obesity, behavioural and emotional disturbances, hypogonadism and partial Growth Hormone (GH) deficiency. The aim of this study was to assess the long‐term effect of GH treatment on the psychological well‐being and Quality of Life (QoL) in an adult PWS group.
Methods A total of 13 PWS patients, their diagnosis confirmed by genetic tests, and their parents were recruited for this study. The participants were administered the 36‐Items Short Form Health Survey (SF‐36) and the Psychological General Well‐Being Index (PGWBI), for the assessment of QoL and psychological well‐being, at the beginning of GH treatment, and at following intervals of 6, 12 and 24 months. Modified versions of the same questionnaires were given to the parents.
Results Significant improvement with respect to the baseline was found, on both scales, in the evaluation of both physical and psychological well‐being, although the parents’ evaluation was less optimistic than that of the patients.
Conclusion Our findings suggest that the amelioration of QoL and psychological status is sustained in patients who continue GH treatment. |
| doi_str_mv | 10.1111/j.1365-2788.2006.00878.x |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_919970603</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><ericid>EJ754593</ericid><sourcerecordid>771801382</sourcerecordid><originalsourceid>FETCH-LOGICAL-c5198-a596e7c67cb54931c606eb68f1849bac441a3bba015e2a136b21608a161780163</originalsourceid><addsrcrecordid>eNqNkUtv1DAURi0EotPCP0DIG-iGBN84fiE2qCrTDuX9qMTGchxn8JBJhjhRJ_8ehxlNd4A3tnTPd32vDkIYSArxPF-lQDlLMiFlmhHCU0KkkOn2DpodCnfRjCieJxmn9Agdh7AikYSc30dHIGjGJcAMlR8HU_t-xG2Fa185bJoSb8Jof7R1u_TW1PjG1XVSON8ssW_w_CLpO2d6Vz7DphzqHn-4_ow3pveu6cMLbHDdNkvfD6VvYjjEx_gA3atMHdzD_X2Cvr4-_3J2kVy9n1-evbpKLAMlE8MUd8JyYQuWKwqWE-4KLiuQuSqMzXMwtCgMAeYyExctMuBEGuAgJAFOT9Dpru-ma38NLvR67YON45vGtUPQCpQShBP6T1IIiB2pzCL59O8kyaIBmEC5A23XhtC5Sm86vzbdqIHoyZpe6UmOnuToyZr-Y01vY_Tx_o-hWLvyNrjXFIEne8CEqKTqTGN9uOUkE1IBi9yjHec6bw_l84VgOVPT1i935Rtfu_G_59OLy0_xEePJLu5D77aHuOl-ai6oYPr63VzDQr3NxPdv-g39DStIyP4</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>70220012</pqid></control><display><type>article</type><title>Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study</title><source>Applied Social Sciences Index & Abstracts (ASSIA)</source><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><source>EBSCOhost Education Source</source><creator>Bertella, L. ; Mori, I. ; Grugni, G. ; Pignatti, R. ; Ceriani, F. ; Molinari, E. ; Ceccarelli, A. ; Sartorio, A. ; Vettor, R. ; Semenza, C.</creator><creatorcontrib>Bertella, L. ; Mori, I. ; Grugni, G. ; Pignatti, R. ; Ceriani, F. ; Molinari, E. ; Ceccarelli, A. ; Sartorio, A. ; Vettor, R. ; Semenza, C.</creatorcontrib><description>Background Prader–Willi syndrome (PWS) is a congenital alteration of chromosome pair 15. It is characterized by short stature, muscular hypotonia, hyperphagia, obesity, behavioural and emotional disturbances, hypogonadism and partial Growth Hormone (GH) deficiency. The aim of this study was to assess the long‐term effect of GH treatment on the psychological well‐being and Quality of Life (QoL) in an adult PWS group.
Methods A total of 13 PWS patients, their diagnosis confirmed by genetic tests, and their parents were recruited for this study. The participants were administered the 36‐Items Short Form Health Survey (SF‐36) and the Psychological General Well‐Being Index (PGWBI), for the assessment of QoL and psychological well‐being, at the beginning of GH treatment, and at following intervals of 6, 12 and 24 months. Modified versions of the same questionnaires were given to the parents.
Results Significant improvement with respect to the baseline was found, on both scales, in the evaluation of both physical and psychological well‐being, although the parents’ evaluation was less optimistic than that of the patients.
Conclusion Our findings suggest that the amelioration of QoL and psychological status is sustained in patients who continue GH treatment.</description><identifier>ISSN: 0964-2633</identifier><identifier>EISSN: 1365-2788</identifier><identifier>DOI: 10.1111/j.1365-2788.2006.00878.x</identifier><identifier>PMID: 17326811</identifier><identifier>CODEN: JIDREN</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adaptation, Psychological - drug effects ; Adult ; Adults ; Behavior Problems ; Biological and medical sciences ; Body Composition ; Chromosomes ; Congenital Impairments ; Emotional Disturbances ; Female ; Genetics ; Growth hormones ; growth-hormone therapy ; Hormones. Endocrine system ; Human Growth Hormone - therapeutic use ; Humans ; Intervals ; Longitudinal Studies ; Male ; Medical sciences ; Mental Retardation ; Metabolism ; Obesity ; Outcomes of Treatment ; Parent Attitudes ; Parents ; Patients ; Pharmacology. Drug treatments ; Physical Health ; Prader-Willi syndrome ; Prader-Willi Syndrome - drug therapy ; Prader-Willi Syndrome - psychology ; Psychiatric Status Rating Scales - statistics & numerical data ; Psychological Patterns ; psychological well-being ; Psychological wellbeing ; Psychology ; Quality of Life ; Quality of Life - psychology ; Questionnaires ; Short forms ; Surveys and Questionnaires ; Time Factors ; Treatment Outcome ; Well Being</subject><ispartof>Journal of intellectual disability research, 2007-04, Vol.51 (4), p.302-311</ispartof><rights>2007 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5198-a596e7c67cb54931c606eb68f1849bac441a3bba015e2a136b21608a161780163</citedby><cites>FETCH-LOGICAL-c5198-a596e7c67cb54931c606eb68f1849bac441a3bba015e2a136b21608a161780163</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1365-2788.2006.00878.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1365-2788.2006.00878.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,30977,45550,45551</link.rule.ids><backlink>$$Uhttp://eric.ed.gov/ERICWebPortal/detail?accno=EJ754593$$DView record in ERIC$$Hfree_for_read</backlink><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=18578915$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17326811$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bertella, L.</creatorcontrib><creatorcontrib>Mori, I.</creatorcontrib><creatorcontrib>Grugni, G.</creatorcontrib><creatorcontrib>Pignatti, R.</creatorcontrib><creatorcontrib>Ceriani, F.</creatorcontrib><creatorcontrib>Molinari, E.</creatorcontrib><creatorcontrib>Ceccarelli, A.</creatorcontrib><creatorcontrib>Sartorio, A.</creatorcontrib><creatorcontrib>Vettor, R.</creatorcontrib><creatorcontrib>Semenza, C.</creatorcontrib><title>Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study</title><title>Journal of intellectual disability research</title><addtitle>J Intellect Disabil Res</addtitle><description>Background Prader–Willi syndrome (PWS) is a congenital alteration of chromosome pair 15. It is characterized by short stature, muscular hypotonia, hyperphagia, obesity, behavioural and emotional disturbances, hypogonadism and partial Growth Hormone (GH) deficiency. The aim of this study was to assess the long‐term effect of GH treatment on the psychological well‐being and Quality of Life (QoL) in an adult PWS group.
Methods A total of 13 PWS patients, their diagnosis confirmed by genetic tests, and their parents were recruited for this study. The participants were administered the 36‐Items Short Form Health Survey (SF‐36) and the Psychological General Well‐Being Index (PGWBI), for the assessment of QoL and psychological well‐being, at the beginning of GH treatment, and at following intervals of 6, 12 and 24 months. Modified versions of the same questionnaires were given to the parents.
Results Significant improvement with respect to the baseline was found, on both scales, in the evaluation of both physical and psychological well‐being, although the parents’ evaluation was less optimistic than that of the patients.
Conclusion Our findings suggest that the amelioration of QoL and psychological status is sustained in patients who continue GH treatment.</description><subject>Adaptation, Psychological - drug effects</subject><subject>Adult</subject><subject>Adults</subject><subject>Behavior Problems</subject><subject>Biological and medical sciences</subject><subject>Body Composition</subject><subject>Chromosomes</subject><subject>Congenital Impairments</subject><subject>Emotional Disturbances</subject><subject>Female</subject><subject>Genetics</subject><subject>Growth hormones</subject><subject>growth-hormone therapy</subject><subject>Hormones. Endocrine system</subject><subject>Human Growth Hormone - therapeutic use</subject><subject>Humans</subject><subject>Intervals</subject><subject>Longitudinal Studies</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Mental Retardation</subject><subject>Metabolism</subject><subject>Obesity</subject><subject>Outcomes of Treatment</subject><subject>Parent Attitudes</subject><subject>Parents</subject><subject>Patients</subject><subject>Pharmacology. Drug treatments</subject><subject>Physical Health</subject><subject>Prader-Willi syndrome</subject><subject>Prader-Willi Syndrome - drug therapy</subject><subject>Prader-Willi Syndrome - psychology</subject><subject>Psychiatric Status Rating Scales - statistics & numerical data</subject><subject>Psychological Patterns</subject><subject>psychological well-being</subject><subject>Psychological wellbeing</subject><subject>Psychology</subject><subject>Quality of Life</subject><subject>Quality of Life - psychology</subject><subject>Questionnaires</subject><subject>Short forms</subject><subject>Surveys and Questionnaires</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><subject>Well Being</subject><issn>0964-2633</issn><issn>1365-2788</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>7QJ</sourceid><recordid>eNqNkUtv1DAURi0EotPCP0DIG-iGBN84fiE2qCrTDuX9qMTGchxn8JBJhjhRJ_8ehxlNd4A3tnTPd32vDkIYSArxPF-lQDlLMiFlmhHCU0KkkOn2DpodCnfRjCieJxmn9Agdh7AikYSc30dHIGjGJcAMlR8HU_t-xG2Fa185bJoSb8Jof7R1u_TW1PjG1XVSON8ssW_w_CLpO2d6Vz7DphzqHn-4_ow3pveu6cMLbHDdNkvfD6VvYjjEx_gA3atMHdzD_X2Cvr4-_3J2kVy9n1-evbpKLAMlE8MUd8JyYQuWKwqWE-4KLiuQuSqMzXMwtCgMAeYyExctMuBEGuAgJAFOT9Dpru-ma38NLvR67YON45vGtUPQCpQShBP6T1IIiB2pzCL59O8kyaIBmEC5A23XhtC5Sm86vzbdqIHoyZpe6UmOnuToyZr-Y01vY_Tx_o-hWLvyNrjXFIEne8CEqKTqTGN9uOUkE1IBi9yjHec6bw_l84VgOVPT1i935Rtfu_G_59OLy0_xEePJLu5D77aHuOl-ai6oYPr63VzDQr3NxPdv-g39DStIyP4</recordid><startdate>200704</startdate><enddate>200704</enddate><creator>Bertella, L.</creator><creator>Mori, I.</creator><creator>Grugni, G.</creator><creator>Pignatti, R.</creator><creator>Ceriani, F.</creator><creator>Molinari, E.</creator><creator>Ceccarelli, A.</creator><creator>Sartorio, A.</creator><creator>Vettor, R.</creator><creator>Semenza, C.</creator><general>Blackwell Publishing Ltd</general><general>Blackwell Publishing</general><general>Blackwell</general><scope>BSCLL</scope><scope>7SW</scope><scope>BJH</scope><scope>BNH</scope><scope>BNI</scope><scope>BNJ</scope><scope>BNO</scope><scope>ERI</scope><scope>PET</scope><scope>REK</scope><scope>WWN</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7QJ</scope></search><sort><creationdate>200704</creationdate><title>Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study</title><author>Bertella, L. ; Mori, I. ; Grugni, G. ; Pignatti, R. ; Ceriani, F. ; Molinari, E. ; Ceccarelli, A. ; Sartorio, A. ; Vettor, R. ; Semenza, C.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5198-a596e7c67cb54931c606eb68f1849bac441a3bba015e2a136b21608a161780163</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Adaptation, Psychological - drug effects</topic><topic>Adult</topic><topic>Adults</topic><topic>Behavior Problems</topic><topic>Biological and medical sciences</topic><topic>Body Composition</topic><topic>Chromosomes</topic><topic>Congenital Impairments</topic><topic>Emotional Disturbances</topic><topic>Female</topic><topic>Genetics</topic><topic>Growth hormones</topic><topic>growth-hormone therapy</topic><topic>Hormones. Endocrine system</topic><topic>Human Growth Hormone - therapeutic use</topic><topic>Humans</topic><topic>Intervals</topic><topic>Longitudinal Studies</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Mental Retardation</topic><topic>Metabolism</topic><topic>Obesity</topic><topic>Outcomes of Treatment</topic><topic>Parent Attitudes</topic><topic>Parents</topic><topic>Patients</topic><topic>Pharmacology. Drug treatments</topic><topic>Physical Health</topic><topic>Prader-Willi syndrome</topic><topic>Prader-Willi Syndrome - drug therapy</topic><topic>Prader-Willi Syndrome - psychology</topic><topic>Psychiatric Status Rating Scales - statistics & numerical data</topic><topic>Psychological Patterns</topic><topic>psychological well-being</topic><topic>Psychological wellbeing</topic><topic>Psychology</topic><topic>Quality of Life</topic><topic>Quality of Life - psychology</topic><topic>Questionnaires</topic><topic>Short forms</topic><topic>Surveys and Questionnaires</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><topic>Well Being</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bertella, L.</creatorcontrib><creatorcontrib>Mori, I.</creatorcontrib><creatorcontrib>Grugni, G.</creatorcontrib><creatorcontrib>Pignatti, R.</creatorcontrib><creatorcontrib>Ceriani, F.</creatorcontrib><creatorcontrib>Molinari, E.</creatorcontrib><creatorcontrib>Ceccarelli, A.</creatorcontrib><creatorcontrib>Sartorio, A.</creatorcontrib><creatorcontrib>Vettor, R.</creatorcontrib><creatorcontrib>Semenza, C.</creatorcontrib><collection>Istex</collection><collection>ERIC</collection><collection>ERIC (Ovid)</collection><collection>ERIC</collection><collection>ERIC</collection><collection>ERIC (Legacy Platform)</collection><collection>ERIC( SilverPlatter )</collection><collection>ERIC</collection><collection>ERIC PlusText (Legacy Platform)</collection><collection>Education Resources Information Center (ERIC)</collection><collection>ERIC</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Applied Social Sciences Index & Abstracts (ASSIA)</collection><jtitle>Journal of intellectual disability research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bertella, L.</au><au>Mori, I.</au><au>Grugni, G.</au><au>Pignatti, R.</au><au>Ceriani, F.</au><au>Molinari, E.</au><au>Ceccarelli, A.</au><au>Sartorio, A.</au><au>Vettor, R.</au><au>Semenza, C.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><ericid>EJ754593</ericid><atitle>Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study</atitle><jtitle>Journal of intellectual disability research</jtitle><addtitle>J Intellect Disabil Res</addtitle><date>2007-04</date><risdate>2007</risdate><volume>51</volume><issue>4</issue><spage>302</spage><epage>311</epage><pages>302-311</pages><issn>0964-2633</issn><eissn>1365-2788</eissn><coden>JIDREN</coden><abstract>Background Prader–Willi syndrome (PWS) is a congenital alteration of chromosome pair 15. It is characterized by short stature, muscular hypotonia, hyperphagia, obesity, behavioural and emotional disturbances, hypogonadism and partial Growth Hormone (GH) deficiency. The aim of this study was to assess the long‐term effect of GH treatment on the psychological well‐being and Quality of Life (QoL) in an adult PWS group.
Methods A total of 13 PWS patients, their diagnosis confirmed by genetic tests, and their parents were recruited for this study. The participants were administered the 36‐Items Short Form Health Survey (SF‐36) and the Psychological General Well‐Being Index (PGWBI), for the assessment of QoL and psychological well‐being, at the beginning of GH treatment, and at following intervals of 6, 12 and 24 months. Modified versions of the same questionnaires were given to the parents.
Results Significant improvement with respect to the baseline was found, on both scales, in the evaluation of both physical and psychological well‐being, although the parents’ evaluation was less optimistic than that of the patients.
Conclusion Our findings suggest that the amelioration of QoL and psychological status is sustained in patients who continue GH treatment.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>17326811</pmid><doi>10.1111/j.1365-2788.2006.00878.x</doi><tpages>10</tpages></addata></record> |
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| subjects | Adaptation, Psychological - drug effects Adult Adults Behavior Problems Biological and medical sciences Body Composition Chromosomes Congenital Impairments Emotional Disturbances Female Genetics Growth hormones growth-hormone therapy Hormones. Endocrine system Human Growth Hormone - therapeutic use Humans Intervals Longitudinal Studies Male Medical sciences Mental Retardation Metabolism Obesity Outcomes of Treatment Parent Attitudes Parents Patients Pharmacology. Drug treatments Physical Health Prader-Willi syndrome Prader-Willi Syndrome - drug therapy Prader-Willi Syndrome - psychology Psychiatric Status Rating Scales - statistics & numerical data Psychological Patterns psychological well-being Psychological wellbeing Psychology Quality of Life Quality of Life - psychology Questionnaires Short forms Surveys and Questionnaires Time Factors Treatment Outcome Well Being |
| title | Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study |
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