Yoonjeong Lim, Consuelo M Kreider, Mary Alvarez, Roxanna M Bendixen
{"title":"Barth综合征患者的HRQoL:儿童自我报告与家长代理报告的一致性及其与家长HRQoL的关系","authors":"Yoonjeong Lim, Consuelo M Kreider, Mary Alvarez, Roxanna M Bendixen","doi":"10.29245/2690-0009/2019/2.1104","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>Barth syndrome is an X-linked rare disorder that typically affects only males. This study investigates 1) agreement between child self-reports and parent proxy-reports of HRQoL in boys with Barth syndrome and 2) relationship between parental HRQoL and parent proxy-reports of HRQoL for the child.</p><p><strong>Materials and methods: </strong>Twenty-eight boys with Barth syndrome and their parents participated in this study. The PedsQL™ 4.0 and the PedsQL™ Family Impact Module were used to measure HRQoL of the boys, and the parents' HRQoL, respectively. The Intraclass Correlation Coefficient was used to test agreement between the child self-reports and parent proxy-reports of HRQoL. The Spearman correlation coefficient was used to test the relationship between parental HRQoL and parent proxy-reports of HRQoL for the child.</p><p><strong>Results: </strong>The agreement between the child self-reports and the parent proxy-reports showed moderate-to-good agreement. Higher parental HRQoL was significantly related to higher ratings of the parents on their children's HRQoL (<i>p</i> < .05).</p><p><strong>Conclusions: </strong>This study broadens understanding of HRQoL of boys with Barth syndrome using both child self-reports and parent proxy-reports. The findings indicate that the parent proxy-report of HRQoL should be used in conjunction with the child self-report when making client-centered health decisions.</p>","PeriodicalId":87301,"journal":{"name":"Journal of human & clinical genetics","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7971423/pdf/","citationCount":"4","resultStr":"{\"title\":\"HRQoL in Barth Syndrome: Agreement between Child Self-reports and Parent Proxy-reports and Its Relationship to Parental HRQoL.\",\"authors\":\"Yoonjeong Lim, Consuelo M Kreider, Mary Alvarez, Roxanna M Bendixen\",\"doi\":\"10.29245/2690-0009/2019/2.1104\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Purpose: </strong>Barth syndrome is an X-linked rare disorder that typically affects only males. This study investigates 1) agreement between child self-reports and parent proxy-reports of HRQoL in boys with Barth syndrome and 2) relationship between parental HRQoL and parent proxy-reports of HRQoL for the child.</p><p><strong>Materials and methods: </strong>Twenty-eight boys with Barth syndrome and their parents participated in this study. The PedsQL™ 4.0 and the PedsQL™ Family Impact Module were used to measure HRQoL of the boys, and the parents' HRQoL, respectively. The Intraclass Correlation Coefficient was used to test agreement between the child self-reports and parent proxy-reports of HRQoL. The Spearman correlation coefficient was used to test the relationship between parental HRQoL and parent proxy-reports of HRQoL for the child.</p><p><strong>Results: </strong>The agreement between the child self-reports and the parent proxy-reports showed moderate-to-good agreement. Higher parental HRQoL was significantly related to higher ratings of the parents on their children's HRQoL (<i>p</i> < .05).</p><p><strong>Conclusions: </strong>This study broadens understanding of HRQoL of boys with Barth syndrome using both child self-reports and parent proxy-reports. The findings indicate that the parent proxy-report of HRQoL should be used in conjunction with the child self-report when making client-centered health decisions.</p>\",\"PeriodicalId\":87301,\"journal\":{\"name\":\"Journal of human & clinical genetics\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7971423/pdf/\",\"citationCount\":\"4\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of human & clinical genetics\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.29245/2690-0009/2019/2.1104\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2019/6/13 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of human & clinical genetics","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.29245/2690-0009/2019/2.1104","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2019/6/13 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
HRQoL in Barth Syndrome: Agreement between Child Self-reports and Parent Proxy-reports and Its Relationship to Parental HRQoL.
Purpose: Barth syndrome is an X-linked rare disorder that typically affects only males. This study investigates 1) agreement between child self-reports and parent proxy-reports of HRQoL in boys with Barth syndrome and 2) relationship between parental HRQoL and parent proxy-reports of HRQoL for the child.
Materials and methods: Twenty-eight boys with Barth syndrome and their parents participated in this study. The PedsQL™ 4.0 and the PedsQL™ Family Impact Module were used to measure HRQoL of the boys, and the parents' HRQoL, respectively. The Intraclass Correlation Coefficient was used to test agreement between the child self-reports and parent proxy-reports of HRQoL. The Spearman correlation coefficient was used to test the relationship between parental HRQoL and parent proxy-reports of HRQoL for the child.
Results: The agreement between the child self-reports and the parent proxy-reports showed moderate-to-good agreement. Higher parental HRQoL was significantly related to higher ratings of the parents on their children's HRQoL (p < .05).
Conclusions: This study broadens understanding of HRQoL of boys with Barth syndrome using both child self-reports and parent proxy-reports. The findings indicate that the parent proxy-report of HRQoL should be used in conjunction with the child self-report when making client-centered health decisions.