Health status of children and young persons with congenital adrenal hyperplasia in the UK (CAH-UK): a cross-sectional multi-centre study
Objective There is limited knowledge on the onset of comorbidities in congenital adrenal hyperplasia (CAH) during childhood. We aimed to establish the health status of children with CAH in the UK. Design and methods This cross-sectional multicentre study involved 14 tertiary endocrine UK units, recr...
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| Veröffentlicht in: | European journal of endocrinology 2022-10, Vol.187 (4), p.543-553 |
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| creator | Bacila, Irina Lawrence, Neil Richard Mahdi, Sundus Alvi, Sabah Cheetham, Timothy D Crowne, Elizabeth Das, Urmi Dattani, Mehul Tulsidas Davies, Justin H Gevers, Evelien Krone, Ruth E Kyriakou, Andreas Patel, Leena Randell, Tabitha Ryan, Fiona J Keevil, Brian Ahmed, S Faisal Krone, Nils P |
| description | Objective There is limited knowledge on the onset of comorbidities in congenital adrenal hyperplasia (CAH) during childhood. We aimed to establish the health status of children with CAH in the UK. Design and methods This cross-sectional multicentre study involved 14 tertiary endocrine UK units, recruiting 101 patients aged 8–18 years with classic 21-hydroxylase deficiency and 83 controls. We analysed demographic, clinical and metabolic data, as well as psychological questionnaires (Strengths and Difficulties (SDQ), Paediatric Quality of Life (PedsQL)). Results Patient height SDS in relation to mid-parental height decreased with age, indicating the discrepancy between height achieved and genetic potential height. Bone age was advanced in 40.5% patients, with a mean difference from the chronological age of 1.8 (±2.3) years. Patients were more frequently overweight (27%) or obese (22%) compared to controls (10.8% and 10.8%, respectively, P |
| doi_str_mv | 10.1530/EJE-21-1109 |
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We aimed to establish the health status of children with CAH in the UK. Design and methods This cross-sectional multicentre study involved 14 tertiary endocrine UK units, recruiting 101 patients aged 8–18 years with classic 21-hydroxylase deficiency and 83 controls. We analysed demographic, clinical and metabolic data, as well as psychological questionnaires (Strengths and Difficulties (SDQ), Paediatric Quality of Life (PedsQL)). Results Patient height SDS in relation to mid-parental height decreased with age, indicating the discrepancy between height achieved and genetic potential height. Bone age was advanced in 40.5% patients, with a mean difference from the chronological age of 1.8 (±2.3) years. Patients were more frequently overweight (27%) or obese (22%) compared to controls (10.8% and 10.8%, respectively, P < 0.001). No consistent relationship between glucocorticoid dose and anthropometric measurements or hormonal biomarkers was detected. A small number of patients had raised total cholesterol (3.0%), low HDL (3.0%), raised LDL (7.0%) and triglycerides (5.0%). SDQ scores were within the ‘high’ and ‘very high’ categories of concern for 16.3% of patients. ‘School functioning’ was the lowest PedsQL scoring dimension with a median (interquartile range) of 70 (55–80), followed by ‘emotional functioning’ with a median of 75 (65–85). Conclusions Our results show an increased prevalence of problems with growth and weight gain in CAH children and suggest reduced quality of life. This highlights the urgent need to optimise management and monitoring strategies to improve long-term health outcomes.</description><identifier>ISSN: 0804-4643</identifier><identifier>EISSN: 1479-683X</identifier><identifier>DOI: 10.1530/EJE-21-1109</identifier><identifier>PMID: 36001026</identifier><language>eng</language><publisher>Bristol: Bioscientifica Ltd</publisher><subject>Original ; Original Research</subject><ispartof>European journal of endocrinology, 2022-10, Vol.187 (4), p.543-553</ispartof><rights>The authors</rights><rights>The authors 2022 The authors</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b400t-11141dc2b1a407a5261ff394928160e3f7029aa67c81a187ac238b16bfbfda1b3</citedby><cites>FETCH-LOGICAL-b400t-11141dc2b1a407a5261ff394928160e3f7029aa67c81a187ac238b16bfbfda1b3</cites><orcidid>0000-0003-0689-5549 ; 0000-0002-3402-4727</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,776,780,881,27903,27904</link.rule.ids></links><search><creatorcontrib>Bacila, Irina</creatorcontrib><creatorcontrib>Lawrence, Neil Richard</creatorcontrib><creatorcontrib>Mahdi, Sundus</creatorcontrib><creatorcontrib>Alvi, Sabah</creatorcontrib><creatorcontrib>Cheetham, Timothy D</creatorcontrib><creatorcontrib>Crowne, Elizabeth</creatorcontrib><creatorcontrib>Das, Urmi</creatorcontrib><creatorcontrib>Dattani, Mehul Tulsidas</creatorcontrib><creatorcontrib>Davies, Justin H</creatorcontrib><creatorcontrib>Gevers, Evelien</creatorcontrib><creatorcontrib>Krone, Ruth E</creatorcontrib><creatorcontrib>Kyriakou, Andreas</creatorcontrib><creatorcontrib>Patel, Leena</creatorcontrib><creatorcontrib>Randell, Tabitha</creatorcontrib><creatorcontrib>Ryan, Fiona J</creatorcontrib><creatorcontrib>Keevil, Brian</creatorcontrib><creatorcontrib>Ahmed, S Faisal</creatorcontrib><creatorcontrib>Krone, Nils P</creatorcontrib><title>Health status of children and young persons with congenital adrenal hyperplasia in the UK (CAH-UK): a cross-sectional multi-centre study</title><title>European journal of endocrinology</title><description>Objective There is limited knowledge on the onset of comorbidities in congenital adrenal hyperplasia (CAH) during childhood. We aimed to establish the health status of children with CAH in the UK. Design and methods This cross-sectional multicentre study involved 14 tertiary endocrine UK units, recruiting 101 patients aged 8–18 years with classic 21-hydroxylase deficiency and 83 controls. We analysed demographic, clinical and metabolic data, as well as psychological questionnaires (Strengths and Difficulties (SDQ), Paediatric Quality of Life (PedsQL)). Results Patient height SDS in relation to mid-parental height decreased with age, indicating the discrepancy between height achieved and genetic potential height. Bone age was advanced in 40.5% patients, with a mean difference from the chronological age of 1.8 (±2.3) years. Patients were more frequently overweight (27%) or obese (22%) compared to controls (10.8% and 10.8%, respectively, P < 0.001). No consistent relationship between glucocorticoid dose and anthropometric measurements or hormonal biomarkers was detected. A small number of patients had raised total cholesterol (3.0%), low HDL (3.0%), raised LDL (7.0%) and triglycerides (5.0%). SDQ scores were within the ‘high’ and ‘very high’ categories of concern for 16.3% of patients. ‘School functioning’ was the lowest PedsQL scoring dimension with a median (interquartile range) of 70 (55–80), followed by ‘emotional functioning’ with a median of 75 (65–85). Conclusions Our results show an increased prevalence of problems with growth and weight gain in CAH children and suggest reduced quality of life. This highlights the urgent need to optimise management and monitoring strategies to improve long-term health outcomes.</description><subject>Original</subject><subject>Original Research</subject><issn>0804-4643</issn><issn>1479-683X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><recordid>eNp9kcFuEzEQhi0EomnhxAv4WIQMnvXGu8sBqYpSAq3EhUjcrFmvnTVy7LD2gvIGPDZOUyFx4TQj-Zv_n_FPyCvgb2Ep-Lv15zWrgAHw7glZQN10TLbi21Oy4C2vWS1rcUEuU_rOOZSePycXQpaeV3JBfm8M-jzSlDHPiUZL9ej8MJlAMQz0GOewowczpRgS_eUKqWPYmeAyeoonrtTxWIiDx-SQukDzaOj2jl6vbjZse_f6PUWqp5gSS0ZnF08T-9lnx7QJeTLFex6OL8gziz6Zl4_1imxv119XG3b_5eOn1c0962vOc7kSahh01QPWvMFlJcFa0dVd1YLkRtiGVx2ibHQLCG2DuhJtD7K3vR0QenFFPpx1D3O_N8PDCujVYXJ7nI4qolP_vgQ3ql38qbolCCm6InD9KDDFH7NJWe1d0sZ7DCbOSVUNl9CW3-UFfXNGH86fjP1rA1ydslMlO1WBOmVXaDjTvYtJu2LvrNP435k_8Facog</recordid><startdate>20221001</startdate><enddate>20221001</enddate><creator>Bacila, Irina</creator><creator>Lawrence, Neil Richard</creator><creator>Mahdi, Sundus</creator><creator>Alvi, Sabah</creator><creator>Cheetham, Timothy D</creator><creator>Crowne, Elizabeth</creator><creator>Das, Urmi</creator><creator>Dattani, Mehul Tulsidas</creator><creator>Davies, Justin H</creator><creator>Gevers, Evelien</creator><creator>Krone, Ruth E</creator><creator>Kyriakou, Andreas</creator><creator>Patel, Leena</creator><creator>Randell, Tabitha</creator><creator>Ryan, Fiona J</creator><creator>Keevil, Brian</creator><creator>Ahmed, S Faisal</creator><creator>Krone, Nils P</creator><general>Bioscientifica Ltd</general><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0003-0689-5549</orcidid><orcidid>https://orcid.org/0000-0002-3402-4727</orcidid></search><sort><creationdate>20221001</creationdate><title>Health status of children and young persons with congenital adrenal hyperplasia in the UK (CAH-UK): a cross-sectional multi-centre study</title><author>Bacila, Irina ; Lawrence, Neil Richard ; Mahdi, Sundus ; Alvi, Sabah ; Cheetham, Timothy D ; Crowne, Elizabeth ; Das, Urmi ; Dattani, Mehul Tulsidas ; Davies, Justin H ; Gevers, Evelien ; Krone, Ruth E ; Kyriakou, Andreas ; Patel, Leena ; Randell, Tabitha ; Ryan, Fiona J ; Keevil, Brian ; Ahmed, S Faisal ; Krone, Nils P</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b400t-11141dc2b1a407a5261ff394928160e3f7029aa67c81a187ac238b16bfbfda1b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Original</topic><topic>Original Research</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bacila, Irina</creatorcontrib><creatorcontrib>Lawrence, Neil Richard</creatorcontrib><creatorcontrib>Mahdi, Sundus</creatorcontrib><creatorcontrib>Alvi, Sabah</creatorcontrib><creatorcontrib>Cheetham, Timothy D</creatorcontrib><creatorcontrib>Crowne, Elizabeth</creatorcontrib><creatorcontrib>Das, Urmi</creatorcontrib><creatorcontrib>Dattani, Mehul Tulsidas</creatorcontrib><creatorcontrib>Davies, Justin H</creatorcontrib><creatorcontrib>Gevers, Evelien</creatorcontrib><creatorcontrib>Krone, Ruth E</creatorcontrib><creatorcontrib>Kyriakou, Andreas</creatorcontrib><creatorcontrib>Patel, Leena</creatorcontrib><creatorcontrib>Randell, Tabitha</creatorcontrib><creatorcontrib>Ryan, Fiona J</creatorcontrib><creatorcontrib>Keevil, Brian</creatorcontrib><creatorcontrib>Ahmed, S Faisal</creatorcontrib><creatorcontrib>Krone, Nils P</creatorcontrib><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>European journal of endocrinology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bacila, Irina</au><au>Lawrence, Neil Richard</au><au>Mahdi, Sundus</au><au>Alvi, Sabah</au><au>Cheetham, Timothy D</au><au>Crowne, Elizabeth</au><au>Das, Urmi</au><au>Dattani, Mehul Tulsidas</au><au>Davies, Justin H</au><au>Gevers, Evelien</au><au>Krone, Ruth E</au><au>Kyriakou, Andreas</au><au>Patel, Leena</au><au>Randell, Tabitha</au><au>Ryan, Fiona J</au><au>Keevil, Brian</au><au>Ahmed, S Faisal</au><au>Krone, Nils P</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Health status of children and young persons with congenital adrenal hyperplasia in the UK (CAH-UK): a cross-sectional multi-centre study</atitle><jtitle>European journal of endocrinology</jtitle><date>2022-10-01</date><risdate>2022</risdate><volume>187</volume><issue>4</issue><spage>543</spage><epage>553</epage><pages>543-553</pages><issn>0804-4643</issn><eissn>1479-683X</eissn><abstract>Objective There is limited knowledge on the onset of comorbidities in congenital adrenal hyperplasia (CAH) during childhood. We aimed to establish the health status of children with CAH in the UK. Design and methods This cross-sectional multicentre study involved 14 tertiary endocrine UK units, recruiting 101 patients aged 8–18 years with classic 21-hydroxylase deficiency and 83 controls. We analysed demographic, clinical and metabolic data, as well as psychological questionnaires (Strengths and Difficulties (SDQ), Paediatric Quality of Life (PedsQL)). Results Patient height SDS in relation to mid-parental height decreased with age, indicating the discrepancy between height achieved and genetic potential height. Bone age was advanced in 40.5% patients, with a mean difference from the chronological age of 1.8 (±2.3) years. Patients were more frequently overweight (27%) or obese (22%) compared to controls (10.8% and 10.8%, respectively, P < 0.001). No consistent relationship between glucocorticoid dose and anthropometric measurements or hormonal biomarkers was detected. A small number of patients had raised total cholesterol (3.0%), low HDL (3.0%), raised LDL (7.0%) and triglycerides (5.0%). SDQ scores were within the ‘high’ and ‘very high’ categories of concern for 16.3% of patients. ‘School functioning’ was the lowest PedsQL scoring dimension with a median (interquartile range) of 70 (55–80), followed by ‘emotional functioning’ with a median of 75 (65–85). Conclusions Our results show an increased prevalence of problems with growth and weight gain in CAH children and suggest reduced quality of life. This highlights the urgent need to optimise management and monitoring strategies to improve long-term health outcomes.</abstract><cop>Bristol</cop><pub>Bioscientifica Ltd</pub><pmid>36001026</pmid><doi>10.1530/EJE-21-1109</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0003-0689-5549</orcidid><orcidid>https://orcid.org/0000-0002-3402-4727</orcidid><oa>free_for_read</oa></addata></record> |
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| source | Oxford University Press Journals All Titles (1996-Current) |
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| title | Health status of children and young persons with congenital adrenal hyperplasia in the UK (CAH-UK): a cross-sectional multi-centre study |
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