Neil R Lawrence, Irina Bacila, Joseph Tonge, Jeremy Dawson, Gary S Collins, Zi-Qiang Lang, Jillian Bryce, Malika Alimussina, Minglu Chen, Salma Rashid Ali, Safwaan Adam, Erica L T van den Akker, Tânia Aparecida Sartori Sanchez Bachega, Federico Baronio, Niels Holtum Birkebæk, Walter Bonfig, Hedi Claahsen van der Grinten, Martine Cools, Eduardo Correa Costa, Miguel Debono, Liat de Vries, Christa E Flück, Gabriella Gazdagh, Ayla Güven, Sabine E Hannema, Violeta Iotova, Hetty J van der Kamp, Ruth Krone, Sofia Leka-Emiri, María Clemente-León, Corina Raducanu Lichiardopol, Renata L Markosyan, Tatjana Milenkovic, Mirela Costa de Miranda, Uta Neumann, John Newell-Price, Şükran Poyrazoğlu, Ursina Probst-Scheidegger, Gianni Russo, Luisa De Sanctis, Sumudu Nimali Seneviratne, Marianna Rita Stancampiano, Rieko Tadokoro-Cuccaro, Ajay Thankamony, Ana Vieites, Malgorzata Wasniewska, Diego Yeste, Jeremy Tomlinson, S Faisal Ahmed, Nils Krone
{"title":"Blood pressure and its associations in 554 children and young people with CAH.","authors":"Neil R Lawrence, Irina Bacila, Joseph Tonge, Jeremy Dawson, Gary S Collins, Zi-Qiang Lang, Jillian Bryce, Malika Alimussina, Minglu Chen, Salma Rashid Ali, Safwaan Adam, Erica L T van den Akker, Tânia Aparecida Sartori Sanchez Bachega, Federico Baronio, Niels Holtum Birkebæk, Walter Bonfig, Hedi Claahsen van der Grinten, Martine Cools, Eduardo Correa Costa, Miguel Debono, Liat de Vries, Christa E Flück, Gabriella Gazdagh, Ayla Güven, Sabine E Hannema, Violeta Iotova, Hetty J van der Kamp, Ruth Krone, Sofia Leka-Emiri, María Clemente-León, Corina Raducanu Lichiardopol, Renata L Markosyan, Tatjana Milenkovic, Mirela Costa de Miranda, Uta Neumann, John Newell-Price, Şükran Poyrazoğlu, Ursina Probst-Scheidegger, Gianni Russo, Luisa De Sanctis, Sumudu Nimali Seneviratne, Marianna Rita Stancampiano, Rieko Tadokoro-Cuccaro, Ajay Thankamony, Ana Vieites, Malgorzata Wasniewska, Diego Yeste, Jeremy Tomlinson, S Faisal Ahmed, Nils Krone","doi":"10.1093/ejendo/lvaf060","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) affects approximately 1 in 15,000 individuals. We leveraged the power of multicentre registry data to assess the trend and predictors of blood pressure (BP) within children and young persons with 21OHD to inform monitoring strategies.</p><p><strong>Method: </strong>Data from the International CAH Registry in patients younger than 20 years was compared to normative values. Values of BP were modelled to create reference curves, multiple change point analysis applied to quantify the difference with normative data. Covariate adjustment was informed by a directed acyclic graph, prior to joint outcome regression modelling to accurately assess predictors of BP.</p><p><strong>Results: </strong>A total of 6436 visits within 554 patients (52.5% females) showed BP-Standard deviation scores (SDS) were higher at younger ages. Patients under five years had systolic BP-SDS of 1.6 (Q1:0.6-Q3:2.7) decreasing to 1.0 (Q1:0.2-Q3:1.8) over five years, equating to 31.0% over the 95th centile decreasing to 15.0%. Higher doses of fludrocortisone were associated with a small increase in systolic BP equivalent to 1.2mmHg with every 100 micrograms extra fludrocortisone. Renin of 100µU/ml was associated with 4.6mmHg lower systolic BP than a renin of 1µU/ml, higher 17OH-progesterone and androstenedione also predicted lower systolic and diastolic BP (p<0.05).</p><p><strong>Conclusion: </strong>Higher BP in children with 21OHD is common and particularly pronounced at a younger age, but may not be attributable to excessive mineralocorticoid replacement. There is a need to improve our understanding of the determinants of this raised BP as well as its long-term effects.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":""},"PeriodicalIF":5.3000,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Endocrinology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/ejendo/lvaf060","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ENDOCRINOLOGY & METABOLISM","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) affects approximately 1 in 15,000 individuals. We leveraged the power of multicentre registry data to assess the trend and predictors of blood pressure (BP) within children and young persons with 21OHD to inform monitoring strategies.
Method: Data from the International CAH Registry in patients younger than 20 years was compared to normative values. Values of BP were modelled to create reference curves, multiple change point analysis applied to quantify the difference with normative data. Covariate adjustment was informed by a directed acyclic graph, prior to joint outcome regression modelling to accurately assess predictors of BP.
Results: A total of 6436 visits within 554 patients (52.5% females) showed BP-Standard deviation scores (SDS) were higher at younger ages. Patients under five years had systolic BP-SDS of 1.6 (Q1:0.6-Q3:2.7) decreasing to 1.0 (Q1:0.2-Q3:1.8) over five years, equating to 31.0% over the 95th centile decreasing to 15.0%. Higher doses of fludrocortisone were associated with a small increase in systolic BP equivalent to 1.2mmHg with every 100 micrograms extra fludrocortisone. Renin of 100µU/ml was associated with 4.6mmHg lower systolic BP than a renin of 1µU/ml, higher 17OH-progesterone and androstenedione also predicted lower systolic and diastolic BP (p<0.05).
Conclusion: Higher BP in children with 21OHD is common and particularly pronounced at a younger age, but may not be attributable to excessive mineralocorticoid replacement. There is a need to improve our understanding of the determinants of this raised BP as well as its long-term effects.
期刊介绍：
European Journal of Endocrinology is the official journal of the European Society of Endocrinology. Its predecessor journal is Acta Endocrinologica.
The journal publishes high-quality original clinical and translational research papers and reviews in paediatric and adult endocrinology, as well as clinical practice guidelines, position statements and debates. Case reports will only be considered if they represent exceptional insights or advances in clinical endocrinology.
Topics covered include, but are not limited to, Adrenal and Steroid, Bone and Mineral Metabolism, Hormones and Cancer, Pituitary and Hypothalamus, Thyroid and Reproduction. In the field of Diabetes, Obesity and Metabolism we welcome manuscripts addressing endocrine mechanisms of disease and its complications, management of obesity/diabetes in the context of other endocrine conditions, or aspects of complex disease management. Reports may encompass natural history studies, mechanistic studies, or clinical trials.
Equal consideration is given to all manuscripts in English from any country.
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