ESPE2022 Poster Category 1 Adrenals and HPA Axis (52 abstracts)
Blood pressure (BP) status in Congenital Adrenal Hyperplasia (CAH) – longitudinal analysis of real world data from the I-CAH registry
Neil R. Lawrence 1 , Irina Bacila 1 , Jeremy Dawson 1 , Salma R. Ali 2,3 , Erica LT van den Akker 4 , Tânia Aparecida Sartori Sanchez Bachega 5 , Federico Baronio 6 , Niels Holtum Birkebæk 7 , Walter Bonfig 8,9 , Hedi Claahsen van der Grinten 10,10 , Martine Cools 11 , Eduardo Correa Costa 12 , Liat de Vries 13 , Christa Flück 14 , Gabriella Gazdagh 15 , Ayla Güven 16 , Sabine Hannema 17,18 , Violeta Iotova 19 , Hetty J van der Kamp 20 , Ruth Krone 21 , Sofia Leka-Emiri 22,23 , María Clemente León 24 , Corina Raducanu Lichiardopol 25 , Renata L. Markosyan 26 , Tatjana Milenkovic 27 , Mirela Costa de Miranda 5 , Uta Neumann 28 , John Newell-Price 29,1 , Şükran Poyrazoğlu 30 , Ursina Probst-Scheidegger 31 , Gianni Russo 32 , Luisa De Sanctis 33,34 , Adam Safwaan 35 , Sumudu Seneviratne 36 , Marianna Rita Stancampiano 37 , Rieko Tadokoro-Cuccaro 38 , Ajay Thankamony 38 , Ana Vieites 39 , Malgorzata Wasniewska 40 , Diego Yeste 24 , Eleni Daniel 29 , Jeremy Tomlinson 41 , S. Faisal Ahmed 2 & Nils Krone 42,43,44
1The University of Sheffield, Sheffield, United Kingdom; 2University of Glasgow, Glasgow, United Kingdom; 3Royal Hospital for Children & Queen Elizabeth University Hospital, Glasgow, United Kingdom; 4Sophia Children’s Hospital, Rotterdam, Netherlands; 5University of Sao Paulo, Sao Paulo, Brazil; 6S.Orsola-Malpighi University Hospital, Bologna, Italy; 7Aarhus University Hospital, Aarhus, Denmark; 8Technical University Munich, Munich, Germany; 9Klinikum Wels-Grieskirchen, Wels, Austria; 10Radboud University Medical Centre, Nijmegen, Netherlands; 11Ghent University Hospital, Ghent, Belgium; 12Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil; 13Schneider's Children Medical Center of Israel, Petah-Tikvah, Israel; 14Medizinische Universitätskinderklink, Bern, Switzerland; 15Southampton General Hospital, Southampton, United Kingdom; 16Baskent University Istanbul Hospital, Istanbul, Turkey; 17University Medical Center Rotterdam, Rotterdam, Netherlands; 18Leiden University Medical Centre, Leiden, Netherlands; 19Medical University of Varna, Varna, Bulgaria; 20University Medical Centre Utrecht, Utrecht, Netherlands; 21Birmingham Women's & Children's Hospital, Birmingham, United Kingdom; 22"P.& A. KYRIAKOU" Children's Hospital, Athens, Greece; 23DIU Paris VI University, Athens, Greece; 24Hospital Universitario Vall d'Hebron, Barcelona, Spain; 25University of Medicine and Pharmacy Craiova, Craiova, Romania; 26Yerevan State Medical University, Yerevan, Armenia; 27Institute for Mother and Child Healthcare of Serbia “Dr Vukan Čupić”, Belgrade, Serbia; 28Charite - Universitätsmedizin, Berlin, Germany; 29Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom; 30Istanbul University, Istanbul, Turkey; 31Kantonsspital Winterthur, Winterthur, Switzerland; 32Scientific Institute San Raffaele, Milan, Italy; 33Regina Margherita Children's Hospital, Torino, Italy; 34, University of Torino, Torino, Italy; 35The Christie Hospital, Manchester, United Kingdom; 36Colombo University, Colombo, Sri Lanka; 37Endocrinologia Pediatrica presso Ospedale San Raffaele, Milano, Italy; 38University of Cambridge, Cambridge, United Kingdom; 39Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina; 40Università degli Studi di Messina, Messina, Italy; 41University of Oxford, Oxford, United Kingdom; 42University of Sheffield, Sheffield, United Kingdom; 43Technische Universität Dresden, Dresden, Germany; 44Sheffield Children's NHS Foundation Trust, Sheffield, United Kingdom
Introduction: Data on BP in children with classic congenital adrenal hyperplasia (CAH) is inconsistent.
Aim: To assess the trend in BP throughout childhood using data from the International CAH registry and compare this to normative data from the American Academy of Paediatrics (AAP).
Method: This retrospective multi-centre study included 37 centres from 20 countries. BP data was analysed by Lambda-Mu-Sigma (LMS) modelling to create smoothing reference curves. We subtracted the AAP median BP for age from the I-CAH registry median BP for age, and conducted Bayesian multiple change point analysis to assess the age at which BP in the two populations converged.
Results: A total of 546 patients (53% girls, 86% prescribed Fludrocortisone) with 6357 visits between 1988-2021 were available for analysis and 3181 visits had BP measurements available. Each centre contributed a median of 12 patients (Interquartile Range (IQR) 5, 23), with data from a median of 6.0 years (IQR 3.2, 12.7) follow up per patient. Median age at visit was 5.4 years (IQR 2.4, 10.6), maximum age at visit was 19.9 years, and median number of visits available per patient was 5 (IQR 2, 9). When compared against AAP normative BP values, the difference in median BP for age decreased with a clear plateau in both sexes. Systolic BP was higher in 1 year old CAH patients by 17.5 mmHg in boys and 13.6mmHg in girls, decreasing to a plateau of 6.2mmHg higher after age 7.7 years in boys and 5.3mmHg after age 13.3 years in girls. Diastolic BP was 22.9mmHg higher in boys and 18.3mmHg higher in girls at the age of 1 year, decreasing to a plateau of 3.2mmHg higher after age 6.7 years in boys and 3.3mmHg higher after age 6.3 years in girls.
Conclusions: Analysis of real world data shows BP in children with classic CAH to be higher than normative values at an early age, but to plateau at age 8 years in males and 13 years in females to a level marginally higher than reference values. The difference was greater in diastolic BP at early ages, but greater in systolic BP after plateau. The impact of higher BP at these young ages on vascular modelling and long term cardiovascular risk in CAH is unknown and warrants further investigation. Further analysis with covariates within this data set is ongoing, seeking to clarify other determinants of BP in CAH.
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