ESPE2024 Poster Category 1 Adrenals and HPA Axis 1 (9 abstracts)
Variation in Blood Pressure in Young Adults with 21a-Hydroxylase Deficiency: Longitudinal Multilevel Modelling of Data from the I-CAH Registry
Neil R. Lawrence 1 , Irina Bacila 1 , Joseph Tonge 1 , Jeremy Dawson 1 , Gary S. Collins 2 , Zi-Qiang Lang 1 , Jillian Bryce 3 , Malika Alimussina 3 , Minglu Chen 3 , Salma R. Ali 4,3 , Safwaan Adam 5 , Erica L.T. van den Akker 6 , Tânia A.S.S. Bachega 7 , Federico Baronio 8 , Walter Bonfig 9,10 , Hedi Claahsen-van der Grinten 11 , Martine Cools 12 , Eduardo C. Costa 13 , Miguel Debono 14 , Liat de Vries 15,16 , Christa Flück 17 , Gabriella Gazdagh 18 , Ayla Güven 19 , Sabine Hannema 20,21 , Violeta Iotova 22 , Hetty J van der Kamp 23 , Ruth Krone 24 , Sofia Leka-Emiri 25 , María Clemente-León 26 , Corina R. Lichiardopol 27 , Renata L. Markosyan 28 , Tatjana Milenkovic 29 , Mirela C. Miranda 7 , Uta Neumann 30 , John Newell-Price 1 , Şükran Poyrazoğlu 31 , Ursina Probst-Scheidegger 32 , Gianni Russo 33 , Luisa De Sanctis 34,35 , Sumudu N. Seneviratne 36 , Marianna R. Stancampiano 33 , Rieko Tadokoro-Cuccaro 37 , Ajay Thankamony 37 , Ana Vieites 38 , Malgorzata Wasniewska 39 , Diego Yeste 40 , Jeremy Tomlinson 2 , S. Faisal Ahmed 4 & Nils Krone 1,41
1University of Sheffield, Sheffield, United Kingdom. 2University of Oxford, Oxford, United Kingdom. 3Royal Hospital for Children & Queen Elizabeth University Hospital, Glasgow, United Kingdom. 4University of Glasgow, Glasgow, United Kingdom. 5The Christie Hospital, Manchester, United Kingdom. 6Sophia Children’s Hospital, Rotterdam, Netherlands. 7University of Sao Paulo, Sao Paulo, Brazil. 8IRCCS AOUBO, Bologna, Italy. 9Technical University Munich, Munich, Germany. 10Klinikum Wels-Grieskirchen, Wels, Austria. 11Radboud University Medical Centre, Nijmegen, Netherlands. 12Ghent University and Ghent University Hospital, Ghent, Belgium. 13Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. 14Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom. 15Schneider's Children Medical Center of Israel, Petah-Tikvah, Israel. 16Tel-Aviv University, Tel-Aviv, Israel. 17Medizinische Universitätskinderklink, Bern, Switzerland. 18Southampton General Hospital, Southampton, United Kingdom. 19Baskent University Istanbul Hospital, Istanbul, Turkey. 20Sophia Children's Hospital, Rotterdam, Netherlands. 21Leiden University Medical Centre, Leiden, Netherlands. 22Medical University of Varna, Varna, Bulgaria. 23University Medical Centre Utrecht, Utrecht, Netherlands. 24Birmingham Women's & Children's Hospital, Birmingham, United Kingdom. 25"P.& A. KYRIAKOU" Children's Hospital, Athens, Greece. 26University Hospital Vall d'Hebron, Barcelona, Spain. 27University of Medicine and Pharmacy Craiova, Craiova, Romania. 28Yerevan State Medical University, Yerevan, Armenia. 29Institute for Mother and Child Healthcare of Serbia “Dr Vukan Čupić”, Belgrade, Serbia. 30Charite - Universitätsmedizin, Berlin, Germany. 31Istanbul University, Istanbul, Turkey. 32Kantonsspital Winterthur, Winterthur, Switzerland. 33Scientific Institute San Raffaele, Milan, Italy. 34Regina Margherita Children's Hospital, Torino, Italy. 35University of Torino, Torino, Italy. 36University of Colombo, Colombo, Sri Lanka. 37University of Cambridge, Cambridge, United Kingdom. 38Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina. 39University of Messina, Messina, Italy. 40Hospital Universitario Vall d'Hebron, Barcelona, Spain. 41Sheffield Children's Hospital NHS Foundation Trust, Sheffield, United Kingdom
Background: Alterations in blood pressure in treated CAH due to 21 hydroxylase deficiency is contentious, with studies reporting different effects. We used data from young adults entered into the I-CAH registry to assess the change in blood pressure readings recorded within patients over time.
Methods: We used longitudinal mixed effects modelling (LMEM) in R to account for repeated measures in two levels within patients attending different centres. Multilevel quantile regression was used to assess variability as patients aged. Sex, weight, total daily hydrocortisone equivalent and fludrocortisone were incorporated into the models as covariates. Missing data was addressed using interpolation and multilevel imputation, with complete case analysis applied as a sensitivity analysis.
Results: A total of 109 patients (44 male (median age 27 years (Q1-Q3: 23-32) and weight 76 kg (63-88); 65 female (median age 28 years (24-32) and weight 65 kg (52-72)) with 425 clinic visits from 10 centres between 20-40 years of age were available for modelling. Total daily hydrocortisone equivalent was median 20 mg (20-30), mineralocorticoid replacement median 50micrograms (0-150), with 3/109 having diagnosed hypertension. Systolic blood pressure was different between sexes but did not vary overall with age (median BP male:130/76, female: 121/72), although there was greater between patient variability at older ages (range of Q5 to Q95: systolic increasing 21 to 42, diastolic 19 to 37 across the age range). Multivariable LMEM showed that an increase in weight had a significant effect on systolic (estimate=0.2, standard error (SE)=0.05, P <0.001, R 2cond=0.44) and diastolic (estimate=0.1, SE=0.04, P = 0.02, R 2cond=0.29) blood pressure, whereas the models showed an insignificant effect of either hydrocortisone or fludrocortisone dosing (P >0.05).
Discussion: The effect of dosing on blood pressure in patients with CAH is likely to be variable in different patients impacted by their overall level of disease control and adherence to treatment, with increasing variability between patients with age. In comparison to the consistent effect of weight on blood pressure equivalent to an increase in systolic blood pressure of 2mmHg and diastolic blood pressure of 1mmHg for every 10 kg of increase weight, this highlights the variability in appropriate replacement doses between patients.
Conclusion: Blood pressure in young adults between 20 and 40 with CAH did not change with age. Increases that were seen within adults were more attributable to an increase in weight than changes in dosing, highlighting the importance of healthy lifestyle advice and adequate mineralocorticoid replacement without any indication that this will drive hypertension.
Article tools
My recent searches
My recently viewed abstracts
Authors
ESPE Abstracts
© Bioscientifica 2024 |
Privacy policy |
Cookie settings
BiosciAbstracts
Bioscientifica Abstracts is the gateway to a series of products that provide a permanent, citable record of abstracts for biomedical and life science conferences.
Find out more