Blood pressure and its associations in 554 children and young people with CAH.
Lawrence NR., Bacila I., Tonge J., Dawson J., Collins GS., Lang Z-Q., Bryce J., Alimussina M., Chen M., Ali SR., Adam S., van den Akker ELT., Bachega TASS., Baronio F., Birkebæk NH., Bonfig W., Claahsen van der Grinten H., Cools M., Costa EC., Debono M., de Vries L., Flück CE., Gazdagh G., Güven A., Hannema SE., Iotova V., van der Kamp HJ., Krone R., Leka-Emiri S., Clemente-León M., Lichiardopol CR., Markosyan RL., Milenkovic T., de Miranda MC., Neumann U., Newell-Price J., Poyrazoğlu Ş., Probst-Scheidegger U., Russo G., De Sanctis L., Seneviratne SN., Stancampiano MR., Tadokoro-Cuccaro R., Thankamony A., Vieites A., Wasniewska M., Yeste D., Tomlinson J., Ahmed SF., Krone N.
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.