ESPE Abstracts (2014) 82 P-D-1-3-13

Assessment of Blood Pressure and Left Ventricular Parameters in Children with Classical CAH-due to 21 Hydroxylase Deficiency

Shereen Abdelghaffara, Reem Ibrahima, Osama Abdelaziza, Mona Hafeza, Mona Mamdouha, Abeer Alashmawya, Fatma Elmougyb, Sahar Abdelatyb, Amany Ibrahima, Hend Mehaweda & Isis Ghalya


aDepartment of Pediatrics, DEMPU, Cairo University, Cairo, Egypt; bDepartment of Chemical Pathology. Cairo University, Cairo, Egypt


Background: Patients with congenital adrenal hyperplasia (CAH), due to 21 hydroxylase (OH) deficiency may develop an adverse cardiovascular risk profile as reported by few previous studies.

Objective and Hypotheses: Blood pressure (BP) and echo parameters in children with CAH due to 21-OH deficiency were evaluated.

Method: This cross-sectional study included 53 children. BP, echocardiographic left ventricular functions as well as growth parameters were compared to results of 30 healthy control children. Association of results with androgen levels as well as with glucocorticoids (GCs), and mineralocorticoids (MCs) treatment doses were studied.

Results: By using BP International centiles, 23 (43.3%) patients had hypertension. BP levels in cases were positively correlated with GCs doses and negatively correlated with 17-hydroxyprogesterone (OHP) levels. Fifteen (28.8%) patients were obese (>2SDS), and generally, cases had significantly higher BMI-SDS than controls. Growth velocity (GV)-SDS was negatively correlated with MCs dose and plasma renin activity (PRA level. By echocardiography, mean left ventricular mass (LVM) and left ventricular mass index (LVMI) (when compared to published paediatric criteria: LVMI>38.6 g/m2.7) were found to be elevated. In cases, inter ventricular septum in diastole (IVSd) and left ventricular posterior wall in diastole (LVPWd) were statistically significantly increased while Left ventricular internal dimension in systole (LVIDs) and left ventricular mass index (LVMId) were statistically significantly decreased (P<0.05). Multivariable linear regression showed that glucocorticoid dose was a predictor of hypertension and that age and hypertension were predictors of left ventricular (LV) hypertrophy (as evidenced by IVS, LVM, and LVMI) (P<0.05 for all).

Conclusion: Patients with CAH due to 21 OH deficiency can suffer from hypertension as well as affection of left ventricular functions. The systematic monitoring of BP, height, BMI, and echocardiographic parameters are important to prevent cardiovascular morbidity in adult CAH patients.