Monitoring
The main concerns in children are optimizing growth and preventing early puberty. The preservation of fertility and healthy sexual function, and maintenance of general wellbeing are the focus of care for adults. At all ages, maintaining a healthy body mass index (BMI), reducing cardiovascular risk factors including hypertension and maintenance of general wellbeing are priorities. Optimal treatment adults requires a multidisciplinary approach, including psychologic support by dedicated providers.[38]
Successful treatment of affected children hinges on the delicate balance of suppressing adrenal androgen secretion with glucocorticoid administration while maintaining normal growth.
In growing children, we recommend a follow-up at least every 3 months. In adulthood, the appointment frequency can be spaced to every 6 to 12 months.[11][14]
Growth data including BMI, pubertal assessment, and blood pressure measurements are examples of clinical parameters necessary for each visit. In some clinics, serum concentrations of important adrenal corticosteroids (17-hydroxyprogesterone, delta-4-androstenedione, testosterone) are monitored in the morning at each clinic visit. Renin is monitored in salt-wasting CAH patients. Bone maturation is assessed by bone age of the left hand, usually every 6 months using the Greulich and Pyle method.[39] Additional laboratory evaluation to determine cardiovascular risk factors can be considered.
Beginning at adolescence, testicular ultrasound assessments should begin to screen for testicular adrenal rest tumors (TARTs). Expert consensus recommends every 1 to 2 years if asymptomatic.[14] No routine screening is recommended for ovarian adrenal rest tumors, which are far more rare. In either sex, increasing hydrocortisone dosing and poor control should prompt consideration of adrenal rest tumor.
In adult patients, bone mineral density study is recommended for a baseline exam, with plans for follow-up determined based on the results.[14]
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