- Credits - Section Writer: Dr. Om J Lakhani - Section Editor: Dr. Om J Lakhani Support us: 1. Support us by purchasing our book - Click here for more details: [[Volume 1- THE BEST OF NOTES IN ENDOCRINOLOGY BOOK SERIES]] 2. [Support you by Becoming a YouTube member (Click here)](https://www.youtube.com/channel/UC6zQSf7dLDqfQOeM4mNUBTQ/join) - Q. What are clinical presentation of non classical CAH (NCCAH) at various ages ? - Children - Premature Pubarche - Accelerated growth - Medication resistent acne - Adolescent girl - PCOS type presentation in young girls - Adult women - Infertility - PCOS type features - Male - Generally asymptomatic - May have TART and infertility - Adrenal incidentaloma - Many adults present with unilateral or bilateral incidentaloma - Q. Who should be tested for NCCAH ? - Children having premature Pubarche, accelerated growth or treatment resistant acne - Postpubertal girls and women with Oligo-amenorrhea, Hyperandrogenism and infertility - Adrenal incidentaloma with history/ examination s/o of CAH - Relative of patients with CAH - Q. How is diagnosis of NCCAH made ? ![[Pasted image 20220411084840.png]] - Please note- the values are in ng/ml - to convert to ng/dl multiply the values by 100 - Q. What value is diagnostic in children ? - Uptodate says: - < 0.82 ng/ml (82 ng/dl) - rules out Non-classical CAH in children - More than 2 ng/ml (200ng/dl)s surely NCCAH and ACTH stimulation is generally not needed - 0.82-2 ng/ml (82-200 ng/dl) – may consider doing a ACTH stimulation - Q. What is the diagnostic value in an adolescent ? - A baseline 17 OHP >15 ng/ml is diagnostic of non-classical CAH - Q. At what time after giving ACTH is sample repeated ? - 60 min - Q. Describe the management of NCCAH in children ? - In children with hyperandrogenism and bone age advancement -Start hydrocortisone 10-15 mg/m2 - Continue treatment till: - Early to mid puberty in boys - 2-3 years post menarche in girls - If the girl continues to have symptoms of menstrual irregularity and Hyperandrogenism then give OC pills and not glucocorticoids - Q. Give the outline of management of NCCAH in adults ? - Adult male with NCCAH: No treatment - Adult women - Not desiring fertility - OC pills (1st line) - anti-androgens (2nd line) - Glucocorticoids (3rd line) - Infertility - Glucocorticoids (1st line) - Clomiphene (2nd line) - Q. Are stress dose of Glucocorticoids required in people with NCCAH ? - If they are on glucocorticoids - yes - If they are not on Glucocorticoids- then no - Q. What should be the targets while treating NCCAH ? - They have not been clearly defined - 17-OHP should be in upper limit of normal - Progesterone is helpful for monitoring in women with infertility with value <0.6 ng/ml is associated with improved fertility outcomes - Q. Should asymptomatic patients with NCCAH be treated ? - No