• Credits
  • Section Writer: Dr. Om J Lakhani
  • Section Editor: Dr. Om J Lakhani

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• 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 ?

  

  • 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