CAH Management Quiz

A female newborn is born with abnormal external genitalia. Evaluation for CAH should be considered in which of the following situations?

A) Bilateral palpable gonads
B) Unilateral nonpalpable gonad
C) Bilateral nonpalpable gonads
D) Absent uterus

A 40-year-old woman with NCCAH presents with infertility. What is the recommended next step in management?

A. Start GC (glucocorticoid) treatment
B. Discontinue GC treatment if currently receiving it
C. Increase HC dose for stress situations
D. Start fertility treatment

A 25-year-old man with NCCAH is asymptomatic and has normal bone age. What is the recommended management?

A. Start GC treatment
B. Discontinue GC treatment if currently receiving it
C. Monitor without Treatment
D. Increase HC dose for stress situations

A 10-year-old girl with classic CAH is being treated with HC and fludrocortisone. Her height velocity has decreased over the past year. What is the recommended next step in management?

A. Increase the HC dose
B. Decrease the HC dose
C. Change to a long-acting GC
D. Add an androgen antagonist

A 16-year-old girl with classic CAH is being treated with HC and fludrocortisone. She reports increasing acne and hirsutism. Which of the following additional medications may be helpful for controlling her symptoms?

A. Spironolactone
B. Metformin
C. Drospirenone-containing oral contraceptives
D. Leuprolide

A 5-year-old child with classic CAH is being treated with hydrocortisone (HC) and fludrocortisone. During a routine check-up, the patient is found to have high blood pressure. What should be the next step in management?

A. Increase the HC dose
B. Decrease the HC dose
C. Increase the fludrocortisone dose
D. Decrease the fludrocortisone dose

A 14-year-old girl with classic CAH is experiencing hyperandrogenic symptoms. What Treatment may be needed in addition to glucocorticoid (GC) and mineralocorticoid (MC) therapies?

a) Oral contraceptives containing drospirenone
b) Spironolactone
c) Prednisone
d) Methylprednisolone

What is the suggested dose of fludrocortisone in fully-grown patients with classic CAH?

a) 0.2-0.5 mg/d, three times a day
b) 0.05-0.2 mg/d, once or twice a day
c) 0.1-0.3 mg/d, once a day
d) 0.3-0.5 mg/d, two times a day

A 5-year-old child is diagnosed with classic 21OHD. What is the recommended maintenance therapy with hydrocortisone (HC)?

a) 5-10 mg/m2⋅d, once a day
b) 10-15 mg/m2⋅d, three times a day
c) 20-25 mg/m2⋅d, two times a day
d) 15-20 mg/m2⋅d, once a day

A 10-year-old male with classic CAH is being treated with hydrocortisone. Which of the following should be monitored to optimize the adrenal steroid treatment profile?

A. Signs of glucocorticoid (GC) excess
B. Signs of inadequate androgen normalization
C. Signs of mineralocorticoid (MC) deficiency or excess
D. All of the above

A 25-year-old female presents with symptoms of classic CAH. What is the recommended timing for hormone measurement?

A. Any time of day
B. Early morning (before 8 AM)
C. Late afternoon (after 4 PM)
D. During menstruation

A 2-month-old female infant is referred to a pediatric endocrinologist after a positive newborn screen for CAH. Which diagnostic test should be performed?

A. Serum electrolyte measurement
B. Random serum 17OHP measurement
C. LC-MS/MS panel measurement of steroids
D. Cosyntropin stimulation test

A 6-year-old female has borderline 17OHP levels. What is the recommended next step?

A. Repeat the 17OHP measurement
B. Obtain a complete adrenocortical profile after a cosyntropin stimulation test
C. Refer to a genetic counselor for genotyping
D. No further evaluation is needed

A 20-year-old female presents with hirsutism and irregular menstrual cycles. What is the recommended screening test for CAH?

A. Early morning baseline serum cortisol measurement
B. Early morning baseline serum 17OHP measurement by LC-MS/MS
C. ACTH stimulation test
D. Genotyping

A 3-day-old male infant is found to have elevated 17OHP on newborn screening. What is the recommended next step?

A. Repeat filter paper specimen
B. Evaluate with a complete adrenocortical profile
C. Refer to a pediatric endocrinologist for cosyntropin stimulation testing
D. Monitor without further evaluation

A 28-year-old pregnant woman with a previous child with CAH presents for prenatal counseling. She is considering prenatal Treatment with Dex for the current pregnancy. What should be recommended to her regarding the balance of benefits and harms of prenatal Treatment with Dex?

A. The benefits of prenatal Treatment with Dex outweigh the risks, and it is recommended for all pregnancies at risk for CAH.
B. Prenatal Treatment with Dex is experimental and poses unresolved ethical questions, and the risks may outweigh the benefits.
C. Prenatal Treatment with Dex is recommended only if the fetus is male and affected with CAH.
D. Prenatal Treatment with Dex is contraindicated in all pregnancies.

A 32-year-old pregnant woman presents for prenatal counseling. She is concerned about the potential long-term effects of fetal exposure to Dex. What should be advised to her?

A. Long-term effects of fetal exposure to Dex are not well described.
B. Long-term effects of fetal exposure to Dex are well described, and studies have shown no adverse effects on brain development.
C. Long-term effects of fetal exposure to Dex are well described, and studies have shown adverse mental health sequelae in childhood and adolescence.
D. Long-term effects of fetal exposure to Dex are well described, and studies have shown improved cognitive function in adulthood.

A 26-year-old pregnant woman with a previous child with CAH presents for prenatal counseling. She is considering prenatal Treatment with Dex for the current pregnancy. What should be recommended to her?

A. Prenatal Treatment with Dex is recommended for all pregnancies at risk for CAH.
B. Prenatal Treatment with Dex should only be obtained through protocols approved by Institutional Review Boards at centers capable of collecting outcomes from a sufficiently large number of patients.
C. Prenatal Treatment with Dex is contraindicated in all pregnancies.
D. Prenatal Treatment with Dex is recommended only if the fetus is female and affected with CAH.

A pregnant woman at risk for carrying a fetus affected with CAH is considering prenatal Treatment. Which of the following statements regarding prenatal therapy is true?

a) Prenatal therapy is appropriate for male fetuses with CAH
b) Prenatal therapy can change the need for lifelong hormonal replacement therapy
c) Prenatal Treatment is associated with major risks to the mother
d) Prenatal diagnosis and treatment research should not be performed in countries where prenatal sex determination is illegal

A newborn female is found to have abnormal development of the external genitalia with virilization. Which of the following conditions is the most likely diagnosis?

a) Congenital adrenal hyperplasia (CAH)
b) Turner syndrome
c) Klinefelter syndrome
d) Androgen insensitivity syndrome

What is the dose of dexamethasone typically used for prenatal Treatment of CAH?

A) 10 μg/kg prepregnancy maternal body weight
B) 20 μg/kg prepregnancy maternal body weight
C) 30 μg/kg prepregnancy maternal body weight
D) 40 μg/kg prepregnancy maternal body weight

A patient with 46,XX CAH and complete virilization (Prader 5/normally formed “penis” with the urethral meatus within the glans) is undergoing gender assignment. What should be discussed with the patient and their family?

A) Pros and cons of female vs male gender assignment
B) Fertility implications of gender assignment
C) Both A and B
D) None of the above

A newborn female patient with CAH is diagnosed with clitoromegaly. What are the advantages of early complete reconstruction for this patient?

A) Ability to use excess common urogenital sinus tissue to reconstruct the anterior vaginal wall
B) Diminished risk of vaginal stenosis
C) Both A and B
D) None of the above

A 16-year-old patient with CAH and high confluence (Fig. 3) is considering surgical repair. What is the recommended approach?

A) Delayed reconstruction
B) Early complete surgical repair
C) Observation only
D) Clitoroplasty

A 6-year-old female patient with CAH presents with severe virilization and has not undergone surgery. What should be the ongoing observation in this patient?

A) Obstruction of menstrual flow
B) Risk of urinary tract infections
C) Both A and B
D) None of the above

A 3-year-old female patient with CAH is diagnosed with a low urogenital confluence of the vagina and urethra. What is the recommended approach for surgical repair?

A) Delayed reconstruction
B) Early complete surgical repair
C) Observation only
D) Clitoroplasty

A pregnant woman with CAH presents to the clinic. She has been on stable doses of hydrocortisone and fludrocortisone prior to her pregnancy. What is the recommended management for this patient during pregnancy?

A) Use of GCs that traverse the placenta, such as Dex
B) Discontinuation of all GC therapy
C) Continued prepregnancy doses of HC/prednisolone and fludrocortisone therapy, with dosage adjustments if symptoms and signs of GC insufficiency occur
D) Increased doses of fludrocortisone only

A 10-year-old girl with CAH presents to the clinic. Her parents are interested in surgical options to restore functional anatomy. What is the recommended advice?

A) Delay surgery until the child is older
B) Perform surgery immediately
C) Do not perform surgery at all
D) Recommend surgery only for severely virilized girls

A 25-year-old female with classic CAH presents for a routine check-up. She has been on stable doses of hydrocortisone and fludrocortisone for the past year. Which of the following should be recommended for this patient?

A) Routine adrenal imaging
B) Regular testicular ultrasounds
C) Lifestyle counseling for maintaining normal BMI
D) BMD screening

A 28-year-old male with classic CAH presents for a routine check-up. What is the recommended surveillance for this patient?

A. Routine adrenal imaging
B. Screening for BMD
C. Testicular ultrasound to assess for TARTs
D. Evaluation for cardiac and metabolic disease

A 32-year-old woman with classic CAH becomes pregnant. What is the recommended management during pregnancy?

A. Discontinuation of HC/prednisolone and fludrocortisone therapy
B. Decrease in HC/prednisolone and fludrocortisone therapy
C. Continued prepregnancy doses of HC/prednisolone and fludrocortisone therapy, with dosage adjustments if necessary
D. Switching to Dex therapy during pregnancy

A 26-year-old female with NCCAH presents with infertility. What is the recommended Treatment?

A. Treatment with a GC that traverses the placenta
B. No Treatment is required
C. Treatment with a GC that does not traverse the placenta
D. Treatment with prednisone

A 35-year-old woman with classic CAH has impaired fertility. What should be the next step?

a) Suppression of follicular-phase progesterone
b) Testicular sperm extraction (donor sperm)
c) Referral to a reproductive endocrinologist
d) Evaluation for adrenal rest tumors

A 25-year-old male with NCCAH is interested in having children. What is the recommended step prior to pregnancy planning?

a) Referral to a reproductive endocrinologist
b) Genetic counseling
c) Evaluation for TARTs
d) Sperm banking

A 16-year-old girl with classic CAH is transitioning to adult care. What should be included in her gynecological evaluation?

a) Evaluation for osteoporosis
b) Evaluation for vaginal stenosis or abnormalities in menstruation
c) Evaluation for hypertension
d) Evaluation for obesity

Which of the following is a recommendation for prenatal Treatment of CAH?

A) Use of prenatal Treatment for male fetuses
B) Start of Treatment at 10 to 12 weeks of gestation
C) Use of chorionic villous biopsy for genetic diagnosis
D) Obtaining prenatal therapy only through protocols approved by Institutional Review Boards at centers capable of collecting outcomes from a sufficiently large number of patients

What is the aim of prenatal Treatment with Dexamethasone for CAH?

A) Prevention of lifelong hormonal replacement therapy
B) Prevention of social stigma associated with female genital virilization
C) Prevention of life-threatening salt-losing crises
D) Reduction of potential androgenization of the female fetal brain

A woman has previously delivered a child with CAH and is pregnant again via the same partner. What is the chance that the fetus will be female and have CAH?

A) 1:2
B) 1:4
C) 1:8
D) 1:16

Which of the following is the main advantage of newborn screening for CAH?

A) Reduced learning disabilities in patients with salt-wasting crises
B) Shorter hospitalizations for infants diagnosed with CAH
C) Prevention of serious morbidity and mortality
D) Reduction in delayed or incorrect diagnosis in males with salt-wasting CAH

A newborn is found to have nonpalpable gonads. Which of the following conditions should be considered in the differential diagnosis?

A) Turner syndrome
B) Klinefelter syndrome
C) Classic CAH
D) Simple virilizing CAH

A newborn is diagnosed with salt-losing CAH. Which of the following complications is most commonly associated with missed diagnosis of this condition?

A) Hypothyroidism
B) Hypovolemia
C) Hyperkalemia
D) Hypoglycemia

A newborn male is noted to have rapid postnatal growth and virilization. Which of the following conditions should be considered in the differential diagnosis?

A) Turner syndrome
B) Klinefelter syndrome
C) Simple virilizing CAH
D) Salt-losing CAH

Management of Congenital Adrenal Hyperplasia: Endocrine Society Guidelines

CAH Management Quiz

CAH Management Quiz

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