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ACTH stimulation panel; for 21 hydroxylase deficiency This panel must include the following: Cortisol (82533 x 2) 17 hydroxyprogesterone (83498 x 2)

CPT4 code

Name of the Procedure:

ACTH Stimulation Panel for 21 Hydroxylase Deficiency
Common Name(s): ACTH stimulation test, Cosyntropin stimulation test
Technical/M edical Term: Adrenocorticotropic hormone (ACTH) stimulation panel

Summary

The ACTH stimulation panel is a diagnostic test used to evaluate the functioning of the adrenal glands by measuring cortisol and 17-hydroxyprogesterone levels before and after administration of synthetic ACTH.

Purpose

Medical Condition: 21 Hydroxylase Deficiency
Goals: To assess adrenal gland function and diagnose conditions such as congenital adrenal hyperplasia (CAH) due to 21 hydroxylase deficiency.

Indications

  • Symptoms of adrenal insufficiency like fatigue, muscle weakness, and salt craving.
  • Ambiguous genitalia or other signs of CAH in newborns.
  • Unexplained low blood pressure or dehydration.
  • Screening in patients with a family history of 21 hydroxylase deficiency.

Preparation

  • Patients may be instructed to fast for a certain period before the test.
  • Medications affecting cortisol levels should be adjusted as directed by the healthcare provider.
  • A preliminary blood draw may be required to establish baseline levels.

Procedure Description

  1. Baseline Blood Draw: A blood sample is taken to measure initial cortisol (82533) and 17-hydroxyprogesterone (83498) levels.
  2. Administration of ACTH: Synthetic ACTH (cosyntropin) is administered via injection.
  3. Post-ACTH Blood Draws: Additional blood samples are taken at specified intervals (usually 30 and 60 minutes after administration) to measure the levels of cortisol and 17-hydroxyprogesterone again.

Tools and Equipment: Needles, syringes, synthetic ACTH (cosyntropin), blood collection tubes.
Anesthesia/Sedation: Not typically required.

Duration

The entire procedure typically takes about 1-2 hours.

Setting

The procedure is usually performed in an outpatient clinic or hospital laboratory.

Personnel

  • Trained nurses or phlebotomists for blood draws.
  • A healthcare provider such as an endocrinologist or pediatrician to supervise the test.

Risks and Complications

  • Minor risks include pain or bruising at the injection site.
  • Rarely, allergic reactions to synthetic ACTH.

Benefits

  • Accurate diagnosis of 21 hydroxylase deficiency or other adrenal disorders.
  • Guidance for appropriate treatment and management of diagnosed conditions.

Recovery

  • Post-procedure care is minimal and typically involves monitoring for any immediate reactions to the injection.
  • Patients can typically resume normal activities shortly after the procedure.
  • Follow-up appointments may be scheduled to discuss test results and further treatment plans.

Alternatives

  • Other diagnostic tests, such as genetic testing or different hormonal assays.
  • Pros: Genetic testing is non-invasive and can directly indicate CAH.
  • Cons: ACTH stimulation test provides functional information about adrenal performance not available from genetic tests.

Patient Experience

  • Patients may feel a slight discomfort from needle sticks for blood draws and the injection.
  • Pain management measures include local anesthetics or cold sprays to numb the area.
  • Overall, the procedure is well-tolerated and low-risk.

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