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HYDROCORTISONE THERAPY AND GROWTH TRAJECTORY IN CHILDREN WITH CLASSICAL CONGENITAL ADRENAL HYPERPLASIA

Carla Bizzarri, Nicola Improda, Chiara Maggioli

    Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
    |February 23, 2017
    PubMed

    Insights

    Optimizing hydrocortisone dosage in children with congenital adrenal hyperplasia (CAH) is crucial for improving growth. Lower doses during puberty enhance growth spurts and final height in CAH patients.

    Area of Science:

    • Pediatric Endocrinology
    • Genetics and Inherited Diseases

    Background:

    • Congenital adrenal hyperplasia (CAH) is a group of genetic disorders affecting the adrenal glands.
    • Poor linear growth is a significant concern for children diagnosed with CAH.
    • Understanding growth trajectory factors is essential for managing CAH patients.

    Purpose of the Study:

    • To analyze factors influencing growth trajectory in children with classical CAH.
    • To evaluate the impact of treatment on height velocity, pubertal growth spurt, and final height.
    • To identify optimal treatment strategies for improving growth outcomes in CAH.

    Main Methods:

    • Retrospective review of clinical records from two Italian centers.
    • Inclusion of 57 children (31 males) with classical CAH treated with hydrocortisone and fludrocortisone.
    • Categorization of clinical observations into three age groups: 0-2 years, 2 years to puberty onset, and after puberty onset.

    Main Results:

    • Final height standard deviation score (SDS) was lower than target height SDS.
    • Higher hydrocortisone doses were associated with decreased height-velocity SDS during puberty.
    • Adrenocorticotropic hormone (ACTH) levels positively correlated with height velocity and total pubertal growth.
    • Mineralocorticoid therapy and ACTH levels positively impacted target-adjusted final height SDS.

    Conclusions:

    • Height outcomes for CAH patients have improved compared to previous reports.
    • Using the lowest effective hydrocortisone dose during puberty is recommended.
    • Optimizing treatment can enhance pubertal growth and final height in children with CAH.
    Abstract

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