Skip to main content
SpringerLink
Log in

Congenital adrenal hypoplasia: two new cases

  • Case Report
  • Published:
Journal of Endocrinological Investigation Aims and scope Submit manuscript

Abstract

Two male adolescents with the X-linked form of congenital adrenal hypoplasia are described. Both grew slowly during childhood and adolescence and did not undergo pubertal development because of hypogonadotropic hypogonadism associated with the congenital adrenal hypoplasia. The severely delayed bone age in childhood is probably due to the adrenal androgen deficiency and suggests a role of these hormones in the prepubertal skeletal maturation. The failure of gonadotropin secretion still remains unexplained. A hypothalamic defect has been suggested, but further studies are necessary to clarify this hypothesis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Prader A., Zachmann M., Illig R. Luteinizing hormone deficiency in hereditary congenital adrenal hypoplasia. J. Pediatr. 86:421, 1975.

    Article  PubMed  CAS  Google Scholar 

  2. Golden M.P., Lippe B.M., Kaplan S.A. Congenital adrenal hypoplasia and hypogonadotropic hypogonadism. Am. J. Dis. Child. 131: 1117, 1977.

    Article  PubMed  CAS  Google Scholar 

  3. Kelly W.F., Joplin G.F., Pearson G.W. Gonadotropin deficiency and adrenocortical insuffi ciency in children: a new syndrome. Br. Med. J. 2: 98, 1977.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  4. Block S., Brook C.G.D., Cox P.J.N. Congenital adrenal hypoplasia and gonadotropin deficiency. Br. Med. J. 2:996, 1977.

    Article  Google Scholar 

  5. Hay I.D., Smail P.J., Forsyth C.C. Familial cytomegalic adrenocortical hypoplasia: an X-linked syndrome of pubertal failure. Arch. Dis. Child. 56: 715, 1981.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  6. Tanner J.M., Whitehouse R.H. Clinical longitudinal standard for height, weight, height velocity, weight velocity, and stages of puberty. Arch. Dis. Child. 51: 170, 1976.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  7. Greulich W.W., Pyle S.I. Radiographic atlas of skeletal development of hand and wrist, ed. 2. Stanford Press, Stanford, 1959.

    Google Scholar 

  8. Korth-Schutz S., Levine L.S., New M.I. Serum androgens in normal prepubertal and pubertal children and in children with precocious puberty. J. Clin. Endocrinol. Metab. 42: 117, 1976.

    Article  PubMed  CAS  Google Scholar 

  9. Korth-Schutz S., Levine L.S., New M.I. Dehydroepiandrosterone sulfate (DS) levels, a rapid test for abnormal adrenal androgen secretion. J. Clin. Endocrinol. Metab. 42: 1005, 1976.

    Article  PubMed  CAS  Google Scholar 

  10. Weiss L, Mellinger R.C. -Congenital adrenal hypoplasia: an X-linked disease. J. Med. Genet. 7:27, 1970.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  11. Brook C.G.D., Bambach M., Zachmann M., Prader A. Familial congenital adrenal hypoplasia. Helv. Paediatr. Acta 28: 277, 1973.

    PubMed  CAS  Google Scholar 

  12. Zachmann M., Illig R., Prader A. Gonadotropin deficiency and cryptorchidism in three prepubertal brothers with congenital adrenal hypoplasia. J. Pediatr. 97: 255, 1980.

    Article  PubMed  CAS  Google Scholar 

  13. Richards G.E., Conte F.A., Kaplan S.L., Grumbach M.M. Congenital adrenal hypoplasia and isolated gonadotropin deficiency: ability of gonadotropes to respond to LRF. Clinical Research 26: 171A, 1978.

    Google Scholar 

  14. Grumbach M.M., Richards G.E., Conte F.A., Kaplan S.L. Clinical disorders of adrenal function and puberty: an assessment of the role of the adrenal cortex in normal and abnormal puberty in man and evidence for an ACTH-like pituitary adrenal androgen stimulating hormone. In: Jones V.S.H., Serio M., Giusti G., Martini L. (Eds.), The endocrine function of the human adrenal cortex. Academic Press, New York, 1978, p. 583.

    Google Scholar 

  15. Sizonenko P.C., Paunier L., Carmignac D. Hormonal changes during puberty. IV. Longitudinal study of adrenal androgen secretion. Horm. Res. 7:288, 1976.

    Article  PubMed  CAS  Google Scholar 

  16. Sizonenko P.C., Paunier L. Hormonal changes in puberty. III. Correlation of plasma dehydroepiandrosterone, testosterone, FSH, LH with stages of puberty and bone age in normal boys and girls and in patients with Addison’s disease, or hypogonadism or with premature or late adrenarche. J. Clin. Endocrinol. Metab. 47: 894, 1975.

    Article  Google Scholar 

  17. Copeland K.C., Paunier L., Sizonenko P.C. The secretion of adrenal androgens and growth patterns of patients with hypogonadotropic hypogonadism and idiopathic delayed puberty. J. Pediatr. 91:985, 1977.

    Article  PubMed  CAS  Google Scholar 

  18. Hay I.D. Pubertal failure in congenital adrenocortical hypoplasia. Lancet 2: 1035, 1977.

    Article  PubMed  CAS  Google Scholar 

  19. Marsden H.B., Zakhour H.D. Cytomegalic adrenal hypoplasia with pituitary cytomegaly. Virchows Arch. Pathol. Anat. 378: 105, 1978.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. The Division of Pediatric Endocrinology, Department of Pediatrics, The New York Hospital, Cornell Medical Center, 525 East 68 Street, New York, New York, 10021, USA

    R. Virdis, L. S. Levine, D. Levy, S. Pang, R. Rapaport & M. I. New

Authors
  1. R. Virdis
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. L. S. Levine
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. D. Levy
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. S. Pang
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. R. Rapaport
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. M. I. New
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Virdis, R., Levine, L.S., Levy, D. et al. Congenital adrenal hypoplasia: two new cases. J Endocrinol Invest 6, 51–54 (1983). https://doi.org/10.1007/BF03350561

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03350561

Key-words

Search

Navigation