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European Journal of Pediatrics

, Volume 150, Issue 10, pp 696–699 | Cite as

Adrenocorticotropic hormone insensitivity associated with autonomic nervous system disorders

  • M. Dumić
  • A. Radica
  • Z. Sabol
  • V. Plavšić
  • Lj Brkljačić
  • V. Sarnavka
  • J. Vuković
Endocrinology

Abstract

Five children with adrenocorticotropic hormone (ACTH) insensitivity associated with autonomic nervous system disorders are described. At the time of diagnosis, four of them had osteoporosis. The fifth patient died and skeletal roentgenograms were not done. Osteoporosis was subsequently discovered in one of our previously reported patients with ACTH insensitivity. We assume that osteoporosis is, at least partly, the result of decreased adrenal androgen production. Human leucocyte antigen typing failed to establish any linkage.

Key words

ACTH insensitivity Adrenal insufficiency Autonomic nervous system disorders Osteoporosis HLA typing 

Abbreviations

ACTH

adrenocorticotropic hormone

HLA

human leucocyte antigen

PRA

plasma renin activity

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References

  1. 1.
    Allgrove J, Clayden GS, Grant DB, Macaulay JC (1978) Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet I:1284–1286Google Scholar
  2. 2.
    Aust W (1970) Prüfung der Tränensekretion und Funktion der ableitenden Tränenwege. In: Staub W (ed) Die ophtalmologischen Untersuchungen. Enke Stuttgart, pp 90–91Google Scholar
  3. 3.
    Counahan R, West R (1974) Ocular and fingertip abnormalities in isolated glucocorticoid deficiency. J Pediatr 85:580–581Google Scholar
  4. 4.
    Crilly RG (1987) Bone mineral density in Addison's disease. Br Med J 294:1486Google Scholar
  5. 5.
    Devogelaer JP, Crabbe J, De Deuxchaisnes CN (1987) Bone mineral density in Addison's disease: evidence for an effect of adrenal androgens on bone mass. Br Med J 294:798–800Google Scholar
  6. 6.
    Dumić M, Radica A, Jušić A, Stefanović N, Murko Z (1987) Selective ACTH insensitivity associated with autonomic nervous system disorders and sensory neuropathy. Eur J Pediatr 146:592–594PubMedGoogle Scholar
  7. 7.
    Gefner ME, Lippe BM, Kaplan SA, Berquist WE, Batteman JB, Paterno W, Seegan R (1983) Selective ACTH insensitivity, achalasia and alacrimia: a multisystem disorder presenting in childhood. Pediatr Res 17:532–536PubMedGoogle Scholar
  8. 8.
    Kaštelan A, Kerhin-Brkljačić V, Hors J, Brkljačić LJ, Maćašović P (1974) The distribution of HLA antigens and genes in the Yugoslav population. Tissue Antigens 4:69–75PubMedGoogle Scholar
  9. 9.
    Kelch RP, Kaplan SL, Beglieri EG, Daniels GH, Epstein CJ, Grumbach MM (1972) Hereditary adrenocortical unresponsiveness to adrenocorticotropic hormone. J Pediatr 81:726–736PubMedGoogle Scholar
  10. 10.
    Lanes R, Plotnick LP, Bynum TE, Lee PA, Casella JF, Fox CE, Kowarski AA, Migeon JM (1980) Glococorticoid and partial mineralocorticoid deficiency associated with achalasia. J Clin Endocrinol Metabol 50:268–270Google Scholar
  11. 11.
    Migeon CJ, Kenny FM, Kowarski A, Snipes CA, Spaulding JS, Finkelstein JW, Blizzard RM (1968) The syndrome of congenital adrenocortical unresponsiveness to ACTH. Report of six cases. Pediatr Res 2:501–513PubMedGoogle Scholar
  12. 12.
    Preson SL, McMorris FA (1984) Adrenalectomy of rats results in hypomyelination of the central nervous system. J Neurochem 42:262–267PubMedGoogle Scholar
  13. 13.
    Schliack H (1977) Methoden zur Untersuchung der Schweißsekretionsstörungen. In: Mumenthaler M, Schliak H (eds) Läsionen peripherer Nerven. Thieme, Stuttgart, pp 70–71Google Scholar
  14. 14.
    Stuckey BG, Mestaglia FL, Reed WD, Pullan PT (1987) Glucocorticoid insufficiency, achalasia, alacrimia with autonomic and motor neuropathy. Ann Intern Med 106:62–64Google Scholar

Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • M. Dumić
    • 1
  • A. Radica
    • 1
  • Z. Sabol
    • 1
  • V. Plavšić
    • 1
  • Lj Brkljačić
    • 1
  • V. Sarnavka
    • 1
  • J. Vuković
    • 1
  1. 1.Department of Paediatrics and Tissue Typing CentreUniversity Hospital RebroZagrebYugoslavia

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