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Isolated adrenocorticotropic hormone deficiency associated with common variable immunodeficiency

  • Endocrinology
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Abstract

A 14-year-old girl has been suffering from an isolated adrenocorticotropin hormone (ACTH) deficiency with secondary glucocorticoid deficiency and common variable immunodeficiency since the age of 6.6 years. Human corticotropin releasing hormone administration did not increase ACTH and cortisol levels, strongly suggesting a pituitary deficiency. Despite the profound humoral defect, severe infections have never developed and the antibody response to herpes viruses was intact. We speculate that the association between two rare disorders, simultaneously diagnosed 2 months after measles, is not coincidental but caused by close interactions between neuro-endocrine and immune systems.

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Abbreviations

ACTH:

adrenocorticotropic hormone

CVI:

common variable immunodeficiency

IFN-γ:

interferon-γ

References

  1. Alvarez-Mon M, Kehrl JH, Fauci AS (1985) A potential role of adrenocorticotropin in regulating human B lymphocyte functions. J Immunol 135:3823–3826

    PubMed  Google Scholar 

  2. Aynsley-Green A, Moncrieff MW, Ratter S, Benedict CR, Storrs CN, Wilkinson RH (1978) Isolated ACTH deficiency. Arch Dis Child 53:499–502

    PubMed  Google Scholar 

  3. Carey DE (1985) Isolated ACTH deficiency in childhood: lack of response to corticotropin-releasing hormone alone and in combination with arginine vasopressin. J Pediatr 107:925–928

    PubMed  Google Scholar 

  4. Conley ME, Park CL, Douglas SD (1986) Childhood common variable immunodeficiency with autoimmune disease. J Pediatr 108:915–922

    PubMed  Google Scholar 

  5. Cotman CW, Brinton RE, Galaburola A, McEwen B, Shneider DM (1987) The neuro-immune endocrine connection. Raven Press, New York

    Google Scholar 

  6. Eibl M, Griscelli C, Seligman M, Aiuti F, Kishimoto T, Matsumoto S, Hanson LA, Hitzig WH, Thompson RA, Cooper MD, Good RA, Rosen FS, Waldmann TA, Wedgood RJ (1989) Primary immunodeficiency diseases. Immunol Rev 1:173–205

    Google Scholar 

  7. Fleisher TA, White RM, Broder S, Nissley SP, Blaese RM, Mulvihill JJ, Olive G, Waldmann TA (1980) X-linked hypogammaglobulineamia and isolated growth hormone deficiency. N Engl J Med 302:1429–1434

    PubMed  Google Scholar 

  8. Hagen GA, Bolman RM, Frank JP (1975) Atypical adrenal insufficiency with failure of the pituitary feedback receptor. Am J Med 59:882–888

    PubMed  Google Scholar 

  9. Hashimoto K, Takahara J, Takaja Y, Yunoki S, Ofuji T (1980) Isolated ACTH and B-LPH deficient patient having anaphylactic shock after 1-18 ACTH. J Clin Endocrinol Metab 51: 1175–1179

    PubMed  Google Scholar 

  10. Hausser C, Virelizier JL, Buriot D, Griscelli C (1983) Common variable hypogammaglobulinemia in children. Am J Dis Child 137:833–837

    PubMed  Google Scholar 

  11. Hochberg Z, Hardoff D, Atias D, Spindel A (1985) Isolated ACTH deficiency with transitory GH deficiency. J Endocrinol Invest 8:67–70

    PubMed  Google Scholar 

  12. Hung W, Migeon CJ (1968) Hypoglicemia in a two-year-oldboy with adrenocorticotropic hormone (ACTH) deficiency (probably isolated) and adrenal medullar unresponsiveness to insulin-induced hypoglicemia. J Clin Endocrinol Metab 28: 146–152

    PubMed  Google Scholar 

  13. Ichiba Y, Goto T (1983) Isolated corticotropin deficiency. Am J Dis Child 137:1202–1203

    PubMed  Google Scholar 

  14. Jensen MD, Handwergen BS, Scheithauer BW, Carpenter PC, Mirakian R, Banks PM (1986) Lymphocyte hypophysitis with isolated corticotropin deficiency. Am Intern Med 105:200–203

    Google Scholar 

  15. Kojima I, Nejima I, Ogata E (1982) Isolated adrenocorticotropin deficiency associated with polyglandular failure. J Clin Endocrinol Metab 54:182–186

    PubMed  Google Scholar 

  16. Martin MM, Martin ALA (1971) Idiopathic hypoglicemia: a defect in hypothalamic ACTH-releasing factor. Pediatr Res 5:396

    Google Scholar 

  17. Neighbour PA, Grayel AI (1981) Interferon production in vitro by leucocytes from patients with systemic herpes erythematosus and rheumatoid arthritis. Clin Exp Immunol 25:576–582

    Google Scholar 

  18. Pastorelli G, Roncarolo MG, Touraine JL, Tovo PA, Vries JE de (1989) Peripheral blood lymphocytes of patients with common variable immunodeficiency (CVI) produce reduced levels of IL-4, IL-2 and IFN- but poliferate normally upon activation by mitogens. Clin Exp Immunol 78:334–340

    PubMed  Google Scholar 

  19. Richtsmeier AJ, Henvy RA, Bloodworth JMB, Ehrich EN (1980) Lymphoid hypophysitis with selective adrenocorticotropic hormone deficiency. Arch Intern Med 140:1243–1245

    PubMed  Google Scholar 

  20. Stacpoole PW, Interlandi JW, Nicholson WE, Rabin D (1982) Isolated ACTH deficiency: a heterogeneous disorder. Critical review and report of four new cases. Medicine 61:13–24

    PubMed  Google Scholar 

  21. Stalla GK, Hartwimmer J, Werder K von, Muller OA (1984) Ovine (o) and human (h) corticotrophin releasing factor (CRF) in man: CRF-stimulation and CRF-immunoreactivity. Acta Endocrinol 106:289–297

    PubMed  Google Scholar 

  22. Tsukada T, Nakai Y, Koh T, Tsuji S, Inada M, Nishikawa M, Shinoda H, Kaway I, Takesawa N, Imura H (1984) Plasma adrenocorticotropin and cortisol responses to ovine cortitropinreleasing factor in patients with adrenocortical insufficiency due to hypothalamic and pituitary disorders. J Clin Endocrinol Metab 58:758–760

    PubMed  Google Scholar 

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Tovo, P.A., Lala, R., martino, S. et al. Isolated adrenocorticotropic hormone deficiency associated with common variable immunodeficiency. Eur J Pediatr 150, 400–402 (1991). https://doi.org/10.1007/BF02093717

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  • DOI: https://doi.org/10.1007/BF02093717

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