Transient Adenocorticotropic Hormone deficiency in an infant

Abstract

Background: Isolated Adenocorticotropin Hormone (ACTH) deficiency is a rare disorder, characterized by secondary adrenal insufficiency with a low or absent cortisol production, normal secretion of other pituitary hormones and absence of structural pituitary defects.

Case summary: The patient was the product of a 28 weeks gestation, clomiphen induced to a 40-year-old, gestational diabetic mother. He was ventilated for 30 days, and was diagnosed to have grade IV Retina of Prematurity (ROP). He suffered from recurrent attacks of hypoglycemic, blood sugar of 1.6 mmol/L, low serum cortisol 65 nmol/L (normal; 150-630), and ACTH of 1.5 pmol/L (normal; 1.6-13.9), with suppressed serum insulin and normal thyroid, growth hormone and gonadal functions. Magnetic Resonance Imaging (MRI) was unremarkable. He was started on hydrocortisone 2.5 mg twice daily for two years, which was then slowly tapered and stopped. Later Serum cortisol was 175 nmol/L, and ACTH of 5.5 pmol/L with a normal shot ACTH stimulation test.

Conclusions: This case of a premature baby who presented with recurrent hypoglycemia had an isolated ACTH deficiency that proved to be transient. The pediatricians need to be aware of the existence of such condition.

Keywords

Adenocorticotropin Hormone / Isolated / Hypoglycemic / Transient

ACKNOWLEDGEMENTS

The authors would like to thank Mrs. Cecile Sael and Miss Hadeel N. Al Jurayyan for her help in preparing this manuscript.

CONFLICTS OF INTEREST DISCLOSURE

The authors declare no conflicts of interest.

References

[1] Andriloli M, Pecori Giraldi F, Cavagnini F. Isolated Corticotrophin deficiency pituitary. 2006; 9(4): 289-95.

[2] Al Jurrayan NAM. Isolated Adrenocorticotropin deficiency as a rare cause of hypoglycaemia in children. Horm Res. 1995; 44: 238-40. https://doi.org/10.1159/000184633

[3] Escobar-Morreale H, Serrano-Gotarreadona J, Varela C. Isolated Adrenocorticotropic hormone deficiency due to probable lympho-cytic hypophysitis in a man. Journal of Endocrinological Investigation. 1994; 17: 127-31. PMid: 8006333. https://doi.org/10.1007/BF03347700

[4] Kasperlik-Zaluska AA, Czarnocka B, Czech W, et al. Secondary adrenal insufficiency associated with autoimmune disorders, A report of twenty five cases. Clin Endocrinol (Oxf). 1998; 49(6): 779-83. https://doi.org/10.1046/j.1365-2265.1998.00611.x

[5] Scoble JE, Harvard CW. Anosmia and Isolated ACTH deficiency following a road traffic accident: Case report. J Neurosurg. 1990; 73(3): 453-4. PMid:2166780. https://doi.org/10.3171/jns.1990.73.3.0453

[6] Sakai H, Yoshioka K, Yamagami K, et al. Complete adrenocorti-cotropin deficiency after radiation therapy for brain tumor with a normal growth hormone reserve. Intern med. 2002; 41: 453-7. PMid: 12135178. https://doi.org/10.2169/internalmedicine.41.453

[7] Metherell LA, Savage MO, Dattani M, et al. TPIT mutations are associated with early onset, but not late onset isolated ACTH deficiency. Eur J Endocrinol. 2004; 151: 463-5.

[8] Hochberg Z, Hardoff D, Atias D, et al. Isolated ACTH deficiency with transitory GH deficiency. J Endocrinol Invest. 1985; 8(1): 67-70. PMid: 2985687. https://doi.org/10.1007/BF03350645

[9] Hiraiwa T, Furutama D, Sakane S, et al. Isolated Adrenocorti-cotropic hormone deficiency with transient thyroiditis inducing an adrenal crisis. Med Princ Pract. 2007; 16: 230-3. PMid: 17409760. https://doi.org/10.1159/000100396

[10] AL Jurrayan NA. Primary Adrenal Insufficiency (PAI):A major teaching hospital experience, Riyadh, Saudi Arabia. Int J Clinical Therapeutic and Diagnosis (IJCTD). 2015; 3(4): 92-4.

[11] Nishi H, Nishi Y, Yagita M, et al. A case of transient ACTH deficiency associated with polymyalgia Rheumatica. Jpn Clin Med. 2010; 1: 25-7. PMid: 23946678.

[12] Kidess AI, Caplan RH, Reynertson RH, et al. Transient Corticotropin deficiency in Critical illness. Mayo Clinic proceedings. https://doi.org/article/S0025-6196(12)60188-8

[13] Quintos JB, Boney CM. Transient adrenal insufficiency in the premature newborn. Curr Opin Endocrinol Diabetes Obes. 2010; 17(1): 8-12. PMid: 19881342. https://doi.org/10.1097/MED.0b013e32833363cc

[14] Alfitori G, Mahmood M, Malik S. Transient secondary hypoadrenalism after a single intra-articular steroid injection-a case report. Endocrine Abstracts. 2009; 19: 89.

[15] Matsuoka Y, Furuyashiki T, Bito H, et al. Impaired adrenocorti-cotropic hormone response to bacterial endotoxin in mice deficient in prostaglandin E receptor EP1 and EP3 subtypes. Proceeding of the National Academy of Science of the United States of America PNAS. 2003; 4132-7. https://doi.org/10.1073/pnas.0633341100

[16] Yoshida M, Murakami M, Ogawa K, et al. Repeated hypoglycemia caused by the overproduction of anti-insulin antibodies and isolated ACTH deficiency in a type 2 diabetic patient receiving insulin therapy. Diabetes Care. 2013; 36(2): 22. https://doi.org/10:2337/dc12-1565.