Isolated Adrenocorticotropin Deficiency as a Rare Cause of Hypoglycaemia in Children

Issa

2016

CRCP

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.

Section: Discussionmentioning

confidence: 97%

“…[1] The defect is usually at the pituitary level but can be at the hypothalamus. [2] Most cases are secondary to autoimmune process such lymphocytic hypophysitis. [3,4] It may also occur after traumatic head injury [5] and pituitary irradiation.…”

Section: Introductionmentioning

confidence: 99%

Transient Adenocorticotropic Hormone deficiency in an infant

Issa

2016

CRCP

“…[2][3][4][5][6][7][8][9][10][14][15][16] Insulin Tolerance Test (ITT) is considered the gold standard test to evaluate the hypothalamic pituitary adrenal (HPA), but has its disadvantages in clinical practice and that is the reason we prefer to the synacthen test, in most cases. 17 The patients presented here 18,19 were diagnosed as having isolated Adrenocorticotrophic Hormone (ACTH) deficiency according to the typical endocrinological findings as well as hormonal revaluation after a sufficient time interval on replacement therapy. The possible path physiologic mechanisms leading to this is poorly understood.…”

Section: Discussionmentioning

confidence: 99%

Isolated Adrenocorticotropic Hormone Deficiency not Always Permanent

Jurayyan¹

2017

ICPJL

Isolated Adrenocorticotrophic hormone (ACTH) deficiency is rare but could be a lifethreatening condition characterized by hypocortisolism. Most causes are secondary to autoimmune process such as lymphocytic hypophysitis. It may also occur after traumatic brain injury, tumors, pituitary irradiation and ischemic brain insult. Genetic causes are not infrequent. We present two causes of Isolated Adrenocorticotrophic Hormone (ACTH) deficiency who experienced different outcomes and highlight the importance of understanding the various possible aetio-pathophysiology.

Clin Pediatr Endocrinol

“…Finally, control of hypoglycemia is necessary to prevent severe ACs in young children less than 5 yr of age. There have been several reports of children with primary or secondary adrenal insufficiency presenting severe ACs with hypoglycemia (4,5,6,7,8,9,10,11,12), and some of these crises resulted in death (6, 10,11,12). We recommend measurement of the patient’s glucose level at home, especially in the morning (12), in the same manner as is now widely performed in patients with type1 diabetes mellitus.…”

Section: Discussionmentioning

confidence: 99%

Stress Doses of Glucocorticoids Cannot Prevent Progression of All Adrenal Crises

Aso

Izawa

Higuchi

et al. 2009

Adrenal crises (ACs) sometimes progress rapidly and can be fatal. The aims of the present study were to reveal whether stress doses of glucocorticoids (SDGs) can prevent progression of severe ACs and to suggest a method of prevention, through analysis of its clinical features. We studied 24 severe ACs (nine patients) that occurred after diagnosis of primary or secondary adrenal insufficiency, retrospectively. The following information was analyzed: 1) whether SDGs were given orally and/or sc; 2) duration from the time when some symptoms started to the time when the patient came to the hospital; and 3) presence of hypoglycemia and electrolyte disturbance (hyponatremia, hyperkalemia). Eleven crises occurred after taking SDGs. Ten crises progressed within 3 h. Six of these ten crises progressed to severe ACs despite the fact that the patients took SDGs. Six crises were observed in association with hypoglycemia, and five of these six crises occurred in patients under 5 yr of age. Three of the six crises in association with hypoglycemia progressed to ACs within 3 h. Two of the three crises progressed to severe status within 3 h despite the fact that the patients took SDGs. Electrolyte disturbance was observed in only one crisis. In conclusion, SDGs cannot prevent progression of all ACs. Progression can be associated with hypoglycemia, particularly in patients under 5 yr of age. Patients should be given guidance on an ongoing basis on how to prevent ACs and hypoglycemia.