Introduction: Recurrent hypoglycemia is a life threatening condition. And its early diagnosis and correct diagnosis can be crucial to entail survival of the child.
Objectives: To review this case with respect to the clinical scenario and critical pathways we should remember while investigating a case of recurrent hypoglycemia.
Methods: History: A 5 year old, Pakistani origin, male child presented to the Pediatric endocrine clinic with chief complaint of recurrent hypoglycemia. Patient had presented to Emergency room with an episode of documented hypoglycemia( blood sugar 2.2 mmol/l).Patient was given some IV fluids, stabilised and sent home without any critical sample saved. There was past history of documented recurrent hypoglycemia associated with high fever, vomiting, diarrhea.There was also history of sweating episodes.Child was first born, product of non-consanguineous marriage, full term with average birth weight. There was history of neonatal intensive care stay for pneumonia and respiratory distress with h/o one episode of convulsion related with hypoglycemia. There was a positive history of diagnosed with Dextrocardia and Situs inversus.The patient had been apparently evaluated previously by Pediatricians at various hospitals locally and in their home country for hypoglycemia including extensively for hyperinsulinemia ( but never correlated).The parents had been even given glucometer to keep a measure of glucose levels and instructions for taking sugar in case of hypoglycemia. He was also diagnosed as subclinical hypothyroid lately and had been prescribed thyroxine supplements. He had been also diagnosed with autism because of poor school performance and poor attention span.
Clinical examination: Anthropometry -Weight at 10th percentile and Height at 50th percentile.General examination revealed alert child with a sallow complexion, frequent eye blinking because of dry eyes, dark greyish discoloration of around gums. Mild to moderate development delay (predominant speech) was also noted. Rest of the systemic exam was normal other than the systemic finding related to situs inversus and dextrocardia.
The labs were ordered including fasting sugars, insulin and complete hormonal panel.The significant results showed ACTH of 425 pmol/l (1.6-13.9) and nearly undetectable cortisol levels at 0.5 nmol/L(171-536).CT scan adrenal showed relatively thinned out (possibly atrophic ) adrenal glands with tiny right calcific focus with situs inversus totalis.
The child was diagnosed as a case of primary adrenocortical insuffiency and started immediately on age and body surface area appropriate dose of hydrocortisone. Further investigations,other relevant findings and management challenges for such a case will be discussed.
19 Sep 2019 - 21 Sep 2019