Congenital adrenal hyperplasia with salt-wasting crisis and arrhythmia: a case study

BMJ Case Rep. 2019 Jan 29;12(1):e227565. doi: 10.1136/bcr-2018-227565.

Abstract

We report a case of a 10-day-old male infant who presented to the emergency department with severe electrolyte imbalance and life-threatening arrhythmia. The parents reported a 3-day history of poor feeding and lethargy. On examination, he was bradycardic (heart rate of 65 beats/min) with signs of dehydration. His ECG showed broad complex bradycardia. Blood gas showed metabolic acidosis with hyponatraemia and hyperkalaemia. A probable diagnosis of congenital adrenal hyperplasia (CAH) with salt-wasting crisis was made and treatment was commenced. He was given saline bolus, nebulised salbutamol, calcium gluconate and hydrocortisone. Following the above interventions, his heart rate rose to 150 beats/min with a regular sinus rhythm within a period of 40 min. The diagnosis of CAH secondary to 21-hydroxylase deficiency with mutation in CYP21A2 was confirmed by genetic studies. He was discharged home with hydrocortisone, fludrocortisone and sodium chloride.

Keywords: endocrine system; paediatrics (drugs and medicines).

Publication types

  • Case Reports

MeSH terms

  • Adrenal Hyperplasia, Congenital / complications*
  • Adrenal Hyperplasia, Congenital / diagnosis
  • Adrenal Hyperplasia, Congenital / therapy
  • Anti-Inflammatory Agents / therapeutic use
  • Arrhythmias, Cardiac / complications*
  • Arrhythmias, Cardiac / diagnosis
  • Arrhythmias, Cardiac / therapy
  • Diagnosis, Differential
  • Fludrocortisone / therapeutic use
  • Humans
  • Hydrocortisone / therapeutic use
  • Infant, Newborn
  • Male
  • Sodium Chloride / therapeutic use
  • Water-Electrolyte Imbalance / complications*
  • Water-Electrolyte Imbalance / diagnosis
  • Water-Electrolyte Imbalance / therapy

Substances

  • Anti-Inflammatory Agents
  • Sodium Chloride
  • Fludrocortisone
  • Hydrocortisone

Supplementary concepts

  • Congenital adrenal hyperplasia due to 21 hydroxylase deficiency