MosaicED Brain Teaser!
You're on your second shift as a paediatric resident in the ED. Your palms are sweaty, knees weak, stethoscopes heavy. There's vomit on your sweater already, from some kids belly. A one week old boy is brought in by his parents. He looks unwell and dehydrated. His parents tell you that he vomited twice this morning and once last night.
On examination he has a sunken fontanelle and is crying but not producing any tears. His scrotum in darker than the rest of his body and you think his penis is bigger than normal, or is yours smaller?
What is the immediate treatment of this boy?
- A-Air enema
- B-Emergency surgery
- D-IM adrenalin
- E-IM benzyl penicillin
- F-IM insulin
- G-IV immunoglobulins
- H-IV vancomycin and ceftriaxone
- I-Nebulized salbutamol
What is the long term treatment of this boy?
- B-Dietary advice
- C-Daily insulin
- D-Hydrocortisone and fluticosone
- E-Trigger avoidance
- F-Reliever inhaler
- G-RIPE therapy
Answers:K-Rehydration and D-Hydrocortisone and fluticosone. This baby is dehydrated and needs rehydration immediately, 10-20ml/kilo NaCl. He is having a salt crisis as he has congenital adrenal hyperplasia. It presents with a salt crisis; an unwell, lethargic vomiting, and dehydrated baby. It is due to 21 hydroxylase deficiency which means no aldosterone or cortisone is produced. Females with it have ambiguous genitalia and males have a big penis and hyperpigmented scrotum. It will also cause early puberty. Investigations will show very low salt, high potassium, low BSL, and metabolic acidosis. Treatment is hydrocortisone (glucocorticoids replacement) and fluticasone (mineralocorticoids replacement) for life.