Congenital Hyperinsulinism – Board-Style Questions

What this deck covers

congenital hyperinsulinism in 18 board-style clinical cases with worked explanations. Each case gives you a clinical vignette, four options, the correct answer, and an explanation of why each of the other three is wrong, with the guideline or textbook it follows named on the answer.

Board-style teaching questions for paediatricians and trainees preparing for paediatric board, MRCPCH and licensing examinations. Written from published guidelines and standard paediatric textbooks, with the source named on every answer.

The 18 cases, in order

1. More sugar than any baby needs
2. A result that looks normal
3. Glucose that was hidden
4. How low is too low
5. Breathing harder on the drug
6. When the first drug fails
7. Looking for one spot
8. One spot, one operation
9. What the operation costs
10. Feeding the problem
11. After a protein meal
12. A small baby’s high insulin
13. After the wrap
14. A teenager with new lows
15. Watching the brain grow
16. Still needed?
17. The next baby
18. At home, at night

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: Poor feeding that has not yet been properly corrected, Inappropriate, as secretion should have stopped, His liver has no stored glycogen left to release, The same as any healthy newborn, A heart scan, as diazoxide can raise lung pressures, A higher dose of diazoxide than the maximum, A contrast CT scan of the whole abdomen and pelvis, Removing the lesion, which can cure the disease, Her growth will be permanently slowed, Stop all feeds and rely on the glucose drip, A urea cycle disorder with liver failure, It usually settles over weeks to months, Congenital hyperinsulinism appearing late, Ketotic hypoglycaemia of childhood.

Clinical pearls from this deck

• A glucose need of eight or more per kilogram per minute: think insulin.
• Hypoglycaemia with any insulin at all: the insulin is the problem.
• Glucose jumps after glucagon at a low: insulin was holding it back.
• Hyperinsulinism: keep the glucose higher, the brain has no backup.
• Breathless on diazoxide: scan the heart for pulmonary hypertension.
• Diazoxide fails: add octreotide.

Work through the deck once for recognition, then again a week later to test yourself — each question gives the answer and explains why every other option fails.

More from this system: all Endocrinology Board Questions case decks · pediatric reference values.