Autosomal Recessive Polycystic Kidney Disease – Board-Style Questions

What this deck covers

Autosomal Recessive Polycystic Kidney Disease 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. What the scan predicts
2. Telling it apart
3. Breathing first
4. When the kidneys must come out
5. Salt going out
6. More than one drug
7. Why he is not growing
8. A well child with a big spleen
9. Fever from the bile ducts
10. Two organs, one operation
11. Making room
12. Rugby on Saturday
13. The next pregnancy
14. The well brother
15. The lungs that survived
16. What the family expect
17. The number that matters
18. Who follows him

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: The volume of amniotic fluid over the coming weeks, Several large discrete cysts of differing sizes, Respiratory support, since the lungs are the immediate threat, Continue ventilation and wait for the kidneys to shrink, Restrict his fluid intake in order to correct the low sodium, It usually settles on one drug once the dose is right, Assess him for growth hormone treatment in chronic disease, To biopsy the liver through the stomach wall, A urinary infection presenting with abdominal pain, A kidney transplant on its own, as the liver still functions, Because there is no room in the abdomen for the graft, Carry on as normal, since the spleen is not fragile, Reassurance, as lightning does not strike twice, No testing at all, as he is well and has no symptoms.

Clinical pearls from this deck

• The liquor is the lungs, and the lungs are the first week.
• Big and uniformly bright, with the cortex and medulla blurred together.
• They die of lungs, not of kidneys, in the first week.
• When the kidneys stop the chest from working, they come out.
• These kidneys leak salt: give salt, not water.
• Expect to need two or three drugs, and start early.

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 nephrology case decks · pediatric reference values.