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Polycystic Liver Disease — ESENeph MCQ

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HardPolycystic Kidney DiseasePolycystic Liver DiseaseESENeph

A 38-year-old with ADPKD has troublesome abdominal fullness from diffuse polycystic liver disease but preserved kidney function. Which first specialist assessment is most appropriate?

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Correct answer: ADefine whether dominant cysts or diffuse hepatomegaly cause symptoms

The best answer is “Define whether dominant cysts or diffuse hepatomegaly cause symptoms”. Management depends on anatomy and burden: a dominant accessible cyst, segmental disease and diffuse massive hepatomegaly lead to different interventional, medical and transplant pathways. “Use tolvaptan when rapidly progressive renal ADPKD also qualifies” is less appropriate because its licensed disease-modifying indication concerns renal ADPKD progression, not liver-volume treatment “Aspirate a dominant symptomatic cyst after anatomical assessment” is less appropriate because aspiration is suited to selected dominant cysts and cannot treat diffuse disease this way “Refer for transplant assessment when severe diffuse disease warrants it” is less appropriate because transplantation is reserved for severe refractory disease “Use supportive management when symptoms and complications remain limited” is less appropriate because mass effect can materially impair nutrition and quality of life

Reference: KDIGO 2024 CKD guideline: https://kdigo.org/wp-content/uploads/2024/03/KDIGO-2024-CKD-Guideline.pdf