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AL Amyloidosis Cardiac Staging — ESENeph MCQ

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ModerateChronic Kidney DiseaseAL Amyloidosis Cardiac StagingESENeph

A 45-year-old man presents with AKI (creatinine 400 umol/L) and nephrotic-range proteinuria. He has a known paraprotein (IgG lambda). Kidney biopsy shows Congo red-positive deposits with apple-green birefringence. Amyloid typing confirms AL (lambda). What cardiac investigation is essential?

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Correct answer: B — Echocardiography and NT-proBNP – cardiac amyloidosis must be assessed as it determines prognosis and treatment eligibility

AL amyloidosis is a systemic disease, and cardiac involvement is the most important determinant of prognosis. Up to 70% of AL amyloidosis patients have cardiac involvement at diagnosis. Echocardiography (looking for increased wall thickness, diastolic dysfunction, granular sparkling) and NT-proBNP/troponin are essential staging investigations. The Mayo 2004/2012 staging systems use NT-proBNP, troponin, and free light chain difference to stratify prognosis and guide treatment intensity.

Reference: BSH 2020 – AL Amyloidosis; Mayo 2012 Staging System