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Dr PP Varma, New Delhi 29 December 2018

Rapidly progressive autosomal-dominant polycystic kidney disease (ADPKD) deserves treatment.
Assessment should be done with imaging and clinical data.

MRI based ADPKD diagnostic criteria: >10 renal cysts - ADPKD (<30 years); Total <10 renal cysts exclude ADPKD; Living kidney donors - Total <5 cysts; At-risk subjects <40 years of age with equivocal MRI findings (cysts 5-19), molecular testing.
USS diagnosis of ADPKD - Criteria for ADPKD1, at risk patients: <30 years: at least 2 cysts in 1 or 1 cyst in each kidney; 30-59 years: at least 2 cysts in each kidney; ≥60 years: at least 4 cysts in each kidney. Diagnostic criteria with family history, unknown genotype: 15-39 years: 3 or more cysts (unilateral/bilateral); 30-59 years: 2 or more cysts in each kidney.

Side effects are common with 15-20% dropout rate.
Monitor urine Osm, hyper/hyponatremia/LFT-ALT/AST/S bilirubin.
Indications of Tolvaptan: Japan: Documented 5% rate of TKV increase. No age or CKD restriction; Canada: Consensus recommendation of Nephrologists; European Union: Adults with CKD1 through CKD3. Evidence of rapidly progressive disease; USA: Adults with risk of rapidly progressive disease. Also approved in Switzerland, Australia, Hong Kong and Nordic countries.

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