FF 80 KDIGO ADPKD Guidelines

The Filtrate

Joel Topf

AC Gomez

Sophia Ambruso

Nayan Arora

Special Guest

Charles Edelstein, MD, PhD Professor, Medicine-Renal Med Diseases/Hypertension

Extra-Special Guest

Michelle Rheault, MD Professor of Pediatrics, University of Minnesota

Editing by

Simon and Joel Topf

The Kidney Connection written and performed by by Tim Yau

Show Notes

KDIGO ADPKD Guidelines:

Website

Guideline PDF

Executive Summary PDF

NephJC coverage

Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP)

Hy’s Law (Wikipedia) has three components:

ALT or AST by 3-fold or greater above the upper limit of normal

And total serum bilirubin of greater than 2× the upper limit of normal, without findings of cholestasis (defined as serum alkaline phosphatase activity less than 2× the upper limit of normal)

And no other reason can be found to explain the combination of increased aminotransferase and serum total bilirubin, such as viral hepatitis, alcohol abuse, ischemia, preexisting liver disease, or another drug capable of causing the observed injury

Meeting this definition yields a very high risk of fulminant kidney failure (76% in one series)

Clinical Pattern of Tolvaptan-Associated Liver Injury in Subjects with Autosomal Dominant Polycystic Kidney Disease: Analysis of Clinical Trials Database (PubMed) Two of 957 patients on tolvaptan met Hy’s law criteria. None had fulminant kidney failure.

Effects of Hydrochlorothiazide and Metformin on Aquaresis and Nephroprotection by a Vasopressin V2 Receptor Antagonist in ADPKD: A Randomized Crossover Trial (PubMed) Patients had a baseline urine volume on tolvaptan of 6.9 L/24 h. Urine volume decreased to 5.1 L/24 h with hydrochlorothiazide and to 5.4 L/24 h on metformin.

TEMPO 3:4 Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease (NEJM)

Reprise Trial Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease ( .css-j9qmi7{display:-webkit-box;display:-webkit-flex;display:-ms-flexbox;display:flex;-webkit-flex-direction:row;-ms-flex-direction:row;flex-direction:row;font-weight:700;margin-bottom:1rem;margin-top:2.8rem;width:100%;-webkit-box-pack:start;-ms-flex-pack:start;-webkit-justify-content:start;justify-content:start;padding-left:5rem;}@media only screen and (max-width: 599px){.css-j9qmi7{padding-left:0;-webkit-box-pack:center;-ms-flex-pack:center;-webkit-justify-content:center;justify-content:center;}}.css-j9qmi7 svg{fill:#27292D;}.css-j9qmi7 .eagfbvw0{-webkit-align-items:center;-webkit-box-align:center;-ms-flex-align:center;align-items:center;color:#27292D;}