logo
episode-header-image
May 13
1h 18m

FF 80 KDIGO ADPKD Guidelines

NephJC Team
About this episode

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 ( NEJM | NephJC )

Unified ultrasonographic diagnostic criteria for polycystic kidney disease by Edelstein in JASN (PubMed)

Tolvaptan and Kidney Function Decline in Older Individuals With Autosomal Dominant Polycystic Kidney Disease: A Pooled Analysis of Randomized Clinical Trials and Observational Studies (PubMed)

Charles’ draft choice Recommendation 4.1.1.1: We recommend initiating tolvaptan treatment in adults with ADPKD with an estimated glomerular filtration rate (eGFR) ‡25 ml/min per 1.73 m2 who are at risk for rapidly progressive disease (1B).

Sophia’s draft choice Recommendation 1.4.2.1: We recommend employing the Mayo Imaging Classi cation (MIC) to predict future decline in kidney function and the timing of kidney failure (1B).

Progression to kidney failure in ADPKD: the PROPKD score underestimates the risk assessed by the Mayo imaging classification (Frontiers of Science)

AC’s draft choice Recommendation 9.2.1: We recommend targeting BP to ≤ 50th percentile for age, sex, and height or ≤ 110/70 mm Hg in adolescents in the setting of ADPKD and high BP (1D).

HALT-PKD Blood Pressure in Early Autosomal Dominant Polycystic Kidney Disease (NEJM)

Nayan’s draft choice Recommendation 6.1.2: We recommend screening for ICA in people with ADPKD and a personal history of SAH or a positive family history of ICA, SAH, or unexplained sudden death in those eligible for treatment and who have a reasonable life expectancy (1D).

Screening for Intracranial Aneurysms in Patients with Autosomal Dominant Polycystic Kidney Disease (CJASN)

Surgical Clipping Versus Endovascular Coiling in the Management of Intracranial Aneurysms (PubMed) Clipping is associated with a higher rate of occlusion of the aneurysm and lower rates of residual and recurrent aneurysms, whereas coiling is associated with lower morbidity and mortality and a better postoperative course.

Joel’s editorial pick Recommendation 6.1.1: We recommend informing adults with ADPKD about the increased risk for intracranial aneurysms (ICAs) and subarachnoid hemorrhage (1C).

Joel’s first draft pick The bring out your dead pick:

Recommendation 4.3.1: We recommend not using mammalian target of rapamycin (mTOR) inhibitors to slow kidney disease progression in people with ADPKD (1C).

Recommendation 4.4.1: We suggest not using statins specfiically to slow kidney disease progression in people with ADPKD (2D).

Recommendation 4.5.1: We recommend not using metformin specifically to slow the rate of disease progression in people with ADPKD who do not have diabetes (1B).

Recommendation 4.6.1: We suggest that somatostatin analogues should not be prescribed for the sole purpose of decreasing eGFR decline in people with ADPKD (2B).

Perfect match: mTOR inhibitors and tuberous sclerosis complex (Orphanet Journal of Rare Diseases)

Navitor Pharmaceuticals Announces Janssen Has Acquired Anakuria Therapeutics, Inc. (BioSpace) This is press release about acquiring the mTor1 inhibitor.

Joel’s second draft pick Recommendation 4.2.1.1: We suggest adapting water intake, spread throughout the day, to achieve at least 2–3 liters of water intake per day in people with ADPKD and an eGFR ≥ 30 ml/min per 1.73 m2 without contraindications to excreting a solute load (2D).

Nayan’s bonus draft Practice Point 4.7.1: Sodium-glucose cotransporter-2 inhibitors (SGLT2i) should not be used to slow eGFR decline in people with ADPKD.

Open-Label, Randomized, Controlled, Crossover Trial on the Effect of Dapagliflozin in Patients With ADPKD Receiving Tolvaptan (KIReports)

SMART Trial of GLP-1ra in non-diabetics: Semaglutide in patients with overweight or obesity and chronic kidney disease without diabetes: a randomized double-blind placebo-controlled clinical trial (PubMed)

Tubular Secretions

Nayan: Landman on Paramount Plus (IMDB)

Sophia: Pass

Nayan: steps in with The Pitt on HBO (Wikipedia)

Charles: The White Lotus, Yellowstone 1923, Poirot (IMDB)

AC: The Pitt

Michael Crichton’s Estate Sends The Pitt to the Courtroom (Vulture)

Joel: I Must Betray you by Ruta Sepetys (Amazon)

Up next
Jun 9
NephJC RoboPod Episode 1: The Stamp Act Tac v MMF in Pediatric Nephrotic Syndrome
One of NephJC signature moves has been to adapt new technologies and communication techniques to delivering state of the art nephrology education. Podcasts, Visual Abstracts, Tweetorials. NephJC has been on the front of these waves. Clearly one of the most exciting and controvers ... Show More
19m 27s
Apr 22
FF 79 REGENCY, Efficacy and Safety of Obinutuzumab in Active Lupus Nephritis
The Filtered Fragments (OG Filtrate)Joel TopfJennie LinSwapnil HiremathSpecial Guest Brad Rovin GN God and second author from The Ohio StateKoyal Jain GN Specialist from UNCAlfred Kim Rheumatologist from Washington UniversityEditing bySimon Topf and Nayan AroraThe Kidney Connecti ... Show More
1h 42m
Mar 2025
FF 78 NephMadness, the dialysis region
The FiltrateJoel TopfJordy CohenNayan AroraSpecial Guest Katie Kwon @katiekwonmd.bsky.socialMariana Murea @MarianaMureaEditing bySimon Topf and Joel TopfShow NotesNephMadness at AJKDblog.orgThe Hemodialysis RegionVote for your favorites 
1h 8m
Recommended Episodes
Jul 2023
Prof. John Myburgh talks Fluids, Catecholamines, South Africa and a little bit of Research.
In this episode, we have the honor of speaking with Professor John Myburgh, a former South African physician and researcher who has dedicated his life to improving the field of critical care medicine. Professor Myburgh has made significant contributions to the field of fluid resu ... Show More
33m 24s
Jun 2024
Episode 906: Case Study of Hypernatremia
Contributor: Aaron Lessen MD Educational Pearls: The case: A gentleman came in from a nursing home with symptoms concerning for sepsis. He was hypotensive, hypoxic, febrile, and mentally altered. His past medical history included previous strokes which had left him with deficits ... Show More
3m 46s
Jul 2021
COVID-19: Vaccine immunogenicity, interleukin-6 inhibitors, mucormycosis
Kieran Walsh, clinical director at BMJ, asks editors from BMJ Learning and BMJ Best Practice to summarise the latest clinical guidance related to Covid-19. Abigail Davis, GP and section editor, covers the latest evidence around vaccine immunogenicity in different immunosuppressed ... Show More
12m 6s
Jul 2024
Acute Kidney Injury; Roadside to Resus
Acute Kidney Injury is common, complicated and holds significant morbidity and mortality. But...if we recognise it, we can make a real difference to our patients' outcomes. In this episode we run through the anatomy, physiology and aetiologies. We have a think about the multitude ... Show More
59m 55s
Jul 2024
Jul 19 2024 This Week in Cardiology
Vulnerable plaque and scientific method; industry payments to trainees; tirzepatide or semaglutide; trial interpretation; and PFA are the topics John Mandrola, MD, covers in today’s podcast. This podcast is intended for healthcare professionals only. To read a partial transcript ... Show More
25m 26s
Dec 2023
Dec 01 2023 This Week in Cardiology
More from AHA including SGLT2 inhibitors, blood transfusion after MI; cracks in the armor of Factor XI inhibitors; and renal denervation are the topics John Mandrola, MD, covers in this week’s podcast. This podcast is intended for healthcare professionals only. To read a partial ... Show More
27m 48s
Mar 2025
Therapeutic Interventions for Autoimmune Retinopathy
Dr. Edmund Tsui is joined by Dr. Majda Hadziahmetovic to discuss her meta-analysis and systematic review investigating the effectiveness of systemic and local treatments in slowing autoimmune retinopathy progression. From the Ophthalmology Science article, “Clinical Outcomes of T ... Show More
21m 1s
Oct 2023
S2 Episode 5: When Is It Time to Talk About Kidney Transplantation?
Join experts Drs Matthew Sparks and Samira Farouk as they pull back the curtain to help patients and providers better understand the transplant journey. Relevant disclosures can be found with the episode show notes on Medscape (https://www.medscape.com/viewarticle/991605). The to ... Show More
28m 50s
Aug 2024
DKA Management: Balanced Electrolyte Solutions vs. Saline
In this episode of the Saving Lives Podcast, Eddy Joe delves into the latest research comparing balanced electrolyte solutions to 0.9% saline for treating diabetic ketoacidosis (DKA). Discover how BES could potentially revolutionize DKA management by speeding up recovery and redu ... Show More
9m 7s