2) This #accredited #tweetorial on #ADPKD and assessment of risk progression with emphasis on imaging is supported by an independent educational grant from Otsuka Pharmaceuticals and is intended for healthcare providers. YOU CAN EARN 1β£HOUR CREDIT WITH THIS PROGRAM!
β @CKD_ce (@ckd_ce) May 31, 2022
4a) Let’s start with a case or rather a family since #ADPKD is a #familyaffair!
β @CKD_ce (@ckd_ce) May 31, 2022
You are seeing in clinic 2 brothers, 28 and 32 y.o., who have a strong family history of #ADPKD. Both their maternal GF and mother have ADPKD and reached kidney failure at age 48 & 54 respectively.
5) The two brothers ask you how to interpret their test results. What do YOU think?
β @CKD_ce (@ckd_ce) May 31, 2022
a. Both brothers have ADPKD
b. Neither has ADPKD
c. The younger brother has ADPKD and the older brother does not have ADPKD
d. Cannot provide answers without genetic testing
6b) #ADPKD is a clinical diagnosis based on the family history and radiological findings. The performance of ultrasound-based unified criteria for diagnosis or exclusion of ADPKD is summarized here (from πhttps://t.co/3baZZsvOuF): pic.twitter.com/5QZtX8oBMR
β @CKD_ce (@ckd_ce) May 31, 2022
7a) Genetic testing is required in select scenarios as summarized in this proposed flowchart for diagnosis of #APDKD @ecorneclegall @thelancet (https://t.co/Mm9xOKTJFm)
β @CKD_ce (@ckd_ce) May 31, 2022
Arguably, as accessibility to genetic testing increases, it will become standard of care . . .
8a) The older brother proceeds successfully to donate a kidney to their mother after confirming he has no cysts on CT abdomen. The younger brother has additional questions for you as you have confirmed he has #ADPKD. He asks you if you could predict his genotype …
β @CKD_ce (@ckd_ce) May 31, 2022
9a) The answer is that family history of renal disease severity predicts the mutated gene in ADPKD. The presence of at least one affected family member who developed ESKD at age β€55 was highly predictive of a PKD1 mutation (positive predictive value 100%; sensitivity 72%).
β @CKD_ce (@ckd_ce) May 31, 2022
10a) Now the younger brother is asking you if he would reach kidney failure at an age similar to his mother or grandfather. His current creatinine is 0.8 mg/dL with eGFR >90 ml/min/1.73m2. He would really like to know as planning his life will hinge on this important information.
β @CKD_ce (@ckd_ce) May 31, 2022
11) Mark your best answer & return TOMORROW for the correct answer & more intensive education on #ADPKD!
β @CKD_ce (@ckd_ce) May 31, 2022
π@BasuNephro @dr_nikhilshah @shubharthikar @Elektra @kidneydoc101 @NephRodby @edgarvlermamd @FLAVIAZH @dguerrot @KorstUwe @kkalra_22 @AlanYuNeph @GlassockJ @nephJC @ndahl48
13a) Yesterday’s poll? Tweet 11? Did you answer?
β @CKD_ce (@ckd_ce) June 1, 2022
It’s 4: there is a large interfamilial variability, & each patient has his/her own prognosis. We will explore how to assess the risk of progression and predict ESKD onset in later tweets.
14a) Interfamilial variability in ADPKD has been described in more than one cohort. In a large cohort of 1153 ADPKD patients (375 families), at least 12% of family members have discordant ADPKD presentation regardless of responsible mutation type. @MattLanktree
β @CKD_ce (@ckd_ce) June 1, 2022
14c) The interested reader is referred to
β @CKD_ce (@ckd_ce) June 1, 2022
πhttps://t.co/HJuuH76Wr1
πhttps://t.co/yjBvPbftli pic.twitter.com/8SAcMFPqRz
15b) Whatβs your view?
β @CKD_ce (@ckd_ce) June 1, 2022
1. Assessing disease progression in ADPKD is not very imp’t in tailoring tx plan
2. Rapid prog only exists in #Glomerulonephritis
3. Rapid prog defined as reaching ESKD by age 62
4. There are no tools to assess risk of rapid prog unless GFR starts toβ¬οΈ
16b) Others have considered different definitions such as reaching #CKD stage 3 before age 40, early onset #hypertension, or larger kidney volumes than expected for given age. pic.twitter.com/AER7o1rrae
β @CKD_ce (@ckd_ce) June 1, 2022
17b) I will share later which is considered the most practical with ability to individualize the prediction of ESKD onset. Pending that discussion, whatβs YOUR go-to method to assess risk of progression in ADPKD? Be sure to answer before scrolling β¬οΈ
β @CKD_ce (@ckd_ce) June 1, 2022
(cont)
18a) Going back to your patient, he asks you if the kidney ultrasound gives you enough information to predict his renal outcome. As a reminder, his kidney length on ultrasound was 18 cm. You tell him what?
β @CKD_ce (@ckd_ce) June 1, 2022
(cont)
19a) Letβs discuss the use of kidney ultrasound in assessment of risk of progression. This is particularly important as US is the most accessible and least expensive. An US kidney length >16.5 cm corelates with TKV>750 ml & predicts the development of CKD stage 3 in 8 years.
β @CKD_ce (@ckd_ce) June 1, 2022
19c) . . . especially given that the risk assessment has larger cost implications down the line. πhttps://t.co/3ZapWy8K6R and https://t.co/6wYz8v9VL1. pic.twitter.com/JE9TIZXZTY
β @CKD_ce (@ckd_ce) June 1, 2022
20b)
β @CKD_ce (@ckd_ce) June 1, 2022
1. You are at high risk of early progression to ESKD before age 60, PPV 90.9%
2. Your risk of evolution to kidney failure before age 60 is low, NPV 81.4%
3. We donβt have a tool to predict your risk based on the provided information
4. Your risk is indeterminate
21b) We discussed in earlier tweets the limitations of using the family history as the sole factor in determining risk of progression and how the large interfamilial variability limits the practical value of this marker. pic.twitter.com/s3WJOVkOlb
β @CKD_ce (@ckd_ce) June 1, 2022
23) You can calculate a #PROPKD score (Predicting Renal Outcome in Polycystic Kidney Disease Scoring System) to predict the likelihood of kidney failure before 60 years old, using the online tool at https://t.co/eKmusEaUdL pic.twitter.com/LnFYL4ijDN
β @CKD_ce (@ckd_ce) June 1, 2022
24b) You tell him that there is another way using Total Kidney Volume or #TKV. He is excited. You order an abdominal MRI and he returns for follow up. He really wants to know about all the hype around TKV. What can you tell him? (choices in 24c)
β @CKD_ce (@ckd_ce) June 1, 2022
24c part 2)
β @CKD_ce (@ckd_ce) June 1, 2022
3. Age-adjusted Ht-TKV estimates intrinsic rate of TKV growth; only 1 measurement needed
4. All of the Above
26) Welcome back. In today's closing session, we'll talk about the potential contribution of #MRI to predicting #rapidprogression of #ADPKD. I am @fouadchebib and we have videos to share! pic.twitter.com/tkrc6OGZ8o
— @CKD_ce (@ckd_ce) June 2, 2022
28) The #CRISP (Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease) study (https://t.co/rsamBteEaQ) provided additional support for a causal link between growth in kidney volume and GFR decrease. pic.twitter.com/3e6cf68OiM
— @CKD_ce (@ckd_ce) June 2, 2022
30) However, measurements using the ellipsoid equation are comparable to the gold standard method. See πhttps://t.co/KzZRgUdUVN, https://t.co/Mm9xOKTJFm and πhttps://t.co/SvReSPArZj. pic.twitter.com/DHTgGMconU
— @CKD_ce (@ckd_ce) June 2, 2022
32a) Do you have trouble convincing your radiology department to measure the TKV for you? Here is a way you can do this in your office through the ellipsoid equation. If unable to obtain sagittal length, substitute with coronal length. #empower #nephrologist
— @CKD_ce (@ckd_ce) June 2, 2022
(Part 1 of video) pic.twitter.com/9kL7D9Q0ww
33a) Each of the imaging modalities have its pros and cons. The advantages of MRI are no radiation, no need for contrast , & allows obtaining accurate TKV and advanced biomarkers (not yet implemented clinically).
— @CKD_ce (@ckd_ce) June 2, 2022
33c) What is your favorite imaging modality in #ADPKD? Please comment on your rationale
— @CKD_ce (@ckd_ce) June 2, 2022
1. Renal ultrasound anytime, all the time
2. Abdominal CT scans with contrast
3. Abdominal CT scans without contrast
4. Abdominal MRI
35a) After obtaining ht-TKV, it is essential to analyze the value in the context of age, #GFR and cystic burden, rather than blindly using htTKV as an absolute number. Age-adjusted #htTKV, represented by the Mayo Imaging Classification . . .
— @CKD_ce (@ckd_ce) June 2, 2022
36a) The Mayo imaging classification allows prediction of future eGFR & thus age of #ESKD onset. This is THE MOST important question that you should discuss in your clinic with the patient. Your patients might be pleasantly or unpleasantly surprised.
— @CKD_ce (@ckd_ce) June 2, 2022
37) Here is more information for those interested:
— @CKD_ce (@ckd_ce) June 2, 2022
πhttps://t.co/GqXrbL1BMV pic.twitter.com/Jk1SYez4A3
39) Keep in mind natural history of #ADPKD & other renal cystic disease. If cystic burden doesnβt fit renal function,π€ADPKD mimickers or comorbidities. ADPKD patients also get #DM, other nephropathies, GNs, medications induced nephrotoxicity, etchttps://t.co/6wYz8v9VL1 pic.twitter.com/9LO12GB3HO
— @CKD_ce (@ckd_ce) June 2, 2022
40b) Limitations included delay in treatment initiation, need for β₯3 TKV each β₯6 mo apart. Might not differentiate #PKD1 vs. #PKD2 and would not exclude atypical #ADPKD. pic.twitter.com/gSm1l55YWh
— @CKD_ce (@ckd_ce) June 2, 2022
41b) If you prefer video . . . pic.twitter.com/gN0EHYTb3y
— @CKD_ce (@ckd_ce) June 2, 2022
43) Take home message 1/5:
— @CKD_ce (@ckd_ce) June 2, 2022
Always confirm the diagnosis. #bilateral #kidneycysts does not always mean #ADPKD
45) Take home message 3/5
— @CKD_ce (@ckd_ce) June 2, 2022
Use all the practical tools available to you. You can predict onset of #ESKD in your office. #empower your patient with this knowledge!
47) Take home message 5/5
— @CKD_ce (@ckd_ce) June 2, 2022
Accurate diagnosis and prognosis have large implications on implementing disease-modifying treatments:
πhttps://t.co/GqXrbL1BMV pic.twitter.com/1FOD50CvYA
48) And that's it! You made it! You just earned 1β£ hour ofπCE/#CME. Go claim it at https://t.co/GCce6vQOkO and please FOLLOW @ckd_ce (and @cardiomet_ce!) for more education by the world's leading authorities! I am @fouadchebib and I thank you for joining us!
— @CKD_ce (@ckd_ce) June 2, 2022