1b) .@KidneyCathy, our famous international expert, is @UCDMedicine grad, trained @CHI_Ireland @GreatOrmondSt working @MCRI_for_kids @RCHMelbourne @UniMelbMDHS🇦🇺🇮🇪 pic.twitter.com/oicGVkqqoa
— @CKD_ce (@ckd_ce) March 9, 2023
3) This program is supported by an educational grant from Otsuka Pharmaceuticals & is intended for an audience of #healthcare providers. Statement of accreditation & faculty disclosures at https://t.co/PHlIppl6Yw.
— @CKD_ce (@ckd_ce) March 9, 2023
📢FOLLOW US for more programs by expert faculty! 🆓CE/#CME!
5) First off, how do we know a child has #ADPKD & do we need to confirm the diagnosis?
— @CKD_ce (@ckd_ce) March 9, 2023
👉As covered in my previous #tweetorial the diagnosis of #ADPKD can be via ultrasound or genetic testing. Either can rule in the dx. But a neg ultrasound in a child does NOT rule out #ADPKD.
7a) 🧬#Sanger sequencing of a gene of interest eg PKD1
— @CKD_ce (@ckd_ce) March 9, 2023
➕Quick
➕Specific
➕No risk of incidental findings
➕Affordable
➖If patient has an #ADPKD phenocopy–eg #PKD2, #GANAB, #IFT140, #DNAJB11–then this will be missed pic.twitter.com/KjN3u6iopo
🧬Genomic sequencing with analysis of a pre-defined list of genes
— @CKD_ce (@ckd_ce) March 9, 2023
➕Comprehensive
➕Limited risk of incidental findings
➕Future proof – can reanalyse
➖But if family variant is unknown then the result may be missed => negative test is not 💯https://t.co/sJNUe2wScp
9) Furthermore, @TheACMG guidelines for variant interpretation work best for patients with a phenotype as they assess the variant against #population, #computational, #functional, #segregation, #allelic and other data.https://t.co/oiqxmMMYsK
— @CKD_ce (@ckd_ce) March 9, 2023
11) 🛑 it’s also reasonable to ask whether you SHOULD be testing an asymptomatic, at-risk child. Since they are too young to consent to predictive testing for an adult-onset disease they should be reviewed in a multidisciplinary renal genetics clinic.https://t.co/wMNiMWbwHN pic.twitter.com/SBypzaqIQV
— @CKD_ce (@ckd_ce) March 9, 2023
13) The multidisciplinary renal genetic team will reframe the request as WHEN not WHETHER to test. Any predictive testing should be provided with genetic counselling support.https://t.co/JEAnxyp6QT pic.twitter.com/TplDOdc8oO
— @CKD_ce (@ckd_ce) March 9, 2023
15) What is the most important topic to discuss during the annual review of a teenager with #ADPKD and normal kidney function?
— @CKD_ce (@ckd_ce) March 9, 2023
Mark your best answer and RETURN TOMORROW for the correct response, more education, AND your 🔗to🆓CE/#CME!
17) Well, yesterday’s quiz was a trick question: ALL of these topics are important when reviewing a teenager with #ADPKD & normal kidney function! So settle in for part 2⃣ of this #tweetorial on non-clinical considerations in the management of children & young people with #ADPKD
— @CKD_ce (@ckd_ce) March 10, 2023
19) #Obesity is an established risk factor for the development and progression of #CKD. Higher #BMI is strongly and independently associated with the rate of progression in early-stage #ADPKD.
— @CKD_ce (@ckd_ce) March 10, 2023
See 🔓https://t.co/BSJSMzGAVV. pic.twitter.com/lKM34NpIti
21) Targeting prevention of the development of overweight and #obesity should be a core part of the annual review of each child at risk of #ADPKD.https://t.co/cThGKLcMeR
— @CKD_ce (@ckd_ce) March 10, 2023
23) Growing children should maintain age-appropriate dietary protein requirements though there is some evidence that plant-focused diets could be considered for adults with #ADPKD. 🔓 https://t.co/tJwVurzDOk
— @CKD_ce (@ckd_ce) March 10, 2023
25) While the evidence for #protein is shaky the evidence for #salt is strong. Kramers and colleagues followed 589 patients for 4 years and showed that 🧂intake was significantly associated with annual change in #eGFR while protein intake was not. pic.twitter.com/7JEuYX07R0
— @CKD_ce (@ckd_ce) March 10, 2023
27) We can't talk enough about #SALT: Americans eat an average of 3.4g of salt each day & the majority comes from processed foods rather than the salt we add at the table. https://t.co/dNn5FlMIuh pic.twitter.com/FSC9NrGfki
— @CKD_ce (@ckd_ce) March 10, 2023
29) No RCTs but post-hoc analysis of HALT A/B trials – each 1g /day ⬇️🧂associated w/ ⬇️kidney growth (just 0.43%/year) & a ⬇️rate of eGFR decline (-0.09ml/min/1.73m2/yr). Not a huge impact but worth educating family about 🧂 intake https://t.co/TADEolBDnC
— @CKD_ce (@ckd_ce) March 10, 2023
31) Until recently this had not been explored in an #RCT. @gopi_rangan randomised 184 patients to ad libitum or prescribed water aimed to reduce measured urine osmolality to 270 mOsmol/kg or less.
— @CKD_ce (@ckd_ce) March 10, 2023
Do I get bonus points for using Latin in a #tweetorial??
⭐️⭐️⭐️ pic.twitter.com/nIcu0G0riC
33) This is despite an average of 2.3L of water each day in treatment group w/ a mean 0.6L ⬆️ urine volume & 91 mOsmol/kg ⬇️urine osmolality w/in 12 weeks – sustained over 3 yrs. That’s some stellar patient education, individualised tx, & adherence right there. pic.twitter.com/jkC9xV0Psl
— @CKD_ce (@ckd_ce) March 10, 2023
35) Potential detrimental actions on the #kidneys include:
— @CKD_ce (@ckd_ce) March 10, 2023
🚬 lesions
🚬Inflammatory and pro-proliferative effects
🚬Redox imbalance
🚬#Albuminuria
🚬Decrease in renal plasma flow & #GFR
🚬Increase in #sympathetic activity & renal vascular resistance
37) In Australia 🇦🇺, the Generation #Vape study showed teenagers were readily accessing illegal #nicotine containing vapes – 32% have vaped & 80% got their last vape from their friends.
— @CKD_ce (@ckd_ce) March 10, 2023
🔓 https://t.co/KmPiV7g3p3
39) Talking about #ADPKD in a paed clinic feels different:
— @CKD_ce (@ckd_ce) March 10, 2023
❤️Parents may be contemplating further pregnancies
❤️Child/Teen considering future parenthood
❤️Parent w/ #ADPKD may be scared about their own future, struggling w/ feelings of guilt & scared about their parent's future pic.twitter.com/9dQFM6Zbx7
41) Stop now and refer to a reproductive genetic counsellor or your local renal genetics clinic if you feel out of your depth discussing reproductive options or if you need an update on the options available to your patients. pic.twitter.com/RYsw64UNcI
— @CKD_ce (@ckd_ce) March 10, 2023
43) to wit,
— @CKD_ce (@ckd_ce) March 10, 2023
1. Donor-assisted conception
2. #IVF w/ pre-implantation #genetic testing
3. Genetic testing in early #pregnancy w/ termination of affected pregnancy
4. #Adoption
*assuming these options are funded and legally available in your jurisdiction
45) Children & their families living w/ #ADPKD will need psychosocial support to help w/ the burden of this multi-system multi-generational disease.
— @CKD_ce (@ckd_ce) March 10, 2023
🔓https://t.co/H5YTJMQzgO pic.twitter.com/ab66WmwG2O
47) It's c, vaping/smoking. Lots of indirect evidence that nicotine exposure harms kidney health, but no direct association yet between 🚬and #ADPKD progression.
— @CKD_ce (@ckd_ce) March 10, 2023
So . . . let's close with a few 🔑practice points:
49) 2⃣ Educate and manage #lifestyle risks including #diet, #exercise, #smoking, #vaping, hydration, and #salt. pic.twitter.com/yVo4SyuybH
— @CKD_ce (@ckd_ce) March 10, 2023
51) 4⃣ Consider referring to a renal genetics clinic +/- involving a #genetic_counsellor in these discussions.
— @CKD_ce (@ckd_ce) March 10, 2023
See 🔓 https://t.co/wMNiMWbwHN pic.twitter.com/t1A0QySJDz
53) and just like that . . . you have earned 0.75hr 🆓CE/#CME! Point your 🖱️to https://t.co/jo7fi46YSN and claim your certificate. I am @KidneyCathy and I so appreciate your following this 🧵. Please CONTINUE following @ckd_ce for more CE/#CME from expert authors–always 🆓!
— @CKD_ce (@ckd_ce) March 10, 2023