2) Prior #tweetorials about #ADPKD, still available for CE/#CME credit, can be found at https://t.co/Uuliqfop4x and feature expert authors @fouadchebib, @KidneyCathy, @edgarvlermamd, and @dguerrot. FOLLOW US for more expert #MedEd in #kidneydisease.#FOAMed @MedTweetorials pic.twitter.com/GLAUXu9kEQ
— @CKD_ce (@ckd_ce) September 5, 2023
4a) Now, on to #ADPKD. Let's start with some #MedHistory. Recognition of #PKD begins with the death of Polish King Stephen Bathory in 1586. He died at the age of 53 years.
— @CKD_ce (@ckd_ce) September 5, 2023
5) #PKD incidence:
— @CKD_ce (@ckd_ce) September 5, 2023
1 in 1000 live births
Responsible for 10% of #ESKD
Affected #genes:
Chromosome 16 (#PKD1 locus)- 78%
Chromosome 4 (#PKD2 locus)- 14%#GANAB#ALG9#DNAJB11
7) Clinical features:
— @CKD_ce (@ckd_ce) September 5, 2023
often asymptomatic with kidney #cysts detected by USG#HTN#hematuria#proteinuria#kidney function impairment
Flank pain
Median age of onset of #ESKD#PKD1 – 54 years#PKD2 – 74 years
9a) When to suspect #ADPKD ?
— @CKD_ce (@ckd_ce) September 5, 2023
Clinical features + #FamHx of ADPKD
Diagnosis is confirmed with USG & family h/o ADPKD
9c)
— @CKD_ce (@ckd_ce) September 5, 2023
๐ซ sporadic #PKD w/o FamHx
๐ซ early severe PKD
๐ซ PKD + syndromic features
๐ซ Reproductive counseling
11) If the results of #USG are equivocal, #MRI may be performed because of its superior sensitivity.
— @CKD_ce (@ckd_ce) September 5, 2023
Some experts perform genetic testing to confirm the diagnosis, if available, as an alternative to follow-up imaging with an MRI. pic.twitter.com/U90HIcNUn4
13a) Dx in pts w/o #FamHx #ADPKD:
— @CKD_ce (@ckd_ce) September 5, 2023
10 or > cysts (>5mm) in each kidney, esp if kidneys are enlarged & liver cysts are present.#TKV is an accurate estimate of kidney cyst burden & correlates to pain, hypertension, gross hematuria, proteinuria/albuminuria & loss of kidney function.
14) Identification of high risk pts:
— @CKD_ce (@ckd_ce) September 5, 2023
The #Mayo classification provides an age-adjusted assessment of progression risk:
Classes 1C, 1D & 1E are defined as high risk for progression to #ESKDhttps://t.co/ywkm4DLi5z pic.twitter.com/UabgYk9IQo
16) Management of blood pressure:
— @CKD_ce (@ckd_ce) September 5, 2023
First-line anti-hypertensives – #ACEI or #ARBs
Salt ๐งrestriction <5g/day, Na <2g/day
18a) #Tolvaptan:
— @CKD_ce (@ckd_ce) September 5, 2023
Vasopressin V2-receptor (V2R) antagonist
Indications:
๐ซ Adult patients with #ADPKD who have #eGFR โฅ25 mL/min1.73 m2 & who are at risk of rapid progression, defined by at least one of the following (cont):
19) Side effects of #tolvaptan:
— @CKD_ce (@ckd_ce) September 5, 2023
Thirst โ 4 to 55 percent
Polyuria โ 5 to 38 percent
Nocturia โ 5 to 29 percent
Polydipsia โ 2 to 10 percent
Hypernatremia โ 1 to 4 percent
Increased liver enzymes โ 1 to 6 percent
21a) Indications for intracranial #aneurysm #ICA screening in patients with good life expectancy:
— @CKD_ce (@ckd_ce) September 5, 2023
โ family history of ICA or subarachnoid hemorrhage,
โ previous ICA rupture,
โ high-risk professions (e.g., airline pilots)
โ patient anxiety despite adequate information.
22a) What are the #reproductive issues of #ADPKD?
— @CKD_ce (@ckd_ce) September 5, 2023
โ potential aggravation of polycystic liver disease (PLD) with exogenous estrogen or progesterone exposure
โ Development of Pregnancy-induced hypertension and preeclampsia
(cont)
23) Indications for #nephrectomy: pic.twitter.com/Sd1sHtkZqN
— @CKD_ce (@ckd_ce) September 5, 2023
25a) What have you learned?
— @CKD_ce (@ckd_ce) September 5, 2023
Which of the following is the most common genetic lesion identified in #ADPKD?
25c) Itโs c, a nontruncating #PD1 mutation. Hereโs a reminder: pic.twitter.com/3kEk37l6bV
— @CKD_ce (@ckd_ce) September 5, 2023
26) And you just earned 0.5hr ๐ CE/#CME! You can claim your certificate at https://t.co/qnExevP6KX. Thanks for following this ๐งต! Get more #MedEd #nephtwitter by FOLLOWING US here! And thanks to guest faculty Mythri Shankar ๐ฎ๐ณ @nephromythri !
— @CKD_ce (@ckd_ce) September 5, 2023