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KDIGO 2025 ADPKD Guidelines Review
#NephJC Chat
Tuesday, March 25, 2025, 9 pm Eastern on Bluesky
Kidney Int. 2025 Feb;107(2S):S1-S239. doi: 10.1016/j.kint.2024.07.009.
KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD)
Kidney Disease: Improving Global Outcomes (KDIGO) ADPKD Work Group
PMID: 39848759
More information
Introduction
KDIGO has been on a revamping spree and updating multiple guidelines over the past few years. It is no surprise that they decided that Autosomal Dominant Polycystic Kidney Disease (ADPKD) should have its own guidelines. After all, it is estimated to affect up to 12 million individuals and is the 4th most common cause of renal replacement therapy worldwide. ADPKD patients are distinct in many ways- including diagnostic criteria, prognostic markers and treatment options to delay progression- and hence should not be lumped under other etiologies of CKD. These are the first-ever KDIGO guidelines to specifically address the diagnosis and disease management of patients with ADPKD. These guidelines are a result of a decade of global collaboration between physicians, researchers, advocates, and patients. The guidelines incorporate ADPKD-specific care as well as many general CKD management practice points.
As with previous KDIGO guidelines, there is a grading system to help judge the quality of evidence supporting guidance. There are 2 grades (1 for “we recommend”, 2 for “we suggest”) and four grades of evidence (A-D) from high to very low. There are also a number (in this case > 200) of ungraded practice points.
Chapter 1: Diagnosis
Recommendation 1.3.1: For screening adults at risk of ADPKD, we recommend first using abdominal imaging by ultrasound, in the context of the family history, kidney function, and comorbidities (1B).
Practice Point 1.3.4: Follow-up magnetic resonance imaging (MRI), computed tomography (CT) imaging, and/or genetic testing may clarify the diagnosis and further characterize the disease.
Imaging the kidneys (and liver) is central to diagnosing ADPKD. Criteria have been established based upon a family history of ADPKD, age, and the number of cysts found on imaging. Certain modalities, particularly ultrasound, can be user and/or patient (i.e. body habitus, adequate prep for imaging) dependent. Cost and availability of imaging modalities may also play a role in which type of image is chosen for diagnosis and prognosis. Height-adjusted total kidney volume (htTKV) is used to predict the risk of rapid kidney decline and is preferentially done with MRI imaging, when possible (Lanktree M, Kidney Int Rep, 2023). MRI is more sensitive at visualizing small cysts, and thus the number of cysts visualized for a diagnosis of ADPKD varies widely by modality.
Figures 3, 4 and 5. Ultrasound and MRI diagnostic criteria, with family history of ADPKD. KDIGO ADPKD, 2025
Genetic testing
Genetics is a hot topic and even a region in NephMadness this year. Genetic testing, which has become more available in some parts of the world, is not required for diagnosis but may be helpful in very young patients, those without a family history of ADPKD, and patients with equivocal imaging studies (table 4). Ideally, counseling should be discussed and offered even before genetic testing is undertaken. A multidisciplinary team led by a nephrologist might include geneticists, genetic counselors, and radiologists when discussing difficult diagnostic cases. Genetic testing may also be offered to patients with radiologically obvious ADPKD, as it may provide prognostic value and increase certainty for the patient and their family.
Figure 1. Diagnosis algorithm in at-risk adults with a family history of ADPKD. KDIGO ADPKD, 2025
Table 4. Genetic testing to clarify diagnosis and aid in prognosis. KDIGO ADPKD, 2025
Currently, a PKD and/or nephrology next-generation sequencing (tNGS) panel is the most targeted and cost-effective means to genetically screen people with suspected ADPKD. In families with previously identified mutation, a Sanger sequencing of the target gene is usually sufficient for diagnosis or exclusion of the disease. Whole gene sequencing (WGS) is also used if there are atypical features or reasons to suspect variants other than PKD1 and PKD2. Genetic testing is not always definitive for diagnosis and identifies approximately 75% of pathologic variants. Databases have been established to identify variants of unknown significance (VUS). Negative or uncertain genetic testing, in a patient with an ADPKD phenotype, does not absolutely exclude the disease.
Several inherited diseases can clinically mimic ADPKD with kidney and/or liver cysts as part of their phenotype. Non-inherited acquired cysts can also develop in kidneys due to a multitude of reasons. KDIGO provides a detailed list of features in Table 5.
Adapted from Table 5. KDIGO ADPKD. 2025
Figure 3. from Spheroids, organoids, and kidneys‑on‑chips: how complex human cellular models have assisted in the study of kidney disease and renal ciliopathies. Dewhurst RM, et al. Microfluidics and Nanofluidics, 2023.
Prognostics
Several characteristics may be linked with worse outcomes in patients with ADPKD summarised in Figure 6. Important ones include male sex, obesity (especially in early stages of disease- HALT-PKD Study A), high salt intake (Torres et al, Kidney International 2017), and genetic variants.
Amongst genetic variants, PKD1 is associated with more rapid progression to kidney failure, especially if associated with a truncating mutation, and the disease progression in a family member with ADPKD may also be predictive of outcomes in presymptomatic relatives. Amongst the minor ADPKD genes, ADPKD-NEK8 (only specific variants of kinase domain) is associated with kidney failure in childhood (age 1-17 years). Mutations in ALG9 and ALG5 are associated with a moderate risk of kidney failure and GANAB and IFT140 with low risk of kidney failure.
Figure 6. Factors associated with worse prognosis in patients with ADPKD. KDIGO ADPKD, 2025
Recommendation 1.4.2.1: We recommend employing the Mayo Imaging Classification (MIC) to predict future decline in kidney function and the timing of kidney failure (1B)
Figure 10. Predicting eGFR decline by MIC (A-E). KDIGO ADPKD, 2025
The Mayo Imaging Classification (MIC) has only been validated in patients with PKD1 or PKD2 genetic variants (in studies of tolvaptan a subset of patients where 97% PKD 1 or 2). Additionally, when using the MIC for prognosis, physicians should exclude people with atypical imaging patterns. Kidney disease prognosis is based upon htTKV and divided into 5 classes (1A-1E). Once htTKV has been calculated, and the patient has been classified as a slow to rapid progressor, it is not usually necessary to re-image the patient unless there is a sudden, unexpected change in eGFR. Patients with more severe disease (C-E) may qualify for further interventions, including tolvaptan. Kidney size, even from an early disease stage, has strong predictive value for determining later decline in kidney function. Given that the significance of htTKV is highly age-dependent, these data alone can be difficult to interpret; therefore, htTKV/age groups have been defined to categorize people with ADPKD according to the MIC. Five studies reported various multivariable analyses that evaluated MIC as a predictor of future kidney function (>4 years), as measured by eGFR slope or development of kidney failure. The grade of the certainty of the evidence was downgraded from high to moderate, based primarily on some inconsistencies regarding how strongly each of the MIC classes was associated with change in kidney function.
Additional tools include the Predicting Renal Outcomes in Polycystic Kidney Disease (PROPKD) score and longitudinal data of individual eGFR slope (when multiple measurements are captured over time) can help identify people at higher risk of rapidly progressive CKD. United Kingdom Kidney Association (UKKA) guidelines integrate both MIC and PROPKD scores in the prescription algorithm. The PROPKD score combines genetic and sex data with details of early onset of disease symptoms, to provide prognostic information. The PROPKD score has been mostly validated in Caucasian ADPKD patients.
Figure 13. PROPKD risk stratification. KDIGO ADPKD, 2025
Chapter 2: Treatment
Hypertension
Briefly, there are not many deviations from the treatment of hypertension in patients with ADPKD beyond those with CKD from other etiologies. RAS inhibitors remain a first-line therapy, although the certainty of evidence is low. The only other point worth noting is a recommended blood pressure of ≤110/75 as measured by home BP monitoring in adults aged 18-49 years with CKD G1-G2. This target is the lowest of any etiology of CKD, but is graded as very low (1D) certainty (and maybe even that is generous), and is based on intensive blood pressure control using RAS inhibitors in the HALT PKD study (Schrier RW et al, NEJM 2014) and was associated with lower TKV, LV mass and albuminuria, but no difference in eGFR slope. It is important to note the population for HALT-PKD was super young (<40 years), with an eGFR> 90 ml/min. The results are likely not applicable for patients with ADPKD on dialysis, who are older, and often have more comorbidities.
Pain
Pain is common in patients with ADPKD due to pressure effects, cyst rupture, or infections. The experience of pain and its severity can vary greatly between patients. It is suggested that an evaluation be undertaken to rule out other causes (stones, spinal abnormalities, malignancy) of back pain as cyst size does not necessarily correlate with the severity of symptoms. As with all chronic pain, shared decision-making, consideration of both nonpharmacological and pharmacological options, and starting with noninvasive therapies are recommended.
Figure 15. Pharmacologic and nonpharmacologic therapies for ADPKD pain. KDIGO ADPKD, 2025
Infections
Asymptomatic pyuria is relatively common in people with ADPKD and does not necessarily indicate a UTI. Asymptomatic pyuria and asymptomatic bacteriuria should not be treated with antibiotics (except during pregnancy), which is the same recommendation as that for the general population. The clinical presentation of UTI may overlap with cyst hemorrhage or kidney stone. A lipid-soluble antibiotic (e.g., fluoroquinolones, trimethoprim-sulfamethoxazole) should be used to treat kidney cyst infection in people with ADPKD, if possible. Finally, kidney cyst infections are treated with oral antibiotics, unless a person is septic or actively vomiting. In people with ADPKD and kidney cyst infection, it is suggested to treat with 4–6 weeks of antibiotic therapy rather than a shorter course (2D).
Figure 16. Infected kidney cyst algorithm in ADPKD. KDIGO ADPKD, 2025
Chapter 3: CKD Progression and KRT
Management of CKD (hypertension, gout, anemia, secondary hyperparathyroidism, hyperlipidemia) is similar to other etiologies of CKD with a few exceptions. Notably, SGLT2 inhibitors have been used widely in patients with and without diabetes to slow CKD progression. SGLT2 inhibitors, however, are not recommended in ADPKD due to their diuretic effect (glucosuria) leading to increased serum vasopressin with mixed results in cyst progression (at least in animal models). There is at least one short-term (6-month crossover design) study that looked at patients on tolvaptan and dapagliflozin that suggested slowing of eGFR chronic slope on combination therapy, though the trial was small with only twenty-seven participants. (Uchiyama K, et al. Kidney Int Rep, 2025) Longer studies in patients with less severe disease will need to be done before SGLT2 inhibitors can be recommended to slow CKD progression in patients with ADPKD. (Bahlmann-Kroll E, et al, BMJ Open, 2024) Finally, HIF PHIs should not be used to treat anemia in ADPKD since cyst growth is associated with HIF-1𝝰 induction. The use of HIF PHIs in an animal model led to rapid loss of kidney function and cyst growth. (Kraus A et al, Kidney Int 2018) More recent CKD medication breakthroughs, like nsMRAs and GLP1 antagonists, are not even mentioned as part of these guidelines and will have to wait for formal future studies.
Transplant
In ADPKD, most care in the peri-transplant period is similar to other etiologies of ESKD.
Special considerations for ADPKD patients seeking kidney transplantation:
In patients seeking a living-related donor, exclusion of the diagnosis of ADPKD is an important consideration. Imaging and/or genetic testing should be done on all related donors.
During the pre-transplantation work-up for candidates with ADPKD, the total kidney and liver weight (derived from total kidney and liver volumes) should be calculated and subtracted from the patient’s total body weight for a more accurate assessment of weight and body mass index. In some patients, this may allow certain patients to make BMI cut-offs of some institutions, and increase eligibility.
Native nephrectomy: Indications for native nephrectomy are seen in figure 21, if the benefits outweigh the risks.
KDIGO suggests unilateral rather than bilateral nephrectomy when indicated (2D)
The procedure should be done at the time of or after transplantation, but not before transplantation, where feasible (2C) and with hand-assisted laparoscopic nephrectomy as a preference over open nephrectomy (2D)
A meta-analysis of 5 studies found an increased risk of all-cause mortality in pre-transplant nephrectomy, possibly related to higher blood loss and long operative time. A recent meta-analysis by ERA (Geertseema P et al, NDT 2025) demonstrated a lower risk of major complications in post-transplant versus pre-transplant nephrectomy but no difference in mortality, duration of procedure, or blood loss. Pre-transplant nephrectomy was suggested as a last treatment option. Again, the preferred timing of nephrectomy evidence is weak at best.
Figure 21. Indications for nephrectomy in ADPKD transplant patients. KDIGO ADPKD, 2025
Kidney replacement therapy
Recommendation 3.3.1: We suggest that in people with ADPKD, the selection of dialysis modality (hemodialysis [HD] or peritoneal dialysis [PD]) for treatment of kidney failure should be determined by patient-related factors, patient choice, and availability of facilities (2C)
Shared decision-making should be employed when choosing a dialysis modality. The choice of PD versus HD should be determined by patient factors, preferences, and availability of facilities.
Peritoneal dialysis should be considered a viable kidney replacement therapy (KRT) for people with ADPKD complicated by kidney failure, with caution indicated only when massive kidney and/ or liver enlargement are present.
Studies have found no significant difference in all-cause mortality between PD and HD in patients with ADPKD, but the quality of evidence suggesting modality superiority is low.
Chapter 4: Delaying CKD Progression
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)
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)
Tolvaptan (V2 Vasopressin receptor antagonist) studies in ADPKD
TEMPO 3:4
Tolvaptan slowed the increase in htTKV and decline in eGFR versus placebo over a 3-year period in patients with ADPKD. (Torres VE, et al, NEJM, 2012) Patients who received tolvaptan had a higher frequency of side effects related to increased aquaresis (thirst, polyuria, nocturia, and polydipsia as a result of the excretion of electrolyte-free water). There were higher discontinuation rates in the treatment arm due to adverse events (15.4% versus 5%), including the risk of liver toxicity.
REPRISE
The REPRISE study included patients with ADPKD and CKD 3b/4 (eGFR of 25- 44 ml/min). Tolvaptan resulted in a slower decline than placebo in eGFR at 1-year follow-up. (Torres VE, et al, NEJM, 2017) The decline of eGFR, even in patients with severe CKD was slowed by tolvaptan (eGFR −2.34 ml/min in the tolvaptan group versus −3.61 ml/min in the placebo group; difference, 1.27 ml/min/1.73 m2; 95% CI, 0.86 to 1.68; P<0.001).
Featured
OVERTURE
OVERTURE (Perrone RD et al, KI reports 2023) was an observational, longitudinal study that included 3409 ADPKD patients from 20 countries. Higher baseline measured htTKV was once again confirmed to be associated with worse ADPKD-related clinical outcomes. The relationship of htTKV to eGFR decline was attenuated in subjects at low risk of rapid progression (Mayo class 1A–1B). This finding is consistent with data from the Mayo Clinic.
Technically, increasing water intake should have similar effects to vasopressin receptor antagonists, and suppress endogenous vasopressin. Additionally, water intake is significantly cheaper and has fewer serious side effects. Most studies on increased water intake in patients with ADPKD have failed to show significant benefits in TKV or slope of eGFR decline. It is important to note that adherence to protocols of increased fluid intake is difficult, and most patients in the PREVENT-ADPKD trial were not able to achieve a goal urine osmolarity < 300 mOsm/kg. (Wong ATY, et al, BMJ Open, 2018) It is also unclear if 2-3 liters daily, as recommended in 4.2.1.1, would actually achieve this urine osmolarity goal in most patients.
Table 8. Water intake versus tolvaptan. KDIGO ADPKD, 2025
Mayo Imaging Classification (MIC) class 1C-1E or historic eGFR decline ≥3 ml/min per 1.73 m2 are deemed eligible for tolvaptan treatment. A PROPKD score > 6 may be helpful if imaging is indeterminate. Other causes of eGFR decline need to be considered before initiating tolvaptan, and patients should have an eGFR >25 ml/min per 1.73 m2 before initiation. Tolvaptan should be used with caution in people with gout and liver disease, and frequent lab monitoring is required (at initiation, monthly for 18 months, then quarterly). Tolvaptan is started at a low dose and then slowly titrated to limit polyuria and risks for hypernatremia (Figure 28 below).
Tolvaptan should be discontinued in patients who are pregnant, breastfeeding, or who initiate KRT. Treatment with tolvaptan can be maintained close to the initiation of KRT, and the timing of withdrawal depends on individual patient circumstances. The withdrawal of tolvaptan may be associated with a 5%–10% increase in eGFR on average. Although current studies have shown a reduction in eGFR decline, no long-term studies are showing a decreased incidence of kidney failure.
Figure 28. Titration of tolvaptan dosing. KDIGO ADPKD, 2025
Figure 29. Monitoring for drug-induced liver injury during tolvaptan therapy KDIGO ADPKD, 2025
Patients should be educated regarding the effect of tolvaptan on increasing urinary water loss (which can cause thirst, polyuria, and nocturia) and strategies to manage anticipated aquaretic effects. People with ADPKD and their physicians should be advised that tolvaptan treatment should be immediately interrupted in clinical situations causing volume depletion (i.e., vomiting, diarrhea), and people with ADPKD should have a “sick-day plan”. There is currently insufficient evidence for using thiazide diuretics to mitigate aquaresis associated with tolvaptan. Treatment with tolvaptan (potentially life-long) is expensive (roughly $5700/month USD) and may be prohibitive to some patients depending on coverage.
Other medications have been evaluated for their potential to shrink cysts and slow CKD progression. There are four recommendations (grade 1B-2D) concerning these medications in patients with ADPKD. Specifically, mTOR inhibitors, statins, metformin, flozins, and somatostatin analogs are not recommended for use in ADPKD patients to slow CKD progression.
Chapter 5: Cystic Liver Disease
Recommendation 5.2.3.1: We recommend prescribing long-acting somatostatin analogues in people with ADPKD and markedly enlarged polycystic livers with severe volume-related symptoms (1B).
Four RCTs with a follow-up of >1 year assessed the role of somatostatin analogs in ADPKD and autosomal dominant polycystic liver disease (ADPLD). These drugs reduce total liver volume (TLV) in people with ADPKD and ADPLD, as compared to placebo. They also reduce the rate of growth of polycystic kidneys but have not been shown to slow the rate of eGFR decline.
Also note that ADPLD is a more aggressive condition in women than in men, which is thought to be estrogen-driven. Therefore, patients should be counselled about oestrogen-containing contraceptives associated with liver cyst growth, and consideration should be given to alternative contraception. Furthermore, a risk versus benefit discussion should be had about hormone replacement therapy after menopause.
Chapter 6: Intracranial Aneurysms and Other Extrarenal Manifestations
Recommendation 6.1.1: We recommend informing adults with ADPKD about the increased risk for intracranial aneurysms (ICAs) and subarachnoid hemorrhage (1C)
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)
Perhaps the most devastating consequence of a diagnosis of ADPKD, aside from kidney failure, is the associated formation of cerebral vascular aneurysms. ADPKD is associated with a 6.9-fold increased risk of developing an intracranial aneurysm (ICA). A detailed personal history of subarachnoid hemorrhage (SAH) and a family history of ICAs, or unexplained sudden death, should be obtained to identify people with ADPKD. General CV risk modification including stopping smoking and controlling hypertension is recommended to decrease risk of aneurysmal rupture. MR angiography (time of flight) without gadolinium should be used for screening for ICA, although high-resolution CT angiography may also be used. Most aneurysms detected by screening are small (<5 mm), and approximately 90% occur in the anterior circulation.
Patients with identified ICA, and their families, should be educated about the occurrence of “thunderclap” headaches, and that they should seek immediate medical attention if they occur.
Screening for unruptured ICA also can be discussed in the context of evaluation for kidney and/or liver transplantation or before major elective surgery although ironically, the guideline does not elaborate on this practice point despite kidney transplantation being KRT modality of choice for ADPKD patients. In addition, people with ADPKD who are not considered at increased risk for ICA, and who prefer being screened for ICA, should be given access to screening. If the screening is negative in people with a high risk of ICA, the timing of rescreening should be individualized, possibly every 5–10 years, based on risk factors, age, and life expectancy. Routine screening of vascular abnormalities of non-intracranial large arteries has no role in people with ADPKD with no familial history of vascular aneurysms or dissections. People with ADPKD with first-degree relatives who have a family history of aortic root or thoracic aortic aneurysms should be screened for aortic aneurysms.
Figure 35. Prevalence of ICA and SAH in ADPKD and the general population. KDIGO ADPKD, 2025
Other extrarenal manifestations of ADPKD are fairly rare. Cardiovascular diseases including mitral valve prolapse, pericardial effusions, cardiomyopathies, and congenital malformations are uncommon. Liver, splenic, and pancreatic cysts are quite common, but other GI manifestations including liver failure are rare. Bronchiectasis (seen on x-ray), and pleural effusions (21% versus 8% in controls) related to an ADPKD diagnosis have also been described in the literature.
Chapter 7: Lifestyle
Patients with ADPKD, similar to other patients with CKD from other causes, should adopt healthy lifestyles, diets, and activity levels. One point of consideration though is that direct trauma to the kidneys and/or liver is a particular concern for contact sports, and bleeding is more likely to occur if cysts are superficially located and are large. Therefore, people with ADPKD and large and/or superficial cysts are advised to avoid contact sports (e.g., American football, rugby, boxing, hockey, lacrosse, wrestling, judo).
Another aspect of lifestyle for patients living with ADPKD has been in the headlines lately. Recent literature suggests that cystic cells are subject to several metabolic dysregulations, particularly in the glucose pathway, and mitochondrial abnormalities, leading to decreased oxidative phosphorylation and impaired fatty acid oxidation. The latest studies on the use of ketogenic dietary interventions (daily calorie restriction, intermittent fasting, time-restricted feeding, ketogenic diets, and exogenous ketosis), appear to have a positive impact on the progression of ADPKD, reducing kidney cyst sizes and improving kidney function. (Pezzuoli C, et al, Nutrients, 2024) However, further large-scale clinical studies are needed to confirm these preliminary findings and evaluate their long-term safety.
Finally, physicians should also recognize and treat the multiple psychosocial stressors in patients with ADPKD.
Figure 43. Psychosocial screening and approach. KDIGO ADPKD, 2025
Chapter 8: Pregnancy
Women with ADPKD and liver cysts should be educated regarding their contraceptive choices, given that estrogen and possibly progesterone exposure may be associated with an increased risk of polycystic liver disease (PLD) progression. As a general rule, however, contraception should not be restricted in patients with ADPKD.
Genetic counseling should be made available to all couples with a history of ADPKD in their personal or family histories. Tolvaptan and RAAS inhibition should be stopped during preconception counseling. Before pregnancy, screening for ICA should be considered in women with a family history of ICA, women with de novo ADPKD, those with unknown familial history, and those with a personal or familial history of extracerebral vascular phenotype. Women with ADPKD are at an increased risk of preeclampsia and preterm delivery and should be monitored carefully throughout their pregnancy and in the postpartum period. Assessment of the sFlt-1/ PlGF ratio in plasma, from 24 weeks of gestation and every 4–6 weeks, should be done to monitor for preeclampsia (as guidelines recommend for all patients with CKD). Low-dose aspirin (75-150 mg) can be taken from week 12-36 to mitigate patients’ pre-eclampsia risk.
Figure 45. Management of women with ADPKD and childbearing age. KDIGO ADPKD, 2025
Chapter 9: Pediatrics
Recommendation 9.2.1: We recommend targeting BP to ≤50th percentile for age, sex, and height or ≤110/70 mmHg in adolescents in the setting of ADPKD and high BP (1D).
Recommendation 9.2.2: We recommend use of RASi (i.e., ACEi or ARBs) as the first-line pharmacologic therapy for high BP in children and adolescents with ADPKD (1D).
Genetic testing, in conjunction with counseling, in young children may be appropriate in families with a strong history of ADPKD. On the other hand, a wait-and-watch strategy is often employed in children with a single kidney cyst (on ultrasound), normal BP and urine findings, negative family history for ADPKD, and negative ultrasound findings in parents. The workgroup suggests not performing routine screening for extrarenal manifestations including liver, pancreas, or spleen cysts, cardiac valvular disease, or ICA in children and adolescents with ADPKD. Do not use vasopressin analogs to treat nocturnal enuresis in children with or at risk of ADPKD. Different phenotypic entities exist in children and may be recognized as early <18 months. Once identified by imaging or genetic testing, blood pressure control and lifestyle modification may be recommended.
Table 21. Phenotypical entities in children with ADPKD. KDIGO ADPKD, 2025
Figure 52. Monitoring disease progression in pediatric patients. KDIGO ADPKD, 2025
Conclusion
KDIGO is making further efforts to provide the world’s nephrology community with guidance on specific disease entities that contribute to significant CKD burden. As the care of patients with CKD and kidney failure becomes more complex, in an age of increasing therapeutics and pathophysiologic understanding, consensus expert guidance is appreciated. In this first iteration of ADPKD guidelines from KDIGO, it becomes quite obvious that we have come quite far in disease management, but still have room to grow. Most guidelines are practice points and the level of evidence of most recommendations is low. It is extremely pertinent that these guidelines, written by physicians and scientists, continue to honor the voice of the patient when making suggestions and recommendations about patient care.
Summary prepared by
Brian Rifkin
Hattiesburg Clinic
Hattiesburg, MS
Reviewed by
Jamie Willows, Pallavi Prasad, Cristina Popa, Swapnil Hiremath