Materiały źródłowe

• #1 Autosomal dominant polycystic kidney disease (ADPKD) | Kidney Care UK

  https://kidneycareuk.org/kidney-disease-information/kidney-conditions/autosomal-dominant-polycystic-kidney-disease-adpkd/

  ADPKD is a genetic condition. It is caused by an abnormality or mutation in one of two genes, PKD1 or PKD2. Most people get ADPKD when they inherit a faulty copy of one of these genes from a parent. […] There is currently no cure for ADPKD so treatment focuses on maintaining a good level of health to protect the kidneys. This includes: […] Avoid smoking. Smoking damages the blood vessels in the kidneys and speeds up kidney damage. […] Control blood pressure. High blood pressure damages the kidneys and increases the risk of stroke and heart disease. Blood pressure should therefore be checked regularly. […] Eat a balanced diet. It is especially important to limit the amount of salt in the diet as too much salt can cause high blood pressure. […] Exercise regularly. This will help to control blood pressure. […] Drink enough fluid. […] Avoid non-steroidal anti-inflammatory drugs (NSAIDs). These are a type of medication used to relieve pain, reduce inflammation and bring down a high temperature.

• #2 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Autosomal dominant polycystic kidney disease is the most common genetic kidney disease affecting adults. Approximately 60% of patients develop kidney failure by 60 years of age due to slowly expanding kidney cysts. […] A healthy lifestyle and rigorous control of blood pressure slow kidney cyst growth. These interventions can be effective in reducing progression to kidney failure and cardiovascular disease, especially if started in early adulthood. […] Encouraging young adults with ADPKD to engage in their health during the early asymptomatic period provides the best opportunity to significantly delay the onset of kidney failure and prevent cardiovascular disease. In particular, there is good evidence that lifestyle modifications (smoking cessation, weight reduction, aiming for a body mass index less than 25 kg/m2, reduction in dietary sodium intake to 80-100 mmol/day and regular physical activity) slow kidney cyst growth and the decline in kidney function. Providing education via PKD Australia fact sheets and genetic counselling are also recommended.

• #3 Kidneys – polycystic kidney disease (PKD) | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/kidneys-polycystic-kidney-disease-pkd

  Polycystic kidney disease (PKD) is an inherited condition characterised by the growth of cysts on the kidneys. […] There is currently no cure for PKD, but early detection and treatment can reduce or prevent some complications. […] Currently there is no cure for PKD. However, early detection and treatment can reduce or prevent some of the complications of PKD.

• #4

  https://www.nhs.uk/conditions/autosomal-dominant-polycystic-kidney-disease-adpkd/

  There’s currently no cure for ADPKD, however: […] a healthy lifestyle may help to protect your kidney function […] various treatments are available to manage problems caused by the condition.

• #5 Autosomal Dominant Polycystic Kidney Disease: From Pathophysiology of Cystogenesis to Advances in the Treatment

  https://www.mdpi.com/1422-0067/23/6/3317

  Healthy lifestyle and diet, maintenance of optimal weight, regular cardiovascular exercise and avoidance of smoking are generally recommended in ADPKD. […] Strict control of hypertension is the most important therapeutical option. Hypertension often presents in children and young adults with ADPKD and later promotes decline of renal function. […] A monthly protocol for monitoring liver enzymes is obligatory for 18 months as a prevention of severe hepatic toxicity, followed by 3-month check-up. […] Nowadays, therapy with tolvaptan should be recommended to all ADPKD patients with probable rapid progression of the disease. […] Criteria for tolvaptan use are proposed as follows: 1. age 18–55 years, 2. chronic kidney disease CKD1–4 (eGFR ≥ 25 mL/min per 1.73 m²), 3. high risk measured by available risk scores (longitudinal diameter > 17 cm by ultrasound, total kidney volume > 750 mL, Mayo imaging classification 1C, 1D, 1E or PROPKD score > 6), and 4. rapid decline of eGFR 3 mL/min per > 1.73 m² for 5 years. […] Nowadays, there is no evidence that somatostatin analogues should be used in patients with polycystic renal disease. However, they should come into account in patients with high volume polycystic liver to put off liver transplantation.

• #6 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Autosomal dominant polycystic kidney disease is the most common genetic kidney disease affecting adults. Approximately 60% of patients develop kidney failure by 60 years of age due to slowly expanding kidney cysts. […] A healthy lifestyle and rigorous control of blood pressure slow kidney cyst growth. These interventions can be effective in reducing progression to kidney failure and cardiovascular disease, especially if started in early adulthood. […] Encouraging young adults with ADPKD to engage in their health during the early asymptomatic period provides the best opportunity to significantly delay the onset of kidney failure and prevent cardiovascular disease. In particular, there is good evidence that lifestyle modifications (smoking cessation, weight reduction, aiming for a body mass index less than 25 kg/m2, reduction in dietary sodium intake to 80-100 mmol/day and regular physical activity) slow kidney cyst growth and the decline in kidney function. Providing education via PKD Australia fact sheets and genetic counselling are also recommended.

• #7 Polycystic kidney disease (PKD) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/polycystic-kidney-disease

  High blood pressure is the most common sign of PKD. […] Because high blood pressure can cause kidney damage, it is very important to treat it. In fact, treatment of high blood pressure can help slow or even prevent kidney failure. […] At present, there is no cure for PKD. However, a lot of research is being done. Recent studies suggest that drinking plain water throughout the day and avoiding caffeine in beverages can slow the growth of cysts. […] Studies also suggest that some treatments may slow the rate of kidney disease in PKD, but further research is needed before these treatments can be used in patients. […] Careful control of blood pressure […] Healthy lifestyle with regard to quitting smoking, exercise, weight control and reduced salt intake […] Drinking lots of plain water throughout the day

• #8 Autosomal Dominant Polycystic Kidney Disease: From Pathophysiology of Cystogenesis to Advances in the Treatment

  https://www.mdpi.com/1422-0067/23/6/3317

  Healthy lifestyle and diet, maintenance of optimal weight, regular cardiovascular exercise and avoidance of smoking are generally recommended in ADPKD. […] Strict control of hypertension is the most important therapeutical option. Hypertension often presents in children and young adults with ADPKD and later promotes decline of renal function. […] A monthly protocol for monitoring liver enzymes is obligatory for 18 months as a prevention of severe hepatic toxicity, followed by 3-month check-up. […] Nowadays, therapy with tolvaptan should be recommended to all ADPKD patients with probable rapid progression of the disease. […] Criteria for tolvaptan use are proposed as follows: 1. age 18–55 years, 2. chronic kidney disease CKD1–4 (eGFR ≥ 25 mL/min per 1.73 m²), 3. high risk measured by available risk scores (longitudinal diameter > 17 cm by ultrasound, total kidney volume > 750 mL, Mayo imaging classification 1C, 1D, 1E or PROPKD score > 6), and 4. rapid decline of eGFR 3 mL/min per > 1.73 m² for 5 years. […] Nowadays, there is no evidence that somatostatin analogues should be used in patients with polycystic renal disease. However, they should come into account in patients with high volume polycystic liver to put off liver transplantation.

• #9

  https://link.springer.com/article/10.1007/s00467-021-04974-4

  In terms of drug choice, RAS inhibition with ACE inhibitor or ARB blocker is generally preferred for adults with CKD due to the additional renoprotective effects in proteinuric patients. […] The vasopressin 2 receptor antagonist tolvaptan is thought to reduce cyst growth by blocking the effect of arginine vasopressin on collecting duct cells, which otherwise transmits proliferative stimuli via cyclic AMP and promotes secretion of fluids into the cyst lumen via the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. […] From a pediatric perspective, the fact that loss of GFR is a very gradual, continuous process, has both upsides and downsides: on the one hand, there is a theoretical benefit of starting to delay disease progression as early as possible in order to maximize the achieved delay of kidney failure.

• #10 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Early detection and treatment of hypertension have significant benefits, as they prevent left ventricular hypertrophy, reduce albuminuria and slow kidney cyst growth. […] The treatment of hypertension follows standard guidelines and should be integrated with routine screening for other cardiovascular disease risk factors (such as hyperlipidaemia and impaired glucose tolerance). […] In patients with early-stage disease (eGFR 60 mL/min/1.73 m2), the recommended blood pressure target is between 120/70 mmHg and 130/80 mmHg. […] Tolvaptan is a specific oral vasopressin type 2 receptor antagonist and is indicated in patients with ADPKD at high risk of developing kidney failure. […] The main adverse effect of tolvaptan is massive aquaresis (mean urine volume of 57 L/day) due to the off-target suppression of vasopressin-mediated water reabsorption in the collecting duct. This occurs in all patients and requires behavioural adaptation to increase daily fluid intake.

• #11 Polycystic kidney disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/symptoms-causes/syc-20352820

  If you have polycystic kidney disease and you’re thinking about having children, a genetic counselor can help you know your risk of passing the disease to your children. […] Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. It’s most important to manage your blood pressure. […] Here are some tips for keeping your blood pressure in check: […] Take the blood pressure medicines your healthcare professional prescribes as directed. […] Eat a low-salt diet that has plenty of fruits, vegetables and whole grains. […] Get to and stay at a healthy weight. […] Exercise regularly. Aim for at least 30 minutes of moderate physical activity most days of the week. […] Limit alcohol use. […] Don’t smoke.

• #12 Autosomal Dominant Polycystic Kidney Disease: Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/digestive-disorders/autosomal-dominant-polycystic-kidney-disease

  You can slow the damage and prevent some of the complications by making healthy habits (especially those that help you lower and then maintain your blood pressure) part of your life and taking medications as necessary. […] There is no cure for ADPKD, but you can treat the health problems that the disease causes and possibly prevent kidney failure. […] It’s important to stay as healthy as possible to protect your kidneys and keep them working as long as you can. Follow your doctor’s advice carefully. You can also keep up these habits to stay well: […] Stick to a healthy, well-balanced diet that’s low in calories. Try to limit salt, because it can raise your blood pressure. […] Exercise can help control your weight and blood pressure. Just avoid any contact sports where you might injure your kidneys. […] If you smoke, get help from your doctor to quit. Smoking damages the blood vessels in the kidneys, and it may create more cysts. […] Dehydration may cause you to have more cysts.

• #13 ADPKD basics

  https://pkdcharity.org.uk/adpkd/what-is-adpkd/adpkd-basics

  What can I do to help my kidneys? There are things you can do to help to protect your kidneys and stay as healthy as possible: […] Avoid smoking. Smoking can increase the speed at which your ADPKD progresses and damages your kidneys. It is also bad for your general health. […] Take your blood pressure medication. If you have high blood pressure, it’s important to control it. High blood pressure increases the chance of having a heart attack or bleed on the brain (intracranial haemorrhage). It can also cause your ADPKD to progress faster. Blood pressure can be treated with lifestyle changes and medications. […] Eat healthily. There is no recommended special diet that people with ADPKD should follow, but a balanced diet is important for everyone for controlling weight. Being overweight or obese may lead to a faster decline in your kidney function and can increase your risk of high blood pressure or cardiovascular problems. Limit how much salt you eat because salt makes high blood pressure worse and may increase the speed that your kidney function declines.

• #14 Autosomal Dominant Polycystic Kidney Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532934/

  Implement a systematic screening protocol for ADPKD, including regular imaging studies and genetic testing, in patients with a family history of the disease. […] Develop appropriate management strategies, including lifestyle modifications and pharmacological interventions, in patients diagnosed with ADPKD to slow disease progression and manage associated complications. […] Apply evidence-based guidelines for the management of ADPKD, including the use of tolvaptan when indicated, to optimize patient outcomes. […] Facilitate coordinated care among healthcare providers to ensure seamless transitions between primary care, nephrology, and other subspecialties, optimizing the management of ADPKD patients’ complex healthcare needs. […] Lifestyle modifications like increased oral intake of water, restricted salt intake, and avoidance of NSAIDs are associated with long-term benefits.

• #15 Polycystic kidney disease (PKD) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/polycystic-kidney-disease

  High blood pressure is the most common sign of PKD. […] Because high blood pressure can cause kidney damage, it is very important to treat it. In fact, treatment of high blood pressure can help slow or even prevent kidney failure. […] At present, there is no cure for PKD. However, a lot of research is being done. Recent studies suggest that drinking plain water throughout the day and avoiding caffeine in beverages can slow the growth of cysts. […] Studies also suggest that some treatments may slow the rate of kidney disease in PKD, but further research is needed before these treatments can be used in patients. […] Careful control of blood pressure […] Healthy lifestyle with regard to quitting smoking, exercise, weight control and reduced salt intake […] Drinking lots of plain water throughout the day

• #16 Autosomal Dominant Polycystic Kidney Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532934/

  Patients are instructed to drink three liters of fluids daily to suppress vasopressin, thereby decreasing cAMP production and inhibiting cyst production. […] All ADPKD patients are advised to limit their sodium intake to less than 2 grams per day. […] Management of hypertension is essential in reducing cardiovascular mortality and slowing the progression of renal failure. […] Tolvaptan slows the progression of autosomal dominant polycystic kidney disease by blocking the reception of vasopressin signaling at the V2 receptor, lowering the intracellular cyclic AMP that would otherwise stimulate cystic proliferation and growth. […] The decision to undergo genetic testing or renal ultrasonography for asymptomatic children should be collaborative, involving caretakers, parents, and healthcare professionals.

• #17 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Maintaining adequate hydration also reduces vasopressin, but high-quality evidence indicates that drinking more than 2.25 L of water a day does not slow disease progression in patients with ADPKD. […] In young asymptomatic patients, a focus on lifestyle modifications, the monitoring and treatment of blood pressure, and the selected use of disease-modifying drugs reduce the risk of kidney failure and cardiovascular disease.

• #18 Polycystic kidney disease (PKD) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/polycystic-kidney-disease

  Avoiding caffeine in all beverages. […] In April 2018, the FDA approved a new drug called tolvaptan for the treatment of autosomal dominant polycystic kidney disease (ADPKD). The drug can be used to help slow kidney function decline in adults at risk for this type of PKD. […] Reducing salt intake helps control blood pressure in patients with PKD who have high blood pressure. […] Physical exercise is recommended for people with PKD, however exercises that are potentially harmful to the kidney, such as contact sports, should be avoided.

• #19 Polycystic kidney disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/symptoms-causes/syc-20352820

  If you have polycystic kidney disease and you’re thinking about having children, a genetic counselor can help you know your risk of passing the disease to your children. […] Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. It’s most important to manage your blood pressure. […] Here are some tips for keeping your blood pressure in check: […] Take the blood pressure medicines your healthcare professional prescribes as directed. […] Eat a low-salt diet that has plenty of fruits, vegetables and whole grains. […] Get to and stay at a healthy weight. […] Exercise regularly. Aim for at least 30 minutes of moderate physical activity most days of the week. […] Limit alcohol use. […] Don’t smoke.

• #20 ADPKD basics

  https://pkdcharity.org.uk/adpkd/what-is-adpkd/adpkd-basics

  Exercise. Regular exercise is an important part of a healthy lifestyle because it helps to control your weight and blood pressure. […] Stay hydrated. There haven’t been any studies proving that drinking extra fluid slows the growth of kidney cysts. For the moment, kidney experts recommend that you drink enough that you don’t get thirsty, but not excessively. […] Use pain medications other than NSAIDs. Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are commonly used for pain and inflammation. However, using them repeatedly or for a long time can damage the kidneys. Long term use is generally not recommended for people with ADPKD, particularly if your kidney function is already impaired. Ask your doctor or pharmacist to suggest safer alternatives for you.

• #21

  https://www.nhs.uk/conditions/autosomal-dominant-polycystic-kidney-disease-adpkd/treatment/

  There’s currently no cure for autosomal dominant polycystic kidney disease (ADPKD), and it’s not possible to stop cysts forming in the kidneys. […] But there are some potentially useful medications, such as tolvaptan, that can sometimes be used to reduce the growth rate of cysts. […] Avoiding all types of contact sport, such as rugby and football, is usually recommended.

• #22 Polycystic kidney disease (Causes, Symptoms and Treatment)

  https://patient.info/doctor/autosomal-dominant-polycystic-kidney-disease

  Screening offers a means of detecting asymptomatic individuals. There should be appropriate counselling prior to screening. […] The option for screening should be given in advance of an individual starting their own family. […] Advise against participating in contact sports which risk abdominal trauma, such as rugby or boxing, as this can provoke haematuria or even cause the rupture of an enlarged kidney. […] Much of the morbidity and mortality of ADPKD is due to CVD. Thus, lifestyle advice regarding avoiding smoking, maintenance of a healthy body weight, diet (eg, limiting salt intake) and regular exercise (ideally of a low-impact nature, such as swimming or walking) would seem sensible, even if not studied within the ADPKD population specifically.

• #23 Polycystic kidney disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/symptoms-causes/syc-20352820

  If you have polycystic kidney disease and you’re thinking about having children, a genetic counselor can help you know your risk of passing the disease to your children. […] Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. It’s most important to manage your blood pressure. […] Here are some tips for keeping your blood pressure in check: […] Take the blood pressure medicines your healthcare professional prescribes as directed. […] Eat a low-salt diet that has plenty of fruits, vegetables and whole grains. […] Get to and stay at a healthy weight. […] Exercise regularly. Aim for at least 30 minutes of moderate physical activity most days of the week. […] Limit alcohol use. […] Don’t smoke.

• #24 Autosomal Dominant Polycystic Kidney Disease: From Pathophysiology of Cystogenesis to Advances in the Treatment

  https://www.mdpi.com/1422-0067/23/6/3317

  Healthy lifestyle and diet, maintenance of optimal weight, regular cardiovascular exercise and avoidance of smoking are generally recommended in ADPKD. […] Strict control of hypertension is the most important therapeutical option. Hypertension often presents in children and young adults with ADPKD and later promotes decline of renal function. […] A monthly protocol for monitoring liver enzymes is obligatory for 18 months as a prevention of severe hepatic toxicity, followed by 3-month check-up. […] Nowadays, therapy with tolvaptan should be recommended to all ADPKD patients with probable rapid progression of the disease. […] Criteria for tolvaptan use are proposed as follows: 1. age 18–55 years, 2. chronic kidney disease CKD1–4 (eGFR ≥ 25 mL/min per 1.73 m²), 3. high risk measured by available risk scores (longitudinal diameter > 17 cm by ultrasound, total kidney volume > 750 mL, Mayo imaging classification 1C, 1D, 1E or PROPKD score > 6), and 4. rapid decline of eGFR 3 mL/min per > 1.73 m² for 5 years. […] Nowadays, there is no evidence that somatostatin analogues should be used in patients with polycystic renal disease. However, they should come into account in patients with high volume polycystic liver to put off liver transplantation.

• #25 Autosomal Dominant Polycystic Kidney Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532934/

  Implement a systematic screening protocol for ADPKD, including regular imaging studies and genetic testing, in patients with a family history of the disease. […] Develop appropriate management strategies, including lifestyle modifications and pharmacological interventions, in patients diagnosed with ADPKD to slow disease progression and manage associated complications. […] Apply evidence-based guidelines for the management of ADPKD, including the use of tolvaptan when indicated, to optimize patient outcomes. […] Facilitate coordinated care among healthcare providers to ensure seamless transitions between primary care, nephrology, and other subspecialties, optimizing the management of ADPKD patients’ complex healthcare needs. […] Lifestyle modifications like increased oral intake of water, restricted salt intake, and avoidance of NSAIDs are associated with long-term benefits.

• #26 ADPKD basics

  https://pkdcharity.org.uk/adpkd/what-is-adpkd/adpkd-basics

  Exercise. Regular exercise is an important part of a healthy lifestyle because it helps to control your weight and blood pressure. […] Stay hydrated. There haven’t been any studies proving that drinking extra fluid slows the growth of kidney cysts. For the moment, kidney experts recommend that you drink enough that you don’t get thirsty, but not excessively. […] Use pain medications other than NSAIDs. Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are commonly used for pain and inflammation. However, using them repeatedly or for a long time can damage the kidneys. Long term use is generally not recommended for people with ADPKD, particularly if your kidney function is already impaired. Ask your doctor or pharmacist to suggest safer alternatives for you.

• #27 Polycystic kidney disease (PKD) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/polycystic-kidney-disease

  Avoiding caffeine in all beverages. […] In April 2018, the FDA approved a new drug called tolvaptan for the treatment of autosomal dominant polycystic kidney disease (ADPKD). The drug can be used to help slow kidney function decline in adults at risk for this type of PKD. […] Reducing salt intake helps control blood pressure in patients with PKD who have high blood pressure. […] Physical exercise is recommended for people with PKD, however exercises that are potentially harmful to the kidney, such as contact sports, should be avoided.

• #28 Treatment Options for Autosomal Dominant Polycystic Kidney Disease (ADPKD): What Works and What Does Not – AJKD Blog

  https://ajkdblog.org/2023/06/21/treatment-options-for-autosomal-dominant-polycystic-kidney-disease-adpkd-what-works-and-what-does-not/

  Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder. However, there is still a scarcity in treatments that prevent or slow the progression to end stage kidney disease (ESKD). […] Tolvaptan, a vasopressin receptor antagonist, is the only medication approved to slow kidney function decline in ADPKD patients at risk for rapid progression. […] Major research efforts in both the clinical and preclinical setting in the last two decades resulted in a number of pivotal clinical trials aimed to address ADPKD. Several medications have been suggested to alter the course of kidney disease progression. However, the only medication that was proven effective in slowing kidney function decline has been Tolvaptan.

• #29 Autosomal Dominant Polycystic Kidney Disease: From Pathophysiology of Cystogenesis to Advances in the Treatment

  https://www.mdpi.com/1422-0067/23/6/3317

  Healthy lifestyle and diet, maintenance of optimal weight, regular cardiovascular exercise and avoidance of smoking are generally recommended in ADPKD. […] Strict control of hypertension is the most important therapeutical option. Hypertension often presents in children and young adults with ADPKD and later promotes decline of renal function. […] A monthly protocol for monitoring liver enzymes is obligatory for 18 months as a prevention of severe hepatic toxicity, followed by 3-month check-up. […] Nowadays, therapy with tolvaptan should be recommended to all ADPKD patients with probable rapid progression of the disease. […] Criteria for tolvaptan use are proposed as follows: 1. age 18–55 years, 2. chronic kidney disease CKD1–4 (eGFR ≥ 25 mL/min per 1.73 m²), 3. high risk measured by available risk scores (longitudinal diameter > 17 cm by ultrasound, total kidney volume > 750 mL, Mayo imaging classification 1C, 1D, 1E or PROPKD score > 6), and 4. rapid decline of eGFR 3 mL/min per > 1.73 m² for 5 years. […] Nowadays, there is no evidence that somatostatin analogues should be used in patients with polycystic renal disease. However, they should come into account in patients with high volume polycystic liver to put off liver transplantation.

• #30

  https://link.springer.com/article/10.1007/s00467-021-04974-4

  In terms of drug choice, RAS inhibition with ACE inhibitor or ARB blocker is generally preferred for adults with CKD due to the additional renoprotective effects in proteinuric patients. […] The vasopressin 2 receptor antagonist tolvaptan is thought to reduce cyst growth by blocking the effect of arginine vasopressin on collecting duct cells, which otherwise transmits proliferative stimuli via cyclic AMP and promotes secretion of fluids into the cyst lumen via the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. […] From a pediatric perspective, the fact that loss of GFR is a very gradual, continuous process, has both upsides and downsides: on the one hand, there is a theoretical benefit of starting to delay disease progression as early as possible in order to maximize the achieved delay of kidney failure.

• #31 Autosomal Dominant Polycystic Kidney Disease: From Pathophysiology of Cystogenesis to Advances in the Treatment

  https://www.mdpi.com/1422-0067/23/6/3317

  Healthy lifestyle and diet, maintenance of optimal weight, regular cardiovascular exercise and avoidance of smoking are generally recommended in ADPKD. […] Strict control of hypertension is the most important therapeutical option. Hypertension often presents in children and young adults with ADPKD and later promotes decline of renal function. […] A monthly protocol for monitoring liver enzymes is obligatory for 18 months as a prevention of severe hepatic toxicity, followed by 3-month check-up. […] Nowadays, therapy with tolvaptan should be recommended to all ADPKD patients with probable rapid progression of the disease. […] Criteria for tolvaptan use are proposed as follows: 1. age 18–55 years, 2. chronic kidney disease CKD1–4 (eGFR ≥ 25 mL/min per 1.73 m²), 3. high risk measured by available risk scores (longitudinal diameter > 17 cm by ultrasound, total kidney volume > 750 mL, Mayo imaging classification 1C, 1D, 1E or PROPKD score > 6), and 4. rapid decline of eGFR 3 mL/min per > 1.73 m² for 5 years. […] Nowadays, there is no evidence that somatostatin analogues should be used in patients with polycystic renal disease. However, they should come into account in patients with high volume polycystic liver to put off liver transplantation.

• #32 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Early detection and treatment of hypertension have significant benefits, as they prevent left ventricular hypertrophy, reduce albuminuria and slow kidney cyst growth. […] The treatment of hypertension follows standard guidelines and should be integrated with routine screening for other cardiovascular disease risk factors (such as hyperlipidaemia and impaired glucose tolerance). […] In patients with early-stage disease (eGFR 60 mL/min/1.73 m2), the recommended blood pressure target is between 120/70 mmHg and 130/80 mmHg. […] Tolvaptan is a specific oral vasopressin type 2 receptor antagonist and is indicated in patients with ADPKD at high risk of developing kidney failure. […] The main adverse effect of tolvaptan is massive aquaresis (mean urine volume of 57 L/day) due to the off-target suppression of vasopressin-mediated water reabsorption in the collecting duct. This occurs in all patients and requires behavioural adaptation to increase daily fluid intake.

• #33

  https://link.springer.com/article/10.1007/s00467-021-04974-4

  However, starting pharmacological treatment early in life requires drugs with very good safety profiles. […] Nonetheless, in children with ADPKD who already received ACE inhibition, a prospective randomized controlled trial of pravastatin could show a significant reduction of height-adjusted kidney growth over 3 years. […] Higher salt intake is prospectively associated with worse kidney outcomes in the general population, as well as with higher kidney volume and growth in ADPKD. […] While in the general CKD population the negative effects of sodium intake are probably mediated via increased blood pressure and activation of the renin-angiotensin system, in ADPKD sodium-induced increase of vasopressin is a likely additional mechanism leading to cyst growth. […] However, a small interventional trial suggests the opposite clinical effect, so results of an ongoing larger randomized trial are eagerly awaited.

• #34 Autosomal Dominant Polycystic Kidney Disease: From Pathophysiology of Cystogenesis to Advances in the Treatment

  https://www.mdpi.com/1422-0067/23/6/3317

  Healthy lifestyle and diet, maintenance of optimal weight, regular cardiovascular exercise and avoidance of smoking are generally recommended in ADPKD. […] Strict control of hypertension is the most important therapeutical option. Hypertension often presents in children and young adults with ADPKD and later promotes decline of renal function. […] A monthly protocol for monitoring liver enzymes is obligatory for 18 months as a prevention of severe hepatic toxicity, followed by 3-month check-up. […] Nowadays, therapy with tolvaptan should be recommended to all ADPKD patients with probable rapid progression of the disease. […] Criteria for tolvaptan use are proposed as follows: 1. age 18–55 years, 2. chronic kidney disease CKD1–4 (eGFR ≥ 25 mL/min per 1.73 m²), 3. high risk measured by available risk scores (longitudinal diameter > 17 cm by ultrasound, total kidney volume > 750 mL, Mayo imaging classification 1C, 1D, 1E or PROPKD score > 6), and 4. rapid decline of eGFR 3 mL/min per > 1.73 m² for 5 years. […] Nowadays, there is no evidence that somatostatin analogues should be used in patients with polycystic renal disease. However, they should come into account in patients with high volume polycystic liver to put off liver transplantation.

• #35 A new drug candidate can shrink kidney cysts | MIT News | Massachusetts Institute of Technology

  https://news.mit.edu/2024/new-drug-candidate-can-shrink-kidney-cysts-0122

  Researchers at MIT and Yale University School of Medicine have now found that a compound originally developed as a potential cancer treatment holds promise for treating ADPKD. […] The results also suggest that in patients, treatment with 11beta compounds once every few months, or even once a year, could significantly delay disease progression, and thus avoid the need for continuous, burdensome antiproliferative therapies such as tolvaptan. […] Based on what we know about the cyst growth paradigm, you could in theory treat patients in a pulsatile manner once a year, or perhaps even less often and have a meaningful impact on total kidney volume and kidney function, Sorin Fedeles says.

• #36 Autosomal Dominant Polycystic Kidney Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532934/

  Implement a systematic screening protocol for ADPKD, including regular imaging studies and genetic testing, in patients with a family history of the disease. […] Develop appropriate management strategies, including lifestyle modifications and pharmacological interventions, in patients diagnosed with ADPKD to slow disease progression and manage associated complications. […] Apply evidence-based guidelines for the management of ADPKD, including the use of tolvaptan when indicated, to optimize patient outcomes. […] Facilitate coordinated care among healthcare providers to ensure seamless transitions between primary care, nephrology, and other subspecialties, optimizing the management of ADPKD patients’ complex healthcare needs. […] Lifestyle modifications like increased oral intake of water, restricted salt intake, and avoidance of NSAIDs are associated with long-term benefits.

• #37 Polycystic kidney disease (Causes, Symptoms and Treatment)

  https://patient.info/doctor/autosomal-dominant-polycystic-kidney-disease

  Screening offers a means of detecting asymptomatic individuals. There should be appropriate counselling prior to screening. […] The option for screening should be given in advance of an individual starting their own family. […] Advise against participating in contact sports which risk abdominal trauma, such as rugby or boxing, as this can provoke haematuria or even cause the rupture of an enlarged kidney. […] Much of the morbidity and mortality of ADPKD is due to CVD. Thus, lifestyle advice regarding avoiding smoking, maintenance of a healthy body weight, diet (eg, limiting salt intake) and regular exercise (ideally of a low-impact nature, such as swimming or walking) would seem sensible, even if not studied within the ADPKD population specifically.

• #38 Autosomal Dominant Polycystic Kidney Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532934/

  Patients are instructed to drink three liters of fluids daily to suppress vasopressin, thereby decreasing cAMP production and inhibiting cyst production. […] All ADPKD patients are advised to limit their sodium intake to less than 2 grams per day. […] Management of hypertension is essential in reducing cardiovascular mortality and slowing the progression of renal failure. […] Tolvaptan slows the progression of autosomal dominant polycystic kidney disease by blocking the reception of vasopressin signaling at the V2 receptor, lowering the intracellular cyclic AMP that would otherwise stimulate cystic proliferation and growth. […] The decision to undergo genetic testing or renal ultrasonography for asymptomatic children should be collaborative, involving caretakers, parents, and healthcare professionals.

• #39 Polycystic kidney disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/symptoms-causes/syc-20352820

  If you have polycystic kidney disease and you’re thinking about having children, a genetic counselor can help you know your risk of passing the disease to your children. […] Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. It’s most important to manage your blood pressure. […] Here are some tips for keeping your blood pressure in check: […] Take the blood pressure medicines your healthcare professional prescribes as directed. […] Eat a low-salt diet that has plenty of fruits, vegetables and whole grains. […] Get to and stay at a healthy weight. […] Exercise regularly. Aim for at least 30 minutes of moderate physical activity most days of the week. […] Limit alcohol use. […] Don’t smoke.

• #40 Preventive project of Autosomal Dominant Polycystic Kidney Disease (ADPKD) | Nefrología

  https://www.revistanefrologia.com/en-preventive-project-autosomal-dominant-polycystic-articulo-S2013251421001334

  Autosomal dominant polycystic kidney disease (ADPKD, Q61.2) is a multisystemic genetic pathology with renal impact; half of the affected patients require renal replacement therapy (RRT) and almost all die with some degree of renal failure. […] Since 2009, our group has studied its impact and recommends an approach based on prevention: to encourage couples with affected members to have healthy children who do not transmit the disease. […] Our proposal, summarized in Fig. 1 using the Preventive Hexagon, is based on: a) locating unknown cases within affected families, b) recovering lost cases for follow up and control, c) diagnosing the disease before having the first child d) adequate information to patients and relatives, e) facilitate the most appropriate reproductive options for each couple aiming to have healthy children and f) promote informed parenthood without coercion.

• #41 Preventive project of Autosomal Dominant Polycystic Kidney Disease (ADPKD) | Nefrología

  https://revistanefrologia.com/en-preventive-project-autosomal-dominant-polycystic-articulo-S2013251421001334

  Autosomal dominant polycystic kidney disease (ADPKD, Q61.2) is a multisystemic genetic pathology with renal impact; half of the affected patients require renal replacement therapy (RRT) and almost all die with some degree of renal failure. […] Since 2009, our group has studied its impact and recommends an approach based on prevention: to encourage couples with affected members to have healthy children who do not transmit the disease. […] Our proposal, summarized in Fig. 1 using the Preventive Hexagon, is based on: a) locating unknown cases within affected families, b) recovering lost cases for follow up and control, c) diagnosing the disease before having the first child d) adequate information to patients and relatives, e) facilitate the most appropriate reproductive options for each couple aiming to have healthy children and f) promote informed parenthood without coercion.

• #42 Preventive project of Autosomal Dominant Polycystic Kidney Disease (ADPKD) | Nefrología

  https://www.revistanefrologia.com/en-preventive-project-autosomal-dominant-polycystic-articulo-S2013251421001334

  Autosomal dominant polycystic kidney disease (ADPKD, Q61.2) is a multisystemic genetic pathology with renal impact; half of the affected patients require renal replacement therapy (RRT) and almost all die with some degree of renal failure. […] Since 2009, our group has studied its impact and recommends an approach based on prevention: to encourage couples with affected members to have healthy children who do not transmit the disease. […] Our proposal, summarized in Fig. 1 using the Preventive Hexagon, is based on: a) locating unknown cases within affected families, b) recovering lost cases for follow up and control, c) diagnosing the disease before having the first child d) adequate information to patients and relatives, e) facilitate the most appropriate reproductive options for each couple aiming to have healthy children and f) promote informed parenthood without coercion.

• #43 Polycystic Kidney Disease (PKD) Symptoms, Treatments & Causes – American Kidney Fund (AKF)

  https://www.kidneyfund.org/all-about-kidneys/types-kidney-diseases/polycystic-kidney-disease

  How can I prevent ADPKD from causing kidney failure? After you find out that you have ADPKD, it is important to work with your doctor to understand your risk for progressing to kidney failure and learn about what you can do to manage it. Following your management plan and making healthy life changes may be able to help you keep your kidneys working longer and slow progression to kidney failure. […] Work with your doctor to prevent or manage diabetes and high blood pressure. […] Keep a healthy weight. […] Hydration: Drinking more fluid can help slow the progression of ADPKD. It is important for you to discuss the amount with your doctor. […] Take all of your prescription medicines as your doctor tells you. […] Follow a kidney-friendly eating plan. A dietitian can help you make a plan that works for you. […] Be active for at least 30 minutes most days of the week. This can include activities such as: […] Drink less alcohol. The healthy guidelines for drinking alcohol are: […] Quit smoking or using tobacco.

• #44

  https://link.springer.com/article/10.1007/s00467-021-04974-4

  Significant progress has been made in understanding the genetic basis of autosomal dominant polycystic kidney disease (ADPKD), quantifying disease manifestations in children, exploring very-early onset ADPKD as well as pharmacological delay of disease progression in adults. […] We propose an algorithm for work-up and management based on current recommendations that integrates the need to screen regularly for hypertension and proteinuria in offspring of affected parents with different options regarding diagnostic testing, which need to be discussed with the family with regard to ethical and practical aspects. […] Pharmacological management includes renin-angiotensin system blockade as first-line therapy for hypertension and proteinuria. […] The vasopressin receptor antagonist tolvaptan is licensed for delaying disease progression in adults with ADPKD who are likely to experience kidney failure.

• #45 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Early detection and treatment of hypertension have significant benefits, as they prevent left ventricular hypertrophy, reduce albuminuria and slow kidney cyst growth. […] The treatment of hypertension follows standard guidelines and should be integrated with routine screening for other cardiovascular disease risk factors (such as hyperlipidaemia and impaired glucose tolerance). […] In patients with early-stage disease (eGFR 60 mL/min/1.73 m2), the recommended blood pressure target is between 120/70 mmHg and 130/80 mmHg. […] Tolvaptan is a specific oral vasopressin type 2 receptor antagonist and is indicated in patients with ADPKD at high risk of developing kidney failure. […] The main adverse effect of tolvaptan is massive aquaresis (mean urine volume of 57 L/day) due to the off-target suppression of vasopressin-mediated water reabsorption in the collecting duct. This occurs in all patients and requires behavioural adaptation to increase daily fluid intake.

• #46 Drug management of autosomal dominant polycystic kidney disease

  https://australianprescriber.tg.org.au/articles/drug-management-of-autosomal-dominant-polycystic-kidney-disease.html

  Autosomal dominant polycystic kidney disease is the most common genetic kidney disease affecting adults. Approximately 60% of patients develop kidney failure by 60 years of age due to slowly expanding kidney cysts. […] A healthy lifestyle and rigorous control of blood pressure slow kidney cyst growth. These interventions can be effective in reducing progression to kidney failure and cardiovascular disease, especially if started in early adulthood. […] Encouraging young adults with ADPKD to engage in their health during the early asymptomatic period provides the best opportunity to significantly delay the onset of kidney failure and prevent cardiovascular disease. In particular, there is good evidence that lifestyle modifications (smoking cessation, weight reduction, aiming for a body mass index less than 25 kg/m2, reduction in dietary sodium intake to 80-100 mmol/day and regular physical activity) slow kidney cyst growth and the decline in kidney function. Providing education via PKD Australia fact sheets and genetic counselling are also recommended.

• #47

  https://link.springer.com/article/10.1007/s00467-021-04974-4

  The ongoing trial on the effect of tolvaptan in children is an important step forward to studying disease modifying agents for pediatric ADPKD. […] However, less industry-driven research into the effects of statins in children and non-pharmacological interventions such as low-salt and high-water intake is also needed. […] Considering the high prevalence of cardiovascular morbidity in adults with ADPKD, which often precedes loss of kidney function, opportunities for early control of hypertension and establishing healthy lifestyle habits should not be missed.

• #48 ADPKD: Symptoms, Treatment, Screenings, Diagnosis, and More

  https://www.healthline.com/health/adpkd/everything-to-know-symptoms-treatments

  To help slow the development of ADPKD, your doctor may prescribe tolvaptan (Jynarque). […] This medication may help delay or prevent kidney failure. […] Managing ADPKD and sticking with your treatment plan can be challenging, but its vital for slowing the progression of the disease. […] Practicing healthy habits and following your doctors recommended treatment plan may help prevent complications and keep your kidneys healthier for longer.

• #49 Polycystic kidney disease – Symptoms, Causes, Types,Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact Num

  https://www.pacehospital.com/polycystic-kidney-disease-types-symptoms-causes-prevention-treatment

  There is currently no known method for polycystic kidney disease prevention. On the other hand, kidney damage and other polycystic kidney disease issues brought on by high blood pressure might be slowed down by managing blood pressure at 120/80mmhg. […] However, some preventive strategies are available to help manage symptoms, prevent or delay kidney function loss in patients with polycystic kidney disease, and help decrease the growth of cysts. They consist of: […] A healthy way of living that includes smoking cessation, exercising, controlling weight, and consuming less salt […] Consuming a large amount of plain water all day long […] Limiting the amount of caffeine in all drinks […] Consuming a lot of liquids as soon as blood in the urine is noticed […] Cautious blood pressure monitoring.

• #50

  https://www.nhs.uk/conditions/autosomal-dominant-polycystic-kidney-disease-adpkd/

  There’s currently no cure for ADPKD, however: […] a healthy lifestyle may help to protect your kidney function […] various treatments are available to manage problems caused by the condition.

• #51 Preventive project of Autosomal Dominant Polycystic Kidney Disease (ADPKD) | Nefrología

  https://www.revistanefrologia.com/en-preventive-project-autosomal-dominant-polycystic-articulo-S2013251421001334

  Public Health Systems (SSP) must be sensitive and understand the needs of these patients and families and, just as dialysis and transplantation are facilitated, it must also provide primary prevention measures, which will contribute to a decrease in prevalence and incidence of this disease in two or three generations. […] Lastly, reorienting the focus towards primary prevention also has its economic advantages since it is a cost-efficient model and its implementation will make the health system more sustainable.

• #52

• #53

  https://link.springer.com/article/10.1007/s00467-021-04974-4

  The ongoing trial on the effect of tolvaptan in children is an important step forward to studying disease modifying agents for pediatric ADPKD. […] However, less industry-driven research into the effects of statins in children and non-pharmacological interventions such as low-salt and high-water intake is also needed. […] Considering the high prevalence of cardiovascular morbidity in adults with ADPKD, which often precedes loss of kidney function, opportunities for early control of hypertension and establishing healthy lifestyle habits should not be missed.

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