Materiały źródłowe

• #1 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK373382/

  Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threatening single-gene disease. It affects up to 15 million people worldwide with 50% risk for end-stage kidney disease, 80% risk for hypertension, 60% risk for painful kidney complications, 20% risk for symptomatic polycystic liver disease and 3% risk for intra-cerebral aneurysm rupture. […] For a long time, the treatment and management strategies of this disease have not progressed in comparison with the treatment of other kidney diseases. Recently, there have been new therapeutic hopes with identification of specific drugs based on the mechanisms of kidney progression. […] In this chapter, we will summarize the key findings, highlight recent developments, and look ahead to the changes in clinical practice that will likely arise from the adoption of an updated management framework for this major kidney disease.

• #2 Autosomal dominant polycystic kidney disease with ectopic unilateral multicystic kidney: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-023-04305-1

  Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and the fourth cause of death of end-stage renal disease. The disease has a prevalence of 1:4001:1000 accounting for 10% of patients on dialysis. In most ADPKD patients, bilateral kidneys are similarly affected, with numerous fluid-filled cysts arising from different nephron segments. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic. […] He was put on antihypertensives, analgesia for the left flank pain and to have follow up at the urology and nephrology departments.

• #2 Autosomal Dominant Polycystic Kidney Disease – NIDDK

  https://www.niddk.nih.gov/health-information/kidney-disease/polycystic-kidney-disease/autosomal-dominant-pkd

  Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of PKD. […] Health care providers usually diagnose ADPKD between the ages of 30 and 50, when signs and symptoms start to appear, which is why it is sometimes called adult PKD. […] Most people with ADPKD have pain, high blood pressure, and kidney failure at some point in their lives. […] High blood pressure increases your chances of heart disease and stroke. High blood pressure can also damage your kidneys even more. Keep your blood pressure under control to help delay kidney damage. […] More than half of people with ADPKD progress to kidney failure by age 70. […] Although a cure doesn’t exist yet for ADPKD, treatment can help reduce your complications, which can help you live longer. […] A health care provider needs to find the source of your pain before he or she can treat it.

• #3 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: […] People with ADPKD will usually be seen once a year by a kidney specialist to monitor their general health and kidney function. More frequent monitoring will be needed if blood tests show signs of kidney damage. […] Half of people with ADPKD will develop kidney failure by the age of 60 and will need dialysis or a kidney transplant. However, with the right treatments most people with ADPKD remain in good health for decades.

• #4 Polycystic Kidney Disease condition | Children’s Wisconsin

  https://childrenswi.org/medical-care/nephrology/conditions/polycystic-kidney-disease

  Autosomal dominant PKD (inherited)- „Autosomal dominant” means that if one parent has the disease, there is a 50 percent chance that the disease will pass to any child, and that boys and girls are equally affected. […] If a positive diagnosis is made, we design a treatment plan specifically for your child, which may include one or more of the following: […] Monitoring and treatment for high blood pressure […] Specific dietary modifications […] Regular monitoring of kidney function, and kidney imaging (ultrasound) […] Specific guidelines on the use of medications which may adversely affect disease progression Guidelines on participation in athletics, including the use of kidney shields […] Education; including regular updates of new disease-specific therapies now in clinical trials

• #5 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK373382/

  Hypertension is a common and serious complication of ADPKD, often occurring in 50% – 70% of ADPKD patients early in the disease, before appearance of renal dysfunction. […] Early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients. […] In patients with renal disease, the goal is a blood pressure of less than 130/80 mm Hg. In patients with ADPKD we have to adopt this blood pressure target. Hypertension is the most important modifiable risk factor in ADPKD and better blood pressure control allows slowing down the progression of kidney disease. […] Current published data confirm that patients with ADPKD in the United States, Denmark and Great Britain are having a better prognosis. […] Therefore, early control of hypertension is very important in patients with ADPKD to slow down kidney disease progression and prevent occurrence of cardiovascular events.

• #6 Autosomal Dominant Polycystic Kidney Disease – Cystic Urologic Disorders for Medicine

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/cystic-urologic-disorders-39445/autosomal-dominant-polycystic-kidney-disease_50337

  ADPKD is the leading genetic cause of end-stage renal disease (ESRD) and is the fourth overall cause of ESRD. Over the course of their lifetimes, patients will experience progressive renal failure related to enlargement of tubular cysts. Patients with ADPKD have a 50% chance of progressing to ESRD by age 70. […] Early polycystic kidney disease is typically asymptomatic, and diagnosis is therefore often delayed in patients with no known family history of ADPKD. Hypertension is most commonly one of the first presenting signs, and is seen in 40-50% of patients on initial presentation. […] Patients with ADPKD are likely to form cysts in locations other than the kidneys. The most common extrarenal site of cyst formation is the liver, as over 80% of patients with ADPKD have been shown to develop hepatic cysts at some point over the course of their lifetime.

• #7 Polycystic Kidney Disease (PKD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5791-polycystic-kidney-disease

  Polycystic kidney disease (PKD) is a genetic disorder that causes cysts to grow in the kidneys. Health complications include high blood pressure and kidney failure. Most people with PKD will eventually need dialysis or a kidney transplant. PKD causes chronic kidney disease, which can progress to kidney failure. If you receive a PKD diagnosis, it’s important to work with your healthcare provider on a treatment plan to manage complications of the disease. […] ADPKD is the most common form of PKD. Most people receive an ADPKD diagnosis in adulthood between the ages of 30 and 50. People with the dominant type of PKD often don’t show signs of the disease until they reach adulthood. Symptoms of ADPKD include: Pain in your back or sides (flank pain), High blood pressure, Headaches, Hematuria (blood in your pee), Urinary tract infections (UTIs), Kidney stones.

• #8 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. […] Autosomal dominant PKD (ADPKD) is the most common inherited form of polycystic kidney disease. […] Autosomal dominant PKD can lead to kidney failure. […] Symptoms usually develop between the ages of 30 and 40 (but can begin earlier), and may include: high blood pressure (may occur before cysts appear), pain in the back or sides, headaches, enlarged and painful abdomen, blood in the urine (haematuria), urinary tract infections, kidney stones, liver, intestine, brain and pancreatic cysts, abnormal heart valves, abdominal wall hernias, reduced kidney function or kidney failure.

• #9

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  Pain related to increasing kidney size and cyst complications is reported in 50% of patients with ADPKD, with 20% reporting pain often, usually, or always. […] Pain remains an issue as disease progresses with 44% of transplant and 59% of dialysis patients reporting pain. […] Patients desire support from their providers, open and honest conversations regarding pain management, and information and resources to learn more about their condition. […] Despite patients reporting pain management as a top priority in ADPKD outcomes, many feel that their pain is underestimated by their physician and wish for more discussion surrounding pain management and strategies. […] The ADPKD Pain and Discomfort Scale was developed and validated to specifically evaluate the severity and type of pain experienced by patients with ADPKD.

• #10 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of kidney disease. […] Early detection and management of hypertension are important to delay the progression of renal dysfunction and development of cardiovascular complications. […] Pain management includes evaluation of concomitant illnesses, use of analgesics, and adjuvant therapy. […] Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers are recommended for initial hypertension management in patients with ADPKD. […] The goal of therapy should be blood pressure of less than 140/90 mm Hg in those who have developed chronic kidney disease; otherwise, the blood pressure targets are the same as those for the general population. […] Pain is the most common symptom in those with ADPKD and may present as back, chest, abdominal, or flank pain, or a combination.

• #11 Autosomal Dominant Polycystic Kidney Disease – Cystic Urologic Disorders for Medicine

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/cystic-urologic-disorders-39445/autosomal-dominant-polycystic-kidney-disease_50337

  ADPKD is the leading genetic cause of end-stage renal disease (ESRD) and is the fourth overall cause of ESRD. Over the course of their lifetimes, patients will experience progressive renal failure related to enlargement of tubular cysts. Patients with ADPKD have a 50% chance of progressing to ESRD by age 70. […] Early polycystic kidney disease is typically asymptomatic, and diagnosis is therefore often delayed in patients with no known family history of ADPKD. Hypertension is most commonly one of the first presenting signs, and is seen in 40-50% of patients on initial presentation. […] Patients with ADPKD are likely to form cysts in locations other than the kidneys. The most common extrarenal site of cyst formation is the liver, as over 80% of patients with ADPKD have been shown to develop hepatic cysts at some point over the course of their lifetime.

• #12 Polycystic Kidney Disease | UCSF Department of Surgery

  https://hpbsurgery.ucsf.edu/condition/polycystic-kidney-disease

  The UCSF PKD Center of Excellence is a comprehensive clinic dedicated to the care of individuals with polycystic kidney disease. […] The PKD Center of Excellence is experienced in navigating all related medical conditions and engaging appropriate experts to provide patients with the best possible care. […] Autosomal dominant PKD is the most common inherited disorder of the kidneys. […] Many people with autosomal dominant PKD live for several decades without developing symptoms. […] The kidneys enlarge along with the cysts-which can number in the thousands-while roughly retaining their kidney shape. […] High blood pressure is common and develops in most patients by age 20 or 30. […] People with autosomal dominant PKD also can experience the following complications: urinary tract infections-specifically, in the kidney cysts, hematuria-blood in the urine, liver and pancreatic cysts, abnormal heart valves, high blood pressure, kidney stones, aneurysms-bulges in the walls of blood vessels-in the brain, diverticulosis-small pouches bulge outward through the colon.

• #13 Polycystic Kidney Disease | UCSF Department of Surgery

  https://hpbsurgery.ucsf.edu/condition/polycystic-kidney-disease

  Autosomal dominant PKD is usually diagnosed by kidney imaging studies. […] In most cases of autosomal dominant PKD, patients have no symptoms and their physical condition appears normal for many years, so the disease can go unnoticed. […] Once cysts have grown to about one-half inch, however, diagnosis is possible with imaging technology. […] Although a cure for autosomal dominant PKD is not available, treatment can ease symptoms and prolong life. […] Pain in the area of the kidneys can be caused by cyst infection, bleeding into cysts, kidney stone, or stretching of the fibrous tissue around the kidney with cyst growth. […] People with autosomal dominant PKD tend to have frequent urinary tract infections, which can be treated with antibiotics. […] Keeping blood pressure under control can slow the effects of autosomal dominant PKD.

• #14 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Intracranial aneurysm represents the most severe extra-renal manifestation in those with ADPKD. […] Fertility rates for both men and women with ADPKD who have not developed significant renal dysfunction are comparable to those of the general population. […] All patients with ADPKD develop a progressive loss of renal function, with nearly 80% of patients dying or developing end-stage renal disease by 70 years of age. […] Patients with a family history of ADPKD are candidates for screening, typically with imaging studies. […] Although computed tomography and magnetic resonance imaging are slightly more sensitive for detecting renal cysts, ultrasonography is the preferred screening modality because of lower cost, increased availability, and lack of radiation exposure.

• #15 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK373382/

  Patients with ADPKD should avoid violent sports. […] Non-hypertensive ADPKD patients with normal kidney function should undergo blood and urinary testing and ultrasonography of the kidneys every year. ADPKD patients with high blood pressure, chronic kidney disease or cardiovascular complications require more frequent monitoring, based on the severity of hypertension and stage of chronic kidney disease. […] Improvements in screening and diagnosis of ADPKD have allowed earlier diagnosis of disease, later onset of ESKD and better survival. However, the main and most effective therapy remains control of hypertension. Therefore, early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients.

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

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

  If blood pressure and blood chemistry are normal then annual blood tests and ultrasound are sufficient to monitor the disease. […] Current advice is that blood pressure should be controlled to a target of 130/80 mm Hg. […] Treat UTIs as they occur, as for the general population. […] Patients should become familiar with self-management for straightforward repeated episodes. […] As end-stage kidney disease is reached, dialysis is required. […] Renal transplantation has similar outcomes for patients with ADPKD as for those without the disease.

• #17 Autosomal dominant polycystic kidney disease (ADPKD): Treatment – UpToDate

  https://www.uptodate.com/contents/autosomal-dominant-polycystic-kidney-disease-adpkd-treatment

  Autosomal dominant polycystic kidney disease (ADPKD): Treatment […] INTRODUCTION — Polycystic kidney disease (PKD) includes inherited diseases that cause an irreversible decline in kidney function. PKD is most commonly encountered as an autosomal dominant disease, while the rare autosomal recessive form represents a different entity. The autosomal dominant form (autosomal dominant PKD [ADPKD]) is the most common genetic cause of chronic kidney disease (CKD). The majority of individuals with PKD eventually require kidney replacement therapy. […] IDENTIFICATION OF HIGH-RISK PATIENTS — The identification of individuals who are at high risk for progression of chronic kidney disease (CKD) is important for prognostic reasons and to identify patients who may benefit from specific therapies, such as tolvaptan. Our preferred method for identifying high-risk patients with ADPKD is the Mayo classification system, which categorizes patients into five prognostic classes from the lowest to the highest risk for disease progression (classes 1A, 1B, 1C, 1D, and 1E). Classes 1C, 1D, and 1E are defined as high risk for progression to end-stage kidney disease (ESKD).

• #18

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

  Non-pharmacological interventions in this population also deserve further study. […] Good long-term care often still requires overcoming a mindset of ADPKD as an adult disease and should be viewed as repeated opportunities for screening and counselling, two pediatric key areas of expertise. […] Based on the relatively high incidence of hypertension and proteinuria detailed above, two recent clinical guidelines recommend to examine urine and blood pressure in all at-risk children at regular intervals. […] Antihypertensive and antiproteinuric treatment have the dual aims of lowering cardiovascular mortality and preserving kidney function, because blood pressure is one of the important modifiable risk factors. […] There are universal recommendations to lower blood pressure in children with hypertension and especially children with CKD with a host of evidence, but we will focus on reviewing ADPKD-specific evidence here.

• #19

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

  Testing for hypertension and proteinuria can be done without revealing transmission status in children who have neither signs nor symptoms of ADPKD while children with positive findings can undergo focused work-up. […] Initiating age-appropriate communication about the risk of disease early on helps to improve family communication and improves long-term coping strategies. […] The only randomized controlled trial in children with ADPKD was much smaller and stratified into three groups of children with normal (75th percentile), high-normal (75th95th percentile) and high blood pressure (95th percentile). […] 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. […] However, before tolvaptan or other drugs can be used in children with ADPKD, valid prediction models are urgently needed to select appropriate patient populations and the long-term safety and sustained effect need to be verified.

• #20 International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-019-0155-2

  We recommend that children with ADPKD should have their blood pressure measured at least once a year (that is, at the same interval as healthy children). […] We suggest that children with ADPKD receive antihypertensive treatment if their blood pressure repeatedly exceeds the ninetieth percentile or is 130/85mmHg if age 16 years. […] We recommend using angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers as first-line antihypertensive treatment in children with ADPKD who have hypertension and albuminuria. […] We recommend that children with ADPKD should aim to achieve the recommended intake for healthy children, which may require extra assistance (for example, advice from a dietician). […] Families should be encouraged to openly discuss their disease and future genetic risks with their children, for example, by provision of age-appropriate information and by providing support for family members in managing their own and their children’s emotions.

• #21 Polycystic Kidney Disease, Autosomal Dominant | UCSF Health

  https://www.ucsfhealth.org/conditions/polycystic-kidney-disease-autosomal-dominant

  Autosomal dominant PKD causes fluid-filled cysts to grow in the kidneys. Cysts may also form in other organs, including the liver and pancreas. For many patients, so many cysts develop that they eventually cause kidney failure, making dialysis or a transplant necessary. […] UCSF provides comprehensive care for patients with the autosomal dominant form of polycystic kidney disease (PKD). Our team includes specialists in nephrology, hepatology, cardiology, radiology, reproductive endocrinology and infertility, maternal-fetal medicine, nutrition and genetics. Because PKD can affect many organ systems in addition to the kidneys, we are experienced in navigating all PKD-related medical conditions and work together to provide you with the best possible care at every stage of the disease. […] Unfortunately, there is no cure for autosomal dominant PKD. Once a diagnosis is made, your doctor will want to work with you to reduce pain and discomfort, minimize complications and keep your kidneys healthy for as long as possible.

• #22 Polycystic Kidney Disease | The University of Kansas Health System

  https://www.kansashealthsystem.com/care/conditions/polycystic-kidney-disease

  At The University of Kansas Health System, we provide: A multidisciplinary approach, including shared care with referring physicians. A nationally recognized, complete continuum of kidney care, including dialysis and transplant, as well as use of tolvaptan, the first and only FDA-approved medication to treat PKD, slowing the disease’s progression. […] Our multidisciplinary dialysis team includes physicians, registered nurses, dietitians and social workers to provide education and support, and to help you manage your condition and maximize your quality of life.

• #23

  https://www.nursingcenter.com/journalarticle?Article_ID=5685377&Journal_ID=54012&Issue_ID=5685268

  Autosomal dominant polycystic kidney disease causes chronic kidney disease and end-stage renal disease. […] Although there is no known cure, NPs are uniquely positioned to help patients manage their symptoms and delay onset of kidney failure and need for dialysis.

• #24 Autosomal Dominant Polycystic Kidney Disease –… | FreeCME.com

  https://www.freecme.com/products/autosomal-dominant-polycystic-kidney-disease-understanding-the-disease-and-improving-treatment-adherence-and-outcome-2

  In the real-world setting, treatment adherence in patients with autosomal dominant polycystic kidney disease (ADPKD) can be a challenge. […] This CME program will discuss the diagnosis and management of ADPKD as well as strategies on improving treatment adherence. […] Develop evidence-based strategies to counter the aquaretic effect from disease modifying therapy. […] Dr. Noce is a nephrology nurse practitioner at Yale-New Haven Hospital in New Haven, CT, where provides care for patients across the spectrum of renal diseases. […] She is a core member of the PKD Foundation Center of Excellence program at Yale, where she provides expertise in the holistic care of patients with ADPKD, including patient counseling and medication management. […] The maximum number of hours awarded for this Nursing Continuing Professional Development activity is 1.0 contact hours.

• #25

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We encourage patients to check BP at home, with a goal BP of 110/75 for younger high-risk patients and 120/80 for older patients with kidney function decline. […] For patients with preserved kidney function, we encourage a low salt diet, high in potassium, with 3 L of fluid intake, and maintenance of an ideal body weight. […] We discuss the use of hormonal therapy with our female patients, with a recommendation for avoidance for those at higher risk of PLD progression and individualized counseling for those at lower risk. […] Somatostatin analogs administered by intramuscular injection (octreotide, lanreotide) are effective in decreasing liver growth and may be considered for symptomatic patients, for whom surgical intervention is not appropriate. […] Liver transplantation is warranted in patients with extremely debilitating symptoms, seriously compromised functional status and reduced quality of life, untreatable complications, such as portal hypertension, and severe malnutrition with bilobar extensive cystic liver disease lacking adequate areas of parenchymal sparing. […] Kidney stone management in patients with ADPKD generally follows the same guidance as management in patients without ADPKD. […] Tolvaptan may confer protective effects against stone formation.

• #26

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  Patients with autosomal dominant polycystic kidney disease benefit from specialized care over their lifetimes, starting with diagnosis of the condition with ongoing discussion of both the renal course and extra-renal issues. […] Both renal and extra-renal issues may continue to cause major morbidity even after successful kidney transplant or initiation of RRT, and extra-renal disease aspects should always be considered as part of routine management. […] In this review, we will focus on updates in pain/depression screening, cardiac manifestations, liver and pancreatic cysts, kidney stone management, and genetic counseling. […] ADPKD significantly affects health-related quality of life. […] Pain is a primary driver of the mental and physical burden of the disease, contributing substantially to reductions in health-related quality of life.

• #27

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We propose asking about each pain type (sharp, dull, or fullness or discomfort) at each clinical encounter, with further exploration of the domains of severity or interference with a positive response as a way of engaging in a discussion of individual patient concerns. […] Between 22% and 60% of patients with ADPKD have depression, and therefore, we also recommend screening. […] Given the negative effects of depression on mental and physical health of patients with ADPKD, it is important to screen for depression early on in ADPKD disease course and at regular intervals throughout management. […] The US Preventative Services Taskforce recommends that all adults should be screened for depression using evidence-based protocols, such as various iterations of the Patient Health Questionnaire.

• #28

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We propose asking about each pain type (sharp, dull, or fullness or discomfort) at each clinical encounter, with further exploration of the domains of severity or interference with a positive response as a way of engaging in a discussion of individual patient concerns. […] Between 22% and 60% of patients with ADPKD have depression, and therefore, we also recommend screening. […] Given the negative effects of depression on mental and physical health of patients with ADPKD, it is important to screen for depression early on in ADPKD disease course and at regular intervals throughout management. […] The US Preventative Services Taskforce recommends that all adults should be screened for depression using evidence-based protocols, such as various iterations of the Patient Health Questionnaire.

• #29 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK373382/

  Hypertension is a common and serious complication of ADPKD, often occurring in 50% – 70% of ADPKD patients early in the disease, before appearance of renal dysfunction. […] Early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients. […] In patients with renal disease, the goal is a blood pressure of less than 130/80 mm Hg. In patients with ADPKD we have to adopt this blood pressure target. Hypertension is the most important modifiable risk factor in ADPKD and better blood pressure control allows slowing down the progression of kidney disease. […] Current published data confirm that patients with ADPKD in the United States, Denmark and Great Britain are having a better prognosis. […] Therefore, early control of hypertension is very important in patients with ADPKD to slow down kidney disease progression and prevent occurrence of cardiovascular events.

• #30 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK373382/

  Patients with ADPKD should avoid violent sports. […] Non-hypertensive ADPKD patients with normal kidney function should undergo blood and urinary testing and ultrasonography of the kidneys every year. ADPKD patients with high blood pressure, chronic kidney disease or cardiovascular complications require more frequent monitoring, based on the severity of hypertension and stage of chronic kidney disease. […] Improvements in screening and diagnosis of ADPKD have allowed earlier diagnosis of disease, later onset of ESKD and better survival. However, the main and most effective therapy remains control of hypertension. Therefore, early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients.

• #31

  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. […] If you’re diagnosed with ADPKD, you’ll usually see a kidney specialist, who can help draw up a suitable treatment plan. […] Medication is usually used to treat high blood pressure in people with ADPKD. […] Angiotensin-converting enzyme (ACE) inhibitors and angiotensin-2 receptor blockers (ARBs) are the 2 medications most widely used. […] In many cases of ADPKD, any pain you experience can be relieved by treating the underlying cause, such as kidney stones or a urinary tract infection (UTI). […] If you need to take a painkiller, paracetamol is the best medication to try first.

• #32 Autosomal Dominant Polycystic Kidney Disease (ADPKD) – Genitourinary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/genitourinary-disorders/cystic-kidney-disease/autosomal-dominant-polycystic-kidney-disease-adpkd

  Autosomal dominant polycystic kidney disease usually causes no symptoms initially; half of patients remain asymptomatic, never develop renal insufficiency or failure, and are never diagnosed. Most patients who develop symptoms do so by the end of their 20s. […] Symptoms include low-grade flank, abdominal, and lower back pain due to cystic enlargement and symptoms of infection. Acute pain, when it occurs, is usually due to hemorrhage into cysts or passage of a calculus. Fever is common with acute pyelonephritis, and rupture of cysts into the retroperitoneal space may cause a fever that can last for weeks. […] Strict control of hypertension is essential. Typically an angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB) is used. In addition to controlling blood pressure, these medications help block angiotensin and aldosterone, growth factors that contribute to renal scarring and loss of renal function.

• #33 Autosomal dominant polycystic kidney disease (ADPKD): Treatment – UpToDate

  https://www.uptodate.com/contents/autosomal-dominant-polycystic-kidney-disease-adpkd-treatment

  INITIAL MANAGEMENT FOR ALL PATIENTS — Management of blood pressure — In young, healthy individuals aged 18 to 50 years with estimated glomerular filtration rate (eGFR) >60 mL/min/1.73 m², we generally use an angiotensin-converting enzyme (ACE) inhibitor or an angiotensin receptor blocker (ARB) to target a blood pressure of <110/75 mmHg using home blood pressure measurements or daytime ambulatory blood pressure monitoring. In such patients, intensive blood pressure lowering may reduce the rate of kidney growth and, in high-risk patients (Mayo class ID and IE), may slow the rate of decline in eGFR. Dietary sodium restriction — We advise all PKD patients to restrict dietary sodium, with a goal of 2 grams intake per day or less (approximately 5 grams of salt). In order to ensure adherence, the authors measure a 24-hour urine sodium and obtain a nutrition consult at least once during the course of the management. Increased fluid intake — We advise patients with ADPKD to drink >3 L of fluid per day, unless the eGFR is <30 mL/min/1.73 m² or the patient is at risk for hyponatremia (eg, taking a thiazide diuretic).

• #34

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

  Antidepressants or anticonvulsants, which are normally used to treat epilepsy, may be prescribed for long-term (chronic) pain. These medications can also be used to relieve some types of pain. […] You’ll have blood tests at varying intervals to monitor your kidney function. […] You should discuss with your doctors what treatment you’d like if your condition reaches a stage where your kidneys stop working altogether (kidney failure). […] The 2 main treatment options for kidney failure are: dialysis, where a machine replicates some of your kidneys’ functions; a kidney transplant, where a healthy kidney is removed from a living or recently deceased donor and implanted into someone with kidney failure. […] Tolvaptan is a medication that’s recommended by the National Institute for Health and Care Excellence (NICE) to treat ADPKD in adults. […] It can be used to slow down the growth of cysts, reducing overall kidney growth and preserving kidney function for longer. […] If you have ADPKD, your kidneys will be more vulnerable to injury. […] Avoiding all types of contact sport, such as rugby and football, is usually recommended.

• #35 Polycystic kidney disease – Medthority

  https://www.medthority.com/specialty/nephrology/polycystic-kidney-disease/

  Autosomal dominant PKD is one of the most common genetic disorders and the fourth leading cause of end-stage kidney disease (ESKD) in children and adults. […] Treatment of polycystic kidney disease consists of lifestyle modifications to enhance hydration and limit sodium intake, pharmacological therapy to manage complications, and general supportive measures, such as counselling. […] Antihypertensive agents have been the mainstay of treatment as hypertension is one of the most common complications of PKD; 50-70% of people with autosomal dominant PKD and up to 80% of children with autosomal recessive PKD develop hypertension. […] Identification of vasopressin as a key agent in the pathogenesis of PKD, through activation of vasopressin 2 receptors (V2R), has enabled the development of disease-modifying drugs that target cyst-forming pathways. […] Tolvaptan, a V2R antagonist, is the first disease-modifying drug to have been developed for the treatment of PKD. […] Tolvaptan has now been approved in several countries for treatment of autosomal dominant PKD in patients with rapidly progressing disease.

• #36 Autosomal dominant polycystic kidney disease (ADPKD): Treatment – UpToDate

  https://www.uptodate.com/contents/autosomal-dominant-polycystic-kidney-disease-adpkd-treatment

  ADDITIONAL MANAGEMENT FOR HIGH-RISK PATIENTS — Patients at high risk for progression may be identified based upon criteria described above. The major clinical challenge is to define the patients at risk for rapid progression who are the most likely to derive benefit from tolvaptan. Tolvaptan is a vasopressin V2-receptor (V2R) antagonist with proven beneficial results in ADPKD. Candidates for treatment — Unless contraindications are present or cost is prohibitive, we prescribe tolvaptan to adult patients with ADPKD who have an estimated glomerular filtration rate (eGFR) ≥25 mL/min1.73 m² and who are at risk of rapid progression. […] SPECIFIC MANAGEMENT OF END-STAGE KIDNEY DISEASE — Patients with ADPKD who progress to end-stage kidney disease (ESKD) require kidney replacement therapy. Peritoneal dialysis is less commonly performed than hemodialysis because it is perceived to be difficult for the patients to accommodate large volumes of peritoneal dialysate fluid in the setting of massively enlarged kidneys. Patients with ADPKD and ESKD are therefore most commonly treated with hemodialysis or undergo kidney transplantation.

• #37 Evaluating Treatments for Autosomal Dominant PKD

  https://www.uspharmacist.com/article/evaluating-treatments-for-autosomal-dominant-pkd

  Autosomal dominant polycystic kidney disease (ADPKD) is a progressively debilitating and often painful genetic disorder in which fluid-filled noncancerous cysts develop in the kidneys, causing the kidneys to enlarge and lose function over time. Approximately 140,000 people in the United States are diagnosed with ADKPD, which is the fourth leading cause of end-stage renal disease (ESRD). The treatment of ADPKD has been limited to the management of symptoms and complications. The only drug that is currently approved by the FDA for ADPKD is tolvaptan (Jynarque). Recent research has shown that a few other drugs are being studied and potentially repurposed for investigational usage for ADPKD. […] Tolvaptan, a selective vasopressin 2 receptor antagonist, has been proven to inhibit kidney cyst growth and preserve kidney function in multiple animal models of PKD. It is FDA approved to slow kidney function decline in adults who are at risk of rapidly progressing ADPKD. The TEMPO 3:4 trial was able to show that tolvaptan slowed the increase in total kidney volume and a decline in kidney function in patients with ADPKD; however, it was associated with a higher discontinuation rate due to the adverse effects.

• #38 Evaluating Treatments for Autosomal Dominant PKD

  https://www.uspharmacist.com/article/evaluating-treatments-for-autosomal-dominant-pkd

  Tolvaptan has a beneficial effect on ADPKD by slowing disease progression; however, it has a few drawbacks, such as cost and an increase in adverse events at higher doses when compared with placebo (including increased urination, thirst, nocturia, and liver injuries, which require frequent monitoring). Tolvaptan (Jynarque) is available only through a restricted distribution program under a Risk Evaluation and Mitigation Strategy (REMS) called the Jynarqu REMS Program because of the risks of liver injury. […] The drugs discussed can have an impact on reducing the decline in kidney function in both CKD and ADPKD, therefore delaying ESRD. The results of these studies suggest a range of potential therapies for ADPKD, but each has limitations. Everolimus showed some benefit in reducing kidney size but is not suitable for long-term use due to side effects, such as acute kidney injury. Tolvaptan, while effective in slowing disease progression by lowering an increase in total kidney volume and kidney function decline compared with placebo, has high cost and adverse effects (such as liver toxicity) that limit its use, especially in the long term.

• #39

  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. […] If you’re diagnosed with ADPKD, you’ll usually see a kidney specialist, who can help draw up a suitable treatment plan. […] Medication is usually used to treat high blood pressure in people with ADPKD. […] Angiotensin-converting enzyme (ACE) inhibitors and angiotensin-2 receptor blockers (ARBs) are the 2 medications most widely used. […] In many cases of ADPKD, any pain you experience can be relieved by treating the underlying cause, such as kidney stones or a urinary tract infection (UTI). […] If you need to take a painkiller, paracetamol is the best medication to try first.

• #40

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

  Antidepressants or anticonvulsants, which are normally used to treat epilepsy, may be prescribed for long-term (chronic) pain. These medications can also be used to relieve some types of pain. […] You’ll have blood tests at varying intervals to monitor your kidney function. […] You should discuss with your doctors what treatment you’d like if your condition reaches a stage where your kidneys stop working altogether (kidney failure). […] The 2 main treatment options for kidney failure are: dialysis, where a machine replicates some of your kidneys’ functions; a kidney transplant, where a healthy kidney is removed from a living or recently deceased donor and implanted into someone with kidney failure. […] Tolvaptan is a medication that’s recommended by the National Institute for Health and Care Excellence (NICE) to treat ADPKD in adults. […] It can be used to slow down the growth of cysts, reducing overall kidney growth and preserving kidney function for longer. […] If you have ADPKD, your kidneys will be more vulnerable to injury. […] Avoiding all types of contact sport, such as rugby and football, is usually recommended.

• #41 Autosomal Dominant Polycystic Kidney Disease – NIDDK

  https://www.niddk.nih.gov/health-information/kidney-disease/polycystic-kidney-disease/autosomal-dominant-pkd

  Always talk with a health care provider before taking any OTC medicines because some may be harmful to your kidneys. […] Controlling your blood pressure can slow the effects of ADPKD. […] ADPKD can eventually cause your kidneys to fail. People with kidney failure must have dialysis or a kidney transplant to replace their kidney function. […] Managing PKD successfully will probably include several lifestyle changes, such as changes in your physical activity level and what you eat. […] Visiting with a health care team on a regular basis is an important part of your routine as you work to limit your kidney problems.

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

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

  If you have hypertension and PKD, you can take steps to control your high blood pressure, which includes quitting smoking or using tobacco products and taking prescribed medicines for hypertension. […] Your doctor can recommend strategies to manage some of the symptoms and health problems caused by ADPKD. […] If you have ADPKD and are in pain, there are many ways to help you feel better. […] For long-lasting pain, your doctor might recommend stronger medicines like opioids or gabapentin, which can help with nerve pain. […] In some cases, if the pain is really bad and won’t go away, your doctor might talk to you about special procedures like nerve blocks or even surgery to remove cysts. […] Doctors usually use two classes of medicines, ACE inhibitors and ARBs, to help lower your blood pressure.

• #43 Polycystic Kidney Disease (PKD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5791-polycystic-kidney-disease

  Theres no cure for PKD. The goal of treatment is to slow the progression of the disease and control the symptoms it causes. The most common treatments for PKD include: Blood pressure management, Breathing support, Dialysis, Growth therapy, Kidney transplant, Pain management. […] Polycystic kidney disease isn’t preventable. But you may be able to slow the disease or prevent kidney failure by practicing a healthy lifestyle. People with ADPKD can live long and full lives if they receive the proper treatment and manage the condition. About half of people with ADKPD will need dialysis or a kidney transplant due to kidney failure by age 70. […] Living a healthy lifestyle can go a long way in managing PKD. Some tips for healthy living include: Eat a kidney-friendly diet, Exercise for 30 minutes most days, Maintain a weight that’s healthy for you, Manage blood pressure, Quit smoking or using tobacco, Avoid beverages containing alcohol, Reduce stress, Drink enough water and other noncaffeinated beverages, Get at least seven hours of sleep each night, Take all of your medication exactly as your provider prescribes. […] Working with healthcare providers who have experience treating polycystic kidney disease is important.

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

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

  Currently there is no cure for PKD. However, early detection and treatment can reduce or prevent some of the complications of PKD. […] Self-care suggestions for PKD generally include: changing your diet, making healthier lifestyle choices, avoiding non-steroidal anti-inflammatory drugs (NSAIDs), considering avoiding contact sports.

• #45

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We encourage patients to check BP at home, with a goal BP of 110/75 for younger high-risk patients and 120/80 for older patients with kidney function decline. […] For patients with preserved kidney function, we encourage a low salt diet, high in potassium, with 3 L of fluid intake, and maintenance of an ideal body weight. […] We discuss the use of hormonal therapy with our female patients, with a recommendation for avoidance for those at higher risk of PLD progression and individualized counseling for those at lower risk. […] Somatostatin analogs administered by intramuscular injection (octreotide, lanreotide) are effective in decreasing liver growth and may be considered for symptomatic patients, for whom surgical intervention is not appropriate. […] Liver transplantation is warranted in patients with extremely debilitating symptoms, seriously compromised functional status and reduced quality of life, untreatable complications, such as portal hypertension, and severe malnutrition with bilobar extensive cystic liver disease lacking adequate areas of parenchymal sparing. […] Kidney stone management in patients with ADPKD generally follows the same guidance as management in patients without ADPKD. […] Tolvaptan may confer protective effects against stone formation.

• #46 International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-019-0155-2

  We recommend that children with ADPKD should have their blood pressure measured at least once a year (that is, at the same interval as healthy children). […] We suggest that children with ADPKD receive antihypertensive treatment if their blood pressure repeatedly exceeds the ninetieth percentile or is 130/85mmHg if age 16 years. […] We recommend using angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers as first-line antihypertensive treatment in children with ADPKD who have hypertension and albuminuria. […] We recommend that children with ADPKD should aim to achieve the recommended intake for healthy children, which may require extra assistance (for example, advice from a dietician). […] Families should be encouraged to openly discuss their disease and future genetic risks with their children, for example, by provision of age-appropriate information and by providing support for family members in managing their own and their children’s emotions.

• #47 Polycystic Kidney Disease | UCSF Department of Surgery

  https://hpbsurgery.ucsf.edu/condition/polycystic-kidney-disease

  Autosomal dominant PKD is usually diagnosed by kidney imaging studies. […] In most cases of autosomal dominant PKD, patients have no symptoms and their physical condition appears normal for many years, so the disease can go unnoticed. […] Once cysts have grown to about one-half inch, however, diagnosis is possible with imaging technology. […] Although a cure for autosomal dominant PKD is not available, treatment can ease symptoms and prolong life. […] Pain in the area of the kidneys can be caused by cyst infection, bleeding into cysts, kidney stone, or stretching of the fibrous tissue around the kidney with cyst growth. […] People with autosomal dominant PKD tend to have frequent urinary tract infections, which can be treated with antibiotics. […] Keeping blood pressure under control can slow the effects of autosomal dominant PKD.

• #48 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Psychological assessment and depression screening should be considered for patients with chronic pain despite appropriate medical management. […] Although symptomatic lower urinary tract infections are much more common in those with ADPKD than in the general population, the pattern of infections (more common in women) and the infectious organisms (gram-negative bacteria) are similar to those in the general population. […] Nephrolithiasis is twice as common in those with ADPKD than in the general population. […] Gross hematuria occurs in more than 40% of persons with ADPKD and is a risk factor for the rapid progression of renal dysfunction. […] Because of the proliferative effect of estrogen on hepatic cysts, oral contraceptives containing estrogen and menopausal estrogen therapy should be administered at the lowest effective dose or avoided in patients with ADPKD.

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

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

  If blood pressure and blood chemistry are normal then annual blood tests and ultrasound are sufficient to monitor the disease. […] Current advice is that blood pressure should be controlled to a target of 130/80 mm Hg. […] Treat UTIs as they occur, as for the general population. […] Patients should become familiar with self-management for straightforward repeated episodes. […] As end-stage kidney disease is reached, dialysis is required. […] Renal transplantation has similar outcomes for patients with ADPKD as for those without the disease.

• #50 Autosomal Dominant Polycystic Kidney Disease | Consultant360

  https://www.consultant360.com/articles/foresee-your-next-patient-autosomal-dominant-polycystic-kidney-disease

  Autosomal Dominant Polycystic Kidney Disease is a commonly encountered late-onset multisystem illness with a variety of manifestations and severity of symptoms. […] The mainstay of treatment for all patients with ADPKD consists of blood pressure control with renin-angiotensin-aldosterone system blockers (eg, angiotensin-converting enzyme inhibitors, angiotensin II receptor blockers, direct renin inhibitors). […] Supportive measures such as decreased dietary sodium intake, maintenance of hydration, and management of chronic abdominal pain, as well as treatment of nephrolithiasis and cyst infection with trimethoprim-sulfamethoxazole or fluoroquinolones, are also an essential part of management. […] Ultimately, individuals who progress to end-stage kidney disease require renal replacement therapy or renal transplant.

• #51 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Psychological assessment and depression screening should be considered for patients with chronic pain despite appropriate medical management. […] Although symptomatic lower urinary tract infections are much more common in those with ADPKD than in the general population, the pattern of infections (more common in women) and the infectious organisms (gram-negative bacteria) are similar to those in the general population. […] Nephrolithiasis is twice as common in those with ADPKD than in the general population. […] Gross hematuria occurs in more than 40% of persons with ADPKD and is a risk factor for the rapid progression of renal dysfunction. […] Because of the proliferative effect of estrogen on hepatic cysts, oral contraceptives containing estrogen and menopausal estrogen therapy should be administered at the lowest effective dose or avoided in patients with ADPKD.

• #52

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We encourage patients to check BP at home, with a goal BP of 110/75 for younger high-risk patients and 120/80 for older patients with kidney function decline. […] For patients with preserved kidney function, we encourage a low salt diet, high in potassium, with 3 L of fluid intake, and maintenance of an ideal body weight. […] We discuss the use of hormonal therapy with our female patients, with a recommendation for avoidance for those at higher risk of PLD progression and individualized counseling for those at lower risk. […] Somatostatin analogs administered by intramuscular injection (octreotide, lanreotide) are effective in decreasing liver growth and may be considered for symptomatic patients, for whom surgical intervention is not appropriate. […] Liver transplantation is warranted in patients with extremely debilitating symptoms, seriously compromised functional status and reduced quality of life, untreatable complications, such as portal hypertension, and severe malnutrition with bilobar extensive cystic liver disease lacking adequate areas of parenchymal sparing. […] Kidney stone management in patients with ADPKD generally follows the same guidance as management in patients without ADPKD. […] Tolvaptan may confer protective effects against stone formation.

• #53 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Psychological assessment and depression screening should be considered for patients with chronic pain despite appropriate medical management. […] Although symptomatic lower urinary tract infections are much more common in those with ADPKD than in the general population, the pattern of infections (more common in women) and the infectious organisms (gram-negative bacteria) are similar to those in the general population. […] Nephrolithiasis is twice as common in those with ADPKD than in the general population. […] Gross hematuria occurs in more than 40% of persons with ADPKD and is a risk factor for the rapid progression of renal dysfunction. […] Because of the proliferative effect of estrogen on hepatic cysts, oral contraceptives containing estrogen and menopausal estrogen therapy should be administered at the lowest effective dose or avoided in patients with ADPKD.

• #54 Polycystic Kidney Disease (PKD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5791-polycystic-kidney-disease

  Theres no cure for PKD. The goal of treatment is to slow the progression of the disease and control the symptoms it causes. The most common treatments for PKD include: Blood pressure management, Breathing support, Dialysis, Growth therapy, Kidney transplant, Pain management. […] Polycystic kidney disease isn’t preventable. But you may be able to slow the disease or prevent kidney failure by practicing a healthy lifestyle. People with ADPKD can live long and full lives if they receive the proper treatment and manage the condition. About half of people with ADKPD will need dialysis or a kidney transplant due to kidney failure by age 70. […] Living a healthy lifestyle can go a long way in managing PKD. Some tips for healthy living include: Eat a kidney-friendly diet, Exercise for 30 minutes most days, Maintain a weight that’s healthy for you, Manage blood pressure, Quit smoking or using tobacco, Avoid beverages containing alcohol, Reduce stress, Drink enough water and other noncaffeinated beverages, Get at least seven hours of sleep each night, Take all of your medication exactly as your provider prescribes. […] Working with healthcare providers who have experience treating polycystic kidney disease is important.

• #55 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Intracranial aneurysm represents the most severe extra-renal manifestation in those with ADPKD. […] Fertility rates for both men and women with ADPKD who have not developed significant renal dysfunction are comparable to those of the general population. […] All patients with ADPKD develop a progressive loss of renal function, with nearly 80% of patients dying or developing end-stage renal disease by 70 years of age. […] Patients with a family history of ADPKD are candidates for screening, typically with imaging studies. […] Although computed tomography and magnetic resonance imaging are slightly more sensitive for detecting renal cysts, ultrasonography is the preferred screening modality because of lower cost, increased availability, and lack of radiation exposure.

• #56 Autosomal Dominant Polycystic Kidney Disease – Cystic Urologic Disorders for Medicine

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/cystic-urologic-disorders-39445/autosomal-dominant-polycystic-kidney-disease_50337

  Patients with ADPKD are at an increased risk of developing intracranial berry (or saccular) aneurysms, which have been found to be present in as many as 5-10% of patients with ADPKD. There is a strong familial pattern to this phenomenon, and patients should be screened with MRI, as failure to recognize or manage an aneurysm could result in stroke or death.

• #57

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We encourage patients to check BP at home, with a goal BP of 110/75 for younger high-risk patients and 120/80 for older patients with kidney function decline. […] For patients with preserved kidney function, we encourage a low salt diet, high in potassium, with 3 L of fluid intake, and maintenance of an ideal body weight. […] We discuss the use of hormonal therapy with our female patients, with a recommendation for avoidance for those at higher risk of PLD progression and individualized counseling for those at lower risk. […] Somatostatin analogs administered by intramuscular injection (octreotide, lanreotide) are effective in decreasing liver growth and may be considered for symptomatic patients, for whom surgical intervention is not appropriate. […] Liver transplantation is warranted in patients with extremely debilitating symptoms, seriously compromised functional status and reduced quality of life, untreatable complications, such as portal hypertension, and severe malnutrition with bilobar extensive cystic liver disease lacking adequate areas of parenchymal sparing. […] Kidney stone management in patients with ADPKD generally follows the same guidance as management in patients without ADPKD. […] Tolvaptan may confer protective effects against stone formation.

• #58 Autosomal Dominant Polycystic Kidney Disease – NIDDK

  https://www.niddk.nih.gov/health-information/kidney-disease/polycystic-kidney-disease/autosomal-dominant-pkd

  Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of PKD. […] Health care providers usually diagnose ADPKD between the ages of 30 and 50, when signs and symptoms start to appear, which is why it is sometimes called adult PKD. […] Most people with ADPKD have pain, high blood pressure, and kidney failure at some point in their lives. […] High blood pressure increases your chances of heart disease and stroke. High blood pressure can also damage your kidneys even more. Keep your blood pressure under control to help delay kidney damage. […] More than half of people with ADPKD progress to kidney failure by age 70. […] Although a cure doesn’t exist yet for ADPKD, treatment can help reduce your complications, which can help you live longer. […] A health care provider needs to find the source of your pain before he or she can treat it.

• #59 Autosomal Dominant Polycystic Kidney Disease – NIDDK

  https://www.niddk.nih.gov/health-information/kidney-disease/polycystic-kidney-disease/autosomal-dominant-pkd

  Always talk with a health care provider before taking any OTC medicines because some may be harmful to your kidneys. […] Controlling your blood pressure can slow the effects of ADPKD. […] ADPKD can eventually cause your kidneys to fail. People with kidney failure must have dialysis or a kidney transplant to replace their kidney function. […] Managing PKD successfully will probably include several lifestyle changes, such as changes in your physical activity level and what you eat. […] Visiting with a health care team on a regular basis is an important part of your routine as you work to limit your kidney problems.

• #60

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

  Antidepressants or anticonvulsants, which are normally used to treat epilepsy, may be prescribed for long-term (chronic) pain. These medications can also be used to relieve some types of pain. […] You’ll have blood tests at varying intervals to monitor your kidney function. […] You should discuss with your doctors what treatment you’d like if your condition reaches a stage where your kidneys stop working altogether (kidney failure). […] The 2 main treatment options for kidney failure are: dialysis, where a machine replicates some of your kidneys’ functions; a kidney transplant, where a healthy kidney is removed from a living or recently deceased donor and implanted into someone with kidney failure. […] Tolvaptan is a medication that’s recommended by the National Institute for Health and Care Excellence (NICE) to treat ADPKD in adults. […] It can be used to slow down the growth of cysts, reducing overall kidney growth and preserving kidney function for longer. […] If you have ADPKD, your kidneys will be more vulnerable to injury. […] Avoiding all types of contact sport, such as rugby and football, is usually recommended.

• #61 ADPKD basics

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

  If your kidneys fail, you’ll need treatment to take over some of their work. This is known as kidney replacement therapy. The best option is usually a kidney transplant, where you receive a donated kidney from a living or deceased donor. If this isn’t possible, or if you would prefer, you could have dialysis. […] Most adults with early ADPKD experience symptoms occasionally, such as back pain and feeling tired. These are often associated with a particular problem (such as an infection) and clear up with treatment. […] As your kidney health falls, you may have some restrictions on your diet and other aspects of your life (for example, being advised to avoid rougher sports). Not everybody with ADPKD will experience kidney failure. If your kidneys do fail and you start dialysis, this is likely to impact on your day-to-day life due to the time (and possibly travel) needed for dialysis sessions.

• #62 Autosomal dominant polycystic kidney disease (ADPKD): Treatment – UpToDate

  https://www.uptodate.com/contents/autosomal-dominant-polycystic-kidney-disease-adpkd-treatment

  ADDITIONAL MANAGEMENT FOR HIGH-RISK PATIENTS — Patients at high risk for progression may be identified based upon criteria described above. The major clinical challenge is to define the patients at risk for rapid progression who are the most likely to derive benefit from tolvaptan. Tolvaptan is a vasopressin V2-receptor (V2R) antagonist with proven beneficial results in ADPKD. Candidates for treatment — Unless contraindications are present or cost is prohibitive, we prescribe tolvaptan to adult patients with ADPKD who have an estimated glomerular filtration rate (eGFR) ≥25 mL/min1.73 m² and who are at risk of rapid progression. […] SPECIFIC MANAGEMENT OF END-STAGE KIDNEY DISEASE — Patients with ADPKD who progress to end-stage kidney disease (ESKD) require kidney replacement therapy. Peritoneal dialysis is less commonly performed than hemodialysis because it is perceived to be difficult for the patients to accommodate large volumes of peritoneal dialysate fluid in the setting of massively enlarged kidneys. Patients with ADPKD and ESKD are therefore most commonly treated with hemodialysis or undergo kidney transplantation.

• #63 Polycystic Kidney Disease, Autosomal Dominant | UCSF Health

  https://www.ucsfhealth.org/conditions/polycystic-kidney-disease-autosomal-dominant

  If you have PKD, you’re at increased risk of high blood pressure and it’s important to keep your pressure within the normal range to avoid damaging your kidneys. You may be able to control your blood pressure with diet, exercise and other lifestyle modifications. If not, medications, including angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs), can help. […] When PKD progresses to kidney failure, waste products and fluid begin to build up in the blood. Initial symptoms may include nausea, loss of appetite, fatigue, and vomiting. At this point, either dialysis or a transplant is necessary. […] A transplant often offers a better prognosis, but a shortage of organs means that even patients who desire a transplant are likely to rely on dialysis first. […] For transplants, kidneys from living donors usually work best. A donor need not be a relative. Younger people with few other health problems are the best candidates for transplant. Your doctor can help you choose the best course of action for you.

• #64 Autosomal Dominant Polycystic Kidney Disease (ADPKD) – Genitourinary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/genitourinary-disorders/cystic-kidney-disease/autosomal-dominant-polycystic-kidney-disease-adpkd

  Urinary tract infections (UTIs) should be treated promptly. […] Nephrectomy is an option to relieve severe symptoms due to massive kidney enlargement (eg, pain, hematuria) or recurrent UTIs. […] Hemodialysis, peritoneal dialysis, or kidney transplantation is required in patients who develop chronic renal failure. ADPKD does not recur in grafts. […] Supportive measures include increased fluid intake (particularly water) to suppress vasopressin release, even if only partially, in patients who are able to safely excrete the load. […] Tolvaptan appears to slow increase in renal volume and decline in renal function, but it can cause adverse effects due to free water diuresis (eg, thirst, polydipsia, polyuria) that can make adherence difficult. […] Genetic counseling is recommended for first-degree relatives of patients with ADPKD.

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

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

  If blood pressure and blood chemistry are normal then annual blood tests and ultrasound are sufficient to monitor the disease. […] Current advice is that blood pressure should be controlled to a target of 130/80 mm Hg. […] Treat UTIs as they occur, as for the general population. […] Patients should become familiar with self-management for straightforward repeated episodes. […] As end-stage kidney disease is reached, dialysis is required. […] Renal transplantation has similar outcomes for patients with ADPKD as for those without the disease.

• #66 Autosomal dominant polycystic kidney disease (ADPKD): Treatment – UpToDate

  https://www.uptodate.com/contents/autosomal-dominant-polycystic-kidney-disease-adpkd-treatment

  NEPHRECTOMY IN SELECTED CASES — Nephrectomy is avoided whenever possible in ADPKD because of potential complications and the adverse effects of removing a partially functioning kidney. However, it may be a reasonable therapeutic option in certain circumstances: Debilitating abdominal discomfort and/or marked limitation of daily activities due to massively enlarged kidneys, or chronic kidney pain refractory to treatment. […] SUMMARY AND RECOMMENDATIONS — Course of autosomal dominant polycystic kidney disease — Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of chronic kidney disease (CKD). The majority of individuals with ADPKD eventually require kidney replacement therapy. Identification of high-risk patients — The identification of individuals who are at high risk for progression of CKD is important for prognostic reasons and to identify patients who may benefit from specific therapies, such as tolvaptan. Blood pressure management — In hypertensive patients with ADPKD, we suggest using an angiotensin-converting enzyme (ACE) inhibitor or an angiotensin receptor blocker (ARB) rather than another antihypertensive drug. Dietary recommendations — We advise all ADPKD patients to restrict dietary sodium, with a goal of 2 grams intake per day or less. Treatment with tolvaptan — In adult patients with ADPKD who have an eGFR ≥25 mL/min1.73m² and who are at high risk of CKD progression, we suggest treatment with tolvaptan. Nephrectomy — Nephrectomy is generally avoided in ADPKD because of potential complications and the adverse effects of removing a partially functioning kidney. However, nephrectomy may be an option in patients with disabling symptoms due to massively enlarged kidneys.

• #67

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  Patients with autosomal dominant polycystic kidney disease benefit from specialized care over their lifetimes, starting with diagnosis of the condition with ongoing discussion of both the renal course and extra-renal issues. […] Both renal and extra-renal issues may continue to cause major morbidity even after successful kidney transplant or initiation of RRT, and extra-renal disease aspects should always be considered as part of routine management. […] In this review, we will focus on updates in pain/depression screening, cardiac manifestations, liver and pancreatic cysts, kidney stone management, and genetic counseling. […] ADPKD significantly affects health-related quality of life. […] Pain is a primary driver of the mental and physical burden of the disease, contributing substantially to reductions in health-related quality of life.

• #68

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  Pain related to increasing kidney size and cyst complications is reported in 50% of patients with ADPKD, with 20% reporting pain often, usually, or always. […] Pain remains an issue as disease progresses with 44% of transplant and 59% of dialysis patients reporting pain. […] Patients desire support from their providers, open and honest conversations regarding pain management, and information and resources to learn more about their condition. […] Despite patients reporting pain management as a top priority in ADPKD outcomes, many feel that their pain is underestimated by their physician and wish for more discussion surrounding pain management and strategies. […] The ADPKD Pain and Discomfort Scale was developed and validated to specifically evaluate the severity and type of pain experienced by patients with ADPKD.

• #69

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  Pain related to increasing kidney size and cyst complications is reported in 50% of patients with ADPKD, with 20% reporting pain often, usually, or always. […] Pain remains an issue as disease progresses with 44% of transplant and 59% of dialysis patients reporting pain. […] Patients desire support from their providers, open and honest conversations regarding pain management, and information and resources to learn more about their condition. […] Despite patients reporting pain management as a top priority in ADPKD outcomes, many feel that their pain is underestimated by their physician and wish for more discussion surrounding pain management and strategies. […] The ADPKD Pain and Discomfort Scale was developed and validated to specifically evaluate the severity and type of pain experienced by patients with ADPKD.

• #70 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Psychological assessment and depression screening should be considered for patients with chronic pain despite appropriate medical management. […] Although symptomatic lower urinary tract infections are much more common in those with ADPKD than in the general population, the pattern of infections (more common in women) and the infectious organisms (gram-negative bacteria) are similar to those in the general population. […] Nephrolithiasis is twice as common in those with ADPKD than in the general population. […] Gross hematuria occurs in more than 40% of persons with ADPKD and is a risk factor for the rapid progression of renal dysfunction. […] Because of the proliferative effect of estrogen on hepatic cysts, oral contraceptives containing estrogen and menopausal estrogen therapy should be administered at the lowest effective dose or avoided in patients with ADPKD.

• #71

  https://journals.lww.com/kidney360/fulltext/2023/12000/beyond_loss_of_kidney_function__patient_care_in.22.aspx

  We propose asking about each pain type (sharp, dull, or fullness or discomfort) at each clinical encounter, with further exploration of the domains of severity or interference with a positive response as a way of engaging in a discussion of individual patient concerns. […] Between 22% and 60% of patients with ADPKD have depression, and therefore, we also recommend screening. […] Given the negative effects of depression on mental and physical health of patients with ADPKD, it is important to screen for depression early on in ADPKD disease course and at regular intervals throughout management. […] The US Preventative Services Taskforce recommends that all adults should be screened for depression using evidence-based protocols, such as various iterations of the Patient Health Questionnaire.

• #72 ‘A sword of Damocles’: patient and caregiver beliefs, attitudes and perspectives on presymptomatic testing for autosomal dominant polycystic kidney disease: a focus group study | BMJ Open

  https://bmjopen.bmj.com/content/10/10/e038005

  For patients with ADPKD, presymptomatic testing provides an opportunity to take ownership of their health through family planning and preventive measures. However, these decisions can be wrought with tensions and uncertainty about prognostic implications, and the psychosocial and financial burden of testing. Healthcare professionals should focus on genetic counselling, mental health and providing education to patients families to support informed decision-making. Policymakers should consider the cost burden and risk of discrimination when informing government policies. Finally, patients are recommended to focus on self-care from an early age. […] Clinical management involves pharmacological and lifestyle interventions to control hypertension, slow the progression of cysts, manage complications (kidney and extra-kidney manifestations) and maintain quality of life (QoL).

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

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

  This is the most common inherited cause of serious renal disease. It is an autosomal dominant condition and is thought to be of high or complete penetrance. […] Diagnosis of ADPKD has implications both for the patient and for their family. […] Consider the information needs of the patient and their family members. Provide written information and sources of support relating to the disease and be prepared to discuss the nature of the disease, possible complications and likely prognosis as well as the genetic implications for other family members. […] Care and monitoring would usually be undertaken jointly by primary care and renal teams. […] The TEMPO (Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and Its Outcomes) 3:4 trial, showed that the vasopressin receptor antagonist tolvaptan slowed the growth of cystic kidneys and the deterioration of renal function.

• #74 Autosomal dominant polycystic kidney disease — Knowledge Hub

  https://www.genomicseducation.hee.nhs.uk/genotes/knowledge-hub/autosomal-dominant-polycystic-kidney-disease/

  Autosomal dominant polycystic kidney disease is a genetic disorder characterised by the growth of numerous cysts in the kidneys, leading to renal failure and other systemic complications. […] ADPKD is generally a late-onset multisystem condition. Genomic counselling/testing should therefore be delayed until an appropriate age (at least the age of 16). There are, however, important management considerations for children and young people see Management below. […] Risk stratification to predict the age at which the patient might reach kidney failure, with the help of existing prognostic tools, is the first step in management. If the risk of developing kidney failure is high (rapidly progressive disease), treatment with a vasopressin V2-receptor antagonist (tolvaptan), to slow cyst growth, should be discussed.

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

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

  Couples on reproductive age who wish to have offspring must know the risk of having children in a natural way: autosomal dominant inheritance conditions a 50% probability of conceiving a healthy or affectionate child, and this rule of chance applies on each occasion. […] 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.

• #76 Autosomal Dominant Polycystic Kidney Disease – NIDDK

  https://www.niddk.nih.gov/health-information/kidney-disease/polycystic-kidney-disease/autosomal-dominant-pkd

  Always talk with a health care provider before taking any OTC medicines because some may be harmful to your kidneys. […] Controlling your blood pressure can slow the effects of ADPKD. […] ADPKD can eventually cause your kidneys to fail. People with kidney failure must have dialysis or a kidney transplant to replace their kidney function. […] Managing PKD successfully will probably include several lifestyle changes, such as changes in your physical activity level and what you eat. […] Visiting with a health care team on a regular basis is an important part of your routine as you work to limit your kidney problems.

• #77 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. […] The doctor taking care of these patients, in addition to solving the specific questions of each individual, must address how to reduce the health impact of the disease; this should be done by providing information to individual and family, recommending to study those members of affected families not yet evaluated and persuading those who left our consultations to come back to the clinics. […] Patient registration and pedigree trees are necessary and the analysis of this information will allow us to: a) know the general magnitude of the disease within a given healthcare setting and in each family, b) its geographical distribution, c) the evolution of incidence and prevalence, d) identify and locate non-studied cases, which in our province, Granada, with a population of 914,678 inhabitants, should range between 198240 people.

• #78 Autosomal Dominant Polycystic Kidney Disease: Early intervention and lifestyle crucial – Mayo Clinic News Network

  https://newsnetwork.mayoclinic.org/discussion/autosomal-dominant-polycystic-kidney-disease-early-intervention-and-lifestyle-crucial/

  „This drug improves care and paves the way for future therapies that enhance the quality of life and delay kidney failure,” explains Neera Dahl, M.D., Ph.D., Mayo Clinic nephrologist and senior author of the review. […] The review recommends that a kidney specialist who manages patients with ADPKD should share decision-making with the patient regarding genetic testing, treatments, monitoring and aneurysm screening. This collaborative approach recognizes the importance of the physician’s medical expertise and the patient’s values, preferences, and goals. It also says patients with ADPKD should be aware of the symptoms of ruptured aneurysms (sudden, severe headache) and the need for immediate medical attention should they experience those symptoms.

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

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

  Couples on reproductive age who wish to have offspring must know the risk of having children in a natural way: autosomal dominant inheritance conditions a 50% probability of conceiving a healthy or affectionate child, and this rule of chance applies on each occasion. […] 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.

• #80 Polycystic Kidney Disease | The University of Kansas Health System

  https://www.kansashealthsystem.com/care/conditions/polycystic-kidney-disease

  At The University of Kansas Health System, we provide: A multidisciplinary approach, including shared care with referring physicians. A nationally recognized, complete continuum of kidney care, including dialysis and transplant, as well as use of tolvaptan, the first and only FDA-approved medication to treat PKD, slowing the disease’s progression. […] Our multidisciplinary dialysis team includes physicians, registered nurses, dietitians and social workers to provide education and support, and to help you manage your condition and maximize your quality of life.

• #81 Pediatric Polycystic Kidney Disease Treatment & Management: Approach Considerations, Long-Term Monitoring

  https://emedicine.medscape.com/article/983281-treatment

  In addition to the significant medical problems, the psychosocial stress on the patient and family can be overwhelming. A team approach in which the skills of the nephrologist are used together with those of other medical specialists (eg, gastroenterologist), specialized nurses, nutritionists, social workers, psychiatrists, and other support staff provides optimal comprehensive care.

• #82 Pediatric Polycystic Kidney Disease Treatment & Management: Approach Considerations, Long-Term Monitoring

  https://emedicine.medscape.com/article/983281-treatment

  In addition to the significant medical problems, the psychosocial stress on the patient and family can be overwhelming. A team approach in which the skills of the nephrologist are used together with those of other medical specialists (eg, gastroenterologist), specialized nurses, nutritionists, social workers, psychiatrists, and other support staff provides optimal comprehensive care.

• #83 Polycystic Kidney Disease (PKD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5791-polycystic-kidney-disease

  Theres no cure for PKD. The goal of treatment is to slow the progression of the disease and control the symptoms it causes. The most common treatments for PKD include: Blood pressure management, Breathing support, Dialysis, Growth therapy, Kidney transplant, Pain management. […] Polycystic kidney disease isn’t preventable. But you may be able to slow the disease or prevent kidney failure by practicing a healthy lifestyle. People with ADPKD can live long and full lives if they receive the proper treatment and manage the condition. About half of people with ADKPD will need dialysis or a kidney transplant due to kidney failure by age 70. […] Living a healthy lifestyle can go a long way in managing PKD. Some tips for healthy living include: Eat a kidney-friendly diet, Exercise for 30 minutes most days, Maintain a weight that’s healthy for you, Manage blood pressure, Quit smoking or using tobacco, Avoid beverages containing alcohol, Reduce stress, Drink enough water and other noncaffeinated beverages, Get at least seven hours of sleep each night, Take all of your medication exactly as your provider prescribes. […] Working with healthcare providers who have experience treating polycystic kidney disease is important.

• #84 Treatment and Management of Autosomal Dominant Polycystic Kidney Disease – Polycystic Kidney Disease – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK373382/

  Hypertension is a common and serious complication of ADPKD, often occurring in 50% – 70% of ADPKD patients early in the disease, before appearance of renal dysfunction. […] Early and effective treatment of hypertension is very important to decrease the morbidity and mortality of ADPKD patients. […] In patients with renal disease, the goal is a blood pressure of less than 130/80 mm Hg. In patients with ADPKD we have to adopt this blood pressure target. Hypertension is the most important modifiable risk factor in ADPKD and better blood pressure control allows slowing down the progression of kidney disease. […] Current published data confirm that patients with ADPKD in the United States, Denmark and Great Britain are having a better prognosis. […] Therefore, early control of hypertension is very important in patients with ADPKD to slow down kidney disease progression and prevent occurrence of cardiovascular events.

• #85 Autosomal Dominant Polycystic Kidney Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2014/0901/p303.html

  Intracranial aneurysm represents the most severe extra-renal manifestation in those with ADPKD. […] Fertility rates for both men and women with ADPKD who have not developed significant renal dysfunction are comparable to those of the general population. […] All patients with ADPKD develop a progressive loss of renal function, with nearly 80% of patients dying or developing end-stage renal disease by 70 years of age. […] Patients with a family history of ADPKD are candidates for screening, typically with imaging studies. […] Although computed tomography and magnetic resonance imaging are slightly more sensitive for detecting renal cysts, ultrasonography is the preferred screening modality because of lower cost, increased availability, and lack of radiation exposure.

• #86 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: […] People with ADPKD will usually be seen once a year by a kidney specialist to monitor their general health and kidney function. More frequent monitoring will be needed if blood tests show signs of kidney damage. […] Half of people with ADPKD will develop kidney failure by the age of 60 and will need dialysis or a kidney transplant. However, with the right treatments most people with ADPKD remain in good health for decades.

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

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

  Medicine to prevent kidney failure. Tolvaptan (Jynarque) can slow the decline of kidney function for adults whose disease is at risk of quickly getting worse. […] 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: […] Eat right. Stick to a healthy, well-balanced diet that’s low in calories. Try to limit salt, because it can raise your blood pressure. […] Stay active. Exercise can help control your weight and blood pressure. Just avoid any contact sports where you might injure your kidneys. […] Don’t smoke. If you smoke, get help from your doctor to quit. Smoking damages the blood vessels in the kidneys, and it may create more cysts. […] Drink plenty of water. Dehydration may cause you to have more cysts. […] Cysts often grow very slowly. They can grow even slower when you control your blood pressure and make healthy lifestyle choices. But after many years, they can get large enough to damage your kidneys. As time goes on, some people have kidney failure and need dialysis or a kidney transplant.

• #88

  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.

• #89 Polycystic Kidney Disease Treatment | Autosomal Dominant Polycystic Kidney Disease

  https://resources.healthgrades.com/right-care/kidney-disease/treating-autosomal-dominant-polycystic-kidney-disease

  Currently, other drugs are also being studied to determine if they may be effective in treating autosomal dominant polycystic kidney disease. A wide variety are under investigation, including medications used to treat type 2 diabetes, vitamins, and dietary supplements. The more researchers learn about what causes cysts to develop and how the disease progresses, the better they can try to stop it. If you’re interested in joining a clinical trial for one of these new medications, talk to your doctor about your options.

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

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

  Currently there is no cure for PKD. However, early detection and treatment can reduce or prevent some of the complications of PKD. […] Self-care suggestions for PKD generally include: changing your diet, making healthier lifestyle choices, avoiding non-steroidal anti-inflammatory drugs (NSAIDs), considering avoiding contact sports.

• #91 Polycystic Kidney Disease Treatment | Autosomal Dominant Polycystic Kidney Disease

  https://resources.healthgrades.com/right-care/kidney-disease/treating-autosomal-dominant-polycystic-kidney-disease

  Currently, other drugs are also being studied to determine if they may be effective in treating autosomal dominant polycystic kidney disease. A wide variety are under investigation, including medications used to treat type 2 diabetes, vitamins, and dietary supplements. The more researchers learn about what causes cysts to develop and how the disease progresses, the better they can try to stop it. If you’re interested in joining a clinical trial for one of these new medications, talk to your doctor about your options.

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