# Polycystic kidney disease - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - Sources: Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK532934/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class UD55.00 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care - Verified date: 2026-08 ## Verified against - No dose - referral pathway, no medicine given in primary care - Polycystic kidney disease - disease-level clinical article (polycystic-kidney-disease-clinical.txt) ## Treatment metadata - No drug therapy in primary care (Referral & Advice) ## Complete treatment card ```text POLYCYSTIC KIDNEY DISEASE Sources: Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK532934/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class UD55.00 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care Review status: REVIEWED against No dose - referral pathway, no medicine given in primary care, Polycystic kidney disease - disease-level clinical article (polycystic-kidney-disease-clinical.txt) (2026-08) IS IT THIS? - reference only, to read alongside your own examination SYMPTOMS - what the patient reports (7) - Once GFR begins to fall, the decline is typically rapid, averaging 4.0 to 5.0 mL/minute per year. - High blood pressure is the earliest and most common presenting feature. [hypertension] - Visible blood in the urine can be the first symptom, from a bleeding cyst that communicates with the collecting system. [bleeding · blood in the urine · cyst] - Cyst hemorrhage causing gross hematuria is a frequent complication when the cyst opens into the collecting system. [bleeding · blood in the urine · cyst] - Kidney stones occur in about 20 to 25 percent of patients, most often uric acid or calcium oxalate. [kidney stones] - Polycystic liver disease is suspected once four or more cysts are seen in the liver parenchyma. [cyst] - Affected children usually have normal kidney function but higher rates of hypertension and protein in the urine than unaffected children. [hypertension · proteinuria] SIGNS - what you find (3) - The most frequently seen heart abnormalities are mitral valve prolapse together with aortic regurgitation. - As many as half of patients also have diverticulosis. [diverticulosis] - Kidneys may be palpable on abdominal exam, particularly with a strong family history of the disease. TESTS (6) - The diagnosis is suspected with renal impairment plus multiple bilateral cysts on ultrasound or CT, with or without a known family history. - Diagnostic ultrasound thresholds rise with age: 2 or more cysts (either kidney) at 15-29 years, 2 or more per kidney at 30-59, and 4 or more per kidney at 60 and older. - Three or more cysts total between ages 15 and 39 has a 100% positive predictive value for the diagnosis. - Two or fewer cysts after age 40 has a 100% negative predictive value against the diagnosis. - Genetic testing for PKD1 and PKD2 is about 99% accurate but is not routinely needed for diagnosis. - If a child's initial screening ultrasound is negative, repeat screening is deferred until adolescence, around age 15 to 18. IF NOT THIS - what else fits (6) - Bardet-Biedl syndrome combines renal cysts with vision problems and obesity, so cysts alone do not define it. - HNF1B mutation causes renal cysts alongside early-onset diabetes, early-onset gout, a small pancreas, abnormal liver enzymes, and genital tract defects. - Unlike ADPKD, medullary sponge kidney doesn't lead to renal failure, though it's closely tied to nephrocalcinosis and kidney stones. - Tuberous sclerosis also causes renal cysts but comes with characteristic skin lesions like facial angiofibromas and connective tissue nevi. - Autosomal recessive PKD, unlike the dominant form, begins at or shortly after birth rather than in adulthood. - Orofaciodigital syndrome type I features facial, oral, and digit abnormalities, with renal cysts as a secondary finding. Source StatPearls "Autosomal Dominant Polycystic Kidney Disease" - disease-level clinical article Status traced to the source above 1. NO DRUG THERAPY IN PRIMARY CARE (REFERRAL & ADVICE) [1st line] Adult An inherited disorder causing progressive kidney cysts and eventual kidney failure; the GP recognises the diagnosis, often via family history or imaging, and refers to nephrology for monitoring. - Refer, with advice Peds Children follow the same pathway: recognise and refer. No primary-care medicine is implied. Source No dose - referral pathway, no medicine given in primary care Why An inherited disorder causing progressive kidney cysts and eventual kidney failure; the GP recognises the diagnosis, often via family history or imaging, and refers to nephrology for monitoring. Caution RED FLAG - Infected renal cyst or acute pyelonephritis requiring urgent admission and targeted parenteral antibiotics: assess urgently and refer. RED FLAG - Ruptured intracranial berry aneurysm presenting as sudden thunderclap headache: assess urgently and refer. High blood pressure, blood in the urine, or declining kidney function in a known polycystic kidney disease patient needs prompt nephrology involvement. No medicine is prescribed for this in primary care - this entry is for recognition and referral. Anything given is decided by the service it is referred to. RED FLAG - Cerebral aneurysm is the most serious extrarenal complication of ADPKD (4x higher prevalence than general population). RED FLAG - Severe cyst hemorrhage causing hemodynamic instability requires emergency hospitalization and transfusion. Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)