# 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/ · Egyptian National Drug Formulary - Cardiovascular Chapter 2024 (enalapril monograph) · Jinarc 45 mg + Jinarc 15 mg tablets SmPC section 4.2 Posology (eMC product 6847) · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class UD55.00 - condition scope only, no dose - Verified date: 2026-09 ## Verified against - Egyptian National Drug Formulary - Cardiovascular Chapter 2024 (enalapril monograph) - Jinarc 45 mg + Jinarc 15 mg tablets SmPC section 4.2 Posology (eMC product 6847) - Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf (NBK532934) - https://www.ncbi.nlm.nih.gov/books/NBK532934/, Treatment / Management ## Treatment metadata - Enalapril — 10 mg — oral.solid - Tolvaptan — 15 mg — oral.solid - Fluid, salt and the complications (Advice & Referral) ## Complete treatment card ```text POLYCYSTIC KIDNEY DISEASE Sources: Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK532934/ · Egyptian National Drug Formulary - Cardiovascular Chapter 2024 (enalapril monograph) · Jinarc 45 mg + Jinarc 15 mg tablets SmPC section 4.2 Posology (eMC product 6847) · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class UD55.00 - condition scope only, no dose Review status: REVIEWED against 3 sources listed above (2026-09) 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 Rx: Blood pressure - the treatment that changes the outcome | Slowing the disease - specialist- initiated | Non-drug management and the complications BLOOD PRESSURE - THE TREATMENT THAT CHANGES THE OUTCOME 1. ENALAPRIL [1st line] Adult 5 to 10 mg once daily initially; titrate after 2 to 4 weeks if needed up to 40 mg/day in 1 or 2 divided doses. If creatinine clearance is 10 to 30 ml/min, start at 2.5 mg once daily with a maximum of 20 mg/day - long-term Peds 0.1-1 mg/kg/day (The formulary gives two separate paediatric rules. "Children under 12 years and over 20 KGs weight: Oral: Initial: 0.1 mg/kg once daily increased to 1mg/kg in 1-2 divided doses according to response with close monitoring." The calculated figures above are that rule, which is why they do not apply below 20 kg. For a child of 12 to 17 years the formulary gives a set amount instead: "Initially 2.5 mg once daily, monitor blood pressure carefully for 1-2 hours, maintenance 10-20 mg daily in 1-2 divided doses." Enalapril is not recommended at or under 16 years of age where GFR is below 30 ml/min/1.73 m2, and the formulary states no dose for that situation.) Not for children under 20 kg 20kg -> 2-20 mg/day 21kg -> 2.1-21 mg/day 22kg -> 2.2-22 mg/day 23kg -> 2.3-23 mg/day 24kg -> 2.4-24 mg/day 25kg -> 2.5-25 mg/day 26kg -> 2.6-26 mg/day 27kg -> 2.7-27 mg/day 28kg -> 2.8-28 mg/day 29kg -> 2.9-29 mg/day 30kg -> 3-30 mg/day 31kg -> 3.1-31 mg/day 32kg -> 3.2-32 mg/day 33kg -> 3.3-33 mg/day 34kg -> 3.4-34 mg/day 35kg -> 3.5-35 mg/day 36kg -> 3.6-36 mg/day 37kg -> 3.7-37 mg/day 38kg -> 3.8-38 mg/day 39kg -> 3.9-39 mg/day 40kg -> 4-40 mg/day 41kg -> 4.1-41 mg/day 42kg -> 4.2-42 mg/day 43kg -> 4.3-43 mg/day 44kg -> 4.4-44 mg/day 45kg -> 4.5-45 mg/day 46kg -> 4.6-46 mg/day 47kg -> 4.7-47 mg/day 48kg -> 4.8-48 mg/day 49kg -> 4.9-49 mg/day 50kg -> 5-50 mg/day Choice Alternatives, in the order the article gives them: "Beta-blockers and calcium-channel blockers are second-line treatments. Thiazides are preferred in patients with normal renal function as a third-line and in patients with hypercalciuria, while loop diuretics are recommended in patients with impaired renal function as an alternative to thiazides." An angiotensin inhibitor leads because the article names it the preferred agent and because it protects the glomerulus, not only the blood pressure. Source Egyptian National Drug Formulary - Cardiovascular Chapter 2024 (enalapril monograph, Hypertension, chronic; and Dosing: Altered Kidney Function). The choice of an angiotensin inhibitor and the blood pressure target come from Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf (NBK532934) - https://www.ncbi.nlm.nih.gov/books/NBK532934/, Treatment / Management Why The article: "Management of hypertension is essential in reducing cardiovascular mortality and slowing the progression of renal failure" and "Angiotensin inhibitors are the preferred agents if there is no contraindication. ACE inhibition also protects the glomeruli by decreasing intraglomerular pressure and reducing the rate of GFR decline in those with proteinuria." Blood pressure control is the treatment that changes the outcome in this disease, and it is the part a GP actually delivers between nephrology visits. Caution Blood pressure target. The article cites the HALT-PKD study for a target of "less than 120 to 125/80 mm Hg, similar to other patients with chronic kidney disease", and adds that where GFR is preserved or nearly preserved, "a lower blood pressure goal of less than 110/75 mm Hg is associated with a decreased incidence of cardiovascular events and a slower rate of cyst growth". This is a tighter target than ordinary hypertension. RED FLAG - Contraindicated in ACE-inhibitor hypersensitivity and in anyone with a history of angioedema. Stop it in pregnancy. Use the lower starting dose in hyponatraemia, hypovolaemia, severe heart failure, reduced kidney function, or in a patient already on a diuretic. Where creatinine clearance is under 10 ml/min the formulary says to consider alternative therapy because of the risk of hyperkalaemia and kidney failure; if it is used, 2.5 mg every other day with a maximum of 10 mg/day. Check potassium and creatinine after starting or increasing the dose - this is a patient whose kidney function is already the thing being protected. Egypt PRES 10MG 30 TAB. GLOBAL NAPI P... 20.25 EGP (0.68/unit) PRESSLIGHT 10 MG 10 TAB. EL-OBOUR 10.00 EGP (1.00/unit) EZAPRIL 10 MG 30 TABS. MULTI-APEX 54.00 EGP (1.80/unit) SLOWING THE DISEASE - SPECIALIST-INITIATED 2. TOLVAPTAN [2nd line] Adult Split dose, twice daily. Start at 60 mg/day as 45 mg on waking and at least 30 minutes before the morning meal, plus 15 mg 8 hours later; titrate upward at intervals of at least one week to 90 mg/day (60 mg + 30 mg) and then to the target 120 mg/day (90 mg + 30 mg) if tolerated - long-term, while the benefit outweighs the monitoring burden Peds The SmPC: "The safety and efficacy of tolvaptan in children and adolescents has not yet been established. No data are available. Tolvaptan is not recommended in the paediatric age group." Source Jinarc 45 mg + Jinarc 15 mg tablets SmPC section 4.2 Posology (eMC product 6847); the indication and the restriction to high-risk patients from Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf (NBK532934) - https://www.ncbi.nlm.nih.gov/books/NBK532934/, Treatment / Management Why Vasopressin V2 receptor antagonist. The article: tolvaptan "is the only FDA-approved medicine for ADPKD at high risk for disease progression; however, due to its high cost and adverse effects, its use is recommended only in patients at high risk of disease progression or who demonstrate rapidly declining kidney function." It works by "blocking the reception of vasopressin signaling at the V2 receptor, lowering the intracellular cyclic AMP that would otherwise stimulate cystic proliferation and growth." It is started by nephrology, not in primary care; it is here so the GP who sees the patient in between knows the regimen and the monitoring it commits them to. Caution RED FLAG - Liver injury. Blood testing for hepatic transaminases and bilirubin is required before starting, then monthly for 18 months, then every 3 months. Stop permanently if ALT or AST exceeds 8 times the upper limit of normal; or exceeds 5 times for more than 2 weeks; or exceeds 3 times together with bilirubin above twice normal or INR above 1.5; or exceeds 3 times with persistent symptoms of liver injury. RED FLAG - Contraindicated in anuria, volume depletion, hypernatraemia, pregnancy, breast-feeding, and in any patient who cannot perceive or respond to thirst. Also contraindicated where liver enzymes or signs of liver injury before starting already meet the criteria for permanent discontinuation. The patient must be instructed to drink sufficient water or other aqueous fluids. The drug makes them pass large volumes of urine, so a patient who cannot get to water, or cannot feel thirst, is the one it harms. Cost. The Egyptian register lists the split-dose packs (DAVOLTAN 15 mg and 45 mg, 30 mg and 60 mg, 30 mg and 90 mg, 56 tablets each) at 12,200 EGP per pack, which is 28 days of treatment. Discuss what this actually costs before the patient is committed to it; the article's own reason for restricting it is the cost. Not for every patient with the disease. The article's threshold is a yearly GFR decline of at least 3.0 ml/min attributed to ADPKD, estimated from at least five measurements over four years, with preference for patients aged 55 or younger who have fewer comorbidities - and it states there is no definite age cut-off. That assessment is nephrology's, not primary care's. Egypt DAVOLTAN 15 MG 10 TABS. HIKMA SPECIAL... 1524.00 EGP (152.40/unit) DAVOLTAN 15 MG & 45 MG 56 TABS. HIKMA SPECIAL... 12200.00 EGP (217.86/unit) NON-DRUG MANAGEMENT AND THE COMPLICATIONS 3. FLUID, SALT AND THE COMPLICATIONS (ADVICE & REFERRAL) [3rd 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. Alongside the blood pressure control above, the article advises three litres of fluid a day to suppress vasopressin and a sodium intake under 2 grams a day - neither is proven to prevent progression, and both are stated as recommended. - Refer, with advice Peds A child with a family history is monitored rather than treated; the enalapril row above carries the paediatric weight-based dose for the blood pressure, and tolvaptan is not recommended in children at all. Source No dose - lifestyle advice and the referral pathway, from Autosomal Dominant Polycystic Kidney Disease - StatPearls - NCBI Bookshelf (NBK532934) - https://www.ncbi.nlm.nih.gov/books/NBK532934/, Treatment / Management 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. Alongside the blood pressure control above, the article advises three litres of fluid a day to suppress vasopressin and a sodium intake under 2 grams a day - neither is proven to prevent progression, and both are stated as recommended. Caution Cyst infection needs immediate treatment to stop retrograde seeding of the kidney. The article names the agents with good cyst penetration - trimethoprim-sulfamethoxazole, ertapenem, chloramphenicol, fluoroquinolones and clindamycin - and states no dose for any of them, so no dose is printed here. If fever persists after 1 to 2 weeks of appropriate treatment, the infected cyst is drained. Stones are much commoner than in the general population and are usually uric acid. The article suggests urinary alkalinisation where possible, with potassium citrate and a higher fluid intake as the treatments of choice; it states no dose. Flank pain is not assumed to be the cysts. Exclude infection, stone, herpes zoster and neoplasm first. Cyst haemorrhage is usually self-limiting and settles with rest, analgesia and more fluid. RED FLAG - Severe bleeding causing haemodynamic instability needs hospital admission, transfusion and supportive care. 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)