{ "schema_version": 1, "kind": "condition", "id": "polycystic-kidney-disease", "name": "Polycystic kidney disease", "category": "chronic", "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", "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", "verified_date": "2026-09", "treatments": [ { "id": 1473, "generic": "Enalapril", "line": 1, "is_adjunct": false, "form": "oral.solid", "strength_mg": 10.0, "adult_dose": "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", "adult_duration": "long-term", "dose_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", "rationale": "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.", "cautions": [ "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." ], "peds_mgkg_low": 0.1, "peds_mgkg_high": 1.0, "peds_max_mg": null, "peds_basis": "day", "peds_unit": "mg", "peds_note": "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.", "peds_min_weight_kg": 20.0, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": null, "peds_doses": [ { "weight_kg": 20, "low_mg": 2, "high_mg": 20, "capped": false }, { "weight_kg": 30, "low_mg": 3, "high_mg": 30, "capped": false }, { "weight_kg": 40, "low_mg": 4, "high_mg": 40, "capped": false } ], "brands": [ { "trade_name": "PRES 10MG 30 TAB.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "GLOBAL NAPI PHARMACEUTICALS", "price_egp": 20.25, "pack_count": 30, "unit_price": 0.68, "strength_mg": 10.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "PRESSLIGHT 10 MG 10 TAB.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "EL-OBOUR", "price_egp": 10.0, "pack_count": 10, "unit_price": 1.0, "strength_mg": 10.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "EZAPRIL 10 MG 30 TABS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "MULTI-APEX", "price_egp": 54.0, "pack_count": 30, "unit_price": 1.8, "strength_mg": 10.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null } ], "condition_id": "polycystic-kidney-disease" }, { "id": 1474, "generic": "Tolvaptan", "line": 2, "is_adjunct": false, "form": "oral.solid", "strength_mg": 15.0, "adult_dose": "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", "adult_duration": "long-term, while the benefit outweighs the monitoring burden", "dose_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", "rationale": "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.", "cautions": [ "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." ], "peds_mgkg_low": null, "peds_mgkg_high": null, "peds_max_mg": null, "peds_basis": null, "peds_unit": null, "peds_note": "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.\"", "peds_min_weight_kg": null, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": null, "peds_doses": null, "brands": [ { "trade_name": "DAVOLTAN 15 MG 10 TABS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "HIKMA SPECIALIZED PHARMACEUTICALS", "price_egp": 1524.0, "pack_count": 10, "unit_price": 152.4, "strength_mg": 15.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "DAVOLTAN 15 MG & 45 MG 56 TABS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "HIKMA SPECIALIZED PHARMACEUTICALS", "price_egp": 12200.0, "pack_count": 56, "unit_price": 217.86, "strength_mg": 15.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null } ], "condition_id": "polycystic-kidney-disease" }, { "id": 1475, "generic": "Fluid, salt and the complications (Advice & Referral)", "line": 3, "is_adjunct": false, "form": null, "strength_mg": null, "adult_dose": "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.", "adult_duration": "Refer, with advice", "dose_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", "rationale": "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.", "cautions": [ "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." ], "peds_mgkg_low": null, "peds_mgkg_high": null, "peds_max_mg": null, "peds_basis": null, "peds_unit": null, "peds_note": "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.", "peds_min_weight_kg": null, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": null, "peds_doses": null, "brands": [], "condition_id": "polycystic-kidney-disease" } ] }