eMedicine Specialties > Nephrology > Cystic Diseases of the Kidney
Polycystic Kidney Disease: Treatment & Medication
Updated: Sep 17, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Ensure that a patient with ADPKD who is nonhypertensive and has normal renal function undergoes blood testing and ultrasonographic scanning once a year.
- Schedule more frequent follow-up studies for patients with high blood pressure. Hypertension is common, occurring in as many as 50-70% of patients before the onset of renal failure.
- Patients with renal failure require more frequent monitoring, based on the severity of their condition.
- Medical therapy is necessary to accomplish the following:
- Control blood pressure. In patients with renal disease, the goal is a blood pressure of less than 130/88 mm Hg. If more than 1 g/d of urinary protein is present, the target blood pressure is less than 125/75 mm Hg. Achieving good blood pressure control helps slow the progression of renal disease.
- The best drugs for this condition are ACE inhibitors (ie, captopril, enalapril, lisinopril) or angiotensin II receptor antagonist blockers (ie, telmisartan, losartan, irbesartan, candesartan). Calcium channel blockers are not recommended.
- Control abnormalities related to renal failure (ie, hyperkalemia, hyperphosphatemia, hypocalcemia, hyperparathyroidism, acidosis).
- Treat urinary tract infections, which occur in 30-50% of patients and most frequently in women. Gram-negative bacteria are the most common pathogens.
- Reduce abdominal pain produced by enlarged kidneys.
- Avoid nonsteroidal anti-inflammatory drugs (NSAIDs).
- With heart murmurs, institute routine American Heart Association antibiotic prophylaxis.
- Treatment involves surgical cyst decompression, which is effective for pain relief in 60-80% of patients. See Surgical Care.
- Distinguishing between infections of the bladder, renal parenchyma, and cysts is important because the treatment for each condition is different. Treating infected cysts requires antibiotics that penetrate into the cyst. Useful agents are ciprofloxacin, trimethoprim-sulfamethoxazole, clindamycin, and chloramphenicol.
- Patients with ADPKD and ESRD may undergo hemodialysis, peritoneal dialysis, or renal transplantation.
Surgical Care
- Infected renal or hepatic cysts
- If infected cysts do not respond to conventional antibiotic therapy, surgical drainage may be necessary.
- This procedure is usually performed with ultrasonographically guided puncture.
- Large cysts causing abdominal pain
- Cysts may become large enough to cause abdominal discomfort or pain. Typically, acute pain is from cyst hemorrhage or an obstruction by a clot, stone, or infection.7
- When one or more cysts can be identified as causing the pain, the symptoms can often be abated by open- or fiber optic–guided surgery to excise the outer walls and to drain them.
- In approximately one half of patients, however, candidate cysts cannot be identified as directly causing the pain. In these cases, indiscriminate excision of dozens of cyst walls that abut the capsule have produced complete symptomatic relief for months or years. Volumetric reduction of these kidneys usually exceeds 50% but still leaves kidneys larger than normal size. Not every cyst can be removed, and, with time, the residual cysts enlarge and symptoms may reappear.
- Approximately one quarter of patients with the most severe pain do not gain relief from surgery or pharmacologic therapy with narcotics. These individuals usually have inaccessible cysts in the medullary portions of the kidneys. Nephrectomy is used as a last resort to control the pain in these patients.
- Massive polycystic liver diseases (see image below and Image 2)
- When the liver becomes so large that it prevents the patient from obtaining normal nutrition or causes severe abdominal discomfort, a surgical procedure is necessary.
- Surgical intervention may range from unroofing several cysts to a partial hepatectomy.
- Partial hepatectomy is difficult because of the characteristics of the polycystic liver. Only expert surgeons should proceed with this surgical procedure.
- When the polycystic liver causes portal hypertension or is very large with nonresectable areas, liver transplantation may be necessary.
- Special attention should be paid when bilateral nephrectomy has to be carried out in patients with severe liver involvement. Several cases of refractory ascites after bilateral nephrectomy have been reported in these patients.
Consultations
- Nephrologist upon evidence of renal insufficiency, hypertension, microalbuminuria, or concentrating defect
- Invasive radiologist for cyst sclerosis or drainage
- General surgeon for nephrectomy, cyst decompression, unroofing, or surgical hepatic procedures
- Neurosurgeon for ICAs
- Cardiologist for valvular abnormalities
Diet
- Although a low-salt diet is recommended when hypertension or renal failure is present, no other special diet reportedly is of benefit.
Activity
- Patients should avoid contact sports in which direct trauma to the back or abdomen is likely. This is especially important with larger, palpable kidneys in order to minimize the risk of rupture.
Medication
No specific medication is available for ADPKD; however, clinical trials with vasopressin 2 receptor antagonists (Tolvaptan), somatostatin, and rapamycin are ongoing. The drugs of choice for hypertension are ACE inhibitors and angiotensin II receptor antagonist blockers. Do not treat abdominal pain with NSAIDs because of its potential nephrotoxic effect.
Cyst infections require gyrase inhibitors (eg, ciprofloxacin, chloramphenicol, clindamycin). Trimethoprim-sulfamethoxazole is also an effective antibiotic for reaching the inner cavity of the cyst. Renal failure requires drugs to maintain electrolyte levels (eg, calcium carbonate, calcium acetate, sevelamer, lanthanum carbonate, calcitriol [possibly], diuretics, blood pressure medications). Approximately 62% of patients with renal insufficiency require at least 2 antihypertensive agents for optimal blood pressure control.
ACE inhibitors
These peptides suppress the renin-angiotensin-aldosterone system.
Enalapril (Vasotec)
Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
2.5-5 mg/d PO, increase prn
Dosing range: 10-40 mg/d PO in 1-2 divided doses
Alternatively: 1.25 mg/dose IV over 5 min q6h
Pediatric
Not established
NSAIDs may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; hypotensive effects may be enhanced when administered concurrently with diuretics
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Renal impairment, valvular stenosis, or severe congestive heart failure
Lisinopril (Prinivil, Zestril)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Adult
10 mg/d PO; increase 5-10 mg/d at 1- to 2-wk intervals; not to exceed 40 mg
Pediatric
Not established
NSAIDs may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; hypotensive effects may be enhanced when administered concurrently with diuretics
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Renal impairment, valvular stenosis, or severe congestive heart failure
Captopril (Capoten)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Adult
12.5-25 mg PO bid/tid; may increase by 12.5-25 mg/dose at 1- to 2-wk intervals; not to exceed 50 mg tid
Pediatric
6.25-12.5 mg/dose PO q12-24h; not to exceed 6 mg/kg/d
NSAIDs may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; hypotensive effects may be enhanced when administered concurrently with diuretics
Documented hypersensitivity; renal impairment
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Renal impairment, valvular stenosis, or severe congestive heart failure
Electrolyte supplements
Administer to maintain electrolyte levels in renal failure.
Calcium carbonate (Oystercal)
Reduces phosphorus load.
Adult
1-2 g divided PO bid/qid
Pediatric
45-65 mg/kg/d PO divided qid
May decrease effects of tetracyclines, atenolol, salicylates, iron salts, and fluoroquinolones; IV administration antagonizes effects of verapamil; large intakes of dietary fiber may decrease calcium absorption and levels
Renal calculi; hypercalcemia; hypophosphatemia; renal or cardiac disease; patients with digitalis toxicity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Digitalized patients and respiratory failure or acidosis
Antibiotics
Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the clinical setting.
Ciprofloxacin (Cipro)
Inhibits bacterial DNA synthesis and, consequently, growth. Fluoroquinolone with activity against pseudomonads, streptococci, MRSA, Staphylococcus epidermidis, and most gram-negative organisms, but no activity against anaerobes. Levofloxacin (Levaquin) overcomes many of these limitations. Continue treatment for at least 2 d (7-14 d typical) after signs and symptoms have disappeared
Adult
250-500 mg PO bid for 7-14 d
Pediatric
<18 years: Not recommended
>18 years: Administer as in adults
Antacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones; cimetidine may interfere with metabolism; reduces therapeutic effects of phenytoin; probenecid may increase serum concentrations; may increase toxicity of theophylline, caffeine, cyclosporine, and digoxin (monitor digoxin levels); may increase effects of anticoagulants (monitor PT)
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
In prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Levofloxacin (Levaquin)
Inhibits growth of susceptible organisms by inhibiting DNA gyrase and promoting breakage of DNA strands.
Adult
750 mg PO q24h for 7-14 d
Pediatric
Not established
Antacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones; cimetidine may interfere with metabolism; reduces therapeutic effects of phenytoin; probenecid may increase serum concentrations; may increase toxicity of theophylline, caffeine, cyclosporine, and digoxin (monitor digoxin levels); may increase effects of anticoagulants (monitor PT)
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
In prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Trimethoprim-sulfamethoxazole (Bactrim, Bactrim DS, Septra, Septra DS, Cotrim C)
Inhibits bacterial growth by inhibiting synthesis of dihydrofolic acid.
Adult
20 mg TMP/kg/d IV divided qid
Pediatric
Administer as in adults
May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration with diuretics increases incidence of thrombocytopenic purpura in elderly people; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
Documented hypersensitivity; megaloblastic anemia due to folate deficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Discontinue at first appearance of skin rash or sign of adverse reaction; obtain CBC counts frequently; discontinue therapy if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, administer 5-15 mg/d leucovorin); caution in folate deficiency (eg, chronic alcoholism, elderly people, anticonvulsant therapy, malabsorption syndrome); hemolysis may occur in G-6-PD deficiency; AIDS patients may not tolerate or respond to TMP-SMZ; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); administer fluids to prevent crystalluria and stone formation
Clindamycin (Cleocin)
Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.
Adult
150-450 mg/dose PO q6-8h; not to exceed 1.8 g/d
600-1200 mg/d IV/IM divided q6-8h depending on degree of infection
Pediatric
8-20 mg/kg/d PO as hydrochloride and 8-25 mg/kg/d as palmitate divided tid/qid
20-40 mg/kg/d IV/IM divided tid/qid
Increases duration of neuromuscular blockade induced by tubocurarine and pancuronium; erythromycin may antagonize effects; antidiarrheals may delay absorption
Documented hypersensitivity; regional enteritis; ulcerative colitis; hepatic impairment; antibiotic-associated colitis
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in severe hepatic dysfunction; no adjustment necessary in renal insufficiency; associated with severe and possibly fatal colitis
Chloramphenicol (Chloromycetin)
Binds to 50S bacterial-ribosomal subunits and inhibits bacterial growth by inhibiting protein synthesis. Effective against gram-negative and gram-positive bacteria.
Adult
50-100 mg/kg/d PO/IV divided q6h for 10 d; not to exceed 4 g/d
Pediatric
50-75 mg/kg/d PO/IV divided q6h
With concurrent administration with barbiturates, serum levels may decrease while barbiturate levels may increase, causing toxicity; manifestations of hypoglycemia may occur with sulfonylureas; rifampin may reduce serum levels, presumably through hepatic enzyme induction; may increase effects of anticoagulants; may increase serum hydantoin levels, possibly resulting in toxicity (chloramphenicol levels may increase or decrease)
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Use only for indicated infections or as prophylaxis for bacterial infections; serious and fatal blood dyscrasias (eg, aplastic anemia, hypoplastic anemia, thrombocytopenia, granulocytopenia) can occur; evaluate baseline and perform periodic blood studies approximately q2d while on therapy; discontinue upon appearance of reticulocytopenia, leukopenia, thrombocytopenia, anemia, or findings attributable to chloramphenicol; adjust dose in liver or kidney dysfunction; caution in pregnancy at term or during labor because of potential toxic effects on fetus (gray syndrome)
Phosphate binders
Administer to maintain phosphate levels in renal failure.
Lanthanum carbonate (Fosrenol)
Noncalcium, nonaluminum phosphate binder indicated for reduction of high phosphorus levels in patients with ESRD. Directly binds dietary phosphorus in upper GI tract, thereby inhibiting phosphorus absorption.
Adult
Initial: 250-500 mg PO tid pc (chewable tabs); adjust dose q2-3wk to target serum phosphorus level
Maintenance: 500-1000 mg PO tid pc
Pediatric
Not established
Drugs known to interact with antacids (eg, alendronate, amprenavir, ciprofloxacin, itraconazole, tetracycline, thyroid hormones) should not be administered within 2 h
Documented hypersensitivity; bowel obstruction; hypophosphatemia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Deposited into developing bone, including growth plate (long-term effects unknown); common adverse effects typically diminish over time but include headache, abdominal pain, nausea, diarrhea, constipation, and vomiting; in clinical trials, dialysis graft occlusion occurred more frequently than with placebo; caution with GI motility diseases (eg, Crohn disease, ulcerative colitis) or recent GI surgery
Sevelamer hydrochloride (Renagel)
Polymeric phosphate binder for oral administration. Does not contain aluminum and, thus, aluminum intoxication is not a concern.
Adult
2-4 cap PO pc; adjust based on serum phosphorus concentrations to lower serum phosphorus to <6 mg/dL
Pediatric
Not established
May reduce absorption of drugs co-administered with sevelamer
Documented hypersensitivity; bowel obstruction; hypophosphatemia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in patients with dysphagia, severe GI motility disorders, or swallowing disorders; can cause hypophosphatemia in patients with low or normal serum phosphate levels; when changes in absorption of oral medications may have clinical consequences (eg, antiseizure or antiarrhythmic drugs), medications should be taken 1 h before or 3 h after a dose of sevelamer
Angiotensin II receptor antagonists
These agents interfere with the binding of formed angiotensin II to its endogenous receptor.
Valsartan (Diovan)
Prodrug that produces direct antagonism of angiotensin II receptors. Displaces angiotensin II from AT1 receptor and may lower blood pressure by antagonizing AT1-induced vasoconstriction, aldosterone release, catecholamine release, arginine vasopressin release, water intake, and hypertrophic responses. May induce more complete inhibition of renin-angiotensin system than ACE inhibitors, does not affect response to bradykinin, and is less likely to be associated with cough and angioedema. For use in patients unable to tolerate ACE inhibitors.
Adult
80 mg/d PO; may increase to 160 mg/d if needed
Pediatric
<6 years: Not established
>6 years: 1.3 mg/kg PO qd initially, not to exceed 40 mg/d; may adjust dose according to blood pressure response up to 2.7 mg/kg/d (not to exceed 160 mg/d)
May increase digoxin, lithium, and allopurinol levels; probenecid may increase valsartan levels; coadministration with diuretics increases hypotensive effects; NSAIDs may reduce hypotensive effects of valsartan; may increase risk of hyperkalemia if taken concurrently with potassium supplements or other potassium-sparing diuretics
Documented hypersensitivity; severe hepatic insufficiency; biliary cirrhosis or obstruction; primary hyperaldosteronism; bilateral renal artery stenosis
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimesters; caution in hyperkalemia, suspected bilateral renal artery stenosis, or suspected solitary kidney with unilateral renal artery stenosis
Losartan (Cozaar)
Angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. May induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors, does not affect the response to bradykinin, and is less likely to be associated with cough and angioedema. For patients unable to tolerate ACE inhibitors.
Adult
25-100 mg PO qd or divided bid
Pediatric
<6 years: Not established
6-16 years: 0.7 mg/kg PO qd; not to exceed 50 mg/d if <50 kg or 100 mg/d if >50 kg
CrCl <30 mL/min: Not established
May increase digoxin, lithium, and allopurinol levels; probenecid may increase losartan levels; coadministration with diuretics increases hypotensive effects; NSAIDs may reduce hypotensive effects of losartan; may increase risk of hyperkalemia if taken concurrently with potassium supplements or other potassium-sparing diuretics
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimesters; caution in patients with unilateral or bilateral renal artery stenosis
Candesartan (Atacand)
Blocks vasoconstriction and aldosterone-secreting effects of angiotensin II. May induce more complete inhibition of renin-angiotensin system than ACE inhibitors, does not affect response to bradykinin, and is less likely to be associated with cough and angioedema. Use in patients unable to tolerate ACE inhibitors.
Angiotensin II receptor antagonist blockers reduce blood pressure and proteinuria, protecting renal function and delaying onset of ESRD.
Adult
8-16 mg/d PO initially; not to exceed 32 mg/d
Pediatric
Not established
May increase digoxin, lithium, and allopurinol levels; probenecid may increase candesartan levels; coadministration with diuretics increases hypotensive effects; NSAIDs may reduce hypotensive effects of candesartan; may increase risk of hyperkalemia if taken concurrently with potassium supplements or other potassium-sparing diuretics
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimesters; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; watch for serum potassium
Olmesartan (Benicar)
Blocks vasoconstrictor effects of angiotensin II by selectively blocking binding of angiotensin II to AT-1 receptor in vascular smooth muscle. Action is independent of pathways for angiotensin II synthesis.
Adult
20 mg PO qd initially; may increase to 40 mg/d after 2 wk if further BP reduction required; lower dose in volume- or salt-depleted patients
Pediatric
Not established
Diuretics may enhance hypotensive effect
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pregnancy category D in second and third trimesters; may cause injury or even death to the developing fetus due to effect on renin-angiotensin system if given in second or third trimesters of pregnancy; serum levels and AUC increase with renal and hepatic insufficiency, respectively; may cause oliguria, azotemia, and acute renal failure; facial edema, angioedema, or decreased hemoglobin or hematocrit occur rarely
More on Polycystic Kidney Disease |
| Overview: Polycystic Kidney Disease |
| Differential Diagnoses & Workup: Polycystic Kidney Disease |
Treatment & Medication: Polycystic Kidney Disease |
| Follow-up: Polycystic Kidney Disease |
| Multimedia: Polycystic Kidney Disease |
| References |
| Further Reading |
| « Previous Page | Next Page » |
References
Rahman E, Niaz FA, Al-Suwaida A, et al. Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: A single center study. Saudi J Kidney Dis Transpl. Sep-Oct 2009;20(5):806-10. [Medline].
Schrier RW. Renal volume, renin-angiotensin-aldosterone system, hypertension, and left ventricular hypertrophy in patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol. Sep 2009;20(9):1888-93. [Medline].
Cadnapaphornchai MA, McFann K, Strain JD, et al. Prospective change in renal volume and function in children with ADPKD. Clin J Am Soc Nephrol. Apr 2009;4(4):820-9. [Medline].
Doulton TW, Saggar-Malik AK, He FJ, Carney C, Markandu ND, Sagnella GA, et al. The effect of sodium and angiotensin-converting enzyme inhibition on the classic circulating renin-angiotensin system in autosomal-dominant polycystic kidney disease patients. J Hypertens. May 2006;24(5):939-45. [Medline].
Sawicki M, Walecka A, Rozanski J, et al. Doppler sonography measurements of renal vascular resistance in autosomal-dominant polycystic kidney disease. Med Sci Monit. Aug 2009;15(8):MT101-4. [Medline].
Ravine D, Gibson RN, Walker RG, et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. Apr 2 1994;343(8901):824-7. [Medline].
Sallee M, Rafat C, Zahar JR, et al. Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. Jul 2009;4(7):1183-9. [Medline].
Arnold HL, Harrison SA. New advances in evaluation and management of patients with polycystic liver disease. Am J Gastroenterol. Nov 2005;100(11):2569-82. [Medline].
Chauveau D, Pirson Y, Verellen-Dumoulin C, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int. Apr 1994;45(4):1140-6. [Medline].
Gabow PA. Autosomal dominant polycystic kidney disease. N Engl J Med. Jul 29 1993;329(5):332-42. [Medline].
Grantham JJ, Chapman AB, Torres VE. Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol. Jan 2006;1(1):148-57. [Medline].
Grantham JJ, Torres VE, Chapman AB, et al. Volume progression in polycystic kidney disease. N Engl J Med. May 18 2006;354(20):2122-30. [Medline]. [Full Text].
Hateboer N, v Dijk MA, Bogdanova N, et al. Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. Jan 9 1999;353(9147):103-7. [Medline].
Hossack KF, Leddy CL, Johnson AM, et al. Echocardiographic findings in autosomal dominant polycystic kidney disease. N Engl J Med. Oct 6 1988;319(14):907-12. [Medline].
Huston J 3rd, Torres VE, Wiebers DO, et al. Follow-up of intracranial aneurysms in autosomal dominant polycystic kidney disease by magnetic resonance angiography. J Am Soc Nephrol. Oct 1996;7(10):2135-41. [Medline].
Masoumi A, Reed-Gitomer B, Kelleher C, et al. Potential pharmacological interventions in polycystic kidney disease. Drugs. 2007;67(17):2495-510. [Medline].
Ong AC, Wheatley DN. Polycystic kidney disease--the ciliary connection. Lancet. Mar 1 2003;361(9359):774-6. [Medline].
Parfrey PS, Bear JC, Morgan J, et al. The diagnosis and prognosis of autosomal dominant polycystic kidney disease. N Engl J Med. Oct 18 1990;323(16):1085-90. [Medline].
Pei Y. Diagnostic approach in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. Sep 2006;1(5):1108-14. [Medline].
Qian F, Watnick TJ, Onuchic LF, et al. The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell. Dec 13 1996;87(6):979-87. [Medline].
Ramos A, Torres VE, Holley KE, et al. The liver in autosomal dominant polycystic kidney disease. Implications for pathogenesis. Arch Pathol Lab Med. Feb 1990;114(2):180-4. [Medline].
Rossetti S, Harris PC. Genotype-phenotype correlations in autosomal dominant and autosomal recessive polycystic kidney disease. J Am Soc Nephrol. May 2007;18(5):1374-80. [Medline].
Russell RT, Pinson CW. Surgical management of polycystic liver disease. World J Gastroenterol. Oct 14 2007;13(38):5052-9. [Medline].
Schrier RW. Optimal care of autosomal dominant polycystic kidney disease patients. Nephrology (Carlton). Apr 2006;11(2):124-30. [Medline].
Tahvanainen E, Tahvanainen P, Kääriäinen H, et al. Polycystic liver and kidney diseases. Ann Med. 2005;37(8):546-55. [Medline].
Torra R, Badenas C, Darnell A, et al. Linkage, clinical features, and prognosis of autosomal dominant polycystic kidney disease types 1 and 2. J Am Soc Nephrol. Oct 1996;7(10):2142-51. [Medline].
Torres VE. Vasopressin antagonists in polycystic kidney disease. Kidney Int. Nov 2005;68(5):2405-18. [Medline]. [Full Text].
Torres VE, Harris PC. Mechanisms of Disease: autosomal dominant and recessive polycystic kidney diseases. Nat Clin Pract Nephrol. Jan 2006;2(1):40-55; quiz 55. [Medline]. [Full Text].
Torres VE, Harris PC. Polycystic kidney disease: genes, proteins, animal models, disease mechanisms and therapeutic opportunities. J Intern Med. Jan 2007;261(1):17-31. [Medline].
Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. Apr 14 2007;369(9569):1287-301. [Medline].
Walz G. Therapeutic approaches in autosomal dominant polycystic kidney disease (ADPKD): is there light at the end of the tunnel?. Nephrol Dial Transplant. Jul 2006;21(7):1752-7. [Medline].
Weimbs T. Regulation of mTOR by polycystin-1: is polycystic kidney disease a case of futile repair?. Cell Cycle. Nov 1 2006;5(21):2425-9. [Medline].
Wilson PD. Polycystic kidney disease. N Engl J Med. Jan 8 2004;350(2):151-64. [Medline].
Further Reading
Related eMedicine topics:
Autosomal Dominant Polycystic Kidney Disease
Autosomal Recessive Polycystic Kidney Disease
Caroli Disease [Pediatrics: General Medicine]
Caroli Disease [Radiology]
Cystic Diseases of the Kidney
Hepatic Cysts
Neonatal Hypertension
Polycystic Kidney Disease [Pediatrics: General Medicine]
Potter Syndrome
Clinical guidelines:
ACR Appropriateness Criteria® renal failure. American College of Radiology - Medical Specialty Society. 1995 (revised 2008). 10 pages. NGC:007019
Urinary tract infections in renal insufficiency, transplant recipients, diabetes mellitus and immunosuppression. In: Guidelines on the management of urinary and male genital tract infections. European Association of Urology - Medical Specialty Society. 2008 Mar. 12 pages. [NGC Update Pending] NGC:006489
Clinical trials:
Autosomal Dominant Polycystic Kidney Disease (ADPKD) Pain Study
Effect of Statin Therapy on Disease Progression in Autosomal Dominant Polycystic Kidney Disease (ADPKD)
Efficacy, Safety and Tolerability of Everolimus in Preventing End-Stage Renal Disease in Patients With Autosomal Dominant Polycystic Kidney Disease
Evaluation of Autosomal Recessive Polycystic Kidney Disease and Congenital Hepatic Fibrosis
Polycystic Kidney Disease Data Repository
The Effect of High and Low Sodium Intake on Urinary Aquaporin-2 in Autosomal Dominant Polycystic Kidney Disease
Keywords
polycystic kidney disease, PKD, polycystic kidney, kidney cyst, kidney cysts, kidney disease, renal cyst, renal cysts, autosomal dominant polycystic kidney disease, ADPKD, adult polycystic kidney disease, polycystic kidney disease type 1, PKD1, polycystic kidney disease type 2, PKD2, kidney failure, renal failure, dialysis-dependent kidney disease, end-stage renal disease, ESRD, end-stage kidney disease, ESKD, renal transplantation, renal transplant, kidney transplantation, kidney transplant, hemodialysis, peritoneal dialysis, ADPKD type 1, ADPKD1, ADPKD type 2, ADPKD2


Treatment & Medication: Polycystic Kidney Disease