eMedicine Specialties > Urology > Congenital Urologic Conditions
Cystic Diseases of the Kidney: Treatment & Medication
Updated: Jul 22, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Effective means of prevention or modulation of disease have not yet been identified. Current treatment is aimed at symptom control. In general, therapy is reserved for pain, hypertension, infection, renal salt wasting, and nephrolithiasis.
- Inherited cystic renal disease
- Autosomal dominant polycystic kidney disease
- Patients have decreased ability to concentrate urine and should be encouraged to drink 1-2 L of water daily.
- Generally, 130/80 is considered the treatment goal for hypertension in this population. Moderate hypertension may be treated with sodium restriction (ie, <100 mEq/d), exercise, and weight control. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) are effective in controlling hypertension in autosomal dominant polycystic kidney disease (ADPKD). However, ACE inhibitors have been associated with reversible renal failure in polycystic kidney disease. Calcium channel blockers also are effective in managing hypertension in ADPKD.
- Prevention of infection with appropriate precautions is important, particularly in women. Avoid urinary tract instrumentation whenever possible.
- Treatment of infection involving cystic kidneys requires a prolonged course of antibiotics. Most cyst walls are permeable to polar antibiotics, including cephalosporins, penicillin derivatives, and aminoglycosides. Occasionally, cysts are relatively impermeable to these agents and require parenteral lipophilic antibiotics, such as ciprofloxacin, erythromycin, chloramphenicol, or a tetracycline. Clinical evaluation findings, including sterile urine, lack of fever, and no renal pain on deep palpation, should guide the route and duration of antibiotic therapy.
- Autosomal recessive polycystic kidney disease (ARPKD): The newborn is provided supportive therapy while the degree of pulmonary insufficiency and the etiology is reviewed. Dialysis may be required for renal failure. With less severe childhood disease, edema often is a problem and is managed with sodium restriction and loop diuretics. Hypertension is controlled with salt restriction and antihypertensives, with particular emphasis on the use of ACE inhibitors and ARBs.
- Juvenile nephronophthisis (JNPHP) and medullary cystic kidney disease: With severe salt wasting, salt supplementation may improve renal function and slow renal demise. End-stage renal insufficiency necessitates dialysis or renal transplantation.
- Autosomal dominant polycystic kidney disease
- Acquired cystic renal disease
- Acquired renal cystic disease (ARCD): Mild bleeding episodes may be managed with bed rest and analgesics.
- Medullary sponge kidney (MSK): Encourage patients with nephrolithiasis to produce 2 L of urine daily. Patients with hypercalcuria may benefit from oral thiazide diuretics. Patients may develop UTI and should be taught preventative measures.
- Simple cyst: An infected simple cyst usually requires a combination of antimicrobial and surgical management. Pathogens encountered most frequently in infected simple cysts include Enterobacteriaceae, staphylococci, and Proteus species.
- Inhibitors of the EGF receptor tyrosine kinase have been shown to slow cyst development and extend the life span in polycystic mice. Clinical trials with these agents are underway.21
- The identification of mTOR as a possible common pathway to cyst development makes this protein an attractive target for therapy. Rapamycin inhibits mTOR and has been shown to stop kidney growth and even allow regression in kidney size in a mouse model. Additionally, a retrospective comparison of patients treated with rapamycin to those not treated demonstrated a 25% decrease in kidney volume in the treatment group.15,16 Further study is required to determine the relevance and efficacy of such therapy in a clinical population.
Surgical Care
- Multicystic dysplastic kidney (MCDK): Previously, the involved kidney was routinely removed to prevent the subsequent development of symptoms. Today, however, surgical excision is indicated only if the dysplastic kidney interferes with respiratory or digestive function or if significant hypertension has developed. Additionally, cyst rupture, which can occur spontaneously or secondary to trauma, may require emergent surgical intervention.
- Inherited cystic renal disease
- ADPKD: Significant chronic pain may result from expansion of renal cysts. Percutaneous aspiration and sclerosis may control these symptoms successfully. However, for the management of severe pain, particularly for large kidneys with innumerable cysts, surgical excision may be preferred. Laparoscopic techniques have been used with good outcomes. Nephrectomy may be performed simultaneously with renal transplantation in order to create space for the transplanted kidney and to relieve symptoms associated with the native polycystic kidney. Overall, patients may obtain relief that lasts several years with surgical intervention. In extreme cases of liver enlargement, severe pain and wasting may result. Partial hepatectomy may alleviate these symptoms.
- ARPKD: In patients with severe portal hypertension, sclerotherapy or portosystemic shunt placement may be necessary to control bleeding. Splenectomy may be indicated for splenomegaly with significant complications.
- JNPHP and medullary cystic kidney disease (MCKD): If transplantation is considered, selecting an older or unrelated donor is advisable to minimize the risk of the transplanted kidney also being affected with these diseases.
- Acquired cystic renal disease
- ARCD: Persistent or severe hemorrhage may necessitate nephrectomy or renal embolization. If a 3-cm renal mass suggestive of renal cell carcinoma (RCC) is noted, a partial or radical nephrectomy is indicated.
- Simple, intermediate, and suspicious cysts: Simple renal cysts rarely require surgical management to relieve pain or obstruction. Treatment options include aspiration, sclerosis, open resection, endoscopic marsupialization and fulguration, percutaneous resection, and laparoscopic resection.
- Bosniak category III and IV renal cysts require surgical exploration. Approximately 50% of Bosniak category III cystic renal lesions are malignant. Management depends on the appearance of the lesion and varies from exploration and biopsy to nephrectomy. The current standard approach is open exploration with anticipated partial nephrectomy. However, as the experience with laparoscopic exploration and nephrectomy grows, this technique may prove equally reasonable.
Medication
No specific medical therapies are available for the renal cysts themselves. Complications of cystic renal diseases, such as hypertension, infection, and pain, are treated with standard medical therapy. Some examples are listed below.
Angiotensin-converting enzyme inhibitors
These agents reduce aldosterone secretion.
Lisinopril (Zestril, Prinivil)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Adult
10 mg/d PO; increase to 5-10 mg/d at 1- to 2-wk intervals; not to exceed 40 mg
Pediatric
Not established
May increase digoxin, lithium, and allopurinol levels; probenecid may increase lisinopril levels; coadministration with diuretics increases hypotensive effects; possible enhanced hypotensive effects of lisinopril when administered concurrently with diuretics or NSAIDs
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal impairment, valvular stenosis, or severe congestive heart failure
Angiotensin II receptor antagonists
These agents antagonize the effects of angiotensin II.
Losartan (Cozaar)
Nonpeptide 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/bid
Pediatric
Not established
Ketoconazole, sulfaphenazole, and phenobarbital may decrease effects; cimetidine may increase effects
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Patients with unilateral or bilateral renal artery stenosis
Calcium channel blockers
In specialized conducting and automatic cells in the heart, calcium is involved in the generation of the action potential. The calcium channel blockers inhibit movement of calcium ions across the cell membrane, depressing both impulse formation (automaticity) and conduction velocity.
Diltiazem (Cardizem, Dilacor, Tiazac)
During depolarization, inhibits calcium ions from entering the slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium.
Adult
Cardizem SR: 60-120 mg PO bid
Cardizem CD for hypertension: 180-240 mg PO qd
Dilacor for hypertension: 180-240 mg PO qd
Pediatric
Not established
May increase carbamazepine, digoxin, cyclosporine, and theophylline levels; possible bradycardia and decrease in cardiac output when administered with amiodarone; possible increase in cardiac depression when administered with beta-blockers; cimetidine may increase diltiazem levels
Documented hypersensitivity; severe CHF; sick sinus syndrome; second-degree or third-degree AV block; hypotension (<90 mm Hg systolic)
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
Impaired renal or hepatic function; may increase LFT levels, and hepatic injury may occur
Antimicrobials
These agents are used to treat renal parenchymal infection (to be used in combination with gentamicin) and infected renal cysts.
Ampicillin (Principen, Omnipen, Marcillin)
Bactericidal activity against susceptible organisms. Alternative to amoxicillin when patients are unable to take medication orally. Used to treat parenchymal infection.
Adult
250-500 mg PO q6h
500 mg to 1.5 g IM q4-6h
500 mg to 3 g IV q4-6h; not to exceed 12 g/d
Pediatric
50-100 mg/kg/d PO divided q4-6h
100-400 mg/kg/d IV/IM divided q4-6h
Probenecid and disulfiram elevate ampicillin levels; allopurinol decreases ampicillin effects and has additive effects on ampicillin rash; may decrease effects of oral contraceptives
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal failure; evaluate rash and differentiate from hypersensitivity reaction
Gentamicin (Garamycin, Gentacidin)
Aminoglycoside antibiotic for gram-negative coverage. Used in combination with both an agent against gram-positive organisms and one that covers anaerobes. Not the DOC, but consider if penicillins or other less toxic drugs are contraindicated, when clinically indicated, and in mixed infections caused by susceptible staphylococci and gram-negative organisms. Dosing regimens are numerous; adjust dose based on CrCl and changes in volume of distribution. May be administered IV/IM.
Adult
Serious infections and normal renal function: 3 mg/kg/d IV q8h
Loading dose: 1-2.5 mg/kg IV q8h
Maintenance: 1-1.5 mg/kg IV q8h
Extended dosing regimen for life-threatening infections: 5 mg/kg/d IV/IM q6-8h
Follow each regimen by at least a trough level drawn on the third or fourth dose (0.5 h before dosing); may draw a peak level 0.5 h after 30-min infusion
Pediatric
<5 years: 2.5 mg/kg/dose IV/IM q8h
>5 years: 1.5-2.5 mg/kg/dose IV/IM q8h or 6-7.5 mg/kg/d divided q8h; not to exceed 300 mg/d; monitor as in adults
Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; aminoglycosides enhance effects of neuromuscular blocking agents (prolonged respiratory depression may occur); coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur (monitor regularly)
Documented hypersensitivity; non–dialysis-dependent renal insufficiency
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
Narrow therapeutic index (not intended for long-term therapy); caution in renal failure (not on dialysis), myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission; adjust dose in renal impairment
Sulfamethoxazole and trimethoprim (Bactrim, Bactrim DS, Septra, Septra DS)
Inhibits bacterial growth by inhibiting synthesis of dihydrofolic acid. Antibacterial activity of TMP-SMZ includes common urinary tract pathogens, except Pseudomonas aeruginosa. Used to treat infected renal cyst.
Adult
160 mg TMP/800 mg SMZ PO q12h for 10-14 d
Pediatric
<2 months: Do not administer
>2 months: 15-20 mg/kg/d (based on TMP) PO tid/qid for 14 d
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 of diuretics increases incidence of thrombocytopenia 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 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, advanced age, those receiving anticonvulsant therapy, those with malabsorption syndrome); hemolysis may occur in individuals with G-6-PD deficiency; patients with AIDS 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
Ciprofloxacin (Cipro)
Fluoroquinolone with activity against pseudomonads, streptococci, MRSA, S epidermidis, and most gram-negative organisms, but no activity against anaerobes. Inhibits bacterial DNA synthesis and, consequently, growth. Trovafloxacin (Trovan) overcomes many of these limitations. Continue treatment for at least 2 d (7-14 d typical) after signs and symptoms have disappeared.
Used to treat infected renal cyst either in patients intolerant to or not adequately covered by trimethoprim-sulfasalazine.
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 of fluoroquinolones; ciprofloxacin reduces therapeutic effects of phenytoin; probenecid may increase ciprofloxacin 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, periodically evaluate organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Thiazide diuretics
These agents are beneficial in the treatment of fluid retention.
Hydrochlorothiazide (Microzide, Esidrix, HydroDIURIL)
Inhibits reabsorption of sodium in distal tubules, increasing excretion of sodium and water, as well as potassium and hydrogen ions.
Adult
25-100 mg PO qd; not to exceed 200 mg/kg/d
Pediatric
<6 months: 2-3 mg/kg/d PO divided bid
>6 months: 2 mg/kg/d PO divided bid
Thiazides may decrease effects of anticoagulants, antigout agents, and sulfonylureas; thiazides may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Documented hypersensitivity; anuria; renal decompensation
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 renal disease, hepatic disease, gout, diabetes mellitus, and erythematosus
Analgesics
Pain control is essential to quality patient care. Analgesics ensure patient comfort and have sedating properties, which are beneficial for patients who experience pain.
Oxycodone and acetaminophen (Percocet, Roxicet, Roxilox, Tylox)
Drug combination indicated for the relief of moderate-to-severe pain.
Adult
1-2 tab or cap PO q4-6h prn
Pediatric
0.05-0.15 mg/kg/dose oxycodone PO q4-6h prn; not to exceed 5 mg/dose oxycodone
Phenothiazines may decrease analgesic effects; toxicity increases with coadministration of either CNS depressants or tricyclic antidepressants
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
Duration of action may increase in elderly people; be aware of total daily dose of acetaminophen patient is receiving; do not exceed 4000 mg/24 h of acetaminophen; higher doses may cause liver toxicity
More on Cystic Diseases of the Kidney |
| Overview: Cystic Diseases of the Kidney |
| Differential Diagnoses & Workup: Cystic Diseases of the Kidney |
 Treatment & Medication: Cystic Diseases of the Kidney |
| Follow-up: Cystic Diseases of the Kidney |
| Multimedia: Cystic Diseases of the Kidney |
| References |
| Further Reading |
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Keywords
acquired renal cystic disease, ARCD, acquired cystic renal disease, acquired cystic kidney disease, ACKD, dialysis-associated cystic renal disease, autosomal dominant polycystic kidney disease, ADPKD, autosomal recessive polycystic kidney disease, ARPKD, multicystic dysplastic kidney, MCDK, cystic renal dysplasia, cystic dysplasia, congenital multicystic kidney, end-stage renal disease, ESRD, medullary sponge kidney, MSK, nephronophthisis–medullary cystic kidney disease complex, NMCD, juvenile nephronophthisis, JNPHP, medullary cystic kidney disease, MCKD, nephronophthisis-uremic medullary cystic disease complex, renal cell carcinoma, RCC, tuberous sclerosis, TS, von Hippel-Lindau syndrome, VHLS, renal cysts, congenital cystic dysplasia, glomerulocystic kidney disease, GCKD
