Sandbox:khurram
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
Treatment of ADPKD is targeted at the management of symptoms and complications of the disease including tight blood pressure control with ACEIs or ARBs, pain control, antibiotics in cases of cyst infections or pyelonephritis, and avoidance of abdominal trauma.
Treatment
Treatment of ADPKD is targeted at managing symptoms and disease complications. There have been no treatments associated with disease regression or slowing of cysts formation.[1] No professional society has proposed guidelines for the management of patients with ADPKD.
Hypertension
Up to 75% of patients with ADPKD on imaging without any renal insufficiency are hypertensive.[2] Even in young patients, 50% of those aged 20-34 years are hypertensive despite normal renal function.[3] Treatment is recommended when blood pressure exceeds 130/80 mm Hg for adults, or sex and age adjusted norms for children. ACE inhibitors and ARBs are highly recommended as observational studies have shown better preservation of renal function with these agents. Eventual resistance or blunting of the effects of the these agents with increased volume retention can be encountered. Salt restriction, with addition of thiazide diuretics initially or loop diuretics as a second line, is justified.[1] The first randomized clinical trial evaluating the use of lisinopril and telmisartan and their effect on progression of kidney disease in ADPKD patients, the HALT Progression of Polycystic Kidney Disease (HALT PKD), is expected to conclude by June 2014.[4]
Hematuria
Gross hematuria with significant renal colic secondary to collecting system clots is not uncommon. Treatment usually involves analgesia, bed rest and hydration to increase urinary flow rate to 2-3 liters daily. In most patients, gross hematuria regresses to microscopic hematuria in a few days. Patients should be advised to avoid sports with risk of abdominal trauma to prevent recurrent episodes.[1]
Urinary Tract Infections
Women are at a higher risk for UTIs and cyst infections with gram negative rods being the most common pathogens. Symptomatic cyst infections or acute pyelonephritis require hospital admission with floroquinolone therapy until pain, fever, and leukocytosis resolve.[5]
Pain
Severe flank or abdominal pain in the absence of cyst complications is very common. Several options for treatment exist including regular analgesics, local anesthetic injections, surgical cyst unroofing, transcutaneous stimulation, and surgical renal denervation. No randomized control trials have been conducted to evaluate the efficacy of each of these treatments. Generally, invasive procedures are reserved for patients with intractable pain that is not responding to classical pain management. [6]
Renal Failure
Renal failure is the eventual complication of almost all patients with ADPKD. Treatment is usually with the 3 major forms of renal replacement with special considerations required for some cases. Peritoneal dialysis is usually not recommended in patients with aortic aneurysms. Hemodialysis, although the most common form of renal replacement, required significant anticoagulation that may complicate existing hematuria. Ultimately, renal transplantation is the preferred renal replacement modality with outcomes similar to patients with other baseline kidney diseases leading to ESRD.[1]
References
- ↑ 1.0 1.1 1.2 1.3 Grantham JJ (2008). "Clinical practice. Autosomal dominant polycystic kidney disease". N Engl J Med. 359 (14): 1477–85. doi:10.1056/NEJMcp0804458. PMID 18832246.
- ↑ Gabow PA (1990). "Autosomal dominant polycystic kidney disease--more than a renal disease". Am J Kidney Dis. 16 (5): 403–13. PMID 2239929.
- ↑ Kelleher CL, McFann KK, Johnson AM, Schrier RW (2004). "Characteristics of hypertension in young adults with autosomal dominant polycystic kidney disease compared with the general U.S. population". Am J Hypertens. 17 (11 Pt 1): 1029–34. doi:10.1016/j.amjhyper.2004.06.020. PMID 15533729.
- ↑ Torres VE, Chapman AB, Perrone RD, Bae KT, Abebe KZ, Bost JE; et al. (2012). "Analysis of baseline parameters in the HALT polycystic kidney disease trials". Kidney Int. 81 (6): 577–85. doi:10.1038/ki.2011.411. PMC 3580956. PMID 22205355.
- ↑ Gibson P, Watson ML (1998). "Cyst infection in polycystic kidney disease: a clinical challenge". Nephrol Dial Transplant. 13 (10): 2455–7. PMID 9794537.
- ↑ Bajwa ZH, Gupta S, Warfield CA, Steinman TI (2001). "Pain management in polycystic kidney disease". Kidney Int. 60 (5): 1631–44. doi:10.1046/j.1523-1755.2001.00985.x. PMID 11703580 Check
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