Evaluation and Management of Pain in Autosomal Dominant Polycystic Kidney Disease
2010; Elsevier BV; Volume: 17; Issue: 3 Linguagem: Inglês
10.1053/j.ackd.2010.01.005
ISSN1548-5609
AutoresMarie C. Hogan, Suzanne M. Norby,
Tópico(s)Hedgehog Signaling Pathway Studies
ResumoTransient episodes of pain are common in autosomal dominant polycystic kidney disease (ADPKD). A small fraction of patients have disabling chronic pain. In this review, we discuss the etiologies of pain in ADPKD; review how ADPKD patients should be assessed; and discuss medical, surgical, and other management options. Transient episodes of pain are common in autosomal dominant polycystic kidney disease (ADPKD). A small fraction of patients have disabling chronic pain. In this review, we discuss the etiologies of pain in ADPKD; review how ADPKD patients should be assessed; and discuss medical, surgical, and other management options. Pain is a common symptom in patients with autosomal dominant polycystic kidney disease, often occurring early during the course of the disease and leading to the diagnosis.1Bajwa Z.H. Gupta S. Warfield C.A. Steinman T. Pain management in polycystic kidney disease.Kidney Int. 2001; 60: 1631-1644Crossref PubMed Scopus (105) Google Scholar, 2Bajwa Z.H. Sial K.A. Malik A.B. Steinman T.I. Pain patterns in patients with polycystic kidney disease.Kidney Int. 2004; 66: 1561-1569Crossref PubMed Scopus (83) Google Scholar, 3Dalgaard O.Z. Bilateral polycystic disease of the kidneys: A follow-up of two hundred and eighty-four patients and their families.Acta Med Scand. 1957; 328: 1-255Google Scholar, 4Gabow P.A. Autosomal dominant polycystic kidney disease–more than a renal disease.Am J Kidney Dis. 1990; 16: 403-413PubMed Scopus (214) Google Scholar, 5Grantham J.J. Renal pain in polycystic kidney disease: When the hurt won't stop.J Am Soc Nephrol. 1992; 2: 1161-1162PubMed Google Scholar, 6Iglesias C.G. Torres V.E. Offord K.P. Holley K.E. Beard C.M. Kurland L.T. Epidemiology of adult polycystic kidney disease, Olmsted County, Minnesota: 1935-1980.Am J Kidney Dis. 1983; 2: 630-639PubMed Google Scholar, 7Segura J.W. King Jr., B.F. Jowsey S.G. Martin P. Zince H. Chronic pain and its medical and surgical management in renal cystic diseases.in: Torres V.E. Watson M.L. Polycystic Kidney Disease. Oxford University, New York, NY1996: 462-480Google Scholar It can be managed effectively in most patients, but a minority of patients develop chronic pain that limits their ability to function; causes sleep disturbance, fatigue, anxiety, and depression; and negatively affects social relationships.8Clarke K.A. Iphofen R. A phenomenological hermeneutic study into unseen chronic pain.Br J Nurs. 2008; 17: 658-663PubMed Google Scholar, 9Heiwe S. Bjuke M. "An evil heritage": Interview study of pain and autosomal dominant polycystic kidney disease.Pain Management Nursing. 2009; 10: 134-141Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar Health care providers often fail to discuss pain during encounters with patients with ADPKD, leading to suboptimal management.2Bajwa Z.H. Sial K.A. Malik A.B. Steinman T.I. Pain patterns in patients with polycystic kidney disease.Kidney Int. 2004; 66: 1561-1569Crossref PubMed Scopus (83) Google Scholar, 9Heiwe S. Bjuke M. "An evil heritage": Interview study of pain and autosomal dominant polycystic kidney disease.Pain Management Nursing. 2009; 10: 134-141Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar Understanding the spectrum of causes of pain in ADPKD helps to guide diagnostic evaluations and specific treatments. Recent studies have sought to characterize pain in ADPKD both quantitatively2Bajwa Z.H. Sial K.A. Malik A.B. Steinman T.I. Pain patterns in patients with polycystic kidney disease.Kidney Int. 2004; 66: 1561-1569Crossref PubMed Scopus (83) Google Scholar and qualitatively.9Heiwe S. Bjuke M. "An evil heritage": Interview study of pain and autosomal dominant polycystic kidney disease.Pain Management Nursing. 2009; 10: 134-141Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar This review focuses on the causes of ADPKD-related pain and discusses management options. Patients with ADPKD report pain located in the low back (71%), abdomen (61%), head (49%), chest (30%), and legs (27%), often with radicular features.2Bajwa Z.H. Sial K.A. Malik A.B. Steinman T.I. Pain patterns in patients with polycystic kidney disease.Kidney Int. 2004; 66: 1561-1569Crossref PubMed Scopus (83) Google Scholar Although cyst hemorrhage, nephrolithiasis, and urinary tract infection are often associated with acute episodes of pain, the exact cause of chronic flank pain cannot always be determined.7Segura J.W. King Jr., B.F. Jowsey S.G. Martin P. Zince H. Chronic pain and its medical and surgical management in renal cystic diseases.in: Torres V.E. Watson M.L. Polycystic Kidney Disease. Oxford University, New York, NY1996: 462-480Google Scholar, 10Torres V.E. Harris P.C. Pirson Y. Autosomal dominant polycystic kidney disease.Lancet. 2007; 369: 1287-1301Abstract Full Text Full Text PDF PubMed Scopus (945) Google Scholar Chronic pain related to cysts has been attributed to traction of the renal pedicle, distension of the capsule, and compression of nearby structures.2Bajwa Z.H. Sial K.A. Malik A.B. Steinman T.I. Pain patterns in patients with polycystic kidney disease.Kidney Int. 2004; 66: 1561-1569Crossref PubMed Scopus (83) Google Scholar Sometimes, the patient may be able to indicate the location of pain with 1 finger, but frequently the pain is more diffuse. It can be described as "razor sharp," knife-like, dull, aching, cramping, or as a "fullness," generally lasting for hours to days and rated 4 to 5 out of 10 on a visual analog scale.2Bajwa Z.H. Sial K.A. Malik A.B. Steinman T.I. Pain patterns in patients with polycystic kidney disease.Kidney Int. 2004; 66: 1561-1569Crossref PubMed Scopus (83) Google Scholar, 9Heiwe S. Bjuke M. "An evil heritage": Interview study of pain and autosomal dominant polycystic kidney disease.Pain Management Nursing. 2009; 10: 134-141Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar Exacerbations occur unpredictably, several times per day or per week or only a few times a month. The inability to anticipate the onset of episodes or exacerbations of pain in ADPKD can cause patients to worry and to limit social activities.9Heiwe S. Bjuke M. "An evil heritage": Interview study of pain and autosomal dominant polycystic kidney disease.Pain Management Nursing. 2009; 10: 134-141Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar A small study using the Short-Form 36 Health Status Questionnaire (SF-36), a self-report tool to assess physical and mental well-being, showed that patients reporting use of pain medication within the previous month had lower scores on the Physical Component Summary than those who did not report any recent pain medication use.11Rizk D. Jurkovitz C. Veledar E. et al.Quality of life in autosomal dominant polycystic kidney disease patients not yet on dialysis.Clin J Am Soc Nephrol. 2009; 4: 560-566Crossref PubMed Scopus (50) Google Scholar The detection of kidney pain relies on input from sympathetic, parasympathetic, and sensory nerves.12Ansell J, Gee W, d B.J, Diseases of the kidney and ureter. In the management of pain (2nd edition) (ed J Bonica) Vol. 2 pp 1232-49. Lea & Febiger, Philadelphia, PA, in The management of pain., B. J, Editor. 1990, Lea & Febiger: Philadelphia, PA. p. 1232–1249.Google Scholar Chronic pain, regardless of the initial precipitating factor, is likely maintained by aberrant activity of sensory and autonomic neurons innervating the kidney, renal pelvis, and ureters (Fig 1). The sympathetic nerves supplying the kidneys originate in spinal cord segments T10 to L1 and travel via white rami to the paravertebral ganglia.12Ansell J, Gee W, d B.J, Diseases of the kidney and ureter. In the management of pain (2nd edition) (ed J Bonica) Vol. 2 pp 1232-49. Lea & Febiger, Philadelphia, PA, in The management of pain., B. J, Editor. 1990, Lea & Febiger: Philadelphia, PA. p. 1232–1249.Google Scholar, 13Naidoo N. Partab P. Pather N. Moodley J. Singh B. Satyapal K.S. Thoracic splanchnic nerves: implications for splanchnic denervation.J Anat. 2001; 1995: 585-590Crossref Scopus (27) Google Scholar The sympathetic nerves travel via the lesser splanchnic nerves from the T10 and 11 thoracic paravertebral ganglia to the synapse at the ipsilateral aorticorenal and celiac ganglia (Fig 1). From the 12th thoracic paravertebral ganglion, nerves travel via the least splanchnic nerve to the synapse either in the aorticorenal ganglion or in the renal plexus. The first lumbar splanchnic nerve and the postganglionic sympathetic nerves from the aorticorenal and celiac plexi synapse in the renal plexus. The parasympathetic innervation originates from the vagus nerve. These parasympathetic nerves traverse through the celiac plexus or pass directly to the renal plexus.14Brown J.A. Torres V.E. King B.F. Segura J.W. Laparoscopic marsupialization of symptomatic polycystic kidney disease.J Urol. 1996; 156: 22-27Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar Sensory renal nerves travel via the renal plexus, splanchnic nerves, thoracic sympathetic ganglia, T10-12 spinal nerves, and spinal cord dorsal horn neurons. Extensive cross-connection with innervation to other visceral structures explains the complex patterns of referred pain in some patients. Patients can complain of abdominal, back, and flank pain, all of which can be severe and require evaluation, in addition to the sensation of flank heaviness, which is not a "pain" as such. Kidney and nonkidney sources of pain not related to cystic disease must be considered; for example, abdominal wall hernias, colon diverticulitis, and possibly abdominal aneurysms, which occur with increased frequency in ADPKD patients. A detailed history should be obtained focusing pain location, duration, associated symptoms and relieving factors. The history will frequently help to identify whether pain relates directly or indirectly (eg, mechanical back pain related to organomegaly) to the cystic disease or whether is a manifestation of extrarenal associations of the disease. Cyst hemorrhage, urinary tract infection, and nephrolithiasis are common causes of transient pain in ADPKD. The frequency of cyst hemorrhage, gross hematuria, and nephrolithiasis correlate directly with the size of the kidneys.15Bennett W.M. Elzinga L.W. Clinical management of autosomal dominant polycystic kidney disease.Kidney Int Suppl. 1993; 42: S74-S79PubMed Google Scholar Pain caused by cyst hemorrhage is very common and reported as sharp, localized, and sudden in onset. It is thought that the pain is caused by an acute expansion in the cyst and distension of the renal capsule.4Gabow P.A. Autosomal dominant polycystic kidney disease–more than a renal disease.Am J Kidney Dis. 1990; 16: 403-413PubMed Scopus (214) Google Scholar Cyst hemorrhage is frequently associated with gross hematuria and passage of clots may be associated with renal colic. Occasionally subcapsular and retroperitoneal hemorrhage or hemoperitoneum may occur.16Ravich L. Lerman P.H. Drabkin J. Ruptured renal cyst in polycystic disease.Urology. 1976; 7: 60-61Abstract Full Text PDF PubMed Google Scholar, 17Tarrass F. Benjelloun M. Acute abdomen caused by spontaneous renal cyst rupture in an ADPKD haemodialysed patient.Nephrology. 2008; 13: 177-178Crossref PubMed Scopus (8) Google Scholar Vascular endothelial growth factor produced by the cystic epithelium promotes angiogenesis, which increases the risk of hemorrhage into cysts and gross hematuria. Symptomatic episodes probably underestimate the frequency of cyst hemorrhage because more than 90% of patients with ADPKD have cysts that are hyperdense (on computed tomography [CT] scan) or high signal (on magnetic resonance imaging [MRI]), indicative of blood or high protein content. Most hemorrhages will resolve within 2 to 7 days. If hematuria persists longer than 1 week or if the initial episode occurs after the age of 50 years, investigation to exclude neoplasm should be undertaken. Combined unenhanced and contrast-enhanced CT scan may be required (when kidney function permits) to make a correct diagnosis and differentiate among the various complications affecting patients with ADPKD.18Gupta S. Seith A. Sud K. et al.CT in the evaluation of complicated autosomal dominant polycystic kidney disease.Acta Radiol. 2000; 41: 280-284Crossref PubMed Scopus (39) Google Scholar, 19Nishiura J.L. Neves R.F. Eloi S.R. Cintra S.M. Ajzen S.A. Heilberg I.P. Evaluation of nephrolithiasis in autosomal dominant polycystic kidney disease patients.Clin J Am Soc Nephrol. 2009; 4: 838-844Crossref PubMed Scopus (60) Google Scholar Patients should be forewarned of pain episodes associated with cyst hemorrhage by their physicians and advised on what measures to take (see management). Urinary tract infections occur more frequently in women than men. Patients with ADPKD are at risk for acute pyelonephritis and for cyst infections. When there is suspicion of urinary tract infection in a patient with ADPKD, urine for Gram stain and culture should be immediately obtained and appropriate antibiotic management quickly instituted. Because genitourinary instrumentation is associated with an increased risk, prophylactic antibiotics are indicated when this instrumentation is necessary. Patients with upper urinary tract infections usually present with fever, leukocytosis, and flank pain. Acute pyelonephritis and cyst infection may be different to separate. Failure to respond to or recurrence of symptoms after an appropriate course of antibiotics should suggest a diagnosis of cyst infection. Cyst hemorrhage can also present with flank pain and fever but usually without leukocytosis. Negative urine and blood cultures do not entirely exclude cyst infection. In the appropriate clinical setting, cyst aspiration and culture of complex cysts detected by imaging studies may be indicated. Diagnostic criteria have recently been proposed for both kidney and hepatic cyst infection: presence of all of the following: fever (temperature >38.5 °C for >3 d), abdominal pain (particularly a palpable area of renal or liver tenderness), increased C-reactive protein (C-reactive protein >50 mg/L), and the absence of any significant recent intracystic bleeding (based on the results of an abdominal computed tomography [CT] scan) or other causes of fever.20Sallee M. Rafat C. Zahar J.-R. et al.Cyst infections in patients with autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol. 2009; 4: 1183-1189Crossref PubMed Scopus (133) Google Scholar Kidney (and liver) ultrasound data should be considered positive if debris with a thick wall and/or a distal acoustic enhancement is detected in at least one cyst. Kidney CT scan and magnetic resonance imaging (MRI) data are considered positive when enhanced wall thickening and/or perilesional inflammation is detected in at least 1 cyst.20Sallee M. Rafat C. Zahar J.-R. et al.Cyst infections in patients with autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol. 2009; 4: 1183-1189Crossref PubMed Scopus (133) Google Scholar Diffusion-weighted MRI or nuclear imaging (67-Ga or 111-In-labeled leucocyte scans) can also be useful, but false-negative and -positive results are possible with the latter techniques.20Sallee M. Rafat C. Zahar J.-R. et al.Cyst infections in patients with autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol. 2009; 4: 1183-1189Crossref PubMed Scopus (133) Google Scholar, 21Yuki K. Takeshi S. Akito T. Diagnosis and localization of infected renal cyst by diffusion-weighted magnetic resonance imaging in polycystic kidney disease.Int J Urol. 2009; 16: 918-919Crossref PubMed Scopus (7) Google Scholar Positron emission tomography scans may be helpful particularly for infected liver cysts.20Sallee M. Rafat C. Zahar J.-R. et al.Cyst infections in patients with autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol. 2009; 4: 1183-1189Crossref PubMed Scopus (133) Google Scholar, 22Bleeker-Rovers C.P. de Sevaux R.G. van Hamersvelt H.W. Corstens F.H. Oyen W.J. Diagnosis of renal and hepatic cyst infections by 18-F-fluorodeoxyglucose positron emission tomography in autosomal dominant polycystic kidney disease.Am J Kidney Dis. 2003; 41: E18-E21Abstract Full Text Full Text PDF PubMed Google Scholar, 23Soussan M. Sberro R. Wartski M. Fakhouri F. Pecking A.P. Alberini J.L. Diagnosis and localization of renal cyst infection by 18F-fluorodeoxyglucose PET/CT in polycystic kidney disease.Ann Nucl Med. 2008; 22: 529-531Crossref PubMed Scopus (25) Google Scholar Kidney stones occur in ∼20% ADPKD patients and often present with renal colic.24Torres V.E. Erickson S.B. Smith L.H. Wilson D.M. Hattery R.R. Segura J.W. The association of nephrolithiasis and autosomal dominant polycystic kidney disease.Am J Kidney Dis. 1988; 11: 318-325PubMed Scopus (100) Google Scholar, 25Torres V.E. Wilson D.M. Hattery R.R. Segura J.W. Renal stone disease in autosomal dominant polycystic kidney disease.Am J Kidney Dis. 1993; 22: 513-519PubMed Scopus (120) Google Scholar, 26Grampsas S.A. Chandhoke P.S. Fan J. et al.Anatomic and metabolic risk factors for nephrolithiasis in patients with autosomal dominant polycystic kidney disease.Am J Kidney Dis. 2000; 36: 53-57Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar Stones are usually made of calcium oxalate or uric acid. Urinary stasis secondary to the distorted renal anatomy might also play a part and metabolic factors include decreased ammonia excretion, low urinary pH, and low urinary citrate concentration. Stones can be difficult to differentiate from cyst wall and parenchymal tissue calcification. Patients with larger kidneys are more prone to develop stones.19Nishiura J.L. Neves R.F. Eloi S.R. Cintra S.M. Ajzen S.A. Heilberg I.P. Evaluation of nephrolithiasis in autosomal dominant polycystic kidney disease patients.Clin J Am Soc Nephrol. 2009; 4: 838-844Crossref PubMed Scopus (60) Google Scholar To rule out obstruction (eg, because of large cysts near the pelvis or calculus), CT imaging with contrast (where feasible) can be most informative.18Gupta S. Seith A. Sud K. et al.CT in the evaluation of complicated autosomal dominant polycystic kidney disease.Acta Radiol. 2000; 41: 280-284Crossref PubMed Scopus (39) Google Scholar Early consultation with urology or interventional radiology for management with relief of obstruction should be considered. Renal cell carcinoma is a rare cause of pain in ADPKD. It occurs no more commonly in ADPKD patients than in the general population. However, it may present at an earlier age with frequent constitutional symptoms and a higher proportion of sarcomatoid, bilateral, multicentric, and metastatic tumors.27Keith D.S. Torres V.E. King B.F. Zincki H. Farrow G.M. Renal cell carcinoma in autosomal dominant polycystic kidney disease.J Am Soc Nephrol. 1994; 4: 1661-1669PubMed Google Scholar The presence of a solid mass on ultrasonography, speckled calcifications on CT, contrast enhancement, tumor thrombus, or regional lymphadenopathy should raise concern for carcinoma. The definition of chronic pain is daily pain lasting more than 4 to 6 weeks. Some authors prefer three months to be the dividing line between acute and chronic pain. Pain related directly to a specific cyst tends to be a steady nagging discomfort, with the standing position and walking exacerbating the discomfort.1Bajwa Z.H. Gupta S. Warfield C.A. Steinman T. Pain management in polycystic kidney disease.Kidney Int. 2001; 60: 1631-1644Crossref PubMed Scopus (105) Google Scholar Patients can often localize the pain with 1 finger, and the location tends to be most frequently identified as the anterior abdominal area more so than localized back pain. The area pinpointed may not readily correlate with the largest-sized cysts seen on imaging. Often, pain begins with an acute episode and persists as chronic kidney pain after the eliciting cause has been treated and resolved, suggesting sensitization. On other occasions, it develops gradually and becomes severe over several years. There is little relation between the appearance of the kidneys and the severity of chronic pain: patients with moderate or even mild cystic disease may still have disabling pain. This condition is very frustrating to patients and physicians because extensive laboratory testing of the serum and urine and exhaustive imaging studies fail to provide evidence for associated findings to explain the pain. Careful assessment is needed to identify non-renal pain and evaluation by a physical medicine physician can be helpful to exclude musculoskeletal causes. Sometimes a large kidney cyst compressing the greater curvature of the stomach and /or duodenal loop can lead to early satiety. Hepatic cysts have been detected by MRI in 94% of patients between the ages of 35 and 46 although liver function remains preserved.28Bae K.T. Zhu F. Chapman A.B. et al.Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease Cohort.Clin J Am Soc Nephrol. 2006; 1: 64-69Crossref PubMed Scopus (209) Google Scholar Most patients have no symptoms from these. Patients with marked hepatomegaly may experience a sensation of heaviness, dull ache, mechanical low back, and gastrointestinal symptoms (Figure 3, Figure 4).29Vauthey J.N. Maddern G.J. Blumgart L.H. Adult polycystic disease of the liver.Br J Surg. 1991; 78: 524-527Crossref PubMed Scopus (78) Google Scholar Episodes of cyst hemorrhage, rupture, torsion, and infection may be associated with acute episodes of pain. Rarely, patients may develop symptoms from compression of vessels (hepatic venous outflow obstruction, compression of the inferior vena cava or portal vein) or bile ducts.10Torres V.E. Harris P.C. Pirson Y. Autosomal dominant polycystic kidney disease.Lancet. 2007; 369: 1287-1301Abstract Full Text Full Text PDF PubMed Scopus (945) Google Scholar, 27Keith D.S. Torres V.E. King B.F. Zincki H. Farrow G.M. Renal cell carcinoma in autosomal dominant polycystic kidney disease.J Am Soc Nephrol. 1994; 4: 1661-1669PubMed Google ScholarFigure 4The CT scan shows location of larger right upper lobe liver cyst in a 39-year-old female patient with autosomal dominant polycystic liver disease. Images are shown (before and after) 1,100 mL of fluid was aspirated from the cyst without apparent complication under CT guidance; 150 mL of saline and contrast were then reinjected into the cyst (second image). Imaging of the entire liver was then performed that showed no evidence of extravasation from the cyst or connection to the biliary tree. This material was then removed from the cyst, and 20 mL of alcohol were used to sclerose the liver cyst over a 15-minute period. Alcohol was then removed from the cyst. The patient tolerated the procedure well.View Large Image Figure ViewerDownload Hi-res image Download (PPT) It is extremely unusual to have pain because of pancreatic disease. When it occurs, it is caused by pancreatic duct obstruction causing pancreatitis. Pancreatic cysts can be detected by ultrasound in 5% to 9% of patients30Nicolau C. Torra R. Bianchi L. et al.Abdominal sonographic study of autosomal dominant polycystic kidney disease.J Clin Ultrasound. 2000; 28: 277-282Crossref PubMed Scopus (40) Google Scholar, 31Torra R. Nicolau C. Badenas C. et al.Ultrasonographic study of pancreatic cysts in autosomal dominant polycystic kidney disease.Clin Nephrol. 1997; 47: 19-22PubMed Google Scholar although it may be closer to 19% by MRI.10Torres V.E. Harris P.C. Pirson Y. Autosomal dominant polycystic kidney disease.Lancet. 2007; 369: 1287-1301Abstract Full Text Full Text PDF PubMed Scopus (945) Google Scholar, 32NiMhaille B.A. Norby S.M. Rosetti S. et al.Pancreatic cysts and intraductal papillary mucinous neoplasms in ADPKD.J Am Soc Nephrol. 2008; 19: 126AGoogle Scholar, 33Malka D. Hammel P. Vilgrain V. Flejou J.F. Belghiti J. Bernades P. Chronic obstructive pancreatitis due to a pancreatic cyst in a patient with autosomal dominant polycystic kidney disease.Gut. 1998; 42: 131-134Crossref PubMed Scopus (20) Google Scholar An intraductal papillary mucinous neoplasm has been reported in patients with ADPKD.34Sato Y. Mukai M. Sasaki M. et al.Intraductal papillary-mucinous neoplasm of the pancreas associated with polycystic liver and kidney disease.Pathol Int. 2009; 59: 201-204Crossref PubMed Scopus (16) Google Scholar In the majority of patients, episodes of pain associated with cyst hemorrhage and gross hematuria are transient and resolve spontaneously. Patients should be advised to avoid aspirin and physical activities, and symptoms may necessitate time off work, use of acetaminophen or propoxyphene (Darvocet, Aaipharma, Wilmington, NC, USA, Table 1) in scheduled doses until the pain subsides, and staying well hydrated. Hypertension should be strictly controlled. If the pain is severe or if gross hematuria is persistent or recurrent, more thorough evaluation including CT scans or MRI is indicated. Hospitalization may be necessary for pain control or for close observation when the hemorrhage is severe, extends into the retroperitoneal space, or causes urinary tract obstruction by clots. Rarely, interventions to stop the hemorrhage (embolization or surgery) may be necessary.Table 1Starting doses of selected short and long acting opioids for chronic non-cancer painaCR = controlled-release; ER = extented release; IR = immediate-release; IV = intravenous; TD = transdermal; TM = transmucosal.Oral administrationDrugDose (mg)bDoses are given in milligrams unless otherwise indicated.Frequency (h)Duration of effect (h)Plasma half-life (h)Codeine15-603-64-63Fentanyl100-200 μg6cNot more than 4 doses per day.0.5-1 (IV), 72 (TD), 2-4 (TM)3.7Hydrocodone2.5-10.03-64-82.5-4.0Hydromorphone2-43-44-52-3Levorphanol2-46-86-812-16Methadone5-106-84-624Morphine15-30 (IR)3-4 (IR)3-62.0-3.5Oxycodone10 (CR), 5-10 (IR)12 (CR), 3-6 (IR)8-12 (CR), 3-4 (IR)2.5-3.0Oxymorphone10 (IR), 5-10 (ER)4-6 (IR), 12 (ER)3-67.0-9.5Propoxyphone65-10044-66-12Tramadol50-100 (IR), 100 (ER)4-6 (IR), 24 (ER)4-6 (IR), 24 (ER)5-7Data from references 1Bajwa Z.H. Gupta S. Warfield C.A. Steinman T. Pain management in polycystic kidney disease.Kidney Int. 2001; 60: 1631-1644Crossref PubMed Scopus (105) Google Scholar,32NiMhaille B.A. Norby S.M. Rosetti S. et al.Pancreatic cysts and intraductal papillary mucinous neoplasms in ADPKD.J Am Soc Nephrol. 2008; 19: 126AGoogle Scholar, and 34Sato Y. Mukai M. Sasaki M. et al.Intraductal papillary-mucinous neoplasm of the pancreas associated with polycystic liver and kidney disease.Pathol Int. 2009; 59: 201-204Crossref PubMed Scopus (16) Google Scholar.Copyright permission Argoff, CE et al. Mayo Clin Proc, 84: 602-12, 2009.a CR = controlled-release; ER = extented release; IR = immediate-release; IV = intravenous; TD = transdermal; TM = transmucosal.b Doses are given in milligrams unless otherwise indicated.c Not more than 4 doses per day. Open table in a new tab Data from references 1Bajwa Z.H. Gupta S. Warfield C.A. Steinman T. Pain management in polycystic kidney disease.Kidney Int. 2001; 60: 1631-1644Crossref PubMed Scopus (105) Google Scholar,32NiMhaille B.A. Norby S.M. Rosetti S. et al.Pancreatic cysts and intraductal papillary mucinous neoplasms in ADPKD.J Am Soc Nephrol. 2008; 19: 126AGoogle Scholar, and 34Sato Y. Mukai M. Sasaki M. et al.Intraductal papillary-mucinous neoplasm of the pancreas associated with polycystic liver and kidney disease.Pathol Int. 2009; 59: 201-204Crossref PubMed Scopus (16) Google Scholar. Copyright permission Argoff, CE et al. Mayo Clin Proc, 84: 602-12, 2009. The efficacy of antibiotic treatment and infection eradication is defined by the disappearance of fever, normalization of C-reactive protein levels, and at least 2 negative blood and/or urine cultures (these criteria also apply for hepatic cyst infection).20Sallee M. Rafat C. Zahar J.-R. et al.Cyst infections in patients with autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol. 2009; 4: 1183-1189Crossref PubMed Scopus (133) Google Scholar If unresponsive to these measures, percutaneous or surgical drainage should be considered. Extracorporeal shock wave lithotripsy can be performed safely in selected patients with ADPKD; however, the presence of residual fragments in ∼50% of patients is higher than that in patients without ADPKD.25Torres V.E. Wilson D.M. Hattery R.R. Segura J.W. Renal stone disease in autosomal dominant polycystic kidney disease.Am J Kidney Dis. 1993; 22: 513-519PubMed Scopus (120) Google Scholar, 26Grampsas S.A. Chandhoke P.S. Fan J. et al.Anatomic and metabolic risk factors for nephrolithiasis in patients with autosomal dominant polycystic kidney disease.Am J Kidney Dis. 2000; 36: 53-57Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar Studies have shown that drainage and antibiotics prove more efficacious than antibiotics alone in hepatic cyst infections (Fig 3), and another larger study confirmed that in the case of large-diameter (>5 cm) infected cysts, early drainage is necessary because antibiotics alone do not usually resolve the infection.20Sallee M. Rafat C. Zahar J.-R. et al.Cyst infections in patients with autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol. 2009; 4: 1183-1189Crossref PubMed Scopus (133) Google Scholar, 35Telenti A. Torres V.E. Gross Jr., J.B. Van Scoy R.E. Brown M.L. Hattery R.R. Hepatic cyst infection in autosomal dominant polycystic kidney disease.Mayo Clin Proc. 1990; 65: 933-942Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar There have been no formal stud
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