Flexible ureteroscopy and holmium laser lithotripsy for treatment of upper urinary tract calculi in patients with autosomal dominant polycystic kidney disease

The objective of the study was to investigate the safety and efficacy of flexible ureteroscopy and holmium laser lithotripsy for the management of patients with autosomal dominant polycystic kidney disease (ADPKD) and associated nephrolithiasis. Between 2005 and 2010, flexible ureteroscopic stone treatment was attempted in 13 patients with ADPKD. Two patients had bilateral renal stones. Most of the stones were located in the renal pelvis and/or calices with a stone size 1.7 cm in the largest diameter. The success rate and morbidity and complications were recorded. A total of 45 intrarenal calculi with a mean stone size of 5.6 mm (range 3–17) were identified. The mean number of stones per patients was 3.2 (range 2–5). The mean number of primary procedures was 1.2 (range 1–2). The overall stone-free rates after one and two procedures were 84.5 and 92.3%, respectively. Complications occurred in three procedures and consisted of low-grade fever in one procedure, flank pain in another procedure and stent pain in another procedure. No patient died. Flexible ureteroscopy with holmium laser lithotripsy is a safe and effective method for the treatment of patients with ADPKD and associated nephrolithiasis.     

PERCUTANEOUS NEPHROLITHOTOMY IN THE PEDIATRIC POPULATION

PURPOSE: Percutaneous nephrolithotomy is an established technique used in children with renal calculi. We review our experience with percutaneous nephrolithotomy for treating nephrolithiasis in childhood. MATERIALS AND METHODS: We retrospectively reviewed the records of children who underwent percutaneous nephrolithotomy procedures for renal calculi from 1985 to 1996. Antegrade percutaneous access was obtained in all patients and the tract was dilated to 24F. Grasper forceps, ultrasound and/or electrohydraulic lithotripsy was used to remove and disintegrate stones. In all patients a nephrostomy tube was placed intraoperatively, and a plain abdominal x-ray and nephrostogram were done postoperatively. The nephrostomy tube was removed after ensuring free drainage down the ureter and no untoward effects from clamping. Complete anatomical and metabolic evaluation was performed in all cases. Patients were followed 2 to 6 weeks, and 3 and 6 months postoperatively with a plain abdominal x-ray and excretory urography or renal ultrasound. RESULTS: In 5 boys and 3 girls (9 renal units) 4 to 11 years old (mean age 6.4) a total of 10 percutaneous nephrolithotomy procedures were performed. At presentation 6 children had flank and/or abdominal pain, 5 gross hematuria and 3 urinary tract infection. Three patients had associated metabolic abnormalities. One patient with a staghorn calculus had hydronephrosis and multiple infundibular stenoses. No underlying urological anatomical abnormalities were noted in the remaining cases. Four renal units that were obstructed at presentation required initial nephrostomy tube insertion. Average operative time was 131.8 minutes (range 58 to 240). An 87.5% stone-free rate was achieved using percutaneous nephrolithotomy monotherapy. Percutaneous nephrolithotomy was not successful for eradicating a staghorn stone in 1 patient. Hypothermia developed in 2 patients in whom operative time exceeded 150 minutes. No blood transfusions were required. CONCLUSIONS: Percutaneous nephrolithotomy is safe and effective in children, and should be considered a viable management option. However, staghorn calculi may require alternative management, particularly in the setting of underlying anatomical abnormalities. Children with renal calculi should undergo a complete anatomical and metabolic assessment with the institution of medical therapy, as appropriate.     

Selective, concurrent bilateral nephrectomies at renal transplantation for autosomal dominant polycystic kidney disease

PURPOSE: An algorithm was developed for performing bilateral nephrectomies for specific indications before or at renal transplantation in patients with autosomal dominant polycystic kidney disease. Outcomes for the living donor arm of the algorithm are reported. MATERIALS AND METHODS: Patients with autosomal dominant polycystic kidney disease and end stage renal disease were evaluated for transplantation. Patients with recurrent pyelonephritis, hemorrhage, pain, early satiety or kidneys that extended into the true pelvis underwent bilateral nephrectomies. Bilateral nephrectomies with concurrent renal transplantation were performed if a living renal donor was identified. If no living donor was identified, pre-transplantation bilateral nephrectomies were done and the patients were listed for cadaveric donor renal transplantation. The living renal donor arm of the algorithm was evaluated by comparing certain parameters for 15 and 17 patients with autosomal dominant polycystic kidney disease who underwent pre-transplantation and concurrent bilateral nephrectomies, respectively, including patient and graft survival, delayed graft function, graft function, length of stay for each surgery, transfusions and complications. RESULTS: No deaths, graft failures or delayed graft function occurred. In the delayed renal transplant group median time from nephrectomy to living donor transplantation was 124 days. Serum creatinine at discharge home and 1 year after transplantation for the pre-transplantation nephrectomy cohort was 2.0 and 1.3 mg/dl, respectively. Seven of the 17 patients with concurrent nephrectomy underwent transplantation before starting renal replacement therapy. A longer mean total hospital stay in the pre-transplantation nephrectomy cohort was the only statistically significance outcome variable. CONCLUSIONS: Selective bilateral nephrectomies at living donor renal transplantation results in decreased total length of stay without compromising patient or graft outcomes and it allows preemptive renal transplantation. Concurrent nephrectomy is safe and it further validates the algorithm for selective, concurrent bilateral nephrectomies for patients with autosomal dominant polycystic kidney disease who undergo living donor renal transplantation.     

常染色体显性遗传性多囊肾病263例患者的临床特点

目的:分析常染色体显性遗传性多囊肾病(ADPKD)患者的临床特点。方法:回顾性分析2008年8月至2018年8月收治的263例成人型ADPKD患者的临床资料,包括性别、年龄、是否合并多囊肝、泌尿系结石、泌尿系感染;有无血尿、蛋白尿;有无糖尿病、高血压病、冠心病;血脂状况等。结果:高血压[60.1%(158/263)]、...     

Percutaneous nephrolithotomy for caliceal diverticular calculi: a novel single stage approach

PURPOSE: Current percutaneous treatment of symptomatic caliceal diverticular calculi involves renal access, stone removal, dilation of the diverticular communication, fulguration of the cavity and placement of a nephrostomy tube. We reviewed the outcomes of patients undergoing a novel single stage percutaneous nephrolithotomy technique for radiopaque caliceal diverticular stones that eliminates ureteral catheterization and entry into the renal collecting system. MATERIALS AND METHODS: A total of 21 patients (8 male and 13 female including 1 bilateral) with a mean age of 42.4 years underwent percutaneous nephrolithotomy for caliceal diverticular stones from February 2001 to May 2003. Of the diverticula 12 were upper pole, 4 were interpolar and 6 were lower pole. Infracostal access was established by the urologist directly onto the radiopaque stones without the aid of a ureteral catheter. After balloon tract dilation a 30Fr Amplatz sheath was placed and following stone removal the diverticulum was fulgurated. The infundibulum was neither cannulated nor dilated. A 20Fr red rubber catheter or an 8.5Fr Cope loop was placed into the diverticulum. Stone-free status was assessed by noncontrast computerized tomography on postoperative day 1 (POD1). The drainage tube was removed if there was no urine drainage and the kidney was stone-free. Excretory urography was performed at 3 months to evaluate diverticular resolution. RESULTS: Of 21 patients 20 were discharged home tubeless on POD1 and 18 of 21 (85.7%) renal units were stone- free on POD1 noncontrast computerized tomography. Mean operative time was 58.5 minutes and mean stone burden was 138.9 mm. Mean stone diameter was 11.6 mm and mean diverticular diameter was 15.3 mm. Of 22 renal units 16 had followup excretory urography. All diverticula decreased in size and 14 (87.5%) had complete resolution. CONCLUSIONS: In patients with symptomatic radiopaque caliceal diverticular stones, a single stage procedure without the need for ureteral catheterization combined with direct infracostal diverticular puncture allows for a rapid procedure with little morbidity.     

Follow-up for laparoscopic renal denervation and nephropexy for autosomal dominant polycystic kidney disease-related pain in pediatrics

PURPOSE: To present our medium-term experience with laparoscopic renal denervation and nephropexy for autosomal dominant polycystic kidney disease (ADPKD)-related pain in the pediatric patient. MATERIALS AND METHODS: Twelve patients aged 8 to 19 years (mean age 12.4 years) with ADPKD presented with chronic pain refractory to narcotic analgesics. These 12 patients underwent laparoscopic renal denervation of 16 kidneys. RESULTS: Mean operative time was 152 minutes and mean hospital stay was 2.17 days. All patients were pain-free at discharge and remain pain-free at a mean follow-up of 25.5 months. Three adolescent patients each had an episode of flank pain. One was associated with pyelonephritis, another with stones, and the third with trauma and a hematoma. CONCLUSIONS: Laparoscopic renal denervation and nephropexy is a promising option for pediatric patients with uncontrolled ADPKD-related pain.     

Atteintes cardiovasculaires associées à la polykystose rénale autosomique dominante

Autosomal dominant polycystic kidney disease is the most frequent genetic kidney disease. Cardiovascular disorders associated with autosomal dominant polycystic kidney disease are multiple and may occur early in life. In autosomal dominant polycystic kidney disease cardiovascular morbidity and mortality are related both to the nonspecific consequences of chronic kidney disease and to the particular phenotype of autosomal dominant polycystic kidney disease. Compared to the general population, patients with autosomal dominant polycystic kidney disease present an increased prevalence of hypertension, left ventricular hypertrophy, atrial fibrillation, valvular diseases, aneurisms and arterial dissections. This review article provides an update on cardiovascular disorders associated with autosomal dominant polycystic kidney disease and recent pathophysiological developments.     

Limitations of laparoscopy for bilateral nephrectomy for autosomal dominant polycystic kidney disease

PURPOSE: We retrospectively studied outcomes following bilateral hand assisted laparoscopic nephrectomy. MATERIALS AND METHODS: We retrospectively reviewed the charts of 18 patients with symptomatic autosomal dominant polycystic kidney disease who underwent bilateral hand assisted laparoscopic nephrectomy. Preoperative radiographic imaging was reviewed retrospectively to determine kidney size based on an ellipsoid shape. A visual analog pain scale with scores of 0 to 10 to assess pain related to autosomal dominant polycystic kidney disease was measured preoperatively and postoperatively. RESULTS: Average patient age was 48.2 years (range 30 to 64). Of the patients 14 successfully underwent bilateral hand assisted laparoscopic nephrectomy, while 4 required open conversion. A total of 16 patients underwent nephrectomy for pain and 2 underwent surgery for frequent recurrent symptomatic urinary tract infections. All patients except 1 underwent renal transplantation before bilateral nephrectomy. There was a significant difference in the volume of the right and left kidneys between the hand assisted laparoscopic and open groups (mean +/- SD 1,043 +/- 672 and 1,058 +/- 603.8 vs 4,052 +/- 548 and 3,592 +/- 1,752 cm(3), p <0.001 and 0.06 respectively). There were 5 complications, including wound infection and protracted ileus in 2 patients each, and incisional hernia in 1. In addition, the difference in mean preoperative and postoperative visual analog pain scores was statistically significant (6.9, range 3 to 10 and 0.5, range 0 to 2, p <0.05). CONCLUSIONS: Bilateral laparoscopic hand assisted nephrectomy is a safe and reliable option in patients requiring removal of the 2 kidneys in a single setting. Rather than performing staged nephrectomies, hand assisted laparoscopic nephrectomy allows the single administration of general anesthesia and provides effective relief of bothersome symptoms in patients with symptomatic autosomal dominant polycystic kidney disease. This procedure is safe in patients with renal transplants. Patients with massive polycystic kidneys with a kidney volume of greater than 3,500 cc are at increased risk for open conversion and they may have improved outcomes if open nephrectomy is attempted from the outset.     

Intermediate followup of hand assisted laparoscopic nephroureterectomy for urothelial carcinoma: factors associated with outcomes

PURPOSE: We report our experience with hand assisted laparoscopic (HALS) nephroureterectomy and describe the associations of preoperative, operative and pathological factors with outcome. MATERIALS AND METHODS: HALS nephroureterectomy was performed in 54 consecutive patients using modified transurethral resection of the ureteral orifice (TURUO) or a 1 port transvesical endoscopic cuff technique for the distal ureter in all except 8. Data were collected prospectively and retrospectively, and followup was distinguished for bladder, contralateral upper tract and nonurothelial (local recurrence and distant metastases) sites. RESULTS: The endoscopic cuff was associated with significantly shorter mean operative time than the transurethral resection of the ureteral orifice method (234 vs 295 minutes, p = 0.002) but the comparison was confounded by the effect of experience. With 28% of patients having stage II or greater tumors and 49% having high grade bladder disease, contralateral upper tract and nonurothelial recurrences developed in 55%, 11% and 25% of evaluable patients at a median followup of 25.1, 24.4 and 24.9 months, respectively, in those without recurrence. At a median followup of 25.0 months cancer specific survival was 94%, 86% and 80% at 1 to 3 years, respectively. Three-year cancer specific survival was 100% in patents with grade 1 or 2, or stage 0 or I tumors but only 57% and 36% in patients with grade 3 and stage II or IV tumors, respectively. CONCLUSIONS: HALS nephroureterectomy is associated with 3-year outcomes that are strongly associated with stage and grade. We prefer the endoscopic cuff method for the distal ureter because it is performed after nephrectomy, does not require patient repositioning and is expedient.     

Autosomal dominant polycystic kidney disease associated with aortic dissection: Two case reports

We describe 2 patients with autosomal dominant polycystic kidney disease associated with aortic dissection. Both patients presented with chronic renal failure arising from autosomal dominant polycystic kidney disease, as well as with aortic dissection, DeBakey type IIIb.     

Autosomal dominant polycystic kidney disease with primary hyperaldosteronism.

We report three cases of primary aldosteronism associated with autosomal dominant polycystic kidney disease. The diagnosis of primary hyperaldosteronism was based on the presence of hypokalaemia with excessive urinary potassium excretion and/or the characteristic hormonal changes. Renal function impairment due to autosomal dominant polycystic kidney disease could mask hypokalaemia. The interpretation of adrenal imagery may be hindered by adjacent renal cysts. In one case an adrenal adenoma was detected and surgically removed, with only partial correction of the blood pressure. This could be explained by the persisting underlying autosomal dominant polycystic kidney disease. We conclude that in a hypertensive patient with polycystic kidney disease, extrarenal causes of hypertension may be present.     

Predictors of Autosomal Dominant Polycystic Kidney Disease Progression

Autosomal dominant polycystic kidney disease is a genetic disorder associated with substantial variability in its natural course within and between affected families. Understanding predictors for rapid progression of this disease has become increasingly important with the emergence of potential new treatments. This systematic review of the literature since 1988 evaluates factors that may predict and/or effect autosomal dominant polycystic kidney disease progression. Predicting factors associated with early adverse structural and/or functional outcomes are considered. These factors include PKD1 mutation (particularly truncating mutation), men, early onset of hypertension, early and frequent gross hematuria, and among women, three or more pregnancies. Increases in total kidney volume and decreases in GFR and renal blood flow greater than expected for a given age also signify rapid disease progression. Concerning laboratory markers include overt proteinuria, macroalbuminuria, and perhaps, elevated serum copeptin levels in affected adults. These factors and others may help to identify patients with autosomal dominant polycystic kidney disease who are most likely to benefit from early intervention with novel treatments.     

Native Nephrectomy in Patients With Autosomal Dominant Polycystic Kidney Disease Evaluated for Kidney Transplantation

Native nephrectomy (NN) in patients with autosomal dominant polycystic kidney disease (ADPKD) is indicated in cases of recurrent urinary tract infections and hematuria, neoplastic degeneration, and encumbrance. Timing, indication, and surgical approach of NN depends on the symptoms or policy of the center. The aim of our study is to evaluate our experience.In our retrospective study, we included 130 patients with a diagnosis of ADPKD from 530 patients evaluated for renal transplantation from 2011 to 2017. We analyzed the etiologic indication, the timing, and the complications of NN.In our cohort, 53 patients underwent open NN, 85% pre-kidney transplantation (KT), 13% post-KT, and only 1 case simultaneous with KT. In the pre-KT group, indications included: major indication was encumbrance in the. In the post-KT group, the major indication was infection followed by encumbrance, which developed after KT. Complications were: 3 cases of bleeding (1 required relaparotomy, 2 evolved into hematoma and radiological derange); 1 iatrogenic iliac artery injury, which was contextually repaired, and 5 cases of incisional hernia. At 35 ± 7.2 months follow-up, patients’ survival was 96%; 1 patient died at the induction of anesthesia and 1 patient from sepsis after double NN and removal of nonfunctional transplanted kidney.NN is not without complications and should be performed when clearly indicated. In our experience, we preferred to perform NN before KT.     

Incidence of urolithiasis in cystectomy patients after intestinal conduit or continent urinary diversion

The purpose of this study was to determine the incidence of nephrolithiasis in radical cystectomy patients treated with either intestinal conduit or continent urinary diversion. The charts from 94 patients who had undergone radical cystectomy with urinary diversion at our institution from 1988 to 1998 were reviewed retrospectively for this study. Charts and radiographs from all patients were examined for renal function and evidence or urinary tract calculi. Two groups were compared: group I patients had undergone diversion with an intestinal conduit, and group II patients had received a continent diversion (primarily involving an Indiana pouch). Conduit diversions were typically done with a freely refluxing anastomosis (Bricker), whereas continent diversions were done with a nonrefluxing ureteral-intestinal anastomosis. Group I consisted of 54 patients who had undergone ileal conduit (50) or colon conduit (4) diversion with a mean follow-up of 2.5 years (range 0.6–7.0 years). Group II consisted of 40 patients who had undergone continent diversion (33 Indiana pouches, 7 orthotopic diversions) with a mean follow-up of 3.1 years (range 0.5–10.5 years). Laboratory studies of serum blood urea nitrogen, creatinine, and CO₂ were similar between the two groups. Six patients in group I developed urolithiasis, all in the upper tract. Stones developed at a mean of 3.1 years after urinary diversion. Three patients required operative intervention, whereas the others were managed expectantly. One patient in group II had an upper tract stone at the time of presentation for his bladder cancer, but no patient developed new upper tract stones during the present study period. Two patients in group II developed pouch calculi at a mean of 5 years after diversion; both required surgical intervention. In our study the risk for upper tract urolithiasis seemed higher in the intestinal conduit group (group I), with 11% of the patients developing stones. In the continent diversion group, no patient developed upper tract stones, although two patients (5%) developed pouch stones. Refluxing urine may contribute to an increased risk for stone formation after urinary diversion, whereas pouch stasis may contribute to stone formation in the continent diversion group.     

Intracranial aneurysms and autosomal dominant polycystic kidney disease: followup study by magnetic resonance angiography

PURPOSE: Intracranial aneurysms are known to complicate autosomal dominant polycystic kidney disease. We assess the value of magnetic resonance angiography to detect intracranial aneurysms early in patients with autosomal dominant polycystic kidney disease. MATERIALS AND METHODS: We evaluated 15 patients with asymptomatic autosomal dominant polycystic kidney disease treated at our hospital between 1992 and 1998. Magnetic resonance angiography was performed at presentation and was repeated 18 to 72 months after treatment. RESULTS: On the initial magnetic resonance angiogram 3 intracranial aneurysms were detected in 3 patients. The intracranial aneurysms ranged from 4 to 8 mm. in diameter, and were in the anterior communicating artery in 1, in the vertebral artery in 1, and at the bifurcation of the internal carotid artery and ophthalmic artery in 1 case. Repeat magnetic resonance angiography 18 to 72 months after treatment revealed new intracranial aneurysms in 2 patients. In 1 case the lesion was 7 mm. in diameter, in the internal carotid artery and posterior communicating artery, and detected 69 months after the initial angiogram. In the other patient the lesion was 4 mm. in diameter, in the anterior communicating artery and detected 71 months after treatment. CONCLUSIONS: Since new intracranial aneurysms were demonstrated in patients followed for a long time periodic repeat magnetic resonance angiography is important.     

Nephron sparing surgery for central renal tumors: experience with 33 cases

PURPOSE: Nephron sparing surgery is standard treatment for small, peripherally located renal cell carcinoma. In patients with a solitary kidney, bilateral tumors or impaired renal function nephron sparing surgery provides the only option to nephrectomy and subsequent hemodialysis or transplantation. We retrospectively investigated the value of nephron sparing surgery for centrally located renal cell carcinoma. MATERIALS AND METHODS: Between 1969 and 1997, 311 renal tumor enucleations were performed at our institution. The tumor was centrally located in 33 cases. The indication for enucleation was elective in 7 cases and imperative in 26, including bilateral tumor in 16 (metachronous in 9 and synchronous in 7), chronic renal failure in 4 and solitary kidney in 6. Four patients had metastasis at enucleation. RESULTS: Convalescence was unremarkable in 28 cases. Hemorrhage occurred in 1 patient, a urinary fistula in 2 and a local abscess secondary to a urinary fistula in 1. One patient died postoperatively of heart failure. Average serum creatinine was 1.25, 1.63 and 1.33 mg./dl. preoperatively, at hospital discharge and at a mean followup of 33 months, respectively. Hemodialysis was necessary transiently during convalescence in 1 patient and permanently starting 6 years after enucleation in another. Definitive histology revealed oncocytoma in 4 cases and renal cell carcinoma in 29. Disease was stages pT1 to pT3 in 9, 18 and 2 cases, and grades 1 to 3 in 6, 18 and 5, respectively. Local recurrence developed in 2 patients. Mean followup was 5.2 years (range 0.3 to 16.7). At a mean followup of 6.2 years (range 0.7 to 16.7) 20 patients were free of disease. In addition to the patient who died postoperatively, 9 died of renal cell carcinoma at a mean of 1.6 years (range 0.3 to 5.3) and 3 died of other causes at 5, 11 and 12 years postoperatively, respectively. No patient who underwent elective enucleation died. CONCLUSIONS: Nephron sparing surgery for centrally located kidney tumors is technically feasible and associated with an acceptable complication rate. Local tumor control is excellent, and the overall prognosis depends on contralateral disease and metastasis. Benign tumors may be diagnosed and removed without loss of the kidney. By avoiding hemodialysis quality of life is improved.     

The association of nephrolithiasis and autosomal dominant polycystic kidney disease

Despite the frequency and morbidity of nephrolithiasis in autosomal dominant polycystic kidney disease (ADPKD), this association has not been subject to a detailed study. One hundred fifty-one of 751 ADPKD patients seen at the Mayo Clinic between 1976 and 1986 had nephrolithiasis. Seventy-four had passed calculi or had stones surgically removed. Stone analysis was available in 30 patients: uric acid, calcium oxalate, calcium phosphate, and struvite were present in 56.6%, 46.6%, 20%, and 10%, respectively. Calculi were observed in 71 of 79 patients with excretory urograms available for review. Faintly opaque and bull's eye stones, probably containing uric acid, were present in 12.7% and 14.1% of these patients, respectively. Precaliceal tubular ectasia was observed in 15.5%. Ninety-seven patients had preserved renal function (serum creatinine less than 1.5 mg/dL) at the initial evaluation. Six were excluded because they had other known causes of stone disease. The most common metabolic abnormality in the remaining 91 patients was hypocitric aciduria (ten of 15 patients with measurements). The urine pH in the first voided morning specimens (5.66 +/- 0.05) was significantly lower than that of an unselected control population (5.92 +/- 0.03, P less than 0.001). Hyperuricosuria, hyperoxaluria, and hypercalciuria were observed in six of 32 (18.8%), six of 31 (19.4%), and three of 39 (9.7%) patients with preserved renal function. The composition of the stones, the frequency of hypocitric aciduria, and the low urine pH (possibly related to the defect in excretion of ammonia described in ADPKD), suggest that metabolic, along with mechanical, factors are responsible for the frequent occurrence of nephrolithiasis in this disease.     

Vasopressin antagonists in polycystic kidney disease

Increased cell proliferation and fluid secretion, probably driven by alterations in intracellular calcium homeostasis and cyclic adenosine 3,5-phosphate, play an important role in the development and progression of polycystic kidney disease. Hormone receptors that affect cyclic adenosine monophosphate and are preferentially expressed in affected tissues are logical treatment targets. There is a sound rationale for considering the arginine vasopressin V2 receptor as a target. The arginine vasopressin V2 receptor antagonists OPC-31260 and tolvaptan inhibit the development of polycystic kidney disease in cpk mice and in three animal orthologs to human autosomal recessive polycystic kidney disease (PCK rat), autosomal dominant polycystic kidney disease (Pkd2/WS25 mice), and nephronophthisis (pcy mouse). PCK rats that are homozygous for an arginine vasopressin mutation and lack circulating vasopressin are markedly protected. Administration of V2 receptor agonist 1-deamino-8-D-arginine vasopressin to these animals completely recovers the cystic phenotype. Administration of 1-deamino-8-D-arginine vasopressin to PCK rats with normal arginine vasopressin aggravates the disease. Suppression of arginine vasopressin release by high water intake is protective. V2 receptor antagonists may have additional beneficial effects on hypertension and chronic kidney disease progression. A number of clinical studies in polycystic kidney disease have been performed or are currently active. The results of phase 2 and phase 2-3 clinical trials suggest that tolvaptan is safe and well tolerated in autosomal dominant polycystic kidney disease. A phase 3, placebo-controlled, double-blind study in 18- to 50-yr-old patients with autosomal dominant polycystic kidney disease and preserved renal function but relatively rapid progression, as indicated by a total kidney volume >750 ml, has been initiated and will determine whether tolvaptan is effective in slowing down the progression of this disease.     

Polycystic kidney disease at end-stage renal disease in the United States: patient characteristics and survival

BACKGROUND: The patient characteristics and mortality associated with autosomal dominant polycystic kidney disease have not been characterized for a national sample of end-stage renal disease (ESRD) patients. METHODS: 375,152 patients in the United States Renal Data System were initiated on ESRD therapy (including patients who eventually received renal transplants) between January 1, 1992 and June 30, 1997 and analyzed in an historical cohort study of polycystic kidney disease. RESULTS: Of the study population, 5,799 (1.5%) had polycystic kidney disease. In logistic regression, polycystic kidney disease was associated with Caucasian race (odds ratio 3.31, 95% CI, 3.09-3.54), women (1.10, 1.04-1.16), receipt of renal transplant (4.15, 3.87-4.45), peritoneal dialysis (vs. hemodialysis, 1.37, 1.27-1.49), younger age, and more recent year of first treatment for ESRD. Use of pre-dialysis EPO but not the level of serum hemoglobin at initiation of ESRD was significantly higher in patients with polycystic kidney disease. Patients with polycystic kidney disease had lower mortality compared to patients with other causes of ESRD, but patients with polycystic kidney disease had a higher adjusted risk of mortality associated with hemodialysis (vs. peritoneal dialysis) compared to patients with other causes of ESRD (hazard ratio 1.40, 1.13-1.75). CONCLUSIONS: Hematocrit at presentation to ESRD was not significantly different in patients with polycystic kidney disease compared with patients with other causes of ESRD. Peritoneal dialysis is a more frequent modality than hemodialysis in patients with polycystic kidney disease, and patients with polycystic kidney disease had an adjusted survival benefit associated with peritoneal dialysis, compared to patients with other causes of renal disease.     

Autosomal dominant polycystic kidney disease with liver and pancreatic involvement in early childhood

In a patient with autosomal dominant polycystic kidney disease, liver and kidney cysts were found by biopsy at the age of 8 months. Computed tomography at the age of 16 years confirmed the presence of liver and kidney cysts and also revealed pancreatic cysts. Such early onset of liver cysts in a patient with autosomal dominant polycystic kidney disease has not been reported previously.