Unilateral renal cystic disease: a case report study

Unilateral renal cystic disease (URCD) of kidney is a non-familial, extremely rare condition, characterized by replacement of the renal parenchyma of one kidney by a cluster of multiple cysts of varying size with a normal contralateral kidney. It is morphologically indistinguishable from autosomal dominant polycystic kidney disease (ADPKD); as such, hepatic and pancreatic cysts is not seen and shows no progressive deterioration in renal function; thus, differentiating ADPKD from URCD becomes important. We report a case of URCD documented by clinical and radiological imaging. A 21 year-old female, presented with history of mild lancinating pain in the left flank for 6 years which aggravated in the past 3 days, with no history of lower urinary tract symptoms. No significant family illnesses reported. Examination showed normal vitals and ballotability present and associated tenderness on deep palpation in left lumbar region. Laboratory findings were within normal limits. Ultrasonography of abdomen and pelvis showed left hydronephrosis with multiple cysts. CECT Abdomen revealed an enlarged left kidney (∼15×16×10 cm) filled with variable sized round, well-marginated multiple cysts. Renal ultrasound was performed on patient’s parents and her siblings and ruled out cystic renal disease. Hence, authors considered the diagnosis of URCD in this patient. In conclusion, treatment and managing guidelines of URCD have not been mentioned in any of the medical literatures. There is little information regarding the progression of URCD. Hence there is a need for further understanding of pathogenesis, progression and management of these patients.

Ruptured Sinus of a Valsalva Associated with Autosomal-Dominant Polycystic Kidney Disease in a young male

Autosomal-dominant polycystic kidney disease (ADPKD) is a systemic hereditary disorder characterized by localized cellular proliferation and extracellular matrix abnormalities resulting in both renal and extra renal manifestations, with multiple structural flaws such renal cysts, cerebral and aortic aneurysms, annulo-aortic ectasia, and valvular insufficiency states. Association between Autosomal-dominant polycystic kidney disease (ADPKD) and ruptured sinus of Valsalva aneurysm (SVA) has rarely been documented before. We herein report a case of Autosomal-dominant polycystic kidney disease (ADPKD) who presented with shortness of breath, was diagnosed as a case of ruptured sinus of Valsalva aneurysm (SVA) and defect was closed successfully.

Mechanism of male reproductive failure in autosomal dominant polycystic kidney disease and outcomes of assisted reproductive technology treatment / 上海交通大学学报（医学版）

Objective: To explore the potential mechanism of male reproductive failure in autosomal dominant polycystic kidney disease (ADPKD) patients and analyze the outcomes of assisted reproductive technology treatment. Methods: Next-generation sequencing was performed for genetic diagnosis of 8 ADPKD patients, who came to International Peace Maternity & Child Health Hospital, Shanghai Jiao Tong University School of Medicine, for genetic counseling. The semen of ADPKD patients and normal males who came for pre-pregnancy consultation was collected by masturbation for sperm analysis. The ultrastructure of sperm was observed by transmission electron microscopy. Outcomes of 7 patients with ADPKD who chose preimplantation genetic testing (PGT) were compared with those of 7 patients who were dystrophin (DMD) gene mutation carriers, undergoing the PGT in the same period. Results: Eight patients with ADPKD were heterozygous for polycystin 1 (PKD1) gene. Key parameters of sperm motion including progressive motility sperm percentage, curvilinear velocity, straight-line velocity, average path velocity, amplitude of lateral head displacement were much lower than those of normal semen, showing mild to severe oligozoospermia. One ADPKD patient with severe oligoathenospermia manifested bilateral seminal vesicle cysts. Transmission electron microscopy showed that the central microtubules of the sperm flagella of ADPKD patients were absent and the surrounding double microtubules were disorganized. There was no significant difference in the number of eggs, fertilization rate, cleavage rate, effective embryo rate and excellent embryo rate between the ADPKD patients and the DMD gene mutation carriers, but the ADPKD patients were prone to early abortion. Conclusion: Male reproductive failure caused by ADPKD may be related to many factors such as abnormal structure of sperm flagella and genital cysts. Further, PKD1 mutation may play a role in embryo implantation and early development.

Autosomal Dominant Polycystic Kidney Desease Coexisting with Renal Dysplasia. First Case Described and Followed Since Prenatal Period

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

Autosomal Dominant Polycystic Kidney Desease Coexisting with Renal Dysplasia. First Case Described and Followed Since Prenatal Period

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

Esophageal Artery Pseudoaneurysm and Takayasu Arteritis in a Patient with Autosomal Dominant Polycystic Kidney Disease

A 47-year-old female previously diagnosed with ADPKD visited the hospital due to sudden pain in her upper abdomen and back. Esophagogastroduodenoscopy, contrast-enhanced abdominal computed tomography (CT), and CT angiography identified an esophageal artery pseudoaneurysm and hematoma in the esophagus. Urgent angiography and embolization were performed. After the procedure, CT angiography and positron emission tomography were performed due to differences in blood pressure between the arms. The patient was also found to have Takayasu arteritis and subsequently received outpatient follow-up care. The possible mechanisms that cause vascular abnormalities in ADPKD patients include damaged vascular integrity due to abnormal polycystin expression caused by PKD mutations and connective tissue abnormalities. Further research is needed to confirm these mechanisms, and ADPKD patients should be assessed for vascular abnormalities.

Ganoderic acid A from Ganoderma lucidumretard ADPKD renal cyst development via down-regulating Ras/MAPK signaling pathway / 中国药理学与毒理学杂志

OBJECTIVE Autosomal dominant polycystic kidney disease (ADPKD) is a common-monogenetic disease characterized by progressive development of renal cysts. Thereis still further need for effective therapy.Based on our precious study that Ganoderma triterpenes(GT),which is the major secondary metabolites of Ganoderma lucidum,is able to attenuate renal cyst development.The aim of this study was to investigate the effect of a monomer,Ganoderic acid A(GA-A)that was purified from the GT,which has been reported to exhibit antinociceptive,antioxidative,hepatoproctive and anti-cancer activities,to have a potent anti-cyst effect in ADPKD. METHODS We first evaluated the potential cytotoxicity of GA-A on MDCK cells using a CCK-8 assay.Then we used MDCK cyst model,cultivated MDCK cells in vitro to form fluid-filled cysts surrounded by monolayer cells.MDCK cells were co-incu-bated with 10 μmol·L-1FSK with or without GA-A(25 μg·mL-1)and equal concentration GT as positive control from day 0 to day 6 to investigate the inhibitory effect of GA-A on cyst formation.And to further investigate the inhibitory effect of GA-A on cyst enlargement, MDCK cysts were treated with different concentration of GA-A(6.25,25 and 100 μg·mL-1)from day 5 to day 12.Next we used an embryonic kidney cyst model, wile-type mice kidneys were taken out on embryonic day 13.5 to form renal cysts stimulated with 8-Br-cAMP to prove the renal cyst inhibition at organ level.Meanwhile,we explored the possible mechanisms underlying GA-A inhibition on renal cyst development using MDCK cells treated with 10 μmol·L-1FSK co-incubated with GA-A(25 μg·mL-1)and equal concentration GT.Several key components of Ras/MAPK pathway was evaluated by Western blot,the protein expression of H-ras,B-raf, p-ERK, Egr-1 and c-fos was evaluated. RESULTS MDCK cell viability was not affected by GA-A that were used ofincreasing concentrations up to 200 μg·mL-1. GA-A had no significant influence on cyst formation,but inhibited cyst enlargement dose-dependently and the inhibitory effect is significantly better than that of the same concentration of GT which proved that GA-A may be an effective monomer from GT.And after washing out GA-A on day 8,MDCK cysts regrew to a large size,suggesting that the inhibitory effect of GA-A on MDCK cyst enlargement was reversible. GA-A inhibited embryonic kidney cyst development significantly in a dose-dependent and reversible manner proving GA-A cyst inhibition at organ level,which is also more effective than equal concentration GT.Treatment of MDCK cells with FSK caused a significant elevation of H-ras,B-raf,p-ERK,Egr-1 and c-fos signaling molecules,while treatment with GA-A reduced the level of H-ras,B-raf,p-ERK,Egr-1 and c-fos expression.GA-A down-regulated Ras/MAPK signaling pathway could contribute to its inhibitory effect on cyst development. CONCLUSION Ganoderic acid A from Ganoderma lucidum retard ADPKD renal cyst development via down-regulating Ras/MAPK signaling pathway.

Intraductal Papillary Mucinous Neoplasm (IPMN) in association with Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Intraductal Papillary Mucinous Neoplasm (IPMN) of the pancreas in association with Autosomal Dominant Polycystic Kidney Disease (ADPKD) is extremely rare, even though 10% of ADPKD patients may develop simple pancreatic cyst. The first case report was published by Yasunori Sato from Japan in 2009. Since then less than 10 case reports are available worldwide to describe about this condition. We reported such a rare case of a 67-year-old man with ADPKD who was referred to our centre because of chronic abdominal pain and diagnosed as IPMN based on the serial imaging procedures. Despite of the high risk comorbidities, he successfully underwent pylorus preserving total pancreaticoduodenectomy with splenectomy.

Influence of angiotensin converting enzyme (ACE) gene rs4362 polymorphism on the progression of kidney failure in patients with autosomal dominant polycystic kidney disease (ADPKD).

Background & objectives: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited systemic disorder, characterized by the fluid filled cysts in the kidneys leading to end stage renal failure in later years of life. Hypertension is one of the major factors independently contributing to the chronic kidney disease (CKD) progression. The renin-angiotensin aldosterone system (RAAS) genes have been extensively studied as hypertension candidate genes. The aim of the present study was to investigate the role of angiotensin converting enzyme tagging - single nucleotide polymorphisms (ACE tag-SNPs) in progression of CKD in patients with ADPKD. Methods: In the present study six ACE tagSNPs (angiotensin converting enzyme tag single nucleotide polymorphisms) and insertion/deletion (I/D) in 102 ADPKD patients and 106 control subjects were investigated. The tagSNPs were genotyped using FRET-based KASPar method and ACE ID by polymerase chain reaction (PCR) and electrophoresis. Genotypes and haplotypes were compared between ADPKD patients and controls. Univariate and multivariate logistic regression analyses were performed to assess the effect of genotypes and hypertension on CKD advancement. Mantel-Haenszel (M-H) stratified analysis was performed to study the relationship between different CKD stages and hypertension and their interaction. Results: All loci were polymorphic and except rs4293 SNP the remaining loci followed Hardy-Weinberg equilibrium. Distribution of ACE genotypes and haplotypes in controls and ADPKD patients was not significant. A significant linkage disequilibrium (LD) was observed between SNPs forming two LD blocks. The univariate analysis revealed that the age, hypertension, family history of diabetes and ACE rs4362 contributed to the advancement of CKD. Interpretation & conclusions: The results suggest that the ACE genotypes are effect modifiers of the relationship between hypertension and CKD advancement among the ADPKD patients.

An Adult with Haemoptysis: A Rare Case of Congenital Anomaly.

A non-smoker adult male presented with haemoptysis of short duration. Chest radiograph (postero-anterior view) suggested an opaque left hemithorax. Further evaluation of lung lesion pointed towards a left lung hypoplasia with absent left pulmonary artery and a right-sided aortic arch (RAA). Both kidneys were enlarged with multiple cysts and thinning of parenchyma. This case describes a unique coexistence RAA and probable autosomal dominant polycystic kidney disease.

“ADPKD presenting as congestive cardiac failure secondary to dilated cardiomyopathy” - a case report.

ADPKD presenting as congestive cardiac failure due to dilated cardiomyopathy is rare. ADPKD is an inherited systemic disorder with major renal manifestation and in some cases extrarenal manifestation or combination of both. In this report 45 year male patient presented with complains of dyspnoea, abdominal distension, pain right hypochondriac region. He was hospitalized, examined clinically and advised various bio-chemical and imaging tests. The finding was suggestive of ADPKD with dilated cardiomyopathy with congestive cardiac failure. He was managed with diuretics, ACE inhibitors, digoxin, Moist oxygen inhalation and he responded to the treatment.

Autosomal Dominant Polycystic Kidney Disease with an Asymptomatic Type I Choledochal Cyst / 대한내과학회지

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disease. Polycystic liver disease is commonly observed in ADPKD but rarely has it been associated with a choledochal cyst. We report a case of a choledochal cyst with ADPKD in a 60-year-old female patient with ADPKD and concomitant chronic renal failure who was admitted because of acute deterioration of kidney function due to poor oral intake. There was no history of a biliary disorder and her kidney function recovered promptly after fluid replacement. Non-enhanced computed tomography was performed to evaluate ADPKD and revealed numerous cysts in both kidneys and liver. It also showed fusiform dilatation of the extrahepatic bile ducts, a finding indicative of a choledochal cyst. Liver function was within the normal range and there was no evidence of extrahepatic biliary obstruction. Magnetic resonance cholangiopancreatography confirmed the diagnosis of a type I choledochal cyst combined with ADPKD.

Caffeine intake by patients with autosomal dominant polycystic kidney disease

Because caffeine may induce cyst and kidney enlargement in autosomal dominant polycystic kidney disease (ADPKD), we evaluated caffeine intake and renal volume using renal ultrasound in ADPKD patients. Caffeine intake was estimated by the average of 24-h dietary recalls obtained on 3 nonconsecutive days in 102 ADPKD patients (68 females, 34 males; 39 ± 12 years) and compared to that of 102 healthy volunteers (74 females, 28 males; 38 ± 14 years). The awareness of the need for caffeine restriction was assessed. Clinical and laboratory data were obtained from the medical records of the patients. Mean caffeine intake was significantly lower in ADPKD patients versus controls (86 vs 134 mg/day), and 63% of the ADPKD patients had been previously aware of caffeine restriction. Caffeine intake did not correlate with renal volume in ADPKD patients. There were no significant differences between the renal volumes of patients in the highest and lowest tertiles of caffeine consumption. Finally, age-adjusted multiple linear regression revealed that renal volume was associated with hypertension, chronic kidney disease stage 3 and the time since diagnosis, but not with caffeine intake. The present small cross-sectional study indicated a low level of caffeine consumption by ADPKD patients when compared to healthy volunteers, which was most likely due to prior awareness of the need for caffeine restriction. Within the range of caffeine intake observed by ADPKD patients in this study (0-471 mg/day), the renal volume was not directly associated with caffeine intake.

The Role of PEC Progenitors in ADPKD Progression

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease is a pathology mainly characterized by the progressive development and enlargement of cysts in each kidneys. Such as many adult epithelial tissue, renal tubule replaces damaged or death cells through the presence of stem/progenitor cells CD133+CD24+ Obviously, in ADPKD the repair of damages is insufficient to block the disease, but renal stem cells could have a role in the pathology. In this study we investigate the localization and the involvement of cells CD133+CD24+ in ADPKD progression. METHODS AND RESULTS: Two normal kidneys and two ADPKD kidneys were examined. CD133 and CD24 expression was investigated by confocal microscopy and immunoblotting. Renal tissue and cells were analyzed. CD133 and CD24 have the same localization in ADPKD tissues and in normal kidneys: a subset of epithelial cells (PEC) of Bowman's capsule and luminal side of tubules. It is interesting that CD133+CD24+ cells are statistically more represented in ADPKD tubules (p<0.001) and in healthy glomeruli (p=0.0016). Cysts express CD133 and CD24. CONCLUSIONS: Renal epithelial progenitors demonstrate to be involved in ADPKD pathogenesis but their role will have to be clarified and possibly managed to obtain improvement, or at least stabilization, of disease.

Successful Renal Transplantation in Patients with Polycystic Kidneys after Renal Contraction by Renal Artery Embolization: Report on 2 Cases / 대한이식학회지

Autosomal polycystic kidney disease is responsible for about 10% of the cases of end stage renal disease. The increase in kidney size is usually proportional to the degree of deterioration in renal function. At the time of transplantation, these nonfunctional kidneys can be massively enlarged and nephrectomy is required before renal transplantation. However, pretransplantation nephrectomy of polycystic kidneys has the potential risk of surgical complications, including ileus, hernias, infection, excessive bleeding and/or intestinal injury. We report here on two cases successful renal transplantation in patients with polycystic kidneys after renal contraction by renal artery embolization and without nephrectomy. The volume reduction was evaluated by CT before and 3 months after renal artery embolization and the reduction in volume was 48% and 44% in each case, respectively. The embolization was well tolerated in both cases without immediate or delayed complications except for fever and lumbar flank pain. Four months after renal artery embolization, both of the patients successfully received a transplant from living donors.

Factors determining renal function in Korean patients with autosomal dominant polycystic kidney disease / 대한내과학회지

BACKGROUND/AIMS: Previous studies of autosomal dominant polycystic kidney disease (ADPKD) in Koreans have been predominantly cross-sectional and insufficient to elucidate factors determining renal function. METHODS: We retrospectively reviewed the medical records of 60 patients who were diagnosed with ADPKD in our hospital from 1995 to 2005. We surveyed the basal characteristics, symptoms, signs, blood and urine laboratory findings, radiologic extrarenal abnormalities, and kidney length through ultrasonography, and these measures were analyzed to identify their relationship to decreased renal function. RESULTS: The clinical characteristics, such as symptoms and signs, were similar to those reported previously. Following t-tests and simple regression analyses, the statistically significant variables related to renal function deterioration were as follows: hypertension (with decreased renal function, 2.5 mL/min/1.73 m2/year more rapidly than the no-hypertension group, p=0.006), hypercholesterolemia (p=0.007), hypernatremia (p=0.011), mean kidney length (beta=0.378, p=0.029), and albumin (beta=-2.067, p=0.003). The multiple regression analysis revealed that the significant factors were hypertension (beta=0.261, p=0.016), mean kidney length (beta =0.211, p=0.047), and hypernatremia (beta=0.244, p=0.024). CONCLUSIONS: The independent risk factors for deteriorating renal function in Korean patients with ADPKD were hypertension, hypernatremia, and mean kidney length.

Mycophenolate mofetil versus Rapamycin in Han: SPRD rats with Polycystic Kidney Disease

Objective: We aimed to explore the effect of Mycophenolate mofetil (MMF) on loss of renal function and cyst progression compared to rapamycin in Han: SPRD rats. We also sought to assess whether the effect of combination therapy of MMF plus rapamycin was better than that of monotherapy. Methods: Sixty heterozygous (Cy/+) and littermate control (+/+) male Han: SPRD rats were weaned at 4 weeks of age, then divided into four groups randomly to receive different treatments by intragastric administration for 2 months: vehicle-treated group as control, MMF-treated group (20mg/kg/day), rapamycin-treated group (2mg/kg/day), and MMF+Rapa- treated group (MMF 20mg/kg/day plus Rapamycin 2mg/kg/day). Resulls: After 2 months of treatment, rapamycin caused a 22 percent decrease in body weight in comparison to the control group, whereas MMF had no significant effect on weight gain. The steady increase of BUN in Cy/+ rats was reduced by 15 percent in MMF-treated Cy/+ rats. However, rapamycin and combination therapy reduced BUN by 42 percent and 43 percent, respectively. CCr was 0.93±0.11ml/min in vehicle-treated Cy/+ rats, 1.67±0.23 ml/min in MMF-treated Cy/+ rats (P<0.05), 1.72±0.44 ml/min and 1.83±0.21 ml/min in rapamycin- and MMF+Rapa-treated Cy/+ rats, respectively (.P<0.01). Cyst volume density was 57.1 percent in vehicle-treated Cy/+ rats, 45.2 percent in MMF-treated Cy/+ rats (P<0.05), 32.9 percent and 37.7 percent in rapamycin- and MMF+Rapa-treated Cy/+ rats, respectively (P<0.01). MMF markedly ameliorated interstitial inflammation and fibrosis. Rapamycin showed a similar effect on interstitial inflammation and fibrosis, but to a lesser degree. Conclusion : MMF is more tolerable than rapamycin and can retard deterioration of renal function in Han: SPRD rats, though its effect is weaker than that of rapamycin. Combination therapy does not exert more favorable effect than monotherapy.

Multilocular Cystic Renal Cell Carcinoma Accompanied by Autosomal Dominant Polycytstic Kidney Disease / 대한신장학회잡지

No abstract available.

No Association of TGF-beta Gene Polymorphism with the Progression of Autosomal Dominant Polycystic Kidney Disease(ADPKD) in Korean Patients / 대한신장학회잡지

BACKGROUND: Two genetic loci, PKD1 and PKD2, have been identified as being responsible for ADPKD, and PKD1 is known to be associated with poor prognosis. However, the presence of intrafamilial clinical diversity suggests the presence of disease-modifying loci. Because the mechanism of renal failure in ADPKD includes cystic growth and tubulointerstitial atrophy and fibrosis, we studied the associations between two cytokine gene polymorphisms in the TGF-beta gene, which are known to be related with chronic tubulointerstitial inflammation, and ADPKD progression in Korean patients. METHODS: 47 normal controls and 114 individuals with ADPKD were genotyped by PCR-RFLP, and the TGF-beta gene leader sequence of T869C(Leu10Pro) variant was compared with MspA1I and G915C (Arg25Pro) with BglI. Statistic significances were determined using the Chi-square test. RESULTS: The distribution of alleles for the TGF-beta Leu10Pro polymorphism in ADPKD was : T 52%, C 48%, which was similar to the Korean(56 : 44, p= 0.670) and Western controls(65 : 35), and in addition, no differences were found between the CRF and the non-CRF groups(p=0.571) or the early hypertension and the normotension groups(p=0.252). The distribution of alleles for the TGF-beta Arg25Pro polymorphism was all GG type, which was different from Western controls(90 : 10, p=0.000). CONCLUSION: Our results suggest that the polymorphism at Arg25Pro of TGF-beta in Korean population has different allele distribution from Western, and the polymorphism at Leu10Pro of TGF-beta has no association with the renal progression of Korean ADPKD patients.

Cytokine Profile in the Aspirated Cystic Fluids in Autosomal Dominant Polycystic Kidney Disease (ADPKD) Patients / 대한신장학회잡지

BACKGROUND: The pathogenesis of ADPKD is still unknown but the proliferation of cystic epithelia and the fluid secretion to cystic lumen are thought to be important. Cytokines play a pivotal role in growth, differentiation, and apoptosis in general, but there were few reports about the cytokine profile in ADPKD cysts. METHODS: In this study, we measured cytokine content in aerobic culture-negative cystic fluids from 23 patients with symptomatic normal to end-stage (n=3) ADPKD in order to elucidate the possibility that cytokines are related to the development and progression of disease. Enzyme-linked immunosorbent assays (ELISAs) were used to detect IL-1beta, IL-2, IL-4, IL-6, IL-10, and IFN-gamma with commercial kits. RESULTS: Male to female ratio was 6 : 17 and the median age at examination was 52 years (range 36 to 78). IL-1beta was present in 18 of 23[78%] (11 to 173 pg/mL), IL-2 in 18 of 23[78%] (5 to 159 pg/ mL), IL-4 in 9 0f 23[39%] (8 to 156 pg/mL) and IL-6 in 10 of 23[43%] (16 to 1498 pg/mL). IL-10, and IFN-gamma were not detected. IL-1beta concentrations correlated directly with those of IL-2 (r=0.7671). IL- 6 levels in patients with azotemia (n=7) [288.4+/-26.2 (mean+/-S.D.)] were significantly higher than those of normal renal function group (98.3+/-413.9)(p<0.01). Such difference was not found in other cytokines. Cytokine concentrations did not correlate with sodium concentrations, nor with cystic fluid osmolality, indicating that differences in concentrations among fluids could not be explained by differences in water content. And, there was no significant correlation between the intracystic concentrations of these cytokines and the corresponding cyst diameters. CONCLUSION: These data identify proinflammatory cytokines as possible mediators to the morbidity of ADPKD. Especially, IL-6 levels of cystic fluid were elevated in the azotemic ADPKD patients.