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Identifying genetic mutations in individuals with inherited cystic kidney disease is necessary for precise treatment. We aimed to elucidate the genetic characteristics of cystic kidney disease in the Korean population. Methods: We conducted a 3-year prospective, multicenter cohort study at eight hospitals from May 2019 to May 2022. Patients with more than three renal cysts were enrolled and classified into two categories, typical autosomal dominant polycystic kidney disease (ADPKD) and atypical PKD. We identified the clinical characteristics and performed a genetic analysis using a targeted gene panel. Results: A total of 725 adult patients were included in the study, of which 560 (77.2%) were diagnosed with typical ADPKD and 165 (22.8%) had atypical PKD. Among the typical ADPKD cases, the Mayo imaging classification was as follows: 1A (55, 9.9%), 1B (149, 26.6%), 1C (198, 35.8%), 1D (90, 16.3%), and 1E (61, 11.0%). The atypical PKD cases were classified as bilateral cystic with bilateral atrophic (31, 37.3%), lopsided (27, 32.5%), unilateral (nine, 10.8%), segmental (eight, 9.6%), bilateral cystic with unilateral atrophic (seven, 8.4%), and asymmetric (one, 1.2%). Pathogenic variants were found in 64.3% of the patients using the ciliopathy-related targeted gene panel. The typical ADPKD group demonstrated a higher discovery rate (62.3%) than the atypical PKD group (41.8%). Conclusion: We present a nationwide genetic cohort’s baseline clinical and genetic characteristics for Korean cystic kidney disease.
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It is important for the dialysis specialist to provide essential and safe care to hemodialysis (HD) patients. However, little is known about the actual effect of dialysis specialist care on the survival of HD patients. We therefore investigated the influence of dialysis specialist care on patient mortality in a nationwide Korean dialysis cohort. Methods: We used an HD quality assessment and National Health Insurance Service claims data from October to December 2015. A total of 34,408 patients were divided into two groups according to the proportion of dialysis specialists in their HD unit, as follows: 0%, no dialysis specialist care group, and ≥50%, dialysis specialist care group. We analyzed the mortality risk of these groups using the Cox proportional hazards model after matching propensity scores. Results: After propensity score matching, 18,344 patients were enrolled. The ratio of patients from the groups with and without dialysis specialist care was 86.7% to 13.3%. The dialysis specialist care group showed a shorter dialysis vintage, higher levels of hemoglobin, higher single-pool Kt/V values, lower levels of phosphorus, and lower systolic and diastolic blood pressures than the no dialysis specialist care group. After adjusting demographic and clinical parameters, the absence of dialysis specialist care was a significant independent risk factor for all-cause mortality (hazard ratio, 1.10; 95% confidence interval, 1.03–1.18; p = 0.004). Conclusion: Dialysis specialist care is an important determinant of overall patient survival among HD patients. Appropriate care given by dialysis specialists may improve clinical outcomes of patients undergoing HD.
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Tolvaptan reduces height-adjusted total kidney volume (htTKV) and renal function decline in autosomal dominant polycystic kidney disease (ADPKD). This study was aimed at investigating the efficacy and safety of tolvaptan in Korean patients with ADPKD during the titration period. Methods: This study is a multicenter, single-arm, open-label phase 4 study. We enrolled 108 patients with ADPKD (age, 19–50 years) with an estimated glomerular filtration rate (eGFR) of >30 mL/min/1.73 m2 and factors defined as indicative of rapid disease progression. After tolvaptan titration, we evaluated efficacy and side effects and assessed factors associated with the effects. Results: After titration for 4 weeks, eGFR and htTKV decreased by 6.4 ± 7.9 mL/min/1.73 m2 and 16 ± 45 mL/m, respectively. No serious adverse drug reactions were observed during the titration period. The greatest eGFR decline was observed in the first week, with a starting tolvaptan dose of 45 mg. Multivariate linear regression for htTKV decline showed that the greater the change in urine osmolality (Uosm), the greater the decrease in htTKV (β, 0.436; p = 0.009) in the 1D group stratified by the Mayo Clinic image classification. Higher baseline eGFR was related to a higher htTKV reduction rate in the 1E group (β, –0.642; p = 0.009). Conclusion: We observed short-term effects and safety during the tolvaptan titration period. The decline of htTKV can be predicted as a short-term effect of tolvaptan by observing Uosm changes from baseline to end of titration in 1D and baseline eGFR in 1E groups.
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Background@#Factors related to the development and severity of polycystic liver disease (PLD) have not been well established. We aimed to evaluate the genetic and epidemiologic risk factors of PLD in patients with autosomal dominant polycystic kidney disease (ADPKD). @*Methods@#Adult patients with inherited cystic kidney disease were enrolled from May 2019 to May 2021. Demographic, clinical, and laboratory data were collected at the initial study visit. The severity of PLD was graded based on the height-adjusted total liver volume: 1,800 mL/m (Gr3). Targeted exome sequencing was done by a gene panel including 89 ciliopathy-related genes. We searched out the relative factors to the presence and the severity of PLD using logistic regression analysis. @*Results@#Of 602 patients with typical ADPKD, 461 (76.6%) patients had PLD. The patients with PLD showed female predominance and a higher frequency of other ADPKD-related complications. The genetic variants with truncating mutation of PKD1 (PKD1-proteintruncating [PT]) or PKD2 commonly affected the development and severity of PLD. An older age, female sex, and higher kidney volume with Mayo classification 1C-1E was significantly associated with the development of PLD, but not with the severity of PLD. On the other hand, higher body mass index, lower hemoglobin, and higher alkaline phosphatase (ALP) were the significant risk factors of severe PLD (≥ Gr2). @*Conclusion@#Hepatic involvement in ADPKD could be related to kidney manifestations and genetic variants including PKD1-PT or PKD2. Monitoring hemoglobin and ALP and evaluating the genetic variants might help predict severe PLD.
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Many countries have their own hemodialysis (HD) quality assurance programs and star rating systems for HD facilities. However, the effects of HD quality assurance programs on patient mortality are not well understood. Therefore, in the present study, the effects of the Korean HD facility star rating on patient mortality in maintenance HD patients were evaluated. Methods: This longitudinal, observational cohort study included 35,271 patients receiving HD treatment from 741 facilities. The fivestar ratings of HD facilities were determined based on HD quality assessment data from 2015, which includes 12 quality measures in structural, procedural, and outcome domains. The patients were grouped into high (three to five stars) and low (one or two stars) groups based on HD facility star rating. Cox proportional hazards model was used to evaluate the effects of star rating on patient mortality during the mean follow-up duration of 3 years. Results: The patient ratio between high and low HD facility star rating groups was 82.0% vs. 18.0%. The patients in the low star rating group showed lower single-pool Kt/V and higher calcium and phosphorus levels compared with subjects in the high star rating group. After adjusting for sociodemographic and clinical parameters, the HD facility star rating independently increased the mortality risk (hazard ratio, 1.11; 95% confidence interval, 1.04–1.18; p = 0.002). Conclusion: The HD facilities with low star rating showed higher patient mortality.
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Plastic cannulae have attracted increasing interest as an alternative to traditional metal needles with the aim of reducing cannulation-related complications. We investigated whether the substitution volumes during hemodiafiltration differ using these two types of needles in dialysis patients. Methods: An intervention study involving 26 hemodialysis patients was conducted in Korea between March and September in 2021. Patients first received online hemodiafiltration using traditional metal needles, and thereafter plastic cannulae were used in a stepwise protocol. Repeated-measures design and linear mixed-effect models were used to compare substitution volumes between the two needle types with the same inner diameter. Results: The mean patient age was 62.7 years, and their mean dialysis vintage was 95.2 months. Most patients (92.3%) had an arteriovenous fistula as the vascular access. The substitution volume increased as blood flow and needle size increased for both plastic cannulae and metal needles. The substitution volume was significantly higher with 17-gauge (G) plastic cannulae than with 16-G metal needles at blood flow rates of 280, 300, and 330 mL/min. Similar results were obtained for 15-G metal needles and 16-G plastic cannulae at a blood flow rate of 330 mL/min. However, the patient ratings of pain on a visual analogue scale were higher for plastic cannulae. Conclusion: Higher substitution volumes were obtained at the same prescribed blood flow rate with plastic cannulae than with metal needles during online hemodiafiltration. Plastic cannulae are an option for achieving high-volume hemodiafiltration for patients with low blood flow rates.
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Patients with end-stage kidney disease face increased risk of cardiovascular events, and left ventricular diastolic dysfunction (LVDD) contributes to the high occurrence of cardiovascular mortality (CM). Although a high serum aldosterone (sALD) level is involved in the development of cardiovascular complications in the general population, this association is unclear in patients undergoing hemodialysis. We aimed to determine the impact of sALD on LVDD and CM among hemodialysis patients (HDPs). Methods: We performed a prospective cohort study of maintenance HDPs without cardiovascular disease. The patients were divided into two groups according to the median level of sALD. All patients underwent baseline echocardiography to evaluate diastolic dysfunction (E/e’ ratio > 15). The LVDD and CM rates were compared between the high and low aldosterone groups. Results: We enrolled a total of 60 adult patients (mean age, 57.9 ± 12.1 years; males, 30.0%). The low aldosterone group had an increased left ventricular diastolic dimension compared with the high aldosterone group (52.2 ± 8.4 mm vs. 50.3 ± 5.2 mm, respectively; p = 0.03). Low log-aldosterone (odds ratio [OR], 0.40; 95% confidence interval [CI], 0.19–0.86) and large left atrial dimension (OR, 1.31; 95% CI, 1.11–1.54) were independent risk factors for LVDD at baseline. In addition, Cox regression analysis demonstrated that low sALD was an independent predictor of CM in HDPs (hazard ratio, 0.46; 95% CI, 0.25–0.85; p = 0.01) during follow-up. Conclusion: Low sALD was not only associated with LVDD but was also an independent predictor of CM among HDPs regardless of their interdialytic weight gain.
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Methods@#This is a post hoc analysis of a prospective, controlled, randomized, unblinded study with 78 Korean hemodialysis patients receiving intravenous (n = 40) or subcutaneous (n = 38) erythropoietin therapy. We evaluated hemoglobin variability by calculating the frequency of hemoglobin measurements outside the target range during all visits. The high-frequency group was defined by those with hemoglobin variability over the median value (25%) while the low-frequency group was defined by those with hemoglobin variability of <25%. @*Results@#In this analysis, 37 patients (51.1%) were men, and the mean age was 50.6 ± 12.5 years. Twenty-five patients (35.2%) had diabetes mellitus. The frequency of the value being outside the target hemoglobin range was higher in the subcutaneous group compared to the intravenous group (0.36 ± 0.19 vs. 0.27 ± 0.12/visit, p = 0.03). The low-frequency group required significantly lower erythropoietin doses compared to the high-frequency group. In the adjusted Cox analysis, the parameter high-frequency group was a significant independent risk factor for cardiovascular events (hazard ratio, 3.53; 95% confidence interval, 1.15–10.83; p = 0.03). @*Conclusion@#The risk of missing the target hemoglobin range increased with subcutaneous administration compared with intravenous erythropoietin administration in hemodialysis patients. An increased frequency of the value being outside the target hemoglobin range was also associated with an increased risk of cardiovascular events.
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Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by cyst growth in the kidneys, resulting in kidney enlargement and end-stage kidney disease. The polycystic kidney disease 1 (PKD1) and PKD2 have been identified as genes related to ADPKD and their significance in the molecular pathology of the disease has been studied. A disease-modifying drug has been approved; therefore, it has become important to identify patients at a high risk of kidney disease progression. Genetic tests, image analysis methods, and clinical factors for kidney disease progression prediction have been established. This review describes genetic and clinical characteristics, and discusses ongoing studies in Korean ADPKD patients.
ABSTRACT
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by cyst growth in the kidneys, resulting in kidney enlargement and end-stage kidney disease. The polycystic kidney disease 1 (PKD1) and PKD2 have been identified as genes related to ADPKD and their significance in the molecular pathology of the disease has been studied. A disease-modifying drug has been approved; therefore, it has become important to identify patients at a high risk of kidney disease progression. Genetic tests, image analysis methods, and clinical factors for kidney disease progression prediction have been established. This review describes genetic and clinical characteristics, and discusses ongoing studies in Korean ADPKD patients.
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Coronavirus disease 2019 (COVID-19) is a highly contagious viral disease that is caused by the novel virus Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2). COVID-19 has become pandemic since December 2019, when the first case developed in Wuhan, China. Patients receiving hemodialysis are more vulnerable to viral transmission because their immune functions are impaired and they receive treatment within a narrow space. Calling on previous experience with Middle East Respiratory Syndrome during the 2015 outbreak, the joint committee of the Korean Society of Nephrology and the Korean Society of Dialysis Therapy quickly formed a COVID-19 task force team to develop a manual before the first index case was diagnosed in the hemodialysis unit. This special article introduces clinical practice guidelines to prevent secondary transmission of COVID-19 within hemodialysis facilities, which were developed to protect patients, healthcare workers, and caregivers from this highly transmissible virus. The areas of infection control covered by these guidelines include standard precautions, performing dialysis therapy for confirmed or suspected cases, performing cohort isolation for contact patients, and disease monitoring and contact surveillance. We hope these guidelines help healthcare workers and hemodialysis patients around the world cope with the COVID-19 pandemic.
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Background@#Intrarenal renin-angiotensin system (RAS) is known to play the major role in the development of hypertension and renal progression in autosomal dominant polycystic kidney disease (ADPKD). Urinary angiotensinogen to creatinine ratio (AGT/Cr) was suggested as a novel biomarker to reflect intrarenal RAS activity. This study was performed to evaluate urinary AGT/Cr as a predictive biomarker for renal function decline in addition to imaging classification in a prospective ADPKD cohort. @*Methods@#From 2011 to 2016, a total of 364 ADPKD patients were enrolled in the prospective cohort called the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD). Among them, a total of 207 subjects in chronic kidney disease stage 1–4 with baseline urinary AGT and total kidney volume and subsequent renal function follow-up data over more than 1 year were included in the analysis. Patients were defined as slow progressors (SP) if they are classified as 1A or 1B by imaging classification whereas rapid progressors (RP) if they are classified as 1C–1E. Patients were divided according to AGT/Cr quartiles and annual estimated glomerular filtration rate (eGFR) slope was compared among highest quartile (hAGT group) and the rest of quartiles (lAGT group). Patients were divided into 4 groups to evaluate the predictive value of urinary AGT/Cr in addition to imaging classification: SP/lAGT, SP/hAGT, RP/lAGT, and RP/hAGT. The Cox regression model was used to evaluate the hazard ratio (HR) between groups. @*Results@#The mean age was 45.9 years and 88.9% had hypertension. Baseline eGFR was 79.0 ± 28.4 mL/min/1.73 m2 and median height-adjusted total kidney volume was 788.2 (471.2;1,205.2) mL/m. The patients in the hAGT group showed lower eGFR (72.4 ± 24.8 vs. 81.1 ± 29.2 mL/min/1.73 m2, P = 0.039), lower plasma hemoglobin (13.0 ± 1.4 vs. 13.7 ± 1.6 g/dL, P = 0.007), higher urinary protein to creatinine ratio (0.14 [0.09, 0.38] vs. 0.07 [0.04, 0.12] g/g, P = 0.007) compared to the lAGT group. The hAGT group was an independent risk factor for faster eGFR decline after adjusting for gender, RP, baseline eGFR, and other known risk factors. During median follow-up duration of 4.6 years, a total of 29 renal events (14.0%) occurred. The SP/hAGT group showed significantly higher risk of developing renal outcome compared to SP/lAGT group (HR, 13.4; 95% confidence interval, 1.282–139.324; P = 0.03). @*Conclusion@#Urinary AGT/Cr can be a useful predictive marker in the patients with relatively small ADPKD. Various biomarkers should be considered to define RP when implementing novel treatment in the patients with ADPKD.
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BACKGROUND: Elevated serum alkaline phosphatase (AP) and γ-glutamyl transferase (γ-GT) are commonly observed in patients with acute pyelonephritis. The goal of this study was to examine the clinical significance of elevated serum AP and γ-GT levels and to explore the mechanisms underlying these changes. METHODS: We examined serum AP and γ-GT levels in 438 patients with acute pyelonephritis. Urine AP/creatinine (Cr), urine γ-GT/Cr, fractional excretion of AP, and fractional excretion of γ-GT (FE(γ-GT)) were evaluated in patients with elevated and normal serum levels. AP isoenzymes were also examined. RESULTS: We identified 77 patients (17.6%) with elevated serum AP and 134 patients (30.6%) with elevated serum γ-GT. Among them, both enzymes were elevated in 64 patients (14.6%). Older age, longer hospital stay, elevated baseline serum Cr, and complicated pyelonephritis were associated with increases in serum AP and γ-GT. Multivariate analysis showed that high serum AP levels were significantly correlated with renal impairment (odds ratio, 2.13; 95% confidence interval, 1.08–4.19; P = 0.029). FE(γ-GT) was significantly lower in patients with elevated serum enzyme levels. The liver fraction for AP isoenzyme profile did not increase in patients with elevated serum AP. CONCLUSION: Our results demonstrated that elevated serum AP and γ-GT levels are associated with complicated pyelonephritis and renal impairment. Lower FE(γ-GT) levels in patients with elevated serum enzymes may be the result of decreased urinary excretion of these enzymes.
Subject(s)
Humans , Alkaline Phosphatase , gamma-Glutamyltransferase , Isoenzymes , Length of Stay , Liver , Multivariate Analysis , Pyelonephritis , TransferasesABSTRACT
Patients receiving hemodialysis are vulnerable to infectious diseases due to their impaired immunity and high risk of exposure to pathogens. To protect patients, staff, and visitors from potential infections, each hemodialysis unit should establish and follow standard infection control and prevention measures. Therefore, clinical practice guidelines were developed by a working group of nephrologists and infection control specialists to provide evidence-based guidance for dialysis physicians and nurses, with the aim of preventing infection transmission and controlling infection sources in hemodialysis facilities. The areas of infection control covered by these guidelines include standard precautions, isolation strategies, vascular access, water treatment, cleaning/disinfecting/sterilizing, and vaccination. This special report summarizes the key recommendations from the Korean clinical practice guidelines for preventing the transmission of infections in hemodialysis facilities.
Subject(s)
Humans , Communicable Diseases , Dialysis , Disease Transmission, Infectious , Infection Control , Renal Dialysis , Specialization , Vaccination , Water PurificationABSTRACT
The Korean Society of Nephrology participated in the task force team consisting of government authorities and civilian experts to prevent and control the spread of Middle East respiratory syndrome (MERS) in 2015. The Korean Society of Nephrology MERS Task Force Team took an immediate action and drafted ‘the clinical recommendation for hemodialysis facilities’ to follow when the first and the only confirmed case was reported in the hemodialysis unit. Owing to the dedicated support from medical doctors, dialysis nurses, and related medical companies, we could prevent further transmission of MERS infection successfully in hemodialysis units. This special report describes the experience of infection control during MERS outbreak in 2015 and summarizes the contents of ‘the clinical practice guideline for hemodialysis facilities dealing with MERS patients’ built upon our previous experience.
Subject(s)
Advisory Committees , Coronavirus Infections , Dialysis , Infection Control , Middle East Respiratory Syndrome Coronavirus , Middle East , Nephrology , Quarantine , Renal DialysisABSTRACT
No abstract available.
Subject(s)
Humans , Carcinoma, Renal Cell , Polycystic Kidney, Autosomal Dominant , Translocation, GeneticABSTRACT
BACKGROUND: For various reasons, kidney transplant recipients with autosomal dominant polycystic kidney disease (ADPKD) often undergo native nephrectomy in preparation for the transplantation. Simultaneous nephrectomy can result in hypotensive events perioperatively and affect transplant outcome adversely. Our aim was to evaluate the effect of simultaneous native nephrectomy (SNx) on perioperative blood pressure and graft outcome compared to non-nephrectomy (NNx) in renal transplant recipients with ADPKD. METHODS: Data regarding renal function and blood pressure were collected from 42 renal transplant recipients with ADPKD. The primary outcome was graft function over 1 year post-transplant. The secondary outcomes were patient and graft survival, postoperative hypotensive events, and blood pressure control. We compared units of anti-hypertensive medication used by transplanted ADPKD patients in the SNx and NNx groups. RESULTS: Patients with SNx during kidney transplantation showed similar rates of patient and graft survival and renal function. Although they had significantly more hypotensive events during the perioperative period (69.2% vs. 37.5% in NNx, P=0.045), no harmful influence on renal function was observed. No difference in mean blood pressure during the 1-year post-transplant period was observed between the two groups; however, the SNx group required fewer units of anti-hypertensive medication. CONCLUSIONS: SNx is a relatively safe procedure. Graft outcome in the SNx group was not inferior to that of the NNx group, and patients with SNx can have well-controlled blood pressure.
Subject(s)
Humans , Blood Pressure , Graft Survival , Kidney , Kidney Transplantation , Nephrectomy , Perioperative Period , Polycystic Kidney, Autosomal Dominant , Transplantation , TransplantsABSTRACT
A 62-yr-old woman with an autosomal dominant polycystic kidney disease (ADPKD) was admitted to our hospital for further evaluation of intermittent fever, nausea and left flank discomfort. The computed tomography (CT) scan revealed a gas-forming, infectious cyst of approximately 8.1 cm in size in left kidney lower pole. Escherichia coli was identified from the cyst fluid culture examination. Her symptoms improved only after the concomitant use of intravenous ciprofloxacin and an intracystic irrigation of ciprofloxacin through a percutaneous cystostomy drainage. Our case presents the successfully treated emphysematous cyst infection with combination of intravenous antibiotics and intracystic antibiotic therapy instead of surgical management.
Subject(s)
Female , Humans , Middle Aged , Anti-Bacterial Agents/therapeutic use , Ciprofloxacin/therapeutic use , Cystostomy , Cysts/microbiology , Escherichia coli Infections/complications , Injections, Intravenous , Polycystic Kidney, Autosomal Dominant/complications , Therapeutic Irrigation , Tomography, X-Ray ComputedABSTRACT
No abstract available.
Subject(s)
Adult , Humans , Male , Antiphospholipid Syndrome/complications , Catastrophic Illness , Coronary Angiography , Fibrinolytic Agents/therapeutic use , Multiple Organ Failure/etiology , Recurrence , Thrombosis/diagnosisABSTRACT
We retrospectively evaluated demographic and biochemical parameters associated with depression and health-related quality of life (HRQOL) in maintenance peritoneal dialysis (PD) patients. This study included 105 patients maintaining PD at Seoul National University Hospital. Data were collected from electronic medical record. Korean Beck's Depression Inventory and Korean version of Kidney Disease Quality of Life short form, version 1.3 were used to evaluate depression and HRQOL, respectively. Moderate to severe depression was found in 24.8% of patients. Patients with lower normalized protein equivalent of nitrogen appearance (nPNA) (< 1.2 g/kg/day), lower weekly renal Kt/V(urea) (< 0.2), and lower serum albumin level (< or = 4.0 g/dL) were associated with depression (P < 0.05). Among them, lower weekly renal Kt/V(urea) was the only independent risk factor associated with depression (OR = 3.1, P = 0.007). Depressed patients showed significantly lower scores in every dimension of HRQOL (P < 0.001). Lower weekly renal Kt/V(urea) (beta = 0.24, P = 0.005) and lower nPNA (beta = 0.15, P = 0.03) were the independent risk factors associated with lower kidney dialysis component summary, whereas lower plasma hemoglobin level was the consistent risk factor for lower physical component summary (beta = 0.22, P = 0.03) and mental component summary (beta = 0.22, P = 0.01). Depression is a prevalent psychological problem in PD population. Residual renal function is the most important factor associated with depression and impaired HRQOL in PD patients.