Executive summary of the Korean Society of Nephrology 2021 clinical practice guideline for optimal hemodialysis treatment

The Korean Society of Nephrology (KSN) has published a clinical practice guideline (CPG) document for maintenance hemodialysis (HD). The document, 2021 Clinical Practice Guideline on Optimal HD Treatment, is based on an extensive evidence-oriented review of the benefits of preparation, initiation, and maintenance therapy for HD, with the participation of representative experts from the KSN under the methodologists’ support for guideline development. It was intended to help clinicians participating in HD treatment make safer and more effective clinical decisions by providing user-friendly guidelines. We hope that this CPG will be meaningful as a recommendation in practice, but not on a regulatory rule basis, as different approaches and treatments may be used by health care providers depending on the individual patient’s condition. This CPG consists of eight sections and 15 key questions. Each begins with statements that are graded by the strength of recommendations and quality of the evidence. Each statement is followed by a summary of the evidence supporting the recommendations. There are also a link to full-text documents and lists of the most important reports so that the readers can read further (most of this is available online).

Executive Summary of the Korean Society of Nephrology 2021 Clinical Practice Guideline for Optimal Hemodialysis Treatment

The Korean Society of Nephrology (KSN) has published a clinical practice guideline (CPG) document for maintenance hemodialysis (HD). The document, 2021 Clinical Practice Guideline on Optimal HD Treatment, is based on an extensive evidence-oriented review of the benefits of preparation, initiation, and maintenance therapy for HD, with the participation of representative experts from the KSN under the methodologists’ support for guideline development. It was intended to help clinicians participating in HD treatment make safer and more effective clinical decisions by providing user-friendly guidelines. We hope that this CPG will be meaningful as a recommendation in practice, but not on a regulatory rule basis, as different approaches and treatments may be used by health care providers depending on the individual patient’s condition. This CPG consists of eight sections and 15 key questions. Each begins with statements that are graded by the strength of recommendations and quality of the evidence. Each statement is followed by a summary of the evidence supporting the recommendations. There is also a link to full-text documents and lists of the most important reports so that the readers can read further (most of this is available online).

Urinary Angiotensinogen in addition to Imaging Classification in the Prediction of Renal Outcome in Autosomal Dominant Polycystic Kidney Disease

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.

Effect of Simultaneous Nephrectomy on Perioperative Blood Pressure and Graft Outcome in Renal Transplant Recipients with Autosomal Dominant Polycystic Kidney Disease / 대한이식학회지

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.

Recent Advances in the Management of Autosomal Dominant Polycystic Kidney Disease / 대한내과학회지

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by the dysregulated growth of kidney cysts, resulting in end-stage kidney failure. By identifying the genes involved in ADPKD and detailing the molecular pathology of the disease, putative therapeutic agents have been developed. However, clinical trials of vasopressin receptor antagonists and somatostatin analogues have raised several concerns among researchers and clinicians. Questions regarding when and who to treat and what surrogate marker to use for describing endpoints have been raised. This review focuses on the current methods for managing ADPKD and describes recent findings from clinical trials. The main difficulties associated with implementing therapeutic agents in patients with ADPKD and considerations for clinical settings will also be discussed.

Successful Pregnancy in a Patient with Autosomal Dominant Polycystic Kidney Disease on Long-Term Hemodialysis

Recent advances in dialysis and a multidisciplinary approach to pregnant patients with advanced chronic kidney disease provide a better outcome. A 38-yr-old female with autosomal dominant polycystic kidney disease (ADPKD) became pregnant. She was undergoing hemodialysis (HD) and her kidneys were massively enlarged, posing a risk of intrauterine fetal growth restriction. By means of intensive HD and optimal management of anemia, pregnancy was successfully maintained until vaginal delivery at 34.5 weeks of gestation. We discuss the special considerations involved in managing our patient with regard to the underlying ADPKD and its influence on pregnancy.

First Case of Continuous Ambulatory Peritoneal Dialysis-Related Peritonitis Caused by Cryptococcus arboriformis

No abstract available.

Subcutaneous sarcoidosis presenting as a suprapubic mass, acute kidney injury, and hypercalcemia

No abstract available.

Successfully Treated Escherichia coli-Induced Emphysematous Cyst Infection with Combination of Intravenous Antibiotics and Intracystic Antibiotics Irrigation in a Patient with Autosomal Dominant Polycystic Kidney Disease

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.

Outcome of Early Initiation of Peritoneal Dialysis in Patients with End-Stage Renal Failure

Recent studies reported that early initiation of hemodialysis may increase mortality. However, studies that assessed the influence of early initiation of peritoneal dialysis (PD) yielded controversial results. In the present study, we evaluated the prognosis of early initiation of PD on the various outcomes of end stage renal failure patients by using propensity-score matching methods. Incident PD patients (n = 491) who started PD at SNU Hospital were enrolled. The patients were divided into 'early starters (n = 244)' and 'late starters (n = 247)' on the basis of the estimated glomerular filtration rate (eGFR) at the start of dialysis. The calculated propensity-score was used for one-to-one matching. After propensity-score-based matching (n = 136, for each group), no significant differences were observed in terms of all-cause mortality (P = 0.17), technique failure (P = 0.62), cardiovascular event (P = 0.96) and composite event (P = 0.86) between the early and late starters. Stratification analysis in the propensity-score quartiles (n = 491) exhibited no trend toward better or poorer survival in terms of all-cause mortality. In conclusion, early commencement of PD does not reduce the mortality risk and other outcomes. Although the recent guidelines suggest that initiation of dialysis at higher eGFR, physicians should not determine the time to initiate PD therapy simply rely on the eGFR alone.

A Case of Delftia acidovorans Peritonitis in a Peritoneal Dialysis Patient Managed with Preserving the Dialysis Catheter / 대한신장학회지

Delftia acidovarans is aerobic, nonfermentative Gram-negative rod commonly found in soil and water. Generally it is nonpathogenic but it unusually can cause bacteremia in immunocompromised patients. We present a case of peritonitis due to D. acidovorans in a patient on continuous ambulatory peritoneal dialysis. A 75-year-old woman was admitted with abdominal pain and cloudy peritoneal effluent. She was empirically treated with intraperitoneal (IP) cefazolin and ceftazidime, and then IP ceftazidime and oral ciprofloxacin, but peritonitis did not improve. Seven days after admission, D. acidovorans was identified from the peritoneal effluent, which was sensitive to amikacin, ceftazidime, ciprofloxacin and imipenem. Catheter removal was considered with regard to poor response to adequate antibiotics; however, 4 days after changing to IP imipenem/cilastatin, abdominal pain, the leukocyte count of peritoneal effluent and C-reactive protein decreased. She was treated with imipenem/cilastatin for two weeks and discharged with the dialysis catheter intact.

Bleeding Complications Associated With the Use of Low Molecular Weight Heparin According to the Degree of Renal Impairment / 대한신장학회지

PURPOSE: Low molecular weight heparin (LMWH) is safe and effective in the treatment of acute coronary syndrome (ACS) and venous thromboembolism. Compared with unfractionated heparin (UFH), it is known to have less bleeding tendency in the general population. However, it is not certain whether bleeding complications are decreased by LMWH in patients with renal failure. We postulated that the use of LMWH may lead to increase in bleeding tendency in patients with renal dysfunction. METHODS: We conducted a retrospective study in 486 hospitalized patients who were diagnosed as cerebral infarction or ACS, and treated with enoxaparin or nadroparin from January 2008 to December 2009. Bleeding complications were compared in 3 groups according to estimated glomerular filtration rate (GFR> or =60, 30-59, and <30 mL/min/1.73m2). Age, hypertension (HTN), diabetes mellitus (DM), smoking and usage of antithrombotics were examined and the relationship of these variables with bleeding tendency was analyzed. RESULTS: Compared with group I, the frequency of total bleeding complications increased in patients with group II (p=0.002) and III (p=0.005) regardless of adequate dose reduction. Multiple logistic regression analysis after adjustment for age, HTN, DM, and usage of antithrombotics revealed that decreased GFR groups [odds ratio (OR) of group II was 5.79 (95% confidence interval (CI), 1.23-29.97; p=0.042), OR of group III 5.92 (95% CI, 1.22-27.61; p=0.029)] and DM [OR of DM 7.88 (95% CI; 1.46-46.32, p=0.026)] were two independent factors which affect major bleeding. CONCLUSION: These findings suggest that renal insufficiency, even if it is mild, could affect major bleeding complications in the use of LMWH.

Erratum to "Assessment of Deceased Donor Kidneys Using a Donor Scoring System" by Bang K, et al. (Yonsei Med J 2010;51:870-876)

No abstract available.

Erratum to "Assessment of Deceased Donor Kidneys Using a Donor Scoring System" by Bang K, et al. (Yonsei Med J 2010;51:870-876)

No abstract available.

Central Venous Stenosis Caused by Traction of the Innominate Vein due to a Tuberculosis-Destroyed Lung

We report a case of central venous stenosis due to a structural deformity caused by a tuberculosis-destroyed lung in a 65-year-old woman. The patient presented with left facial edema. She had a history of pulmonary tuberculosis, and the chest X-ray revealed a collapsed left lung. Angiography showed leftward deviation of the innominate vein leading to kinking and stenosis of the internal jugular vein. Stent insertion improved her facial edema.

Waldenstrom Macroglobulinemia with CD5+ Expression Presented as Cryoglobulinemic Glomerulonephropathy: A Case Report

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.

The Usefulness of Urinary Angiotensinogen as a Biomarker of Renal Progression in Autosomal Dominant Polycystic Kidney Disease / 대한신장학회지

PURPOSE: The renin-angiotensin-aldosterone system activation has been suggested as a potential risk factor for renal progression in autosomal dominant polycystic kidney disease (ADPKD). This study was performed to evaluate urinary angiotensinogen as a biomarker of renal progression in ADPKD. METHODS: Patients with estimated glomerular filtration rate (eGFR) > or =30 mL/min/1.73m2 were enrolled in the study. Specimens (blood and urine) and computed tomography (CT) were taken from each subject. The eGFR was calculated by 4-variable MDRD equation and total kidney volume (TKV) was measured from CT images by modified ellipsoid method. Urinary angiotensinogen (AGT) and neutrophil gelatinaseassociated lipocalin (NGAL) were measured by ELISA. The concentration of AGT was adjusted with random urine creatinine (Cr). The association between urinary biomarkers, TKV and eGFR were evaluated. RESULTS: A total of 59 (M:F=31:28) subjects were enrolled in the study and their mean age was 46 years. The eGFR and TKV at the enrollment were 77.3+/-15.6 mL/min/1.73m2 and 1389.8+/-925.1 mL, respectively. Log AGT/Cr was associated with TKV (r2=0.117, p=0.01) in the earlier stage of disease (TKV<3,000 mL). However, it did not show significant correlation with eGFR. Log NGAL was not associated with either TKV or eGFR. Urinary AGT/Cr was closely related to the number of anti-hypertensive medication, TKV, and the presence of albuminuria, although there was no correlation with plasma renin activity or aldosterone level. CONCLUSION: Urinary angiotensinogen may be a useful biomarker of disease progression in ADPKD patients.

An Infrarenal Aortic Hypoplasia Presented with Claudication

We describe a case of infrarenal aortic hypoplasia in a 52-yr-old woman who presented with claudication. Computed tomographic angiography revealed an abrupt absence of the infrarenal aorta, with collateral flow reconstituting the iliofemoral systems. After a polytetrafluoroethylene graft was interposed between the aortic stump and the iliac bifurcation, the patient's claudication resolved.

Rapid Extension of Renal Angiomyolipoma to Perinephric Space: A Case Report / 대한신장학회지

Angiomyolipoma is a benign neoplasm composed of fat, smooth muscles and blood vessels. We report a case of a 64-year-old man with a renal angiomyolipoma which rapidly extended to perinephric space. He had been diagnosed of having a small renal angiomyolipoma, and seven years later, a large perinephric mass was newly detected. Right nephrectomy with mass excision revealed an exophytic mass in the lower pole of right kidney which extended to perinephric space. A histological examination showed large angiomyolipomatosis. Clinicians who follow the renal angiomyolipoma should be aware of the unusual perinephric progression.

Assessment of Deceased Donor Kidneys Using a Donor Scoring System

PURPOSE: Marginal grafts should be used more actively in Asian countries where deceased donor transplantation is unpopular. We modified a quantitative donor scoring system proposed by Nyberg and his colleagues and developed a donor scoring system in order to assess the quality of deceased donor grafts and their prognostic value as an initial effort to promote usage of marginal donors. MATERIALS AND METHODS: We retrospectively evaluated 337 patients. RESULTS: A scoring system was derived from six donor variables [age, 0-25; renal function, 0-4; history of hypertension, 0-4; Human Leukocyte Antigen (HLA) mismatch, 0-3; body weight, 0-1; cause of death, 0-3 points]. Donor grafts were stratified by scores: grade A, 0-10; grade B, 11-20; grade C, 21-30; and grade D, 31-40 points. Donor grades significantly correlated with estimated glomerular filtration rate (eGFR) at 6 months (A, 64.0 mL/min/1.73 m2; B, 57.0 mL/min/1.73 m2; C, 46.8 mL/min/1.73 m2; p < 0.001). The five-year graft survival rate was also lower in grade C than grade A (74% vs. 93%, p = 0.002). Donors in grade C and D were regarded as marginal donors. The proportion of marginal donors was much lower in Korea, compared with data from the United Network for Organ Sharing (15.2% vs. 29%). CONCLUSION: Considering the scarcity of deceased donor kidneys and the relatively better graft outcome with lower grade-donors in Korea, it is worth increasing the usage of marginal grafts.