A case of proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) that responded favorably to steroid therapy.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) generally has a poor prognosis and the consensus is that it needs to be treated with clone-directed therapy. However, the prognosis of PGNMID is heterogenous and some cases have been successfully treated using other therapeutic strategies. We herein report a case of PGNMID that responded favorably to steroids without clone-directed therapy. An 18-year-old woman was referred to a nephrologist with proteinuria detected in an annual health check-up. Over a 3-year period, the concentration of creatinine (Cr) increased from 0.76 to 1.00 mg/dL and proteinuria from 0.35 to 1.9 g/g Cr. Monoclonal gammopathies were not detected in her serum or urine. Based on the findings of kidney biopsy at the age of 21 years, the patient was diagnosed with proliferative glomerulonephritis with monoclonal IgG1-kappa deposits. The histological feature was mesangial proliferative glomerulonephritis with advanced glomerulosclerosis, which is a rare presentation of PGNMID. Intravenous methylprednisolone pulse therapy was initiated, followed by oral prednisolone at a dose of 30 mg daily. One year later, a second kidney biopsy revealed a significant decrease in mesangial deposits of IgG1-kappa. Prednisolone was gradually tapered and discontinued 2 years after the first kidney biopsy. At the time of prednisolone withdrawal, urinalysis showed proteinuria of 0.2 g/g Cr without hematuria. Kidney function remained stable throughout the treatment period.

Need for evidence on long-term prognosis of PD+HD: a commentary.

Combination therapy with peritoneal dialysis and hemodialysis (PD+HD) is an alternative dialysis method for patients with end-stage kidney disease (ESKD). The complementary use of once-weekly HD expedites to achieve adequate dialysis and enables to prolong PD duration. Although PD+HD has been widely employed among Japanese PD patients, it is much less common outside Japan. Clinical evidences are still not enough, especially in long-term prognosis and appropriate treatment duration, suitable patients, and generalizability. A retrospective cohort study by Chung et al. (BMC Nephrol 21:348, 2020) compared the risk of mortality and hospitalization between PD patients who were transferred to PD+HD and those who were transferred to HD in Taiwan. Because the mortality and hospitalization rates did not differ between the groups, the authors concluded that, PD+HD may be a rational and cost-effective treatment option. It should be noted that the effects of PD+HD on long-term prognosis are still unknown due to too-short PD+HD duration. However, the study identified the high-risk patient population and showed the generalizability of PD+HD. PD+HD is a treatment of choice in patients with ESKD who prefer PD lifestyles even after decline in residual kidney function.

Ultrafiltration volume by once-weekly hemodialysis is a predictor of technique survival of combination therapy with peritoneal dialysis and hemodialysis.

Overhydration is a major cause of technique failure of peritoneal dialysis (PD). Hence, we investigated the impact of ultrafiltration (UF) volume by once-weekly hemodialysis (HD), excess volume beyond their dry weight, on technique survival of PD and HD combination therapy (PD+HD). Forty-six anuric PD+HD patients were divided into three groups according to baseline UF volume by HD: low-UF (

Hospitalization for Patients on Combination Therapy With Peritoneal Dialysis and Hemodialysis Compared With Hemodialysis.

INTRODUCTION: Combination therapy with peritoneal dialysis and hemodialysis (PD+HD) is widely used for PD patients with decreased residual kidney function in Japan; however, hospitalization for this combined dialysis has not been investigated so far. We compared the risk of hospitalization for PD+HD with that for HD. METHODS: A multicenter, prospective observational study was conducted on 42 PD+HD and 42 HD patients matched for age and diabetic nephropathy. The main outcome measure was the cumulative incidence of hospitalization for any cause assessed with the Kaplan-Meier method. Hospitalization rates (the number of admissions per 100 patient-years) associated with dialysis modality were also calculated. The impact of dialysis modality on time to hospitalization was analyzed using the Cox proportional hazard model. RESULTS: There was no significant difference between groups in terms of age, sex, dialysis vintage, diabetic nephropathy, and comorbidities. The cumulative incidence of hospitalization did not significantly differ between the groups (log-rank test, P = 0.36). Although total hospitalization rates were 66.0 in PD+HD and 59.2 in HD, hospitalization rates for the sum of PD-related infections (a composite of catheter-related infection and peritonitis) and vascular access troubles were 21.7 in PD+HD and 7.2 in HD. On univariate Cox proportional hazard analysis, dialysis modality had no significant impact on time to hospitalization. CONCLUSION: The risk of hospitalization was not significantly different between PD+HD and HD, although PD+HD patients had a higher risk of dialysis access-related complications than HD patients.

Health-related quality of life on combination therapy with peritoneal dialysis and hemodialysis in comparison with hemodialysis and peritoneal dialysis: A cross-sectional study.

BACKGROUND: The health-related quality of life (HRQOL) of dialysis patients has not been well examined, especially in combination therapy with peritoneal dialysis and hemodialysis (PD+HD) patients. We compared the HRQOL of PD+HD patients with that of HD and PD patients. METHODS: A multicenter, cross-sectional study was conducted on 36 PD+HD, 103 HD, and 90 PD patients in Japan who completed the Kidney Disease Quality of Life Short Form 36, version 1.3. HRQOL scores were summarized into physical- (PCS), mental- (MCS), role/social- (RCS), and kidney disease component summaries (KDCS). RESULTS: Of the PD+HD patients, 31 (86%) transferred from PD and 5 (14%) transferred from HD. They had the longest dialysis vintage and the smallest urine volume. PCS, MCS, and KDCS HRQOL scores of PD+HD patients were comparable with those of HD and PD patients. However, the RCS score for PD+HD was significantly higher than that for HD (p = 0.020) and comparable with that for PD. PD+HD and PD were associated with significantly higher RCS scores than HD after adjusting for age, gender, diabetic nephropathy, dialysis vintage, ischemic heart disease, and peripheral arterial disease. CONCLUSIONS: For RCS, HRQOL in PD+HD patients was better than that in HD and comparable with that in PD patients, whereas the PCS, MCS, and KDCS HRQOL scores of PD+HD patients were comparable with those of HD and PD patients.

Bioimpedance Spectroscopy-Based Fluid Status in Combined Dialysis Compared With Hemodialysis and Peritoneal Dialysis: A Cross-Sectional Study.

Combination therapy with peritoneal dialysis and hemodialysis (PD+HD) is widely used in Japan for PD patients with decreased residual renal function. However, fluid status in PD+HD patients has not been well studied. In this cross-sectional study, we compared fluid status in 41 PD+HD patients with that in 103 HD and 92 PD patients using the bioimpedance spectroscopy. Extracellular water normalized to patient height (NECW, kg/m) was the highest in pre-HD (8.3 ± 1.6) followed by PD (7.9 ± 2.7), PD+HD (7.5 ± 2.5), and post-HD patients (6.9 ± 1.5) (P < 0.01). By multiple linear regression analysis, PD+HD was associated with a significantly lower NECW than pre-HD (ß = -0.8, P = 0.03) and similar to PD (ß = -0.5, P = 0.24) and post-HD (ß = 0.6, P = 0.08) after adjustment for age, sex, diabetic nephropathy, ischemic heart disease, dialysis period, and daily urine volume. There was no correlation between NECW and daily urine volume in all dialysis groups. Average daily fluid removal (a sum of urine volume and ultrafiltration volume by dialysis) was positively correlated with NECW in PD+HD and pre-HD, but not in PD and post-HD patients. Our results suggest that fluid status in PD+HD patients with decreased residual renal function is acceptable as compared with that in HD and PD patients.

Incidence and risk factors of contrast-induced nephropathy after transcatheter arterial chemoembolization in hepatocellular carcinoma.

BACKGROUND: Transcatheter arterial chemoembolization (TACE) is widely used for unresectable hepatocellular carcinoma (HCC). The purpose of this study was to investigate incidence and risk factors of contrast-induced nephropathy (CIN) after TACE in patients with HCC. METHODS: In this single-center retrospective study, we examined 461 consecutive TACE sessions in 260 patients between January 2003 and October 2015. CIN was defined as an increase in serum creatinine levels by ≥ 0.5 mg/dl or ≥ 25% from baseline within 72 h after TACE. We calculated incidence rate of CIN and tried to identify its risk factors by logistic regression analysis. RESULTS: Twenty-one cases of CIN (5%) were observed in 461 TACE sessions. One patient required subsequent hemodialysis transiently. In univariate analysis, tumor size > 5 cm [odds ratio (OR) 5.76, 95% confidence interval (CI) 2.34-14.14, p < 0.001], chronic kidney disease (OR 2.54, 95% CI 1.05-6.14, p = 0.04), serum hemoglobin level [OR 0.79 (per 1 g/dl increase), 95% CI 0.64-0.98, p = 0.03] and serum albumin level [OR 0.44 (per 1 g/dl increase), 95% CI 0.19-1.02, p = 0.05] were associated with the development of CIN. Stepwise logistic regression methods showed that tumor size > 5 cm (OR 7.81, 95% CI 2.99-20.46, p < 0.001) and serum albumin [OR 0.29 (per 1 g/dl increase), 95% CI 0.11-0.75, p = 0.01] were risk factors of CIN. CONCLUSIONS: In this study, HCC tumor size and lower serum albumin level were independent predictors of CIN after TACE.

Eicosapentaenoic Acid and Prevalence of Cardiovascular Disease in Hemodialysis Patients.

Eicosapentaenoic acid (EPA)/arachidonic acid (AA) ratio showed inverse associations with cardiovascular disease (CVD) in general population. However, this has not been examined enough in dialysis patients. We cross-sectionally investigated the relationship between EPA/AA ratio and prevalence of CVD in 321 chronic hemodialysis patients (64 ± 11 years old; 110 women; dialysis vintage 10 ± 8 years) in an urban area of Tokyo. CVD was defined as a composite of ischemic heart disease, ischemic stroke and hemorrhagic stroke. The frequency of dietary fish intake was also examined. Logistic regression was used to quantify the association of EPA/AA ratio with CVD. EPA/AA ratio was 0.31 ± 0.19 and 154 patients (48%) consumed fish once or less weekly. One hundred and thirty patients (41%) had CVD, including 65 with ischemic heart disease, 70 with ischemic stroke, and 20 with hemorrhagic stroke. Age (odds ratio [OR], 1.04; P = 0.01), hypertension (OR, 2.25; P = 0.002), and dialysis vintage (OR, 1.04; P = 0.02) were associated with CVD; however, EPA/AA was not after adjustment for other risk factors. A similar relationship was observed between fish intake and CVD prevalence. We did not find any significant association between EPA/AA ratio and prevalence of CVD, although traditional risk factors such as age, hypertension and dialysis vintage were associated with CVD. These results might have been influenced by the fact that only a small proportion of our patients showed a high EPA/AA ratio.

Warfarin-related nephropathy in a patient with renal pelvic cancer.

An 83-year-old Japanese man had a history of chronic heart failure due to bradycardia-tachycardia syndrome. He was admitted to our hospital because of macrohematuria and acute kidney injury (AKI), which were detected by an urologist at an outpatient visit. He had a history of recurrent macrohematuria and transurethral resection of bladder tumors twice in the preceding 2 years. He had been on warfarin for 12 years, with a stable international normalized ratio (INR) that was usually less than 2.1. Urinalysis revealed numerous red blood cells (RBCs) and mild proteinuria without RBC casts. His serum creatinine level was elevated to 2.41 mg/dL from 0.96 mg/dL at 3 weeks before admission. INR was 1.44. Hydronephrosis was not observed. Ureteroscopy detected invasive urothelial carcinoma of the renal pelvis, and right laparoscopic nephroureterectomy was performed at 41 days after diagnosis of AKI. The background renal parenchyma displayed tubular obstruction by red blood cell casts and acute tubular injury, which were changes compatible with warfarin-related nephropathy (WRN). Warfarin was discontinued, and the serum creatinine level recovered to 1.66 mg/dL after 3 months. In the present patient with nephrosclerosis, WRN occurred at a therapeutic INR level after 12 years of uneventful warfarin therapy, and the coexisting urothelial malignancy was a unique feature.

Age-Related Differences of Organism-Specific Peritonitis Rates: A Single-Center Experience.

Peritonitis remains an important cause of morbidity and mortality in peritoneal dialysis (PD) patients, but its incidence and the distribution of causative organisms vary widely between institutions and age groups. This study was performed to investigate the recent status and risk factors of PD-related peritonitis and to clarify differences between age groups. We retrospectively reviewed the medical records of 119 PD patients treated at our department between January 2002 and January 2013. We calculated both overall and organism-specific peritonitis rates and also analyzed risk factors. Sixty-three episodes of peritonitis occurred during 261.5 patient-years for an incident rate of 0.24 episodes/patient-year. Multivariate analysis showed that older age (≥65 years) and hypoalbuminemia (<3.0 g/dL) were associated with an increased risk of peritonitis (P = 0.035 and P = 0.029, respectively). In elderly patients (≥65 years old), the rate of peritonitis due to Gram-positive and Gram-negative bacteria was 0.17 and 0.08 episodes/patient-year, respectively, and Gram-positive peritonitis was markedly more frequent than in younger patients (<65 years old). In particular, there was a high frequency of Staphylococcus aureus peritonitis in elderly patients (0.09 episodes/patient-year) and it had a poor outcome. At our department, the risk of peritonitis was increased in older patients and patients with hypoalbuminemia. The distribution of causative organisms was markedly different between age groups and analysis of organism-specific peritonitis rates helped to identify current problems with our PD program.

Dialysis Amyloid Deposition in the Aortic Valve and Its Association with Aortic Stenosis.

BACKGROUND: The relationship between dialysis amyloid (DA) deposition in the aortic valve (AV) and aortic stenosis (AS) is unknown. METHODS: This was a cross-sectional study. AV specimens of dialysis patients (median vintage: 8.8 years) consecutively collected from cardiac surgeries (n = 56) or autopsies (n = 13) were examined by a board-certified pathologist blinded to clinical data. DAs were considered to be present if deposits were stained both by Congo red with apple-green birefringence under polarized light and by anti-ß2-microblobulin antibody. Degree of deposition was graded as follows: Amyloid (-), no deposit; Amyloid (1+), occasional small deposits; Amyloid (2+), multiple small to large deposits or a single large deposit. Calcification was defined as a calcified deposit with a diameter >1 mm in the specimen. Severe AS (sAS) was defined as a mean gradient >50 mm Hg by echocardiogram. We examined the proportion of DAs and the association between DAs and the sAS. RESULTS: DAs were present in 71% (n = 49) of specimens and primarily co-localized with calcification. Non-dialysis related amyloid was found in one specimen. After excluding this specimen, sAS was associated with 'Amyloid (1+) and Calcification >1 mm' and 'Amyloid (2+) and Calcification >1 mm' (vs. 'Amyloid (-) and Calcification ≤1 mm', odds ratios (ORs): 13.5 and 34.2, respectively). Furthermore, after adjustment for covariates, sAS was found to be associated with 'Amyloid (2+) and Calcification >1 mm' (OR: 24.3). CONCLUSIONS: DA deposition in the AV was prevalent among dialysis patients. DA deposition with accompanying calcification might contribute to the severity of AS.

AA amyloid nephropathy with predominant vascular deposition in Crohn's disease.

A 44-year-old man with a 17-year history of Crohn's disease (CD) was referred to our nephrology department on suspicion of drug-induced nephrotoxicity. Over the preceding 18 months, he had slowly progressive renal insufficiency with slight urinary abnormalities. His disease activity had been well controlled up to that point with 5-aminosalicylic acid and azathiopurine. Laboratory examination revealed slight proteinuria without hematuria and an elevated serum creatinine level of 1.4 mg/dl. Pathological examination revealed amyloid A (AA) deposition in the kidney, predominantly in the arterial and arteriolar walls with little to none in the glomerular capillaries. AA amyloidosis is typically accompanied by glomerular amyloid deposition and massive proteinuria. In the present case, however, vascular amyloid deposition was predominant, and the renal function was deteriorated with slight urinary abnormalities. The present case confirmed the importance of conducting a definitive pathological diagnosis of renal insufficiency in CD patients.

AP-VAS 2012 case report: two patients with rheumatoid arthritis suspected of relapsed microscopic polyangiitis after initiation of dialysis.

We report two patients with rheumatoid arthritis (RA) who were suspected of microscopic polyangiitis during maintenance dialysis. Case 1 was a 52-year-old woman with RA diagnosed at the age of 38 years and treated successfully with gold compounds. At the age of 43 years, she presented with progressive renal dysfunction and abnormal urine sediments, and a renal biopsy revealed crescentic nephritis with advanced glomerular sclerosis. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was not measured on that occasion. She reached end-stage renal failure within 4 months and started peritoneal dialysis. Eight years later, soon after she was switched to hemodialysis, she developed fever of unknown origin. MPO-ANCA was elevated to 37 EU, although there were no other signs or symptoms suggestive of vasculitis. After taking prednisolone orally (10 mg/day), her fever withdrew, and MPO-ANCA became undetectable. Case 2 was a 71-year-old woman with RA diagnosed at the age of 60 years and treated with gold compounds. She developed renal failure of unknown cause (no biopsy was performed), and started hemodialysis at the age of 69 years. One year later, she presented with fever and subsequently developed cough with hemoptysis. MPO-ANCA was elevated to 62 EU. Treatment with azathioprine 50 mg and prednisolone 35 mg daily brought remarkable clinical improvement, and MPO-ANCA became undetectable. These cases highlight the importance of measuring ANCA even in RA patients on dialysis who present with fever of unknown origin or with underlying kidney disease of uncertain etiology.

Acute systemic hypotension after arteriovenous fistula construction in a patient with severe aortic stenosis.

We report the case of a 53-year-old hemodialysis patient with severe aortic stenosis, who developed acute systemic hypoperfusion after arteriovenous fistula (AVF) construction. He presented with hypotension and repeated syncope soon after distal radiocephalic AVF construction, and finally developed a respiratory arrest. His blood pressure and hemodynamics recovered promptly by sub-emergent aortic valve replacement surgery. In the present case, the heart with severe aortic stenosis could not increase cardiac output in response to the reduction in peripheral vascular resistance caused by the AVF. High-output heart failure, a relatively rare AVF-associated disorder, occurs with an excessive AVF flow, usually more than 3 L/min or 30% of cardiac output. However, heart failure may develop soon after construction of an AVF with a moderate blood flow if a patient's cardiac function is severely impaired. In addition, heart failure may improve with AVF preservation if the underlying heart disease is treatable.

[Case of mesangial proliferative glomerulonephritis complicated with multicentric Castleman's disease].

We report a case of a 47-year-old man with multicentric Castleman's disease (MCD) and progressive renal dysfunction due to mesangial proliferative glomerulonephritis, possibly from IgA nephropathy. At age 36 years, he was referred to a hematologist due to hypergammaglobulinemia. Because of systemic lymph node swelling, he underwent right cervical lymph node biopsy at age 41 years and MCD (plasma cell type)was diagnosed. During this period, microscopic hematuria and persistent proteinuria occurred and his renal function deteriorated (serum creatinine (Cr) rising from 0.7 mg/dL to 1.4 mg/dL). Treatment with intravenous methylprednisolone at the dose of 1 g daily for 3 days followed by oral prednisolone at 20 mg daily reduced his lymphadenopathy and improved the renal function. However, his renal function deteriorated again, from Cr 0.8 mg/dL to 1.8 mg/dL over 6 years in line with gradual prednisolone tapering to 6 mg daily. At age 47 years, he was referred to our nephrology department and underwent a renal biopsy. The microscopic examination showed IgA nephropathy with crescent formation, accompanied by mild lymphoplasmacytic tubulointerstitial nephritis. Treatment with the same dose of intravenous methylprednisolone therapy followed by oral prednisolone at 40 mg daily, improved his proteinuria, hematuria and renal dysfunction. The coexistence of MCD and IgA nephropathy is a rare phenomenon. In addition, IL-6, overproduced by MCD might have influenced the mesangial cell proliferation and the activity of IgA nephropathy in the present case.