Successful treatment of nephrotic syndrome due to pregnancy-related crescentic IgA nephropathy: a case report.

BACKGROUND: Crescentic immunoglobulin A (IgA) nephropathy, defined as > 50% of the glomeruli with crescents, often has a poor renal prognosis. Because of the high prevalence of pre-eclampsia in the second trimester of pregnancy, we often fail to investigate the new onset of glomerulonephritis and the aggravation of subclinical nephropathies. We report a case of nephrotic syndrome suggestive of crescentic IgA nephropathy possibly triggered by pregnancy. CASE PRESENTATION: A 33-year-old multipara was referred for persistent proteinuria, hematuria, and hypoalbuminemia two months postpartum. The patient was diagnosed with proteinuria for the first time at 36 weeks of gestation. The patient was normotensive during pregnancy. Renal biopsy revealed crescentic IgA nephropathy, with cellular crescents in 80% of the glomeruli and no global sclerosis. After treatment with pulse steroids followed by high-dose oral glucocorticoids and tonsillectomy, a gradual improvement was seen in proteinuria, hematuria, and hypoalbuminemia. CONCLUSION: Although the precise mechanism remains unclear, pregnancy possibly triggered the new onset of crescentic IgA nephropathy or the aggravation of subclinical IgA nephropathy.

A complicated case of Serratia marcescens catheter-related bloodstream infection misdiagnosed as hypersensitivity reactions to bicarbonate dialysate containing acetate.

Recurrent fever during/post-dialysis can occur due to infectious or non-infectious causes. We present the case of a 79-year-old patient who had persistent post-dialysis fever after long-term tunneled central venous catheterization with acetate-containing bicarbonate dialysate. Drug-induced lymphocyte stimulation test (DLST) was positive for acetate dialysate, and he was suspected of having acetate dialysate-induced hypersensitivity reaction. However, switching to acetate-free dialysate did not attenuate the fever. Since Serratia marcescens had been isolated twice from the blood, catheter-related bloodstream infection (CRBSI) was suspected. The culture of the catheter tip confirmed CRBSI caused by S. marcescens. Elevation of ß-d-glucan levels and appearance of pulmonary nodular shadow on chest computed tomography images indicated complicated fungal infections. Administration of antibiotics and antifungals led to resolution of the pulmonary nodular shadow with attenuation of fever and C-reactive protein levels. DLST for acetate dialysate was negative, and its reuse did not aggravate the symptoms; hence, the first result was considered false-positive. An indwelling catheter is a risk factor for S. marcescens-related CRBSI, which leads to post-dialysis fever. Hypersensitivity reactions to dialysates must be diagnosed considering the clinical course and DLST results.

Successful Treatment of Nephrotic Syndrome Due to Collapsing Focal Segmental Glomerulosclerosis Accompanied by Acute Interstitial Nephritis.

A 39-year-old woman was hospitalized for nephrotic syndrome. Laboratory test results showed increased serum creatinine levels and urinary excretions of beta-2-microglobulin, and N-acetyl-beta-D-glucosaminidase. A renal biopsy revealed collapsing focal segmental glomerulosclerosis (FSGS) and acute interstitial nephritis. Despite treatment with pulse steroid followed by oral high-dose glucocorticoids and cyclosporines, heavy proteinuria persisted. After low-density lipoprotein apheresis (LDL-A) therapy was initiated, her proteinuria gradually decreased, leading to complete remission. A repeat renal biopsy after treatment revealed no collapsing glomeruli. Immediate LDL-A should be performed to treat cases of collapsing FSGS poorly responding to other treatments.

Risk factors for peritoneal dialysis withdrawal due to peritoneal dialysis-related peritonitis.

BACKGROUND: Peritoneal dialysis has become commonly used for renal replacement therapy; however, some patients withdraw from peritoneal dialysis due to complications, including peritoneal dialysis-related peritonitis, resulting in the low number of patients on peritoneal dialysis. Risk factors for peritoneal dialysis withdrawal due to peritoneal dialysis-related peritonitis are less certain. This retrospective study aimed to investigate these risk factors. METHODS: We retrospectively analyzed clinical characteristics, laboratory data, and causative microorganisms of 204 episodes of peritoneal dialysis-related peritonitis between 2007 and 2018 at our institution. RESULTS: Of the 204 episodes, 38 resulted in withdrawal from peritoneal dialysis due to peritoneal dialysis-related peritonitis. The number of peritonitis episodes per patient-year and the incidence of cardiovascular disease were significantly higher in the withdrawal group. Similarly, this group had low levels of serum creatinine, urea nitrogen, serum albumin, alanine aminotransferase, cholinesterase and high C-reactive protein, and second dialysate cell counts after antibiotic administration. Multivariate logistic regression analysis revealed that serum albumin (odds ratio: 0.465; 95% confidence interval: 0.249-0.868; P=0.016) and cardiovascular disease (odds ratio: 2.508; 95% confidence interval: 1.184-5.315; P=0.016) exhibited significant differences. CONCLUSIONS: The results of this study suggest that hypoalbuminemia and the presence of cardiovascular disease were independent risk factors for withdrawal from peritoneal dialysis due to peritoneal dialysis-related peritonitis.

Clinical Characteristics and Risk Factors for Mortality due to Bloodstream Infection of Unknown Origin in Hemodialysis Patients: A Single-Center, Retrospective Study.

INTRODUCTION: Hemodialysis patients are at a high risk of bloodstream infection (BSI). The risk factors for BSI-associated mortality, especially of unknown origin, remain uncertain. BSI of unknown origin is highly prevalent and related to high mortality. The present study aimed to investigate the clinical and microbiological characteristics of BSI and risk factors for BSI-associated mortality, including BSI of unknown origin, in hemodialysis patients. METHODS: This study was a single-center, retrospective study conducted from August 2012 to July 2019 in hemodialysis patients with BSI at Kawashima Hospital. Data related to demographics, clinical parameters, BSI sources, causative microorganisms, and initial treatments were collected from the medical records. The predictors for mortality associated with BSI were evaluated by logistic regression. RESULTS: Among 174 patients, 55 (30.9%) had the infection from unknown origin. The most frequent bacterium was Staphylococcus aureus. Low serum albumin level was an independent predictor of mortality due to BSI (odds ratio [OR]: 0.28, 95% confidence interval [CI]: 0.13-0.59). A lower serum albumin level (≤2.5 g/dL) was associated with poorer mortality. Methicillin-resistant Staphylococcus aureus (MRSA) was independently associated with mortality due to BSI of unknown origin (OR: 6.20, 95% CI: 1.04-37.1); 87.5% cases with BSI of unknown origin due to MRSA were not initially administrated anti-MRSA antibiotics, and in such patients, the mortality rate was 85.7%. CONCLUSIONS: Serum albumin level of 2.5 g/dL is a cutoff value, which could predict the mortality due to BSI in hemodialysis patients. Considering the high mortality rate of MRSA-associated BSI of unknown origin, wherein no focus of infection was identified in the present study, initial empiric treatment should be considered for MRSA-associated BSI of unknown origin.

Parathyroidectomy for tertiary hyperparathyroidism after second kidney transplantation: a case report.

Successful kidney transplantation usually resolves secondary hyperparathyroidism (SHPT). However, some patients fail to normalize, and their condition is often referred to as tertiary hyperparathyroidism (THPT). Surgical consensus on the timing of post-transplant parathyroidectomy (PTX) for THPT has not been reached. Herein, we report a case of a 58-year-old post-transplant woman, considering the concrete timing of PTX for both SHPT and THPT. She initiated hemodialysis with end-stage renal disease at the age of 24, and underwent first kidney transplantation at the age of 28. When peritoneal dialysis (PD) was induced due to the worsening kidney function at the age of 50, the serum intact parathyroid hormone (iPTH) level remarkably increased (2332 pg/mL). Although cinacalcet was administered, the patient's iPTH levels were not sufficiently suppressed for seven years. Diagnostic images including ultrasound, computed tomography, and 99mTc-methoxyisobutylisonitrile scintigraphy indicated THPT as the reason for prolonged post-transplant hypercalcemia. Therefore, PTX was performed 14 months after the second transplantation. Histology showed nodular hyperplasia of all parathyroid glands, indicating autonomous secretion of parathyroid hormone. In general, patients with more severe THPT are recognized with more severe SHPT prior to transplantation during the dialysis period. We should consider a referral for surgery based on the individual risk factors. We recommend to perform parathyroidectomy earlier, before the kidney transplantation in the clinical suspicion of severe SHPT.

Clinical characteristics associated with 1-year tolvaptan efficacy in autosomal dominant polycystic kidney disease with a wide range of kidney functions.

Autosomal dominant polycystic kidney disease (ADPKD) develops into end-stage kidney disease by 65 years of age in an estimated 45%-70% of patients. Recent trials revealed that tolvaptan inhibits disease progression both in early-stage or late-stage ADPKD ; however, stratified analysis showed a difference of favorable factors correlated with tolvaptan efficacy between early-stage and late-stage ADPKD. Thus, we examined the efficacy of tolvaptan in ADPKD with a wide range of estimated glomerular filtration rates (eGFR). We enrolled 24 patients with eGFR 35.3 (28.0-65.5) ml / min / 1.73m2 and evaluated treatment effect as ΔΔeGFR (ml / min / 1.73m2 / year) or ΔΔtotal kidney volume (TKV) (% / year) that was calculated as post-treatment annual change - pre-treatment annual change. Pre ΔeGFR was significantly low in eGFR responders, defined as ΔΔeGFR > 0 ml / min / 1.73m2 / year. In eGFR responders, pre ΔeGFR, post ΔeGFR, eGFR, TKV, and proteinuria were significantly correlated with ΔΔeGFR. In TKV responders defined as ΔΔTKV > 5 % / year, we identified hypertension history, proteinuria, TKV, and post ΔTKV as significantly correlated factors with ΔΔTKV. In conclusion, pre ΔeGFR may be a predictive factor of therapeutic efficacy on kidney function. Tolvaptan may have greater efficacy in early-stage ADPKD with rapid GFR decline or with well-controlled blood pressure. J. Med. Invest. 67 : 315-320, August, 2020.

Pulmonary sarcoidosis as a cause of intermittent fever of unknown origin in a hemodialysis patient with renal cell carcinoma: A case report and literature review.

Hemodialysis patients have weakened immune systems and can exhibit fever due to various causes. Herein, we describe the case of a 61-year-old hemodialysis patient who exhibited intermittent low-grade fever after a pacemaker had been implanted 2 months before due to sick sinus syndrome. She had a medical history of subcutaneous sarcoidosis and uveitis. Active pulmonary sarcoidosis was diagnosed based on elevated soluble interleukin-2 receptor, elevated lysozyme level, and gallium-67 scintigraphy uptake in hilar and mediastinal lymph nodes. She was also diagnosed with renal cell carcinoma via contrast computed tomography. However, because her C-reactive protein level remained normal, the possibility of neoplastic fever was considered low. After the initiation of prednisolone administration, her fever gradually disappeared. Her serum soluble interleukin-2 receptor and lysozyme level improved in parallel with the enlargement of the mediastinal lymph node and gallium-67 scintigraphy uptake.

Alogliptin-Induced Minimal Change Nephrotic Syndrome and Interstitial Nephritis.

Alogliptin is one of the dipeptidyl peptidase-4 inhibitors used to treat patients with type 2 diabetes. Little is known about the nephrotoxicity associated with alogliptin, such as nephrotic syndrome or interstitial nephritis. We report a biopsy-proven rare case of minimal change nephrotic syndrome and interstitial nephritis induced by alogliptin. A 68-year-old man who had been prescribed alogliptin was hospitalized for nephrotic syndrome. On admission, serum creatinine level was elevated with increased urinary ß2-microglobulin and N-acetyl-ß-d-glucosaminidase excretion. Kidney biopsy revealed minor glomerular abnormalities and interstitial nephritis, and gallium-67 scintigraphy showed uptake in both kidneys. A drug lymphocyte stimulation test for alogliptin was positive. With discontinuation of alogliptin treatment alone, serum creatinine level normalized in parallel with urine ß2-microglobulin and N-acetyl-ß-d-glucosaminidase levels. In addition, complete remission of nephrotic syndrome was observed. Drug-induced dual pathology has not been previously reported with alogliptin. In summary, clinicians should keep in mind that alogliptin can induce minimal change nephrotic syndrome and interstitial nephritis.

Association between periodontal condition and kidney dysfunction in Japanese adults: A cross-sectional study.

Recent studies have demonstrated that chronic kidney disease (CKD) may be associated with the progression of periodontal disease. Diabetes mellitus (DM) is a major risk factor for CKD. The objective of this study was to clarify the relationship between periodontal condition and kidney dysfunction in patients who had kidney failure with or without DM. One hundred sixty-four patients with kidney dysfunction were enrolled (male: N = 105; female: N = 59), and the relationship between periodontal condition and kidney dysfunction was analyzed in a cross-sectional study. The subjects were divided into three groups: (a) patients with DM, (b) dialysis patients with nephropathy due to various kidney diseases, and (c) dialysis patient with nephropathy due to DM (diabetic nephropathy). Then, the effect of DM on the periodontal condition was analyzed. The patients were also stratified by CKD stage (into G1-G5) using the estimated glomerular filtration rate (eGFR), and the G5 group was divided in patients with or without DM. Correlations between eGFR and parameters of periodontal condition were calculated in patients from G1 to G4. The number of missing teeth was significantly higher in dialysis patients with diabetic nephropathy than in patients with DM, whereas alveolar bone loss did not show a significant difference among the three groups. In addition, the G5 patients with DM had a significantly higher number of missing teeth than the other CKD groups, whereas alveolar bone loss did not show a significant difference. In G5 patients with DM, Community Periodontal Index and Oral Hygiene Index scores were significantly higher than in G1-4 patients with DM. There was a significant negative correlation between eGFR and the number of missing teeth. Patients with diabetic nephropathy have a higher rate of periodontal problems such as missing teeth in Japanese adults.

Treatment of renal anaemia with erythropoiesis-stimulating agents in predialysis chronic kidney disease patients: Haemoglobin profile during the 6 months before initiation of dialysis.

AIM: Erythropoiesis-stimulating agents (ESAs) are all effective for renal anaemia in patients with chronic kidney disease (CKD). However, it was reported that the haemoglobin (Hb) concentration decreases to 8.4 g/dL during the initial phase of dialysis despite treatment with recombinant human erythropoietin (rHuEPO). This study compared Hb at the initiation of dialysis among patients treated with three different ESAs (rHuEPO, darbepoetin alfa [DA], and a continuous erythropoietin receptor activator [CERA]). METHODS: The subjects were 82 CKD patients who started dialysis at Kawashima Hospital between 1 January 2009 and 28 February 2015 and who received only one kind of ESA for at least 6 months before initiation of dialysis. Baseline characteristics and laboratory data at initiation of dialysis were compared among the three groups. Then changes of the Hb, ESA dose, and erythropoiesis resistance index were assessed over time during the 6 months before initiation of dialysis. Differences of Hb at the initiation of dialysis were also assessed. RESULTS: Among the 82 patients, 36 received rHuEPO, 13 received DA, and 33 received CERA. Baseline characteristics and laboratory data of the patients showed no significant differences among the three groups. The monthly Hb decreased gradually during the 6-month period before initiation of dialysis in all three groups. Hb was significantly higher in the CERA group than the rHuEPO group at the initiation of dialysis. CONCLUSION: Long-acting ESAs may be more useful for predialysis patients with CKD because they do not attend hospital frequently, unlike haemodialysis patients.

Stringent glycaemic control prolongs survival in diabetic patients with end-stage renal disease on haemodialysis.

AIM: No suitable index or optimal target for diabetic control has been established for diabetic patients with end-stage renal disease (ESRD) undergoing haemodialysis. To address these issues, the single-centre observational study was conducted. METHODS: Two hundred and forty-five diabetic ESRD patients (23.3% female; age at initiation of haemodialysis 61.7 ± 10.7 years) at start of haemodialysis between 1 January 1995 and 31 December 2004 were enrolled. Subjects were grouped according to glycaemic control level throughout the observational period as follows: mean postprandial plasma glucose (PPG) <8.9 mmol/L, 8.9 mmol/L ≤ PPG < 10.0 mmol/L, 10.0 mmol/L ≤ PPG < 11.1 mmol/L, 11.1 mmol/L ≤ PPG < 12.2 mmol/L and PPG ≥ 12.2 mmol/L; and HbA1c < 6.0%, 6.0-6.4%, 6.5-6.9% and ≥ 7.0%. Survival was then followed until 31 December 2005. RESULTS: Cumulative survival of groups of 10.0 mmol/L ≤ PPG < 11.1 mmol/L, 11.1 ≤ PPG < 12.2 and PPG ≥ 12.2 mmol/L was significantly lower than that for PPG < 8.9 mmol/L as determined by Kaplan-Meier estimation (P = 0.016, 0.009 and 0.031, respectively; log-rank test). In both uni- and multivariate Cox proportional hazard models, mortality hazard ratios were significantly higher for PPG ≥ 10.0 mmol/L than for PPG < 8.9 mmol/L (P = 0.002-0.021). Kaplan-Meier survival curves grouped by HbA1c levels showed no correlation between HbA1c and survival during the observational period. No significant difference in mortality hazard ratios was seen for any HbA1c groups evaluated by Cox proportional hazard model. CONCLUSION: Intensive management of diabetic control at a stringent mean on-study PPG < 10.0 mmol/L will improve the life expectancy in diabetic dialysis patients. However, no range of HbA1c values obtained in this study showed any clear difference in clinical outcomes.

Effect of protein and energy intakes on body composition in non-diabetic maintenance-hemodialysis patients.

This cross sectional study was performed to find the adequate amount and combination of dietary protein and energy for maintaining better nutritional status for stable non-diabetic maintenance hemodialysis (MHD) patients. The body composition including body fat, total body water, body cell mass and body protein were measured by multi-frequency bioelectrical impedance analysis in 200 stable MHD patients without diabetes (124 men, 76 women). Dietary energy intake (DEI) and dietary protein intake (DPI) were assessed by a brief self-administered diet history questionnaire (BDHQ), the DPI value being confirmed by calculating the normalized protein equivalent of total nitrogen appearance (nPNA). The nutritional status and the body composition were compared among 4 groups of patients in each gender that were divided by the combination of DEI and DPI; high energy (HE)/high protein (HP), HE/low protein (LP), low energy (LE)/HP and LE/LP groups. The mean DPI ranged between 1.17-1.23 and 0.89-0.95 g/kg IBW/d in the HP and LP groups, respectively for both genders, and the mean DEI was 35-37 and 24-25 kcal/kg IBW/d in HE and LE groups, respectively. BMI and serum albumin concentration were not different among the 4 groups. Body cell mass index (BCMI) was maintained in the HE groups regardless of DPI, and it was significantly higher in the HE/HP group than in the LE/LP group. Multiple regression analysis also showed that the BCMI was more greatly affected by DEI than DPI. These results indicated that a DPI of 0.89-0.95 g/kg IBW/d could be sufficient for maintaining BCMI, if DEI is kept over 35 kcal/kg IBW/d in stable non-diabetic MHD patients. This DPI level is lower than the recommended DPI proposed by dietary guidelines in the US and Japan.