A 31-year-old man sought medical evaluation for a 2-year history of edema and proteinuria, with prior pathology suggesting atypical membranous nephropathy (MN). Despite treatment with a combination of steroids, calcineurin inhibitors, and four courses of rituximab (1 g, intravenous injection), the patient′s nephrotic syndrome showed no relief (24 h urine protein peaked at 31.18 g/d), indicating refractory nephrotic syndrome. Later in the disease course, a sudden surge of creatinine level (322.5 μmol/L) prompted a renal biopsy, which revealed concurrent acute interstitial nephritis. Further treatment involving steroids, cyclophosphamide, and a fifth rituximab infusion (1 g, intravenous injection) resulted in improvement in renal function (serum creatinine: 322.5?147 μmol/L), but the MN failed to achieve partial relief. Subsequent treatment with the novel humanized CD20 monoclonal antibody obinutuzumab (1 g, intravenous injection) was initiated. In the latest follow-up, anti-phospholipase-A2-receptor antibody (PLA2R) antibody were negative, B cells were eliminated, serum albumin was 36 g/L, urine protein-to-creatinine ratio was 4 810 mg/g, and serum creatinine was 162 μmol/L. This case underscores the potential efficacy of obinutuzumab in refractory MN. For advanced MN cases, prompt identification of the cause of acute kidney injury is crucial, emphasizing the need for targeted interventions to potentially stall renal function decline.

Objective:To analyze the clinical and pathological characteristics, treatment and prognosis of renal changes in patients with Kimura disease and improve the clinicians′ understanding on renal manifestations of Kimura disease.Methods:The clinical data of Kimura disease patients with definite diagnosis and detailed data in Peking Union Medical College Hospital from January 1980 to August 2020 were retrospectively analyzed. The patients were divided into renal impairment group and non-renal impairment group according to whether the kidney was involved or not and the related clinical data between the two groups were compared. The patients presenting with nephrotic syndrome were followed up.Results:There were 60 patients with Kimura disease confirmed by pathological diagnosis with 48 males. The median age was 33(3, 62) years old, and the median duration was 36(12, 111) months. There were 18 cases complicated with renal injury in 49 patients with complete routine urine and renal function examination and the main manifestations of renal injury were proteinuria and/or microscopic hematuria. There was no significant difference at age, sex and absolute value of eosinophils between the two groups (all P>0.05). Compared with the renal inpairment group, patients in non-renal inpairment group had longer course of disease, higher levels of hypersensitive C-reactive protein and erythrocyte sedimentation rate, and lower median values of total eosinophils and total IgE, but there was no statistically significant difference (all P>0.05). Among the patients with renal involvement, 6 patients met the diagnostic criteria for nephrotic syndrome, and 5 of them completed renal biopsies. The renal pathological diagnosis was membranous nephropathy in 2 cases and minimal change disease in 3 cases, and no interstitial eosinophil infiltration was found in renal biopsy tissues. These patients had a good response to glucocorticoids and/or immunosuppressive therapy, and achieved complete remission of nephrotic syndrome; at the same time, lymphadenopathy caused by Kimura disease could be well controlled. Conclusions:Kimura disease can combine with various renal lesions, and the pathology of nephrotic syndrome can be membranous nephropathy or minimal change nephropathy. After energetic treatment of glucocorticoids and/or immunosuppressive therapy, nephrotic syndrome can be completely relieved, and lymphadenopathy can be well controlled. The relationship between Kimura disease and renal disease needs further study.

A young female patient presented with fever, arthralgia, and rash was diagnosed with adults still's disease. When treated with glucocorticoid steroid, the above patient progressed to anuria, sudden, and confusion. After a teamwork involving different departments, the patient was finally diagnosed with atypical hemolytic uremic syndrome (aHUS) and treated with good outcome. aHUS is a rare disease, while Eculizumab is an orphan drug. The diagnosis and treatment of the patient reveals the importance of multidisciplinary team on the diagnosis and treatment of rare and difficult diseases.

Objective:To evaluate whether dialysis modality will affect cognitive function in dialysis population.Methods:This was a cross-sectional study. Chronic dialysis patients in our center was screened from July 2013 to July 2014. All of the subjects received brain magnetic resonance imaging (MRI) examination and comprehensive cognitive function evaluation.Results:A total of 189 chronic dialysis patients were enrolled in this study, 122 cases on hemodialysis (HD) and 67 cases on peritoneal dialysis (PD). There was no significant difference in age between HD and PD groups [(56.4±13.2) years vs (56.4±16.1) years, t=0.004, P=0.997]. The dialysis vintage and serum albumin of HD patients was higher than those of PD patients[58.0(16.8, 107.5) months vs 31.0(7.0, 67.0) months, Z=-3.490, P<0.001; (39.6±3.9) g/L vs (35.3±3.8) g/L, t=7.328, P<0.001, respectively]. The prevalence of cerebral small vessel diseases (CSVDs) was comparable between HD and PD groups (all P>0.05). Compared with HD patients, PD patients presented a 11.90-fold risk of immediate memory impairment (95% CI 1.40-101.08, P=0.023) and a 6.18-fold risk of long-delayed memory impairment (95% CI 2.12-18.05, P=0.001). After adjusting for age, educational lever, dialysis vintage, serum creatinine, and CSVDs, the influence of dialysis modality on memory still worked. PD patients presented a 43% risk of executive function impairment of HD patients ( OR=0.43, 95% CI 0.17-1.04, P=0.061). Conclusions:HD patients manifested better memory than PD patients, while PD probably performed better in executive function than HD patients. There was no significant difference in language function between the two groups. The difference in cognitive function may not be related to CSVDs.

A 43-year-old male presented with elevated serum creatinine for 4 years and developed abdominal pain for 3 days. He started peritoneal dialysis 2 months ago. Dialysis-related peritonitis was ruled out and acute gastroenteritis was diagnosed. The patient was administrated with ertapenem 500 mg/d. An acute mental abnormality developed 3 days later. After excluded organic encephalopathy, ertapenem was discontinued for the suspicion of antibiotic-related encephalopathy. The frequency of peritoneal dialysis was increased to accelerate the clearance of antibiotics. However, the metal abnormality became even more severe. Then a diagnosis of Wernick-Korsakoff syndrome was considered. After the administration of high dose vitamin B₁, the mental disorder dramatically relieved. Vitamin B₁ 30 mg/d is maintained during peritoneal dialysis and the mental disorder does not relapse.

Objective To investigate the clinical and pathological features of patients with a combination of Sjogren's syndrome (SS) and antineutrophil cytoplasmic antibody (ANCA) associated vasculitis with renal involvement.Methods By searching the Peking Union Medical College Hospital medical database and literature between January 1990 and June 2017,patients had a combination of SS and ANCA associated vasculitis with renal involvement were included.Data of clinical information,autoimmune antibodies,renal manifestations and renal pathology were retrieved and analyzed.Results Eighteen patients were enrolled:4 from our hospital and 14 from literature.SS was diagnosed no later than ANCA associated vasculitis in all the patients,among which 83.3％(15/18) of patients had extra-glandular and extra-renal organs involved.All the patients were tested positive for myeloperoxidase (MPO)-ANCA,and only two were protein 3 (PR3)-ANCA positive concurrently.The positivity rates of antinuclear antibody (ANA),rheumatoid factor (RF),anti-SSA antibody,and anti-SSB antibody were 83.3％(15/18),55.6％(10/18),77.8％(14/18),and 38.9％(7/18),respectively.The renal manifestations were characterized by renal insufficiency with a median serum creatinine of 174 μmol/L,hematuria,moderate proteinuria with a median 24 hour urine protein of 1.70 g,and necrotizing vasculitis with oligo-immune complex and varying degrees of interstitial damage in pathology.Conclusions A combination of Sjogren's syndrome and ANCA associated vasculitis with renal involvement is rare in clinical setting,and almost all of the patients are MPO-ANCA positive,with high probability of ANA positivity and extra-glandular involvement.Physicians should beware of ANCA associated glomerulonephritis in SS patients with inexplicable renal dysfunction and renal biopsy should be carried out in time.

Objective To compare glycemic profile between diabetic patients receiving peritoneal dialysis and diabetic patients with normal kidney function, and to investigate the impact of peritoneal dialysis on glycemic control through continuous glucose monitor system ( CGMS). Methods 19 diabetic patients with end-stage renal disease receiving regular peritoneal dialysis (DMPD group) and 8 patients with non-diabetic ne-phropathy receiving regular peritoneal dialysis ( PD group) were randomly selected and matched with 20 diabetic patients with normal kidney function (DM group) based on age, gender and 72 hours mean glucose. CGMS were applied on all patients for 72 hours. Glycemic variability parameters were compared among the three groups. Results Peritoneal transport function was positively correlated with mean glucose, glucose standard deviation and mean amplitude of glycemic excursion. Compared with PD group, multiple variation parameters, such as intraday glycemic standard deviation (P<0. 001), covariant efficiency (P=0. 009) and mean of daily difference (P=0. 043), were significantly lower in DMPD group. Though both DMPD and DM group exhibited profile as trough in wee hours and post-prandial hyperglycemia, DMPD had higher glycemic level in wee hours (P<0. 001). Conclusion Diabetic patients with end-stage renal disease receiving regular peritoneal dialysis have smaller glucose variability than diabetic patients with normal renal function.

Objectives To compare the clinical characteristics, long - term survival and associated risk factors of automated peritoneal dialysis (APD) patients and continuous ambulatory peritoneal dialysis (CAPD) patients. Methods As a retrospectively study, adult patients started peritoneal dialysis in Peking Union Medical College Hospital (PUMCH) from September 1st, 2002 to September 30th, 2016 were enrolled. Baseline information and dialysis associated parameters were collected. The primary outcome was death and the secondary outcome was technical failure. The risk factors of death were analyzed in APD patients by Cox's regression model. Homochromous gender and age matched CAPD patients were analyzed as control. Results The baseline condition of 69 APD patients were similar to those of 138 CAPD patients. The survival rates of APD patients at 1-year、3-year and 5-year were 95.4%, 88.0% and 73.0% respectively, which were superior to CAPD patients. No significant difference in technical survival was found between APD and CAPD patients. Single-factor Cox's regression analysis showed that all-cause mortality of CAPD patients was 2.2 times higher than that of APD patients (95% CI 1.221-3.837). In the multi-factor Cox regression analysis model, adjusted by age, complications (including cardiovascular disease and diabetes), nPCR and serum creatinine, dialysis modality was not an independent risk factor of dialysis patients. Age (HR=1.077, 95%CI 1.016-1.142, P=0.013), diabetes (HR=3.608, 95%CI 1.117-11.660, P=0.032) and serum albumin (HR=0.890, 95%CI 0.808-0.982, P=0.020) were independently associated with all-cause death of APD patients. Conclusions Dialysis modality was not an independent risk factor for the all-cause mortality of peritoneal dialysis patients. Age, diabetic nephropathy and hypoalbuminemia were independently associated with the death of APD patients.

Objective To investigate the clinicopathological characteristics of IgAN patients with massive proteinuria,as well as their treatment response to glucocorticoids and long-term prognosis.Methods Clinical and pathological parameters were collected in patients diagnosed with IgA nephropathy in our hospital from Jan 2003 to Oct 2015.Patients were followed up for at least six months under the treatment with full dosage of glucocorticoids.Responses of patients with and without nephrotic syndrome were compared.Results A total of 156 patients were enrolled for the analysis (86 patients in the nephropathic proteinuria group,and 70 patients in the nephrotic syndrome group).Patients presented with nephrotic syndrome showed higher proportion of IgM deposition in renal slides.There exited no difference in treatment response to glucocorticoids between the two groups.Patients with full or partial remission showed a better prognosis by Kaplan-Meier analysis than no remission group (P ＜ 0.001).The ratio of segmental sclerosis was negatively correlated with treatment response to glucocorticoids by multiple linear regression (3 value=-0.330,P ＜ 0.001).Multivariate Cox regression model showed that glomerular density (HR=0.45,P=0.02) and eGFR (HR=0.95,P=0.001)were independent influential factors for renal survival.Conclusions Patients presented with nephrotic syndrome show higher proportion of IgM deposition in renal slides.Patients in remission after treatment with 6-month glucocorticoids present a better prognosis than no remission patients,and glomerular density as well as eGFR are independent influential factors for renal survival.

Objective: To analyze the clinical data of the elderly peritoneal dialysis (PD) patients in Peking Union Medical College Hospital (PUMCH), and to find the risk factors for the long-term survival. Methods: Baseline data and the outcome of maintenance PD patients from 1996-03 to 2015-09-30 were collected for a retrospective cohort study. Patients were divided into the non-elderly group (<65 years old), the 65-79 years old group and the ≥80 years old group, and were follow to 2016-09-30. The survival rate was calculated by Kaplan-Meier method and the risk factors of outcome were analyzed by the Cox's regression model. Results: Among 577 PD patients, about 243(42.1%) were elderly patients, including 207 patients aged between 65 and 79 years (35.9%) and 36 patients aged 80 or more (6.2%). The most common primary disease causing PD was diabetic nephropathy (DN) for both elderly and non-elderly patients. The 1-year, 3-year, 5-year survival rate of patients aged between 65 and 79 years were 87.0%, 61.9%, 32.4% respectively, and 72.5%, 48.5%, 27.3% for the ≥80 years old group. The dominating reasons of death were cardiovascular events and infection. There was no difference of technical survival rates among three groups, and the most common reason for technical failure was peritonitis. For elderly patients, diabetes (HR=2.193, 95% CI 1.445-3.328, P<0.001) and lower baseline serum albumin (HR=0.968, 95%CI 0.940-0.996, P=0.026) were independent risk factors for death. However, for non-elderly patients, diabetes (HR=3.746, 95%CI 2.149-6.529, P<0.001) was the only independent risk factor for death. Conclusions Cardiovascular diseases and infection are the main reasons for death among the elderly PD patients in PUMCH. Diabetes and lower baseline serum albumin may predict the mortality of elderly PD patients independently. Better management of nutrition might improve survival in elderly PD patients.