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 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.

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.

Objectives To analyze the spectrum of renal diseases associated with monoclonal gammopathy and unrelated renal diseases.Methods Hospitalized patients in Peking Union Medical College Hospital who underwent renal biopsy between January,2013 and December,2015.They had monoclonal gammopathy on serum protein electrophoresis (SPE),serum immunofixation electrophoresis (IFE),urine IFE and/or serum free light chain (FLC).64 patients met the inclusion criteria and were classified as monoclonal gammopathy of renal significance (MGRS) (n=36),monoclonal gammopathy of undetermined significance (MGUS) (n=17) and hematologic malignancy (n=11).Results Renal lesions in MGRS subgroup included light chain amyloidosis (n=28,77.8％),light chain deposition disease (n=7,19.4％),and fibrillary glomerulopathy (n=l,2.8％).eGFR in light chain amyloidosis subgroup differed significantly,compared with light chain deposition disease [eGFR 93 ml· min-1 · (1.73m2)-1 vs 28 ml· min-1 · (1.73 m2) 1,P ＜ 0.01],as well as HTN incidence (35.7％ vs 100.0％,P ＜ 0.01).Renal diseases in MGUS subgroup included membranous nephropathy (n=10,58.8％),focal segmental glomerulosclerosis (n=3,17.6％),diabetic glomerulopathy (n=l,5.9％),Henoch-Schonlein purpura nephritis (n=l,5.9％),anti-glomerular basement membrane disease concurrent with membranous nephropathy (n=l,5.9％) and glomerulomegaly (n=l,5.9％).Various renal lesions related/unrelated to hematologic malignancy were seen in third subgroup,including light chain cast nephropathy (n=3,27.3％),tubulo-interstitial lesions (n=2,18.2％),light chain amyloidosis (n=1,9.1％),light chain deposition disease(n=1,9.1％),IgA nephropathy (n=1,9.1％),mesangial proliferative glomerulonephritis (n=l,9.1％),endocapillary proliferative glomerulonephritis (n=1,9.1％) and acute tubular necrosis (n=1,9.1％).Positive rates of SPE,serun IFE and urine IFE in MGRS subgroup were 40.6％,52.8％ and 69.4％,respectively.Positive rates of SPE,serum IFE and urine IFE in MGUS subgroup were 68.8％,100.0％ and 37.5％,respectively.Positive rates of SPE,serum IFE and urine IFE in hematologic malignancy subgroup were 54.5％,72.7％ and 81.8％ respectively.MGRS and MGUS subgroups differed significantly in positive rate of serum IFE (P ＜ 0.001).Abnormal rates of serum FLC ratio in above three subgroups were 83.3％,17.6％ and 90.9％,respectively,with that in MGUS group being significantly lower than the rates in other two groups (P ＜ 0.001,respectively).Conclusions The significance of monoclonal gammopathy in patients with renal disease should be evaluated by other clinical data,as well as renal pathology.

Objective To assess the predictive value of Berden classification in ANCA associated glomerulonephritis.Methods Patients with confirmed ANCA associated glomerulonephritis were included,by retrieving the medical database in Peking Union Medical College Hospital from January 2000 to May 2015.Their detailed information during hospitalization and follow-up was recorded.The patients were divided into four categories based on Berden classification.The differences in clinical characters,renal function and response for treatment were compared.Results Among the 88 patients with ANCA-associated glomerulonephritis,19 (21.6％),21 (23.9％),32 (36.4％)and 16 (18.2％) patients were classified as focal,mixed,crescentic and sclerotic category.22 patients developed ESRD,and 19 patients died during follow up (1 patient developed ESRD before died).The mean estimated glomerular filtration rate (eGFR) at baseline was 68.04,25.45,30.04,15.16 ml·min-1·(1.73 m2)-1 (P ＜ 0.05) in focal,crescentic,mixed and sclerotic category,respectively.During follow-up period,focal category always had the best renal function,while sclerotic category had the worst renal function.Crescentic category and mixed category were similar and in the middle.Remission rate at 6m was 62.5％,73.7％,57.5％,30.8％(P ＞ 0.05).And crescentic category had the greatest improvement in eGFR at 6m.Conclusions Focal category had relatively preserved renal function and favorable renal outcome,while the sclerotic category had the worst renal outcome.Crescentic and mixed category had an intermediate outcome.We support the use of the Berden classification in predicting the renal prognosis of patients with ANCA associated glomerulonephritis.

Objective To observe the clinical parameters, self-management, and compliance in peritoneal dialysis (PD) patients before and during Spring Festival, and to describe holiday syndrome in PD patients.Methods We prospectively observed PD patients with regular follow-up in our center before Chinese Spring Festival (January 8-February 7, 2016) and during Chinese Spring Festival (February 8-March 8, 2016).Demographic and clinical characteristics, self-management, and compliance with its influencing factors were collected by face-to-face method in outpatient care.Results Totally 130 PD patients were enrolled.The average age was (58.5±15.4) years, and women accounted for 53.1％.The primary diagnosis was diabetic kidney disease (32.3％), followed by primary glomerulonephritis (26.2％).Fourteen patients (10.8％) received automated peritoneal dialysis (APD), and most of the remaining patients chose continuous ambulatory peritoneal dialysis (CAPD,73.8％), with a median dialysis age of 34 months.During the 4-week followed-up, the appetite was stable, while the serum phosphate and pre-albumin increased [(1.5±0.4)mmol/L vs.(1.6±0.4)mmol/L, P=0.025;325.3 (272.2,355.1)mmol/L vs.326.0(284.2,376.5)mmol/L, P<0.01, respectively].No difference was observed in edema, dyspnea, and nighttime lying flat.However, the weight elevated significantly [(61.3±13.9)kg vs.(63.4±13.0)kg, P=0.002], with the median peritoneal ultrafiltration volume increased from 783.3 ml to 900.0 ml (P=0.005).During the holiday, the incidences of dialysis-related infections were unchanged, while the incidences of digestive system comorbidities increased significantly (5.4％ vs.13.8％, P=0.021).As for the compliance, 8 patients reduced their PD cycles during the holiday, which was more frequent than before holiday (P=0.018).One patient changed his PD pattern, and 6 patients did not follow the medication orders.Demographic characteristics such as gender, age, and marriage conditions were not associated with the compliance.Conclusions Holiday syndrome remarkably affects PD patient''s volume load, metabolism, and compliance.Poor self-management of PD patients during holidays needs further concern.