A 65-year-old woman presented with intermittent right hand numbness and elevated serum creatinine for more than 2 months. The histological examination of kidney biopsy showed renal arterioles occlusion and interstitial fibrosis. Pathological abnormality was originally considered as a part of systemic atherosclerosis. Thus, rosuvastatin 20 mg/d, fosinopril 10 mg/d, metoprolol 47.5 mg/d and aspirin 0.1g/d were administrated. No improvement of renal function was seen. Further Congo red staining was applied. Diffuse amorphous eosinophilic substance was deposited in interlobular artery and small arteriolar artery. Combined with the abnormal free light chain (FLC) level and ratio (serum κ 340 mg/L, κ/λ 10.932), the diagnosis of systematic light-chain amyloidosis was confirmed. The patient received 3 courses of chemotherapy regimen as BCD (bortezomib 2 mg d1, 8, 15, 22, cyclophosphamide 0.3 g d1, 8, 15, 22 and dexamethasone 40 mg d1, 8, 15, 22). A hematologic partial response was achieved and serum creatinine decreased to 180 μmol/L.

Objective To investigate the clinical and pathological features of Fabry disease in Chinese patients. Methods This retrospective study included all in-patients Fabry disease patients at PUMC Hospital from 2004 to 2014.Results The 12 patients included 10 males and 2 females.The middle age at onset was 10.The most fre-quent symptoms in our group were pain(10/12), angiokeratoma(9/12), renal abnormalities (7/12), hearing loss ( 6/12 ) , hypohidrosis ( 5/12 ) , cardiac abnormalities ( 5/12 ) , cerebrovascular abnormalities ( 3/12 ) and ophthalmological abnormalities ( 3/12 ) .The severe clinical manifestations were more frequently found in males . Electron microscopy plays a very important role in pathological diagnosis of Fabry disease .7 patients were suspec-ted to have family history .5 patients were examined regularly in which 4 cases were stable , and 1 patient re-ceived hemodialysis after 3 years'follow-up.Conclusions Onset of Fabry disease is early, many organs could be involved and patients may have different clinical manifestations.There are great differences in clinical features be-tween male and female patients .

Objective To evaluate the predictive factors and renal outcomes of idiopathic membranous nephropathy (IMN) in patients with type 2 diabetes (T2DM).Methods In this retrospective study,clinical data of 101 IMN patients with T2DM and 96 patients with diabetic nephropathy (DN) were consecutively collected.Logistic regression was used to assess potential clinical factors indicating IMN and COX regression was employed to analyze risks of IMN in developing to endstage renal disease (ESRD),as compared with that of DN,in patients with T2DM.Results In a multivariate model,age ≥55 years old,presence of nephrotic syndrome,estimated glomerular filtration rate (eGFR) ＞ 60 ml · min-1 · (1.73 m2)-1,duration of diabetes≤5 years and absence of diabetic retinopathy,were associated with IMN,as compared with DN,in patients with T2DM.In T2DM patients presented with nephrotic syndrome,age≥55 years old,eGFR ＞ 60 ml· min1· (1.73 m2)-1,duration of diabetes≤5 years and absence of diabetic retinopathy,were also associated with IMN,as compared with DN.Receiver operating characteristic curve (ROC) showed eGFR 65.5 ml · min-1 · (1.73 m2) 1 was an optimal cutoff in differentiating DN and IMN.DN was associated with 16.8 times as high risk of incident ESRD as compared with IMN in T2DM patients.Conclusions In patients with T2DM,age≥55 years,presence of nephrotic syndrome,early stage of CKD,duration of diabetes≤5 years and absence of retinopathy,may indicate IMN rather than DN.T2DM patients with IMN have much better renal prognosis as compared with DN.

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 analyze the clinicopathological features and prognosis of antiglomerular basement membrane(GBM)disease,and evaluate the efficacy and safety of double filtration plasmapheresis(DFPP). Methods A total of 35 hospitalized patients diagnosed as anti-GBM disease in our department were enrolled in the study.All the patients were divided into 3 groups according to the manifestations at admission.Group Ⅰ∶24 patients with severe pulmonary hemorrhage or rapidly progressive glomerulonephritis(RPGN)received pulse methylprednisolone with or without DFPP,and then followed by prednisone and CTX.Group Ⅱ∶5 patients without severe pulmonary hemorrhage and RPGN received prednisone and CTX.Group Ⅲ∶5 ESRD patients and 1 normal renal function patient did not receive immunosuppression therapy.Anti-GBM antibody titer of pre-and post-DFPP in 4 patients was measured consecutively,and removal rate was calculated.Results The mean age of all the patients was(41.1±16.6)years.Sixteen patients(45.7%)presented Goodpasture's syndrome.Eighteen patients(51.4%)had anti-GBM glomerulonephritis alone,whereas one suffered solely from pulmonary hemorrhage.20%patients had positive P-ANCA serology.54.2%crescentic glomerulonephritis and 7 with other glomerulonephritis were revealed by kidney biopsy in 24 patients.Patients in Group Ⅰ showed more severe manifestation at admission:higher Scr level,higher titer of anit-GBM antibody,greater percentage of crescents.Within the follow-up period,7 patients died and kidneys of 50%patients survived.No patient died in Group Ⅱ and Ⅲ.The elder age,anemia,higher Scr(＞300 μmol/L),oliguria or anuria,emergency hemodialysis at admission,and more glomerular sclerosis were predictors of poor prognosis.The anti-GBM antibody was negative after 4 to 6 sessions of DFPP.and the mean removal rate was 55%.During total 94 DFPP sessions,there was no unacceptable morbidity. Conclusions Different therapy strategy is necessary for anti-GBM disease with different clinical manifestations.DFPP is an effective and safe clearance way of anti-GBM antibody.

Objective To retrospectively evaluate the relevant factors for hepatitis B virus-associated glomerulonephritis (HBV-GN).Methods A total of 86 patients with pathologyproven HBV-GN and 135 HBV carriers with non-HBV-GN were included in this retrospective casecontrol study.Logistic regression analysis was used to detect the relevant factors for HBV-GN.Results On univariate analysis,the factors associated with HBV-GN were as follows: male (OR 2.79,95%CI 1.48-5.25,P=0.001),HBeAg positivity (OR 2.60,95%CI 1.49-4.53,P=0.001),HBV replication (OR 3.63,95%CI 1.80-7.33,P＜0.01),liver cirrhosis (OR 4.58,95%CI 1.41-14.91,P=0.011),and elevated alanine aminotransferase (ALT) (OR 2.53,95%CI 1.42-4.51,P=0.002).On multivariate analysis,the associations remained significant for male (OR 2.21,95%CI 1.12-4.33,P=0.022),HBV replication (OR 2.77,95%CI 1.28-5.97,P=0.01),liver cirrhosis (0R 4.55,95%CI 1.29-16.10,P=0.019) and elevated ALT (OR 1.96,95%CI 1.04-3.69,P=0.037).Compared with HBV-associated IgA nephritis (HBV-IgAN) in multivariate model,HBV-associated membranous nephropathy (HBV-MN) or membranoproliferative glomerulonephritis (HBV-MPGN) was significantly associated with male (OR 6.51,95%CI 1.76-24.11,P=0.005) and HBV replication (OR 7.22,95%CI 1.68-30.97,P=0.008).Conclusions Male,HBV replication,liver cirrhosis and elevated ALT may be predictive factors for HBV-GN.Compared with HBV-IgAN,HBV-MN or HBV-MPGN is significantly associated with male and HBV replication.

4D Flow MRI is a new phase contrast MR technique,which can spatially encode velocity in three-direction,collect multi-directional blood flow data,and result in complex three-dimensional dynamic parameters.4D Flow MRI is more accurate and comprehensive than 2D phase contrast MRI and Doppler echocardiography.However,4D Flow MRI have some disadvantages,such as long scanning time,noise,etc.,which are the future key technical issues to be addressed.4D Flow MRI technology has a great potential in clinical applications,such as intracranial vascular diseases,cardiac magnetic resonance imaging,and other aspects of aortic diseases.Research advances in clinical application of 4D Flow MRI were reviewed in this paper.

Primary biliary cirrhosis/cholangitis is an autoimmune disease. Renal tubular acidosis is a common form in PBC cases, but Fanconi syndrome is rarely reported. The paper reported a 66-year-old female patient with fatigue, renal insufficiency and elevated bile duct enzymes. The patient presented with type 2 proximal renal tubular acidosis and complete Fanconi syndrome. Laboratory examinations showed high-titer-positive anti-mitochondrial antibodies, elevated serum IgM, and type 3 cryoglobulinemia. Renal biopsy revealed interstitial nephritis, and electron micrographs showed abnormal mitochondria in proximal tubular epithelial cells. The patient's renal function ameliorated, and acid-base imbalance and electrolyte disturbances were corrected after high-dose glucocorticoid treatment.

The article reported one case of renal damage caused by lenvatinib in the treatment of advanced primary liver cancer. The patient was a 63-year-old male who was admitted to the hospital due to "liver cancer for 4 years, blood pressure elevation for nearly 2 years, and edema for 7 months". During the treatment of liver tumors with atezolizumab combined with lenvatinib, blood pressure increased and renal insufficiency aggravated progressively. Pathological light microscopy of renal biopsy showed endothelial cell lesion and tubulointerstitial damage, and electron microscopy showed moderate proliferation of mesangial cells and deposition of mesangial matrix. There were many agglomerated low-electron density deposits in the mesangial area, and a small amount of electron dense deposits in the subendothelium. The pathological diagnosis was endothelial cell disease (thrombotic microangiopathy) and secondary focal segmental glomerulosclerosis. Renal injury was considered as secondary to lenvatinib. After discontinuing lenvatinib and giving angiotensin receptor antagonist treatment, blood pressure was normal, urine protein turned negative, and renal function improved significantly after 8 months of outpatient follow-up.

Objective To analyze the clinic-pathological data and peritubular capillary (PTC) injuries of malignant nephrosclerosis (MN) patients and their correlations with the long term renal survival.Methods This was a retrospective cohort study of 52 MN patients in Peking Union Medical College Hospital from January 2003 to March 2012.Their clinical data and renal biopsy samples were carefully studied.CD34 staining was performed to evaluate the PTC area,using Benign nephrosclerosis (BN,n=17) patients and glomerular minimal lesions (GML,n=19) patients as controls.Multivariate Cox proportional hazard model was used to identify the potential independent risk factors for long term renal survival.Results Fifty-two MN patients were enrolled.The sex ratio of male to female was 12:1 and the average age was (34.0±8.2) years.The maximum blood pressure (SBP/DBP) was (230.4 ± 25.0)/(156.4 ± 20.6) mmHg,companied with significant loss of eGFR and proteinuria.Glomerular sclerosis index,tubular atrophy and interstitial fibrosis correlated with eGFR and proteinuria (P ＜ 0.05).After aggressive treatment,BP control rate improved significantly (76.9％ vs 3.7％,P ＜0.01),Scr [(376.4±263.8) μmol/L vs (486.8±375.7) μmol/L,Wilcoxon test,P＜ 0.01] and proteinuria [(1.10±0.70) g/24 h vs (2.04± 1.26) g/24 h,P ＜ 0.01,n=21] also improved.PTC area in MN patients was significantly lower than those in BN patients and GML patients,and it correlated well with Scr (r=-0.553,P=0.001) and eGFR (r=0.476,P=0.004).The median follow-up time was 74 months,the cumulative renal survival rate at 1 year,5 year and 10 year was 90％,64％ and 23％,respectively.Kaplan-Meier analysis showed that the patients with higher PTC area had longer renal survival time [(114.8± 12.4) months vs (63.0±8.3) months, x2=5.312,P ＜ 0.05].Univariate Cox proportional hazard model found that unsatisfied BP control,eGFR ＜ 30 ml · min-1 · (1.73 m2)-1 upon discharge,lower PTC area,severer tubular-interstitial damage and anemia were associated with poor renal outcome.Multivariate Cox model showed that unsatisfied BP control (RR=3.89,95％ CI 1.75-8.65,P=0.001),eGFR ＜ 30 ml · min-1 · (1.73 m2)-1 upon discharge (RR=4.27,95％ CI 1.40-13.09,P=0.011) were independent risk factors for long-term renal survival.Conclusions The correlation between PTC area and renal functions in MN patients are much better than that of classic vascular changes.Unsatisfied BP control and eGFR ＜ 30 ml · min-1 · (1.73 m2)-1 upon discharge are independent risk factors for long-term renal survival.