Chronic kidney disease patients have an increased prevalence of subclinical cerebrovascular diseases. Dialysis patients have severe vascular diseases burden. The cerebral small vessel diseases (CSVD) are difficult to find by clinical assessment. The evaluation of CVSD needs MRI. Cognitive impairment is a consequence of CVSD which is diagnosed by cognitive testing. These limited the study of CVSD and cognitive function in dialysis patients. Peritoneal dialysis (PD) patients are minority of dialysis population. We know even fewer about the CVSD in this special population. In this cross-sectional study, we enrolled 72 PD patients who received care at the Peking Union Medical College hospital peritoneal dialysis center. CSVD were assessed by brain MR images. Cognitive function was evaluated with the Chinese version of the MMSE and MoCA. In our PD patients, the brain MRI showed the prevalence different signs of CSVD were: lacunar infarcts 38.9%, microbleeds 36.1%, abnormal brain white matter hyperintensities (WMHs) 48.6%, and intracerebral hemorrhage 4.2%. 25% and 86.8%of our patients could be diagnosed as cognitive impairment, according to the MMSE and MoCA test, respectively. nPCR was lower in patients with a lacunar infarct or intracerebral hemorrhage, and relative to the MMSA/MoCA score; hsCRP was higher in patients with lacunar infarct or abnormal WMHs and negative relative to the MMSA/MoCA score. In logistic regression analyses, nPCR was an independent risk factor for lacunar infarcts and impaired cognitive function. The presence of lacunar infarct was an independent risk factor for cognitive function decline. We demonstrated a high prevalence of CSVD and cognitive impairment in our PD patients. Lacunar infarct was the main kind of CVSD responsible for PD patients cognitive function decline. Our novel observation also revealed an association between malnutrition-inflammation and CSVD.

Background . IgG4‐related disease is a new disease entity, but little attention was drawn to urinary system involvement besides nephritis or nephropathy. Here, we described clinical, radiological, and pathological manifestations of IgG4‐related urinary disease (IgG4‐RUD) and assess its treatment responses. Methods . We conducted a retrospective study enrolling 65 IgG4‐RUD patients from an IgG4‐related disease (IgG4‐RD) cohort of the Peking Union Medical College Hospital. Clinical, laboratory, radiological, pathological data were collected, and treatment response to immunosuppressants were analysed. Results . IgG4‐related interstitial nephritis (TIN, 32.3%), glomerular nephritis (GN, 7.7%), renal pelvis and ureter involvement (21.5%), abnormal radiology with quiescent clinical presentation (13.8%), and renal parenchymal lesion plus retroperitoneal fibrosis (RPF, 18.5%) were major lesion types of IgG4‐RUD. All patients had elevated serum IgG4, 76.9% had hyperglobulinemia, and 92.3% had elevated serum IgE at diagnosis. IgG4‐TIN patients presented with renal dysfunction, and 94.3% had low serum complement C3 and IgG4‐GN presented with nephrotic syndrome, while renal pelvis and ureter involvement had normal renal function and urinalysis. IgG4‐RPF with renal parenchymal involvement presented with acute renal dysfunction and required emergency medical intervention. Renal cortex low‐density areas, parenchyma or pelvis nodular mass, bilateral enlargement of the kidney, and renal pelvis and ureter mass/wall thickening were specific image patterns of IgG4‐RUD. Infiltration of plasma lymphocytes and storiform fibrosis were histopathological features of IgG4‐RUD. Patients showed satisfactory responses to immunosuppressive treatment, but complete recovery of renal function was difficult to achieve in IgG4‐TIN. Four patients (6.2%) experienced clinical relapses during the maintenance period. Conclusion . IgG4‐RUD had diverse lesion types and distinctive manifestations. Radiological examinations were helpful for diagnosis and treatment evaluation. Patients showed good initial response to immunosuppressive treatment but relapses could occur at the maintenance period.

Immune checkpoint inhibitors (ICIs) are nowadays widely used in clinical oncology treatment, and significantly improve the prognosis of cancer patients. However, overactivation of T cells and related signaling pathways caused by ICIs can also induce immune‐related adverse effects (irAEs). Renal immune side‐effects are relatively rare, but some are serious and fatal. Acute kidney injury (AKI), diagnosed mainly by percentage increases in serum creatinine (sCr), is the most common clinical manifestation, while acute tubulointerstitial nephritis (ATIN) is the main cause of ICI‐related AKI. Urinalysis analysis and sediment evaluation, 24 hour urine protein and sCr are helpful in screening and monitoring renal irAEs. Multiple potential causes for AKI are involved during cancer therapy, and should be differentiated from the immune mechanisms of ICIs. Under these circumstances, a renal biopsy should be considered which is essential for clinical decision‐making. Steroids are an effective treatment option for renal irAEs. Most patients who experience ICI‐related ATIN achieve a partial or complete renal recovery with prompt diagnosis and treatment. Multidisciplinary collaborations of different specialists will improve the effectiveness and outcome in the management of ICI irAEs.

Lipodystrophy syndromes are rare disorders of variable body fat loss associated with potentially serious metabolic complications. Familial partial lipodystrophy (FPLD) is mostly inherited as an autosomal dominant disorder. Renal involvement has only been reported in a limited number of cases of FPLD. Herein, we present a rare case of proteinuria associated with type 4 FPLD, which is characterized by a heterozygous mutation in PLIN1 and has not been reported with renal involvement until now. A 15-year-old girl presented with insulin resistance, hypertriglyceridaemia, hepatic steatosis and proteinuria. Her glucose and glycated haemoglobin levels were within normal laboratory reference ranges. A novel heterozygous frameshift mutation in PLIN1 was identified in the patient and her mother. The kidney biopsy showed glomerular enlargement and focal segmental glomerulosclerosis under light microscopy; the electron microscopy results fit with segmental thickening of the glomerular basement membrane. Treatment with an angiotensin-converting enzyme inhibitor (ACEI) decreased 24-h protein excretion. We report the first case of proteinuria and renal biopsy in a patient with FPLD4. Gene analysis demonstrated a novel heterozygous frameshift mutation in PLIN1 in this patient and her mother. Treatment with ACEI proved to be beneficial.

Glomerular filtration rate (GFR) is essential for renal function evaluation and classification of chronic kidney disease (CKD), while the reference method in children are cumbersome. In the Chinese children, there was no data about GFR measured through plasma or renal clearance of the exogenous markers, and therefore no validated GFR-estimating tools in this population. We measured GFR with double-sample plasma clearance of ^99mTc-DTPA (mGFR) in 87 hospitalized children with renal injury. Using mGFR as the golden standard, we evaluate the efficiency of four different GFR estimation equations (the original and update Schwartz equation, the Filler equation, the CKiD equation) by statistical parameters of correlation, precision and accuracy. In our population, mGFR was 97.0± 31.9 mL/min/1.73m². The updated Schwartz equation, the Filler equation and the CKiD equation, produced eGFR with strong correlation with mGFR, strong explanation capacity of variance in mGFR, small bias, satisfactory performance in Bland-Altman analysis, high intra-class correlation coefficients, high ratio of eGFR within mGFR±10% and eGFR within mGFR±30%, good agreement in CKD staging between eGFR and mGFR. The original Schwartz equation produced eGFR with large bias, poor precision and accuracy. The validated equations to estimate GFR in our patients are the updated Schwartz equation, which is simple for bedside use, the Filler equation and the CKiD equation, which provide more accurate eGFR. The original Schwartz equation should not be applied to estimate GFR in Chinese children with kidney injuries.