To provide scientific guidance and standardization for perioperative clinical nursing practice in patients undergoing percutaneous renal biopsy, relevant evidence on percutaneous renal biopsy nursing care was systematically retrieved and synthesized through an evidence-based approach. After two rounds of Delphi expert consultation and expert panel discussions, expert consensus on perioperative clinical nursing standards for patients undergoing percutaneous renal biopsy (hereinafter referred to as the "Consensus") was developed. The Consensus includes three primary themes: preoperative care, intraoperative care, and postoperative care, encompassing 21 secondary themes. It is characterized by scientific rigor, practical applicability, and comprehensiveness, and serves as a valuable reference and guide for clinical nursing professionals across medical institutions.

To provide scientific guidance and standardization for perioperative clinical nursing practice in patients undergoing percutaneous renal biopsy, relevant evidence on percutaneous renal biopsy nursing care was systematically retrieved and synthesized through an evidence-based approach. After two rounds of Delphi expert consultation and expert panel discussions, expert consensus on perioperative clinical nursing standards for patients undergoing percutaneous renal biopsy (hereinafter referred to as the "Consensus") was developed. The Consensus includes three primary themes: preoperative care, intraoperative care, and postoperative care, encompassing 21 secondary themes. It is characterized by scientific rigor, practical applicability, and comprehensiveness, and serves as a valuable reference and guide for clinical nursing professionals across medical institutions.

Objective:To analyze the clinical and pathological data of 15 patients with light chain amyloidosis initially diagnosed with other kidney diseases, and identify possible misdiagnosis reasons.Methods:It was a retrospective observational study. The clinical and pathological data of 15 patients, whose initial kidney biopsies failed to diagnose light chain-amyloidosis but were confirmed by a subsequent kidney biopsy or pathology consultation at Peking University First Hospital from January 2010 to December 2022 were collected. The results of immunofluorescence, Congo red staining, and electron microscopy of two renal biopsies were analyzed.Results:The median age of 15 patients was 56 years old, with a male-to-female ratio of 7∶8. The main clinical manifestation was massive proteinuria with normal kidney function, and there were 10 cases presenting as nephrotic syndrome. The initial diagnosis based on the first kidney biopsy included minimal change disease (8 cases), IgA nephropathy (3 cases), membranous nephropathy (3 cases), and type Ⅲ collagen glomerulonephritis (1 case). M proteinemia was not evaluated in 13 patients during the first kidney biopsy. Light chain immunofluorescence staining was not performed in 12 cases. Congo red staining was not performed in 13 cases. All fifteen patients received glucocorticoids combined with immunosuppressive therapy after their initial diagnosis, and 5 patients developed severe infection. After 12.0 (7.5, 20.0) months of treatment, none of them achieved clinical remission. Thirteen had evidences for M protein before the second kidney biopsy. The renal tissues of all patients underwent immunofluorescence light chain examination, Congo red staining, and immunoelectron microscopy examination when necessary. The repeat kidney biopsies of 14 cases and pathology consultation of one case consistently indicated light chain-amyloidosis. The kidney tissues in 13 cases were confirmed to be light chain restricted, 11 cases by immunofluorescence, and 2 cases by immune electron microscopy. After diagnosis of light chain-amyloidosis, all patients received targeted plasma cell therapy except for 1 patient lost to follow-up, 6 patients achieved hematologic remission, 5 patients achieved renal remission, 1 patient entered end-stage renal disease, and 3 patients died.Conclusions:In middle and elderly-aged patients with nephrotic syndrome, if conventional immunosuppressive therapy yields unsatisfactory results, it is crucial to focus on identifying evidences of monoclonal immunoglobulinemia, if necessary, kidney biopsy should be actively repeated. Kidney biopsy pathology should include comprehensive examinations such as light chain immunofluorescence, Congo red staining, and electron microscopy to avoid misdiagnosis of light chain-amyloidosis.

The paper reports a case of coenzyme Q10 deficiency nephrotic syndrome associated with coenzyme Q2 gene mutation and reviews the literature on this topic. The patient presented with hematuria, proteinuria, and a diminution of vision as clinical manifestations. But the proteinuria was not relieved after sufficient doses of glucocorticoids for over 2 months. The patient′s birth history was unremarkable, and his parents were both healthy and not consanguineous. Whole exome sequencing revealed that the patient had a mutation of coenzyme Q2 gene at c.973A>G(p.T325A) and c.517C>T(p.R173C). Combined with renal biopsy pathology, the patient was diagnosed with hereditary nephropathy and started the supplements of coenzyme Q10 after stopping glucocorticoid treatments immediately. After 5 weeks of therapy, the patient′s 24-h urine protein quantification decreased from 6.01 g to 1.53 g.

Objective:To summarize and analyze the clinicopathological characteristics of patients with DNAJ heat shock protein family member B9 (DNAJB9)-positive fibrillary glomerulonephritis (FGN).Methods:The clinical and pathological data of 5 patients with DNAJB9-positive FGN diagnosed in Peking University First Hospital from January 2011 to January 2021 were retrospectively collected and analyzed.Results:Among the 5 patients, the female to male ratio was 4∶1, and the median age was 29 years old (24-71 years old). The clinical manifestations included 2 cases with nephrotic syndrome and 3 cases with proteinuria. One patient had gross hematuria, and 4 cases had mild microscopic hematuria. None of the 5 patients had evidence of monoclonal gammopathy. The renal pathological pattern of FGN showed mesangial-proliferative glomerulonephritis, mesangial nodular sclerosis, membranoproliferative glomerulonephritis, and atypical membranous nephropathy. Crescents formation could be accompanied. Immunofluorescence staining showed smudgy and granular IgG and C3 deposition in the mesangial region and capillary wall, and the subtypes of IgG were mainly IgG1 and IgG4. Under electron microscopy, fibrillary deposits with a diameter of 8-30 nm were observed in the mesangial and subendothelial area, accompanied by deposition in basement membrane and occasionally subepithelial area. The renal prognosis of FGN patients was poor. One patient entered end-stage renal disease within one week, and another patient entered end-stage renal disease within one year despite immunosuppressant therapy in 2 cases with nephrotic syndrome at onset. One patient had worsening proteinuria despite renin-angiotensin system (RAS) blocker treatment. Two patients achieved complete renal remission and stable renal function after RAS blocker treatment.Conclusions:Most FGN patients in China are young people. The main clinical manifestations are proteinuria or mild microscopic hematuria. The diagnosis depends on the discovery of fibrillary deposits in the mesangial area and subendothelial area with a diameter of about 10-30 nm under the electron microscope. DNAJB9 protein immunohistochemical staining can be used as an important marker for the diagnosis of FGN. The prognosis of FGN kidney is poor, and there is no effective targeted treatment option now.

Objective:To investigate the clinicopathological characteristics of renal leukocyte chemotactic factor 2 amyloidosis (ALECT2).Methods:The patients with renal ALECT2 diagnosed by renal biopsy in Peking University First Hospital, Shanxi Medical University Second Hospital and Shanxi Bethune Hospital from January 2001 to October 2021 were retrospectively enrolled. According to whether the patients had concurrent glomerular diseases, they were classified into two groups: isolated ALECT2 group and ALECT2 with concurrent renal diseases group. Clinicopathological data of the two groups were compared. Light microscopy, immunofluorescence and immunoelectron microscopy were applied to investigate pathological characteristics of renal tissues. Mass spectrometry was used to analyze the composition of renal amyloid deposits. Gene sequencing was employed to detect the leukocyte chemotactic factor 2 ( LECT2) gene sequence in peripheral blood of the patients. Results:Sixteen patients with ALECT2 were enrolled in this study and nine of them had concurrent renal diseases. The age of 16 patients was (65.00±8.45) years old. The sex ratio of males to females was 7 to 9. Most of patients were Han ethnicity (15/16). Eight patients came from Shanxi province. Fifteen patients presented with varying degree of proteinuria [2.16(1.07, 4.72) g/24 h]; 5 patients had nephrotic syndrome; 11 patients had renal insufficiency; 12 patients had microscopic hematuria. Part of patients also had hypertension (12/16) and diabetics (6/16). Compared with isolated ALECT2, the ALECT2 group with concurrent renal diseases had a higher proportion of nephrotic syndrome (5/9 vs 0/7, P=0.034). Renal biopsy results showed that all patients (16/16) had amyloid deposits in the interstitium of renal cortex with varying degree of inflammatory cell infiltration and fibrosis, and glomeruli (12/16) and arterioles (14/16) were involved by amyloid deposits. The amyloid deposits were strongly congophilic and immunohistochemistry for LECT2 was positive. By semi-quantitative analysis, the proportions of glomerular and overall amyloid loads in ALECT2 with concurrent renal diseases group were lower than those in isolated ALECT2 group (both P<0.05). Electron microscopy revealed randomly oriented and non-branching fibrils with a diameter of 8-12 nm. The LECT2 peptides were detected by mass spectrometry in renal amyloid deposits of 8 patients, and homozygous G allele of LECT2 was found in 7 patients by gene sequencing. Complete follow-up data of 13 patients showed that 2 patients died, 1 patient developed end-stage renal disease at the time of renal biopsy, and most of the rest patients had stable renal function (8/10). Conclusions:Patients with renal ALECT2 mainly present with proteinuria, along with a high incidence of renal insufficiency, microscopic hematuria, and concurrent renal diseases. The pathologic feature is the preferential deposition of amyloid in renal cortical interstitium.

Objective:To provide clinical experience for the diagnosis and treatment of spontaneous renal hemorrhage through retrospective analysis of clinical features, imaging manifestations, underlying causes, treatment , and prognosis of spontaneous renal hemorrhage. Methods:By searching hospital information system, medical records scanning system, department of the interventional vascular surgery registry system, and picture archiving and communication systems, the patients with spontaneous renal hemorrhage admitted to Peking University First Hospital between January 1, 2000 to April 10, 2020 were enrolled. The clinical manifestations, investigations, imaging features, treatment, and prognosis of patients were retrospectively reviewed. The diagnostic efficiency and the accuracy of etiological diagnosis of renal hemorrhage by imaging examinations such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and angiography were compared and evaluated.Results:A total of 50 patients with 51 events of spontaneous renal hemorrhage were enrolled in the study. Laboratory tests showed hemoglobin was (103.3±29.4) g/L. The most common clinical features were flank or abdominal pain (48 cases, 96.0%), fever (17 cases, 34.0%), nausea (10 cases, 20.0%), vomiting (9 cases, 18.0%), and gross hematuria (4 cases, 8.0%). Nine patients (18.0%) experienced hypovolemic shock (systolic pressure<90 mmHg). With an initial assessment of the imaging method, the diagnostic accuracy of bleeding was 98.0%(49/50), yet the accuracy of underlying causes was 56.0%(28/50). The diagnostic accuracy of bleeding was 100.0%(25/25) by non-contrast abdominopelvic CT. The most common cause of spontaneous renal hemorrhage syndrome was renal tumors (27 cases, 54.0%), among which angiomyolipoma occurred most frequently (20 cases, 40.0%). Other causes included renal cyst (10 cases, 20.0%), autoimmune diseases (4 cases, 8.0%), bleeding diathesis (3 cases, 6.0%), and idiopathic renal hemorrhage (6 cases, 12.0%). Twelve patients (24.0%) received conservative management, 29 patients (58.0%) underwent interventional embolization therapy, and 11 patients (22.0%) received nephrectomy. The success rate on first embolization therapy was 86.2%(25/29), and approximately 13.8%(4/29) required second embolization therapy or nephrectomy.Conclusions:Spontaneous renal hemorrhage has no specific clinical features and is easy to be underdiagnosed or misdiagnosed. Non-contrast CT scan has a high diagnostic value for renal bleeding. Comprehensive judgement consisting of clinical features, laboratory tests, imaging manifestations and pathological examinations should be relied on for finding the underlying causes. Prompt diagnosis and management can guarantee a better prognosis.

Objective:To investigate the clinicopathological characteristics of renal light and heavy chain amyloidosis (AHL).Methods:Ten patients with renal AHL diagnosed by renal biopsy in Peking University First Hospital and Institute of Nephrology of Peking University from January 2015 to June 2020 were enrolled. Clinicopathological data of these patients was collected and reviewed.Results:AHL typically affected older patients, with a male/female ratio of 7:3. The clinical manifestations were mainly edema and heavy proteinuria. At the same time, 7/10 of patients presented with nephrotic syndrome, 7/10 presented with microscopic hematuria, and 3/10 presented with renal insufficiency. Laboratory examinations showed monoclonal immunoglobulin in blood and urine in all patients, and IgGλ was the most common one (5/10). Decreased serum complement could be seen in some patients. The ratio of serum free κ light chain and free λ light chain was abnormal in all patients who underwent serum free light chain test. None of the 10 patients met the diagnostic criteria of multiple myeloma. Except for one of the 10 patients who was diagnosed as Waldenstrom's macroglobulinemia, the rest were diagnosed as monoclonal gammopathy of renal significance (MGRS). Bone marrow of 2/6 of patients were positive for amyloid. Cardiac involvement was confirmed in only one patient. Renal biopsy demonstrated amorphous eosinophilic material, which was Congo red positive, was deposited in glomerular mesangial area (10/10), capillary vessels (8/10), renal interstitium (9/10), peritubular capillary walls (9/10) and arterioles (8/10). This material showed apple green birefringence under polarized light. Immunofluorescence showed that single heavy chain and single light chain were positive at the same time, which was consistent with the results of mass spectrometry analysis. Ultrastructural evaluation revealed randomly oriented, non-branching fibrils with a diameter of 8-12 nm.Conclusions:Main clinical manifestations of AHL amyloidosis are edema and massive proteinuria, along with a high incidence of hematuria, a low portion of heart involvement and high frequency of whole molecule of monoclonal immunoglobulin (IgGλ dominant) by serum immunofixation electrophoresis. Renal pathology shows the commonly involved kidney compartments of amyloid deposits are glomerular capillary walls and peritubular capillary walls in patients with AHL amyloidosis.

Objective:To explore the possible correlation between allostatic load (AL) and clinical symptoms in patients with first-episode schizophrenia (FES) and AL influence upon the onset.Methods:Patients with FES (schizophrenia group, n=93) who received treatment at Beijing Huilongguan Hospital from October 2017 to July 2019 and the healthy controls (control group, n=111) recruited during the same period were enrolled in the research. Systolic blood pressure, diastolic blood pressure, resting heart rate, body mass index, waist-hip ratio, high-density lipoprotein cholesterol, total cholesterol, glycosylated hemoglobin, high-sensitivity C-reactive protein, 12-hour urine adrenaline, 12-hour urine norepinephrine, 12-hour urine cortisol, and serum dehydroepiandrosterone of all subjects were collected to calculate the AL index. The positive and negative symptom scale (PANSS) was used to assess the psychopathological symptoms of patients. Covariance analysis was used to compare the differences of AL index and the thirteen biological components between FES patients and controls. Pearson correlation analysis was used to explore the correlation between AL index and clinical symptoms, and the Logistic regression analysis was used to explore the risk factors of schizophrenia. Results:AL index among schizophrenic patients was higher than the controls (5.87±1.90 vs. 3.50±2.21, F=65.97, P<0.001). AL index was possitively correlated with PANSS positive symptoms ( r=0.25, P=0.019) and was a risk factor of schizophrenia ( OR=1.76, 95% CI: 1.47-2.11, P<0.01). Conclusions:The AL is possitively correlated with positive symptoms in the FES, and the higher AL may be one of important inducements to the schizophrenia onset.