Zhonghua Nei Ke Za Zhi
· 2025 Jun · PMID 40484823
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A retrospective analysis was conducted on the clinical characteristics, renal pathology, genetic testing, and treatment of five patients -two males and three females-diagnosed with primary atypical hemolytic uremic syndr...A retrospective analysis was conducted on the clinical characteristics, renal pathology, genetic testing, and treatment of five patients -two males and three females-diagnosed with primary atypical hemolytic uremic syndrome (aHUS) in the Department of Nephrology at the Affiliated Hospital of Qingdao University from February 2022 to June 2024. The patients' ages at disease onset ranged from 14 to 29 years. Four patients experienced prodromal infection symptoms. At disease onset, serum creatinine levels ranged from 168.5 to 1 230.2 μmol/L. All patients presented with hematuria, proteinuria, hypertension, non-immune hemolytic anemia, thrombocytopenia, elevated lactate dehydrogenase (LDH), and fragmented red blood cells in peripheral blood (0.5%-6.0%). Serum haptoglobin levels were below the normal lower limit in all cases. Four patients demonstrated decreased serum complement C3, while one maintained normal serum complement C3 throughout the course of the disease. One patient exhibited serum factor H concentrations below the normal lower limit. Another patient tested positive for anti-factor H antibodies. Renal biopsies were performed on four patients. Electron microscopy revealed typical acute-phase pathological features of aHUS in three cases, including glomerular endothelial cell swelling and widened subendothelial spaces. One patient demonstrated ischemic and atrophic changes in the glomerular capillaries, while another had concurrent membranous nephropathy. Whole-exome high-throughput sequencing related to aHUS was performed in all five patients, revealing heterozygous gene mutations in each case. Complement-related gene mutations, typically occurring in a heterozygous state, are prevalent in aHUS patients. The eight heterozygous gene variations identified in this study were absent from existing databases of known aHUS-associated pathogenic mutations. Four patients received eculizumab treatment at varying time points following diagnosis, resulting in differing clinical outcomes. The patient positive for anti-factor H antibodies was treated with rituximab. The patient with membranous nephropathy initiated combination therapy with rituximab and eculizumab after six months of eculizumab monotherapy. Following treatment, all five patients achieved complete cessation of intravascular mechanical hemolysis, with normalization of LDH and platelet levels, as well as varying degrees of renal function recovery. From a pathophysiological perspective, the timely administration of the complement C5 inhibitor eculizumab can rapidly induce clinical remission, reduce the incidence of end-stage renal disease, and improve prognosis in patients with aHUS.
Jia G, Ding DW, Fang YQ
… +4 more, Li T, Cui LN, Shang YL, Han Y
Zhonghua Nei Ke Za Zhi
· 2025 Jun · PMID 40484822
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To investigate the effect of thrombocytopenia and coagulation dysfunction on bleeding complications in patients undergoing percutaneous liver biopsy. The clinical, laboratory, and demographic data of patients undergoing...To investigate the effect of thrombocytopenia and coagulation dysfunction on bleeding complications in patients undergoing percutaneous liver biopsy. The clinical, laboratory, and demographic data of patients undergoing percutaneous liver biopsy at the First Affiliated Hospital of Air Force Medical University from January 2005 to January 2024 were retrospectively analyzed. The incidence of bleeding was recorded. Univariate and multivariate logistic regression analyses were used to assess the effects of thrombocytopenia and coagulation dysfunction on the risk of postoperative bleeding. Furthermore, we assessed the bleeding risk in patients with autoimmune hepatitis. A total of 2 885 liver perforations were performed in 2 364 patients, 98.4% of whom had an autoimmune liver disease. There were 27 cases of postoperative bleeding (0.9%). The univariate logistic regression analysis showed that platelet count (PLT)(<0.05, =0.975), coagulation dysfunction (international normalized ratio; INR)(<0.05, =6.954), and cirrhosis (<0.05, =3.857) were associated with bleeding. The multivariate logistic regression analysis showed that PLT was an independent risk factor for bleeding (<0.05, =0.975). PLT scores of 40×10/L and 65×10/L can classify the bleeding risk of patients with thrombocytopenia into high, medium, and low risk. There was no difference in the risk of bleeding between the 40×10/L<PLT≤50×10/L and 50×10/L<PLT≤99×10/L groups (>0.05, =0.10). The risk of bleeding in the 31×10/L≤PLT≤40×10/L group was higher than that in the 40×10/L<PLT≤99×10/L group (<0.05, =11.50) and was 0 in the 19×10/L≤PLT≤30×10/L group. The risk of bleeding in the 1.15<INR≤1.5 group was higher than that in the INR≤1.15 group, and the risk of bleeding was 0 when 1.5<INR≤2.5. Compared with other liver diseases, the risk of bleeding was not increased in patients with autoimmune hepatitis (>0.05, =0.10). Furthermore, the bleeding cases were considered mild as the bleeding stopped after pressure hemostasis. PLT is an independent risk factor for bleeding after hepatocentesis. When the PLT is >40×10/L, hepatocentesis can still be performed safely with appropriate management measures.
Gao JW, Peng Z, Liu Y
… +3 more, Yu HX, Wu Y, Tian XP
Zhonghua Nei Ke Za Zhi
· 2025 Jun · PMID 40484821
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To determine the correlation between serum soluble CD146 (sCD146) levels and disease activity in patients with systemic vasculitis and the potential of sCD146 as a novel biomarker. We recruited 304 patients from the sys...To determine the correlation between serum soluble CD146 (sCD146) levels and disease activity in patients with systemic vasculitis and the potential of sCD146 as a novel biomarker. We recruited 304 patients from the systemic vasculitis cohort at Peking Union Medical College Hospital from July 2013 to December 2022. The cohort comprised 200 patients with Takayasu arteritis (TAK) and 104 with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The patient's demographic and clinical data, including age, sex, disease duration, disease type, laboratory results, and disease status, were extracted from the database. The serum sCD146 concentration was measured using a sandwich enzyme-linked immunosorbent assay (ELISA). Continuous variables were presented as mean±standard deviation if normally distributed, with between-group comparisons conducted using the -test. For non-normally distributed data, median (,) was used, and comparisons between groups were performed using the Mann-Whitney test. Categorical data were expressed as percentages, and comparisons between groups were conducted using the Chi-square test or Fisher's exact test,as appropriate. Kendall's tau-b's rank correlation coefficient was calculated to evaluate the correlation between sCD146 and variables associated with systemic vasculitis. A two-sided value <0.05 was considered statistically significant. Serum sCD146 levels were significantly lower in patients with active disease compared to those in remission in both cohorts [TAK: 246 (218, 287) vs. 277 (230, 322) μg/L, =-2.58, =0.010; AAV: (301±90) vs. (344±81) μg/L, =-2.56, =0.007]. Serum sCD146 levels were positively correlated with age and disease duration (TAK: =0.09, 0.12, =0.040, =0.009; AAV: =0.28, 0.15, <0.001, =0.020). In patients with TAK, sCD146 levels were negatively correlated with IL-6, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and disease activity status (=-0.17, -0.18, -0.16, -0.16; =0.001, <0.001, =0.003, =0.010). In patients with AAV, sCD146 levels were negatively correlated with platelet count (PLT),disease activity status,and the Birmingham Vasculitis Activity Score (=-0.36, -0.27, -0.27; <0.001, =0.007, 0.001). Serum sCD146 levels were significantly lower in patients with active systemic vasculitis than in remission, displaying a negative correlation with disease activity. These findings suggest that sCD146 has potential as a novel biomarker for assessing disease activity in systemic vasculitis.
Zhonghua Nei Ke Za Zhi
· 2025 Jun · PMID 40484820
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To investigate the correlation between the neutrophil-to-lymphocyte ratio (NLR), monocyte-to-high-density lipoprotein cholesterol ratio (MHR), and cardiovascular calcification in patients on maintenance hemodialysis (MHD...To investigate the correlation between the neutrophil-to-lymphocyte ratio (NLR), monocyte-to-high-density lipoprotein cholesterol ratio (MHR), and cardiovascular calcification in patients on maintenance hemodialysis (MHD), and to evaluate their predictive value for cardiovascular calcification. This retrospective case-control analysis was conducted on the clinical data of 362 patients with chronic kidney disease who underwent regular hemodialysis for >3 months at the Hemodialysis Center of the Second People's Hospital of Hefei from December 2018 to December 2023. Patients were divided into a cardiovascular calcification group (216 cases) and a cardiovascular non-calcification group (146 cases). The cardiovascular calcification group was further categorized according to different calcification sites, with 69 cases in the vascular calcification group, 79 in the valve calcification group, and 68 in the vascular and valve calcification group. Spearman correlation analysis was used to assess the correlation between cardiovascular calcification and various indicators. Risk factors for cardiovascular calcification in patients with MHD were analyzed using binary logistic regression analysis. The predictive value of the NLR and MHR for cardiovascular calcification was analyzed using the receiver operating characteristic (ROC) curve. This study enrolled 362 cases, including 233 males and 129 females aged 29-89 years. Age, the NLR, and the MHR were positively correlated with cardiovascular calcification in patients on MHD (=0.338, 0.383, and 0.391, respectively, all <0.05). In contrast, serum magnesium was negatively correlated with cardiovascular calcification (=-0.169, <0.05). Age (=1.063, 95% 1.036-1.092, <0.001), male sex (=2.017, 95% 1.104-3.685, =0.023), neutrophil count (=1.737, 95% 1.326-2.276, <0.001), the NLR (=1.722, 95% 1.310-2.263, <0.001), and the MHR (=1.352, 95% 1.153-1.586, <0.001) were identified as independent risk factors for cardiovascular calcification in patients on MHD. Serum magnesium (=0.034, 95% 0.001-0.797, =0.036) was a protective factor. The combined area under the curve (AUC) of the NLR and MHR was the largest (AUC=0.804, 95% 0.759-0.850); the AUC for the NLR and MHR used alone was 0.725 (95% 0.672-0.779) and 0.730 (95% 0.677-0.783), respectively. The MHR, and the NLR are independent risk factors for cardiovascular calcification in patients with MHD. The combination of the MHR and NLR has a greater clinical predictive value for cardiovascular calcification.
Zhang Q, Wang H, Li XL
… +9 more, Miao M, Ma HX, Shen YY, Wei N, Zou K, Su WX, Yu JQ, Wu DP, Liu LM
Zhonghua Nei Ke Za Zhi
· 2025 Jun · PMID 40484819
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To investigate the infection rate of cytomegalovirus (CMV) and Epstein-Barr virus (EBV) in patients with severe aplastic anemia (SAA) after intensive immunosuppressive therapy in combination with a thrombopoietin recepto...To investigate the infection rate of cytomegalovirus (CMV) and Epstein-Barr virus (EBV) in patients with severe aplastic anemia (SAA) after intensive immunosuppressive therapy in combination with a thrombopoietin receptor agonist (lST+TPO-RA) as well as assess the clinical impact of treatment. A retrospective, case series study was undertaken involving patients with SAA who were admitted to Soochow Hopes Hematonosis Hospital, The First Affiliated Hospital of Soochow University, and Zhengzhou Third People's Hospital from June 2022 to February 2025. Thirty patients with complete CMV and EBV monitoring data after IST+TPO-RA treatment were enrolled. The first activation time of CMV and EBV, the maximum viral load, the first negative conversion time, and blood routine tests within 3 days before CMV and EBV positivity, during the positive period, and within 3 days after turning negative were recorded. The patients were followed up for 9 months after the completion of IST. One-way analysis of variance was used to compare the changes of blood routine before and after virus positivity and after turning negative. The test was used to compare the viral infection rate and the therapeutic effect of IST between the two groups. The 30 SAA patients comprised 15 males and 15 females with an average age of (40.0±16.9) years. Of the 30 patients, 18 (60.0%) were infected with CMV and 6 (20.0%) with EBV. Among them, 17 cases received rabbit anti-human thymocyte immunoglobulin (r-ATG) treatment (r-ATG group), 13 cases received porcine anti-human lymphocyte immunoglobulin (p-ALG) treatment (p-ALG group). The CMV infection rate was significantly higher in the r-ATG group than in the p-ALG group (15/17 vs. 3/13, =13.03, <0.001); meanwhile, the rate of EBV infection was only slightly higher in the r-ATG group than in the p-ALG group, and the difference did not reach statistical significance (5/17 vs. 1/13, =2.17, =0.196). In patients infected with CMV, neutrophil, hemoglobin, and platelet counts were significantly decreased during the infection phase, followed by significant increases after CMV clearance (=14.48, 11.38, 4.73; all <0.05). No significant differences in treatment efficacy were found between the r-ATG and p-ALG groups at 3, 6, and 9 months post-IST (all >0.05). This preliminary study showed that the incidence of CMV and EBV infection in patients with SAA increased after IST, with CMV infections occurring significantly more frequently than EBV infections. The CMV infection rate was significantly higher in patients treated with r-ATG than in those receiving p-ALG. CMV infection was associated with notable alterations in hematological parameters, highlighting the need for close clinical monitoring.
Xia Y, He J, Gu WY
… +27 more, Jia T, Lu TX, Li YL, Zhou JH, Li BZ, Hua HY, Liu P, Miao YQ, Cheng YX, Xie XY, Zhang YP, Wu WZ, Jia ZX, Lu XZ, Wang CL, Yu L, Xu M, Shi JN, Chen WF, Zhuang WC, Qian Z, Qian J, Ni HW, Chen YF, Shen QD, Li JY, Shi WY
Zhonghua Nei Ke Za Zhi
· 2025 Jun · PMID 40484818
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To summarize the clinical characteristics of elderly patients with stage Ⅰ diffuse large B-cell lymphoma (DLBCL) and analyze the factors associated with prognosis. A case series study was conducted by retrospectively co...To summarize the clinical characteristics of elderly patients with stage Ⅰ diffuse large B-cell lymphoma (DLBCL) and analyze the factors associated with prognosis. A case series study was conducted by retrospectively collecting clinical data from patients aged over 60 years with newly diagnosed stage Ⅰ DLBCL across 20 medical centers in Jiangsu Province, China, between June 2010 and April 2023. The involved site, classification and treatment plan were summarized. The primary endpoints were progression-free survival (PFS) and overall survival (OS). Statistical analyses were performed using the Kaplan-Meier method, and Cox regression model. The study included 255 patients with a median age of 69 years, of whom 130 (51.0%) were male, 66 (25.9%) were aged ≥75 years and 26 (10.1%) had a high Charlson Comorbidity Index (CCI) score of ≥2. Extranodal involvement was observed in 163 (63.9%) patients, with the stomach (37.4%, 61/163), intestine (19.0%, 31/163), testes (11.0%, 18/163), and breast (7.4%, 12/163) being the most frequently affected sites. The non-germinal center B-cell (non-GCB) subtype was prevalent in 63.7% of patients (142/223), with no significant difference between the nodal and extranodal groups (=0.681). Furthermore, 73.9% (184/249) and 11.7% (29/249) of patients received the R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) and R-miniCHOP regimen, respectively. The overall 3-year PFS rate was 81.5%, and the 3-year OS rate was 85.6%. Patients aged ≥75 years (=2.910, 95% 1.565-5.408, =0.001) and/or with a CCI score ≥2 (=2.324, 95% 1.141-4.732, =0.020) had a significantly poorer PFS. Incorporating age ≥75 years and CCI score ≥2 into the stage-modified international prognostic index (sm-IPI) can better stratify the prognosis of elderly patients with stage Ⅰ DLBCL. The 3-year PFS rate was 48.7% in the high-risk group versus 85.7% in the low-risk group (<0.001). Our findings show that the elderly patients with stage Ⅰ DLBCL were predominantly characterized by extranodal involvement (particularly in the stomach and intestinal tract) and non-GCB subtype. Age ≥75 years and CCI ≥2 were identified as independent prognostic factors. The newly established sm-IPI-75-CCI incorporating these factors demonstrated superior prognostic discrimination compared to conventional risk assessment systems.