Arthritis, Juvenile ; therapy ; Child ; Humans ; Macrophage Activation Syndrome ; therapy ; Plasmapheresis

Objective To investigate the expression of interferon-induced protein 44 (IFI44) gene in the leukocytes of the peripheral blood samples from patients with systemic lupus erythematosus (SLE), and to evaluate the relationship between the expression level and disease activity. Methods Mononuclear cells in the peripheral blood samples from 100 SLE patients were compared with those of 40 disease controls and 40 healthy donors (HD) and the expression of the IFI44 was evaluated by quantitative real-time PCR.Comparisons between groups were performed with ANOVA, and the correlation analysis between the level of expression was higher in SLE patients than disease controls and healthy donors (26.8±5.3, 7.4±2.7, 5.2±2.0,respectively) (P=0.0012, P=0.005), but no difference was found between disease controls and healthy donors. Mild disease activity and the SLE patients with stable disease (63.1±22.4, 28.0±7.2, 9.2±1.8, respectively)and 24 hours urine protein level (r=0.42, P=0.000). Conclusion IFI44 is demonstrated to be highly expressed in SLE patients. The level of IFI44 may be a promising candidate biomarker for identifying SLE activity.

Objective:To investigate the serum level of C-C chemokine ligand 19 (CCL19)and its clinical significance in rheumatoid arthritis.Methods:The serum CCL19 levels in both rheumatoid ar-thritis (RA)patients and health controls were detected by ELISA.The proportion of peripheral blood B cells and memory B cell subsets were also detected in some patients.Then the clinical and laboratory data of the patients were collected.The CCL19 levels in patients with different clinical features were analyzed. And the correlation between the clinical data,laboratory parameters,B cell subsets proportion and serum CCL19 levels were also analyzed.Independent samples t test,paired t test,Pearson and Spearman corre-lation were used for statistical analysis.Results:The levels of CCL19 was higher in the RA patients than the health controls (P <0.05).The serum CCL19 levels were decreased in the RA patients who accep-ted disease-modifying anti-rheumatic drugs (DMARDs)treatment for 6 months (P <0.001 ).Serum CCL19 levels were correlated with the titers of both rheumatoid factor (RF)and anti-cyclic citrullinated peptide (CCP)antibody (r =0.42,P =0.002;r =0.33,P =0.013),but not with erythrocyte sedi-mentation rate (ESR),C-reactive protein (CRP)and disease activity score in 28 joints (DAS28)(P >0.05).The levels of CCL19 were higher in the serum positive (RF and anti-CCP antibody)patients,but there were no differences between low and high disease activity RA,as well as early and non-early RA. There was no correlation between the serum CCL19 levels and the proportion of B cells as well as memory B subsets.All the proportion of peripheral blood CD27 + memory B cell subsets in RA was lower than the healthy controls,including CD27 +IgD +,CD27 +IgD - and CD27 + B cells.Conclusion:The increased serum CCL19 levels in RA patients are associated with the activity of B cells,so CCL19 might predict whether the RA type is a B cell mediated RA,and specify the treatment directions for the rheumatologist.

BACKGROUND:Hemiarthroplasty is a proven method for reconstruction of the hip joint function, especialy for the elderly patients who cannot tolerate the total hip arthroplasty. However, for the patients with osteoporosis, there are stil more controversial in clinical practice about using cemented or cementless femoral prosthesis for hemiarthroplasty. OBJECTIVE: To observe the repair effect of cemented or cementless femoral prosthesis when were used for hemiarthroplasty in patients with osteoporosis. METHODS:The clinical data from 105 patients treated with hemiarthroplasty were retrospectively analyzed. Osteoporosis was diagnosed in these patients using the dual energy X-ray absorptiometry before surgery.Patients were divided into bone cement group (n=56) and cementless group (n=49) depending on different types of femoral stem prosthesis. The difference of efficacy between cemented or cementless femoral prosthesis when were used for hemiarthroplasty in patients with osteoporosis was analyzed by comparing the operative time, volume of drainage, post-operative Harris scores of hip joint function and radiographic assessment (prosthesis subsidence, osteolysis, severe stress shielding, heterotopic ossification) during the folow-up after the replacement. RESULTS AND CONCLUSION:In the process of hemiarthroplasty in patients with osteoporosis, the operative time in the cement group was significantly longer than that in the cementless group (P < 0.05). However, there was no significant difference in the postoperative volume of drainage between these two groups (P > 0.05). At the 1, 3, 6 and 12 of folow-up after replacement, there were no significant differences in the Harris score of hip joint function, total effective rate and radiographic assessment between these two groups (P> 0.05). These results suggest that in the process of hemiarthroplasty in patients with osteoporosis, the clinical efficacy of cementless femoral prosthesis is similar to the cement femoral prosthesis, therefore two prostheses can al be selected; however, the operative time of the cement femoral prosthesis is longer than the cementless femoral prosthesis, so we can give priority to the cementless femoral prosthesis for patients with osteoporosis who can’t tolerate a long operative time because of the poor basic condition.

Objective To investigate the common initial clinical presentations of primary Sj(o)gren's syndrome (pSS) with pulmonary complications,and to explore the differences between patients with extraglandular manifestations at disease onset (EGM) and those with glandular manifestations at disease onset (GM).Methods A total of 1 341 hospitalized SS patients from 2003 to 2012 were retrospectively reviewed.Of them,102 hospitalized patients with pSS'associated lung disease were analyzed and included.Case control study was performed to explore the differences between the EGM group and the GM group.Results Fifty-one percent of patients were presented with EGM at onset,with significantly shorter disease duration [36 (12,156) m vs 102 (48,159) m,x2=-2.41,P=0.016].Although the mean diagnose time was similiar,only 4％ of the EGM group could be confirmed the pSS diagnose at onset,which was significantly less frequently than that of the GM group (34％,22=15.29,P＜0.01).Case control study revealed that hyperglobulinemia,elevated RF titers and anti-SSA and/or anti-SSB test positive were less predominant in the EGM group [IgG 16(12,21) g/L vs 21 (15,28) g/L,x2=-2.15,P=0.032;22 (20,171) U/ml vs 104 (20,238) U/ml,x2=-l.98,P=0.048;33％ vs 72％,x2=15.78,P＜0.01].The predicted value of TLC and FVC were lower [(87±23)％ vs (97±20)％,x2=-1.96,P=0.050;(8±28)％ vs (100±27)％,x2=-1.70,P=0.089] and HRCT score was higher in EMG group [12(88,15) vs 8(5,13),x2=-1.82,P=0.070].Conclusion EMG at onset is the common initial manifestation of pSS'associated lung involvement.Pulmonary complication is more progressively and severe than those with MG at onset.Anti'SSA positive,elevated RF titer and hyperglobulinemia are not predominant for patients with EMG at onset.

Objective To explore the feasibility and clinical effect of laparoscopic radical cystectomy with intracorporeal Xing's orthotopic neobladder.Methods Forty-one patients who underwent laparoscopic radical cystectomy with intracorporeal Xing's orthotopic neobladder from July 2013 to August 2019.There were 31 cases performed in Beijing Chaoyang hospital and 10 cases in National Cancer Center.Mean age was 59 (range 44-78) years,mean BMI was 25.3 (range 20.1-34.7) kg/m2,and mean CCI was 3 (range 2-6).No urethral stricture or urinary incontinence was found by preoperative examination.No distant metastasis was identified by bone scans,chest X-ray and sonography.Cystoscopy or TURBT was performed on all patients and biopsy was taken to confirm the diagnosis.Preoperative pathology showed 30 cases (73.2％) of MIBC,9 cases of NMIBC (22.0％) and 2 cases (4.9％) of in-situ cancer.Laparoscopic radical cystectomy and lymphadenectomy were performed under general anesthesia.Urinary diversion was completed in the peritoneal cavity,by intercepting the terminal ileum about 60 cm,and taking the proximal ileum 10 cm as input loop on the right side with proximal to distal way,and the middle 40 cm ileum was detubated.After u-shaped suture,the ileum was folded back and stitched into a sphere building a novd orthotopic neobladder with bilateral isoperistaltic afferent limbs.The prognosis of perioperative data and postoperative satisfaction regarding continence were analyzed,continence was defined as 0-1 pad/day.The 41 patients were divided into two groups to compare the difference in term of operation time and blood loss between the first 21 patients and the last 20 patients.Results Mean total operative time was 324.9 mins (range 210-480) mins,and mean estimated blood loss was 177.6(range 50-700) ml.There were significant statistical differences in term of total operation time,construction time and blood loss between the first 21 patients and the next 20 patients (P ＜ 0.05).Postoperative pathological results were urothelial carcinoma in 40 cases (2 in situ carcinoma) and small cell carcinoma in 1 case.Mean number of dissected lymph nodes was 19 (range 11-58),with 7 cases(17.1％)of positive lymph nodes,and 3 cases(7.3％) had positive surgical margin.At a mean follow up of 17.6 (range 2-64) months,36 patients (87.8％) survived,including 2 patients (4.9％) with metastasis and 1 patient (2.4％) with recurrence,and 5 cases (12.2％)died.All patients were able to urinate without catheterization.Thirty-seven patients (90.2％) were satisfied with voiding control during the daytime (0-1 urinal pad),and 29 patients (70.7％) were satisfied with voiding control at nighttime (0-1 urinal pad) by the follow-up 12 months after the operation.Conclusions Total laparoscopic radical cystectomy combined with Xing's orthotopic ileum neobladder is a simple method with fewer postoperative complications and a satisfactory continence rate.

BACKGROUND: Human neutrophil lipocalin (HNL) has been used extensively to differentiate acute bacterial infection from febrile diseases as a biomarker to reflect the activation of the neutrophil. The serum HNL levels in the adult-onset Still's disease (AOSD) patients with and without infection, as well as the healthy controls (HCs), were analyzed statistically in this study to evaluate the value of HNL for the diagnosis of AOSD. METHODS: A total of 129 AOSD patients were enrolled, from whom blood samples were drawn and the AOSD diagnosis was confirmed through the review of the medical records, where the systemic score, demographic characteristics, clinical manifestations, and laboratory parameters were also collected for the patients; in addition, a total of 40 HCs were recruited among the blood donors from the healthcare center with the relevant information collected. The HNL test was done for the blood samples with the enzyme-linked immunosorbent assay and the analyses were done for the correlations of HNL with clinical manifestations and diagnostic effectiveness. RESULTS: The serum HNL increased significantly in the patients with only AOSD as compared with that in the HCs (139.76 ± 8.99 ng/mL vs . 55.92 ± 6.12 ng/mL; P  < 0.001). The serum HNL level was correlated with the white blood cell (WBC) count ( r  = 0.335, P  < 0.001), neutrophil count ( r  = 0.334, P  < 0.001), erythrocyte sedimentation rate ( r  = 0.241, P  = 0.022), C-reactive protein ( r  = 0.442, P  < 0.0001), and systemic score ( r  = 0.343, P  < 0.0001) in the AOSD patients significantly. Patients with fever, leukocytosis ≥15,000/mm 3 , and myalgia in the HNL-positive group were observed relatively more than those in the HNL-negative group ( P  = 0.009, P  = 0.023, and P  = 0.007, respectively). HNL was a more sensitive indicator than ferritin and C-reactive protein (CRP) to differentiate the AOSD patients with bacterial infection from AOSD-only patients, and the Youden index was 0.6 for HNL and 0.29 for CRP. CONCLUSION Serum HNL can be used as a biomarker for the diagnosis of the AOSD, and HNL is also observed to be associated with the disease activity.
Adult ; Humans ; Still's Disease, Adult-Onset/diagnosis* ; C-Reactive Protein/metabolism* ; Neutrophils/metabolism* ; Clinical Relevance ; Biomarkers ; Bacterial Infections