Please wait a minute...
Table of Content
18 December 2018, Volume 50 Issue 6
For Selected: View Abstracts Toggle Thumbnails
  • Article
    Diagnosis and treatment of antiphospholipid antibody:related recurrent spontaneous abortion and analysis of therapeutic drugs and pregnancy outcome in 75 patients with antiphospholipid syndrome
    Xin yi LI,Jin xia ZHAO,Xiang yuan LIU
    2018, (6):  956-961.  doi: 10.19723/j.issn.1671-167X.2018.06.003     PMID: 30562764
    Abstract ( 1516 )   HTML ( 102 )   PDF (882KB) ( 911 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Recurrent spontaneous abortion is one of the common complications in women of childbearing age during pregnancy. The immune factor accounts for a large proportion of many causes. Antiphospholipid antibody syndrome is the most common type of acquired thrombophilia disease. Autoimmune di-seases that cause thrombosis and obstetric complications under the action of antibodies are also the most common type of immune-related recurrent abortion. At present, there is no unified opinion on the treatment of this disease, especially the treatment of immunoglobulins and other drugs like glucocorticoid. Here we reviewed the progress of diagnosis and treatment of antiphospholipid antibody-related recurrent abortions and retrospectively analyzed and summarized the drug regimens and pregnancy outcomes of this disease with pregnancy patients in our hospital. A total of 75 patients were included. According to their clinical manifestations and laboratory results, these patients were basically divided into two categories:classical antiphospholipid syndrome and non-classical antiphospholipid syndrome. The latter was further divided into serum-negative antiphospholipid syndrome and antiphospholipid antibody-related recurrent abortion patients based on their clinical manifestations and antiphospholipid antibody results. The patients were divided into four categories:aspirin + hydroxychloroquine, aspirin + low molecular weight heparin, aspirin + low molecular weight heparin + hydroxychloroquine, aspirin + hydroxychloroquine + low molecular weight heparin + low dose glucocorticoids. Among them, aspirin + hydroxychloroquine + low molecular weight heparin + low-dose glucocorticoid treatment regimen was most commonly used. Most of the patients who received the above different treatment regimens achieved full-term infants, and a small number of patients had adverse pregnancy outcomes, such as premature delivery, placental abruption, eclampsia, and fetal malformation. And adverse pregnancy outcomes also occurred in this group. It might be related to the severity of the disease and the potential adverse effects of maternal fetal. However, further statistical analysis is needed for the risk factors affecting the pregnancy outcome of this part of patients.

    Effects of integrin metalloproteinases on osteogenic differentiation
    Xia LIU,Ying ni LI,Xiao li SUN,Qing lin PENG,Xin LU,Guo chun WANG
    2018, (6):  962-967.  doi: 10.19723/j.issn.1671-167X.2018.06.004     PMID: 30562765
    Abstract ( 1077 )   HTML ( 37 )   PDF (2948KB) ( 271 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To study the effects of disintegrin and metalloproteinase (ADAM)9, 15 and 17 on the osteogenic differentiation of bone marrow mesenchymal stem cells (BMMSCs).Methods: BMMSCs of ADAM9, ADAM15, ADAM17 conditional knockout mice and wild type mice (WT) were induced and cultured. Alkaline phosphatase (ALP) activity was measured by colorimetry, early osteogenic transcription factors Runx and Osterix were detected by Real-time PCR, and mineral formation was analyzed by alizarin red staining. Results:ALP activity was lower in ADAM9 group (8.08±0.34), ADAM15 group (6.46±3.40), ADAM17 group (9.30±2.30) than that in WT group (9.44±2.50), but there was no significant difference (P>0.05). Stimulated with bone morphogenetic protein 2(BMP2),there was significant difference (P<0.05) between ADAM9 group (14.22±3.25), ADAM15 group (10.14±2.40) and WT group (20.89±3.40), and ADAM 17 group (23.56±2.50) was higher than WT group (20.89±3.40), but no significant difference (P>0.05).Similarly, cultured by osteogenic induction medium (OST), compared with WT group(12.97±1.30), ADAM9 group(9.63±1.00) and ADAM15 group(7.75±1.30)were lower,ADAM17 group (20.09±1.68) was higher, and the difference was statistically significant (P<0.05). Using stimulated culture by BMP2 and OST combined , ADAM9 group (15.75±1.30), ADAM 15 group (12.43±1.30) were less than WT group (26.15 ±1.50), while ADAM17 group (29.55±2.10) was higher than WT group were statistically significant (P<0.05). The expression of Runx2 in ADAM9 group (2.02±0.24), ADAM15 group (3.09±0.19), ADAM17 group (3.89±0.91) had no significant difference compared with WT (2.02±0.21) group (P>0.05). ADAM9 group stimulated by BMP2 (7.00±0.23), ADAM15 group (6.04±0.23) were lower than WT group (12.6±0.23), ADAM17 group (18.52±1.39) was higher than WT group (12.6±0.23), and the difference was statistically significant (P<0.05).In non-stimulating culture,there was no significant difference in Osterix expression between ADAM9 group (9.60±3.87) , ADAM17 group (12.40±3.00) and WT group (10.9±1.10, P>0.05),but in ADAM15 group (6.50±1.51) it was slightly lower than that in WT group (P<0.05). After BMP2 stimulation, ADAM9 group (39.20±3.23) and ADAM15 group (20.50±4.80) were less than WT group (60.30±5.93), while ADAM17 group (80.20±3.30) was higher than WT group (P<0.05). Alizarin red staining showed no obvious orange-red mass in the non-induction group. Local calcified nodules could be seen in the BMP2, OST, OST + BMP2 induction culture conditions in all the experimental groups, but there was no significant difference in quantitative analysis (P>0.05). Conclusion: ADAM9, 15, 17 took part in the osteogenic differentiation of BMMSCs, and provided new targets for its regulation.

    Increased receptor activator of nuclear factor kappa B ligand expressed on B10 cells in rheumatoid arthritis
    Hong jiang LIU,Xiao feng GUO,Fan lei HU,Cui ping YAN,Xiang jun CUI,Xue liang YAN,Zhan guo LI,Yuan JIA,Shu lin ONG
    2018, (6):  968-974.  doi: 10.19723/j.issn.1671-167X.2018.06.005     PMID: 30562766
    Abstract ( 1047 )   HTML ( 40 )   PDF (2090KB) ( 293 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To detect receptor activator of nuclear factor kappa B ligand (RANKL) expressed on B10 cells in rheumatoid arthritis (RA) and to evaluate the correlation between RANKL-producing B10 cells in RA and clinical features and laboratory parameters, trying to reveal the possible role of B10 cells in the pathogenesis of RA and the potential mechanism of impaired immunosuppressive capacities.Methods:25 RA patients and 20 healthy volunteers were enrolled. These RA patients did not received treatment with glucocorticoids, disease-modifying anti-rheumatic drug and biologics during the recent half of a year. The levels of RANKL-producing B10 cells were measured by flow cytometry (FCM) and polymerase chain reaction (PCR). The correlation between the frequencies of RANKL-producing B10 cells in RA and clinical data, laboratory parameters were analyzed. The role of tumor necrosis factor α (TNF-α) and interleukin 1β (IL-1β) in inducing RANKL expression in B10 cells was evaluated by in vitro stimulation assay. Independent samples t test, Pearson and Spearman correlation were used for statistical analysis.Results:B10 cells were capable of producing RANKL at a low level in health controls. The frequencies of RANKL-producing B10 cells were markedly higher in RA patients than in health controls (3.65%±1.59% vs. 2.25%±0.68%, P<0.01). The frequencies of these cells correlated po-sitively with RA tender joint counts, swollen joint counts and disease activity score in 28 joints (DAS28) (r=0.479, P=0.035;r=0.519, P=0.008;r=0.526, P=0.019). However, no correlation was found between these cells and RA patient age, disease duration, or the levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF) and anti-citrullinated peptide antibody (ACPA). After in vitro stimulation by TNF-α, but not IL-1β, B10 cells isolated from healthy donors demonstrated fundamentally upregulated expression of RANKL.Conclusion:Our studies showed the frequencies of RANKL-producing B10 cells were markedly higher in RA patients, and their frequencies were positively correlated with RA tender joint counts, swollen joint counts and DAS28. These findings suggested that B10 cells might be involved in RA bone destruction.

    Clinical significance of detection of soluble interleukin 2 receptor alpha chain in the assessment of rheumatoid arthritis disease activity
    Jia jia XU,Yan WANG,He SUN,Ru lin JIA,Xue wu ZHANG,Yang MENG,Li li REN,Xiao lin SUN
    2018, (6):  975-980.  doi: 10.19723/j.issn.1671-167X.2018.06.006     PMID: 30562767
    Abstract ( 1226 )   HTML ( 32 )   PDF (1012KB) ( 242 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To evaluate soluble interleukin-2 receptor alpha chain (sIL-2Rα, sCD25) in serum for the determination of rheumatoid arthritis (RA) activity.Methods:Peripheral blood was collected from 108 patients with RA, 39 patients with osteoarthritis (OA) and 50 healthy control subjects, and synovial fluids were from 40 patients with RA. The sera from the patients with RA, the disease control group (osteoarthritis), the healthy control group, and the synovial fluids of the RA patients were detected by enzyme-linked immunosorbent assay (ELISA).The clinical manifestations and laboratory parameters of the patients with RA were recorded and the correlation with the serum sCD25 level was analyzed.Results:The serum sCD25 concentration in RA group was (2 886±1 333) ng/L, the serum sCD25 concentration in OA group was (2 090±718) ng/L, and the serum sCD25 concentration in healthy group was (1 768±753) ng/L. The serum sCD25 level in the patients with RA was significantly higher than that in the disease controls and healthy controls (P<0.001). Sensitivity of serum sCD25 in the diagnosis of RA was 66.1% and specificity was 83.0%;serum sCD25 levels and erythrocyte sedimentation rate (r = 0.321, P = 0.001), C-reactive protein (r=0.446, P<0.001), DAS28 score (r = 0.324, P<0.001), joint tenderness count (r=0.203, P=0.024), D-dimer levels (r=0.383, P<0.001), age (r=0.24, P=0.007), IgG (r=0.207, P=0.028), HRF-IgG (r=0.345, P=0.034) showed a significant positive correlation, and disease duration (r=-0.206, P=0.021) showed a negative correlation with sCD25;In patients with rheumatoid arthritis, the positive rates of serum ESR, CRP, and sCD25 were 14.3% (2 cases), 14.3% (2 cases), and 71.4% (10 cases) in the low disease activity group. The positive rates of serum ESR, CRP and sCD25 in the moderate disease activity group were 94.2% (49 cases), 82.7% (43 cases), and 86.5% (45 cases). The positive rates of serum ESR, CRP, and sCD25 in the high disease activity group were 100% (42 cases), 95.2% (40 cases), and 90.5% (38 cases);36 cases of ESR and/or CRP were negative (about 33.3%) in 108 patients, serum sCD5 levels of 17 cases in these 36 cases (about 47.2%)increased, of which 14 cases (about 82.4%) had a DAS28 score higher than 3.2.Conclusion:The serum sCD25 has a high specificity for diagnosis of RA and a poor sensitivity. The serum level is closely related to the activity of RA, indicating that sCD25 may be involved in the inflammatory process of RA and may become a new inflammatory marker of RA.It is more meaningful for detection of serum sCD25 when RA is active, but ESR and/or CRP is negative.

    Value of serum matrix metalloproteinase 3 in the assessment of early rheumatoid arthritis
    Meng-ke LIU,Lu-chen WANG,Fan-lei HU
    2018, (6):  981-985.  doi: 10.19723/j.issn.1671-167X.2018.06.007     PMID: 30562768
    Abstract ( 2383 )   HTML ( 26 )   PDF (961KB) ( 461 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the expression level of serum matrix metalloproteinase 3 (MMP3) in early rheumatoid arthritis (ERA) patients with normal C-reaction protein (CRP) or erythrocyte sedimentation rate (ESR), and the significance in disease assessment.Methods:In the study, 133 cases of early RA patients, 25 osteoarthritis (OA) patients and 60 healthy controls in Peking University People’s Hospital from 2011 to 2015 were included. The RA patients were further divided into 4 groups according to levels of CRP and ESR: 88 patients with increased CRP and increased ESR, 15 patients with normal CRP and normal ESR, 17 patients with normal CRP but increased ESR, and 13 patients with increased CRP but normal ESR. All the clinical information of the patients was collected, and the serum MMP3 levels of both patients and healthy controls were detected by enzyme-linked immune sorbent assay (ELISA).Results:The serum MMP3 level of RA patients with normal CRP and/or normal ESR [(72.89±6.34) μg/L] was obviously higher than that of OA patients [(42.87±4.14) μg/L](P=0.002) and healthy controls [(31.62±2.88) μg/L](P<0.001). The serum MMP3 levels of the patients with normal CRP and normal ESR[(47.04±9.64) μg/L] were higher than those of the healthy controls, and there was statistical significance between the two groups (P<0.05). The serum MMP3 levels of the patients with increased CRP but normal ESR [(94.18±9.11) μg/L] and the patients with normal CRP but increased ESR [(79.42±10.60) μg/L] were both higher than those of the OA patients and healthy controls, and there was obvious statistical difference (P<0.05). In the early RA patients with normal CRP and/or normal ESR, the serum MMP3 level was positively correlated with the CRP level (r=0.336,P=0.024). The positive rate of MMP3 in the patients with normal CRP and/or normal ESR was 44.44%, higher than the positive rate of CRP (28.89%) and the positive rate of ESR (37.78%).In these early RA patients, the positive rate was 52.94% in the patients with normal CRP but increased ESR and 53.85% in the patients with increased CRP but normal ESR.Conclusion:The detection of the serum MMP3 level was significant in the assessment of early RA patients within 2-year duration who had normal CRP or ESR value.

    Clinical characteristic of 74 cases of malignant tumor in rheumatoid arthritis
    Yu-hua WANG,Guo-hua ZHANG,Ling-ling ZHANG,Jun-li LUO,Lan GAO,Mian-song ZHAO
    2018, (6):  986-990.  doi: 10.19723/j.issn.1671-167X.2018.06.008     PMID: 30562769
    Abstract ( 1206 )   HTML ( 21 )   PDF (877KB) ( 554 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the clinical characteristics of rheumatoid arthritis (RA) patients with malignant tumor.Methods:Retrospective summary was made of 1 562 in patients of RA from January 2011 to June 2017.In the study, 74 RA patients with malignant tumor were reviewed and analyzed, and the general conditions, tumor types, RA and tumor onset sequence, and the medication situation were analyzed.Results:The incidence of malignant tumor in the patients with rheumatoid arthritis in our center was 4.16 %. The 74 patients were complicated with malignant tumor, of whom 53 were female,and 21 male. The age of RA at presentation was (52.6±17.8) years. The average disease duration of malignant tumor was (63.4 ± 12.7) years. The onset time of rheumatoid arthritis was earlier than that of malignant tumors in 51 cases (51/74), with an average of (17.2±14.2) years between 2 and 60 years. The incidence of malignant tumor was earlier than that of rheumatoid arthritis in 16 cases (16/74), with an average of (6.2±5.9) years between 1 and 21 years, of which 10 cases were sex hormone related tumors. Seven cases (7/74) were diagnosed with RA at the same time, and the time interval between the two diseases was within 1 year. All the patients were over 60 years old with digestive tract tumors. All the 7 patients showed polyarthritis, significantly increased erythrocyte sedimentation rate and C-reactive protein, including 4 rheumatoid factor positive cases and 2 anti-CCP antibody positive cases. The effect of non-steroidal anti-inflammatory drugs and traditional drugs to improve the condition of the disease was poor in the 7 patients, and the condition was relieved after using low-dose glucocorticoids. Gastrointestinal tumors, breast and reproductive system tumors were the most common, followed by respiratory, urological and blood system tumors.Conclusion:The risk in patients of rheumatoid arthritis complicated with malignant tumor is higher than that of the general population. A variety of factors play an important role in cancer risk of RA, including disease activity, some estrogen metabolites, the use of drugs and so on. Therefore, all RA patients should be screened for malignant tumor during diagnosis, and malignant tumor surveillance is mandatory for all rheumatoid arthritis patients after diagnosis.

    Effect of functional exercises on patients with rheumatoid arthritis: a meta-analysis
    Li WANG,Chao GAO,Di ZHU,Li-hong CHEN
    2018, (6):  991-997.  doi: 10.19723/j.issn.1671-167X.2018.06.009     PMID: 30562770
    Abstract ( 2094 )   HTML ( 234 )   PDF (1232KB) ( 1306 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To evaluate the effect of functional exercises on disease activity, joint function and quality of life of patients with rheumatoid arthritis (RA). Methods: Randomized controlled trials were searched in Cochrane Library, PubMed, China National Knowledge Infrastructure (CNKI), VIP database and Wanfang database with keywords being “rheumatoid arthritis/RA”, “function exercise (training)/joint exercise (training)/physical exercise (training)/resistance movement (exercise)/isotonic and isometric/stretching exercise/muscle exercise”, and “trials/clinical trials”. Then literature selection, extraction and literature quality evaluation were carried out by two of the authors independently following the including and excluding standards. Then the outcome indicators were analyzed with Review Manager 5.3 software. Results: In the study, 2 173 articles were achieved by searching in databases, including 1 522 English papers and 651 Chinese papers. Then 913 duplicated papers were identified and removed using EndNote software. After reading the titles and abstracts, 1 194 papers were excluded that did not satisfy the including standards. Finally, the full texts of these papers were read and papers with insufficient data were excluded, resulting in 13 included papers for systematic review, including 8 English and 5 Chinese papers. A total of 812 cases were studied in these papers, including 426 in the experimental groups and 386 in the conventional groups. For the outcome index in these articles, disease activity score 28 (DAS28) was used in 5 of them, health assessment questionnaire (HAQ) was used in 8 articles, visual analogue scale (VAS) for pain was used in 6 articles, and morning stiffness duration was used in 3 articles. The meta-analysis showed that functional exercises could delay the development of the disease activity of RA patients (mean difference=-0.76; 95%CI: -1.13, -0.38; P<0.001), improve the joint function (mean difference=-0.36; 95%CI: -0.47, -0.24; P<0.001), alleviate the pain of joints (mean difference=-1.75; 95%CI: -1.98, -1.53; P<0.001), and reduce the duration of morning stiffness (mean difference=-17.65; 95%CI: -22.09, -13.21; P<0.001). Conclusion: This study showed the positive effects of functional exercises on alleviating the pain of joints, reducing the morning stiffness duration, and delaying the disease exacerbation of RA patients. It has a positive effect on improving the joint function and improving the quality of life in patients with RA.

    Study of bone mineral density and serum bone turnover markers in newly diagnosed systemic lupus erythematosus patients
    Hai-hong YAO,Su-mei TANG,Zhi-min WANG,Xia ZHANG,Xu-yong CHEN,Li GAO,Jing LIU,Yi-jun DAI,Zhao-heng HU,Xue-wu ZHANG,Zhan-guo LI
    2018, (6):  998-1003.  doi: 10.19723/j.issn.1671-167X.2018.06.010     PMID: 30562771
    Abstract ( 1162 )   HTML ( 35 )   PDF (1101KB) ( 409 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the changes of bone mineral density (BMD) and serum bone turnover factor in newly diagnosed systemic lupus erythematous (SLE) patients.Methods:Eighty newly diagnosed SLE patients and 80 age and gender matched healthy controls were enrolled. None of the SLE patients had ever received glucocorticoid,immunosuppressive agents or vitamin D. BMD was measured at radius,lumbar spine and hip by dual X ray absorptiometry (DXA). Bone turnover markers including se-rum levels of tartrate-resistant acid phosphatase 5b (TRAP5b),bone alkaline phosphatase (BAP) and 25-hydroxy vitamin D3 (25-OH-VD3) were measured by enzyme-linked immunosorbent assay (ELISA). Logistic regression was employed to analyze the risk factors associated with decreased BMD.Results:Mean age of the SLE patients was (32.8±12.4) years,and 85% were female,none of whom were post-menopausal. BMD was significantly reduced in all the measured sites,compared with the healthy controls. Sixteen (20%) of the patients were osteopenic in at least one site measured locations. The serum levels of 25-OH-VD3 were markedly reduced in the newly diagnosed SLE patients than those of the normal controls [(46.1+12.3) nmol/L vs. (25.4+11.2) nmol/L,P<0.001)]. The serum levels of 25-OH-VD3 in the SLE patients with nephritis were much lower than those without nephritis (P=0.04). A significant negative correlation was demonstrated between the serum concentration of 25-OH-VD3 and the disease activity scores as measured by SLE disease activity index (SLEDAI) (r=-0.3,P=0.001). The serum TRAP5b concentration was positively correlated with SLEDAI (r=0.435,P=0.003). Age (P=0.058) and SLEDAI (P=0.085) were probably associated with decreased BMD in Logistic regression analysis.Conclusion:The study showed reduced BMD in untreated SLE patients. The role of chro-nic inflammation was of probable importance in bone metabolism.

    Clinical and immunological characteristics of patients with systemic lupus erythematosus in Tibet plateau, China
    Jiao YANG,Hai-hong YAO,Xiao-dong MO,Zeng LUO,yang-jin Bai-ma
    2018, (6):  1004-1008.  doi: 10.19723/j.issn.1671-167X.2018.06.011     PMID: 30562772
    Abstract ( 1287 )   HTML ( 25 )   PDF (938KB) ( 413 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To describe the clinical, immunological characteristics and organ involvement of patients with systemic lupus erythematosus (SLE) in Tibet plateau, China.Methods:We retrospectively investigated 70 patients admitted in the Tibet Autonomous Region People’s Hospital between May 2014 and April 2016. In the study, 120 hospitalized patients with SLE from the Department of Rheumatology and Immunology of the Peking University People’s Hospital were randomly selected as the control (plain) group. The major organ involvement, clinical and immunological characteristics were compared between the two groups.Results:The female to male ratio of Tibet plateau group was 10.7, while the correspon-ding ratio of plain group was 11.0. The mean age at disease diagnosis was (32.21±11.40) and (35.38±13.25) years, respectively. the most common initial manifestations of SLE were arthritis (78.6%), alopecia (55.7%) and malar rash (48.6%) in Tibet plateau group, the prevalence of arthritis and alopecia was significantly higher than in plain group (P<0.05). The incidence of neuropsychiatric and kidney involvement was significantly lower in Tibet plateau group compared with plain group (P<0.05). As for the serological manifestations, the positivity of anti-double-stranded DNA (dsDNA) (57.1%), anti-Smith (Sm) antibody (55.7%), anti-Sj?gren syndrome A (SSA) antibody (72.3%), anti-Sj?gren syndrome B (SSB) antibody (41.4%) and anti-u1-ribosenuclear protein (u1RNP) antibody (45.7%) was significantly higher in Tibet plateau group (P<0.05). While the incidence of low serum complement C3 (61.4%), C4 (38.6%) less frequent in Tibet plateau group. Mean SLE disease activity index (SLEDAI) score was similar in the Tibet plateau group (12.18±5.58) and plain group (12.69±7.28). Moreover, there were 13 (18.6%) SLE patients suffering from tuberculosis and 7 (10%) SLE patients infected with hepatitis B virus in Tibet plateau group. The number of recent-onset SLE patients with lower 25-dihydroxy-vitamin D3 (25-OH-VD3) in Tibet plateau group was fewer than that in the plain group (76.7% vs. 90.0%, P=0.046). Serum 25-OH-VD3 levels in Tibet plateau plateau group were (31.14±18.74) nmol/L, those in plain group were (26.91±14.27) nmol/L, and the difference was not significant.Conclusion:The age, gender and SLEDAI scores in Tibet plateau group was similar to those in plain group. But there are significant differences in clinical manifestations, distributions of antibodies and immunological changes between Tibet plateau group and plain group. The patients with lower serum 25-OH-VD3 levels were more in plain group than in Tibet plateau group, while there was no signi-ficant difference in the 25-OH-VD3 level between the two groups.

    Multidisciplinary classification of magnetic resonance imaging features of neuropsychiatric lupus
    Jun-ying CHANG,Mei ZHENG,Ying LIU,Rui LIU,Jing-feng ZHANG,Xiao-li DENG
    2018, (6):  1009-1013.  doi: 10.19723/j.issn.1671-167X.2018.06.012     PMID: 30562773
    Abstract ( 877 )   HTML ( 33 )   PDF (991KB) ( 383 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the clinical correlation between the manifestations of neuropsychiatric lupus (NPSLE) and brain magnetic resonance imaging (MRI).Methods:Retrospective analysis of 65 neuropsychiatric lupus patients with brain MRI and clinical data from Peking University Third Hospital from January 2006 to October 2016, which was classified by rheumatologist, neurologists, and radiologists based on their brain MRI findings. The correlation between brain MRI findings and clinical manifestations was analyzed.Results:The characteristics of the brain MRI of the 65 patients were divided into 6 categories: 16 cases (25%) with demyelination in the white matter, 15 cases (23%) with cerebrovascular disease, including 4 cases (6%) with large vascular disease and 11 cases (17%) with small vessel disease, 4 cases (6%) with inflammation, 4 cases (6%) with edema, 13 cases (20%) with multiple manifestation coexistence, and 13 cases (20%) without any abnormality. Except for 4 cases of brain MRI with edema, the clinical manifestations were only epileptic seizures, other patients had complex and diverse clinical manifestations, including epileptic seizures, lupus-like headaches, mental symptoms, blurred vision, peripheral neuropathy and disturbance of consciousness. The incidence of epileptic seizures in patients with edema of MRI is significantly higher than that of other patients, and the therapeutic response time is the shortest.Conclusion:Multidisciplinary collaboration divides the MRI findings of neuropsychiatric lupus patients into six categories. This classification method helps clinicians to predict and intervene early possible neuropsychiatric symptoms to guide clinical treatment.

    Efficacy of mesenchymal stem cells on systemic lupus erythematosus:a meta-analysis
    Shuang LIU,Yu-long GUO,Jing-yi YANG,Wei WANG,Jian XU
    2018, (6):  1014-1021.  doi: 10.19723/j.issn.1671-167X.2018.06.013     PMID: 30562774
    Abstract ( 2038 )   HTML ( 148 )   PDF (1556KB) ( 1296 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: Systemic lupus erythematosus (SLE) is an autoimmune disease with multi-organ involvement and several typical autoantibodies. Mesenchymal stem cells (MSC) are multipotent stem cells with low immunogenicity that can differentiate into various kinds of cells, such as bone, cartilage, fat and skin tissue. MSC have immunomodulatory and reparative properties through interactions with immune cells. MSC have been used in the treatment of refractory SLE and lupus nephritis patients for more than ten years. Most clinical studies were self-controlled studies and only a few were randomized controlled trials. The objective of this study was to use meta-analysis method to evaluate the efficacy and safety of MSC treatment in SLE patients. Methods:The PubMed, Cochrane Library, Wanfang and VIP databases were searched for published randomized controlled trials and self-controlled studies before June 1, 2018. The search terms included the Chinese and English versions of mesenchymal stem cells, Mesenchymal Stromal Cells [Mesh], systemic lupus erythematosus, lupus, Lupus Erythematosus, Systemic [Mesh]. Two authors independently screened the literatures, assessed the quality of the studies and collected data according to the inclusion and exclusion criteria. The endpoints were the SLE disease activity index, 24 h urine protein and complement C3. Meta-analysis was performed with the Revman 5.3 software according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standard. Results:Eight studies involving 213 patients were included and three of the studies were randomized controlled trials with 66 patients involved. The MSC group showed that the SLE disease activity index decreased significantly [standard mean difference (SMD)=-1.76, 95% confidence interval (CI):-2.00 to -1.51, P<0.001), the 24 h urine protein decreased significantly (SMD=-1.74, 95%CI:-2.46 to -1.03, P<0.001), as well as the complement C3 increased significantly (SMD=1.28, 95%CI: 0.93 to 1.62, P<0.001). Four studies reported adverse events including fever, diarrhea and headache during the infusion. Conclusion:Current evidences showed that MSC could improve the disease activity, proteinuria and hypocomplementemia in SLE patients. Large scale and high-quality randomized controlled trials are required to validate the efficacy and safety of MSC treatment in SLE patients.

    Comparison of disease activities and extent measurements for anti-neutrophil cytoplasmic autoantibody-associated vasculitis
    Sheng-guang LI,Qing ZHANG,Hui-qiong ZHOU
    2018, (6):  1022-1026.  doi: 10.19723/j.issn.1671-167X.2018.06.014     PMID: 30562775
    Abstract ( 1475 )   HTML ( 18 )   PDF (881KB) ( 557 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the significance of a set of seven disease activities and extension measurements and their correlations between one and another for anti-neutrophil cytoplasmic autoantibody associated vasculitis (AAV).Methods:A total of 121 patients from Peking University International Hospital and Fouth Medical Center of PLA General Hospital with confirmed diagnoses of AAV clinically were enrolled in the study, including 15 cases of eosinophilic granulomatous with polyangiitis (EGPA), 59 cases of granulomatous with polyangiitis (GPA) and 47 cases of microscopic polyangiitis (MPA). A hundred and twenty-one AAV patients were divided into death group and survival group according to their survival conditions. A set of seven disease assessment scales including Birmingham vasculitis activity score (BVAS)-1994, BVAS-2003, as well as BVAS/GPA, vasculitis damage index (VDI), disease extent index (DEI), five factor score (FFS)-1996, and FFS-2009 were measured and scored one by one, and their relationships which were represented by Spearman correlation coefficient were compared between one and another.Results:BVAS-1994, BVAS-2003, as well as BVAS/GPA, VDI, DEI, and FFS, all of those seven evaluation indexes of the AAV patients in the death group were significantly higher than those in the survival group (P<0.05). Except for BVAS/GPA, all those above indicators in the patients with EGPA were lower than those in the patients with GPA and those in the patients with MPA, and those in all of the AAV patients as a whole group. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA (r values were 0.9 and 0.7, respectively); BVAS-1994 was fairly correlated with BVAS/GPA (r=0.69); FFS-1996 and FFS-2009 were highly correlated (r=0.73) with each other; BVAS-1994, BVAS-2003 and BVAS/GPA were fairly correlated with DEI (with r values of 0.62, 0.65, and 0.62, respectively); VDI was also fairly correlated with BVAS-1994 and with BVAS-2003 (r values were 0.49 and 0.52, respectively).Conclusion:All of those seven AAV assessment indicators above can be used as indicators of disease activity and prognosis in AAV patients, most of which were relevant within one and another. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA, and besides, there were also high correlations between FFS-1996 and FFS-2009.

    Increased serum soluble-endoglin level and its clinical significance in antiphospholipid syndrome
    Ji LI,Li ZHENG,Lian-jie SHI,Jing XU,Jian-long SHU,Xue-wu ZHANG
    2018, (6):  1027-1032.  doi: 10.19723/j.issn.1671-167X.2018.06.015     PMID: 30562776
    Abstract ( 924 )   HTML ( 7 )   PDF (964KB) ( 306 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To detect the serum levels of soluble endothelial glycoprotein endoglin (s-Eng) in patients with antiphospholipid syndrome(APS) and to evaluate the correlation between s-Eng levels and clinical features and laboratory parameters.Methods:The levels of serum s-Eng were measured by enzyme linked immunosorbent assay ( ELISA) in 139 patients with APS, 44 patients with SLE but no APS, 37 patients with primary Sjogren’s syndrome (pSS), 23 patients with Bechet’s disease (BD), 22 patients with systemic sclerosis (SSc) and 22 persistent anticardiolipin antibody (aCL) positive indivi-duals without SLE or APS(simply aCL positive group) and 87 health controls(HC) without any auto-immune diseases. These APS patients included 64 primary APS patients and 75 APS patients secondary to SLE.The correlation between the clinical data, laboratory parameters, and serum s-Eng levels were analyzed.Independent samples t test,paired t test,Chi-square Test, Mann-Whitney U test, Pearson’s χ 2 test were used for statistical analyses. Results:(1)The serum levels of s-Eng were significantly higher in the patients with APS whether primary or secondary to SLE than in the health controls and simply aCL positive group and the patients with other autoimmune diseases, including SLE, pSS, BD and SSc(P<0.001).There was no significant difference in the serum s-Eng levels between simply aCL positive group and health controls[(5.17±2.00) mg/L vs.(5.04±1.11) mg/L, P>0.05]. (2) The best cut-off value for the diagnosis of APS was no less than 8.37 mg/L as mean ± 3SD value, with the sensitivity at 0.772 and the specificity at 0.928. The Youden index was 0.700. These results indicated good validity of s-Eng as a diagnostic marker for APS. (3)The proportions of artery thrombosis and pathological pregnancy were higher in the group of s-Eng -positive APS patients than that in s-Eng -negative group(46/81 vs. 19/58,29/65 vs. 10/44,respectively, all P<0.05).The levels of PLT were lower in the group of s-Eng-positive APS patients (72.00×10 9/L vs. 119.00×10 9/L, P<0.001). (4)The proportions of the presence(93.83% vs. 37.93%, P<0.001) and titer (61.70 U/mL vs.15.45 U/mL, P<0.001) of aCL were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group. The proportions of the presence (61.73% vs. 43.10%, P<0.05)and titer (33.48 U/mL vs.17.40 U/mL,P<0.05) of anti-β2-glycoproteinⅠ antibody were both higher in the group of s-Eng -positive APS patients than in s-Eng -negative group too. Conclusion:S-Eng serum levels were significantly increased in the patients with APS, and it may play a role as acomplementary serological marker for the diagnosis and risk prediction of APS.

    Case series and clinical analysis of 14 cases of catastrophic antiphospholipid syndrome
    Jie-yu GU,Cui LU,Hui SHI,Cheng-de YANG
    2018, (6):  1033-1038.  doi: 10.19723/j.issn.1671-167X.2018.06.016     PMID: 30562777
    Abstract ( 1513 )   HTML ( 45 )   PDF (890KB) ( 524 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson’s syndrome, is a special subtype of antiphospholipid syndrome (APS) characterized by multiple intravascular thrombosis involving multiple organs systems or tissues simultaneously or continuously, high titer antiphospholipid antibodies and high mortality rate. This article’s aims was to analyze the clinical manifestation, laboratory examination and treatment therapy of CAPS for the purpose of improving the understanding, diagnosis and treatment of the disease in clinical practice.Methods:Retrospective analysis and descriptive statistics were applied to the clinical manifestations and laboratory findings of 14 CAPS cases from APS Shanghai Database (APS-SH) with catastrophic antiphospholipid.Results:Of the 14 CAPS patients, 12 cases satisfied the 2003 CAPS Classification Criteria accepted in the 10 th International Congress on Antiphospholipid Antibody, and were diagnosed as definite APS and 2 cases were diagnosed as probable CAPS. Three cases were categorized as primary APS and 11 as APS secondary to systemic lupus erythematosus (SLE). Infection was mostly commonly seen before the onset of CAPS, followed by SLE activity and surgery. Among the involved organs, systems and tissues, brain and lung were most commonly affected sites of arterial thrombosis while peripheral vein was most commonly affected in venous thrombosis events among the clinical events. Triple positivity of anticardiolipin antibody (aCL), anti-β2 glyeoprotein Ⅰ antibody (aβ2GPI), lupus anticoagulant (LA) were detected in 54.55% of the patients. Thrombocytopenia and decreased hemoglobin were frequently seen in the CAPS patients, and the majority proved to be hemolytic anemia. Of all the cases, 6 ended with death. The triple therapy strategy (anticoagulants, glucocorticoid, intravenous immunoglobulin and/or plasma exchange) could help to improve prognosis, cyclophosphamide and rituximab might benefit the patients with other comorbidities such as SLE and micro-angiopathic hemolytic anemia (MHA). Conclusion:CAPS patients suffer from life-threatening acute multiple small vessel thrombosis with high titer of antiphospholipid antibody, potentially leading to multiple organ failure and a poor prognosis, thus early diagnosis and sufficient treatment are critical to prevent the progression of disease and improve the prognosis.

    A cross-sectional study of the clinical features and physician visit patterns at onset of immunoglobulin G4 related disease
    Xing-yun ZHU,Yan-ying LIU,Xue-juan SUN,Ping YU,Shuang LIU,Mei-yan FU,Xu LIU,Li-min REN,Zhan-guo LI
    2018, (6):  1039-1043.  doi: 10.19723/j.issn.1671-167X.2018.06.017     PMID: 30562778
    Abstract ( 944 )   HTML ( 7 )   PDF (925KB) ( 304 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To comprehend clinical features and patient’s physician visit patterns at onset of immunoglobulin G4 related disease (IgG4RD). Methods: In the study, 100 patients with IgG4RD who received treatments in the Department of Rheumatology and Immunology of Peking University People’s Hospital from Apr. 1st, 2017 to Apr. 1st, 2018 were investigated, including gender, age, height, body weight, age of onset, physician visit history, primary history and how did the disease affected their life, etc. Results: In this 100 IgG4RD cohort (57 males and 43 females), the male/female ratio was 1 :0.75, the mean age of onset was (51.51±12.9) years, and the median duration was 49 months (ranging from 4 to 231 months). The onset age of males was significantly older than that of females (P<0.01). The manifestations showed that up to 69% patients had submaxillay glands lesion, 59% patients had lacrimal glands lesion, 28% patients had pancreas involvement and 28% patients had parotid glands involvement. The females had more lacrimal glands involvement (P<0.05). 62% patients were complicated with anaphylactic disease. The primary physician visit departments concentrated upon general surgery department (19/100), oral and maxillofacial surgery department (17/100), rheumatology and immunology department (16/100), ophthalmology department (15/100) and gastroenterology department (10/100). The departments where the confirmed diagnose was made concentrated upon rheumatology department (67/100),oral and maxillofacial surgery department (16/100) and gastroenterology department (7/100). The mean diagnosis duration after 2010 was (16.96±2.163) months, significantly shorter than that before 2010, which was (113.3±11.01) months. Before the definite diagnose was made, 43% patients underwent surgeries and 12% patients had more than one time surgeries. The patients whose first-visit department was a surgery department were more likely to undergo surgeries (P<0.01). 18% patients (18/100) stated that the disease had affected their work. Conclusion: In this cohort of the IgG4RD patients, female is common and has earlier onset age than male. The major manifestations of IgG4RD are salivary glands, lacrimal glands and pancreas involvement. The common chief complains are salivary glands and lacrimal glands enlargement. Accompanied by anaphylactic disease is a marked manifestation of this disease. Delayed diagnoses are not rare, though this situation has been improved since 2010, and more attention still should be paid to the disease.

    A multi-center cross-sectional survey of medicine application in patients with osteoarthritis in China
    Xiao-ying ZHANG,Jia-jing PENG,Chuan-hui LIU,Xiao-yan CAI,Jiang-lin ZHANG,Yi-fang MEI,Hong-tao JIN,Xiao-fei WANG,Hong MO,Zhan-guo LI
    2018, (6):  1044-1048.  doi: 10.19723/j.issn.1671-167X.2018.06.018     PMID: 30562779
    Abstract ( 1078 )   HTML ( 22 )   PDF (889KB) ( 722 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the clinical characteristics, the medicine application and to eva-luate the disease activity in patients with osteoarthritis (OA) in China.Methods:This was a cross-sectional study. Totally 1 066 cases of OA from 40 hospitals in China from April to October 2017 were retrospectively enrolled. Demographic characteristics, clinical data, medicine application, and joint function were evaluated. All the data were analyzed by SPSS software 19.0. t test, Mann-Whitney U test and chi-square test were used for statistical analysis.Results:In the 1 066 cases, the male-to-female ratio was 1 :3.6 and the average age was (61.9±11.0) years, with an age range from 36 to 94 years. The incidence of knee OA, hip OA, and hand OA were respectively 81.9% (873/1 066), 14.1% (150/1 066), and 36.3% (387/1 066). In the study, 242 (22.7%) cases had two kinds of joint areas involved and three joint areas were involved in 51 cases (4.8%), and 56.6% (603/1 066) of the patients used more than one kind of non- steroid anti-inflammatory drugs (NSAIDs) while 61.2% (652/1 066) used disease modifying osteoarthritis drugs (DMOADs), including glucosamine (37.5%, 400/1 066), chondroitin sulfate (2.0%, 21/1 066), diacetate (5.9%, 63/1 066), and the combination of these drugs (15.8%, 168/1 066). 8.6% (92/1 066) patients only took analgesics to relieve the pain, not using any kind of NSAIDs or DMOADs. And 232 patients (21.7%) had intra-articular injections, including 9.2% (98/1 066) sodium hyaluronate, 4.5%(48/1 066) glucocorticoid, and 8.1% (86/1 066) combination of the two drugs. The proportion of the patients taking topical drugs accounted for 26.5% (283/1 066) and physical therapy accounted for 15.8% (168/1 066). Compared with those who suffered from knee OA, the patients who suffered from hip OA had more severe disease assessment. Moreover, there were significant differences in pain (Z=-7.625, P<0.001), morning stiffness (Z=-6.229, P<0.001), and joint function (Z=-6.777, P<0.001) between the two groups of the patients who suffered from knee or hip OA with The Western Ontario and McMaster Universities (WOMAC) osteoarthritis index. Furthermore, patients with hip OA took more analgesics (χ 2=24.838, P<0.001). Conclusion:Oral NSAIDs and DMOADs are wildly used in patients with OA in China. However, the treatment of some patients still need to be improved. Patients with hip OA are more seriously ill and require aggressive treatment.

    Application of artificial ligament in treatment of lower abdominal wall reconstruction after pubic tumor resection
    Jie ZANG,Wei GUO,Xiao-dong TANG,Hua-yi QU,Da-sen LI
    2018, (6):  1049-1052.  doi: 10.19723/j.issn.1671-167X.2018.06.019     PMID: 30562780
    Abstract ( 953 )   HTML ( 17 )   PDF (3972KB) ( 510 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: For patients who had hemipelvectomies involving the resection of a portion or the whole of the pubis, bony reconstruction was not recommended commonly. However, the soft tissue reconstruction of the lower abdominal wall may benefit these patients. The object of the study was to determine the clinical effect of lower abdominal wall reconstruction with LARS ligament after pubic tumor resection interms of patient-reported and objective outcome.Methods:In this series, we reviewed twenty-five patients who underwent pubic tumor resection followed by reconstruction with LARS ligament between February 2012 and February 2018 retrospectively. We evaluated the clinical outcome and complication of this surgical treatment. The function outcome was evaluated according the musculoskeletal tumor society scores (MSTS) for all the patients at the end of the last follow-up.Results:All the patients were stable during the surgery. There were eight patients who underwent resection of superior ramus of pubis, five patients who had resection of inferior ramus of pubis, and twelve patients who received both superior and inferior ramus of pubis. For all the patients, the mean blood loss was (774±580) mL. The mean operation time was (138±25) min. The mean hospital stay was (19±6) d. For the patients who had resection of superior ramus, inferior ramus, as well as both superior and inferior ramus, the mean blood loss were (763±802) mL, (730±315) mL and (808±485) mL, respectively. The mean operation time were (133±27) min, (135±35) min and (143±20) min, respectively. The mean hospital stay were (18±5) d, (22±9) d and (19±6) d, respectively. The mean follow-up time was (37±21) months. Local recurrence was observed in one patient with chondrosarcoma. One patient with renal cancer metastasis died of the disease. No ligament infection, ligament related complication and incisional hernias were observed. Twenty-three patients could ambulate without assistive devices, and the remaining two could walk by crutches. Postoperative pain was reported as none in nineteen patients, mild in three, and mod-erate in three. From a functional point, the mean MSTS score was 87±4.Conclusion:Lower abdominal wall reconstruction with LARS ligament after pubic tumor resection could have satisfactory clinical outcome. It could prevent the occurrence of herniation, decrease the infection rate by minishing the dead space, and achieve good patient-reported outcome.

    Single-center study of laparoscopic radical nephrectomy with Mayo 0-2 level inferior vena cava thrombectomy
    Xiao-jun TIAN,Min QIU,Zhuo LIU,Ruo-tao XIAO,Yi HUANG,Guo-liang WANG,Xiao-fei HOU,Shu-dong ZHANG,Shen-rong ZHUANG,Lu-lin MA
    2018, (6):  1053-1056.  doi: 10.19723/j.issn.1671-167X.2018.06.020     PMID: 30562781
    Abstract ( 1371 )   HTML ( 16 )   PDF (855KB) ( 445 )   Save
    References | Related Articles | Metrics

    Objective: To investigate the safety and feasibility of laparoscopic treatment for renal carcinoma with Mayo 0-2 level venous thrombosis.Methods:From January 2015 to February 2018, 58 renal carcinoma cases with venous thrombus underwent laparoscopic radical nephrectomy with inferior vena cava thrombectomy in Department of Urology, Peking University Third Hospital, of which, 51 cases were male, and 7 female, aged 29-82 years. According to the Mayo grade classification, 20 cases were level 0, 20 cases were level 1, and 18 cases were level 2, with left side being 22 cases, and right side 36 cases. The patients except for those complicated with hemorrhagic diseases, cardiac and pulmonary insufficiency, or those who could not tolerate anesthesia and surgical contraindications, underwent the operation after comprehensive examinations.Results:The 58 cases of renal tumor with venous tumor emboli were successfully completed with the surgeries, including 50 cases of totally laparoscopic surgery, 8 cases of laparoscopy surgery from convert to open (among the patients who were converted to open surgery, 7 were complicated with grade 2 tumor thrombus and 1 with grade 1 tumor thrombus). The main reasons for converting to open surgery were huge tumors (the largest of which was about 16 cm in diameter), severe adhesion and difficulty of separation. For different patients, different surgical methods and procedures were adopted according to the tumor direction and the different grade of tumor thrombus. Radical nephrectomy combined with vena cava tumor thrombus removal was performed in 55 cases and segmental resection of vena cava in 3 cases. The operation time was 132-557 min, and blood loss was 20-3 000 mL. Post-operative pathological types: 51 cases were clear cell carcinoma, 5 cases were type 2 of papillary carcinoma, 1 case was squamous cell carcinoma, and 1 case was chromophobe cell tumor. In the study, 47 cases were followed up for 1-36 months, and 4 cases died (the survival time was 5-15 months, with an average of 10.2 months).Conclusion:Laparoscopic radical nephrectomy with inferior vena cava thrombectomy is a reasonable choice for renal tumor with Mayo 0-2 level venous thrombosis. For diffe-rent tumor directions and different grades of tumor thrombus, an appropriate operation plan can give the maxim benefit to the patients with skillful surgeons.

    Analysis of risk factors for acute lung injury/acute respiratory distress syndrome after esophagectomy
    Jia-xuan XU,Hong-zhi WANG,Jun DONG,Xiao-jie CHEN,Yong YANG,Ren-xiong CHEN,Guo-dong WANG
    2018, (6):  1057-1062.  doi: 10.19723/j.issn.1671-167X.2018.06.021     PMID: 30562782
    Abstract ( 1037 )   HTML ( 15 )   PDF (959KB) ( 400 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To explore the incidence and risk factors for the acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) after resection of esophageal carcinoma.Methods:We retrospectively analyzed 422 consecutive patients admitted to the Department of Critical Care Medicine with eso-phageal carcinoma undergoing esophagectomy from January 2010 to December 2016 in Peking University Cancer Hospital. ALI/ARDS were diagnosed, the patients were divided into ALI/ARDS group and control group without ALI/ARDS, the differences of clinical features were contrasted between the two groups, and the multivariate Logistic regression modeling was used to identify the independent risk factors for ALI/ARDS.Results:In the study, 41 ALI/ARDS cases were diagnosed, making up 9.7% (41/422) of all the enrolled patients undergoing esophagectomy. Comparisons of the ALI/ARDS group and the control group indicated significant statistical differences in the average length of their hospital stay [(18.9±9.7) d vs. (14.8±3.6) d, P=0.011], the proportion of the patients who needed mechanical ventilation support [51.2% (21/41) vs. 9.4% (36/381), P<0.001] and in-hospital mortality [31.7% (13/41) vs. 5.0% (19/381), P<0.001]. Univariate analysis showed significant differences between the patients with ALI/ARDS and without ALI/ARDS in smoking history (P=0.064), preoperative forced expiratory volume in one second/forced vital capacity (FEV1/FVC) (P=0.020), diffusing capacity of the lung for carbon monoxide (DLCO) (P=0.011), body weight index (BMI) (P=0.044), American Society of Anesthesiologists (ASA) physical status classification (P=0.049) and one lung ventilation duration (P=0.008), while multivariate Logistic regression analysis indicated that preoperative FEV1/FVC (OR=1.053, P=0.016, 95%CI 1.010-1.098), ASA physical status classification (OR=2.392, P=0.033, 95%CI 1.073-5.335) and one lung ventilation duration (OR=0.994, P=0.028, 95%CI 0.989-0.999) were the independent risk factors for ALI/ARDS after esophagectomy.Conclusion:ALI/ARDS was a serious complication in patients undergoing esophagectomy associated with increment in length of hospital stay and in-hospital mortality. Multivariate Logistic regression analysis indicated that preoperative FEV1/FVC, ASA classification and one lung ventilation duration were the independent risk factors for ALI/ARDS after esophagectomy. Carefully assessing the patient before operation, shortening one lung ventilation duration were the key points in preventing ALI/ARDS after esophagectomy.

    Clinical analysis of candidemia in immunocompetent patients
    Yan-ling DING,Ning SHEN,Qing-tao ZHOU,Bei HE,Jia-jia ZHENG,Xin-mao ZHAO
    2018, (6):  1063-1069.  doi: 10.19723/j.issn.1671-167X.2018.06.022     PMID: 30562783
    Abstract ( 1064 )   HTML ( 12 )   PDF (905KB) ( 390 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the etiological and clinical characteristics of immunocompetent patients with candidemia.Methods:The clinical and microbiological data of patients diagnosed as candidemia admitted in Peking University Third Hospital from January 2010 to June 2016 were retrospectively analyzed. Underlying diseases, Candida spp. colonization, clinical manifestations, microbiological data, treatment and the outcome were compared between the HIV-negative immunocompromised (IC) and nonimmunocompromised (NIC) patients.Results:A total of 62 cases diagnosed as candidemia were analyzed including 36 men and 26 women, with 16 to 100 years of age [(66.02±17.65) years]. There were 30 NIC and 32 HIV-negative IC patients respectively. In the NIC patients, there were 19 cases (19/30, 63.33%) with admission in intensive care unit (ICU), 21 (21/30, 70.00%) associated diabetes mellitus or uncontrolled hyperglycemia and 22(22/30,73.33%)receiving invasive mechanical ventilation,while in the HIV-negative IC patients, there were 8 (8/32, 25.00%),13 (13/32, 40.63%) and 7 (7/32, 21.88%) respectively (P<0.05). The NIC patients had higher acute physiology and chronic health evaluation (APACHEⅡ) scores and sequential organ failure assessment (SOFA) scores both at admission (19.98±5.81, 6.04±6.14) and candidemia onset (25.61±6.52, 12.75±8.42) than the HIV-negative IC patients (APACHEⅡ 15.09±5.82, 22.15±5.98) and SOFA 2.87±2.73, 7.66±5.64 respectively (P<0.05). In the NIC patients, twenty-one cases (21/30, 70.00%) died in hospital,while 14 cases (14/32, 43.75%) in HIV-negative IC.The crude mortality was significantly different between the two groups (P<0.05). By blood culture, Canidia albicans remained the the most prevalent isolates in all the patients. Clinical manifestation, Candida spp. colonization, etiology and drug susceptibility were also similar between NIC and HIV-negative IC patients (P>0.05).Conclusion:Candidemia in NIC patients tends to occur in those who are much more critically ill, more often admitted in ICU, and more frequently have diabetes mellitus or uncontrolled hyperglycemia and receive invasive mechanical ventilation than HIV-negative IC patients. NIC patients also have poorer prognosis than HIV- negative IC patients. Clinical manifestations, and microbiological characteristics are similar between HIV- negative IC and NIC patients.

    Preparation and evaluation of blank and doxorubicin loaded poly (acrylic acid) microspheres for embolization
    Li-ying GUO,Xiao-xin LIU,Zi-yuan LI,Xiao-ya QIN,Ze-yang FAN,Zhen-zhen LI,Hai-tao GUAN,Li SONG,Ying-hua ZOU,Tian-yuan FAN
    2018, (6):  1070-1077.  doi: 10.3969/j.issn.1671-167X.2018.06.023     PMID: 30562784
    Abstract ( 1008 )   HTML ( 18 )   PDF (9895KB) ( 192 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To prepare ion exchange doxorubicin-loaded poly (acrylic acid) microspheres (DPMs) and evaluate the properties of these chemoembolic agents.Methods:Poly (acrylic acid) microspheres (PMs) without drug were prepared by inverse suspension polymerization method and then doxorubicin was loaded by ion exchange mechanism to prepare DPMs. Optical microscope was used to investigate the morphology and particle size distribution of PMs and DPMs; fluorescence microscope and confocal microscope were used to observe the distribution of doxorubicin after drug loading. Elasticities of both the microspheres were evaluated by texture analyzer. High performance liquid chromatography (HPLC) method was established to determine the drug loading behavior of PMs and releasing behavior of DPMs. The in vivo embolic property was evaluated by embolizing the hepatic artery of a rabbit with 0.1 mL of DPMs.Results:PMs and DPMs were both spherical in shape, smooth in surface and dispersed well. Doxorubicin was mainly in the outer area inside of DPMs and distributed evenly. The average particle size of PMs and DPMs were (283±136) μm and (248±149) μm, respectively. PMs and DPMs both had good compression ability with the Young’s modulus of (62.63±1.65) kPa and (93.94±1.10) kPa separately. PMs reached the drug loading balance at 12 h, and the entrapment efficiency was greater than 99%. Drug loading of PMs in doxorubicin solution at the concentration of 5.0 g/L and 12.5 g/L was (19.78±0.27) g/L and (49.45±0.37) g/L, respectively. Doxorubicin released slowly from DPMs in PBS and the accumulative release percentages of DPMs with corresponding drug loading were 6.82%±0.02% and 2.83%±0.10% after 24 h, respectively. Arterial angiograms showed that the hepatic artery of the rabbit was successfully embolized with DPMs.Conclusion:DPMs with good performance of loading doxorubicin could be a potential embolic agent for transcatheter arterial chemoembolization.

    Effects of intranasal dexmedetomidine for children undergoing dental rehabilitation under general anesthesia: a double-blinded randomized controlled trial
    Ling GAO,Yun LIU,Xu-dong YANG
    2018, (6):  1078-1082.  doi: 10.19723/j.issn.1671-167X.2018.06.024     PMID: 30562785
    Abstract ( 1235 )   HTML ( 24 )   PDF (1027KB) ( 362 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To observe the preoperative sedation, the status of separation from parents, compliance with the mask, hemodynamic parameters and postoperative agitation of intranasal dexmedetomidine (DEX) premedication on children undergoing dental rehabilitation under general anesthesia.Methods:In the study, 60 children of American Society of Anesthesiology classification (ASA Ⅰ-Ⅱ), aged 2-9 years, were randomly assigned to one of two equal groups. Thirty minutes before operation, control group received intranasal placebo (0.9% saline) 0.02 mL/kg, and DEX group received intranasal DEX 2 μg/kg. The preoperative sedation score, the status of separation from parents, comp-liance with the mask and hemodynamic parameters were recorded by an anesthesiologists until anesthesia induction. Recovery conditions, postoperative agitation were also recorded.Results:There was no signi-ficant difference between the two groups in patient characteristics, operation time, extubation time and recovery time. Compared with the children in control group, those in DEX group were significantly more sedated when they were separated from their parents (56.7% vs. 26.7%, P<0.05). Satisfactory compliance with mask application was 40% in control group vs. 73.3% in DEX group (P<0.05). There was no significant difference between the two groups regarding the incidences of postoperative agitation and oxygen saturation (SpO2). Compared with control group, the heart rate (HR) of DEX group was decreased after 20 minutes of drug administration [(97.13±12.93) beats/min vs.(104.53±11.97) beats/min, P<0.05]. The changes of the HR and SpO2 in the two groups were within the normal range. There were no incidences of bradycardia and hypoxemia in either of the groups during study observation.Conclusion:Premedication with intranasal DEX 2 μg/kg for children undergoing dental rehabilitation under general anesthesia produces good preoperative sedation. The levels of sedation, scores of parental separation and compliance with the mask were satisfied. The children have good recovery conditions, and no obvious postoperative agitation and respiratory depression after DEX administration. Intranasal DEX 2 μg/kg is an effective and safe alternative for premedication in children.

    Staining effect of bovine incisors in vitro by tea solutions
    Meng-meng ZHU,Ying-long LI,Jie PAN
    2018, (6):  1083-1087.  doi: 10.19723/j.issn.1671-167X.2018.06.025     PMID: 30562786
    Abstract ( 965 )   HTML ( 12 )   PDF (1167KB) ( 265 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To determine the optimum staining condition of tea solutions on bovine incisors in vitro, by comparing the color stability of tooth surface of different concentrations of tea solutions and methods on bovine incisors in vitro.Methods:Twenty bovine incisors with color surface A1 were chosen, then randomly divided into 4 groups (n=10). Group 1: soaked with 2% tea solution continuously for 6 days; group 2: soaked with 2% tea solution for 6 days, but changed fresh tea solution everyday; group 3: soaked with 1% tea solution continuously for 6 days; group 4: soaked with 1% tea solution for 6 days but fresh tea solution changed every day. After 6 days of staining, the surface color (ΔE value)of all the samples were measured with crystal eye. After brushing 30 times with toothbrushes, the color of bovine incisors were measured again. Then the samples were soaked in artificial saliva at 37 ℃, and ΔE value was measured for 14 days.Results:After staining for 6 days, the ΔE values of the 2% tea solution groups were better than those of the 1% groups (20.21 vs. 16.44, 24.09 vs. 19.22, P<0.05);the groups with the same tea solution concentration, a better result was observed for the group soaked with daily fresh tea solution than for the group that experienced continuous staining (24.09 vs. 20.21, 19.22 vs. 16.44, P<0.05).Groups 1 and 2 were selected for subsequent brushing experiments. The color of both groups became lighter after brushing, and a better result was observed for the continuous staining group than for the group stained in daily fresh solution (3.06 vs. 9.51, P<0.05). The samples with better coloring effect soaked with 2% tea solution continuously for 6 days were put into artificial saliva for 14 days. There was not any significant change in coloring at theend of the first two days (1.51 vs. 1.51, P>0.05), and the color was visibly lighter after the third day (1.51 vs. 5.89, P<0.05), and no further significant change was observed until the 14th day (5.81 vs.5.89, P>0.05), which was darker coloring than that of the pre-staining group.Conclusion:Continuous staining on bovine incisors with 2% tea solution with subsequent soaking in artificial saliva resulted in consistent coloring from day 3 to day 14, and this method could be used as an ideal model for teeth staining in vitro.

    Retrospective analysis of early dental implant failure
    Zhi-yong△ ZHANG,Tian MENG,Quan CHEN,Wen-shu LIU,Yu-huan CHEN
    2018, (6):  1088-1091.  doi: 10.19723/j.issn.1671-167X.2018.06.026     PMID: 30562787
    Abstract ( 1724 )   HTML ( 138 )   PDF (866KB) ( 1241 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To analyze the risk factors of early dental implant failure,treatment and prognosis.Methods:Cases of dental implants in the first clinical division from January 2000 to December 2016 were selected according to inclusion criteria. The differences of gender,age,smoking,location of implants,healing abutments and bone graft were compared between early failed implants and success implants. The general conditions of early failure patients,the early failure occurrence time,treatment and prognosis were recorded. Statistical methods were χ 2 test and descriptive analysis, P<0.05 had statistical significance. Statistical analysis software was IBM SPSS Statistics 19.0. Results:There were 36 patients with 36 early failed implants and 4 381 patients with 6 564 success implants. The rate of early dental implant failure was 0.8% at individual level and 0.5% at implant level. There was no significant difference in gender between the failed implants and success implants(P=0.692). The failure rate of the patients ≥40 years old (1.0%) was higher significantly than that of the patients <40 years old(0.4%, P=0.033). The failure rate of smokers(1.3%) was higher significantly than that of non-smokers(0.3%,P<0.01).There was no significant difference of early failure among four implant locations,which were anterior maxilla,posterior maxilla,anterior mandibular and posterior mandibula(P=0.709).The early failure of implants with bone graft and healing abutments at the same time (1.1%) was significantly higher than that of the implants with bone and healing abutments separately (0.5%,P=0.039)。Ten patents with early failed implants had general diseases,including 5 patients with diabetes,3 with hypertension and 2 with coronary heart disease. All the patients with general diseases were controlled well. The median of early failure occurrence time was 30.5 after implant operations. 83.3% early failure implants was found by dentists at re-examinations. All of the early failure implants were removed when they were found failed. Twenty-six early failure implant sites were inserted with implants again,of which 23 implants were successful.Conclusion:The early dental implant failure was possible to occur in one month after implants inserting. The possible risk factors were age≥40 years old,smoking and using bone graft and healing abutments at the same time. Most early dental implant failure was found by dentists at re-examinations. The implants should be removed when the early dental implant failure was found,which didn’t influence the later implantation.

    Efficacy and safety of applying ultrasonic osteotome in cervical expansive open-door laminoplasty
    Yan LI,Liang JIANG,Zhong-jun LIU,Xiao-guang LIU,Feng WEI,Miao YU,Li-yuan TAO
    2018, (6):  1092-1097.  doi: 10.19723/j.issn.1671-167X.2018.06.027     PMID: 30562788
    Abstract ( 1332 )   HTML ( 17 )   PDF (1584KB) ( 243 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Objective: To investigate the safety and efficacy of applying ultrasonic osteotome in patients undergoning cervical expansive open-door laminoplasty (CEOL).Methods:In the study , 94 consecutive patients who were administrated in the spine group of Orthopedic Department of Peking University Third Hospital from March 2015 to March 2016 were reviewed retrospectively. All the patients were diagnosed as multilevel cervical spondylosis myelopathy and underwent CEOL. These patients were divided into two groups: ultrasonic osteotome group and traditional group, by whether the ultrasonic osteotome device was used in operation. The parameters we studied were as follows: the duration of operation, blood loss in operation, volume of drainage on the first postoperative day, days of remaining the drainage tube, preoperative and postoperative Japanese Orthopedic Association (JOA) scores, complications of cerebrospinal fluid leak and hinge bone nonunion.Results:Compared with the traditional group, the duration of operation of the ultrasonic osteotome group was increased, but the blood loss in operation, volume of drainage on the 1st postoperative day and days of remaining the drainage tube of the ultrasonic osteotome group were all reduced. There was no obvious difference between the two groups when considering the cerebrospinal fluid leak. At the end of the 3-month follow-up, the JOA score and improvement rate of the JOA score were of no obvious difference between the two groups. But the hinge bone union of the traditional group was better than the ultrasonic osteotome group. At the end of the 12-month follow-up, all the JOA score, the improvement rate of the JOA score and the hinge bone union were not obviously different between the two groups.Conclusion:Applying ultrasonic osteotome in patients undergoing cervical expansive open-door laminoplasty is both safe and effective. Compared with the rongeur, ultrasonic osteotome can cause the delayed union of the hinge bone, but it reduces the blood loss in operation, volume of postoperative drainage and days of remaining the drainage tube.

    Clinicopathological analysis of aggressive angiomyxoma of soft tissue in abdomino-pelvic cavity
    Lei LIU,Li-hua WANG,Yu-bo REN,Xiao-song RAO,Shao-min YANG
    2018, (6):  1098-1101.  doi: 10.19723/j.issn.1671-167X.2018.06.028     PMID: 30562789
    Abstract ( 1086 )   HTML ( 20 )   PDF (18927KB) ( 330 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Aggressive angiomyxoma is a rare mesenchymal tumor. To discuss the clinicopathological characteristics, treatment and prognosis of aggressive angiomyxoma, four cases of aggressive angiomyxoma of soft tissue in abdominopelvic cavity were collected from January 2015 to August 2017 in Peking University International Hospital. The clinical data, imaging examination, histopathological features, immunophenotype, therapy and prognosis were analysed. The related literatures were reviewed. All of the patients were adult females, age range from 27 to 49 years and mean 33 years. The clinical complaint was abdominal distention with no definite predisposing factor, or occasional physical-exam finding with no obvious discomfort. Three cases were primary and one case was recurrent. Typical layered or swirled structural sign was presented by CT and MRI scanning of three cases. All tumors located in the pelvic cavity, and attached to the uterus, vagina, rectum, bladder or ureter. One case was involved in the abdominal cavity simultaneously,adhesive to the spine, inferior vena cava and spleen. The gross appearance of tumors was from 5 to 22 cm in maximum diameter. The sectioned surfaces were soft, solid, white or yellow-gray, focally accompanied by edema, mucoid degeneration or cystic change. Microscopic observation showed that tumor cells were short spindle shaped and little atypical, the stroma was loose like ede-matous mucus or collagen, and the vessels were rich in thin and thick-wall. Partially the vessel wall expressed hyaline degeneration. Also tumors might infiltrate surrounding tissue, such as fat or nerve. The immunohistochemistry results of all cases were estrogen receptor and progesterone receptor diffusely mo-derate positive, Desmin and smooth muscle actin mostly positive, whereas CD34 expressed only in vessel and S-100 protein, CD117 and Dog1 all negative. All the tumors were complete surgical excision. During follow-up, one case recurred the second time. Our conclusions are the diagnosis of aggressive angiomy-xoma is based on pathological morphology supplemented by immunohistochemistry, and the tumor may relapse after surgical resection.

    Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a case report
    Xiao-hui ZHANG,Xue-rong DENG,Fan Li,Ying ZHU,Zhuo-li ZHANG
    2018, (6):  1102-1107.  doi: 10.19723/j.issn.1671-167X.2018.06.029     PMID: 30562790
    Abstract ( 1131 )   HTML ( 43 )   PDF (4255KB) ( 450 )   Save
    Figures and Tables | References | Related Articles | Metrics

    This case report concerns a 22-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE). She had intermittent fever, butterfly erythema, photosensitivity, oral ulcers, and multiple arthralgia in the past seven years, but she did not adhere to regular treatments. The edema of the lower extremities and face aggravated in the recent two weeks, so she was admitted to our Department of Rheumatology and Clinical Immunology. Meanwhile, we found she had severe hypertension, the maximal blood pressure was 170/120 mmHg. The patient had high SLE disease activity (the disease activity index score was as high as 23) with blood involvement, acute renal insufficiency, multiple serous effusion and rash. After one week treatments of intravenous methylprednisolone 80 mg daily and other drugs, her conditions made some extent improvement. However, she suffered sudden epileptic attacks. No positive neuro-pathological signs were found, and the blood pressure was up to 190/130 mmHg before the onset of the seizures. Her cerebrospinal fluid (CSF) pressure was 330 mmH2O, the CSF protein level was normal value, and the white blood cell count was 0 cell/mm 3, with no signs of infection. Cranial MRI showed vasogenic edema at bilateral parietal, occipito-parietal regions, and centrum ovale. We prescribed drugs of decreasing intracranial pressure, intravenous drugs of decreasing blood pressure and midazolam for sedation, without corticosteroid impulse therapy. She recovered consciousness in the next day, without epilepsy recurrence. We eventually diagnosed it as posterior reversible encephalopathy syndrome (PRES), according to the history, laboratory results, imaging featuresand clinical outcome. PRES is a disorder of reversible subcortical vasogenic brain edema in patients with acute neurological symptoms (eg, seizures, encephalopathy, headache, and visual disturbances).PRES is mainly caused by blood pressure changes or endothelial injury, which lead to breakdown of the blood-brain barrier and subsequent brain edema. Most patients have a favourable prognosis.SLE complicated with PRES is not rare, especially in patients with disease activity, hypertension, lupus nephritis and/or renal insufficiency, and use of cytotoxic drugs, early recognition and appropriate treatment remain important. Brainstem involvement, intracranial hemorrhage, renal insufficiency and high disease activity of lupus are risk factors for poor prognosis.

    Systemic lupus erythematosus with acquired hemophilia A: a case report
    Fan YANG,Yun-shan ZHOU,Yuan JIA
    2018, (6):  1108-1111.  doi: 10.19723/j.issn.1671-167X.2018.06.030     PMID: 30562791
    Abstract ( 1256 )   HTML ( 24 )   PDF (1491KB) ( 412 )   Save
    Figures and Tables | References | Related Articles | Metrics

    Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor Ⅷ (FⅧ) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions,such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FⅧ, and some cases of AHA may act as the first manifestation of SLE. AHA should be suspected in patients who have spon-taneously hemorrhagic events with an isolated prolonged activated partial thromboplastin time (APTT), reduced FⅧ activity and a negative lupus anticoagulant (LA). When FⅧ inhibitor is found, it can be diagnosed. The management of AHA focuses on the following goals: (1)controlling and preventing blee-ding,(2)eradication of the inhibitor,(3)treatment of the underlying disease. Here, a case of AHA in a patient with lupus is reported. A 53-year-old man with a 4-year history of SLE developed arthralgia and ecchymotic skin lesions after arthrocentesis of knee joint. Ultrasound confirmed the presence of an intramuscular hematoma. Coagulation tests revealed that FⅧ activity reduced to 1% and a prolonged APTT (92.2 s), FⅧ inhibitors were found to be as high as 60.0 Bethesda Units. Initial treatments with me-thylprednisolone 200 mg/d were started but new hemorrhagic manifestation occurred and hisbiological indexes were not good. Then the patient was treated with intravenous pulse corticosteroids (methylprednisolone 500 mg/d),intravenous cyclophosphamide, and also plasma and prothrombin complex infusion. Sub-sequently, FⅧ activity returned within normal ranges, FⅧ inhibitors decreased and clinical improvement was significantly obtained. The patient’s condition kept stable till now.Hemorrhagic events due to produc-tion of antibodies directed against coagulation factors were rarely observed in SLE and attentions should be paid to the association between SLE and AHA.Bypass treatment was considered as the immediate antihemorrhagic treatment. Corticosteroid combined with immunosuppressor was recommended as the main therapy to eradicate the inhibitors. However we still lack the therapeutic guide-lines and standar-dized treatment in patients of AHA with SLE at present.

    Progressive pseudorheumatoid dysplasia misdiagnosed asankylosing spondylitis: a case report
    Rui LIU,Jia-yu ZHAI,Xiang-yuan LIU,Zhong-qiang YAO
    2018, (6):  1112-1116.  doi: 10.19723/j.issn.1671-167X.2018.06.031     PMID: 30562792
    Abstract ( 1250 )   HTML ( 19 )   PDF (6783KB) ( 566 )   Save
    Figures and Tables | References | Related Articles | Metrics

    In this study, we reported a case of progressive pseudorheumatoid dysplasia in Peking University Third Hospital. A 56-year-old male patient presented with hip joint pain for more than 40 years and multiple joints pain with limitation of movements of these joints for 28 years. This patient suffered from joint pain and impaired range of motion of the hip, knee, elbow and shoulder gradually, associated with difficulty in walking and inability to take care of himself. He was diagnosed with “femoral head necrosis” or “ankylosing spondylitis” in local hospitals, but the treatment of nonsteroidal antiinflammatory drugs (NSAIDs) and sulfasalazine was not effective. Up to the age of 14, the patient displayed normal physical development, with the highest height was about 158 cm, according to the patient recall. However, his height was 153 cm at present. There was no history of similar illness in any family member. Physical examinations descried limitation of movement of almost all joints. Enlargement and flexion deformity of the proximal interphalangeal (PIP) joints of the hands resulted in the claw hand appearance. Limited abduction and internal and external rotation of the shoulder and hip could be find. He had normal laboratory findings for blood routine test, biochemical indexes and acute phase reactants such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Furthermore, HLA-B27 and autoimmune antibodies such as rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) antibody and antinuclear antibody (ANA) were all negative. X-ray of the hip showed loss of the joint space and irregularities of the femoral head, both femoral head were flattened, it could be see hyperplasia, osteophytes, bilateral femoral neck thicken, neck dry angle turned smaller. The radiological findings of the spinal vertebra indicated kyphosis deformity, narrowing of the intervertebral discs, vertebral syndesmophytes and flattening of the vertebra. However, there was no clues of bone marrow edema in the lumbar MRI. At last, genetic testing for the Wnt1-inducible signaling pathway protein 3 (WISP3) gene was done and indicated compound heterozygous mutations: 756C>G and c.866dupA. These two mutations were derived from the patient’s mother and father (the patient’s parents each had a heterozygous mutation). Two exons of the WISP3 gene had nucleotide changes leading to amino acid mutations. According to the patient’s history, symptoms, physical examinations, radiological findings and genetic testing, the final definitive diagnosis was progressive pseudorheumatic dysplasia.

    A case of gout secondary to primary myelofibrosis
    Lan-lan JI,Yan-jie HAO,Zhuo-li ZHANG
    2018, (6):  1117-1119.  doi: 10.19723/j.issn.1671-167X.2018.06.032     PMID: 30562793
    Abstract ( 954 )   HTML ( 27 )   PDF (2187KB) ( 432 )   Save
    Figures and Tables | References | Related Articles | Metrics

    A 52-year-old man was referred to our department with a 2-year history of polyarthritis. He was diagnosed as gout due to acute arthritis of bilateral feet dorsum 2 years ago,but he didn’t receive any standard treatment. 1 year ago,there were more and more joints evolved during the gout attack,and many subcutaneous nodules occurred. When he presented to our clinic 1 month ago,the urate acid level was as high as 715 μmol/L. Moreover,we could find bone erosion in the X rays of his hand and foot,as well as synovitis,double contour sign and tophus on the ultrasound examination. The diagnosis of gout was clearly and definitely. However,he had leukocytosis and thrombocytosis for 4 years in the past history,and the urate acid level was only 400 μmol/L at that time. He also had well-controlled hypertension. The family history was unremarkable. Furthermore,we found megalosplenia on his physical examination. The bone marrow examination showed myelofibrosis and JAK2 V617F gene was positive. He was diagnosed as primary myelofibrosis and treated with interferon-α,together with urate acid-lowing therapy (febuxostat 60 mg once daily). Following-up for 1 year,the dosage of febuxostat decreased to 40 mg once daily,and the patient didn’t have gout attack again,some of the tophus diminished,and the urate acid level ranged from 400 to 500 μmol/L. Gout is a common disease in clinical practice,usually combined with metabolic syndrome,chronic renal failure and specific drugs using (diuretic and calcineurin inhibitors). However,it is relatively rare to see gout associated with myeloproliferative diseases,including polycythemia vera,primary thrombocythemia,primary myelofibrosis and chronic myelocytic leukemia.In these diseases,the turnover of nucleic acids is greatly augmented,and an excess of purine metabolites,including uric acid,is released. In the natural course of gout,the appearance of tophus from the first onset of arthritis usually takes several years. This patient only had one traditional risk factor,but his urate acid level was remarkably high and he developed tophus in a short term. After treatment of primary myelofibrosis,the symptom of gout partially alleviated. Careful physical examination and medical history taking lead to the diagnosis of secondary gout,which should be reminded in the daily practice.

    Immunometabolism and systemic lupus erythematosus
    Yu-bing XIAO,Mu-yao GUO,Xiao-xia ZUO
    2018, (6):  1120-1124.  doi: 10.19723/j.issn.1671-167X.2018.06.033     PMID: 30562794
    Abstract ( 1310 )   HTML ( 87 )   PDF (879KB) ( 1013 )   Save
    References | Related Articles | Metrics

    Systemic lupus erythematosus (SLE) is a highly heterogeneous autoimmune disease,characterized by production of pathogenic autoantibodies and wide involvement of multiple systems. Damageofimmune tolerance and imbalance of immune homeostasis lead to the production of autoantibodies and the injuries of multiple organs and systems. In recent years,plenty of studies have identified that immunometabolism affects survival status of certain cells,also cell activation,differentiation and effector functions. Conversely,immune cells with different functions or differentiational status upregulate specific me-tabolic pathways to maintain their identities. In response to outer stimulations,naive immune cells dif-ferentiate into activated cells,accompanied with a series of immunometabolism changes. Therefore,abnormal immunometabolism can induce global imbalance of immune homeostasis,which further results in the initiation and development of autoimmune diseases,including SLE. Multiple abnormalities of immunometabolism have been found in patients with SLE or mouse models of lupus. Immune cells involved in the development of SLE,such as T cells,B cells,dendritic cells and macrophages present various metabolic abnormalities and pathological phenotypes. Among these cells,CD4 + T cells play predominant roles in the pathogenesis of SLE. Lots of studies demonstrated that CD4 + T cells and their subsets were in abnormal immunometabolic status,which further resulted in the development of SLE. In CD4 + T cells from patients with SLE or mouse models of lupus,both levels of glycolysis and oxidative phosphorylation are significantly higher compared with healthy controls. However,mitochondrial abnormalities,decreased ATP production and increased level of oxidative stress also have been found in these cells,which play important roles in the production of reactive oxygen intermediates and autoantibodies. Aggregated lipids rafts and increased synthesis of glycosphingolipid and cholesterol also have been observed in the CD4 + T cells from patients with SLE,leading to the abnormally elevated TCR signaling. Moreover,mechanistic target of rapamycin (mTOR) signaling is activated in the CD4 + T cells from both patients with SLE or mouse models of lupus and participate in the metabolic abnormalities of pathological CD4 + T cells. Progressive understanding of immunometabolism give us new insights of the pathogenesis of SLE and provide us with more therapeutic targets in the treatment of SLE.


WeChat public address

Sponsor: Peking University
Editor-in-Chief: ZHAN Qi-min
Executive Editor-in-Chief: ZENG Gui-fang
Editing and Publishing: Editorial Department of Journal of Peking University (Health Sciences)
ISSN: 1671-167X
CN: 11-4691/R