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Objective:To investigate the prevalence, distribution, and prognosis of knee joint bone marrow edema (BME) in children with juvenile idiopathic arthritis (JIA).Methods:From January 2017 to December 2019, 128 JIA children in the Children′s Hospital, Capital Institute of Pediatrics were analyzed retrospectively, and 136 knees were included totally. BME was evaluated and counted from eight regions according to the juvenile arthritis MRI scoring system (JAMRIS). Chi-square test, independent sample t test or Mann-Whitney U test were used to compare the clinical characteristics between BME group (36 cases) and non-BME group (92 cases). The prognosis of BME were observed. Results:BME was found in 37 of 136 knee joints (27.2%). The ages of the children in BME group and non-BME were (8±4) and (6±4) years old, and the disease duration were 9 (3, 22) and 4 (2, 18) months, respectively, both with statistically significant differences (age: t=-2.63, P=0.010; duration: Z=-5.78, P=0.013). In 23 joints (62.2%,23/37), BME occurred at multiple locations simultaneously in the knee. Locations with BME, according to the frequency of involvement from most to least, were the lateral tibial plateau with 17, the lateral weight-bearing femur with 16, the medial tibial plateau and the medial femoral condyle both with 15, the medial weight-bearing femur with 12, the lateral femoral condyle with 8, the lateral patella with 7, and the medial patella with 5. The MRI score of most of medial femoral conclyle was 1(7/15). Of the 15 BME joints with the MRI follow-up data with interval (7±3) months, BME disappeared in 10 joints, improved in 3 joints and progressed in 2 joints within 12 months after the treatments. Conclusions:There is a low incidence of BME in JIA affected knee joint. Older children and the children with long disease duration have a higher risk for BME, and more likely involved the weight-bearing surfaces of the joint. The overall prognosis is satisfactory after the standard treatments.
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Objective:To clarity the clinical features of juvenile dermatomyositis (JDM) with positive anti-melanoma differentiation associated gene 5 (MDA5) antibody.Methods:Retrospective study.Clinical data of 11 anti-MDA5 autoantibody-positive JDM patients in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2016 to January 2019 were retrospectively recruited for analyzing their clinical characteristics, pulmonary imaging and pulmonary function, thus summarizing treatment experiences.Results:A total of 11 children with anti-MDA5 autoantibody-positive JDM were recruited, involving 2 males and 9 females, with the average onset age of 1-13 (5.8±4.2) years.Clinical manifestations included rash in 11 cases (100.0%), arthritis in 5 cases (45.5%), and myasthenia in 4 cases (36.4%). Muscle enzyme elevated in 10 cases (90.9%) and serum ferritin (SF) elevated in 9 patients (81.8%). Ten cases (90.9%) showed interstitial lung disease (ILD), manifesting as ground glass opacity at subpleural area on CT scans, restrictive ventilation and decreased diffusion function on lung function test, while respiratory symptoms were absent.All patients were treated with glucocorticoid combined with immunosuppressor.Case 2 developed into rapid progressive pulmonary interstitial disease (RPILD), and died of respiratory failure 2 months later.The remaining was followed up for 1-2 years, and the ILD was relieved.Conclusions:All recruited children with anti-MDA5 autoantibody-positive JDM presented typical rash, and mild muscle weakness with a greater tendency to arthritis.Chinese pediatric patients are prone to complicate with ILD with no respiratory symptoms, but ground glass opacity at subpleural area on CT, and restrictive ventilation and decreased diffusion function on lung function test can be detected.Elevated SF is associated with the development of ILD.Glucocorticoid combined with immunosuppressive therapy is effective to JDM with ILD, but ineffective for RPILD.The mortality of anti-MDA5 autoantibody-positive JDM is high without an effective treatment.
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Objective:To investigate the clinical features, diagnosis and treatment of systemic juvenile idiopathic arthritis (SJIA) complicated with macrophage activation syndrome (MAS).Methods:From January 1st, 2018 to January 1st, 2020, 7 cases of SJIA-MAS were diagnosed. Their clinical and laboratory data were collected and summarized.Results:In these 7 cases, 2 were males and 5 were females, the ratio of male to female was 2∶5. The age range was 11 months to 2 years old. The course of disease was 14 to 32 days. The clinical manifestations included fever and rash in 7 without arthritis; hepatomegaly, splenomegaly and lymphadenopathy in 7; hematological involvement in 7; nervous system involvement in 2; digestive system involvement in 7; respiratory system involvement in 7; cardiovascular involvement in 3. White blood cell was decreased in 1 case, platelet was decreased in 1 case and hemoglobin was decreased in 7 cases. Ferritin, triglyceride, alanine transaminas and aspartate aminotransferase were increased in 7 cases, fibrinogen was significantly decreased in 7 cases, and direct bilirubin was increased in 4 cases. IL-2R was significantly increased. Hemophagocytosis was observed in bone marrow of 4 cases. Cerebrospinal fluid protein was 2 005 mg/L in 1 case. All the 7 cases were tested for exon genes, and no pathogenic mutation was found. All of the 7 cases showed lung lesions in chest CT scan. Multiple demyelinating lesions were found in 1 case by head magnetic resonance imaging. One case was treated with high-dose intravenous methylprednisolone combined with IL-6 receptor antagonist(tocilizumab). The other 6 cases were treated with high-dose intravenous methylprednisolone combined with cyclosporine A (CsA). Two cases were treated with Janus kinases inhibitor(tofacitinib). After treatment, 7 cases got relieved, no death, no recurrence oocurred during the follow-up.Conclusion:Acute onset, multiple organ involvement and no joint inflammation are prominent in MAS of infants and toddlers. High fever, proressive reduction of blood cells and increase of SF are significant in SJIA-MAS. High dose glucocorticoid combined with CsA can benefit in most cases, and some severe cases need to be treated with biological agents.
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Objective:To explore the imaging manifestation and the value of imaging follow-up in Takayasu arteritis with pulmonary artery involvement in children.Methods:The data of Takayasu arteritis with pulmonary artery involvement in 7 children in Children Hospital, Capital Institute of Pediatrics from July 2014 to June 2019, were summarized retrospectively, including 1 boy and 6 girls. There were 6 children under 1 year old, the age ranged from 2 to 7 months and the median age was 3 months old. Another child was 12 years old. CT images of all cases in the initial diagnosis and during the follow-up were reconstructed. The diameters and wall thickness of arteries were observed, including ascending aorta, main pulmonary artery, left and right pulmonary artery and each lobe pulmonary artery. The diameter ratio of main pulmonary artery to ascending aorta (MPA/AAO) was calculated. The pulmonary artery pressure, the diameters of left and right coronary artery were measured using echocardiography and compared in the initial diagnosis and during the follow-up.Results:All the 7 cases showed main pulmonary artery, left and right pulmonary artery widened, 6 cases showed lobe pulmonary arteries widened, 2 cases showed main pulmonary artery, left and right pulmonary artery wall thickened, and 6 cases showed lobe pulmonary arteries wall thickened. All the 7 cases showed MPA/AAO>1 in the initial diagnosis, but only 1 case (12-year-old girl) presented pulmonary hypertension from echocardiography. All the 7 cases showed coronary artery dilated in different degrees and ranges in the initial diagnosis, including 3 cases with bilateral coronary artery dilatation and 4 unilaterally. Five children were followed up and showed improvement of pulmonary artery dilatation in 4 cases and progress in 1 case. MPA/AAO decreased in 4 cases and increased in 1 case. The pulmonary hypertension of the 12-year-old patient decreased during the follow-up, and the MPA/AAO also decreased from 1.64 to 1.01. Coronary artery dilatation decreased in 4 of 5 cases during the follow-up.Conclusion:CT imaging can clearly show pulmonary arteries, which plays an important role in the diagnosis and assessment of the treatment effect in Takayasu arteritis with pulmonary artery involvement in children.
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Objective To investigate the safety and feasibility of robotic nephrectomy,work bench surgery with robotic kidney autotransplantation in the treatment of complex renal tumors.Methods The clinical data of 5 patients with renal tumors admitted from January 2018 to July 2018 were analyzed retrospectively.There were 4 males and 1 females.The median age was 49 years old,ranging 32-66 years.The median body mass index was 25.6 kg/m2,ranging 21.1-27.8 kg/m2.Serum creatinine level was 87.2 μmol/L,ranging 78.0-88.9μmol/L before bench surgery.5 patients had multiple bilateral renal tumors and had undergone laparoscopic or robotic partial nephrectomy on the contralateral kidney.For bench surgery kidney,4 cases were on the left side and 1 case was on the right side.Each kidney has more than 2 separate tumors,combined with complete endophytic tumors,tumors larger than 7 cm in diameter or hilar tumors.5 patients were all performed robotic nephrectomy,work bench partial nephrectomy with robotic kidney autotransplantation under general anesthesia.The patient was first in a lateral decubitus position for robotic nephrectomy,and the kidney was removed through a median 6 cm periumbilical incision.After kidney removal,kidney tumors were resected and kidney was reconstructed on a hypothermic working table.Then the kidney was packed in a plastic bag,filling with ice slush.The corresponding parts of the plastic bag were cut to expose the renal artery and vein.Finally,the patient was moved to lithotomy position with Trendelenburg tilt of 20°,and the autologous kidney wrapped in the plastic bag was placed through the previous periumbilical incision into the abdominal cavity for robotic kidney autotransplantation.The renal artery and vein were anastomosed end-to-side with the right external iliac artery and vein.The ureter and bladder were anastomosed.Autologous kidneys were placed in abdominal cavity in 4 cases,and placed in right iliac fossa with retroperitonealization in 1 case.Ice slush on the surface of the autologous kidney did not completely melt before the blood supply was restored during the operation,and the autologous kidney immediately urinated after the blood supply was restored.Results All surgeries were performed successfully without conversion to open surgeries.The total operation time was 460 min,ranging (415-645 min),the time of robotic nephrectomy was 120 min,ranging (74-300 min),the time of robotic kidney autotransphntation was 135 min,ranging(103-163 min),the warm ischemia time was 3 min,ranging (1.5-6.0 min),the cold ischemia time was 182 min,ranging(135-210 min),the rewarming time was 50 min,ranging(45-55 min),the estimated blood loss during operation was 100 ml,ranging(50-300 ml),and the hospital stay was 6 d,ranging(5-9 d).The number of resected tumors was 4,ranging(2-6).The pathology reveals clear cell carcinoma in 3 cases and chromophobe cell carcinoma in 2 cases.The surgical margins were all negative.The serum creatinine levels were 111.1 μmol/L (87-217.6 μ mol/L) and 106.1 μmol/L (87.1-172 μmol/L) on the 7th and 30th day after operation,respectively.One month after operation,CT showed that the function and morphology of the autologous kidneys were fine.No recurrence or metastasis was found in 5 patients during a median follow-up of 7 months,ranging (5.4-11.7 mon).Conclusions For patients with complex renal tumors who cannot undergo in situ partial nephrectomy,robotic nephrectomy,work bench surgery with robotic kidney autotransplantation can completely remove the tumors,maximize the preservation of renal function and minimize the trauma of patients,making the ultimate means of nephron-sparing surgery for patients with complex renal tumors more minimally invasive and safe.
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Objective@#To evaluate the efficacy and side effects of tocilizumab for the treatment of systemic juvenile idiopathic arthritis.@*Method@#In this prospective self case-control study, the children diagnosed with refractory systemic juvenile idiopathic arthritis admitted to Department of Rheumatism and Immunology of Children's Hospital Affiliated to Capital Institute of Pediatrics from December 2013 to June 2016 were enrolled and information before and after treatment of tocilizumab was analyzed. The tocilizumab was introvenously guttae in a dose of 8-12 mg/kg every 2 weeks. Complete blood count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) were tested before and after the application of tocilizumab. Detailed clinical manifestations were recorded. All results were analyzed by χ2 test and t test.@*Result@#Forty patients with a median age of (6.6±3.7) years were enrolled, including 15 males and 25 females. All of the patients presented with fever and 38 patients got normal temperature 24-48 hours after treatment with tocilizumab. Symptoms disappeared in 13 and improved in 4 patients after treatment among the 17 patients who presented with arthritis. Within the 10 patients who manifested with rashes, 9 patients' rashes disappeared without relapse accompanied by the normalization of temperature after the treatment of tocilizumab. One patient got normal temperature but intermittently emerged rashes after symptoms of arthritis improved. In the 40 patients, 38 well tolerated tocilizumab while 2 showed rashes and chill which disappeared shortly after antianaphylaxis treatment. No severe treatment-related infection was found in any patients. According to the study, the white blood cell counts(×109/L), CRP(mg/L) and ESR(mm/1h) tested 2 weeks after the treatment with tocilizumab were significantly lower than that before treatment(12.1±1.2 vs. 16.5±1.8, 47±8 vs. 67±9, 21±5 vs. 57±6, t=2.75, 3.98, 5.22, P=0.009, 0, 0, respectively). No significant changes were found in concentration of IL-6 and TNF-α (65(207) vs. 45(137) ng/L, and 14(6) vs. 17(19)ng/L, Z=-1.247 and-1.285, P=0.212 and 0.199 respectively).@*Conclusion@#Tocilizumab is a treatment with good efficacy and safety for refractory systemic juvenile idiopathic arthritis. Adverse effects would be found in some patients.
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@#With the deepening of research, different new anti-diabetic drug targets have been discovered and reported, several categories of anti-diabetic drugs(linagliptin, exenatide, dapagliflozin, etc. )have been brought to the market and some new drugs acting on different targets are in late-stage clinical trials. All these progresses provide new means for overcoming diabetes. GPR119, GPR120, GPR40, AMPK, apelin receptor and GSK3β are anti-diabetic targets with great research values in current days and the future. This article reviews the mechanisms, drugs and research advances with respect to the above-mentioned targets.
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Central venous catheters are indispensable in modern-day medical practice in neonatal intensive care units.Infection is the most frequent serious complication during these catheter-inserted days.Several strategies to prevent catheter-related infections are disputed all over the world.Studies have shown that some measures played a role in reducing the infection rate,which include maximal sterile barrier precautions,chlorhexidine preparation for skin antisepsis,routine replacement of catheter site dressings and connection sets,time-limited catheter insert,removal catheter after infected,antibiotic locked catheters and so on.
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BACKGROUND: Color Doppler flow imaging can exam the early myocardial disorder in type 2 diabetic patients. What does exercise tolerance test work for the examination of such disorder in combination with color Doppler flow imaging (CDFI) ?OBJECTIVE: To analyze in comparison the early evaluation on reduced diastolic function in left ventricle in patients with type 2 diabetes between the examinations of exercise tolerance test combined with color Doppler flow image and simple color Doppler flow imaging.DESIGN: Cases-controlled comparison and self-comparison.SETTING: Department of electrodiagnosis and department of Endocrinology in a municipal hospital.PARTICIPANTS: Thirty-six cases of inpatients with type 2 diabetes were selected from Department of Endocrinology of Shenyang Red Cross Hospital from March to December in 2004, of which, 25 cases were males and 11cases females. The diabetic patients included had no cardiac vascular complications and participated in the study in volunteer. Thirty-two patients who received annual routine health check at the same period were selected as the control, of which, 20 cases were males and 12 cases females.METHODS: Metronics treadmill exercise test equipment was used for exercise tolerance in two groups. Before exercise(at quiescent state) and after exercise tolerance, Vivid 4 CDFI was used to determine flow velocity at E and A peak values respectively, ratio between flow velocities at E peak value and A peak value as well as isovolumtric relaxation time (IRT).MAIN OUTCOME MEASURES: Comparison of cardiac functional indexes before and after exercise tolerance in two groups.RESULTS: Thirty-four diabetic patients accomplished exercise tolerance test, of which, 1 case presented frequent ventricular extrasystole, another one presented precordial pain and stopped the test. In the control, all of 32 cases ratio between flow rates of E peak value and A peak value was remarkably lower than that in the control (0. 90 ± 0. 25, 1.40 ± 0.30, P ＜ 0.05 ); IRT was remarkably longer than that in the control [ (112. 07 ± 20. 16),imental group, the ratio between flow rates of E peak value and A peak value was remarkably lower than that in the control (0.62 ±0. 12, 1.28 ±0.87, P＜ 0.01 ); IRT was remarkably longer than that in the control[ (138. 10± 19.21), (97.37±9.61) ms, P ＜0.01].CONCLUSION: CDFI can supervise and evaluate at early stage the cardiac functional changes in type 2 diabetic patients. Due to the induction of exercise tolerance, the combination of exercise tolerance test and CDFI provides more accurate, objective and valuable conclusions at early stage.