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Objective:To analyze the clinical characteristics and risk factors of juvenile dermatomyositis (JDM) with relapses by comparing clinical features, treatment and disease course among JDM patients with and without relapses.Methods:A retrospective analysis of 102 JDM patients from Children's Hospital of Nanjing Medical University between March 2017 and March 2021 was carried out. Patients were divided into two groups based on whether a JDM relapse had occurred or not. Initial clinical features, laboratory tests and treatment were compared between the two groups. T-test or Mann-Whitney U test was used for measurement data, chi-square test or fisher exact probability was used for count data. The features associated with risk of relapses were analyzed by multivariate logistic regression. Results:Among 102 children with JDM, twenty patients (19.6%) relapsed during drug reduction or after drug withdrawal. The mean duration to the first relapse was 3.24 years (range: 9 months to 7 years). Myositis specific antibodies (MSA) were positive for 8 (40.0%) patients with relapses. With 5 cases were anti-nuclear matrix protein 2 positive, 2 cases were anti-transcription interme-diary factor 1 gamma positive, 1 case was anti-signal recognition particle (SRP) positive, the other 12 cases were MSA negative. By binary logistic regression analysis, we found that peripheral calcinosis [ OR(95% CI)=17.54(1.55, 198.64), P=0.021], and interstitial lung disease [ OR(95% CI)=3.83(1.27, 11.59), P=0.017] were independently related to JDM with relapses. Fifty-three patients (51.9%) received methylpre-dnisolone pulse therapy for initial treatment and 13 (65.0%) patients with relapses received methylprednisolone pulse for initial treatment. There was no significant difference between the two groups ( χ2=1.70 , P=0.193). Tumor necrosis factor alpha antagonist combined with methotrexate (MTX) had achieved good results in clinical treatment in children with relapses. Conclusion:The risk of relapses is high in children with JDM. Calcinosis and interstitial lung disease at disease onset can predict a relapsing disease course. Aggressive treatment is urgently demanded for patients with JDM, especially those with relapses.
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Objective:To investigate the different effects of different treatment regimens in resistant Kawasaki disease (KD) and to provide evidence for clinical treatment.Methods:Forty-nine inpatient children with resistant KD from July 2017 to June 2019 in Children's Hospital of Nanjing Medical University were enrolled into this study. Treatment and follow-up were still in progress. Rank sum test and χ2/Fisher test were used for statisic. Results:The incidence of resistance in infliximab group was significantly lower than that of intravenous immunoglobulin (IVIG) retreated group ( P<0.05). Sixteen cases were treated with 5 mg/kg infliximab (IFX), and 33 cases received methylprednisolone and an additional dose of IVIG. Nine cases who were resistant to IVIG and methylprednisolone were treated with IFX, 6 patients responded to IFX, 3 of them were treated with cyclosporine. Coronary artery changes were followed up. Coronary artery lesions (CALs) were improved in the IFX group, CALs occurred in 12(36%) patients received IVIG and methylprednisolone, 4 of them were improved( χ2=0.633 , P=0.426). Patients were followed up for 3-24 months, the incidence of CALs persistence was statistically significantly different between the two groups (0 vs 24%, P=0.021]. Conclusion:IFX might be an effective and tolerable treatment for resistant KD.
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Objective Study and analyze the benefits and risks of unilateral thyroidectomy for 1 to 4 cm thyroid cancer in primary surgery.Methods The patients who underwent thyroidectomy for 1 to 4 centimeters WDTC (May 2014 to June 2015) were retrospectively reviewed,and the patients with preoperatively known high-risk characteristics were excluded.One hundred and seventy-one patients would have been eligible for lobectomy as the initial operation based on current American Thyroid Association guidelines.The proportion of patients who need secondary surgery was estimated basing on pathological characteristics.Result In this group,49％ patients would have to undergo a secondary surgery if they took lobectomy as the initial operation.Conclusions Patients with 1 to 4 centimeters WDTC who accept unilateral thyroidectomy may take the risk of secondary surgery.Surgeons and patients need to balance the relative benefits and risks of initial TT versus lobectomy before the operation.
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Objective To investigate the efficacy and safety of tocilizumab inpatients with refractory systemic'onset juvenile idiopathic arthritis (SoJIA),and to provide a new option for the treatment of this severe disease.Methods We retrospectively studied 25 cases of hospitalized patients with refractory SoJIA treated withtocilizumab,of whom 22 had data that fit for analysis,from May 2005 to February 2016.Data of 22 cases were collected retrospectively from physicians in charge of the patients.Children with SoJIA were treated with nonsteroidal antiinflammatory drugs (NSAIDs),Glucocorticoid (GC),methotrexate,cyclosporin A,etanerceptetc before,but still in high disease activity due to inadequate response were involved.Weretrospective analyzedthe laboratory test results like C'reactive protein (CRP),Erythrocyte sedimentation rate (ESR),Ferritin and other inflammatory index.Improvement of pain,fever,rash,hepatosplenomegaly and lymphadenectasis of active SoJIA (disease course ≥6 months,and inadequate response to NSAIDs and GC) after tocilizumab treatment (Body weight ≥30 kg,8 mg/kg;Body weight＜30 kg,12 mg/kg,per 4 weeks) were analyzed.Safety data of 22 cases were collected throughout the treatment period including neutropenia,infections,anaphylaxis and elevated liver enzymes etc.We also retrospectively analyzedthe dose change of GC and the long'term effect.Dichtomous paramenters were compared teween groups using thex2 test.Continuous parameters were compared using the analysis of uariance.Results In comparison to the indices before the treatment,the level of CRP [(8.7±2.2) mg/L vs (111.6±74.4) mg/L,F=5.192,P=0.002],ESR [(6.4±6.3) mm/1 h) vs (65.6±24.3) mm/1 h,F=50.393,P=0.000],white blood cell (WBC) [(8.4±2.5)×109/L vs (17.6±8.6)×109/L,F=9.321,P=0.000],Neutrophil count [(4.9±2.4)×109/L vs.(14.4±8.7)×109/L,F=10.541,P=0.000],blood platelet (PLT) [(269.5±79.2)×109/L vs (405.4± 145.3)×109/L,F=5.704,P=0.000] and globulin [(19.2±4.1) g/L vs (30.1±3.8) g/L,F=22.896,P=0.000] decreased rapidly and hemoglobin [(118.3±9.0) g/L vs (108.5±9.8) g/L,F=4.693,P=0.002] increased significantly at 24 weeks after Tocilizumab (TCZ) treatment.Clinical manifestationssuch as fever,rash,hepatosplenomegaly,joint swelling and pain were significantly improved.GC dose [(1.25±3.8) mg·kg-1·d-1 vs (16.2±12.8) mg·kg-1·d-1,F=8.21,P=0.000] were significantly reduced after TCZ treatment (P＜0.05);American College of Rheumatology (ACR) Pedi 30/50/70/90 was improved after TCZ treatment.Adverse events occurred in 3 cases of 25 children,who were not included in the statistical analysis group.Conclusion This retrospective case series has demonstrated the efficacy of tocilizumab in SoJIA,low incidence of adverse reactions.Further studies are needed to be developed because this case series haslimited sample size.
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Recurrent laryngeal nerve damage is one of the common complications in thyroidectomy.With the wide application of ultrasonic scalpel,intraoperative nuromonitoring,endoscopic thyroidectomy,meticulous dissection technique,the surgery methods has changed obviously,in the same time,new problems to protect recurrent laryngeal nerve in thyroidectomy has appeared.To be familiar with the anatomical characteristics of recurrent laryngeal nerve and to be skilled in using the new technique and new device are the keys to protect recurrent laryngeal nerve under new technologies and advanced conceptions.
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Recurrent laryngeal nerve damage is one of the common complications in thyroidectomy.With the wide application of ultrasonic scalpel,intraoperative nuromonitoring,endoscopic thyroidectomy,meticulous dissection technique,the surgery methods has changed obviously,in the same time,new problems to protect recurrent laryngeal nerve in thyroidectomy has appeared.To be familiar with the anatomical characteristics of recurrent laryngeal nerve and to be skilled in using the new technique and new device are the keys to protect recurrent laryngeal nerve under new technologies and advanced conceptions.
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Objective To explore the diagnosis and treatment of five children with tuberculosis with arthritis as the initial manifestation. Methods The clinical features, laboratory tests and imaging manifestation of 5 children with joint tuberculosis were retrospectively analyzed. Results The course of disease was different. All the five patients were males (mean age 8.5 ±2.9 years old) and suffered from articular symptoms as initial feature. Four of them were diagnosed and treated as rheumatoid arthritis by other hospitals for up to three years, two patients have tuberculosis contact history, and another two patients were found with bone destruction, and one patient has pathologic fracture. Conclusions Tuberculosis is easily misdiagnosed as juvenile idiopathic arthritis , which deserves attention from a pediatric rheumatology physician.
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Objective To investigate the application of intraoperative neuromonitoring (IONM) during thyroidectomy for non-recurrent laryngeal nerve (NRLN).Methods From Oct.2013 to Apr.2016,2846 patients underwent thyroidectomy with the application of IONM,and 11 patients with non-recurrent laryngeal nerve were analyzed.Results 11 cases of NRLN were all accurately identified by IONM,and no injury of NRLN occurred during thyroid surgery.Conclusions NRLN is uncommon in clinical and it is difficult to be predicted before surgery and easy to be injured.The application of IONM can reduce the possibility of NRLN injury remarkably.
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Objective To analyze the clinical features and laboratory data of 10 patients with macrophage activation syndrome (MAS) complicating systemic onset juvenile idiopathic arthritis (soJIA),which were characterized by acute severe liver injury.Methods Data of 10 patients with soJIA/MAS from Nanjing Children's Hospital were collected retrospectively.The clinical features,laboratory findings,treatment,outcomes and prognosis were analyzed.Results In the total 10 patients,female (6/10) outnumbered male.Their age ranged from 1.5 to 9.5 years old (average 5.2±2.6).The most remarkable clinical manifestations were severe liver injury without systemic features,representing as hepatomegaly (10/10),splenomegaly (2/10) and strikingly increased transaminase (10/10,median:ALT 1 445 U/L,AST 885 U/L).Central nervous system dysfunction and hemorrhages were recorded in 20％ of the patients.Two patients had pulmonary infection.Laboratory data showed that platelet count was less than normal or precaution value (10/10,≤262×10g/L).Hyperferritinaemia (10/10,median:17 329 mg/ml) and soluble CD25 elevation (median:3 140 U/ml) were common in the soJIA/MAS patients.Evidence of macrophage hemophagocytosis was found in 90％ of the patients (9/10) who underwent bone marrow aspiration.Pathological findings of liver biopsy from 1 patient revealed massive infiltration of mononuclear cells in the portal tracts.Nearly all patients (9/10) received intravenous pulse methylprednisolone therapy,combined with cyclosporine A and high-dose intravenous immunoglobulin.Eight patients had good outcome.Only 2 patients were complicated with severe interstitial lung disease during 12-months follow-up.Conclusion MAS should be considered when patients with soJIA represents acute severeliver injury without systemic features combined with other laboratory data.Intravenous pulse methylprednisolone and cyclosporine A therapy may improve the prognosis of soJIA/MAS.
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Objective To compare the clinical features, diagnosis, treatment and prognosis between macrophages activation syndrome (MAS) and other hemophagocytic syndrome (HPS). Methods Thirty-six children with HPS were identified at Nanjing Children's Hospital during January 2006 to March 2009. They could be classi-fied into MAS group (13 patients) and other HPS group (23 patients). All relevant clinical features, laboratory data, treatments and outcomes were analyzed with t test,χ2 test and Fisher's exact test.Results Patients with MAS tended to be elder than those with other HPSs [(7.7±1.3) years vs (2.6±0.5)years, t=3.899, P=0.004]. There was no difference in gender distribution. In MAS cases, the central nervous system (69% vs 13%, P=0.001), circulatory system (23% vs 9%, P=0.047) and the urinary system (38% vs 9%, P=0.033) were usually involved. The clinical symptoms of MAS were more sever than other HPS. Serum ferritin [(9703±9819) μg/L vs (4569±1396) μg/L, t=2.854, P=0.015] and erythrocyte sedimentation rate (ESR) [(53±32) mm/1 h vs (20±14) mm/1 h, t=2.708, P=0.020] changed more obviously in MAS cases compared with other HPS. Conclusion, Childhood MAS is different from other HPS in terms of age, etiology, clinical manifestations, laboratory tests and treatments.
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Objective To investigate the cosmetic results of endoscopic thyroidectomy via areola of breasts approach.Methods The incision was chosen to be along the areolar margin.One 12 mm incision was made at 3-clock position of the right areolar to introduce a 30? flexible laparoscope.Another 7 mm incision was made at 9-clock position of the right areolar for a grasping forceps.An additional 12 mm incision for the performance of ultrasonic scalpel was made at 11-clock position of the left areolar.Subcutaneous tissues were separated to create enough space for partial or subtotal thyroidectomy according to patient's conditions.Results The surgery was finished successfully in all 10 cases.The operation time ranged 110~230 min(mean,140 min).The estimated blood loss ranged 3~15 ml(mean,9 ml).There were no surgical complications after operation.Follow-up checkups for 3~5 months(mean,3.5 months) in 10 cases showed inconspicuous scars and no recurrence.Conclusions Endoscopic thyroidectomy via areola of breasts approach is a feasible and safe procedure with satisfactory cosmetic results.
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Objective:To explore the pathogenesis of coronary artery lesions in Kawasaki disease(KD) by detecting expression of CD40L on T-cells from patients with KD.Methods:Blood samples were collected from 26 patients with KD before and after intravenous immunoglobulin(IVIG) treatment. Age-matched febrile 16 children with various diseases were studied in parallel as controls. Age-matched normal control 15 children were studied as controls. CD40L expression on T-cells was detected by flow cytometry, soluble E-selection and soluble CD40L levels were measured by enzyme-linked immunosorbent assay.Results:CD40L expression on CD4+T-cells and soluble E-selection levels were significantly higher in patients with KD than that in the febrile control(FC) group and normal group(P