Objective:To investigate the clinical characteristics, muscle pathological features and treatment in patients with Juvenile idiopathic inflammatory myopathy (JIIM) with positive anti-nuclear matrix protein 2 (NXP2) antibody.Methods:The clinical data of 8 IMM patients with positive anti-NXP2 antibody were collected and the clinical manifestations, auxiliary examinations, muscle pathological changes and therapeutic effects were retrospectively analyzed.Results:The ratio of male to female was 1:3. The median age of disease onset was (6.1±3.8) years. Eight cases had proximal muscle weakness, 7 had dermatomyositis-like rash, 5 had myalgia,4 had calcinosis,3 had skin ulcer, 2 had edema and 1 had abdominal pain. Five cases had elevated serum creatine kinase. Eight cases with lower limb muscle MRI showed abnormal signals in muscle, space between muscles and fat tissue, 3 cases with chest high-resolution CT (HRCT) showed interstitial lung disease. Abdominal CT of 1 case showed irregular thickening, edema and peripheral inflammatory exudation in ascending colon and proximal transverse colon. Pathological biopsy of skeletal muscle showed perifascicular atrophy, inflammatory cell infiltration in fascicular membrane and around small vessels and muscle fiber space. Edema, hyperplasia could be seen in interstitium; but dissolved necrosis, and regenerated muscle fibers were rarely seen. Treatments included glucocorticoids, immunosuppressive agents and biological agents (1 case). After 6 months of follow-up, 5 cases had good outcomes and 3 cases had poor outcomes.Conclusion:Dermatomyositis is the major clinical manifestation of idiopathic inflammatory myopathy with positive anti-NXP2 antibody.It is associated with myasthenia, calcinosis, skin ulcers and intestinal vasculitis. The pathological changes in skeletal muscle are relatively slightmild. Glucocorticoids combined with immunosuppressive agents are effective in most cases.

Objective:To investigate the therapeutic efficacy of Golimumab in the treatment of children with refractory juvenile dermatomyositis(JDM).Methods:The clinical data of a child diagnosed with JDM in the Department of Allergy, Immunology and Rheumatology of Guangzhou Women and Children′s Medical Center in February 2019 were collected.The treatment effect was studied and literature review was conducted.Results:The patient was a 7-year-old boy with subacute onset of the disease.The illness protracted, and main manifestations included skin rashes, limb weakness, and swallowing dysfunction.Physical examination showed heliotropic rashes, Gottron papules, positive Gower, proximal limb muscle strength grade Ⅲ-Ⅳ, distal limb muscle strength grade Ⅳ, and a choking cough when swallowing fluid food.Laboratory tests revealed alanine aminotransferase (ALT) of 36 U/L, aspartate aminotransferase (AST) of 115 U/L, alkaline phosphatase of 69 U/L, lactate dehydrogenase of 941 U/L, creatine kinase of 974 U/L, hypersensitive C-reactive protein of 26 mg/L and an erythrocyte sedimentation rate (ESR) of 52 mm/1 h. Antinuclear antibody spectra were negative.Electromyography suggested myogenic damage.Thigh magnetic resonance imaging indicated diffuse abnormal signal shadows in the subcutaneous fat, muscles and muscle spaces of both hips, thighs and knee joints.The child was diagnosed with JDM, and given standardized treatment of Methylprednisolone, intravenous immunoglobulin, Methotrexate and Hydroxychloroquine sulfate.However, after the treatment, the facial rashes were still red, proximal limb muscle strength and swallowing dysfunction did not improve, the choking cough symptom still existed, and a Cushing face appeared.Recheck results showed ALT of 24 U/L, AST of 32 U/L, alkaline phosphatase of 56 U/L, lactate dehydrogenase of 216 U/L, creatine kinase of 527 U/L, hypersensitive C-reactive protein of 8 mg/L and an ESR of 15 mm/1 h. Refractory JDM was considered.After negotiating with the patient′s family members, they agreed to treat the patient with Golimumab 50 mg by subcutaneous injection once a month.Then tapered prednisone gradually, stopped Hydroxychloroquine sulfate tablets and continued to give the patient oral Methotrexate.After two doses of Golimumab 50 mg, proximal limb muscle strength and swallowing function improved markedly.After the third subcutaneous injection of Golimumab, proximal limb muscle strength improved to grade Ⅳ-Ⅴ, and he was able to go up and down stairs, squat and stand up after squatting.Besides, dysphagia and the choking cough disappeared, and skin rashes improved.Recheck results suggested a normal ESR and creatine kinase levels.Magnetic resonance imaging of thighs indicated no muscle inflammation.Conclusions:Golimumab works well in the treatment of refractory JDM and can effectively improve muscle strength.Therefore, it can be used as a treatment option for refractory JDM.

Objective:To investigate the clinical effect of treatment of valgus impacted proximal humeral fracture in elderly patients with locking plate internal fixation through deltoid approach.Methods:A retrospective case series study was carried out to analyze the clinical data of 25 elderly patients with valgus impacted proximal humeral fracture hospitalized in Central Theater General Hospital of PLA from September 2016 to March 2018, including 7 males and 18 females, aged 60-81 years [(67.9±9.1)years]. According to Neer classification, 21 patients were with three part proximal humeral fracture and 4 with four part fracture. All patients were treated by internal fixation with locking plate through deltoid approach. Rehabilitation exercise training was done after operation. Total length of incision, operation time, intraoperative bleeding volume and fracture healing were recorded. At the last follow-up, the range of motion and abductor muscle strength of the shoulder joint between the affected side and the healthy side were compared, joint function was evaluated using the Constant-Murley score, and the postoperative complications were observed.Results:All patients were followed up for 12-23 months [(14.6±3.2)months]. Total length of incision was 6.4-8.8 cm [(7.1±1.2)cm]. Operation time was 68-106 minutes[(84. 0±5.6)minutes]. Intraoperative bleeding volume was 55-95 ml [(62.5±16.4)ml]. All the fracture lines were well reduced and bony union was observed in all patients at popstoperative 6 months. At the last follow-up, there was no significant difference in flexion, abduction, pronation range and abductor muscle strength between the affected side and the healthy side ( P<0. 05). At the last follow-up, the Constant-Murley score of shoulder joint function was excellent in 9 patients, good in 13, fair in 3, with excellent and good rate of 88%. There was no subacromial impact, humeral head necrosis or other complications after operation. Conclusion:Locking plate through deltoid approach in treatment of valgus impacted proximal humeral fracture in the elderly has the advantages of less operative trauma, shorter operative time, less intraoperative bleeding, less postoperative complications, good reduction of fracture and good functional recovery of shoulder joint.

Objective:To evaluate the efficacy of lateral deltoid muscle approach in the treatment of valgus-impacted three- and four-part fractures of the proximal humerus.Methods:From January 2015 to December 2017, 36 patients were treated at Department of Orthopaedics, General Hospital of Central Military Theater for valgus-impacted three- and four-part fractures of the proximal humerus via the lateral deltoid muscle approach. They were 10 men and 26 women, aged from 38 to 85 years (mean, 65.6 years). The fracture had occurred at the left side in 20 cases and at the right side in 16. According to the Neer classification, 22 cases had a three-part fracture and 14 a four-part one. Their operation time and intraoperative bleeding were recorded. The efficacy was evaluated by their Constant-Murely scores and ranges of motion of the shoulder joint one year after operation.Results:This cohort was followed up for 12 to 36 months (mean, 21.7 months). Their operation time ranged from 55 to 120 min (mean, 75.9 min); their intraoperative bleeding ranged from 50 to 200 mL (mean, 109.7 mL). Their Constant-Murely scores of the affected shoulder one year after operation ranged from 79 to 90 points (mean, 84.1points). At one year after operation, their abduction ranged from 90° to 160° (mean, 120.6°), their uplift from 90° to 170° (mean, 128.1°), their backward extension from 20° to 60° (mean, 38.8°), their internal rotation from 20° to 45° (mean, 30.5°), and their external rotation from 30° to 45° (mean, 35.6°). All patients obtained bony union with no such complications as axillary nerve injury, deep infection, nonunion or ischemic necrosis of the humeral head.Conclusion:Treatment of valgus-impacted three- and four-part fractures of the proximal humerus via the lateral deltoid muscle approach is minimally invasive, simple, safe and effective.

Objective: By studying the efficacy of interleukin (IL)-6 receptor antagonist (tocilizumab) on acute inflammation of systemin juvenile id-iopathic arthritis (sJIA) and its effect on the downstream signaling pathways and inflammatory factors of IL-6 to further reveal the role of tocilizumab in sJIA. Methods: From December 2015 to December 2018, 64 sJIA children were randomly divided into two groups: 31 cases who were treated with tocilizumab+ glucocorticoid+disease-modifying anti-rheumatic drugs (DMARDs) as the tocilizumab group, 33 cases who were treated with placebo (vitamin C) + glucocorticoid+DMARDs as the control group. They were treated for one year. The levels of IL-2, IL-4, IL-6, IL-10 and tumor necrosis factor (TNF)-α were detected by enzyme-linked immunosorbent assay (ELISA). The expressions of p65 and receptor activator for nuclear factor-κB ligand (RANKL) in peripheral blood mononuclear cells (PBMCs) were detected by quantitative polymerase chain reaction (qPCR). The expressions of signal transducer and activator of transcription (STAT3)/phosphates signal transducer and activator of transcription 3 (p-STAT3)/suppressor of cytokine signaling 3 (SOCS3) before and after treatment were detected by Western blotting. The differences between groups were analyzed by variance analysis. Normal distributed data was tested by K-W test. Twenty normal control subjects came from the pediatric clinic in our hospital. Results: There was no significant difference in the demographic data between the two groups (P>0.05). Among them, 2 children who were treated with tocilizumab dropped out after one month treatment and three months due to un-affordability respectively. The C-reactive protein (CRP), ferritin (FER), erythrocyte sedimentation rate (ESR) in the tocilizumab treatment group decreased significantly after 6 months and 1 year when compared with the disease control group. The concentration of IL-6 in the tocilizumab group (77±46) pg/ml, control group (82±40) pg/ml were higher than that in the healthy control group (10±3) pg/ml (F=4.683, P=0.001; F=2.581, P=0.03). After one year, the concentration of IL-6 (316±42) pg/ml in the tocilizumab group was higher than that in the disease control group (62±40) pg/ml (F=11.2, P=0.001). The expression of RANKL and p65 mRNA in treatment group was significantly higher than that in healthy control group (K-W=10.03, P<0.01; K-W=9.42, P<0.01). After one year, the expression of RANKL and p65 mRNA in treatment group was lower than that in disease control group (K-W=9.964, P<0.01; K-W=10.75, P<0.01). The expression of STAT3/p-STAT3/SOCS3 in disease control group before medication was significantly higher than that in healthy control group, while the expression of p-STAT3/SOCS3 in the treatment group was significantly higher than that in healthy control group. The expression of STAT3/p-STAT3 in the tocilizumab group was significantly lower than that in the disease control group (K-W=12.54, P<0.01; K-W=10.52, P<0.01). Conclusion Tocilizumab can effectively alleviate the symptoms of sJIA in active phase, down-regulate the expression of STAT3/p-STAT3 protein, thereby reducing the transcription of downstream nuclear factor (p65, RANKL) mRNA, thereby affecting the proliferation of synovial cells and reducing bone destruction, but has no significant effect on the secretion of IL-6.

OBJECTIVE: To explore whether MRI fusion technology (combined T2-weighted imaging [T2WI] and fat-suppressed T2WI [T2WI-(FS)]) improves signal differences between anal fistulas and surrounding structures. MATERIALS AND METHODS: A total of 32 patients with confirmed diagnoses of anal fistula were retrospectively studied. All available T2WI and T2WI-(FS) images for each patient were used to generate fusion image (T2WI-(Fusion)) based on the addition of gray values obtained from each pixel via an MR post-processing work station. The discriminability of fistula, perianal sphincter, and perianal fat in T2WI, T2WI-(FS), and T2WI-(Fusion) images was quantified with Fisher's scoring algorithm. For subjective visual image assessment by researchers, five-point scale scores were determined using a modified double-stimulus continuous quality-scale test to evaluate T2WI-(FS), T2WI, enhanced axial three-dimensional-volumetric interpolated breath-hold examination (3D-VIBE), and T2WI-(Fusion) sequence images. The differences were subsequently compared. RESULTS: Mean Fisher scores for fistulas vs. sphincters obtained from T2WI-(Fusion) (F(Fusion-fistula) = 6.56) were significantly higher than those from T2WI (F(T2WI-fistula) = 3.35) (p = 0.001). Mean Fisher scores for sphincters vs. fat from T2WI-(Fusion) (F(Fusion-sphincter) = 10.84) were significantly higher than those from T2WI-(FS) (FS(FS-sphincter) = 2.57) (p = 0.001). In human assessment, T2WI-(Fusion) showed the same fistula discriminability as T2WI-(FS), and better sphincter discriminability than T2WI. Overall, T2WI-(Fusion) showed better discriminability than T2WI, T2WI-(FS), and enhanced 3D-VIBE images. CONCLUSION: T2WI and T2WI-(FS) fusion technology improves signal differences between anal fistulas and surrounding structures, and may facilitate better evaluation of anal fistulas and sphincters.
Anal Canal ; Diagnosis ; Fistula ; Humans ; Magnetic Resonance Imaging ; Rectal Fistula ; Retrospective Studies

OBJECTIVETo compare the difference of the diameters of superior mesenteric vein (SMV) and gastrocolic trunk (GCT) between patients with cecum-ascending colon cancer and normal individuals, and to assess the diagnostic value of the diameters of SMV and GCT in cecum-ascending colon cancer.

METHODSPreoperative imaging data of 60 patients with primary cecum-ascending colon cancer confirmed by postoperative pathology at the First Affiliated Hospital of Sun Yat-sen University from June 2014 to December 2016 were retrospectively analyzed. The diameters of SMV and GCT were measured on preoperative CT images. SMV was measured at about 2 cm below the junction of SMV and splenic vein. GCT was measured at 1 cm near the proximal junction of right colon vein, right gastroepiploic vein and anterior pancreaticoduodenal vein. Another 60 people receiving pelvic CT examination without organ illness were collected as control. The diameter differences of SMV and GCT between cancer group and control group were compared. The diagnostic value of the diameters of SMV and GCT in cecum-ascending colon cancer was evaluated by receiver operating characteristic (ROC) curves.

RESULTSAmong 60 cases of cecum-ascending colon cancer, 36 were males and 24 were females with median age of 48 years (range 28-84); 13 were cecum cancer, 47 were ascending colon cancer; 11 had no lymph node and liver metastasis, 40 had lymph node metastasis, 9 had liver metastasis (all with lymph node metastasis). Compared to control group, the diameters of SMV and GCT in cancer group were significantly longer [SMV:(11.2±1.3) mm vs. (9.5±1.7) mm, t=6.04, P<0.001; GCT:(5.5±0.9) mm vs. (3.5±1.0) mm, t=11.51, P<0.001]. However, there were no statistically significant differences in diameters of SMV and GCT among hepatic metastasis, lymph node metastasis and no metastasis cancer groups (all P>0.05). The ROC curve analysis showed that the area under the curve of SMV diameter was 0.777, and the optimal cut-off point was 10.5 mm in the diagnosis of cecum-ascending colon cancer, with the sensitivity and specificity of 95.0%(57/60) and 46.7%(28/60) respectively. The area under the curve of GCT diameter was 0.923, and the optimal cut-off point was 4.5 mm in the diagnosis of cecum-ascending colon cancer, with sensitivity and specificity of 88.3%(53/60) and 85.0%(51/60) respectively.

CONCLUSIONThe dilation of the SMV and GCT may be used as warning factors for cecum-ascending colon cancer, especially the diameter of GCT.

Adult ; Aged ; Aged, 80 and over ; Cecum ; Colon, Ascending ; pathology ; Colonic Neoplasms ; pathology ; Female ; Humans ; Male ; Mesenteric Veins ; anatomy & histology ; Middle Aged ; Retrospective Studies

Objective To investigate application of modified isosceles triangle osteotomy which is designed with the assistance of Picture Archiving and Communication System ( PACS ) in supracondylar surgery for cubitus varus. Methods We reviewed the 31 patients who had been treated for cubitus varus from January 2012 to July 2017. They were 12 males and 19 females, aged from 17 to 24 years ( average, 20. 6 years) . Their elbow varus angles ranged from 14° to 35° (average, 22. 4°) . Preoperatively, the modified isosceles triangle osteotomy was designed using the PACS. The angle of osteotomy = carrying angle of the normal arm + the angle of cubitus varus. The lateral length of the isosceles triangle osteotomy was calculated according to the osteotomy angle. All the cases were fixated with a locking plate. Results The follow-ups lasted from 12 to 40 months ( average, 16 months ) . Bony union was achieved in the osteotomy site by all cases after 7 to 12 weeks ( average, 9 weeks ) . The carrying angles ranged from 8° to 15° ( average, 11°) at final follow-ups. The affected elbow obtained a range of flexion and extension from 126° to 150° ( average, 139°) and a range of rotation from 134° to 160° ( 144°) . According to the Mayo elbow performance score ( MEPS ) one year after operation, 23 cases were rated as excellent, 7 as good and one as fair, yielding an excellent to good rate of 96. 8％. No loss of carrying angle, neural deficit, malunion, delayed union, or myositis ossifi-cans of the elbow was observed during the follow-ups. Conclusion The PACS can be used in preoperative design of the modified isosceles triangle osteotomy for cubitus varus, leading to accuracy in the angle and length of the osteotomy to guarantee fine clinical results.

Objective To summarize the characteristics of deltoid ligament rupture and explore the feasibility and shortterm clinical outcomes of targeted suture anchor repair technique according to the rupture site.Methods From May 2011 to October 2014,19 cases of complete deltoid ligament rupture (17 males and 2 females) were recruited in this study,with an average age of 34.15± 1.23 years (ranged from 15 to 60 years).According to Lauge-Hansen classification,there were 7 cases of pronation external rotation grade ⅣV injury,including 3 cases of Maisonnuve fracture;1 case of pronation abduction type Ⅲ degree injury,1 case of pronation abduction grade ⅣV injury;and 10 cases of supination external rotation grade ⅣV injury.According to AO / OTA classification,there were 9 cases of 43B type injury and 10 cases of 43C type injury.According to the rupture site of deltoid ligament,the targeted suture anchor repair surgery was operated respectively.Early mobilization with the help of hinged ankle brace was encouraged.The evaluation at last follow-up was based on the American Orthopedic and Ankle Association (AOFAS) criteria of ankle and hindfoot,and the visual analogue scale (VAS) scoring system.Results Nineteen patients were all followed up for 24 to 48 months,with an average of 30.42±5.11 months.Fourteen cases (73.7％,14/19) with talus end avulsion were treated by double suture anchor in the talus,with continuous locking suture of the avulsed end.Four cases (21.1％,4/19) with middle part rupture were treated by double suture anchor in the talus,with the sutures crossing three bone tunnels at the medial malleolus.One case (5.3％,1/19) with medial malleolus end avulsion was treated by single suture anchor at the medial malleolus,with continuous locking suture of the avulsed end.At the last follow-up,the AOFAS score was ranged from 70 points to 96 points,with an average of 90.53 points,and excellent in 16 cases,good in 2 cases,fair in 1 case,excellent and good rate was 94.7％.The VAS score was ranged from 0 to 2 points,with an average of 0.42 point.No wide medical clear space was detected.But traumatic arthritis was happened in 2 patients.Conclusion The targeted suture anchor repair technique according to the rupture site was a save technique in treating deltoid ligament rupture,which is conducive to early postoperative functional exercise,with excellent short-term clinical outcomes and few complications.

[Objective] To measure the diameter of the superior rectal vein (SRV) & theinferior mesenteric vein (IMV) by CT and analyze their relationship with lymphatic metastasis of rectal carcinoma.And to discuss the feasibility of utilizing SRV to prcdict lymph node metastasis of rectal cancer.[Methods] The CT imaging and pathological data of 105 rectal carcinoma patients were analyzed retrospectively.We measured and compared the diameter of every patient's SRV & IMV by CT in the presence and absence of microvascular tumor thrombus,signet ring cell,lymph node metastasis and distant metastasis.The accuracy of predicting lymph node metastasis for rectal carcinoma by the diameter of SRV were evaluated by ROC curve.[Results] There were statistical differences in the diameters of SRV and IMV between rectal cancer group with lymph node metastasis (D =4.34 mm,D =5.00 mm) and without (D =3.56 mm,D =4.81 mm;P ＜ 0.001,P =0.023).The differences were significant in the diameter of SRV between rectal cancer group with microvascular tumor thrombus or signet ring cell and without (P =0.019,P =0.044).However,the diameter of IMV showed no statistical difference between rectal cancer group with microvascular tumor thrombus or signet ring cell and without (P =0.605,P =0.663).And there was no statistical difference in the diameter of SRV or IMV between rectal cancer patients with distant metastasis and without (P ＞ 0.05).Regarding 3.75 mm as the cut-off value for the diameter of SRV to predict lymph node metastasis for rectal cancer patients,the sensitivity was 90.9％ and the specificity was 82.0％.Regarding 4.65 mm as the cut-off value for the diameter of IMV to predict lymph node metastasis for rectal cancer patients,the sensitivity was 87.3％ and the specificity was 38％.[Conclusions] It is completely feasible to utilize the diameter of SRV measured by CT to predict lymph node metastasis of rectal carcinoma with high sensitivity,accuracy,and relatively high specificity.