Objective　To better understand the clinical characters of juvenile onset spondyloarthropa-thies (JSpA).Methods　The clinical and laboratory data of 190 in-patients with JSpA were analyzed and the diagnosis,classification and differentiation of juvenile onset arthritis were discussed.Results　Among these 190 patients,163 were male,with a male to female ratio 6∶1.Of them 92.1% had the disease after the age of 8.Peak of age at onset was 12 to 15 years;157(82.6%) patients had peripheral arthritis and only 23(12.1%) patients felt low back pain at onset.During the disease course, peripheral arthritis was found in 187(98.4%) patients and the history of low back pain or buttock pain was recorded in 123(64.7%).The interval between peripheral arthritis and low back pain was from 0 to 20 years,with an average of (3.2±4.5)years.Extra-articular features including enthesitis in 67(35.3%)patients,dactylitis in 20(10.5%),iritis in 9(4.7%) were observed.HLA-B27 was positive in 87.9%(160/182) patients.Sacroiliitis on X-ray was observed in 76.0%(136/179) patients,and 106(55.8%) patients were diagnosed juvenile ankylosing spondylitis (JAS) according to 1984 New York modified criteria.The average disease course in JAS was (6.3±6.2) years,longer than that in JSpA (P＜0.01).Conclusion　The concept of JSpA is helpful to early diagnosis and treatment of juvenile onset arthritis.The JSpA are characterized by asymmetric lower limb predominant oligoarthritis,a wide spectrum of extra-articular features,presence of HLA-B27 and familial history of SpA or psoriasis.It will take an average of 6.3 years for JSpA patients to fulfill the diagnostic criteria of adult AS.

Objective To study the characteristics of IgA nephropathy (IgAN) secondary to ankylosing spondylitis (AS) compared with primary IgAN.Methods Clinical and pathologic data were collected in patients who were diagnosed with IgAN by renal biopsy and admitted to our hospital from Jan 2007 to Sep 2015.Patients with IgAN secondary to AS were recruited by the ratio 1 ∶ 5 of patients with primary IgAN as control group at the same period.Results There were 15 patients in AS group,proportionately 75 patients in the control group.Compared with those in control group,patients in AS group had shorter disease course [(10.1 ± 8.3) months vs (20.2 ± 27.9) months] and lower proportions of renal insufficiency and hypertension[1/15 vs 52.0％ (39/75);1/15 vs 46.7％ (35/75)].In laboratory tests,quantitative 24 hour urinary protein and serum ereatinine were significantly lower in group AS than those in the control group [(1.42±0.67)g vs (2.88 ±1.35)g;(79.0±18.2)mmol/L vs (145.3 ±77.6) mmol/L].The Lee grading of IgAN in two groups was comparable.The treatment in both groups was similar including steroids,immunosuppression agents,angiotensin-converting enzyme inhibitors and angiotensin receptor blockers.After follow-up from 1 to 6 years,3/11 patients in AS group and 22.8％ (13/57) (13/75) in control group developed deterioration of renal function.Conclusion Patients with IgAN secondary to AS have shorter disease course and milder condition compared with patients with primary IgAN.Clinical outcome of renal function in both groups is similar according to comparable treatment.

Objective To analyze the clinical characters of psoriatic arthritis (PA) which antedates psoriasis.Method The clinical characters,laboratory tests and radiographic findings of fifteen PA patients whose arthritis antedated psoriasis were analyzed.Results The male to female ratio was 2 75∶1,the age of onset was 11 to 47 years.The course was 2 month to 30 years.HLA B27 was positive in 86 7%(13/15) of patients and sacroiliitis on X ray was noted in 10 patients.Conclusion There are more sacroiliitis in PA patients whose arthritis antedates psoriasis and they are associated with HLA B27.

Objective To discuss the value of CT prognosis on the Masaoka staging system of thymic epithelial tumors(TET) before surgical resection.Methods The CT images of 102 patients with TET proved by surgery and pathology were reviewed retrospectively.The TET were reclassified according to Masaoka stage system.The size,homogeneity,sharp,contour,infiltration of surrounding tissue,and metastasis on CT were analyzed with Logistic analysis.The diagnostic value was also evaluated with a ROC curve.Results Masaoka pathologic stages were stage Ⅰ for 36 (35.3 ％),stage Ⅱ for 27 (26.5 ％),stage Ⅲ for 30 (29.4 ％),and stage Ⅳ for 9 (8.8 ％).A multivariable Logistic regression model showed that TET with larger size of tumor (20/35,P =0.0371,OR =4.539),irregular or lobulated tumor contour (26/42,P =0.0230,OR =4.870),heterogeneous (21/33,P =0.0154,OR =6.020),infiltration of surrounding fat (25/32,P =0.0019,OR =14.005),and pleural seeding (11/11,P =0.0032,OR =36.153)were more likely to have stage Ⅲ or Ⅳ disease.The area under ROC curve was 0.940.Conclusions The tumor CT imaging features can differentiate between stage Ⅰ,Ⅱ and stage Ⅲ,Ⅳ disease.This helps identified patients more likely to benefit from neoadjuvant therapy.

Objective To investigate the long term efficacy and safety of thalidomide in the treatment of refractory ankylosing spondylitis.Methods A total of 232 patients with refractory ankylosing spondylitis were recruited into open study using thaiidomide at a dose of 150 mg/d, bath ankylosing spondylitis disease activity index ( BASDAI) , spinal pain score and thaiidomide related side effects were observed regularly.Results From the third month, BASDAI and spine pain score decreased significantly when compared with those of the base line ( P < 0.05).Such improvement became more obvious as time went on.A total of 148 patients (63.8% ) got >50% improvement in BASDAI and spine pain score, and 76 cases (32.8% ) reported absence of spine pain.The major side effects were drowsiness, constipation, dry mouth, dizziness and dandruff.Thirty two patients (13.8% ) withdrew from the study because of adverse events.Most of the adverse effects disappeared as thaiidomide was stopped.Conclusion Long term thaiidomide is effective and safe for treating resistant ankylosing spondylitis and it has cumulative effect as duration prolongs.

In the treatment of 30 cases of minor chorea by puncturing Baihui( GV 20), Sishencong ( ExHN 1 ) and Fengchi( GB 20), in combination of oral administration of herbal medicine, the total effective rate is 96.7%.

Child ; DNA Mutational Analysis ; Dystrophin ; genetics ; Exons ; Humans ; Male ; Muscular Dystrophy, Duchenne ; genetics ; Mutation

Objective To explore the sleep problem and its related factors,and to provide evidence for improving sleep quality of patients with ankylosing spondylitis (AS). Methods Pittsburgh sleeping quality index (PSQI),self-rating depression scale (SDS),self-rating anxiety scale (SAS) and a self-designed questionnaire were applied to survey 318 patients with AS.Data were analyzed by the SPSS 17.0 software.The relationship between the various relevant factors were revealed by t-test,x2 test,Wilcoxon rank-sum test and Pearson correlation analysis.Results The mean total score of PSQI was 6.6±3.6, and 35.4％ of AS outpatients showed poor sleep quality.The difference of PSQI scores and detection rate of poor sleep quality between male and female were not statistically significant.There were significant difference of early morning stiffness(Z=-3.077,P=0.002),BASFI(Z=-4.766,P=0.000),BASDAI(Z=-6.880,P=0.000),nocturnal pain VAS(VAS)(Z=-6.502,P=0.000),total back pain VAS(Z=-6.675,P=0.000),ESR(Z=-2.370,P=0.018),CRP(Z=-2.063,P=0.039) between the poor sleep and the good sleep patients (P＜0.05).The PSQI score was positively correlated with age (r=0.165,P=0.003) but not gender and disease duration (r=0.078,P=0.165; r=0.094,P=0.097).PSQI score difference were also observed between patients with negative mood and patients without negative mood (t=-7.613,P=0.000; t=-7.287,P=0.000).Conclusion High incidence of sleep disorder exists among AS outpatients,and it is closely related with age,anxiety,depression and activity of AS.Attention should be paid to AS patients with sleep problems.

Objective To improve clinicians' understanding of tuberculosis as the adverse event of the tumor necrosis factor alpha (TNF-α) antagonists therapy. Methods Two rheumatoid arthritis (RA) patients were reported to develop lymphoid tuberculosis and pulmonary tuberculoma after TNF-α antagonists therapy. The relevant literature were reviewed. Results The first ease was a 62 year-old female RA patient. Her skin PPD reaction was negative and chest X-ray was normal before the therapy. Following 4 doses of infliximab injection (each dose of 3 mg/kg) which were completed within 3 months, the patient developed fight supraclavicular lymphoids tuberculosis in the 5th month after the last treatment. The patient completely recovered after lymphoids excision and antituberculosis therapy with 4 anti-tuberculosis medications combination. The second case was a 44 year-old female RA patient. She did not take the skin PPD test and chest X-ray before the therapy. The patient developed fever and chest distress after etanercept therapy (25 mg hypodermic twice per week ) for 1.5 months. The chest X-ray showed a shadow in the median lobe of the right lung, which had gradually developed to a pulmonary tuberculoma. The patient's physical condition improved after the tubereuloma resection. It has been reported in recent years that ① TNF-α antagonists therapy could increase the incidence of tuberculosis, ② the incidence at which infliximab associated tuberculosis was higher than etanercept, ③ the majority of the patient having tuberculosis were old people, and ④ the incidence of extrapulmonary tuberculosis and the disseminated tuberculosis were higher than regular tuberculosis. Conclusion TNF-α antagonists may decrease the host defense ability against mycobacterium tuberculosis and increase the incidence of tuberculosis. The pre-treatment tuberculosis screening, as well as tuberculosis monitoring during and after treatment is mandatory.

Objective To investigate the value of imaging in the diagnosis of reflex sympathetic dystrophy(RSD).Methods The clinical manifestations and imaging findings of RSD in five patients were analyzed.Results The onset of clinical symptoms of patients was followed by an injury in all cases.Severe pain,swelling,limited range of motion and vasomotor instability were main complaints.Patchy bone demineralization could be found both on plain radiographs and CT.CT scan was superior to plain radiographs in detecting bone demineralization.Increased scintigraphic uptake in the involved extremities were demonstrated in all four patients who examined by bone scintigraphy.Bony erosions which were not easily visible on plain radiographs could be clearly seen on MRI in one patient at early stage.Conclusion Patchy bone demineralization on plain radiographs and CT is the most outstanding imaging finding in RSD.Bone scintigraphy and MRI can help early in diagnosis of the disease.