Objective To discusses the effectiveness of tocilizumab in the treatment of hypomyopathic dermatomysositis (HDM) combined with interstitial lung disease (ILD) in children. Methods The clinical characteristic, treatment, and prognosis of HDM combined with ILD were analyzed in 2 patients. The related literatures were reviewed. Results Both ten-year-old girl and 8-year-old boy had shortness of breath after activities, but had no clinical manifestations of muscle damage; both of them had typical rash, but had nornal muscle strength and muscular tension. Laboratory tests showed the elevation of serum ferritin, lactate dehydrogenase, glutamate aminotransferase, and aspartate aminotransferase. Creatine kinase slightly increased in the initial test, and then was in the normal range in the following tests. The high resolution computed tomography showed that pulmonary interstitial lesions. HDM combined ILD was diagnosed clinically. The girl died after treatment with high-dose hormones, cyclophosphamide, cyclosporine, pirfenidone, and gamma globulin failed. The boy was stabled after conventional hormone treatment plus tocilizumab (240 mg twice). His laboratory indicators were in the normal range in the follow-up. Conclusions The clinical manifestations and laboratory indicators aren't typical in childhood HDM. The mortality is high. Combined with tocilizumab treatment is effective in one case.

0.05). After treatment, the amount of urinary protein and anti-dsDNA antibodies level in leflunomide group were significantly lower than those in dexamethasone and saline groups (P

Objective To assess the effect of low-dose cytarabine and aclarubicin in combination with gran-ulocyte colony-stimulating factor (G-CSF) protocol (CAG) in patients with acute myeloid leukemia (AML),and to understand the potential factors affecting the outcome of CAG induction therapy, therefore to find the optimum pa-tients for CAG therapy. Methods Twenty-one AML patients were enrolled in the current study. All patients were treated with CAG regimen including cytarabine (10 mg/m~2, subcutaneously, every 12 h, days 1 - 14), lacinomycin (5～7 mg/m~2,intravenously,every day, days 1 -8) ,and G-CSF (200 μg/m~2,subcutaneously, every day,12 h be-fore Ara-C was given) priming. Results The overall complete remission (CR) rate of the 21 AML patients was 66.7% (14/21). The CR rates was 87.5% (7/8) in patients older than 60 yrs,60.0% (9/15) in the refractory or relapsed patients,83.3% (5/6) in the MDS transformed AML patients. The CR rates for patients with hyperprolif-erative BM and median to poor proliferative BM were 33.3% and 91.7% ,respectively(P =0.009). The median o-verall survival (OS) time of the 21 AML patients was 450 days. Two-year survival rate estimated by Kaplan-Meier Method was 30.6%. The overall median disease free survival (DFS) was 165 days. The median OS time for those refractory or relapsed was 435 days. The median OS time for those with poor cytogenetic state or standard or good cytogenetic state was 140 days and 620 days, respectively (P = 0.001). The median OS time for patients with hyperproliferative BM and median to poor proliferative BM was 321 days and 620 days, respectively (P = 0.05). The median recovery time of granulocytes above 1.0×10~9/L was 8 days. The median duration of fever was 3.5 days. The rate of infections exceeding WHO grade Ⅱ was 42.9%. No early death occurred. Conclusions The CAG induction therapy may have a higher CR rate in patients with refractory or relapsed AML, elderly AML and secondary AML from MDS transformation, and extend the median overall survival time in refractory or relapsed patients. CAG therapy can not improve the outcome of patients whose BM was in high grade proliferation state or whose cytogenetic state was poor. CAG therapy can shorten the duration of agranulocytosis and decrease the inci-dence of serious infection. Therefore, CAG therapy is worth recommending to patients who can not endure the rou-tine intensive chemotherapy.

Objectives To investigate the etiology, renal pathology, treatment, and prognosis of children's urinary system injury after hematopoietic stem cell transplantation (HSCT). Methods Clinical data of 81 children with urinary dysfunction after HSCT admitted to the Hematology Department in Children's Hospital of Soochow University were analyzed, and relevant literatures were reviewed. Results In 81 cases (50 males and 31 females), the age ranges from 8 months to 17 years old. Thirty cases (37%) with prerenal injury were recovered after active rehydration and other symptom specific treatment. There were 9 (11.1%) children with renal injury, four cases were given up therapy or transferred to other hospitals, thus lead to an unknown prognosis. Kidney biopsy was performed in the remaining five cases for pathological investigation. After active symptom-speific and etiology-based treatment, serum creatinine and glomerular filtration rate of four cases return to normal. But in the long-term follow-up,one case died of recurrence of primary disease, reinfusion of hematopoietic stem cell combined with renal failure. The remaining 3 patients were with chronic kidney disease (CKD). One case with renal thrombotic microangiopathy was in the chronic dialysis. Postrenal renal injuries were mainly hemorrhagic cystitis (28.4%) and urinary tract infection (16%). After a large dose of rehydration, urine alkalization and anti-infection therapy, they were recovered in the short term with a good prognosis. Conclusions Urinary injury after HSCT is mainly divided into three categories: prerenal, renal and postrenal, in which renal injury is prone to frequent recurrence.

Transposition of simple or composite forearm island flaps with a radial vascular pedicle was performed in repairing tissue defects of the hand and reconstruction of the thumb in 16 patients. It was found to be easy to operate and no suturing of blood vessels was necessary. The postoperative swelling was less marked. The flap was good in texture and sensation after the operation. The overall result wassatisfactory. Anatomic-physiological situdy was also carried out. 16 patients have been operated on, including 2 thumb reconstructions, with satisfactoryresults.

Objective To explore nurses′attitudes to patient engagement in patient safety and analyze the influencing factors. Methods With convenient sampling, a total of 580 nurses of 12 hospitals in Shanghai received a questionnaire survey for nurses′attitude to patient participation and patient safety. The higher score after statistical analysis explains the better attitude.Results Average score of nurses′ attitudes to patient engagement in patient safety was (4.039 2±0.518 72), while average score of decision-making participation was (4.000 60± 0.568 75), and caring-related involvement was (94.192 2±0.573 74) and complaint-related involvement was (3.437 4±0.798 40). There is significant difference in attitude of nurses in different levels of hospitals and different educational background to “patient participating in patient safety” ( F=8. 832, 3. 935;P<0. 05 ) . Conclusions Nurses′ attitude to patient engagement in patient safety is quite positive. Caring-related involvement is more welcomed than decision-making participation and complaint-related involvement among Shanghai nurses. Hospital level and educational background are the key influencing factors.

Objective To understand the situation on nurses′ perceptions of patient participation in patient safety and its influencing factors. Methods A total of 551 in-service nurses were recruited by convenience sampling. They were investigated with the scale of medical staff′ perception of patient participation in patient safety.Results The mean of nurses′ perception of patient participation in patient safety was (3.68± 0. 57)with a medium level. The score of decision participation (3.76±0.64) was significantly higher than that of caring participation (3.71±0.63), and the score of caring participation was higher than that of complaining participation (3.33±0.85). There were statistically significant differences in the scores of nurses′perceptions of patient participation in patient safety among different ages, years of working, professional titles, departments and educational background (P<0.05).Conclusions The situation of nurses′ perceptions of patient participation in patient safety is not satisfactory. Nurses should take patient participation in patient safety into account, guide and encourage patients to participate in patient safety.

Objective To evaluate the performance of hook effect of five immunoturbidimetric kits for the detection of specific proteins on biochemical analyzers.Methods Five immunoturbidimetric kits with higher market share that came from Beijing BSBE (A), Sichuan maccura (B), Shenzhen Mindray (C), Ningbo Medical System (D) and Beijing Leadman (E) were used to determine six specific proteins.A series of concentration gradient samples were prepared and tested to compare the performance of hook effect from different manufactures′kits when the analytical measurement ranges were known.Results In the five kits, the upper limits of the safe range of antigen excess about ASO, hs-CRP andβ2-MG were relatively higher in B and C.No hook effect occurred at the approximate concentration of 10 000IU/mL, 1 000mg/L and 226mg/L respectively.The highest upper limits for CysC were C and E kits, and both were greater than 112mg/L.The upper limits of the safety range for other manufacturers were more than 700mg/L about RBP except for D.The maximum upper limit of mALB was D.Hook effect did not appear at the concentration of 43 560mg/L approximately.Conclusion Different manufactures′immunoturbidimetric kits have different hook effect performance.The laboratories should verify the hook effect performance before using the kits, and select the most suitable kit to prevent hook effect.

Objectives To detect the level of soluble programmed death 1 (sPD-1) and soluble programmed death ligand 1 (sPD-L1) in serum and urine of children with primary nephrotic syndrome (PNS),and explore its clinical significance.Methods From July 2017 to November 2017,children with PNS admitted to the Children's Hospital Affiliated to Soochow University were divided into onset group (36 cases) and remission group (33 cases).Thirty healthy children who underwent medical examination for enrollment,undersize or overweight in the outpatient department of pediatric health care and inpatient department of Endocrinology were selected as healthy control group.Serum and urine samples were collected,in which the levels of sPD-1 and sPD-L1 were detected by enzyme-linked immunosorbent assay (ELISA).The correlation between serum and urine sPD-1,sPD-L1 levels and lymphocyte subsets,urinary protein were analyzed by Pearson and Spearman correlation analysis.Results The level of sPD-1 in serum was lower in remission group than those in healthy controlgroup [1.60(0.48,8.15) ng/ml vs 7.38(2.15,19.02) ng/ml,P ＜ 0.01].The level of urinary sPD-1 in onset group was higher than that in remission group [1.21(0.61,2.56) pg/μg vs 0.51(0.31,0.97) pg/μg,P ＜0.001] and healthy control group [1.21(0.61,2.56) pg/μg vs 0.82(0.34,1.15) pg/μg,P ＜ 0.01].The levels of sPD-L1 in serum and urine were higher in onset and remission group than those in healthy control group (P ＜ 0.001).The level of sPD-1 in the serum was positive correlated with the numbers of CD3+,CD3+CD4+,CD3+ CD8+ T lymphocytes and CD3-CD19+,CD19+CD23+ B lymphocytes (r=0.537,0.478,0.454,0.429 and 0.374;P=0.002,0.008,0.012,0.018 and 0.042).The level of sPD-1 in the urine had positive relation with the ratio of 24 hours urinary albumin and weight (24 h UmAlb/Wt),N-acetylglucosaminidase and urinary creatinine (UNAG/Cr) and β2 microglobulin and urinary creatinine (Uβ2MG/Cr) (r=0.409,0.588 and 0.276;P=0.016,0.000 and 0.032).Conclusions The dynamic changes of sPD-1 and sPD-L1 in serum and urine suggested that PD-1/PD-L1 signaling pathway is involved in the development process of childhood primary nephrotic syndrome.