Objective To determine the vitamin D status in children with sepsis/severe sepsis in pediatric intensive care unit (PICU) in order to explor the association between vitamin D status and clinical outcomes,in turn to provide evidence for optimizing nutrition support.Methods It was a prospective,observational,multi-center study,carried out in patients with sepsis/severe sepsis from March 1,2013,to March 30,2014,in the PICUs of three tertiary-care children's hospitals.Total serum 25-hydroxy vitamin D [25 (OH) D] was measured by using an enzyme-linked immunosorbent assay at admission.The association of vitamin D status at admission with length of PICU length of stay,total hospital stay,in-hospital mortality,28-days mortality and costs were analyzed.Results A total of 194 patients includng 117 boys (60.3％)and 77 girls (39.7％) were enrolled.There were 96 patients with sepsis and 98 with severe sepsis.The mortality on discharge and 28 days were 6.7％ and 24.2％ respectively.The median vitamin D level was 9.79 ng/mL (5.32,18.46) at admission.Of them 77.8％ (151/194) had vitamin D deficiency and 50.5％ (98/194) had severe vitamin D deficiency.Patients with severe vitamin D deficiency,had higher mortality on discharge (P =0.011).Vitamin D status had no significant correlations with 28 days mortality,length of PICU stay,total hospital stay and costs.Conclusions More than three-quarters (77.8％) of children with sepsis/severe sepsis in PICUs had Vitamin D deficiency.Patients with severe vitamin D deficiency at admission had higher risk of mortality at discharge.

OBJECTIVE One of the aims of this study is to know the morbidity of LPR in children with chronic cough. And also to explore the relationship among the morbidity of LPR,the pH value in the laryngopharynx, the frequency of ref lux events,the duration of ref lux with age, sex, position, allergy and so on. METHODS A retrospective analysis was conducted on 185 children with pH monitoring in Shanghai Children's Hospital from February 1, 2015 to August 31, 2018. The pH monitoring was taken in those patients for the reasons of chronic cough, and can't be remitted by regular treatment. The sex, diagnosis, allergy and the results of 24h pH monitoring were recorded. RESULTS A total of 185 cases were reviewed. 60％(111/185) were boys and 40％(74/185) were girls. LPR was present in 36.8％ of all the children(68/185). Compared with older patients, children younger than 12m had significant higher morbidity of LPR. Higher morbidity of LPR was also found in patients with food allergy. CONCLUSION LPR has high morbidity in children. In our study, 36.8％ of the chronic cough children combined with LPR. Children younger than 12m had higher morbidity of LPR. There may be a correlation between LPR and food allergy in children.

This paper describes a design for Hospital Information Sys tem on the basis of local area networks.

Objective·To explore the efficacy and safety of compound amino acid capsules in the treatment of malnutrition and calcium and phosphorus metabolism disorders in maintenance hemodialysis patients.Methods·In this prospective,randomized,controlled,single-center study,forty maintenance hemodialysis patients from Renji Hospital,Shanghai Jiao Tong University School of Medicine were randomly divided into two groups,the treatment group(n=21)and the control group(n=19).The treatment group was given oral compound amino acid capsules on the basis of regular hemodialysis treatment,while the control group received no special nutritional intervention.Serum albumin,prealbumin,hemoglobin,ferritin,calcium,phosphorus,1,25-(OH)2-D3 and intact parathyroid hormone levels were analyzed every 3 months,and the incidence of adverse events including death,cardio-cerebrovascular accidents and vascular access failure was recorded.The total follow-up period was 9 months.Results·Serum albumin and prealbumin in the treatment group at 6-month and 9-month were significantly higher than the baseline parameters(albumin,t=3.574,5.599,both P<0.05;prealbumin,t/Z=-2.485,2.921,both P<0.05).Albumin in the control group increased at 9-month with a lower amplification compared to the treatment group(t=3.877,P=0.001),while the difference of prealbumin showed no statistical significance during follow-up.Hemoglobin and serum ferritin in the treatment group started to increase at 3-month(hemoglobin,t=2.192;ferritin,t=2.994;both P<0.05).Phosphorus in treatment group decreased at 3-month and 9-month(t/Z=-2.743,-2.103,both P<0.05),while phosphorus in the control group remained relatively stable during the first 6 months and increased at 9-month(Z=-2.178,P=0.029).Calcium and 1,25-(OH)2-D3 in the treatment group at 3-month and 6-month were significantly higher than the baseline parameters(calcium,t=4.581,4.922,both P=0.000;1,25-(OH)2-D3 t/Z=4.504,-2.374,both P<0.05),while the increase in blood calcium in the control group was significantly smaller than that in the treatment group during the same period.1,25-(OH)2-D3 in the control group showed no significant improvement.There was no significant difference in intact parathyroid hormone level,incidence of adverse events and other laboratory examination results between the two groups.Conclusion·Compound amino acid capsules can ameliorate the nutrition status and regulate calcium and phosphorus metabolism effectively and safely in maintenance hemodialysis patients.

Objective To investigate the correlation between complement C3,C4,C-reactive protein(CRP),erythrocyte sedimentation rate(ESR)and disease activity of systemic lupus erythematosus(SLE).Methods A total of 69 SLE patients admitted to the hospital from January to December 2021 were selected as the SLE group,and 70 healthy individuals were selected as the control group during the same period.The changes of serum related indicators were compared between the SLE group and the control group.According to systemic lupus erythematosus disease activity index(SLEDAI),SLE patients were divided into mild active group(27 cases)and moderate and severe active group(42 cases).The changes of related indicators were compared between the two groups.Spearman correlation analysis was used to analyze the correlation between SLEDAI score and serum related indicators.Receiver operating characteristic(ROC)curve was used to ana-lyze the validity of diagnosis of disease severity.Results Compared with the control group,the levels of serum complement C3 and C4 in the SLE group were decreased,and the levels of CRP and ESR were increased,the differences were statistically significant(P＜0.05).Compared with the mild group,the levels of serum com-plement C3 and C4 in the moderate and severe group were decreased,and the levels of CRP and ESR were in-creased,and the differences were statistically significant(P＜0.05).The SLEDAI score of SLE patients was positively correlated with serum CRP and ESR levels(P＜0.05),and negatively correlated with serum com-plement C3 and C4 levels(P＜0.05).The area under the curve(AUC)of CRP,ESR,complement C3 and C4 were 0.716,0.875,0.872 and 0.856,respectively,the sensitivity were 59.52％,85.71％,78.57％and 78.79％,respectively,and the specificity were 79.83％,81.67％,86.79％and 86.54％,respectively.Conclu-sion Serum complement C3,C4,CRP and ESR are correlated with the progression of SLE,and play auxiliary roles in the disease activity stage of SLE.

Paget's disease of bone (PDB) is a common metabolic bone disease that is characterized by aberrant focal bone remodeling, which is caused by excessive osteoclastic bone resorption followed by disorganized osteoblastic bone formation. Genetic factors are a critical determinant of PDB pathogenesis, and several susceptibility genes and loci have been reported, including SQSTM1, TNFSF11A, TNFRSF11B, VCP, OPTN, CSF1 and DCSTAMP. Herein, we report a case of Chinese familial PDB without mutations in known genes and identify a novel c.163G>C (p.Val55Leu) mutation in FKBP5 (encodes FK506-binding protein 51, FKBP51) associated with PDB using whole-exome sequencing. Mutant FKBP51 enhanced the Akt phosphorylation and kinase activity in cells. A study of osteoclast function using FKBP51V55L KI transgenic mice proved that osteoclast precursors from FKBP51V55L mice were hyperresponsive to RANKL, and osteoclasts derived from FKBP51V55L mice displayed more intensive bone resorbing activity than did FKBP51WT controls. The osteoclast-specific molecules tartrate-resistant acid phosphatase, osteoclast-associated receptor and transcription factor NFATC1 were increased in bone marrow-derived monocyte/macrophage cells (BMMs) from FKBP51V55L mice during osteoclast differentiation. However, c-fos expression showed no significant difference in the wild-type and mutant groups. Akt phosphorylation in FKBP51V55L BMMs was elevated in response to RANKL. In contrast, IκB degradation, ERK phosphorylation and LC3II expression showed no difference in wild-type and mutant BMMs. Micro-CT analysis revealed an intensive trabecular bone resorption pattern in FKBP51V55L mice, and suspicious osteolytic bone lesions were noted in three-dimensional reconstruction of distal femurs from mutant mice. These results demonstrate that the mutant FKBP51V55L promotes osteoclastogenesis and function, which could subsequently participate in PDB development.
Acid Phosphatase ; Animals ; Asian Continental Ancestry Group ; Bone Diseases, Metabolic ; Bone Remodeling ; Bone Resorption ; Femur ; Humans ; Mice ; Mice, Transgenic ; Osteitis Deformans* ; Osteoblasts ; Osteoclasts ; Osteogenesis ; Phosphorylation ; Phosphotransferases ; Tacrolimus Binding Proteins ; Transcription Factors