Objective To investigate the diagnostic value of serum ferritin in children with systemic onset juvenile idiopathic arthritis (SO-JIA). Methods Fifty-seven patients with fever of unknown origin (rectal temperature>38.5 ℃ ) over two weeks and hospitalized in our general medicine ward longer than one week were enrolled in this study. Patients were recorded the course of fever, elinieal symptoms and signs including rash and swollen joints/arthralgia, laboratory tests including complete blood cell count, C-reactive protein, erythroeyte sedimentation rate, lactate dehydrogenase and the level of serum ferritin. SPSS 10.0 was used for statistical analysis. Results Two of 57 patients could not be diagnosed before discharge. The other 55 patients whose diagnosis was confirmed were divided into four groups. Twenty-five patients were SO-JIA,12 patients had hematologic or oncological diseases, 12 patients had infectious diseases and 6 patients had other rheumatic diseases. The level of serum ferritin was significantly higher (P<0.01) in SO-JIA group than in other groups. Moreover, the levels of serum ferritin in SO-JIA group were all higher than normal and the levels of serum ferritin in 76% of SO-JIA group were more than five-fold elevation. Four cut-off levels of serum ferritin level for the diagnosis of SO-JIA were selected based on clinical practice and ROC curve. When the cut-off levels of serum ferritin were 328.25, 529.50, 731.05 ng/ml and 1121.10 ng/ml, the sensitivity for the diagnosis were 100%, 88%, 72%, and 64% respectively, the specificity were 77%, 87%, 90% and 100%,respectively. Conclusion Serum ferritin is valuable for the diagnosis of SO-JIA and 529.50 ng/ml may be a good cut-off level

Objective To explore the association of galactose-deifcient IgA1 levels with clinical features, and further to provide guidance for individualized treatment of HSP. Methods According to the clinical symptoms and curative effect, 57 children with HSP were divided into four groups:non-HSPN group (n=26), HSPN group (n=7), refractory HSP group (n=7) and remission group (n=17). In non-HSPN group, 12 cases received glucorticoid therapy and 14 cases did not. Serum galactose-de-ifcient IgA1 (Gd-IgA1) concentrations were detected using a Helix aspersa-lectin-based enzyme-linked immunosorbent assay (ELISA), and the total IgA1 levels were measured by ELISA. Results The serum Gd-IgA1 level was signiifcantly higher in 40 HSP children who were not cured than that in remission group and control group (P<0.05). However, there was no difference in Gd-IgA1 level between remission group and control group (P>0.05). Compared with the control group, the serum Gd-IgA1 level was signiifcantly higher in HSPN group, non-HSPN group and refractory HSP, and children with refractory HSP had signiifcantly higher Gd-IgA1 level than children in non-HSPN group (P<0.05). No signiifcant difference in Gd-IgA1 level was found either between HSPN group and refractory HSP group or between HSPN group and non-HSPN group (P>0.05). Furthermore, in non-HSPN group, the serum Gd-IgA1 level in HSP children who were not treated with glucorticoid was signiifcantly higher than that in HSP children treated with glucorticoid (P<0.05). Conclusions The serum Gd-IgA1 level is associated with the disease activ-ity and curative effect of HSP, especially in children with refractory HSP, and it is thus likely to be a new non-invasive disease activity marker for guiding the proper usage of glucocorticoid and immunosuppressants in HSP children.

Objective To compare the blood lipid levels of different brachial-ankle pulse wave velocity (baPWV) in young and middle-aged people with normal blood pressure and to explore the related factors affecting baPWV.Methods From January 2014 to December 2017,the clinical data of one thousand two hundred and sixty-eight middle-aged and young people with normal blood pressure who underwent physical examination in Dongying People's Hospital were retrospectively analyzed.Using baPWV＜ 1 400 cm/s as the standard of normal arterial stiffness,the patients were divided into normal arterial stiffness group (normal group,1 128 cases),abnormal arterial stiffness group (abnormal group,baPWV ≥ 1 400 cm/s,140 cases).The blood lipid indexes of the two groups were analyzed and compared.Logistic regression analysis was used for multivariate analysis and linear correlation analysis was used for linear correlation analysis.Pearson correlation analysis was used.Results Compared with the normal group,TC ((4.99 ± 1.10) mmol/L vs.(4.48 ± 1.03) mmoL/L,t =5.830),TG ((1.62 ± 0.27) mmol/L vs.(1.49 ± 0.23) mmol/L,t=5.102),LDL-C[(3.25±0.23) mmol/L vs.(3.11±0.16) mmol/L,t =4.712),Apo B((0.96 ±0.07) g/L vs.(0.87±0.08) g/L,t =4.297)in abnormal group all increased,and HDL-C((1.15±0.09) mmol/L vs.(1.27±0.07) mmol/L,t =4.712) decreased,and the differences were statistically significant (P＜0.05).Smoking,high FPG,high LDL-C,high Apo B,low HDL-C were the independent factors affecting baPWV abnormality (P＜ 0.05).TC,TG,LDL-C,Apo B and baPWV in abnormal group were positively correlated(P＜0.05),and HDL-C and baPWV were negatively correlated(P＜0.05).There was a linear regression relationship between LDL-C,Apo B and baPWV (P＜0.05).Conclusion The increase of LDL-C and Apo B are closely related to early arterial disease in the low-risk populations of normotensive young and middle-aged people,even the risk of blood lipid may already exist within the normal range.

Objective:To analyze the clinical characteristics of children diagnosed with systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)for early recognition.Methods:We retrospectively reviewed the clinical records of 14 SLE patients with TMA hospitalized at Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine from December 2005 to October 2020.Results:The incidence of TMA was 5.65%(14/248)of the hospitalized patients with SLE and 7.87%(14/178)of the hospitalized patients with lupus nephritis.Four patients were boys while ten patients were girls.One boy was six years old and other 13 patients were from 11 to 18 years old.Their SLEDAI scores ranged from 14 to 31, and all of them were severe activity.Renal biopsy of 11 patients during TMA course all revealed lupus nephritis(type Ⅳ, n=8; type Ⅳ+ Ⅴ, n=3). These 14 SLE children were diagnosed with TMA within 3 days to 2 months after admission.At the beginning of the hospitalization, only six patients had both anemia and thrombocytopenia, while eight patients only had moderate anemia.All of the patients had obvious hypocomplementemia.Especially in the patients with first onset of SLE without treatment, their serum levels of C3 were less than 0.17 g/L and C4 were less than 0.07 g/L.Moreover, glomerular filtration rates of these patients were lower than that in normal range.The follow-up time were 0.2-11.3 years(median time was 2.6 years). After treatment, six patients obtained complete remission, and five patients obtained partial remission.One patient had sudden death during the 4th plasmapheresis, and the other two patients deteriorated. Conclusion:Children with SLE and TMA are mostly in severe disease activity, and renal pathology is type Ⅳ lupus nephritis.The SLE children with anemia should be paid special attention to the level of serum complement whether they have thrombocytopenia or not.If the level of serum complements decrease obviously, glomerular filtration rates should be monitored closely and schistocytes should be searched repeatedly in the blood smears of the peripheral blood to facilitate the early recognition of TMA.

Objective To study the intervention effect of systematic nutrition combined with rhythmic exercise on patients with abnormal liver metabolism. Methods According to the theory of system nutrition and health rhythm kinematics, selected 56 subjects with abnormal liver metabolism were selected, and the combined intervention of system nutrition and rhythm movement was conducted regularly every day for 3 consecutive months using the techniques such as liver transient elastography (FibroScan) and bioelectric whole body health scanning system (DDFAO). Results Compared with the pre-intervention period, the liver fat attenuation, liver hardness and liver functional activity of the subjects were significantly improved after intervention. Conclusion The systematic nutrition combined with rhythmic exercise significantly reduced the risk of abnormal liver metabolism in subjects, which may play an important role in preventing liver diseases and promoting the recovery of liver function.

Hematologic Neoplasms ; Hepatitis E virus ; Humans

  Objective  To study the demographic and clinical characteristics, correlation of genotype and phenotype and treatment of Blau syndrome to facilitate early diagnosis and timely treatment of Blau syndrome.  Methods  Seventy-two patients with Blau syndrome from 11 centers from May 2006 to April 2022 were retrospectively analyzed, and their general information, clinical data, laboratory examination and treatment medication were collected.  Results  The distribution of patients with Blau syndrome was uniform in geographical north and south of China, and there was no obvious gender bias. The mean age of onset was (14.30±12.81) months, and the age of diagnosis was (55.18±36.22) months. 35% of patients with Blau syndrome happened before 1 year old, and all patients developed before 5 years old. 87.50% (63/72) had granulomatous arthritis, 65.28% (47/72) had rash, 36.11% (26/72) had ocular involvement, 27.78% (20/72) had fever, and 15.28% (11/72) had pulmonary involvement. Arthritic manifestations of Blau syndrome were most at risk, followed by rash, ocular involvement, and fever.The first 25 months of the disease, the risk of developing a rash was the greatest. The risk of developing arthritis was the greatest between 25 months and 84 months. The main mutations were p.R334Q and p.R334W, and patients with p.R334Q mutation had relatively high incidence of fever (35.71%[5/14] vs. 14.29%[1/7], P=0.43) and ocular involvement (42.86%[6/14]vs. 28.57%[2/7], P=0.51). There was a relatively high incidence of rash (85.71%[6/7] vs. 64.29%[9/14], P=0.59) in patients with the p.R334W mutation. Forty-five patients(62.50%)were treated with a combina-tion of glucocorticoid and methotrexate. Twenty-two patients were treated with tumor necrosis factor antagonist in addition to glucocorticoid and methotrexate.  Conclusions  The risk of different clinical manifestations of Blau syndrome from high to low was arthritis, followed by rash, ocular involvement and fever. The main treatment was glucocorticoid combined with methotrexate, to which biological agents could be added.