BACKGROUND:The influence of Tol-like receptor 2 (TLR2), Tol-like receptor 4 (TLR4), Tol-like receptor 6 (TLR6) signal transduction pathway and active Treg in children with Henoch-Schonlein purpura has been unknown.
OBJECTIVE:To investigate the expression of TLR2, TLR4 and TLR6 in peripheral blood mononuclear cells and Treg immune response in patients with Henoch-Schonlein purpura, and to explore the role of TLR2, TLR4, TLR6 and Treg activation in the pathogenesis of Henoch-Schonlein purpura.
METHODForty-two hospitalized children with Henoch-Schonlein purpura were enrol ed in this study. Another 15 healthy children were selected as controls. TLR2, TLR4 and TLR6 at protein level in peripheral blood mononuclear cells were detected by flow cytometey;reverse-transcription PCR and real-time PCR were used to evaluate the level of MyD88;the levels of transforming growth factor-βand interleukin-10 were measured by enzyme-linked immunosorbent assay. t-test or t’-test was used to compare the levels of these genes and proteins. Pearson’s correlation test was done for correlation analysis.
RESULTS AND CONCLUSION:Compared with the control group, the protein levels of TLR2, TLR4, TLR6 and the relative expression level of MyD88 mRNA were significantly up-regulated (P<0.01). The serum levels of transforming growth factor-βand interleukin-10 were higher in the Henoch-Schonlein purpura children than the healthy children (P<0.05). There was a significant correlation between the protein levels of TLR2, TLR4, TLR6 and mRNA level of MyD88 (P<0.01), but no relationship was found between TLRs and interleukin-10, transforming growth factor-β(P>0.05). The excessive activation of TLR2, TLR4, TLR6 may be involved in the process of Henoch-Schonlein purpura via MyD88-dependent pathway, and the compensatory activation of Treg may participate in protective immunity.

The body mass index (BMI)is one of the important indexes to evaluate the nutrition / health status.BMI is weight in kilograms divided by height in per square meter (unit of kg/m2).BMI is changing with age.However,there are no BMI guidelines for the elderly.It is really necessary to set up a proper BMI range for the elderly.This research analyzed the relationship of BMI with all-cause mortality,cardiovascular mortality and body composition change in the elderly.The result showed that an appropriate higher BMI could help to decrease the all-cause mortality and cardiovascular mortality in the elderly and could also increase bone density and lean body mass,which could prevent the geriatric fracture and sarcopenia.Furthermore,underweight-related mortality was much higher than overweight-related mortality.

Objective: To learn anxiety and depression status in junior high school students with myopia in Zhoushan and their influencing factors,so as to provide reference for vision care and mental health intervention. Methods: A stratified cluster random sampling method was used to select students from two junior high school in Zhoushan. A self-designed questionnaire,self-rating anxiety scale (SAS) and self-rating depression scale (SDS) were employed to collect the demographic information,and to evaluate anxiety and depression status.The logistic regression analysis was used to select the influencing factors for anxiety and depression in myopia students. Results : A total of 775 questionnaires were sent out and 766 valid ones were recovered,with an response rate of 98.84%. There were 662 students with myopia.The rates of anxiety,depression and the two combined in myopic students were 22.36%,25.38% and 14.35%,respectively.The results of the multivariate logistic regression analysis showed that the risk factors for anxiety of myopic students were females (OR=1.514,95%CI:1.054-2.183),the third grade (OR=2.246,95%CI:1.408-3.619),high myopia (OR=1.839,95%CI:1.126-3.017) and wearing glasses for two years or above (OR=2.504,95%CI:1.464-4.306); the risk factors for depression were females (OR=1.182,95%CI:1.253-2.536),above average of school record (OR:1.614-2.026,95%CI:1.034-3.153),high myopia (OR=2.284,95%CI:1.513-3.436) and wearing glasses for two years or above (OR=4.602,95%CI:2.035-10.372). Conclusions The prevalence rates of anxiety and depression are higher in junior high school students with myopia in Zhoushan. The students who are females,are in the third grade,have above average of school record,have high myopia and wearing glasses for two years or above are more likely to suffer from anxiety or depression.

Objective:To investigate the predictive value of mucosal vascular pattern (MVP) under narrow-band imaging (NBI) enteroscopy in patients with ulcerative colitis (UC) in clinical remission for histological healing and clinical recurrence.Methods:A total of 142 patients with UC in clinical remission who visited the First Affiliated Hospital of Weifang Medical University from January 2018 to January 2021 were included in the study and underwent colonoscopy. The white light and NBI endoscopic images were collected and biopsies were obtained. The Mayo endoscopic score (MES) was calculated based on white light images, and MVP staging was evaluated based on mucosal vascular patterns under NBI. Nancy index (NI) was used to evaluate histological healing and patients were followed up for 1 year. The Spearman correlation coefficients of MES and MVP with histological healing and recurrence were calculated. The receiver operator characteristic (ROC) curve was plotted and the area under curve (AUC) was applied to evaluate the accuracy of white light and NBI endoscopy for predicting histological healing of UC in clinical remission.Results:According to the MVP criteria, 47 were defined as clear, 63 blurred, and 32 invisible. Spearman correlation analysis showed a significant correlation between MVP under NBI and histological healing ( r=0.549, P<0.001) and a moderate correlation between MES under white light and histological healing ( r=0.462, P<0.001). Spearman correlation analysis showed a moderate correlation between MVP under NBI and clinical recurrence ( r=0.451, P<0.001) and a moderate correlation between MES under white light and clinical recurrence ( r=0.352, P<0.001). AUC of NBI for diagnosing histological healing of UC in clinical remission was 0.809 (95% CI: 0.738-0.879), with a sensitivity of 84.6% (77/91) and specificity of 64.7% (33/51), superior to the white light endoscopy, of which AUC, sensitivity and specificity were 0.763 (95% CI: 0.678-0.848), 81.3% (74/91) and 66.7% (34/51). Conclusion:MVP staging under NBI could predict histological healing of UC patients in clinical remission and is superior to white light endoscopy.

OBJECTIVE: To explore the clinical characteristics and genetic etiology for a child with atypical Hemolytic uremic syndrome (aHUS) in conjunct with nephrotic level proteinuria. METHODS: A child patient who had visited the Affiliated Hospital of Qingdao University on June 25, 2020 was selected as the study subject. Clinical data of the patient was collected. Whole exome sequencing (WES) was carried out for the child, and candidate variant was verified by Sanger sequencing of the child and his parents. RESULTS: The child, an 8-month-old male, had presented mainly with edema, oliguria, hematuria, nephrotic level proteinuria, anemia, thrombocytopenia, increased creatinine and urea, hypercholesterolemia but normal complement levels. Genetic testing revealed that he has harbored compound heterozygous variants of the DGKE gene, namely c.12_18dupGAGGCGG (p.P7fs*37) and c.1042G>T (p.D348Y), which were respectively inherited from his father and mother. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variants were classified as likely pathogenic and variant of uncertain significance, respectively. By combining his clinical manifestations and results of genetic testing, the child was diagnosed with aHUS with nephrotic level proteinuria. CONCLUSION For infants and young children with aHUS in conjunct with nephrotic level proteinuria, variants of the DGKE gene should be screened. Above finding has expanded the mutational spectrum of the DGKE gene.
Infant ; Female ; Humans ; Child ; Male ; Child, Preschool ; Atypical Hemolytic Uremic Syndrome/diagnosis* ; Mutation ; Genetic Testing ; Thrombocytopenia/genetics* ; Proteinuria/genetics*

Objective:To summarize the clinical phenotype and genotype features of 2 children with adenosine deaminase 2 (ADA2) deficiency, and to review the related literature so as to enhance the understanding of this disease.Methods:The phenotype and genotype of 2 cases with ADA2 deficiency who visited the Affiliated Hospital of Qingdao University from March to December 2019 were analyzed.Literature was searched from foreign and domestic databases and studied to summarize clinical and gene mutation characteristics of children with ADA2 deficiency.Results:(1) ADA2 gene mutation was found in both children.One case was characterized by recurrent fever, livedo reticularis, polyarteritis nodosa and immunodeficiency.The mutation site c. 571delC(p.Q191Sfs*5)of the ADA2 gene detected in this case was a homozygous mutation, which was a new mutation point and not reported in China or abroad previously.The other case was characterized by recurrent fever, panniculitis, vasculitis with legs, and immunodeficiency.The mutation site c. 1358A>G(p.Y453C)was a homozygous mutation that was not reported in China previously.(2)There were 171 cases of children diagnosed with ADA2 deficiency in foreign countries, but only 5 cases (3 previously reported cases and 2 cases in this study) were detected in China.The main clinical phenotypes were recurrent fever(5/5 cases), livedo reticularis(4/5 cases), panniculitis(1/5 cases), cutaneous gangrene(1/5 cases), growth retardation(1/5 cases), cerebral infarction(3/5 cases), humoral immunodeficiency(4/5 cases), blood system involvement(3/5 cases), and myalgia(2/5 cases), elevated inflammatory markers(C-reactive protein, erythrocyte sedimentation rate)(5/5 cases). Conclusions:Children with ADA2 deficiency have various clinical phenotypes, and a good understanding of phenotypes can improve the level of clinical diagnosis and treatment.The mutation point of c. 571delC is a novel ADA2 gene mutation type, which further enriches the ADA2 gene spectrum.

ObjectiveTo explore the occupational needs of hand injury patients and the effects of activity-based analysis and occupation-based interventions. MethodsFrom January, 2021 to June, 2022, 212 patients who accepted occupational therapy for wrist-hand injury in Rehabilitation Center of the First Affiliated Hospital of Nanjing Medical University were interviewed using Canadian Occupational Performance Measure (COPM) to collect the activities that they wanted to do, needed to do, were expected to do, but unable to do. Two senior occupational therapists conducted the activity analysis of the summarized needs, identified occupational components, designed a purposeful occupation-based intervention program, and evaluated the performance and satisfaction using COPM. ResultsA cumulative total of 84 occupational needs were proposed, covering five major domains: work, basic activities of daily living, instrumental activities of daily living, leisure and sleep. The activities mostly impaired involved using computer (8.28%), using chopsticks (6.42%), wringing towels (6.25%), travelling on transport (4.90%) and lifting heavy objects (4.73%). The performance and satisfaction improved after treatment (t > 16.572, P < 0.001). ConclusionThe occupational needs are culturally specific for local hand injury patients; the use of an activity-based analysis and occupation-based intervention is effective on meeting the occupational needs.