BACKGROUND:Shape memory polyurethane has good physical and chemical properties and compatibility, but there are relatively few reports on the compatibility of osteoblasts before and after the deformation-complex of the shape memory polyurethane. OBJECTIVE:To observe the compatibility of osteoblasts with shape memory polyurethane before and after the deformation-complex. METHODS: Shape memory polyurethane membranes were prepared, and its stretching-solid-complex was conducted under the experimental environment, to obtain the membrane materials after the deformation-complex. The Sprague-Dawley neonatal rat osteoblasts were inoculated on the shape memory polyurethane membranes before and after the deformation-complex. After 2 hours of culture, the number of adherent cels was counted, and cel spreading was observed; cel proliferation was determined after 1-11 days of culture. RESULTS AND CONCLUSION:The adhesion amount and proliferation activity of osteoblasts on shape memory polyurethane membranes after the deformation-complex were significantly higher than those before the deformation-complex (P < 0.05). The osteoblasts presented fusiform appearance on the shape memory polyurethane membranes after deformation-complex, and cel arrangement showed a clear orientation, but a smal spreading area; while the osteoblasts presented polygonal shape on the shape memory polyurethane membranes before deformation-complex, arranged in no particular direction, and spread largely. These findings show the shape memory polyurethane has better osteoblast compatibility after the deformation-complex.

A novel avian influenza A (H7N9) virus recently emerged in the Yangtze River delta and caused diseases, often severe, in over 130 people. This H7N9 virus appeared to infect humans with greater ease than previous avian inlfuenza virus subtypes such as H5N1 and H9N2. While there are other potential explanations for this large number of human infections with an avian influenza virus, we investigated whether a lack of conserved T-cell epitopes between endemic H1N1 and H3N2 inlfuenza viruses and the novel H7N9 virus contributes to this observation. Here we demonstrate that a number of T cell epitopes are conserved between endemic H1N1 and H3N2 viruses and H7N9 virus. Most of these conserved epitopes are from viral internal proteins. The extent of conservation between endemic human seasonal inlfuenza and avian inlfuenza H7N9 was comparable to that with the highly pathogenic avian inlfuenza H5N1. Thus, the ease of inter-species transmission of H7N9 viruses (compared with avian H5N1 viruses) cannot be attributed to the lack of conservation of such T cell epitopes. On the contrary, our ifndings predict signiifcant T-cell based cross-reactions in the human population to the novel H7N9 virus. Our findings also have implications for H7N9 virus vaccine design.

Objective: To compare and quantify the differences in size-specific dose estimates (SSDE) obtained by effective diameter and water-equivalent diameter from the central slice of the scan range in head CT examination. Methods: A total of 111 consecutive adult patients who underwent head CT examination were enrolled in this study. All of CTDI_vol values in the dose report were documented. The dataset was assigned into group A and group B, based on the individual size-dependent conversion factors (f) of effective diameter (d_eff) and water-equivalent diameter (d_w) at the central slice multiplied by normalized volume computed tomography dose index (CTDI_vol ) respectively. Body size, f and SSDE were calculated. With SSDE_gross served as the reference level, the performance of SSDE_deff and SSDE_dw was evaluated. Results: Statistically significant differences were found in body size (t=47.587, P<0.05) and f(z=-9.242, P<0.05) between group A and group B. Statistically significant difference also existed in SSDE (t=-46.687, P<0.05), (56.20±2.66) and (53.49±2.48) mGy for group A and group B respectively. Strongly positive correlation was shown in body size (r=0.873, R² =0.761) and SSDE (r=0.974, R² =0.949) between group A and group B(all P<0.05). Positive correlation was also found between SSDE_deff and SSDE_gross(r=0.900, R² =0.809), SSDE_dw and SSDE_gross (r=0.904, R² =0.817, all P<0.05). Mean absolute difference was 2.34 and 0.78 mGy, for SSDE_deff vs. SSDE_gross and SSDE_dw vs. SSDE_gross respectively; mean absolute relative difference was 4.38%, 1.40%; root mean square difference was 1.17 mGy (2.17%), 1.06 mGy (1.91%). Interquartile range and full range of SSDE_deff and SSDE_dw were 3.22 vs. 2.39 mGy, 13.65 vs. 12.48mGy, respectively. A less degree of variation was observed in SSDE_dw than that in SSDE_deff. Conclusions SSDE_dw values based on the water-equivalent diameter at the central slice of the scan range got better agreement with those derived from all slices, which could serve as a simpler and more valid indicator to represent the average value of size-specific dose estimates of the whole scan range in head CT examination.

Objective:To summarize the clinical characteristics, common images, pathogens, and gene mutation types of chronic granulomatosis disease (CGD) in 19 children.Methods:The clinical manifestations, laboratory findings, treatment, and prognosis of 19 patients diagnosed with CGD in Hong Kong University-Shenzhen Hospital from December 2012 to December 2018 were analyzed.Results:The 19 patients were all males and confirmed as CGD by the dihydrorhodamine test and gene sequencing.The age of the first infection was mostly 1 month after birth(13 cases), and the age of clinical diagnosis ranged from 2 months to 10 years.Sixteen mothers were carriers.The patients presented with pulmonary fungal infection (19/19 cases), Bacillus Calmette Guerin (BCG)-osis (14/19 cases), lymphadenitis (14/19 cases), perianal abscess (9/19 cases), skin abscess (5/19 cases) and ulcerative colitis (2/19 cases). There were 59 positive cultures.Pathogens included fungi (9 cases), Klebsiella pneumonia (8 cases), mycobacteria (7 cases), Streptococcus Viridans (5 cases), Escherichia coli (3 cases), gram-positive bacteria (3 cases), Staphylococcus aureus (3 cases), and Burkholderia cenocepacia (2 cases). Gene mutations were found in all 19 patients, including 17 cases of CYBB, 1 case of CYBA and 1 case of NCF2.The type of mutations included nonsense mutations (6 cases), deletion mutations (5 cases, including 2 large fragment deletions), splice mutations (3 cases) and missense mutations (5 cases). Five mutations were novel.Splice mutations in 3 cases often led to skin abscess, perianal abscess and lymphadenitis.Two patients with large deletion mutations had more serious infection than other patients. Conclusions:In China, CGD is characterized with pulmonary infection and disseminated BCG-osis.Mycobacteria are common pathogens of CGD, and fungi are dominant pathogens of CGD.The most common infection is respiratory infection. Klebsiella pneumonia and Escherichia coli often lead to perianal abscess.The relationship between gene mutation types and clinical phenotypes requires further verification by big data.

Currently, there are many reports on the clinical phenotypes and epidemiological data of COVID-19. Understanding the underlying pathogenesis is important for disease management. With the implications from studies on severe acute respiratory syndrome (SARS), this review mainly discussed the pathogenesis of COVID-19 and focused on the host immune response to viruses. Direct damage to tissues and organs and excessive inflammatory responses induced by 2019-nCoV are associated with the pathological process and disease progression. Viral load and high inflammatory response are related to disease severity. Peripheral lymphocytes are closely associated with the acute lung injury, and can be used to predict disease severity. 2019-nCoV-specific antibody and T cell immune response can be detected in patients with 2019-nCoV infection. Given the two sides of inflammation and hormone therapy, steroid as a broad-spectrum immunosuppressant needs to be used with caution. Biologics targeting IL-6 and convalescent plasma therapy are promising treatment for COVID-19. Further research on viral features, pathogenesis, immunopathogenesis and specific immune defense would be conducive to better understanding of COVID-19 and achieving precision medicine.

The clinical data of a child with ABCB1 rs1045642 T/T genotype and skin photosensitivity induced by Voriconazole were analyzed retrospectively in Beijing Children′s Hospital, Capital Medical University in September 2020.Literature was reviewed to discuss the relationship between ABCB1 genetic polymorphism and Voriconazole pharmacokinetics.The patient was a 6.8-year-old boy, who was diagnosed with primary immunodeficiency disease.Long-term oral Voriconazole was administered for prevention and treatment of fungal infections.Skin photodistributed erythema and pigmentation occurred about 3-4 weeks after treatment.The skin lesions were significantly alleviated about 1 month after the withdrawal of Voriconazole.Gene test showed ABCB1 rs1045642 T/T in the patient.Some studies reported that ABCB1 rs1045642 T/T genotype reduced the clearance rate of Voriconazole.Monitoring such adverse reaction of Voriconazole in clinical practice is important. ABCB1 gene polymorphism is possible to correlate with the pharmacokinetics and adverse reactions of Voriconazole.However, further large-scale clinical studies are warranted to verify it.

Objective To explore the validity of the size-specific dose estimate (SSDE) derived from the water-equivalent diameter (Dw)value of the slice located in the middle of the scan range in the head CT examination. Methods A total of 197 patients underwent head CT nonenhanced scan were enrolled in this retrospective study. The Dw, size-dependent conversion factor (f), normalized volume CT dose index (CTDIvol) and SSDE values of all slices were calculated. Two sets of SSDE, SSDEgroand SSDEcenbased on the Dwvalues slice by slice (Dw-gro) and the Dwvalues of the slices in the middle of the scan range (Dw-cen), were obtained across all patients. Pearson correlation analysis and linear regression analysis were performed for Dw-grovs Dw-cen, Spearman correlation analysis and linear regression analysis for SSDEgrovs SSDEcen, SSDE vs Dw, CTDIvolvs Dw. With the reference of SSDEgrovalue, mean absolute relative difference (MARD) of SSDEcen values were calculated to assess its accuracy and the correlated factors of MARD was analyzed with multivariate linear stepwise regression analysis. Results The minimal Dwvalue close to the roof of the skull corresponded to the maximal value of f and SSDE, which was the minimal value of CTDIvol. The significant positive correlation was showed between Dw-grovs Dw-cen, SSDEgrovs SSDEcen, SSDE vs Dw, CTDIvolvs Dw(r=0.947, 0.931, 0.416, 0.626;P＜0.05). The values of Dw,groand Dw-cenwere (16.94±0.69) and (18.50±0.62) cm respectively. The values of SSDEgroand SSDEcenwere [54.10 (52.29, 56.39)] mGy and [53.77 (51.85, 55.25)] mGy respectively. An approximation of SSDEcenvalues with an average of 1.62% of the gross MARD was found to match the reference value. Multivariate linear stepwise regression analysis indicated that MARD had negative correlation with Dw(β=–1.319,P＜0.05), positive correlation with CTDIvol(β=0.202,P＜0.05), and f was not included in the multivariate regression equation. Conclusion SSDEcenbased on the Dwvalue of the slice located at the center of the scan range yields small MARD value and can represent a reliable SSDE estimation in the head CT examination.

Objective:To analyze the clinical course and targeted therapy of pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome.Methods:The clinical history of a 6-year-old boy with PAPA syndrome, who was admitted to Hong Kong University Shenzhen Hospital in September 2017, was reviewed. His genetic diagnosis was confirmed by whole exome sequencing. The response to targeted therapy was evaluated by comparing the inflammatory markers (erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) and serum cytokines (interleukin (IL)-1, IL-6 and tumor necrosis factor-α (TNF-α)) before and after biological agents treatment. For literature review, "PAPA syndrome" and"PSTPIP1 gene"were used as keywords to retrieve papers published from January 1997 to December 2019 from Pubmed, Wanfang and CNKI database.Results:The patient was a 6-year-old boy, admitted to the hospital due to recurrent joint swelling and pain for more than 4 years. Before treatment, the CRP (256 mg/L), ESR (105 mm/1 h) and cytokines including serum TNF-α (7.43 ng/L), IL-1 (<5 ng/L), IL-6 (301 ng/L) were significantly elevated. Culture of the joint effusion was negative, but the IL-6 level was above 1 000 ng/L. MRI showed osteomyelitis at the lower end of the right femur. Gene detection found a heterozygous variation of PSTPIP1 gene (c.748G>A, p.E250K). Arthralgia once alleviated after the initiation of tocilizumab and infliximab, but recurred after 1 year of treatment. Thereafter, the anti-IL-1 receptor antagonist (Anakinra) was commenced, followed by a significant improvement of the arthralgia, and a complete remission during the follow-up. Besides, the level of CRP, ESR, serum TNF-α, IL-1 and IL-6 were all decreased to normal on the last followed up in December 2019. Literature review found 29 articles and 87 patients in total. The initial symptoms included those of arthritis ( n=58), pyoderma gangrenosum ( n=33), and acne ( n=24). Among all the cases, 13 genotypes were confirmed, and 47 variations involved amino acid p.E250. Steroid and/or biological agents were used in most patients. Conclusions:PAPA syndrome should be suspected in children with recurrent pyogenic sterile arthritis, and an early diagnosis could be achieved by genetic test. Targeted treatment with biological agent may control the symptoms effectively. Biological agents can control symptoms of this disorder effectively.

Autoimmune lymphoproliferative syndrome (ALPS) is a human genetic disorder of lymphocyte apoptosis resulting in an accumulation of lymphocytes and childhood onset chronic lymphadenopathy, splenomegaly, multilineage cytopenias, and an increased risk of B-cell lymphoma.Since then, with more than 500 patients with ALPS diagnosed worldwide.In this paper, it is hoped that harmonizing the diagnosis and classification of ALPS will foster be-tter understanding of the pathogenesis of ALPS.

Precision medicine aims at using target therapy on specific diseases by studying the pathogenesis and finding biomarkers. Inborn errors of immunity (IEI) are caused by single gene mutations, providing the perfect human models to study immunology. The technology rapidly developes recently, so scientists have a deeper understandings of the phenotypes, genotypes, and the biological targets, so that doctors are able to use precision medicine on IEIs with many successful cases. The precision medicine have advantages in the treatment of pathogenesis of diseases. This article summarizes successful cases of using precision medicine for IEI recently.