[Different methods in predicting mortality of pediatric intensive care units sepsis in Southwest China].

Objective: To investigate the value of systemic inflammatory response syndrome (SIRS), pediatric sequential organ failure assessment (pSOFA) and pediatric critical illness score (PCIS) in predicting mortality of pediatric sepsis in pediatric intensive care units (PICU) from Southwest China. Methods: This was a prospective multicenter observational study. A total of 447 children with sepsis admitted to 12 PICU in Southwest China from April 2022 to March 2023 were enrolled. Based on the prognosis, the patients were divided into survival group and non-survival group. The physiological parameters of SIRS, pSOFA and PCIS were recorded and scored within 24 h after PICU admission. The general clinical data and some laboratory results were recorded. The area under the curve (AUC) of the receiver operating characteristic curve was used to compare the predictive value of SIRS, pSOFA and PCIS in mortality of pediatric sepsis. Results: Amongst 447 children with sepsis, 260 patients were male and 187 patients were female, aged 2.5 (0.8, 7.0) years, 405 patients were in the survival group and 42 patients were in the non-survival group. 418 patients (93.5%) met the criteria of SIRS, and 440 patients (98.4%) met the criteria of pSOFA≥2. There was no significant difference in the number of items meeting the SIRS criteria between the survival group and the non-survival group (3(2, 4) vs. 3(3, 4) points, Z=1.30, P=0.192). The pSOFA score of the non-survival group was significantly higher than that of the survival group (9(6, 12) vs. 4(3, 7) points, Z=6.56, P<0.001), and the PCIS score was significantly lower than that of the survival group (72(68, 81) vs. 82(76, 88) points, Z=5.90, P<0.001). The predictive value of pSOFA (AUC=0.82) and PCIS (AUC=0.78) for sepsis mortality was significantly higher than that of SIRS (AUC=0.56) (Z=6.59, 4.23, both P<0.001). There was no significant difference between pSOFA and PCIS (Z=1.35, P=0.176). Platelet count, procalcitonin, lactic acid, albumin, creatinine, total bilirubin, activated partial thromboplastin time, prothrombin time and international normalized ratio were all able to predict mortality of sepsis to a certain degree (AUC=0.64, 0.68, 0.80, 0.64, 0.68, 0.60, 0.77, 0.75, 0.76, all P<0.05). Conclusion: Compared with SIRS, both pSOFA and PCIS had better predictive value in the mortality of pediatric sepsis in PICU.

[Continuous positive airway pressure therapy affects the recurrence of atrial fibrillation in patients with obstructive sleep apnea: a systematic review and meta-analysis].

Objective: A higher incidence of atrial fibrillation is associated with obstructive sleep apnea. The effects of continuous positive airway pressure on atrial fibrillation have been studied in observational studies and randomized controlled trials. We therefore conducted this meta-analysis to assess the effect of continuous positive airway pressure on the recurrence of atrial fibrillation after radiofrequency ablation. Methods: A comprehensive search was conducted in Pubmed, Embase, Cochrane, Web of Science, Wanfang Data and CNKI databases from inception to October 2022. We included cohort studies and randomized controlled trials containing atrial fibrillation situation after catheter ablation with and without continuous positive airway pressure therapy. The random effects model was used to assess odds ratios (OR) and confidence intervals (CI). I2 was used to assess the heterogeneity. Results: Eight studies with a total of 1 395 patients with obstructive sleep apnea met the inclusion criteria. Continuous positive airway pressure therapy decreased atrial fibrillation recurrence by 61% (OR=0.392, 95%CI: 0.267-0.576, I2=37.6%). Subgroup analysis showed that the protective effect was more significant in groups with more hypertension patients (OR=0.272 vs. 0.550, 95%CI: 0.165-0.449 vs. 0.329-0.922). Conclusions: Continuous positive airway pressure therapy reduces the recurrence rate of atrial fibrillation. Patients with hypertension are more likely to benefit from it.

[Nontuberculous mycobacteria infection and pulmonary alveolar proteinosis in a patient with hematopoietic defects].

A 28-year-old male with a history of leukopenia was admitted with complaints of fever, cough, and dyspnea for 3 months. Initial work-up identified reduced circulating levels of granulocytes, monocytes, lymphocytes, and NK cells. Computed tomography revealed bilateral reticulonodular opacities and mediastinal lymph node enlargement. Peripheral blood culture and mediastinal lymph node aspiration yielded Mycobacterium avium. Genetic testing revealed a heterozygous germline GATA2 mutation (c.1187G>A, R396Q). Despite standard anti-mycobacterial therapy, the patient's dyspnea worsened and subsequent imaging studies revealed diffuse ground-glass opacification. A transbronchial lung biopsy confirmed the development of pulmonary alveolar proteinosis. Bone marrow transplantation had not been performed due to the unavailability of suitable donors. The disease progressed after whole lung lavage, and the patient died at the age of 31 years from respiratory failure. The current case report emphasized the importance of raising awareness about the rare GATA2 deficiency, which is characterized by hematologic abnormalities, primary immunodeficiency, and pulmonary alveolar proteinosis.

[An investigation on the nutritional status and support of in-patients with common variable immunodeficiency].

The study aimed to reveal for the first time the clinical characteristics, nutritional and metabolic status and support of hospitalized patients with common variant immunodeficiency disease (CVID), and provide reference to improve the long-term nutritional management for such patients. This is a retrospective cross-sectional study. Through searching the electronic medical record system of Peking Union Medical College Hospital, the study included 33 consecutive in-patients with CVID diagnosed in Jan 2016 to Jun 2021, with the male to female ratio of 16∶17. All their medical data, nutritional assessment and intervention retrospectively summarized and analyzed. Data with normal distribution were described using (x¯±s), and analyzed with independent sample t-test. Data with non-normal distribution were compared with non-parametric test. The results showed that the median onset-age of the included patients was 22 (10.0,36.5) years old, and the median duration was 9.0 (2.0,16.0) years. All patients had recurrent infections involving various systems (33/33), with development of autoimmune diseases (8/33) and lymphoproliferative disease or malignancy (9/33) in some cases among them. The nutritional risk screening 2002 (NRS 2002) scores revealed that 85.19% of adults had an NRS 2002≥3 points, and 33.33% of children had a BMI-for-age z score<-2. Weight loss occurred in 66.67% of patients (22/33), while 87.88% (29/33), 69.70% (23/33) and 81.82% (27/33) of patients respectively had anemia, hypoalbuminemia and decreased prealbumin. Among 22 patients with micronutrients status evaluated, 77.27% (17/22), 22.73% (5/22) and 31.82% (7/22) of patients respectively had lowered serum iron, folate deficiency and vitamin B12 insufficiency. Six patients underwent 25-OH-VD3 measurement, and were all testified to have vitamin D deficiency. Among all patients with nutritional risk, 56.00% of them underwent nutritional support: oral nutritional supplements (14 cases), enteral feeding (4 cases) and parenteral nutrition (5 cases). In conclusion, the condition of malnutrition was prevalent in patients with CVID, but was under-recognized and undertreated to some degree.

[Cough, bloody sputum, eosinophil elevation and lung shadow].

A 47-year-old man was referred to the pulmonary clinic with a 2-year history of productive cough and 3-month history of hemoptysis. Two years ago, his chest CT scan revealed a 2 cm×2 cm well-defined nodule in the right upper lung. His cough was alleviated without treatment. Three months ago, he had a productive cough with the bloody sputum after a running to catch the bus. Physical examination was normal. Complete blood count (CBC) showed an elevated eosinophil count (42.61%). Chest CT scan showed that the enlargement of the right upper lobe nodule (3.4 cm×3.3 cm), with bilateral pathy lesions distributed in the right upper lobe and the left lower lobe. Pathological study of needle specimen biopsy showed the lamellated cyst wall of hydatid cyst, brood capsule formation and hooklet. Pulmonary hydatidosis was diagnosed. It was recommended that the patient should be treated by surgery combined with albendazole. His symptoms relieved and lung nodules were shrinking without treatment after 5 years follow-up.

[Waxing and waning pulmonary nodules and cavities].

We reported a case of vascular Ehlers-Danlos syndrome presenting with recurrent pulmonary hemorrhage. A 22-year-old man was admitted for intermittent hemoptysis and chest pain during the past 18 months. Computed tomography of chest showed bilateral nodules and cavities with halo sign. Inflammatory markers, including erythrocyte sedimentation rate, C reactive protein and interleukin 6, were within normal range. The microbiological and pathological examination of bronchoalveolar lavage fluid and CT-guided percutaneous lung biopsy failed to draw a diagnosis. The pulmonary lesions waxed and waned despite empirical antibacterial, antifungal, antimycobacterial, and anti-parasite treatment. Video-assisted thoracoscopic lung biopsy showed pulmonary hemorrhage, hematoma, ossification, and fibrous nodules, suggesting vascular Ehlers-Danlos syndrome. The molecular testing revealed a heterozygous missense variant in the COL3A1 gene which confirmed the diagnosis of vascular Ehlers-Danlos syndrome. The patient had no skin hyperextensibility or joint hypermobility. During 3-year follow-up, there were no evidence of other vascular or organ involvement except he had intermittent minor hemoptysis. Through this clinical pathological discussion, we aimed to remind pulmonologist to consider the possible diagnosis of vascular Ehlers-Danlos syndrome in young patients with recurrent hemoptysis and waxing and waning pulmonary nodules, cavities, or cysts on CT scan who has neither obvious systematic inflammation nor effective reaction on empirical antimicrobial therapy. Molecular testing should be carried out as soon as possible in a suspected patient to avoid unnecessary invasive examinations.

[Efficacy of venetoclax combined azacitidine in newly diagnosed acute myeloid leukemia unfit for standard chemotherapy: a single center experience].

Objective: To investigate the effectiveness and safety of the VA regimen, which combines venetoclax with azacitidine in the treatment of patients with newly diagnosed acute myeloid leukemia (AML) who are not suitable candidates for conventional chemotherapy. Methods: In the Department of Hematology at the Second Hospital of Hebei Medical University, 66 AML patients who received venetoclax and azacitidine treatment from May 2020 to March 2022 were the subject of a retrospective study. The complete remission (CR) rate, cCR rate, ORR rate, MRD negative rate, the incidence of adverse events,1-year EFS, and OS were retrospectively analyzed. Patients subgroups with varying ages, ECOG scores, primary and secondary, risk stratifications, and gene mutation were compared for differences in efficacy and survival. Results: The median follow-up was 4.25 (0.9-19.9) months, and the median number of treatment courses was 2 (1-8) cycles. After the first cycle, the cCR rate was 78.8% , and the MRD negative rate was 51.9% . After prolonged treatment, the cCR rate was 81.8% and MRD negative rate was 66.7% . The median EFS and OS, respectively, were13.2 and 15.3 months. Secondary AML showed inferior efficacy and prognosis. IDH1/2 or NPM1 mutation groups had a significantly higher rate of CR than the control group (P<0.05) . The CR rate and MRD negative rate of patients with rebound thrombocytosis were significantly higher than those without rebound thrombocytosis (P<0.05) . Those who had epigenetic modification mutations (DNMT3, ASXL1, TET2) were more likely to benefit from ongoing therapy. The most common grade 3 and 4 adverse reactions were neutropenia, thrombocytopenia, and anemia. Conclusions: In real-world patients with newly diagnosed AML who are not candidates for standard chemotherapy, the VA regimen produces rapid deep remission. Primary AML patients, rebound thrombocytosis, IDH1/2, and NPM1 gene mutations are favorable factors for treatment benefit, and adverse reactions were tolerable.

Effects of Mo contents on the microstructure, properties and cytocompatibility of the microwave sintered porous Ti-Mo alloys.

The porous Ti-Mo alloys were prepared by microwave sintering, and the effects of Mo contents on the pore structure, phase composition, compressive strength, elastic modulus, bending strength, corrosion resistance and cytocompatibility of porous Ti-Mo alloys were investigated. The results show that the porous Ti-Mo alloys are composed of α phase and ß phase, and the volume fraction of ß phase increases with increasing the Mo contents. The amount of Kirkendall pores distributed over the porous Ti-Mo alloys skeleton increases with increasing the Mo contents, which greatly increases the porosities and pore sizes of the porous Ti-Mo alloys. Correspondingly, all of the compressive strength, elastic modulus and bending strength of the porous Ti-Mo alloys decrease with increasing the Mo contents. The porous Ti-Mo alloys present excellent corrosion resistance in the Hank's solution due to the oxidation film of TiO2, MoO2 and MoO3 naturally formed on the surface, and the Mo contents have no obvious effect on the corrosion resistance. The cell viabilities of the porous Ti-Mo alloys are higher than 94%, indicating the porous Ti-Mo alloys possess favorable cytocompatibility. Moreover, the porous Ti-Mo alloys are beneficial to the spread, proliferation and differentiation of osteoblast-like cells, and the Mo contents have no significant effect on the cytocompatibility of the porous Ti-Mo alloys.

Epigallocatechin gallate inhibits the proliferation and induces apoptosis of multiple myeloma cells via inactivating EZH2.

OBJECTIVE: Epigallocatechin gallate (EGCG), the major chemical constituent of green tea, exhibits remarkable anti-tumor effect properties. In the present work, we aim to explore the effect and underlying mechanism of EGCG on multiple myeloma (MM) cells. MATERIALS AND METHODS: The effects of EGCG on MM cells proliferation and apoptosis were determined by CCK-8 assay and flow cytometry assay. The siRNAs were used to inhibit endogenous expression of EZH2. Enforced expression of EZH2 in U266 cells was accomplished by transfecting EZH2 plasmid. RESULTS: EGCG suppressed proliferation and induced apoptosis in U266 cells, which accompanied by EZH2 inhibition. Moreover, we revealed that enforced expression of EZH2 increased MM cells proliferation and reduced cell apoptosis, whereas EGCG partially reversed the effects of EZH2 on MM cells progression. In addition, qRT-PCR and Western blot showed that EZH2 overexpression increased Bcl-2 expression, and decreased BAX, BAK1 and cytochrome c expression in U266 cells exposed to EGCG. CONCLUSIONS: Our data showed that EGCG inhibited MM cells proliferation and induced apoptosis by targeting EZH2 and modulated mitochondrial apoptosis pathway, indicating EGCG might act as an adjuvant for chemotherapy of MM patients.

Changes in cerebrovascular reactivity in healthy adults after acute exposure to high altitude.

OBJECTIVE: To study changes in the cerebrovascular reactivity (CVR) at different altitude area in healthy adults. SUBJECTS AND METHODS: CVR was tested using transcranial Doppler combined with CO2 inhalation, near-infrared spectroscopy (NIRS) was used to detect the regional cerebral oxygen saturation (rScO2). Blood samples were collected, and the vasoactive substances in serum were detected using the enzyme-linked immunosorbent assay. In this study, 59 healthy adults were divided into 3 groups: low altitude group, medium altitude group and high altitude group. All the indicators in low altitude group were tested at 24h before departure and after arrival from Beijing (at an altitude of 44.4 m) to Xining (at a medium altitude of 2200 m). Then, after resting for 48h, all the indicators were tested at 24h and 48h after arrival from Xining (at a medium altitude of 2200 m) to Yushu Jiegu town (at a high altitude of 3700 m) together with those at the medium altitude. Intergroup comparisons were made for the subjects in the three altitudes. RESULTS: There was an increase in the CVR in low altitude group after acute exposure to high altitude, and the difference was significant (CVR: 1.94re was vs. 0.91±0.53, p<0.001); the CVR index was increased, and the difference was significant [cerebrovascular reserve index (CVRI): 3.65he CVR vs. 1.37e CVR, p<0.001]; the rScO2 level was decreased with the increase of altitude, and the difference was significant [(66.78±4.61)% vs. (70.29±4.52)%, p<0.001]. The levels of vasoactive substances in low altitude group were decreased after acute exposure to high altitude compared with those before exposure: NO: [(79.14±9.54) µmol/L vs. (58.01±9.93) µmol/L, p<0.001]; serum eNOS level was increased, and the difference was significant [(77.23±6.20) pg/ml vs. (65.07±9.82) pg/ml, p<0.001]; EPO: [(84.68±13.16) pg/ml vs. (65.01±5.92) pg/ml, p<0.001]; VEGF: [(71.91±11.62) pg/ml vs. (54.92±11.86) pg/ml, p<0.001]; sFlt: [(384.18±42.73) pg/ml vs. (320.62±78.96) pg/ml, p<0.001]. There was also an increase in CVR in medium altitude group after acute exposure to high altitude, and the difference was significant [CVR: 2.00±0.79 vs. 0.91±0.66, p<0.001]; the difference of CVRI was significant [3.83±0.67 vs. 1.67±0.87, p<0.001]; rScO2 was slightly decreased with the increase of altitude, and the difference was not statistically significant [(67.53±4.61) % vs. (69.63±5.59) %, p<0.001]. Before exposure to high altitude area, the levels of NO, NOS, EPO, VEGF, and sFlt in low and medium altitude groups were higher than those in high altitude group. CVR level of subjects at different altitudes was negatively related to the ScO2 (r=-0.91) but positively related to NO and NOS levels (rs=0.89, r=0.75); CVR was moderately related to VEGF and EPO (rs=0.45, r=0.42). rScO2 was positively related to RBC, HB and VEGF levels (r=0.89, r=0.75, rs=0.86), but had a moderately negative correlation with NO and NOS levels (rs=-0.52, r=-0.57). CONCLUSIONS: After subjects at a low altitude are exposed to high altitude rapidly, CVR is increased, RBC and vasoactive substances in serum, such as NO, eNOS, and EPO, are dramatically increased, VEGF is increased first and then decreased, sFlt-1 level is increased gradually, and rScO2 level is gradually decreased with the increase of altitude, indicating the local brain anoxia of subjects at a high altitude.

[Efficacy and safety of IA regimen containing different doses of idarubicin in de-novo acute myeloid leukemia for adult patients].

Objective: To investigate the efficacy and safety of IA regimen which contains idarubicin (IDA) 8 mg/m(2), 10 mg/m(2) or 12 mg/m(2) as induction chemotherapy for adult patients with de-novo acute myeloid leukemia (AML) . Methods: A total of 1 215 newly diagnosed adult AML patients, ranging from May 2011 to March 2015 in the First Affiliated Hospital of Soochow University and other 36 clinical blood centers in China were enrolled in the multicenter, single-blind, non-randomized, clinical controlled study. To compare the response rate of complete remission (CR) , adverse events between different dose idarubicin combined with cytarabine (100 mg/m(2)) as induction chemotherapy in newly diagnosed patients of adult AML. Results: Of 1 207 evaluable AML patients were assigned to this analysis of CR rate. The CR rates of IDA 8 mg/m(2) group, IDA 10 mg/m(2) group and IDA 12 mg/m(2) group were 73.6% (215/292) , 84.1% (662/787) and 86.7% (111/128) , respectively (P<0.001) . After adjusted for age, blast ratio of bone marrow, FAB classification and risk stratification, the odds ratios (95% CI) of IDA 10 mg/m(2) group and IDA 12 mg/m(2) group were 0.49 (0.34-0.70) and 0.36 (0.18-0.71) , as compared with the IDA 8 mg/m(2) group (P<0.001, P=0.003) . In the intermediate and favorable groups, CR rates was 76.5% (163/213) , 86.9% (506/582) and 86.1% (68/79) in different doses of IDA (P=0.007) . Interestingly, IA regimen with IDA 10 mg/m(2) was the only beneficial factor affecting CR in this group after adjusted for age, blast ratio of bone marrow and FAB classification[OR=0.47 (95% CI 0.31-0.71) , P<0.001]. CR rates in adverse group was 50.0% (18/36) , 60.6% (43/71) and 81.8% (18/22) respectively (P=0.089) . However, the odds ratios (95% CI) of IDA 12 mg/m(2) when compared with the IDA 8 mg/m(2) was 0.22 (0.06-0.80) , after adjusted for age, blast ratio of bone marrow and FAB classification. The median time (days) of neutrophil count less than 0.5×10(9)/L in IDA 8 mg/m(2) group, IDA 10 mg/m(2) group and IDA 12 mg/m(2) group were 14 (11-18) , 15 (11-20) and 18 (14-22) , respectively (P=0.012) and of platelet count lower than 20×10(9)/L were 14 (7-17) , 15 (11-20) and 17 (15-21) , respectively (P=0.001) . The incidences of lung infection in the three groups were 9.8%, 13.5% and 25.2%, respectively (P<0.001) . Conclusions: For young adult patients (aged 18-60 years) with AML in China, intensifying induction therapy with idarubicin 10 mg/m(2) is clinically superior to IDA 8 mg/m(2) and IDA 12 mg/m(2) in favorable intermediate AML subgroup. However, idarubicin 12 mg/m(2) is more suitable to adverse AML subgroup.

[Targeted suppression of miRNA-21 inhibit K562 cells growth through PTEN-PI3K/AKT signaling pathway].

Objective: To investigate the K562 cells biological function and related molecular changes in PTEN-PI3K/AKT signaling pathway of leukemia K562 cells by inhibiting the miRNA-21 expression to explore its pathogenesis of leukemia. Methods: The chemical synthetic miRNA-21 inhibitor was transfered into K562 cells by electrotransfection. RT-PCR was used to detect the miRNA-21 expression changes. Cell proliferation and apoptosis were determined by using MTT and flow cytometry. Western-blot were used to detect the protein expression changes of PTEN, PI3K and p-AKT respectively. Results: The relative expression of miRNA-21 in experimental group was (8.070 ± 5.138)% at 24 hours, which was lower than control groups (P<0.05). The apoptotic rate of (13.370±0.250)% at 24 hours in experimental group was obviously higher than control groups. The cellular proliferation were significantly different at 24 hours. The proliferation inhibition rate was (8.1±0.9)% at 24 hours, which was up to (43.1±2.1)% at 60 hours, but the control groups showed no difference. K562 cell proliferation significantly decreased, while cell apoptosis markedly increased by inhibiting miRNA-21 expression (P<0.01). Western-blot analysis revealed up-regulation of PTEN and down-regulation of PI3K and p-AKT protein expressions after successfully suppressed miRNA-21 expression (P<0.01). Conclusion: Inhibiting miRNA-21 expression in K562 cell could suppress the PI3K/AKT pathway by up-regulation of PTEN expression and promote cell antiproliferative and pro-apoptosis effects.

[A multi-center clinical observation on safety and efficacy of a plasma derived coagulator factor â § for treatment of patients with hemophilia A].

OBJECTIVE: To evaluate the efficacy and safety of homemade plasma derived coagulation factor Ⅷ in patients with hemophilia A. METHODS: Patients with congenital hemophilia A who met the inclusive and exclusive criteria were enrolled in the study after informed consent. The doses of factor Ⅷ were calculated according to the weight, disease severity etc. FⅧ activity and infusion efficacy value at 10 min and 60 min after infusion were recorded, as well as adverse events and validity rating according to the improvement of clinical syndromes. Viral infections including HBV, HCV, HIV and FⅧ inhibitor were determined after 3 and 6 months. RESULTS: A total of 65 patients were enrolled in this study, all of whom were evaluable for drug safety. Treatment efficacy was evaluated in 60 patients and 57 cases completed the trial finally. In this 57 cases, most (52/57) subjects were of middle and severe hemophilia A mainly characterized by joint bleeding. Overall response rate of acute bleeding events was classified as "excellent" (70.00%) or "better" (30.00%). The non-responder was 0. FⅧ activity and infusion efficiency value of first administration after 10 min and 60 min improved significantly [10 min: (123.66±47.54)%; 60 min: (108.05±43.24)%]. The incidence of adverse events was 1.54%. Neither allergic reaction nor reactivation of HBV, HCV, HIV was detected after treatment of 3 and 6 months. No FⅧ inhibitor negative patients converted to positive during follow-up. CONCLUSION: This homemade plasma derived coagulation factor Ⅷ is safe and effective for the treatment of acute bleeding in patients with hemophilia A.

[Expression of porforin and granzyme B in familial hemophagocytic lymphohistiocytosis].

OBJECTIVE: To analyze the correlation between genetic variants of PRF1 and expression level of perforin and granzyme B protein, and further determine the relationship between PRF1 gene variants and cytotoxic T lymphocyte/natural killer (CTL/NK) cell function in famililal hemophagocytic lymphohistiocytosis (FHL2). METHODS: Eight children of FHL2 (P1-P8) after treatment, as well as parents and siblings of P1-P5 were included, and thirty healthy children came for physical examination were designated as controls. PRF1, Unc13D, STX11, STXBP2, RAB27A, LYST, SH2D1A, BIRC4 exons were amplified by PCR and followed by direct sequencing. Bioinformatics analysis of mutant PRF1 was performed by ExPASy online system. Perforin and granzyme B expression on cytotoxic lymphocyte was detected by flow cytometry. RESULTS: ① Three of eight FHL2 children harbored heterozygous missense of PRF1 exons: P1 had compound heterozygous missense mutations (R4C and R33H) and P2 had heterozygous mutations (V50L), P3 had heterozygous mutations (R489W), which confirmed the diagnosis of FHL2. The father (F1) and younger brother (B1) of P1 also had compound heterozygous missense mutation (R4C/R33H), the mother (M2) and younger brother (B2) of P2 had V50L mutation, the father (F3) of P3 had no R489W mutation and the mother of P3 did not participate in this research, so mutation of R4C/R33H of P1 inherited from paternal line, and V50L mutation of P2 came from maternal line, R489W mutation of P3 came from maternal line. ② Comparing to control group, perforin expression of CD8(+) T cells and natural killer (NK) cells of P1, F1, B1, P2, M2 and B2 decreased significantly, but there was no significant difference between two groups in terms of granzyme B expression. CONCLUSIONS: R4C/R33H compound heterozygous mutation and V50L heterozygous mutation all cause lower expression of perforin on CTL/NK cells, and may be causative mutations for familial hemophagocytic lymphohistiocytosis.

Microstructure, mechanical properties and superelasticity of biomedical porous NiTi alloy prepared by microwave sintering.

Porous NiTi alloys were prepared by microwave sintering using ammonium hydrogen carbonate (NH4HCO3) as the space holder agent to adjust the porosity in the range of 22-62%. The effects of porosities on the microstructure, hardness, compressive strength, bending strength, elastic modulus, phase transformation temperature and superelasticity of the porous NiTi alloys were investigated. The results showed that the porosities and average pore sizes of the porous NiTi alloys increased with increasing the contents of NH4HCO3. The porous NiTi alloys consisted of nearly single NiTi phase, with a very small amount of two secondary phases (Ni3Ti, NiTi2) when the porosities are lower than 50%. The amount of Ni3Ti and NiTi2 phases increased with further increasing of the porosity proportion. The porosities had few effects on the phase transformation temperatures of the porous NiTi alloys. By increasing the porosities, all of the hardness, compressive strength, elastic modulus, bending strength and superelasticity of the porous NiTi alloys decreased. However, the compressive strength and bending strength were higher or close to those of natural bone and the elastic modulus was close to the natural bone. The superelastic recovery strain of the trained porous NiTi alloys could reach between 3.1 and 4.7% at the pre-strain of 5%, even if the porosity was up to 62%. Moreover, partial shape memory effect was observed for all porosity levels under the experiment conditions. Therefore, the microwave sintered porous NiTi alloys could be a promising candidate for bone implant.

Effect of micro-arc oxidation surface modification on the properties of the NiTi shape memory alloy.

In this paper, the effects of micro-arc oxidation (MAO) surface modification (alumina coatings) on the phase transformation behavior, shape memory characteristics, in vitro haemocopatibility and cytocompatibility of the biomedical NiTi alloy were investigated respectively by differential scanning calorimetry, bending test, hemolysis ratio test, dynamic blood clotting test, platelet adhesion test and cytotoxicity testing by human osteoblasts (Hobs). The results showed that there were no obvious changes of the phase transformation temperatures and shape memory characteristics of the NiTi alloy after the MAO surface modification and the coating could withstand the thermal shock and volume change caused by martensite-austenite phase transformation. Compared to the uncoated NiTi alloys, the MAO surface modification could effectively improve the haemocopatibility of the coated NiTi alloys by the reduced hemolysis ratio, the prolonged dynamic clotting time and the decreased number of platelet adhesion; and the rough and porous alumina coatings could obviously promote the adherence, spread and proliferation of the Hobs with the significant increase of proliferation number of Hobs adhered on the surface of the coated NiTi alloys (P < 0.05).

Extracorporeal membrane oxygenation and Thoratec pneumatic ventricular assist devices as double bridge to heart transplantation.

INTRODUCTION: Ventricular assist devices have benefited patients with end-stage heart failure as a bridge to heart transplantation (HTx). We present our experiment of HTx using extracorporeal membrane oxygenation (ECMO) with Thoratec pneumatic ventricular assist device (TpVAD). METHODS: From May 1996 to June 2011, among 410 patients who underwent HTx 23 required mechanical circulatory support (MCS) with implantation of the TpVAD and 15 (65%) of them received grafts. RESULTS: The 23 patients included 4 female and 19 male patients of age range 10 to 80 years. Eighteen (78%) of them needed ECMO before TpVAD implantation. Twelve (67%) were implanted with a TpVAD double bridge to HTx. The demand for MCS among patients with acute hemodynamic collapse has led to major improvements in the existing systems such as ECMO with double bridge to TpVAD. CONCLUSION: We used ECMO as a rescue procedure for acute hemodynamic deterioration. However, during ECMO support, left ventricular afterload increased. If prolonged support is required, TpVAD might be required: 15 (65%) of patients supported by ECMO with TpVAD needed to a wait a suitable donor. We recommend the application of ECMO for short-term support (within 1 week), and TpVAD as a bridge for medium- or long-term support.