Fibrous dysplasia of bone (FD) is a tumorlike disease characterized by intramedullary fibrosis, in which the development of the bone in the lesion area stops at the stage of immature braided bone, with the inability to form a normal bone trabecula, resulting in structural changes and reduced mechanical strength of the bone. Repeated pathological fractures often occur with weight bearing, followed by curvature of the affected bone, limb shortening, and abnormal gait. The proximal femur is often involved in FD limb malformations, with complex types and degrees, most of which are manifested as gradually aggravating hip varus and diaphysial curvature. The proximal femur is a common site of limb deformity caused by FD, the types and severity of malformations are complex and varied, which is usually manifested as gradually aggravated varus hip joint and diaphysis bending deformity. The purpose of deformity correction is to restore the normal mechanical axis and length of the femur, thereby restoring the function of the limb, avoiding the progression of deformity and relieving the pain symptoms caused by repeated pathological microfractures, which is more important than the treatment of the lesion itself. The preoperative treatment plan should be made individually for each patient according to the location and extent of the lesion and the type of the lesion. The patients need to be followed up for a long time to adjust the correction plan. Whether the lesion should be curette and bone graft and the type of bone graft material used are still controversial. The femoral deformity of FD should be analyzed based on the principles of deformity correction, the type of deformity and the location of the apex of the deformity should be determined, the osteotomy plan should be designed, and the preoperative simulation should be performed. Both intramedullary and extramedullary fixation after osteotomy can provide sufficient biological stability. The choice of fixation device should be determined according to the specific situation during the operation. There is no obvious abnormality in bone healing and regeneration in FD patients, but dysplastic bone tissue is included in the callus formation. The limb deformity of FD patients is prone to relapse after treatment, long-term close follow-up is needed to adjust the correction plan.

Objective To compare the predictive value of Fried frailty phenotype (FP) and Tilburg frailty Index (TFI) to the quality of life in community elderly people so as to provide a reference for the grass-roots general practitioners to choose the appropriate quality of life screening tool in the community elderly people.Methods Using the convenient sampling method,638 elderly people in the community were selected as the research subjects.FP and TFI were used to assess frailty,and the quality of life was evaluated by the brief health questionnaire (SF-12).The Kappa value was used to evaluate the consistency of the two scales. The Pearson correlation was used to detect the correlation between the two scales and SF-12 respectively,and the Bayes discriminant analysis was used to evaluate and analyze the predictive ability of the two scales on the quality of life.Results Among the 638 community elderly people,the screened incidence rate of frailty was 15.4％ by FP and 28.2％ by TFI,the Kappa value of FP and TFI consistence test was 0.569 (P<0.001). The results of correlation analysis showed that FP and TFI were negatively correlated with the total physical health score (PCS) and total mental health score (MCS) of SF-12 (FP with PCS and MCS,r=-0.772,-0.349,P<0.01;TFI with PCS and MCS,r=-0.738,-0.491,P<0.01).Taking the quality of life as the criterion,the cross-validation accuracy rates of FP and TFI for predicting the PCS decline were 76.65％ and 71.63％ respectively,which for MCS decline was 72.10％ and 75.55％ respectively.Conclusion Both FP and TFI all have the predictive value on the decline of quality of life of the community elderly people,but TFI has the characteristics of multi-dimensions and easy application,so it is more effective and practical for evaluating the quality of life in the community elderly people.

Objective To retrospectively analyze of factors influencing early preterm birth(EPB)and late preterm birth(LPB)in pregnancy women with placenta previa complicated by placenta accreta spectrum disorders(PAS),and assess maternal and infant outcomes.Methods We included 590 cases of pregnancy women with placenta previa complicated by PAS who underwent cesarean sections at five hospitals in Wuhan and Xianning cities between January 2018 and June 2021.These patients were divided into three groups based on delivery gesta-tional age:EPB,LPB,and term birth(TB).A multiple logistic regression model was employed to analyze the risk factors associated with EPB and LPB.Additionally,differences in early maternal and infant outcomes among these groups were examined.Results Among 590 pregnancy women with placenta previa complicated by PAS,the proportions of EPB and LPB were 9.7%and 54.4%.The use of uterine contraction inhibitors prior to cesarean section,vaginal bleeding,and previous cesarean sections history were identified as risk factors for both EPB and LPB.The proportion of severe postpartum hemorrhage was comparable between the EPB group and the LPB group;however,the incidence of neonatal asphyxia,low birth weight infants,and the rate of newborns transferred to the Neonatal Intensive Care Unit(NICU)within 24 hours after cesarean delivery were significantly higher in the EPB group compared to the LPB group.Conclusions Placenta previa complicated by PAS predominantly leads to LPB.The history of prior cesarean sections,uterine contractions,and vaginal bleeding prior to cesarean section,are sig-nificantly associated with both EPB and LPB.During the perinatal period,efforts should be made to extend gesta-tional weeks under close monitoring to minimize the incidence of premature births and thereby improve early mater-nal and infant outcomes.

Background::Pulmonary arterial hypertension (PAH) is characterized by excessive proliferation of small pulmonary arterial vascular smooth muscle cells (PASMCs), endothelial dysfunction, and extracellular matrix remodeling. G protein-coupled receptor kinase 2 (GRK2) plays an important role in the maintenance of vascular tone and blood flow. However, the role of GRK2 in the pathogenesis of PAH is unknown.Methods::GRK2 levels were detected in lung tissues from healthy people and PAH patients. C57BL/6 mice, vascular smooth muscle cell-specific Grk2-knockout mice ( Grk2?SM22), and littermate controls ( Grk2flox/flox) were grouped into control and hypoxia mice ( n = 8). Pulmonary hypertension (PH) was induced by exposure to chronic hypoxia (10%) combined with injection of the SU5416 (cHx/SU). The expression levels of GRK2 and Yes-associated protein (YAP) in pulmonary arteries and PASMCs were detected by Western blotting and immunofluorescence staining. The mRNA expression levels of Grk2 and Yes-associated protein ( YAP) in PASMCs were quantified with real-time polymerase chain reaction (RT-PCR). Wound-healing assay, 3-(4,5)-dimethylthiahiazo (-z-y1)-3,5-di-phenytetrazoliumromide (MTT) assay, and 5-Ethynyl-2′-deoxyuridine (EdU) staining were performed to evaluate the proliferation and migration of PASMCs. Meanwhile, the interaction among proteins was detected by immunoprecipitation assays. Results::The expression levels of GRK2 were upregulated in the pulmonary arteries of patients with PAH and the lungs of PH mice. Moreover, cHx/SU-induced PH was attenuated in Grk2?SM22 mice compared with littermate controls. The amelioration of PH in Grk2?SM22 mice was accompanied by reduced pulmonary vascular remodeling. In vitro study further confirmed that GRK2 knock-down significantly altered hypoxia-induced PASMCs proliferation and migration, whereas this effect was severely intensified by overexpression of GRK2. We also identified that GRK2 promoted YAP expression and nuclear translocation in PASMCs, resulting in excessive PASMCs proliferation and migration. Furthermore, GRK2 is stabilized by inhibiting phosphorylating GRK2 on Tyr86 and subsequently activating ubiquitylation under hypoxic conditions. Conclusion::Our findings suggest that GRK2 plays a critical role in the pathogenesis of PAH, via regulating YAP expression and nuclear translocation. Therefore, GRK2 serves as a novel therapeutic target for PAH treatment.

Humans ; Prognosis ; Multiple Myeloma/diagnosis* ; Neoplasm Staging ; Hematopoietic Stem Cell Transplantation ; Patients ; Retrospective Studies

Objective:To conduct a comparative analysis of the efficacy, safety, and cytokine changes associated with three distinct dose escalation regimens of allergen-specific immunotherapy (AIT), and to provide valuable insights into the implementation of safer and more effective accelerated immunotherapy in clinical practice.Methods:A prospective study of subcutaneous immunotherapy (SCIT) was conducted at Renmin Hospital of Wuhan University, involving patients with allergic rhinitis visited from 2019 to 2022. Participants were allocated to one of three treatment groups based on their preferences: conventional immunotherapy (CIT, 23 cases), cluster immunotherapy (CLIT, 25 cases), or rush immunotherapy (RIT, 18 cases). The RIT group received a single subcutaneous injection of 150 mg of omalizumab one week before commencing treatment. Subjective evaluation indices, including the Combined Symptom and Medication Score (CSMS), Visual Analogue Scale (VAS), and single symptom score, were recorded alongside objective evaluation indices (e.g., sIgE, tIgE, Th1/2 and Th17 cytokines) and adverse reactions. Assessments were conducted at baseline, and at 1, 7, and 15 weeks after treatment. SPSS 22.0 software was used for data processing and analysis.Results:The study included a total of 66 patients, comprising 37 males and 29 females, who completed the treatment regimen. In all three groups, CSMS and VAS scores showed significant reductions at 1, 7, and 15 weeks post-treatment (all P<0.05). Notably, the RIT group demonstrated a significantly lower VAS score (4.33±0.94) compared to the CIT (9.48±1.37) and CLIT (9.44±1.33) groups at 1 week post-treatment ( P<0.05). Additionally, the RIT group (0.62±0.23) exhibited a lower CSMS score than the CIT (1.54±0.21) and CLIT (1.06±0.22) groups at 15 weeks post-treatment ( P<0.05). Furthermore, at the point of reaching the maintenance dose, the RIT group (0.61±0.20) demonstrated superior improvement in nasal itching symptoms compared to the CIT (1.78±0.38) and CLIT groups (1.56±0.32), with P<0.05. The incidence of local adverse reactions in the RIT group (36/11.76%) was lower than that in the CIT (69/20.00%) and CLIT groups (62/16.53%), with P<0.05. Notably, none of the three groups reported grade 3/4 systemic adverse reactions, and there was no statistically significant difference in systemic adverse reactions among the three groups. Following treatment, IL-4, IL-5, IL-6, IL-17, sIgE, sIgE/tIgE, and Eos% exhibited varying degrees of decrease in all three groups, whereas IL-10, TNF, and IFN-γ did not show significant changes. Conclusions:All three distinct dose escalation regimens of SCIT demonstrated substantial clinical efficacy. Of note, the approach of combining a single injection of omalizumab with RIT significantly improved early-stage efficacy and exhibited the advantages of safety, effectiveness, and convenience, establishing it as a reliable immunotherapy method.

The clinical characteristics, laboratory results, response to treatment, and prognosis of 46 macrofocal multiple myeloma(MFMM) patients at our center from January 2013 to December 2019 were analyzed retrospectively. The other 92 patients were selected as matched-controls based on diagnostic period and treatment. Among the 1 137 MM patients, 46 patients met the definition criteria of MFMM (4.0%), with median age 56 years, which was not statistically different from whole MM population ( P=0.066). According to the international staging system (ISS) and Revised ISS, the proportion of patients with advanced stage in MFMM group was less common than that of controls ( P<0.05). More plasmacytomas in MFMM patients were presented (43.5% vs. 18.5%, P<0.05). Regarding cytogenetic abnormalities, there were minor patients manifesting high-risk features in MFMM group (15.8% vs. 32.2%, P=0.058). Translocation(11;14) could be detected in 32.4% MFMM patients and 9.4% typical myeloma patients ( P<0.05). The treatment regimens were comparable. As to the best response of treatment, the complete response (CR) rate in MFMM group was significantly higher than that of controls (78.3% vs. 60.9%, P<0.05). The median follow-up time was 37.9 months. The median progression-free survival in MFMM and control groups were 77.5 vs. 39.8 months, respectively ( P<0.05). The overall survival (OS) of MFMM patients was significantly longer (not reached vs. 68.2 months, P<0.05).

Objective:To study the epidemic status about genotype of the astrovirus, and to provide the epidemic data and control epidemic of infectious disease.Methods:Screening for astrovirus positive nucleic acids was performed using an astrovirus real-time fluorescent PCR kit. RT-PCR amplification was performed with astrovirus-specific primers Mn289/270. The positive products were recovered and purified and directly sequenced. Sequence analysis and phylogenetic analysis of astrovirus sequences were performed using Clustal and MEGA3.0 biological software.Results:From 2016 to 2018, we collected 496 fecal specimens from children with diarrhea from different regions, of them 136 cases had viral diarrhea, and in 17 of them astrovirus (3.4%) was detected, there were more males than females. All the 17 cases were under 5 years of age, and 12 cases (70.58%) were younger than 1 year old. In regional distribution, samples from Xining accounted for 64.70% (11/17), Huangzhong county for 29.41% (5/17), Huangnan Prefecture for 5.88%(1/17). The positive cases were mainly found in April to June, and there was another peak from October to December, which was consistent with the time distribution of rotavirus in past years. Seven strains were HAstV-1, one strain was HAstV-2, and the homology among four strains of HAstV-1 was 99.0%-100%. The homology with the other two strains of the same type was 88.4-95.7%.Conclusions:The dominant genotype of Astrovirus was HAstV-1 in infants with diarrhea in Qinghai, meanwhile there was also HAstV-2.

Objective:To investigate the clinical characteristics, responses, and prognosis of immunoglobulin M multiple myeloma (IgM MM) .Methods:The clinical characteristics, laboratory results, bone marrow biopsy results, response, and prognosis of six cases of IgM MM in the Blood Diseases Hospital, Chinese Academy of Medical Sciences, from December 18, 2009 to October 29, 2020 were collected and analyzed.Results:All six cases met the diagnosis criteria of IgM MM. There were four males and two females. The median age at first diagnosis was 70 (59-81) years. According to Durie-Salmon (DS) staging, 2 cases were in ⅠA, and 4 cases were in ⅢA. According to the International Staging System (ISS) , 4 cases were in Ⅱ, and 2 cases were in Ⅲ. The initial symptoms were as follows: 4 cases of bone pain, 3 cases of hyperviscosity, and 2 cases of lymphadenopathy or hepatosplenomegaly. Laboratory results showed the following: median blood M protein: 39.11 (3.61-75.56) g/L; median serum IgM: 69.35 (4.35-137.00) g/L; median hemoglobin: 87.0 (70-131) g/L; median blood creatinine: 83.6 (53.0-129.6) μmol/L; median blood calcium: 2.12 (2.11-2.50) mmol/L. The median ratio of bone marrow plasma cells was 0.390 (0.255-0.590) , and in four cases, plasma cells were observed in blood smears. Karyotype analysis and fluorescence in situ hybridization (FISH) examination showed the following: 1 case of hypodiploidy, 2 cases of P53 gene deletion, 1 case of 1q21 amplification positive, and 4 cases of RB-1 gene deletion positive. The immunoglobulin heavy chain (IgH) rearrangement was positive in all cases, of which 3 cases were CCND1/IgH fusion gene-positive identified with t (11;14) rearrangement. Immunophenotyping revealed that all cases were positive for CD38, CD138, and monoclonal light chain and four cases were weakly positive for CD20. All cases accepted proteasome inhibitor-based regimens and attained the response of partial remission to strict complete remission.Conclusion:In addition to the typical clinical manifestations of myeloma, IgM MM is also characterized by hyperviscosity, lymphadenopathy, or hepatosplenomegaly, and t (11;14) is the most frequent cytogenetics aberration. Furthermore, the response and prognosis of IgM MM are similar to other common myeloma subtypes.

Objective:To compare the safety and therapeutic effect of bridging therapy versus direct endovascular treatment in patients with acute ischemic stroke(AIS)aged 80 years and over, who received the therapy within 4.5 h of onset.Methods:A total of 89 AIS patients aged 80 years and over receiving the endovascular therapy at our hospital from January 2016 to June 2019 were studied with versus without intravenous thrombolysis before endovascular therapy(the former as bridging therapy group, n=49; the latter as the direct endovascular treatment group, n=40). Baseline information including gender, the modified Rankin scale(mRS)score, medical history, smoking history, preoperative national institute of health stroke scale(NIHSS)score were collected.Clinical data related to the operation including the times from onset to hospital, door-to-puncture and door-to-recanalization, complications(symptomatic cerebral hemorrhage, mortality)and mRS at 90 d after treatment were compared between the two groups.Multiple logistic regression analysis was used to determine whether or not bridging therapy with intravenous thrombolysis was a prognostic factor.Results:There was no significant difference in baseline information between the two groups( P>0.05). The times from onset to hospital, door-to-puncture, door-to-recanalization had no significant difference between the two groups( P>0.05). There was no significant difference in the incidence of symptomatic cerebral hemorrhage and mortality within 90 d between the two groups(26.5% or 13 cases vs. 17.5% or 7 cases, 14.3% or 7 cases vs.7.5% or 3 cases, χ2=1.031 and 1.017, P=0.310 and 0.313). With different clinical outcomes as dependent variables, after adjusting factors such as gender, admission NIHSS and medical history, Logistic regression analysis showed that the bridging therapy with intravenous thrombolysis was not a prognostic factor( OR=0.795, 95% CI: 0.280~2.258, P=0.666). Conclusions:The bridging therapy is as safe and effective as the direct intravascular therapy for AIS patients aged 80 and over within 4.5 hours of onset.The intravenous thrombolysis should be given as soon as possible within time window.