Objective To investigate the overall and subgroup efficacy,subgroup differences,predictors of efficacy and safety of roxadustat in the treatment of refractory non-severe aplastic anemia(NSAA)and low-risk myelodysplastic syndromes(LR-MDS).Methods Patients with refractory NSAA and LR-MDS who were admitted to the Department of Hematology,Peking Union Medical College Hospital from August 2020 to December 2022 were enrolled.All patients received first-line standard treatment,including recombinant human erythropoietin(rhEPO)for at least 3 months before roxadustat treatment.All patients received roxadustat 2.5 mg/kg every other day for at least 3 months,and were followed up for at least 8 months.The clinical characteristics of patients,roxadustat efficacy,predictors of efficacy,adverse effects,relapse and disease clonal evolution were analyzed.Results A total of 40 patients including 24 refractory NSAA and 16 LR-MDS were included.median age was 56(18～81)years and 40%were males.65%of the patients were transfusion dependent.Median follow-up was 21(9～34)months.22.5%,25.0%,47.5%,55.0%,57.5%,60.0%and 50.0%of the patients achieved haemato-logical improvement-erythroid(HI-E)at months 1,2,3,4,5,6,and end of the follow-up period,respectively,and no factors affecting HI-E were identified.The hemoglobin change from baseline was statistically different between the two groups at the end of the follow-up period.50%of patients were relieved from transfusion dependence.Adverse reactions were reported in 22.5%of patients.28.5%of patients relapsed after achieving HI-E,with a median time to relapse of 7(4～12)months.No clonal evolution was observed at the end of the follow-up period.Conclusions Our preliminary findings suggested that Roxadustat may be effective for patients with NSAA or LR-MDS refractory to conventional therapies and rhEPO,with mild adverse effects and low relapse rate.The degree of hemoglobin improvement may be better in the refractory NSAA patients.

The chloroplast genome encodes many key proteins involved in photosynthesis and other metabolic processes, and metabolites synthesized in chloroplasts are essential for normal plant growth and development. Root-UVB (ultraviolet radiation B)-sensitive (RUS) family proteins composed of highly conserved DUF647 domain belong to chloroplast proteins. They play an important role in the regulation of various life activities such as plant morphogenesis, material transport and energy metabolism. This article summarizes the recent advances of the RUS family proteins in the growth and development of plants such as embryonic development, photomorphological construction, VB6 homeostasis, auxin transport and anther development, with the aim to facilitate further study of its molecular regulation mechanism in plant growth and development.
Female ; Pregnancy ; Humans ; Ultraviolet Rays ; Biological Transport ; Chloroplasts/genetics* ; Embryonic Development ; Plant Development/genetics*

Objective:To evaluate the efficacy and safety of eculizumab in the treatment of paroxysmal nocturnal hemoglobinuria (PNH) in China.Methods:Data from PNH patients who received at least 3 months of full-dose eculizumab and were followed for at least 3 months between December 2022 and July 2023 were retrospectively collected. We evaluated changes in clinical and laboratory parameters after 1, 2, 3, and 6 months of eculizumab treatment. The rates of breakthrough hemolysis (BTH), extravascular hemolysis (EVH), and the occurrence of adverse reactions were also monitored.Results:The study included nine patients, six males and three females, with a median age of 54 (28–69) years. 5 of the patients had classic PNH, while 4 had PNH/AA. The number of episodes of hemoglobinuria was 5 (1–25) per month before eculizumab. 4 patients required blood transfusion, 5 had thrombosis and one had renal impairment before eculizumab. The median time to eculizumab was 6 (3–7) months and the followup period was 3 (3–6) months after treatment. The number of episodes of hemoglobinuria following eculizumab was 0 (0–1). During the followup period, no additional thrombotic events occurred. LDH at any time after eculizumab was lower than at baseline, and some patients' HGB increased. All transfused patients became transfusion-independent after receiving eculizumab. The FACIT-Fatigue score improved by an average of 17.3 points following treatment. 2 patients developed BTH and improved with symptomatic treatment. There were three adverse events that caused mild symptoms. There are no serious adverse events or deaths.Conclusion:Eculizumab can effectively control the hemolytic-related symptoms of PNH in China, reducing the need for blood transfusions to some extent, while also demonstrating a higher safety profile.

Objective:To evaluate the efficacy and safety of roxadustat in patients with refractory non-severe aplastic anemia (NSAA) .Methods:The clinical data of patients with refractory NSAA who had been treated with roxadustat continuously for at least 3 months and followed up for more than 6 months at Peking Union Medical College Hospital from October 2020 to August 2022 were retrospectively collected. The demographic information, clinical data, treatment efficacy, adverse reactions, and outcomes were evaluated, and the factors influencing efficacy were analyzed.Results:A total of 41 patients were included. The male-to-female ratio was 16∶25, and the median age was 52 (18-84) years. The median duration of roxadustat treatment was 5 (3-20) months, and the median follow-up was 15 (6-26) months. Hematologic improvement-erythroid (HI-E) was 12.2%, 29.3%, 46.3%, 43.9%, and 30.3% at 1, 2, 3, 6, and 12 months, respectively. The rate of transfusion independence was 28.5%, 38.1%, and 33.3% at 3, 6, and 12 months, respectively. Hemoglobin returned to normal in some patients after treatment with roxadustat. The incidence of adverse events was 22%, all of which were grade Ⅰ-Ⅱ and recoverable. No factors that could affect HI-E were identified. By the end of follow-up, 45% of the patients relapsed, with a median time to relapse of 7 (3-12) months. No clonal evolution was observed, and one patient died.Conclusion:Roxadustat effectively improved anemia with good tolerance in patients with refractory NSAA.

Objective To investigate the expression of long non-coding RNA(LncRNA)nuclera-enriched autosomal transcript(NEAT1)in children with retinoblastoma(Rb)and the effect of down-regulation of NEAT1 in Rb cell Y79 on cell biological function.Methods A total of 83 children with Rb who were diagnosed and treated in Huangshi Central Hospital from March 2015 to March 2021 were collected as the research object.During the same period,50 healthy children(control group)were selected in the children's health center.Quantitative real-time polymerase chain reaction(qRT-PCR)was used to detect the expression of NEAT1 in serum.The differences in the expressions of NEAT1 in serum between Rb children and the control group,and the differences in the expressions of NEAT1 in serum among Rb children with different clinical indicators,were analyzed.Y79 cells were cultured and were divided into si-NEAT1 group(transfected with interference sequence of NEAT1),si-NC group(transfected with control sequence)and Ctl group(only add transfection reagent).The qRT-PCR,MTT,flow cytometry and Transwell were used to detect the NEAT1 expression,cell proliferation,apoptosis,migration and invasion.Results The expression level of NEAT1 in the serum of children with Rb(1.43±0.28)was higher than that in the control group(1.01±0.21),with significant difference(t=9.116,P＜0.001).The expression levels of NEAT1 in serum of children with Rb with Intraocular International Retinoblastoma classification(IIRC)stage CDE,poor differentiation,optic nerve infiltration and lymph node metastasis were higher than those in children with Rb with AB,medium to high differentiation,no optic nerve infiltration and lymph node metastasis,with significant differences(t=2.190～3.693,all P＜0.05).The area under the curve for diagnosing Rb based on NEAT1 expression in serum was 0.882(95％CI:0.826～0.937).When the expression level of NEAT1 was 1.20,the sensitivity and specificity were 80.00％and 79.52％,respectively.Compared with the si-NC group(1.03±0.09)and the Ctl group(1.02±0.15),the expression level of NEAT1 in the si-NEAT1 group(0.35±0.06)was decreased,with significant differences(t=14.829,9.994,all P＜0.001).The absorbance A values in the si-NEAT1 group at 24,48,72 and 96h were significantly lower than those in the si-NC group and the Ctl group(t=si-NC=2.796～4.362,tCtl=2.641～5.555,all P＜0.05),while the apoptosis rate in the si-NEAT1 group was significantly higher than those in the si-NC group and the Ctl group,and the differences were statistically significant(t=4.999,3.915,all P＜0.05).Compared with the si-NC group and the Ctl group,the number of migrating cells(116.50±9.35 vs 132.00±7.32,134.00±7.95)and the number of invasive cells(96.33±8.94 vs 117.67±12.39,119.17±10.05)in the si-NEAT1 group were reduced,and the differences were statistically significant(tsi-NC=3.196,3.421,tCtl=3.492,4.159,all P＜0.05).Conclusion The expression level of NEAT1 in the serum of children with Rb was elevated,which may have a certain diagnostic value for children with Rb.Silencing the expression of NEAT1 in Y79 cells could reduce cell proliferation,accelerate cell apoptosis,and inhibit cell migration and invasion.

Periarticular fracture of the shoulder is a common type of fractures in the elderly. Postoperative adverse events such as internal fixation failure, humeral head ischemic necrosis and upper limb dysfunction occur frequently, which seriously endangers the exercise and health of the elderly. Compared with the fracture with normal bone mass, the osteoporotic periarticular fracture of the shoulder is complicated with slow healing and poor rehabilitation, so the clinical management becomes more difficult. At present, there is no targeted guideline or consensus for this type of fracture in China. In such context, experts from Youth Osteoporosis Group of Chinese Orthopedic Association, Orthopedic Expert Committee of Geriatrics Branch of Chinese Association of Gerontology and Geriatrics, Osteoporosis Group of Youth Committee of Chinese Association of Orthopedic Surgeons and Osteoporosis Committee of Shanghai Association of Chinese Integrative Medicine developed the Chinese expert consensus on the diagnosis and treatment of osteoporotic periarticular fracture of the shoulder in the elderly ( version 2023). Nine recommendations were put forward from the aspects of diagnosis, treatment strategies and rehabilitation of osteoporotic periarticular fracture of the shoulder, hoping to promote the standardized, systematic and personalized diagnosis and treatment concept and improve functional outcomes and quality of life in elderly patients with osteoporotic periarticular fracture of the shoulder.

Objective:To investigate the pathogenesis and clinical efficacy of arthroscopic treatment for hallux ganglion cyst deriving from ankle joint.Methods:The clinical data of 21 patients with ankle arthroscopic in the Department of Hand and Foot Surgery,Affiliated Hospital of Jining Medical College from January 2019 to March 2021 were analyzed retrospectively.There were 15 male cases and 6 female cases,aged (52.6±8.2) years (range:42 to 70 years).There were 9 cases of primary operation and 12 cases of recurrence after operation in other hospital.All the patients were examined by ankle arthrography and MRI before operation.The synovial membrane of the ankle was debrided and the tendon sheath of flexor longus was removed at the ankle canal.One year after operation,MRI was performed,and the American Orthopedic Foot and Ankle Society(AOFAS) score of forefoot function and visual analogue scale (VAS) before and after operation were compared by the paired t test or Mann-Whitney U test.The postoperative complications and recurrence were recorded. Results:All patients were operated successfully.The joint capsule at the back of the ankle joint of the patients were ruptured and communicated with the tendon sheath of the flexor longus tendon at the ankle canal.No wound infection,vascular and nerve injury occurred.The follow-up period was (15.0±2.2) months (range:12 to 18 months).During the follow-up period,there was no recurrence of toe appearance and MRI.At the last follow-up,the AOFAS score (90.8±4.3) was significantly higher than that before operation (72.8±6.3) ( t=-10.810, P<0.01),and the VAS score( M(IQR)) was significantly lower than that before operation,the difference was significant (1.0(1.0) vs. 3.0(0.5), Z=-4.081, P<0.01). Conclusions:The possible mechanism of hallux ganglion cyst deriving from ankle joint is that the joint capsule at the back of the ankle joint ruptures and communicates with the tendon sheath of the flexor longus tendon at the ankle canal,and the intra-articular synovial fluid through the cylinder effect generated by sliding with the flexor tendon of the flexor longus tendon in the tendon sheath sac leads to the heel valange cyst.Ankle-synovial cleansing of the ankle joint under ankle arthroscopy and resection of the flexor tendon sheath of the flexor longus tendon at the ankle canal are effective and less invasive.

Objective:To investigate the pathogenesis and clinical efficacy of arthroscopic treatment for hallux ganglion cyst deriving from ankle joint.Methods:The clinical data of 21 patients with ankle arthroscopic in the Department of Hand and Foot Surgery,Affiliated Hospital of Jining Medical College from January 2019 to March 2021 were analyzed retrospectively.There were 15 male cases and 6 female cases,aged (52.6±8.2) years (range:42 to 70 years).There were 9 cases of primary operation and 12 cases of recurrence after operation in other hospital.All the patients were examined by ankle arthrography and MRI before operation.The synovial membrane of the ankle was debrided and the tendon sheath of flexor longus was removed at the ankle canal.One year after operation,MRI was performed,and the American Orthopedic Foot and Ankle Society(AOFAS) score of forefoot function and visual analogue scale (VAS) before and after operation were compared by the paired t test or Mann-Whitney U test.The postoperative complications and recurrence were recorded. Results:All patients were operated successfully.The joint capsule at the back of the ankle joint of the patients were ruptured and communicated with the tendon sheath of the flexor longus tendon at the ankle canal.No wound infection,vascular and nerve injury occurred.The follow-up period was (15.0±2.2) months (range:12 to 18 months).During the follow-up period,there was no recurrence of toe appearance and MRI.At the last follow-up,the AOFAS score (90.8±4.3) was significantly higher than that before operation (72.8±6.3) ( t=-10.810, P<0.01),and the VAS score( M(IQR)) was significantly lower than that before operation,the difference was significant (1.0(1.0) vs. 3.0(0.5), Z=-4.081, P<0.01). Conclusions:The possible mechanism of hallux ganglion cyst deriving from ankle joint is that the joint capsule at the back of the ankle joint ruptures and communicates with the tendon sheath of the flexor longus tendon at the ankle canal,and the intra-articular synovial fluid through the cylinder effect generated by sliding with the flexor tendon of the flexor longus tendon in the tendon sheath sac leads to the heel valange cyst.Ankle-synovial cleansing of the ankle joint under ankle arthroscopy and resection of the flexor tendon sheath of the flexor longus tendon at the ankle canal are effective and less invasive.

Transcription factors, the proteins with special structures, can bind to specific sites and regulate specific expression of target genes. NAC (NAM, ATAF1/2, CUC1/2) transcription factors, unique to plants, are composed of a conserved N-terminal domain and a highly variable C-terminal transcriptional activation domain. NAC transcription factors are involved in plant growth and development, responses to biotic and abiotic stresses and other processes, playing a regulatory role in flower development. In this paper, we reviewed the studies about NAC transcription factors in terms of discovery, structure, and regulatory roles in anther development, other floral organ development and flowering time. This review will provide a theoretical basis for deciphering the regulatory mechanism and improving the regulatory network of NAC transcription factors in flower development.
Flowers/genetics* ; Gene Expression Regulation, Plant ; Phylogeny ; Plant Proteins/metabolism* ; Plants/metabolism* ; Transcription Factors/metabolism*

Objective:Comparative analysis of total parathyroidectomy (total parathyroidectomy, TPTX) and total parathyroidectomy with autoransplantation (total parathyroidectomy with autoransplantation, TPTX+ AT) for the treatment of uremia secondary hyperparathyroidism Clinical efficacy.Methods:A total of 82 cases of SHPT treated with TPTX or TPTX + AT in First Affiliated Hospital of Chengdu Medical College from June 2015 to July 2019 were collected According to the operation methods, 82 cases were devided into TPTX group and TPTX+ AT group.There were 34 patients in the TPTX group, 17 males and 17 females, with an average age of (45.97±8.96) years, and 48 patients in the TPTX+ AT group, 21 males and 27 females, with an average age of (49.12±7.30) years. SPSS 20.0 software was used for statistical analysis. The measurement data conforming to normal distribution were represented by ( Mean± SD), and the comparison between groups was performed by t test. The data not conforming to normal distribution were represented by quaternary M( P25, P75), and the comparison between groups was performed by non-parametric test. Enumeration data were expressed as cases (%), and comparison was performed by χ2 test. The postoperative complications, postoperative efficacy and recurrence rate were compared between the two procedures. Results:Both TPTX and TPTX+ AT had a low incidence of complications after SHPT, and both of them could effectively relieve symptoms such as bone pain, skin itching and subcutaneous soft tissue calcification.After 1 year of follow-up, both of the two surgical methods can effectively reduce blood calcium (TPTX group: t= 14.522, P<0.05; TPTX+ AT group: t=13.704, P<0.05), blood phosphorus (TPTX group: t=35.022, P<0.05; TPTX+ AT group: t=42.303, P<0.05) and iPTH(TPTX group: t= 20.166, P<0.05; TPTX+ AT group: t= 23.976, P<0.05), but the reduction of iPTH in TPTX group was more effective than that in TPTX+ AT group ( t=-3.416, P<0.05). Conclusion:Both TPTX and TPTX+ AT can effectively treat SHPT, and have high safety and can be used as an alternative for SHPT treatment.