[Optic atrophy in a patient with axonal Charcot-Marie-Tooth disease 2A2A due to MFN2 gene mutations].

A 27-year-old male patient had progressive vision loss in both eyes, which was mainly manifested by impaired ganglion cells in the macular area, accompanied by systemic muscle atrophy in limbs. A complete mitochondrial exon gene detection was performed. The final diagnosis was bilateral optic atrophy and axonal Charcot-Marie-Tooth disease 2A2A caused by mutations of the MFN2 gene. There has been no effective treatment. Applications of nutrients to restore the mitochondrial function may alleviate the clinical symptoms.

[Update on B cell depletion therapy for demyelinating optic neuritis with disease-specific antibody].

Demyelinating optic neuritis coexisting with seropositive aquaporin-4 antibody or myelin oligodendrocyte glycoprotein antibody is different from the typical optic neuritis in immunopathological, clinical and radiological features, as well as therapeutic response. Due to the high recurrence rate and the potential risk of blindness, immunosuppressive therapy is warranted in most cases. The current review tracks and summarizes both the update and the potential controversies of B cell depletion therapy with a special focus on rituximab for demyelinating optic neuritis to optimize clinical decision-making.

[Myelin oligodendrocyte glycoprotein antibody positive optic neuritis with anti-N-methyl-D-aspartate receptor encephalitis: a case report].

A 6-year-old girl had binocular vision loss with pain for one week. The patient presented with symptoms such as non-communication, language deterioration, dysphonia, and choking when drinking and eating during the course. The serum myelin oligodendrocyte glycoprotein antibody was positive. Both the serum and cerebrospinal fluid anti-N-methyl-D-aspartate receptor antibody were also positive. The diagnoses were myelin oligodendrocyte glycoprotein antibody positive optic neuritis and anti-N-methyl-D-aspartate receptor encephalitis. High-dose intravenous glucocorticoids were given. Recurrence was not observed during the 15-month clinical follow-up.

[Research progress of ophthalmic surgery robots].

Ophthalmic surgery has the characteristics of high precision, small operating range, need of microscopic vision, and sensitive feedback, which put forward special requirements for surgical robots. The da Vinci robot, intraocular robotic interventional surgical system, hand-held micromanipulator, and co-manipulation robot have been developed and applied in various ophthalmic surgeries. These surgical robots have shown the advantages of improving the operation accuracy, stability, and dexterity in the ocular surface, cataract, and retinal surgeries.

A nomogram integrating ferroptosis-and immune-related biomarker for prediction of prognosis and diagnosis in kidney renal clear cell carcinoma.

OBJECTIVE: Approximately 60% of patients with kidney renal clear cell carcinoma (KIRC) die within the first 2-3 years. The prognosis for patients with KIRC and its metastases is poor. Ferroptosis and providing immunity are novel treatment targets for several cancers, including KIRC. Therefore, it is important to identify suitable ferroptosis- and immune-related signatures to predict the prognosis and diagnosis of patients with KIRC. MATERIALS AND METHODS: The corresponding data of patients with KIRC were obtained from the Cancer Genome Atlas. Univariate and multivariate Cox regression analyses were used to screen candidate biomarkers in patients with KIRC. RESULTS: We found that four FI-DEGs (BID, MET, LTB4R, and HMOX1) were independently associated with the overall survival of patients with KIRC. The prognosis and diagnosis model constructed using these four biomarkers could predict the outcome of KIRC, as measured by the receiver operating characteristic analyses. CONCLUSIONS: We identified 4 FI-DEGs that could be used as biomarkers in patients with KIRC. The present study not only contributes to understanding the roles of ferroptosis and immunity in the development of KIRC, but also to the diagnosis and prognosis of KIRC, although it remains to be further studied.

[Chlorpromazine hydrochloride plays a tumor suppressive role in diffuse large B lymphoma by promoting the expression of S1PR2].

Objective: To investigate the effect of chlorpromazine hydrochloride on proliferation, apoptosis and cell cycle arrest of human diffuse large B lymphoma cells and its possible mechanism. Methods: In January 2019, OCI-LY3 and TMD8 cells were treated with different concentrations of chlorpromazine hydrochloride for 72 h, the proliferations were detected by Alamar Blue assay. The apoptosis and cell cycle arrest of OCI-LY3 and TMD8 cells were detected by flow cytometry with FITC annexin V/PI (propidium iodide) double staining and PI, respectively. The mRNA levels of cyclin-dependent kinase inhibitor P21, P27, CyclinD1 and S1PR2 were detected by RTqPCR. The protein levels of P21, P27 and S1PR2 were detected by Western blot. Results: With the increase of chlorpromazine hydrochloride concentration, the proliferation inhibition rates of OCI-LY3 and TMD8 cells increased at 72 hours, apoptosis and G1 cell cycle arrest increased at 24 hours. Compared with the control group, the expression levels of P21, P27 and SIPR2 mRNA in OCI-LY3 and TMD8 cells were increased at 10 µmol/L chlorpromazine after 12 hours treatment (P<0.05) . And there was no significant difference in the expression of CyclinD1 mRNA (P>0.05) . There was a similar increase in protein levels of P21, P27 and SIPR2 in OCI-LY3 and TMD8 cells after 24 hours of treatment (P<0.05) . Conclusion: Chlorpromazine hydrochloride at a specific concentration may inhibit the proliferation of ABC diffuse large B lymphoma cells by promoting the expression of S1PR2, and promote cell apoptosis and G1 phase cell cycle arrest.

[IgG4-related ophthalmic disease: three-case report].

The first patient complained visual loss of left eye for 1 week. The second patient complained visual loss of right eye for 2 months with conjunctival edema for more than 10 days. The third patient complained redness and swelling of both eyes for 1 year, poor appetite and fatigue for 4 months. All three patients were male with binocular involvement. Enlargement of lacrimal glands in both eyes with bulbar conjunctival edema were observed in 2 patients and binocular eyelid swelling in 1 patient, all 3 patients were associated with external orbital tissue involvement, which were diagnosed as IgG4-related ophthalmic diseases. After treatment with glucocorticoid, the symptoms of the patients were improved, and the patients were followed up for 58, 54, and 60 months, respectively. Among the 3 patients, 2 had recurrence for once, and 1 had recurrence 3 for times. (Chin J Ophthalmol, 2021, 57: 375-378).

[A summary of oculoplastic and orbital surgery in China in the past 70 years].

Oculoplastic and orbital surgery is concerned with the occurrence, manifestation, diagnosis, treatment and prevention of the eyelid, orbit and lacrimal diseases, as well as the repair and reconstruction of the related deformities and defects. Since the beginning of the new century, the number of patients with eyelid and orbital diseases has increased continuously, and the team of specialists has expanded rapidly. The development of oculoplastic and orbital surgery in China has entered an all-round new stage, especially with the establishment of Chinese Society of Oculoplastic Surgery and Orbital Disease in 2013. Oculoplastic and orbital surgery in China has made outstanding achievements in strengthening the training of professional talents, promoting interdisciplinary and international exchanges, improving the academic level of basic research, and emphasizing on the research of translational medicine. The technique of ocular plastic surgery has been progressing continuously. Cosmetic surgery of the eye has gained widespread popularity and standardization. Orbital surgery has recorded an innovative, interdisciplinary and rapid growth. International exchanges have helped to expand the international influence and competitiveness. This article is written to celebrate the 70th anniversary of Chinese Journal of Ophthalmology. (Chin J Ophthalmol, 2020, 56: 805-810).

Hsa-circ-0068566 inhibited the development of myocardial ischemia reperfusion injury by regulating hsa-miR-6322/PARP2 signal pathway.

OBJECTIVE: In recent years, studies have shown that noncoding RNA (circRNA) is an important regulatory molecule involved in cell physiology and pathology. Herein, we analyzed the role of circRNA-68566 in the regulation of myocardial ischemia-reperfusion (I/R) injury by regulating miR-6322/PARP2 signaling pathway. MATERIALS AND METHODS: Cell viability was checked by CCK-8; LDH concentration, ROS production, MDA, SOD and GSH-Px were measured by corresponding kits; QPCR was used to inspect the expression of circRNA-0068566 and miR-6322 in I/R injury and H9C2 cells; luciferase reporter assay confirmed the direct target effect of circRNA-0068566 and miR-6322; Western blot was used to investigate PARP2 protein expression in I/R injury and H9C2 cells. RESULTS: We analyzed the regulatory effect of circRNA-68566 on I/R injury and found that circRNA-68566 promoted the proliferation of injured cardiomyocytes in vitro and in vivo. circRNA-68566 and miR-6322 were directly combined to regulate the development of I/R injury. We also confirmed that PARP2 was the target of miR-6322 in I/R injury. CONCLUSIONS: We believed that circRNA-68566 participated in myocardial ischemia-reperfusion injury by regulating miR-6322/PARP2 signaling pathway, which provided a new possible strategy for the treatment of I/R injury.

[Acellular xenogenic dermal matrix as a spacer graft for treatment of eyelid retraction related to thyroid associated ophthalmopathy].

Objective: To evaluate the efficacy and safety of an acellular xenogenic dermal matrix (AXDM) as a spacer graft in the surgery to treat upper and/or lower eyelid retraction (UER/LER) in patients with thyroid associated ophthalmopathy (TAO). Methods: This was a retrospective case series study. From January 2016 to December 2017, all the TAO patients who underwent the eyelid retraction surgery with an AXDM as a spacer graft in Shanghai Ninth People's Hospital, Shanghai Jiaotong University School of Medicine were included in the study. The follow-up duration was at least 6 months. Eyelid height, ocular discomfort symptoms, and complications were recorded and used to evaluate the efficacy and safety. The treatment outcomes were divided into good, acceptable and unacceptable outcomes. Nonparametric test was used to compare the efficacy of upper and lower eyelid surgery. Results: Eighty patients (102 eyes) were included, consisting of 43 patients (56 eyes) with UER and 37 patients (46 eyes) with LER. The UER patients included 34 females (median age, 37 years) and 9 males (median age, 47 years). The LER patients included 20 females (median age, 39 years) and 17 males (median age, 40 years). At 6 months after surgery, ocular discomfort was relieved in 79.0% (64/81) eyes; photophobia was relieved in 69.5% (41/59) eyes; tearing was relieved in 94.9% (37/39) eyes; lagophthalmos was relieved in 80.7% (67/83) eyes. In patients with UER, the margin reflex distance was lowered from 8.0 (7.5, 8.0) mm to 3.5 (3.4, 4.0) mm (Z=-6.587, P<0.01). The upper scleral show was lowered from 3.0 (3.0, 3.5) mm to 0 mm (Z=-6.616, P<0.01). In patients with LER, the margin reflex distance was lowered from 8.5 (7.5, 9.0) mm to 5.5 (5.5, 6.0) mm (Z=-5.979, P<0.01). The lower scleral show was lowered from 3.0 (2.5, 3.5) mm to 0.0 (0.0, 0.5) mm (Z=-5.965, P<0.01). In total, 71.3% (57 cases) of the patients acquired good outcomes, 22.5% (18 cases) acquired acceptable outcomes, and 6.3% (5 cases) had recurrence, and no significant difference was found between the patients with UER and LER (33, 8, 2 cases vs. 24, 10, 3 cases, Z=-1.176, P>0.05). No other complications, such as infection, rejection, and dislocation, were observed. Conclusion: AXDM as a spacer graft can be a reasonable and safe alternative for eyelid retraction in TAO patients, achieving both the functional and aesthetic improvements. (Chin J Ophthalmol, 2019, 55: 821-827).

[Hypoxia increases chemotherapy resistance in nasopharyngeal carcinoma via inducing CDK6 deSUMOylation].

Objective: To understand the mechanism of chemotherapy resistance in nasopharyngeal carcinoma under hypoxic conditions through the perspective of protein SUMOylation modification. Methods: Cobalt chloride (CoCl(2)) was used to establish the hypoxic model of human nasopharyngeal carcinoma CNE1 cells. Then, the cell cycle was detected by flow cytometry, and the expression level of small ubiquitin-related modifier(SUMO) and cyclin-dependent kinase 6 (CDK6) proteins were detected by western blotting. MTT assay was used to determine the median lethal dose (IC(50)) of cancer cells against cisplatin, and enzyme-linked immunosorbent assay (ELISA) was used to determine lactate dehydrogenase (LDH) level. Results: The cell cycle of CNE1 induced by hypoxia was arrested in G0/G1 phase.The results of Western blot showed that the protein expression level of CDK6 in CNE1 cells was lower than that in the control group (0.83±0.25 vs. 0.43±0.21, t=14.67, P=0.003). The protein level of conjugated SUMO1 was significantly lower than that in the control group (2.69±0.48 vs. 1.38±0.31, t=17.22, P=0.001), while the level of free SUMO1 protein was significantly higher than that in the control group (2.01±0.43 vs. 2.60±0.59, t=15.45, P=0.002).The LC50 of CNE1 cells in the control group was significantly lower than that in the hypoxic group (29.44 µg/ml vs. 97.72 µg/ml, t=12.79, P=0.001). After CNE1 cells received 50 µg/ml cisplatin for 48 h, the LDH content in the supernatant of the control group was significantly higher than that in the hypoxic group ((541.49±64.59) ng/ml vs. (234.67±41.03) ng/ml, t=11.94, P=0.007)). The apoptosis rate of CNE1 cells in the control group was significantly higher than that in the hypoxic group ((76.64±5.37)% vs. (32.84±4.77) ng/ml, t=8.49, P=0.003)). Conclusion: Hypoxia can dissociate the covalent modification of CDK6 and SUMO1, inhibit cell cycle and increase the chemotherapy resistance of nasopharyngeal carcinoma.

[Outcomes of allogeneic hematopoietic stem cell transplantation in patients with paroxysmal nocturnal hemoglobinuria compared to paroxysmal nocturnal hemoglobinuria-aplastic anemia syndrome].

Objective: To compare the outcomes of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) with paroxysmal nocturnal hemoglobinuria-aplastic anemia (PNH-AA) syndrome. Methods: The outcomes of 46 patients who received allo-HSCT (16 PNH patients, 30 PNH-AA patients) from July 10, 2007 to June 2, 2018 were analyzed retrospectively. The conditioning regimen was busulfan, cyclophosphoramide, and ATG in haploidentical donors and unrelated donors. Patients with matched sibling donors were treated with the fludarabine, cyclophosphamide, and ATG regimen. Results: There were no differences of baseline data between the 2 groups except gender distribution and the numbers of haploidentical donor transplantation. The median values of absolute nucleated cell counts were 10.58 (3.83-13.83) ×10(8)/kg in the PNH group and 10.81 (3.96-33.40) ×10(8)/kg in the PNH-AA group (P=0.668) . The median doses of CD34(+) cells infused were 5.00 (3.14-8.42) ×10(6)/kg and 3.57 (1.97-6.17) ×10(6)/kg (P=0.002) , respectively. All patients obtained complete engraftment. The median time for myeloid engraftment were 11 (7-14) days in the PNH group and 12 (10-26) days in the PNH-AA group (P=0.003) . The median time for platelet engraftment were 13 (11-16) days and 18 (12-75) days (P=0.002) , respectively, after a median follow-up of 36 (4-132) months in the PNH group and 26 (4-75) months in the PNH-AA group (P=0.428) . There were no differences of incidence rates of acute graft-versus-host disease (aGVHD) , chronic GVHD and infection between PNH and PNH-AA groups (P>0.05) . No patient occurred early death and relapse. The estimated 3-year overall survival (OS) of PNH and PNH-AA groups were (100.0±0.0) % and (85.7± 6.6) % (P=0.141) , GVHD-free and failure-free survival (GFFS) were (100.0±0.0) %, (78.7±7.7) % (P=0.067) . Conclusions: allo-HSCT is effective for patients with PNH and PNH-AA syndrome. The preliminary results indicate that myeloid and platelet engraftment in PNH group were faster than PNH-AA group. There were no differences in OS and GFFS between PNH group and PNH-AA group.

[Anxiety and depression in patients with idiopathic tinnitus and its relative factors analysis].

Objective: To investigate the psychological status of anxiety and depression in patients with idiopathic tinnitus, and to analyze its relative factors. Method: One hundred and sixty seven patients with idiopathic tinnitus were assessed by Self-Rating Anxiety Scale(SAS),Self-Rating Depression Scale(SDS) and general condition questionnaire. Patients with anxiety or depression were reassessed after corresponding treatment for 6 weeks. Result: Fifty-five patients(32.93%) were in anxiety state and 43 patients(25.75%) were in depression state. Twenty-seven patients(16.17%) were in both anxiety and depression state. The multivariate logistic regression analysis found that the incidence of anxiety and depression in patients with idiopathic tinnitus were related to sleep disorders, course of disease and THI score(P<0.05). Treatment scores(SAS and SDS) were statistically significantly different between pre- and post-treatment(P<0.01). Conclusion: The anxiety and depression state is highly prevalent in idiopathic tinnitus patients and is related to course of disease, severity of tinnitus and sleep disorders. In clinical practice, we should pay close attention to the psychological status of patients with idiopathic tinnitus, and take timely psychological intervention.

[Comparison of haploidentical hematopoietic stem cell transplantation and matched-sibling donor transplantation for the treatment of paroxysmal nocturnal hemoglobinuria].

Objective: To compare the outcomes between haploidentical donor hematopoietic stem cell transplantation (haplo-HSCT) and matched-sibling donor transplantation (MSD-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) . Methods: The clinical data of 40 PNH patients received HSCT (haplo-HSCT=25, MSD-HSCT=15) from July 2007 to May 2018 were analyzed retrospectively to compare the outcomes between haplo-HSCT and MSD-HSCT groups. Results: There were no differences in terms of gender, age, patients of PNH-AA and median time from diagnosis to transplantation between the 2 groups (P>0.05) . The median values of absolute mononuclear cell counts and CD34+ cells infused were 10.74 (4.80-22.86) ×108/kg and 12.19 (5.14-17.25) ×108/kg (P=0.866) , 3.57 (0.68-7.80) ×106/kg and 4.00 (3.02-8.42) ×106/kg (P=0.151) respectively, in haplo-HSCT and MSD-HSCT groups. All patients attained complete engraftment, no patient occurred graft failure. The median durations for myeloid and platelet engraftment were 12 (range, 9-26) and 11 (range, 7-15) days (P=0.065) , 19 (range, 11-75) and 13 (range, 11-25) days (P=0.027) respectively, in haplo-HSCT and MSD-HSCT groups. During a median follow-up of 26 (4-65) months in haplo-HSCT and 36 (4-132) months in MSD-HSCT groups (P=0.294) , the incidences of grade Ⅰ-Ⅳ acute graft-versus-host disease (aGVHD) were 32.0% and 20.0% (P=0.343) , grade Ⅱ-Ⅳ aGVHD were 16.0%, 13.3% (P=0.759) , chronic GVHD were 30.7% and 24.6% (P=0.418) , moderate-severe chronic GVHD were 12.7% and 7.1% (P=0.522) respectively, in haplo-HSCT and MSD-HSCT groups. The incidences of infection were 32.0% (8/25) and 26.7% (4/15) (P=1.000) respectively, in haplo-HSCT and MSD-HSCT groups. No patients occurred early death and relapse. Three-year estimated overall survival (OS) were (86.5±7.3) % and (93.3 ±6.4) % (P=0.520) , GVHD-free and failure-free survival (GFFS) were (78.3±8.6) % and (92.9±6.9) % (P=0.250) respectively, in haplo-HSCT and MSD-HSCT groups. Conclusion: The preliminary results indicated that haplo-HSCT was a feasible choice for PNH with favorable outcomes, haplo-HSCT and MSD-HSCT produced similar therapeutic efficacy.

[Spectomycin B1 induces VEGFR2 de-SUMO modification to inhibit angiogenesis in nasopharyngeal carcinoma].

Objective:To explore the new mechanism of spectomycin B1 in inhibiting angiogenesis of nasopharyngeal carcinoma and to provide a theoretical basis for targeted gene therapy of nasopharyngeal carcinoma. Method:Human nasopharyngeal carcinoma CNE1 cells were divided into two groups, the control group and spectomycin B1 group. Western blot was used to detect the expression levels of small ubiquitin-related modified protein（SUMO） 1 and vascular endothelial growth factor receptor 2（VEGFR2）. The angiogenesis assay was used to detect the angiogenic ability of CNE1 cells, and the apoptosis was detected by flow cytometry. The model of nasopharyngeal carcinoma-bearing mice was established, spectomycin B1 was administered, tumor volume and weight were measured, and protein expression of CD31 was detected by immunohistochemistry and microvessel density was compared. Result:Spectomycin B1 could reduce deSUMOylation of VEGFR2 protein by 4.05 times, significantly reduce the angiogenic ability of CNE1 cells, and increase the apoptosis rate by 20.68%. In the tumor-bearing mouse model, spectomycin B1 treatment could inhibit subcutaneous tumor growth rate and weight, and the blood vessel density decreased by 40.04%. Conclusion:Spectomycin B1 can inhibit neovascularization of nasopharyngeal carcinoma by inducing deSUMOylation of VEGFR2 protein.

A novel biomarker of serum Histidine-Rich Glycoprotein (HRG) for diagnosing and predicting prognosis of ventilator-associated pneumonia (VAP): a pilot study.

OBJECTIVE: Histidine-Rich Glycoprotein (HRG) has been reported to be associated with idiopathic pulmonary fibrosis, cancer, and sepsis as a novel biomarker. However, there is limited evidence regarding its value in diagnosing or prognosis evaluating of ventilator-associated pneumonia (VAP). PATIENTS AND METHODS: A total of 186 patients intubated in ICU and 65 healthy volunteers were enrolled in this study. Patients were divided into VAP group (n = 116), non-VAP group (n = 70) and control group (n = 65). The HRG, C reactive protein (CRP) and procalcitonin (PCT) levels were measured 72 hours after intubation, while blood sample was acquired from healthy controls for the test. RESULTS: HRG of VAP group was significantly lower than non-VAP group and control group (p < 0.001), while CRP and PCT were significantly higher (p < 0.001). The ROC analysis showed that the AUC of HRG was 0.777 95% CI (0.708-0.847) with a cut-off value of 38.55 µg/mL, which was lower than CRP [AUC = 0.912, 95% CI (0.847-0.950)] and PCT [AUC = 0.818, 95% CI (0.759-0.876)]. No linear correlation was found between HRG and CRP, as well as PCT (p > 0.05). However, the survival analysis showed that patients with higher level of HRG had a significantly higher survival rate (p < 0.001). The multivariate Cox regression analysis also demonstrated that the higher level of HRG was associated with better survival [HR 0.290, 95% CI (0.131-0.641), p = 0.002]. CONCLUSIONS: Serum HRG decreases when the patient develops VAP, which might be used as a biomarker for the diagnosis of VAP, with relatively less accuracy than PCT and CRP. However, HRG is valuable in predicting the clinical outcomes of mechanical ventilation patients.

[Outcome of combination of HLA-haploidentical hematopoietic SCT with an unrelated cord blood unit for 127 patients with acquired severe aplastic anemia].

Objective: To evaluate the outcome of combination of haploidentical donor (HID) hematopoietic stem cell transplantation (HSCT) with an unrelated cord blood unit for severe aplastic anemia (SAA). Methods: The clinical data of 127 SAA patients [including 74 male and 53 female patients, 65 very severe aplastic anemia (vSAA), the median age as 23.5(3-54) years] received HID-HSCT from September 2011 to April 2017 were analyzed retrospectively. The median interval from SAA diagnosis to transplantation was 2 (0.5-180) months. The conditioning was modified Bu/Cy+ATG/ALG-based (Busulfan + cyclophosphamide + antithymocyte immunoglobulin/antilymphocyte immunoglobulin) regimen. Cord blood units were selected based on the results of HLA typing and cell doses evaluated before freezing. Units with at least 4/6 matched HLA loci became the candidates. Prophylaxis for graft-versus host disease (GVHD) was by cyclosporine (CsA), mycophenolate mofetil (MMF) plus short-term methotrexate (MTX). Results: The median values of absolute nucleated cell counts were 10.87 (3.61-24.00)×10(8)/kg in the haploidentical grafts and 2.22 (1.10-7.30)×10(7)/kg in the cord blood units, respectively. The median doses of CD34(+) cells infused were 3.49(1.02-8.89) ×10(6)/kg in the haploidentical grafts and 0.56 (0.16-2.27) ×10(5)/kg in the cord blood units, respectively. Of the 127 patients, 5 patients occurred early death, one patient occurred primary graft failure. All 121 surviving patients attained complete haploidentical engraftment. The median durations of myeloid engraftment were 11 (9-28) days and 15 (9-330) days for platelets, with a cumulative platelet engraftment incidence of 96.1%. The incidence of infection was 58.27% (74/127). During a median follow-up of 20.5 (4-60) months, the incidence of grade Ⅱ-Ⅳ acute GVHD was 24.79% (30/121), moderate-severe chronic GVHD was 14.15% (15/106), 4-year estimated overall survival was (78.5±4.3) %, 4-year estimated failure-free survival was (77.4±4.3) %, respectively. Conclusion: Combination of HID-HSCT and an unrelated umbilical cord blood unit was a feasible choice with favorable outcome for SAA patients without matched donors.

[A multicenter randomized controlled study on the treatment of acute and chronic pharyngitis with Binglianqingye Spray].

Objective:To further evaluate the clinical efficacy and safety of Binglianqingye Spray on acute and chronic pharyngitis by clinical observation. Method:A randomized, double-blind and multicenter clinical trial which served Jinsangzi Tablet and Jinhoujian Spray as the control groups was conducted to observe the effect of Binglianqingye Spray on 360 patients with acute and chronic pharyngitis. Result:Binglianqingye Spray could significantly improve the symptoms with the exact clinical efficacy of the acute pharyngitis or chronic pharyngitis, such as sore throat, pharyngeal mucosa and uvula, dry throat burning, headache, cough, pharynx posterior wall lymphoid follicles hyperemia swelling and mandibular angle lymph node swelling and so on, but it occasionally occurred urine abnormalities and urine sugar adverse reactions. At the same time, the taste satisfaction was 82.1%. Conclusion:The combined effects of Binglianqingye Spray on the treatment of acute pharyngitis or chronic pharyngitis are well, the adverse reaction is rare and the patient's compliance is high.

[Serotype distribution and drug resistance of Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis isolated from nasopharynx of Uygur children].

Objective: To investigate the serotype distribution and antimicrobial susceptibility pattern of Streptococcus pneumoniae (S. pneumoniae), Haemophilus influenzae (H. influenzae) and Moraxella catarrhalis (M. catarrhalis) isolates collected from nasopharyngeal swabs from Uygur children in Kashi. Methods: Nasopharyngeal swabs were collected from inpatient Uygur children aged from 1 month to 5 years with respiratory infections from the pediatric department, the First People's Hospital of Kashi, Xinjiang Uygur Autonomous Region. Antimicrobial susceptibilities of the isolates were determined with E-test and KB disk diffusion methods. The production of ß-lactamase was detected for H. influenzae and M. catarrhalisisolates using nitrocefin disc method. Quellung test and latex agglutination test were adopted to identify serotypes of S. pneumoniae and H. influenzae isolates. Results: Forty-seven S. pneumoniae, 13 H. influenzae and 16 M. catarrhalis isolates were detected. All of the 47 S. pneumoniae isolates were sensitive to parenteral penicillin, amoxicillin-clavulanic acid, vancomycin and levofloxacin; the susceptibility rates to cefotaxime, imipenem and chloramphenicol were 94% (44/47), 89% (42/47), and 98% (46/47). The resistance rate to erythromycin was 74% (35/47). The most common serotype of S. pneumoniae was serotype 19A (10 strains, 21%). The coverage rate of 13-valent conjugate vaccine (PCV13) was 70% (33/47). None of the 13 H. influenzae isolates could be typed. They were highly susceptible to tested ß-lactams antibiotics, except ampicillin. Only one H. influenzae isolate could produce ß-lactamase, and two isolates were identified as ß-lactamase-negative-ampicillin-resistant ones. The sixteen M. catarrhalis isolates were all positive in ß-lactamase detection, but sensitive to amoxicillin-clavulanic acid, cephalosporins and meropenem. Conclusions: In Kashi, Xinjiang Uygur Autonmous Region, S. pneumoniae isolates from Uygur children were highly sensitive to parenteral penicillin and other ß-lactams antibiotics. H. influenzae isolates from Uygur children were highly susceptible to amoxicillin-clavulanic acid, cephalosporins and ciprofloxacin. All M. catarrhalis isolates from Uygur children could produce ß-lactamase, but were sensitive to the enzyme inhibitors and cephalosporins.

[Clinical features and curative effect analysis of patients with myeloid neoplasms and RUNX1 mutations].

Objective: To investigate the survival and prognostic factors of allogeneic hematopoietic stem-cell transplantation (allo-HSCT) for patients with myeloid neoplasms and RUNX1 mutations. Methods: From July 2014 to April 2018, the clinical data of forty-two AML/MDS patients with RUNX1 mutations in the First Affiliated Hospital of Soochow University were retrospectively analyzed. The clinical characteristic features and distribution of the mutations frequently observed with RUNX1 mutations were summarized, the prognosis of allo-HSCT for these patients was also analyzed. Results: Among 42 AML/MDS patients with RUNX1 mutations, 27 were male, 15 were female. The median age was 43.5 (16-68) years old. There are 31 patients in allo-HSCT group and 11 patients in chemotherapy group. RUNX1 mutations co-occurred with many other gene mutations, the most frequent mutations were FLT3 (26.2%, 11/42) . Interestingly, FLT3 mutations only occurred in AML patients compared with MDS patients (P=0.014) . ASXL1 (25%, 3/12) mutations were observed as the most frequent co-mutations in MDS patients. One-year overall survival (OS) , disease-free survival (DFS) of allo-HSCT and chemotherapy patients were (70.6±9.0) %, (61.0±9.4) % and (34.4±16.7) %, (22.4±15.3) %, respectively. When OS and DFS between allo-HSCT and chemotherapy patients were compared, significant differences (χ(2)=4.843, 4.320, P<0.05) were showed. In univariate analysis, transplant age >45 years was a negative effect for OS [HR=4.819 (95% CI 1.145-20.283) , P=0.032] and DFS [HR=5.945 (95% CI 1.715-20.604) , P=0.005]. Also, complex chromosome karyotype abnormality was a negative effect for OS [HR=5.572 (95%CI 1.104-28.113) , P=0.038]. Conclusion: Transplant age (>45 years) and complex chromosome karyotype abnormality were negative prognostic factors in allo-HSCT for myeloid neoplasms patients with RUNX1 mutations.