Objective:To investigate the efficacy and safety of autologous hematopoietic stem cell transplantation (ASCT) in the treatment of multiple myeloma (MM).Methods:The clinical data of 64 MM patients who received ASCT in the Second Affiliated Hospital of Xi'an Jiaotong University from October 2015 to March 2022 were retrospectively analyzed. The clinical characteristics, therapeutic effects and adverse reactions of the patients were summarized.Results:Of the 64 patients, 42 were male and 22 were female; the median age was 54 years old (37-69 years old). The median number of CD34 + cells collected from 46 patients in the CE (cyclophosphamide, etoposide) regimen mobilization group and 17 patients in the plerixafor mobilization group were 7.50×10 6/kg [(1.15-24.73)×10 6/kg] and 4.54×10 6/kg [(0.75-10.40)×10 6/kg], and the difference was statistically significant ( Z = 3.02, P = 0.024). Hematopoietic reconstitution was successful in all 64 patients, and the median time for white blood cell and platelet engraftment was 11 d (8-13 d) and 11 d (8-15 d), respectively. The patients' pretreatment regimens were all high-dose melphalan, the median white blood cell and platelet engraftment time of 29 patients in the oral group were 11 d (8-13 d) and 11 d (8-15 d), respectively, the median white blood cell and platelet engraftment time of 35 patients in the intravenous infusion group were 11 d (8-12 d) and 11 d (8-15 d), respectively, and there were no statistical differences (both P > 0.05). The ≥CR rate was 48.4% (31/64) before transplantation and 70.3% (45/64) three months after transplantation, and the difference was statistically significant ( χ2 = 6.35, P = 0.012). The median follow-up time of all patients was 27 months (2-67 months). The 3-year OS and PFS rates were 77.6% and 54.9%, and the median OS and PFS time were 67 and 52 months. The median hospitalization time was 20 d (15-37 d). There was no transplantation-related mortality, and the main adverse reactions were gastrointestinal reactions (100.0%, 64/64), grade 4 thrombocytopenia (98.4%, 63/64), grade 4 neutropenia, and agranulocytosis with fever (40.6%, 26/64). Conclusions:ASCT is effective for MM patients suitable for transplantation, which can further improve the remission rate and remission depth, prolong the PFS and OS time of patients, and the adverse reactions are controllable.

Objective:To analyze the clinical curative effect of protective sleep nursing on neonatal hyperbilirubinemia.Methods:Eight neonates with hyperbilirubinemia who were admitted from April 2019 to August 2019 were enrolled. They were divided into control group (40 cases) and observation group (40 cases) by random digits table method. Both groups were given routine nursing. On basis of control group, observation group was given protective sleep nursing. The clinical effect, sleep time, discomfort reactions and nursing satisfaction were compared between the two groups.Results:After nursing, the sleep time, crying time and bilirubin level were (18.67 ± 1.45) h/d, (0.82 ± 0.12) h/d, (191.58 ± 12.74) μmol/L in the observation group, and (17.63 ± 1.33) h/d, (1.05 ± 0.15) h/d, (202.42 ± 13.08) μmol/L in the control group, there were significant differences between the two groups ( t values were 3.343, 7.573, 3.755, P<0.05). The duration and regression time of jaundice were (5.26±1.24), (8.70±2.12) d in the observation group, and (7.14±1.18), (12.95±2.31) d in the control group, there were significant differences between the two groups ( t values were 6.946, 8.573, P<0.05). The good rate of sleep quality, incidence rates of vomiting, skin damage and needle falling out, and nursing satisfaction rate were 90.00%(36/40), 7.50%(3/40), 5.00%(2/40), 10.00%(4/40), 100.00%(40/40) in the observation group, and 72.50% (29/40), 27.50%(11/40), 22.50%(9/40), 32.50%(13/40), 87.50%(35/40) in the control group, there were significant differences between the two groups ( χ2 values were 4.021-6.050, P<0.05). Conclusions:The application of protective sleep nursing in treatment of neonatal hyperbilirubinemia can effectively prolong their sleep time, improve their sleep quality, which is conducive to improving their symptoms, reducing discomfort reactions.And satisfaction of their family members is relatively higher.

Objective:To identify the pathogenic mutation in a five-generation Ningxia family with autosomal dominant retinitis pigmentsoa (adRP) and to analyze its associated clinical phenotype.Methods:One adRP pedigree was recruited for this study.All the patients and family members received complete ophthalmic examinations.DNA was abstracted from peripheral blood of three patients, one normal family member and 300 normal controls.Using whole exome sequencing (WES) chip and bioinformatics analysis to screen the candidate disease-causing mutations.PCR and direct sequencing were used to confirm the disease-causing mutations.Genotype-phenotype correlation was also analyzed.This study followed the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of People's Hospital of Ningxia Hui Autonomous Region (No.20160204).Results:PRPF31 c. C1048T (p.Q350X) nonsense mutation was identified as the disease-causing mutation for this family by WES chip, PCR and direct sequencing.This family demonstrated early onset of the disease by presenting nyctalopia from 5 to 6 years, performed rapid disease progression, severely impaired visual function and posterior subcapsular cataract.The fundus presentations and electroretinogram (ERG) results showed typical RP progressions. Conclusions:PRPF31 c. C1048T (p.Q350X) nonsense mutation is the disease-causing mutation of this family.This mutation is first reported in Chinese with distinct phenotypes in the present family, including early onset of the disease, rapid disease progression, severely impaired visual function and posterior subcapsular cataract.

Objective:To evaluate the influence of goal-directed volume management based on cardiac output index (CI), intrathoracic blood volume index (ITBVI) and extravascular lung water index (EVLWI) in patients undergoing off-pump coronary artery bypass surgery.Methods:Forty patients (ASA 2 to 3 grade) undergoing off-pump coronary artery bypass surgery in Lanzhou University Second Hospital from January 2017 to December 2018 were selected. The patients were divided into 2 groups by random digits table method with 20 cases in each group: study group (goal-directed fluid therapy treatment with CI, ITBVI and EVLWI) and control group (conventional fluid therapy). The control group was given central venous pressure (CVP) monitoring rehydration, and the study group was given PiCCO hemodynamic monitoring indicators. The CVP, CI, ITBVI and EVLWI for fluid management were measured. Accurate assessment of volume status of patients was done. The study group received goal-directed fluid therapy based on CVP, CI, ITBVI and EVLWI, with the goal of CI in the 3.0 to 5.0 L/(min·m 2) range, ITBVI in the 800 to 1 000 ml/m 2 range and EVLWI in the 3.0 to 7.0 ml/kg range. The heart rate, mean arterial pressure (MAP), urine volume, central venous oxygen saturation (ScvO 2), lactic acid and renal function were monitored. The ventilator withdrawal time, hospitalization in ICU, length of stay, incidence of acute pulmonary edema, incidence of acute renal failure, mortality of 30 d after surgery were recorded and compared between the two groups. Results:Tissue perfusion and urine volume of the study group was significantly improved compared with that of control group ( P<0.05). ScvO 2 of the study group was higher than that of the routine group ( P<0.05). The concentration of lactic acid of the study group was lower than that of the routine group ( P<0.05). The incidences of acute pulmonary edema, acute renal insufficiency and mortality of the study group were lower than those of the routine group (5.0% vs. 15.0%, 5.0% vs. 10.0% and 5.0% vs. 15.0%), and there were statistical differences ( P<0.05). The length of stay and hospitalization in ICU were both lower than those in the control group ( P<0.01). Conclusions:Goal-directed fluid therapy based on CI, ITBVI and EVLWI can effectively optimize the cardiac preload of patients undergoing off-pump coronary artery bypass surgery, improve cardiac output, ensure microcirculation perfusion, maintain the balance of oxygen supply and demand, and reduce the incidence of complications and mortality.

Chimeric antigen receptor T (CAR-T) cell therapy is a novel cellular immunotherapy that is widely used to treat hematological malignancies, including acute leukemia, lymphoma, and multiple myeloma. Despite its remarkable clinical effects, this therapy has side effects that cannot be underestimated. Cytokine release syndrome (CRS) is one of the most clinically important and potentially life-threatening toxicities. This syndrome is a systemic immune storm that involves the mass cytokines releasing by activated immune cells. This phenomenon causes multisystem damages and sometimes even death. In this study, we reported the management of a patient with recurrent and refractory multiple myeloma and three patients with acute lymphocytic leukemia who suffered CRS during CAR-T treatment. The early application of tocilizumab, an anti-IL-6 receptor antibody, according to toxicity grading and clinical manifestation is recommended especially for patients who suffer continuous hyperpyrexia, hypotensive shock, acute respiratory failure, and whose CRS toxicities deteriorated rapidly. Moreover, low doses of dexamethasone (5-10 mg/day) were used for refractory CRS not responding to tocilizumab. The effective management of the toxicities associated with CRS will bring additional survival opportunities and improve the quality of life for patients with cancer.

Objective To evaluate the effectiveness of a comprehensive hypertension management. Methods Hypertensive patients aged≥35 years in the Zhengfei community of Zhengzhou were selected. The patients were randomly assigned to the intervention and control groups. Those in intervention group received comprehensive hypertension management from October 2015 to September 2016,whereas those in the control group received the original management mode. Scales to assess blood pressure control, biochemical indexes, unhealthy lifestyle, and cardiovascular disease associated risk level were used to evaluate the effectiveness of the management modes.Results Each study groups had 1 051 patients.There were no significant differences in the baseline data of the two groups (P>0.05). At the end of 1 year of receiving the respective hypertension management modes, each group had 941 patients. Findings revealed that after receiving the comprehensive hypertension management mode, the systolic and diastolic blood pressure in the intervention group decreased by(9.87±7.38)mmHg(1 mmHg=0.133 kPa)and(6.33±4.14) mmHg,respectively.Those in the control group decreased by(7.01±6.02)mmHg and(4.52±3.59)mmHg, respectively,statistically significant differences in the extent of reduction of blood pressure between the two groups (P<0.05). Further, the fasting plasma glucose, postprandial blood glucose, low density lipoprotein, serum creatinine,and microalbuminuria levels in the intervention group were significantly lower than those in the control group(all P<0.05).However,the intervention group exhibited a significant increase in the high density lipoprotein level as compared to the control group(P<0.05).There were no significant differences in the total cholesterol,triglyceride,urinary creatinine levels,and body mass index between the two groups(P>0.05), although they had decreased in both groups. After the 1-year management, these proportions of smoking,heavy drinking,high salt diet and need to exercise were 10.0%,3.7%,20.1%,and 48.9% in the intervention group, and 15.3%, 10.0%, 29.0%, and 54.3% in the control group. The proportions were significantly different between the two groups (P<0.05). After the 1-year management, these proportions of low,moderate,and high risk of cardiovascular disease were 13.3%,33.5%,and 53.2% in the intervention group, and 11.2%, 30.1%, and 58.8% in the control group, respectively, with statistically significant differences between the two groups (P< 0.05). After the 1-year management, the proportion of treated, controlled, and control-treated hypertension using medication was 100%, 65.1%, and 75.3% in the intervention group, and 39.5%, 60.3%, and 70.0% in the control group, respectively, with statistically significant differences between the two groups (P< 0.05). Conclusion The comprehensive hypertension management mode was effective in significantly improving the blood pressure and health condition of hypertensive patients.

Background Hereditary retinal diseases (HRDs) are a group of retinal degenerative diseases with significant genetic and clinical heterogeneities.Traditional techniques are challenging for detection of pathogenic mutations.Objective This study was to identify the diseasing-causal genes in 20 Chinese families with a variety of HRDs.Methods Family histories and ophthalmic examinations were obtained from all participants in 20 sporadic families.Targeted sequence capture array technique with next-generation sequencing (NGS) was performed to detect pathogenic mutations in 232 identified genes associated with HRDs.Variants detected by NGS were filtered by bioinformatic analysis HRDs.Genotype-phenotype correlation was also assessed.Results We identified 11 patients with pathogenic mutations,including 8 compound heterozygous mutations and 3 homozygous mutations,which were not yet reported.These findings showed genetic diagnoses in 11 of 20 patients,with the positive rate of 55％.Among them,6 patients were autosomal recessive inheritance and 5 were unspecific.Identification of different mutations and divergent phenotypes revealed 5 patients were affected with cone-rod dystrophy,3 patients with Leber congenital amaurosis,1 patient with congenital stationary night blindness,1 patient with Best vitelliform macular dystrophy and 1 patient with Stargardt disease.Conclusions Targeted NGS is an effective approach for the genetic diagnoses of HRDs.These findings provide insights into understanding the genotype-phenotype correlations in HRDs.

Objective To evaluate the effects of GLYX-13 on cognitive function after long-time isoflurane anesthesia in mice. Methods A total of 192 healthy male C57∕B6J mice, aged 8 weeks, weig-hing 22-25 g, were divided into 4 groups(n=48 each)using a random number table: control group (group C), isoflurane anesthesia group(group I), GLYX-13 group(group G), and isoflurane anesthesia plus GLYX-13 group(group IG). The animals were exposed to 15% isoflurane for 6 h in I and IG groups. GLYX-13 1 mg∕kg was injected via the caudal vein at 2 h before anesthesia in G and IG groups. Novel ob-ject recognition test and contextual fear conditioning test were performed on 1st, 3rd and 7th days after an-esthesia. The expression of 2B subunits-containing NMDA receptor(NR2B)and cyclic adenosine mono-phosphate response element-binding protein(CREB)mRNA in the hippocampus was detected by quantita-tive real-time polymerase chain reaction after the end of behavioral tests on 1st, 3rd and 7th days after anes-thesia. Results Compared with group C, the percentage of time spent in exploring a novel object, dis-crimination index and percentage of freezing time were significantly decreased, and the expression of NR2B and CREB mRNA in the hippocampus was down-regulated in group I(P <005). Compared with group I, the percentage of time spent in exploring a novel object, discrimination index and percentage of freezing time were significantly increased, and the expression of NR2B and CREB mRNA in the hippocampus was up-regulated in group IG(P <005). Conclusion GLYX-13 can significantly improve the cognitive func-tion after long-time isoflurane anesthesia in mice.

Objective To induce human umbilical cord mesenchymal stem cells (hUC-MSCs) to hair-cell like cells in the inner ear, using a two-step neural differentiation method.Methods The hUC-MSCs were obtained from human umbilical cords by tissue adherence culture,whose surface antigen CD29, CD34, CD44, CD45, CD90, HLA-ABC, and HLA-DR could be identified by flow cytometry.In the neural stem cells induced phase, the NSE positive cells were analyzed by microscope and immunohistochemistry.In the second stage, the expression of hair-cell like cells markers (Math1, MyosinⅦa, Brn3c) were tested by qRT-PCR and immunofluorescence method.Results The control group and the protocol group had little NSE after differentiation while the protocol B group presented a neurobiological structure and demonstrated a higher NSE positive ratio after 5 days' neural stem cells induction (P<0.05).Compared to the control group, the mRNA and protein level of Math1, MyosinⅦa, and Brn3c exhibited a significant increase in the differential group,which induced for 4 weeks in the hair-cell like cells in the inner ear's induced phase(P<0.05).Conclusion The two-stage induction (hUC-MSCs-neural stem cells-hair-cell like cells) could produce more MyosinⅦa,Brn3c and Math1,which may provide an appropriate way to treat sensorineural deafness.

Objective:To explore the improvement effect of tirofiban on the myocardial reperfusion of non-ST segmant elevated myocardial infarction (NSTEMI) patients underwent percutaneous coronary intervention (PCI) treatment.Methods:78 NSTEMI patients underwent PCI in our hospital from April 2012 to April 2015 were selected and divided into the observation group (n=38) and the control group (n=40) according to different drugs.Patients in the control group were given asprin,clopidogrel and heparin,while patients in the observation group were additionally given tirofiban.Then the myocardial contrast echocardiography (MCE) was taken to evluate the myocardial reperfusion.Results:No statistical difference was found in the levels of A,β,A β,CK-MB and cTnⅠ before PCI between 2 groups.The levels of β,A β of observation group were obviously higher,CK-MB and cTnⅠ were obviously lower than those of the control group(P＜0.05).The MACE rate of observation group was 2.63％,which was 5.00％ in the control group,no significant difference was between two groups (P＞0.05).Conclusion:Tirofiban could obviously improve the myocardial reperfusion of NSTEMI patients underwent PCI with high safety.