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ObjectiveThis study aimed at conducting retrospective analysis of the clinical symptoms and genetic mutations in 20 children with Gitelman syndrome treated at the Affiliated Children′s Hospital of Nanjing Medical University from August 2015 to November 2022 and also explored the molecular mechanism of the pathogenic high-frequency mutation D486N in the Chinese population.MethodsWe collected the clinical manifestations, growth and development status, laboratory examination results, and SLC12A3 gene variations of the patients. We distinguished the wild-type and mutant SLC12A3 genes overexpressed in human embryonic kidney 293T cells (HEK293T). We used protein immunoblotting to detect the expression level of NCC, and used immunofluorescence techniques to examine the subcellular localization of NCC. In addition, we investigated the impact of the high-frequency SLC12A3 gene mutation D486N on NCC protein expression and localization.ResultsIn the 20 patients with Gitelman syndrome, all of them had hypokalemia. We indemnified twenty-six SLC12A3 gene mutations, 13 of which are missense mutation, 1 of which synonymous mutation, 1 nonsense mutation, 4 frameshift mutation, and 7 splicing site mutation. Among them, four mutations (p.T235K, c.1096-1G > A, p.A464A, and c.2660+1_2660+2insT) were novel mutations.ConclusionsWe found the preliminary evidence that the high-frequency mutation D486N in the Chinese population affected the expression of total and membrane-bound NCC protein and influenced the membrane localization of NCC protein. The findings of this study provides experimental evidence for genetic counseling, diagnosis, and treatment of Gitelman syndrome.
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OBJECTIVES: This study was designed to reveal structural abnormalities in singleton and twin pregnancies in the Chinese population. METHODS: This retrospective study spanned 8 years and included 1228 singleton pregnancies (112,919 examinees) and 49 twin pregnancies (1865 examinees) with structural anomalies diagnosed by ultrasound. Detailed descriptions of anomalies, gestational weeks at diagnosis, and maternal age were recorded. The odds ratio was evaluated in twin pregnancies with detectable structural anomalies. RESULTS: The annual average "ultrasound prevalence of fetal anomalies" among singleton and twin pregnancies were 1.09 and 3.06%, respectively. Mothers with twin anomalies were older (P < .001), and twin pregnancies were diagnosed with anomalies in earlier gestational weeks than singleton (P = .011). No differences were found in the types of anomalies between singleton and twin pregnancies. Central nervous system anomaly was the most common type in singleton and twin pregnancies. Twin pregnancies had higher rates of major anomalies than singleton (total OR 2.45), especially cardiovascular, central nervous, and gastrointestinal systems and ear/eye/face/neck disorders. CONCLUSIONS: Compared with singleton, twin pregnancies had higher odds of detectable structural anomalies. Twin pregnancies with structural anomalies were diagnosed at earlier gestational age and associated with older maternal age. Central nervous system anomaly was the most common type in singleton and twin pregnancies.
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East Asian People , Pregnancy, Twin , Pregnancy , Female , Humans , Retrospective Studies , Maternal Age , Prenatal Diagnosis , Gestational AgeABSTRACT
Primary lung cancer is a malignant tumor with the highest incidence and mortality in China, and lung rehabilitation for lung cancer has become a research hotspot. Many literature and studies have confirmed the feasibility and safety of pulmonary rehabilitation for lung cancer, but the mechanism of pulmonary rehabilitation for lung cancer is not completely clear. This article will discuss the mechanism of pulmonary rehabilitation for lung cancer, the specific content of pulmonary rehabilitation and remote home pulmonary rehabilitation.
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Objective:To explore the applications value of hospital elderly life program in cardiac surgery patients in intensive care unit, and provide reference for improving the prognosis of patients.Methods:This was a prospective study. A total of 84 cardiac surgery patients in intensive care unit from April 2020 to February 2022 in the People′s Hospital of Leshan by convenient sampling method, they were enrolled and divided into the observation group and the control group according to the admission time, each group was 42 cases. Routine nursing care was carried out in both groups, the control group implemented delirium and debility prevention nursing, the observation group adopted hospital elderly life program. The incidence of ICU-acquired delirium and weakness, mechanical ventilation time, duration of ICU stay, the total length of stay and intensive care experience were assessed between the two groups.Results:The 42 cases were included in the final control group and 39 cases in the observation group. The incidence of ICU-acquired delirium and weakness were 17.95% (7/39) and 7.69% (3/39) in the observation group, lower than in the control group 38.10%(16/42) and 23.81%(10/42), the differences were statistically significant ( χ2 = 4.04, 3.90, both P<0.05); the duration of ICU delirium were (1.71 ± 0.95) d in the observation group, shorter than in the control group (2.81 ± 1.05) d, the difference was statistically significant ( t = 2.38, P<0.05); the mechanical ventilation time, duration of ICU stay, the total length of stay, the total score of intensive care experience in hospital in the observation group were (193.54 ± 21.67) h, (9.49 ± 2.11) d, (18.10 ± 3.12) d, (2.72 ± 0.26) points, lower than those in the control group (214.50 ± 27.25) h, (10.90 ± 1.97) d, (20.59 ± 4.07) d, (3.15 ± 0.35) points, the differences were statistically significant ( t values were 3.11-6.35, all P<0.05). Conclusions:Hospital elderly life program can decrease the incidence of ICU-acquired delirium and weakness of cardiac surgery patients in intensive care unit, shorten mechanical ventilation time and hospitalization time, alleviate discomfort in the intensive care experience.
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Objective:To explore the medication law and core TCM prescriptions of Jia Yuejin in the treatment of coronary heart disease (CHD) complicated with depressive disorder (DD) by analyzing target-based network and mining clinical data.Methods:The targets of CHD complicated with DD were obtained by GeneCards, OMIM, TTD and other databases, and then the protein-protein interaction network of the two disease targets was constructed and then screened out the core targets. The Metascape platform was used to perform GO and KEGG pathway enrichment analysis on the intersection targets respectively to analyze the mechanism of action of CHD complicated with DD. Then TCMSP was used to query the active components acting on the targets and the Chinese materia medica containing these active components, and the data were imported into Cytoscape 3.9.0 to construct the core target-active component-Chinese materia medica network for network topology analysis. The outpatient clinical data of Jia Yuejin from January 1, 2015 to January 1, 2021 were collected, and data mining was conducted by using the Ancient and Modern Medical Case Cloud Platform (V2.3.5) to obtain his commonly used prescriptions. The results were fitted with the core TCM prescriptions obtained by target network analysis, and the drugs in the core prescriptions were analyzed.Results:Totally 1 501 intersection targets were obtained by protein interaction network analysis of CHD complicated with DD, which could be divided into 4 core target clusters, including inflammation cause, subclass tumor cause, subclass lipid metabolism factor, and fibrosis factor; a total of 480 active components were obtained by TCMSP, which belonged to 181 types of Chinese materia medica, including 8 core components: quercetin, kaempferol, luteolin, carotene, beta-carotene, acacetin, formononetin and ellagic acid. GO enrichment analysis yielded 61 results, mainly including positive regulation of protein phosphorylation, signal receptor agonist activity, side of membrane , etc.; KEGG pathway enrichment analysis yielded a total of 20 results, mainly including cancer pathways, lipid and atherosclerosis, JAK-STAT signaling pathway, etc. Clinical data mining included 120 cases and 148 prescriptions, including 135 types of Chinese materia medica; the properties were mainly mild, warm, slightly cold and cold; the tastes were mainly sweet, bitter and light, and the medicine mainly belongs to the lung, spleen, liver, heart, stomach, kidney and other meridians; drug association analysis, cluster analysis and complex network analysis were used to synthesize common prescriptions. The core TCM prescriptions obtained from common prescription and target network analysis were fitted: Pinelliae Rhizoma, Glycyrrhizae Radixet Rhizoma, Bupleuri Radix, Cyperi Rhizoma, Salviea Miltiorrhizae Radix et Rhizoma, Corydalis Rhizoma, Codonopsis Radix, Astragali Radix, Acori Tatarinowii Rhizoma. Conclusion:The medication law of Jia Yuejin in the treatment of CHD complicated with DD is in accordance with core TCM prescriptions. This study can provide guidance for clinical treatment and further research of CHD complicated with DD.
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OBJECTIVES@#To investigate the level of neuropsychological development in large for gestational age (LGA) infants at the age of 12 months.@*METHODS@#The infants, aged 12 to <13 months, who attended the Outpatient Service of Child Care in the First Affiliated Hospital of Shandong First Medical University from December 2021 to June 2023, were enrolled as subjects. According to the gestational age and birth weight, they were divided into preterm appropriate for gestational age (AGA) group, preterm LGA group, early term AGA group, early term LGA group, full-term AGA group, and full-term LGA group. A modified Poisson regression analysis was used to investigate the association between LGA and neuropsychological development outcome at 12 months of age.@*RESULTS@#After adjustment for confounding factors, compared with the full-term AGA group at the age of 12 months, the full-term LGA group had a significant increase in the risk of language deficit (RR=1.364, 95%CI: 1.063-1.750), the early term LGA group had significant increases in the risk of abnormal gross motor, fine motor, language, and the preterm LGA group had significant increases in the risk of abnormal language, social behavior, and total developmental quotient (P<0.05); also, the early term AGA group had higher risks of developmental delay across all five attributes and in total developmental quotient at the age of 12 months (P<0.05); except for the language attribute, the preterm AGA group had higher risks of developmental delay in the other 4 attributes (P<0.05).@*CONCLUSIONS@#The neuropsychological development of LGA infants with different gestational ages lags behind that of full-term AGA infants at 12 months of age, and follow-up and early intervention of such infants should be taken seriously in clinical practice.
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Infant, Newborn , Infant , Child , Humans , Birth Weight , Infant, Large for Gestational Age , Infant, Small for Gestational Age , Gestational Age , Child HealthABSTRACT
OBJECTIVES@#To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA.@*METHODS@#A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results.@*RESULTS@#Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05).@*CONCLUSIONS@#There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.
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Child , Humans , Lupus Nephritis/complications , Kidney/pathology , Retrospective Studies , Thrombotic Microangiopathies/therapy , Prognosis , Hypertension/complications , Cholesterol , Lupus Erythematosus, SystemicABSTRACT
Chronic heart failure(CHF) has become a worldwide public health problem due to its high morbidity and mortality, which seriously endangers people's lifespan and quality of life. In recent years, the treatment strategy of CHF has shifted its emphasis on short-term improvement and transformation of hemodynamics to long-term repair as well as improvement of the biological properties of heart failure. At present, with the continuous deepening of medical research, it has been found that histone acetylation is closely related to the occurrence and development of CHF. Traditional Chinese medicine, via regulating histone acetylation, delays ventricular remodeling, improves energy metabolism, inhibits fibrosis and cardiomyocyte hypertrophy, and intervenes in the development process of heart failure, thus reducing the mortality and the readmission rate and ultimately improving long-term prognosis. Therefore, this study reviewed the mechanism of histone acetylation in the treatment of heart failure as well as its prevention and treatment with traditional Chinese medicine, to provide reference for clinical treatment of CHF.
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Humans , Medicine, Chinese Traditional , Histones/therapeutic use , Acetylation , Quality of Life , Heart Failure/prevention & controlABSTRACT
Objective: To evaluate the efficacy and safety of intravenous iron supplementation in patients with recurrent iron deficiency anemia (IDA) . Methods: This retrospective analysis of 90 patients with recurrent IDA from May 2012 to December 2021 was conducted, comparing the efficacy and safety of the intravenous iron therapy group and the oral iron therapy group. Results: Among the 90 patients with recurrent IDA, 20 were males and 70 were females, with a median age of 40 (range: 14-85) years. A total of 60 patients received intravenous iron supplementation and 30 received oral iron supplementation. The hematologic response rates in the intravenous iron group were significantly higher than those in the oral iron group at 4 and 8 weeks after treatment [80.0% (48/60) vs 3.3% (1/30) and 96.7% (58/60) vs 46.7% (14/30), all P<0.001, respectively]. The median increase in hemoglobin levels was also significantly higher in the intravenous iron group than in the oral iron group [38 (4, 66) g/L vs 7 (1, 22) g/L at week 4 and 44.5 (18, 80) g/L vs 19 (3, 53) g/L at week 8, all P<0.001]. The intravenous iron group had a significantly higher proportion of patients who achieved normal hemoglobin levels than the oral iron group (55.0% vs 0 and 90% vs 43.3%, all P<0.001, respectively). Iron metabolism indicators were tested before and after 8 weeks of treatment in 26 and 7 patients in the intravenous and oral iron groups, respectively. The median increase in serum ferritin (SF) levels in the intravenous iron group 8 weeks after treatment was 113.7 (49.7, 413.5) μg/L, and 54% (14/26) of these patients had SF levels of ≥100 μg/L, which was significantly higher than the median increase in SF levels in the oral iron group [14.0 (5.8, 84.2) μg/L, t=4.760, P<0.001] and the proportion of patients with SF levels of ≥100 μg/L (P=0.013). The incidence of adverse reactions was 3.3% (2/60) in the intravenous iron group, which was significantly lower than that in the oral iron group [20.0% (6/30), P=0.015]. Conclusion: Intravenous iron supplementation is more effective for hematologic response, faster hemoglobin increase, and higher iron storage replenishment rates compared with oral iron supplementation in patients with recurrent IDA, and it is well tolerated by patients.
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Male , Female , Humans , Adolescent , Young Adult , Adult , Middle Aged , Aged , Aged, 80 and over , Anemia, Iron-Deficiency/epidemiology , Sucrose/therapeutic use , Ferric Compounds/therapeutic use , Retrospective Studies , Iron/therapeutic use , Hemoglobins/therapeutic useABSTRACT
Objective:To study the learning curve of percutaneous patent foramen ovale (PFO) occlusion guided solely by transthoracic echocardiography (TTE), as well as the success rate and safety of the learning curve.Methods:To retrospectively analyze these patients with indications for PFO occlusion admitted in our department from April 2021 to April 2022, and obtained 100 samples the author's initial cases guided solely by TTE, including 25 men and 75 women, with a mean age of (48.22±10.44) years old.Analyze preoperative baseline data: gender, age, height, weight, body mass index, the tunnel length and size of the PFO measured by transesophageal echocardiography, the grade of contrast-transcranial doppler test, combined atrial septal aneurysm, etc.Operation time, success rate, and complications were analyzed in all patients.Results:With the accumulation of cases, the operation time gradually shortened, accumulated to about 50 cases, the operation time has significantly shortened ( P<0.05), and the learning curve was leveled off after 50 cases ( P<0.05), there was statistical difference.The comparison of the success rate and complication of cases within the learning curve and those after completing the learning curve was no statistical significance( P>0.05). Conclusion:The learning curve of percutaneousc closure of patent foramen ovale guided solely by TTE is long, requiring about 50 cases to complete the learning curve. The success rate and safety of the learning curve are high. This procedure is worth popularizing.
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Objective:To investigate the prevalence of Mycoplasma pneumoniae ( Mp) in children undergoing physical examination. Methods:This study randomly enrolled 1 303 children at the age of 6-12 years who underwent physical examination in 2023. Their oral and pharyngeal swabs as well as venous blood samples were collected. The prevalence of Mp in these subjects was detected using isolation and culturing, nucleic acid detection and serological test. Chi-square test was used for statistical analysis. Results:Among the 1 303 children, the detection rate of Mp was 4.1% (53/1 303) by culturing, 7.3% (95/1 303) by nucleic acid detection and 13.6% (177/1 303) by serological test. Statistical analysis showed that there were significant differences in the the detection rates of Mp among children undergoing physical examination between the three methods ( P<0.05). Conclusions:The detection rate of Mp in children undergoing physical examination in 2023 was about 4.1%. Isolation and culturing was more accurate than nucleic acid detection and serological test in the detection of Mp in healthy population as the latter two methods would overestimate the rate.
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BACKGROUND: Pulmonary infections often lead to poor prognoses in patients with chronic obstructive pulmonary disease (COPD). Activin A and CD64 play crucial pathological roles in the development of COPD. AIM: To explore the bacterial spectrum via analysis of activing A levels, CD64 index, and related mechanisms in COPD patients complicated with pulmonary infection. METHODS: Between March 2015 and January 2018, a total of 85 patients with COPD, who also suffered from pulmonary infections, were enrolled in this study as the pulmonary infection group. In addition, a total of 96 COPD patients, without pulmonary infection, were selected as the control group. Sputum samples of patients in the pulmonary infection group were cultivated for bacterial identification prior to administration of antibiotics. The neutrophil CD64 index was measured using flow cytometry, serum activin A levels were detected via an enzyme-linked immunosorbent assay, and activin A, Smad3, TLR4, MyD88, and NFκB protein expression was analyzed by Western blotting. RESULTS: Gram-negative bacteria were identified in 57.65% of the sputum samples in the pulmonary infection group. The most prevalent Gram-negative species were Pseudomonas aeruginosa and Klebsiella pneumoniae. Conversely, Gram-positive bacteria were identified in 41.18% of the sputum samples in the pulmonary infection group. The most common Gram-positive species was Streptococcus pneumoniae. Fungi were identified in 1.17% of the sputum samples in the pulmonary infection group. The CD64 index was significantly higher in the pulmonary infection group (0.91 ± 0.38) than in the control group (0.23 ± 0.14, P < 0.001). The serum activin A levels were significantly higher in the pulmonary infection group (43.50 ± 5.22 ng/mL), compared to the control group (34.82 ± 4.16 ng/mL, P < 0.001). The relative expression levels of activin A, Smad3, TLR4, MyD88, and NFκB were all significantly higher in the pulmonary infection group, compared to the control group (all P < 0.001). CONCLUSION: Pulmonary infections in COPD patients are mainly caused by Streptococcus pneumoniae, Pseudomonas aeruginosa, and Klebsiella pneumoniae. Pulmonary infections can significantly increase neutrophil CD64 index and serum levels of activin A, thereby activating the activin A/Smad3 signaling pathway, which may positively regulate the TLR4/MyD88/NFκB signaling pathway.
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PURPOSE: To compare the consistency of one-dimensional Response Evaluation Criteria in Solid Tumors (1D-RECIST), two-dimensional WHO criteria (2D-WHO), and three-dimensional (3D) measurement for therapeutic response assessment of nasopharyngeal carcinoma (NPC). MATERIALS AND METHODS: Retrospective data of 288 newly diagnosed NPC patients were reviewed. Tumor size was assessed on magnetic resonance imaging (MRI) according to the 1D-RECIST, 2D-WHO, and 3D measurement criteria. Agreement between tumor responses was assessed using unweighted k statistics. The receiver operating characteristic (ROC) curve was used to determine the optimal cut-off point of the PTV. The Kaplan-Meier method and Cox regression were used for the survival analysis. RESULTS: The optimal cut-off point of the PTV for progression-free survival (PFS) was 29.6%. Agreement with PTV measurement was better for 1D measurement than for 2D and 3D measurements (kappa values of 0.646, 0.537, and 0.577 for 1D, 2D, and 3D measurements, respectively; P < 0.05). The area under the curve of the 1D measurement (AUC=0.596) was similar to that of the PTV measurement (AUC=0.621). Compared with 2D and 3D measurements, 1D measurement is superior for predicting prognosis in NPC (C-index of 0.672, 0.663, and 0.646 were for 1D, 2D, and 3D measurements, respectively; P < 0.005). Survival analysis showed that patients with non-responders had worse prognosis (P < 0.05). CONCLUSIONS: The 1D measurement more closely agreed with the PTV measurement than the 2D and 3D measurements for predicting therapeutic responses in NPC. Therefore, we recommend using the less time-consuming 1D-RECIST criteria in routine clinical practice.
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BACKGROUND/PURPOSE: Immunotherapy has become a research hotspot and is used for head and neck cancer treatment. This research aims to explore the prognostic value of PYHIN1 in oral cancer and the relationship between PYHIN1 and cancer immunity. MATERIALS AND METHODS: The expression of PYHIN1 in clinical specimens was evaluated by bioinformatics analyses and immunohistochemistry. RESULTS: Gene ontology term enrichment analyses and gene set enrichment analyses showed the involvement of PYHIN1 in the modulation of adaptive immunity-associated signaling according to The Cancer Genome Atlas database and Gene Expression Omnibus dataset. Interestingly, the correlation analyses in The Cancer Genome Atlas database revealed a positive correlation between PYHIN1 expression and activated CD8+ T cells infiltration and a negative correlation between PYHIN1 expression and tumor purity. Moreover, activated CD8+ T cells infiltration predicted good patient survival and was negatively correlated with tumor purity. Importantly, PYHIN1 expression was negatively correlated with the pathological stage and was positively associated with a good prognosis in patients with oral cancer. The data obtained from the Gene Expression Omnibus dataset and immunohistochemistry confirmed the positive association between PYHIN1 and CD8+ T cells infiltration in oral cancer tissues. CONCLUSION: We conclude that PYHIN1 is an indicator of cancer immunity, and is an independent prognostic factor that may be an alternative target for oral cancer treatment.
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Objective:To investigate the incidence of anxiety and depression after radiotherapy for nasopharyngeal carcinoma among persons suffering from dysphagia, and to analyze the related factors.Methods:A total of 143 persons with dysphagia after radiotherapy for nasopharyngeal carcinoma were studied. They completed a general information questionnaire and were evaluated using the hospital anxiety and depression scale.Results:Anxiety was detected in 52 of the subjects (36.3%) and depression in 61 (42.7%). Multivariate regression analysis showed that the average total anxiety score was significantly related to whether the respondent used a gastric tube and whether they lived in a religious household. The average total depression score was related to gastric tube use and to the respondent′s district of residence.Conclusion:Persons with dysphagia after radiotherapy for nasopharyngeal carcinoma often suffer anxiety and depression. The use of a gastric tube increases the risk. The depression of rural residents and those with religious beliefs tends to be milder.
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Four cyclic peptides were isolated from the 75% ethanol extract of the fibrous roots of Pseudostellaria heterophylla by silica gel, Sephadex LH-20 column chromatography, and semi-preparative HPLC. Through mass spectrometry, NMR and other methods, they were identified as pseudostellarin L(1), heterophyllin B(2), pseudostellarin B(3), and pseudostellarin C(4). Among them, compound 1 was a new cyclic peptide, and compounds 2-4 were isolated from the fibrous roots of P. heterophylla for the first time. None of these compounds displayed cytotoxic activities against MCF-7, A549, HCT-116, and SGC-7901 cells.
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Caryophyllaceae/chemistry , Chromatography, High Pressure Liquid , Magnetic Resonance Spectroscopy , Peptides, Cyclic/pharmacology , Plant Roots/chemistryABSTRACT
Objective:To investigate the expression levels and clinical significance of glioma-associated oncogene homolog 1 (GLI1) and sonic hedgehog signaling molecule (Shh) in the malignant transformation of ovarian endometriosis (EM).Methods:The expressions of GLI1 and Shh were detected by real-time reverse transcription (RT)-polymerase chain reaction (PCR) and EnVision method in 50 cases of ovarian EM tissues, 35 cases of atypical endometriosis (aEM) and 50 cases of endometriosis-associated ovarian cancer (EAOC). The expression differences of two molecular markers in the malignant transformation of ovarian EM were compared, and the relationships between two molecular markers and the clinicopathological features and prognosis of EAOC were analyzed.Results:(1) RT-PCR showed that the expression levels of GLI1 mRNA in EM, aEM and EAOC group were 1.77±0.40, 3.54±0.44, and 7.80±0.24, respectively. The expression levels of Shh mRNA were 0.95±0.21, 3.14±0.35, and 5.41±0.31, respectively. GLI1 and Shh mRNA in EAOC group were significantly higher than those in EM and aEM group (all P<0.01), and there were statistically significant differences between EM and aEM group (all P<0.01). The percentages of GLI1 in ovarian EM, aEM and EAOC were 32% (16/50), 57% (20/35), and 66% (33/50), respectively, meanwhile, the positive expression rates of Shh were 20% (10/50), 49% (17/35), and 54% (27/50), respectively (all P<0.01). GLI1 mRNA expression was positively correlated with Shh mRNA expression in EAOC tissues ( r=0.721, P<0.01). The expressions of GLI1 protein were proportionated to Shh protein in EAOC tissues ( r=0.608, P=0.001). (2) The expression of GLI1 was significantly related to the International Federation of Gynecology and Obstetrics (FIGO) stage, cancer antigen 125 (CA 125) levels, lymph node metastasis, and Platinum resistance in EAOC patients (all P<0.05). The expression of Shh were related to FIGO stage and lymph node metastasis in EAOC patients (all P<0.05). Logistic regression analysis showed that GLI1 expression was an independent risk factor for poor prognosis in EAOC patients ( P<0.05). Kaplan-meier survival analysis showed that the overall survival rate of EAOC patients with high GLI1 expression and low GLI1 expression was 12.1% and 35.3%, respectively, with statistical significance ( χ2=10.73, P<0.01). The overall survival rate of EAOC patients with high and low expression of Shh protein was 11.1% and 30.4%, in which there was statistically significant difference ( χ2=3.96, P=0.047). Conclusion:GLI1 and Shh are highly associated with the malignant transformation of ovarian EM, which may play a role in promoting malignant degeneration of ovarian EM, and the high expression of GLI1 and Shh indicates a poor prognosis in EAOC patients.
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Objective:To investigate the efficacy and safety of ixazomib combined with lenalidomide and dexamethasone (IRd) regimen in treatment of multiple myeloma (MM) patients in the real world practice.Methods:The clinical data of 24 MM patients treated with IRd regimen from January 2019 to January 2021 in the Union Hospital, Tongji Medical College, Huazhong University of Science and Technology were retrospectively analyzed, and their efficacy and adverse reactions were analyzed. Among the 24 patients, 5 patients were relapsed and refractory (relapsed/refractory group), and 19 newly treated patients (conversion group) who responded to bortezomib induction therapy but converted to IRd regimen due to adverse reactions or other reasons.Results:The 24 patients were treated for a median of 4 cycles (2-7 cycles), with 8 cases of complete remission (CR), 6 cases of very good partial remission (VGPR), 8 cases of partial remission (PR), 1 case of disease progression (PD), 1 case of minimal response (MR), and the overall response rate (ORR) was 91.7% (22/24); the median progression-free survival (PFS) time was 15 months (95% CI 6.6-23.4 months); 6 CR patients were negative for minimal residual disease (MRD). The common adverse reactions were hematological adverse reactions, peripheral neuropathy, fatigue, gastrointestinal reactions, and infections. The incidence rate of grade 3-4 adverse reactions was 25.0% (6/24). In the relapsed/refractory group, the best efficacy was VGPR in 1 case, PR in 3 cases, and MR in 1 case, all patients withdrew from the IRd regimen therapy due to PD after transient remission or poor effect; in the conversion group, the best efficacy was CR in 8 cases, VGPR in 5 cases, PR in 5 cases, and PD in 1 case, 57.9% (11/19) patients maintained their original best response, and 36.8% (7/19) patients improved their best response to CR; the difference in median PFS time between the two groups was statistically significant (7 months vs. not reached, P = 0.018). Conclusions:The IRd regimen is safe and effective for MM patients, especially for the conversion patients after effective bortezomib induction therapy. Although patients with relapsed/refractory MM who have previously used multi-line therapy respond to IRd regimen, the duration of remission is limited.
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A patient with 20-segments multi-planar amputation of the 2nd-5th fingers of the right hand was treated in December 2020 in the Department of Hand and Microsurgery, Affiliated Taihe Hospital of Hubei Universtiy of Medicine. The groupings was performed with microscopic anastomosis and splicing, and the replantation was finally completed. Followed-up at 1 year after surgery, the 17 segments of 20 severed finger segments finally survived. According to the Chinese Medical Association's Hand Surgery Branch's Functional Evaluation Criteria for Replantation of Amputated Fingers, the patient's finger function rating was 57 points and the function was poor at 1-year follow-up. The range of motion of the metacarpophalangeal joint was significantly improved compared with half a year after the operation. The simple grasping, pinching and fingering can be completed. The finger sensation was improved compared with half a year after the operation, but the finger body of right hand atrophied progressively, the interphalangeal joint was stiff, wirheut flexion nor extension range of motion. The function of the replanted finger was limited.
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Objective:To investigate the clinical manifestations, pathological characteristics, treatment and prognosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in 13 children.Methods:The clinical and pathological data of 13 cases of AAV in children′s Hospital of Nanjing Medical University from June 2000 to December 2021 were retrospectively analyzed.Results:Among the 13 cases, 12 cases were diagnosed with microscopic polyangiitis (MPA) and 1 case was granulomatosis with polyangiitis (GPA), including 10 females and 3 males. The onset age ranged from 3 years and 11 months to 13 years and 10 months. The most frequently involved organ was the kidney (12 cases, 92.3%), followed by respiratory system (7 cases, 53.8%), skin (5 cases, 38.5%), digestive system (4 cases, 30.8%), nervous system (4 cases, 30.8%) and cardiovascular system (3 cases, 23.1%). There were 10 cases with orthotic anemia, 7 cases with positive antinuclear antibody, and 3 cases with mildly decreased complement C3. Among the 12 children with renal impairment, 9 cases were accompanied by abnormal renal function at the beginning of the disease. Renal biopsy was classified according to the Berden as follows: sclerotic in 5 cases, crescentic 3 cases, focal in 2 cases and mixed in 2 cases. All children were treated with glucocorticoid combined with immunosuppressant. During the follow-up time from 8 months to 128 months, 4 cases acquired complete remission, 8 cases achieved partial remission and 1 case recurred after complete remission, and 7 cases progressed to chronic kidney disease stage 5. Three children with complete remission underwent repeated renal biopsy, including 2 cases of mixed type and 1 case of crescent type initially, and all changed to focal type.Conclusions:AAV in children occurs mainly in school-age female, and most of AAV in children is MPA. The clinical manifestations are various. Most of them have renal damage and anemia, and lung damage is also common. Patients with skin purpura onset may be misdiagnosed as Henoch-Schonlein purpura, and AAV with ANA positive or complement reduction should exclude systemic lupus erythematosus. Once the renal function is abnormal in AAV, especially estimated glomerular filtration rate<60 ml·min -1·(1.73 m 2) -1 and the pathological classification is sclerotic type or crescent type, it is difficult to reverse even after active treatment. Early diagnosis and treatment are very important for AAV.