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Objective: To evaluate the efficacy and safety of an improved overlength biliary stent in the treatment of bile duct stricture. Methods: Prospective randomized controlled study. Patients with bile duct stricture in Peking University International Hospital from February 2016 to June 2021 were randomly divided into the conventional plastic biliary stents (CPBS) group and the improved overlength biliary stents (IOBS) group by envelope random method. CPBS or IOBS were placed after endoscopic retrograde cholangiopancreatography (ERCP) breast catheterization was successfully conducted and the stenosis length was determined by angiography. The incidence of postoperative complications, median patency time of postoperative stent and reoperation rate within 6 months were compared between the two groups, Kaplan-Meier method was used to draw the survival curve, and log-rank test was conducted to evaluate the safety and efficacy of IOBS in the treatment of bile duct stricture. Results: A total of 90 patients were included. There were 45 patients in IOBS group, including 28 males and 17 females, aged (67.2±11.7) years. There were 45 patients in CPBS group, including 26 males and 19 females, aged (64.6±14.4) years. The patients in the both groups were balanced and comparable. There were no significant differences in success rate of operation, operation time, hospitalization time, operation cost and prospective complication rate between the two groups (all P>0.05). There were no significant differences in the changes of liver function index before and 72 hours after operation between the two groups (both P>0.05). The median patency time of stents in IOBS group was longer than that in CPBS group [M(Q1, Q3), 201(155,246) vs 109(55,167) d, P=0.002].The IOBS group had lower reoperation rate than the CPBS group within 6 months [46.2% (18/39) vs 78.9%(30/38), P=0.003]. Conclusion: IOBS has good safety in the treatment of bile duct stricture and the clinical efficacy is superior to CPBS.
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Procedimientos Quirúrgicos del Sistema Biliar , Colestasis , Femenino , Masculino , Humanos , Constricción Patológica , Estudios Prospectivos , Colangiopancreatografia Retrógrada EndoscópicaRESUMEN
To establish and identify induced pluripotent stem cells (iPSCs) derived from patients with Aicardi-Goutières syndrome (AGS) with TREX1 gene 667G>A mutation, and obtain a specific induced pluripotent stem cell model for Aicardi-Goutières syndrome (AGS-iPSCs). A 3-year-old male child with Aicardi-Goutieres syndrome was admitted to Zhongshan People's Hospital in December 2020. After obtaining the informed consent of the patient's family members, 5 ml peripheral blood samples from the patient were collected, and mononuclear cells were isolated. Then,the peripheral blood mononuclear cells(PBMCs) were transduced with OCT3/4, SOX2, c-Myc and Klf4 by using Sendai virus, and PBMCs were reprogrammed into iPSCs. The pluripotency and differentiation ability of the cells were identified by cellular morphological analysis, real-time PCR, alkaline phosphatase staining (AP), immunofluorescence, teratoma formation experiments in mice. The results showed that the induced pluripotent stem cell line of Aicardi-Goutieres syndrome was successfully constructed and showed typical embryonic stem-like morphology after stable passage, RT-PCR showed mRNA expression of stem cell markers, AP staining was positive, OCT4, SOX2, NANOG, SSEA4, TRA-1-81 and TRA-1-60 pluripotency marker proteins were strongly expressed. In vivo teratoma formation experiments showed that iPSCs differentiate into the ectoderm (neural tube like tissue), mesoderm (vascular wall tissue) and endoderm (glandular tissue). Karyotype analysis also confirmed that iPSCs still maintained the original karyotype (46, XY). In conclusion, induced pluripotent stem cell line for Aicardi-Goutières syndrome was successfully established using Sendai virus, which provided an important model platform for studying the pathogenesis of the disease and for drug screening.
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Enfermedades Autoinmunes del Sistema Nervioso , Malformaciones del Sistema Nervioso , Animales , Preescolar , Humanos , Masculino , Ratones , Enfermedades Autoinmunes del Sistema Nervioso/metabolismo , Enfermedades Autoinmunes del Sistema Nervioso/patología , Diferenciación Celular , Línea Celular , Células Madre Pluripotentes Inducidas/metabolismo , Células Madre Pluripotentes Inducidas/patología , Leucocitos Mononucleares , Malformaciones del Sistema Nervioso/metabolismo , Malformaciones del Sistema Nervioso/patologíaRESUMEN
Objective: To investigate the clinicopathological characteristics of pancreatic lesions in children. Methods: The clinicopathological data of pancreatic lesions in children were analyzed including 42 cases of pancreatic tumors diagnosed from January 2000 to May 2021 in Guangzhou Women's and Children's Medical Center, Guangzhou, China. Histological and immunohistochemical assessments were performed. Related literature was reviewed. Results: The 42 pediatric patients with pancreatic lesions aged 1 day to 12 years (mean, 4.25 years). There were 23 males and 19 females. Clinical presentations included abdominal masses, abdominal pain, vomiting and persistent hypoglycemia after birth. Ultrasound and computerized tomography examination showed space-occupying pancreatic lesions in 31 cases, but no detectable pancreatic lesions in 11 cases. Histologically, among the 42 cases, 22 cases (52.4%) were neoplastic, including 18 cases of epithelial origin. Nine cases of pancreatoblastoma showed that the epithelial tumor cells were arranged in a trabecular pattern, with squamous nests. Six cases of solid-pseudopapillary tumors revealed hemorrhagic and necrotic cysts and monomorphic epithelioid cells arranged in solid sheets, nests or pseudopapillae. Two cases of neuroendocrine tumors showed tumor cells arranged in cords or nests; one case had a mitotic count of about 3/10 high power field, and a Ki-67 index of about 5%, which was consistent with G2 neuroendocrine tumor; the other case showed tumor cells with cytological atypia, brisk mitoses, about 25/10 HPF and a Ki-67 index of about 80%, consistent with small-cell type neuroendocrine carcinoma. The case of acinar cell carcinoma showed high cellularity, tumor cells in solid, cord-like or acinar-like arrangement with little stroma, and monotonous tumor cells with single distinct nucleolus. There were 4 cases of mesenchymal tumors, including 3 cases of Kaposi's hemangioendothelioma and 1 case of inflammatory myofibroblastic tumor. Among the 20 cases (47.6%) of non-neoplastic lesions, there were 11 cases of hyperinsulinism with ATP-sensitive potassium channel abnormality (HAPCA). Severn cases of diffuse type HAPCA in which the islets scattered between the pancreatic acinar tissue, enlarged, and prominent nuclei. Three cases of focal type HAPCA showed pancreatic islet hyperplasia in the form of nested nodules (0.6-1.5 cm). One case of atypical type HAPCA had extensive islet hyperplasia in pancreatic tissue, and scattered proliferation of nest-like nodules was noted. There were also 7 cases of pseudocyst and 2 cases of congenital cyst. Immunohistochemically, pancreatoblastomas were diffusely positive for CKpan, CK8/18, and ß-catenin (nuclear staining of squamous nests only). Solid-pseudopapillary tumors expressed CD10, cyclin D1, CD99, vimentin, CD56, and ß-catenin (nuclear staining). Neuroendocrine tumors were positive for CK, Syn, NSE, CgA, CD56, and ß-catenin (membranous staining). The acinar cell carcinoma was positive for CK8/18, trypsin, and ß-catenin (membranous staining). Conclusions: Pancreatic lesions in children have a wide range of histopathological types. HAPCA is the most common lesion of newborns. Pediatric pancreatic tumors are rare and mostly malignant. It is important to recognize them and make correct pathological diagnoses.
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Carcinoma de Células Acinares , Carcinoma de Células Escamosas , Tumores Neuroendocrinos , Neoplasias Pancreáticas , Carcinoma de Células Acinares/patología , Niño , Femenino , Humanos , Hiperplasia , Recién Nacido , Antígeno Ki-67 , Masculino , Neoplasias Pancreáticas/metabolismo , beta Catenina/análisisRESUMEN
Objective: To investigate the clinical features, treatment and prognosis of chronic eosinophilic pneumonia. Methods: Nine patients with chronic eosinophilic pneumonia diagnosed in Shandong Provincial Qianfoshan Hospital from January 2014 to December 2020 were enrolled and followed up. The data of clinically proven chronic eosinophilic pneumonia were reviewed. Results: The 9 cases included one male and eight females, aged from 16 to 71 years (median 47 years). Among them, 5 cases were complicated with asthma, 1 case was complicated with allergic rhinitis, and 1 case had an allergic history of pollen. All the patients had cough, expectoration, chest tightness and wheezing, and a few had fatigue (3/9), fever (1/9) and chest pain (1/9). Single or multiple patchy high-density shadows (9/9), mediastinal lymphadenopathy (7/9), air bronchogram (2/9), and reticular shadow (1/9) were observed in chest CT. Peripheral eosinophils (EOS) and serum total IgE increased to varying degrees in the 9 patients. Meanwhile, the bronchoscopy of 5 cases showed elevated percentage of eosinophils in alveolar lavage fluid, and the lung biopsy of remaining 4 cases showed EOS infiltration in lung alveolar and interstitium. After receiving glucocorticoid therapy for 0.5 to 1 month, the clinical symptoms of all 9 patients had been improved and lung lesions on CT scans had been obviously absorbed. Four cases relapsed during follow-up. Conclusions: For patients especially women who have a history of allergy, elevated blood eosinophils and serum total IgE with pulmonary high-density shadow or consolidation, chronic eosinophilic pneumonia should be considered, and bronchoscopy or percutaneous lung biopsy is indicated for a definite diagnosis. Glucocorticoid therapy is effective, but the rate of recurrence is high.
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Enfermedades Pulmonares , Eosinofilia Pulmonar , Adolescente , Adulto , Anciano , Líquido del Lavado Bronquioalveolar , Eosinófilos , Femenino , Humanos , Pulmón/patología , Enfermedades Pulmonares/patología , Masculino , Persona de Mediana Edad , Eosinofilia Pulmonar/diagnóstico , Eosinofilia Pulmonar/tratamiento farmacológico , Eosinofilia Pulmonar/patología , Adulto JovenRESUMEN
Objective: To investigate the clinicopathological features of gonadal neoplastic related lesions in children with disorders of sexual development (DsD). Methods: The clinical manifestations, chromosomal karyotype, histology and immunophenotype of 12 cases of neoplastic related lesions from Guangzhou Women and Children's Medical Center, Guangzhou were analyzed during Jan 2015 to May 2020. Results: Twelve cases of neoplastic related lesions were screened in 205 cases of DsD, and 6 patients with gonadal germ cell neoplasia aged 3-13 years with an average age of 8.3 years. There were 2 males and 4 females. Clinical features showed malformation of external genitalia in 2 cases, short stature in 2 cases, clitoral enlargement in 1 case, lower abdominal pain and a huge pelvic mass in 1 case. Chromosomal karyotyping of peripheral blood showed 2 cases of 46XY and 4 cases of 45X/46XY. Fourteen gonadal specimens were examined. Microscopically, 1 case showed dysgerminoma in left ovary, and malignant mixed germ cell tumors in right ovary, as well as gonadoblastoma (GB) and undifferentiated gonadal tissue (UGT). The remaining 5 cases were all precursor lesions of germ cell tumor. Six specimens showed GB, 3 of UGT, and 3 specimens showed germ cell neoplasia in situ (GCNIS), one of which was accompanied by intratubular seminoma and 1 was GB with GCNIS. The other 6 patients with DsD were aged from 8 months to 2 years and 5 months, including 5 males and 1 females. Clinical manifestations showed 5 cases of hypospadias and 1 case of bilateral indirect inguinal hernia. Microscopically, 6 cases showed maturation delay of gonocytes in seminiferous tubules. Immunohistochemically, the primordial germ cells/gonocytes expressed OCT3/4, PLAP and c-KIT in the 12 cases. Conclusion: Gonadal neoplasia in children with DsD is mainly precursor lesions of germ cell tumor and improved understanding of these lesions is of great significance.
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Trastornos del Desarrollo Sexual , Gonadoblastoma , Neoplasias de Células Germinales y Embrionarias , Neoplasias Ováricas , Neoplasias Testiculares , Niño , Femenino , Gonadoblastoma/genética , Gonadoblastoma/cirugía , Humanos , MasculinoRESUMEN
Mutations in coiled-coil-helix-coiled-coil-helix-domain containing 10 (CHCHD10), a mitochondrial twin CX9C protein whose function is still unknown, cause myopathy, motor neuron disease, frontotemporal dementia, and Parkinson's disease. Here, we investigate CHCHD10 topology and its protein interactome, as well as the effects of CHCHD10 depletion or expression of disease-associated mutations in wild-type cells. We find that CHCHD10 associates with membranes in the mitochondrial intermembrane space, where it interacts with a closely related protein, CHCHD2. Furthermore, both CHCHD10 and CHCHD2 interact with p32/GC1QR, a protein with various intra and extra-mitochondrial functions. CHCHD10 and CHCHD2 have short half-lives, suggesting regulatory rather than structural functions. Cell lines with CHCHD10 knockdown do not display bioenergetic defects, but, unexpectedly, accumulate excessive intramitochondrial iron. In mice, CHCHD10 is expressed in many tissues, most abundantly in heart, skeletal muscle, liver, and in specific CNS regions, notably the dopaminergic neurons of the substantia nigra and spinal cord neurons, which is consistent with the pathology associated with CHCHD10 mutations. Homozygote CHCHD10 knockout mice are viable, have no gross phenotypes, no bioenergetic defects or ultrastructural mitochondrial abnormalities in brain, heart or skeletal muscle, indicating that functional redundancy or compensatory mechanisms for CHCHD10 loss occur in vivo. Instead, cells expressing S59L or R15L mutant versions of CHCHD10, but not WT, have impaired mitochondrial energy metabolism. Taken together, the evidence obtained from our in vitro and in vivo studies suggest that CHCHD10 mutants cause disease through a gain of toxic function mechanism, rather than a loss of function.
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Proteínas Mitocondriales/genética , Proteínas Mitocondriales/metabolismo , Esclerosis Amiotrófica Lateral/genética , Esclerosis Amiotrófica Lateral/metabolismo , Animales , Proteínas Portadoras , Proteínas de Unión al ADN , Demencia Frontotemporal/genética , Estudios de Asociación Genética , Células HEK293 , Células HeLa , Humanos , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Mitocondrias/genética , Mitocondrias/metabolismo , Proteínas Mitocondriales/química , Modelos Moleculares , Mutación , Dominios y Motivos de Interacción de Proteínas , Mapeo de Interacción de Proteínas , Elementos Estructurales de las Proteínas , Factores de Transcripción/genética , Factores de Transcripción/metabolismoRESUMEN
Objective: The aim of the present study was to evaluate the effects of ethyl acetate extract of Polygonum orientale L. (POE) on ameliorating postmenopausal osteoporosis in ovariectomized (OVX) rats.Methods: Six-month-old female rats were randomly divided into seven groups: sham-operated; OVX; OVX with estradiol valerate; OVX with alendronate; and OVX with POE in graded doses (3.75, 5.0, or 7.5 g/kg/day). Administration began at week 6 after ovariectomy for 12 weeks. A comprehensive assessment of bone quality was performed, including serum biochemical markers, serum inflammatory factors, bone oxidative stress markers, bone mechanics, and bone histomorphometry.Results: POE treatment significantly decelerated OVX-induced body weight gain without affecting the uterus index and produced a significant decrease in the levels of serum bone turnover markers (p < 0.05 or p < 0.01). Biomechanical testing demonstrated that POE (5.0 and 7.5 g/kg/day) treatments significantly prevented the reduction in maximum stress and Young's modulus in OVX rats (p < 0.05). Compared with the OVX group, POE (3.75, 5.0, or 7.5 g/kg/day) treatments significantly increased trabecular bone mineral density by 35.03, 38.42, and 42.02%, respectively.Conclusion: Our findings suggest that POE has potential effects in regulation of bone metabolism and prevention of bone loss in postmenopausal osteoporosis.
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Osteoporosis Posmenopáusica/tratamiento farmacológico , Extractos Vegetales/uso terapéutico , Polygonum , Animales , Densidad Ósea/efectos de los fármacos , Remodelación Ósea/efectos de los fármacos , Modelos Animales de Enfermedad , Femenino , Humanos , Ovariectomía , Fitoterapia , Extractos Vegetales/farmacología , RatasRESUMEN
Purpose: This study aimed to evaluate the effects of irisin on bones of ovariectomized (OVX) mice, to explore a possible treatment for postmenopausal osteoporosis.Methods: The OVX mice were treated with intraperitoneal injections of recombinant irisin (r-irisin) or saline twice a week for 5 weeks. The trabecular bone structure of the femur, the bone strength of the tibia, and serum parameters were assessed.Results: Treatment with r-irisin prevented the trabecular bone loss of the OVX mice. The r-irisin-treated OVX mice exhibited a greater bone microarchitecture, with significantly increased bone mineral density, bone volume to tissue volume ratio, connection density, and trabecular number parameters compared to those of the saline-treated OVX mice. The improved bone microarchitecture induced an increased bone stiffness in r-irisin-treated OVX mice. Consistently, the OVX mice treated with r-irisin showed a significantly increased number of osteoblasts on the trabecular surface and a significantly decreased number of osteoclasts. The r-irisin-treated OVX mice also had a higher osteocalcin level and a lower tartrate-resistant acid phosphatase concentration in serum.Conclusion: Irisin increases osteoblasts and decreases the number of osteoclasts, which leads to the maintenance of bone mass and quality in OVX mice. Irisin likely preserves the bone microarchitecture via building a 'new balance'. Therefore, our study extended the understanding of the role of irisin in bone metabolism and revealed the possibility of therapeutic application of irisin for postmenopausal osteoporosis.
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Densidad Ósea/efectos de los fármacos , Enfermedades Óseas Metabólicas/tratamiento farmacológico , Fibronectinas/farmacología , Animales , Enfermedades Óseas Metabólicas/etiología , Hueso Esponjoso/metabolismo , Modelos Animales de Enfermedad , Femenino , Fémur/metabolismo , Humanos , Inyecciones Intraperitoneales , Ratones , Osteoblastos/efectos de los fármacos , Osteocalcina/sangre , Osteoclastos/efectos de los fármacos , Osteoporosis Posmenopáusica , Ovariectomía/efectos adversos , Fosfatasa Ácida Tartratorresistente/sangre , Tibia/metabolismoRESUMEN
AIM: To develop liver a computed tomography (CT) radiomics model to predict gastro-oesophageal variceal bleeding (GVB) secondary to hepatitis B-related cirrhosis. MATERIALS AND METHODS: Electronic medical records and image data of liver triple-phase contrast-enhanced CT examinations of 295 patients with hepatitis B-related cirrhosis were collected retrospectively from two hospitals. Two hundred and thirty-six and 59 patients were enrolled randomly into the training and validation cohorts, respectively; and 75 in the training cohort and 16 in the validation cohort endured GVB while the others did not during follow-up period. Radiomics features of the liver were extracted from the portal venous phase images, and clinical features came from medical records. The tree-based method and univariate feature selection were used to select useful features. The radiomics model, clinical model, and integration of radiomics and clinical models were built using the useful image features and/or clinical features. Predicting performance of three models was evaluated with the area under receiver-operating characteristic curve (AUC), accuracy, and F-1 score. RESULTS: Twenty-one useful radiomics features and/or three clinical features were selected to build prediction models that correlated with GVB. AUC of integration of radiomics and clinical models was larger than of clinical or radiomics models for the training cohort (0.83±0.09 versus 0.64±0.08 or 0.82±0.10) and the validation cohort (0.64 versus 0.61 or 0.61). Integration of radiomics and clinical models obtained good performance in predicting GVB for both the training and validation cohorts (accuracy: 0.76±0.07 and 0.73, and F-1 score: 0.77±0.09 and 0.72, respectively). CONCLUSION: Integration of the radiomics and clinical models may be a non-invasive method to predict GVB.
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Várices Esofágicas y Gástricas/etiología , Hemorragia Gastrointestinal/etiología , Hepatitis B/complicaciones , Tomografía Computarizada por Rayos X , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Várices Esofágicas y Gástricas/diagnóstico por imagen , Femenino , Hemorragia Gastrointestinal/diagnóstico por imagen , Hepatitis B/diagnóstico por imagen , Humanos , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico por imagen , Cirrosis Hepática/etiología , Masculino , Persona de Mediana Edad , Modelos Estadísticos , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos , Adulto JovenRESUMEN
Identifying the Single Nucleotide Polymorphisms (SNPs) with functions in insect fecundity promises to provide novel insight into genetic mechanisms of adaptation and to aid in effective control of insect populations. We previously identified several SNPs within the vitellogenin (Vg) promoter region between a high-fecundity population (HFP) and a low-fecundity population (LFP) of the brown planthopper, Nilaparvata lugens Stål (Hemiptera: Delphacidae). Here, we found that an A-to-T (HFP allele to LFP allele) transversion at nucleotide -953 upstream of Vg in a Nilaparvata lugens GATA-1 (NlGATA-1) binding motif is associated with the level of Vg transcription. We also characterized NlGATA-1, containing a double CX2 CX17 CX2 C zinc finger, which has been implicated in the activation of Vg gene expression. Knockdown of the NlGATA-1 gene results in a reduced basal level of expression of the Vg gene and fewer offspring of N. lugens in vivo, whereas overexpression of NlGATA-1 in cells increased Vg promoter activity. Moreover, upon cotransfection with NlGATA-1 expression vector, the luciferase activities of Vg reporter vectors with the A allele were significantly higher than those with the T allele. These findings support a mechanism in which a SNP within the promoter of Vg is associated with the level of Vg transcription by altering the binding activity of NlGATA-1 and subsequently affecting fecundity in N. lugens.
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Factor de Transcripción GATA1/genética , Regulación de la Expresión Génica/genética , Hemípteros/fisiología , Proteínas de Insectos/genética , Polimorfismo de Nucleótido Simple , Vitelogeninas/genética , Animales , Secuencia de Bases , Fertilidad/genética , Factor de Transcripción GATA1/metabolismo , Hemípteros/genética , Hemípteros/metabolismo , Proteínas de Insectos/metabolismo , Vitelogeninas/metabolismoRESUMEN
The first family of extended and fluorinated corannulenes is prepared through a highly efficient and modular synthetic strategy. In this strategy, corannulene aldehyde could be combined with the fluorine-carrying phosphonium ylides to furnish stilbene-like vinylene precursors. A photochemically induced oxidative cyclization process of these precursors gives rise to the fluorinated and curved polycyclic aromatic hydrocarbons. A UV-vis absorption study shows that aromatic extension results in a bathochromic shift of about 12 nm. Fluorination further shifts the absorption spectrum to the red region, and a maximum shift of about 22 nm is detected for a compound carrying two trifluoromethyl groups. A cyclic and square-wave voltammetry investigation reveals that the extension of the corannulene scaffold increases the reduction potential by 0.11 V. Placement of fluorine or trifluoromethyl groups further enhances the electron affinities. In this regard, the presence of one trifluoromethyl group equals the effect of three aromatic fluorine atoms. Molecules with two trifluoromethyl groups, meanwhile, exhibit the highest reduction potentials of -1.93 and -1.83 V. These values are 0.37 and 0.46 V higher than those of the parental corannulene and demonstrate the utility of the present design concept by efficiently accessing effective electron acceptors based on the buckybowl motif.
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Some fire ants of the genus Solenopsis have become invasive species in the southern United States displacing native species by competition. Although the displacement pattern seems clear, the mechanisms underlying competitive advantage remain unclear. The ability of ant workers to produce relatively larger amount of alarm pheromone may correspond to relative greater fitness among sympatric fire ant species. Here we report on quantitative intra-specific (i.e. inter-caste) and inter-specific differences of alarm pheromone component, 2-ethyl-3,6-dimethylpyrazine (2E36DMP), for several fire ant species. The alarm pheromone component was extracted by soaking ants in hexane for 48 h and subsequently quantified by gas chromatography-mass spectrometry at single ion monitoring mode. Solenopsis invicta workers had more 2E36DMP than male or female alates by relative weight; individual workers, however, contained significantly less pyrazine. We thus believe that alarm pheromones may serve additional roles in alates. Workers of Solenopsis richteri, S. invicta, and hybrid (S. richteri × S. invicta) had significantly more 2E36DMP than a native fire ant species, Solenopsis geminata. The hybrid fire ant had significantly less 2E36DMP than the two parent species, S. richteri and S. invicta. It seems likely that higher alarm pheromone content may have favored invasion success of exotic fire ants over native species. We discuss the potential role of inter-specific variation in pyrazine content for the relationship between the observed shifts in the spatial distributions of the three exotic fire ant species in southern United States and the displacement of native fire ant species.
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Hormigas/química , Feromonas/química , Animales , Hormigas/metabolismo , Femenino , Cromatografía de Gases y Espectrometría de Masas , Especies Introducidas , Masculino , Feromonas/metabolismo , Especificidad de la EspecieRESUMEN
Objective: To investigate the pathologic features of gonadal tissues of disorders of sexual development (DSD) in children. Methods: Fifty-three cases of gonadal developmental disorders were collected from July 2015 to August 2017 at Guangzhou Women and Children's Medical Center. Clinical manifestations, karyotypes, sex hormone levels, ultrasound imaging, histology and immunophenotype of gonadal tissues were analyzed. Results: The age of patients ranged from 7 months to 17 years with an average of (50.7 ± 47.1) months. Social genders of the patients included 32 males and 21 females. Forty-eight patients had abnormal sex hormone levels. Clinical presentations included: toward female genitalia in 25 cases, male genitalia tendency in 17 cases and ambiguous external genitalia in 11 cases. Hypospadias was seen in 31 cases and short stature was seen in 8 cases. Chromosomal karyotyping of peripheral blood revealed 23 cases of sex chromosome disorders, 22 cases of 46 XY disorders, of which 3 cases were 5α-reductase deficiency and 8 cases of 46 XX disorders. Ultrasound examination showed cryptorchidism in 30 cases, including 16 cases of unilateral, 14 cases of bilateral and 1 case presenting a huge pelvic tumor. A total of 97 gonadal tissues from 53 cases of DSD were examined, including 9 cases of unilateral and 44 cases of bilateral gonads. Microscopically, 55 gonads (56.7%) showed dysplastic testes including 17 unilateral and 19 bilateral gonads. Fourteen were streak gonads (14.4%) including 8 unilateral and 3 bilateral gonadal tissues. Nine streak gonad with epithelial cord-like structures (9.3%) were found, of which 5 were unilateral and 2 were bilateral lesions. Seven gonads were ovotestis (7.2%), unilateral in 5 cases (the other side of the gonads of ovary in 4 cases, 1 case of dysplastic testes) and bilateral in 1 case. Seven gonads showed follicular-rich ovarian tissue (7.2%). One case showed bilateral dysplastic testes with gonadoblastoma and ectopic adrenal cortex. One case of streak gonad showed epithelial cord-like structures and undifferentiated glandular tissue embedded in malignant mixed germ cell tumors (mixed gonadoblastoma, dysgerminoma, mature teratoma and yolk sac tumor). One case had testicular microlithiasis. Uterus and fallopian tube structures were found in 11 cases. Immunohistochemical stains were performed in 15 cases. D2-40, PLAP and CKIT were expressed in germ cells and Calretinin, WT1 and inhibin were positive in Setoli cells. SALL4 and OCT3/4 were positive in 3 cases. Inhibin highlighted interstitial Leydig cells in 2 cases. GPC3 was positive in yolk sac tumor component. Conclusions: Gonadal dysgenesis presents a broad spectrum of gonadal phenotypes with variable degrees of differentiation. The development of bilateral gonadal tissues has certain variability. Chromosomal karyotypes have no correlation with gonadal phenotypes. Accurate histopathologic diagnosis of gonadal dysgenesis plays an important role in the treatment and prognosis of the patient.
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Trastornos del Desarrollo Sexual/patología , 3-Oxo-5-alfa-Esteroide 4-Deshidrogenasa/deficiencia , Adolescente , Cálculos/patología , Niño , Preescolar , Trastorno del Desarrollo Sexual 46,XY , Trompas Uterinas/patología , Femenino , Humanos , Hipospadias/patología , Lactante , Cariotipificación , Masculino , Neoplasias de Células Germinales y Embrionarias , Ovario/anomalías , Ovario/patología , Errores Congénitos del Metabolismo Esteroideo , Teratoma/patología , Enfermedades Testiculares/patologíaRESUMEN
BACKGROUND: The present study aimed to evaluate the effect of soy or isoflavones on blood homocysteine levels via a systematic review and meta-analysis. METHODS: Pubmed, Embase, Web of Science and the Cochrane Library (up to 16 December 2015) were used for the literature review. Only randomised controlled trials were included. The primary outcome was the standard mean difference (SMD) of blood homocysteine levels between the experimental and control groups. RESULTS: Nineteen randomised controlled studies were included for qualitative analysis. Eighteen studies were included in the data synthesis. Soy or isoflavones were found to have no effect on homocysteine levels, with a SMD of -0.21 (95% confidence interval = -0.43 to 0.00, I2 =67.7%, random effect model). No publication bias was found among those studies (P = 0.296 for Egger's test, and P = 0.198 for Begg's test). CONCLUSIONS: Soy or isoflavones were not found to be associated with a reduction in homocysteine levels. Further studies might still be needed in carefully selected populations.
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Homocisteína/sangre , Isoflavonas/efectos adversos , Alimentos de Soja/efectos adversos , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Ensayos Clínicos Controlados Aleatorios como Asunto , Adulto JovenRESUMEN
OBJECTIVE: To compare enhancement of the central auditory pathway in cats receiving auditory stimulation between manganese enhanced magnetic resonance imaging (MEMRI) with intraperitoneal manganese injection route and MEMRI with intratympanic manganese injection route, and investigate the optimal method for displaying enhancement of the central auditory pathway. METHODS: Twenty-seven normal hearing adult cats were randomly divided into three groups, receiving intraperitoneal manganese injection, left intratympanic manganese injection or left intratympanic gadolinium injection respectively.All cats received white noise stimulation of 80 dB in twenty-four hours after injection.Three dimensionally coronal T1-weighted imaging of the cat brain was obtained with an animal dedicated MRI scanner.The signal noise ratios (SNRs) of bilateral cochlear nuclei (CN), dorsal nuclei of the trapezoid bodies (DNTB), caudal colliculi (CC) and auditory cortices (AC) were measured on reconstructed images and compared. RESULTS: Obvious increased SNRs on both sides were shown in intraperitoneal mangasese injection group while left predilection was shown in intratympanic manganese injection group: left CN 45.7±6.0, 37.4±11.9, 23.9±2.7, F=17.694, P=0.000; left DNTB 50.5±11.2, 37.1±11.2, 27.6±7.3, F=11.781, P=0.000; left CC 37.6±3.9, 22.6±3.1, 17.9±0.7, F=111.898, P=0.000; left AC 27.7±2.5, 17.3±2.3, 14.5±1.0, F=105.132, P=0.000; right CN 42.7±8.3, 23.9±3.0, 22.7±2.1, F=41.492, P=0.000; right DNTB 44.1±8.3, 21.9±3.0, 23.9±4.0, F=27.862, P=0.000; right CC 38.0±4.0, 21.9±3.0, 17.6±0.9, F=120.032, P=0.000; right AC 26.7±3.4, 17.1±2.9, 14.9±1.3, F=64.587, P=0.000.Compared with the left intratympanic gadolinium injection group, the intraperitoneal manganese injection group showed higher SNRs in bilateral CN and CC (P<0.05), and the left intratympanic manganese injection group showed higher SNRs in left CN, AC and bilateral CC.The SNRs of right CN, bilateral DNTB, CC and AC were significantly higher in the intraperitoneal manganese injection group than those in the left intratympanic manganese injection group (P<0.05). CONCLUSION: MEMRI with intraperitoneal manganese injection might be the optimal method for displaying enhancement of the central auditory pathway in cats receiving auditory stimulation.
Asunto(s)
Corteza Auditiva/diagnóstico por imagen , Vías Auditivas , Procesamiento de Imagen Asistido por Computador , Colículos Inferiores/diagnóstico por imagen , Imagen por Resonancia Magnética , Manganeso/administración & dosificación , Estimulación Acústica , Animales , Gatos , Gadolinio/administración & dosificación , Inyección Intratimpánica , Inyecciones Intraperitoneales , Ruido , Distribución AleatoriaRESUMEN
Objective: To compare perioperative outcome of carotid endarterectomy (CEA) between diabetic and non-diabetic patients and discuss the safety of CEA in diabetic patients. Methods: A total of 572 patients with carotid stenosis who underwent CEA from Juanuary 2006 to September 2014 in Peking Union Medical Colloge Hospital were collected and analyzed retrospectively. The patients were distributed into diabetes mellitus (DM) group and non-DM group according to the comorbidities. Their baseline characteristics, intraoperative index and major complications in perioperative period were analyzed and compared. Results: Of 572 cases, 184 had DM (32.2%). Diabetic patients more frequently had a history of coronary artery disease (30.4% vs 18.6%), peripheral artery disease (39.7% vs 27.3%), and hyperlipemia (50.5% vs 36.6%) than non-diabetic patients (all P<0.05). The incidence of perioperative myocardial infarction (0.5% vs 0.8%), stroke (1.6% vs 1.8%) and death (0.5% vs 0.3%) did not differ between two groups (all P>0.05). Data of follow-up was available in 89.6% of patients, with a median duration of 42 months. The incidence of myocardial infarction (4.3% vs 3.4%), stroke (1.8% vs 1.7%), death (1.1% vs 0.9%), and restenosis (2.9% vs 3.1%) did not differ between two groups (all P>0.05). Conclusion: DM does not increase the risk of perioperative complications and impair the long-term outcomes after CEA if the patients are appropriately evaluated and treated in perioperative period.
Asunto(s)
Diabetes Mellitus , Endarterectomía Carotidea , Estenosis Carotídea , Comorbilidad , Enfermedad de la Arteria Coronaria , Complicaciones de la Diabetes , Humanos , Incidencia , Infarto del Miocardio , Enfermedad Arterial Periférica , Estudios Retrospectivos , Factores de Riesgo , Seguridad , Accidente Cerebrovascular , Resultado del TratamientoRESUMEN
OBJECTIVE: To evaluate the prevalence of asymptomatic carotid artery stenosis in patients with arteriosclerosis obliterans of lower extremities, and evaluate its effect on the treatment strategies for lower extremity artery lesions. METHODS: Totally 348 patients with arteriosclerosis obliterans of lower extremities who had no cerebrovascular symptoms or events in the past were enrolled from September 2012 to September 2014 in the Department of Vascular Surgery, Peking Union Medical College Hospital. Preoperative color doppler ultrasonography results of carotid arteries and demographic characteristics were retrospectively collected. The peak systolic velocity and the presence of plaque on gray-scale in the internal carotid arteries were used for diagnosing and grading internal carotid artery stenosis. A stenosis ≥50% was diagnosed as carotid artery stenosis. Binary logistic regression analysis was used to evaluate related risk factors. Treatment strategies for the artery lesions of lower extremities were compared between those with and without asymptomatic carotid artery stenosis. RESULTS: Seventy-seven (22.1%) patients were found to have carotid artery stenosis. Forty-four (12.6%) patients were found to have a stenosis ≥70% or occlusion. Smoking (OR=2.122, 95% CI: 1.143-3.940), coronary artery disease (OR=1.939, 95% CI: 1.058-3.552) and hypertension (OR=1.882, 95% CI: 1.025-3.457) were found to be related risk factors. In patients combined with asymptomatic carotid stenosis, open surgery with general anesthesia was less frequently used than those without asymptomatic carotid stenosis (6.5% vs 18.1%, χ(2)=6.142, P=0.013). CONCLUSIONS: Prevalence of asymptomatic carotid artery stenosis is high in patients with arteriosclerosis obliterans of lower extremities. Smoking, coronary artery disease and hypertension are related risk factors.
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Arteriosclerosis Obliterante , Estenosis Carotídea , Arteria Carótida Interna , Constricción Patológica , Enfermedad de la Arteria Coronaria , Análisis Factorial , Humanos , Hipertensión , Extremidad Inferior , Prevalencia , Factores de Riesgo , Ultrasonografía Doppler en ColorRESUMEN
Objective: To summarize results of endovascular treatment for auto-immune disease related abdominal aorta pseudo-aneurysm(AIPA), and to analysis clinical predictors of long term major adverse clinical events(MACE). Methods: Retrospectively collected endovascular treatment for AIPA cases in Peking Union Medical College Hospital within 2000 to 2015. Twenty-nine cases with AIPA treated by endovascular therapy were enrolled in this study. Twenty five cases were male, range from 23 to 67 years old, mean age was (39.3±11.4) years old.Demographic characters, locations of aneurysms, type to auto-immune disease, immuno medical therapy, operation strategy and long term follow-up data were reported. Statistical analysis was made to verify clinical predictors of long-term MACE. Results: Among the 29 cases, 22 cases with bechet's disease, 4 cases with Takayasu's arteritis, 2 cases with systemic lupus erythematosus, 1 cases with polyarteritis nodosa. Eight cases had ruptured or pending ruptured pesudo-aneurysms, the rest 21 cases had dull pain or no overt symptome. Twenty-four cases had infra-renal artery aneurysms, two were para-and supra-renal artery, two were supra-celiac artery, and the rest one had multiple aneurysms involved thoracic and abdominal aorta.All the cases received regular immune medical therapy except the three emergency cases. All the operations were under general anaesthesia. Nineteen cases underwent classical Endovascular aortic aneurysm repair (EVAR), 5 cases underwent fenestration EVAR, the rest 5 cases underwent hybrid procedure. All the 29 operations were successful, without conversion to open surgery. Major peri-operation complication included 3 incision infection, 3 pulmonary infection. No death occurred. All the cases received regular follow-up from 1 to 120 months. There were five recurrence of pseudo-aneurysm, 1 case suffered from iliac limb occlusion. 5 cases received re-intervention procedure. No occlusion of revascularizal visceral artery was found during follow-up. There were 3 deaths during follow-up, with 1 aneurysm related death, the rest died due to other reasons. Single factors logical regressions analysis showed discontinuing immune medicine therapy and age no less than 40 years significantly related long-term MACE(P<0.05). Meanwhile, type of original auto-immune disease, none classical EVAR were not significant related to MACE. Conclusions: Endovascular therapy is safe and effective for AIPA. Regular peri-operation and long-term immunotherapy is key to success.
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Aorta Abdominal , Aneurisma de la Aorta Abdominal , Adulto , Anciano , Procedimientos Endovasculares , Femenino , Humanos , Enfermedades del Sistema Inmune , Masculino , Persona de Mediana Edad , Procedimientos de Cirugía Plástica , Arteria Renal , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To study the clinicopathologic features of pediatric vascular anomalies and application of ISSVA classification. METHODS: The clinical features, histopathologic findings and immunohistochemical results were analyzed in 117 cases of pediatric vascular anomalies encountered during the period from May 2014 to May 2015. RESULTS: A total of 117 cases of vascular anomalies were studied. The age of patients ranged from 18 hours after birth to 11 years (mean age =34 months and median age =27 months). There were 73 male patients and 44 female patients, with the male-to-female ratio being 1.7â¶1.0. Congenital skin lesions were found in 37 cases (31.6%). The common sites of involvement included head and neck region (46 cases, 39.3%), trunk (28 cases, 23.9%), extremities (14 cases, 12.0%) and internal viscera (31 cases, 26.5%). According to the new ISSVA classification, there were 74 cases of vascular malformations and 43 cases of vascular neoplasms (ratio=1.7â¶1.0). The commonest vascular tumor encountered was infantile hemangioma (21 cases, 48.8%), including 17 cases in proliferative phase and 4 cases in involutive phase. Thirteen cases (23.3%) of congenital hemangioma were found, with 8 cases of rapidly involuting congenital hemangioma and 5 cases of non-involutive congenital hemangioma. Three of the congenital hemangioma occurred in liver. There were 5 cases (11.6%) of pyogenic granuloma, 3 cases (7.0%) of tufted angioma and 1 case (2.3%) of Kaposiform hemangioendothelioma. Amongst the 74 cases of vascular malformations encountered, lymphatic malformation was found in 47 cases (63.5%), venous malformation in 15 cases (20.2%), lymphatic-venous malformation in 11 cases (14.9%) and arteriovenous malformation in 1 case (1.4%). All cases of vascular anomalies were all positive for CD31 on immunostaining. Glut1 and CD15 were positive both in proliferative and involutive phases of the 21 cases of infantile hemangioma, while other vascular tumors and vascular malformations were negative. Forty-seven cases of lymphatic malformation and 11 cases of lymphatic-venous malformation showed D2-40 expression. Focal positivity for D2-40 was demonstrated in 3 cases of tufted angioma and 1 case of Kaposiform hemangioendothelioma. CONCLUSIONS: Vascular anomalies affecting infants and children include tumors and malformations. Accurate histopathologic diagnosis and ISSVA classification of the various types of vascular anomalies play an important role in clinical management.