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Objective: To analyze the HIV positive detection rate from different detection channels in Chinese medical institutions. Methods: A Meta-analysis was conducted. First of all, the literature on HIV testing of medical institutions in China was systematically searched on China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, VIP Information Chinese journal Service platform and PubMed. Secondly, a self-made information table was used to collect the basic information, HIV positive number and test number of the literature. Finally, R 4.0.2 software was used to calculate the pooled HIV detection rate and 95%CI of the whole population, detection approaches subgroups and regions subgroups, and then the forest map was drawn. Funnel plot was used to analyze publication bias. Results: A total of 45 studies which covered 22 provinces. Meta analysis showed that the pooled HIV positive rate was 0.82 (95%CI: 0.62-1.04). Subgroup analysis showed that the HIV positive rate of STD outpatient was the highest (3.01 (95%CI: 1.76-4.58), followed by other patients (1.43 (95%CI: 1.00-1.93)). The HIV positive rate of western China was the highest (1.14 (95%CI: 0.72-1.63)). The HIV positive rate in 2008-2017 was higher than in 2000-2007. The Egger test indicated no publication bias (t=-0.737, P=0.465). Conclusion: The HIV positive detection rate of patients in medical institutions in China was at a low level, but the positive rate of patients in STD clinics was relatively high. Therefore, the HIV testing should be further expanded in this population. Secondly, HIV screening should be strengthened for other patients.
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Infecciones por VIH , Tamizaje Masivo , Pueblo Asiatico , China , Infecciones por VIH/diagnóstico , HumanosRESUMEN
This study investigated the effects of dietary Enteromorpha powder supplementation on the productive performance, egg quality, and antioxidant performance of Zi geese during the late laying period. Three hundred twelve Zi geese (1 yr old) were randomly allocated into 2 cohorts to form a control group and an experimental group (with each cohort including 6 replicates and 21 female geese and 5 male geese in each replicate). The control group was fed a basal diet, and the experimental group was fed a diet containing 3% Enteromorpha powder. The data showed that Enteromorpha powder supplementation significantly improved egg production, laying rate, average daily egg weight (P < 0.01), and egg yolk color (P < 0.05). Supplementation decreased the ADFI and feed conversion rate (P < 0.01). Compared with the control group, glutathione peroxidase (GSH-Px) activity was significantly higher in serum and ovary tissue (P < 0.05), but GSH-Px activity was lower in liver tissue (P < 0.01). Malondialdehyde was reduced in liver and ovary tissue (P < 0.05) in the Enteromorpha powder supplementation group. Meanwhile, the expression of the CAT gene was significantly upregulated in the liver (P < 0.01) in the Enteromorpha group. These results indicate that dietary Enteromorpha powder supplementation improved productive performance and reduced the level of lipid peroxidation in Zi geese during the late laying period.
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Alimentación Animal/análisis , Antioxidantes/metabolismo , Gansos/fisiología , Óvulo/fisiología , Reproducción , Ulva/química , Animales , Dieta/veterinaria , Suplementos Dietéticos/análisis , Relación Dosis-Respuesta a Droga , Óvulo/efectos de los fármacos , Polvos/administración & dosificación , Polvos/metabolismo , Distribución Aleatoria , Reproducción/efectos de los fármacosRESUMEN
UNLABELLED: Essentials Nonacog beta pegol is a recombinant glycoPEGylated factor IX with an extended half-life. This phase 3 trial investigated its safety/efficacy in previously treated hemophilia B boys ≤ 12 years. A 40 IU kg(-1) dose provided effective once-weekly prophylaxis and hemostasis when used to treat bleeds. Nonacog beta pegol was well tolerated in previously treated boys ≤ 12 years with hemophilia B. SUMMARY: Background Nonacog beta pegol is a recombinant glycoPEGylated factor IX with an extended half-life, developed to improve care for patients with hemophilia B. Objectives To investigate the safety, efficacy and pharmacokinetics of nonacog beta pegol for the prophylaxis and treatment of bleeds in previously treated children with hemophilia B. Patients/Methods This phase 3 trial, paradigm(™) 5, enrolled and treated 25 children (aged ≤ 12 years) with hemophilia B (FIX ≤ 2%). Patients were stratified by age (0-6 years and 7-12 years), and received once-weekly prophylaxis with 40 IU kg(-1) nonacog beta pegol for 50 exposure days. Results No patient developed inhibitors, and no safety concerns were identified. Forty-two bleeds in 15 patients were reported to have been treated; the overall success rate was 92.9%, and most bleeds (85.7%) resolved after one dose. The median annualized bleeding rates (ABRs; bleeds per patient per year) were 1.0 in the total population, 0.0 in the 0-6-year group, and 2.0 in the 7-12-year group; the estimated mean ABRs were 1.44 in the total population, 0.87 in the 0-6-year group, and 1.88 in the 7-12-year group. For 22 patients who had previously been receiving prophylaxis, the estimated mean ABR was 1.38 versus a historical ABR of 2.51. Estimated mean steady-state FIX trough levels were 0.153 IU mL(-1) (0-6 years) and 0.190 IU mL(-1) (7-12 years). Conclusion Nonacog beta pegol was well tolerated in previously treated children with hemophilia B; a 40 IU kg(-1) dose provided effective once-weekly prophylaxis and hemostasis when bleeds were treated.
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Factor IX/farmacocinética , Hemofilia B/tratamiento farmacológico , Polietilenglicoles/farmacocinética , Peso Corporal , Niño , Preescolar , Esquema de Medicación , Factor IX/uso terapéutico , Hemofilia B/sangre , Hemofilia B/metabolismo , Hemorragia , Hemostasis , Humanos , Lactante , Recién Nacido , Masculino , Polietilenglicoles/uso terapéutico , Estudios Prospectivos , Proteínas Recombinantes/farmacocinética , Proteínas Recombinantes/uso terapéuticoRESUMEN
BACKGROUND: The aims of our study were to evaluate (i) the relationship between cardiac T2* values and cardiac complications in Asian ß-thalassaemia major (TM) patients, and (ii) the association between cardiac T2* values and other parameters currently used to predict cardiac complications as a result of transfusion iron overload. METHODS: We examined the myocardial iron loads of 88 TM patients from Taiwan with cardiac T2* magnetic resonance imaging (MRI) and assessed the correlation between cardiac T2* values and serum ferritin levels, liver iron concentration and left ventricular ejection fraction (LVEF). We also determined the predictive value of these measurements for the development of arrhythmia. RESULTS AND CONCLUSION: In our group of Taiwanese patients, the relative risk for arrhythmia was 10·36 when cardiac T2* values were less than 10 ms (compared with ≥10 ms) and 1·98 when serum ferritin levels increased >2500 ng mL(-1) (compared with ≤2500 ng mL(-1) ). Serum ferritin levels correlated with cardiac T2* values in patients with abnormal myocardial iron loads (T2* < 20 ms, r = -0·48, P = 0·004, n = 34), but LVEF (measured by echocardiography) gave no indication of excess myocardial iron deposition (r = -0·07, P = 0·52) or of the risk of developing arrhythmia.
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Hierro/metabolismo , Miocardio/metabolismo , Talasemia beta/metabolismo , Adolescente , Adulto , Arritmias Cardíacas/diagnóstico por imagen , Arritmias Cardíacas/etiología , Arritmias Cardíacas/metabolismo , Terapia por Quelación , Niño , Femenino , Ferritinas/sangre , Humanos , Imagen por Resonancia Magnética , Masculino , Miocardio/patología , Radiografía , Factores de Riesgo , Taiwán , Talasemia beta/complicaciones , Talasemia beta/diagnóstico por imagenRESUMEN
OBJECTIVE: To investigate anti-müllerian hormone (AMH) as a best test of ovarian reserve in women with transfusion-dependent ß-thalassaemia, and the relationship between AMH and iron overload. DESIGN AND SETTING: A case-control study in a tertiary medical centre. POPULATION: Twenty-nine women with transfusion-dependent ß-thalassaemia and 29 healthy controls of a similar age were recruited. METHODS: Blood sampling, questionnaires and medical record reviews were used. MAIN OUTCOME MEASURES: The history of iron overload-related morbidities, haematological phenotypes, serum levels of AMH and ferritin, and hormonal profiles were analysed. RESULTS: The serum levels of AMH, luteinising hormone, and estradiol were lower in women with transfusion-dependent ß-thalassaemia than in age-matched normal controls. In women with transfusion-dependent ß-thalassaemia, the serum AMH level was significantly inversely related to the ferritin level, but not related to the presence of hypogonadotrophic hypogonadism, diabetes and haematological phenotypes. The serum ferritin level was positively associated with advanced age and the presence of hypogonadotrophic hypogonadism in the study participants. However, the inverse relationship between AMH and ferritin still exists after further adjustment for advanced age in women with transfusion-dependent ß-thalassaemia. CONCLUSIONS: The present study indicates that the serum AMH levels in women with transfusion-dependent ß-thalassaemia are lower when compared with normal healthy women of a similar age, and are significantly negatively correlated with their serum ferritin levels. This implies that ovarian function might be impaired by the chronic iron overload status in women with transfusion-dependent ß-thalassaemia.
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Hormona Antimülleriana/sangre , Transfusión Sanguínea , Sobrecarga de Hierro/sangre , Talasemia beta/sangre , Talasemia beta/terapia , Adolescente , Adulto , Hormona Antimülleriana/deficiencia , Biomarcadores/sangre , Estudios de Casos y Controles , Niño , Femenino , Ferritinas/sangre , Hospitales Universitarios , Humanos , Valor Predictivo de las Pruebas , Pronóstico , Factores de Riesgo , Sensibilidad y Especificidad , Encuestas y CuestionariosRESUMEN
Interferons (IFNs) are crucial for host defence against viruses. Many IFN-stimulated genes (ISGs) induced by viral infection exert antiviral effects. Microarray analysis of gene expression induced in liver tissues of mice on dengue virus (DENV) infection has led to identification of the ISG gene ISG12b2. ISG12b2 is also dramatically induced on DENV infection of Hepa 1-6 cells (mouse hepatoma cell line). Here, we performed biochemical and functional analyses of ISG12b2. We demonstrate that ISG12b2 is an inner mitochondrial membrane (IMM) protein containing a cleavable mitochondrial targeting sequence and multiple transmembrane segments. Overexpression of ISG12b2 in Hepa 1-6 induced release of cytochrome c from mitochondria, disruption of the mitochondrial membrane potential, and activation of caspase-9, caspase-3, and caspase-8. Treatment of ISG12b2-overexpressing Hepa 1-6 with inhibitors of pan-caspase, caspase-9, or caspase-3, but not caspase-8, reduced apoptotic cell death, suggesting that ISG12b2 activates the intrinsic apoptotic pathway. Of particular interest, we further demonstrated that ISG12b2 formed oligomers, and that ISG12b2 was able to mediate apoptosis through both Bax/Bak-dependent and Bax/Bak-independent pathways. Our study demonstrates that the ISG12b2 is a novel IMM protein induced by IFNs and regulates mitochondria-mediated apoptosis during viral infection.
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Apoptosis , Virus del Dengue/metabolismo , Dengue/metabolismo , Hígado/metabolismo , Membranas Mitocondriales/metabolismo , Proteínas Mitocondriales/metabolismo , Animales , Inhibidores de Caspasas , Caspasas/genética , Caspasas/metabolismo , Línea Celular Tumoral , Dengue/genética , Células HEK293 , Humanos , Interferones/genética , Interferones/metabolismo , Hígado/virología , Ratones , Ratones Noqueados , Inhibidores de Proteasas/farmacología , Proteína Destructora del Antagonista Homólogo bcl-2/genética , Proteína Destructora del Antagonista Homólogo bcl-2/metabolismo , Proteína X Asociada a bcl-2/genética , Proteína X Asociada a bcl-2/metabolismoRESUMEN
The long-term outcome of 1390 children with acute lymphoblastic leukemia (ALL), treated in two successive clinical trials (Taiwan Pediatric Oncology Group (TPOG)-ALL-97 and TPOG-ALL-2002) between 1997 and 2007, is reported. The event-free survival improved significantly (P=0.0004) over this period, 69.3+/-1.9% in 1997-2001 to 77.4+/-1.7% in 2002-2007. A randomized trial in TPOG-97 testing L-asparaginase versus epidoxorubicin in combination with vincristine and prednisolone for remission induction in standard-risk (SR; low-risk) patients yielded similar outcomes. Another randomized trial, in TPOG-2002, showed that for SR patients, two reinduction courses did not improve long-term outcome over one course. Decreasing use of prophylactic cranial irradiation in the period 1997-2008 was not associated with increased rates of CNS relapse, prompting complete omission of prophylactic cranial irradiation from TPOG protocols, beginning in 2009. Decreased use of etoposide and cranial irradiation likely contributed to the low incidence of second cancers. High-risk B-lineage ALL, T-cell, CD10 negativity, t(9;22), infant, and higher leukocyte count were consistently adverse factors, whereas hyperdiploidy >50 was a consistently favorable factor. Higher leukocyte count and t(9;22) retained prognostic significance in both TPOG-97 and TPOG-2002 by multivariate analysis. Although long-term outcome in TPOG clinical trials is comparable with results being reported worldwide, the persistent strength of certain prognostic variables and the lower frequencies of favorable outcome predictors, such as ETV6-RUNX1 and hyperdiploidy >50, in Taiwanese children warrant renewed effort to cure a higher proportion of patients while preserving their quality of life.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Recurrencia Local de Neoplasia/terapia , Neoplasias Primarias Secundarias/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Niño , Preescolar , Aberraciones Cromosómicas , Terapia Combinada , Irradiación Craneana , Femenino , Estudios de Seguimiento , Humanos , Inmunofenotipificación , Lactante , Masculino , Recurrencia Local de Neoplasia/genética , Recurrencia Local de Neoplasia/patología , Neoplasia Residual , Neoplasias Primarias Secundarias/genética , Neoplasias Primarias Secundarias/patología , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología , Pronóstico , Inducción de Remisión , Factores de Riesgo , Tasa de Supervivencia , Taiwán , Factores de Tiempo , Resultado del TratamientoRESUMEN
Between 1993 and July 2008, a total of 354 leukaemic patients received either allogeneic bone marrow transplantation (BMT) [n = 180] or peripheral blood stem cell transplantation (PBSCT) [n = 174] from human leukocyte antigen-matched sibling donors. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporin A and methotrexate. When comparing with BMT group, patients in the PBSCT group received much higher nucleated cells and CD34+ cells, and had much faster recovery of the neutrophil and platelet counts. The probability of developing acute GVHD was slightly higher in PBSCT patients (P = 0.02). The probability of chronic GVHD (cGVHD) in PBSCT was much higher in PBSCT (70 +/- 5.4%, extensive 48 +/- 6.5%) than in BMT (25 +/- 4.7%, extensive 10 +/- 3.4%; P < 0.001). Chronic GVHD was associated with long-term impairment of life quality and decreased quality-adjusted survival. In standard-risk leukaemia, use of PBSCT was associated with higher cGVHD, transplant-related mortality and a trend for decreased overall survival. The results suggest that allogeneic PBSCT is associated with high incidence of cGVHD in Chinese patients and its long-term risk and benefit remains to be defined in early stage of leukaemia.
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Enfermedad Injerto contra Huésped/etiología , Leucemia/terapia , Trasplante de Células Madre de Sangre Periférica/efectos adversos , Hermanos , Enfermedad Injerto contra Huésped/epidemiología , Hospitales Universitarios , Humanos , Incidencia , Estimación de Kaplan-Meier , Calidad de Vida , Sobrevivientes , Taiwán/epidemiología , Tiempo , Trasplante Homólogo/efectos adversosRESUMEN
The significance of hepatitis C viral (HCV)-RNA levels in long-term clinical outcomes of children with chronic HCV infection is not well understood. We conducted a long-term follow-up study of 42 children with chronic HCV infection that included clinical evaluation, biochemical tests, HCV genotyping and repeated quantitative HCV-RNA detection. Patients were divided into low and high viraemia groups according to RNA levels at enrollment (below/above 4.5 x 10(4) IU/mL), and clinical, biochemical and virological factors were evaluated. Overall, 14.3% (6/42) of patients developed spontaneous viral clearance during a median 10.1 years of follow-up. HCV-RNA levels at enrollment and mean RNA levels during follow-up for each patient were significantly correlated (R = 0.9018, 95% CI: 0.6637-0.9038, P < or = 0.001). HCV-RNA level fluctuation was within two log units in 76% of patients. Cumulative viraemia probability during follow-up could be predicted by viraemia levels at enrollment (P = 0.0092). Chronic HCV-infected children, with an RNA level below 4.5 x 10(4) IU/mL at enrollment, have a higher spontaneous viral clearance rate.
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Hepacivirus/fisiología , Hepatitis C Crónica , ARN Viral/sangre , Carga Viral/fisiología , Viremia , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Hepacivirus/genética , Hepacivirus/aislamiento & purificación , Hepatitis C Crónica/epidemiología , Hepatitis C Crónica/inmunología , Hepatitis C Crónica/virología , Humanos , Lactante , Masculino , Taiwán/epidemiología , Factores de Tiempo , Viremia/epidemiología , Viremia/inmunología , Viremia/virologíaRESUMEN
EBV-induced post transplant lymphoproliferative disorder (PTLD) continues to be a major complication after transplantation. Between January 1993 and April 2006, 12 cases of B-cell lymphoproliferative disorder were identified among 577 patients after allogeneic hematopoietic SCT (HSCT) with an overall incidence of 2.51% at 1 year. Grades II-IV acute GVHD, CMV antigenemia and the use of antithymocyte globulin (ATG) were independent risk factors for PTLD. At diagnosis, all of the tumors were CD20-positive and 11 (92%) were EBV-encoded RNA (EBER)-positive. Of the 12 patients with B-cell lymphoproliferative disorder, 8 had pulmonary involvement and 10 had extranodal involvement. Eleven patients received weekly rituximab therapy at a dose of 375 mg/m(2); the median interval between the onset of symptoms and rituximab therapy was 6 days. The overall mortality rate was 92% and seven (64%) of the deaths were directly attributable to disseminated PTLD within days or weeks of presentation. In our series, pulmonary PTLD followed an extremely aggressive course and poor response to current therapy, even though rituximab was included in the therapeutic regimens.
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Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedades Pulmonares/etiología , Trastornos Linfoproliferativos/etiología , Adolescente , Adulto , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales de Origen Murino , Niño , Preescolar , Estudios de Cohortes , Infecciones por Virus de Epstein-Barr/etiología , Infecciones por Virus de Epstein-Barr/inmunología , Infecciones por Virus de Epstein-Barr/terapia , Femenino , Humanos , Lactante , Enfermedades Pulmonares/inmunología , Enfermedades Pulmonares/terapia , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/terapia , Masculino , Persona de Mediana Edad , Análisis Multivariante , Factores de Riesgo , Rituximab , Trasplante Homólogo , Resultado del Tratamiento , Adulto JovenRESUMEN
The thalassemias are a heterogeneous group of inherited hypochromic anemias of varying severity. The mainstay of supportive treatment is regular blood transfusion accompanied by iron-chelating therapy. Hematopoietic stem cell transplantation (HSCT) provides an alternative option when curative therapy is considered. More than 400 patients in Taiwan have beta-thalassemia major or other transfusion-dependent thalassemias, and their treatment costs account for a considerable percentage of the National Health Insurance expenditure. In this report, we estimated the treatment costs of conventional therapy (regular blood transfusion accompanied by iron-chelating agents) and HSCT. The undiscounted medical cost of 20 years of follow-up (20 years from diagnosis) and the undiscounted total lifetime cost were NT$ 4 739 888 (NT$ means New Taiwan Dollars)/US$ 149 288 and NT$ 11 529 990/US$ 363 149, respectively, for patients undergoing conventional therapy, and NT$ 2 639 982/US$ 83 149 and NT$ 3 511 172/US$ 110 588, respectively, for those undergoing successful HSCT. Comparisons of treatment costs and other parameters between these two modalities can add to the information base on which policy is made by health authorities or clinicians.
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Transfusión Sanguínea/economía , Costo de Enfermedad , Trasplante de Células Madre/economía , Talasemia beta/economía , Talasemia beta/terapia , Preescolar , Supervivencia sin Enfermedad , Femenino , Sangre Fetal/citología , Estudios de Seguimiento , Prueba de Histocompatibilidad , Humanos , Lactante , Masculino , Hermanos , Taiwán , Factores de TiempoRESUMEN
To improve treatment results for children with de novo acute myeloid leukemia (AML), we introduced a novel protocol, Taiwan Pediatric Oncology Group-AML-97A, for AML other than acute promyelocytic leukemia (APL), for which modified conventional protocols were used. From January 1, 1997, to December 31, 2002, 141 children younger than 17 years old with de novo AML were enrolled. In total, 117 patients with non-APL AML were treated with induction therapy of idarubicin and cytarabine (Ara-C), postremission therapy with high-dose Ara-C - containing regimens for four monthly courses, and moderate-dose therapy with idarubicin and Ara-C for four monthly courses. The first 19 patients with APL were treated with all-trans retinoic acid, idarubicin and Ara-C, with the remaining five patients receiving all-trans retinoic acid and idarubicin, followed by maintenance therapy for 2 years. Stem cell transplantation was performed in 29 patients in first remission with a similar outcome as chemotherapy alone. The remission rate in the AML-97A study was 90%, the 5-year survival 51 +/- 5.3% (s.e.) and the 5-year event-free survival 50 +/- 4.8%; for APL, these were 100%, 86 +/- 7.0, and 75 +/- 9.8%. For the whole group, the 5-year survival was 57 +/- 4.7% and the 5-year event-free survival 54 +/- 4.4%. The AML-97A regimen was well tolerated.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Leucemia Mieloide Aguda/terapia , Leucemia Promielocítica Aguda/terapia , Trasplante de Células Madre , Adolescente , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Inducción de Remisión , Taiwán , Resultado del TratamientoRESUMEN
OBJECTIVES: To study the effect of hyperbaric oxygen therapy in alleviating acute lung injury induced by lipopolysaccharide (LPS) in rats. DESIGN AND INTERVENTIONS: The rats received an intraperitoneal injection of LPS (15 mg/kg). Animals were either breathing air at 1 ATA or subjected to hyperbaric oxygen (HBO(2)) therapy. The HBO(2) therapy was carried out in a hyperbaric chamber at a pressure of 3 ATA for 90 min. In another two groups, LPS-treated rats also received intraperitoneal injection of N(omega)-nitro-L-arginine (LNAME, 25 mg/kg) or L-N(6)-(iminoethyl)lysine (LNIL, 10 ml/kg). Another two groups of LPS-treated rats were subjected to HBO(2) exposure after the injection of L-NAME or L-NIL. MEASUREMENTS AND MAIN RESULTS: The bronchoalveolar lavage (BAL) was done into the left lung at 7.5 h after intraperitoneal injection of LPS. Parts of the right lung were excised for myeloperoxidase measurement, whereas the rest was collected for wet/dry ratio determination. LPS significantly increased the nitrite/nitrate (NO(x)(-)) concentration (34.4+/-15.7 vs 4.5+/-3.1 microM), LDH activity (66+/-17 vs 46+/-15 mAbs/min), and protein concentration (373+/-119 vs 180+/-90 mg/l) in the BAL fluid. Treatment with HBO(2) immediately after the injection of LPS enhanced the increase of NO(x)(-) production, but reduced the LDH and protein in BAL fluid to the control levels. Pretreatment with either L-NAME or L-NIL abolished the increase of NO(x)(-) in the BAL fluid and further elevated the LDH level and protein concentration. CONCLUSION: Our results suggested that HBO(2) alleviates the LPS-induced acute lung injury, which may be related to the enhancement of nitric oxide production.
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Oxigenoterapia Hiperbárica , Lipopolisacáridos/farmacología , Pulmón/patología , Lisina/análogos & derivados , Análisis de Varianza , Animales , Lavado Broncoalveolar , Inyecciones Intraperitoneales , Lipopolisacáridos/administración & dosificación , Pulmón/efectos de los fármacos , Pulmón/metabolismo , Lisina/administración & dosificación , Lisina/farmacología , Masculino , NG-Nitroarginina Metil Éster/administración & dosificación , NG-Nitroarginina Metil Éster/farmacología , Óxido Nítrico/metabolismo , Peroxidasa/metabolismo , Ratas , Ratas Sprague-DawleyRESUMEN
Nephromegaly, assessed by calculating kidney volume using renal ultrasound, was studied in infants with biliary atresia, neonatal hepatitis, or fulminant hepatitis. We evaluated kidney volume in 29 patients with biliary atresia, 17 patients with neonatal hepatitis, and 10 patients with fulminant hepatitis, as well as 32 healthy infants. Levels of plasma hepatocyte growth factor (HGF) were measured in all infants. Levels of plasma transforming growth factor-beta1 (TGF-beta1) were also measured in diseased infants and 20 healthy infants. Significant nephromegaly was found in infants with biliary atresia compared with healthy infants (P < 0.001 by analysis of covariance). Marked nephromegaly was also noted in all infants with fulminant hepatitis and 35% of infants with neonatal hepatitis. No nephromegaly was found in infants at 2 months of age with biliary atresia or neonatal hepatitis despite mildly elevated plasma HGF levels. Regardless of the duration of HGF exposure and healthy renal growth by a certain age, a positive correlation existed between plasma HGF level and kidney volume (r = 0.529; P < 0.001), but an inverse correlation was found between plasma TGF-beta1 level and nephromegaly (r = -0.505; P < 0.001) in all diseased infants. There was a stronger positive correlation between plasma HGF-TGF-beta1 ratio and kidney volume (r = 0.666; P < 0.001) and degree of nephromegaly (r = 0.717; P < 0.001). These results confirm the presence of large kidneys not only in patients with biliary atresia but also in patients with fulminant hepatitis, which suggests the possible pathogenic role of HGF and manifests as elevated HGF-TGF-beta1 ratios in patients with such conditions. Nephromegaly in patients with severe or chronic liver dysfunction may provide a new in vivo model to study the mechanisms of renal growth.
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Atresia Biliar/sangre , Hepatitis/sangre , Hepatitis/complicaciones , Factor de Crecimiento de Hepatocito/sangre , Enfermedades Renales/etiología , Factor de Crecimiento Transformador beta/sangre , Atresia Biliar/complicaciones , Hepatitis B/sangre , Hepatitis B/complicaciones , Humanos , Lactante , Recién Nacido , Riñón/diagnóstico por imagen , Enfermedades Renales/diagnóstico por imagen , Factor de Crecimiento Transformador beta1 , UltrasonografíaRESUMEN
Langerhans' cell histiocytosis (LCH) is an uncommon disease with variable manifestations. We report a case of LCH with the unusual initial presentations of chest pain and progressive heart failure in a 5-year-old boy. Chest radiography revealed a wide mediastinum with cardiomegaly. Electrocardiography showed first-degree atrioventricular block and an inverted T wave over V4-V6. Echocardiography, computed tomography, and magnetic resonance imaging of the chest all showed an infiltrating lesion that enveloped the entire heart, great vessels, and coronary arteries. Pathologic examination of the biopsy specimen revealed LCH. Chemotherapy, which included prednisolone, vincristine, methotrexate, and 6-mercaptopurine, had only a minimal effect on the tumor. After the addition of etoposide, the lesion decreased in size, and the symptoms and signs of heart failure and chest pain were ameliorated.
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Enfermedades de la Aorta/etiología , Dolor en el Pecho/etiología , Insuficiencia Cardíaca/etiología , Histiocitosis de Células de Langerhans/complicaciones , Preescolar , Humanos , MasculinoRESUMEN
OBJECTIVE: To study the prevalence of and risk factors for abnormal glucose tolerance in transfusion-dependent beta-thalassemic patients. RESEARCH DESIGN AND METHODS: A total of 89 transfusion-dependent beta-thalassemic patients were interviewed. Diabetes was previously diagnosed in 14 of them. In the remaining 75 patients, 68 participated in an oral glucose tolerance test. Potential risk factors were identified using the independent t test, chi2 test, and Fisher's exact test. Logistic regression analysis was used to select the independent risk factors that best predicted abnormal glucose tolerance A two-tailed P value of <0.05 was considered to be statistically significant. RESULTS: The prevalence of impaired glucose tolerance was 8.5% (7 of 82) and that of diabetes was 19.5% (16 of 82). Presentation with diabetic ketoacidosis was 31.1% (5 of 16). The risk factors for abnormal glucose tolerance found in transfusion-dependent beta-thalassemic patients were serum ferritin concentration and hepatitis C infection. CONCLUSIONS: The interaction of iron overload and hepatitis C infection worsened the prognosis of thalassemic patients. Aggressive iron-chelation therapy as well as prevention and treatment of hepatitis C infection should be mandatory in managing glucose homeostasis in transfusion-dependent beta-thalassemic patients in Taiwan.
Asunto(s)
Transfusión Sanguínea , Intolerancia a la Glucosa/epidemiología , Talasemia beta/sangre , Talasemia beta/terapia , Adolescente , Adulto , Niño , Diabetes Mellitus/epidemiología , Femenino , Ferritinas/sangre , Hepatitis B/epidemiología , Hepatitis C/epidemiología , Humanos , Quelantes del Hierro/uso terapéutico , Masculino , Cooperación del Paciente , Prevalencia , Factores de Riesgo , Taiwán , Talasemia beta/complicacionesRESUMEN
Asthma is a chronic inflammatory disease characterized by reversible airway obstruction caused by edematous airway lining, thickened mucosal secretions, and smooth muscle constriction. Beta2-adrenoceptor agonists are widely used in the treatment of bronchial asthma because of their ability to induce relaxation of airway smooth muscle. Evidence indicates that desensitization and down-regulation of beta-adrenoceptors occurs in long-term beta2-agonist therapy, and these medications were thought to cause increased severity of, and mortality in, asthma. The purpose of this study was to delineate further the potential adverse effects of beta2-agonists on the development of T lymphocytes. T cells isolated from umbilical cord blood and adult peripheral blood were cultured in the presence of salbutamol. Intracellular staining with fluorescence-labeled antibodies was used to differentiate the frequency of type 1 T-helper (Th1) and type 2 T-helper (Th2) cells. The results showed a statistically significant inverse relationship between the concentration of salbutamol and the ratio of Th1 over Th2 on cord blood T cells. However, this trend was not observed in adult peripheral blood T cells. The data revealed another potential adverse effect in which chronic beta2-agonist exposure predisposed differentiation of T lymphocytes towards Th2 while that of Th1 was relatively suppressed, especially in cord blood T cells. Hence, beta2-agonists, despite their effect in symptomatic rescue in asthma, should not be used indiscriminately as long-term therapeutic agents.
Asunto(s)
Agonistas Adrenérgicos beta/uso terapéutico , Sangre Fetal/citología , Linfocitos T/citología , Adulto , Albuterol/agonistas , Albuterol/uso terapéutico , Asma/tratamiento farmacológico , Asma/inmunología , División Celular/efectos de los fármacos , Femenino , Humanos , Inmunización/efectos adversos , Masculino , Monocitos/efectos de los fármacos , Fitohemaglutininas/farmacología , Embarazo , Células TH1/efectos de los fármacos , Células Th2/efectos de los fármacosRESUMEN
Allergic rhinitis is a common disease in children, and antihistamines are the key medication. However, traditional tablets are not convenient and lead to low compliance in young children. The aim of this double-blind, placebo-controlled, parallel, randomized study was to evaluate the effectiveness and safety of loratadine syrup for the treatment of children aged 3 to 12 years with allergic rhinitis. Sixty children with allergic rhinitis due to dust mites were enrolled. They were randomized into 2 parallel groups: one group received loratadine syrup 5 mg or 10 mg daily for 3 weeks, and the other group received placebo. The patients returned to special clinics for symptoms evaluation at day 7 and day 21, and the parents were requested to record disease severity daily. Both evaluations, physician's and parents', were recorded with a 4-point scale for 5 symptoms: sneezing, rhinorrhea, nasal congestion, nasal itching and ocular symptoms. Forty-six patients completed the study, 22 in the loratadine group and 24 in the placebo group. At the initial visit, the total symptom score (TSS) in both groups was not significantly different (p = 0.39). The TSS of the loratadine syrup group at day 7 and day 21 was lower than those of the placebo group (p = 0.003, p = 0.06). The daily card scores in the experimental group were also significantly lower than those of the placebo group (week 1, p = 0.014; week 2, p = 0.029; week 3, p = 0.014). No adverse reactions were recorded in both groups. This study revealed that loratadine syrup 5 mg or 10 mg once a day improved symptom scores of children with allergic rhinitis effectively and safely.
Asunto(s)
Antagonistas de los Receptores Histamínicos H1/uso terapéutico , Loratadina/uso terapéutico , Rinitis Alérgica Perenne/tratamiento farmacológico , Administración Oral , Niño , Preescolar , Método Doble Ciego , Femenino , Antagonistas de los Receptores Histamínicos H1/administración & dosificación , Antagonistas de los Receptores Histamínicos H1/efectos adversos , Humanos , Loratadina/administración & dosificación , Loratadina/efectos adversos , Masculino , Soluciones FarmacéuticasRESUMEN
Post-transplant lymphoproliferative disorder (PTLD) is uncommonly of T cell origin, especially following BMT. We describe a 13-year-old boy with severe aplastic anemia (SAA) and no evidence of Fanconi's anemia who underwent BMT at 11 years of age using CY 10 mg/kg once daily i.v. on days -5, -4, antilymphocyte globulin (ALG) 30 mg/kg once daily i.v. on days -5 approximately -3 and CsA from day -1 as conditioning. The BMT failed and he received a further peripheral blood stem cell transplant (PBSCT) 240 days after BMT. Conditioning was with CY 50 mg/kg once daily i.v. on days -5 approximately -2, and ALG 15 mg/kg once daily i.v. on days -4 approximately -2. GVHD prophylaxis included CsA and MTX. Engraftment was later confirmed by cytogenetic studies. Desquamation and ulcers of the oral mucosa and mouth angle developed in the 13th month post PBSCT. A buccal mucosa biopsy on day +524 revealed only plasmacytosis. Immunosuppressants were discontinued at that point. Generalized lymphadenopathy, prolonged fever (waxing and waning) and facial swelling developed in the 18th month post PBSCT. A neck lymph node biopsy on day +601 showed T cell lymphoma of diffuse large cell type with monoclonal TCR gamma-chain gene rearrangement. A FISH study showed that the malignant T cells were of recipient origin. EBV in situ hybridization was negative. He did not receive further treatment apart from discontinuation of immunosuppressants. He was followed up in our out-patient clinic and showed good performance 1170 days post PBSCT. We speculate that a different mechanism was operating in the pathogenesis of T cell lymphoma in this case. Risk factors include SAA and two transplants, conditioned with CY and ALG, long term use of CsA and treatment with azathioprine.
Asunto(s)
Anemia Aplásica/terapia , Trasplante de Médula Ósea/efectos adversos , Trastornos Linfoproliferativos/etiología , Linfocitos T/inmunología , Adolescente , Infecciones por Virus de Epstein-Barr/complicaciones , Humanos , Trastornos Linfoproliferativos/terapia , MasculinoRESUMEN
A nation-wide chemotherapeutic trial for childhood non-Hodgkin's lymphoma (NHL) was conducted by the Taiwan Pediatric Oncology Group (TPOG). Four TPOG-NHL92 protocols based on stage and histology were activated in 1992: TPOG-92LD (treatment duration: 8 months) was used for localized (stages I/II) NHL with any histology, 92LB (2 years), 92SNC (5 months), and 92LC (1 year) for advanced (stages III/IV) lymphoblastic (LB), small non-cleaved cell (SNC), and large cell (LC) lymphoma, respectively. From January 1992 through June 1998, 200 children with newly diagnosed NHL from 13 member hospitals of TPOG were enrolled. There were 140 boys and 60 girls. Their ages at diagnosis ranged from 2.4 months to 18.3 years with a median of 8.2 years. There were 54 (27.3%) patients with LB, 94 (47.5%) with SNC including B-cell acute lymphoblastic leukemia (B-ALL), and 50 (25.2%) with LC. Stages I, II, III, and IV (including B-ALL) of the disease comprised 5%, 10%, 43%, and 42% of cases, respectively. There were 176 patients eligible for evaluation of treatment results. The remission rate of induction was 82.4%, induction failed in 22 (12.5%) patients, and nine patients died during induction. As of August 31, 1999, 26 patients relapsed, six died during remission, one patient developed secondary acute myelomonocytic leukemia, and 105 patients remained in continuous remission with a median remission duration of 49 months. The event-free survival (EFS) at 7 years was 63.5%, 61.5% and 65% for LB, SNC, and LC groups (P = 0.8298). The 7-year EFS for stages I/II, III, and IV of the disease was 73%, 68.9%, and 50.3% (P = 0.0212), respectively. We concluded that following the strategy of stratification of therapy, only disease stages had prognostic significance in this study. More efforts are needed to improve our treatment results.