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1.
Lymphology ; 56(3): 99-109, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38896493

RESUMEN

Primary lymphedema (PLE) is a chronic disease caused by lymphatic dysplasia and progresses to irreversible tissue edema and hypertrophy. Understanding of PLE has been hitherto limited. The aim of this study is to devise an updated classification system for PLE of 1013 patients with PLE of lower limb were enrolled. Sex, age of onset, location, family history and morbidity were documented. The lymphatic imaging findings of magnetic reso-nance lymphography (MRL), indocyanine green lymphography (ICGL) and lymphoscin-tigraphy (LSG), skin tissue immunohisto-chemical staining, whole exome sequencing and the correlation of genotype-phenotype were evaluated. Patients were divided into a congenital onset category and a late onset category. The late onset category was further divided according to developmental age. The ratio of congenital-onset to late-onset PLE was 1:4 and that the highest incidence was in adolescence. The sex ratio was 1.04:1 and 1.5:1 in congenital-onset and late-onset groups, respectively. Three major lymphatic anomalies were identified, in which segmental lymphatic dysfunction, characterized by delayed or partial demonstration of lymph vessels, is the most common and associated with FLT4, GJC2, CELSR1, and PTPN14 mutations. The next most common type is lymphatic hyperplasia, which is associated with FOXC2 and GATA2 variants, followed by initial lymphatic aplasia or dysfunction, which is more common in pa-tients with congenital PLE and associated with FLT4 mutation. A functional and structural combined classification of lymphatic anomalies is proposed, which includes segmental lymphatic dysfunction, lymphatic hyperplasia and initial lymphatic aplasia or dysfunction.

2.
Materials (Basel) ; 16(1)2022 Dec 21.
Artículo en Inglés | MEDLINE | ID: mdl-36614385

RESUMEN

The vapor pressure values of common elements are available in the literature over a limited temperature range and the accuracy and reliability of the reported data are not generally available. We evaluate the reliability and uncertainty of the available vapor pressure versus temperature data of fifty common pure elements and recommend vapor pressure versus temperature relations. By synthesizing the vapor pressure values from measurements reported in the literature with the values computed using the Clausius Clapeyron relation beyond the boiling point, we extend the vapor pressure range from 10-8 atm to 10 atm. We use a genetic algorithm to optimize the fitting of the vapor pressure data as a function of temperature over the extended vapor pressure range for each element. The recommended vapor pressure values are compared with the corresponding literature values to examine the reliability of the recommended values.

3.
Lymphology ; 53(2): 76-80, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33190430

RESUMEN

L ymphedema is a well-known complication of Noonan syndrome (NS) but the lymphatic malformations in NS are poorly understood. We report clinical, genetic, and imaging information about a boy and girl with NS and late-onset lower extremity lymphedema. A de novo missense mutation of RIT1 (NM_006912.5) c.246T>A, p.Phe82Leu was identified in the girl, who also showed systemic lymphatic hyperplasia and dysfunction. Magnetic resonance lymphangiography (MRL) of the boy clearly demonstrated segmental dilated and hyperplastic lymphatics with impaired transport function in an affected limb and pelvic region. Indocyanine green lymphography (ICGL) showed delayed and partial enhancement of the lymph vessels in the affected limb but no lymph reflux was detected. No causative mutation was identified in the second case. Lymphoscintigraphy (LSG) failed to show lymph vessels in either of the children. Our study showed that MRL is a reliable and accurate test that can be used to demonstrate morpho-logical and functional defects of the lymphatic system. Moreover, ICGL is sufficiently sensitive to determine the functional condition of peripheral lymph vessels. The combined use of imaging modalities can give an accurate diagnosis of complex lymphatic system anomalies in NS and other syndromic diseases.


Asunto(s)
Anomalías Linfáticas/complicaciones , Anomalías Linfáticas/diagnóstico , Síndrome de Noonan/complicaciones , Síndrome de Noonan/diagnóstico , Alelos , Niño , Diagnóstico por Imagen , Manejo de la Enfermedad , Femenino , Predisposición Genética a la Enfermedad , Humanos , Procesamiento de Imagen Asistido por Computador , Anomalías Linfáticas/genética , Linfografía , Linfocintigrafia , Imagen por Resonancia Magnética , Masculino , Síndrome de Noonan/genética , Proteínas ras/genética
5.
Zhonghua Hu Li Za Zhi ; 31(7): 373-6, 1996 Jul.
Artículo en Chino | MEDLINE | ID: mdl-9295484

RESUMEN

In order to research the best alcoholic concentration in the humidifying bottle when pneumonedema oxygen inhalation, 32 rabbits, divided into 4 groups, are replicated into pneumonedema models using method of rapid transfusion, and given oxygen inhalation with 20%, 50%, 70%, and 90% alcohol as humidifying agent (shortly called alcoholic oxygen). The results are as follows: using 20% alcohol as humidifying agent, the increasing amplitude of blood PaO2 is 147.30% (P < 0.001), the injury to the pulmonary bronchial mucosa and the wall of pulmonary alveoli is slight; using 50% alcohol, the increasing amplitude of blood PaO2 is 39.46% (P < 0.001), the injury to the parts mentioned above exacerbates and bronchiole cavity mucosa has moderate bleeding; using 70% alcohol, the increasing amplitude of blood PaO2 is 21.97% (P < 0.05), pneumorrhagia occurs; using 90% alcohol, the increasing amplitude of blood PaO2 is 94.46% (P < 0.01), a great number of blood cells aggregate inside the pulmonary alveoli and the bronchiole. This study proves that choosing 20% alcohol as humidifying agent has the best result, and as well, the explanation of the mechanism of alcohol suppressing foam, meaning being able to decrease only the surface tension of the foam inside the pulmonary alveoli, is incomprehensive, and the nature of the material itself forming foam has decisive function.


Asunto(s)
Alcoholes/uso terapéutico , Hipoxia/terapia , Nebulizadores y Vaporizadores , Terapia por Inhalación de Oxígeno , Edema Pulmonar/complicaciones , Administración por Inhalación , Animales , Análisis de los Gases de la Sangre , Evaluación Preclínica de Medicamentos , Humanos , Hipoxia/etiología , Conejos
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