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2.
Immunotherapy ; 13(10): 807-811, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-33969699

RESUMEN

Systemic capillary leak syndrome (SCLS) is a life-threatening disease. It is characterized by severe capillary hyperpermeability to proteins resulting in hemoconcentration, hypoalbuminemia and hypovolemic shock. Its treatment remains supportive, and the prognosis is generally poor. We report on a 51-year old male with melanoma treated with nivolumab for 1 year. 1 month following the completion of the treatment, the patient presented with signs of hypovolemic shock, anasarca, hemoconcentration and hypoalbuminemia. After excluding other diseases, a diagnosis of nivolumab-induced systemic capillary leak syndrome was made. A high dose of intravenous steroid therapy was promptly initiated without any significant clinical improvement. Intravenous immunoglobulin therapy was then administered with normalization of blood pressure, hemoconcentration and complete resolution of anasarca. Intravenous immunoglobulin should be considered a first-line treatment option for this rare phenomenon.


Lay abstract Systemic capillary leak syndrome (SCLS) is a life-threatening disease with a high fatality rate. Patients present with low blood pressure, widespread edema and rapid weight gain. Labs show low albumin levels with highly concentrated blood, seen as high hematocrit and hemoglobin levels. Current treatments aim to support the acute crisis. We are presenting on a 51-year old patient with melanoma, treated with nivolumab for 1 year who developed signs of SCLS 1-month following medication discontinuation. He was first treated with high-dose steroids without symptom resolution. He was then administered immune proteins called intravenous immunoglobulins, resolving all his symptoms. Due to the patient's complete response, we suggest intravenous immunoglobulins as the initial treatment in patients taking nivolumab presenting with SCLS.


Asunto(s)
Síndrome de Fuga Capilar/inducido químicamente , Síndrome de Fuga Capilar/inmunología , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Inmunoglobulinas Intravenosas/uso terapéutico , Nivolumab/efectos adversos , Corticoesteroides/uso terapéutico , Síndrome de Fuga Capilar/terapia , Fluidoterapia/métodos , Humanos , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
3.
Heart Vessels ; 35(1): 46-51, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31278424

RESUMEN

Our prospective study was therefore designed to determine which part of the systemic inflammatory response after cardiac operations resulted from Cardiopulmonary bypass (CPB) in neonates and infants. After approval by the human ethical committee of the Gunma Children's Medical Center (GCMC) and informed consent of the parents, 40 consecutive term congenital heart disease patients aged until 1 year who underwent long CPB time (> 3 h) at surgery were included in the prospective study between January 2012 and December 2014. C1 esterase inhibitor (C1-inh) drug (@Berinert) was generously provided by CSL Behring (King of Prussia, PA). The C1-inh (20 IU/kg) was given intravenously 60 min after CPB. Blood samples for complement factors were obtained before and 48 h after administration of C1-inh. Six patients did not survive and their data were not included. Of 34 patients included, median age was 6.5 months, median body weight was 6050 g, and 16 (47%) were female. According to the Mann-Whitney U test, there were no differences between the two groups concerning demographic and intraoperative data, postoperative chemical data. C1q concentration was only significant lower in patients with C1-inh non-treated group than in patients with C1-inh treated group. But, the consumption of C1q, C3, C4, CH50, and C1-inh in patients with C1-inhibitor non-treated group was observed early postoperatively. There is a significant difference in the values before and after C1-inh treatment between the two groups. The lower value in the C1-inh-treated group is explained by the activation of the classical pathway through the replenishment of complements by C1-inh treatment. This study proposes the administration of C1-inh is an effective therapy to reduce the activation and improve the clinical capillary leak syndrome.


Asunto(s)
Síndrome de Fuga Capilar/prevención & control , Procedimientos Quirúrgicos Cardíacos , Puente Cardiopulmonar , Activación de Complemento/efectos de los fármacos , Proteína Inhibidora del Complemento C1/administración & dosificación , Inactivadores del Complemento/administración & dosificación , Cardiopatías Congénitas/cirugía , Síndrome de Respuesta Inflamatoria Sistémica/prevención & control , Administración Intravenosa , Síndrome de Fuga Capilar/sangre , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/inmunología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Puente Cardiopulmonar/efectos adversos , Proteína Inhibidora del Complemento C1/efectos adversos , Inactivadores del Complemento/efectos adversos , Femenino , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/inmunología , Humanos , Lactante , Recién Nacido , Japón , Masculino , Estudios Prospectivos , Síndrome de Respuesta Inflamatoria Sistémica/sangre , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Síndrome de Respuesta Inflamatoria Sistémica/inmunología , Factores de Tiempo , Resultado del Tratamiento
7.
Curr Mol Med ; 18(5): 335-342, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30289072

RESUMEN

Antibody-toxin fused agents or immunotoxins, are a newly engineered class of cytotoxic agents consisting of a bacterial or plant toxin moiety hooked up either to a monoclonal antibody or a specific growth factor. Nevertheless, acquiring a full potency in clinic is mostly restricted due to the Capillary leak syndrome (CLS), a serious immune provoked, life-threatening side effect, subsequent to the endothelial damage, resulting in fluid escape from the bloodstream into tissues including lungs, muscle and brain, developing organ failure and eventually death. Proposed underlying mechanisms include direct damage to endothelial cells, acute inflammation, Lymphokine-activated killer (LAK) cells engagement, alteration in cell-cell/cell-matrix connectivities and cytoskeletal dysfunction. Very poor biodistribution and heterogeneous extravasation pattern in tumor site result in accumulation of ITs close to the extravasation site, gradual toxin release and initiation of nearby endothelial cells lysis, secretion of pro-inflammatory cytokines, development of acute inflammation and engagement of Lymphokine-activated killer (LAK) cells. Intrinsic immunogenicity of applied toxin moiety is another important determinant of CLS incidence. Toxins with more intrinsic immunogenicity possess more probability for CLS development. Recently, development of new generations of antibodies and mutated toxins with conserved cytotoxicity has partly tapered risk of CLS development. Here, we describe probable mechanisms involved in CLS and introduce some of the recently applied strategies for lessening incidence of CLS as much as possible.


Asunto(s)
Síndrome de Fuga Capilar , Citocinas/inmunología , Inmunotoxinas , Células Asesinas Activadas por Linfocinas , Neoplasias , Animales , Síndrome de Fuga Capilar/inducido químicamente , Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/patología , Síndrome de Fuga Capilar/terapia , Humanos , Inmunotoxinas/efectos adversos , Inmunotoxinas/uso terapéutico , Inflamación/inducido químicamente , Inflamación/inmunología , Inflamación/patología , Inflamación/terapia , Células Asesinas Activadas por Linfocinas/inmunología , Células Asesinas Activadas por Linfocinas/patología , Neoplasias/tratamiento farmacológico , Neoplasias/inmunología , Neoplasias/patología
8.
Biol Blood Marrow Transplant ; 21(12): 2061-2068, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-26327628

RESUMEN

Engraftment syndrome (ES) encompasses a continuum of periengraftment complications after autologous hematopoietic stem cell transplantation. ES may include noninfectious fever, skin rash, diarrhea, hepatic dysfunction, renal dysfunction, transient encephalopathy, and capillary leak features, such as noncardiogenic pulmonary infiltrates, hypoxia, and weight gain with no alternative etiologic basis other than engraftment. Given its pleiotropic clinical presentation, the transplant field has struggled to clearly define ES and related syndromes. Here, we present a comprehensive review of ES in all documented disease settings. Furthermore, we discuss the proposed risk factors, etiology, and clinical relevance of ES. Finally, our current approach to ES is included along with a proposed treatment algorithm for the management of this complication.


Asunto(s)
Enfermedad Injerto contra Huésped/terapia , Trasplante de Células Madre Hematopoyéticas/métodos , Inmunosupresores/uso terapéutico , Linfoma/terapia , Mieloma Múltiple/terapia , Síndrome POEMS/terapia , Encefalopatías/etiología , Encefalopatías/inmunología , Encefalopatías/patología , Encefalopatías/terapia , Síndrome de Fuga Capilar/etiología , Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/patología , Síndrome de Fuga Capilar/terapia , Diarrea/etiología , Diarrea/inmunología , Diarrea/patología , Diarrea/terapia , Exantema/etiología , Exantema/inmunología , Exantema/patología , Exantema/terapia , Fiebre/etiología , Fiebre/inmunología , Fiebre/patología , Fiebre/terapia , Enfermedad Injerto contra Huésped/inmunología , Enfermedad Injerto contra Huésped/patología , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Insuficiencia Hepática/etiología , Insuficiencia Hepática/inmunología , Insuficiencia Hepática/patología , Insuficiencia Hepática/terapia , Humanos , Linfoma/inmunología , Linfoma/patología , Mieloma Múltiple/inmunología , Mieloma Múltiple/patología , Síndrome POEMS/inmunología , Síndrome POEMS/patología , Insuficiencia Renal/etiología , Insuficiencia Renal/inmunología , Insuficiencia Renal/patología , Insuficiencia Renal/terapia , Factores de Riesgo , Acondicionamiento Pretrasplante , Trasplante Autólogo
9.
BMJ Case Rep ; 20142014 May 23.
Artículo en Inglés | MEDLINE | ID: mdl-24859554

RESUMEN

A 54-year-old man presented to our emergency department with fever and dyspnoea. He required vigorous haemodynamic support and mechanical ventilation for hypotensive distributive shock with hypoalbuminaemia, haemoconcentration, rhabdomyolysis and acute renal failure, consistent with idiopathic systemic capillary leak syndrome. Left lung consolidation and hypoxaemia were observed 6 days after admission. Sputum smear revealed the presence of acute angled branching hyphae, consistent with a diagnosis of invasive pulmonary aspergillosis. Antifungal therapy was administered and mechanical ventilation discontinued on day 66. The patient recovered and was discharged from the hospital on day 185.


Asunto(s)
Antifúngicos/uso terapéutico , Síndrome de Fuga Capilar/terapia , Aspergilosis Pulmonar Invasiva/tratamiento farmacológico , Pulmón/diagnóstico por imagen , Infecciones Oportunistas/tratamiento farmacológico , Síndrome de Fuga Capilar/complicaciones , Síndrome de Fuga Capilar/inmunología , Humanos , Aspergilosis Pulmonar Invasiva/complicaciones , Aspergilosis Pulmonar Invasiva/inmunología , Masculino , Persona de Mediana Edad , Infecciones Oportunistas/complicaciones , Infecciones Oportunistas/inmunología , Radiografía
10.
Expert Rev Clin Immunol ; 10(3): 349-52, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24490827

RESUMEN

The systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic attacks of systemic capillary hyperpermeability accompanied by hypoalbuminemia, hemoconcentration and edema. Treatment of the acute phase is supportive, focusing on adequate fluid resuscitation. Many agents have been used to prevent acute attacks, including corticosteroids, ß2-agonists (aminophylline, theophylline, or terbutaline), infliximab, thalidomide and intravenous immunoglobulin (IVIg). ß2-agonists were the first-line maintenance therapy until a few years ago. In more recent years, IVIg became common first-line prophylactic therapy in most patients with benefits at the dose of 2 g/kg once a month. We report the case of a 49-year-old man with SCLS treated successfully with a lower dose of IVIg (1 g/kg monthly) in the maintenance phase. He presented no acute episodes in a follow-up of 28 months. We describe prophylactic treatments for SCLS in literature and compare our patient to another 18 who received IVIg in follow-up.


Asunto(s)
Síndrome de Fuga Capilar/terapia , Edema/prevención & control , Inmunoglobulinas Intravenosas/uso terapéutico , Choque/prevención & control , Síndrome de Fuga Capilar/inmunología , Permeabilidad Capilar/efectos de los fármacos , Ensayos Clínicos como Asunto , Supervivencia sin Enfermedad , Edema/etiología , Europa (Continente) , Humanos , Masculino , Persona de Mediana Edad , Recurrencia , Choque/etiología , Terbutalina/administración & dosificación , Teofilina/administración & dosificación
11.
Handchir Mikrochir Plast Chir ; 44(4): 209-19, 2012 Aug.
Artículo en Alemán | MEDLINE | ID: mdl-22932853

RESUMEN

INTRODUCTION: Thermal injuries with more than 20% of burned body surface area (BSA) lead to systemic shock with generalised oedema in addition to local tissue destruction. This condition, known as burn injury, is caused by immunmodulative mediators whose individual significance is not known in detail. We present an experimental model where plasma of burned animals (burn plasma) is transmitted to healthy animals, to trigger burn iniury without performing direct burn trauma. MATERIAL AND METHODS: The systemic oedema is measured by extravasation of fluorescent albumin in mesenterial venules of Wistar rats. In addition, leukocyte-endothelial interactions ("leukocyte rolling and sticking") is examined. RESULTS: The systemic capillary leak is induced by both direct thermal trauma as well as by infusion of burn plasma. This is evident even after plasma dilution (1% in Ringer's lactate) of the burn plasma. In addition, topical therapy for burned animals (donors) with cerium nitrate led to a significant reduction of plasma extravasation in receiver animals. In addition, systemic antioxidant therapy with high-dose vitamin C of receiver animals, led to a significant reduction of the capillary leak. Leukocyte-endothelial interactions are not significantly affected in either case. CONCLUSION: In summary, for the first time a reliable model of burn injury has been established, which eliminates mediator-independent effects. In addition, our studies show that antioxidant therapy with high doses of vitamin C and topical treatment with cerium nitrate both reduce the systemic capillary leak in receiver animals. Their positive influence could therefore soon be integrated in clinical treatment algorithms.


Asunto(s)
Quemaduras/inmunología , Síndrome de Fuga Capilar/inmunología , Adhesión Celular/inmunología , Citocinas/fisiología , Modelos Animales de Enfermedad , Edema/inmunología , Leucocitos/inmunología , Microcirculación/inmunología , Plasma/inmunología , Choque/inmunología , Animales , Antiinfecciosos Locales/farmacología , Antioxidantes/farmacología , Ácido Ascórbico/farmacología , Velocidad del Flujo Sanguíneo/efectos de los fármacos , Velocidad del Flujo Sanguíneo/fisiología , Quemaduras/tratamiento farmacológico , Síndrome de Fuga Capilar/tratamiento farmacológico , Adhesión Celular/efectos de los fármacos , Cerio/farmacología , Extravasación de Materiales Terapéuticos y Diagnósticos , Leucocitos/efectos de los fármacos , Masculino , Venas Mesentéricas/efectos de los fármacos , Venas Mesentéricas/inmunología , Microcirculación/efectos de los fármacos , Ratas , Ratas Wistar , Choque/tratamiento farmacológico , Vénulas/efectos de los fármacos , Vénulas/inmunología
13.
J Smooth Muscle Res ; 43(4): 117-37, 2007 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-17928746

RESUMEN

Sepsis is the leading cause of mortality in critically ill patients. In this pathological syndrome, septic shock and sequential multiple organ failure correlate with poor outcome. The pathophysiology of sepsis with acute organ dysfunction involves a highly complex, integrated response that includes activation of number of cell types, inflammatory mediators, and the hemostatic system. Central to this process may be alterations in vascular functions. This review article provides a growing body of evidence for the potential impact of vascular dysfunction on sepsis pathophysiology with a major emphasis on the endothelium. Furthermore, the role of apoptotic signaling molecules in the mechanisms underlying endothelial cell injury and death during sepsis and its potential value as a target for sepsis therapy will be discussed, which may help in the assessment of ongoing therapeutic strategies.


Asunto(s)
Endotelio Vascular/fisiopatología , Microcirculación/fisiopatología , Choque Séptico/sangre , Choque Séptico/fisiopatología , Animales , Apoptosis , Factores de Coagulación Sanguínea/metabolismo , Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/metabolismo , Dilatación Patológica/sangre , Dilatación Patológica/inmunología , Dilatación Patológica/fisiopatología , Células Endoteliales/metabolismo , Células Endoteliales/patología , Endotelio Vascular/inmunología , Humanos , Microcirculación/inmunología , Choque Séptico/inmunología
14.
J Immunol ; 179(6): 3715-23, 2007 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-17785808

RESUMEN

Vascular leak syndrome (VLS) is a life-threatening toxicity induced during IL-2 treatment of cancer patients. The mechanism of IL-2-induced VLS is still poorly understood. At present, there is no specific therapy for VLS. Previous studies from our laboratory demonstrated that hyaluronan (HA), a large glycosaminoglycan, abundant in the extracellular matrix and on the cell surface, caused a marked increase of IL-2-induced VLS in the lungs and liver of C57BL/6 mice. Conversely, blockade or knockout of its major receptor, CD44, resulted in a marked decrease of VLS, thereby suggesting a role for HA in VLS. In this study, we report a novel means to prevent IL-2-induced VLS by blocking endogenous HA with HA-specific binding peptide, Pep-1, a newly isolated peptide which specifically binds to soluble, cell-associated, and immobilized forms of HA. Our results demonstrated that blocking HA with Pep-1 dramatically inhibited IL-2-induced VLS in both normal mice as well as in mice bearing melanoma. Moreover, Pep-1 treatment maintained the effectiveness of IL-2 and prevented the metastasis of melanoma. IL-2-induced emigration of lymphocytes across the endothelium and cytotoxicity against tumor by lymphokine-activated killer cells were not affected by Pep-1. Instead, use of Pep-1 maintained endothelial integrity and reduced their apoptosis during IL-2-induced VLS. These data suggested that HA plays a critical role in regulating endothelial cell damage and induction of IL-2-mediated VLS. Also, blockade of HA using Pep-1 could constitute a novel therapeutic modality to prevent IL-2-mediated toxicity, thereby facilitating the effectiveness of high-dose IL-2 in the treatment of metastatic melanomas.


Asunto(s)
Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/prevención & control , Ácido Hialurónico/antagonistas & inhibidores , Interleucina-2/administración & dosificación , Interleucina-2/antagonistas & inhibidores , Neoplasias Pulmonares/prevención & control , Melanoma Experimental/inmunología , Melanoma Experimental/terapia , Animales , Apoptosis/inmunología , Síndrome de Fuga Capilar/patología , Proteínas Portadoras/metabolismo , Diferenciación Celular/inmunología , Citotoxicidad Inmunológica , Endotelio Vascular/citología , Endotelio Vascular/inmunología , Endotelio Vascular/patología , Femenino , Receptores de Hialuranos/metabolismo , Ácido Hialurónico/biosíntesis , Ácido Hialurónico/metabolismo , Interleucina-2/fisiología , Células Asesinas Activadas por Linfocinas/citología , Células Asesinas Activadas por Linfocinas/inmunología , Neoplasias Pulmonares/irrigación sanguínea , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/secundario , Linfocitos Infiltrantes de Tumor/inmunología , Linfocitos Infiltrantes de Tumor/patología , Melanoma Experimental/irrigación sanguínea , Melanoma Experimental/patología , Ratones , Ratones Endogámicos C57BL , Proteínas Mitocondriales
15.
Eur Respir J ; 30(3): 429-35, 2007 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-17537765

RESUMEN

The pathogenesis of acute lung injury includes transendothelial diapedesis of leukocytes into lung tissues and disruption of endothelial/epithelial barriers leading to protein-rich oedema. In vitro studies show that the microtubule network plays a role in the regulation of endothelial permeability as well as in neutrophil locomotion. It was hypothesised that the microtubule-stabilising agent, taxol, might attenuate inflammation and vascular leak associated with acute lung injury in vivo. The effect of intravenously delivered taxol was assessed using a model of murine lung injury induced by intratracheal lipopolysaccharide (LPS) administration. Parameters of lung injury and inflammation were assessed 18 h after treatment. Intravenously delivered taxol significantly reduced inflammatory histological changes in lung parenchyma and parameters of LPS-induced inflammation: infiltration of proteins and inflammatory cells into bronchoalveolar lavage fluid, lung myeloperoxidase activity, and extravasation of Evans blue-labelled albumin into lung tissue. Taxol alone (in the absence of LPS) had no appreciable effect on these parameters. In addition to lung proteins, intravenous taxol reduced accumulation of leukocytes in ascitic fluid in a model of LPS-induced peritonitis. Taken together, the present data demonstrate that microtubule stabilisation with taxol systemically attenuates lipopolysaccharide-induced inflammation and vascular leak.


Asunto(s)
Endotoxemia/inmunología , Lipopolisacáridos/inmunología , Paclitaxel/farmacología , Síndrome de Dificultad Respiratoria/inmunología , Síndrome de Respuesta Inflamatoria Sistémica/inmunología , Moduladores de Tubulina/farmacología , Animales , Líquido del Lavado Bronquioalveolar/inmunología , Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/patología , Endotoxemia/patología , Extravasación de Materiales Terapéuticos y Diagnósticos/inmunología , Extravasación de Materiales Terapéuticos y Diagnósticos/patología , Recuento de Leucocitos , Pulmón/inmunología , Pulmón/patología , Ratones , Ratones Endogámicos C57BL , Peritonitis/inmunología , Peritonitis/patología , Peroxidasa/metabolismo , Edema Pulmonar/inmunología , Edema Pulmonar/patología , Síndrome de Dificultad Respiratoria/patología , Síndrome de Respuesta Inflamatoria Sistémica/patología
16.
Am J Respir Crit Care Med ; 171(8): 858-67, 2005 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-15665321

RESUMEN

The cascade of cellular and molecular pathways mediating acute lung injury is complex and incompletely defined. Although the Src and Jak family of kinases is upregulated in LPS-induced murine lung injury, their role in the development of lung injury is unknown. Here we report that systemic inhibition of these kinases using specific small molecule inhibitors (PP2, SU6656, tyrphostin A1) significantly attenuated LPS-induced lung injury, as determined by histologic and capillary permeability assays. These inhibitors blocked LPS-dependent cytokine and chemokine production in the lung and in the serum. In contrast, lung-targeted inhibition of these kinases in the airway epithelium via adenoviral-mediated gene transfer of dominant negative Src or of suppressor of cytokine signaling (SOCS-1) disrupted lung cytokine production but had no effect on systemic cytokine production or lung vascular permeability. Mice were significantly protected from lethal LPS challenge by the small molecule inhibitors of Jak and Src kinase. Importantly, this protection was still evident even when the inhibitors were administered 6 hours after LPS challenge. Taken together, these observations suggest that Jak and Src kinases participate in acute lung injury and verify the potential of this class of selective tyrosine kinase inhibitors to serve as novel therapeutic agents for this disease.


Asunto(s)
Inhibidores Enzimáticos/farmacología , Lipopolisacáridos/inmunología , Proteínas Tirosina Quinasas/antagonistas & inhibidores , Proteínas Proto-Oncogénicas/antagonistas & inhibidores , Síndrome de Dificultad Respiratoria/inmunología , Familia-src Quinasas/antagonistas & inhibidores , Adenoviridae/genética , Animales , Síndrome de Fuga Capilar/genética , Síndrome de Fuga Capilar/inmunología , Activación Enzimática/efectos de los fármacos , Activación Enzimática/genética , Activación Enzimática/inmunología , Escherichia coli , Regulación de la Expresión Génica/efectos de los fármacos , Técnicas de Transferencia de Gen , Indoles/farmacología , Janus Quinasa 2 , Pulmón/efectos de los fármacos , Pulmón/inmunología , Pulmón/patología , Ratones , Ratones Endogámicos BALB C , Proteínas Tirosina Quinasas/genética , Proteínas Proto-Oncogénicas/genética , Síndrome de Dificultad Respiratoria/patología , Transducción de Señal/efectos de los fármacos , Transducción de Señal/genética , Sulfonamidas/farmacología , Activación Transcripcional/inmunología , Tirfostinos/farmacología , Familia-src Quinasas/genética
17.
Recenti Prog Med ; 96(10): 488-91, 2005 Oct.
Artículo en Italiano | MEDLINE | ID: mdl-16491771

RESUMEN

Generalized edema is commonly due to cardiac failure, renal changes, hepatic and metabolic disturbances, or it can be idiopathic, i.e. primitive lymphedema. Here we describe a patient with several episodes of fluid extravasation characterized by hypotension, hemoconcentration and functional renal insufficiency. These findings, associated to a monoclonal gammopathy, lead to the diagnosis of systemic capillary leak syndrome or Clarkson Syndrome. This rare and perplexing disorder, characterized by a typical three-phase clinical feature, is due to an endothelium permeability alteration, rather responsible of these paroxysmal manifestations. Interleukin-2-pathway is considered as one of the underlying mechanisms. During acute phase the patient underwent therapy with plasma-expanders and glucocorticoids, although in quiescent phase we administered aminophylline, salbutamol and prednisone. After three months, the patient is asymptomatic.


Asunto(s)
Angioedema/diagnóstico , Síndrome de Fuga Capilar/diagnóstico , Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Agonistas Adrenérgicos beta/uso terapéutico , Albuterol/uso terapéutico , Aminofilina/uso terapéutico , Angioedema/inmunología , Angioedema/fisiopatología , Angioedema/terapia , Antiinflamatorios/uso terapéutico , Transfusión Sanguínea , Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/fisiopatología , Síndrome de Fuga Capilar/terapia , Permeabilidad Capilar , Cardiotónicos/uso terapéutico , Quimioterapia Combinada , Humanos , Interleucina-2/metabolismo , Masculino , Persona de Mediana Edad , Gammopatía Monoclonal de Relevancia Indeterminada/inmunología , Gammopatía Monoclonal de Relevancia Indeterminada/fisiopatología , Gammopatía Monoclonal de Relevancia Indeterminada/terapia , Prednisona/uso terapéutico , Resultado del Tratamiento
18.
Lakartidningen ; 101(38): 2880-2, 2004 Sep 16.
Artículo en Sueco | MEDLINE | ID: mdl-15485171

RESUMEN

Systemic capillary leak syndrome (SCLS) is an unusual condition characterized by periodic leakage of plasma proteins through the capillary wall, leading to hypoalbuminaemia, hypovolaemia, haemoconcentration and shock. The pathogenesis is unknown and the mortality high. Various prophylactic treatments have been tried but are difficult to evaluate because of the unpredictable course of the disease. We describe one patient with frequent attacks of SCLS, that did not respond to any kind of treatment regimens over a period of nine years. Subsequently, therapy with regular immunoadsorption of plasma proteins and a leukotriene receptor antagonist was attempted. This treatment has now been given for six years and only one episode of capillary leakage has occurred. The effect may be due to removal of pathogenic immunoglobulins or immune complexes and/or inhibition of leukotriene-induced capillary leakage.


Asunto(s)
Síndrome de Fuga Capilar/terapia , Acetatos/uso terapéutico , Adulto , Proteínas Sanguíneas , Síndrome de Fuga Capilar/tratamiento farmacológico , Síndrome de Fuga Capilar/inmunología , Ciclopropanos , Femenino , Humanos , Técnicas de Inmunoadsorción , Antagonistas de Leucotrieno/uso terapéutico , Quinolinas/uso terapéutico , Sulfuros , Resultado del Tratamiento
19.
J Immunol ; 170(7): 3835-42, 2003 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-12646651

RESUMEN

We evaluated whether IL-4, a cytokine critical for inducing allergic responses, also contributes to the effector phase of allergy. Pretreatment of mice with IL-4 or the related cytokine, IL-13, rapidly and dramatically increased the severity of anaphylaxis induced by cross-linking Fc(epsilon)RI or FcgammaRIII. This effect was inhibited by endogenously produced IFN-gamma, was T cell-, B cell-, and common gamma-chain-independent, and required IL-4Ralpha and Stat6. IL-4Ralpha signaling also enhanced anaphylaxis in mice infected with a nematode parasite that stimulates IL-4/IL-13 production. IL-4 exacerbated anaphylaxis by acting synergistically with vasoactive mediators to increase vascular permeability. Synergy between IL-4 and vasoactive mediators during the effector phase of allergic inflammation may both contribute to allergic immunopathology and enhance protective immunity against gastrointestinal worms.


Asunto(s)
Anafilaxia/inmunología , Interleucina-4/efectos adversos , Interleucina-4/fisiología , Adyuvantes Inmunológicos/efectos adversos , Adyuvantes Inmunológicos/antagonistas & inhibidores , Adyuvantes Inmunológicos/biosíntesis , Adyuvantes Inmunológicos/fisiología , Anafilaxia/patología , Anafilaxia/fisiopatología , Anafilaxia/prevención & control , Animales , Síndrome de Fuga Capilar/sangre , Síndrome de Fuga Capilar/inmunología , Síndrome de Fuga Capilar/fisiopatología , Relación Dosis-Respuesta Inmunológica , Quimioterapia Combinada , Femenino , Inyecciones Intravenosas , Interleucina-12/administración & dosificación , Interleucina-12/uso terapéutico , Interleucina-13/efectos adversos , Interleucina-13/fisiología , Interleucina-18/administración & dosificación , Interleucina-18/uso terapéutico , Interleucina-4/antagonistas & inhibidores , Interleucina-4/biosíntesis , Leucotrieno C4/administración & dosificación , Masculino , Ratones , Ratones Endogámicos BALB C , Ratones Noqueados , Ratones Desnudos , Ratones Transgénicos , Factor de Activación Plaquetaria/administración & dosificación , Factor de Transcripción STAT6 , Serotonina/administración & dosificación , Transducción de Señal/inmunología , Transactivadores/fisiología
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