RESUMEN
Few reports on recurrence after thoracoscopic bullectomy for spontaneous pneumothorax specify the follow-up period and follow-up ratio. Because of the variation in follow-up periods, many reported recurrence rates were not comparable. Some reports compared simple recurrence rate (number of recurrent cases/number of operated cases) of different groups with different follow-up periods. In this study, we employ the Kaplan-Meier method along with a set of optimal follow-up periods and ratios in order to determine a more reliable recurrence rate. Consecutive 68 patients (74 surgical procedures) underwent thoracoscopic bullectomy for spontaneous pneumothorax at our institution between November 2000 and December 2005. A follow-up survey was conducted by phone to determine the rate of recurrent pneumothorax. The follow-up ratio and the mean follow-up period were 92.6% and 1,316 +/- 481 days, respectively. Postoperative recurrence was confirmed for 4 patients. The interval up to recurrence was 144, 345, 476 and 616 days after the bullectomy, respectively. All cases of recurrent pneumothorax occurred within 2 years following the bullectomy. The 1-year, 2-year and 3-year cumulative recurrence rate was 3.0%, 6.3% and 6.3%, respectively. In light of these findings, we feel that comparison analysis of pneumothorax recurrence rates should be evaluated using the Kaplan-Meier method, furthermore, our data suggests that a follow-up period of 2 or more years is advisable.
Asunto(s)
Neumotórax/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias , Recurrencia , Adulto JovenRESUMEN
Behçet's disease is known to be associated with HLA-B51. To address the possibility that a non-human leukocyte antigen (HLA) gene closely linked to the HLA-B gene, such as tumor necrosis factor (TNF)-alpha, TNF-beta, or ECl (the locus that determines the susceptibility to alloreactive natural killer [NK] cells), is involved in the susceptibility to Behçet's disease, NcoI and EcoRI restriction fragment length polymorphisms in the TNF-beta gene and the susceptibility to lysis by alloreactive NK cells were investigated in Behçet's patients. In our NcoI restriction fragment length polymorphism (RFLP) analysis in the TNF-beta gene, the frequency of the NcoI 5.5 kb homozygote was decreased considerably in the patients, especially those with the ocular lesions, in relation to the healthy controls. However, no significant difference was observed between these groups in the EcoRI RFLP band distribution in this gene or the in susceptibility to lysis by alloreactive NK cells. These results indicated that a non-HLA gene located around the TNF gene region centromic of the HLA-B gene was a candidate to control the genetic susceptibility to Behçet's disease.
Asunto(s)
Síndrome de Behçet/genética , Síndrome de Behçet/inmunología , Células Asesinas Naturales/inmunología , Linfotoxina-alfa/genética , Secuencia de Bases , Southern Blotting , Citotoxicidad Inmunológica/inmunología , ADN/análisis , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Antígenos HLA-B/genética , Humanos , Datos de Secuencia Molecular , Hibridación de Ácido Nucleico , Reacción en Cadena de la Polimerasa , Polimorfismo Genético , Polimorfismo de Longitud del Fragmento de RestricciónRESUMEN
We performed a clinical open trial to evaluate the efficacy and the adverse side effects of a single therapy with FK506 in refractory uveitis as a multicenter study in Japan. Fifty-three patients (41 patients with Bechçet's disease, five with Vogt-Koyanagi-Harada disease, four with idiopathic retinal vasculitis, and three with other forms of uveitis) were enrolled in the study. FK506 was given orally for 12 weeks. Treatment with FK506 exhibited therapeutic effects in a dosage-dependent manner: the effectiveness was 38% in patients treated with an initial dosage of 0.05 mg/kg of body weight per day, 60% with 0.10 mg/kg of body weight per day, 83% with 0.15 mg/kg of body weight per day, and 79% with 0.20 mg/kg of body weight per day. Overall efficacy with dosage adjustment when needed was 76.5% at the conclusion of the study at the end of the 12th week. The FK506 therapy induced a variety of adverse side effects, the incidence of which depended on the dosage. The major side effects were renal impairment (28.3%, 15 of 53 patients), neurologic symptoms (20.8%, 11 of 53 patients), gastrointestinal symptoms (18.9%, ten of 53 patients), and hyperglycemia (13.2%, seven of 53 patients). The trough level of FK506 in the whole blood correlated with both the efficacy of the therapy and with the incidence of adverse effects. It is recommended to maintain the trough level between 15 and 25 ng/ml. On the basis of these results, a daily dosage of 0.10 to 0.15 mg/kg of body weight per day was suggested as an appropriate therapeutic dosage for refractory uveitis.
Asunto(s)
Tacrolimus/uso terapéutico , Uveítis/tratamiento farmacológico , Administración Oral , Adulto , Anciano , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Humanos , Japón , Masculino , Persona de Mediana Edad , Tacrolimus/administración & dosificación , Tacrolimus/efectos adversos , Agudeza VisualRESUMEN
Thirteen cerebrospinal fluid specimens (CSFs) from 10 patients with neuro-Behçet's syndrome (nB) were examined for the presence of conversion products of C3 protein by means of crossed immunoelectrophoresis. C3 conversion products were found in 4 CSFs from nB patients, and the proportions of the split products to the total immunoprecipitate zones were 17, 34, 61 and 63%, respectively. CSFs with C3 conversion patterns showed higher C3 protein (beta 1C/A) levels compared with those with non-conversion, whereas the differences of C4 protein (beta 1E) levels were unremarkable. CSF with C3 conversion patterns also showed apparent inflammatory characteristics indicating blood-CSF barrier involvement which would be deduced from increased alpha 2-macroglobulin and IgA, both being regarded as sensitive internal barrier parameters: but IgA measurement was much higher than usually expected values. It is suggested that the increase in CSF C3 might be initiated by accelerated consumption of complement through the alternative pathway, leading to hypersynthetic or hyperinflux response in CSF.
Asunto(s)
Síndrome de Behçet/líquido cefalorraquídeo , Complemento C3/líquido cefalorraquídeo , Humanos , Inmunoelectroforesis Bidimensional , Inmunoglobulina A/líquido cefalorraquídeo , alfa-Macroglobulinas/líquido cefalorraquídeoRESUMEN
The notion that autoimmune mechanisms play a role in the pathogenesis of certain uveitic conditions in humans is supported by the observation that lymphocytes from such patients respond in culture against retinal specific antigens which are uveitogenic in animals. A large proportion of uveitis patients with Behçet's disease are reported to respond well to S antigen, to interphotoreceptor retinoid binding protein (IRBP) and to several of their uveitogenic peptides, in particular, the S antigen derived peptide M. Patients with Behçet's disease without ocular involvement were reported not to differ in their responses to S antigen from the responses in the control group, yet 35% of them responded to IRBP and approximately two thirds of them responded to the peptides (peptide M, peptide N, R-4, or R-14). The responses were inhibited by monoclonal antibodies to CD4 and to class II MHC HLA-DR molecules. The presence of lymphocyte responses to retinal antigens in patients with Behçet's disease without uveitis might indicate a preclinical stage of ocular involvement. Thus, these data support the idea that autoimmunity to retinal specific antigens may play a role in the ocular inflammation in Behçet's disease.
Asunto(s)
Antígenos/inmunología , Síndrome de Behçet/inmunología , Proteínas del Ojo/inmunología , Proteínas de Unión al Retinol/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Secuencia de Aminoácidos , Anticuerpos Monoclonales/inmunología , Formación de Anticuerpos , Antígenos/química , Arrestina , Autoinmunidad , Proteínas del Ojo/química , Femenino , Humanos , Inmunidad Celular , Activación de Linfocitos , Masculino , Persona de Mediana Edad , Datos de Secuencia Molecular , Proteínas de Unión al Retinol/química , Linfocitos T/inmunología , Uveítis/inmunologíaRESUMEN
Two analogs of cyclosporine A (CsA), cyclosporine G (CsG) and cyclosporine D (CsD), were compared to CsA with regards to their effects on experimental autoimmune uveoretinitis (EAU) as well as their adverse effects on renal functions. When administered daily on days 0-14 after immunization with S-antigen, CsA was the most effective of all in inhibiting EAU followed by CsG: 20-30 mg/kg/day of CsG appeared to have the same effect as 5 mg/kg/day of CsA. The effect of CsD was the least. When administered from day 7 after immunization, CsA and CsG were also effective in inhibiting the development of EAU. As for the adverse side effects, CsA was the most nephrotoxic: the toxic changes were morphologically found with doses of CsA at 10 mg/kg/day or higher. CsG and CsD were not nephrotoxic even at 30 mg/kg/day. The effects of CsG on immune responses were very similar to those of CsA. Both agents exhibited selective inhibition on the cell-mediated immune responses to S-antigen, while having no effect on antibody production.
Asunto(s)
Enfermedades Autoinmunes/tratamiento farmacológico , Ciclosporina , Ciclosporinas/farmacología , Retinitis/tratamiento farmacológico , Uveítis/tratamiento farmacológico , Animales , Formación de Anticuerpos/efectos de los fármacos , Antígenos/inmunología , Arrestina , Ciclosporinas/efectos adversos , Ciclosporinas/sangre , Proteínas del Ojo/inmunología , Inmunidad Celular/efectos de los fármacos , Riñón/efectos de los fármacos , Riñón/fisiopatología , Pruebas de Función Renal , Masculino , Ratas , Ratas Endogámicas Lew , Retinitis/inmunología , Pruebas Cutáneas , Uveítis/inmunologíaRESUMEN
In order to investigate the immunogenetic mechanism of Behçet's disease, frequencies of HLA antigens were studied in patients. The subjects consisted of 66 patients and 99 normal controls. A lymphocyte cytotoxicity test was used for typing HLA-A, -B, -C, -DR, -DQ antigens. HLA-DP antigens were analyzed by the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. A significant increase of HLA-B15 was observed in the patients. In contrast, no significant difference was observed in HLA-Bw52 which possesses only two different amino acids from HLA-B15. On the contrary, frequencies of HLA-A11, HLA-Aw33, HLA-B35, HLA-B44 and HLA-DQw1 were significantly lower in the patients than in the controls. No significant difference was observed in HLA-DP antigens. These results suggest that Behçet's disease involves both disease susceptibility factors and disease resistance factors and that such genetic factors are mapped within or very close to the HLA-B gene in the class I gene region. Additionally class II HLA-DQ antigen is associated with disease resistance factors.
Asunto(s)
Síndrome de Behçet/genética , Antígenos HLA/genética , Secuencia de Aminoácidos , Mapeo Cromosómico , Femenino , Antígenos HLA-B/genética , Antígenos HLA-DQ/genética , Humanos , Masculino , Datos de Secuencia MolecularAsunto(s)
Síndrome de Behçet , Adulto , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/epidemiología , Síndrome de Behçet/inmunología , Síndrome de Behçet/patología , Enfermedades Cardiovasculares/etiología , Epididimitis/etiología , Femenino , Enfermedades Gastrointestinales/etiología , Humanos , Inmunidad Celular , Artropatías/etiología , Enfermedades Pulmonares/etiología , Masculino , Persona de Mediana Edad , Enfermedades del Sistema Nervioso/etiología , Enfermedades de la Piel/etiología , Estomatitis Aftosa/etiología , Úlcera/etiologíaRESUMEN
A multicenter open study was conducted to investigate optimum dose schedule of FK506 on refractory uveitis associated with Behçet's disease and allied conditions. Fifty-three patients (41 with Behçet's and 12 with allied conditions) were enrolled in this study. A daily oral dose of FK506 was initially 0.05, 0.1, 0.15 or 0.2 mg/kg, but adjusted in more than half patients during the study based on clinical conditions of patients and/or adverse effects of FK506. The improvement rate of initial daily dose as well as final improvement rate were evaluated in the study. The improvement rate of initial daily dose was increased dose-dependently; 37.5% with 0.05 mg/kg initial daily dose group, 60.0% with 0.1 mg/kg, 91.7% with 0.15 mg/kg and 78.6% with 0.2 mg/kg. The final improvement rate was 76.5%. In patients with Behçet's disease, ocular symptoms improved in 30 (75.0%) of 40 patients evaluable for efficacy and the frequency of ocular attacks was significantly reduced. In eight (66.7%) of 12 patients with Behçet's disease in whom cyclosporin treatment had been failed, their ocular symptoms improved by FK506. Main adverse reactions of FK506 were renal impairment (28.3%), neurologic symptoms (22.6%), gastrointestinal symptoms (20.8%), hypomagnesemia (28.3%), hyperkalemia (13.2%), and hyperglycemia (13.2%. Most of the adverse effects disappeared or ameliorated after FK506 dose reduction or withdrawal from FK506 therapy. It seems that the incidence of the adverse effects depends on the dosage of FK506. The lower dosage (0.05 and 0.1 mg/kg) caused a relatively small number of adverse effects, and the higher dosage (0.15 and 0.2 mg/kg) caused them more frequently. Through level is recommended to maintain between 15-25 ng/ml during early days of treatment based on the safety and efficacy. It is also recommended that a initial daily dose is 0.15 mg/kg on the basis of the efficacy and safety results, and then adjusted based on symptoms of patients and whole blood through level of FK506.
Asunto(s)
Síndrome de Behçet/complicaciones , Inmunosupresores/administración & dosificación , Tacrolimus/administración & dosificación , Uveítis/tratamiento farmacológico , Administración Oral , Adolescente , Adulto , Anciano , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Humanos , Inmunosupresores/efectos adversos , Masculino , Persona de Mediana Edad , Tacrolimus/efectos adversos , Uveítis/complicacionesRESUMEN
Serum dipeptidyl aminopeptidase IV (DAP IV) activity in rats with adjuvant arthritis (AA) were studied. DAP IV activity was significantly low in AA rats (37.7 +/- 14.1 mU/ml, n = 15) compared with control (63.7 +/- 9.0 mU/ml, n = 8) and indomethacin-treated (IDM) AA rats (55.6 +/- 14.2 mU/ml, n = 15). In addition, serum DAP IV activity was inversely correlated with hindpaw arthritis score both in AA rats and IDM-AA rats. Among serum inflammatory index examined, acid soluble glycoprotein, sialic acid were inversely correlated and albumin was positively correlated with DAP IV activity in AA and IDM-AA rats. The role of serum DAP IV remains unknown, but, it seems possible that DAP IV might modify the clinical course of AA rats in connection with its effects on the immune regulatory system.
Asunto(s)
Artritis Experimental/enzimología , Dipeptidil-Peptidasas y Tripeptidil-Peptidasas/sangre , Animales , Artritis Experimental/inmunología , Dipeptidil Peptidasa 4 , Masculino , Ratas , Ratas EndogámicasRESUMEN
A case of Behçet's disease with right hemiplegia and left oculomotor nerve involvement is described. Computed tomography of the brain with or without contrast, and nuclear magnetic resonance demonstrated a low attenuation mass lesion in the right mid brain. Lumbar puncture revealed there was moderate leukocytosis in the cerebrospinal fluid. Corticosteroids seemed effective in reducing her clinical symptoms, and our patient started walking one week after taking 50 mg of prednisolone. This case of neuro-Behçet's disease is the first with Weber's syndrome with documentation of the lesion by means of nuclear magnetic resonance.
Asunto(s)
Síndrome de Behçet/complicaciones , Hemiplejía/etiología , Oftalmoplejía/etiología , Adulto , Tronco Encefálico/diagnóstico por imagen , Femenino , Hemiplejía/diagnóstico por imagen , Humanos , Espectroscopía de Resonancia Magnética , Mesencéfalo/diagnóstico por imagen , Oftalmoplejía/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
The efficacy and safety of oral cyclosporin 10 mg/kg per day in Behçet's disease were compared in a randomised double-masked study with those of colchicine, 1 mg orally per day, and were also investigated in a long-term open study. The double-masked study showed that cyclosporin was effective in treating not only the ocular manifestations of Behçet's disease but also oral aphthous ulcer, dermal lesions, and genital ulceration. Efficacy did not weaken during long-term treatment.
Asunto(s)
Síndrome de Behçet/tratamiento farmacológico , Colchicina/uso terapéutico , Ciclosporinas/uso terapéutico , Administración Oral , Síndrome de Behçet/sangre , Ensayos Clínicos como Asunto , Colchicina/administración & dosificación , Colchicina/efectos adversos , Ciclosporinas/administración & dosificación , Ciclosporinas/efectos adversos , Ciclosporinas/sangre , Método Doble Ciego , Esquema de Medicación , Femenino , Humanos , Enfermedades Renales/sangre , Enfermedades Renales/inducido químicamente , Estudios Longitudinales , Masculino , Distribución Aleatoria , Índice de Severidad de la Enfermedad , Agudeza Visual/efectos de los fármacosRESUMEN
Cardiac involvement in Behçet's disease is quite uncommon. We report a case of Behçet's disease with aortic regurgitation of which histology was examined. A 56-year-old male came to our hospital with a complaint of right cervical pain. Diagnosis of aortic regurgitation was made by echocardiography, cardiac catheterization and aortography. As the cardiac lesion worsened, the operation of the aortic valve replacement was performed. On pathological examination, there was marked fibrous thickening on the aortic valve and the rupture of elastic tissue in the media of aortic wall. There was no history of rheumatic fever nor syphilis, and dilation of orifice of the aorta was not observed. Thus these changes were probably due to Behçet's disease itself.
Asunto(s)
Insuficiencia de la Válvula Aórtica/etiología , Síndrome de Behçet/complicaciones , Insuficiencia de la Válvula Aórtica/patología , Síndrome de Behçet/patología , Humanos , Masculino , Persona de Mediana EdadRESUMEN
The sequential cascades in leukocyte adhesion and migration are important events in the development of inflammatory and immune responses in Behçet's disease. In an attempt to clarify the relation of active ocular lesions to inflammatory reactions covering entire body, we have detected LECAM-1, Mac-1 and CD44 expressed on the peripheral leukocytes in 24 Behçet patients and 15 healthy adults by flow cytometry. LECAM-1 and CD44 expression was dramatically decreased from the polymorphonuclear leukocytes (PMN) upon ocular attacks, whereas the changes in Mac-1 appeared not so striking. This tendency had continued into the recovery stage of ocular inflammation. T lymphocytes showed, on the other hand, no considerable variability in regard to the expression of cell surface LECAM-1, CD44 and also of Mac-1 even at the peak of active ocular inflammation. The results suggest that interaction between PMN and primed vascular endothelial cells might be apt to precede functional modifications of T lymphocytes, and it is also supposed that facilitation of activated PMN recruitment plays an essential role on ocular inflammation in Behçet's disease.
Asunto(s)
Síndrome de Behçet/inmunología , Moléculas de Adhesión Celular/sangre , Leucocitos/inmunología , Proteínas Portadoras/metabolismo , Femenino , Humanos , Receptores de Hialuranos , Selectina L , Antígeno de Macrófago-1/sangre , Masculino , Receptores de Superficie Celular/metabolismo , Receptores Mensajeros de Linfocitos/metabolismoRESUMEN
Esophageal involvement in Behcet's disease is very uncommon, only 21 cases have previously been reported. We have observed five cases with this disease, and investigated the clinical, morphological, and histological aspects of these cases. Esophageal lesions in Behcet's disease occurred more frequently in male patients with the incomplete type. Laboratory data revealed an elevated erythrocyte sedimentation rate and a strongly positive C-reactive protein. The middle portion of the esophagus was most commonly involved. There were several morphological forms of esophageal lesions including erosions, perforated ulcers, widely spreading esophagitis, and severe stenosis. Therefore, there was no unique form to the esophageal lesions in contrast to the ileocolonic ulcers in intestinal Behcet's disease. Histological examination revealed lymphocytic or neutrophilic infiltration in ulcerative lesions. Vasculitis was rarely observed.
Asunto(s)
Síndrome de Behçet/patología , Esófago/patología , Adulto , Síndrome de Behçet/complicaciones , Enfermedades del Esófago/etiología , Estenosis Esofágica/etiología , Esófago/diagnóstico por imagen , Oftalmopatías/etiología , Femenino , Enfermedades de los Genitales Femeninos/etiología , Enfermedades de los Genitales Masculinos/etiología , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de la Boca/etiología , Neutrófilos/patología , Radiografía , Úlcera/etiologíaRESUMEN
The 4th nationwide epidemiological survey on Behçet disease (BD), which included all patients with BD at 1,200 hospitals selected at random from 10,081 hospitals in Japan, was carried out by the BD Research Committee of the Ministry of Health and Welfare in 1991 to examine the epidemiological features of BD in Japan by comparing with previous surveys. 3,938 patients from these hospitals were examined by the Japanese diagnostic criteria of BD (JCBD) revised in 1987 and the International criteria for classification of BD (ICBD). Among these 3,938 patients, 622 patients were only suspected of having BD or clinical signs of the disease were unknown, and most of these patients were incompatible with the ICBD. So these patients were excluded from the study of epidemiological features. The average patients age has risen 7-8 years over the last 20 years and the average age of onset in both sexes increased by about 3 years from 1972 to 1991. While a decrease in the sex ratio was seen in the complete-type and the incomplete-type BD without ocular symptoms, a sustained high sex ratio was shown in incomplete-type BD with ocular symptoms. The positive rate of HLA-B51 antigen was 54.9% (men: 56.9%, women: 52.2%) significantly higher than die 15-16% in healthy subjects but it might have been gradually decreasing. Also the clinical course of BD has become too mild for prognosis. According to diese epidemiological features of BD, the clinical manifestation of BD in Japan might have become the Western type of BD.