RESUMEN
OBJECTIVE: To analyze efficacy and safety of rethymectomy in patients with pathology of thymus. MATERIAL AND METHODS: Nine patients (2 males and 7 females) underwent rethymectomy in the thoracic surgery department of the Hertzen Research Institute of Oncology for the period from March 2009 to December 2019. Initial thymectomy for myasthenia gravis was performed in 6 patients, for thymoma without myasthenia - in 3 patients. Age of patients varied from 27 to 75 years (median 42.8 years). Myasthenia manifested at the age of 25-61 years (median 29.2 years). Period between manifestation and thymectomy varied from 6 to 24 months (median 12.6 months). MGFA grade IIIa was in 1 patient, grade IIIb - in 1, grade IVa - in 1, grade IVb - in 2, grade V - in 1 patient. Rethymectomy was performed via sternotomy in 4 cases, through thoracoscopy - in 5 patients. RESULTS: Postoperative complications occurred in 2 (22.2%) patients. Biopsy revealed residual thymic tissue in all patients. Median follow-up after rethymectomy was 30.2 months (range 12-132 months). Complete stable remission was achieved in 3 (50.0%) patients, remission - in 2 cases, partial remission - in 1 patient. Median dose of steroids before rethymectomy was 40 mg (range 16-96 mg), median dose after rethymectomy - 8 mg (range 0-24 mg). Differences were significant (p=0.04). All patients operated on for thymoma or recurrence are alive within 12-124 months after rethymectomy. CONCLUSION: Rethymectomy is a safe and effective treatment option for patients with refractory myasthenia gravis (especially in case of detected residual thymic tissue) or recurrent thymoma. Radical surgery for recurrent thymoma ensures favorable survival.
Asunto(s)
Miastenia Gravis , Timoma , Neoplasias del Timo , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/diagnóstico , Miastenia Gravis/cirugía , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Timectomía/efectos adversos , Timoma/diagnóstico , Timoma/cirugía , Neoplasias del Timo/complicaciones , Neoplasias del Timo/diagnóstico , Neoplasias del Timo/cirugía , Resultado del TratamientoRESUMEN
Thirty-seven patients with myasthenia gravis (MG) underwent AChR-Ab analysis, clinical study and neurophysiological examination - repetitive nerve stimulation (RNS). About 16,2% of MG patients who were anti-AChR-negative constituted a so-called seronegative MG group (SNMG). Compared to the AChR-Ab positive patients (SPMG), the SNMG was characterized by the higher female/male ratio (6:1), higher frequency of infantile onset of MG (33,3%), absence of association with thymoma and highest frequency of myasthenic crisis (83,3%). The clinical pattern of SNMG differed from SPMG and was characterized by predominant affect of mimic bulbar and respiratory muscles that determined severity of the course and high frequency of myasthenic crises. The identical clinical pattern was found in 19,3% of SPMG patients. However the character of neuromuscular transmission in orbicularis oculi muscle was different in SNMG and this SPMG-group. The pathological decrement was observed in 83,3% muscles of the SPMG-group (from -20% to -74%) and only in one case in the SNMG-group (-48%). Besides, the absence of clinical and neurophysiological responses to anticholinesterase was noted in the SNMG-group. Cholinergic neuromuscular hyperactivity in SNMG patients manifested itself in clinical fasciculations and myokymic contractions of muscles which prevailed in facial muscles in 66,7% of SNMG patients. Neurophysiologic examination displayed extra repetitive discharges after the compound motor action potential (R-CMAP) at low-frequency stimulation after acetylcholine esterase inhibitors in 100% cases.
Asunto(s)
Miastenia Gravis/diagnóstico , Receptores Colinérgicos/inmunología , Adolescente , Adulto , Anciano , Autoanticuerpos/sangre , Niño , Inhibidores de la Colinesterasa/uso terapéutico , Diagnóstico Diferencial , Electrofisiología , Potenciales Evocados Motores , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/sangre , Miastenia Gravis/clasificación , Miastenia Gravis/tratamiento farmacológico , Miastenia Gravis/inmunología , Miastenia Gravis/fisiopatología , Neostigmina/uso terapéutico , Unión Neuromuscular/fisiopatología , Neurofisiología , Receptores Colinérgicos/efectos de los fármacos , Factores SexualesAsunto(s)
Anticuerpos/sangre , Síndrome Miasténico de Lambert-Eaton/sangre , Síndrome Miasténico de Lambert-Eaton/inmunología , Miastenia Gravis/sangre , Miastenia Gravis/inmunología , Receptores Colinérgicos/inmunología , Adulto , Factores de Edad , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Timoma/sangre , Timoma/inmunologíaRESUMEN
A detailed comparative analysis of neurologic symptoms in 45 patients with Lambert-Eatone myastenic syndrome (LEMS) and 42 patients with generalized myasthenia allowed us to single out clinical patterns facilitating disease diagnosis and differentiation. There were no clinical differences in patients having LEMS with or without paraneoplastic process. Electromyography study revealed the presence of typical phenomena: a reduced amplitude of compound muscle action potential and incremental response at 40-Hz stimulation. The compound muscle action potential tripled after 20 s of maximal voluntary contraction. Sera from 89% of patients with LEMS contained IgG antibodies that immunoprecipitate a radiolabeled complex of a selective antagonist of P/Q type voltage-gated calcium channels. The degree of inhibition of calcium influx by patient's IgG correlated with the reduction in amplitude of the resting compound muscle action potential.
Asunto(s)
Síndrome Miasténico de Lambert-Eaton , Potenciales de Acción , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Autoanticuerpos/sangre , Autoinmunidad , Canales de Calcio/inmunología , Carcinoma Broncogénico/complicaciones , Diagnóstico Diferencial , Electromiografía , Electrofisiología , Femenino , Humanos , Inmunoglobulina G/sangre , Síndrome Miasténico de Lambert-Eaton/complicaciones , Síndrome Miasténico de Lambert-Eaton/diagnóstico , Síndrome Miasténico de Lambert-Eaton/inmunología , Síndrome Miasténico de Lambert-Eaton/fisiopatología , Neoplasias Pulmonares/complicaciones , Masculino , Persona de Mediana Edad , Modelos Teóricos , Contracción Muscular , Fatiga Muscular/fisiología , Músculos/fisiopatología , Miastenia Gravis/diagnóstico , Pruebas de Precipitina , Timoma/complicaciones , Neoplasias del Timo/complicacionesAsunto(s)
Autoanticuerpos/inmunología , Síndrome Miasténico de Lambert-Eaton/inmunología , Miastenia Gravis/inmunología , Receptores Colinérgicos/inmunología , Adolescente , Adulto , Anciano , Biomarcadores/sangre , Diagnóstico Diferencial , Femenino , Humanos , Síndrome Miasténico de Lambert-Eaton/sangre , Síndrome Miasténico de Lambert-Eaton/diagnóstico , Masculino , Persona de Mediana Edad , Miastenia Gravis/sangre , Miastenia Gravis/diagnóstico , Radioinmunoensayo , Receptores Colinérgicos/sangreAsunto(s)
Homocigoto , Mutación Missense/genética , Miastenia Gravis/genética , Mutación Puntual/genética , Regiones Promotoras Genéticas/genética , Subunidades de Proteína/genética , Receptores Colinérgicos/genética , Adulto , Análisis Mutacional de ADN , Electromiografía , Femenino , Humanos , Músculo Esquelético/fisiopatología , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , LinajeRESUMEN
Acethylcholine receptor (AChR) antibodies in patients with myasthenia were studied for the first time in Russia with radioimmuno-assay application. Seronegative result (antibody titer lower than 0.2 nmol/l) was obtained for 33% of the patients and seropositive one (antibody titer higher than 0.2 nmol/l)--for 67%. AChR antibodies level varied from 1.9 to 115.0 nmol/l. The AChR antibodies were found more frequently and in higher titers in women than in men. Correlation analysis revealed strong correlation between AChR antibodies level and severity of myasthenia.
Asunto(s)
Anticuerpos/inmunología , Miastenia Gravis/inmunología , Receptores Colinérgicos/inmunología , Adolescente , Adulto , Femenino , Humanos , Masculino , Miastenia Gravis/epidemiología , Miastenia Gravis/cirugía , Cuidados Posoperatorios , Cuidados Preoperatorios , Índice de Severidad de la Enfermedad , TimectomíaRESUMEN
In experiments in ovo mixed chlamydial and gonococcal infection has been obtained by the successive infection of developing chick embryos with C. trachomatis and N. gonorrhoeae into the yolk sack. The competitive interrelations between the associated microorganisms with respect to their pathogenicity characteristics for chick embryos have not been established. This simulator is intended for use in the primary selection of etiotropic chemical preparations capable of producing combined effect on C. trachomatis and N. gonorrhoeae.
Asunto(s)
Infecciones por Chlamydia/microbiología , Modelos Animales de Enfermedad , Gonorrea/microbiología , Animales , Embrión de Pollo , Chlamydia trachomatis/patogenicidad , Neisseria gonorrhoeae/patogenicidad , Factores de Tiempo , Saco Vitelino/microbiologíaRESUMEN
The detection of the differentiated chemotherapeutic activity of tetracyclin and penicillin has been used as an example for demonstrating the possibility of using the experimental in ovo model of mixed chlamydial and gonococcal infection for the detection and primary selection of effective etiotropic preparations, simultaneously affecting Chlamydia trachomatis and Neisseria gonorrhoeae.
Asunto(s)
Infecciones por Chlamydia/tratamiento farmacológico , Modelos Animales de Enfermedad , Gonorrea/tratamiento farmacológico , Penicilina G/uso terapéutico , Tetraciclina/uso terapéutico , Animales , Embrión de Pollo , Infecciones por Chlamydia/microbiología , Chlamydia trachomatis/efectos de los fármacos , Evaluación Preclínica de Medicamentos/métodos , Gonorrea/microbiología , Neisseria gonorrhoeae/efectos de los fármacos , Factores de Tiempo , Saco Vitelino/microbiologíaRESUMEN
N. gonorrhoeae strain b has been found to be capable of retaining its viability in medium 199 with 10% of inactivated cattle serum added and in monolayer cell culture L-929 in the above medium. The characteristics obtained in the present investigation permit simulating the mixed association of gonococci and chlamydiae in the culture system used in this work.
Asunto(s)
Células L/microbiología , Neisseria gonorrhoeae/crecimiento & desarrollo , Animales , Técnicas Bacteriológicas , Bovinos , División Celular , Medios de Cultivo/metabolismo , Sueros Inmunes , Ratones , Neisseria gonorrhoeae/citología , Factores de TiempoRESUMEN
Morphology and ultrastructure of Halprowia arthritidis, strain SR-1 (HSR), isolated from the synovial fluid of a patient with Reiter's syndrome, was studied in the membranes of the yolk sacs of the developing chick embryos and the L-cell culture. In acridine orange staining for light and fluorescent microscopy there was revealed intracellular cytoplasmic inclusions containing HSR structures at various stages of its reproduction characteristic of halprowia (chlamydia). The direct immunofluorescent method demonstrated the presence of a characteristic HSR antigen not only in the developed inclusions, but also at the early stages of infection, when the morphological HSR structures could not be found by light microscopy. The ultrastructure of the HSR inclusions and forms in the cycle of development (of the initial and elementary bodies) of the SR-1 strain was typical of other halprowia. A peculiar structure of a complex of cell wall and cytoplasmic membrane of the elementary body was described. Taking into consideration the biological characteristics of HSR revealed earlier it can be considered to be a typical representative of Halprowiales s. Chlamydiales. The data obtained on other halprowia, pointing out the fact that criteria of compactness and diffuseness of inclusions, the presence of absence of glycogen in the inclusions could not serve as taxonomic signs in classification of halrpowia, were confirmed on a model of the SR-1 studied.