El pico flujo espiratorio y la cuenta máxima son marcadores del compromiso respiratorio en la miastenia gravis / Peak expiratory flow and the single-breath counting test as markers of respiratory function in patients with myasthenia gravis

Introducción: La miastenia gravis (MG) es una enfermedad autoinmune mediada por anticuerpos. El cuadro clínico se caracteriza por debilidad muscular fluctuante y fatigable, con frecuente afectación de músculos fonodeglutorios y respiratorios. Dada la severidad que implica el compromiso respiratorio en la MG, su evaluación rutinaria es esencial.Nuestro objetivo fue identificar un marcador semiológico útil en la pesquisa del compromiso respiratorio en pacientes con MG.Métodos: Se realizó un trabajo observacional en pacientes con diagnóstico de MG. Los pacientes fueron evaluados con test de cuenta máxima, pico flujo espiratorio (PEF), cuestionario de disnea modificado (mMRC) y valoración de fuerza del cuello. Los resultados de estos parámetros fueron correlacionados con la medición de CVF (capacidad vital forzada) y presiones bucales estáticas máximas (PiMáx y PeMáx). Resultados:Cuarenta y cinco pacientes con MG fueron incluidos, dos pacientes tenían MGFA grado I, 35 grado II, siete grado III y uno grado IV al momento de la evaluación. Se halló una correlación positiva entre el test de cuenta máxima y la CVF (r = 0,57, p = 0,000), y entre el PEF y la CVF (r = 0,76, p = 0,000). El grado de disnea, según el mMRC, mostró una correlación negativa con la CVF (r =-0,31 p = 0,03). A su vez, el PEF correlacionó con la PeMáx de forma positiva, estadísticamente significativa (r = 0,51, p = 0,002).Conclusiones: El PEF, el test de cuenta máxima y el mMRC fueron útiles para evaluar la función respiratoria en pacientes con MG.(AU)

Introduction: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterised by fluctuating, fatigable muscle weakness, frequently involving bulbar and respiratorymuscles. Considering the severity of respiratory involvement in MG, routine evaluation ofrespiratory function is essential.The aim of this study was to identify a useful clinical marker of respiratory involvement inpatients with MG. Methods: We performed an observational study of patients with MG. All cases were evaluatedwith the single-breath counting test, peak expiratory flow (PEF), a modified Medical ResearchCouncil dyspnoea scale (mMRC), and a neck strength assessment. The results of these parameters were correlated with forced vital capacity (FVC), maximal inspiratory pressure (MIP), andmaximal expiratory pressure (MEP). Results: The study included 45 patients with MG: 2 patients classified as grade I on the Myasthenia Gravis Foundation of America classification at the time of evaluation, 35 classified asgrade II, 7 classified as grade III, and one classified as grade IV. Positive correlations were foundbetween single-breath counting test scores and FVC values (r = 0.57, p = .000), and betweenPEF and FVC values (r = 0.76, p = .000). Severity of dyspnoea according to the mMRC scaleshowed a negative correlation with FVC values (r = -0.31, p = .03). PEF also showed a significantcorrelation with MEP (r = 0.51, p = .002). Conclusions: PEF, the single-breath counting test, and the mMRC scale are useful measures forevaluating respiratory function in patients with MG.(AU)

Peak expiratory flow and the single-breath count test as markers of respiratory function in patients with myasthenia gravis.

INTRODUCTION: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterised by fluctuating, fatigable muscle weakness, frequently involving bulbar and respiratory muscles. Considering the severity of respiratory involvement in MG, routine evaluation of respiratory function is essential. The aim of this study was to identify a useful clinical marker of respiratory involvement in patients with MG. METHODS: We performed an observational study of patients with MG. All cases were evaluated with the single-breath count test, peak expiratory flow (PEF), a modified Medical Research Council dyspnoea scale (mMRC), and a neck strength assessment. The results of these parameters were correlated with forced vital capacity (FVC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). RESULTS: The study included 45 patients with MG: 2 patients classified as grade I on the Myasthenia Gravis Foundation of America classification at the time of evaluation, 35 classified as grade II, 7 classified as grade III, and one classified as grade IV. Positive correlations were found between single-breath count test scores and FVC values (r = 0.57, P = .000), and between PEF and FVC values (r = 0.76, P = .000). Severity of dyspnoea according to the mMRC scale showed a negative correlation with FVC values (r = -0.31, P = .03). PEF also showed a significant correlation with MEP (r = 0.51, P = .002). CONCLUSIONS: PEF, the single-breath count test, and the mMRC scale are useful measures for evaluating respiratory function in patients with MG.

Characterization of the hierarchical architecture and micromechanical properties of walnut shell (Juglans regia L.).

In the present work a comprehensive characterization of the hierarchical architecture of the walnut shell (Juglans regia L.) was carried out using scanning electron microscopy (SEM), atomic force microscopy (AFM) and confocal laser scanning microscopy (CLSM). Furthermore, micromechanical properties (hardness, HIT and elastic modulus, EIT) of plant tissues were evaluated at cell wall level by applying the instrumented indentation technique (IIT). The complex architecture of the material was described in terms of four hierarchical levels (HL): endocarp (H1), plant tissues (H2), plant cells (H3) and cell wall (H4). Our findings revealed that the walnut shell consists of a multilayer structure (sclerenchyma tissue, ST; interface tissue, IT; porous tissue, PT; and flattened parenchyma tissue, FPT), where differences in the microstructure and composition of plant tissues generate parallel gradients along the cross-section. The indentation tests showed a functional gradient with a sandwich-like configuration, i.e., a lightweight and soft layer (PT, HIT = 0.04 GPa) is located between two dense and hard layers (ST, HIT = 0.33 GPa; FPT, HIT = 0.28 GPa); where additionally there is an interface between ST and PT (IT, HIT = 0.16 GPa). This configuration is a successful strategy designed by nature to improve the protection of the kernel by increasing the strength of the shell. Therefore, the walnut shell can be considered as a functionally graded material (FGM), which can be used as bioinspiration for the design of new functional synthetic materials. In addition, we proposed some structure-property-function relationships in the whole walnut shell and in each of the plant tissues.

Effect of calcium oxalate crystals on the micromechanical properties of sclerenchyma tissue from the pecan nutshell (Carya illinoinensis).

The objective of this study was to evaluate the effect of the presence of calcium oxalate (CaOx) crystals on the micromechanical properties of sclerenchyma tissue from the pecan nutshell (Carya illinoinensis). The microstructure of the cross-section nutshell was examined using light microscopy (LM) and atomic force microscopy (AFM). Using an instrumented indentation system, indentation tests with maximum loads of 500 mN were made on the biological material where the variables studied were the number of crystals present in the evaluated area and the size of individual crystals. Microscopic analysis revealed that the pecan nutshell consists of sclerenchyma tissue with multiple CaOx crystals randomly distributed throughout the material, exhibiting prismatic shapes and various sizes. The results of the indentation tests showed that the examined areas where there were crystals (1, 2 or up to 3) presented values of hardness and elastic modulus significantly higher (P < 0.05) compared to the sclerenchyma (without crystals). Likewise, there were no significant differences (P > 0.05) between the values of the micromechanical properties of the areas evaluated as a function of the number of crystals. On the other hand, it was observed that the size of the crystals did not show a direct correlation with the mechanical properties evaluated as expected. In conclusion, the biomineralization phenomenon is a successful strategy designed by nature to improve the rigidity of the pecan nutshell, where the CaOx crystals strengthen the structure by increasing the micromechanical properties.

Peak expiratory flow and the single-breath counting test as markers of respiratory function in patients with myasthenia gravis. / El pico flujo espiratorio y la cuenta máxima son marcadores del compromiso respiratorio en la miastenia gravis.

INTRODUCTION: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterised by fluctuating, fatigable muscle weakness, frequently involving bulbar and respiratory muscles. Considering the severity of respiratory involvement in MG, routine evaluation of respiratory function is essential. The aim of this study was to identify a useful clinical marker of respiratory involvement in patients with MG. METHODS: We performed an observational study of patients with MG. All cases were evaluated with the single-breath counting test, peak expiratory flow (PEF), a modified Medical Research Council dyspnoea scale (mMRC), and a neck strength assessment. The results of these parameters were correlated with forced vital capacity (FVC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). RESULTS: The study included 45 patients with MG: 2 patients classified as grade I on the Myasthenia Gravis Foundation of America classification at the time of evaluation, 35 classified as grade II, 7 classified as grade III, and one classified as grade IV. Positive correlations were found between single-breath counting test scores and FVC values (r = 0.57, p = .000), and between PEF and FVC values (r = 0.76, p = .000). Severity of dyspnoea according to the mMRC scale showed a negative correlation with FVC values (r = -0.31, p = .03). PEF also showed a significant correlation with MEP (r = 0.51, p = .002). CONCLUSIONS: PEF, the single-breath counting test, and the mMRC scale are useful measures for evaluating respiratory function in patients with MG.

Intermittent inotropic therapy in an outpatient setting: a cost-effective therapeutic modality in patients with refractory heart failure.

Patients with intractable heart failure (New York Heart Association [NYHA] class III and IV) who were receiving maximal conventional treatment were enrolled in an outpatient program that included inotropic infusions, intensive patient education, and close follow-up. The effects of this approach to therapy were evaluated on (1) the number of hospital admissions, (2) length of stay, and (3) number of emergency room visits during the ensuing year. These data were compared with similar data from the year before entry in the program for each patient. Thirty-six patients with stable NYHA class III and IV heart failure received milrinone or dobutamine to manage chronic heart failure in an outpatient setting. The cause of heart failure was ischemic heart disease in 12, idiopathic in 11, hypertension in 8, and pulmonary hypertension in 5. Four patients received dobutamine and 32 patients received milrinone. The mean period of observation was 294 days. For the period before entry in the program, patients had 21 emergency room visits, 75 admissions, and 528 days spent in the hospital. After enrollment, patients had 10 emergency room visits, 34 admissions, and 150 days spent in the hospital. In conclusion, this therapeutic regimen reduced the number of hospital admissions, days spent in the hospital, and emergency room visits. Our study supports the concept that the use of intermittent inotropic therapy in the outpatient setting plays an important role in managing this severely ill group of patients.

[Paracoccidioidomycosis of intramedullar and cerebral localization]. / Paracoccidioidomicose de localização intramedular e cerebral.

It is reported our diagnosis and therapeutical experience with a patient that presented clinical, radiological and tomographical manifestations of paracoccidioidomycosis with involvement of lungs, brain and spinal cord. Besides being a rare case the authors call attention for the extreme difficulty in defining the final diagnosis, which was achieved only after laminectomy and surgical biopsy of the intra spinal cord tumor at the C5 level. The patient received two series of treatment with ketoconazole (400-600 mg/day). After both the patient presented tonic and clonic convulsive disorders. We speculated about a possible perigranulomatous inflammatory response related to the therapeutical intervention, not yet described in literature.

Paracoccidioidomicose de localizaçäo intramedular e cerebral / Paracoccidioidomycosis of intramedullary and cerebral localization

O presente relato de caso, descreve nossa experiência diagnóstica e terapêutica com um paciente que apresentou manifestaçöes clínicas, radiológicas e tomográficas de paracoccidioidomicose comprometendo os pulmöes, cérebro e, particularmente, a medula espinhal. Além da raridade do caso, chama-se a atençäo para a extrema dificuldade na elucidaçäo diagnóstica definitiva, obtida somente após laminectomia e biópisa cirúrgica de tumoraçäo iintramedular ao nvel de C5. O paciente recebeu duas séries de tratamento com ketoconazol (400 a 600 mg/dia). No início de ambas apresentou crise convulsiva tònico-clónica. Especula-se sobre uma possível resposta inflamatória perigranulomatosa intracerebral, relacionada à intervençäo terapêutica, ainda sem relato na literatura

[Correlation of HLA and myasthenia gravis in a Brazilian population]. / Estudo da correlação HLA e miastenia gravis em uma população brasileira.

Based on the fact that Brazilian ethnic groups are mixed and therefore different from caucasian, chinese or japanese populations the authors studied the HLA antigen distribution in 37 myasthenic patients. The control group consisted of 69 healthy individuals of the same population studied in the same laboratory. The antigens HLA B8 showed the highest relative risk for female, younger than 40 years old, with thymic hyperplasia. Also the HLA B8 and A1 showed high frequency for black myasthenic patients.

[Multimodal evoked potentials: standardized study of 51 individuals in a Brazilian population]. / Potenciais evocados multimodais: Estudos normativos em 51 indivíduos de uma população brasileira.

The authors report the results of normative studies of pattern-shift visual, brainstem auditory and somatosensory evoked potentials of upper and lower limbs carried out in a sample of 51 normal subjects of a Brazilian population. Normal values were thus established for the main components of each modality of evoked potentials, with special emphasis on those having more clinical utility. The results were compared to the data available in the literature.

Carotid artery disease following external cervical irradiation.

A retrospective study of 910 patients surviving at least five years after cervical irradiation for Hodgkin's disease, non-Hodgkin's lymphoma, or primary head an neck neoplasms showed the incidence of stroke following cervical irradiation was 63 of 910 patients (6.3%) during a mean period of observation of nine years. This represents a trend toward an increased risk for this population observed over the same period of time (p = 0.39). A prospective study of 118 similar patients currently living five years after cervical radiotherapy was performed to determine the incidence of carotid artery disease occurring as a consequence of neck irradiation. Abnormal carotid phonangiograms (CPA) were found in 25% of the patients and abnormal oculoplethysmographs (OPG) were found in 17%. These studies represent significant carotid lesions that are not expected in such a population. It is concluded that the carotid stenoses demonstrated are most likely a consequence of prior irradiation. Patients that are five-year survivors of cervical irradiation should have noninvasive vascular laboratory studies performed as part of their routine follow-up examinations in order to detect these carotid lesions while they are occult.

Pseudomyxoma peritonei.

Thirty-eight patients with pseudomyxoma peritonei were treated at the M. D. Anderson Hospital, Houston, from 1954 to 1978. The various treatment regimens used have provided actuarial survival rates of 54% at five years and 18% at ten years. Local or regional disease was the cause of death in 68% of patients, and no patient died of metastatic disease. Initial definitive surgery should consist of effective tumor reduction, omentectomy, appendectomy, and, in the female subject, bilateral oophorectomy. Most patients have been treated adjunctively with either fluorouracil or melphalan (L-phenylalanine mustard) depending on the presumed site of origin, but results in a small number of patients treated with either whole abdominal or strip abdominal radiotherapy suggest that this modality may offer improved survival. Treatment with adjunctive radiotherapy alone has provided a five-year survival rate of 75%, compared with 44% for chemotherapy.