Thymectomy for myasthenia gravis in children.
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Biomedical subjects
Publications and source records attributed to C Herrmann.
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Two disorders of neuromuscular transmission producing muscle weakness and easy fatigability which may confront the physician are myasthenia gravis and the myasthenic syndrome. The former has early symptoms and signs of oculobulbar and then extremity weakness with rapid decline of action potential and contractile strength with repetitive use and nerve-muscle stimulation. Anticholinesterases improve strength. The myasthenic syndrome has early symptoms and signs of pelvic girdle, pectoral girdle and proximal limb muscle weakness. This is worst when first starting to use or carry out nerve muscle stimulation in the rested muscles. It improves significantly for a time with use or on rapid stimulation, and then declines with continued activation. Deep tendon reflexes are sluggish or absent. Small cell carcinoma of the lung is often associated. Guanidine improves the strength. Other features and possible underlying mechanisms of the two disorders help to differentiate and treat them.
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Physicians should consider the possibility of myasthenia gravis in patients complaining of fluctuating muscle weakness and easy fatigability. The disorder may occur in either sex at any age. Repeated measurements of multiple muscle groups and functions to include baseline strength and response to a control as well as anticholinesterase drugs are desirable in reaching a diagnosis. Appropriate doses of anticholinesterase medication reduce weakness and fatigability in most patients. Selected patients may benefit from thymectomy. Patients in crisis failing to respond to anticholinesterase drugs, having difficulty maintaining a patent airway or adequate respiratory exchange are best managed by prompt tracheostomy using a cuffed tube, with adequate tracheobronchial toilet and mechanical respiratory assistance.
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Microscopical imaging of natural, unstressed draglines or of untreated bulk samples showed two types or threads with diameters of either approximately 1-2 microm or 4-5 microm, which could be identified as products of the minor or major ampullate glands. The threads had a circular profile in serial cross sections and are surrounded by a thin outer layer of a different material within the section. Such fibrillar configurations were also found in untreated threads or in the same serial sections of transmission electron microscopy (TEM) samples by means of the special technique of laser scanning microscopy. In TEM slides, numerous cavities with the same circular profile were detectable, and the length of these cavities is variable from 40-300 nm. The threads are oriented parallel and twisted around themselves to construct a double thread. In the interface between the two single threads, bridge-like structures are prominent. The single untreated thread consists of cylindrical fibers with a diameter of approximately 1-1.5 microm. Apparently more than eight fibers are within a thread and each fiber is composed of a great number of fibrils with a diameter of about 150 nm. The surface of threads is coated with a characteristic layer approximately 150-250 nm thick that contains glycoproteins. These were demonstrated for the first time by labeling with concanavalin A lectin-gold complex and are dependent on the diameter and length of the thread. The same substances could also be detected inside the single thread. The skin can be removed completely or partially by mechanical treatment, or by washing with phosphate-buffered saline or trypsin.
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OBJECTIVE: Beyond acute myocardial infarction, little is known about the effect of depression, and especially anxiety, on prognosis in cardiology patients. The present study aims to examine the effect of anxiety and depression on 5-year mortality in patients referred for exercise testing. METHODS: A total of 5,057 patients referred for routine exercise testing completed the Hospital Anxiety and Depression Scale (HADS) before undergoing the exercise test. Survival data were obtained from 5,017 (99.2%) of those patients after 5.7 +/- 0.8 years. HADS scores and cardiological baseline data were used to predict mortality. RESULTS: In univariate analyses, HADS depression was not a significant predictor; high anxiety was associated with improved survival. Logistic regression revealed nine independent objective predictors from which we computed a composite somatic risk index. When controlling for this physical risk index, anxiety and depression had independent, opposite effects; that is, anxiety was associated with a lower mortality and depression with a higher mortality. CONCLUSION: Anxiety and depression scores have different predictive effects on mortality in patients referred for exercise testing. These effects are independent of a highly effective physical risk index, suggesting that psychological screening of cardiology patients might improve risk stratification.
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OBJECTIVE: While depression has been found to predict mortality in acute myocardial infarction, results from many other groups of medical patients are inconclusive. It is, therefore, unclear whether depression also predicts mortality in the typical mixed patient populations treated on medical hospital wards and whether an increased risk can be identified by means of patients' self ratings of depression. METHOD: The Hospital Anxiety and Depression scale was used as a routine screening tool in consecutive admissions to the general medical wards of a university hospital. The official survival data were obtained 22 months later. For all 454 patients who completed the screening questionnaire, complete survival data were available. RESULTS: High depression scores significantly predicted mortality in univariate comparisons (odds ratio 3.2; 95% CI 1.9-5.5) and in multivariate Cox regression analyses controlling for demographic and medical baseline variables (multivariate odds ratio 1.9; 95% CI 1.2-3.1; p < .01). Other significant predictors in the multivariate model were having a principal diagnosis of hematological disease or cancer, and older age. Disability, as assessed by nurses' ratings, and gender were not related to mortality. Subgroup analyses showed that the effect of depression scores was greatest in cardiopulmonary patients, but there was also a uniform trend toward higher mortality in depressed patients with other diagnoses. CONCLUSION: Depressed mood is an independent risk factor for all-cause mortality in medical inpatients. Identifying patients at risk does not require formal psychiatric diagnoses, but can be achieved by means of a short, routinely administered self-rating questionnaire.
The visual observer performance for detecting low-contrast patterns by employing both the receiver operating characteristic (ROC) and the 9-alternative forced choice (9-AFC) method is investigated. For each observer and each experimental technique, the results were reproducible with respect to a repeated observation. When the results of different observers for each method are compared, a strong variation of values is found. From the ROC data obtained, the expected percentages of correct detection in a 9-AFC experiment is calculated and compared with the corresponding values of the actual 9-AFC experiment. For some observers, the latter values were significantly higher than the computed ones, whereas for others the results showed good agreement with predictions from theory.
BACKGROUND: In a cross-sectional study we investigated associations between self-ratings of anxiety and depression, physical factors and physicians' assessments of patients' medical status. METHODS: Hospital inpatients (n = 574) consecutively admitted to internal medical wards were evaluated for the presence of anxiety and depressive symptoms using the Hospital Anxiety and Depression Scale (HADS). Physicians were asked for their perception of psychiatric problems in their patients and for their assessments of patients' severity of illness, functional impairment and 1-year prognosis on 3-point ranking scales. Detailed somatic data including comorbidity and accepted risk factors were taken from the patients' records. RESULTS: Almost two thirds of all patients with abnormal HADS scores (65.2%) were not identified by their attending physicians as suffering from psychiatric problems. Using multiple-regression models, HADS scores for depression, but not for anxiety, proved to be an independent predictor for the clinicians' judgements of disease severity [exp(beta) = 1.08; 95% confidence interval (CI) 1. 03-1.13; p < 0.01] and functional impairment [exp(beta) = 1.11; 95% CI 1.05-1.17; p < 0.01]. The estimation of prognosis, however, was only related to physical predictors and showed no association with depressive symptoms or other psychosocial factors. CONCLUSIONS: Our data demonstrate that internists' ratings of severity of illness and functional impairment, but not prognosis, are associated with HADS depression scores, whereas there is no such association with self-rated anxiety.