Pulmonary function loss in patients on long-term anticonvulsive therapy.
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Analyzing international 15 years experiences the authors characterize the present situation of knowledges and practical possibilities concerning brain death diagnostics. It must be differentiated between generally accepted obliging criterions and the remaining space of responcibility of the neurologist, who is acting as a member of a brain death commission. In this frame procedures have to be chosen, which allow to diagnosticate without any doubt and as early as possible. Further developmental possibilities are shown.
12 patients, whose neurological findings had induced the procedure of brain death determination, and further 4 young children were examined correspondently by special CT methods. In conclusion cranial computerized tomography as a non-invasive method seems to be useful alternatively beside cerebral angiography for the documentation of cerebral circulation arrest.
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Motor lesions following herpes zoster are quite common. Hemiparesis, paraparesis, pareses of the facial and other cranial nerves as well as segmental pareses can be observed. We report on a patient suffering from zoster ophthalmicus complicated by paresis of the third cranial nerve. As a cause, a partial brain stem-encephalitis was diagnosed. The patient recovered after antiviral treatment (Aciclovir, Inosiplex).
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Patients with functional loss of visual acuity or visual fields range from the "deliberate malingerer" to the "suggestible innocent." Between these extremes are patients with varying mixtures of fraud and suggestibility. These patients do not, as a rule, have psychiatric disease and do not need to see a psychiatrist. The ophthalmologist must be able to control frustration with these patients to prove that the patient has better visual fields and visual acuity than admitted to, and so that he can perform a careful, dispassionate examination to establish that no organic disease is present. This examination makes it possible to offer believable reassurance to the patient. Simple reassurance seems to be effective therapy.
Visual function loss has been documented in diabetes mellitus in relation to flicker and contrast. However, no direct correlation between the degree of loss in sensitivity and the level of retinopathy has been established. It has been suggested that such non-invasive psychophysical procedures actually reflect metabolic disturbances within the diabetic retina. This study investigates the possibility of whether early nephropathy demonstrated by microalbuminuria, is an indicator of microangiopathy which may be a cause of retinal disturbance leading to a loss of visual function. The visual function of a group of diabetics showing microalbuminuria was studied. Contrast and flicker threshold were measured and the results compared with those obtained with an age-matched control diabetic group. The procedures used effectively separated the two groups and raises the issue of incorporating psychophysics in retinal screening programmes.
Hirschsprung disease (HSCR) is a congenital enteric neuropathy caused by disrupted development of enteric neural crest-derived cells (ENCDCs). Although pathogenic coding variants in RET account for many cases, the largest genetic contribution to HSCR risk arises from a common noncoding variant (rs2435357) within a SOX10-bound RET enhancer (MCS+9.7) that reduces RET gene expression in vivo and triggers expression changes in other ENS genes in the human fetal gut. However, the ENS cell types affected by this enhancer and the mechanisms by which these transcriptional changes lead to HSCR remain unknown. Here, we investigated the role of this enhancer by generating mice carrying a deletion of the orthologous Ret mcs+9.7 enhancer (Δmcs+9.7). Single-cell RNA sequencing of E14.5 embryonic gut demonstrated that enhancer deletion reduced Ret expression by 8% without altering ENS cell composition. However, reduced Ret expression was restricted to differentiating neurons and inhibitory motor neuron lineages, revealing cell type-specific enhancer activity. To determine the functional consequences of further reducing Ret dosage, we generated compound heterozygous mice carrying both the enhancer deletion and a Ret coding null allele (+/Δmcs+9.7;+/CFP). These mice exhibited additive reductions in Ret expression, altered Sox10 expression, dysregulation of cell-cycle and neuronal differentiation programs, and selective depletion of developing inhibitory motor neuron lineages. These findings establish a cell type-specific role for the mcs+9.7 enhancer in modulating Ret dosage and reveal how subtle enhancer perturbations alter neural subtype specification without overt hypoganglionosis, suggesting that HSCR arises from a cascade of cellular defects triggered by >50% loss of Ret function.
Loss of differentiated function by type II pneumocytes plated on plastic surfaces was demonstrated by decreased lamellar body content, increased cellular protein, and rapid cellular flattening, changes that were retarded modestly by plating cells on laminin-coated surfaces. Laminin surfaces also inhibited [3H]thymidine (THM) incorporation into cellular DNA by 40% compared with plastic at 40 h, but did not alter an additional mitogenic effect of rat serum over fetal calf serum. In contrast, cells plated on the laminin-rich basement membrane-like gel formed from an extract of EHS mouse sarcoma, matrix gel (MG), maintained a high content of intracellular lipids in lamellar inclusions and retained a rounded morphology for at least 3 days. MG markedly inhibited THM incorporation and morphological changes when cells were cultured on this surface or when MG was formed over cells initially plated on plastic for various intervals. The importance of the laminin component of MG was demonstrated when these surfaces were pretreated with a highly specific antilaminin serum. Type II cells commenced flattening on the treated MG surface, and THM incorporation increased with the same time course as did control cells on plastic. The data suggest that short-term culture and study of differentiated type II pneumocytes may require a laminin-rich substratum. THM incorporation into type II cell DNA provides an important early and sensitive index of cell-basement membrane interaction and subsequent maintenance of function.
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Thirty children with functional hearing loss were seen for psychological assessment. The sample included twice as many girls as boys. Most had experienced middle-ear problems and scored outside normal limits on the Junior Eysenck Personality Inventory. Introversion alone or combined with neuroticism was the most important personality dimension, especially for girls. The relationship between introverted behaviour, hearing impairment and previous experience of hearing problems is discussed.
The terminology used to describe functional hearing loss (FHL) and some explanations of the phenomenon are discussed briefly. Previous studies of FHL in children are reviewed. Characteristics of 30 children seen for psychological assessment following diagnosis of FHL are described. There were twice as many girls as boys in the sample. A large proportion of the children had experienced middle ear problems. The mean IQ for the sample was below average, but the range of intellectual ability was wide. Nine children showed serious educational retardation. The children were assigned to one of three psychological problem groups depending on whether they had minor, school-based, or deeper, psychological problems. Those with deeper psychological problems tended to show greater hearing losses on pure tone audiometry. FHL seemed to be related to attentional factors in those with only minor or school-based problems but not for those with deeper psychological problems. These findings are discussed with reference to the need for psychological assessment of children with FHL.
This paper presents a general decision model for quantitative risk analysis to help in solving the problem of setting the optimal exposure level of a potential carcinogen in regulatory decision-making. This model consists of a probability function and two loss functions. The probability function describes the dose-response relationship for a potential carcinogen at various exposure levels. The two loss functions include the cost of using a potential carcinogen, e.g., health loss, and the cost of not using the compound, e.g., economic loss. Using the principle of minimum expected loss, a fundamental formula for setting the optimal beneficial dose level is derived. The formula equates the probability function to a ratio of loss functions. The general form of loss functions is described in the paper. Under certain conditions, the current approach for quantitative risk assessment is a special situation of this general model.
Functional and morphological changes of the rat sciatic nerve after local hyperthermia (30 min, 45 degrees C) and crush treatment were compared. After hyperthermic injury nerve function loss developed in a time period of about 7 h. Nerve crush led to an immediate loss of nerve function. Nerve function loss was assessed by a motor and a sensory function test. Recovery from function loss took place in both treatment groups and was complete in 4-5 weeks. Early (within 8 h post-treatment) histopathological changes in the nerve after heating included edema, possible blood stasis and changes in the blood vessel wall, like swelling of the media. During this period some axonal changes were observed. Immediate after crushing axons were severely damaged, while many blood vessels remained normal. Within one week after both treatments, degeneration of axons and myelin was observed at the site and distal from the site of the lesion (Wallerian degeneration). Three weeks after treatment a major part of the axons had regenerated and remyelinated. Vascular changes at the site of lesion could still be observed in the heat-treated nerves. Twelve weeks after both treatments, blood vessels appeared to be normal again. Morphometrical analysis of the treated nerves confirmed the histological observations. Three and 12 weeks after treatment average axon diameters were significant smaller and average myelin sheaths were significant thinner compared to untreated nerves. These parameters did not differ significantly when the two treatment groups were compared.