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131.
132.
Evidence that TDP-43 is not the major ubiquitinated target within the pathological inclusions of amyotrophic lateral sclerosis 总被引:3,自引:0,他引:3
Sanelli T Xiao S Horne P Bilbao J Zinman L Robertson J 《Journal of neuropathology and experimental neurology》2007,66(12):1147-1153
Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by the presence of various types of ubiquitinated inclusions in the cytoplasm of affected motor neurons. The identification of the ubiquitinated targets within these inclusions has represented a major challenge, as this may provide new gene candidates and/or clues to understanding the neurodegenerative mechanism(s) underlying the disease. As such, the nuclear factor TAR DNA-binding protein (TDP-43) was recently identified as a component of ubiquitinated skein-like inclusions and round inclusions in ALS. This identification combined with biochemical evidence led to the suggestion that TDP-43 is the key ubiquitinated target and major disease protein in ALS. Here, using 3-dimensional deconvolution imaging, we have obtained remarkable resolution of skein-like inclusions and round inclusions in ALS. Surprisingly we have found that in contrast to current thinking, TDP-43 is not the major ubiquitinated target within these types of inclusions. These findings raise the possibility that TDP-43 may not necessarily be the key disease protein in ALS and indicate that the major target(s) of ubiquitination remain to be identified. 相似文献
133.
134.
Thyrotoxicosis in children: thirty years''experience 总被引:3,自引:0,他引:3
Optimal treatment for thyrotoxicosis remains controversial in adults, but more so in paediatric practice. We have conducted a retrospective review of the records of 76 paediatric patients seen between 1965 and 1995 to determine management practice and outcome of therapeutic interventions. Seventeen are currently on antithyroid drug (ATD) treatment, while four have had their care transferred. Of the remaining 55, 21 (38%) achieved long-term remission with ATD alone following a mean treatment duration of 3.3 y (range 0.5-7 y). Block-replacement (high dose of ATD with thyroxine replacement) was more convenient than the titration regimen (3.4+/-0.3 visits to hospital per year versus 6.1+/-0.4, p<0.001). Surgery (subtotal/total thyroidectomy) was carried out in 27 patients, of whom 24 subsequently became hypothyroid and were treated with thyroxine. I131 was used successfully in six patients, two following surgery. ATD should remain the first-line therapy; a block-replacement regimen is more convenient. Surgery in a specialized centre carries a low risk. Caution should still be exercised in the use of I131 in young children. 相似文献
135.
136.
AA Hutchison JA Wozniak H-G Choi RA Otto RM Abrams PC Kosch 《Acta paediatrica (Oslo, Norway : 1992)》1994,83(3):241-248
Laryngeal and pump muscle activities are important in the establishment and maintenance of functional residual capacity (FRC) after birth. The aim of this study was to determine the expiratory mechanisms by which laryngeal and diaphragmatic activities achieve the increments in FRC postnatally. Wire electrodes were placed in: the laryngeal abductor, a major laryngeal adductor, the inferior pharyngeal constrictor and the diaphragm of six fetal sheep. The lambs were delivered prematurely by cesarean section and a face mask with a pneumotachograph applied. A grunting respiratory pattern was characterized by severe expiratory airflow retardation, associated with laryngeal adductor activity. In grunting breaths, minimal volume loss at end-expiration and incremental increases in FRC occurred when the onset of diaphragmatic activity preceded the onset of laryngeal muscle activities associated with laryngeal opening. Thus the timing order of laryngeal and diaphragmatic muscle activities near end-expiration is a determinant of increments in FRC. 相似文献
137.
Features of both systemic lupus erythematosus and scleroderma developed in a young Chinese girl while on multiple anticonvulsant therapy. These were reversed after withdrawal of the drugs. Readministration of ethosuximide for control of epilepsy caused a relapse with predominant sclerodermatous features. These clinical signs again resolved after ethosuximide withdrawal. 相似文献
138.
Introduction: This article reports a case of posterior reversible encephalopathy syndrome on compyted tomography (CT) perfusion in a patient
on “Triple H” (hypertension, hypervolemia, and hemodilution) therapy following aneurysmal rupture repair.
Case Report: “Triple H” therapy is used in the postoperative course for treatment of vasospasm to prevent stroke and hemorrhage by maintaining
cerebral perfusion pressure.
Discussion: A potential complication includes vasogenic edema from dysfunction of cerebral blood vessel autoregulation. CT perfusion
can detect alterations in cerebral blood flow and volume caused by these hemodynamic changes. 相似文献
139.
Rath TJ Sundgren PC Brahma B Lieberman AP Chandler WF Gebarski SS 《Neuroradiology》2005,47(3):183-188
Subependymomas are benign intraventricular tumors with an indolent growth pattern, which are usually asymptomatic, and most commonly occur in the fourth and lateral ventricles. When symptomatic, subependymomas often obstruct critical portions of the cerebrospinal fluid (CSF) pathway, causing hydrocephalus, and range from 3 cm to 5 cm in size. We report a case of an unusually massive subependymoma of the lateral ventricles treated with subtotal resection, ventriculoperitoneal shunt, and post-surgical radiation. The clinical course, radiographic and pathologic characteristics of this massive intraventricular subependymoma are discussed, as well as the differential diagnosis of lateral ventricular masses and a review of the literature concerning subependymomas. 相似文献
140.