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Magnetic resonance reveals mitochondrial dysfunction and muscle remodelling in spinal muscular atrophy.
Brain
May 2022
Centre for Child Development, Exercise and Physical Literacy, Wilhelmina Children's Hospital, University Medical Centre Utrecht, P.O. Box 85090, 3508 AB Utrecht, the Netherlands.
Genetic therapy has changed the prognosis of hereditary proximal spinal muscular atrophy, although treatment efficacy has been variable. There is a clear need for deeper understanding of underlying causes of muscle weakness and exercise intolerance in patients with this disease to further optimize treatment strategies. Animal models suggest that in addition to motor neuron and associated musculature degeneration, intrinsic abnormalities of muscle itself including mitochondrial dysfunction contribute to the disease aetiology.
View Article and Find Full Text PDFAttempts at optimizing classification of chronic obstructive pulmonary disease (COPD) reflect clinical heterogeneity of this pathology and provide a basis for the search of new phenotypic markers (especially at the early stages of the disease) that could be useful for prognostication of its severity in individual patients. One of the potential makers is phenotyping of COPD with distinguishing bronchitic, emphysemic, and mixed phenotypes. This paper presents results of analysis of functional characteristics of the patients with these phenotypes.
View Article and Find Full Text PDFThe article is dedicated to analysis of psychological features of 152 women with coronary artery disease (CAD), who had had acute myocardial infarction and had begun sanatorium rehabilitation in Belokurikha sanatorium. The role of the neurophysiological method of bioadaptive regulation in improvement of the efficacy of early post-infarction rehabilitation and secondary CAD prophylaxis in women with alexithymia was evaluated. The authors make their conclusion on the basis of clinico-functional and psychological studies and statistical evaluation of the data received in the course of the study.
View Article and Find Full Text PDF[The assessment of the activity of idiopathic pulmonary fibrosis by high-resolution computed tomography].
Radiol Med
March 1996
Radiodiagnoistica Padiglione Pneumonefrologico, Policlinico S. Orsola-Malpighi, Bologna.
The authors report the results of the study performed with high resolution CT (HRCT) in a group of 29 patients affected with idiopathic pulmonary fibrosis (IPF). Each patient underwent HRCT at the beginning of the study and after one year. A complete clinico-functional assessment was available in 20 cases and functional CT correlation was made in these patients; 15/20 subjects underwent immunosuppressive therapy with corticosteroids and cyclophosphamide.
View Article and Find Full Text PDFAs many as 50 patients presenting with unstable angina were examined for clinicofunctional status, using bicycle ergometry [correction of veloergometry], transesophageal electrocardiostimulation and 24-hour Holter ECG monitoring during 1-yr follow-up. The results of exercise tests at enrollment into the study suggested a dramatic decrement in the patients' exercise tolerance, as to Holter, with the painless myocardial ischemia tending to be more common among them than the painful one. A follow-up study a year later showed the course of the illness to be most variable among those cases presenting with unstable angina.
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