Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0729233 (Thoracic)
 6,478 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Respiratory failure is the major cause of death in patients with Duchenne muscular dystrophy (DMD). In this report we retrospectively examined the clinical courses, pulmonary function, progression of scoliosis and the time of assisted ventilation. Forty nine patients, aged 2 to 27 years were studied and thirty eight patients of them were examined twice in some intervals. The subjects of examinations included pulmonary function studies consisting of spirometry, measurement of the thoracic scoliosis measured according to the method of Cobb and room air arterial blood gas values. The results were the following; 1) The decline in percentage values of vital capacity (%VC) began at age 8 or 9 and decreased consistently with age. Finally %VC reached under 10 percent at mean age of 21.53 with respiratory failure. 2) Thoracic scoliosis measured according to the method of Cobb increased further with advancing age. The greater the angle of curvature, the more likely the development of respiratory failure. 3) Sixteen patients received negative pressure ventilator (chest respirator: CR) between 16.5 and 25.1 years of age. Twelve patients used part-time (mainly night-time) ventilator and four patients used it in full-time. The average PaCO2 and PaO2 before administration of ventilator were 67.49 mmHg and 71.46 mmHg, respectively. The levels after ventilation were 58.01 mmHg (PaCO2) and 82.09 mmHg (PaO2).
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PMID:[Respiratory failure and its care in Duchenne muscular dystrophy]. 206 Feb 38

Recently body respirator (BR) has been used to control respiratory failure in patients with late stage Duchenne muscular dystrophy (DMD). We examined the effect of BR using a pulse oximeter. Arterial oxygen saturation (SaO2) for the night (21:00-7:00) was monitored in 15 DMD patients (5 cases without BR, 3 cases with BR partially for the night and 6 cases with BR all night long) and the desaturation (SaO2 less than 90%) time was followed three times (Jan. '87, Nov. '87, Apr. '88) in each patient. Desaturation time did not increase in 4 cases without BR. But in one case without BR it increased so much that we decided to put the patient on BR. In 3 cases with BR partially for the night, desaturation was well controlled when they used BR. No marked increase of desaturation was found in 4 cases with BR all night long. 2 of these cases were changed from cuirass type BR to jacket type BR and were getting on satisfactorily. Thoracic cage expansion of jacket type was larger than that of cuirass type, and it was found that jacket type was valuable. Also, we investigated the cause of desaturation by recording SaO2, nasal flow, thoracic cage motion and abdominal motion at the same time by making use of a polygraphy. The result showed that the main cause of desaturation was the resistance of thoracic cage motion against BR. And we think research and development is needed.
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PMID:[The effect of body respirator on the desaturation during the night in Duchenne muscular dystrophy]. 280 9

Spinal deformity in the form of kyphosis or kyphoscoliosis occurs in most patients with Duchenne muscular dystrophy (DMD), a fatal X-linked disorder caused by an absence of the subsarcolemmal protein dystrophin. Mdx mice, which also lack dystrophin, show thoracolumbar kyphosis that progresses with age. We hypothesize that paraspinal and respiratory muscle weakness and fibrosis are associated with the progression of spinal deformity in this mouse model, and similar to DMD patients there is evidence of altered thoracic conformation and area. We measured kyphosis in mdx and age-matched control mice by monthly radiographs and the application of a novel radiographic index, the kyphotic index, similar to that used in boys with DMD. Kyphotic index became significantly less in mdx at 9 mo of age (3.58 +/- 0.12 compared with 4.27 +/- 0.04 in the control strain; P < or = 0.01), indicating more severe kyphosis, and remained less from 10 to 17 mo of age. Thoracic area in 17-mo-old mdx was reduced by 14% compared with control mice (P < or = 0.05). Peak tetanic tension was significantly lower in mdx and fell 47% in old mdx latissimus dorsi muscles, 44% in intercostal strips, and 73% in diaphragm strips (P < or = 0.05). Fibrosis of these muscles and the longissimus dorsi, measured by hydroxyproline analysis and histological grading of picrosirius red-stained sections, was greater in mdx (P < 0.05). We conclude that kyphotic index is a useful measure in mdx and other kyphotic mouse strains, and assessment of paralumbar and accessory respiratory muscles enhance understanding of spinal deformity in muscular dystrophy.
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PMID:Progression of kyphosis in mdx mice. 1523 60

This is a summary of the presentation "A 2009 Perspective on the 2004 American Thoracic Society Statement, 'Respiratory Care of the Patient With Duchenne Muscular Dystrophy,'" presented as part of the program on pulmonary management of pediatric patients with neuromuscular disorders at the 30th annual Carrell-Krusen Neuromuscular Symposium on February 20, 2008.
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PMID:A 2009 perspective on the 2004 American Thoracic Society statement, "respiratory care of the patient with Duchenne muscular dystrophy". 1942 Jan 52

We recently described a modified version of the 6-minute walk test (6MWT) for Duchenne muscular dystrophy (DMD) based partly on the American Thoracic Society (ATS) guidelines. This measure has shown reliability, validity and utility as a primary outcome measure in DMD clinical trials. Because loss of muscle function in DMD occurs against the background of normal childhood growth and development, younger children with DMD can show increase in distance walked during 6MWT over ~1 year despite progressive muscular impairment. In this study, we compare 6-minute walk distance (6MWD) data from DMD boys (n=17) and typically developing control subjects (n=22) to existing normative data from age- and sex-matched children and adolescents. An age- and height-based equation fitted to normative data by Geiger and colleagues was used to convert 6MWD to a percent-predicted (%-predicted) value in boys with DMD. Analysis of %-predicted 6MWD data represents a method to account for normal growth and development, and shows that gains in function at early ages represents stable rather than improving abilities in boys with DMD. Boys with DMD from 4-7 years of age maintain a stable 6MWD approximately 80% of that of typically developing peers, with the deficit progressing at a variable rate thereafter.
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PMID:Percent-predicted 6-minute walk distance in duchenne muscular dystrophy to account for maturational influences. 2230 89

We recently described a modified version of the 6-minute walk test (6MWT) for Duchenne muscular dystrophy (DMD) based partly on the American Thoracic Society (ATS) guidelines. This measure has shown reliability, validity and utility as a primary outcome measure in DMD clinical trials. Because loss of muscle function in DMD occurs against the background of normal childhood growth and development, younger children with DMD can show increase in distance walked during 6MWT over ~1 year despite progressive muscular impairment. In this study, we compare 6-minute walk distance (6MWD) data from DMD boys (n=17) and typically developing control subjects (n=22) to existing normative data from age- and sex-matched children and adolescents. An age- and height-based equation fitted to normative data by Geiger and colleagues was used to convert 6MWD to a percent-predicted (%-predicted) value in boys with DMD. Analysis of %-predicted 6MWD data represents a method to account for normal growth and development, and shows that gains in function at early ages represents stable rather than improving abilities in boys with DMD. Boys with DMD from 4-7 years of age maintain a stable 6MWD approximately 80% of that of typically developing peers, with the deficit progressing at a variable rate thereafter.
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PMID:Percent-predicted 6-minute walk distance in duchenne muscular dystrophy to account for maturational influences. 0

Golden retriever and Labrador retriever muscular dystrophy are inherited progressive degenerative myopathies that are used as models of Duchenne muscular dystrophy in man. Thoracic lesions were reported to be the most consistent radiographic finding in golden retriever dogs in a study where radiographs were performed at a single-time point. Muscular dystrophy worsens clinically over time and longitudinal studies in dogs are lacking. Thus our goal was to describe the thoracic abnormalities of golden retriever and Labrador retriever dogs, to determine the timing of first expression and their evolution with time. To this purpose, we retrospectively reviewed 390 monthly radiographic studies of 38 golden retrievers and six Labrador retrievers with muscular dystrophy. The same thoracic lesions were found in both golden and Labrador retrievers. They included, in decreasing frequency, flattened and/or scalloped diaphragmatic shape (43/44), pulmonary hyperinflation (34/44), hiatal hernia (34/44), cranial pectus excavatum (23/44), bronchopneumonia (22/44), and megaesophagus (14/44). The last three lesions were not reported in a previous radiographic study in golden retriever dogs. In all but two dogs the thoracic changes were detected between 4 and 10 months and were persistent or worsened over time. Clinically, muscular dystrophy should be included in the differential diagnosis of dogs with a combination of these thoracic radiographic findings.
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PMID:Age-related thoracic radiographic changes in golden and labrador retriever muscular dystrophy. 2270 94

The Golden Retriever Muscular Dystrophy (GRMD) dog is the closest animal counterpart of Duchenne muscular dystrophy in humans and has, for this reason, increasingly been used in preclinical therapeutic trials for this disease. The aim of this study was to describe the abnormalities in canine dystrophic muscle non-invasively, quantitatively, thoroughly and serially by means of NMR imaging. Thoracic and pelvic limbs of five healthy and five GRMD dogs were imaged in a 3T NMR scanner at 2, 4, 6 and 9months of age. Standard and fat-saturated T(1)-, T(2)- and proton-density-weighted images were acquired. A measurement of T(1) and a two-hour kinetic study of muscle enhancement after gadolinium-chelate injection were also performed. Ten out of the 15 indices evaluated differed between healthy and GRMD dogs. The maximal relative enhancement after gadolinium injection and the proton-density-weighted/T(2)-weighted signal ratio were the most discriminating indices. Inter-muscle heterogeneity was found to vary significantly for most of the indices. The body of data that has been acquired here will help in designing and interpreting preclinical trials using dystrophin-deficient dogs.
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PMID:Comprehensive longitudinal characterization of canine muscular dystrophy by serial NMR imaging of GRMD dogs. 2298 Jul 71

Decline in pulmonary function in Duchenne Muscular Dystrophy (DMD) contributes to significant morbidity and reduced longevity. Spirometry is a widely used and fairly easily performed technique to assess lung function, and in particular lung volume; however, the acceptability criteria from the American Thoracic Society (ATS) may be overly restrictive and inappropriate for patients with neuromuscular disease. We examined prospective spirometry data (Forced Vital Capacity [FVC] and peak expiratory flow [PEF]) from 60 DMD patients enrolled in a natural history cohort study (median age 10.3 years, range 5-24 years). Expiratory flow-volume curves were examined by a pulmonologist and the data were evaluated for acceptability using ATS criteria modified based on the capabilities of patients with neuromuscular disease. Data were then analyzed for change with age, ambulation status, and glucocorticoid use. At least one acceptable study was obtained in 44 subjects (73%), and 81 of the 131 studies (62%) were acceptable. The FVC and PEF showed similar relative changes in absolute values with increasing age, i.e., an increase through 10 years, relative stabilization from 10-18 years, and then a decrease at an older age. The percent predicted, FVC and PEF showed a near linear decline of approximately 5% points/year from ages 5 to 24. Surprisingly, no difference was observed in FVC or PEF by ambulation or steroid treatment. Acceptable spirometry can be performed on DMD patients over a broad range of ages. Using modified ATS criteria, curated spirometry data, excluding technically unacceptable data, may provide a more reliable means of determining change in lung function over time.
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PMID:Characterization of pulmonary function in Duchenne Muscular Dystrophy. 2573 12