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Query: UNIPROT:Q86TM3 (cage)
 29,987 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We measured the breathing pattern of normal subjects, asymptomatic smokers, asymptomatic and symptomatic asthmatic patients, and patients with chronic obstructive pulmonary disease, restrictive lung disease, primary pulmonary hypertension and anxiety state utilizing respiratory inductive plethysmography. Respiratory rate was increased above the normal in smokers and in patients with COPD, restrictive lung disease and pulmonary hypertension, but remained normal in asthmatic patients. Inspiratory times (T1) of one second or less often occurred in patients with COPD, restrictive lung disease, and pulmonary hypertension. Smokers and patients with symptomatic asthma, COPD, restrictive lung disease and pulmonary hypertension showed heightened respiratory center drive as reflected by elevated mean inspiratory flow (VT/TI). Fractional inspiratory time was reduced to a variable extent in smokers, symptomatic asthmatic patients and patients with COPD, and was a weak indicator of airways obstruction. Patients with COPD often had major fluctuations of expiratory timing, periodic fluctuations of end-expiratory level, and asynchrony between rib cage and abdominal movements. Chronic anxiety was characterized by frequent sighs; episodic rapid rates alternating with apneas were less common. We conclude that analysis of breathing patterns provides diagnostic discrimination among normal subjects and disease states.
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PMID:Breathing patterns. 2. Diseased subjects. 688 4

Patients with cystic fibrosis (CF) often hypoventilate during sleep with marked falls in oxygen saturation (SaO2%). This occurs most commonly during REM sleep, when there is a reduction in rib cage excursion and a fall in end-expiratory lung volume (EELV). The aim of this study was to examine the effect of nocturnal nasal continuous positive airway pressure (nCPAP) on SaO2 and the respiratory disturbance index (RDI) during sleep in patients with CF and severe lung disease. Seven patients (FEV1% pred, 23 +/- 5; range, 14 to 28%) were evaluated during sleep on two nights, control and nCPAP (11 +/- 2 cm H2O; range, 8 to 16 cm H2O), with four patients breathing room air and three patients breathing supplemental oxygen on both nights. Mean awake SaO2 was 91 +/- 1% (range, 89 to 93%). All patients showed significant oxyhemoglobin desaturation and respiratory disturbance in the control study. The maximal falls in SaO2 (15 +/- 10%) were most often associated with phasic eye movements, and a decline in rib cage excursion and the sum signal (Respitrace) during REM sleep. Nasal CPAP resulted in a significant improvement in the mean minimum oxygen saturation (MMOS) during both NREM (nCPAP 91 +/- 3% vs control 88 +/- 2%, p < 0.05) and REM sleep (nCPAP 89 +/- 6% vs control 83 +/- 6%, p < 0.05). Transcutaneous CO2 measurements were not significantly different between the control and the nCPAP studies. The RDI was also significantly reduced with nCPAP especially during REM sleep (9 +/- 7 events per hour vs control 25 +/- 11 events per hour, p < 0.05). Nasal CPAP caused no change in total sleep time or sleep efficiency yet significantly reduced the RDI and improved baseline SaO2 during both NREM and REM sleep.
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PMID:Benefits of nocturnal nasal CPAP in patients with cystic fibrosis. 798 90

Respiratory muscle weakness is the primary cause of respiratory dysfunction in neuromuscular disease (NMD), but structural abnormalities of the chest wall also play a role. In adults with NMD, restrictive lung disease is in part caused by reduced chest wall compliance (C(W)), believed to reflect stiffening of connective tissue resulting from chronically reduced chest wall motion in the presence of respiratory muscle weakness. We hypothesized that chronic limitation of chest wall motion in young children with NMD leads to structural underdevelopment of the chest wall, and results in increased, rather than decreased, C(W). In 18 subjects with NMD, ranging from 3 mo to 3.8 yr of age, we compared C(W) with values obtained in children without NMD. A modification of the Mead-Whittenberger technique was used, with respiratory muscle relaxation provided by brief manual ventilation. Respiratory system compliance (Crs) and lung compliance (C(L)) were calculated from airway opening pressure, transpulmonary pressure, and tidal volume. C(W) was calculated as 1/C(W) = 1/Crs - 1/C(L) during manual ventilation. C(W)/kg was higher in subjects with NMD than in controls, at 5.2 +/- 2.8 (mean +/- SD) versus 2.4 +/- 0.8 ml/cm H2O (p < 0.001). In subjects who had normal lung compliance values during spontaneous breathing (C(Lspont)), C(W)/C(Lspont) was significantly greater in subjects with NMD (5.5 +/- 3.2) than in controls (1.9 +/- 1.0) (p < 0.001). By predisposing to rib cage deformation and reduced end-expiratory lung volume, abnormally high C(W) in infants and young children with NMD may contribute to respiratory dysfunction.
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PMID:Chest wall compliance in infants and children with neuromuscular disease. 888 5

Previous studies from this laboratory showed that the decreases in Tco and associated functional parameters often observed in rodents following exposure to xenobiotic agents are capable of modulating the subsequent toxic response and that the magnitude of this induced hypothermic response may itself be modified by a number of experimental conditions. A moderate hypothermic response, characterized by a temperature drop of approximately 2 degrees C, appears to afford the optimal protection. Studies in which exposures occur through inhalation of harmful gases or particles present a special set of problems. In such studies, the dose of the toxic agent to which the animal is exposed is a function of the concentration of the agent in the atmosphere and the minute ventilation of the animal. Although ambient concentrations is generally held constant in laboratory studies, minute ventilation varies directly with metabolism, and both of these parameters may change significantly across experimental conditions. Thus, at low Tas, metabolism and minute ventilation are relatively high and uptake of inhalable toxic agents is increased. However, the development of the hypothermic response during the exposure entails a directly correlated reduction in these parameters and, presumably, in dose. For the most part, inhalation toxicological studies are conducted using resting animals or exercising humans. Animals are sometimes concurrently exposed to CO2 to simulate the increased ventilation of exercise and more closely mimic human studies. The experimental protocols employed in the above inhalation studies permitted examination of (1) the impact of species, size, handling stress, and changes in Ta on both the induced hypothermic response and the concomitant pulmonary toxicity; (2) the additive impact of exercise stress on O3 toxicity; and (3) the toxicity of ambient-derived particulate matter in normal rats and in rats with preexisting pulmonary inflammation. The results of these studies demonstrate that the magnitude of the induced hypothermic response is directly proportional to the uptake of the toxic agent by the lung and inversely proportional to the mass of the animal and the ambient temperature at which the exposure is conducted. The hypothermic response is sensitive to a number of experimental stresses including handling and changes in cage conditions. Exercise attenuates the hypothermic response, whereas CO2-stimulated increases in ventilation employed as an exercise surrogate may potentiate the response. Toxic exposures conducted in animals with lung disease or compromised pulmonary function may induce a severe hypothermic response while comparable exposures in normal animals produce only mild or moderate responses. In general, the development of the hypothermic response in the presence of ambient pollutants serves to decrease the minute ventilation of the animal and therefore limits the uptake and dose of the airborne toxicant. The results of these inhalation studies support our previous conclusions concerning the impact of the hypothermic response on toxicity and emphasize the need to monitor and incorporate these changes in functional parameters into analyses of toxicological data. Furthermore, because humans do not demonstrate a robust hypothermic response following exposure to toxic agents, extrapolation of the results obtained from animal studies and comparisons with data from human studies are considerably more complicated.
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PMID:Impact of the hypothermic response in inhalation toxicology studies. 910 Sep 77

The authors describe a radiographic method to quantify a surgical procedure of thoracic expansion in a 2-year-old patient with achondroplasia, small chest cage, and severe restrictive lung disease. The patient had undergone three surgical procedures of thoracic expansion since birth. The authors utilized computer-generated lung volume histograms after spiral computed tomographic scanning and three-dimensional imaging of the lungs to calculate his lung volumes before and after the third surgical thoracic expansion. The lung volumes, calculated by the histograms, were 363 mL and 406 mL before and after surgery, respectively. This 40-mL difference in the patient's lung volumes (4 mL/kg) accounted for a significant clinical improvement. Lung volume histograms obtained by this radiographic method are very helpful in substantiating a successful surgical chest expansion or provide an explanation for an unsuccessful repair.
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PMID:Lung volume histograms after computed tomography of the chest with three-dimensional imaging as a method to substantiate successful surgical expansion of the rib cage in achondroplasia. 960 82

Jarcho-Levin syndrome is a genetically transmitted rare entity characterized by multiple vertebral and rib anomalies. The multilevel skeletal involvement causes short stature, neck and thoracic cage deformities, and restrictive lung disease that is usually the cause of early death. The authors describe a 33-year follow-up of a patient with this syndrome who represents, to their best knowledge, the longest survival of a patient with this entity.
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PMID:Jarcho-Levin syndrome: report on a long-term follow-up of an untreated patient. 1021 83

Multi-detector row computed tomography (CT) with three-dimensional (3D) volume rendering provides a unique perspective on thoracic anatomy and disease. Multi-detector row CT allows shorter acquisition times, greater coverage, and superior image resolution. Three-dimensional volume rendering now permits real-time, interactive modification of relative pixel attenuation in an infinite number of planes and projections. In vascular imaging, this technique provides image quality that equals or surpasses that of conventional angiography. Its use has expanded to aid in diagnosis and surgical planning, often obviating conventional or digital angiography and reducing costs. It is reliable in depicting clot and the pulmonary vasculature and may also be used to evaluate thoracic venous anomalies (eg, pulmonary arteriovenous malformations) and to plan therapy. Airway imaging with multi-detector row CT with 3D volume rendering is particularly useful in the planning and follow-up of stent placement. In diffuse lung disease, this technique can increase nodule detection and help differentiate between small nodules and vessels. It is also helpful in imaging the musculoskeletal system and the thoracic cage. Multi-detector row CT with 3D volume rendering has enhanced the conventional roles of thoracic CT and challenged the supremacy of other imaging modalities. It will likely play a leading role in future radiologic research and practice.
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PMID:Multi-detector row CT of thoracic disease with emphasis on 3D volume rendering and CT angiography. 1155 32

No transgenic cystic fibrosis (CF) mouse model developed to date mimics the major clinical phenotype found in humans with CF, chronic Pseudomonas aeruginosa lung infection. In a transgenic CF transmembrane conductance regulator (cftr) mouse colony, we found WT, heterozygous, and homozygous CF mice housed in the same cage became chronically colonized in the oropharynx with environmental P. aeruginosa when the bacterium was present in drinking water. Elimination of P. aeruginosa from drinking water resulted in clearance in most WT and CF heterozygous, but not homozygous mice. For experimental evaluation, a combination of specific animal husbandry techniques and an oral infection route showed cftr(-/-) mice but not WT mice can be chronically colonized by P. aeruginosa with subsequent lung translocation, yielding a pathologic picture indicative of chronic lung infection. In some instances, mucoid isolates of P. aeruginosa were recovered from lungs, indicating conditions were present for conversion to mucoidy. Overexpression of human CFTR in the lungs of WT mice markedly accelerated the clearance rate of P. aeruginosa, demonstrating that lung levels of CFTR play an important role in defense against infection. P. aeruginosa mutants unable to express the surface polysaccharide alginate or the global regulator GacA were deficient in their ability to colonize the mice. CF mice made potent immune responses to P. aeruginosa outer membrane antigens. Overall, we found that under the proper conditions, transgenic CF mice are hypersusceptible to P. aeruginosa colonization and infection and can be used for evaluations of lung pathophysiology, bacterial virulence, and development of therapies aimed at treating CF lung disease.
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PMID:Hypersusceptibility of cystic fibrosis mice to chronic Pseudomonas aeruginosa oropharyngeal colonization and lung infection. 1257 88

1. Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. 2. The rare condition of fused ribs and congenital scoliosis may result in a three-dimensional thoracic deformity with adverse effects on thoracic growth and function with development of thoracic insufficiency syndrome. 3. The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume. Volume depends on the width and depth of the rib cage, and the thoracic spine provides height. The ability to change volume, termed thoracic function, is provided by the diaphragm and the secondary muscles of respiration. 4. On radiographs, the loss of the vertical height of the lung of the concave, restricted hemithorax is defined by the percentage of space available for the lung. 5. Spine rotation causes a windswept thorax, with both restriction of the volume of the convex hemithorax and restriction of the motion of the involved ribs. 6. Constrictive three-dimensional deformity of the thorax may cause extrinsic, restrictive lung disease. 7. Progressive thoracic insufficiency syndrome is diagnosed on the basis of clinical signs of respiratory insufficiency, loss of chest wall mobility as demonstrated by the thumb excursion test, worsening indices of three-dimensional thoracic deformity on radiographs and computed tomography scans, or a relative decline in percent predicted vital capacity due to thoracic "failure to thrive," as demonstrated by pulmonary function tests. 8. Treatment of progressive thoracic insufficiency syndrome should provide an acute increase in the thoracic volume with stabilization of any flail chest-wall defects and maintain these improvements as the patient grows, without the need for spine fusion.
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PMID:The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. 1263 23

A 37-year-old woman with severe interstitial lung disease associated with dermatomyositis sine myositis is reported. A thoracoscopic lung biopsy revealed organizing diffuse alveolar damage. Significantly elevated serum levels of the tumor markers CA 15-3 and CASA (cancer-associated serum antigen) were detected, but no evidence of an underlying malignancy (including breast and ovarian) was found on serial clinical and radiologic examinations. These levels gradually normalized as the interstitial lung disease responded to a combination of cyclophosphamide and corticosteroids. The use of the CA 15-3 and CASA assays to measure serum levels of the highly glycosylated, high-molecular-weight mucin MUC1 in interstitial lung disease has not been previously described. Clinicians should therefore be aware that elevation of these tumor markers may reflect the presence of interstitial lung disease rather than an underlying malignancy in patients with dermatomyositis, especially if the levels normalize after successful treatment of the lung disease.
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PMID:Transient elevation of the tumor markers CA 15-3 and CASA as markers of interstitial lung disease rather than underlying malignancy in dermatomyositis sine myositis. 1704 61


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