Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q86TM3 (cage)
 29,987 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Muscular diseases are characterized by progressive loss of muscle strength, resulting in cough ineffectiveness with its deleterious effects on the respiratory system. Assessment of cough effectiveness is therefore a prominent component of the clinical evaluation and respiratory care in these patients. Owing to uneven distribution of muscle weakness in neuromuscular patients, we hypothesized that forces acting on the chest wall may impact on the compartmental distribution of gas volume resulting in a decrease in cough effectiveness. Pulmonary volumes, respiratory muscle strength, peak cough flow and chest wall kinematics by optoelectronic plethysmography were studied in 8 patients and 12 healthy subjects as controls. Chest wall volume was modeled as the sum of volumes of the rib cage and abdomen. The plot of the volumes of upper to lower rib cage allowed assessment of rib cage distortion. Unlike controls, patients were unable to reduce end-expiratory chest wall volume, and exhibited greater rib cage distortion during cough. Peak cough flow was negatively correlated with rib cage distortion (the greater the former, the smaller the latter), but not with respiratory muscle strength. In conclusion, insufficient deflation of chest wall compartments and marked rib cage distortion resulted in cough ineffectiveness in these neuromuscular patients.
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PMID:Chest wall kinematics during voluntary cough in neuromuscular patients. 1824 70

We report a 65-years-old man suffering from slowly progressive dyspnea of four years' clinical history followed by dysphagia. His range of motion was severely restricted in the four extremities and trunk, however, neither motor weakness nor sensory disturbance was noted. Radiographic examination showed diffuse osteophyte formation in front of whole vertebrae but no apparent change was observed in the sacroiliac joint. These radiographic features suggested the diagnosis of diffuse idiopathic skeletal hyperostosis (DISH) in this patient. We consider that the restrictive ventilatory impairment in the present patient was due to the decreased thoracic cage compliance caused by hyperostosis of thoracic costovertebral joints, and the prominent osteophyte formation in front of the cervical vertebrae compressing esophagus was responsible for dysphagia.
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PMID:[A case of diffuse idiopathic skeletal hyperostosis presenting dysphagia and restrictive ventilatory impairment]. 1830 65

Asphyxiating thoracic dystrophy (ATD) is a rare autosomal recessive form of chondrodysplasia characterized by short ribs. Respiratory failure is due to the reduced volume and complete immobility of the thoracic cage. There is no consensus on the treatment of this restrictive pulmonary disease. Surgical attempts to enlarge the thoracic cage are disappointing. We report the cases of nine children with ATD treated by periodic respiratory hyperinsufflation. Their clinical outcome was related to the severity of their respiratory distress and their age at the beginning of this treatment. It is possible to use periodic hyperinsufflation very early after birth to prevent secondary respiratory failure. Periodic insufflation can also be used to treat older children with severe restrictive respiratory insufficiency requiring tracheostomy and endotracheal management. This treatment promotes alveolar multiplication and thoracic growth. Four children had laboratory and/or clinical evidence of hepatic dysfunction that improved on ursodeoxycholic acid therapy. Three children who had muscle weakness at birth improved during childhood.
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PMID:[Jeune'disease (asphyxiating thoracic dystrophy) and respiratory failure: importance of early respiratory management with periodic hyperinsufflation]. 1844 62

Osteomalacia (OM) is a condition that usually is overlooked and neglected when compared with other metabolic bone disease such as osteoporosis. Presenting with a wide spectrum of nonspecific clinical, radiographic, and biochemical manifestations, OM is a treatable metabolic disease that is precisely diagnosed by anterior iliac crest bone biopsy. Clinical clues to lead one to suspect OM in the context of a diffuse bone disease include the presence of generalized bone pain affecting mainly shoulders, hips and rib cage, proximal muscle weakness, low serum calcium x phosphorus product, increased serum alkaline phosphatase, low calcium in the 24-h urine test, and low serum 25 hydroxyvitamin D. Radiographic examination may show a characteristic "erased" or "fuzzy" type of demineralization, pseudofractures, or bone deformities. OM is confined usually to elderly individuals or to those patients with intestinal malabsorption and hypophosphatemia.
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PMID:Musculoskeletal manifestations of osteomalacia. 1907 64

A 53-year-old man was referred to our hospital for dry cough and a mass in the hilum of the right lung on chest CT which was diagnosed as small cell lung cancer by bronchofiberscopy (T3N2M0, stage IIIB). Also, he was aware of progressive muscle weakness in the lower extremities on the first consultation. An electromyogram showed neuropathic changes and did not show waxing phenomenon in response to high frequency repetitive stimulation. Sensory nerve conduction velocity was low, so we diagnosed small cell cancer associated with paraneoplastic sensory neuropathy. Serum antineuronal antibodies were negative. His neurological symptoms improved dramatically after chemoradiotherapy for small cell lung cancer. A complete response was obtained by concurrent chemoradiotherapy and prophylactic cranial irradiation was administrated. He is alive without recurrence at 11 months after the treatment.
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PMID:[Improvement of neurologic symptoms after chemoradiotherapy in a patient with small cell lung cancer associated with paraneoplastic neurologic syndrome]. 1919 34

Lung transplantation and lung volume reduction surgery have opened a new therapeutic era for patients with advanced emphysema. In addition to providing impressive clinical benefits, they have helped us better understand how the chest wall and respiratory muscles adapt to chronic hyperinflation. This article reviews the effects of these procedures on respiratory muscle and chest wall function. Inspiratory (including diaphragm) and expiratory muscle strength are often close to normal after unilateral and bilateral transplantation, although some patients have marked weakness. After bilateral transplantation for emphysema, graft volume is normal at full inflation but remains greater than normal at end expiration, which results from structural changes in the chest wall. In contrast, patients with unilateral transplantation have a reduction in graft volume at full inflation. The mediastinum is displaced toward the graft at end expiration, which reduces the surface area of the diaphragm on the transplanted side, and it moves toward the native lung during tidal and full inspiration and toward the graft during tidal and forced expiration. Lung volume reduction produces an increase in contractility, length and surface area of the diaphragm, and increases its contribution to tidal volume; at the same time, neural drive to the muscle and respiratory load are reduced, such that diaphragm neuromechanical coupling is improved. Diaphragm configuration and rib cage dimensions are only minimally affected by the procedure. Single-lung transplantation and lung volume reduction favorably impact on the disadvantageous size interaction by which the lungs are functionally restricted by the chest wall in emphysema.
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PMID:Effect of lung transplant and volume reduction surgery on respiratory muscle function. 1935 7

The idiopathic inflammatory myopathies (IIMs) comprise polymyositis, myositis overlapping with another connective tissue disease, dermatomyositis (DM) and inclusion-body myositis (IBM). IIMs are characterized by the presence of proximal muscle weakness, increased levels of muscle-specific enzymes, specific electromyographic abnormalities, and the presence of inflammatory cell infiltrates in skeletal muscle. Clinical, serological and histological criteria can be used to define individual IIM subtypes. In the first of this two-part review series, we examine the evidence for the existence of cancer-associated myositis (CAM), and in part 2, we discuss recent discoveries that provide insight into identification of patients with DM, who may be most at risk of developing CAM.
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PMID:Defining cancer risk in dermatomyositis. Part I. 1952 81

A 3D finite element model of rib cage movement is developed and used to study the role of age-related costal cartilage and sternocostal joint calcification, as well as respiratory muscle weakness on the 'bucket-handle' movement of human rib. The volume displacement of the rib cage is related to changes in its circumference using an empirical equation presented by Agostoni et al. (1965, J Appl Physiol, 20:1179-1186). A systematic study is carried out to quantify the role of costal cartilage, sternocostal joint calcification and muscle weakness on the volume displacement of the rib cage. The results provide insight into some of the mechanisms underlying age-related changes in the respiratory system.
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PMID:Computational model of rib movement and its application in studying the effects of age-related thoracic cage calcification on respiratory system. 1992 42

Hemiparesis, unilateral weakness or partial paralysis, is a common outcome following hemispherectomy in humans. We use the non-human primate as an invaluable translational model for our understanding of developmental plasticity in response to hemispherectomy. Three infant vervet monkeys (Chlorocebus sabeus) underwent hemispherectomy at a median age of 9 weeks and two additional monkeys at 48 months. Gross motor assessment was conducted in a large open field that contained a horizontal bar spanning the width of the cage. Subjects were assessed yearly following surgery in infantile lesions for a period of 3 years. Adult-lesioned subjects were assessed 40 months following surgery. Shortly after surgery both infant and adult-lesioned subjects were unable to move the contralateral side of their body, but all subjects were able to walk within 6 months following surgery. At each time point the lower limb gait was normal in infant-lesioned subjects with no apparent limp or dragging, however the upper limb demonstrated significant impairment. Horizontal bar crossing was significantly impaired during the first 24 months following surgery. Adult-lesioned subjects also displayed upper limb movement impairments similar to infant-lesioned subjects. In addition the adult-lesioned subjects displayed a noticeable lower limb limp, which was not observed in the infant-lesioned group. Both groups at each time point showed a propensity for ipsiversive turning. The upper limb gait impairment and horizontal bar crossing of lesioned subjects are reminiscent of hemiparesis seen in hemisperectomized humans with the young-lesioned subjects showing a greater propensity for recovery.
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PMID:Partial recovery of hemiparesis following hemispherectomy in infant monkeys. 1996 43

Posttraumatic syringomyelia may result from a variety of inherent conditions and traumatic events, or from some combination of these. Many hypotheses have arisen to explain this complex disorder, but no consensus has emerged. A 28-year-old man presented with progressive lower extremity weakness, spasticity, and decreased sensation below the T4 dermatome five years after an initial trauma. Magnetic resonance imaging (MRI) revealed a large, multi-septate syrinx cavity extending from C5 to L1, with a retropulsed bony fragment of L2. We performed an L2 corpectomy, L1-L3 interbody fusion using a mesh cage and screw fixation, and a wide decompression and release of the ventral portion of the spinal cord with an operating microscope. The patient showed complete resolution of his neurological symptoms, including the bilateral leg weakness and dysesthesia. Postoperative MRI confirmed the collapse of the syrinx and restoration of subarachnoid cerebrospinal fluid (CSF) flow. These findings indicate a good correlation between syrinx collapse and symptomatic improvement. This case showed that syringomyelia may develop through obstruction of the subarachnoid CSF space by a bony fracture and kyphotic deformity. Ventral decompression of the obstructed subarachnoid space, with restoration of spinal alignment, effectively treated the spinal canal encroachment and post-traumatic syringomyelia.
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PMID:Decompressive surgery in a patient with posttraumatic syringomyelia. 2037 79


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