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PubMedDate not supplied

Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates.

The purpose of this study was to determine rates of pneumonia and hospitalization for patients receiving oxygen therapy, patients having indwelling tracheostomy tubes, and those using tracheostomy or noninvasive methods of home mechanical ventilation. Six hundred eighty-four users of assisted ventilation for 13,751 patient-years or 19.8 years per patient were surveyed by mail and twice by telephone over a span of four years. Pneumonia and hospitalization rates were significantly higher for ventilator users with chronic obstructive pulmonary disease or with neuromuscular ventilatory insufficiency and gastrostomy tubes than for ventilator users with neuromuscular ventilatory insufficiency without gastrostomy tubes. Of the latter group, more than 90% of the pneumonias and hospitalizations were triggered by otherwise benign intercurrent upper respiratory tract infections. Oxygen therapy was associated with a significantly (P < 0.001) higher rate of pneumonias and hospitalizations than that seen for untreated patients after initial episodes of respiratory distress or during the use of either tracheostomy intermittent positive pressure ventilation or noninvasive ventilatory assistance methods. The lowest pneumonia and hospitalization rates (P < 0.001) were by full-time, noninvasive intermittent positive pressure ventilation users. We conclude that oxygen therapy is not an effective substitute for assisted ventilation for patients with primarily ventilatory insufficiency. Noninvasive ventilatory aids can be used effectively for up to full-time ventilatory support for patients with neuromuscular conditions whose bulbar muscle function is adequate to avert the need for gastrostomy tube placement.

Analysis of Variance↗
PubMed2000

Apoptotic features accompany acute quadriplegic myopathy.

OBJECTIVE: To investigate the role of apoptosis in acute quadriplegic myopathy. BACKGROUND: Acute quadriplegic myopathy is a muscular disease characterized by diffuse flaccid weakness occurring in patients with severe systemic illness and exposure to corticosteroids or neuroblocking agents. Myofiber atrophy and thick filament loss are the distinguishing pathologic features on muscle biopsy. Increased calpains expression and lysosomal and nonlysosomal proteolytic pathways have been claimed as possible pathogenic factors. Nevertheless, the mechanisms leading to myofiber atrophy and thick filament loss need further investigation. PATIENTS AND METHODS: The expression of ubiquitin and proapoptotic proteases as well as DNA fragmentation in muscle biopsies from three patients with acute quadriplegic myopathy were studied. RESULTS: All patients exhibited an important overexpression of caspases, calpain, cathepsin B, and ubiquitin, and the presence of numerous apoptotic nuclei in over 70% of myofibers. CONCLUSIONS: These data suggest that apoptosis mediated by proteolytic proteases may play a role in the pathogenesis of acute quadriplegic myopathy.

Acute Disease↗
PubMed1998

An elderly man with Klinefelter syndrome associated with hypertrophic cardiomyopathy, sick sinus syndrome, and coronary arteriovenous fistula.

Hypertrophic cardiomyopathy (HCM) is a rare cardiac complication in patients with Klinefelter syndrome. We report the case of a 67-year-old Japanese man with Klinefelter syndrome, HCM, sick sinus syndrome, and coronary arteriovenous fistula, in whom the 47XXY/46XY mosaic pattern was revealed by chromosomal study. Echocardiography revealed HCM with an interventricular septum thickness of 17 mm and a left ventricular posterior wall thickness of 10 mm. Sick sinus syndrome type III was diagnosed by paroxysmal atrial fibrillation (longest sinus arrest 9.0 sec) on 24-h Holter ECG recording. Coronary arteriovenous fistula was detected from the left anterior descending artery to the right ventricle by coronary arteriography. To our knowledge, this is the first case report of Klinefelter syndrome with HCM. As there have been a few reports of patients with Klinefelter syndrome in association with skeletal muscular diseases such as Becker-type muscular dystrophy or myotonic dystrophy, the gene mutation that causes Klinefelter syndrome may occur in the cardiac muscle. HCM may represent another variable expression of this chromosomal abnormality.

Aged↗
PubMed1999

Bcl-2 and Bax protein expression in human myopathies.

Expression of the apoptosis-related proteins Bcl-2 and Bax was analysed by means of immunohistochemistry in muscle biopsies from 13 patients suffering from different muscular diseases (inclusion body myositis n=4, polymyositis n=3, Becker muscular dystrophy n=4, necrotizing myopathy n=2, and controls n=4), in an attempt to learn about the role of these proteins in human muscle diseases associated with muscle fiber necrosis and regeneration. Increased Bcl-2 and Bax immunoreactivity was observed as fine granular precipitates in the cytoplasm or as subsarcolemmal aggregates in pathological cases. Increased Bcl-2 and Bax immunoreactivity was detected in some necrotic fibers, regenerating fibers, ring fibers and some apparently normal muscle fibers. In addition, increased Bcl-2 and Bax immunoreactivity was observed in the periphery of rimmed vacuoles and in muscle fibers with abnormal mitochondria in patients suffering from inclusion body myositis. Double-labelling immunohistochemistry disclosed co-localization of both proteins in about 50% of Bcl-2-immunoreactive fibers. Finally, double-labelling immunohistochemistry using N-CAM antibodies revealed that some Bax-positive fibers were regenerating fibers. Since increased Bax immunoreactivity was not restricted to necrotic muscle fibers, the present results suggest that over-expression of this protein in human myopathies is probably independent of the process of cell death.

Adolescent↗
PubMed1985

The significance of x-ray examinations of limb musculature in neuromuscular diseases with special regard to childhood diseases.

On the basis of examinations performed on 29 children suffering from various muscular diseases, the authors give a detailed discussion of the application, indication and advantages of muscles x-rays in diagnosing neuromuscular diseases. With the help of x-rays of the musculature an inside view can be obtained in vivo, in a non-invasive manner, of structural changes in the muscles, and the progress of the disease can be followed up objectively. The method opens up new possibilities of diagnosis and promotes a more thorough knowledge of the pathological processes.

Adolescent↗
PubMed2001

Lack of type XV collagen causes a skeletal myopathy and cardiovascular defects in mice.

Type XV collagen occurs widely in the basement membrane zones of tissues, but its function is unknown. To understand the biological role of this protein, a null mutation in the Col15a1 gene was introduced into the germ line of mice. Despite the complete lack of type XV collagen, the mutant mice developed and reproduced normally, and they were indistinguishable from their wild-type littermates. However, Col15a1-deficient mice showed progressive histological changes characteristic for muscular diseases after 3 months of age, and they were more vulnerable than controls to exercise-induced muscle injury. Despite the antiangiogenic role of type XV collagen-derived endostatin, the development of the vasculature appeared normal in the null mice. Nevertheless, ultrastructural analyses revealed collapsed capillaries and endothelial cell degeneration in the heart and skeletal muscle. Furthermore, perfused hearts showed a diminished inotropic response, and exercise resulted in cardiac injury, changes that mimic early or mild heart disease. Thus, type XV collagen appears to function as a structural component needed to stabilize skeletal muscle cells and microvessels.

Animals↗
PubMed1985

[Unusual muscular involvement in ankylosing spondylitis].

Muscle involvement in ankylosing spondylitis has been little studied. The authors report two cases with marked muscular atrophy and functional impotence, which had directed the diagnosis towards a myopathy over a period of several years in the first case, and a suspected primary muscular disease associated with ankylosing spondylitis in the second. Muscle biopsies eliminated the diagnosis of myopathy in both cases, with rapid functional recovery with proper treatment. Following a review of the literature, two hypotheses can be considered to explain the muscular involvement in ankylosing spondylitis: one mechanism which appears well-established is a radiculitis with involvement of the paravertebral muscles: other authors suggest that there is nonspecific, generalized muscular involvement in this disorder.

Adult↗
PubMedDate not supplied

Tubular aggregates in a case of osteomalacic myopathy due to anticonvulsant drugs.

We describe a case of severe osteomalacia and secondary myopathy due to anticonvulsant drugs--barbiturates and phenytoin. The clinical course of the patient, a 29-year-old epileptic woman subject to anorexia, demonstrates the importance of alimentary intake in the pathogenesis of the disease. Muscle biopsy performed before and after treatment showed the efficacy of therapy with 25-hydroxy cholecalciferol. However, in both biopsies tubular aggregates were found: this unusual ultrastructural finding probably indicates the direct action of the anticonvulsant drugs on the sarcoplasmic reticulum membranes. This is the second case of osteomalacic myopathy due to anticonvulsants confirmed by histologic and ultrastructural examinations; it is also the first report of tubular aggregates in this muscular disease.

Adult↗
PubMed1983

Demonstration of early lesions of muscle by infrared fluorescence microscopy.

Previous investigations demonstrated decrease or loss of IR fluorescence in certain lesions of smooth and striated muscle. These investigations were extended to diseases of skeletal muscle. Sections were stained and photographed as described (Puchtler et al. 1980). Various diseases caused significant changes of IR fluorescence. Often, IR fluorescence demonstrated lesions in muscle fibers which appeared unremarkable in direct light and/or conventional darkfield microscopy. The patterns of IR fluorescence varied widely, even within groups of diseases, e.g. muscular dystrophies. However, owing to the heterogeneity of the available material, it is not yet possible to draw conclusions concerning the diagnostic significance of different IR fluorescence patterns. Hence this report should be regarded only as a pilot study.

Adolescent↗
PubMed2003

[Antilipemic therapy and rhabdomyolysis].

The authors review the pathomechanism and frequency of side effects (myopathy and rhabdomyolysis) of the lipid lowering agents, statins and fibrates, on the basis of large prospective studies. They review in detail the possible pathomechanism of myopathy as caused by statins and fibrates. The cytochrome P450 enzyme system plays a role in the interaction of other drugs with long term statin therapy. The long term administration of statin and fibrate to patients with primary and secondary hyperlipidemia was considered safe on the basis of our experience and literature data. The combination of statin and fibrate is also considered safe and useful in the treatment of combined hyperlipidemia. As a precautionary measure, we emphasize the ruling out of liver, kidney and muscular diseases in such patients. Lipid lowering therapy is efficient in the primary and secondary prevention of cardiovascular diseases if applied cautiously.

Anticholesteremic Agents↗