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muscular dystrophies/hypoxie
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Hypoxemia during sleep in Duchenne muscular dystrophy.
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Overnight polysomnography after acclimatization was performed on 14 patients with Duchenne muscular dystrophy (mean age, 18.3 yr; mean VC, 1.24 L). Despite their lack of sleep-related symptoms and normal daytime blood gas tensions, periods of hypopnea and/or apnea (H/A) were observed in all patients
[Nasal flaring during hypoxemia in myotonic dystrophy and duchenne muscular dystrophy].
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We investigated the relationship between nasal flaring and SpO2 in 19 patients with Duchenne muscular dystrophy (DMD) and 26 patients with myotonic dystrophy (DM1). In DMD patients, nasal flaring was observed when SpO2 was lower than 96%, while it was not seen even at 82% of SpO2 in DM1. None of the
Chronic hypoxia impairs muscle function in the Drosophila model of Duchenne's muscular dystrophy (DMD).
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Duchenne's muscular dystrophy (DMD) is a severe progressive myopathy caused by mutations in the DMD gene leading to a deficiency of the dystrophin protein. Due to ongoing muscle necrosis in respiratory muscles late-stage DMD is associated with respiratory insufficiency and chronic hypoxia (CH). To
[The effect of clomipramine to nocturnal periodic hypoxemia in Duchenne muscular dystrophy].
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Respiratory insufficiency is a major cause of death in Duchenne muscular dystrophy (DMD). The earliest sign is a hypoventilation which appears in REM sleep. We administered 20 mg of clomipramine hydrochloride before sleep to two DMD patients with periodic nocturnal hypoxemia. The complaints such as
[Prandial hypoxia in progressive muscular dystrophy].
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Progressive muscular dystrophy patients often show progressive body weight loss in early adolescence. This severe body weight loss frequently causes superior mesenteric artery syndrome which may result in a fatal outcome. We performed prandial pulse oximetry and found 12 out of 35 Duchenne muscular
[Treatment of nocturnal periodic hypoxemia with safrazine hydrochloride in a patient with Duchenne muscular dystrophy under nasal intermittent positive pressure ventilation].
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Nocturnal periodic hypoxemia occurring in a 25-year-old Duchenne muscular dystrophy patient under NIPPV control was successfully treated with monoamine oxydase inhibitor (MAOI), safrazine hydrochloride. Five mg of safrazine hydrochloride was administered before sleep, and the periodic hypoxemia
[Nocturnal hypoxia and treatment in the patients with Duchenne muscular dystrophy].
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Overnight monitoring using pulse oximeter was performed on 10 patients with Duchenne muscular dystrophy (mean age, 22; mean %FVC, 26.6%; mean PaCO2, 54.1 Torr; mean PaO2 76.6 Torr). At the same time, spinal deformity and obesity were examined. In 4 patients, the measurement of the chest and
Inhibiting hypoxia in muscular dystrophy.
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[Question of hypoxia in progressive muscular dystrophy].
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[Progressive muscular dystrophy. VII. Detection of heterozygotes of Duehenne muscular dystrophy by determining serum creatine kinase with localized work load in anoxia].
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Noninvasive nasal mask-assisted ventilation in respiratory failure of Duchenne muscular dystrophy.
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The effects of noninvasive nasal mask-assisted ventilation were studied in two patients with chronic respiratory failure due to Duchenne's muscular dystrophy. Observations were made with continuous recordings of transcutaneous CO2 and O2 and ear oximetry. In one case, the mean tcPCO2 fell from 72 mm
[Facioscapulohumeral muscular dystrophy with sinus dysfunction].
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We report a 47-year-old man with facioscapulohumeral muscular dystrophy (FSHD) presenting with sinus dysfunction. He became unable to roll over and stand up at the age of 42, but he could still walk. Facial muscle involvement, scapular winging, asymmetrical involvement, funnel chest, and the absence
[Increased troponin I level in a Duchenne muscular dystrophy patient with normal coronarography].
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Duchenne muscular dystrophy (DMD) is a neuromuscular disease characterized by a progressive skeletal muscular weakness. As the respiratory care of such patients has been dramatically improved during the past few years, the DMD associated cardiomyopathy is becoming a new concern. We report a clinical
Pulmonary hypertension in progressive muscular dystrophy of the Duchenne type.
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Right heart catheterization was performed in 8 patients with progressive muscular dystrophy of the Duchenne type (DMD) at the advanced stage. A mean pulmonary arterial pressure in excess of 20 mmHg was observed in all cases. Five of them showed severe pulmonary hypertension with a mean pressure
[The usefulness of tracheostomy in Duchenne muscular dystrophy ventilated by a chest respirator].
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The respiratory dysfunction in Duchenne muscular dystrophy (DMD) patients increases with age. We have attempted various methods of artificial respiration for them. Recently, a non-invasive positive pressure ventilation (NIPPV) became the first choice of respiration, but the chest respirator (CR) was
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