myotonic dystrophy/hypoxie

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[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

[Aggravation of hypoxemia in supine position in myotonic dystrophy].

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Myotonic dystrophy (MyD) involves a variety of systems. Respiratory disorders are common, namely elevation of diaphragm, alveolar hypoventilation, aspiration pneumonia and sleep apnea. We evaluated respiratory involvement. The subjects were 11 patients with MyD. Also 6 patients with limb girdle

[Anesthetic management of a child with congenital myotonic dystrophy and perioperative hypoxia].

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A 7-year-old boy with congenital myotonic dystrophy (MD) and developmental retardation underwent an emergency surgery for strangulation ileus. General anesthesia was maintained using sevoflurane and fentanyl. While intraoperative arterial blood pressure, pulse and rectal temperature remained stable,

[Hypoxia after cesarean section in a patient with myotonic dystrophy].

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A 28-year-old woman, 35 week-pregnant, underwent emergency cesarean section. Although she had no apparent symptoms before the operation, myotonic dystrophy was suspected from physical examination and laboratory data. The anesthesiologist in charge recognized only high creatine kinase. Combined

Sleep hypoxia in myotonic dystrophy and its correlation with awake respiratory function.

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BACKGROUND Tiredness and daytime respiratory failure occur frequently in myotonic dystrophy. Sleep hypoxaemia was studied in 12 patients with myotonic dystrophy and correlations were sought with their daytime lung and respiratory muscle function. METHODS All patients underwent overnight sleep

[Excessive daytime sleepiness, central type sleep apnea and myotonic dystrophy].

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Two cases of myotonic dystrophy with excessive daytime somnolence are described. All-night polysomnographic studies were performed revealing high number of central sleep apnea which triggered micro-arousals and awakenings leading to decrease of sleep efficiency as well as of stage 3, 4 and REM.

Respiratory and hemodynamic study during wakefulness and sleep in myotonic dystrophy.

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Six young male patients with grade I (mild) myotonic dystrophy and a complaint of excessive daytime sleepiness were studied during wakefulness and sleep. Pulmonary function tests during wakefulness showed evidence of mild abnormality related to respiratory muscle weakness. During sleep, some

Perioperative hazards in myotonic dystrophy.

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A 32-year-old man with myotonic dystrophy underwent a thoracotomy for removal of a mediastinal thymoma. Pre-operative examination revealed features of myotonic dystrophy, the only other abnormality was mild restrictive pulmonary disease. Anaesthesia was induced with thiopentone and maintained with

[A case of myotonic dystrophy with morning headache following sleep apnea syndrome].

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We reported a 39-year-old man with myotonic dystrophy. He suffered from morning headache. Respiratory function tests showed restrictive pattern and arterial gas analysis showed hypoxia and hypercapnia with respiratory acidosis (PaCO2 50.8 mmHg, PaO2 63.8mmHg, pH 7.317, SaO2 89.8%). Polysomnograph

Ventilatory response in myotonic dystrophy.

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Patients with myotonic dystrophy often develop respiratory failure caused by alveolar hypoventilation. Abnormalities in the ventilatory response to hypoxia and hypercapnia may explain this phenomenon. Accordingly, hypoxic and hypercapnic responses were measured in seven patients with myotonic

Control and modulation of respiration in Steinert's myotonic dystrophy.

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Respiratory impairment in patients with Steinert's muscular dystrophy is known to lead to respiratory failure. Both the blunted chemical drive of breathing and the respiratory muscle weakness have been cited in the pathophysiology of premature death in these patients. To further assess the chemical

[Muscular disability and organ impairments in myotonic dystrophy type 1].

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BACKGROUND Myotonic dystrophy (DM1) is a multisystemic disorder characterized by myotonic muscular weakness, and numerous organ impairments, especially cardiac and respiratory disorders. The goal of this study was to evaluate in DM1 patients the relationships between a new muscular disability scale,

Pulmonary function and electromyographic study of respiratory muscles in myotonic dystrophy.

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Ten adult myotonic dystrophy patients underwent measurements of lung function, maximal dynamic and static ventilatory efforts, and respiratory muscle electromyography (EMG). EMG studies were performed during spontaneous breathing or when subjects breathed through high inspiratory or expiratory

[Diurnal sleep apnea in myotonic dystrophy].

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We investigated diurnal sleep apnea in myotonic dystrophy with respiratory inductive plethysmography. Five of eight patients met criteria for sleep apnea syndrome and had central apnea mainly. In a case showing periodic breathing with apnea like Cheyne-Stokes type breathing, the duration of apnea

[Respiratory pathophysiology during sleep in patients with myotonic dystrophy].

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Myotonic dystrophy is a genetic disorder inherited as an autosomal dominant trait. It is known to be associated with endocrine dysfunction, polar cataracts, cardiac abnormalities and other conditions. Respiratory distress constituents the principal problem in myotonic dystrophy. The author

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