myotonic dystrophy/offita

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[Persistent apnea in an obese patient with myotonic dystrophy].

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We report a case of a 35-year-old woman with myotonic dystrophy and severe obesity of BMI 43.3 who showed persistent apnea at emergence after ovarian resection. The patient received an iv induction with minimum dose of propofol and vecuronium 3 mg. Anesthesia was maintained with propofol, 50%

Anesthetic management of a patient combining myotonic dystrophy and obesity.

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We describe the anesthetic management of an obese myotonic patient who underwent bilio-pancreatic diversion for severe obesity. A female, 35 years old (weight 137 kg, height 160 cm, excess body weight 120%) suffered from myotonic dystrophy and obesity, complicated by a mild heart failure and

Mivacurium in an obese patient with myotonic dystrophy--a case report.

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Mivacurium is a short-acting non-deplolarizing neuromuscular blocking agent. Its use in an obese patient with myotonic dystrophy undergoing laparoscopic cholecystectomy is reported. Mivacurium was used as a bolus dose of 0.07 mg. kg-1, followed by a continuous infusion at the rate of 0.003 mg. kg-1.

Overweight Is an Independent Risk Factor for Reduced Lung Volumes in Myotonic Dystrophy Type 1.

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BACKGROUND In this large observational study population of 105 myotonic dystrophy type 1 (DM1) patients, we investigate whether bodyweight is a contributor of total lung capacity (TLC) independent of the impaired inspiratory muscle strength. METHODS Body composition was assessed using the

Organ dysfunction and muscular disability in myotonic dystrophy type 1.

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Myotonic dystrophy type 1 (DM1) is a multisystemic disorder characterized by muscle weakness and multiple organ impairment, especially the eyes, lung, and heart. We conducted the current study to analyze the prevalence and intercorrelation among these disorders and their respective relationships

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

Environmental influence on altered receptor function in a genetic disease: insulin and glucose affect insulin receptors in myotonic dystrophy.

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Insulin action in vivo and insulin binding to monocytes in vitro were correlated in patients with myotonic dystrophy (MyD) and compared with healthy controls. Confirming our previous studies and those of others, the present results show that the glucose infusion rate (DR), an estimate of in vivo

Swallowing impairments in Amyotrophic Lateral Sclerosis and Myotonic Dystrophy type 1: Looking for the portrait of dysphagic patient in neuromuscular diseases.

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BACKGROUND Dysphagia is a critical symptom of Neuromuscular Diseases and is often associated with considerable morbidity and mortality. OBJECTIVE This study is designed to investigate the prevalence of dysphagia and to identify different clinical profiles of swallowing disorders in Myotonic

Dramatic improvement of blood glucose control after pioglitazone treatment in poorly controlled over-weight diabetic patients with myotonic dystrophy.

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Insulin resistance is mainly present in skeletal muscle in non-obese patients with myotonic dystrophy. Thiazolidinediones are reported to reduce insulin resistance in these patients. However, the effects of pioglitazone in overweight patients with myotonic dystrophy and type 2 diabetes mellitus have

Experiences with bariatric surgery in patients with facioscapulohumeral dystrophy and myotonic dystrophy type 1: A qualitative study.

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Overweight and obesity are common in patients with facioscapulohumeral dystrophy (FSHD) and myotonic dystrophy type 1 (DM1). Lifestyle change is often challenging for patients with neuromuscular diseases, especially to increase physical activity. When lifestyle changes have not been effective,

Surgical treatment of obesity in DM1--a case report and a review of the literature.

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In patients with myotonic dystrophy type 1 overweight and obesity are frequent. When present this has further negative effects on the patients' pulmonary and muscle function as well as social participation. Anesthesia in myotonic dystrophy type 1 patients is associated with increased risks,

Postoperative ventilatory failure in an obese, myopathic woman with periodic somnolence: A case report.

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An obese woman with periodic sommolence developed postoperative ventilatory problems following an uncomplicated cholecystectomy. A severe myopathic process involving the respiratory muscles was found at necropsy. A brother, who died a week later, had myotonic dystrophy.

Glucocorticoid metabolism and adrenocortical reactivity to ACTH in myotonic dystrophy.

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Dysfunction of the hypothalamic-pituitary-adrenal axis might contribute to metabolic disturbances frequently encountered in myotonic dystrophy. We hypothesized that abnormal adrenocortical sensitivity to ACTH and/or glucocorticoid metabolism could be important in myotonic dystrophy. We assessed

Assessment of sleep studies in myotonic dystrophy.

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Myotonic dystrophy (MD) is the commonest adult muscular dystrophy and is associated with respiratory muscle weakness. The role of screening sleep studies is unclear in MD. We prospectively evaluated polysomnography/overnight oximetry in a group of MD patients and related this to the daytime

Non-alcoholic fatty liver disease later diagnosed as myotonic dystrophy

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Background: Myotonic dystrophy (MD) is sometimes accompanied by metabolic/endocrine disorders, including dyslipidemia, central obesity, and hypogonadism. Due to considerable individual differences in the severity and progression of

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