myotonic dystrophy/uppköst

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A case of myotonic dystrophy (MD) associated with glucose-induced hyperinsulinemia followed by reactive hypoglycemia and increased number of cytosine-thymine-guanine (CTG) trinucleotide repeats in MD gene.

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A 39-year-old man with myotonic dystrophy consulted our hospital for nausea, vomiting and dizziness that occurred after 75 g oral glucose tolerance test (OGTT). Reexamination of OGTT revealed remarkable hyperinsulinemia (622 microU/ml) followed by reactive hypoglycemia (50 mg/dl) and such

Steakhouse Syndrome in Myotonic Dystrophy.

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A 70-year-old man with myotonic dystrophy (MD) showed repetitive vomiting and decreased food ingestion. These symptoms were caused by acute mass of steak impaction occluding the esophagus, known as "steakhouse syndrome," which may have occurred in response to esophageal functional changes following

Dysphagia and dystrophia myotonica. A case report.

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A 26-year-old Indian man who presented with a long history of vomiting, upper abdominal pain and dysphagia is described. The dysphagia had been largely overlooked and investigation delayed. The diagnosis of dystrophia myotonica (DM) was apparent on clinical examination and his symptoms responded

Anaesthetic management of a patient with myotonic dystrophy.

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A 13-year-old boy with myotonic dystrophy underwent insertion of a percutaneous gastrostomy feeding tube under general anaesthesia. We used a laryngeal mask airway and a spontaneously breathing technique with propofol total intravenous anaesthesia. Postoperative vomiting and aspiration, 12 h after

Gastric emptying in myotonic dystrophy.

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BACKGROUND Gastrointestinal symptoms are common and important for the quality of life in patients with myotonic dystrophy (MD). Gastric emptying was studied in patients with MD who suffered from symptoms suggesting slow gastric emptying and the effect of prokinetic treatment was

Gastric volvulus complicating myotonic dystrophy.

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Myotonic dystrophy is an autosomal inherited disorder of both striated and smooth muscle, and is considered to be a rare cause of gastrointestinal dilatation and abnormal peristalsis. We report on a patient with myotonic dystrophy complicated by gastric volvulus. A 57-year-old female with myotonic

Continuous propofol anaesthesia for patients with myotonic dystrophy.

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Myotonic dystrophy, a rare genetic disorder, may pose a serious problem to the anaesthesiologist due to muscular and extramuscular involvement. Thirteen patients, median age 21 yr were anaesthetized by continuous propofol infusion, fentanyl, atracurium and N2O to evaluate this combination in

Gastrointestinal symptoms in myotonic dystrophy.

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BACKGROUND Gastrointestinal complaints may be the initial symptom in myotonic dystrophy (MD). However, the frequency of gastrointestinal symptoms has never been prospectively evaluated. METHODS Forty patients with MD were interviewed with regard to their GI symptoms with a standardized

[Upper abdominal pain, nausea, and vomiting in a 63-year-old woman].

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We present a case of myotonic dystrophy type I and its complications including cholangitis and cardiac arrhythmias. The therapy options focus on the consequences of the disease and result in an endoscopic retrograde cholangiography (ERC) and implantation of an implantable cardioverter-defibrillator

Isolated neonatal swallowing dysfunction: a case series and review of the literature.

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Our purpose was to describe the natural history of isolated neonatal swallowing dysfunction (INSD). Nine infants with INSD are described. Eight presented within two weeks of birth. Symptoms included choking and cyanotic spells with feeds, recurrent aspiration, apnea, stridor, and vomiting. Three had

Intestinal pseudoobstruction as a feature of myotonic muscular dystrophy.

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We report two cases of intestinal pseudoobstruction caused by visceral smooth muscle involvement due to myotonic muscular dystrophy. Two patients with myotonic muscular dystrophy presented with abdominal pain, distention, constipation, and vomiting. The exclusion of mechanical obstruction by plain

Management of pneumatosis intestinalis in children over the age of 6 months: a conservative approach.

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BACKGROUND Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising

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