Gastric emptying in myotonic dystrophy

Rönnblom, Anders; Andersson, Staffan; Hellström, PM; Danielsson, Åke

doi:10.1046/j.1365-2362.2002.01028.x

Cited by 36 publications

(27 citation statements)

References 19 publications

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“…Researchers highlight that up to 40% of children and young adults can experience GI symptoms, which include pain, bloating, altered bowel habit, and constipation. 9 Furthermore, Rönnblom et al 10 conducted a study in 2002 to monitor gastric emptying in 10 patients with MD; in this study, they suggested that MD is associated with reduced gastric motility. However, because of a lack of high-quality studies, it is still up for debate whether MD causes CIPO via damage to the enteric nervous system, smooth muscle, or secondary endocrine changes.…”

Section: Figurementioning

confidence: 99%

The Use of Pyridostigmine in a Child With Chronic Intestinal Pseudo-Obstruction

et al. 2018

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Chronic intestinal pseudo-obstruction is a rare disorder that affects the motility of the gastrointestinal tract. It results in acute or subacute intestinal obstruction symptoms in the absence of mechanical lesion. It can lead to intestinal failure in children with significant strain on nutrition, growth, and development. There is no universally agreed protocol for management of chronic intestinal pseudo-obstruction in children, and there is wide variation in clinical practice.

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Section: Figurementioning

confidence: 99%

The Use of Pyridostigmine in a Child With Chronic Intestinal Pseudo-Obstruction

et al. 2018

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“…In 28% of patients, gastrointestinal problems occurred before the diagnosis of DM1 was made and 25% of patients find the worst symptoms are gastrointestinal related. The burden for patients of gastrointestinal related symptoms has generally been underecognised 47 48…”

Section: Myotonic Dystrophy Typementioning

confidence: 99%

The myotonic dystrophies: diagnosis and management

Turner

Hilton‐Jones

2010

Journal of Neurology, Neurosurgery & Psychiatry

268

208

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There are currently two clinically and molecularly defined forms of myotonic dystrophy: (1) myotonic dystrophy type 1 (DM1), also known as 'Steinert's disease'; and (2) myotonic dystrophy type 2 (DM2), also known as proximal myotonic myopathy. DM1 and DM2 are progressive multisystem genetic disorders with several clinical and genetic features in common. DM1 is the most common form of adult onset muscular dystrophy whereas DM2 tends to have a milder phenotype with later onset of symptoms and is rarer than DM1. This review will focus on the clinical features, diagnosis and management of DM1 and DM2 and will briefly discuss the recent advances in the understanding of the molecular pathogenesis of these diseases with particular reference to new treatments using gene therapy.

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“…91,92 Of these, abdominal pain, dysphagia, vomiting, diarrhea, coughing while eating, and fecal incontinence were the most common in one survey 92 ; up to 25% of patients consider gastrointestinal involvement as the most disabling feature of their disease. 93 Delayed gastric emptying and gastroparesis have indeed been well documented, [94][95][96] and instances of gastric volvulus described. 97 In Duchenne dystrophy, gastric motor dysfunction is an early feature, 98 resulting in hypomotility and gastroparesis, 99 which can be profound and result in acute gastroparesis and gastric dilatation.…”

Section: Muscle Diseasementioning

confidence: 98%