Cyclic Vomiting Associated With Excessive Dopamine in Riley-day Syndrome

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The Riley-Day syndrome

Familial dysautonomia (Riley et al., 1949) is an autosomal recessive disease affecting Jews of Eastern European ancestry (Brunt and McKusick, 1970). As the name implies the autonomic nervous system is defective, and this is seen clinically as defective control of temperature, blood pressure, and swallowing, by skin blotching, excessive sweating, lack of tearing, and bowel disturbance. Abnormali...

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Familial dysautonomia (Riley-Day syndrome).

Familial dysautonomia, also known as Riley-Day syndrome, is a disorder of autonomic nervous system with an autosomal recessive mode of inheritance. Reduction and/or loss of unmyelinated and small myelinated fibers is found, as reduction of dopamine beta-hydroxylase in blood. The diagnosis is based on clinical features: diminished lacrimation, insensitivity to pain, poor temperature control, abo...

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Riley-Day Syndrome (congenital familial dysautonomia).

THIS rare disease is of particular importance to ophthalmologists because of the congenital failure of tear production and corneal anaesthesia. It was first described as an entity by Riley, Day, Greeley, and Langford (1949). Since then, many further cases have been reported (Braun-Vallon and Bessman, 1960; Levin, 1960; Laxdal, Khera, and Haworth, 1961; Thieffry, Joseph, Martin, Job, and Lorthol...

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Cyclic Vomiting Syndrome

Obscure gastrointestinal bleeding constitutes approximately fi ve percent of all gastrointestinal bleeding cases. Patients with this condition are often diffi cult to diagnose and frequently undergo extensive GI testing. This is no news item to all gastrointestinal specialists. The following cases highlight several different presentation scenarios, the long road to correct diagnosis, and some i...

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Cyclic Vomiting Syndrome

Cyclic vomiting syndrome (CVS) is a chronic functional disorder characterized by episodes of severe nausea and vomiting that alternate with prolonged symptom-free intervals.1 Symptoms are often triggered by social stress; episodes can occur after the loss of a loved one, job-related stress, during exams, and even on vacations.2 The etiology of CVS is not known, but several theories have been pr...

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ژورنال

عنوان ژورنال: Journal of Clinical Gastroenterology

سال: 2013

ISSN: 0192-0790

DOI: 10.1097/mcg.0b013e3182582cbf