Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Esophageal achalasia (EA) has been historically treated by esophageal dilatation or myotomy with or without fundoplication. Botulinum toxin (Botox-Allergan) use in pediatric EA has not been previously described. The authors' objective was to observe the efficacy of botulinum toxin injection into the lower esophageal sphincter (LES) for EA. An 11-year-old boy presented with a 9-month history of frequent pneumonia, productive cough, and a 1-year history of chest discomfort and odynophagia. Chest radiograph showed changes compatible with aspiration. Upper gastrointestinal (UGI) series showed typical narrowing of the LES, and 24-hour pH study showed no reflux. Esophageal manometry showed classic findings of achalasia. An upper gastrointestinal endoscopy was performed showing a huge volume of retained food. A direct four-quadrant injection was performed with a total of 100 U of botulinum toxin into the LES. UGI series showed improvement in esophageal emptying. Esophageal manometry showed impressive improvement in LES pressure (preinjection, 44.1 mm Hg to postinjection mean of 16.6 mm Hg), percent relaxation (preinjection, 30% to postinjection, 58.8%), and duration of relaxation (preinjection, 1.9 seconds to postinjection, 11 seconds). The patient has not had any further respiratory symptoms, chest pain, or odynophagia in 8 months of follow-up. Botulinum toxin injection is simple and effective for EA and merits its study in a prospective manner in the pediatric population.
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PMID:Botulinum toxin use in pediatric esophageal achalasia: a case report. 920 Jan

Acute airway obstruction from mega-esophagus is a rare presentation of achalasia. Mega-esophagus is generally considered when the transverse width of the esophagus is more than 7 cm. A 78 year old lady presented with longstanding history of productive cough and nocturnal dyspnoea. She was seen in the emergency department with acute exacerbation of dyspnoea after a bout of vomiting, containing semi-solid food with foetid smell. Her respiratory status deteriorated rapidly with onset of stridor, and cyanosis. Chest x-ray showed widening of mediastinum due to dilated esophagus with air-fluid level. Prompt, repeated, upper respiratory tract suction was carried out. A wide bore nasogastric tube was introduced, esophagus was decompressed with a gush of air and fluid, relieving the respiratory distress. This case illustrates an unusual presentation of achalasia underscoring the need for urgent, life-saving esophageal decompression. Hypotheses, regarding the mechanism of airway compromise, as well as, treatment options are reviewed.
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PMID:Achalasia esophagus; presenting as acute air way obstruction. 1790 30