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)

A patient with a progressive, inherited disease of the CNS developed remarkable esophageal dilatation without the lower esophageal sphincter dysfunction characteristic of achalasia. Postmortem examination included neuropathologic study of the CNS and evaluation of esophageal myenteric plexus histology, but the pathogenesis of the megaesophagus remains unknown.
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PMID:Megaesophagus and hereditary nervous system degeneration. 796 58

Megaoesophagus was observed in 82 Long-Evans rats aged 3-32 months. Clinically, the hair was coarse, the neck distended, the mouth opened, wetted by saliva and soiled by bedding material, and the respiration characterized by tachypnoea and inspiratory crackles. By radiography, after barium administration, the oesophagus was seen to be dilated and filled with impacted dry food in the precardial region. The size of the dilatation varied depending on the oesophageal region and reached a diameter of 12 mm in the most severe case. Histologically, the muscular layers of the dilated portions showed focal inflammation and single fibre necrosis. Each affected oesophagus had (1) an increased circumference and radius and a reduced number of myenteric ganglion cells in both the thoracic and abdominal portions, and (2) a decreased thickness of the muscular layers in the thoracic portion and at the level of the cardia. A simple geometrical model showed that the reduced ganglion cell number was not due to a change in shape of the dilated oesophagus. Since no sign of infectious disease was found in these animals, and rats of other strains kept at the same time under the same conditions were not affected, a hereditary aetiology is suggested.
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PMID:Megaoesophagus in rats: a clinical, pathological and morphometrical study. 831 55

The aim of this paper is to describe a patient with severe postpolio problems who developed achalasia. A 66-year-old patient came to our observation for severe dysphagia. He had suffered from paralytic poliomyelitis at the age of 7 months and had severe residual deficits. At the age of 62 he presented with sudden pain localized in the distribution of the C4 and C5 dermatomes and an inability to abduct the left arm. At the time, he experienced only occasional and mild dysphagia; his esophagus was not dilated and emptied normally. Over the following months his muscular function improved, but dysphagia worsened. We found a megaesophagus with a sigmoid appearance and the manometric features of achalasia. Pneumatic dilatation produced good resolution of dysphagia. A year later manometry showed the reappearance of peristalsis after all wet swallows. In patients with postpolio dysphagia, the possible presence of achalasia must be considered.
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PMID:Achalasia. A possible late cause of postpolio dysphagia. 861 25

From 1985 to 1995, 60 patients with a mean age of 52 +/- 12 years [24-78] underwent colon interposition for esophageal replacement. Indications were esophageal cancer (n = 37), benign stricture (n = 13), iatrogenic esophageal fistula (n = 5), achalasia with megaesophagus (n = 3), and necrosis of a previous substitute (n = 2). Colon interposition represented only 18.5% of all operations performed for oesophageal replacement during the same period. The colon was selected because of inadequate stomach in 33 cases (55%). Long-segment conduit based on the ascending branch of the left colonic artery was the preferred method and could be used in 52 patients (86.7%). The colon was placed in the esophageal bed in 38 patients (63.3%), substernally in 21 (35%), and subcutaneously in 1. Overall operative mortality and morbidity were 8.3% and 65% respectively. Five-year survival rate was 9% in the 37 patients with esophageal cancer. Seven patients (13.5%) required one or more dilatations of the esophagocolonic anastomosis. At last follow-up, 34 patients (65.4%) had no difficulty eating. Multivariate analysis identified the conduit position in the posterior mediastinum as an independent predictor of good functional result (p = 0.0018). We conclude that colon interposition for esophageal replacement provides satisfactory and durable function; however, early mortality and morbidity are substantial.
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PMID:[Technique and results of colonic esophagoplasties]. 876 61

The association between upper gastrointestinal (GI) motility disorders and respiratory problems is reviewed. Upper GI motility disorders, such as gastroesophageal reflux disease, gastroparesis, and achalasia, have been associated with respiratory problems, including aspiration, airway obstruction, asthma, bronchospasm, chronic cough, and laryngitis. These associations, which had been based solely on clinical observation, have recently been supported by physiologic studies and treatment trials. The association of reflux disease with asthma has the most support. Up to 80% of persons with asthma have evidence of pathologic gastroesophageal reflux, and in several studies antireflux therapy with prokinetic agents, antisecretory drugs, or fundoplication surgery has been found to reduce asthma symptoms and the need for medication in some patients. Reflux has also been associated with chronic cough and laryngitis, and antireflux therapy can reduce respiratory symptoms. Gastroesophageal reflux, gastroparesis, and achalasia are all associated with aspiration. In addition, in rare instances, the megaesophagus associated with achalasia can produce mechanical airway obstruction. Effective therapy for these GI motility disorders can eliminate complicating respiratory problems.
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PMID:Upper gastrointestinal motility disorders and respiratory symptoms. 893 26

An accurate preoperative diagnosis of an esophageal motor disorder, including its location (the LES, the body of the esophagus, or both), is essential before proceeding with esophagomyotomy. The operative procedure should be performed with careful attention to certain technical details to ensure completeness of the myotomy, to prevent later healing of the myotomy, and to avoid radical cardiomyotomy that might facilitate the subsequent development of GER. Potential hazards are associated with performing a 360 degrees antireflux procedure in the presence of an aperistaltic esophagus. Early operation before the development of megaesophagus is recommended. The excellent results achieved by resection coupled with antrectomy and Roux-en-Y diversion suggest its wider application to patients with one or more previous failed myotomies, particularly patients with stricture and megaesophagus. Long-term surveillance of patients with achalasia is mandatory in view of the known risk of late development of squamous cell carcinoma.
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PMID:Failure after esophagomyotomy for esophageal motor disorders. Causes, prevention, and management. 924 98

The objective of this study was to determine whether myenteric denervation of the abdominal esophagus using benzalkonium chloride (BAC) leads to esophageal achalasia with changes of the muscle propria and epithelial cell proliferation. The treatment led to megaesophagus 3 months after BAC application. Denervation of the esophagus induced muscle hypertrophy and increased epithelial cell proliferation. The imbalance of the neurotransmitters may play a role in these morphokinetic changes.
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PMID:Megaesophagus in rats. 938 Sep 50

Megaoesophagus resulting from achalasia is a rare but serious cause of airway obstruction. The exact aetiology remains unclear. Although 29 cases have previously been reported, the potential need for urgent treatment has not been sufficiently emphasized. Some forms of treatment with drugs or decompression with a fine tube have been advocated but emergency tracheal intubation may become necessary. A 90-year-old lady had a bolus of food lodged in her oesophagus but with no respiratory symptoms. The bolus was removed under anaesthesia. Six weeks later she suffered similar symptoms after eating but developed severe airway obstruction over 10 min. Emergency intubation of the trachea was necessary before removing the food bolus under general anaesthesia. This case demonstrates the urgency with which these patients may need to be treated. The condition can rapidly worsen due to swallowing of air and saliva.
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PMID:Rapidly developing airway obstruction resulting from achalasia of the oesophagus. 1092 42

Achalasia presenting as acute airway obstruction is an uncommon complication. We report the case of an elderly woman with previously undiagnosed achalasia who presented with acute respiratory distress due to megaesophagus. Emergency endotracheal intubation and insertion of a catheter into the esophagus, with continuous aspiration was required. Upon introduction of the esophageal catheter an abruptand audible air decompression occurred, with marked improvement of the clinical picture. Endoscopic injection of botulinum toxin was chosen as the definitive treatment with good clinical result. The pathophysiology of the phenomenon of esophageal blowing in achalasia is unclear, but different hypothetical mechanisms have been suggested. One postulated mechanism is an increase in upper esophageal sphincter (UES) residual pressure or abnormal UES relaxation with swallowing in achalasia patients. We reviewed the UES manometric findings in 50 achalasia patients and compared it with measurement performed in 45 healthy controls. We did not find any abnormalities in UES function in any of our achalasia patients group, or in the case under study. An alternative hypothesis postulates that airway compromise in patients with achalasia results from the loss UES belch reflex (abnormal UES relaxation during esophageal air distension), and in fact, an abnormal UES belch reflex was evidenced in our case.
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PMID:Achalasia presenting as acute airway obstruction. 1111 88

As the few randomized controlled trials available in the literature comparing botulinum toxin (BT) injection with established endoscopic treatment of achalasia cardia, i.e. pneumatic dilatation, showed conflicting results, we conducted a prospective randomized trial. Seventeen consecutive patients with achalasia cardia diagnosed during a period between December 1997 and February 2000 were randomized into two treatment groups [pneumatic dilatation by Rigiflex dilator (n=10), BT injection by sclerotherapy needle into four quadrants of lower esophageal sphincter (LES) (n=7) 80 units in five cases, 60 units in two cases] after dysphagia grading, endoscopy, barium esophagogram, and manometry, all of which were repeated 1 week after treatment. Patients were followed up clinically for 35.2+/-14 weeks. Chi-squares, Wilcoxon rank-sum test, Kaplan-Meier method and log-rank tests were used for statistical analysis. After 1 week, 6/7 (86%) BT-treated vs. 8/10 (80%) dilatation-treated patients improved (P=NS). There was no difference in LES pressure and maximum esophageal diameter in the barium esophagogram in the two groups before therapy. Both therapies resulted in significant reduction in LES pressure. The cumulative dysphagia-free state using the Kaplan-Meier method decreased progressively in BT-treated compared with dilatation-treated patients (P=0.027). Two patients with tortuous megaesophagus, one of whom had failed dilatation complicated by perforation previously, improved after BT. One other patient in whom pneumatic dilatation had previously failed improved in a similar manner. BT is as good as pneumatic dilatation in achieving an initial improvement in dysphagia of achalasia cardia. It is also effective in patients with tortuous megaesophagus and previous failed pneumatic dilatation. However, dysphagia often recurs during 1-year follow up.
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PMID:Randomized controlled trial of intrasphincteric botulinum toxin A injection versus balloon dilatation in treatment of achalasia cardia. 1186 25


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