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 dilation is the treatment of choice for most patients with esophageal dysphagia (functional and mechanical). Multiple forms of esophageal dilators are available. Mechanical dilators (guidewire/nonguidewire assisted) are the major forms of dilators used. Balloon dilator use has increased but they offer only a marginal advantage over traditional mechanical dilators at a greatly increased cost (2 degrees to single use). Comparative trials are biased in favor of balloon dilators, but balloon dilators are not indicated for empiric dilation for dysphagia. Empiric dilation for solid food dysphagia is still controversial. Dilation is rarely associated with complications and is rarely contraindicated unless previous dilation attempts have been unsuccessful. Special circumstances such as caustic strictures, radiation stricture, and dysphagia associated with eosinophilic esophagitis should engender cautious dilation. Attention to detail about placement of guidewires and stricture type are still critical for safety. Predilation barium studies are not needed in all patients but should be employed if the endoscope is not able to pass the stricture and stricture length and angulation are unknown. Intralesional steroids and proton pump inhibitor therapy are important adjuvant treatments for resistant strictures and reflux associated strictures. Balloon dilation for achalasia is still a viable alternative, but it is likely to decrease in usage with the advent of more widespread laparoscopic myotomy.
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PMID:Esophageal Dilation / Dilators. 1562 37

The lower oesophageal sphincter (LOS) is a specialized segment of the circular muscle layer of the distal oesophagus, accounting for approximately 90% of the basal pressure at the oesophago-gastric junction. Together with the crural diaphragm, it functions as an antireflux barrier protecting the oesophagus from the caustic gastric content. During swallowing or belching, the LOS muscle must relax briefly in order to allow passage of food or intragastric air. These swallow-induced and prolonged transient lower oesophageal sphincter relaxations (TLOSRs) respectively result from activation of the inhibitory motor innervation of the sphincter. Both in man and animals, the main neurotransmitter released by the inhibitory neurones is nitric oxide. The two typical examples of dysfunction of the LOS are achalasia and gastro-oesophageal reflux disease (GORD). Achalasia is characterized by reduction or even absence of the inhibitory innervation to the LOS, leading to impaired LOS relaxation with dysphagia and stasis of food in the oesophagus. On the contrary, GORD results from failure of the antireflux barrier, with increased exposure of the oesophagus to gastric acid. This leads to symptoms such as heartburn and regurgitation, and in more severe cases to oesophagitis, Barrett's oesophagus and even carcinoma. To date, TLOSRs are recognized as the main underlying mechanism, and may represent an important target for treatment. More insight in the pathogenesis of both diseases will undoubtedly lead to new treatments in the near future.
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PMID:The lower oesophageal sphincter. 1583 51

The goals in the treatment of achalasia are threefold: 1) relieving the symptoms, particularly dysphagia and bland regurgitation; 2) improving esophageal emptying by disrupting the poorly relaxing lower esophageal sphincter (LES); and 3) preventing the development of megaesophagus. Although achalasia cannot be permanently cured, excellent palliation is available in over 90% of patients, especially those with pneumatic dilation and laparoscopic Heller myotomy. The efficacy for short- and long-term therapy seems to be similar when performed by experts. Pneumatic dilation done as an outpatient surgery disrupts the LES muscle from within by using balloons of progressively larger diameter (3.0, 3.5, and 4.0 cm). Repeat dilations may be required; secondary severe gastroesophageal reflux disease (GERD) is rare, but approximately 2% of patients will have an esophageal perforation. A surgical Heller myotomy is now being done laparoscopically through the abdomen that cuts the LES and extends the myotomy 2 to 3 cm onto the stomach. Usually 2 days of hospitalization is required, and patients can normally return to work in 1 to 2 weeks. Severe GERD with esophagitis and peptic stricture is a common complication; therefore, most surgeons combine the myotomy with an incomplete fundoplication. Medical therapy is much less effective than these invasive procedures. Smooth muscle relaxants (nitrates and calcium channel blockers) taken immediately before meals improve dysphagia, but side effects and drug tolerance are common. The injection of botulinum toxin (100 to 200 units) endoscopically into the LES gives short-term relief of symptoms and improves esophageal emptying. This treatment is most effective in the elderly, as symptom relief can last up to 1 to 2 years with a single injection. Several studies suggest the most cost-effective management of achalasia is initial treatment with pneumatic dilation.
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PMID:Modern management of achalasia. 1600 28

The barium esophagram is a valuable diagnostic test for evaluating structural and functional abnormalities of the esophagus. The study is usually performed as a multiphasic examination that includes upright double-contrast views with a high-density barium suspension, prone single-contrast views with a low-density barium suspension, and, not infrequently, mucosal-relief views with either density of barium suspension. The double-contrast phase optimizes the ability to detect inflammatory or neoplastic diseases, whereas the single-contrast phase optimizes the ability to detect hiatal hernias and lower esophageal rings or strictures. Fluoroscopic examination of the esophagus is also important for assessing motility disorders such as achalasia and diffuse esophageal spasm. This article is a review of gastroesophageal reflux disease, other types of esophagitis, benign and malignant esophageal tumors, varices, lower esophageal rings, diverticula, and esophageal motility disorders, all of which can be diagnosed with the aid of esophagography.
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PMID:Diseases of the esophagus: diagnosis with esophagography. 1617 17

We report a case of esophageal achalasia (EA) in Downa9s syndrome. A six-year-old girl with DS was referred from another hospital for further management of dysphagia and growth retardation (weight 16.3 kg, height 105.4 cm: both below -1.5 SD). Dysphagia commenced when she was one year old and gastroesophageal reflux (GER) was diagnosed when she was four. Routine investigations suggested EA or esophageal stenosis secondary to GER. While dissecting the esophago-gastric junction in preparation for a Heller-Dor esophagocardiomyotomy (HD-ECM), the crus of the diaphragm was noted to be narrowed, severely fibrosed and attached to the lower esophagus which was covered by dense scar tissue suggestive of an old esophageal perforation secondary to GER esophagitis. A Nissen fundoplication was performed, but dysphagia persisted postoperatively, and a narrowing 2 cm above the fundoplication wrap with proximal dilatation was found on repeated barium studies. At re-laparotomy, the fundoplication was unwrapped and HD-ECM performed. Eight months postoperatively, she is well with no recurrence. EA must be considered in any DS patient presenting with dysphagia.
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PMID:Down's syndrome and esophageal achalasia: a rare but important clinical entity. 1626 71

When patients with the typical reflux symptoms of heartburn, regurgitation, or both, undergo endoscopy, up to 75% will not have endoscopic oesophagitis or evidence of Barrett's oesophagus. These patients have been described as having endoscopic negative or, more commonly, non-erosive reflux disease (NERD). Patients without oesophagitis, but with a positive pH test, can be diagnosed with gastro-oesophageal reflux disease (GERD). Some experts also consider a response to proton pump inhibitor therapy as proof of GERD in a patient with the correct symptoms and a negative endoscopy. Patients with normal acid exposure, but who report symptoms with a majority of their reflux episodes documented during an ambulatory pH study, have also been considered to have NERD, although others have labelled them as having 'functional heartburn'. Finally, there are some patients who have reflux symptoms and respond to reflux therapy, but have no demonstrable reflux by either endoscopy or ambulatory reflux testing. Whether these patients are part of the GERD spectrum or have another diagnosis is not clear. It seems that the most widely used definition of functional disease (the Rome II criteria) would include these patients as having functional heartburn, as it was defined as 'greater than or equal to 12 weeks of either continuous or intermittent symptoms of burning retrosternal discomfort or pain without pathologic GERD, achalasia, or other motility disorders with a recognized pathologic basis'. This article reviews potential differences in pathophysiology between erosive oesophagitis and NERD; explores whether symptoms can help distinguish NERD patients from erosive oesophagitis patients; and explores the evaluation and therapy of these patients.
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PMID:Review article: the role of acid suppression in patients with non-erosive reflux disease or functional heartburn. 1648 68

Achalasia is a motility disorder of the oesophagus of unknown origin in which loss of relaxation of the lower oesophageal sphincter (LOS) and aperistalsis in the distal oesophagus leads to functional oesophageal obstruction. The treatment is symptomatic, aimed at lowering of the LOS pressure, and may be accompanied by various side effects, including gastro-oesophageal reflux, a risk factor for oesophagitis and its complications. Stasis and fermentation can also lead to inflammation of the oesophageal mucosa, giving rise to hyperplasia of the epithelium, multifocal dysplasia and in some patients eventually squamous cell carcinoma. Unfortunately, the sensitivity and specificity of endoscopical inspection to assess inflammation or dysplasia of the oesophageal lining is low, such that biopsy sampling is necessary for accurate assessment. Although it is generally accepted that achalasia is a pre-malignant disorder, the reported increased risk of patients with achalasia developing a squamous cell carcinoma varies from 0 to 140 times that of the normal population. In addition, achalasia may predispose to Barrett's metaplasia and oesophageal adenocarcinoma, which have been described in case reports after myotomy. Surveillance endoscopy with tissue sampling to detect pre-neoplastic lesions has been recommended, even though this can be very difficult due to mucosal adherence of food as well as hyperplastic changes of the mucosa. In the event of moderate to severe inflammation and/or persisting stasis of food despite adequate LOS pressure-lowering therapy, the surveillance interval should be shortened and performed after a 3-day liquid diet. The exact technique and time intervals still need to be established, however.
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PMID:Long-term risk of oesophagitis, Barrett's oesophagus and oesophageal cancer in achalasia patients. 1678 16

A 58-year-old female with a recurrent history of upper abdominal pain and intermittent dysphagia underwent endoscopic evaluation that demonstrated an irregular and nodular esophago-gastric (EG) junction and grade I erosive esophagitis. Biopsies showed prominent intestinal metaplasia of Barrett's type without dysplasia, chronic inflammation and multiple aggregates of large cells within the mucosal lamina propria, some with spindle shaped nuclei. Immunohistochemistry stains for keratins AE-1/AE-3 were negative, while S-100 and NSE were positive. This, together with routine stains, was diagnostic for mucosal ganglioneuromatosis. The background of chronic inflammation with intestinal type metaplasia was consistent with long-term reflux esophagitis. No evidence of achalasia was seen. Biopsies of gastric antrum and fundus were unremarkable, without ganglioneural proliferation. Colonoscopy was unremarkable. No genetic syndromes were identified in the patient including familial adenomatous polyposis and multiple endocrine neoplasia type IIb (MEN IIb). Iansoprazole (Prevacid) was started by oral administration each day with partial relief of symptoms. Subsequent esophagogastroscopy repeated at 4 mo showed normal appearing EG junction. Esophageal manometry revealed a mild non-specific lower esophageal motility disorder. Mild motor dysfunction is seen with gastro-esophageal reflux disease (GERD) and we feel that the demonstration of localized ganglioneuromatosis was not likely related etiologically. In the absence of findings that might suggest neural hypertrophy, such as achalasia, the nodular mucosal irregularity seen with this instance of ganglioneuromatosis may, however, have exacerbated the patient's reflux.
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PMID:Sporadic ganglioneuromatosis of esophagogastric junction in a patient with gastro-esophageal reflux disorder and intestinal metaplasia. 1720 37

Multi-detector computed tomography (CT) offers new opportunities in the imaging of the gastrointestinal tract. Its ability to cover a large volume in a very short scan time, and in a single breath hold with thin collimation and isotropic voxels, allows the imaging of the entire esophagus with high-quality multiplanar reformation and 3D reconstruction. Proper distention of the esophagus and stomach (by oral administration of effervescent granules and water) and optimally timed administration of intravenous contrast material are required to detect and characterize disease. In contrast to endoscopy and double-contrast studies of the upper GI tract, CT provides information about both the esophageal wall and the extramural extent of disease. Preoperative staging of esophageal carcinoma appears to be the main indication for MDCT. In addition, MDCT allows detection of other esophageal malignancies, such as lymphoma and benign esophageal tumors, such as leiomyma. A diagnosis of rupture or fistula of the esophagus can be firmly established using MDCT. Furthermore, miscellaneous esophageal conditions, such as achalasia, esophagitis, diverticula, and varices, are incidental findings and can also be visualized with hydro-multi-detector CT. Multi-detector CT is a valuable tool for the evaluation of esophageal wall disease and serves as an adjunct to endoscopy.
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PMID:Dedicated multi-detector CT of the esophagus: spectrum of diseases. 1765 87

Dysphagia can be caused by a number of disorders such as benign or malignant obstruction of the esophagus, inflammatory alterations of the mucosa or primary esophageal motility disorders. Endoscopic evaluation is recommended for all patients to exclude malignancy and to establish or confirm a diagnosis. This article provides an overview of the most frequent inflammatory and functional esophageal disorders causing dysphagia. Clinical findings, diagnostic procedures and therapeutic management of primary esophageal motility disorders such as achalasia and diffuse esophageal spasm as well as of GERD and eosinophilic esophagitis are discussed. The diagnosis of achalasia is made by barium swallow with fluoroscopy and by manometry. Therapeutic options for achalasia are pneumatic dilatation of the esophagogastric junction, laparoscopic cardiomyotomy combined with fundoplication and botulinum toxin injection of the lower esophageal sphincter Diffuse esophageal spasm is manometrically characterized by normal peristalsis intermittently interrupted by simultaneous contractions. Potential medical therapies are PPIs for underlying GERD, smooth-muscle relaxants and antidepressant medications. GERD is a multifaceted disease caused by abnormal reflux of gastric contents into the esophagus leading to chronic symptoms or mucosal damage. Therapy includes lifestyle modifications, acid suppressive medications mainly by PPI and laparoscopic fundoplication in selected patients. Eosinophilic esophagitis is a chronic inflammatory disorder of the esophagus diagnosed histologically. The main symptom of eosinophilic esophagitis is dysphagia for solid food with imminent risk of food impaction. Systemic or topical corticosteroids are the therapy of choice.
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PMID:[Esophageal dysphagia]. 1766 9


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