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)

We describe a patient who developed an ataxic sensory syndrome associated with xerophthalmia and progressive dysphagia with regurgitation. Electrophysiological findings were consistent with an axonal sensory neuropathy, and superficial peroneal nerve biopsy showed a reduction in number of myelinated fibers with epineurial inflammation. Rheumatoid factor, anti-SSA/SSB and antinuclear antibodies were positive and a diagnosis of Sjogren's syndrome was made. An endoscopic investigation revealed esophageal achalasia. We suggest that there may be a common autoimmune mechanism directed to different targets on the basis of this rare association.
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PMID:Sensory ataxic neuropathy and esophageal achalasia in a patient with Sjogren's syndrome. 1722 76

Achalasia is an incurable neuromuscular disorder of the esophagus, resulting from destruction of the esophageal myenteric plexus. This leads to aperistalsis and failure of the lower esophageal sphincter to relax after swallowing. Symptoms of achalasia are gradual in onset and include dysphagia, regurgitation, and weight loss. Severe malnutrition can ensue. Wernicke's encephalopathy (WE) is a serious, potentially fatal, neurologic disorder caused by thiamine deficiency (vitamin B(1)), classically described as presenting with a triad of ocular abnormalities, ataxia, and confusion. The incidence is uncertain, and many cases likely go unrecognized. It is usually diagnosed in the alcoholic population. We describe its onset after the successful surgical treatment of achalasia.
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PMID:Wernicke's encephalopathy after laparoscopic cardiomyotomy for achalasia. 1759 43

Achalasia is a rare motor disorder of the oesophagus, characterised by the absence of peristalsis and impaired swallow-induced relaxation. These motor abnormalities result in stasis of ingested food in the oesophagus, leading to clinical symptoms, such as dysphagia, regurgitation of food, retrosternal pain and weight loss. Although it is well demonstrated that loss of myenteric oesophageal neurons is the underlying problem, it still remains unclear why these neurons are preferentially attacked and destroyed by the immune system. This limited insight into pathophysiology explains the fact that treatment is limited to interventions aimed at reducing the pressure of the lower oesophageal sphincter. The most successful therapies are clearly pneumatic dilatation and Heller myotomy with short-term success rates of 70-90%, declining to 50-65% after more than 15 years. The challenge for the coming years will undoubtedly be to get more insight into the underlying disease mechanisms and to develop a treatment to restore function.
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PMID:Achalasia. 1764 3

Idiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition. It is a rare disease with an annual incidence of approximately 1/100,000 and a prevalence rate of 1/10,000. The disease can occur at any age, with a similar rate in men and women, but is usually diagnosed between 25 and 60 years. It is characterized predominantly by dysphagia to solids and liquids, bland regurgitation, and chest pain. Weight loss (usually between 5 to 10 kg) is present in most but not in all patients. Heartburn occurs in 27%-42% of achalasia patients. Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. The diagnosis is based on history of the disease, radiography (barium esophagogram), and esophageal motility testing (esophageal manometry). Endoscopic examination is important to rule out malignancy as the cause of achalasia. Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents) aimed at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Although it cannot be permanently cured, excellent palliation is available in over 90% of patients.
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PMID:Idiopathic (primary) achalasia. 1789 99

We aimed to evaluate the clinical outcomes of patients undergoing laparoscopic esophageal myotomy for achalasia and identify the factors that might predict postoperative dysphagia or symptomatic reflux. A retrospective analysis of all patients undergoing laparoscopic Heller myotomy from January 1997 to June 2004 was performed. Postoperative frequency and severity of reflux, dysphagia, chest pain, and regurgitation were evaluated using a standardized telephone interview. Forty-eight males and 53 females, with an average age of 45 years, underwent laparoscopic Heller myotomy during the study period. Prior to presentation, 65% of patients had undergone pneumatic dilatation (52%) and/or Botox injection (28%). The mean lower esophageal sphincter pressure was 44 mmHg. A Toupet fundoplication was performed in 89 patients, and 12 patients had no fundoplication. There were no intraoperative complications and 10 minor postoperative complications. During an average follow-up of 34 months (range 2-90), 15% of patients had a weekly dysphagia, and 16% had subjective reflux. Only an older age predicted higher incidence of postoperative dysphagia. No factors were identified to predict postoperative symptomatic reflux. Eighty-one percent of patients rated their outcome as excellent, 14% good, 4% fair, and 1% poor. Ninety-nine percent of patients would choose surgery over other treatment options again. Laparoscopic anterior esophageal myotomy is a safe and effective treatment for achalasia. Improvement in dysphagia can be expected in more than 95% of patients. Younger patients tended to have better improvement of dysphagia. Predicting the patients at higher risk for postoperative reflux remains elusive at this time.
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PMID:Laparoscopic Heller myotomy for achalasia in 101 patients: can successful symptomatic outcomes be predicted? 1792 16

This study reports the usefulness and safety of endoscope-guided pneumatic dilatation (PD) technique without using fluoroscopy. From January 1998 to June 2004, a total of 33 patients with achalasia, including 20 males and 13 females, received PD in our unit. The mean age was 48.5+/-17.5 years (range: [corrected] 18 to 93 y). All patients underwent endoscopic-guided PD by using a 3.0 cm [corrected] diameter "Regiflex" balloon dilator (Microvasive, Watertown, MA). The results of PD were recorded and evaluated by symptom scores based on the frequency of attack of 3 major symptoms: dysphagia, regurgitation, and chest pain, both before, and then 6 weeks, 6 months, 1 year after PD was performed. Thirty-three patients were treated, excellent results in 27, good results in 3, and failure in 3 (1 requiring surgical treatment later). Overall, we demonstrated that the technique is effective and safe option for achalasia treatment.
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PMID:Endoscope-guided pneumatic dilatation of esophageal achalasia without fluoroscopy is another safe and effective treatment option: a report of Taiwan. 1828 75

Achalasia is a primary motor disorder of the esophagus characterized by an abnormal hypertensive, nonrelaxing lower esophageal sphincter (LES) and nonfunctioning, aperistaltic esophageal body resulting in significant regurgitation and dysphagia. The primary goal of treatment is palliation of symptoms. At present, all treatment techniques are directed at relieving the functional obstruction at the level of the LES by disruption or paralysis of the esophageal muscle constituting the LES. Destruction of the LES function also places the patient at risk for pathologic gastroesophageal reflux disease. Therefore, the treatment of patients with achalasia must strike a balance between the relief of dysphagia and potential creation of pathologic gastroesophageal reflux. The advent of laparoscopic esophageal myotomy for the treatment of achalasia over the past decade has resulted in most patients with the disease being referred to surgeons for definitive treatment. At the time of consultation the patient may present with a myriad of symptoms, investigative results, and previous treatments. Based on our experience of over 300 patients treated with surgery at our institution between 1990 and 2007, this review will address the practical problems encountered in the surgical management of achalasia.
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PMID:Practical approaches to the surgical management of achalasia. 1830 56

Megaoesophagus resulting from achalasia is a rare but serious cause of airway obstruction. The exact aetiology remains unclear. Achalasia normally presents as weight loss, dysphasia and regurgitation but frequently considerable oesophageal distension can occur without complain and very rarely cause of achalasia presents with stridor or respiratory distress. The authors presents a 19 -year old young lady who had respiratory symptoms and had been treated as a cause of chronic asthma, was found by us to have oesophagus achalasia. She had complained of cough, dispneia and had a pulmonary function studies that showed a severe airway obstruction. After surgery the symptoms disappeared and she had a marked improvement in the flow-volume curve. Oesophagus achalasia should be considered as one of the differential diagnoses of airway obstruction.
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PMID:[Oesophagus achalasia: differencial diagnosis of asthma]. 1836 27

The aim of surgical treatment in achalasia cardia is symptom relief. Most studies have evaluated the results of laparoscopic cardiomyotomy with an antireflux procedure. However, data on the effectiveness of laparoscopic cardiomyotomy without an antireflux procedure is sparse. We describe our experience of laparoscopic cardiomyotomy without antireflux procedure in 40 consecutive patients with respect to symptom relief and complications. There was no mortality and 1 conversion. Preoperatively dysphagia, regurgitation, and heartburn were present in 40, 39, and 11 patients. At a mean follow-up of 26 months, there was a significant improvement in symptom scores. Two patients (5%) had persistent postoperative dysphagia. One improved on conservative therapy, whereas other was treated with relaparoscopic cardiomyotomy. Three patients (7.5%) developed heartburn in the postoperative period, which was well controlled with proton pump inhibitors. Laparoscopic cardiomyotomy without antireflux procedure results in excellent relief of dysphagia without producing significant symptomatic reflux in the follow-up.
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PMID:Symptomatic outcome of laparoscopic cardiomyotomy without an antireflux procedure: experience in initial 40 cases. 1842 30

Achalasia is a rare motor disorder of the esophagus, characterized by the absence of peristalsis and impaired swallow-induced relaxation. These motor abnormalities result in stasis of ingested food in the esophagus, leading to clinical symptoms, such as dysphagia, regurgitation of food, retrosternal pain and weight loss. Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. In terms of diagnosis, esophageal manometry is the gold standard to diagnose achalasia. Still, its role in post-treatment surveillance remains controversial. Radiological studies support the initial diagnosis of achalasia and have been proposed for detecting preclinical symptomatic recurrence. Although endoscopy is considered to have a poor sensitivity and specificity in the diagnosis of achalasia, it has an important role in ruling out secondary causes of achalasia. Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents) aimed at reducing the lower esophageal sphincter (LES) pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids.
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PMID:Achalasia. 1861 76


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