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)

1. Esophageal achalasia is a rare disease of unknown origin that occurs with the same frequency in both sexes. 2. During the preoperative workup as well as the postoperative time the following studies should be performed: upper GI series, esophageal manometry and endoscopy with biopsy. 3. In most instances conservative treatment with dilations only provides temporal relief of the symptoms and moreover, is not exempt from complications; it should be employed only in incipient achalasia, in patients who refuse to be operated upon and in those whose general conditions make surgery inadvisable. 4. Surgery can provide a permanent cure for the symptomatology in the majority of the patients, with a very low mortality and morbidity; an operation should be performed early in those who retain more than 10 mls. in the esophagus with persistence of the symptomatology as well as in those patients in whom cancer is suspected. 5. A modified Heller type operation, with abdominal approach with gastrostomy, diaphragmatic hernioplasty and an anti-reflux procedure and when necessary a piloroplasty is the treatment of choice for esophageal achalasia yielding good results in 88.8% of the cases. It should be noted that the degree of postoperative gastroesophageal regurgitation is directly related to the extent to which the gastric incisons is extended below the esophageal-gastric junction.
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PMID:[Surgical treatment of achalasia. Analysis of 27 cases]. 71 47

Diverticula of the oesophagus are a rare disease with an interesting patho-physiology which is the basis for a correct indication and surgical procedure. Our experience bases on 72 operated cases with a long follow-up. The pharyngo-oesophageal or Zenker's diverticulum and the epiphrenic diverticulum are pulsion diverticula above an achalasic sphincter: the upper cervical and the LES. A surgical treatment is necessary in the most cases of Zenker's, in the epiphrenic form depending on symptoms and risk. The treatment of Zenker's is a cervicotomy with diverticulectomy (we use a stapler) and accurate myotomy of the cricopharyngeus. A beginning little D or a cervical achalasia without D are often the cause of serious and lasting symptoms. Here the alone cricomyotomy is the procedure of choice. Reflux disease is frequently associated with Zenker's, sometimes a surgical problem too. The operation of the epiphrenic D is a left thoracotomy with diverticulectomy and the very important abolition of the causal achalasia with myotomy and antireflux (by us a fundoplication). The traction diverticula of the thoracic oesophagus are frequently asymptomatic. Operation with right thoracotomy is exceptionally necessary on painful segmental dyskinesia of bezoar. It is essential on oesophago-respiratory fistula.
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PMID:[Surgical indication and technique in diverticulosis of the esophagus]. 184 11

The authors present the case of 36 years old patient with dysphagia. This symptom appeared three years ago and became more severe for six month. The endoscopic and radiologic examinations revealed achalasia of the cardia. The surgical treatment was performed. Two weeks after surgery all symptoms disappeared. We present this case because achalasia of the cardia is comparatively rare disease which diagnostic is not easy.
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PMID:[Difficulties in diagnosis of esophageal achalasia]. 764 13

Achalasia is a disorder of the esophagus characterized by loss of esophageal motility and elevated lower esophageal sphincter pressure. The etiology of this rare disease is unknown, and there is no cure. This article describes the disorder and the palliative treatments currently available. Nursing care of the individual with achalasia also is presented.
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PMID:Esophageal achalasia. 768 95

Esophageal achalasia (EA) is a rare disease in man and animals and there are many discussions on its higher risk of esophageal cancer. N-Amyl-N-methylnitrosamine (AMN) which specifically induces esophageal tumors in mice and rats was given to three mutant mouse strains, i.e. 101/N, STX/Le and BXH-8, which develop a high incidence of EA. The incidence of EA in 101/N, STX/Le, BXH-8 and normal C57BL/6J mice was 38.5% (110/286), 30.1% (43/143), 91.8% (190/207) and 0% (0/167), respectively. The average numbers of AMN-induced esophageal tumors in EA(+) were significantly higher than those of EA(-) in all of the 101/N, STX/Le and BXH-8 mice. Furthermore, significantly larger size tumors and invasive squamous cell carcinomas were found in EA(+) mice than in EA(-) mice. These results indicate the higher sensitivity of EA for both tumor induction and promotion, possibly due to the longer retention of AMN. In fact, relaxation of the lower esophagus by a smooth muscle relaxing calcium-channel blocker, nicardipine hydrochloride, significantly prevented the induction of esophageal tumors.
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PMID:High incidence of esophageal cancer in esophageal achalasia by the oral administration of N-amyl-N-methylnitrosamine and its prevention by nicardipine hydrochloride in mice. 961 58

Current concepts of esophageal motility disorders are summarized. Primary data sources were located via MEDLINE or cross-citation. No attempt was made to be comprehensive or inclusive of the literature because fewer than 10% of citations are discussed. Instead, emphasis was placed on new developments in diagnosis, therapeutics and practice patterns. Controlled therapeutic trials and pathophysiological observations are emphasized. Achalasia is a rare disease of failed lower sphincter relaxation and aperistalsis. Diffuse esophageal spasm (DES), an equally rare disease, is defined by nonpropagated esophageal contractions. Nonspecific motility disorders, including nutcracker esophagus and hypertensive lower esophageal sphincter, are identified only by manometry and are 10 times as prevalent. Neuromuscular pathology is evident only with achalasia (myenteric plexus neuron destruction). Pharmacological therapies have limited efficacy with achalasia, more limited efficacy with DES and no efficacy with the nonspecific motility disorders. More efficacious therapies for the nonspecific disorders are directed at associated reflux disease or psychiatric disorders. Pneumatic dilation is effective therapy for achalasia in 72% of instances but frequently requires repeat dilation and is complicated by a 3% perforation rate. Surgical myotomy is effective in 88% of patients with achalasia; morbidity from thoracotomy has been the major limitation but has been sharply reduced with a laparoscopic approach. In conclusion, although it has been suggested that esophageal motility disorders are distinct clinical entities, critical review of the literature supports this only in the case of achalasia, a disease of well defined pathophysiology, functional disturbance and therapies. This clarity diminishes progressively for DES and nonspecific esophageal motility disorders.
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PMID:Esophageal motility disorders: current concepts of pathogenesis and treatment. 1075 19

Swallowing is a complex mechanism based on the coordinated collaboration of tongue, pharynx and esophagus. Disturbances of this interplay or disorders of one or several of these components lead to dysphagia, non-cardiac chest pain or regurgitation. The major primary esophageal motility disorders--achalasia, diffuse esophageal spasm, hypercontractile esophagus ('nutcracker esophagus') and non-specific motility disorder--are of unknown etiology. Other esophageal diseases, such as cervical diverticula or gastroesophageal reflux disease, might also be caused by a primary esophageal motility disorder. Medical treatment of esophageal disorders with esophageal hyper- or dysmotility requires agents that reduce esophageal contractile force (anticholinergic agents, nitrates, calcium antagonists). Despite the beneficial effect of the various drugs on esophageal motility parameters, the clinical benefit of medical treatment of esophageal motility disorders is rather disappointing. Calcium channel antagonist, alone or in combination with anticholinergics or nitrates, can be used as a medical trial, especially in mild achalasia. However, medical therapy is clearly inferior to pneumatic balloon dilation therapy. Recently, botulinum toxin injection was suggested as a therapeutic option in achalasia patients with good results on lower esophageal sphincter pressure (LESP) and symptom scores that were similar to the results achieved by pneumatic balloon dilation. Hypercontractile esophagus shows a good manometric response to calcium channel antagonists, but only little clinical effect in terms of improvement of symptoms. Diffuse esophageal spasm is a relatively rare disease and few clinical studies are available. The use of calcium channel antagonists can be beneficial, at least in some patients with diffuse esophageal spasm. From clinical and epidemiological studies, there is some evidence of a 'psychological' component in the pathogenesis or perception of esophageal symptoms. There is some clinical benefit from centrally acting drugs such as benzodiazepines or antidepressants. With the exception of botulinum toxin for achalasia, medical therapy of primary esophageal motility disorders is rather limited and the clinical results are poor. Further understanding of esophageal pathophysiology as well as development of new receptor-selective drugs might increase our chances of a successful treatment of primary esophageal motility disorders.
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PMID:Esophageal pharmacology and treatment of primary motility disorders. 1077 Mar 58

Generalized leiomyomatosis of the esophagus and stomach is a rare disease. According to literature data, age of the patients ranges from 9 to 84 years, in men this disease is diagnosed 2 times more often than in women. It is necessary to differentiate this disease with cancer, esophageal diverticulum, achalasia of the cardia, hiatal hernia. In our case a woman of 32 years was ill from 5 years of age. Correct diagnosis was not made before surgery. Endoscopic examination suspected leiomyoma. Only repeated urgent histological tests during surgery resulted in accurate diagnosis (subtotal leiomyomatosis of the esophagus and proximal stomach) and in adequate surgical policy--subtotal resection of the esophagus and proximal resection of the stomach with Toprover gastrostoma creation. Then, retrosternal esophagocoloplasty was performed. 2 years after the first surgery the state of the patients is satisfactory, there are no symptoms of recurrence of the disease.
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PMID:[Subtotal leiomyomatosis of the esophagus and proximal part of the stomach]. 1292 38

Achalasia is a primary oesophageal motor disorder characterised by the abnormal relaxation of the lower oesophageal sphincter (LES) and absent oesophageal peristalsis. It is a rare disease, with an estimated incidence of approximately 1/100,000 and a prevalence close to 10/100,000 [1]. Its exact aetiology remains unknown. Autoimmune, infectious, degenerative and hereditary processes have all been proposed as factors that lead to a chronic inflammatory response in the myenteric plexus, thus resulting in selective loss of inhibitory neurons [2] and failure of the LES to relax and aperistalsis in the body of the oesophagus. The most common symptoms of achalasia are dysphagia for solids and liquids, regurgitation, chest pain, weight loss and heartburn in > 90 approximately 75, 40 - 50, approximately 60, approximately 40%, respectively [3,4]. The diagnosis is based on symptoms, barium swallow and manometry. A barium oesophagram typically shows a dilated oesophagus that tapers into a 'bird-beak' at the gastro-oesophageal junction with lack of normal peristalsis on fluoroscopic evaluation. The characteristic manometric features of achalasia are abnormal LES relaxation and aperistalsis; additionally, the LES pressure is frequently high, but can also be normal. Current practice of medicine is faced with rising healthcare costs and limited budgets [5]. We are therefore confronted with an increasing demand to justify the value of our therapeutic interventions, not only from the risk/benefit standpoint but also from the cost perspective [6,7].
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PMID:Cost-assessment of alternative management strategies for achalasia. 1459 55

Primary crycopharyngeal achalasia consists of a failure of the crycopharyngeus muscle to relax at the appropriate time during the swallowing process in the abscence of other motor abnormalities. The principle treatment options for cricopharyngeal achalasia include either dilatation of the upper esophagus or surgical myotomy of the cricopharyngeus muscle. We report a primary cricopharyngeal achalasia case, who was diagnosed early in 1st week of life and treated successfully by balloon dilatation, and discuss the treatment options for this very rare disease. The presented case is the first successfully treated newborn case during the neonatal period in the literature with respect to balloon dilatation.
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PMID:Primary cricopharyngeal achalasia in a newborn treated by balloon dilatation: a case report and review of the literature. 1697 10


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