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)

Dysphagia is a common reason for radiographic examination of the oesophagus to assess structural or functional abnormalities. From February 1, 1989 to August 28, 1993, six hundred and sixty eight patients had barium swallow examination. Out of 668 patients complaining of dysphagia, 173 patients had either histologically confirmed diagnoses and/or surgical diagnoses or oesophagoscopic diagnoses. The histological, oesophagoscopic and surgical diagnoses were: malignant tumours of the oesophagus 137 patients, achalasia of the cardia 21 patients, diverticula of the oesophagus six patients, peptic structures, five patients and non specific oesophagitis, four patients. Barium swallow agreed with 166 (96%) histological, oesophagoscopic and surgical diagnoses. The Kappa statistic was high (> .8). The mean age for patients with malignant tumours of the oesophagus was 53.5 years (range: 32-75 years), and for achalasia of the cardia was 36 years (range: 14-58 years). Patients with malignancy are in higher age group categories in comparison to the non-malignant patients (OR = 0.07 (0.02, 0.17). The mean duration of dysphagia for achalasia of the cardia was 8.5 years. The major cause of dysphagia was found to be malignant tumour of the oesophagus. Further study is recommended to determine the pattern of oesophageal pathologies to substantiate the finding.
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PMID:Relative sensitivity of barium swallow examination in the diagnosis of oesophageal pathology. 869 23

Achalasia is a motor disorder of the oesopagus characterized by decrease in ganglion cell density in Auerbach's plexus. The cause of the lesion is unknown. This is to repeat on the occurrence of autoimmune phenomena in patients with achalasia, in particular circulating antibodies against Auerbach's plexus and its possible meaning. IgG-antibodies against Auerbach's plexus were determined by standard indirect immunofluorescence. Antibodies to the cytoplasm of Auerbach's plexus were found in 37 of 58 patients with achalasia at variable stages of the disease (I-IV) with a disease duration ranging from 1 to 20 years but only in 4 out of 54 healthy controls (specificity 93%, sensitivity 64%, p < 0.0001), and in none of 12 patients with Hirschsprung's disease as well as 12 patients with cancer of oesophagus and only in one of 11 patients with peptic oesophagitis as well as in one of 13 patients with myasthenia gravis. The present observations suggest that autoimmunity to Auerbach's plexus plays a role in the pathogenesis of achalasia, the mechanism of action is unknown.
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PMID:Autoantibodies to Auerbach's plexus in achalasia. 874 84

Achalasia is a primary esophageal motor disorder characterized by lack of esophageal peristalsis and poor lower esophageal sphincter (LES) relaxation. Clinically, achalasia manifests as progressive dysphagia to solids and liquids and mild weight loss. Predisposition to esophageal cancer is not prevalent, but certain tumors may mimic achalasia. The diagnosis of achalasia is relatively easy to make with a good history, radiography, and esophageal motility testing. The esophagogram reveals a typical bird-beak narrowing of the esophagogastric junction and esophageal dilation, the degree of which depends on the stage of the disease. Esophageal manometry reveals poor LES relaxation, aperistalsis, and often elevated intraesophageal pressure. Endoscopic examination is important to rule out malignancy as the cause of achalasia. The traditional treatment of achalasia is forceful dilation of the LES. Bougienage may be helpful in some cases. Pharmacological agents, such as nitroglycerin and calcium channel blockers, provide some relief by decreasing LES pressure. However, they are not a viable, long-term choice. Surgical myotomy offers slightly better results than pneumatic dilation, but it is accompanied by some increased gastroesophageal reflux. Laparoscopic and thoroscopic myotomy are in their infancy, and, if successful, they will make surgical treatment much more attractive. Intrasphincteric botulinum toxin injection is the newest form of therapy. Its safety and ease of administration are very encouraging, but long-term results are not available.
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PMID:Achalasia. 877 90

Peritoneal mesothelioma has been increasing in frequency since the 1960s. Although still a rare malignant neoplasm, early diagnosis influences prognosis. More common presenting features include abdominal pain, abdominal distension or a palpable mass; more uncommon presentations have included dysphagia secondary to achalasia, chronic pancreatitis and regional lymphadenopathy. We report two recent cases at the Southern General Hospital in Glasgow.
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PMID:Peritoneal mesothelioma with gastric outlet obstruction. 887 13

Cancer of the oesophagus has great diversity in geographical distribution and incidence. The rate of oesophageal cancer has been increasing in some areas and the reasons for this are not clear. This review outlines fascinating epidemiological aspects and the risk factor for squamous cell carcinoma of the oesophagus. While in the Western world the effects of alcohol and tobacco are substantial preconditions, worldwide other factors, such as diet, nutritional deficiencies, environmental exposure and infectious agents (especially papillomavirus and fungi), play a significant role. Chronic irritation of the oesophagus appears to participate in the process of carcinogenesis, particularly in patients with thermal and/or mechanical injury, achalasia, oesophageal diverticulum, chronic lye stricture, radiation therapy, injection sclerotherapy and gastric resection before the appearance of oesophageal tumour. The association of Plummer-Vinson syndrome, coeliac disease, tylosis and scleroderma with oesophageal cancer has also been reviewed.
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PMID:Risk factors for squamous cell carcinoma of the oesophagus. 905 60

Two cases of a rare combination of conditions, achalasia and adenocarcinoma in Barrett's esophagus are reported. Cancer developed 26 years after the onset of gastroesophageal reflux in one and 30 years after esophagomyotomy in the other. Twenty-one cases of Barrett's esophagus and achalasia have now been reported; adenocarcinoma developed in six patients. Only one has survived more than five years after treatment. Long-term surveillance of patients with achalasia is recommended.
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PMID:Esophageal achalasia and adenocarcinoma in Barrett's esophagus: a report of two cases and a review of the literature. 907 76

Carcinosarcoma of the esophagus is a rare malignant neoplasm that consists of both carcinomatous and sarcomatous elements. The histogenesis of the sarcomatous component is generally considered to result from metaplasia of carcinomatous cells toward mesenchymal differentiation. True carcinosarcoma, characterized as a collision between a carcinoma and a sarcoma, is extremely rare. We describe a patient with primary achalasia who developed a true carcinosarcoma of the esophagus in which clonal differences between carcinomatous and sarcomatous elements were genetically and immunohistochemically demonstrated. A polypoid tumor located in the middle third of the esophagus developed in a 51-year-old man with longstanding achalasia. The tumor was predominantly composed of spindle-shaped sarcomatous cells. Squamous cell carcinoma in situ and islands of well-differentiated squamous cell carcinoma in the sarcomatous element were histologically observed. The sarcomatous element was immunoreactive for both mesenchymal and myoid markers. The carcinomatous component expressed type I and type II cytokeratins as well as epithelial membrane antigen. Analysis for chromosomal loss of heterozygosity performed in multiple microdissected samples of each sarcomatous and carcinomatous element revealed distinct genetic clonalities. These differences in immunohistochemical and genetic clonalities suggest that the tumor composed of squamous cell carcinoma and leiomyosarcoma originated separately from epithelial and mesenchymal precursors.
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PMID:Esophageal carcinosarcoma: a genetic analysis. 928 91

Cancer of the oesophagus is a challenging clinical problem. Overall survival is poor, but patients who present early are eminently curable. Most cancers of the middle and upper oesophagus are squamous cell carcinoma. Adenocarcinoma is the most common cancer of the third of the oesophagus; this is not surprising when the usual distribution of Barrett's mucosa is considered. The geographical variation in the prevalence of oesophagus cancer is important. In most parts of the world, alcohol consumption and tobacco usage are the principal risk factors. Other risk factors have been identified in "the high-risk areas": a diet high in nitrosamines, deficient in trace elements, in vitamins (C.A, E) and the hereditary conditions like: Barrett's oesophagus, achalasia, caustic strictures.
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PMID:[The etiopathogenesis of esophageal cancer]. 945 31

Type 1 antineuronal nuclear autoantibody (ANNA-1, also known as "anti-Hu") is a marker of neurologic autoimmunity that is highly associated with small-cell lung carcinoma (SCLC). To determine the spectrum of symptoms and signs as well as the frequency of cancer in adult patients who are seropositive for ANNA-1, we reviewed 162 sequential patients (67% female) identified as ANNA-1-positive in a comprehensive immunofluorescence screening test. In 21% of these patients, the antibody test requested by the physician was not ANNA-1. By the end of the follow-up period, cancer had been found in 142 patients (88%). Ten of these lacked evidence of SCLC (4 had prostate carcinoma, 3 breast carcinoma, 1 both prostate carcinoma and melanoma, 1 lymphoma, and 1 squamous-cell lung carcinoma). Of the 132 patients (81%) with proven SCLC, 17 had one or more coexisting malignant neoplasms (6 had renal carcinoma, 4 another lung primary carcinoma, 3 prostate carcinoma, 3 breast carcinoma, and 4 assorted neoplasms). The diagnosis of SCLC in 128 patients (97%) followed the onset of paraneoplastic symptoms. SCLC was identified in 10 patients by chest MRI after an equivocal chest radiograph or CT; in 28 by bronchoscopy, mediastinoscopy, or thoracotomy; and in 7 at autopsy. Neurologic signs in decreasing frequency were neuropathy (sensory > mixed somatic > autonomic > cranial [especially cranial nerve VIII] > motor), cerebellar ataxia, limbic encephalitis, polyradiculopathy, associated Lambert-Eaton myasthenic syndrome, myopathy, myelopathy, opsoclonus/myoclonus, motor neuronopathy, brachial plexopathy, and aphasia. Nineteen patients had a solely gastrointestinal initial presentation, including gastroparesis, pseudo-obstruction, esophageal achalasia, or other dysmotility. We conclude that seropositivity for ANNA-1 can expedite the diagnosis and treatment of otherwise occult cancer in patients, especially tobacco abusers, with varied neurologic and gastroenterologic presentations. The search for SCLC should not end on discovering a different neoplasm.
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PMID:Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. 952 Dec 51

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.
Cancer Lett 1998 May 15
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


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