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)

It has been reported that patients who refuse blood transfusions, such as Jehovah's witnesses, can undergo major surgery. In a review of the literature, however, we critically examined the severity of anaemia in relation to operative mortality and morbidity rates. We report three cases of Jehovah's witnesses who underwent major surgery and presented complication during the postoperative period. Case 1: a 50-year-old man with oesophageal achalasia who underwent Heller's myotomy and Nissen's fundoplication. The postoperative period was complicated by massive haemorrhage and the patient was reoperated on postoperative day 1. After four years, he underwent total oesophagectomy because of severe chronic oesophagitis. On postoperative day 13 the patient suffered anteroseptal myocardial ischaemia, which was treated with medical therapy. Case 2: a 40-year-old man, admitted for ulcerative rectocolitis, who underwent total colectomy. On postoperative day 1 he presented massive haemorrhage and shock. He was reoperated and the postoperative period was complicated by myocardial ischaemia, renal failure and an enterocutaneous fistula. Case 3: a 65-year-old woman with ulcerative rectocolitis who underwent total colectomy and a temporary ileostomy. She suffered venous thrombosis of the lower limbs and pulmonary oedema. The patient died 14 months after surgery as a result of massive haemolysis by cryoagglutinins and cardiac arrest.
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PMID:[Severity of anemia and operative morbidity and mortality. Report on 3 clinical cases in Jehovah's Witnesses and review of the literature]. 1194 8

Chagas' disease causes degeneration and reduction of the number of intrinsic neurons of the esophageal myenteric plexus, with consequent absent or partial lower esophageal sphincter relaxation and loss of peristalsis in the esophageal body. The impairment of esophageal motility is seen mainly in the distal smooth muscle region. There is no study about esophageal striated muscle contractions in the disease. In 81 patients with heartburn (44 with esophagitis) taken as controls, 51 patients with Chagas' disease (21 with esophageal dilatation) and 18 patients with idiopathic achalasia (11 with esophageal dilatation) we studied the amplitude, duration and area under the curve of esophageal proximal contractions. Using the manometric method and a continuous perfusion system we measured the esophageal striated muscle contractions 2 to 3 cm below the upper esophageal sphincter after swallows of a 5-ml bolus of water. There was no significant difference in striated muscle contractions between patients with heartburn and esophagitis and patients with heartburn without esophagitis. There was also no significant difference between patients with heartburn younger or older than 50 years or between men and women or in esophageal striated muscle contractions between patients with heartburn and Chagas' disease. The esophageal proximal amplitude of contractions was lower in patients with idiopathic achalasia than in patients with heartburn. In patients with Chagas' disease there was no significant difference between patients with esophageal dilatation and patients with normal esophageal diameter. Esophageal striated muscle contractions in patients with Chagas' disease have the same amplitude and duration as seen in patients with heartburn. Patients with idiopathic achalasia have a lower amplitude of contraction than patients with heartburn.
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PMID:Esophageal striated muscle contractions in patients with Chagas' disease and idiopathic achalasia. 1204 32

Benign esophageal lesions occur in various diseases. Barium studies are useful for the evaluation of mucosal surface lesions but provide little information about the extramucosal extent of disease. Computed tomography and magnetic resonance imaging, on the other hand, permit the assessment of wall thickness, mediastinal involvement, adjacent lymphadenopathy, and distant spread. In diseases such as fibrovascular polyps, duplication cysts, scleroderma, trauma, caustic esophagitis, hiatal hernia, esophageal diverticulum, achalasia, and paraesophageal varices, the findings of imaging studies are specific, obviating the need for further invasive diagnostic work-up. The advent of helical computed tomography and its volume data set allows the acquisition of multiplanar images, and magnetic resonance imaging is useful both for this and for tissue characterization. Thus, multiplanar cross-sectional imaging further extends the role of imaging modalities to the evaluation of benign esophageal lesions. Through an awareness of the multiplanar cross-sectional appearances of various benign esophageal lesions, the radiologist can play an important role in the detection, diagnosis, further diagnostic planning, and treatment of the diseases in which they occur.
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PMID:The spectrum of benign esophageal lesions: imaging findings. 1227 Nov 66

Dysphagia is a rare manifestation in a patient with Crohn's disease. We report on the case of a patient with long-standing Crohn's disease who developed progressive dysphagia over 3 years. Endoscopy showed minimal distal oesophagitis with non-specific histological findings. Further investigation with cinematography, barium swallow and manometry established an achalasia-like motility disorder. Biopsies obtained from the oesophagus were non-specific. Balloon dilatation was performed. Initial success was followed by recurrent dysphagia. At repeat endoscopy, an oesophageal fistula was detected. An attempt at conservative medical management failed and oesophagectomy was successfully performed. Pathology results of the resected specimen confirmed the suspected diagnosis of oesophageal Crohn's disease. Even if achalasia is suspected in a Crohn's patient, it should be taken into consideration that the motility disorder could be the result of a transmural inflammation with or without fibrosis caused by Crohn's disease.
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PMID:Dysphagia in Crohn's disease: a diagnostic challenge. 1240 54

Both open and laparoscopic myotomies have been used in the treatment of achalasia. Postoperative gastro-oesophageal reflux is among the commonly reported side effects of myotomy. The addition of an antireflux procedure to the standard surgical approach has given rise to controversy. The objective of our study was to determine whether or not an antireflux procedure should be used in addition to Heller myotomy. Over the period from 1980 to 1990, 94 patients (mean age: 47.9 years) with achalasia underwent Heller myotomy calibrated by intraoperative oesophageal manometry without fundoplication. In 1999-2000, all patients filled in a clinical questionnaire: all underwent radiographic oesophageal imaging, oesophageal manometry, ambulatory 24-h oesophageal pH monitoring, and oesophagogastroduodenoscopy, when necessary. Ten healthy age-matched subjects were compared in the manometric and radiological studies. Myotomy improved the clinical profiles and instrumental data results in all patients. Gastro-oesophageal reflux was present in 10 patients (10.6%); none of these 10 subjects presented oesophagitis. Heller open myotomy yields good long-term results. Intraoperative manometric calibration reduces the side effects of myotomy, such as gastro-oesophageal reflux. The addition of fundoplication is not justified in all patients.
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PMID:Long-term results of Heller myotomy without an antireflux procedure in achalasic patients. 1246 52

Achalasia is a disease of unknown origin in which there is a denervation of the myenteric plexus on the smooth muscle of the lower oesophageal sphincter, causing a cardial stenosis and a loss of efficacy of oesophageal peristalsis. The predominant symptoms are dysphagia for solids and liquids and regurgitation of the retained food. Occasionally, there may be oesophageal haemorrhage as a consequence of oesophagitis and stasis ulcers. An important but uncommon complication is the development of oesophageal cancer, which is typically squamous cell carcinoma. We report an exceptional case of a 77-year-old woman with a long-term achalasia and mega-oesophagus who presented four episodes of upper gastrointestinal bleeding in a 2 month period. The patient underwent surgical resection of the 10 cm of distal oesophagus, performing a partial fundoplication, and the pathological study revealed an oesophageal infiltration by a low-grade non-Hodgkin's lymphoma. After an insidious outcome, she died on the 47th day after admission.
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PMID:Relapsing upper bleeding in non-Hodgkin's oesophageal lymphoma associated with achalasia. 1450 23

We studied the premalignant nature of achalasia using anti-Ki-67 and anti-p53 monoclonal antibodies immunohistochemically. In this study, four patients with esophageal carcinoma and achalasia were investigated. Three tumors were pT4 (UICC pTNM) and one tumor was pT1. The majority of non-malignant esophageal epithelium showed esophagitis and/or dysplasia histologically. Esophageal epithelial cells in the lesions of esophagitis and/or dysplasia had a higher number of Ki-67-positive cells than normal epithelial cells. p53 protein was expressed in two tumors and it was not expressed in non-malignant epithelium. From these results, we found that esophageal epithelium in achalasia lesions is changed to varying degrees of esophagitis and/or dysplasia by stagnation of intake foods, and these abnormal epithelial cells showed a high proliferative state compared with the normal cells without the p53 gene mutation. We suggest that the distinct proliferative status is a cause of carcinogenesis.
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PMID:Histopathological analysis of non-malignant and malignant epithelium in achalasia of the esophagus. 1460

Heller's esophagomyotomy relieves dysphagia but does not restore esophageal peristalsis. The myotomy may induce reflux and the addition of a 360 degrees fundoplication may be hazardous with regard to the remaining aperistaltic esophagus. The aim of this prospectively randomized clinical trial was to compare the outcome for patients with uncomplicated achalasia who underwent an anterior Heller's esophagomyotomy (H group) with or without an additional floppy Nissen fundoplication (H + N group). Between 1984 and 1995, 20 patients were prospectively randomized to one or other of the performed operations, 10 patients per group. Esophagitis including Barrett's esophagus (n = 2) was seen under medical treatment, in 6 of 9 in the H group but none in the H + N group. No patient in the H + N group required postoperative continuous acid-reducing drugs. Twenty-four-hour esophageal pH-studies in median 3.4 years after surgery showed pathological reflux expressed as a percentage of time below pH 4 of 13.1% in the H group compared to 0.15% (P < 0.001) in H + N group. One patient with recurrent dysphagia in the H + N group later had an esophagectomy. The remaining patients reported significant improvement of dysphagia without symptoms of reflux at 8.0 years follow-up. Heller's esophagomyotomy eliminates dysphagia, but can induce advanced reflux that requires medical treatment. The addition of a 360 degrees fundoplication eliminates reflux without adding dysphagia in the majority of patients and can be recommended for most patients with uncomplicated achalasia.
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PMID:Heller's esophagomyotomy with or without a 360 degrees floppy Nissen fundoplication for achalasia. Long-term results from a prospective randomized study. 1464 Dec 90

A 72 year-old man presented severe dysphagia and weight loss of recent onset. Repeated oesophageal endoscopy and biopsies with macroforceps were normal. Oesophageal manometry disclosed features compatible with achalasia. Oesophageal EUS endoscopy localized an infiltrating process between muscular layers of the oesophageal wall and CT scan delimited a circular thickening in the inferior part of the oesophagus. Because of severe clinical presentation mimicking a possible oesophageal neoplasm like a lymphoma, partial oesophagectomy was performed and revealed eosinophilic oesophagitis. This unusual presentation emphasizes that idiopathic eosinophilic oesophagitis must be proposed in the differential diagnosis of dysphagia, even in old patient without apparent oesophageal lesion at endoscopy.
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PMID:Idiopathic eosinophilic oesophagitis: atypical presentation of a rare disease. 1528 82

Endoscopy-negative reflux disease is used to describe a heterogeneous group of disorders with symptoms that mimic those of gastroesophageal reflux disease in the absence of visible esophageal injury at endoscopy. Compared with patients who have gastroesophageal reflux-related erosive esophagitis, those with endoscopy-negative disease are more likely to be younger, female, of lower body weight, and without a hiatal hernia. Approximately 50% of those with endoscopy-negative reflux have abnormal intraesophageal acid exposure and are considered to have nonerosive acid reflux disease. Those with symptoms of >12 consecutive or intermittent weeks' duration during the prior year, with normal acid exposure and without achalasia or other motility disorder with a recognized pathologic basis, are considered to have functional heartburn. In the absence of pathologic reflux, a number of etiologies may contribute to the symptoms of heartburn, including motor events, reflux of nonacidic gastric contents, minute changes in intraesophageal pH (pH <4), visceral hypersensitivity, and emotional or psychological abnormalities. Although persons with endoscopy-negative reflux disease experience decrements in their quality of life that are similar to those for individuals with erosive esophagitis, the response to traditional therapies for acid reflux may differ between the 2 groups. Studies have found that approximately 50% of patients with endoscopy-negative reflux disease experience complete symptom relief after 4 weeks of proton pump inhibitor treatment. In those with persistent heartburn symptoms, other structural or nonacid reflux etiologies for their symptoms should be explored.
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PMID:Endoscopy-negative reflux disease: concepts and clinical practice. 1547 51


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