Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic sclerosis (SSc) is a multisystem disease of unknown etiology. Esophageal involvement affects 50-90% of patients and is characterized by abnormal motility and hypotonic lower esophageal sphincter. Data on the association of esophageal abnormalities and age, gender, SSc subset or duration, autoantibody profile, esophageal symptoms, and medication are lacking or conflicting. The aim of this study was the evaluation of these associations in Brazilian sclerodermic patients from the Rheumatology Division, Clinics Hospital, Federal University, Minas Gerais. They underwent medical records review, clinical interview, and esophageal manometry. The normal cutoff level for lower esophageal sphincter pressure was 14 mmHg. Abnormal peristalsis occurred when less than 80% of peristaltic waves were propagated. P-values less than 0.05 were considered significant. Twenty-eight patients were included: 71% were women. The population presented medium age and disease duration of 46 years and 12 years, respectively. Cutaneous diffuse SSc occurred in 39% and its limited form in 61%. Dysphagia, pyrosis, and regurgitation occurred, respectively, in 71%, 43%, and 61% of patients. Lower esophageal sphincter pressure and number of peristaltic waves-propagated medias were, respectively, 17.2 mmHg and 2.3. SSc-related manometric abnormalities were present in 86% of patients. Manometry revealed distal esophageal body hypomotility, hypotonic lower esophageal sphincter, or both, respectively, in 82%, 39%, and 36% of patients. One patient presented the manometric pattern of esophageal achalasia. Male patients more frequently presented hypotonic inferior esophageal sphincter. Manometric findings have had no relationship with the other variables. Nifedipine use did not influence manometric findings.
Dis Esophagus 2009
PMID:Esophageal manometry in 28 systemic sclerosis Brazilian patients: findings and correlations. 1966 79

Limited access to esophageal manometry (EM) may delay identification and treatment of patients with achalasia. In order to assess predictors to fast-track patients for manometric confirmation of achalasia, we compared the clinical, radiographic, and endoscopic characteristics of achalasia patients to patients with functional dysphagia without manometric features of achalasia (controls). Patients referred for esophageal manometry to assess functional dysphagia prospectively identified over a 12-month period were asked to participate in this study. The Achalasia Symptom Questionnaire (ASQ), a structured 11-question survey (score: 0-best, 67-worst), was completed by all consenting patients. ASQ scores, esophago-gastro-duodenoscopy and upper gastro-intestinal (UGI) contrast study findings were compared between patents with subsequently confirmed achalasia and those in whom achalasia was excluded by EM. Univariate logistic regression identified predictors that were tested by multivariate logistic regression to generate the model. Of the 803 EM performed over this 12-month period, 95 patients were referred specifically to assess functional dysphagia. Of these, 50 were confirmed to have achalasia, and 45 had dysphagia without manometric evidence for achalasia and hence comprised the control group. ASQ scores were higher in achalasia patients (37+/-13 versus 23+/-10). Endoscopy and/or contrast esophagogram reports were available in 92% achalasia patients and 80% controls. Significant predictors for achalasia identified on univariate logistic regression included ASQ score, abnormal findings on endoscopy, and contrast UGI study. Using multivariate logistic regression, we were able to accurately predict the probability of achalasia to be P where P=ey/(1+ey) and y=5.6+(0.089xASQ)+(2.088xEGD)+(3.083xUGI), e=exponential constant 2.7182, esophagogastroduodenoscopy (EGD) and UGI=0 if normal and 1 if abnormal. Dropping the predictor ASQ, the formula changes to y=-2.7+(1.987xEGD)+(2.861xUGI). Using only noninvasive investigations (i.e. eliminating EGD), the formula changes to y=-4.9653+(0.0951xASQ)+(3.4312xUGI). The probability of achalasia can be calculated in patients with functional dysphagia based on clinical, endoscopic, and radiographic findings allowing for a prioritization of EM studies.
Dis Esophagus 2010 Jan
PMID:Clinical predictors of achalasia. 1973 27

Injection of botulinum toxin (BT) and pneumatic dilatation are available methods in nonsurgical treatment of achalasia. Authors anticipate beneficial effect of prior BT injection on the success of pneumatic dilatation and duration of its effect. There are no long-term data available to assess efficacy of combined treatment. From 1998 to 2007, 51 consecutive patients (20 men and 31 women, age 24-83) with achalasia were included and prospectively followed up. Each patient received injection of 200 IU of BT into the lower esophageal sphincter (LES) during endoscopy and 8 days later pneumatic dilatation (PD) under X-ray control was performed. The follow-up was established every 3 months first year and then annually. The efficacy was evaluated by a questionnaire concerning patient's symptoms and manometry. Results were compared with 40 historical controls (16 men and 24 women, age 26-80) treated by PD alone using the same method and follow-up. Fifty-one patients underwent combined treatment. Four patients failed in follow-up and were not included for analysis. The mean duration of follow-up was 48 months with range 12-96 months. Thirty-four of forty-seven (72%) patients were satisfied with results with none or very rare and mild troubles at the time of the last visit. Forty-one patients were followed up more than 2 years. Effect of therapy lasted in 75% (31/41) of them. In 17 patients, more than 5 years after treatment, effect lasted in 12 (70%). Mean tonus of LES before therapy was 29 mm Hg (10-80), 3 months after therapy decreased to 14 mmHg (5-26). The cumulative 5 years remission rate (+/-95% CI) in combined treated patients 69% +/- 8% was higher than in controls 50% +/- 9%; however it, was not statistically significant (P= 0.07). In control group 1, case of perforation (2.5%) occurred. Eight patients (17%) with relapse of dysphagia were referred to laparoscopic Heller myotomy with no surgical complication. The main adverse effect was heartburn that appeared in 17 patients (36%). Initial injection of BT followed by PD seems to be effective for long-term results with fewer complications. But the combined therapy is not significantly superior to PD alone.
Dis Esophagus 2010 Feb
PMID:Combined treatment of achalasia - botulinum toxin injection followed by pneumatic dilatation: long-term results. 1973 28

The objectives of this study were to define the utility of esophagogastroduodenoscopy in the diagnosis and management of patients presenting with dysphagia and to determine the relative incidence of the various causes of dysphagia in Sudan. This is a prospective, cross-sectional, descriptive, hospital-based study carried out at the endoscopy unit of Soba University Hospital, Khartoum, Sudan. All patients complaining of dysphagia underwent upper gastrointestinal endoscopy with therapeutic intervention when necessary. A total of 114 patients were enrolled in the study, with a mean age of 47 years SD +/- 19 and a male to female ratio of 1 : 1.04. A benign condition was diagnosed in 56% of the cases; this included esophageal strictures in 21% of the cases and achalasia in 14%. Malignant causes were mainly due to esophageal cancer (40.4%) and cancer of the stomach cardia (3.5%). Therapeutic intervention was attempted in 83% of the cases. Risk factors predictive of a malignant etiology were age over 40 years (P < 0.000), dysphagia lasting between 1 month and 1 year (P < 0.000), and weight loss (P < 0.000). A barium study was performed in 35 cases (31%) prior to endoscopic examination and proved to be inaccurate in three cases (8.6%). Upper gastrointestinal endoscopy in our African setting is an accurate and useful investigation in the diagnosis and management of patients presenting with dysphagia. Patients over the age of 40 years presenting with dysphagia and weight loss are more likely to have a neoplastic disease and should be referred for urgent endoscopy.
Dis Esophagus 2010 Apr
PMID:Use of endoscopy in diagnosis and management of patients with dysphagia in an African setting. 1990 93

Laparoscopic anterior cardiomyotomy in addition to anterior Dor's fundoplication is the procedure of choice for achalasia of the esophagus with approximately 95% success rate. Redo cardiomyotomy is complicated and associated with rerecurrence of dysphagia. Twelve patients with failed redo myotomy were clinically evaluated with radiology, endoscopy, and manometry in whom achalasia type III or IV was confirmed. We propose as treatment for these selected cases an inversed Y cardioplasty + truncal vagotomy, a partial distal gastrectomy and Roux-en-Y gastrojejunostomy in order to facilitate esophageal emptying and avoid the appearance of postoperative gastroesophageal reflux as a side effect of this procedure. One patient was reoperated on in order to enlarge the cardioplasty. Disappearance of dysphagia was confirmed in all patients. Three patients presented reflux symptoms and were treated with 20 mg of Omeprazole 20 twice/day. No food retention, erosive esophagitis, or Barrett's esophagus were observed. The mean resting pressure decreased from 24.9 +/- 8.5 mm Hg to 7.5 +/- 2.5 mm Hg (P = 0.0001). Furthermore, esophageal diameter decreased significantly after a 5-year follow-up. This procedure could be an option for treating patients in which repeated Heller operations have failed.
Dis Esophagus 2010 Apr
PMID:Inversed Y cardioplasty plus a truncal vagotomy-antrectomy and a Roux-en-Y gastrojejunostomy performed in patients with stricture of the esophagogastric junction after a failed cardiomyotomy or endoscopic procedure in patients with achalasia of the esophagus. 1990 94

The aim of the present study was to evaluate the long-term outcomes of laparoscopic Heller myotomy with Dor fundoplication (LHD) and its effect on chest pain. Between June 1995 and August 2009, a total of 35 patients with achalasia underwent an LHD. The symptom scores were calculated by combining the frequency and the severity. Pre- and postoperative evaluations included symptom score, radiology, manometry, and 24-hour pH manometry. Median total symptom score was significantly lower than the preoperative score (19 vs 4, P < 0.001) at a median follow-up of 94 months. Among the 35 patients, 18 (51%) had chest pain. The frequency of chest pain was similar for the pre- and postoperative scores, but the severity tended to be less. Median esophageal diameter (5.4 cm vs 3.5 cm, P < 0.001) and lower esophageal sphincter pressure (41 mmHg vs 8.9 mmHg, P < 0.001) were significantly reduced after surgery. Median age, duration of symptoms, esophageal diameter, and lower esophageal sphincter pressure were similar between patients with and without chest pain prior to surgery. No significant differences were observed between the two groups in terms of amplitude, duration, and frequency of contractions from the findings of postoperative 24-hour esophageal manometry. Chest pain resolved in three patients (17%) and improved in seven patients (39%) after surgery. LHD can durably relieve achalasic symptoms of both dysphagia and regurgitation, and it can be considered the surgical procedure of choice. However, achalasic chest pain does not always seem to be related with patient characteristics and manometric findings.
Dis Esophagus 2010 May
PMID:Laparoscopic Heller myotomy with Dor fundoplication for achalasia: long-term outcomes and effect on chest pain. 2000

Treatment of achalasia aims at reducing the pressure of the lower esophageal sphincter (LES) and palliate symptoms. Our objective in this study was to investigate functional changes of the esophagus after Heller myotomy and evaluate their influence on postoperative gastroesophageal reflux and esophageal morphologic changes. Between 1980 and 2003, 216 patients with achalasia underwent Heller myotomy, associated with anterior partial fundoplication (Dor fundoplication). Preoperative and long-term outcome data were collected from these patients at our hospital. The objective was to analyze esophageal functional results after Heller myotomy in the long term. Results were classified as excellent, good, fair, or poor, according to Vantrappen and Hellemans' modified classification. One-year, 2-year, 5-year, 10-year, and 20-year postoperative follow-up information was available in 100% of all patients, 91.7%, 85.1%, 60%, 52.6%, and 45.9%, respectively. There were no perioperative deaths. One year after the surgery, all patients had a significant reduction in symptoms of dysphagia and regurgitation. Five years, 10 years, 15 years, and 20 years after surgery, there were 77.2% of patients (142 in 184), 68.1%, 57.1%, and 54.5%, respectively, who were satisfied (excellent to good) with surgery. No esophageal peristalsis was demonstrated in patients during follow-up. Contractile waves in the body of the esophagus were simultaneous. The difference in the distal esophageal amplitude, the LES relaxation rate, and LES pressures in the anterior wall and/ or two sides was significant (P < 0.05) when compared before and after operation. However, there was no significant difference in the LES length and LES pressure in the posterior side. The change of direction of the LES pressure and the relaxation of LES correlate with long-term outcomes. Postoperative gastroesophageal reflux rates, including nocturnal reflux, increased with time. The percentage of patients whose esophageal diameter became normal or remained mildly increased with time in the first 10 years after surgery changed significantly. Myotomy is an effective way to palliate symptoms in patients with achalasia. Adequate myotomy can lead to reduction of LES pressure in two or three directions, which may facilitate esophageal emptying by gravity. Surgical intervention does not lead to the return of esophageal peristalsis. Functional damage of LES in patients with achalasia is irreversible.
Dis Esophagus 2010 May
PMID:Functional analysis of long-term outcome after Heller's myotomy for achalasia. 2000 1

The current state of research into the etiology of achalasia only allows for speculation. To date, several studies have been performed investigating genetic, immune, and infectious disease mechanisms; however, none of these have been conclusive. Further research into this topic is warranted given the severity of the disease, and it may be possible that all of these mechanisms are involved in the pathophysiology of the disease.
Dis Esophagus 2012 May
PMID:Synopsis of investigations into proposed theories on the etiology of achalasia. 2000 2

Patients with longstanding achalasia have an increased risk of developing esophageal cancer. Surveillance is hampered by chronic stasis. We investigated whether aberrant expressions of tumor suppressor gene p53 and proliferation marker ki67 are early predictors for progression to malignancy. In 399 achalasia patients, 4% died of esophageal cancer despite surveillance. We performed a cohort study, using surveillance biopsies from 18 patients (11 carcinoma, one high-grade dysplasia [HGD], and six low-grade dysplasia [LGD]) and 10 controls (achalasia patients without cancer or dysplasia development). One hundred sixty-four biopsies were re-evaluated and studied for p53 and ki67 expression using immunohistochemistry. Eighty-two percent of patients with cancer/HGD showed p53 overexpression in surveillance biopsies at a mean of 6 (1-11) years prior to cancer development. In 67% of patients with LGD and only in 10% of the controls p53 overexpression was present. The proportion of samples with p53 overexpression increased with increasing grades of dysplasia. We found no difference for ki67 overexpression. p53 overexpression may identify achalasia patients at increased risk of developing esophageal carcinoma. Further study is needed to determine if patients with p53 overexpression would benefit from intensive surveillance to detect esophageal neoplasia at a potential curable stage.
Dis Esophagus 2010 Aug
PMID:Expression of p53 as predictor for the development of esophageal cancer in achalasia patients. 2011 24

The management of achalasia remains controversial, with little consensus on the optimal patient treatment pathway. In our own esophageal unit, we offer pneumatic dilatation as the initial therapy in most patients as first-line therapy. In this study, we aimed to examine the safety and efficacy of our own approach to the management of patients with a diagnosis of achalasia, examining symptomatic outcomes, patient satisfaction, and need for further intervention, as well as examining patient factors associated with treatment failure. Sixty-seven consecutive patients underwent pneumatic dilatation as first-line therapy (53% male, mean age 46 years). All attended regular outpatient follow-up (mean 37, range 3-132 months). Twenty-five percent of patients required a second intervention because of symptom recurrence, at a median period of 4.5 months. Symptomatic outcomes were excellent or good in 80%. Significant predictors of treatment failure and poor symptom score included a younger age at the time of diagnosis and increased esophageal diameter on barium swallow. This study suggests that pneumatic dilatation is a safe and effective approach as first-line therapy in patients with newly diagnosed achalasia.
Dis Esophagus 2010 Aug
PMID:Outcomes in achalasia from a surgical unit where pneumatic dilatation is first-line therapy. 2035 40


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>