Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014848 (achalasia)
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This study aims to evaluate by the use of 24-hour combined multichannel intraluminal impedance and pH monitoring (MII-pH) the efficacy of the Nissen fundoplication in controlling both acid and nonacid gastroesophageal reflux (GER) in patients that underwent Heller myotomy for achalasia. It has been demonstrated that fundoplication prevents the pathologic acid GER after Heller myotomy, but no objective data exists on the efficacy of this antireflux surgery in controlling all types of reflux events. The study population consisted of 20 patients that underwent laparoscopic Heller myotomy and Nissen fundoplication for achalasia. All patients were investigated with manometry and MII-pH. MII-pH showed no evidence of postoperative pathologic GER. The overall number of GER episodes was normal in both the upright and recumbent position. This reduction was obtained because of the postoperative control of both the acid and nonacid reflux episodes. The Nissen fundoplication adequately controls both acid and nonacid GER after extended Heller myotomy. Further controls with MII-pH are warranted to check at a longer follow-up for the efficacy of this antireflux procedure in achalasic patients.
Dis Esophagus 2008
PMID:Objective assessment of gastroesophageal reflux after extended Heller myotomy and total fundoplication for achalasia with the use of 24-hour combined multichannel intraluminal impedance and pH monitoring (MII-pH). 1856 68

The laparoscopic Heller-Dor operation has been the procedure of choice for the treatment of achalasia. However, because the incidence of achalasia is low, reports on the outcome of surgical treatment for achalasia are limited. In this study, the therapeutic results after laparoscopic Heller-Dor operation for achalasia at a single university hospital were evaluated. Between August 1994 and July 2006, 100 consecutive patients underwent laparoscopic Heller-Dor operation. The therapeutic results after laparoscopic Heller-Dor operation were assessed based on complications, operation time, blood loss, postoperative hospital stay, and the standardized questionnaire for satisfaction by telephone or outpatient clinic interview. With respect to perioperative complications, lower esophageal mucosal perforation occurred in 14 patients, but all of them could be suture-obliterated laparoscopically. One patient was converted to laparotomy because of uncontrolled bleeding from the short gastric artery. The mean operative time was 169 minutes, and the mean perioperative blood loss was 22 mL. The median postoperative hospital stay was 7 days. Reflux esophagitis, which was seen in five patients, was treated successfully with a proton pump inhibitor. According to the standardized questionnaire for satisfaction, 77 patients rated their recovery as 'excellent', 17 as 'good', 4 as 'fair', and 2 as 'poor'; thus, the overall success rate was 94%. There were no significant differences in surgical outcomes by morphologic type and severity of esophageal dilatation; however, the success rate deteriorated significantly with progression of the morphologic type. Laparoscopic Heller-Dor operation is a safe and effective surgical treatment for achalasia.
Dis Esophagus 2009
PMID:Results after laparoscopic Heller-Dor operation for esophageal achalasia in 100 consecutive patients. 1901 50

Only a few studies have been performed comparing subjective symptom improvements with objective improvement in esophageal emptying after pneumatic dilation (PD), and discrepancy existed. We evaluated whether esophagogram measurements adds to the traditional subjective symptoms scores in assessing achalasia patients after PD. We enrolled 32 new patients with achalasia who received endoscope-guided PD treatment between January 1998 and June 2004. Postdilation investigations were performed by using esophageal emptying on esophagogram prospectively in a blinded manner, along with symptom scores before and after PD at the initial investigation, 6 weeks later, and every 1 year thereafter. Our results showed that seven patients who noted complete relief showed less than 50% improvement in barium column height and esophageal diameter. There was no linear correlation between the degree of patient symptom improvement and esophageal emptying measured by esophagogram (r = 0.181, P = 0.322). A trend of association existed in the relationship between clinical remissions and initial post-PD esophageal emptying improvement (P = 0.067). In summary, the association between the post-PD symptom score improvement and degrees of esophageal emptying may be hampered by the small sample size in the current study. An additional objective parameter like esophagogram to the subjective symptom scores may be more optimal in assessing clinical remissions.
Dis Esophagus 2009
PMID:The role of barium esophagogram measurements in assessing achalasia patients after endoscope-guided pneumatic dilation. 1901 53

Pneumatic dilation (PD) has been widely used in the treatment of idiopathic achalasia with a 70-90% response. The aim of this study was to evaluate the effectiveness of PD and its predictive factors by means of clinical assessment. In addition, we evaluated its safety and the need for subsequent surgical intervention. Fifty-six patients were treated with a Witzel dilator. The response was evaluated at medium (1-5 years) and long term (>5 years). Diverse possible predictive factors to response were analyzed. After the first PD, 85.7% of the 56 patients improved and passed from clinical stage II-III to clinical stage 0-I (P < 0.005). After the second dilation, 84.6% of the patients (13) passed to clinical stage 0-I (P < 0.05). Only patients who were not young (>40 years) avoided a second dilation and/or surgery (P < 0.001). During the first 5 years of follow-up, 80% of patients maintained their response; this percentage decreased to 58% after 10 years. PD therapy of achalasia is a safe technique, with few adverse effects (4% perforations and 10% gastroesophageal reflux). It offers a medium-term response of 80% and long-term response of around 60%. Age was the only predictive response factor.
Dis Esophagus 2009
PMID:Endoscopic pneumatic balloon dilation in primary achalasia: predictive factors, complications, and long-term follow-up. 1902 91

Pseudoachalasia is a rare clinical entity with clinical, radiographic, and manometric features often indistinguishable from achalasia. Primary adenocarcinomas arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes of pseudoachalasia. Rarely, processes other than esophagogastric cancers including chronic idiopathic intestinal pseudo-obstruction, amyloidosis, sarcoidosis, Chagas' disease, vagotomy, antireflux surgery, pancreatic pseudocysts, von Recklinghausen's neuroinomatosis, gastrointestinal stromal tumor, and other malignancies and rare genetic syndromes, may lead to the development of pseudoachalasia. Secondary achalasia is extremely rare, with less than 100 cases reported in the literature so far. Gastrointestinal manifestations in primary or secondary amyloidosis include abdominal pain, diarrhea, constipation, malabsorption, obstruction, motility disturbance, intestinal infarction, perforation, and hemorrhage; however, gastrointestinal tract involvement is asymptomatic in most instances. We present here a rare case of multiple myeloma initially presenting with dysphagia because of esophageal amyloidosis and manometric findings typical of achalasia.
Dis Esophagus 2009
PMID:A rare case of multiple myeloma initially presenting with pseudoachalasia. 1920 46

Megaesophagus is the end-stage of achalasia cardiae. It is the result of peristaltic disorders and slow decompensation of the muscular layer of the esophagus. The aim of this article is to detail the diagnostic criteria and surgical management of megaesophagus. Criteria were acute bending of esophagus axis; lack of esophagus peristalsis, and no response to stimulation in the manometric test; and Los Angeles C/D esophagitis in the endoscopic examination. Between 1991 and 2004 seven patients (5 females, 2 males; age, 51-67 years; average age, 59 +/- 8 years) were treated. A bypass made from the pedunculated part of the jejunum connecting the part of esophagus above the narrowing with the praepyloric part of the stomach was made. Access was by an abdominal approach. A jejunum bypass was made in six patients with megaesophagus. A transhiatal esophageal resection was carried out, and in the second stage a supplementary esophagus was made from the right half of the colon on the ileocolic vessels in one patient who had experienced two earlier unsuccessful operations. Symptoms of dysphagia, recurrent inflammation of the respiratory tract, and pain subsided in all patients. Complications were not reported in the postoperative period. All patients survived. Subsequent radiographic and endoscopic examination showed very good outcome. The jejunum bypass gave very good results in the surgical treatment of megaesophagus.
Dis Esophagus 2009
PMID:Diagnostic criteria and surgical procedure for megaesophagus--a personal experience. 1920 50

Achalasia is a motility disorder characterized by the absence of coordinated peristalsis and incomplete relaxation of the lower esophageal sphincter. The etiology remains unclear although dense inflammatory infiltrates within the myenteric plexus have been described. The nature of these infiltrating cells is unknown. The aim of this study was to evaluate the expression of proinflammatory cytokines - namely, tumor necrosis factor alpha and interleukin-2 - in the distal esophageal muscle in patients with achalasia. Lower esophageal sphincter muscle from eight patients undergoing myotomy or esophagectomy for achalasia of the esophagus were obtained at the time of surgery. Control specimens consisted of similar muscle taken from eight patients undergoing operation for cancer or Barrett's esophagus. The expression of tumor necrosis factor alpha and interleukin-2 were assessed by immunohistochemistry. The total number of inflammatory cells within the myenteric plexus were counted in five high power fields. The percentage of infiltrating cells expressing tumor necrosis factor alpha or interleukin-2 was calculated. Clinical data including demographics, preoperative lower esophageal sphincter pressure, duration of symptoms, and dysphagia score (1 = no dysphagia to 5 = dysphagia to saliva) were obtained through electronic medical records. Statistical comparisons between the groups were made using the unpaired t-test, Fisher's exact test, or Mann-Whitney U test, with a two-tailed P-value less than 0.05 being considered significant. The total number of inflammatory cells was found to be similar between the groups. A significantly higher proportion of inflammatory cells expressed tumor necrosis factor alpha in achalasia as compared with controls (22 vs. 11%; P= 0.02). A similar percentage of infiltrating cells expressed interleukin-2 (40 vs. 41%; P= 0.87). Age, gender, preoperative lower esophageal sphincter pressure, or dysphagia score were not correlated to expression of these cytokines. There was, however, a significant inverse correlation between duration of symptoms and the proportion of inflammatory cells expressing tumor necrosis factor alpha in achalasia (P= 0.007). In conclusion, a higher proportion of infiltrating inflammatory cells expressed tumor necrosis factor alpha in achalasia. Furthermore, this proportion appears to be highest early in the disease process. Further studies are required to more clearly delineate the role of tumor necrosis factor alpha in the pathogenesis of this idiopathic disease.
Dis Esophagus 2009
PMID:An increased proportion of inflammatory cells express tumor necrosis factor alpha in idiopathic achalasia of the esophagus. 1920 53

The clinical significance of nonspecific esophageal motility disorder (NEMD) is unclear. Our aim was to investigate the natural history of NEMD. All manometries performed at Meir Hospital from 1997 to 2004 and diagnosed as NEMD were reviewed. Manometric criteria for NEMD included either low-amplitude peristalsis, nonprogression of peristalsis, prolonged retrograde or triple-peaked waves, or incomplete relaxation of the lower sphincter. Patients determined to have NEMD were contacted and asked to complete a questionnaire and undergo a second manometry. NEMD had been diagnosed in 137 patients. Upon review of manometry results, 65 patients were eligible for the study (36 men and 29 women). The other 72 patients did not have NEMD when we reviewed their manometry tracing, applying strict criteria as specified in Table 1. The average age was 64 +/- 16 years (range 24-83 years). The average follow-up period was 7 +/- 2 years. All 65 patients were symptomatic at their initial prestudy visit. By the second visit, symptoms had resolved in 33 (51%) patients and improved in 13 (19%). Dysphagia, chest pain, and food regurgitation had improved, whereas heartburn and respiratory symptoms had not. Of 37 patients with triple-peaked waves, only 11 (30%) had improved clinically. Of the 65 study patients, 17 (26%) had a second manometry during the study, which was normal in 2 (12%), unchanged in 11 (69%), and revealed achalasia in 4 (23%), representing 6% of all study patients. NEMD is generally a benign disorder that improves clinically in most cases. Nevertheless, in about 6% of patients, NEMD may evolve into achalasia.
Dis Esophagus 2009
PMID:Nonspecific esophageal motility disorders may be an early stage of a specific disorder, particularly achalasia. 1930 17

Achalasia surgical treatment alters the esophagogastric junction anatomy (cardiomyotomy plus fundoplication or esophagectomy and gastric pull-up), thus favoring a certain degree of gastroesophageal reflux. Gastric secretory and hormonal functioning is not completely known in chagasic patients. The aim of this study was to evaluate the gastric secretory and hormonal response in patients with end-stage chagasic achalasia compared with normal subjects. Gastric secretion and hormonal response were assessed by estimation of gastric acid secretion (GAS) in basal condition and after pentagastrin stimulation, basal serum gastrin, and serum pepsinogen (SP) in basal condition and after betazole hydrochloride (Histalog; Eli Lilly and Company, Indianapolis, IN, USA) stimulation in 27 patients with chagasic achalasia. The results were then compared with those of 24 normal subjects. In the chagasic group, the mean basal and stimulated GAS were significantly lower than in the control group (basal: 1.277 vs. 3.13, P = 0.002; stimulated: 15.9 vs. 35.8, P = 0.0001). Chagasic patients' SG levels showed a significantly higher basal value than the control group (83.3 vs. 36.8, P = 0.0001). There was a significant increase of SP after stimulation compared with the basal levels in both chagasic and control groups. Although the chagasic patients' SP values were higher than the controls, this difference was not statistically significant, either in basal and stimulated conditions (basal: 122.0 vs. 108.9, stimulated 120 min: 177.1 vs. 158.9). In patients with chronic Chagas' disease (ChD), although autonomic denervation does not suppress the strength of the gastric mucosal cells' secretory response to stimulation, it reduces GAS (parietal cell) without, however, affecting SP production (chief cells). On the other hand, the gastrin-producing cells have continuously been stimulated by low GAS.
Dis Esophagus 2009
PMID:Gastric secretory and hormonal patterns in end-stage chagasic achalasia. 1930 18

To choose which treatment would be most effective for the individual patient with newly diagnosed achalasia is difficult for the tending physician. A diagnostic tool that would allow prediction of the symptomatic and functional response after treatment for achalasia is therefore needed. The timed barium esophagogram (TBE) is a method that allows objective assessment of esophageal emptying, but the value of TBE in the clinical management of achalasia remains to be clarified. The aim of this study was first, to assess the ability of TBE to predict symptoms and treatment failure during post-treatment follow-up. Second, to determine whether esophageal emptying as assessed by TBE differs after treatment with pneumatic dilatation or laparoscopic myotomy. Fifty-one patients with newly diagnosed achalasia were prospectively randomized to pneumatic dilatation (n = 26) or laparoscopic myotomy (n = 25). Evaluation with TBE was performed before (n = 46) and after treatment (n = 43). The median interval between treatment and post-treatment TBE was 6 months, and the median follow-up time after the post-treatment TBE was 18 months. Following therapeutic intervention, TBE parameters did not differ significantly between treatment groups. However, significant correlations were found between the height of the barium column at 1 min and the symptom scores at the end of follow up for 'dysphagia for liquids' (P < 0.05, rho = 0.47), 'chest pain' (P < 0.05, rho = 0.42), and the 'Watson dysphagia score' (P < 0.05, rho = 0.46). Patients with less than 50% improvement in this TBE-parameter (height at 1 min) post-treatment had a 40% risk of treatment failure during follow-up. In summary, pneumatic balloon dilatation and laparoscopic myotomy similarly affected esophageal function as assessed by TBE-emptying. Lack of improvement in barium-column height post-treatment was associated with an increased risk of treatment failure which should motivate close surveillance in order to detect symptomatic recurrence at an early stage.
Dis Esophagus 2009
PMID:Evaluation of the response to treatment in patients with idiopathic achalasia by the timed barium esophagogram: results from a randomized clinical trial. 1943 Dec 19


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