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)

Older patients with achalasia presumably have had a longer, more indolent course than younger patients. This study was undertaken to determine the impact of patient age and duration of symptoms on symptom severity and outcome after Heller myotomy. Two hundred sixty-two patients (142 men and 120 women), of average age 49 years +/- 17.7 (SD), have undergone laparoscopic Heller myotomy. Patients scored their symptoms using a Likert scale and subjectively rated their overall outcome. Data are presented as median, mean +/- SD, when appropriate. Follow-up is 25 months, 32 months +/- 28.7. Symptom severity scores improved after myotomy (P < 0.001 for all, paired Student's t test). Before myotomy, older patients had less dysphagia, regurgitation, choking, and chest pain (P < 0.05). Duration of preoperative symptoms did not correlate with frequency of symptoms. After myotomy, older patients had lower scores for dysphagia, chest pain, choking, and heartburn (P < 0.01); patients with prolonged durations of symptoms had lower dysphagia and choking scores. Neither age nor duration of symptoms had a significant effect on overall subjective outcomes. All patients should expect significant reductions in symptoms of achalasia following myotomy. Age and duration of symptoms impact symptoms before and after myotomy, but neither seem to impact subjective measure of outcome.
 ...
PMID:Age affects presenting symptoms of achalasia and outcomes after myotomy. 1598 75

The goals in the treatment of achalasia are threefold: 1) relieving the symptoms, particularly dysphagia and bland regurgitation; 2) improving esophageal emptying by disrupting the poorly relaxing lower esophageal sphincter (LES); and 3) preventing the development of megaesophagus. Although achalasia cannot be permanently cured, excellent palliation is available in over 90% of patients, especially those with pneumatic dilation and laparoscopic Heller myotomy. The efficacy for short- and long-term therapy seems to be similar when performed by experts. Pneumatic dilation done as an outpatient surgery disrupts the LES muscle from within by using balloons of progressively larger diameter (3.0, 3.5, and 4.0 cm). Repeat dilations may be required; secondary severe gastroesophageal reflux disease (GERD) is rare, but approximately 2% of patients will have an esophageal perforation. A surgical Heller myotomy is now being done laparoscopically through the abdomen that cuts the LES and extends the myotomy 2 to 3 cm onto the stomach. Usually 2 days of hospitalization is required, and patients can normally return to work in 1 to 2 weeks. Severe GERD with esophagitis and peptic stricture is a common complication; therefore, most surgeons combine the myotomy with an incomplete fundoplication. Medical therapy is much less effective than these invasive procedures. Smooth muscle relaxants (nitrates and calcium channel blockers) taken immediately before meals improve dysphagia, but side effects and drug tolerance are common. The injection of botulinum toxin (100 to 200 units) endoscopically into the LES gives short-term relief of symptoms and improves esophageal emptying. This treatment is most effective in the elderly, as symptom relief can last up to 1 to 2 years with a single injection. Several studies suggest the most cost-effective management of achalasia is initial treatment with pneumatic dilation.
 ...
PMID:Modern management of achalasia. 1600 28

Only two treatment modalities-pneumatic dilation and Heller myotomy-promise long-term relief from dysphagia and regurgitation in patients with achalasia. The objective of this study was to determine whether both options differ in their effects on esophageal function, morphology, and improvement in symptoms. Eighty-nine patients diagnosed with achalasia between January 1980 and December 2002 at a single center were enrolled in this study. Sixty-four patients underwent pneumatic dilation and 25 Heller myotomy in combination with an anterior semifundoplication (Dor procedure). Clinical evaluation (Eckardt-Score), esophageal manometry, and barium swallow were performed before and within 6 months after treatment. Our data shows that Heller myotomy reduces the LES resting pressure more markedly (7.9 [3.7-16.9] mm Hg) than pneumatic dilation (14.5 [2.7-36.0] mm Hg) (P < 0.0001) with similar pressures at diagnosis in both groups. Morphologic changes, assessed by the diameter of the esophageal corpus, were also more pronounced after surgical therapy (P > 0.05). Both options will lead to an immediate and significant improvement in symptoms, although the two treatment modalities did not differ in their subjective results. As only objective findings, such as those obtained by manometry and the timed barium swallow, have proven relevance for the assessment of long-term results, surgical therapy is the superior and more effective treatment option in patients with achalasia.
 ...
PMID:Effects of pneumatic dilation and myotomy on esophageal function and morphology in patients with achalasia. 1602 11

Achalasia, a poorly relaxing lower esophageal sphincter, produces a functional obstruction and the expected symptoms of dysphagia, regurgitation and eventually weight loss. The cause of achalasia remains largely unknown in Western countries, Chagas' disease being the most frequent etiology in Brazil. We report on two sets of monozygotic male twins with typical manifestations of achalasia. The majority of authors attribute a limited contribution unless achalasia is related to a multisystem disorder, like the triple-A or Allgrove's syndrome, an autosomal recessive disease characterized by the triad of adrenocorticotropic hormone (ACTH) resistant adrenal insufficiency, achalasia and alacrima. The four cases reported demonstrated the genetic influence of achalasia in patients without multisystem disorders. We believe that idiopathic achalasia is a syndrome with similar clinical, pathological, radiological and manometric evolution, but with a great variety of etiological agents, one of them being the congenital form.
 ...
PMID:Congenital achalasia: facts and fantasies. 1619 35

An eight-year-old, male boxer dog was presented with a one-month history of hindlimb weakness followed by compulsive ineffective drinking, dysphagia, regurgitation and nasal reflux during drinking. A neurological examination revealed weakness and conscious proprioception deficits in both hindlimbs with normal spinal reflexes. The dog's swallowing function was examined by fluoroscopy. This showed normal prehension of the barium paste, bolus formation and contraction of the pharyngeal muscle, but no opening of the upper oesophageal sphincter was detected. A serum thyroid stimulating hormone level of 0.402 ng/dl and serum total T4 of 0-01 microg/dl were determined. The dog fully recovered one month after L-thyroxine therapy. The association found between cricopharyngeal achalasia and hypothyroidism suggests that hypothyroidism should be included in the list of differential diagnoses for dogs with cricopharyngeal achalasia.
 ...
PMID:L-thyroxine responsive cricopharyngeal achalasia associated with hypothyroidism in a dog. 1630 Jan 18

When patients with the typical reflux symptoms of heartburn, regurgitation, or both, undergo endoscopy, up to 75% will not have endoscopic oesophagitis or evidence of Barrett's oesophagus. These patients have been described as having endoscopic negative or, more commonly, non-erosive reflux disease (NERD). Patients without oesophagitis, but with a positive pH test, can be diagnosed with gastro-oesophageal reflux disease (GERD). Some experts also consider a response to proton pump inhibitor therapy as proof of GERD in a patient with the correct symptoms and a negative endoscopy. Patients with normal acid exposure, but who report symptoms with a majority of their reflux episodes documented during an ambulatory pH study, have also been considered to have NERD, although others have labelled them as having 'functional heartburn'. Finally, there are some patients who have reflux symptoms and respond to reflux therapy, but have no demonstrable reflux by either endoscopy or ambulatory reflux testing. Whether these patients are part of the GERD spectrum or have another diagnosis is not clear. It seems that the most widely used definition of functional disease (the Rome II criteria) would include these patients as having functional heartburn, as it was defined as 'greater than or equal to 12 weeks of either continuous or intermittent symptoms of burning retrosternal discomfort or pain without pathologic GERD, achalasia, or other motility disorders with a recognized pathologic basis'. This article reviews potential differences in pathophysiology between erosive oesophagitis and NERD; explores whether symptoms can help distinguish NERD patients from erosive oesophagitis patients; and explores the evaluation and therapy of these patients.
 ...
PMID:Review article: the role of acid suppression in patients with non-erosive reflux disease or functional heartburn. 1648 68

Achalasia is a motor disorder of the esophageal smooth muscle in which the lower esophageal sphincter does not relax normally with swallowing, and the esophageal body undergoes nonperistaltic contractions. The underlying abnormality is the loss of intramural neurons. Achalasia affects men and women of all ages. Dysphagia, chest pain, and regurgitation are the main symptoms. Information on the effects of achalasia on pregnancy outcome and the effects of pregnancy on the natural course of achalasia is limited. Two studies, including 30 patients altogether, and several case reports have been published. Treatment options include nitrates, calcium channel antagonists, botulinum toxin injection, pneumatic dilation, and esophagomyotomy.
 ...
PMID:Achalasia in pregnancy. 1649 Jan 20

Laparoscopic Heller myotomy for achalasia has a 10-20% failure rate and may require re-operation to control persistent or recurrent symptoms. We report follow-up of 15 patients who underwent laparoscopic re-operation for failed Heller myotomy. Between 1993 and 2004, 15 patients underwent laparoscopic re-operation for failed Heller myotomy at our center. The mean duration between procedures was 23 months. Follow-up was completed at a mean duration of 30 months in 14 patients (93%) via a telephone questionnaire. Our overall failure rate for primary surgery (n = 106) was 5.6%. The mechanisms of failure were incomplete myotomy (33%), myotomy fibrosis (27%), fundoplication disruption (13%), too tight fundoplication (7%) and a combination of myotomy fibrosis and incomplete myotomy (20%). Significant symptom improvement was observed with postoperative symptom resolution seen in 71% of patients with dysphagia, 89% for regurgitation, 58% for heartburn and 40% for chest pain. Fifty percent reported excellent results and 79% would recommend the procedure to a friend. Subsequent dilations were performed in four patients (29%). Two patients required conversion to open surgery (13%). Three patients (20%) failed the re-operation and required further revisional surgery. Complications included intraoperative perforation in three (none of which resulted in postoperative morbidity) and a pneumothorax in one patient. Prior endoscopic therapies (pneumatic dilation or Botulinum toxin) were not associated with poor results. Laparoscopic re-operation for failed Heller myotomy is feasible and results are encouraging.
 ...
PMID:Laparoscopic re-operation for failed Heller myotomy. 1672 98

We report on a 12-year-old girl presenting with mental retardation, trigonocephaly, midface hypoplasia, upward-slanting palpebral fissures, arched eyebrows, bilateral epicanthal folds, hypertelorism, a flattened nasal bridge, a short nose, anteverted nares, a long philtrum, a small mouth, micrognathia, low-set ears, a short neck, long digits, flexion deformity of the fingers of the hands, hypoplasia of the labia majora, hyperplasia of the labia minora, flat feet, dysphagia, frequent regurgitation, prominent esophageal dilation, and achalasia. Seizures were noted since 5 years of age. Cytogenetic analysis of her peripheral blood revealed a karyotype of 46,XX, der(9)t(1;9)(p36.22;p22.2)pat. Achalasia, an uncommon esophageal motor disorder, has not been previously described in association with either a deletion of 9p or a duplication of 1p.
 ...
PMID:Partial trisomy 1p (1p36.22-->pter) and partial monosomy 9p (9p22.2-->pter) associated with achalasia, flexion deformity of the fingers and epilepsy in a girl. 1710 Jan 98

The incidence and prevalence of achalasia is 0.6/100,000 people per year and 1/10,000, respectively. It is difficult then for one center to accumulate a large cohort of patients. One study (Gut 33:1011,1992) described the presenting symptoms in only 38 patients. To approach this problem differently, we used the internet to access a larger patient population. Using search engines at Google and Yahoo, Inc., we identified achalasia support groups. We examined the most populated support group (YSG) and assembled the exchanged messages into 4 categories: support, symptoms, treatment, and diagnosis. Next, a survey modeled after a previous study (ibid) was composed and posted on a university-sponsored Web site for March 2003, advertised to YSG members, and then removed from the server. The results were entered into a database and analyzed. There were 6 support groups identified; 1 was foreign. The most populated site was the Yahoo, Inc., group with 298 active members. We analyzed the 3,222 messages posted from October 6, 2002 through October 5, 2003. The message composition was as follows: 67% support, 16% symptoms, 12% treatment, and 5% diagnosis. Of 298 Yahoo site members, we had 88 respondents to the survey from 9 countries. The data from 83 were analyzed (5 were not completed). Respondents were 66 women (55%), 29 men (24%), and 5 non-gender-identified respondents. They ranged in age from 6 to 72 years at time of diagnosis. In the 83 respondents, dysphagia symptoms were reported by 98%, regurgitation by 68%, chest pain by 81%, weight loss by 69%, and epigastric pain by 67%, similar to those reported in the study cited in Gut. The modalities used to evaluate these achalasia patients were as follows: 83% had monometry, 87% had radiography, and 89% had endoscopy. The mean time from symptom onset to diagnosis was 5 years. The treatments attempted on this population were balloon dilatation in 57%, botulinum toxin injection (Botox) in 8%, myotomy in 12%, by lifestyle/diet modification in 12%, and other treatments in 11%. We believe that the Internet can provide physician-researchers with useful information about common and uncommon diseases on a global basis. The Internet allows patients to voice concerns that they may not freely express to their doctor and provides a modality to collect data from a larger number of patients than may be available at any single institution.
 ...
PMID:The use of the internet in data assimilation in rare diseases. 1721 76


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