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)

Four recent reports describe a multisystem disorder in which ACTH insensitivity is associated with achalasia and alacrima. We report studies on a male patient with this rare triad. The patient had alacrima from birth; isolated glucocorticoid deficiency had been diagnosed at 3.5 years of age and achalasia at age 6. The possibility that this syndrome could be due to a parasympathetic degeneration has already been proposed; the cause of the glucocorticoid deficiency, however, remains unclear. Parasympathetic function in other areas was investigated to determine whether there might be a more generalized abnormality. Specific cardiac tests of parasympathetic function showed that parasympathetic input to the heart was affected in the patient, while the same tests in an Addisonian child were normal. We show, then, a hitherto undetected parasympathetic abnormality in a patient with this syndrome, suggesting a generalized disturbance of this system. On this basis we may hypothesize that the glucocorticoid failure may be a consequence of the loss of parasympathetic input to the adrenal gland, although this remains to be demonstrated experimentally.
Clin Endocrinol (Oxf) 1985 Sep
PMID:Glucocorticoid deficiency with achalasia of the cardia and lack of lacrimation. 407 37

Experimental achalasia dogs produced with Deloyer's method showed higher resting pressure at the gastroesophageal junction and the increase in LES pressure in response to tetragastrin and cholecystokinin. Dose-response curve of the LES to each dose of tetragastrin in achalasia dog shifted to the left. Resting LES pressure in 11 patients with achalasia was 42.73 +/- 23.31 cm H2O. It increased significantly after intramuscular injection of 5 micrograms/kg of tetragastrin and fluoroscopic observation showed the tonic contraction of the lower esophagus and cardia. After the performance of Jekler-Lhotka operation, LES pressure decreased to lower values sufficient to prevent the gastroesophageal reflux. Comparing 5 kinds of hiatal herniorrhaphies in dogs, LES pressure increased postoperatively in the following order: Nissen, Belsey Mark IV, Stensrud, Hill and Harrington methods. Responses to tetragastrin increased after Nissen and Belsey Mark IV methods. In 12 out of 21 clinical cases of sliding esophageal hiatal hernia who had undergone Nissen-Rossetti method adding fundopexy and posterior gastropexy, preoperative esophageal manometry showed HPZ of 24.98 +/- 8.87 cm H2O in peak value and 5.1 +/- 3.46 cm in length. Seven cases showed the biphasic pattern and 5 cases showed the negative response to tetragastrin. Postoperative manometry showed HPZ of 31.42 +/- 18.46 cm H2O in peak value and 4.5 +/- 1.73 cm in length. One case showed the biphasic pattern and 3 cases showed the negative response to tetragastrin.
Nihon Geka Gakkai Zasshi 1985 Sep
PMID:[Gastrointestinal hormones and operations for achalasia of the esophagus and sliding esophageal hiatal hernia: their surgical significance]. 408 31

The disturbance of defecation in the patient with Hirschsprung's disease and the pathophysiology of constipation in the constipated patient with simple megarectum were investigated. In Hirschsprung's disease, an existence of aganglionosis and the sphincter achalasia are two main factors which cause clinical symptoms such as chronic constipation and megacolon. As a surgical treatment, we have routinely performed our modified Duhamel's operation. Postoperative followup study indicated that the most cases in our series have gained a satisfactory defecatory function 1 to 3 years after operation. In chronically constipated patient with simple megarectum, on the other hand, it was found manometrically that the most of them had significantly high anal canal pressure and incomplete anal relaxation after rectal stimulation. These findings indicate that those patients have hypertonic and achalasic sphincter. Therefore, the authors propose "high anal pressure syndrome (HAPS)" for such constipated patient with simple megarectum. As to the surgical treatment, 6 patients with simple megarectum had a complete posterior internal sphincterotomy and the postoperative results were excellent or good in our series.
Nihon Geka Gakkai Zasshi 1985 Sep
PMID:[Pathophysiology of defecatory disturbance in the patient with Hirschsprung's disease and chronically constipated patients with simple megarectum]. 408 53

A 38-year-old patient, complaining of dysphagia and substernal pain of nine months' duration, was found by manometric studies to have diffuse oesophageal spasm. As has similarly been reported for cholinergic agents in achalasia and in some cases of diffuse oesophageal spasm the oesophageal body responded in a hypersensitive fashion to subcutaneous and intravenous injections of pentagastrin. This effect was inhibited by the intravenous administration of atropine and completely abolished by nitroglycerine. When pentagastrin was subcutaneously injected before a barium examination a distortion of the oesophageal contour, described as curling or corkscrew oesophagus, regularly appeared.
Gut 1974 Sep
PMID:Supersensitivity to pentagastrin in diffuse oesophageal spasm. 421 3

This article explores how the mediastinum accommodates a significantly dilated esophagus, using achalasia as an anatomic model. This accommodation introduces the concepts of vascular tethering and the paratracheal esophagus as part of a unifying theory of "the path of least resistance." Practical examples demonstrate the importance of understanding these concepts in any disease involving esophageal dilatation.
Radiol Clin North Am 1984 Sep
PMID:Mediastinal impressions on the dilated esophagus. 638 21

Between January, 1970, and January, 1984, 113 patients with esophageal achalasia underwent 115 esophagomyotomies at the Lahey Clinic. Twenty-nine patients had been treated on one or more occasions by forceful dilation, and 18 had been operated upon before. Results are based on follow-up studies of 103 patients operated on 1 to 13.5 years ago (average follow-up period, 6.75 years). Six patients were lost to follow-up study, and six were operated upon less than a year ago. The condition of 94 patients (91%) was improved by operation. The improvement rate was 94% for those who underwent a primary operation and 76% for those who underwent reoperation. Only four of the nine poor results were caused by reflux esophagitis, and these patients are satisfactorily managed medically. Multiple regression analysis of risk factors including age, sex, duration of symptoms, severity of disease, length of follow-up, previous operation, and forceful dilations revealed that only previous operation correlated significantly with poor results (p = 0.0004). Preoperative and postoperative manometric assessment of the lower esophageal sphincter was made on some of these patients. The amplitude of lower esophageal sphincter pressure dropped from 32.5 +/- 1.6 (SEM) to 14.5 +/- 1.4 mm Hg, and the length of the lower esophageal sphincter decreased from 3.7 +/- 0.1 to 2.2 +/- 0.1 cm. These differences were highly significant (p = 0.001). After myotomy a short subhiatal remnant of the lower esophageal sphincter remains with pressure within the normal range, which minimizes the risk of postoperative gastroesophageal reflux. Because of the high success rate of limited esophagomyotomy and the low incidence of significant reflux symptoms after its use, we recommend that it be performed without an associated antireflux procedure.
J Thorac Cardiovasc Surg 1984 Sep
PMID:Operation for esophageal achalasia. Results of esophagomyotomy without an antireflux operation. 647 85

We reviewed the hospital records of 36 patients who underwent modified Heller's myotomy for achalasia between January, 1961, and December, 1982. There were 18 male and 18 female patients ranging between 17 months and 75 years old. The most frequent symptom was dysphagia, followed by regurgitation of ingested food and weight loss. Modified Heller's myotomy was performed through a transthoracic incision in 35 patients and a transabdominal incision in 1. An antireflux procedure in addition to esophagomyotomy was performed in 20 patients. There was 1 postoperative death. Thirty-three patients were followed up for periods ranging from 9 months to 21 years. The results were considered good in 27, fair in 2, and poor in 4. One of the 4 underwent repeat esophagomyotomy 71/2 years after the initial operation with a good result. The remaining 3 had an antireflux procedure at the time of esophagomyotomy. Because of recurrence of symptoms, esophagogastrostomy was performed in 1 and colon interposition in 2. These results suggest that an antireflux procedure should not be added to modified Heller's operation in the treatment of achalasia.
Ann Thorac Surg 1984 Sep
PMID:Two decades of experience with modified Heller's myotomy for achalasia. 647 42

We measured quantitative esophageal transit, expressed as percentage of esophageal retention, before and after pneumatic dilatation in two patients with achalasia. In the sitting position they ingested a 500 ml liquid meal containing 500 muCi technetium Tc 99m sulfur colloid. Radioactivity counts of the entire esophagus were plotted at five-minute intervals for 30 minutes. In five normal control subjects the esophagus essentially cleared in less than one minute. Both patients with achalasia had definite retention 30 minutes before dilatation and had quantitative improvement after dilatation. Radionuclide scintigraphic esophageal transit probably correlates better than other parameters with the physiologic degree of obstruction in achalasia.
South Med J 1983 Sep
PMID:Radionuclide esophageal transit: an evaluation of therapy in achalasia. 661 92

Esophageal transit of a small volume of watery liquid has been observed scintigraphically in 200 studies performed on patients aged between 6 days and 16 years. Qualitative information concerning esophageal morphology and function in the various phases of deglutition, and scintigraphic features of achalasia, stenosis, and other pathologies are described. Measured esophageal transit time and its normal variation, its relevance to the diagnosis of esophagitis, and the monitoring of treatment are discussed. This technique observing distinct deglutitions has proven a useful diagnostic tool. Its advantages and limitations are discussed in comparison with other methods.
Clin Nucl Med 1983 Sep
PMID:Pediatric esophageal scintigraphy. Results of 200 studies. 664 Oct 76

To avoid gastroesophageal reflux which is reported to occur in about 4-25 per cent after Heller esophago-cardiomyotomy, a modified gastro-fundoplication was designed using a transabdominal approach. Forty patients with idiopathic esophageal achalasia were subjected to this surgery. There were no serious complications and suppression of dysphagia and gastroesophageal reflux manifestations were excellent.
Jpn J Surg 1983 Sep
PMID:Cardiomyotomy and fundoplication for esophageal achalasia. 666 73


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