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)

Achalasia of the esophagus may be associated with abnormalities of the myenteric plexus (hypo- and anganglionosis). This report evaluates this relationship by studying the effect of benzalkonium chloride (BK, a topical neurotoxin) on the lower esophagus. Following midline laparotomy, topical BK (0.5%) was applied to the muscularis of the lower 1.0 cm of the esophagus for 30 min in 38 Sprague-Dawley rats (200 g). Thirty-eight additional rats acted as controls (unoperated, n = 19; sham laparotomy, n = 19). At 1 and 3 months animals were evaluated for weight gain, daily food intake (g/100 gm body wt), lower esophageal sphincter (LES) manometry, and contrast esophagram. At 3 months, the esophagus was evaluated for histologic study and acetylcholinesterase staining. Esophagram showed distal narrowing with proximal dilatation in BK rats (inner diameter 4.71 +/- 0.61 vs 6.17 +/- 0.58 in controls, P less than 0.001). Daily food intake was significantly less in BK rats (5.57 +/- 0.41 g vs controls 7.69 +/- 0.33 g P less than 0.001). Daily weight gain was also less in BK rats (1.13 +/- 0.34 vs controls 1.83 +/- 0.25, P less than 0.001). An increased LES pressure was noted in BK rats (5.45 +/- 0.89 mmHg vs controls 4.04 +/- 1.04 mmHg; P less than 0.1). A histologic study showed aganglionosis in BK rats with positive cholinesterase staining fibers compared to controls. Topical BK results in distal esophageal aganglionosis characterized by distal narrowing, proximal dilatation, decreased food intake, and limited weight gain when compared to controls. These findings are similar to those observed in achalasia and support a primary neurogenic cause for its etiology.
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PMID:The effect of a neurotoxin (benzalkonium chloride) on the lower esophagus. 275 16

Lower oesophageal sphincter supersensitivity to gastrin I and cholinergic stimulation has recently been described in patients with achalasia. To determine the pathogenesis of this finding, the lower oesophageal sphincter was tested to a cholinesterase inhibitor, edrophonium chloride. Edrophonium chloride significantly increased the lower oesophageal sphincter pressure both in normal subjects and in patients with achalasia. The preservation of this response in the presence of denervation supersensitivity suggested intact postganglionic cholinergic nerves and, thus, a preganglionic site of denervation in achalasia.
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PMID:The site of denervation in achalasia. 506 32

Pseudocholinesterase deficiency is a rare genetic as well as an acquired disorder that affects the body's ability to metabolize choline esters such as succinylcholine and mivacurium. It can be inherited as an autosomal recessive trait, occurring in approximately 1 in 3,200 to 1 in 5,000 people. In most cases of pseudocholinesterase deficiency, no signs or symptoms of the condition exist. It is first suspected after a prolonged recovery from paralysis following general anesthesia in which succinylcholine or mivacurium are administered. We emphasize the importance of obtaining a detailed history prior to any endoscopic intervention or surgery requiring monitored anesthesia care or general anesthesia. In addition, a family history of anesthesia complications may help identify patients at risk. This article examines a case of a patient who underwent a successful endoscopic pneumatic dilation under general anesthesia for the treatment of achalasia, but was subsequently admitted to the intensive care unit, requiring mechanical ventilator support, for 18 hours. The patient made a complete recovery and was discharged home with no further complications. This case stimulated a review of this entity and we provide recommendations relevant to all proceduralist and anesthesia staff, as well as all personnel involved in procedures.
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PMID:Pseudocholinesterase Deficiency: What the Proceduralist Needs to Know. 3057 21