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)

A child is reported with adrenocortical unresponsiveness to ACTH and autonomic dysfunction. The latter consisted of cold extremities, progressive loss of tear production, the development of achalasia of the esophagus, pupillary dysfunction, and an abnormal histamine skin test. These findings suggest progressive parasympathetic denervation as a cause for the adrenocortical abnormality.
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PMID:Autonomic dysfunction and adrenocortical unresponsiveness to ACTH. 285 Mar 14

Clinical uses of calcium channel blockers are expanding. In addition to the established uses in patients with arrhythmias, angina pectoris or hypertension, newer and to some extent investigational uses indicate widespread application. For instance, their use has been reported in hypertrophic cardiomyopathy and cold cardioplegia, as well as in pulmonary hypertension, antiplatelet therapy, asthma, achalasia and oesophageal spasm, increased intraocular pressure and in cerebral vasospasm. Their use in obstetrical practice has been proposed. Thus, the presentation of a patient who is treated with calcium channel blockers and who requires anaesthesia will become more common. Calcium channel blockers may, under certain circumstances, potentiate haemodynamic and MAC depressive effects of inhalation agents. There is also evidence that the effects of neuromuscular blocking agents may be potentiated. The anaesthetist should be aware that the potential for interactions exists with digoxin, propranolol, quinidine, theophylline or dantrolene. Of interest and some significance are the anaesthetic implications of pathophysiological alterations that can be induced by calcium channel blockers, by affecting lower oesophageal tone, intracranial hypertension, bronchomotor tone (asthma), muscular dystrophy, neuromuscular function, hypoxic pulmonary vasoconstriction, malignant hyperthermia, inhibition of platelet aggregation and hyperkalemia. Despite these significant potential anaesthetic implications and because, at this time, in some instances withdrawal has clearly demonstrated increase in the signs of myocardial ischaemia, it would not seem necessary to recommend preoperative discontinuation of calcium channel blocker medication in patients presenting for anaesthesia. It is, however, appropriate that there is a high index of awareness of potential problems, unless there is some modification in inhalation anaesthetic concentrations and neuromuscular blocker dosage. Monitoring of cardiovascular and neuromuscular functions is essential. Calcium channel blockers would appear to be currently the drugs of choice for angina pectoris, arrhythmias or hypertension in patients with associated chronic obstructive pulmonary disease.
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PMID:Anaesthetic implications of calcium channel blockers. 286 80

Various swallowing disorders have been viewed to be psychogenic and an altered contractile activity of the oesophagus has been reported to occur during emotional stress. Non-propulsive activity and "cardiospasm" were suggested to represent a symbolic revulsion, referring to deep, unconscious conflict or an ambivalent attitude towards incorporation (Kronfeld, 1934; Weiss, 1944; Alexander, 1950). However, these concepts were not corroborated by subsequent research. "Globus hystericus" was shown to result from webs and folds in the hypopharynx or from gastrooesophageal reflux, whilst the term "cardiospasm" has even become obsolete: there is no spasm of the lower oesophageal sphincter but an "achalasia", i.e., a failure to relax upon swallowing, which is not induced by psychic factors, but by lesions affecting the intrinsic and extrinsic innervation. On the other hand, it has been shown that the oesophagus reacts with non-propulsive contractions not only emotional tension, but also to cold or hot food and even to stimuli not related to ingestion such as intense short sounds, and that these contractions are likely to form part of the defence reaction of the healthy organism. Despite this responsiveness of the oesophagus and the fact that strategies aimed at inducing relaxation and reassurance can help patients to cope with their swallowing disorders, the psychosomatic concept that "exaggerated" oesophageal responses can lead to organic diseases has remained purely speculative. Present knowledge of the physiology and pathophysiology of the oesophagus clearly indicates that disorders such as globus sensation, achalasia, and diffuse oesophageal spasm should not be treated by psychotherapeutic but by appropriate surgical, or medical, means.
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PMID:[Swallowing the psyche]. 663 83

We describe two sisters with distal, slowly progressive muscular weakness and hypotrophy since childhood, autonomic dysfunction characterized by profuse sweating, distal cyanosis related to cold weather, orthostatic hypotension, and esophageal achalasia. Nerve conduction velocity of several motor nerves was slow, and although no sensory abnormalities were present, sural nerve biopsy revealed severe nonspecific demyelination. No similar patients could be found in the literature and we therefore suggest the possibility that these individuals have a newly recognized hereditary syndrome.
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PMID:Peripheral motor neuropathy associated with autonomic dysfunction in two sisters: new hereditary syndrome? 728 84

It has been previously shown that patients with achalasia may have motor abnormalities of the stomach, small bowel and biliary system. This study investigates whether a disturbance of extraintestinal autonomic function occurs. Autonomic function studies were performed in 15 patients with achalasia and 15 age- and sex-matched healthy controls. Pupillograms were obtained during darkness, light exposure and after pilocarpine administration. Cardiovascular function studies included determinations of heart rate variation during deep breathing and orthostasis. In addition, we determined blood pressure changes in response to sustained handgrip, cold exposure and orthostasis. Neurohormonal function was investigated by measuring serum pancreatic polypeptide (PP) levels prior to and following sham feeding. Pupillary function did not differ in patients as compared with controls. However, 9 of 15 patients (95% CI: 32-84%) and none of the controls showed at least one abnormal autonomic cardiovascular response. A significant difference between the two groups was observed in sympathetic function (P = 0.023). More patients than controls did not respond to sham feeding with a PP increase. It is concluded that some patients with achalasia exhibit an abnormality of the autonomic nervous system that extends beyond the gastrointestinal tract. These abnormalities mainly concern cardiovascular function but may also involve neurohormonal responses.
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PMID:Autonomic dysfunction in patients with achalasia. 762 67

Achalasia is considered a primary motility disorder confined to the oesophagus. The lower oesophageal sphincter (LOS) in achalasia is frequently hypertonic and manifests absent or incomplete relaxation in response to deglution. On the other hand, the LOS and the proximal stomach act physiologically as a functional unit whereby relaxation of the LOS during deglution is associated with receptive relaxation of the proximal stomach. Thus, this study investigated the hypothesis that impaired LOS relaxation in patients with achalasia might be associated with impaired relaxation of the proximal stomach. The study consisted of 20 patients with achalasia and 10 healthy controls. Gastric tone variations were quantified using an electronic barostat. Firstly, the study established the basal gastric tone (intragastric volume at the minimal distending pressure+1 mm Hg) and gastric compliance (volume/pressure relation) during isobaric distension (increasing stepwise the intragastric pressure from 0 to 20 mm Hg up to 600 ml). Secondly, the gastric tone response to cold stress (hand immersion into ice water for five minutes) or to control stimuli (water at 37 degrees) was determined. Basal gastric tone mean (SEM) was similar in achalasia and in healthy controls (125 (9) ml v 138 (9) ml, respectively). Compliance was linear and similar in both groups, which also showed similar gastric extension ratios (58 (7) ml/mm Hg v 57 (6) ml/mm Hg). Cold stress induced a gastric relaxatory response that, as a group, was significantly lower in achalasia than in healthy controls (volume: 43 (20) ml v 141 (42) ml; p < 0.05). The responses in each group were not uniform, five of the 20 patients with achalasia showed definite (volume > 100 ml) relaxatory responses whereas four of the 10 healthy controls did not. In conclusion, reflex gastric relaxation is impaired in most patients with achalasia showing that the proximal stomach, and not exclusively the oesophagus, may be effected by the disease.
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PMID:Impaired gastric relaxation in patients with achalasia. 769 93