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)

This report describes paraneoplastic visceral neuropathy including achalasia, gastroparesis, subileus and constipation in a 59 year old patient with metastasising atypical bronchial carcinoid. Achalasia was successfully treated by cardiomyotomy and fundoplication; additionally, extramucosal pylorectomy was undertaken to improve gastric emptying. Endoscopic papillotomy was necessary because of a functional stenosis of the sphincter of Oddi with development of obstructive jaundice. Symptoms of intestinal pseudoobstruction did not improve with cisapride or corticosteroid treatment. Histological examination of gastrointestinal specimens revealed a lymphocytic infiltration of the myenteric plexus associated with loss of neurones. The rheumatoid factor was positive, there was evidence of circulating immune complexes and antibodies to Sm-antigen were present, suggesting a possible autoimmune pathogenesis.
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PMID:Paraneoplastic chronic intestinal pseudoobstruction as a rare complication of bronchial carcinoid. 164 19

The regulation of gastrointestinal function is known to involve elements of the enteric nervous system. Processes such as secretion, motility, blood flow, and immune function are all influenced by a complex network of neurons whose cell bodies lie in the gut. These neurons use a wide spectrum of substances as neurotransmitters, although the majority use peptides once thought to function only as gut hormones. It has been increasingly recognized that abnormalities of this neuroendocrine regulatory system underlie many gastrointestinal disorders. The most obvious are states of peptide excess found in patients with gut endocrine tumors such as carcinoid, gastrinoma, and somatostatinoma. Conversely, other disorders appear to be related to deficiency states. Examples include both achalasia and Hirschsprung's disease (congenital megacolon), where the loss of inhibitory neural action leads to abnormalities of peristalsis and sphincter function. Evidence for abnormal neuroendocrine regulation leading to disease states is increasing for many other gastrointestinal disorders.
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PMID:Neuroendocrine disorders of the gut. 853 22