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)

Vasoactive intestinal peptide (VIP), first isolated from the gut, was originally considered a candidate gastrointestinal hormone. Since about 1975, however, it has become increasingly clear that it is primarily a neurotransmitter or neuromodulator and that it exerts its functions mainly by local release from nerve endings. VIP plays a hormonal role only when it is released in large amounts from a tumor, with a consequent overflow into the circulation and grossly elevated plasma concentrations of the peptide. Moderately increased VIP plasma and tissue concentrations that cause mainly local effects are found in intestinal ischemia. Crohn's disease and some other chronic inflammatory diseases of the bowel. VIP is also measured in increased amounts in the normal fetus and neonate, where it may play an important physiological role. Such an increase of VIP levels in the circulation could enhance perfusion and metabolic activity of tissues during their rapid-growth period. On the other hand, disorders with a disturbed VIP function such as achalasia and Hirschsprung's disease and possibly also asthma and cystic fibrosis seem to be characterized mainly by a derangement of smooth muscle activity and/or exocrine secretion. Considering this list of disorders where VIP has either a proven or suspected role, it is easy to imagine the significance of this peptide in pediatric pathophysiology.
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PMID:[Vasoactive intestinal polypeptide (VIP)--possible importance in diseases of childhood]. 299 82

The records of 14 patients who underwent surgical revision for anastomotic strictures after hypopharyngocolostomy or esophagocolostomy were reviewed. The esophageal reconstruction was originally performed for esophageal strictures or resections after corrosive injury in 13 patients and for achalasia in 1. The esophageal substitutes used consisted of right ileocolon in 12 patients and left colon in 2. Routes of colon positioning were substernal in 13 patients and subcutaneous in 1. One-half of all strictures were located at the hypopharynx and the other half at the cervical esophagus. Causes of the strictures were anastomotic leakage in 5 patients, progressive caustic scarring in 4, graft ischemia in 3, combined caustic and tuberculous scar in 1, and technical error in 1. The interval from esophageal reconstruction to the revision was 1 month to 15 years with a median of 7 months. Surgical approaches included cervical incision only in 9 patients, cervical incision plus sternotomy in 3, and cervical incision plus partial resection of sternal manubrium in 2. Revisional procedures consisted of excision of scar with reanastomosis in 12 patients, skin graft in 1, and free jejunal graft in 1. After revision, all but 1 patient had excellent results. On the basis of these experiences we conclude that most strictures after pharyngocolostomy or esophagocolostomy can be surgically corrected after excision of the scar and mobilization of the esophageal substitute through a cervical incision only or a cervical incision plus sternotomy.
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PMID:Prevention and management of strictures after hypopharyngocolostomy or esophagocolostomy. 803 6

Nitric oxide (NO), produced by the neural nitric oxide synthase enzyme (nNOS) is a transmitter of inhibitory neurons supplying the muscle of the gastrointestinal tract. Transmission from these neurons is necessary for sphincter relaxation that allows the passage of gut contents, and also for relaxation of muscle during propulsive activity in the colon. There are deficiencies of transmission from NOS neurons to the lower esophageal sphincter in esophageal achalasia, to the pyloric sphincter in hypertrophic pyloric stenosis and to the internal anal sphincter in colonic achalasia. Deficits in NOS neurons are observed in two disorders in which colonic propulsion fails, Hirschsprung's disease and Chagas' disease. In addition, damage to NOS neurons occurs when there is stress to cells, in diabetes, resulting in gastroparesis, and following ischemia and reperfusion. A number of factors may contribute to the propensity of NOS neurons to be involved in enteric neuropathies. One of these is the failure of the neurons to maintain Ca(2+) homeostasis. In neurons in general, stress can increase cytoplasmic Ca(2+), causing a Ca(2+) toxicity. NOS neurons face the additional problem that NOS is activated by Ca(2+). This is hypothesized to produce an excess of NO, whose free radical properties can cause cell damage, which is exacerbated by peroxynitrite formed when NO reacts with oxygen free radicals.
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PMID:The involvement of nitric oxide synthase neurons in enteric neuropathies. 2189 78

Paraneoplastic neurological syndromes (PNS) are rare, remote effects of cancer that are usually caused by an altered immune response to the tumor and not due to the tumor mass, metastasis, infection, ischemia, or metabolic derangements. PNSs can affect any area of the central, the peripheral, and the autonomic nervous systems. These are rare in lymphomas compared with solid tumors attributed to their presentation even in late stages and the absence of onconeural antibodies. We present a child with stage IIB Hodgkin lymphoma who presented with dual PNS, achalasia cardia, and Holmes Adie pupil occurring synchronously with the cancer.
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PMID:Two Uncommon Paraneoplastic Neurological Syndromes in a Child With Hodgkin Lymphoma. 2716 30