Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical features of the multiple mucosal neuromas (MMN) syndrome permit the recognition of these patients and their potential development of the associated medullary thyroid carcinoma (MTC). The distinctive physical appearance caused by the mucosal neuromas, the Marfanoid habitus and, occasionally, the positive family history aid in establishing the diagnosis. Neurogangliomas are frequently present in the gastrointestinal tract of these patients who may have megacolon, constipation and diarrhea. The third instance of the MMN syndrome is reported in the newborn as intestinal obstruction. It is suggested that the syndrome be considered in the differential diagnosis of Hirschsprung's disease and bowel obstruction in the neonate. Serum calcitonin measurements following stimulation by calcium or pentagastrin infusion reliably detect incipient MTC and may be used to select those MMN patients requiring thyroid surgery. Recognition of patients with the MMN syndrome and subsequent calcitonin screening and early surgical intervention will significantly reduce the chance of their developing terminal MTC. All MMN patients with mucosal neuromas or intestinal neurogangliomas should have such evaluations at least yearly. Relatives who are at risk for inheriting this dominant disease should be similarly evaluated, regardless of their normal appearance.
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PMID:Medullary carcinoma of the thyroid in the multiple mucosal neuromas syndrome. 53 32

A case of multiple mucosal neuromata and a calcitonin-secreting medullary carcinoma of the thyroid is described. Unusual features were the neonatal presentation with large bowel obstruction and severe feeding difficulties associated with giant neuromata of the intestine; severe hypotonia; developmental delay; and the early recognition and treatment of an associated medullary carcinoma of the thyroid.
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PMID:Mucosal neuroma syndrome presenting in a neonate. 89 50

Three patients with the carcinoid syndrome received intravenous somatostatin (3.5 micrograms/min) for one day; intravenous salmon calcitonin (8 IU/hr) for one day; subcutaneous salmon calcitonin (100 IU three times daily) for ten days; and subcutaneous octreotide (150 micrograms three times daily) for ten days. Octreotide (SMS-201.995) is a stable analogue of somatostatin. There was a five-day washout period between each treatment. During each of these treatments, reductions in the numbers of daily flushes and bowel movements, stool weight, and urinary 5-hydroxyindoleacetic acid (5-HIAA) levels were observed. Relief of cramping abdominal pains was also reported. Patients 1 and 3 chose to continue receiving the subcutaneous calcitonin and patient 2 chose the octreotide. Patient 1 (aged 67 years) reported relief of symptoms for five months until she developed an intestinal obstruction as a result of tumor infiltration. Patient 3 (aged 67 years) has received the calcitonin for about 16 months with relief of symptoms and reduced urinary 5-HIAA levels. Patient 2 (aged 57 years) has continued octreotide treatment for one year and reports relief of symptoms.
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PMID:Treatment of the carcinoid syndrome with somatostatin, salmon calcitonin, or octreotide. 137 97

Pyloric biopsies obtained at pyloromyotomy from 46 infants were studied by light and electron microscopy and compared to 8 autopsy control cases without any evidence of infantile hypertrophic pyloric stenosis (IHPS). A positive family history of this disorder was recorded in 8 cases (2 girls and 6 boys). The most frequent changes in the myenteric plexus comprised axonal alterations. In glial cells, cytoplasmic vacuolisation or an increase of intermediate filaments occurred. In ganglion cells, vacuolisation of perikaryal cytoplasm or dense bodies were observed. No obvious differences were seen between sporadic and hereditary cases. In addition to these fine structural alterations, immunohistochemistry in 6 IHPS cases and 4 controls revealed differences in the distribution of substance P, bombesin, calcitonin gene related peptide and enkephalin-like immunoreactivity within the myenteric plexus between IHPS and control cases. The immunoreactivity, however, was unevenly distributed from case to case and even within individual cases. The reduction of immunoreactivity corresponded, at least in part, to an increase of neurofilaments or abnormal organelles within axons. Increased immunoreactivity was apparently related to focal accumulation of dense cored vesicles noted in the preceding study. It is suggested that these and other changes reported interfere with the normal gastrointestinal reflex mechanisms leading to intestinal obstruction.
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PMID:Immunohistochemical reactivity of neuropeptides in plastic-embedded semithin sections of the myenteric plexus in infantile hypertrophic pylorus stenosis. 158 86

A 55-year-old man underwent an ileocecal resection because of an intestinal obstruction. The resected specimen contained a sessile tumor near the ileal end. This tumor was 9 x 7 cm in size, with a nodular surface of brown tint. Microscopically, it was a well-differentiated adenocarcinoma, the tumor tissue extending into the muscularis propria. The malignant tissue was associated partially with adenomatous areas and, in malignant nests, with foci of lamellar and intracellular cornification. Further, there was focal ossification in the fibrous stroma. Stains for argyrophil and argentaffin cells revealed strong positive reactions, and immunohistochemical studies were positive for calcitonin and CEA. These findings suggest the multi-potentiality of a small intestinal carcinoma.
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PMID:[Primary ileal adenocarcinoma, showing squamous differentiation and ossification]. 313 24

Multiple Endocrine Neoplasia type 2b (MEN 2b) is a rare syndrome. The principal features are: medullary thyroid carcinoma (MTC), dysmorphism, a ganglioneuromatosis and pheochromocytomas. Eight cases of MEN 2b have been observed at the Institute Gustave Roussy, between 1968 and 1983. Seven involved children under 15 years of age. Eight had a bilateral MTC; six had dysmorphism; six had mucosal tongue neuromas. Six were troubled with visceral ganglioneuromatosis of whom two had intestinal obstruction and one urinary chronic retention. One patient had pheochromocytoma with hypertension. From this experience and other data it appears that: the dysmorphism is frequently poorly interpreted; the visceral ganglioneuromatosis is an early and severe feature; it is important to examine the patient for pheochromocytoma; the MTC must be detected by calcitonin dosage after stimulation, and requires total thyroidectomy; familial screening must be done. To improve the poor prognosis of MEN 2b, early diagnosis and aggressive treatment are necessary.
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PMID:[Multiple endocrine neoplasia type 2b. Clinical, diagnostic and therapeutic aspects]. 614 75

During a study of 80 subjects from the same family, 47 persons underwent clinical examination and blood sampling for carcinoembryonic antigen and thyrocalcitonin. A preliminary investigation had shown that two members of the family had histologically confirmed amyloid stroma MTC, 2 others had probable malignant disease, 3 children died of intestinal obstruction in the neonatal period, and a fourth child operated for megacolon when 3 days old was the descendant of 2 subjects with probable thyroid carcinoma. Three years later, the study was updated and concentrated on the patients with the highest risk of developing malignant disease. The diagnosis was confirmed in one patient considered to have probable MTC. One case of malignant disease was discovered in a child considered to be normal 6 years previously. Three other patients, considered to be normal 6 years before had very probable MTC. These results indicated that familial investigations including plasma calcitonin measurements after pentagastrin stimulation, are valuable when amyloid stroma MTC is diagnosed in one member of the family. This test should be repeated periodically. The surgical indications in patients with abnormal responses should take into consideration the psychological context.
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PMID:[Value of the assay of plasma carcinoembryonic antigen and thyrocalcitonin in the detection of medullary cancer of the thyroid with amyloid stroma. Results of a familial survey of 47 subjects]. 648 13

Three infants, who presented with intestinal obstruction due to diffuse transmural intestinal ganglioneuromatosis, are described. Mutation analysis of exon 16 of the RET proto-oncogene revealed germline M918T and thus, a molecular diagnosis of multiple endocrine neoplasia type 2B (MEN 2B). Two infants developed medullary carcinoma of the thyroid. The third had a prophylactic thyroidectomy despite no obvious thyroid masses and normal calcitonin concentrations, but microscopic multifocal medullary carcinoma was found on histological examination. Early recognition of intestinal ganglioneuromatosis with germline RET M918T mutation in pseudo-Hirschsprung's disease is an indication for prophylactic thyroidectomy.
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PMID:Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2B: implications for treatment. 1036 18

Adhesions in the peritoneal cavity have been implicated in the cause of intestinal obstruction and infertility, but their role in the aetiology of chronic pelvic pain is unclear. Nerves have been demonstrated in human pelvic adhesions, but the presence of pain-conducting fibres has not been established. The purpose of this study was to use an animal model to examine the growth of nerves during adhesion formation at various times following injury and to characterize the types of fibres present. Adhesions were generated in mice by injuring the surface of the caecum and adjacent abdominal wall, with apposition. At 1-8 weeks post-surgery, adhesions were processed and nerve fibres characterized histologically, immunohistochemically, and ultrastructurally. Peritoneal adhesions had consistently formed by 1 week after surgery and from 2 weeks onwards, all adhesions contained some nerve fibres which were synaptophysin, calcitonin gene-related peptide, and substance P-immunoreactive, and were seen to originate from the caecum. By 4 weeks post-surgery, nerve fibres were found to originate from both the caecum and the abdominal wall, and as demonstrated by acetylcholinesterase histochemistry, many traversed the entire adhesion. Ultrastructural analysis showed both myelinated and non-myelinated nerve fibres within the adhesion. This study provides the first direct evidence for the growth of sensory nerve fibres within abdominal visceral adhesions in a murine model and suggests that there may be nerve fibres involved in the conduction of pain stimuli.
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PMID:Growth of nerve fibres into murine peritoneal adhesions. 1105 24

Adjuvant analgesics are defined as drugs with a primary indication other than pain that have analgesic properties in some painful conditions. The group includes numerous drugs in diverse classes. Although the widespread use of these drugs as first-line agents in chronic nonmalignant pain syndromes suggests that the term "adjuvant" is a misnomer, they usually are combined with a less-than-satisfactory opioid regimen when administered for cancer pain. Some adjuvant analgesics are useful in several painful conditions and are described as multipurpose adjuvant analgesics (antidepressants, corticosteroids, alpha(2)-adrenergic agonists, neuroleptics), whereas others are specific for neuropathic pain (anticonvulsants, local anesthetics, N-methyl-D-aspartate receptor antagonists), bone pain (calcitonin, bisphosphonates, radiopharmaceuticals), musculoskeletal pain (muscle relaxants), or pain from bowel obstruction (octreotide, anticholinergics). This article reviews the evidence supporting the use of each class of adjuvant analgesic for the treatment of pain in cancer patients and provides a comprehensive outline of dosing recommendations, side effects, and drug interactions.
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PMID:Adjuvant analgesics in cancer pain management. 1547 43


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