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)

A female patient with Gardner's syndrome was treated with delta1-testololactone (200 mg daily) because of growth of a large desmoid tumor in the pelvis and lower abdomen and a tumor in a scar from a previous laparotomy. There was also pain and swelling of the left leg. An immediate effect of the drug therapy was complete relief of pain followed shortly thereafter by disappearance of the edema of the leg. After two months, the numerous sebaceous cysts were less prominent. The gross measurements of the diameter of the pelvic and lower abdominal tumor clearly demonstrated tumor shrinkage following therapy. Small polyps scattered over the rectal mucosa and numerous osteomata were not demonstrably affected. After one year of treatment with delta1-testololactone, a laparotomy for partial small bowel obstruction was necessary. Obstruction was caused by the involvement of small bowel mesentery and the bowel itself in a contracted residuum of dense fibrous tissue. Substitution of theophylline and chlorothiazide for the testololactone in Januray 1974 was followed by further diminution of the measurable abdominal and pelvic desmoids. All of these compounds synergize the action of 3',5'-adenosine monophosphate and at least the latter two may function by inhibiting the action of 3',5'-adenosine monophosphate diesterase.
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PMID:Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3',5'-adenosine monophosphate. 16 90

Desmoids are histologically benign but locally aggressive fibrous tumors. Although overall they are rare lesions, they are a common manifestation of Gardner syndrome. We retrospectively reviewed clinical records and CT scans of 25 patients with abdominal desmoids. The number, location, and CT characteristics of the lesions were recorded for each patient. Tumors were solitary in 72% of patients and multiple in 28%. Fifty percent were located in the abdominal wall, 41% in the mesentery, and 9% in the retroperitoneum. More than two thirds of the lesions had well-defined borders, with the remainder displaying an infiltrative outer margin. The majority of tumors had attenuation values equal to (47%) or greater than (41%) the attenuation of muscle on contrast-enhanced CT scans. Complications attributable to the desmoid were commonly detectable on CT (hydronephrosis occurred in 36% and small-bowel obstruction in 20%). Our results detail the spectrum of CT findings and complications caused by abdominal desmoids.
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PMID:Abdominal desmoids: CT findings in 25 patients. 185 6

A case of omento-mesenterial fibromatosis in a 6-year-old boy revealing through acute intestinal obstruction is reported. Mesenterial fibromatosis, also known as aggressive fibromatosis or desmoid tumor is generally associated with Gardner's syndrome but otherwise an extremely rare disease. Fibromatoses do not metastasize but are characterized by a high incidence of local recurrence. Primary treatment is operative. After multiple recurrence irradiation, cytostatics and antiestrogens have been used additionally to control the disease.
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PMID:[Mesenteric fibromatosis in childhood]. 321 Nov 72

Patients with familial polyposis coli or Gardner's syndrome are at risk for a variety of extracolonic manifestations. In a series of patients followed at the University of Washington, we have found several recurring and unusual manifestations, including upper gastrointestinal polyposis, small bowel obstruction secondary to desmoid tumors or adhesions, recurring pancreatitis, and adenoma of the papilla of Vater. In one family with familial polyposis only, a set of twins had different manifestations; one twin had familial polyposis only, whereas the second had classic extracolonic manifestations of Gardner's syndrome. Multiple rectal adenomas developed in a woman with an ileorectal anastomosis with each of three pregnancies. Spontaneous regression occurred after each delivery. Multiple rectal adenomas developed in her daughter on two occasions while taking birth control pills. Physicians caring for these patients should look for such manifestations.
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PMID:Unfamiliar aspects of familial polyposis coli. 372 22

Mesenteric desmoid tumors are a recognized sequela of colectomy for polyposis coli of Gardner's type. Relentless growth and recurrence carry a poor prognosis. Recently, nonsteroidal anti-inflammatory drugs have been used to halt the growth of these tumors, presumably by interfering with prostaglandin metabolism. A 36-year-old man presented with small-bowel obstruction secondary to a large, diffuse mesenteric desmoid six years following colectomy and ileoproctostomy. Laparotomy revealed it to be unresectable. Postoperatively, he was started on sulindac (Clinoril) 100 mg twice a day. His obstruction resolved, and he remains well at 11 months. A CT scan shows diminution in the size of the tumor. Nonsteroidal anti-inflammatory agents may be an alternative to chemotherapy and radiotherapy in treating mesenteric desmoids.
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PMID:Mesenteric desmoid tumor in Gardner's syndrome treated by sulindac. 669 Feb 65

Intra-abdominal desmoid tumors are known to be associated with familial polyposis coli, but have not been previously described in association with carcinoma of the sigmoid colon. This is a case report of a patient with intra-abdominal desmoid tumor associated with carcinoma of the sigmoid colon which manifested as a small-bowel obstruction. This appears to be the first case of its kind reported.
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PMID:Intra-abdominal desmoid tumor. 741 82

The authors briefly describe the principal late complications of ileo-anal anastomoses: anal fistulae, anal strictures, bowel obstruction, gallstones, renal stones, desmoid tumours. The aetiological hypotheses and the therapeutic possibilities are analysed for each complication.
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PMID:[Late complications of proctocolectomy treated by ileo-anal anastomosis]. 816 Nov 46

Abdominal desmoid tumors (ADT) have become an important problem because of the increased survival of patients with familial adenomatous polyposis (FAP). Of 240 patients operated upon for FAP between 1978 and 1991, 29 (16 men and 13 women) had ADT. Diagnosis was made at laparotomy in 19 patients, while an abdominal mass or intestinal obstruction was the principal cause of discovery in the ten remaining patients. Twenty ADT were discovered after a previous laparotomy performed an average of three years earlier. Nine ADT occurred in 146 patients who had previously undergone a coloproctectomy and ileal pouch-anal anastomosis and eight occurred in 74 patients who had abdominal colectomy and ileorectal anastomosis. Desmoid tumors were responsible for eight instances of intestinal obstruction and one instance of small intestinal perforation. One death was directly related to ADT. In four of nine patients, ADT was responsible for a deterioration of the functional results of ileoanal anastomosis, but pouch removal was not necessary. Conversion of ileorectal anastomosis to ileoanal anastomosis and excision of carcinomas that occur in the residual rectum after ileorectal anastomosis were impossible because of ADT in three and two patients, respectively. Complete surgical removal of the tumor was possible in seven patients only and four patients had a recurrence. None of the medical therapies used (sulindac, tamoxifen and chemotherapy) were effective. The results of this study confirm the high incidence, severity and absence of effective treatment of ADT in FAP. Desmoid tumors occurring after ileorectal anastomosis seem to be more severe than after ileoanal anastomosis because, in the former instance, they may interfere with the further management of the rectal stump.
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PMID:Operation and abdominal desmoid tumors in familial adenomatous polyposis. 839 84

Gardner's syndrome is a genetic disease that carries a 100% risk of malignant degeneration if left untreated. The syndrome is characterized by the presence of numerous colon polyps, along with a wide variety of extracolonic manifestations. The presence of these extracolonic manifestations increase morbidity and make treatment more difficult. The surgical procedure commonly used to manage the disease is total colectomy with creation of an ileoanal reservoir. The incidences of intestinal obstruction, genitourinary obstruction, septicemia, and fistula formation increase with the growth of desmoid tumors, which are one of the extracolonic manifestations of the disease. The ET nurse plays an essential role in the care of the patient with Gardner's syndrome. Whether acting as a consultant or primary care provider, the ET nurse provides emotional support, educates, and establishes a plan of care to manage the ostomies, skin problems, complex wounds, and fistulas associated with Gardner's syndrome.
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PMID:The role of the ET nurse in managing Gardner's syndrome. 884 93

The management of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis (FAP) is very difficult. Non-steroidal anti-inflammatory drugs (NSAIDs), anti-estrogenic agents, and steroids are most commonly used, because surgical removal of these tumors may result in severe morbidity, with local recurrence being common. We report a patient with FAP and intra-abdominal desmoid tumors that regressed markedly after prednisolone therapy. The patient, a 38-year-old woman, had undergone total colectomy and ileorectal anastomosis with a diagnosis of FAP with colon cancer. Approximately 17 months after the surgery, she noticed an elastic firm lump in the abdominal wall. She also experienced lower abdominal distension. Computed tomography (CT) of the lower abdomen showed an invasive heterogenous low-density mass occupying the intra-abdominal space. She was treated with sulindac, NSAID, at 300 mg/day, the diagnosis being intra-abdominal desmoid tumors. She exhibited an intestinal obstruction about 9 months after the initiation of sulindac therapy. We changed the treatment and began prednisolone (initial dose, 40 mg/day). This treatment was continued for two years; subsequently, the lesions regressed markedly. She is currently well, more than 3 years after the withdrawal of prednisolone.
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PMID:Prednisolone therapy for intra-abdominal desmoid tumors in a patient with familial adenomatous polyposis. 908 78


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