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Query: UMLS:C0021933 (intussusception)
 3,822 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Peutz-Jeghers syndrome is a familial disease characterized by mucocutaneous pigmentation and intestinal polyposis. Small-intestinal intussusception is a common complication. Preservation otion technique to prevent intussusception is described.
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PMID:Prevention of intussusception in Peutz-Jeghers syndrome. 46 86

Peutz-Jeghers syndrome is a familial disease characterized by spots on the lips and intestinal polyposis. A review of the history and the anatomic features of the disease are presented. One of us (AJM) has the largest personal series in the literature with twenty patients in five generations. Pedigree charts and aspects relating to the development and interrelationships of spots and polyps are explained. A previously unreported instance of colon intussusception is presented along with a discussion of the clinical presentation and the malignant potential.
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PMID:Peutz-Jeghers syndrome: experience with twenty patients in five generations. 59 34

A rare case of recurrent cecocolic intussusception in an adult patient with multiple lymphomatous polyposis of the gastrointestinal tract is presented. Clinical features, especially the difficulty in distinguishing this entity from adenomatous polyposis on colonoscopy, and histopathology are discussed. It is important that surgeons and colonoscopists be aware of this rare form of diffuse gastrointestinal lymphoma because of therapeutic implications.
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PMID:Cecocolic intussusception in multiple lymphomatous polyposis of the gastrointestinal tract. Report of a case. 232 32

Peutz-Jeghers syndrome is a disease manifested by a combination of mucocutaneous pigmentation and gastrointestinal (GI) polyposis. The major morbidity results from intussusception, obstruction, and bleeding. Standard surgical management has been to perform enterotomies at the site of palpable polyps. A method of treating Peutz-Jeghers syndrome surgically with combined intraoperative enteroscopy is presented. The patient had eight large polyps that required three enterotomies for removal. Only three of the polyps were palpable. Two intussusceptions were reduced. The advantages of intraoperative enteroscopy are that 1) it provides accurate assessment of the extent of the disease, 2) smaller polyps can be treated endoscopically, preventing enterotomies, and 3) the endoscopist can direct the surgeon to the appropriate enterotomy sites. A combined surgical and endoscopic approach for the management of Peutz-Jeghers is successful. It more accurately removes the cause of the major morbidity associated with the disease and may allow the patient a longer interval between laparotomies.
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PMID:Peutz-Jeghers syndrome. A call for intraoperative enteroscopy. 233 77

A review was conducted of children with intussusception admitted to the Royal Children's Hospital over a 16 year period: of 630 episodes of intussusception, 28 represented recurrences (4.4%): 10 of these occurred in children aged 2 years or over. Duration of symptoms and the incidence of an incorrect initial diagnosis were markedly reduced with recurrence. The clinical presentation was similar to the first episode apart from rectal bleeding which was less common, reflecting the shorter history. Sixteen barium enemas were performed, of which 10 were successful. There were 18 operations with eight resections; seven for localized lesions and one for failure of manual reduction in a patient with an inverted Meckel's diverticulum. Barium reduction for recurrence should not be attempted in children over 2 years of age in whom no laparotomy was performed for their first episode, for most second recurrences, or in multiple polyposis.
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PMID:Recurrent intussusception: barium or surgery? 347 5

Generalized juvenile polyposis occurred in five patients (age range, 18 months to 16 years). Clinical findings included abdominal pain, weakness, rectal bleeding, diarrhea, rectal prolapse, intussusception, clubbing, and failure to thrive. Laboratory findings included anemia, hypoalbuminemia, hypokalemia, and skin test anergy. Diagnosis is achieved by double contrast enema, endoscopy, and biopsy. Unlike patients with solitary juvenile polyps, patients with generalized involvement require surgical intervention. Subtotal colectomy and ileoproctostomy are the procedures of choice, and we performed them in four cases. An ileoanal-endorectal pull-through procedure was required in one patient with continued rectal disease. All five patients are currently alive and well. Long-term follow-up is important as polyps may persist into adult life. Family members are at risk for developing gastrointestinal tract tumors and should be screened.
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PMID:Generalized juvenile polyposis coli. Clinical management based on long-term observations. 370 30

During the past 30 years, authors observed and followed 5 patients with Peutz-Jeghers' syndrome. Four of them had diffuse polyposis of stomach, small bowel and colon. They also had severe clinical presentation of the disease, with recurring colicky pain, haemorrhage, anaemia and intussusception, all of which necessitated frequent surgical treatment. Excised polyps presented as benign hamartomas, without malignant alteration. Mucocutaneous pigmentations were present in 4 patients. Family history was revealing in only 2 cases. One patient, a girl aged 2 years, died due to the complications of the surgical intervention (intestinal obstruction). She has had the most severe form of the disease with diffuse polyposis.
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PMID:Peutz-Jeghers' syndrome--juvenile intestinal polyposis--review of five cases. 373 31

Over an 18-year period we have diagnosed nodular lymphoid polyposis of the intestinal tract in 6 patients. The site of the polyposis, which was due to prominent lymphoid hyperplasia, was ileal (3), colonic (2), and rectal (1). The diagnosis was made following complications arising from the polyps, which included recurrent intussusception (2), rectal prolapse (1), intestinal or pseudointestinal obstruction (2), and rectal bleeding (1). Immunoglobulin staining was performed on all the bowel specimens and in every case secretory IgA was present on the mucosal surfaces and IgG and IgA were seen in the lamina propria, thus excluding immunodeficiency in these patients. Viral studies were performed in 3 patients and all were positive. In one patient Echovirus II was seen in tissue homogenate from a mesenteric lymph node and in another, adenovirus type II was cultured from lymphoid polyps of the rectum. A further patient had positive serological tests for adenovirus. Thus it appears that nodular lymphoid hyperplasia is part of the generalized lymphoid hyperplasia associated with viral infections in infancy and childhood. Immunodeficiency states as a cause of the lymphoid hyperplasia should always be excluded by estimation of serum immunoglobulins.
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PMID:Nodular lymphoid hyperplasia of the intestinal tract in infancy and childhood. 397 8

The authors present their experience on a case of Peutz-Jeghers syndrome. Initially described in 1921, the association of intestinal polyposis with pigmentation of the skin and mucous membranes has been called the Peutz-Jeghers syndrome; the pigment, which is melanin, usually involves the lips and mouth and at times the face and extremities. The syndrome appears to be a hereditable disease. Polyps may be present in the stomach, small bowel and colon, but are most frequent in the small bowel, where they produce intussusception or bleeding. Our patient, a 35-year-old man, presented abdominal pain and recurrent intestinal bleeding. Our aim was to evidence radiological signs of this syndrome. the radiographic examination of small bowel, showing multiple polyps and signs of intussusception, definitely confirmed the diagnosis. The Authors finally discuss the possibility of other syndromes associated with gastrointestinal polyposis such as familial polyposis, Cronkhite-Canada syndrome, Gardner syndrome etc.
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PMID:[Radiological aspect of Peutz-Jeghers syndrome: considerations on a case]. 621 Oct 21

Cystic fibrosis is the most common fatal inherited disease of Caucasians. At present, cystic fibrosis accounts for most cases of chronic progressive pulmonary disease and for many other clinical features in the first three decades of life. Thus, it is a challenge to both pediatricians and internists, particularly chest physicians. The diagnosis is based on the triad of chronic obstructive pulmonary disease, pancreatic insufficiency, and increased levels of electrolytes in the sweat. The cardinal test for confirmation of the diagnosis is the "sweat test," which is an excellent discriminant for cystic fibrosis, even in adults. Ancillary features of cystic fibrosis may be of diagnostic assistance (eg, nasal polyposis, Pseudomonas aeruginosa in sputum, azoospermia, and others). Treatment of the pulmonary disease must be emphasized. Choice of antibiotics should be based on the results of sputum culture, but P aeruginosa is the most common pathogen. Removal of secretions by regular postural drainage and percussion is an integral part of the program. Pneumothorax, massive hemoptysis, cor pulmonale, and other complications may be encountered. Sinusitis is almost universal, and nasal polyposis is frequently present. Pancreatic insufficiency occurs in over 80 percent of the patients with cystic fibrosis and may result in intestinal malabsorption. Massive salt loss through the sweat in hot weather, a distinctive type of biliary cirrhosis without jaundice, gallbladder abnormalities, cholelithiasis, and diabetes mellitus also may be found. Of special importance are intestinal obstructive complications (meconium ileus in newborn infants with cystic fibrosis and intestinal obstruction due to fecal accumulation or intussusception in adults). Azoospermia is present in 95 percent of men and there is reduced fertility in women; however, pregnancy does occur in cystic fibrosis. This chronic and ultimately fatal disease produces a predictable set of psychosocial complications.
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PMID:Diagnosis and treatment of cystic fibrosis. An update. 637 70


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