1/41. Familial enteropathy: a syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy.We have studied 5 infants with persistent severe diarrhea from birth and marked abnormalities of absorption associated with failure to thrive leading to death in 4 infants. Three had siblings who died and a sibling of a 4th is ill at present, all with a similar illness; 2 were the products of consanguinous marriages. Exhaustive investigation failed to identify a recognized disease entity in any patient. steatorrhea, sugar malabsorption, dehydration, and acidosis were severe in all patients, whatever the diet fed. Total parenteral nutrition was used, but excessive stool water and electrolyte losses persisted even when nothing was fed by mouth. There was no evidence of a hematological or consistent immunological defect in any infant and no abnormalities of intestinal hormones were noted. In the duodenal mucosa of all infants we saw similar abnormalities characterized by villus atrophy, crypt hypoplasia without an increase in mitoses or inflammatory cell infiltrate in the lamina propria and in villus enterocytes absence of a brush border, increase in lysosome-like inclusions, and autophagocytosis. In 3 infants studied by marker perfusion of the proximal jejunum we found abnormal glucose absorption and a blunted response of Na absorption to actively transported nonelectrolytes; in 2 there was net secretion of Na and H2O in the basal state. Our patients evidently suffered from a congenital enteropathy which caused profound defects in their capacity to assimilate nutrients. The similar structural lesion seen in the small intestinal epithelium of all of our cases undoubtedly contributed to their compromised intestinal function, but the pathogenesis of this disorder, if indeed it is a single disease, remains obscure.- - - - - - - - - - ranking = 1keywords = lamina propria, propria (Clic here for more details about this article) |
2/41. Bannayan-Riley-Ruvalcaba syndrome: spectrum of intestinal pathology including juvenile polyps.Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a disorder that includes juvenile polyposis as part of its pathologic spectrum, and it recently has been shown to share phenotypic and genotypic features with Cowden's disease. In existing literature, descriptions of intestinal pathology in patients with BRRS are relatively sparse and occasionally erroneous. We describe the intestinal pathology in multiple specimens from three children with BRRS. Examination of gastrointestinal biopsies from these children revealed predominantly colonic and rectal polyps with the histology of juvenile polyps. Additionally, two cases with clusters of ectopic ganglion cells within the lamina propria, one in a colonic polyp and one in a duodenal biopsy, and an atypical polyp were observed. Bannayan-Riley-Ruvalcaba syndrome should be included in the list of differential diagnostic considerations when a child or young adult presents with a juvenile polyp, particularly if unusual histologic features such as atypical polyps or ectopic ganglion cells are encountered.- - - - - - - - - - ranking = 1keywords = lamina propria, propria (Clic here for more details about this article) |
3/41. Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy.We report the case of a woman with systemic lupus erythematosus initially manifesting with fever, rash and arthritis, and two years later with Class IV lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persistence of symptoms resulted in segmental resection of the ileum which showed widespread myocyte necrosis and active inflammation within the muscularis propria. A subsequent, more extensive ileocolic resection showed severe diffuse atrophy and fibrosis of the muscularis propria throughout the resected bowel. The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy secondary to systemic lupus erythematosus, and may have an auto-immune etiology.- - - - - - - - - - ranking = 0.99584423161842keywords = muscularis, propria (Clic here for more details about this article) |
4/41. Localized amyloid tumor in small bowel.We report a case of localized jejunal amyloidosis occurring in a 74-year-old man who experienced an episode of digestive bleeding while he was receiving oral anticoagulation. It illustrates a rare entity, characterized by an endoscopic aspect of polypoid, pseudotumoral formations. Histologically, submucosal connective tissues, muscularis mucosae, and blood vessel walls are massively infiltrated by amyloid, giving a typical red/green birefringence under polarized light.- - - - - - - - - - ranking = 0.38851829908049keywords = muscularis (Clic here for more details about this article) |
5/41. Jejunal diverticulosis and chronic pneumoperitoneum.A unique case of pneumoperitoneum, without peritonitis or perforation, associated with jejunal diverticulosis is presented. pneumoperitoneum is documented over the course of many years, with recurrence after partial small bowel resection. Jejunal diverticulosis is one of the leading gastrointestinal causes of pneumoperitoneum without peritonitis or surgery. The distended diverticular mucosa may function as a semipermeable membrane allowing transmural gas equilibration.- - - - - - - - - - ranking = 0.00037257075981982keywords = membrane (Clic here for more details about this article) |
6/41. Intestinal spirochetosis in eight pediatric patients from Southern sweden.Intestinal spirochetes in humans have been recognized for more than a century, but it is still a matter of debate whether they are just commensal organisms or whether they cause colorectal disease. Most descriptions to date are of adult patients, while reports in the pediatric literature have been scarce. In a retrospective study we found eight children with intestinal spirochetosis. The findings, clinical as well as pathological, with light- and electron microscopy, are presented. In all patients, a 3 microm-thick layer of spirochetes was visualised on the luminal aspect of the epithelial cells covering the enterocytes and part of the gland openings. In five of the eight cases an inflammatory cell reaction was seen by light microscopy and in one patient a picture suggesting intracytoplasmatically located spirochetes was seen by electron microscopy. Despite partial or complete destruction of microvilli, spirochetes were still able to adhere to the enterocyte membranes. In three children there was a clear correlation between treatment and relief of symptoms. In four there was partial improvement and in one child no change in bowel-related symptoms. We believe that intestinal spirochetes may cause colorectal disease in children. Possible pathogenic mechanisms are discussed.- - - - - - - - - - ranking = 0.00037257075981982keywords = membrane (Clic here for more details about this article) |
7/41. capsule endoscopy: a case study of an 11-year-old girl.Small-bowel disorders have typically been challenging to diagnose because of difficulty visualizing this organ. The recent development of an innovative M2A trade mark Capsule that provides video visualization of the small bowel may have a unique niche in the diagnosis of small bowel disorders. This article highlights a pediatric patient's experience of capsule endoscopy to help the reader more clearly understand the procedure and appropriate nursing care.- - - - - - - - - - ranking = 0.10940381672872keywords = propria (Clic here for more details about this article) |
8/41. Mesenteric ischemia affects young adults with predisposition.An 8-year retrospective review of indiana University hospital records consisting of any patient age 18 to 40 years old who presented with arterial mesenteric ischemia was performed. Three patients were identified that met our criteria. The first patient was discovered to have a protein c deficiency. The second patient was afflicted with afibrinoginemia, a protein C and an antithrombin iii deficiency. The third patient had been previously diagnosed with Takayasu's arteritis and had an elevated ESR. Each patient had a protracted course of symptoms before mesenteric disease was considered, confirmed by angiography, and treated by arterial bypass with/without bowel resection. All patients survived and are currently asymptomatic at an average of 2 years postoperatively. Mesenteric ischemia in patients under the age of 40, especially in the absence of cocaine use, is rare and often causes a delay in diagnosis and appropriate treatment. The high incidence of hypercoagulable states in our study cases suggests the need for a search for such disorders and the possible need for long-term anticoagulation therapy as a deterrent to recurrence.- - - - - - - - - - ranking = 0.10940381672872keywords = propria (Clic here for more details about this article) |
9/41. Megacystis-microcolon-intestinal hypoperistalsis syndrome. Antenatal appearance in two cases.Two cases with severe congenital megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) are presented. This is a rarely encountered syndrome in neonates and 45 cases have earlier been reported. The disease is usually lethal and it now seems clear that MMIHS is an autosomal recessive disorder. The enlarged bladder, typical of this syndrome, is however easy to define by ultrasound, sometimes even in early pregnancy. The concomitant finding of a dilatation of the urinary tract and the absence of oligohydramnios may lead the physician to suspect the diagnosis. Because of the information available from sonography, appropriate investigations can be undertaken immediately after delivery. Prenatal ultrasound examination in subsequent pregnancies is recommended.- - - - - - - - - - ranking = 0.10940381672872keywords = propria (Clic here for more details about this article) |
10/41. Management of recurrent anastomotic stricture and iatrogenic stenosis by circular stapler.Anastomotic stricture is an increasingly common clinical finding. It is thought to arise because of ischemia, disruption, or leakage at an anastomosis site. Its management can be difficult and strictures often are resistant to standard dilation therapy. Major corrective surgery is possible; however, it is technically challenging and not without risk. We have used a circular stapler to excise colorectal strictures, introducing the anvil of the stapler via a proximal stoma or colotomy, drawing the anvil through the stricture with a snare via a colonoscope and affixing it to the body of a circular staple gun and excising the stricture. We have with found this to be an effective treatment in appropriately selected patients.- - - - - - - - - - ranking = 0.10940381672872keywords = propria (Clic here for more details about this article) |
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