1/217. 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 = disease (Clic here for more details about this article) |
2/217. A morbid course in a girl with mixed connective tissue disease.We describe an 18-year-old girl with a 13-year history of mixed connective tissue disease whose clinical course was unique: she ultimately developed end-stage kidney and bowel disease that led to a protracted morbid clinical course. We report this case to alert pediatric nephrologists to the importance of early recognition of possible intestinal disease in these patients. Unfortunately, no therapy is currently known to reverse the pathological process in the bowel, but multiorgan transplantation might be an option if the numerous medical complications of end-stage bowel disease can be successfully controlled.- - - - - - - - - - ranking = 4keywords = disease (Clic here for more details about this article) |
3/217. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis).We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease.- - - - - - - - - - ranking = 3.5keywords = disease (Clic here for more details about this article) |
4/217. A case of pediatric Behcet's disease with intestinal involvement.We report a 6-year-old girl with recurrent oral aphthae, genital and perianal ulcers, and folliculitis-like erythema followed by intestinal symptoms. No ocular lesions were found. Her ailment was diagnosed as incomplete Behcet's disease with intestinal involvement. According to the literature, pediatric Behcet's disease is characterized by a low incidence of ocular lesions and a high incidence of intestinal involvement, as exhibited in this case. As intestinal lesions in pediatric Behcet's disease are often life-threatening, a barium enema must be given to exclude the possibility of intestinal involvement when pediatric patients with Behcet's disease complain of abdominal pain.- - - - - - - - - - ranking = 4keywords = disease (Clic here for more details about this article) |
5/217. immunoblastic lymphadenopathy-like T-cell lymphoma complicated by multiple gastrointestinal involvement.We report a rare case of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma complicated by multiple gastrointestinal involvement, which appeared to be ameliorated by chemotherapy but resulted in perforative peritonitis. A 66-year-old Japanese woman who had generalized lymphadenopathy and eruptions was admitted to our hospital because of bloody stool. Colonoscopic examination revealed hemorrhagic ulcers in the terminal ileum and a saucer-like ulcer in the cecum. Gastrointestinal endoscopy revealed several ulcerative or elevated lesions in stomach and duodenum. biopsy specimens of these lesions and of a lymph node showed characteristic histological features of IBL-like T-cell lymphoma. The initial treatment with prednisolone (PSL) and cyclophosphamide (CPA) was effective. Six months after the treatment, however, she developed bloody stool again caused by multiple ulcerative lesions in the large intestine. The recurrence of the disease was determined histologically, and four courses of CPA, PSL, vinblastine sulfate and doxorubicin hydrochloride (CHOP) therapy were administered. One month after completing the CHOP therapy, she developed intestinal obstruction and then acute peritonitis resulting from perforation at an ulcer scar in the jejunum. Surgical treatment was successful, and histological examination demonstrated no lymphoma cells in the resected specimen. A gastrointestinal perforation should be recognized as a potential complication of IBL-like T-cell lymphoma, even during remission.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
6/217. Abdominal cerebrospinal fluid pseudocyst in a severely handicapped patient with hydrocephalus.We present a case of an abdominal cerebrospinal fluid pseudocyst as a rare complication of a ventriculoperitoneal shunt. The patient is a severely handicapped bedridden spastic quadriplegic with hydrocephalus. He underwent surgical reconstruction of a shunt tube because of shunt disconnection at the age of 12 years. fever and frequent vomiting developed 2 months after surgery, and abdominal fullness gradually became severe. He was diagnosed as having an abdominal cerebrospinal fluid pseudocyst on abdominal ultrasonography. If a shunt dysfunction is suspected, this type of cyst should also be considered, and abdominal ultrasonography should be performed as one of the screening tests for determining the cause of the shunt dysfunction.- - - - - - - - - - ranking = 0.0019193206965236keywords = tube (Clic here for more details about this article) |
7/217. Posttraumatic intestinal stenosis presenting as a perforation: report of a case.A 78-year-old woman was admitted to the hospital after falling into a ditch approximately 1 m deep and sustaining a blunt abdominal trauma with a left femur fracture. On the tenth day after admission, symptoms of a small bowel obstruction occurred. A nasogastric tube was inserted, and the symptoms thus improved. She sometimes complained of abdominal pain during the 12 months after the fall, but recovered with conservative management. The next year, she was readmitted to the hospital for a pin extraction of the left femur bone. During this admission, 15 months since her admission after her fall, she again complained of abdominal pain. abdominal pain increased with a muscular defense, and abdominal x-rays revealed free air. She was referred to our hospital with a diagnosis of perforative peritonitis, and emergency surgery was performed. Upon laparotomy, circumferential stenoses of the small bowel were recognized in the proximal segments about 40cm, 80cm, and 100cm from the ileocecal region. In addition, a perforation and prominent dilatation of the bowel segment was observed just proximal to the stenosis about 100cm from the ileocecal region. She underwent a small intestinal resection at two sites. There were no findings of an intestinal specific ulcer, such as Crohn's disease, intestinal tuberculosis, or malignancy, based on the results of a histopathological examination.- - - - - - - - - - ranking = 0.50383864139305keywords = disease, tube (Clic here for more details about this article) |
8/217. Unusual presentation of graft-versus-host disease in pediatric liver transplant recipients: evidence of late and recurrent disease.Graft-versus-host disease (GvHD) is a multi-organ disease caused by mature donor T cells that are activated by alloantigens expressed by the host antigen-presenting cells. GvHD has been reported after solid organ transplantation with two principal presentations: humoral and cellular. In the cellular type of GvHD after liver transplantation the symptoms are identical to the GvHD after bone marrow transplant, except that the liver is spared because it lacks host antigens. We have described three cases of intestinal GvHD after pediatric liver transplant with an unusual recurrent late presentation in two patients. Two patients were female, and their age at the time of transplant was 8 and 9 months, respectively, and one was an 8-month-old male. They all received reduced liver allografts of identical blood type from three different donors. One patient received two doses of donor bone marrow cell infusion. Two patients received double immunosuppressive therapy constituted by tacrolimus at a dose of 0.05 mg/kg p.o. b.i.d. and steroids 10 mg p.o. daily. One patient received a triple drug immunosuppression with tacrolimus (0.05 mg/kg p.o. b.i.d.), steroids (10 mg p.o. daily) and mycophenolate mofetil (125 mg p.o. b.i.d.). diagnosis of intestinal GvHD was confirmed histologically on intestinal biopsies performed at the time of presentation of the clinical symptoms or at autopsy.- - - - - - - - - - ranking = 5keywords = disease (Clic here for more details about this article) |
9/217. Stool smears for diagnosis of intestinal acute graft-versus-host disease.A patient with severe diarrhea was successfully diagnosed as having acute intestinal GVHD on stool smear through detection of detached intestinal epithelial cells with apoptosis. Since a stool smear can be easily obtained non-invasively, it is a possible tool for the diagnosis of acute intestinal GVHD.- - - - - - - - - - ranking = 2keywords = disease (Clic here for more details about this article) |
10/217. Intestinal angioedema mimicking Crohn's disease.angioedema usually presents as episodic attacks of swelling of the face, airway and extremities, but it may also involve visceral tissues. A 58-year-old woman with repeated episodes of abdominal pain, nausea and vomiting had two laparotomies and was treated for Crohn's disease for two years before a diagnosis of acquired intestinal angioedema was made. This case provides important insights into the presentation of intestinal angioedema.- - - - - - - - - - ranking = 2.5keywords = disease (Clic here for more details about this article) |
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