1/44. Autoimmune enteropathy with distinct mucosal features in T-cell activation deficiency: the contribution of T cells to the mucosal lesion.BACKGROUND: Autoimmune enteropathy is normally characterised by crypt hyperplastic villous atrophy with enterocyte autoantibodies, activation of mucosal lymphocytes and increased epithelial HLA-DR. This case involved a severely affected Portuguese infant who was found to have lymphocyte activation deficiency and demonstrated correspondingly distinct mucosal features. methods: A female infant of nonconsanguineous parents was treated for vomiting and diarrhoea, first with milk exclusion and then with parenteral nutrition. lymphocyte subsets and immunoglobulin concentrations were normal, but in vitro testing showed no activation in response to phytohaemagglutinin, candida, or purified protein derivative, although the response to interleukin (IL)-2 was intact. interleukin-2 deficiency was excluded. Analysis of jejunal biopsy specimens revealed only mild villous blunting with absent goblet cells, normal epithelial proliferation, and no crypt hyperplasia. The dense infiltrate of CD8 and CD4 T lymphocytes showed normal CD2 and CD3 expression but no activation or proliferation markers. HLA-DR was not increased on epithelium or lymphocytes. Thus, in addition to in vitro evidence for lymphocyte activation deficiency, the mucosal specimens showed no evidence of in situ T-cell activation. RESULTS: After development of overwhelming septicaemia, the patient died at 18 months, just before a planned bone marrow transplant. CONCLUSIONS: These findings confirm significant heterogeneity within autoimmune enteropathy. Formal immune function testing should be performed in all affected infants to identify T-cell activation deficiencies. The distinct mucosal findings suggest that activated T cells usually induce the crypt hyperplastic villous atrophy characteristic of classic autoimmune enteropathy.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/44. 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 = 0.5keywords = life (Clic here for more details about this article) |
3/44. Internal hernias and gastric perforation after a laparoscopic gastric bypass.A 27-year-old woman underwent laparoscopic Rouxen-Y gastric bypass. A retrocolic-retrogastric herniation of most of the small bowel and later a gastric perforation due to internal hernia at the mesenteric defect of the jejuno-jejunostomy occurred. These unusual, but not rare, complications are directly related to the neoanatomy that follows gastric bypass and can lead to rapidly progressing and life-threatening situations. Proper evaluation of clinical signs and symptoms, early abdominal CT scan, and urgent operative intervention are mandatory to achieve a successful outcome.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
4/44. Intestinal involvement and vasculopathy in von Recklinghausen's neurofibromatosis.Involvement of the gastrointestinal system and vascular lesions are well-known features of von Recklinghausen's disease, but they are rarely detected in childhood. We report a case of von Recklinghausen's disease with intestinal involvement. The excised right hemicolectomy material of a 16-year-old girl was examined, and diffuse neurofibromatous proliferation with ganglioneuromatous features were observed. Vasculopathy was also seen in some arteries throughout the intestinal segment.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
5/44. Superior vena cava thrombosis causing respiratory obstruction successfully resolved by stenting in a small bowel transplant candidate.A 4 year old child was referred for small bowel transplantation. He had superior vena cava obstruction secondary to numerous central venous line placements; alternative routes for long term central venous access were compromised by extensive venous occlusive disease. Patency for the superior vena cava was re-established with stenting, which allowed for radiological placement of another central venous line. Long term survival in infants and young children with intestinal failure is dependent on adequate central venous access for the administration of parenteral nutrition. Line sepsis and physical damage to the catheter often necessitates multiple central venous catheter placements during their early life and these children are at risk of catheter related veno-occlusive disease. Recurrent sepsis and the loss of satisfactory venous access for the administration of parenteral nutrition is life threatening and is an indication for intestinal transplantation in up to 41% of patients reported by the small bowel registry.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/44. Small bowel herniation around an anterior gastropexy for a gastric volvulus: a case report.Gastric volvulus can be a medical emergency with life-threatening complications. Early surgical intervention is important to avoid potential ischemic complication that may lead to infarction of the stomach. The condition has been reported in children and in the elderly, but the majority of cases are reported in the fifth decade of life. We present a case of a complication arising from corrective laparoscopic surgery for gastric volvulus, whereby most of the small bowel herniated around the anterior laparoscopically performed gastropexy. The herniation was reduced during a laparotomy, and the space through which the herniation occurred was closed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/44. secretory component deficiency. A disorder of the IgA immune system.We studied a 15-year-old boy with chronic intestinal candidiasis who had normal serum IgA levels without IgA in his secretions. There was an elevated number of peripheral blood lymphocytes bearing surface IgA. In addition, the lymphocytes cultured in vitro with pokeweed mitogen produced IgA as well as other immunoglubulins. Despite this evidence of normal IgA synthetic capacity, the patient had greatly diminished levels of IgA in the saliva and jejunal fluid, and, as estimated by 14C-L-leucine incorporation, could not synthesize IgA locally at intestinal-mucosal sites. Finally, the patient had no detectable free secretory component in saliva or jejunal fluid in contrast to normal persons and to patients with iga deficiency. The basis of this disorder is probably a defect in the homing of IgA precursor cells to secretory sites or in the selective proliferation/differentiation of IgA cells at such sites.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
8/44. Disseminated aspergillosis inciting intestinal ischaemia and obstruction.Invasive aspergillosis is an opportunistic infection that characteristically affects the immunocompromised host, resulting in a high degree of morbidity and mortality. Although the portal of entry is usually pulmonary, there are rare reports of invasive aspergillosis localized to the gastrointestinal tract. In addition, haematological spread may develop, with life threatening disseminated infection involving the vital organs and the gastrointestinal tract. Although disseminated infection is well recognized, the CT findings of gastrointestinal disease have not been reported to our knowledge. We describe the CT findings in a patient with invasive aspergillosis involving the gastrointestinal tract, which resulted in intestinal ischaemia complicated by small bowel obstruction.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
9/44. Anaesthetic management of a patient with microvillus inclusion disease for intestinal transplantation.We report the anaesthetic management of a 3-year-old-child with microvillus inclusion disease undergoing isolated small bowel transplantation. He required long-term total parenteral nutrition which was complicated with numerous episodes of catheter related sepsis. This resulted in thrombosis of the major blood vessels which critically restricted vascular access available for intravenous nutrition, becoming a life-threatening condition for the patient. Haemodynamic, respiratory parameters and urinary output were well preserved throughout the procedure. Besides a transitory increase in potassium following graft revascularization, biochemical changes were small. Anaesthetic management included comprehensive preoperative assessment, central venous angiography to depict accessibility of central and peripheral veins, assurance of additional vascular access through the intraoperative catheterization of the left renal vein, perioperative epidural analgesia and preservation of splanchnic perfusion to ensure implant viability.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
10/44. Effects of isolated small bowel transplantation on liver dysfunction caused by intestinal failure and long-term total parenteral nutrition.It has not been fully determined whether isolated small bowel transplantation (ISBTx) can reverse liver dysfunction caused by intestinal failure requiring long-term total parenteral nutrition (TPN). A boy with congenital microvillus inclusion disease presented with vomiting and severe diarrhea since the first day of life and had been managed by TPN since then. He suffered from catheter-related sepsis several times. At 14 yr of age he developed progressive hepatosplenomegaly with thrombocytopenia and coagulopathy. He underwent ISBTx with an ileal graft from his blood-identical grandmother at the age of 16 yr. Oral feeding was started on the 14th day after ISBTx and gradually increased. TPN was completely withdrawn after 5 months. liver was palpated 5 cm below the costal margin before ISBTx, while it became non-palpable 5 months after ISBTx. serum liver enzyme levels and prothrombin time normalized in the 5 months following ISBTx. liver biopsy showed marked steatosis, slight cholestasis, and mild bridging fibrosis before ISBTx. Although histological examination of liver biopsy revealed complete disappearance of steatosis 7 and 11 months after ISBTx, liver fibrosis remained unchanged. This clinical experience has shown that although steatosis and cholestasis are reversible after successful ISBTx and withdrawal of TPN, liver fibrosis may remain unchanged.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
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