1/145. 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) |
2/145. Persistent crying as predominant manifestation of sepsis in infants and newborns.Acute episodes of unexplained crying in infants may be due to serious and even life-threatening conditions. We present six infants in whom excessive crying was the predominant initial manifestation of sepsis for a period of time that ranged from 2 to 10 hours, before other symptoms or signs became evident. This led to a diagnostic delay in two patients who were considered initially to have infant colic. sepsis should be considered in the differential diagnosis of acute unexplained crying in infants.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
3/145. life-threatening retroperitoneal sepsis after hemorrhoid injection sclerotherapy: report of a case.We present a case of life-threatening retroperitoneal sepsis after injection sclerotherapy for first-degree hemorrhoids.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
4/145. Lemierre's syndrome.Postanginal sepsis, or Lemierre's syndrome, is rare but with life-threatening potential involving mainly infants and adolescents. The morbidity or mortality is caused mainly by lack of knowledge of the syndrome. The 18-year-old boy described here developed a jugular thrombosis 7 days after an angina. fusobacterium necrophorum was isolated from the culture of the excised jugular vein. Secondary embolism involved the lungs, associated with an iliac osteomyelitis and sacroiliitis. Computed tomography was used for diagnosis and follow-up.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
5/145. Tunneled right atrial catheter infection presenting as renal failure.We report two cases of progressive renal failure secondary to membranoproliferative glomerulonephritis associated with subclinical septicemia from a tunneled right atrial catheter used for home parenteral nutrition administration. Although the occurrence of line infection and septicemia is a common complication of central venous catheters, a review of the literature reveals only one case report of renal failure secondary to an infected implanted central venous device. Both patients presented with azotemia and had biopsy-proven membranoproliferative glomerulonephritis, accompanied by leukocytoclastic vasculitis. In both cases, removal of the right atrial catheter and prolonged antibiotic therapy was effective in resolving the ongoing infection and resulted in marked improvement in renal function. A high index of suspicion for catheter sepsis should be maintained in patients with tunneled right atrial catheters presenting with subacute renal failure.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/145. gas gangrene in a patient with severe haemophilia A.The haemophilia patient tends to live a more protected life than his normal counterpart, this is particularly so in underdeveloped and developing countries where due to poor health infrastructure, financial constraints and nonavailability of factor concentrates, patients quickly learn that they need to live a protected life. Under such circumstances, gas gangrene seems to be a very unusual infection for this group of patients. We report here a 25-year-old male with severe haemophilia who developed gas gangrene due to inadequate medical management following a road traffic accident. Subsequently, his affected limb was salvaged by conservative therapy. A literature search failed to reveal any reports of similar patients in the English literature.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/145. 46,XY gonadal dysgenesis associated with congenital nephrotic syndrome and sepsis.The occurrence of nephrosis in the first 3 months of life is rare and is termed 'congenital nephrotic syndrome.' The congenital nephrotic syndrome is a group of heterogeneous diseases with a clinical course that differs markedly from the childhood nephrotic syndrome. The coexistence of a congenital nephrotic syndrome and gonadal dysgenesis in a 46,XY karyotype with normal female external genitalia is extremely rare. Frequent severe infections are often seen in the Finnish type, but sepsis leading to death is rare in the neonatal onset of gonadal dysgenesis. This report describes an unusual case of complete XY gonadal dysgenesis in a 46,XY female neonate with the congenital nephrotic syndrome and overwhelming sepsis.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
8/145. lemierre syndrome in an immunocompromised patient.lemierre syndrome or septic thrombophlebitis of the internal jugular vein is a potentially life-threatening complication. This condition may result from oropharyngeal infection, central venous catheterization, and intravenous drug abuse. Immunocompromised patients and individuals with systemic disease are at higher risk of developing the syndrome. We present here a case of septic jugular vein thrombosis in an adolescent with systemic lupus erythematosus. The etiological factors, pathogenesis, and diagnostic and therapeutic measures are discussed.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
9/145. Fatal cerebroembolism from nonbacterial thrombotic endocarditis in a trauma patient: case report and review.Nonbacterial thrombotic endocarditis (NBTE) is a rare condition that may result in an unexpected and usually fatal cerebroembolism. It occurs in a variety of clinical situations, including malignancy, immune disorders, and sepsis, but it has rarely been reported after trauma. The formation of NBTE appears to require a hypercoagulable state and changes in valvular morphology, e.g., during a hyperdynamic state. patients with disseminated intravascular coagulation have a 21% incidence of NBTE. Although NBTE is usually found at autopsy, premorbid detection by echocardiography is currently possible and feasible. Untreated patients have a high incidence of embolic events, but anticoagulation with heparin may be life-saving. A lethal case of NBTE in a severely injured patient is reported here with the purpose of increasing awareness among medical personnel caring for trauma patients. Recommendations have been made for surveillance echocardiography in high-risk patients, e.g., critically ill patients with sepsis or disseminated intravascular coagulation.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
10/145. Congenital neutropenia. Report of a case and a biorationale for dental management.Congenital neutropenia is characterized by a marked decrease in or lack of circulating PMN's in children with no prior history of drug intake. The neutropenia is persistent and the clinical course is one of early onset of severe, recurrent, and eventually fatal infections. bone marrow studies show a maturation arrest of neutrophilic precursors. Because of their greatly increased susceptibility to infection, patients with congenital neutropenia present a difficult dental management problem. A case of congenital neutropenia has been presented, as well as a biorationale for dental treatment. On the basis of reports in the literature, the following recommendations for the management of patients with congenital neutropenia are made: 1. The prevention and control of infection and the interception of dental disease before surgical intervention becomes necessary should be the overriding considerations in the management of patients with congenital neutropenia. 2. The carious breakdown of teeth should be prevented by the daily application of a 0.4 per cent stannous fluoride gel in addition to oral hygiene and limitation of sucrose intake. 3. Periodontal therapy should be palliative only, since alveolar bone loss is progressive despite frequent oral hygiene instruction and prophylaxis. The goal of periodontal therapy for patients with congenital neutropenia should therefore be a decrease in gingival inflammation to make the patient's mouth more comfortable and to slow down alveolar bone loss. Periodontal surgery is contraindicated. 4. bacteremia and subsequent septicemia should be prevented since a minor infection can become life threatening in patients with congenital neutropenia. The patient should rinse for 30 seconds and the gingival sulci should be irrigated with a phenolated antiseptic mouthwash prior to all dental manipulations of the soft tissue. This will significantly reduce the incidence of bacteremia. 5. Surgery should be avoided if at all possible because of the high risk of post-operative infection. All surgery sholld be performed in the hospital, and the patient should be given antibiotics as determined by his physician. Primary closure should be done with fine polyglycolic acid sutures to reduce the chance of infection. If postoperative infection can be prevented, wound healing will progress normally despite the complete absence of PMN's.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
| | Next -> |