1/85. Two separate episodes of hemophagocytic syndrome at a two-year interval in an apparently immunocompetent male.We describe two separate episodes of hemophagocytic syndrome (HPS) at an interval of two years in a seemingly immunocompetent male. This case suggests the possible existence of an inherent predisposition to HPS, in which otherwise negligible self-limited viral infection may trigger HPS. Laboratory data for a 16-year-old boy admitted with persistent high grade fever and severe thrombocytopenia disclosed coagulation abnormality, liver damage, and hypercytokinemia. A bone marrow aspiration revealed a proliferation of histiocytes with fresh hemophagocytosis. We diagnosed that he was suffering from HPS. Responding to steroid pulse therapy, he recovered completely and was discharged. After two years of healthy life, he became febrile again and was readmitted. The fever was refractory to antibiotics and was associated with a sudden drop in platelet count. Laboratory data and the bone marrow picture were consistent with those of HPS. He was again successfully treated with steroid. After the second episode, he has been healthy for more than two years.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
2/85. Leukocyte functions in 2 cases of Papillon-Lefevre syndrome.AIM: To investigate the role of leukocytes in the pathogenesis of Papillon-Lefevre syndrome (PLS). methods: Peripheral blood polymorphonuclear neutrophils (PMNs), monocytes (MNs) and gingival crevicular fluid (GCF) were obtained from 2 cases of PLS with typical features. The chemotaxis of PMNs and MNs were evaluated using a modified Boyden chamber. The adherence of PMNs was determined by adherence of PMNs to petri dishes. interleukin-8 (IL-8) in GCF was detected by sandwich ELISA. Elastase activity in GCF was measured with a low molecular weight substrate (S-2484) specific for granulocyte elastase. RESULTS: PMNs from both patients showed depressed chemotactic response to FMLP and IL-8. Total amounts of IL-8 in GCF from the 2 patients were much higher than those of the normal controls. Elastase activity was not significantly different from that of the controls. The adherence of PMN and the chemotaxis of MN in the 2 patients were normal. CONCLUSION: The depressed chemotactic response of PMN leads to decreased recruitment of PMN and/or release of lysozyme from PMN in the diseased gingival tissue, increasing the susceptibility of PLS patients to periodontal infection.- - - - - - - - - - ranking = 0.2keywords = rate (Clic here for more details about this article) |
3/85. Primary pseudomonas meningitis in an adult, splenectomized, multitransfused thalassaemia major patient.A 19-year-old splenectomized, multitransfused female patient with beta-thalassaemia major developed primary meningitis due to P. putida. Her blood cultures were negative. P. putida is an unusual nosocomial organism to cause primary meningitis. infection due to this organism carries high mortality. However, owing to early diagnosis and energetic treatment this patient survived without any sequelae. A review of serious infections over the last 7 years in patients in our thalassaemia care centre revealed 11 serious infections among our splenectomized patients (n = 46) and none in the non-splenectomized group (n = 106). Surprisingly, all overwhelming infections (23.8% in the splenectomized group) were caused by Gram-negative bacilli like klebsiella, pseudomonas, aeromonas and campylobacter species. As all our splenectomized patients had prior pneumococcal vaccination and oral penicillin prophylaxis, overwhelming septicaemia due to S. Pneumoniae was successfully prevented, but an increasing incidence of overwhelming sepsis due to Gram-negative bacilli, against which no vaccination or suitable prophylactic antibiotics are available, is now posing a new threat to this vulnerable group of patients.- - - - - - - - - - ranking = 1.3975866979401keywords = mortality (Clic here for more details about this article) |
4/85. Good's syndrome presenting with cytomegalovirus pneumonia.A 61-year-old woman who had undergone an operation for thymoma 17 years previously suddenly became dyspneic and showed bilateral pulmonary infiltrates on a chest radiograph. In the bronchoalveolar lavage fluid cells contained characteristic cytomegalic inclusion bodies, as well as cytomegalovirus dna demonstrated by a polymerase chain reaction. Immunological findings included hypogammaglobulinemia, deficient numbers of circulating B cells, and impaired blast transformation of peripheral blood T cells in response to mitogens in vitro. Considering all of the findings, the patient was diagnosed with Good's syndrome presenting with cytomegalovirus pneumonia.- - - - - - - - - - ranking = 0.4keywords = rate (Clic here for more details about this article) |
5/85. Synchronous high-risk melanoma and lymphoid neoplasia.Large population-based studies have shown a significant association between melanoma and lymphoid neoplasia, particularly non-Hodgkin's lymphoma (NHL) and chronic lymphocytic leukaemia (CLL), that is independent of any treatment received for the initial tumour. This study examines the presentation, diagnosis, treatment and progress of three patients who developed advanced melanoma concurrently with a lymphoid neoplasm (one NHL, two CLLs), in order to illustrate their association, discuss common aetiological factors and examine possible therapeutic options. As it is the melanoma rather than the lymphoid neoplasm that represents the bigger threat to overall survival, initial treatment should be targeted towards this cancer. However, because of the interplay between the diseases and the possible side-effects of the various treatments, the choice of adjuvant therapy requires careful consideration. immunosuppression associated with chemotherapy may permit a more aggressive course for the melanoma, while locoregional radiotherapy is contraindicated following lymph node dissections. As immunotherapy is of benefit in the treatment of melanoma and has also been recently shown to be effective in the management of lymphoid neoplasia, we instituted interferon-alpha as adjuvant therapy for these patients, thereby utilizing a single agent to treat the dual pathologies. The three patients have now been followed-up for 6 months without evidence of disease recurrence or progression.- - - - - - - - - - ranking = 0.2keywords = rate (Clic here for more details about this article) |
6/85. Immunologic and virologic analyses of an acutely hiv type 1-infected patient with extremely rapid disease progression.The immunologic and virologic factors that impact on the rate of disease progression after acute infection with human immunodeficiency virus (hiv) type 1 are poorly understood. A patient with an extraordinarily rapid disease course leading to AIDS-associated death within 6 months of infection was studied intensively for the presence of anti-hiv immune reactivities as well as changes in the genetic and biologic properties of virus isolates. Although altered humoral responses were evident, the most distinctive immunologic feature was a nearly complete absence of detectable hiv-specific CTL responses. In addition to a rapid decline in CD3 CD4 cells, elevated percentages of CD8 CD45RA and CD8 CD57 cells and diminished CD8 CD45R0 and CD8 CD28 cells were evident. Primary viral isolates recovered throughout the course of infection exhibited limited sequence diversity. Cloned viral envelopes were found to have unusually broad patterns of coreceptor usage for cell-cell fusion, although infectivity studies yielded no evidence of infection via these alternative receptors. The infectivity studies demonstrated that these isolates and their envelopes maintained an R5 phenotype throughout the course of disease. The absence of demonstrable anti-hiv CTL reactivities, coupled with a protracted course of seroconversion, highlights the importance of robust hiv-specific immune responses in the control of disease progression.- - - - - - - - - - ranking = 0.48995125413372keywords = rate, death (Clic here for more details about this article) |
7/85. Very low-density lipoprotein apolipoprotein b-100 turnover in glycogen storage disease type Ia (von Gierke disease).Mixed hyperlipidaemia is a common finding in glycogen storage disease type Ia (GSD Ia). Although cross-sectional studies have demonstrated increases in intermediate-density lipoproteins (IDLs) and reductions in lipoprotein lipase activity, no studies have investigated the dynamics of apolipoprotein b-100 (apo B) metabolism in GSD Ia. This study investigated apoB turnover in GSD Ia using an exogenous labelling method in one sib from a kinship with established GSD Ia. The study demonstrated normal hepatic secretion of very low-density lipoprotein (VLDL), but hypocatabolism of VLDL, probably due to lack of lipoprotein lipase activity. The production rate of IDL was slightly increased, but the turnover rate of low-density lipoprotein was normal. The findings suggest that, as well as a corn starch diet and dietary fat restriction, treatment of severe mixed hyperlipidaemia in GSD Ia and its attendant risk of pancreatitis should possibly involve fibrates that activate lipoprotein lipase and may enhance the clearance of IDL, rather than omega-3 fatty acids, which principally suppress hepatic secretion of VLDL.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
8/85. Bleeding diathesis in amyloidosis with renal insufficiency associated with Crohn's disease: response to desmopressin.A 53-yr-old man with a 33-yr history of Crohn's ileocolitis, complicated by arthritis and cologastric fistulization, was diagnosed with GI amyloidosis at the time of proctocolectomy. He had marked proteinuria (4.2 g/24 h) and moderate renal insufficiency (BUN of 35 mg/dl and serum creatinine of 2.5 mg/dl). During the operation, he had severe bleeding that required 11 U of blood. Postoperatively, desmopressin was administered, which resulted in a prompt cessation of bleeding. This case demonstrates the efficacy of desmopressin in reversing the bleeding diathesis in surgical patients with amyloidosis complicated by renal insufficiency.- - - - - - - - - - ranking = 0.4keywords = rate (Clic here for more details about this article) |
9/85. Primary cutaneous mucormycosis in infants and neonates: case report and review of the literature.A case of angioinvasive cutaneous mucormycosis in a premature infant, eventually requiring extracorporeal membrane oxygenation therapy, is described. The fungal infection began at the site of a brachial artery catheter that had been covered with an adhesive dressing in the left antecubital fossa. The infection progressed rapidly over a 5-day period, and a left arm amputation was required. Fungal hyphae were present at the margins of resection. The patient eventually had disseminated mucormycosis and died. A second case of cutaneous mucormycosis in another premature infant also is presented. This infant had the infection at an intravenous catheter site. Rapid initiation of surgical debridement of the wound and amphotericin b therapy resulted in patient survival. Eighteen reported cases of cutaneous mucormycosis in neonates were found and are reviewed. Prematurity, low birth weight, broad-spectrum antibiotics, corticosteroid therapy, and local trauma to the skin site were common risk factors. Only 7 of the 18 patients survived. Therapy consisted of local debridement and intravenous amphotericin b. High index of suspicion, early diagnosis, and rapid institution of therapy can improve survival rate. The key to prevention appears to be appropriate skin care.- - - - - - - - - - ranking = 0.2keywords = rate (Clic here for more details about this article) |
10/85. Lethal fulminate S. aureus sepsis in M. Behcet overnight cold exposure.Whether patients with Behcet's disease (BD) and immunosuppressive therapy are generally prone to acquire severe infectious diseases is unknown. A 48-year-old man under corticosteroids and azathioprine for BD since 1995 was admitted because of a transitory ischemic attack. Between the third and fourth hospital day he was accidentally locked up, insufficiently dressed, in the hospital's chapel over night. On the following day, he developed fever and deteriorating consciousness until he became comatose. CT scans of the brain were normal and there was only a slight pleocytosis. Despite adequate therapy, the patient's condition further deteriorated such that he died. Responsible for his decline was a fulminate sepsis, diagnosed upon fever, increased c-reactive protein, thrombocyte decline, multi-organ failure, rhabdomyolysis, growth of S. aureus on blood culture, and autopsy. patients with BD and immunosuppressive therapy, may be more vulnerable to infections and may develop lethal overwhelming sepsis already after overnight cold exposure.- - - - - - - - - - ranking = 0.2keywords = rate (Clic here for more details about this article) |
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