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排序方式: 共有1774条查询结果,搜索用时 9 毫秒
971.
972.
Touma EH Rashed MS Vianey-Saban C Sakr A Divry P Gregersen N Andresen BS 《Archives of disease in childhood》2001,84(1):58-60
A patient with very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is reported. He had a severe neonatal presentation and cardiomyopathy. He was found to be homozygous for a severe mutation with no residual enzyme activity. Tandem mass spectrometry on dried blood spots revealed increased long chain acylcarnitines. VLCAD enzyme activity was severely decreased to 2% of control levels. Dietary management consisted of skimmed milk supplemented with medium chain triglycerides and L-carnitine. Outcome was good and there was no acute recurrence. 相似文献
973.
Cross-reactivity of cytomegalovirus-specific CD8+ T cells to allo-major histocompatibility complex class I molecules 总被引:1,自引:0,他引:1
Gamadia LE Remmerswaal EB Surachno S Lardy NM Wertheim-van Dillen PM van Lier RA ten Berge IJ 《Transplantation》2004,77(12):1879-1885
BACKGROUND: In transplantation settings, cytomegalovirus (CMV) infection is a common complication. CMV infection is associated with a higher incidence of graft rejection in solid organ transplantation and graft-versus-host disease in bone marrow transplantation. The underlying mechanism of this association could be the generation of CMV-specific CD8 T cells capable of cross-reacting with alloantigens present on graft and host, respectively. METHODS: Whereas as to date, no direct ex vivo analysis can be performed of the CD8 T-cell repertoire directed at allo-major histocompatibility complex (MHC) class I molecules, virus-specific cells can be readily enumerated by use of MHC-peptide tetrameric complexes. In this study, the authors used this technique to analyze potential overlapping CD8 T-cell repertoires between self-MHC-viral peptide and allo-MHC complexes by stimulating CMV-specific CD8 T cells with alloantigens. RESULTS.: The authors found that CMV-specific CD8 T cells are activated and proliferate on stimulation with alloantigens. CONCLUSIONS: Although these cells are cytotoxic against CMV-peptide pulsed target cells, no cytotoxicity of CMV-specific cells to alloantigens could be detected, inferring that there are other mechanisms of graft damage by alloantigen-stimulated virus-specific CTL. 相似文献
974.
Abbas EE Afioni N Al Wakeel J Bakr MA Dham R Donia A Droubi N Khidir E Mathew CM Mitwali AH Naga S Pingle A Rashed A Roshdy A Shaheen F Shaibani B Shaibani FM Shaker DS Sheiban A Solieman M 《Transplantation proceedings》2004,36(6):1805-1811
Recombinant human erythropoietin has proved to be effective to treat anemia of end-stage renal disease (ESRD). The aim of this study was to assess the efficacy and safety profile of Epotin, a rHuEPO produced in the Middle East. One hundred thirty patients with Hct = 27%; Hb = 9 g/dL maintained on hemodialysis thrice weekly from 19centers in eight countries in the Middle East were recruited into this 13-week study. Depleted iron stores (TSTAT <20% and/or Serum ferritin < 100 microg/dL) were replenished prior to initiation of Epotin therapy, which was delivered intravenously in a dose of 150 U/kg body weight/week in three equal doses postdialysis and titrated according to hemoglobin (Hb) and hematocrit (Hct) response. Efficacy was assessed in terms of Hb/Hct response. Epotin raised the mean Hb level from 7.7 (+/- 1.2) g/dL to 12.0 (+/- 1.7) g/dL and Hct from 22.7 (+/- 4.1) % to 36.2 (+/- 5.7) % by week 13. The increase started to show significance at week 3. Targeting an absolute increase in Hb of 2.5 g/dL (Hct 7.5%) over a 13-week period, the success rate was of <85.71%. Segregating patients into subgroups of men and women and chronic ESRD versus recent ESRD failed to reveal a significant differences in either the severity of the anemia or the response to Epotin. Side effects were similar to other erythropoietins; no dropouts were reported. In conclusion, Epotin is effective to treat anemia in patients on maintenance hemodialysis with an acceptable safety profile. No difference in response was observed between men and women, nor between patients with different levels of chronicity of ESRD. 相似文献
975.
Pharmacologic interventions designed to control hyperparathyroidism (HPT) in uremic patients have limitations and potentially serious adverse clinical consequences. Hence, one still has to resort to surgical parathyroidectomy (PTX) in a considerable number of dialysis patients. The aim of the present study was to illustrate our experience with 26 renal dialysis patients who underwent surgical PTX. The main indications for PTX included iPTH > 1000 pg/mL associated with severe osteitis fibrosa, debilitating pruritus, marked soft tissue calcification, or hypercalcemia with hyperphosphatemia, which sometimes complicated vitamin D therapy. All patients were resistant to more conservative measures, including control of serum phosphate, attention to oral intake and dialysate calcium levels, and oral/intravenous administration of active vitamin-D-pulse therapy. Ultrasound and technetium 99-sestamibi scan were used to image the thyroid and the parathyroid glands. Total PTX with autotransplantation was performed in 23 patients; subtotal PTX was performed in 3 patients. Histology of frozen sections taken intraoperatively showed nodular changes in 14 and diffuse hyperplasia in 12 cases. During the 2-year follow-up period significant reductions in parathyroid hormone, alkaline phospatase blood levels, skeletal changes, and soft tissue calcifications were observed. Pruritis improved in half the cases. Some improvement in hemoglobin and hematocrit was also noticed. The complication rate after PTX was low. Transient postoperative hypocalcemia requiring intensification of calcium and vitamin D therapy was seen in cases with high preoperative alkaline phosphatase levels. Recurrence was observed in two cases. Hypoparathyroidism was not recorded. We conclude that surgical reduction of parathyroid mass is a safe and effective treatment for symptomatic disease not suppressible by pharmacologic means. 相似文献
976.
We present a case of an extensive gas-forming abdominal wall abscess secondary to ruptured retroperitoneal appendicitis in a diabetic patient. Computed tomography (CT) demonstrated a retroperitoneal abscess extending to the abdominal subcutaneous tissue through the superior lumbar triangle pathway, a known anatomical defect of the lumbar musculature. This case not only represents an unusual manifestation of acute appendicitis, but also alerts us to the importance of anatomical considerations in the imaging interpretation of disease extent. The applied anatomy of the lumbar triangle as a conduit of intra-abdominal and retroperitoneal processes into the abdominal wall is reviewed. 相似文献
977.
978.
979.
Cardiovascular disease in renal allograft recipients is associated with elevated sialic acid or markers of inflammation 总被引:1,自引:0,他引:1
Bakri RS Afzali B Covic A Sriskantharan R Bharma-Ariza P Park WH Sriharan M Dalton N Wierzbicki AS Crook MA Goldsmith DJ 《Clinical transplantation》2004,18(2):201-204
BACKGROUND: Cardiovascular disease (CVD) is more common in patients with renal failure, even after renal transplantation. We wished to examine the relationship between markers of inflammation and CVD in stable renal transplant patients. METHODS: Ninety stable renal transplant outpatients with no recent illnesses or rejection were invited for study. Blood was drawn for a variety of inflammatory markers including total plasma sialic acid (SA) levels. RESULTS: Patients with CVD were significantly older than patients without (54 +/- 12 vs. 42 +/- 14 yr, p < 0.01) and had significantly lower total cholesterol (4.5 +/- 1.6 vs. 5.1 +/- 1.0 mmol/L, p < 0.01). Time from transplantation, present creatinine and blood pressure, smoking history were similar in both groups. Patients with CVD had significantly higher levels of SA (89.2 +/- 22.3 vs. 77.4 +/- 13.9 mg/dL, p = 0.01); fibrinogen [4.6 (2.2-6.7) vs. 3.6(1.9-5.7) g/L; p = 0.05); and C-reactive protein (CRP) [2.2 (1.5-8.0) vs 1.5 (0.7-3.0) microg/dL] than those without CVD. A logistic multiple linear regression analysis of the data with CVD as the dependent variable, and all the other parameters as independent variables, showed significant associations (F = 16.9; p < 0.001) with diastolic blood pressure (beta = 5.6; p = 0.02) and CRP (beta = 4.4; p = 0.04). CONCLUSIONS: This study suggests that inflammation is associated with a higher prevalence of cardiovascular disease in patients with renal allografts. The measurement of sialic acid as a risk factor may be superior to that of CRP in this group as its concentration is independent of renal function. 相似文献
980.
Repetto HA Rodríguez-Rilo L Mendaro E Basso L Galvez H Morrone G Vazquez LA 《Pediatric nephrology (Berlin, Germany)》2004,19(12):1400-1403
Percutaneous treatment of renal artery stenosis (RAS) is an accepted procedure and numerous reports have been published. However, experience with its use in RAS in the transplanted kidney in children is scarce. Since 1994 we have diagnosed RAS in seven children with the use of Doppler ultrasonography (US), confirming it with percutaneous angiography (PAG). In six of the seven patients percutaneous transluminal angioplasty (PTA) was performed. In one patient a metallic stent was placed due to the extension of the arterial lesion, and a second stent was placed in another child when a re-stenosis was diagnosed 1 month after the PTA. All patients presented with hypertension (de novo or 30% increase over previous values). After ruling out acute rejection, calcineurin inhibitor toxicity, and urinary obstruction, US was performed and, when an increase in arterial flux velocity was registered, PAG was also performed. Six children showed an increase in serum creatinine (Cr) and proteinuria. Blood pressure decreased after the procedure and Cr returned to previous levels in all children. One of the grafts was lost due to chronic transplant rejection 7 years later. The other children have a functioning kidney. Although this is a small group of patients, the consistently good results and the lack of reported experience prompted us to communicate our preliminary observation. 相似文献