Transplantation from non-heart-beating donors.
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Biomedical subjects
Publications and source records attributed to G Mallory.
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PURPOSE: To determine whether the morphologic features of posttransplant lymphoproliferative disease (PTLD) correlated to a response to therapy. PATIENTS AND METHODS: We reviewed our experience with PTLD in the pediatric population. We identified 32 patients with a total of 36 episodes of PTLD. The diagnosis was confirmed by tissue examination and classified according to the degree of monomorphic features of the lesion. Thirty-four of 36 episodes were managed with immunosuppression reduction, and the patients were assessed for their response to this strategy. Chemotherapy was used to treat 10 of 15 patients who had progressive disease, and their subsequent course was also analyzed. RESULTS: Sixteen of 17 (94%) patients with polymorphic morphology responded to immunosuppression reduction compared with only 5 of 17 (29%) patients with monomorphic features (P < 0.001). All of the patients with progressive disease who did not receive additional therapy died. Standard chemotherapy regimens for lymphoma were administered to 10 patients with progressive disease, with a high response rate (90%), durable remissions, and acceptable toxicity. CONCLUSIONS: We conclude that the morphologic characteristics of PTLD provide information to potentially help guide treatment strategies in the management of this disease. Standard chemotherapy regimens for malignant lymphoma appear to be a viable treatment option for patients with progressive disease, although further investigation is needed.
Approximately 700 transplants world-wide have been performed in patients aged less than 18 years; in contrast, over 11,000 lung transplants have been performed in adults. The major diagnostic group is cystic fibrosis. An emerging group of patients are infants born with congenitally based pulmonary diseases such as surfactant protein B deficiency. Survival in children is very similar to that in adults, although it is generally perceived that children are at higher risk. For instance, no children have been transplanted for chronic obstructive lung disease (a low-risk diagnostic group) while this disease comprises approximately 40% of all adult lung transplantations. Immunosuppression generally consists of cyclosporine, azathioprine and prednisone. Post-transplant complications in children are similar to those observed in adults. Around 40% of patients will develop bronchiolitis obliterans approximately 3 years after transplantation and this remains the major impediment to long term survival. Donor shortage is also a significant obstacle, especially in adolescents. As a partial solution to this problem, living donor lung transplantation has taken on a greater role in our program. Although this is a complex, expensive treatment strategy, lung transplantation remains the most effective therapy for end-stage pulmonary parenchymal and vascular diseases even in children.
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There is more to losing weight, maintaining weight and choosing a healthy diet after gastric bypass than simply limiting food intake and avoiding sugars. Patients must learn to make food selections that have a high nutrient:energy ratio. Considerable research indicates that the dietary fat level, independent of total caloric intake, is a predictor of fat storage. A contributing factor is the difference in metabolic efficiency of converting dietary fats to body fat compared to the relative inefficiency of converting carbohydrates to body fat. The recommendation that no more than 25% of daily caloric intake come from fat is in keeping with good nutritional practices and may also lower the risk of heart disease and cancer. Patients should have nutrition counseling supplemented with written materials on label reading and recognizing high fat foods, types of fat and cholesterol, serving sizes, meal planning, low fat cooking and dining out. Choosing foods with a high nutrient:energy ratio allows the patient to eat an increased volume and thus a wider variety of foods to meet their nutrient and energy requirements.
A 12-year-old girl had sarcoidlike syndrome and hypogammaglobulinemia. Pancytopenia and hepatosplenomegaly were noted at age 4 years. Histopathologic study showed typical sarcoidlike granulomas. Chronic lung disease, along with recurrent infections, developed. Immunologic studies revealed common variable hypogammaglobulinemia, abnormal cellular immune functions, and decreased C4 levels. An immunoregulatory defect is suggested as the pathogenesis of this immunodeficiency syndrome, with multisystem sarcoidlike granulomas, hypersplenism, pulmonary disease, and abnormal cellular and humoral immunity.
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