Increasingly, there is a trend to deliver chemotherapy, where possible, in the outpatient ambulatory setting. In the few studies that have explored the setting of cancer care, long wait times are frequently linked to dissatisfaction. Several factors contribute to lengthy waiting times for patients and their families: long registration processes, lag times associated with obtaining laboratory results, time required for patient assessments and preparation of chemotherapeutic agents, adequacy of nursing resources, and physical space constraints in relation to patient volumes. With the goal of improving care delivery in the outpatient clinic, a fast-tracking system was established. Program planning included establishing patient eligibility criteria, protocol and treatment appropriateness, interdepartmental collaboration, development of a communication plan for families and staff, negotiation of physical space, and allocation of human resources. This was instituted by re-allocating existing resources and establishing an autonomous nurse-managed chemotherapy clinic. This fast-tracking program has enabled us to use our existing resources with greater efficiency and improve patient care from safety and quality-of-life perspectives for those included in the program.
Patients with GATA2 (Emberger syndrome) deficiency needs early hematopoietic stem cell transplant (HSCT) before evolving in to myelodysplastic syndrome or acute myeloid leukemia and with time given compromised organ dysfunction leads to increase regimen-related toxicities. Most published cases have used nonmyeloablative conditioning regimens, show higher incidences of rejection and relapse rates and umbilical cord blood transplant has been reported to be suboptimal in patients with GATA2 deficiency because of longer period of engraftment leads to more infections and mortality. We report a 4.5-year-old girl with GATA2 deficiency who underwent matched unrelated donor HSCT utilizing a myeloablative conditioning regimen including intravenous busulfan (total dose of 12.8 mg/kg) and fludarabine (total dose of 160 mg/m) She tolerated the conditioning regimen and bone marrow infusion well. Her initial chimerism was mixed (90% donor), cyclosporine was gradually weaned and discontinued at day+85 and this resulted in conversion to full-donor chimerism. Bone marrow assessment 3 months post-HSCT revealed normal hematopoiesis and absence of monosomy 7. At 20 months of follow-up she had full-donor chimerism with complete reconstitution of the all hematopoietic stem cells. Myeloablative matched unrelated donor HSCT represents an effective option for cure in patients with GATA2 deficiency and Emberger syndrome.
The processing of foods is a large and important industry and has become a necessity in our modern way of life. Through processing, the excess from one harvest may be carried over to the next and perishable foods may be served out of season. I n addition, the processing of foods often makes them more acceptable to the consuming public. Flavors and palatability are often improved and in soqe cases the food value of the nutrient constituents is also increased.On the other hand, some food processing methods are deleterious. Perhaps the most widely recognized food nutrients which are destroyed to various degrees by commercial processing as well as by home cooking are the vitamins. Less recognized but just as important is the loss in protein nutritive quality. Although the deleterious effects of heat treatment on proteins have been known f o r a long time, it is only lately that this important problem has received the attention that it merits.Our interest has been centered around the effects of heat on protein nutritional quality and more specifically on the effects of heat on proteins when reducing sugars such as dextrose are present during the processing.The reaction that takes place is apparently that of the nonenzymatic browning (Maillard) reaction between the free amino groups of the protein and the reducing sugar. This reaction is important in that the storage life of many foodstuffs is shortened because of this reaction which causes darkening, flavor changes, and sometimes formula failure. There is evidence now to indicate that the nutritive value of the protein is also impaired. Likewise, during the processing of some food materials such a reaction also occurs, resulting in color and flavor changes, and more important, in nutritional changes which have only recently been recognized. METHODS Preparation of Protein-dextrose Complexes.In these experiments casein * was used as the protein. Unless otherwise stated the casein-dextrose complexes were prepared by mixing one gram of casein with one gram of dextrose' per gram of pure protein (N X 6.38) and 0.5 ml. of water per gram of casein, and autoclaving for the desired period and a t the designated temperature. Usually one-kilo batches were prepared in shalIow Pyrex pans.Animal Assay Methods. Diets were prepared from treated and untreated casein 80 that the protein content was approximately ten per cent and f a t was added to six per cent.
Reactivation of HSV and VZV is common following HSCT. Consensus guidelines do not support the use of routine screening for viremia following HSCT in adults, but no such clear guidelines exist in pediatrics. In our center, routine practice was to screen patients weekly for HSV and VZV viremia until engraftment in autologous transplant patients and up to day +100 in allogeneic transplant patients. We conducted a retrospective study of over 500 patients to establish whether this screening identified any patients with HSV or VZV viremia who would not have been identified by clinical signs or symptoms. Over a 4.5-yr period, routine screening identified three cases of HSV viremia and one case of VZV viremia. Two patients had persistent, unexplained fever and two patients had skin or mucosal lesions suggestive of HSV/VZV. We conclude that routine screening for HSV and VZV viremia in pediatric HSCT patients has a very low yield and that viremia can be reliably identified by targeted testing in patients with vesicular skin lesions, oral or genital ulceration, unexplained fever, neurological symptoms, or unexplained abnormal liver transaminases.
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