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PURPOSE - To identify variations in practices used by nurses for pediatric patients with sickle cell disease (SCD) receiving chronic blood transfusion therapy for strokes.
DATA SOURCES - Descriptive study of a convenience sample of 11 nurses who care for children with SCD from nine institutions completed a closed-ended questionnaire consisting of 37 items. Responses reflected practice experience with a total of 189 transfused patients with SCD.
CONCLUSIONS - A wide range of nursing practices exists for blood transfusion therapy for children with SCD and strokes. Manual partial exchange transfusion (66%) was the most commonly used method for blood transfusion in children with strokes reported among the nurses surveyed. Simple transfusions and erythrocytapheresis account for 21% and 13% of the practices reported. Opportunities exist to establish evidence-based nursing care guidelines to improve the care of children with strokes receiving blood transfusion therapy.
IMPLICATIONS FOR PRACTICE - A wide range of local standard care guidelines for blood transfusion therapy exists. The results of this survey indicate that partial manual exchange transfusion is the most commonly used method of chronic blood transfusion therapy in children with SCD and stroke despite the fact that the magnitude of benefit in comparison with simple transfusion has not been established. Factors such as peripheral venous access, compliance with current chelation regimen, and the presence of antibodies are important considerations in the choice of method.
Behavioural risk factors for HIV/AIDS in Bangladesh were reviewed in a preceding article in this journal. Omitted from that review was a discussion of potential biomedical risk factors including: (i) an unregulated blood supply system in which blood used in transfusions is not screened for HIV and is donated primarily by professional donors: (ii) unsterile injections in non-formal and formal health-care settings; and (iii) a high prevalence in high-risk groups of other sexually transmitted diseases (STDs) which may function as co-factors for HIV transmission, particularly if chronically untreated. Studies elsewhere in the world suggest that the unregulated blood supply system, in particular, poses a serious danger in terms of the spread of the HIV epidemic. While certain socio-cultural factors may be contributing to low levels of HIV in Bangladesh, the prevalence of biomedical and behavioural risk factors suggest the importance of implementing targeted cost-effective interventions now.
Iron overload from repeated transfusions of RBCs in long-term hemodialysis patients is a problem of increasing clinical significance. We report on the prevalence of and diagnostic criteria for identification of hemodialysis patients with iron overload. In 150 unselected hemodialysis patients, 62 (41%) had ferritin levels greater than 2,000 ng/mL (normal = 10 to 360 ng/mL). In 16 of these patients, accurate transfusion histories were obtained and ferritin levels correlated with calculated transfusional iron burden (r = 0.553, P less than .05). These patients could be divided into two distinct groups on the basis of their response to a single dose (2 g, IV) of deferoxamine: "high" responders had twice the level of feroxamine (the chelated product of deferoxamine and iron) of the "low" responders (P less than .001). High responders also had significantly higher prevalence of the "hemochromatosis" alleles A3, B7, and B14 than a large group of dialysis patients awaiting transplantation (71% v 37%, P less than .001). In two patients with iron overload and clinically significant bone disease, bone histology revealed prominent iron staining at the calcification front. We conclude that transfusional iron overload is a significant clinical problem in long-term hemodialysis patients, that may also be associated with bone pathology.
Adolescents engaging in certain sexual or drug-related behavior are at risk of contracting the human immunodeficiency virus infection in endemic locales. Local and national surveillance data were analyzed to determine the characteristics of the acquired immunodeficiency syndrome (AIDS) epidemic on adolescents. Of the 605 cases of AIDS in people aged 13 to 21 years reported through 1987, 518 were males (83 from New York City [NYC], NY), and 87 were females (28 from NYC). Over half of all adolescent males with AIDS reported homosexual contact. Transfusion/blood product-related human immunodeficiency virus acquisitions (especially in males with hemophilia) represented 11% of adolescent cases from NYC (1% of NYC adults) and 22% of adolescent cases in the United States (US) outside of NYC (4% of adults in the US). Intravenous drug use was more frequently reported among adolescents with AIDS from NYC (23%) than among adolescents outside NYC (14%). In females, heterosexual transmission accounts for about half of all adolescent AIDS cases and 29% of all adult cases. Age-appropriate services and behavioral interventions are urgently needed for high-risk adolescents.
Two children with prototypic hemolytic-uremic syndrome had prolonged acute dialysis-dependent renal failure (74 and 84 days) associated with a state of hyperproteinemia induced by extensive infusion of fresh frozen plasma (283 and 307 units). We believe that the hyperproteinemia prolonged the duration of renal failure. Following cessation of plasma therapy, the hyperproteinemic state reversed, the degree of proteinuria decreased and renal function quickly recovered. Although the pathophysiological mechanism requires further evaluation, we speculate that an alteration in the colloid oncotic pressure and/or aggravation of tubulointerstitial injury due to overload-proteinuria may have increased the duration of renal failure.
Five long-term hemodialysis patients with clinical iron overload were treated with 300 U/kg of recombinant human erythropoietin (rHuEPO) intravenously (IV) after each hemodialysis. The patients were phlebotomized after each hemodialysis at any time the predialysis hematocrit was 35% or greater. Over a period of 1 year, the average phlebotomy rate varied from 0.5 to 1.1 U/wk with a mean phlebotomy rate of 45.8 +/- 5.6 U/yr (range, 27 to 57 U). The mean serum ferritin decreased from 8,412 +/- 1,599 micrograms/L (ng/mL) to 3,007 +/- 1,129 micrograms/L (ng/mL), and the mean iron removal over this period was 9.5 g. Liver iron deposition, as measured by density on computed tomographic (CT) scan, improved, while skin color lightened significantly. Patients tolerated phlebotomy with no major symptoms or complications and exhibited no change in the hemogram or serum chemistries. In patients with severe iron overload, changes in serum ferritin with erythropoietin treatment alone may not reflect true change in iron burden. Use of high-dose erythropoietin and phlebotomy is an effective and safe (at least for 1 year) method of reducing iron overload in long-term hemodialysis patients.