β-Thalassaemia: from hospital to the community

published_or_final_versio

A boy with fever and sore throat

published_or_final_versio

Oral complications related to cancer therapy and bone marrow transplantation (BMT) amongst Chinese children

Abstract no. 271published_or_final_versio

Urate oxidase for the prevention and treatment of tumour lysis syndrome in children with cancer

published_or_final_versio

Malignancies in Chinese patients with neurofibromatosis type 1

published_or_final_versio

Effects of chelators (desferal, deferiprone & deferaairox) on the growth of klebsiella and aeromonas isolated from transfusion dependent thalassemia patients

Poster Presentation (Doctor’s Session)Infection is among the leading causes of death for thalassemia major patients. The known predisposing factors of infection include prior splenectomy, iron overload and use of iron chelator such as desferal (desferrioxamine). While encapsulated organisms frequently found in splenectomized patients were readily controlled by prophylactic vaccination and vigilant antibiotic treatment, ferrophilic organisms such as Yersinia and Klebsiella remains common among Thalassemic patients. The inductive iron overloaded environment favours the growth of these organisms but their growth is also affected by the environment temperature. For example, Yersinia infection is more prevalent in temperate regions and Klebsiella infection is commonly found in subtropical areas. Furthermore, the use of iron chelator in the form of desferal further aggravates the risk of Yersinia infection. It is because the iron membrane transport protein siderophore found in desferal can be adopted by the bacteria for iron acquisition. However, oral chelators such as deferiprone do not enhance growth of Yersinia in vitro or in vivo. In order to find out whether such observation can be extended to Klebsiella and Aeromona infection, in vitro culture assay using Klebsiella pneumoniae and Aeromonas hydrophila obtained directly from our transfusion dependent thalassaemic patients were performed. The growth rates of the bacteria under iron rich, iron poor with or without different chelators were assessed. The growth rates were analyzed by both: (1) optic density of bacterial broth; and (2) colony count by bacterial agar plate. We found that the growth of Klebsiella was marginally enhanced by desferal in vitro when compared to Yersinia. Such unfavourable effect was not found in either deferiprone or deferasirox in vitro. On the other hand, the growth of Aeromonas was not affected by the presence of any of the 3 chelators. Therefore, we suggested that factors other than desferal may account for the increase prevalence of Klebsiella and Aeromonas infection among Asian thalassemic patients. It also suggests that oral chelators are safe for thalassemic patients during febrile illness. Unlike desferal, withholding iron chelator during infectious period may not be mandatory. But care has to be exercised especially for patients on deferiprone, since neutropenia has to be ruled out during febrile illness. This project was supported by the Children's Thalassaemia Foundationspublished_or_final_versio

Vaccines for prophylaxis of viral infections in patients with hematological malignancies.

Viral infections cause significant morbidity and mortality in patients with hematological malignancies. It remains uncertain whether viral vaccinations in these patients are supported by good evidence. We aimed to determine the effectiveness and safety of viral vaccines in patients with hematological malignancies. We searched Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, CINAHL (June 2010), reference lists of relevant papers, abstracts from scientific meetings and contacted vaccine manufacturers. Randomized controlled trials (RCTs) evaluating viral vaccines in patients with hematological malignancies were included. Relative risk (RR) was used for binary data and mean difference (MD) for continuous data. Primary outcome was incidence of infection. Secondary outcomes were mortality, incidence of complications and severe viral infection, hospitalization, immune response and adverse effects. Fixed-effect model was used in meta-analyses. Eight RCTs were included, with 305 patients in the intervention groups and 288 in the control groups. They evaluated heat-inactivated varicella zoster virus (VZV) vaccine (two trials), influenza vaccines (five trials) and inactivated poliovirus vaccine (IPV) (one trial). Seven trials had high and one trial had moderate risk of bias.VZV vaccine might reduce herpes zoster compared to no vaccine (RR 0.54, 95% CI 0.3 to 1.0, P=0.05), but not statistically significant. Vaccination also demonstrated efficacy in immune response but frequently caused local adverse effects. One trial reported severity score of zoster, which favored vaccination (MD 2.6, 95% CI 0.94 to 4.26, P=0.002).Two RCTs compared inactivated influenza vaccine with no vaccine and reported lower risk of lower respiratory infections (RR 0.39, 95% CI 0.19 to 0.78, P=0.008) and hospitalization (RR 0.17, 95% CI 0.09 to 0.31, P<0.00001) in vaccine recipients. However, vaccine recipients more frequently experienced irritability and local adverse effects. There was no significant difference in seroconversion between one and two doses of influenza vaccine (one trial), or between recombinant and standard influenza vaccine (one trial), or influenza vaccine given with or without re-induction chemotherapy (one trial).The IPV trial comparing vaccination starting at 6 versus 18 months after stem cell transplant (SCT) found no significant difference in seroconversion. Inactivated VZV vaccine might reduce zoster severity in adult SCT recipients. Inactivated influenza vaccine might reduce respiratory infections and hospitalization in adults with multiple myeloma or children with leukemia or lymphoma. However, the quality of evidence is low. Local adverse effects occur frequently. Further high-quality RCTs are needed.link_to_subscribed_fulltex

Left ventricular myocardial deformation and mechanical dyssynchrony in children with normal ventricular shortening fraction after anthracycline therapy

Objective: The M-mode-derived left ventricular shortening fraction is incorporated into most of the paediatric oncology protocols for monitoring of cardiotoxicity. This study tested the hypothesis that alteration of left ventricular myocardial deformation and mechanical dyssynchrony may occur in asymptomatic children after anthracycline therapy despite having left ventricular shortening fractions within the limits of normal. Design: Cross-sectional study. Setting: Tertiary paediatric cardiac centre. Methods: Left ventricular longitudinal, circumferential and radial myocardial deformation was determined using speckle tracking echocardiography in 45 patients aged 15.3±5.8 years. Real-time three-dimensional echocardiographic data were acquired for the measurement of left ventricular volumes and systolic dyssynchrony index (SDI), the latter derived from the dispersion of time-to-minimum regional volume using a 16-segment model. The results were compared with those of 44 controls. Results: Compared with controls, patients had reduced left ventricular global systolic longitudinal strain (p=0.012), circumferential strain (p4.96%) in patients was 16% (95% CI 6% to 29%). In patients, SDI correlated negatively with left ventricular ejection fraction (r=-0.52, p<0.001), radial strain (r=-0.35, p=0.021), circumferential strain (r=-0.37, p=0.015) and circumferential SR (r=-0.43, p=0.004), but not with the cumulative anthracycline dose (p=0.82). Conclusions: Impaired left ventricular myocardial deformation and mechanical dyssynchrony may exist in children after anthracycline therapy despite having normal left ventricular shortening fractions.published_or_final_versio