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We recently read the article about coagulation abnormalities in type 1 Gaucher disease in the December 2006 issue of Pediatric Hematology and Oncology [1]. A child with Gaucher associated with von ...

Cardiac complications are the leading cause of death in ß-thalassemia major (TM) patients. The aim of this study was to investigate the impact of iron overload on ventricular functions using conventional and tissue Doppler imaging (TDI) in patients with TM and compare them with children with thalassemia trait (TT) and healthy controls. This prospective study includes 3 groups: group 1: 29 patients with ß-TM; group 2: 28 patients with TT; group 3: 29 healthy controls. Peak late relaxation velocity determined by conventional echocardiography for the right ventricle was significantly higher and the E/A ratio for the right ventricle and left ventricle were significantly lower in TM patients than the other groups (P < .05). Peak late relaxation velocity determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly higher in TM patients than the TT subjects and controls (P < .001). The E/A ratio determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly lower in group 1 than the other 2 groups (P < .001). There was a negative correlation between the ferritin level and E/A ratio for the left ventricle, interventricular septum, and right ventricle using TDI (P < .05). Conventional echocardiographic techniques have failed to distinguish ventricular functions of asymptomatic patients with TM from the subjects with TT and from normal controls when global functions were examined. The present study indicates that TDI should be used for screening of TM and TT subjects' cardiac functions. © 2011 Informa Healthcare USA, Inc.

In this study, the authors aimed to evaluate the frequency of pulmonary hypertension (PHT) in asymptomatic thalassemia major (TM) patients, and to investigate the impact of pulmonary function test (PFT) and CO diffusion results on the evaluation of pulmonary hypertension. Data from 50 asymptomatic patients with TM over age 10 were evaluated. Pulmonary hypertension was diagnosed in 10 patients (20%). High tricuspid regurgitant jet velocity was found in 14% of adults and in 25% of children. Pulmonary function test including CO diffusion testing results were not different between the TM patients with or without pulmonary hypertension. In conclusion, PHT was frequent among TM patients even they were asymptomatic and although PFT results has shown lack of association, it should be investigated in larger population.

The authors report on a 4-year-old child with the diagnosis of tetralogy of Fallot (TOF) and infective endocarditis. Methicillin-resistant Staphylococcus aureus (MRSA) was isolated from the blood culture of the patient. While receiving imipenem, amikacin, and linezolid therapies, the boy's general condition improved, acute phase reactants decreased, and his blood culture became negative for MRSA. On his follow-up echocardiography, the vegetation had also disappeared. However, he developed progressive bicytopenia following linezolid therapy for 5 weeks. During linezolid therapy, his hemoglobin level decreased from 12.1 to 5.3 g/dL and his platelet count from 242 x 10(9) to 14 x 10(9)/L. His white blood cell count (WBC) did not decrease during linezolid therapy. Six days following termination of linezolid therapy, his hemoglobin had increased to 8.2 g/dL and platelet count to 192 x 10(9)/L. Thus, it should be kept in mind that linezolid may induce cytopenias in children. If these side effects of linezolid are known, unnecessary laboratory investigations may be prevented and cessation of the drug may be sufficient for reversal of the cytopenias.