The agreement between the HJHS scores and the MRI scores suggests that the HJHS may be used safely as a first-line tool. We recommend that the FISH should be used in the routine follow-up of hemophilia patients as a functional evaluation tool. Painful joints may be useful in deciding to apply MRI, whereas the bleeding frequency may not be useful.

Obesity is associated with a number of risk factors, such as hyperlipidemia, hyperinsulinemia, hypertension, and early atherosclerosis. Evidence indicates that atherosclerosis begins in childhood and progresses over decades. In this work, we examined the relationship between cardiovascular risk factors and ultrasonographic signs of subclinical atherosclerosis in 77 obese children and adolescents compared to 40 non-obese healthy peers. Carotis intima media thickness (cIMT), carotid artery compliance (CAC), brachial artery flow-mediated dilatation (FMD), and established cardiovascular risk factors were studied. In the obese patients, cIMT was significantly increased (0.57 mm vs 0.45 mm, p < 0.001) whereas CAC (1.84% vs 3.29%, p < 0.001) and FMD (9.67 % vs 14.81%, p < 0.001) were significantly decreased. In multiple linear regression analysis, a relation was observed between cIMT, CAC, brachial FMD on one hand, and body mass index (BMI) on the other. Among the lipid anomalies, only hypertriglyceridemia was found to be positively correlated with cIMT. Additionally, we found a significant association between waist circumference (WC) and FMD. These findings indicate that obesity in children is associated with arterial wall alterations and endothelial dysfunction. In hyperlipidemic situations, only hypertriglyceridemia was found to be positively correlated with cIMT. This finding has consistently indicated TG to be a risk factor for the development of atherosclerosis. To our knowledge this is the first study to determine the relation between FMD and WC, which is used as a parameter of obesity in childhood.

Pediatric cancer patients have an increased risk of potentially life-threatening fungal infections such as Candida parapsilosis, associated with long-term CVADs. The Infectious Diseases Society of America (IDSA) guidelines on Candida catheter-related bloodstream infections recommend systemic antifungal therapy and catheter removal. In this study, we focused on our experience with antifungal failure due to totally implanted catheter-associated C. parapsilosis bloodstream infections. We investigated cases leading to port removal in pediatric malignancy patients and the associated patient outcomes. In the first phase of the study, a retrospective chart review was performed to collect patient information, including primary disease; time from hospitalization to port-related candidemia; antifungal drug choice; and the time at which port removal occurred. During the second phase, antifungal susceptibility tests for C. parapsilosis were performed in our microbiology laboratory. All patients had fevers and were neutropenic at the time of candidemia diagnosis. The mean duration between the first isolation of Candida parapsilosis from the port samples to the port removal was 9.75 ± 5.29 days for 11 patients. Patient fevers lasted for a mean time of 16.22 ± 6.51 days. The median recovery duration from fever after CVC removal was four days (range 2-12 days). The median duration for achieving negative blood cultures, following antifungal treatment was 18 days (range 10-27 days). Our data favored the removal of catheters in the presence of ongoing fever, as suggested by the guidelines, independent of the chosen antifungal treatment. Future studies with large samples are needed to evaluate the effects of catheter removal on mortality rates and patient outcomes.

Pandemic H1N1 influenza virus caused mortality in patients with ARDS or HLH; an unexpected finding for pandemic H1N1 (2009) influenza virus. Thus, for children with leukemia and infected with H1N1 virus, short- and long-term complications should be kept in mind during evaluation.

Hemophagocytic lymphohistiocytosis is clinically characterized by fever, hepatosplenomegaly, pancytopenia, and coagulopathy and is histologically characterized by excessive proliferation and activation of histiocytes or macrophages. It occurs with systemic infections, immunodeficiency, and underlying malignancies. Brucellosis is one of the rare causes of hemophagocytosis. Here, we report the case of a 15-year-old male with pancytopenia due to hemophagocytosis during the course of brucellosis that responded favorably to therapy. Although rare, concomitant coronavirus infection and hemophagocytosis should be considered as a possible cause of pancytopenia in patients with brucellosis, particularly in regions where brucellosis is frequently encountered.