Brain perfusion changes in beta-thalassemia.

BACKGROUND: Brain injury in hereditary hemoglobinopathies is commonly attributed to anemia-related relative hypoperfusion in terms of impaired oxygen blood supply. Supratentorial and infratentorial vascular watershed regions seem to be especially vulnerable, but data are very scarce. AIMS: We investigated a large beta-thalassemia sample with arterial spin labeling in order to characterize regional perfusion changes and their correlation with phenotype and anemia severity. METHODS: We performed a multicenter single-scanner cross-sectional 3T-MRI study analyzing non-invasively the brain perfusion in 54 transfusion-dependent thalassemia (TDT), 23 non-transfusion-dependent thalassemia (NTDT) patients and 56 Healthy Controls (HC). Age, hemoglobin levels, and cognitive functioning were recorded. RESULTS: Both TDT and NTDT patients showed globally increased brain perfusion values compared to healthy controls, while no difference was found between patient subgroups. Using age and sex as covariates and scaling the perfusion maps for the global cerebral blood flow, beta-thalassemia patients showed relative hyperperfusion in supratentorial/infratentorial watershed regions. Perfusion changes correlated with hemoglobin levels (p = 0.013) and were not observed in the less severely anemic patients (hemoglobin level > 9.5 g/dL). In the hyperperfused regions, white matter density was significantly decreased (p = 0.0003) in both patient subgroups vs. HC. In NTDT, white matter density changes correlated inversely with full-scale Intelligence Quotient (p = 0.007) while in TDT no correlation was found. CONCLUSION: Relative hyperperfusion of watershed territories represents a hemodynamic hallmark of beta-thalassemia anemia challenging previous hypotheses of brain injury in hereditary anemias. A careful management of anemia severity might be crucial for preventing structural white matter changes and subsequent long-term cognitive impairment.

Atypically Protracted Course of Liver Involvement in Kawasaki Disease. Case Report and Literature Review.

Hypertransaminasemia in patients with Kawasaki disease (KD) is reported to be transient. Here, we describe a child with an atypically protracted course of liver tests abnormalities and review the inherent literature. The patient was hospitalized at age 7-months for isolated hypertransaminasemia detected during a classical KD diagnosed 3 months before, and persistent since then. KD clinical evolution had been favorable, with rapid response to acetylsalicylic acid and intravenous immunoglobulins. Liver enzymes however remained persistently elevated with a fluctuating pattern (ALT > AST levels; peak of AST 186 IU/L and ALT 240 IU/L). During follow-up, the main causes of liver dysfunction had to be excluded through appropriate and extensive laboratory investigations. Transaminases values become steadily normal only 7 months after the acute presentation of KD. Conclusions: Our report shows that an atypically protracted courses of KD-related hypertransaminasemia above the previously reported temporal limits should be taken into account during the stepwise diagnostic approach to the patient's liver dysfunction. Insidious acetylsalycilic acid-hepatotoxicity warrants consideration in the differential diagnosis.

Healthy Lifestyle Management of Pediatric Obesity with a Hybrid System of Customized Mobile Technology: The PediaFit Pilot Project.

Pediatric obesity management strategies suffer from a high rate of dropout and persistence of weight excess, despite the use of new tools, such as automated mobile technology (MT). We aimed to compare the efficacy of two 6-month personalized MT protocols in terms of better engagement, adherence to follow-up visits and improved anthropometric and lifestyle parameters. MT contacts consisted of three personalized/not automated What's App® self-monitoring or challenge messages per week. Messages, sent by a dedicated coach were inserted between three-monthly in-presence regular visits with (PediaFit 1.2) or without (PediaFit 1.1) monthly free-of charge short recall visits carried out by a specialized pediatric team. The sample included 103 children (mean age 10 years, range 6-14) recruited in the Pediatric Obesity Clinic between January 2017 and February 2019, randomized into Intervention group (IG) (n = 24 PediaFit 1.1; n = 30 PediaFit 1.2) and Control group (CG) (total n = 49). Controls received standard treatment only (indications for healthy nutrition and physical activity, and three months in presence regular visits). Overall, both IGs achieved significantly better results than the CGs for all considered parameters. Comparison of the two IGs at the sixth month in particular showed an IG 1.2 statistically significantly lower drop-out rate (10% vs. 62%, p = 0.00009), along with significantly improved BMI (p = 0.003), Screen Time (p = 0.04) and fruit and vegetables consumption (p = 0.02). The study suggests that the hybrid association of messaging through personalized/not automated MT plus monthly free-of charge recall visits may improve the prefixed outcomes of MT weight loss intervention programs.