Correlation of Asymmetric Dimethyl Arginine Level to Sickle Retinopathy in Children With Sickle Cell Disease.

Asymmetric dimethyl arginine (ADMA) is a competitive inhibitor of nitric oxide synthetase especially in L-arginine deficiency, which is the case in sickle cell disease (SCD). we aimed to assess the level of ADMA in children with sickle retinopathy and to correlate it to the degree of retinopathy. In this cross-sectional study 40 children with SCD were included, 20 of them with sickle cell retinopathy (SCR) (group I), 20 with normal fundus examination (group II), and another 20 healthy children served as controls (group III). We measured ADMA level by ELISA and performed fundus examination. Seventeen of the 20 children included in group I had Grade I retinopathy (85%), 2 children had Grade II retinopathy (10%), and 1 child had Grade III retinopathy (5%). ADMA was significantly higher in SCD than controls ( P -value <0.001), and it was even higher in patients with SCR compared those without retinopathy ( P -value <0.002), and there was positive linear correlation between ADMA and the grade of retinopathy. The type of retinopathy detect in the studied patients was the nonproliferative type. In conclusion, ADMA is elevated in children with SCD, and its level is even higher in those who develop SCR.

Right ventricular function in ß-thalassemia children: comparing three-dimensional echocardiography with other functional parameters.

BACKGROUND: Cardiomyopathy is a major cause of mortality and morbidity in beta-thalassemia major (ß-TM), and its early detection is critical for prompt management. We aimed to evaluate right ventricle (RV) function in ß-TM, in absence of cardiac symptoms, using 3D echocardiography, and compare it with other functional parameters. METHODS: Cross-sectional cohort study was conducted on 50 ß-TM children with no cardiac manifestations and 50 healthy controls of matched age and sex. We evaluated RV function using; Fraction Area Change (2DE-RV FAC), Ejection Fraction (3D-RVEF), Tricuspid annular plane systolic excursion (TAPSE), Tissue Doppler imaging (TDI) systolic (S') and diastolic (E', A'), Myocardial performance index (MPI), and speckle tracking (2D-STE) of RV global longitudinal strain (LSS), systolic strain rate (SSR), early diastolic strain rate (DSR E), and late diastolic strain rate (DSR A). RESULTS: 3D-RVEF, MPI, and 2D-STE showed significant differences between the two groups. ROC curve analysis measurements had an AUC above 0.7-which indicate at least a fair discriminatory power between the ß-TM group with RV dysfunction and normal controls. CONCLUSIONS: ß-TM patients have decreased RV function indices at a pre-symptomatic stage. Early detection of RV dysfunction is feasible and can allow for closer follow-up to detect pre-clinical changes. IMPACT: The key message of this article is to emphasize the importance of the evaluation of right ventricular function in children with beta-thalassemia major. This article adds to the existing literature the use of recent echocardiographic modalities as three-dimensional echocardiography and two-dimensional speckle tracking in the evaluation of right ventricular dysfunction in beta-thalassemia children while cardiac affection is still subclinical. IMPACT: Recent echocardiographic functional parameters can be effectively used to evaluate the right ventricle in beta-thalassemia and can detect dysfunction in asymptomatic patients allowing for early intervention.

Impact of CD105 Flow-Cytometric Expression on Childhood B-Acute Lymphoblastic Leukemia.

BACKGROUND: CD105 (Endoglin) is a receptor of the transforming growth factor-Beta (TGF- ß) superfamily. It is expressed in angiogenic endothelial cells and is considered a powerful marker of angiogenesis and a potential main player in the pathogenesis of vascular diseases as well as tumor progression. CD105 expression was correlated with poor prognosis in many types of solid malignancies, however, its influence on hematological neoplasms is still an area of interest. PURPOSE: To assess the flow-cytometric expression of CD105 in childhood B-acute lymphoblastic leukemia (B-ALL) and its relation to disease response after the induction chemotherapy. SUBJECTS AND METHODS: Eighty children newly diagnosed with B-ALL were screened for flow-cytometric expression of CD105 at time of diagnosis, then they were followed up to detect their response to induction therapy. RESULTS: CD105 was expressed in 41.2% of B-ALL patients. Higher expression of CD105 was observed in high and very high-risk groups. The multivariate analysis considered CD105 positivity as an independent prognostic marker for response to induction therapy. Values higher than 2.5 Specific fluorescence indices (SFIs) and 35% expression were sensitive predictors to induction failure. CONCLUSION: CD105 can be considered as a potential prognostic marker for the detection of response to induction therapy in childhood B-ALL, and it can serve to optimize treatment decisions.