Brodie M.J.,Epilepsy Unit |
Covanis A.,Agia Sophia Childrens Hospital |
Gil-Nagel A.,Epilepsy Programme |
Lerche H.,University of Tubingen |
And 4 more authors.
Epilepsy and Behavior | Year: 2011
This article represents a synthesis of presentations made by the authors during a scientific meeting held in London on 7 June 2010 and organized by GlaxoSmithKline. Each speaker produced a short précis of his lecture to answer a specific question, resulting in an overview of what we know about the relevance of the mechanisms of action of antiepileptic drugs in determining appropriate combination therapies for the treatment of drug-resistant epilepsy. © 2011 Elsevier Inc. Source
Papadakis V.,Agia Sophia Childrens Hospital |
Meletis J.,National and Kapodistrian University of Athens
Archives of Hellenic Medicine | Year: 2010
Aplastic anemia (AA) is a severe, acquired hematological disease, which is usually idiopathic. There are two peaks of incidence, at 15-25 years and above 60 years. In children under the age of 15 years, there are 1-3 cases per 106 children, and at this age it is of utmost importance to recognize possible congenital bone marrow failure syndromes. There is no single hallmark for the diagnosis of AA. Decreased bone marrow cellularity and function are evidenced by a decrease in the peripheral blood count, with concomitant hemorrhage, infection and pallor. In the majority of cases, AA is the result of an autoimmune destruction mechanism targeted at the subject's own bone marrow cells. A usually unidentified triggering event leads to a Th1 type cell response, with overproduction of myelosuppressant cytokines, such as IFN-α, TNF-α and IL-2, in parallel with activation of Fas mediated, increased bone marrow cell apoptosis. Appropriate therapy results in reversal of the above pathways and clinical cure. There is a possibility of later evolution to clonal disease, i.e., myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML). The forms of treatment available for AA are appropriate immunosuppressive therapy (IST) or stem cell transplantation (SCT). IST comprises anti-thymocyte globulin and cyclosporine. SCT is the treatment preferred for younger patients with severe AA who have an available HLA compatible sibling donor, and in the case of IST failure (with the use of volunteer donors). IST is reserved for older patients and for younger patients without an available sibling donor. Currently treatment results achieve a 75% to 85% 5-year survival rate. Extremely important issues in the care of patients with AA are prompt diagnostic evaluation and the meticulous supportive care. © Athens Medical Society. Source
Mavrogeni S.,Onassis Cardiac Surgery Center |
Smerla R.,Aglaia Kyriakou Childrens Hospital |
Grigoriadou G.,Aglaia Kyriakou Childrens Hospital |
Servos G.,Aglaia Kyriakou Childrens Hospital |
And 6 more authors.
Lupus | Year: 2016
Objectives: To evaluate the cardiovascular magnetic resonance (CMR) findings in a paediatric population with systemic lupus erythematosus (SLE) and cardiac symptoms. Methods: Twenty-five SLE children, aged 10.2 ± 2.6 years, with cardiac symptoms and normal routine non-invasive evaluation were examined by CMR, using a 1.5 T system and compared with sex-matched SLE adults. Left ventricular (LV) volumes, ejection fraction, T2 ratio, early (EGE) and late (LGE) gadolinium enhancement were assessed. Acute and chronic lesions were characterised as LGE-positive plus T2 > 2, EGE > 4 or T2 < 2, EGE < 4, respectively. According to LGE, lesions were characterized as: (a) diffuse subendocardial, (b) subepicardial and (c) subendocardial/transmural, due to vasculitis, myocarditis and myocardial infarction, respectively. Results: LV ejection fraction (LVEF) was normal in all SLEs. T2 > 2, EGE > 4 and positive epicardial LGE wall was identified in 5/25 children. Diffuse subendocardial fibrosis was documented in 1/25. No evidence of myocardial infarction was identified in any children. In contrast, in SLE adults, LGE indicative of myocardial infarction was identified in 6/25, myocarditis in 3/25, Libman-Sacks endocarditis in 1/25 and diffuse subendocardial fibrosis in 2/25. The incidence of heart disease in SLE children was lower compared to SLE adults (p < 0.05), with a predominance of myocarditis in children and myocardial infarction in adults. A significant correlation was documented between disease duration and CMR lesions (p < 0.05). Conclusion: CMR identifies a predominance of myocarditis in paediatric SLE with cardiac symptoms and normal routine non-invasive evaluation. However, the incidence of cardiac lesions is lower compared to SLE adults, probably due to shorter disease duration. Significance and Innovation: CMR identifies heart involvement in a significant percentage of SLE children with cardiac symptoms and normal routine noninvasive evaluation. The incidence of heart disease is lower in SLE children compared with SLE adults. Predominance of myocarditis and myocardial infarction is observed in SLE children and SLE adults, respectively. © The Author(s) 2015. Source
Severino M.,G. Gaslini Childrens Hospital |
Schwartz E.S.,Childrens Hospital of Philadelphia |
Thurnher M.M.,Medical University of Vienna |
Rydland J.,center |
And 2 more authors.
Neuroradiology | Year: 2010
Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease-free survival. © 2010 Springer-Verlag. Source
Dedoussis G.V.Z.,Harokopio University |
Kapiri A.,Harokopio University |
Samara A.,University of Lorraine |
Dimitriadis D.,Agia Sophia Childrens Hospital |
And 4 more authors.
European Journal of Clinical Investigation | Year: 2010
Background Adipose tissue secrets several adipokines that have been proposed to be enrolled in many inflammatory pathways. Our aim was to investigate the adipokine expression in adipose tissue and peripheral blood mononuclear cells (PBMCs) in children. Materials and methods Thirty-one (17 males and 14 females) healthy children aged 10·9 ± 1·8 years with a body mass index (BMI) of 19·3 ± 3·5 kg m -2 were enrolled. Adipokines (TNF-alpha, IL-6 and leptin) gene expression was quantified by real-time quantitative PCR in adipose tissue and PBMCs from the same children. Their serum levels were also measured. Results BMI was positively correlated with leptin gene expression in adipose tissue and with leptin serum levels (β = 0·476, P = 0·006 and β = 0·576, P = 0·003 respectively). Leptin's serum levels were positively correlated with leptin gene expression in adipose tissue (β = 0·462, P = 0·02). Adipose tissue gene expression of leptin and TNF-alpha and serum leptin and TNF-alpha serum levels were positively correlated (β = 0·752, P < 0·001, β = 0·311 and P = 0·015 respectively). In PBMCs, a positive correlation between TNF-alpha and IL-6 expression was found (β = 0·526, P = 0·042). Conclusion We demonstrated powerful correlations of adipokines gene expression in adipose tissue and PBMCs in children, underlying that these molecules share common pathways related to childhood obesity. © 2010 Stichting European Society for Clinical Investigation Journal Foundation. Source