To develop instruments that measure the severity of infantile hemangiomas (Hemangioma Severity Scale [HSS]) and the complications of infantile hemangiomas for longitudinal use (Hemangioma Dynamic Complication Scale [HDCS]).
Instrument development and reliability study.
The HSS and the HDCS were developed through the collaborative effort of members of the Hemangioma Investigator Group Research Core, an expert multi-institutional research group. After development of the scales, 13 pediatric dermatologists used the HSS to score 20 different hemangiomas. In addition, 12 pediatric dermatologists used the HDCS to score hemangioma-related complications for 24 clinical scenarios. Interrater and intrarater reliability was measured for both scales.
Interrater and intrarater reliability.
For the HSS, interrater reliability and intrarater reliability exceeded 99%. Similarly, the HDCS had a high rate of interrater agreement; for individual items, agreement among raters was 67% to 100%, with most clinical scenarios demonstrating greater than 90% agreement. Intrarater reliability was excellent for all individual items of the HDCS.
The HSS and the HDCS are reliable scales that can be used to measure the severity of infantile hemangiomas, including the severity of complications for longitudinal use.
"IHs as a whole are largely understudied considering the high prevalence of these lesions in children and the serious threat to health they pose in certain instances. To date, there remains a great deal of uncertainty as to the origin of these tumors, with evidence suggesting they may be caused by aberrant transplantation of placental endothelial cells (27), predisposing genetic factors (28,29) and/or tumor stem cell components (30). Despite the controversial origin of these tumors, proliferating IHs are characterized by an enhanced angiogenic capacity largely due to modulation of signaling pathways that regulate the VEGF signaling axis, while involuting IHs display a chronic inflammatory response and downregulation of angiogenesis regulators (31). "
[Show abstract][Hide abstract] ABSTRACT: Infantile hemangiomas (IHs) are non-malignant, largely cutaneous vascular tumors affecting approximately 5-10% of children to varying degrees. During the first year of life, these tumors are strongly proliferative, reaching an average size ranging from 2 to 20 cm. These lesions subsequently stabilize, undergo a spontaneous slow involution and are fully regressed by 5 to 10 years of age. Systemic treatment of infants with the non-selective β-adrenergic receptor blocker, propranolol, has demonstrated remarkable efficacy in reducing the size and appearance of IHs. However, the mechanism by which this occurs is largely unknown. In this study, we sought to understand the molecular mechanisms underlying the effectiveness of β blocker treatment in IHs. Our data reveal that propranolol treatment of IH endothelial cells, as well as a panel of normal primary endothelial cells, blocks endothelial cell proliferation, migration, and formation of the actin cytoskeleton coincident with alterations in vascular endothelial growth factor receptor-2 (VEGFR-2), p38 and cofilin signaling. Moreover, propranolol induces major alterations in the protein levels of key cyclins and cyclin-dependent kinase inhibitors, and modulates global gene expression patterns with a particular affect on genes involved in lipid/sterol metabolism, cell cycle regulation, angiogenesis and ubiquitination. Interestingly, the effects of propranolol were endothelial cell-type independent, affecting the properties of IH endothelial cells at similar levels to that observed in neonatal dermal microvascular and coronary artery endothelial cells. This data suggests that while propranolol markedly inhibits hemangioma and normal endothelial cell function, its lack of endothelial cell specificity hints that the efficacy of this drug in the treatment of IHs may be more complex than simply blockage of endothelial function as previously believed.
Experimental and therapeutic medicine 10/2012; 4(4):594-604. DOI:10.3892/etm.2012.654 · 1.27 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: This paper is based on a review of the literature focused on pediatric dermatology, from October 2011 to september 2012. Our objective was to highlight the main advances in fields such as atopic dermatitis, infantile hemangiomas, infectious diseases, inflammatory disorders, and genodermatoses.
Annales de Dermatologie et de Vénéréologie 12/2012; 139:S202–S216. DOI:10.1016/S0151-9638(12)70135-7 · 0.92 Impact Factor
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