Background: Guidelines and expert recommendations on infantile hemangiomas (IH) are aimed at increasing homogeneity in clinical decisions based on the risk of sequelae.Objective: The objective was to analyze the inter-and intra-observer agreement among pediatric dermatologists in the choice of treatment for IH.Methods: We performed a cross-sectional inter-rater and intra-rater agreement study within the Spanish infantile hemangioma registry. Twenty-seven pediatric dermatologists were invited to participate in a survey with 50 clinical vignettes randomly selected within the registry. Each vignette contained a picture of an infantile hemangioma with a clinical description. Raters chose therapy among observation, topical timolol, or oral propranolol. The same survey reordered was completed 1 month later to assess intra-rater agreement. Vignettes were stratified into hemangioma risk categories following the Spanish consensus on IH. The agreement was measured using kappa statistics appropriate for the type of data (Gwet's AC 1 coefficient and Gwet's paired t test).Results: Twenty-four dermatologists completed the survey. Vignettes represented 7.8% of the Spanish hemangioma registry. The inter-rater agreement on the treatment decision was fair (AC 1 = 0.39, 95% confidence interval [CI]: 0.30-0.47). When stratified by risk category, good agreement was reached for high-risk hemangiomas (AC 1 = 0.77, 95% CI: 0.51-1.00), whereas for intermediate-and low-risk categories, the agreement was only fair (AC 1 0.31, 95% CI: 0.16-0.46 and AC 1 = 0.38, 95% CI: 0.27-0.48, respectively). Propranolol was the main option for high-risk hemangiomas (86.4%), timolol for intermediate-risk (36.8%), and observation for low-risk ones (55.9%). The intrarater agreement was good. The inter-rater agreement between pediatric dermatologists on the treatment of IH is only fair. Variability was most significant with intermediate-and low-risk hemangiomas.
Congenital melanocytic nevus syndrome (CMNS) is the result of an abnormal proliferation of melanocytes in the skin and central nervous system caused by progenitor-cell mutations during embryonic development. Mutations in the NRAS gene have been detected in many of these cells. We present 5 cases of giant congenital melanocytic nevus, 3 of them associated with CMNS; NRAS gene mutation was studied in these 3 patients. Until a few years ago, surgery was the treatment of choice, but the results have proved unsatisfactory because aggressive interventions do not improve cosmetic appearance and only minimally reduce the risk of malignant change. In 2013, trametinib was approved for use in advanced melanoma associated with NRAS mutations. This drug, which acts on the intracellular RAS/RAF/MEK/pERK/MAPK cascade, could be useful in pediatric patients with CMNS. A better understanding of this disease will facilitate the development of new strategies.
Migrations, mass tourism, and international real estate investments are causing irreversible effects on the genuine identity evolution of Mouraria’s urban landscape heritage. This research analyses the emotional and multisensorial responses to the urban space of Mouraria’s residents using the methodology of three pedagogical-artistic laboratories. We enquire how this multicultural community is involved in the creation of an active and predicative experience of space through artistic activities. This community-based research reaffirms the understanding of the urban heritage in its intangible dimension as a social participative construct, in a constant process of identity reformulation, reinvention, and artistic creation.
A 1-month-old female Kuwaiti infant was referred to us with a history of an asymptomatic dusky red, stable, hairy plaque on the right thigh, present since birth. She had been delivered at term by lower segment cesarean section because her mother had had a previous cesarean section and had a birth weight of 3.5 kg. The antenatal history of her mother, the infant's birth history, and family history were unremarkable.On examination, her length, weight, and head circumference were all at the 50th percentile. She was observed to have a 9-· 7-cm indurated, dusky red, nontender plaque on the right thigh with local hypertrichosis in the area (Fig. 1). Her general parameters and examination of the rest of her skin and mucosae were normal. Histologic examination of a biopsy taken from the lesion is shown in Figs. 2 and 3.
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