P21 - Evaluation of modifications in SCORMA Index and grading in the follow-up of a pediatric population with mastocytosis
© Del Mastro et al; licensee BioMed Central Ltd. 2014
Published: 28 February 2014
Mastocytosis is a rare disease affecting both children and adults. It is characterized by accumulation of mast-cells in the skin and/or other tissues so that two main variants are distinguished: cutaneous (CM) and systemic (SM) mastocytosis. Most pediatric patients have primarily CM and complete remission will develop in a considerable number of patients during puberty. Less frequently, skin lesions persist and these patients are often diagnosed with SM in adulthood.
Our study investigates the correlation between SCORMA (SCORing MAstocytosis) Index (SC-I), a scoring system that assesses the clinical extent and intensity of cutaneous mastocytosis, and Grading system, that evaluates skin-specific symptoms and treatment status, in the follow-up of pediatric patients with mastocytosis. Furthermore, we evaluate the role of SC-I modifications in the assessment of the severity of disease.
Twenty-two patients aged less than 12 years were enrolled from our centre. SC-I and Grading were assessed for each patient at first visit and after 2 years, with determination of Grading modifications and calculation of differences in absolute value of SC-I (ΔSC-I). Data were analyzed using SPSS software. Pearson’s correlation coefficient (ρ) was used to assess the relationship between SC-I and Grading at first visit (ρ1) and after 2 years (ρ2). Chi-square test was performed to evaluate the association of ΔSC-I with Grading modifications, by dividing patients with SC-I changed (ΔSC-I > 10) and unchanged (ΔSC-I ≤ 10) in two groups (Grading modified and unmodified).
We found ρ1= 0, 47 (p < 0,05) and ρ2 =0,36 (p < 0,05), while there was no statistical difference in ΔSC-I in the two groups (p < 0,05).
According to our data, in pediatric patients with mastocytosis SC-I and Grading show a moderate correlation at first visit which weakens not significantly at the subsequent assessment after 2 years. Moreover, SC-I modifications (ΔSC-I) seem to be independent from Grading modifications and, as a consequence, from the clinical evolution of disease.
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