Unit:
Κατεύθυνση Γενική και Εξειδικευμένη Παιδιατρική: Κλινική Πράξη και ΈρευναLibrary of the School of Health Sciences
Author:
Palioura Alexia-Eleftheria
Supervisors info:
Ευαγγελία Χαρμανδάρη Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ, Επιβλέπουσα
Αθανάσιος Μίχος Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Μανθούλα Βαλάρη, Διευθύντρια ΕΣΥ, A' Παιδιατρική Κλινική, Νοσοκομείο Παίδων «Η Αγία Σοφία»
Original Title:
Κλινικά- εργαστηριακά ευρήματα και πρόγνωση, σε παιδιά με μαστοκυττάρωση
Translated title:
Clinical-laboratory findings and prognosis in children with mastocytosis
Summary:
BACKGROUND: Mastocytosis is characterized by the accumulation of mast cells in the skin and other organs. In children, it is mainly located in the skin and usually resolves spontaneously by adolescence.
AIM: To study the natural course, causative factors, prognostic factors, complications of cutaneous mastocytosis in children, and its impact on their quality of life.
METHODOLOGY: Patients under the age of 15 who were diagnosed with cutaneous mastocytosis between 2004 and 2009 were included in the study. Information was collected regarding symptoms, clinical manifestations, age of onset, laboratory findings, and the impact on the quality of life of children, with the assistance of a questionnaire for parents.
RESULTS: A total of 35 patients were followed up for 10 years. The male-to-female ratio was 2:1. In 82.8% of cases, the onset of the disease occurred before the age of one. Twenty patients had maculopapular cutaneous mastocytosis (MCM) (57%), 12 had solitary mastocytomas (SM) (34.2%), and 2 had diffuse cutaneous mastocytosis (DCM) (5.7%). Significant improvement or resolution after 10 years was observed in 83% of patients with SM and in all patients with MCM and DCM. A greater number of skin lesions, more pronounced symptoms, and elevated tryptase levels were associated with persistent disease. None of the patients developed systemic mastocytosis. Allergies were reported in 9 patients (25.7%), and one had type I diabetes mellitus. No patient reported a significant impact of mastocytosis on their quality of life.
CONCLUSIONS: Most children with mastocytosis experience partial or complete resolution of the rash before adolescence. Mastocytosis has minimal impact on the quality of life of young patients.
Main subject category:
Health Sciences
Keywords:
Cutaneous mastocytosis, Urticaria pigmentosa, Prognosis, Children
