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Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis.
- Source :
- Allergy; Jun2012, Vol. 67 Issue 6, p813-821, 9p
- Publication Year :
- 2012
-
Abstract
- Background Despite the good prognosis of pediatric mastocytosis, some patients suffer from severe mast cell ( MC) mediator-associated symptoms. The aim of this study was to identify predictors for severe MC mediator release symptoms in children with mastocytosis in the skin ( MIS). Methods Serum baseline total tryptase (sb T) levels in 111 children with MIS - 80 maculopapular cutaneous mastocytosis/plaque mastocytosis, 22 nodular mastocytosis, and nine diffuse cutaneous mastocytosis - were investigated as a predictive biomarker for the occurrence of MC mediator-related signs and symptoms within the first 18 months after disease onset. Results Twelve children (11%) who showed extensive cutaneous disease involving >90% of body surface area ( BSA) suffered from severe symptoms requiring hospitalization, with ( n = 5) or without ( n = 6) management in the intensive care unit ( ICU) owing to life-threatening complications. The median sbT was significantly ( P < 0.001) higher in patients with extensive cutaneous disease vs those with <90% of BSA involved (45.5 vs 5.2 μg/l, respectively), as well as in children with grade 4 (severe mastocytosis-related symptoms requiring emergency therapy and hospitalization) vs those with grade <4 (46.2 vs 5.2 μg/l, respectively). Receiver operating characteristics curve analyses showed that the optimal cutoff s for sbT to predict the need for daily antimediator therapy, hospitalization, and the management in an ICU were 6.6, 15.5, and 30.8 μg/l, respectively (sensitivity and specificity of 77% and 79%, 100% and 95%, and 100% and 96%, respectively). Conclusions Increased sb T in association with extensive cutaneous involvement identifies patients at risk for severe MC activation events in pediatric mastocytosis. [ABSTRACT FROM AUTHOR]
- Subjects :
- MAST cell disease
SKIN diseases
TRYPTASE
INTENSIVE care units
PEDIATRICS
Subjects
Details
- Language :
- English
- ISSN :
- 01054538
- Volume :
- 67
- Issue :
- 6
- Database :
- Complementary Index
- Journal :
- Allergy
- Publication Type :
- Academic Journal
- Accession number :
- 75061718
- Full Text :
- https://doi.org/10.1111/j.1398-9995.2012.02812.x