PD34 - Childhood mastocytosis: serum baseline total tryptase levels and extent of cutaneous disease as predictors of mast cell mediator release symptoms
نویسندگان
چکیده
Methods A total of 25 children (median age of onset 14,2 months (range, 0 mth–12ys) diagnosed with mastocytosis have been studied. Patients were classified according to the type of skin lesions following the WHO classification. Mastocytosis-related symptoms and data on specific triggering factors were systematically recorded for 3 years after diagnosis. Severity was graded according to standard recommendations. Serum baseline total tryptase (sbT) levels (CAP;Phadia Diagnostics) were measured at the time of referral. The extent of cutaneous disease was determined using the technique used to assess burns and is given as percentage of total body surface area (BSA).
منابع مشابه
Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis.
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 (sbT) levels in 111 children with MIS - 80 maculopapular cutaneous mastocyto...
متن کاملThe role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience
INTRODUCTION In children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determination is recommended in the diagnosis and follow-up. AIM To compare clinical presentation and the cou...
متن کاملSerum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis.
BACKGROUND Skin lesions are the predominant clinical feature of the commonest form of mastocytosis. Mastocytosis is classified according to World Health Organization criteria. Determination of the levels of mast-cell mediators or their metabolites reflects the mast-cell burden. The extent of cutaneous mastocytosis can be assessed clinically using a scoring system (SCORing MAstocytosis; SCORMA I...
متن کاملPresence or absence of elevated acute total serum tryptase by itself is not a definitive marker for an allergic reaction.
713 March 2015 To the Editor: We read with interest the study by Laroche et al.1 who examined the value of plasma histamine and serum or plasma tryptase in evaluating severe perioperative anaphylaxis. We would like to add some thoughts to further explore and clarify the significance of tryptase as a “predictable marker of allergic reaction.” Indeed, elevated serum total tryptase associated with...
متن کاملCutaneous Mastocytosis with Persistent Blistering: Successful Treatment with Methylprednisolone and 3-Year Follow-Up Management
BACKGROUND Mastocytosis is a rare disorder with diverse clinical manifestations. In cutaneous mastocytosis the mast cell infiltration is limited to the skin, but is often associated with systemic symptoms due to the release of mast cell mediators. CASE REPORT We report a 6-month-old male infant who had skin lesions of various morphologies (macules, papules, plaques, and nodules) and sizes, pe...
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عنوان ژورنال:
دوره 4 شماره
صفحات -
تاریخ انتشار 2014