Systemic mastocytosis in a 5-year-old child: successful treatment with disodium cromoglycate. 1984

L Businco, and A Cantani, and E Businco, and J Pepys

Most clinical signs and symptoms of systemic mastocytosis (SM) are attributed to histamine release. We report here a 5-year-old male child with SM, who suffered from the age of 4 months from disseminated skin lesions, vomiting, diarrhoea, abdominal pain, flushing, tachycardia, hypotension, somnolence, and transient blindness, triggered by heat and egg ingestion. Oral disodium cromoglycate (DSCG) or placebo were started in a single blind trial at a dose of 100 mg/kg/day in four divided doses. The child was studied for 21 months during the administration of three courses of DSCG, each of 6 months' duration, interspersed with three 1-month courses of placebo. During treatment with DSCG all the systemic manifestations improved, and the histaminaemia decreased. During the placebo periods the symptoms, signs, and histaminaemia recurred.

UI MeSH Term Description Entries
D007073 Immunoglobulin E An immunoglobulin associated with MAST CELLS. Overexpression has been associated with allergic hypersensitivity (HYPERSENSITIVITY, IMMEDIATE). IgE
D008297 Male Males
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004205 Cromolyn Sodium A chromone complex that acts by inhibiting the release of chemical mediators from sensitized MAST CELLS. It is used in the prophylactic treatment of both allergic and exercise-induced asthma, but does not affect an established asthmatic attack. Disodium Cromoglycate,Sodium Cromoglycate,Aarane,Bicromat Spray,Cromoglicic Acid,Cromoglycate,Cromoglycic Acid,Cromolyn,FPL-670,Intal,Lomudal,Nalcrom,Nasalcrom,Opticrom,Vicrom,Acid, Cromoglicic,Acid, Cromoglycic,Cromoglycate, Disodium,Cromoglycate, Sodium,FPL 670,FPL670
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012882 Skin Tests Epicutaneous or intradermal application of a sensitizer for demonstration of either delayed or immediate hypersensitivity. Used in diagnosis of hypersensitivity or as a test for cellular immunity. Skin Test,Test, Skin,Tests, Skin
D014582 Urticaria Pigmentosa The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules. Maculopapular Cutaneous Mastocytosis,Cutaneous Mastocytoses, Maculopapular,Cutaneous Mastocytosis, Maculopapular,Maculopapular Cutaneous Mastocytoses

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