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[Mastocytosis in children]. | LitMetric

[Mastocytosis in children].

Med Sestra

Published: March 1986

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Background: Hymenoptera venom allergy is a potentially life-threatening allergic reaction. Venom immunotherapy (VIT) is recommended to prevent severe allergic reactions. Field stings indicate the effectiveness of immunotherapy.

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Mastocytosis is characterized by the clonal expansion of mast cells, with deposition into various organs and variable clinical presentation depending on subtype. It generally results from a mutation in the KIT gene, which encodes for production of receptor tyrosine kinases, the constitutive activity of which results in abnormal cell growth and proliferation. In pediatric patients, the cutaneous mastocytosis (CM) form predominates, and systemic mastocytosis (SM) is rarely reported.

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Cutaneous mastocytosis (CM) is a rare skin disease with limited data on its prevalence across different racial groups. This retrospective cohort study examines the characteristics of CM at Nationwide Children's Hospital (NCH) from January 2010 to June 2022, identifying 192 confirmed cases of CM and further stratifying this cohort to 155 patients with documented Fitzpatrick phototypes. There were two important findings: first, nine (4.

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Article Synopsis
  • CD2, CD25, and CD30 expression in extracutaneous mast cells is a minor diagnostic criterion for systemic mastocytosis (SM), as categorized by the World Health Organization and International Consensus Classification.
  • A study of 5,034 patients revealed that lower percentages of CD2, CD25, and/or CD30 in mast cells are seen in indolent SM compared to advanced forms like aggressive SM and mast cell leukemia.
  • The absence of CD2 in mast cells is linked to significantly lower overall survival and indicates the potential for disease spread outside the bone marrow to organs like the spleen and liver.
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This literature review aims to comprehensively evaluate the clinical and dermoscopic presentations of common pediatric diseases among children with skin of color (SoC) while also addressing potential variations based on racial backgrounds. This review encompasses various conditions, such as nevi subtypes, viral infections, infestations, and inflammatory dermatoses, as well as hair diseases and abnormal vascular formations, occurring in pediatric populations. Overall, we identified 7 studies on nevi subtypes, 24 studies on skin infections, 6 on inflammatory dermatoses, 10 on hair diseases and disorders, and 14 on miscellaneous disorders that also satisfied our SoC- and race-specific criteria.

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