THÔNG TIN TÀI LIỆU
against the cytokines responsible for the hyperactive response are now widely used in in the treatment of MAS Specifically, IL1-R antagonists, like anakinra (Kineret), can lead to rapid clinical improvement in children and are now used alone or in conjunction with corticosteroids as first-line therapy In addition, adjunctive therapy with IVIG may be used, especially if there is concern for infection Additional medications that may be used include the calcineurin inhibitors Notably, a new medication that blocks interferon gamma, emapalumab, was recently approved to treat refractory FHL and may have use in the treatment of MAS HLH and MAS are severe life-threatening immune dysregulatory syndromes that may progress rapidly when untreated As such, prompt consideration for these pathologic processes with early decision to admit to the hospital is essential to the successful care of these patients As the process evolves, affected children may develop sepsis-like physiology with cytokine storm and ultimately cardiopulmonary collapse Thus, they often require hemodynamic and respiratory support in addition to the treatment for potential infectious complications related to both treatment with immunosuppressive medications and underlying illnesses KAWASAKI DISEASE CLINICAL PEARLS AND PITFALLS Fever, often exceeding 40°C, is the most consistent manifestation of the disease Extreme irritability is commonly observed Initiation of IVIG within the first 10 days of the illness shortens disease duration and minimizes complications Some children present with a clinical picture that does not fulfill the classic clinical criteria (incomplete KD) The majority of complications are cardiac in nature so close monitoring of the cardiac system is imperative Infants ≤6 months should have laboratory studies if febrile for days or longer, even if there are no other clinical manifestations of KD present Current Evidence KD is an acute, self-limited vasculitis that occurs in children of all ages It is an idiopathic vasculitis of medium-sized vessels that has surpassed acute rheumatic fever to become the leading cause of acquired heart disease in children in the developed world KD is 50% more common in boys than in girls, and it usually affects children younger than years of age The highest incidence occurs in children who live in East Asia (e.g., Japan, Korea, and Taiwan) or are of Asian ancestry living elsewhere in the
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