Pediatric emergency medicine trisk 3001 3001

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 7 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 5 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