Pediatric emergency medicine trisk 2991 2991
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anti-ds DNA), and rarely patients may develop multiple sclerosis–like CNS
abnormalities. These associations should be kept in mind if a patient on anti-TNF
therapy develops new neurologic or rheumatologic complaints.
Sulfasalazine is a sulfa drug, and its most severe side effects are typical of this class
of medications. Headache and GI upset—especially with preparations that are not
enterically coated—occur most commonly. Although rare, more concerning are bone
marrow suppression, agranulocytosis, photosensitive eruptions, and hypersensitivity
reactions, including Stevens–Johnson syndrome.
Antimalarial agents such as hydroxychloroquine must be administered judiciously
because of their ability to cause irreversible ocular toxicity at high doses. Even at lower
doses, children may develop rashes, gastric upset, or reversible visual disturbances
secondary to altered accommodation. Finally, children with glucose-6-phosphate
deficiency who receive hydroxychloroquine may develop hemolytic anemia, especially
during intercurrent infections.
The long list of potential side effects of systemic corticosteroids is enumerated
elsewhere. In the acute setting, immunosuppressive effects of systemic steroids are
most salient. It is important to remember that these agents dramatically increase
susceptibility to herpes viruses (especially disseminated varicella) and intracellular
pathogens, such as mycobacteria and listeria. Although they have little effect on
susceptibility to other bacterial pathogens, their anti-inflammatory effects tend to mask
clinical signs of infection, accentuating the need for attentiveness on the part of
clinicians. In addition, patients receiving chronic corticosteroid therapy are at risk for
adrenal insufficiency, and treatment with stress dose steroids may be necessary in the
setting of acute illness or injury.
Management of Complications and Emergencies
Patients with all subtypes of JIA may have extra-articular complications either due to
their underlying disease or as a result of treatment with immunosuppressive
medications. It should be noted, however, that children with sJIA are at higher risk for
more severe complications of disease, including life-threatening cardiopulmonary
manifestations of disease as well as MAS, a cytokine storm syndrome. As such, extra
care to evaluate for such complications should be taken when children with sJIA
present to the ED.
Fever. Marked elevation of body temperature is characteristic of sJIA, whereas a lowergrade fever often accompanies polyarticular disease. The diagnosis of sJIA is one of
exclusion and fever is a common symptom, so diligent efforts should be made to rule
out infections and malignancies. This may require hospitalization for a diagnostic
evaluation, particularly in infants and young children. A bone marrow examination to
rule out malignancy may be necessary in patients who have or are suspected to have
