Pediatric emergency medicine trisk 2973 2973
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enzymes, weakness, or GI symptoms do not rapidly improve, steroid-sparing agents
such as methotrexate (0.75 to 1.25 mg/kg/wk), IV immune globulin (2 g/kg/mo), or
cyclosporine A (2 to 4 mg/kg/day) are introduced within 4 to 8 weeks. In more
recalcitrant cases, it may be necessary to add immunosuppressive drugs such as
cyclophosphamide or rituximab. Under all circumstances, the goal is to rapidly control
disease activity while minimizing toxicity from medications. Fortunately, active disease
generally does not recur if a complete remission can be induced and maintained for 1 to
2 years.
During the initial evaluation of JDM, it is essential to monitor the function of the
palatopharyngeal and respiratory muscles; palatal weakness increases the risk of
aspiration. Eating only in the upright position, frequent suctioning, or placement of a
nasogastric tube may be necessary to avoid aspiration. Support of weak muscles, such
as wearing a soft neck collar while riding in automobiles, helps minimize the risk of
complications until children regain their strength.
Management of Complications and Emergencies
The most serious emergencies in JDM relate to the respiratory and GI tracts ( Table
101.9 ). In addition, complications may occur as a result of therapy with corticosteroids
and immunosuppressive agents (e.g., infection and GI hemorrhage).
Respiratory Complications. (See also Chapters 71 Respiratory Distress and 99
Pulmonary Emergencies. ) Respiratory emergencies seen in JDM have diverse
etiologies. Entities to be considered include (i) aspiration pneumonia secondary to
weakness of velopalatine muscles; (ii) atelectasis and pneumonia secondary to
difficulty in clearing secretions as respiratory muscles become involved; (iii)
respiratory failure secondary to profound involvement of respiratory musculature,
including the diaphragm; (iv) progressive interstitial lung disease; and (v) opportunistic
infection (tuberculosis, fungi, viruses, or Pneumocystis ) in the immunocompromised
host. Because fever may also occur with active JDM, it is necessary to differentiate
pyrexia caused by infection from that caused by underlying disease.
In addition to usual care, preliminary investigations should include measurement of
muscle enzymes (including CK, aldolase, AST, ALT, and LDH) as well as vWF:Ag.
Depending on the seriousness of the symptoms and the cooperativeness of the child,
pulmonary function studies should be assessed.
If the etiology of the respiratory deterioration remains in doubt, more sensitive tests
of disease activity, including MRI of the thigh muscles, may be necessary to determine
whether more aggressive control of the underlying myositis is necessary.
Corticosteroids are essential for treating weakness of respiratory muscles and
interstitial lung disease. Plasmapheresis is reserved for children who deteriorate even
after pulse steroid therapy.
