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Pediatric emergency medicine trisk 2954 2954

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nitroglycerine paste or intra-arterial reserpine) may be tried. Excessive peripheral
vasodilation may also precipitate cardiovascular collapse due to a “steal syndrome,” so
caution must be exercised to ensure cardiac filling pressures are maintained.
Consequently, these procedures should be performed only with intensive monitoring. If
gangrene has developed and there is no infection, spontaneous separation of the tips of
the digits will occur and carries less risk and morbidity than surgical amputation.
GI Complications. Abnormal esophageal motility with reflux may result in esophagitis.
The major symptom of this condition is retrosternal pain that is made worse by certain
foods and recumbent positioning. The pain may be severe and incapacitating, and the
risk of aspiration is increased. Although children with the complaint of retrosternal pain
do not require admission to the hospital, they often need an evaluation of their lower
esophageal sphincter with esophageal manometry.

Mixed Connective Tissue Disease
Mixed connective tissue disease (MCTD) is a rare disorder among pediatric patients.
The median age at onset is approximately 11 years, and like many other rheumatologic
conditions occurs more frequently in girls than in boys by a ratio of 3:1. MCTD was
initially described by Sharp et al. in 1972, and includes features of rheumatoid arthritis,
scleroderma, SLE, and dermatomyositis (DM). RP and polyarteritis are the most
common manifestations at onset. Children present with features of more than one
connective tissue disease, and have a speckled ANA pattern and high titers of antiRNP.
There is no specific treatment for pediatric MCTD and treatment is typically directed
at an individual’s particular disease manifestations. Many patients will respond to lowdose corticosteroids, NSAIDs, hydroxychloroquine, or a combination of these
medications. Patients with severe myositis, renal, or visceral disease often require highdose corticosteroids and sometimes cytotoxic agents.
Pediatric MCTD is associated with a higher risk of nephritis than adult MCTD.
Other complications include pulmonary hypertension, pulmonary fibrosis, severe
thrombocytopenia, and esophageal dysfunction.

VASCULITIS
CLINICAL PEARLS AND PITFALLS
Vasculitis may affect any body system and may lead to life-threatening


complications, including stroke, myocardial infarction, hypertensive crisis,
and acute renal failure.
Systemic signs of inflammation are often present.



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