Pediatric emergency medicine trisk 2977 2977
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Calcinosis. During the period of formation of subcutaneous calcification, children with
JDM may develop high fever, chills, and one or more areas of swelling under the skin.
The inflammation caused by the subcutaneous calcium deposit may be
indistinguishable from that of cellulitis or abscess formation, with warmth, erythema,
and tenderness. Eventually, the lesion may spontaneously extrude calcium, at which
time the fever often subsides. Although this is the natural history of subcutaneous
calcifications, it is often hard to exclude an infectious etiology. If doubt exists, needle
aspiration of the site may be performed and the fluid examined for calcium crystals and
organisms. In the face of uncertainty, it is best to treat for infection with antibiotics
until culture results are available. Incision and drainage or surgical debridement should
be avoided, as the inflamed skin rarely heals satisfactorily. Complete control of the
underlying disease offers the best hope for resolution of calcinosis, although this may
be incomplete or may require many years.
Cardiac Emergencies. Although EKG abnormalities may be seen in up to 50% of
children with JDM, development of myocarditis is uncommon. Involvement of the
conduction system by edema and fibrosis leads to electrical abnormalities and
dysrhythmias.
BEHÇET DISEASE
BD is a rare vasculitis in children, especially in nonendemic areas such as the United
States. BD is the only vasculitis that affects both arteries and veins. The classical
description of BD is a clinical triad consisting of recurrent buccal aphthous ulcers,
recurrent genital ulcers, and uveitis with hypopyon. In addition to these cardinal
features of BD, there are a host of associated clinical manifestations, including arthritis,
neurologic involvement, GI manifestations, vascular/thrombotic disease, and various
dermatologic lesions, including erythema nodosum and necrotic folliculitis.
Clinical Considerations
Recurrent oral ulcerations are the most common presenting sign and ongoing
manifestation of pediatric BD. While ulcerative mucocutaneous lesions are far from
specific for BD, the oral lesions in BD tend to scar, unlike those associated with
inflammatory bowel disease, SLE, chronic oral aphthosis, and Sweet syndrome.
Although oral and genital ulcers may markedly negatively impact quality of life,
there are other less common but more serious complications of BD that may lead to
significant morbidity and even mortality. Ocular disease can be devastating, ultimately
resulting in blindness. GI disease can result in perforation. Neurologic complications
are varied, including headache, meningoencephalitis, idiopathic intracranial
hypertension, and quadriparesis. Psychiatric symptoms, including depression,
personality changes, and memory loss, are also reported. Vascular/thrombotic
complications are a particularly ominous development in BD patients; these can
include dural sinus thrombosis and arterial lesions. In one multinational pediatric BD
