Pediatric emergency medicine trisk 2964 2964
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Initial management of each of these GI catastrophes includes volume replacement,
gastric decompression, and stress doses of corticosteroids. Very large volumes of IV
fluids may be required. Technetium scan, angiography of the celiac axis vessels, and
peritoneal aspiration may be indicated in some cases, and direct examination of the GI
tract by endoscopy may yield valuable information concerning the nature, location, and
extent of lesions. Surgical consultation should be obtained immediately, and in the
presence of bleeding aneurysms or infarcted bowel, exploratory laparoscopy or
laparotomy should be performed as soon as the patient can be stabilized.
CNS Complications. Clinical signs of CNS disease are less frequent than those of
peripheral nervous system involvement. Seizures and hemiparesis are the most
common manifestations of CNS involvement in PAN. CT angiogram, MRI with MRA,
and/or carotid angiography may help localize the lesion.
Management of hypertensive encephalopathy and increased intracranial pressure are
described elsewhere (see Chapters 97 Neurologic Emergencies and 100 Renal and
Electrolyte Emergencies ). Surgical correction of a ruptured aneurysm should be
undertaken if the bleeding vessel can be localized and is accessible.
Miscellaneous Complications. As with all vasculitides, PAN may involve testicular
vessels, leading to acute scrotal pain and purpura and accompanying dysuria. Once
other causes of scrotal pain are excluded, including epididymitis and testicular torsion,
treatment may proceed with steroids and immunosuppressive medications.
JUVENILE DERMATOMYOSITIS
CLINICAL PEARLS AND PITFALLS
Patients are at risk for aspiration pneumonia and respiratory insufficiency
because of muscular weakness, including weakness of the diaphragm.
Poorly controlled disease may be associated with GI manifestations of
vasculitis, including GI bleeding.
A rare complication is the development of cardiac conduction abnormalities.
Current Evidence
JDM is a rare rheumatic disorder characterized by inflammation of the blood vessels,
skin, and striated muscle. The annual incidence rate is roughly three cases per 1 million
children in the United States. Girls are more often affected than boys (2:1), as is typical
of most autoimmune conditions. The mean age of onset is estimated at 6.9 years in the
United States, with almost 20% of patients diagnosed at 4 years of age or younger.
Before steroid therapy was available, as many as one-third of patients died of the
disease and another one-third developed permanent disabilities. More recently, the
