Pediatric emergency medicine trisk 2953 2953
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Esophagitis
Retrosternal pain,
pyrosis, melena
CBC
Barium swallow
Esophageal ph probe
and manometry
Antacids/cimetidine
Surgical manipulation
for chronic
unremitting
complaints
EKG, electrocardiogram; CO, carbon monoxide; CT, computed tomography; ACE, angiotensin-converting enzyme;
PA, posteroanterior; BUN, blood urea nitrogen; CBC, complete blood count.
Pulmonary hypertension is the most common cause of dyspnea in patients with JSSc.
On auscultation, there is a wide or fixed splitting of the second heart sound and the
pulmonic component is accentuated. The EKG shows right ventricular hypertrophy.
Echocardiography and right heart catheterization may be necessary to differentiate
cardiac from pulmonary etiologies of respiratory deterioration. Corticosteroids and
cyclophosphamide (50 mg/day orally or 500 to 750 mg/m2 by monthly IV infusion) are
the treatment of choice in patients without established interstitial fibrosis.
Renal Complications. Sclerodermatous involvement of the vessels of the kidney is the
most common cause of renal failure in adults with JSSc. Risk factors include
proteinuria, hypertension, rapid progression of skin thickening early in the illness,
anemia, pericardial effusion, and CHF. The development of a microangiopathic
hemolytic anemia suggests imminent renal failure. These complications appear to be
less common in children than in adults.
Renal failure may develop gradually or acutely in a patient with known renal
disease, and use of corticosteroids may precipitate its appearance. Scleroderma renal
crisis is characterized by precipitous hypertension. Immediate investigation should
include urinalysis, measurement of urine output and urinary electrolytes, serum
electrolytes, BUN, creatinine, and plasma renin level.
A major advance in the pharmacologic management of scleroderma renal crisis has
been the use of ACE inhibitors such as captopril. Patients who fail to respond to this
drug may still respond to potent vasodilators such as minoxidil, along with β-blockers
and diuretics; regimens involving multiple drugs may also be necessary (see Chapters
37 Hypertension and 100 Renal and Electrolyte Emergencies ). Because most patients
with severe scleroderma renal disease have a component of myocarditis and ventricular
stiffness, maintenance of blood volume is essential to ensure adequate preload to
support the circulation.
Peripheral Vascular Complications. RP can often be incapacitating, particularly in cold
weather. Symptoms include severe pain in the extremities and loss of sensation in the
tips of the digits. Treatment with calcium-channel antagonists, such as slow-release
nifedipine, may decrease the frequency or severity of attacks. In urgent cases with
impending gangrene, systemic (e.g., iloprost) or topical vasodilators (e.g.,
