0071643192.pdf

PEDIATRICS

■ Supportive care

■ Consider PRBC transfusion if hemoglobin < 6 g/dL.

■ Avoid platelet transfusion unless active bleeding or a surgical procedure is

required.

■ Plasma exchange, if CNS symptoms

COMPLICATIONS

■ About 40–50% of children with HUS develop renal failure and require

dialysis (with up to 70% of those who require dialysis eventually recovering

normal renal function).

■ About 15–20% have seizure or coma.

■ Mortality 3–5%

IMMUNOLOGY

Juvenile Rheumatoid Arthritis

The term JRA encompasses a variety of conditions (see Table 5.6), which all

share the common feature of joint pain lasting longer than 6 weeks. Affected

joints are painful, swollen, stiff, warm to the touch, often have reduced range

of motion, and may be erythematous. The etiology of JRA is unknown, with

possible connections to antecedent viral infections or related to host immune

characteristics.

SYMPTOMS/EXAM

Variable, often with at least some of the following:

■ Warm, swollen joint

■ Morning stiffness that improves with activity

■ Increased pain after periods of rest

■ Low-grade fever

■ Fatigue

■ Anorexia and weight loss

Systemic onset JRA typically includes some combination of the following

symptoms:

■ Daily, spiking fever

■ Rash (transient macules on torso, proximal extremities)

TABLE 5.6. Types of JRA

NUMBER SYSTEMIC AGE AT

OFJOINTS SYMPTOMS ONSET SEX ANA

Polyarticular Usually ≥ 5 Mild or Early–late > Positive 25%

onset symmetric moderate

childhood

Pauciarticular <5; mostly Iridocyclitis Early > Positive 60%

onset knees, ankles, (anterior uveitis) childhood

elbows

Systemic onset Variable See text Any age > Negative

Avoid antibiotics and

antidiarrheal agents in

children who have

hemorrhagic diarrhea; they

increase risk of HUS!

JRA is the most common form

of arthritis in children.