Pediatric Primary Care (76 page)

1.  Fever.

2.  Reddened, edematous, indurated palms/soles progressing to desquamation. May be unwilling to walk/use hands to grasp objects.

3.  Erythematous rash; most prominent on trunk, groin.

4.  Bilateral conjunctival injection; particularly bulbar conjunctiva; nonexudative.

5.  Red or peeling lips, strawberry tongue, injected oral, pharyngeal mucosa.

6.  Cervical lymphadenopathy.

7.  Irritability: particularly in infants.

8.  Evidence of vasculitis in other systems.

a.  Cardiovascular: murmur, gallop, ECG changes, cardiomegaly, peripheral arterial aneurysms.

b.  Gastrointestinal: diarrhea, vomiting, abdominal pain, ileus, jaundice, increased liver enzymes, distention of gallbladder.

c.  Hematologic: elevated WBC count, elevated platelet count.

d.  Renal: proteinuria, hypoalbuminemia.

e.  Respiratory: cough, rhinorrhea.

f.  Joint: pain, swelling.

g.  CNS: pleocytosis of cerebral spinal fluid (CSF), seizure.

E.  Diagnostic tests.

1.  No specific laboratory test to confirm diagnosis.

2.  Diagnostic criteria to establish diagnosis.

3.  Fever of greater than or equal to 5 days and 4 of the following 5 criteria:

a.  Erythematous, edematous, indurated palms/soles progressing to desquamation later in course.

b.  Polymorphous exanthem.

c.  Bilateral conjunctival injection.

d.  Red or peeling lips, strawberry tongue, injected oral, pharyngeal mucosa.

e.  Cervical lymphadenopathy.

f.  OR: Fever of greater than or equal to 5 days, less than 4 of the above 5 criteria, and coronary aneurysms documented by echocardiography or cardiac angiography.

4.  Kawasaki should be considered in any young child with unexplained fever for 5 or more days, leukocytosis, elevated erythromycin sedimentation rate or C-reactive protein, and thrombocytosis.

a.  “Incomplete” Kawasaki, with symptoms inadequate to meet classic criteria is more common in younger than 1 year of age and adolescents.

b.  Echocardiography may show evidence of coronary arteritis.

F.  Differential diagnosis.

1.  Infectious illnesses, particularly streptococcal and staphylococcal infections.

2.  Drug reactions or hypersensitivity reactions.

3.  Stevens-Johnson syndrome: erythema, swelling, peeling all advance beyond hands, feet.

4.  Other vasculitic diseases.

5.  Polyarteritis nodosa, systemic lupus erythematosus.

6.  Juvenile rheumatoid arthritis: smaller joints, morning stiffness, fingers involved, joint deformities.

7.  Rheumatic fever: positive antistreptococcal antibody.

8.  Viral exanthems: do not meet all criteria for Kawasaki disease.

9.  Measles: rash more prominent on face and neck.

10.  Toxic shock syndrome: sandpaper rash, more toxic-appearing child, often hypotension with evidence of shock.

G.  Treatment.

1.  Referral to pediatrician and specialists.

2.  Hospitalization.

3.  Aspirin.

a.  Used for antipyretic, anti-inflammatory, and antiplatelet effects.

b.  High-dose aspirin for at least 48 hours.

c.  Low-dose aspirin to complete 6-8 weeks of therapy if no coronary artery abnormalities.

d.  Aspirin continued indefinitely if coronary artery aneurysms, second antiplatelet agent may be added.

4.  IV immunoglobulin.

a.  Decreases incidence of coronary artery aneurysms.

b.  Mechanism of protection unknown.

c.  If fever persists > 36 hours after IVIG repeat IVIG dose.

H.  Follow up.

1.  Pediatric cardiology for echocardiographic evaluation of coronary artery aneurysms. Aneurysms may not develop until after acute stage of illness.

I.  Complications.

1.  Coronary artery aneurysms: develop in < 20% of individuals, fewer if treated with immunoglobulin.

2.  If coronary artery aneurysms develop, increased risk of coronary artery thrombosis, stenosis, and resultant myocardial infarction.

3.  May also develop valvular heart disease; mitral valve is affected more frequently than aortic valve.

4.  Pericarditis, myocarditis, pericardial effusion, congestive heart failure may occur.

5.  Can have development of vascular aneurysms elsewhere in body.

6.  Can have liver dysfunction from intrahepatic vasculitis.

J.  Education.

1.  Serious nature of illness.

2.  Importance of specialty care and follow up.

3.  Possibility of complications after initial phase of illness.

4.  Importance of adherence to prescribed medication: aspirin.

5.  Importance of not using aspirin to treat fever in other childhood illnesses: association with Reye's syndrome.

6.  Consider influenza vaccine due to association of Reye's syndrome with flulike illnesses and aspirin.

V.  RHEUMATIC FEVER

A.  Etiology.

1.  Complication of Group A beta-hemolytic streptococcal (GABHS) pharyngitis.

2.  Thought to be unique host factors that make certain individuals more susceptible to rheumatic fever after GABHS pharyngitis.

3.  Rheumatic fever is classified as collagen vascular disease or connective tissue disease resulting in diffuse vasculitis.

B.  Occurrence.

1.  Most common in underdeveloped countries but resurgence in United States.

2.  Occurs in 2-3% of patients with untreated GABHS.

3.  Rheumatic fever is rare in infants, toddlers. Most cases younger than 5 years of age.

C.  Clinical manifestations.

1.  Inflammation of joints and heart.

2.  Rarely, inflammation of brain and skin.

D.  Physical findings.

1.  Migratory polyarthritis.

a.  Occurs in 75% of initial episodes, 50% of recurrent episodes.

b.  Arthritis is typically of larger joints (knees, elbows, wrists, ankles).

c.  Each joint typically affected for < 1 week, often overlap so that more than 1 joint is affected at a time.

d.  Joints are painful to touch, red, swollen; painful with movement.

2.  Carditis.

a.  Up to 50% of patients with rheumatic fever have evidence of carditis.

b.  May manifest as myocarditis with poor function, valvular disease with audible murmur, pericarditis with friction rub, or as general symptoms of tachycardia, cardiomegaly, pulmonary edema, or symptoms of congestive heart failure.