• Evaluate ABCs. Note general appearance, color, and mental status. Check for increased work of breathing; assess perfusion by checking capillary refill and pulses.
• Vital signs: Tachycardia is a sign of compensated shock. Hypotension signifies decompensated shock
• Pay close attention to oral mucosa. Bright red lips or strawberry tongue suggest Kawasaki disease. Ulcerative lesions of erythema multiforme (EM) or vesicles of enterovirus are diagnostic clues.
• Note altered mental status (confusion, agitation, or lethargy) and meningeal signs such as a positive Kernig or Brudzinski sign.
• Perform a complete joint examination. There may be a painless joint effusion in Lyme disease. Joint swelling, warmth, morning stiffness or pain may occur in rheumatologic processes such as juvenile idiopathic arthritis (JIA).
• The rashes of RMSF, ehrlichiosis, erythema multiforme, Stevens-Johnson syndrome, enterovirus, group A streptococcus, Kawasaki disease, and drug reactions may involve palms and soles.
Tests for Consideration
• Complete blood count (CBC) with differential:
Leukopenia suggests overwhelming infection or viral suppression;
Leukocytosis and thrombocytosis are nonspecific for infection;
Thrombocytopenia in sepsis, RMSF, and ehrlichiosis;
Thrombocytosis in Kawasaki disease $116
• Blood culture: If ill-appearing $152
• Complete metabolic panel: Hyponatremia occurs in RMSF and other tick-borne illnesses $237
• Liver transaminase levels: Elevated levels may signal organ inflammation or inadequate perfusion $132
• RMSF acute and convalescent titers $349
• Group A streptococcus antigen $148
• Rapid respiratory panel to check for adenovirus $325
• Lumbar puncture if clinically indicated:
• Enterovirus polymerase chain reaction (PCR) $300
• Lyme PCR $180
• Gram stain $180
• Culture $152
• Cell count $150
• Glucose and protein $75
• Urinalysis: To check for hematuria, proteinuria, or casts $95
• Urine culture $148
• Prothrombin time (PT) and partial thromboplastin time (PTT): If petechiae and/or purpura $105
• Erythrocyte sedimentation rate (ESR): To monitor inflammatory response in HSP or Kawasaki disease $85
• Renal biopsy: For persistent HSP $1325
IMAGING CONSIDERATIONS
→Head computed tomography (CT): Before lumbar puncture if altered mental status $1827
→ Echocardiogram in Kawasaki disease: To identify coronary artery aneurysms $1630
→ Abdominal ultrasound: If suspect Henoch-Schönlein purpura with intussusception $846
Kawasaki Disease |
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Pφ | KD represents a febrile multisystem vasculitis with preferential involvement of medium-size arteries. Inflammation may involve all three layers of the vessel wall, with possible aneurysm formation. An infectious etiology has been postulated. |
TP | The child is extremely irritable. In classic Kawasaki disease, there is fever for at least 5 days, with at least four of the following:- 1
Bilateral nonexudative conjunctivitis - 2
Erythema of oral and pharyngeal mucosa with strawberry tongue, dry cracked lips - 3
Edema and erythema of hands - 4
Rash - 5
Cervical lymphadenopathy, unilateral >1.5 cm
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In incomplete or atypical Kawasaki disease, patients present with 5 days of fever and fewer than four additional findings. This is more common in infants under 1 year, in whom diagnosis is more difficult but who have higher rates of coronary artery aneurysms. Also possible are myocarditis, hydrops of the gallbladder, mild hepatitis, aseptic meningitis, arthritis, and urethritis. |
Dx | Diagnosis is clinical, and there are three phases:- 1
Acute febrile phase lasts 1 to 2 weeks: fever and other signs and symptoms, including perineal desquamation - 2
Subacute phase lasts 2 to 4 weeks. Fever and other symptoms have resolved, but irritability, anorexia, and conjunctivitis persist; new periungual desquamation of fingers and toes, marked thrombocytosis of up to 1,000,000/mm 3 , and coronary artery aneurysm formation. - 3
Convalescent phase up to 8 weeks from onset; all symptoms resolve. ESR and C-reactive protein (CRP) are normal. Echocardiogram is done at diagnosis and if normal, is repeated at 2 to 3 weeks and again 6 to 8 weeks after onset. |
Tx | Intravenous immunoglobulin (IVIG) 2 g/kg within 10 days of onset decreases symptoms and markedly reduces coronary artery aneurysm formation. Children with KD are also treated with high-dose aspirin (antiinflammatory dosing) for 14 days, or until afebrile for 3 to 4 days, followed by low-dose aspirin (antithrombotic dosing) until 6 to 8 weeks from onset. Persistent or recurrent disease may require a second course of IVIG. Factors associated with poor outcome include male gender, age <1 year, prolonged fever, or fever recurrence after an afebrile period. 1 See Nelson Essentials 88. |