Kawasaki disease By: Brittni McClellan

Kawasaki disease - description An idiopathic, multisystem disease of young children Characterized by vasculitis of the small- and medium-sized blood vessels. The acute phase is self-limited;. Signs and symptoms evolve over the 1st 10 days, then resolve spontaneously. 20–25% of untreated patients will develop coronary artery aneurysms. Etiology: Unknown

Kawasaki disease – H&P Patient History: 3 Phases: Acute Phase (1-2 Weeks from Onset) Febrile: >39 degrees C Oral Changes Rash – Prone to Perineal Area Edema/Erythema of Feet Painful, Limits Ambulation Subacute Phase (2-8 Weeks from Onset) Gradual Improvement, Decrease in Fever Desquamation of the Perineal Area, Palms, Soles, and Periungal Areas Coronary Artery Aneurysms/MI Arthritis/Arthralgias Convalescent Phase (Months-Years from Onset) Resolution of Remaining Symptoms/Normal Labs Aneurysms may resolve or be persistent.

Kawasaki disease – H&P Physical Exam: High, Unremitting Fever for 1-2 Weeks Unilateral, Cervical Rash: Erythematous and Polymorphous Aseptic Meningitis Prominent on Trunk, Perineum Pancarditis Maculopapular, May Coalesce Tachycardia, Gallop Rhythms Conjunctivitis: Bilateral Muffled Heart Sounds, Signs of CHF, or Murmurs Oral Changes Abdominal Changes Erythema, Fissures, Crusting of Lips RUQ Mass Abdominal Pain Diffuse Oropharyngeal Erythema Diarrhea Strawberry Tongue Hydrops of Gallbladder Extremity Changes Hepatosplenomegaly/Jaundice Metatitis/Vulvitis Erythema/Desquamation of the Palms/Soles Beau Lines (Transverse Grooves Across Fingernails) Arthritis/Anthralgias Adenopathy Uveitis

Kawasaki disease – signs and symptoms

Kawasaki disease - Diagnosis Fever for >45 Days >4 of the Criteria: Bilateral nonexudative conjunctival injection Polymorphous, nonvesicular rash Mucosal involvement of the upper respiratory tract that may include erythema, fissured lips, crusting of the lips and mouth, strawberry tongue, or injected, nonexudative pharyngitis. Edema or erythema of the hands and feet Cervical adenopathy (>1.5 cm diameter), which is often unilateral

Kawasaki disease - Diagnosis CBC: WBC usually increased with a left shift; >15,000 Anemia for age Elevated ESR and C-reactive protein Other laboratory abnormalities: Sterile pyuria (70%) Mild increases in hepatic transaminases A CSF pleocytosis with a normal protein/glucose Mild hypoalbuminemia Imaging Chest x-ray: Dilated heart during acute phase Echocardiography: Decreased shortening fraction and effusion Coronary ectasia, dilation, or aneurysms may be detected as early as 6 days into the illness; peak onset is between 3 and 4 weeks.

Kawasaki disease – treatment Medication IV Immunoglobulin Aspirin Complications: Aneurysms: Noted 12-28 days after onset of disease May Thrombose, or Rupture Reticulocyte count increases in 3-4 days

Question 1 A 9 month old boy is brought to the clinic because he has had temperatures up to 104°F. He has been fussy, had been eating less, and his mother explains that the fever began 5 days ago. The child does not have any close sick contacts. His temperature is currently 103.5°F. A physical exam reveals the following findings: cracked fissured lips, redness of oral mucosa, redness of conjunctiva, enlarged cervical lymph node. There is also a maculopapular rash over the trunk and dorsal edema of the hands. The patient’s hematocrit is 32%, leukocyte count is 15,000/mm³, and platelet count is 560,000/mm³. What is the most feared complication of this disease? Hepatosplenomegaly and Jaundice Erythematous, Maculopapular Rash, covering over 90% of the body surface area. Aneurysms, especially 3-4 weeks post- disease. Anaphylaxis from the IVIG used to treat the disease. Aneurysms, especially during the first week of the disease. c) Aneurysms, especially 3-4 weeks post-disease.