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With Kawasaki Disease, Time Is Coronary Health Clinical AND Health Affairs With Kawasaki Disease, Time is Coronary Health BY CLAIRE JANSSON-KNODELL AND RHAMY MAGID, M.D. previously healthy 3-year-old Hmong his shins believed to be an allergic reac- per minute). His blood pressure was boy presented to Children’s Hospitals tion. During a follow-up visit to his pe- 110/66 mm Hg, and he was irritable. He A and Clinics of Minnesota with a his- diatrician, the boy had a low-grade fever, weighed 15.2 kg. His sclerae were injected tory of fever that was unremitting despite swelling in his legs, and an erythematous bilaterally without exudate. His lips ap- antipyretics. Two weeks prior to admission rash. He was referred to the hospital, but peared bright pink with cheilosis with- at Children’s, he presented to an outside his mother chose to keep him home be- out frank cracking. His oropharynx was hospital with a fever accompanied by left- cause she assumed he had improved. He erythematous. He did not have cervical sided neck swelling. A neck ultrasound was “playful and interactive” at home and lymphadenopathy. His hands and feet were showed a lymph node measuring 3.5 x the neck swelling had lessened. A week edematous bilaterally. He had no rash, pe- 2.7 x 2.2 cm, without abscess or fluid col- later, the child returned to the clinic with techiae or ecchymosis. He had nonbleed- lection. He was treated for acute cervical persistent fever and pain in his foot that ing desquamation of his hands circumfer- lymphadenitis with antibiotics (ceftriaxone caused him to limp. The child also had entially around his thumbs. initially, then ampicillin-sulbactam). His red eyes, which his mother attributed to The boy had a WBC of 25,000/mm3 undulating fevers continued, but he was frequent crying. The pediatrician sent the with a neutrophil predominance, hemo- discharged after two days with oral antibi- boy directly to Children’s Hospital for ad- globin level of 8.1g/dL and a platelet count otics (amoxicillin-clavulanate). At home, mission. of 895,000/mm3. He had a mild transa- his family discontinued this medication On exam, the boy had a fever of minitis with an alanine aminotransferase after his mother noticed a spotted rash on 103.1° F and was tachycardic (161 beats level of 60 U/L. His C-reactive protein was 9.48 mg/dL and his erythrocyte sedimen- TABLE tation rate was >140 mm/hour. This boy’s history, physical exam and Clinical Criteria for Diagnosis of Kawasaki Disease lab values were suggestive of Kawasaki Fever of at least five days disease (Table).1 Because of his late pre- Presence of four or more of the following clinical features: sentation, there was concern for coronary • Conjunctival injection: bilateral, painless, without exudate aneurysm. He was immediately started • Cervical lymphadenopathy: >1.5 cm, usually unilateral on intravenous immune globulin therapy Oral mucosal changes: erythema and cracking of lips, strawberry tongue, diffuse injection of oral and • (IVIG) and high-dose aspirin. pharyngeal mucosa • Polymorphous rash Transthoracic echocardiogram showed • Changes in extremities: acute stage — erythema and edema of hands and feet, three aneurysms—one in each of his convalescent stage — membranous desquamation of fingertips coronary arteries. The aneurysm in the Exclusion of alternative diagnosis left circumflex artery measured 5.6 mm, a saccular aneurysm in the right coronary Source: Dajani AS, Taubert KA, Gerber MA, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993;87:1776–80. artery was 6.5 mm in size, and the giant 50 | MINNESOTA MEDICINE | OCTOBER 2014 Clinical AND Health Affairs aneurysm in the left anterior descending Learning points artery measured • Early recognition is key to pre- 8.2 mm.2 (A giant venting cardiovascular sequelae. aneurysm is defined • Treatment within the first 10 as >8mm and is a days of illness is critical to the prognostically poor prevention of coronary aneu- indicator.) Aggres- rysms, reducing the risk five- sive anticoagula- fold. tion was started to • If there is concern regarding prevent coronary patient compliance in cases thrombosis and where the index of suspicion for subsequent myocar- Kawasaki disease is high, the dial infarction. seriousness should be clearly communicated to the family Discussion and close follow up may be This case illustrates beneficial. the potential for severe consequences when there is late diagnosis of Kawasaki disease. Without IVIG treatment, about 25% of children with Kawasaki disease develop an aneurysm; with IVIG administration that number is reduced to 4%.3 Recognition of this constellation of findings as Kawasaki disease is crucial to the administration of appropriate IVIG ther- apy for prevention of aneurysms in coronary arteries. Addition- ally, this case highlights the value of culturally appropriate care. Perhaps if the gravity of the situation was communicated to the family in a way that was clearly understood, this poor outcome could have been avoided. MM Claire Jansson-Knodell is a medical student at the University of Minnesota and Rhamy Magid is a pediatrician at Children’s Hospitals and Clinics of Minnesota. REFERENCES 1. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association [Published correction appears in Pediatrics 2005;115:1118]. Pediatrics. 2004;114:1708–33. 2. Terai M, Shulman ST. Prevalence of coronary artery abnormalities in Kawasaki disease is highly dependent on gamma globulin dose but independent of salicylate dose. J Pediatr 1997; 131:888. 3. Dajani AS, Taubert KA, Gerber MA, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993;87:1776–80. OCTOBER 2014 | MINNESOTA MEDICINE | 51.
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