Patients with fever for <5 days and <4 principal features are labeled as "incomplete-atypical" cases,
Figure 5. Peeling of the fingertips involving the entire finger and palm.
a diagnosis that often is based on echocardiograph-ic findings of CAA (Rowley et al., 1987). The term "incomplete" may be preferable to "atypical" because these patients lack sufficient clinical signs of the disease to fulfill the classic criteria; they do not demonstrate atypical clinical features. The phrase "atypical Kawasaki disease" should be reserved for patients who have a problem, such as renal impairment, that generally is not seen in Kawasaki disease. Incomplete KD is more common in young infants than in older children, making accurate diagnosis and timely treatment especially important in these young patients who are at substantial risk of developing coronary abnormalities. Children with high persistent fever and incomplete onset represent a challenge for the pediatrician. In fact, associated to the classical clinical picture we can observe adjunctive clinical features that sometimes can lead to inappropriate diagnoses (Sundel et al., 1992). Fig. 6 summarizes the presence of these associated clinical features observed in 171 patients followed at the Pediatric Department, University of Padua. Gastrointestinal symptoms such as nausea, vomiting, and abdominal pain due to acute distension of the gallbladder (hydrops), identified by abdominal US, occur in ~50% of patients during the first 2 weeks of the illness (Sunddleson et al., 1987). Hepatic enlargement and jaundice can occur.
One third of patients can start with respiratory symptoms while around 40% can show early cardiac involvement such as pericarditis, myocarditis, and EKG abnormalities. Osteoarticular involvement such as arthritis or arthralgia can occur in almost one third of the patients. Signs of lower urinary tract inflammation such as sterile pyuria or uretritis may be observed in some patients while acute surgical abdomen can be a rare but threatening presentation of KD (Zulian et al., 2003).
Transient unilateral peripheral facial nerve palsy and high-frequency sensorineural hearing loss can occur during acute Kawasaki disease (Knott et al., 2001).
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