OBJECTIVE-The 2004 American Heart Association (AHA) statement included a clinical case definition and an algorithm for diagnosing and treating suspected incomplete Kawasaki disease (KD).We explored the performance of these recommendations in a multicenter series of US patients with KD with coronary artery aneurysms (CAAs).METHODS-We reviewed retrospectively records of patients with KD with CAAs at 4 US centers from 1981 to 2006. CAAs were defined on the basis of z scores of >3 or Japanese Ministry of Health and Welfare criteria. Our primary outcome was the proportion of patients presenting at illness day ≤21 who would have received intravenous immunoglobulin (IVIG) treatment by following the AHA guidelines at the time of their initial presentation to the clinical center. RESULTS-Of195patients who met entry criteria, 137 (70%) met the case definition and would have received IVIG treatment at presentation. Fifty-three patients (27%) had suspected incomplete KD and were eligible for algorithm application; all would have received IVIG treatment at presentation. Of the remaining 5 patients, 3 were excluded from the algorithm because of fever for <5 days at presentation and 2 because of <2 clinical criteria at >6 months of age. Two of these 5 patients would have entered the algorithm and received IVIG treatment after follow-up monitoring. Overall, application of the AHA algorithm would have referred ≥190 patients (97%) for IVIG treatment. CONCLUSIONS-Application of the 2004AHA recommendations, compared with the classic criteria alone, improves the rate of IVIG treatment for patients with KD who develop CAAs. Future multicenter prospective studies are needed to assess the performance characteristics of the AHA algorithm in febrile children with incomplete criterion findings and to refine the algorithm further.Address correspondence to Elizabeth S. Yellen, MD, Children's Hospital Boston, Department of Cardiology, 300 Longwood Ave, Boston, MA 02115. elizabeth.yellen@cardio.chboston.org. FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. NIH Public Access Author ManuscriptPediatrics. Author manuscript; available in PMC 2010 July 2. In 2004, the American Heart Association (AHA) published a statement on the diagnosis, treatment, and long-term management of KD, which subsequently was endorsed by the American Academy of Pediatrics. 5 The report included an algorithm to aid clinicians in the evaluation of patients with suspected KD who do not meet the complete case definition and thus may not be identified or receive timely referral for IVIG treatment. The AHA algorithm for patients with suspected incomplete KD was based on expert opinion and anecdotal reports, rather than large clinical trials or registries. In a retrospective, multicenter study, we sought to assess the performance of the AHA recommendations for IVIG treatment for children with CAAs attributable to KD. METHODS SubjectsWe performed a retrospective chart review of data for childre...

Objective To determine the real world effectiveness of statins and impact of baseline factors on low-density lipoprotein cholesterol (LDL-C) reduction among children and adolescents. Study design We analyzed data prospectively collected from a quality improvement initiative in the Boston Children’s Hospital Preventive Cardiology Program. We included patients ≤ 21 years of age initiated on statins between September 2010 and March 2014. The primary outcome was first achieving goal LDL-C, defined as <130 mg/dL, or <100 mg/dL with high-level risk factors (e.g. diabetes, etc.). Cox proportional hazards models assessed the impact of baseline clinical and lifestyle factors. Results Among the 1521 pediatric patients evaluated in 3813 clinical encounters over 3.5 years, 97 patients (6.3%) were started on statin therapy and had follow-up data (median age 14 [IQR 7] years), 54% were female, 24% obese, 62% with at least one lifestyle risk factor. The median baseline LDL-C was 215 (IQR 78) mg/dL and median follow-up after starting statin was 1.0 (IQR 1.3) year. The cumulative probability of achieving LDL-C goal within 1 year was 60% (95% CI 47, 69). Male sex (HR 0.5 [95% CI 0.3, 0.8]) and higher baseline LDL-C (HR 0.92 [95% CI 0.87, 0.98] per 10 mg/dL) were associated with not achieving LDL-C goals, but not age, BMI percentile, lifestyle factors or family history. Conclusions The majority of pediatric patients started on statins reached LDL-C treatment goals within 1 year. Males and those with higher baseline LDL-C were less likely to be successful and may require increased support.

In a real-world practice, pediatric patients using statins did not experience higher CK levels, nor was there a meaningful CK increase with statin initiation. These data suggest the limited utility to checking CK in the absence of symptoms, supporting current guidelines.