Impact of Z score system on the management of coronary artery lesions in Kawasaki disease.

BACKGROUND: Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation and management was assessed in a large real-world dataset. METHODS: Using a combined dataset of patients with acute Kawasaki disease from the Children's Hospital at Montefiore and the National Heart, Lung, and Blood Institute Kawasaki Disease Study, coronary Z scores and the rate of coronary lesions (Z ≥ 2.0) and aneurysms (Z ≥ 2.5) were determined using four Z score systems. Agreement among Z scores and the effect on Kawasaki management were assessed. RESULTS: Of 333 patients analysed, 136 were from Montefiore and 197 from the Kawasaki Disease Study. Age, sex, body surface area, and rate of coronary lesions did not differ between the samples. Among the four Z score systems, the rate of acute coronary lesions varied from 24 to 55%. The mean left anterior descending Z scores from Pediatric Heart Network and Boston had a large uniform discrepancy of 1.3. Differences in Z scores among the four systems may change anticoagulation management in up to 22% of a Kawasaki population. CONCLUSIONS: Choice of Z score system alone may impact Kawasaki disease diagnosis and management. Further research is necessary to determine the ideal coronary Z score system.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract-based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non-White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08-1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline-based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Predictors of inadequate initial echocardiography in suspected Kawasaki disease: Criteria for sedation.

OBJECTIVE: Kawasaki disease is the primary cause of acquired pediatric heart disease in developed nations. Timely diagnosis of Kawasaki disease incorporates transthoracic echocardiography for visualization of the coronary arteries. Sedation improves this visualization, but not without risks and resource utilization. To identify potential sedation criteria for suspected Kawasaki disease, we analyzed factors associated with diagnostically inadequate initial transthoracic echocardiography performed without sedation. DESIGN: This retrospective review of patients < 18 years old undergoing initial transthoracic echocardiography for the inpatient evaluation of suspected Kawasaki disease from 2009 to 2015 occurred at a medium-sized urban children's hospital. The primary outcome was diagnostically inadequate transthoracic echocardiography without sedation due to poor visualization of the coronary arteries, determined by review of clinical records. The associations of the primary outcome with demographics, Kawasaki disease type, laboratory data, fever, and antipyretic or intravenous immunoglobulin treatment prior to transthoracic echocardiography were analyzed. RESULTS: In total, 112 patients (44% female, median age 2.1 years, median BSA 0.54 m2 ) underwent initial transthoracic echocardiography for suspected Kawasaki disease, and 99 were not sedated. Transthoracic echocardiography was diagnostically inadequate in 19 out of these 99 patients (19.2%) and was associated with age ≤ 2.0 years, weight ≤ 10.0 kg, and antipyretic use ≤ 6 hours before transthoracic echocardiography (all P < .05). These variables did not reach statistical significance on multivariable analysis. CONCLUSIONS: Patients ≤ 2.0 years or ≤ 10.0 kg or those recently receiving antipyretics, potentially a surrogate for irritability, were associated with diagnostically inadequate transthoracic echocardiography during the inpatient workup of Kawasaki disease. These factors should be considered when deciding which patients to sedate for initial Kawasaki disease transthoracic echocardiography.

Differences in resting-state functional magnetic resonance imaging functional network connectivity between schizophrenia and psychotic bipolar probands and their unaffected first-degree relatives.

BACKGROUND: Schizophrenia and bipolar disorder share overlapping symptoms and genetic etiology. Functional brain dysconnectivity is seen in both disorders. METHODS: We compared 70 schizophrenia and 64 psychotic bipolar probands, their respective unaffected first-degree relatives (n = 70, and n = 52), and 118 healthy subjects, all group age-, gender-, and ethnicity-matched. We used functional network connectivity analysis to measure differential connectivity among 16 functional magnetic resonance imaging resting state networks. First, we examined connectivity differences between probands and control subjects. Next, we probed these dysfunctional connections in relatives for potential endophenotypes. Network connectivity was then correlated with Positive and Negative Syndrome Scale (PANSS) scores to reveal clinical relationships. RESULTS: Three different network pairs were differentially connected in probands (false-discovery rate corrected q < .05) involving five individual resting-state networks: (A) fronto/occipital, (B) anterior default mode/prefrontal, (C) meso/paralimbic, (D) fronto-temporal/paralimbic, and (E) sensory-motor. One abnormal pair was unique to schizophrenia, (C-E), one unique to bipolar, (C-D), and one (A-B) was shared. Two of these three combinations (A-B, C-E) were also abnormal in bipolar relatives but none was normal in schizophrenia relatives (nonsignificant trend for C-E). The paralimbic circuit (C-D), which uniquely distinguished bipolar probands, contained multiple mood-relevant regions. Network relationship C-D correlated significantly with PANSS negative scores in bipolar probands, and A-B with PANSS positive and general scores in schizophrenia. CONCLUSIONS: Schizophrenia and psychotic bipolar probands share several abnormal resting state network connections, but there are also unique neural network underpinnings between disorders. We identified specific connections that might also be candidate psychosis endophenotypes.