Trends and Resource Utilization in Kawasaki Disease Hospitalizations in the United States, 2008-2017.

OBJECTIVES: To explore trends in hospitalization rate, resource use, and outcomes of Kawasaki Disease (KD) in children in the United States from 2008 to 2017. METHODS: This was a retrospective, serial cross-sectional analysis of pediatric hospitalizations with International Classification of Disease diagnostic codes for KD in the National Inpatient Sample. Hospitalization rates per 100 000 populations were calculated and stratified by age group, gender, race, and US census region. Prevalence of coronary artery aneurysms (CAA) were expressed as proportions of KD hospitalizations. Resource use was defined in terms of length of stay and hospital cost. Cochran-Armitage and Jonckheere-Terpstra trend tests were used for categorical and continuous variables, respectively. P <.05 was considered significant. RESULTS: A total of 43 028 pediatric hospitalizations identified with KD, yielding an overall hospitalization rate of 5.5 per 100 000 children. The overall KD hospitalization rate remained stable over the study period (P = .18). Although KD hospitalization rates differed by age group, gender, race, and census region, a significant increase was observed among Native Americans (P = .048). Rates of CAA among KD hospitalization increased from 2.4% to 6.8% (P = .04). Length of stay remained stable at 2 to 3 days, but inflation-adjusted hospital cost increased from $6819 in 2008 to $10 061 in 2017 (Ptrend < 0.001). CONCLUSIONS: Hospitalization-associated costs and rates of CAA diagnostic codes among KD hospitalizations increased, despite a stable KD hospitalization rate between 2008 and 2017. These findings warrant further investigation and confirmation with databases with granular clinical information.

Assessing Health Care Utilization and Compliance in Kawasaki Disease.

OBJECTIVE: To characterize health care utilization and costs associated with care after diagnosis of Kawasaki disease including adherence to guidelines for echocardiograms. STUDY DESIGN: We analyzed children hospitalized for Kawasaki disease using 2015-2017 national Truven MarketScan commercial claims data. The mean 90-day prehospitalization utilization and costs were quantified and compared with the 90 days posthospitalization via Wilcoxon 2-sample test. Adherence to echocardiogram guidelines was examined using multivariable logistic regression to identify factors associated with adherence. RESULTS: The mean total payments 90 days prior to hospitalization ($2090; n = 360) were significantly lower than those after discharge ($3778), though out of pocket costs were higher ($400 vs $270) (P < .0001). There was an increase in office visits, medical procedures, and echocardiograms after discharge. A majority of health care utilization before hospitalization occurred in the 7 days immediately prior to the date of admission; 51% obtained an echocardiogram within the first 2 weeks, and 14% were completely adherent with recommendations. Children with greater utilization prior to admission were more likely to adhere to American Heart Association guidelines for follow-up echocardiograms (OR 1.03, 95% CI 1.01-1.06). CONCLUSIONS: Outpatient health care expenditure nearly doubles after Kawasaki disease hospital discharge when compared with prehospitalization, suggesting the financial ramifications of this diagnosis persist beyond costs incurred during hospitalization. A significant portion of patients do not receive guideline recommended follow-up echocardiograms. This issue should be explored in more detail given the morbidity and mortality associated with this diagnosis.

Kawasaki Disease and Clinical Outcome Disparities Among Black Children.

OBJECTIVE: To determine whether Black children with Kawasaki disease exhibit disparities in prevalence, sequelae, and response to intravenous gamma globulin (IVIG) treatment. STUDY DESIGN: International Classification of Diseases codes were used to identify children with Kawasaki disease admitted to a tertiary center in the southeastern US. Subjects diagnosed and treated according to American Heart Association criteria were included. Demographic, laboratory, clinical, and echocardiographic data from the electronic medical record (2000-2015) were compared between Blacks and Whites. RESULTS: Data from 369 subjects (52% Whites and 48% Blacks) were included in our analysis. No significant differences related to timely admission, IVIG treatment, or coronary artery (CA) abnormalities during hospitalization were observed. Blacks showed lower IVIG response rates than Whites for patients administered IVIG within 10 days of fever onset (86.6% vs 95.6%; P = .007). Blacks received more ancillary drugs (9.6% vs 2.6%; P = .003), and endured longer hospitalizations (mean, 5 ± 3.9 days vs 3.4 ± 2.2 days; P = .001). Blacks presented with higher C-reactive protein level and erythrocyte sedimentation rate and lower hemoglobin, albumin, and sodium levels. Blacks had a higher proportion of persistent CA abnormalities than Whites at second follow-up echocardiogram (14.5% vs 6.3%; P = .03), and at third follow-up echocardiogram (21.2% vs 6.9%; P = .01). CONCLUSIONS: Compared with White children, Black children with Kawasaki disease had higher IVIG refractory prevalence, more severe inflammation, more ancillary treatments, and longer hospitalizations. Despite no racial differences in time to diagnosis or initial treatment, there was greater CA abnormality persistence among Black children at follow-up.

An update on the epidemiology, length of stay, and cost of Kawasaki disease hospitalisation in the United States.

BACKGROUND: Kawasaki disease is an acute vasculitis of childhood and is the leading cause of acquired heart disease in the developed countries. METHODS: Data from hospital discharge records were obtained from the National Kids Inpatient Database for years 2009 and 2012. Hospitalisations by months, hospital regions, timing of admission, insurance types, and ethnicity were analysed. Length of stay and total charges were also analysed. RESULTS: There were 10,486 cases of Kawasaki disease from 12,678,005 children hospitalisation. Kawasaki disease was more common between 0 and 5 years old, in male, and in Asian. The January-March quarter had the highest rate compared to the lowest in the July-September quarter (OR=1.62, p < 0.001). Admissions on the weekend had longer length of stay [4.1 days (95 % CI: 3.97-4.31)] as compared to admissions on a weekday [3.72 days (95 % CI: 3.64-3.80), p < 0.001]. Blacks had the longest length of stay and whites had the shortest [4.33 days (95 % CI: 4.12-4.54 days) versus 3.60 days (95 % CI: 3.48-3.72 days), p < 0.001]. Coronary artery aneurysm was identified in 2.7 % of all patients with Kawasaki disease. Children with coronary artery aneurysm were hospitalised longer and had higher hospital charge. Age, admission during weekend, and the presence of coronary artery aneurysm had significant effect on the length of stay. CONCLUSIONS: This report provides the most updated epidemiological information on Kawasaki disease hospitalisation. Age, admissions during weekend, and the presence of coronary artery aneurysm are significant contributors to the length of stay.

Impact of Socioeconomic Status on Outcomes of Patients with Kawasaki Disease.

OBJECTIVE: To evaluate the association of neighborhood socioeconomic status (SES) with time to intravenous immunoglobulin treatment, length of stay (LOS), and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. STUDY DESIGN: We examined the relationship of SES in 915 patients treated at a large academic center between 2000 and 2017. Neighborhood SES was measured using a US census-based score derived from 6 measures related to income, education, and occupation. Linear and logistic regression were used to examine the association of SES with number of days of fever at time of treatment, LOS, and CAA. RESULTS: Patients in the lowest SES quartile were treated later than patients with greater SES (7 [IQR 5, 9] vs 6 [IQR 5, 8] days, P = .01). Patients in the lowest SES quartile were more likely to be treated after 10 days of illness, with an OR 1.9 (95% CI 1.3-2.8). In multivariable analysis, SES remained an independent predictor of the number of days of fever at time of treatment (P = .01). Patients in the lowest SES quartile had longer LOS than patients with greater SES (3 [IQR 2, 5] vs 3 [IQR 2, 4], P = .007). In subgroup analysis of white children, those in the lowest SES quartile vs quartiles 2-4 were more likely to develop large/giant CAA 17 (12%) vs 30 (6%), P = .03. CONCLUSIONS: Lower SES is associated with delayed treatment, prolonged LOS, and increased risk of large/giant CAA. Novel approaches to diagnosis and education are needed for children living in low-SES neighborhoods.

Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I - definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase.

The primary purpose of these practical guidelines related to Kawasaki disease (KD) is to contribute to prompt diagnosis and appropriate treatment on the basis of different specialists' contributions in the field. A set of 40 recommendations is provided, divided in two parts: the first describes the definition of KD, its epidemiology, etiopathogenetic hints, presentation, clinical course and general management, including treatment of the acute phase, through specific 23 recommendations.Their application is aimed at improving the rate of treatment with intravenous immunoglobulin and the overall potential development of coronary artery abnormalities in KD. Guidelines, however, should not be considered a norm that limits treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or complications.

Kawasaki Disease: Issues in Diagnosis and Treatment--A Developing Country Perspective.

Kawasaki disease (KD) is a common vasculitis in children and is the commonest cause of pediatric acquired heart disease in children in Japan and countries in North America and the European Union. It is now being increasingly reported from several developing countries, including China and India. Diagnosis of KD is based on a set of clinical criteria, none of which is individually pathognomonic for this condition. Further, these clinical features appear sequentially over a few days and all findings may not be present at a given point of time. Like many other vasculitides, there is no confirmatory laboratory test for KD. Treatment of KD involves use of intravenous immunoglobulin (IVIg) and aspirin. IVIg is an expensive product and poses several difficulties for patients in developing countries who may find it difficult to access therapy even if a diagnosis of KD has been made in time. In this review, the authors discuss some of these challenges that pediatricians have to face while managing KD in resource constrained settings.

[The algorithms of acute phase treatment for patients with Kawasaki disease].

Approximately one decade has passed when the original version of "Guidelines for Medi cal Treatment of Acute Kawasaki Disease" was published. Since then, many clinical stud ies have investigated and new evidences regarding diagnosis and acute phase treatment have been piled up. Based upon the background, Research Committee of the Japanese Society of Pediatric Cardiology and Cardiac Surgery revised the guideline in December 2012. The revised guideline proposed new therapeutic algorithm for acute Kawasaki disease patients. The present review overviews the revised guideline especially focused on the algorithm, and explains how to treat acute phase Kawasaki disease patients.

[Plasma exchange therapy].

By the Kawasaki disease, it is important that treatment is effective before the tenth day of illness when a coronary lesion can occur, and it is a well-known fact that early disappearance of inflammation leads directly to the onset restraint of the coronary lesions. Plasma exchange (PE) removes inflammatory cytokine and chemokines in the blood for Kawasaki disease directly and suppresses the inflammation early. The treatment result of the PE for the IVIG-resistant Kawasaki disease is so good, and if it could be started before particularly coronary lesion develops, we will expect an extremely big effect. In addition, when immunoglobulin therapy, steroid pulse therapy or neutrophilic elastase inhibitor therapy is invalid, we can perform PE therapy with the insurance applica- tion in Japan.

Epidemiology and management of Kawasaki disease.

Kawasaki disease (KD) is an acute systemic vasculitis affecting young children and is rising in incidence worldwide. It is most common in children <5 years of age, males and those of Asian ethnicity. It is an important cause of acquired heart disease in children. Standard treatment with high-dose aspirin (acetylsalicylic acid; ASA) and intravenous immune globulin (IVIG) has been shown to decrease the rate of coronary artery aneurysm development. Anti-coagulation has an important place in the management of KD, although guidance based on evidence is lacking. Treatment of refractory KD is an area under intense study and may include IVIG, corticosteroids and/or tumour necrosis factor (TNF)-α inhibitors among immunosuppressive agents. Acute complications of KD include myocarditis/KD shock syndrome and macrophage activation syndrome, which necessitate appropriate awareness in order to initiate proper management.

Assessment of intravenous immunoglobulin non-responders in Kawasaki disease.

The authors compared pre-treatment and post-treatment characteristics of 206 prospectively enrolled patients with Kawasaki disease (KD) responsive to intravenous immunoglobulin (IVIG) with those of 23 (10% of total) IVIG non-responders. Demographic characteristics were similar in both groups. Compared to IVIG responders, non-responders had a longer total duration of fever and a higher incidence of coronary artery lesions. Prior to IVIG, non-responders had higher neutrophil differential and C-reactive protein, and lower cholesterol. 24 hours after the IVIG infusion, a total leucocyte count >13.1×10(9)/l, neutrophil differential >51% and total protein <72 g/l showed reasonable sensitivity (91%, 91% and 64%, respectively) and specificity (89%, 76% and 78%, respectively) as independent characteristics of non-response to IVIG. Laboratory parameters before and shortly after IVIG may reflect the severity of inflammation in KD patients and assist in informing further management.

Kawasaki disease.

Kawasaki disease is currently the leading cause of acquired heart disease in children in the United States and Japan. The case of an 18-year-old insurance applicant is used to review the pathogenesis, complications, treatment and prognostic implications of this disorder.

Positron emission tomography for the assessment of myocardial viability in Kawasaki disease using different therapies.

13N-ammonia and 18F-fluorodeoxyglucose positron emission tomography (PET) of the heart were performed on 30 children with a history of Kawasaki disease. The results indicated PET abnormalities in 61.1% of patients during the acute and subacute stages and in 41.2% of patients in the convalescent stage of Kawasaki disease. Two-dimensional echocardiography and coronary angiography could not predict the myocardial viability and perfusion as well as PET. Different therapies during the acute stage of the disease did not effectively prevent myocardial damage, despite the absence of coronary arterial abnormalities. The patients who received 400 mg x kg(-1) x day(-1) of intravenous immunoglobulin (IVIG) for 5 days had a significantly lower incidence of PET abnormalities than those who received a single dose of 2000 mg x kg(-1) IVIG (P < 0.05).

Selective high dose gamma-globulin treatment in Kawasaki disease: assessment of clinical aspects and cost effectiveness.

BACKGROUND: High-dose intravenous gamma-globulin (IVGG) plus aspirin (ASA) treatment is effective in preventing coronary artery complications in acute Kawasaki disease (KD). However, gamma-globulin is very expensive, especially in Japan. Furthermore the indication for IVGG treatment and the optimal dose of gamma-globulin remain controversial. OBJECTIVES: To examine these two issues, we used Harada's scoring system to investigate whether a single 2 g/kg dose therapy has any advantage over the 5 day 400 mg/kg per day therapy. METHODS: We studied 203 patients with KD who had no coronary artery complications on admission. Of these, 145 patients scored 4 or more on Harada score within the first 9 days of illness and were treated with IVGG treatment. Using a random number table, 72 patients were selected to receive a single 2 g/kg dose (2 g group), while the remaining 73 patients were treated with 400 mg/kg per day for 5 consecutive days (400 mg group). Those who had a Harada score of three or less received no IVGG (non-IVGG group) treatment (58 patients). RESULTS: The incidence rate of coronary artery complications in the 2 g group was significantly lower than in the 400 mg group. The duration of high fever, positive duration of C-reactive protein and the number of hospital days in the 2 g group were each significantly shorter than in the 400 mg group. The total medical expense in the 2 g group was significantly lower than in the 400 mg group. There were no coronary artery complications in the non-IVGG group. CONCLUSIONS: It was found to be clinically more effective and more cost effective to select a patient by Harada's scoring system and, where a score of four or more was obtained, to administer a single 2 g/kg intravenous dose of gamma-globulin for acute KD.

Epidemiology, etiology, and management of Kawasaki disease: state of the art.

Since its first description in Japan 30 years ago, Kawasaki disease has been reported worldwide. Although an infectious etiology is suspected based on the epidemiology and clinical features, a causative agent has not been identified. The majority of the morbidity and mortality associated with this condition is attributable to the development of coronary artery aneurysms. Treatment during the acute phase with intravenous immunoglobulin and acetylsalicylic acid results in significant reductions in coronary complications. The long-term management of patients with persistent coronary abnormalities is less well-defined. This article reviews the epidemiology, possible etiologies, and management of Kawasaki disease.