Complete KD Research Articles

NLRC4 methylation and its response to intravenous immunoglobulin therapy in Kawasaki disease: a case control study

BackgroundKawasaki disease (KD) is a systemic vasculitis accompanied by many systemic physiological and biochemical changes. Elucidating its molecular mechanisms is crucial for diagnosing and developing effective treatments. NLR Family CARD Domain Containing 4 (NLRC4) encodes the key components of inflammasomes that function as pattern recognition receptors. The purpose of this study was to investigate the potential of NLRC4 methylation as a biomarker for KD.MethodsIn this study, pyrosequencing was utilized to analyze NLRC4 promoter methylation in blood samples from 44 children with initial complete KD and 51 matched healthy controls. Methylation at five CpG sites within the NLRC4 promoter region was evaluated.ResultsCompared to controls, NLRC4 methylation significantly decreased in KD patients (CpG1: p = 2.93E-06; CpG2: p = 2.35E-05; CpG3: p = 6.46E-06; CpG4: p = 2.47E-06; CpG5: p = 1.26E-05; average methylation: p = 5.42E-06). These changes were significantly reversed after intravenous immunoglobulin (IVIG) treatment. ROC curve analysis demonstrated remarkable diagnostic capability of mean NLRC4 gene methylation for KD (areas under ROC curve = 0.844, sensitivity = 0.75, p = 9.61E-06, 95% confidence intervals were 0.762–0.926 for mean NLRC4 methylation). In addition, NLRC4 promoter methylation was shown to be significantly negatively correlated with the levels of central granulocyte percentage, age, mean haemoglobin quantity and mean erythrocyte volume. Besides, NLRC4 promoter methylation was positively correlated with lymphocyte percentage, lymphocyte absolute value.ConclusionsOur work revealed the role of peripheral NLRC4 hypomethylation in KD pathogenesis and IVIG treatment response, could potentially serve as a treatment monitoring biomarker, although its precise functions remain to be elucidated.

The impact of inappropriate steroid exposure before the diagnosis of Kawasaki disease

BackgroundKawasaki disease (KD) is a systemic inflammatory disease characterized by vasculitis. In South Korea, some pediatric doctors empirically prescribe steroids to control febrile pediatric patients. This study aimed to evaluate the clinical characteristics of patients with KD after steroid exposure. MethodsThis was a single-center, retrospective, observational study. This study included patients (aged ≤15 years) between January 2020 and July 2022. We compared two groups, one group exposed to steroids and the other group who were not, using the Student's t-test or analysis of variance; otherwise, the Mann–Whitney U test or Kruskal–Wallis test was conducted. Statistical significance was set at p < 0.05. ResultsIn total, 190 patients with KD were enrolled; of these, 64 (33.7 %) had a history of steroid exposure, and 126 (66.3 %) had no history of steroid exposure. In the steroid exposure group, prolonged fever duration (6.72 ± 1.72 versus 5.61 ± 1.19, p-value = <0.001), a lower proportion of complete KD (29.69 % vs. 88.10 %, p-value = <0.001), and a significantly lower level of C-reactive protein were observed. However, no significant correlations were observed between the Transthoracic Echocardiography (TTE) results (coronary artery aneurysm, existence of pericardial effusion) and prognostic factors (days of hospitalization, the number of intravenous immunoglobulin administrations, and Kobayashi score) between the two groups. ConclusionsPatients with KD and previous steroid exposure may exhibit an incomplete KD phenotype with prolonged fever. Although previous steroid exposure does not affect the prognosis of KD, including coronary artery aneurysms, it may mask the classic features of KD, resulting in a delayed diagnosis.

Predictive value of monocyte to HDL-C ratio for coronary artery lesions and intravenous immunoglobulin resistance in Kawasaki disease.

As a practical, cost-effective inflammatory biomarker, MHR has a significantly predictive value in complete KD children complicated with CALs and IVIG-resistance. Paying more attention to the changes of MHR in KD children may contribute to better understanding of KD development and prognosis in clinical practice. • CALs are the most prevalent serious sequela of KD, and approximately 10%~20% of patients do not respond to IVIG therapy. • MHR could be a convenient biomarker to predict the development and progression of CVDs. It has been reported that the MHR is a new prognostic biomarker in several CVDs. • MHR has a significantly predictive value in KD children complicated with CALs and IVIG-resistance. • Compared with the molecular and immunological biomarkers that have been reported, MHR has the characteristics of practical, cost-effective, higher sensitivity and specificity, which can be used as a predictive indicator in complete KD patients.

Epidemiological data of national Kawasaki disease registry in Iran, 2007-2019.

Kawasaki disease(KD) is a vasculitis of childhood that tends to influence the coronary arteries. There is no national data about the prevalence of KD in Iran. This study aimed to perform a national registry in Iran for 13 years. In this retrospective study, the data for KD extracted from medical records of <19 year-old patients admitted to tertiary hospitals in Iran between 2007 and 2019 were recorded in the national KD registry system. Age, admission date, gender, location, and presence of KD criteria, laboratory and echocardiography findings, and treatment modalities were evaluated. Complete KD was considered if ≥4 clinical criteria of the KD existed and otherwise, incomplete KD was considered. Data from 1,682 KD patients including 999(59.39%) boys and 683(40.61%) girls and male/female ratio of 1.46 were evaluated. The mean age was 3.08 ± 2.49 years and 1465(87%) were living in urban regions. The yearly incidence of the disease was between 2.62 to 3.03 from 2015 to 2019. The highest age-specific incidence was observed in children <1-year-old. Incomplete and resistant KD included 1,321(78.54%) and 9(0.54%) patients, respectively. Abnormal echocardiography was detected in 619(36.80%) patients. Leukocytosis, with dominancy of neutrophils, anemia, thrombocytosis and increased ESR and CRP were the most noticeable laboratory findings. No death due to KD disease was reported. Based on this study, most of the KD cases are presented with atypical presentation in Iran. So, increasing awareness of primary healthcare workers by educating and updating their data is very important in timely diagnosis and management of the disease.

Minimally globally rigid graphs

A graph G=(V,E) is globally rigid in Rd if for any generic placement p:V→Rd of the vertices, the edge lengths p(u)−p(v),uv∈E uniquely determine p, up to congruence. In this paper we consider minimally globally rigid graphs, in which the deletion of an arbitrary edge destroys global rigidity. We prove that if G=(V,E) is minimally globally rigid in Rd on at least d+2 vertices, then |E|≤(d+1)|V|−d+22. This implies that the minimum degree of G is at most 2d+1. We also show that the only graph in which the upper bound on the number of edges is attained is the complete graph Kd+2. It follows that every minimally globally rigid graph in Rd on at least d+3 vertices is flexible in Rd+1.As a counterpart to our main result on the sparsity of minimally globally rigid graphs, we show that in two dimensions, dense graphs always contain nontrivial globally rigid subgraphs. More precisely, if some graph G=(V,E) satisfies |E|≥5|V|, then G contains a subgraph on at least seven vertices that is globally rigid in R2. If the well-known “sufficient connectivity conjecture” is true, then our methods also extend to higher dimensions.Finally, we discuss a conjectured strengthening of our main result, which states that if a pair of vertices {u,v} is linked in G in Rd+1, then {u,v} is globally linked in G in Rd. We prove this conjecture in the d=1,2 cases, along with a variety of related results.

Enumeration of Labeled and Unlabeled Hamiltonian Cycles in Complete k-Partite Graphs

Labeled and unlabeled Hamiltonian cycles in complete n-partite graphs Kd,d,...,d having exactly d vertices in each part are enumerated. Recurrence relations for the exact values $$ {b}_n^{(d)} $$ of such cycles for arbitrary n and d are obtained.

Importance of Serum Ferritin Level for Early Diagnosis and Differentiation in Patients with Kawasaki Disease with Macrophage Activation Syndrome.

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.

Comparison of IVIG resistance predictive models in Kawasaki disease.

We aimed to compare the ten different scores (by Kobayashi, Egami, Harada, Formosa, Sano, Piram et al., Wu et al., Yang et al., Tan et al., and Kanai et al.) to assess their performance in predicting IVIG resistance in Turkish children. Complete and incomplete KD patients diagnosed with KD at Hacettepe University between June 2007 and September 2019 were evaluated retrospectively. A total of 129 patients, 79 boys (61.2%), with a median age 36 (IQR 19.5-57.0) months were evaluated. Sixteen patients (12.4%) had IVIG resistance. Sensitivity was low for all the ten scores. Tan, Sano, and Egami predictive models had the highest specificity (97.3, 89.4, 86.7%, respectively). Almost all scoring systems distinguished the group of patients with low risk for IVIG resistance but could not differentiate IVIG-resistant patients. Multivariate analysis for the laboratory features showed that platelet count <300 × 109/L and GGT serum levels were independent risk factors for IVIG resistance (OR: 3.896; 95% CI: 1.054-14.404; p = 0.042 and OR: 1.008; 95% CI: 1.001-1.015; p = 0.050). The current scoring systems had a low sensitivity for predicting the risk for IVIG resistance in Turkish children. On the other hand, increased serum GGT levels and low platelet count were risk factors for predicting IVIG resistance. Intravenous immunoglobulin (IVIG) resistance may be observed in 10-20% of patients diagnosed with Kawasaki disease. Coronary artery involvement is more frequent in IVIG-resistant patients. It is important to predict the patients who might develop IVIG resistance to improve prognosis. The performance of the IVIG resistance predictive models in Kawasaki disease in our population is limited due to the low sensitivity.

High risk of coronary artery aneurysm in Kawasaki disease.

ObjectiveKawasaki disease (KD) is a vasculitis of unknown aetiology with a high risk of coronary aneurysms if untreated. Timely treatment with intravenous immunoglobulin decreases the risk for coronary artery aneurysms (CAA). In this study, we set out to elucidate the factors associated with the risk of developing CAA.MethodsRecords of all KD-diagnosed children in Skåne between 2004 and 2014 were collected and clinical and demographic data were compiled. KD is defined according to the revised American Heart Association diagnostic criteria and classified as either complete KD (cKD) or incomplete KD (iKD).ResultsKD was diagnosed in 77 children and CAA was found in 31% (n = 24). Children with CAA were younger compared with children without (median; 20 vs 34 months) and intravenous immunoglobulin treatment within 10 days was less likely to be received (75% vs 91%). In children presenting with iKD, 47% developed CAA compared with 21% in cKD patients. Using multivariate analysis, an association between the risk of CAA with low age in children with iKD was observed.ConclusionThe risk of CAA development is disturbingly high in young children with iKD. This highlights the importance of rapid intense treatment and vigilance in infants, who are the most difficult to diagnose, in order to reduce the frequency of CAA.

Treatment change and coronary artery abnormality in incomplete Kawasaki disease.

Incomplete Kawasaki disease (iKD) showed a higher incidence of coronary artery abnormalities (CAAs) than complete KD. However, the incidence of CAAs among iKD patients may have changed recently. We examined KD patients from recent nationwide surveys conducted between 2013 and 2016 and compared them with the results of a previous survey (2001-2002). Of 63270 KD patients, 13770 patients (22%) had iKD. They showed a higher incidence of convalescent-phase CAAs (cCAAs, 2.8%) than complete KD (2.1%). The incidence of cCAAs in patients with one or two symptoms (6.7%) was significantly higher than those with three or four symptoms (2.6%) (P < 0.0001). Intravenous immunoglobulin (IVIG) treatment was administered to 80% of iKD patients; 30% of them received IVIG before the fifth illness day (early treatment) and 12% of patients received IVIG after the seventh illness day (late treatment). In the previous survey, the incidence of cCAAs was higher in both iKD (5.9%) and cKD (4.4%). Intravenous immunoglobulin was administered to 62% of iKD patients; 26% of them received early treatment, and 16% received late treatment. The incidence of cCAAs remained higher among iKD patients than cKD patients but this difference was reduced by the increased proportion of iKD patients treated with IVIG and those at an earlier time point. It is important to recognize the possibility that patients may have iKD and perform echocardiography even if they present with a few principal symptoms.

Gastrointestinal hemorrhage before anticoagulant therapy in Kawasaki disease: a case report

BackgroundKawasaki disease (KD) is an acute febrile multisystem vasculitis and has been recognized to be the most common cause of acquired heart disease in children. Owing to its propensity to involve vessels throughout the entire body, KD often mimics other disease processes. The diagnosis might be delayed if other prominent symptoms appear before the characteristic clinical features of KD. Although gastrointestinal symptoms including vomiting, diarrhea, and abdominal pain are not uncommon in KD patients, KD with gastrointestinal bleeding is quite rare.Case presentationA previously healthy 4-year-old boy initially presented with abdominal pain, followed by fever, rash, and gastrointestinal hemorrhage, eventually diagnosed as complete KD. The patient recovered smoothly after appropriate management and no subsequent complications occurred in the following months.ConclusionThe diagnosis of KD should be considered in children presenting with abdominal symptoms and fever without definable cause. Pediatricians should be aware of the risk of gastrointestinal bleeding in patients with KD, especially in those with prominent abdominal symptoms.

Kawasaki disease: Clinico-laboratory spectrum and outcome in a cohort of children treated at a tertiary care hospital in Islamabad, Pakistan

Objective:To describe the demographics; clinical, laboratory, echocardiographic findings; treatment and outcome in a cohort of children with Kawasaki disease in a tertiary care hospital.Methods:This is a descriptive, observational, retrospective cohort study conducted at Shifa International Hospital, Islamabad, from January 2013-June 2019. Children who met the criteria for Kawasaki disease according to the American Heart Association and American Academy of Pediatrics guidelines were included.Results:A total of 25 children who met the criteria of Kawasaki disease were included. Their mean age was 43 months (4-150 months). Majority (76%) were males. Eighteen (72%) had complete Kawasaki disease and 7 (28%) had incomplete Kawasaki disease. Fever (> five days) was present in 20 (80%) patients. Eight patients (32%) had echocardiographic changes, out of which two patients (25%) had complete Kawasaki disease and six patients (75%) had incomplete Kawasaki disease. Intravenous immunoglobulin was given to all patients. Fifteen children (60%) received intravenous immunoglobulin within 10 days of fever. None required a second dose. All patients received high dose aspirin at diagnosis which was reduced to antiplatelet dose after resolution of fever for ≥48hrs. Eighteen patients (72%) came for regular follow up. Follow up at 6 months showed complete resolution of echocardiographic changes in six patients (75%), 1 (12.5%) was lost to follow up and one (12.5%) child had persistent coronary artery dilatation.Conclusion:Complete KD was present in 72% of our cohort of children. Coronary artery abnormalities were present in one third of these children, at younger age and more common in those with incomplete KD but recovered in most.

Kawasaki disease: a prospective population survey in the UK and Ireland from 2013 to 2015

ObjectiveKawasaki disease (KD) is an increasingly common vasculitis with risk of coronary artery aneurysms (CAAs). The last UK survey was in 1990, whereas current epidemiology, treatment patterns and complication rates...

Circulating endothelial glycocalyx components as a predictive marker of coronary artery lesions in Kawasaki disease

BackgroundKawasaki disease (KD) is acute and self-limited vasculitis caused by unknown origin, and the critical complication in KD patients is coronary artery lesions (CALs). The endothelial glycocalyx is a network of membranes luminally covering the endothelium. This study aimed to evaluate the clinical utility of serum glycocalyx components as biomarkers of predicting the onset CALs in KD. MethodsSeventy subjects with complete type KD, 18 subjects as febrile control (FC), and 15 subjects as afebrile controls (AC) were enrolled. Medical, demographic, echocardiography, and laboratory data from the medical records were retrospectively analyzed. Serum syndecan-1 and hyaluronan levels prior to intravenous immunoglobulin (IVIG) therapy were measured at the acute phase, immediately after IVIG, the subacute phase, and the time of discharge at the convalescent phase. ResultsSerum syndecan-1 and hyaluronan levels were higher in the KD group than in the AC and FC groups at all three phases. Further, these levels were compared between KD patients with and without the development of CALs. Serum syndecan-1 and hyaluronan levels at the acute phase were significantly elevated in KD patients with the CALs than in those without CALs. Serum hyaluronan, not syndecan-1, was determined as the most contributory parameter to predict CALs by a multiple logistic analysis. ConclusionsCirculating syndecan-1 and hyaluronan can be useful biomarkers to predict the development of CALs in KD.

Laboratory Markers in Incomplete Kawasaki Disease according to Coronary Artery Outcome.

Background and ObjectivesWe defined laboratory marker profiles typical of incomplete Kawasaki disease (iKD) during illness, especially with respect to the presence of a coronary artery abnormality such as coronary artery dilation or aneurysm.MethodsThis retrospective study examined the clinical and laboratory markers of patients with iKD over time, along with those of patients with complete KD (cKD) and febrile controls.ResultsOf 795 patients, 178 had iKD, 504 had cKD and 113 were febrile controls. During the transition from the acute to subacute phase, the age-adjusted hemoglobin levels and platelet counts were significantly lower and higher, respectively, in the subacute phase than in the acute phase in both iKD and cKD patients, which differed from those of febrile controls. Lower levels of acute and subacute age-adjusted hemoglobin levels in iKD patients (odds ratio [OR], 0.538 and 0.583; p=0.006 and 0.018, respectively) and higher subacute platelet counts in cKD patients (OR, 1.004; p=0.014) were correlated with the risk of coronary dilation. A higher acute neutrophil-to-lymphocyte ratio was associated with aneurysm only in cKD patients (OR, 1.059; p=0.044).ConclusionsThe iKD patients share KD-specific laboratory marker profiles in terms of complete blood cell counts and acute phase reactant levels with cKD patients. However, the factors predicting coronary dilation differ according to the phenotype; lower acute and subacute age-adjusted hemoglobin levels predict coronary dilation only in iKD patients.

Changes in clinical and laboratory features of Kawasaki disease noted over time in Daejeon, Korea

BackgroundKawasaki disease (KD) becomes one of the common diseases in Korea. Changes in clinical features and laboratory findings of KD were evaluated over a period of 10 years.MethodsWe reviewed the medical records of KD patients and compared the clinical and laboratory features of two KD patient groups: those admitted from 2000 to 2004 (group A, 284 cases) and those admitted from 2010 to 2014 (group B, 331 cases).ResultsThere were a total of 615 KD patients (mean age: 29.7 months; male-to-female ratio = 1.6:1), including 228 incomplete KD patients. Incomplete KD patients had milder values in some laboratory indices. The preadmission and total fever durations were longer in group A than in group B. The proportion of incomplete KD was higher in group B, but incidence of coronary artery lesions (CALs) was lower. For laboratory indices, the C-reactive protein and follow-up platelet values were lower, and the hemoglobin and albumin values were higher in group B. The same clinical and laboratory findings were confirmed in the KD subgroups; those with the same fever duration of 5 or 6 days and same ages, those with complete KD, and those with incomplete KD in the two different time periods.ConclusionsOur findings suggest that clinical features of KD tend to be milder over time and manifest in a higher incidence of incomplete KD, lower incidence of CALs, and less severe laboratory findings in recent KD patients in Korea compared with their historic counterparts.

Proof of Komlós's conjecture on Hamiltonian subsets

Komlós conjectured in 1981 that among all graphs with minimum degree at least d, the complete graph Kd+1 minimises the number of Hamiltonian subsets, where a subset of vertices is Hamiltonian if it contains a spanning cycle. We prove this conjecture when d is sufficiently large. In fact we prove a stronger result: for large d, any graph G with average degree at least d contains almost twice as many Hamiltonian subsets as Kd+1, unless G is isomorphic to Kd+1 or a certain other graph which we specify.

Abstract 161: Investigating the Added Value Of Repeated ECHO Imaging In Patients With Kawasaki Disease and Always-Normal Coronary Arteries

Background: Echocardiography (echo) is recommended in patients (pts) with KD at baseline, 2 wks, and 6 wks after fever onset to detect coronary artery (CA) abnormalities. However, echo imaging is expensive and in infants and toddlers often requires sedation, which is burdensome and carries some risk. Objective: To assess the benefit of additional echo imaging in uncomplicated KD pts with normal CAs at baseline and 2 wks. Methods: In this retrospective two-center study, eligibility criteria were: 1) admission and treatment at center for acute KD; 2) no significant congenital heart disease; 3) available echo measurements of both the right (R) CA and left anterior descending (LAD) at ≤ Day 10 (baseline), 2 (±1) wks and 6 (±3) wks; and 4) normal CAs = RCA and LAD z scores [zMax] ≤2.0 and no aneurysms. Results: Of 2,600 pts treated for KD from 1976 - 2014, 457 had normal CAs at baseline and 2 wks and met other inclusion criteria. Of these, 57% were male; median age was 3.3 yrs [IQR 1.8 to 5.4 yrs]; 77% had complete KD; and 14% received IVIG retreatment. At 6 wks, 450 (98.5%) had normal CAs. Of the remaining 7 pts, zMax was 2.0-2.4 in 5 (1.1%); their z scores subsequently normalized at 1 yr. The zMax was 2.5-2.9 in 1 (0.2%) pt who remained on low dose aspirin until 6 mos, when ECHO normalized. The zMax was ≥3.0 in 1 pt (0.2%, 95% CI [0.0%, 1.2%]) with a dilated LAD, which was normal 3 wks later. Sensitivity analysis using zMax of &lt;2.5 was similar (98.0% with nl CAs at 6 wks). Conclusion: New CA abnormalities are rarely detected at 6 wks in KD pts with normal CAs at baseline and 2 wks. The 6 wk echo may be unnecessary for uncomplicated KD pts with normal CAs at baseline and 2 wks. Figure - CA zMax shown as mean (+), median, 25th to 75th %tile.

Abstract 189: Natural History of Coronary Artery Aneurysms in Kawasaki Disease in US population and Risk Factors for Persistent Aneurysms

Background: The late natural history of coronary artery aneurysms (CAA) after IVIG treatment in the US is not well described. Methods: We evaluated all KD patients (pts) at 2 centers from 1984-2014. Entry criteria were: 1) IVIG treatment; 2) CAA, defined as LAD or RCA z-score ≥ 3 or Japanese Ministry of Health criteria; and 3) ≥1 follow-up (f/u) echo. Kaplan Meier curves evaluated time to CAA regression (z &lt; 2.5) and Cox regression examined factors associated with persistent CAA and major adverse cardiac events (MACE= death, MI, CABG, PCI, occluded CA). Results: Of 2592 KD pts, 408 (15%) met entry criteria and were 72% male; 54% white, 21% Asian, 7% black, 18% other race. Median age at fever onset was 1.8 y [IQR: 0.7-4.4y], 74% had complete KD, and fever days before 1 st IVIG were 7d [IQR 6-10d]. IVIG retreatment occurred in 35% and adjunctive anti-inflammatory therapy in 37%, both increased over time (p&lt;.001). LAD and RCA CAA occurred in 31%, LAD alone in 47% and RCA alone in 22%. Median z-scores at CAA diagnosis were: LAD 3.61 (IQR 3.1-5.1) and RCA 3.1 (1.7-4.1) with 93 (23%) pts having giant CAA (z ≥10). Over median f/u of 2.6 y (0.01-29.0y), 313 (77%) had CAA regression at median of 1.1 mo (IQR =0.3-16.9mo). Univariate risk factors for CAA persistence were z ≥ 8 at diagnosis (HR 0.22, p &lt;.001), earlier era (2010-2014 HR =1.0 , 2000-2009 HR = 0.60, 1990-1999 HR =0.38, 1984-1989=0.15, all p&lt;.001), multi-vessel CAA (LCA alone HR =1, RCA alone HR =0.99 p=.99, LCA+RCA HR=0.35, p&lt;.001) IVIG retreatment (HR 0.68, p=.003), IVIG treatment &gt;Day 10 (HR 0.46, p&lt;0.001), adjunctive anti-inflammatory therapy (HR 0.65, p=.03), and non-Asian race (HR 0.64, p=.001). Multivariable model for persistent CAA included earlier era (p&lt;.001), z-score &gt;8 at diagnosis (p&lt;.001), multi-vessel CAA (&lt;.001) and treatment after Day 10 (p=.01). MACE occurred in 25 (1%) pts, including 3 deaths, with univariate risk factors of CAA z-score ≥ 8 (p&lt;.001), earlier era (p&lt;.001), treatment after 10 d (p=.03), and non-Asian race (p=.03) Conclusion: Most CAA regress within the first year after treatment. Persistent CAA are more likely in pts with larger CAA, multi-vessel CAA and late IVIG treatment. In pts with large CAA, time to regression has gotten shorter in more recent eras, possibly related to greater use of adjunctive therapies.

Abstract 214: Outcomes of KD by the KD type: Linking Inpatient and Outpatient Follow-up Data

Background: The relationship between KD type and outcomes is not well described. We sought to describe the incidence of KD outcomes based on type of KD presentation. Methods: Using an electronic medical record, we prospectively recorded clinical data for each KD encounter (initial treatment, 2 and 6 week follow up visits) for all pts treated with IVIG at our hospital and followed in our KD clinic from 11/2012-9/2014. Pts were grouped by KD type as determined on the day of treatment - Complete, Incomplete, or questionable KD (qKD), defined as incomplete KD and &lt; 3 supplemental lab criteria. Late treatment was defined as IVIG given at &gt; 10 days of fever. The correlation of KD type with outcomes of persistent fever requiring repeat IVIG, development of periungual peeling (PP), and coronary abnormalities (CAA) was assessed using chi-square. Results: We studied 109 pts treated with IVIG; 95 were treated within 10 days of fever onset (14 late). Late treatment was most common in qKD (9/19=47%) compared to Incomplete (4/22=18%) and Complete (1/68=1.5%), p&lt;0.01. PP was least common in qKD (4/19=21%), compared to Incomplete (12/22=54%) and Complete (49/68=72%), p&lt;0.05. No pts with qKD developed CAA, compared to 5/67 (7%) of Complete and 3/18 (16%) of Incomplete pts treated within 10 days of fever, (p=0.16, NS). Late treatment more commonly had CAA (5/14=36%, v. 8/95=8%), relative risk = 4.2 (p=0.003). In pts with CAA, 10 /13 (77%) had CAA on the first echocardiogram. The remaining 3 pts (2 Complete, 1 Incomplete) had a pericardial effusion initially and developed CAA by the second echocardiogram. Need for repeat IVIG was not different between groups (Complete 16/68 = 24%, Incomplete 3/22=14%, qKD 2/19=11%, p=0.33). Of the 2 qKD pts who required a second dose of IVIG, one was subsequently diagnosed with juvenile idiopathic arthritis and the other with Bartonella henselae; neither developed PP or CAA. Conclusions: The incidence of CAA was not statistically different between KD types, though no qKD pts developed CAA despite a higher incidence of late treatment with IVIG. In most pts who developed CAA, the initial echocardiogram showed either CAA or a pericardial effusion. PP was least common in qKD. In qKD, failure of initial IVIG should prompt re-evaluation of the diagnosis of KD.