Classic Kawasaki Disease Research Articles

Current Approach to the Evaluation and Management of Incomplete Kawasaki Disease in the Emergency Department.

Kawasaki disease (KD) is one of the most common vasculitides of childhood and frequently presents to the emergency department. Although the diagnosis of KD is based on clinical criteria, children who do not fulfill the criteria but have sufficient supportive features of KD are diagnosed as having incomplete KD and warrant the same course of therapy as children with classic KD. The diagnosis of incomplete KD is challenging and requires a high index of suspicion. The purpose of this article is to review presenting features of incomplete KD and the diagnostic approach and management of children in the emergency department.

Echocardiographic Findings in Pediatric Multisystem Inflammatory Syndrome Associated With COVID-19 in the United States

BackgroundCenters from Europe and United States have reported an exceedingly high number of children with a severe inflammatory syndrome in the setting of coronavirus disease 2019, which has been termed multisystem inflammatory syndrome in children (MIS-C). ObjectivesThis study aimed to analyze echocardiographic manifestations in MIS-C. MethodsA total of 28 MIS-C, 20 healthy control subjects and 20 classic Kawasaki disease (KD) patients were retrospectively reviewed. The study reviewed echocardiographic parameters in the acute phase of the MIS-C and KD groups, and during the subacute period in the MIS-C group (interval 5.2 ± 3 days). ResultsOnly 1 case in the MIS-C group (4%) manifested coronary artery dilatation (z score = 3.15) in the acute phase, showing resolution during early follow-up. Left ventricular (LV) systolic and diastolic function measured by deformation parameters were worse in patients with MIS-C compared with KD. Moreover, MIS-C patients with myocardial injury were more affected than those without myocardial injury with respect to all functional parameters. The strongest parameters to predict myocardial injury in MIS-C were global longitudinal strain, global circumferential strain, peak left atrial strain, and peak longitudinal strain of right ventricular free wall (odds ratios: 1.45 [95% confidence interval (CI): 1.08 to 1.95], 1.39 [95% CI: 1.04 to 1.88], 0.84 [95% CI: 0.73 to 0.96], and 1.59 [95% CI: 1.09 to 2.34], respectively). The preserved LV ejection fraction (EF) group in MIS-C showed diastolic dysfunction. During the subacute period, LVEF returned to normal (median from 54% to 64%; p < 0.001) but diastolic dysfunction persisted. ConclusionsUnlike classic KD, coronary arteries may be spared in early MIS-C; however, myocardial injury is common. Even preserved EF patients showed subtle changes in myocardial deformation, suggesting subclinical myocardial injury. During an abbreviated follow-up, there was good recovery of systolic function but persistence of diastolic dysfunction and no coronary aneurysms.

Are Kawasaki Disease and Pediatric Multi-Inflammatory Syndrome Two Distinct Entities? Results from a Multicenter Survey During SARS-CoV-2 Epidemic in Italy

Background: There is mounting evidence on the existence of a childhood multi-inflammatory syndrome related to SARS-CoV-2, sharing similarities with Kawasaki Disease (KD). Methods: On April 24th,2020 the Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD or KD-like disease. Classification was: 1) classical and incomplete KD, named Kawasaki Disease Group (KDG); 2) KD-like multi-inflammatory syndrome, named KawaCOVID Group (KCG). Demographic, clinical, and laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAPO). Chi square test or exact Fisher test and non parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Findings: One-hundred-forty-nine patients were recruited (96 KDG and 53 KCG). KCG children significantly differed for older age at onset and more frequent occurrence of mucocutaneous manifestations, irritability, gastrointestinal and respiratory symptoms, oxygen supplementation and ventilation, ICU recovery, and secondary Hemophagocytic Lymphohistiocytosis. Myocarditis was more common in KCG, while coronary artery abnormalities were more common in the KDG. 23/149 patients showed heart abnormalities at follow-up. KDG characteristics were compared to a historic cohort of 505 KD: no significant difference was found. Interpretation: KCG and KDG patients significantly differed in clinical manifestations and heart outcome. KDG children did not differ from a historic cohort of KD. Our data would suggest that SARS-CoV-2 infection may be linked to two inflammatory conditions in children: the classic KD, possibly triggered by SARS-CoV-2, and the multisystem inflammatory syndrome, that has already been referred as PIMS-TS or MIS-C. More data, on larger series with prospective analysis are warranted to define the best treatment and long-term outcome. Funding: The research was conducted with no funding. Declaration of Interests: AR has received grant support and/or speaking or consultant fees from AbbVie, Angelini, Bristol-Myers Squibb, Novartis, Pfizer, Reckitt-Benkiser, Roche and Johnson & Johnson; MC, SDP, FZ, CB, LV, AM, RS, RC, GZ, MF, DM, AM, AC, RMD, MCM, FLT, AM, GS, AV, RC and AT do not have any conflict of interest to declare. Ethics Approval Statement: Institutional Review Board approval was achieved (IRCCS Burlo Garofolo – 03/2020).

Síndrome Kawasaki temporalmente relacionado a COVID-19 (Síndrome Kawasaki- Like) en pacientes pediátricos

Introducción: El Síndrome Multisistémico Inflamatorio surge como manifestación de COVID-19 en la edad pediátrica, con diversos fenotipos dentro de los cuales se encuentra un Síndrome similar a Kawasaki, que responde a terapéutica habitual para la enfermedad clásica. Reporte de casos: Se reportan dos casos procedentes de la ciudad de Chiclayo, con edades de 7 y 9 años, sexo femenino, historia de contactos probables y confirmados para COVID-19, y una de las pacientes con serología positiva IgG e imágenes tomográficas compatibles con COVID-19 en resolución. Ambas cumplieron con criterios de Síndrome Multisistémico Inflamatorio Pediátrico y Enfermedad de Kawasaki clásica, además con respuesta favorable a terapéutica habitual, sin complicaciones y en seguimiento actual. Conclusiones: los casos reportados responden a terapéutica habitual y las características de presentación contribuirán a la documentación de dicha patología emergente en la comunidad científica.

Multi-System Inflammatory Syndrome in a Child Mimicking Kawasaki Disease.

There have been recent reports of children presenting with severe multi-system hyperinflammatory syndrome resembling Kawasaki disease (KD) during current COVID-19 pandemic. Exact pathophysiology is unknown, however, most of the children have multi-organ dysfunction and respiratory system involvement is less common compared to adults. These patients have certain characteristic laboratory parameters different from those seen in children with KD. However, only limited literature is available at present for identification and management of such patients. We report a young girl who presented with fever, rash and other manifestations mimicking classic KD and fulfilling the case definitions for pediatric multi-system inflammatory syndrome. She had lymphopenia, thrombocytopenia and hyponatremia in the absence of macrophage activation syndrome, similar to that seen in patients reported from UK and Italy. Clinical manifestations resolved and laboratory parameters improved with intravenous immunoglobulin and corticosteroids. Early recognition is important to administer immunomodulatory therapy which may be life saving for these patients.

Multisystem Inflammatory Syndrome in Children and Kawasaki Disease: Two Different Illnesses with Overlapping Clinical Features

Multisystem Inflammatory Syndrome in Children and Kawasaki Disease: Two Different Illnesses with Overlapping Clinical Features

Novel paediatric presentation of COVID-19 with ARDS and cytokine storm syndrome without respiratory symptoms.

Novel paediatric presentation of COVID-19 with ARDS and cytokine storm syndrome without respiratory symptoms.

Kawasaki-like disease: emerging complication during the COVID-19 pandemic

Kawasaki-like disease: emerging complication during the COVID-19 pandemic

Predicting IVIg Nonresponse in Children With Kawasaki Disease

Research Article| January 01 2020 Predicting IVIg Nonresponse in Children With Kawasaki Disease AAP Grand Rounds (2020) 43 (1): 8. https://doi.org/10.1542/gr.43-1-8 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn MailTo Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Predicting IVIg Nonresponse in Children With Kawasaki Disease. AAP Grand Rounds January 2020; 43 (1): 8. https://doi.org/10.1542/gr.43-1-8 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: immunoglobulins, intravenous, kawasaki's disease Source: Hester GZ, Watson D, Nickel AJ, et al. Identifying patients with Kawasaki disease safe for early discharge: development of a risk prediction model at a US children’s hospital. Hosp Pediatr. 2019; 9(10): 749– 756; doi: https://doi.org/10.1542/hpeds.2019-0049Google Scholar Investigators from multiple institutions conducted a retrospective study to develop a model for predicting which children with Kawasaki disease (KD) will be IV immunoglobulin (IVIg) nonresponders in hopes of identifying low-risk patients who could be discharged from the hospital earlier after receiving an initial dose of IVIg. Study participants were children 0–18 years old with a primary diagnosis of KD and hospitalized at a single, large, urban US children’s hospital. The medical records of study participants were reviewed, and clinical and laboratory data abstracted. The primary study outcome was IVIg nonresponse defined by one or more of the following criteria: (a) documented fever (temperature ≥38.0°C) 36 hours to 7 days after completion of IVIg dose, (b) receipt of a second dose of IVIg ≥20 hours after first dose, or (c) readmission within 7 days for the second dose of IVIg. Candidate clinical and laboratory parameters for inclusion in the prediction model were chosen, and backward stepwise regression was used to develop the final model, with only variables statistically associated with IVIg nonresponse (defined as P<.157) included. The concordance index (area under the receiver operator curve) was used to assess the accuracy of the final model after adjusting for over-optimism. Based on the model, the authors used a cutoff value for estimate of nonresponse of 10% and computed the sensitivity, specificity, and positive and negative predictive value (PPV and NPV) of the model for predicting IVIg nonresponse. Data on 430 children with KD who received an initial dose of IVIg were analyzed. A total of 81 (19%) met criteria for IVIg nonresponse. Variables included in the prediction model for IVIg nonresponse included white blood cell count, platelet count, hemoglobin, aspartate aminotransaminase level, serum sodium, serum albumin, maximum temperature 0–6 hours after IVIg, and presentation with classic KD (ie, fever for ≥5 days with at least 4 of 5 clinical features) versus incomplete KD. The adjusted concordance index of the model was 0.70. Using a cutoff value for predicted nonresponse of 10%, the model had a sensitivity of 90.1%, specificity of 30.1%, PPV of 23.0%, and NPV of 92.9%, with 26.3% of study participants classified as low risk. The authors conclude that a model combining clinical and laboratory parameters can be used to predict risk of IVIg nonresponse, and that this model can be used in identifying children who might be discharged sooner after an initial dose of IVIg. Dr Brady has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device. KD is the most common cause of acquired heart disease in children in developed countries.1 The incidence of KD... You do not currently have access to this content.

Clinical characteristics and risk factors of coronary artery abnormities in 320 children with Kawasaki disease

Objective To compare the clinical features of classic Kawasaki disease (CKD) and incomplete Kawasaki disease (IKD), and to explore the risk factors of coronary artery abnormities in Kawasaki disease. Methods We collected the clinical data of 320 children with Kawasaki disease from July 1, 2017 to June 30, 2018, including 244 children with CKD (CKD group) and 76 children with IKD (IKD group). The risk factors of coronary artery abnormities in children with Kawasaki disease were explored. Results In addition to coronary artery injury(5%), some patients showed other rare complications such as cholestasis (1.6%), intestinal obstruction(0.6%), atlantoaxial subluxation (0.9%), macrophage activation syndrome (0.9%) and Kawasaki disease shock syndrome (0.3%). The age onset in CKD group was earlier than that in IKD group.WBC, CPR, NT-proBNP in CKD group were significantly higher than those in IKD group (P<0.05). After one week of treatment, WBC and CRP decreased significantly in group IKD, and there was no significant difference in PLT count.Multivariate logistic model was used to analyze the risk factors of coronary artery abnormities in Kawasaki disease.It was found that 25-OH VitD reduction (OR=2.851, P<0.05) was a high risk factor for coronary artery injury in KD. Conclusion Kawaski disease should be diagnosed early, treated for organ damage and supplemented with vitamin D. After discharge, coronary artery changes should be monitored regularly. Key words: Kawasaki disease; Coronary artery abnormities; Risk factors,

Abstract 17: Environmental Epidemiology of Kawasaki Disease

Background: The etiology of Kawasaki disease (KD) is commonly described as an exaggerated immune response to an environmental or infectious trigger in developmentally, immunologically and genetically susceptible children. The trigger for KD has not yet been identified. Methods: Patients with newly diagnosed KD (n=73) were enrolled within 6 weeks of KD diagnosis. Control subjects (n=65) were enrolled during pediatric annual visits and from friends of enrolled KD patients. All participants completed an extensive questionnaire about medical history, family and environment. Geographic localisation software characterized the participants’ neighborhoods. A hypothetical scenario was used for control patients to simulate similar recall bias to that of the KD patients. Results: There were no differences in age, gender, allergies, child and family medical history or recent vaccination. Children with KD were more likely to be unwell prior to KD symptom onset than controls, including fever, stomach pain, lethargy, jaundice, loss of appetite and irritability, but not infectious disease symptoms (16% vs. 21%, p=0.49). Infectious symptomatology developed concurrently to classic KD symptoms. Children with KD had less exposure to environmental allergens and were more likely to be exposed to chemical irritants. Conclusions: The presence of symptoms for up to 1 month before onset of KD may suggest that a preceding illness/environmental agents may alter barriers to entry/exposure or prime an immunologic reaction to an etiologic trigger. Children with fewer allergen/infectious exposures and chronic chemical exposure may be immunologically more susceptible.

Clinical and epidemiological characteristics of kawasaki disease.

Background:Kawasaki disease (KD) is an acute multisystem vascular syndrome of unknown etiology that is the leading cause of acquired heart disease in children of developed counties.Objectives:We aimed to evaluate the epidemiological characteristics and clinical manifestations of KD in children residing in the southwest of Iran.Patients and Methods:In this retrospective study, we reviewed the medical records of all children with KD who had been admitted to the main children’s hospital of Ahvaz, southwest Iran, from March 2000 to March 2010. Data regarding clinical and epidemiological characteristics, management, and the outcome of disease for each patient were obtained. The patients were divided into cardiac and non-cardiac groups based on echocardiographic results.Results:In total, 104 patients with KD (66 boys and 38 girls) were enrolled in this study. The male to female ratio was 1.7:1. The mean ± SD age of the patients was 33.6 ± 24.2 months. Most (87.2%) cases were from urban areas. The disease occurred more frequently during winter and spring. Furthermore, 61.5% of the children had the criteria of classic KD, and 38.5% were labeled as incomplete KD. The mean ± SD of the duration of hospital stay was 6.9 ± 2.4 days. The mean time between illness and admission to the hospital was 6.47 ± 2.6 days. The most common sign was fever, followed by conjunctivitis and oral changes. In total, 20% of the patients had cardiac abnormalities. There was no significant statistical difference between the cardiac and non-cardiac groups according to age, sex, clinical manifestations, laboratory findings, and cessation of fever. The duration of hospital stay and the time between onset of illness and diagnosis were longer in the cardiac group. All patients received intravenous immunoglobulin and aspirin. Only one patient continued to have cardiac abnormalities after 6 months of follow-up.Conclusions:Kawasaki disease is not rare in southwest of Iran. The age, gender distribution and clinical findings are similar to that of other reports. Patients with cardiac abnormalities had delayed treatment and prolonged hospital stays.

A case of orange-brown chromonychia in a patient with kawasaki disease.

Dear Editor: Kawasaki disease (KD) is an acute inflammatory syndrome of middle-sized arteries such as the coronary artery, and its pathogenesis and etiology remain unclear1. The diagnosis of classic KD is based on the presence of fever lasting longer than 5 days and four of five specific clinical criteria, including bilateral nonpurulent conjunctival injection, oral mucosal changes, redness and swelling of the hands and feet, skin rash, and cervical lymphadenopathy1. Among the cutaneous features of KD, typical nail changes have previously been described, such as transverse leukonychia, onychomadesis, and pincer nails2,3. In addition, Pal and Giri4 reported that patients with KD may show transverse orange-brown chromonychia and suggested to incorporate it as add-on clinical finding of KD. A 5-year-old Korean girl presented with transverse orange-brown discoloration of the finger and toe nails with periungual desquamation. There was no evidence of any other disease or use of drugs that could lead to chromonychia. Ten days previously, she was found to have KD, and was then treated with aspirin at a local hospital. On her visit, she showed orange-brown discoloration at the distal portion of the nail (Fig. 1). Conjunctival hyperemia, cracking of the lips, and strawberry tongue were also observed (Fig. 2). On the basis of the clinical findings, we diagnosed her nail discoloration as orange-brown chromonychia that occurred with KD on the basis of the clinical findings. Two weeks later, the almost transverse orange-brown discoloration disappeared with other symptoms. Fig. 1 (A) Orange-brown discoloration at the distal portion of the nail. (B) Closer view. Fig. 2 (A) Conjunctival hyperemia. (B) Cracking of the lips. (C) Strawberry tongue. A variety of nail abnormalities have been reported in KD. Ciastko2 reported onychomadesis in a boy with KD, that started 6 weeks after a fever. Pincer nail deformity and Beau's lines in KD were also reported2,3. These nail abnormalities commonly develop in the chronic phase and spontaneously resolve. Otherwise, Lindsley5 was the first to describe red transverse nail-bed lines in four KD patients during the acute phase. He considered that chromonychia may be related to localized vasculitis or nail-bed hyperemia, because the lines occurred during active inflammation. Thereafter, Pal amd Giri4 studied about the typical transverse orange-brown discoloration of the nails observed in KD patients, and reported that it was present in almost 75% of the patients (29 of 40). Also, they recognized that this orange-brown chromonychia started appearing between the fifth and eight day of the onset of fever and slowly faded away with time. Therefore, they suggested to include orange-brown chromonychia as an additional clinical feature in the diagnosis of KD, differently to other nail changes. Chromonychia is described after the use of antineoplastic drugs, including adriamycin, cyclophosphamide, and vincristine. In addition, thermal injury, contact exposure to elemental iron, angiotensin-receptor blockage therapy, use of a nail hardener, systemic lupus erythematosus, and hyperbilirubinemia may cause these changes. In summary, we report a case of orange-brown chromonychia in a KD patient, and suggest that it should be considered as a reference of in the diagnosis of KD, based on the basis of its appearance in the acute phase.

Clinical spectrum of incomplete Kawasaki disease in Thailand

Background: Inadequate diagnostic criteria in incomplete Kawasaki disease (KD) patients may lead to misdiagnosis and delayed treatment. However, the risk of coronary artery aneurysm in these patients remains uncertain.Aim: To investigate differences in clinical, laboratory and echocardiographic variables between patients with incomplete KD and classic KD.Method: The medical records of 208 KD patients treated between January 2001 and December 2009 in the Department of Pediatrics, Chiang Mai University Hospital were reviewed retrospectively. Patients with three or fewer major criteria were defined as having incomplete KD.Results: Of the 208 KD patients, 61 (29%) had incomplete KD. In those with incomplete KD, a significantly higher proportion were male (73·8% vs 59·2%, P = 0·03), the diagnosis was made later [mean (SD) day 9·0 (4·2) vs 7·2 (2·5), P = 0·003], there was a higher rate of delayed diagnosis (>10 days, 21% vs 10%, P = 0·02) and the presence of five major criteria was less common. The proportion of associated symptoms (irritability, upper respiratory tract symptoms, diarrhoea, vomiting and reactivation of BCG) and laboratory findings (pyuria, haemoglobin level, white blood count, polymorphonuclear cells, platelet count, erythrocyte sedimentation rate and serum albumin) were comparable in patients with incomplete KD and classic KD. The incomplete KD group tended to have a higher proportion of coronary artery abnormalities but the difference was not statistically significant (38% vs 25%, P = 0·09). However, a significantly greater proportion of the group with incomplete KD had large aneurysms (10% vs 1%, P = 0·009).Conclusions: Incomplete KD and classic KD have the same disease spectrum. Owing to the absence of some major criteria, incomplete KD can be more difficult to diagnose, which can result in delayed diagnosis and a greater risk of large coronary aneurysms.

Comparison of classic Kawasaki disease v/s incomplete Kawasaki disease in children of Dr. Sotero del Rio Hospital, Santiago, Chile

Comparison of classic Kawasaki disease v/s incomplete Kawasaki disease in children of Dr. Sotero del Rio Hospital, Santiago, Chile

Increased Incidence of Incomplete Kawasaki Disease at a Pediatric Hospital After Publication of the 2004 American Heart Association Guidelines

We sought to study the impact of the 2004 American Heart Association guidelines on diagnosis and treatment of patients with Kawasaki disease (KD). We reviewed patient records from July 2000 to June 2002 (group 1) and July 2007 to June 2009 (group 2) at a tertiary children's hospital. The proportion of patients with incomplete KD in group 2 (56 of 118 [47%]) was significantly higher than that in group 1 (20 of 85 [24%], p = 0.001). Median age (months) and interquartile ranges for group 1 was 26 (range 12.5-52) and for group 2 was 38.5 (range 18-63; p = 0.072). The number of patients diagnosed with KD having just 2 symptoms other than fever was significantly higher in group 2 (2.4 vs. 16.9%, p < 0.001). Erythrocyte sedimentation rate, albumin, and alanine aminotransferase levels were obtained in a significantly greater number of patients with KD after the guidelines were published. Thirty-two of the 203 patients studied had coronary artery (CA) involvement (15.8%), 4 of whom had CA aneurysms (2%) and 28 had CA ectasia only (13.8%). CA involvement was seen in 13 of 85 (15.3%) patients in group 1 and 19 of 118 (16.1%; p = 1) patients in group 2. After publication of the 2004 AHA guidelines, diagnoses of incomplete KD and laboratory use increased at our center; however, the rate of CA involvement remained stable. There also was a trend towards older age in children diagnosed with KD. Laboratory parameters and CA involvement between incomplete KD and classic KD were comparable.

Childhood Eye Problems

The articles in this issue of Pediatric Annals focus on common ocular problems that usually present initially to the child’s primary care provider. Pediatricians and other primary care providers are typically the ones who need to recognize the child with strabismus, cataract, amblyopia, and leukocoria for referral for ophthalmologic care. In addition, acute conjunctivitis may be viral or bacterial, and there are a number of therapeutic options. These topics are all covered well in the articles in this issue. Assessment of the normal red refl ex, the normal corneal light refl ex, and normal cover tests are all important in screening for signifi cant ocular abnormalities, some with very important long-term consequences. With respect to conjunctivitis, I would be remiss if I did not take this opportunity to suggest that Kawasaki disease should be considered in the child who presents with some days of fever and bilateral injected bulbar conjunctivae without exudate or drainage from the eyes. This presentation is one of the most consistent fi ndings in Kawasaki disease and should prompt a careful search (by history and physical exam) for other classic Kawasaki disease symptoms or signs. Should there be enough other features to prompt concern about the possibly of a complete or an incomplete (or atypical) case of Kawasaki disease, laboratory studies, including C-reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR), can be very valuable.

Isolated abducens palsy in adolescent girl with Kawasaki disease

Kawasaki disease (KD) represents one of the most common types of pediatric vasculitis. To date, there has been one case that is associated with abducens palsy.1 Here, we present a case of classic KD with right abducens palsy that developed during the subacute phase of the illness. A 12-year-old Caucasian girl with an unremarkable medical history was referred to our hospital with a 4-day history of fever, weakness and a rash. On the second day of fever, she developed rash in the lower extremities, vomiting, headache and complete blood count revealed leukocytosis (19 000/mm3). Physical examination revealed a maculopapular rash that blanched with central clearing over her trunk and lower extremities, unilateral lymphadenopathy, bilateral conjunctival injection, oropharynx hyperemia and strawberry tongue. There was no organomegaly, costovertebral tenderness or meningeal findings. Complete blood count and biochemical parameters were non-specific. Diagnosis of KD was made and 2D-echocardiography was performed, which showed a dilated proximal left coronary artery measuring 5.6–5.8 mm. Intravenous immunoglobulin (IVIG) and high-dose acetylsalicylate (80 mg/kg) were given. By her 4th hospital day, 2 days after IVIG therapy, she complained of double vision and lateral rectus palsy. There were no characteristic findings on computed cranial tomography. Lumbar puncture was not abnormal. Her cranial magnetic resonance imaging study was normal. Neurological exam and laboratory findings were consistent with right abducens palsy (Fig. 1). Oral dexamethasone 0.5 mg/kg/dose was started twice a day on her 5th hospital day. On the seventh hospital day the patient was afebrile and her general status was improved, but cranial nerve palsy persisted. Her erythrocyte sedimentation rate was relatively decreased to 40 mm/h. After her 13th hospital day, a dose of steroid treatment was decreased to a half-dose and after another week steroid treatment was ceased. The patient was discharged on the 20th day of hospitalization with acetylsalicylate therapy. A follow-up examination 4 weeks later presented resolution of right abducens palsy and disappearance of coronary involvement. Loss of abduction of the right eye is seen, demonstrating isolated sixth-nerve palsy. The diagnosis of KD can be made when there are fewer than four clinical features if coronary artery involvement is documented.2 Our case had four of the five criteria at the time of presentation (conjunctivitis, mucosa changes, lymphadenopathy and rash). Although the coronary artery involvement of KD is the most important cause of morbidity and mortality, neurological findings can be expected as KD has a multisystemic nature. Irritability and lethargy are common features during the clinical course of KD due to aseptic meningitis.3, 4 Nevertheless, cranial nerve palsy associated with KD has rarely been seen and the mortality rate is higher in KD associated with facial nerve palsy, as compared with KD without facial nerve palsy.1 Although this single case is not enough to be evidence, we suggest that recovery of the neurological finding may be related with acetylsalicylate and steroid treatment. This report reinforces the need to be aware of various manifestations of KD beside coronary artery involvement. It is also helpful to keep in mind the diagnosis of KD in any child with high fever and without cranial palsy.

Kawasaki Disease at the Extremes of the Age Spectrum

We sought to determine outcomes of Kawasaki disease (KD) and to explore factors associated with poor clinical outcomes for patients diagnosed outside the age range of 1 to 4 years. A retrospective review of data for all patients seen between January 1990 and April 2007 was performed. Patients were stratified into 5 groups on the basis of age at diagnosis. A total of 1374 patients were identified; 61 (4%) were <6 months of age at diagnosis, 114 (8%) 6 months to <1 year, 854 (62%) 1 to 4 years, 258 (19%) 5 to 9 years, and 87 (6%) >9 years. Patients <1 year of age and those >9 years of age were more likely to have coronary artery abnormalities than were patients diagnosed between 1 and 4 years of age. Patients diagnosed between the ages of 5 and 9 years were at the lowest risk. Patients at both extremes of the age spectrum were more likely to present with <4 of the classic KD features, but only those <6 months or >5 years of age were at increased risk of being diagnosed >12 days after illness onset. Patients <6 months of age had lower albumin levels, and those <1 year of age had higher white blood cell and platelet counts, all of which are known predictors of coronary artery abnormalities. Patients >9 years of age were less likely to receive intravenous immunoglobulin treatment. Outcomes for children diagnosed with KD at either extreme of the age spectrum are suboptimal, although the associated factors are different.

Acute Respiratory Distress Syndrome in a Child with Kawasaki Disease

This report presents a case of classic Kawasaki disease with progression to acute respiratory distress syndrome. The severity of the patient's pulmonary disease led clinicians to suspect toxic shock syndrome. Clinicians need to be aware that pulmonary manifestations of Kawasaki disease can include acute respiratory distress syndrome.