Involvement In Kawasaki Disease Research Articles

Role of Kobayashi Risk Scoring for Determining Refractory Kawasaki Disease

Background: Coronary complications are common in children with Kawasaki disease (KD). Administration of intravenous immunoglobulin (IVIG) during the first 10 days will result in lower coronary complications. It is useful to predict if a patient will develop coronary complications or not. Objectives: We designed this study to evaluate the role of Kobayashi risk scoring for determining refractory KD. Methods: Medical records of all cases with KD were reviewed and demographic data, previous history, coronary artery involvement, laboratory findings, and days with fever were extracted for all cases. Kobayashi score was calculated. Results: A total of 168 cases enrolled. There was no significant difference regarding Kobayashi score in different groups regarding coronary involvement. In cases without coronary involvement, 93.5% were classified as low risk, in one and two vessels involvement groups, all were categorized as low risk, and among three vessels involvement cases, 75% were known as low risk. Conclusions: Kobayashi score is not useful for predicting severity of coronary involvement in Kawasaki disease.

Abdominal-pain-first presentation of Kawasaki disease

Clinicians from Italy cared for a child with high fever and abdominal pain as first complaints of Kawasaki disease. Abdominal computed tomography showed concentric thickening of jejunal bowel, dilatation of small and large intestine, ascitic fluid, and enlargement of mesenteric lymph nodes. As typical mucocutaneous signs and laboratory findings appeared, the diagnosis was considered. IGIV therapy was associated with rapid defervescence and resolution of abdominal pain. The authors reviewed 48 published cases of Kawasaki disease with intestinal involvement. Fever, abdominal pain, and vomiting generally were first or early manifestations, before appearance of mucocutaneous findings. Pseudo-obstruction was present in >80% of cases by plain radiograph or another imaging study. Hydrops of the gallbladder was not common. Kawasaki disease was not an early consideration and 25% of patients underwent surgery. Coronary artery aneurysms were present in 43% of cases at the time of Kawasaki disease diagnosis. Symptoms resolved following IGIV therapy. Authors of published cases did not report intestinal sequelae (but follow up was variable and usually was short). This editor has cared for a very young infant with Kawasaki disease before the IGIV therapy era who had bowel obstruction due to jejunal stenosis 2 months after the acute phase. Compilation of 57 cases of lymph-node-first presentation of Kawasaki disease was reported in The Journal in 2013 (J Pediatr 2013;162:1259-63).1 We now add abdominal-pain-first presentation. It should not be too surprising that intestinal vasculitis could be significant in some cases. Might it not suggest an intestinal portal of microbe or super antigen entry, as might cervical lymphadenitis a respiratory tract portal of entry? The 43% rate of coronary artery aneurysms at the time of diagnosis in cases of abdominal-pain-first Kawasaki disease in the literature likely represents both diagnosis and reporting biases. However, it behooves us all to consider Kawasaki disease in the differential when a child has high fever and abdominal pain. Article page 186 ▶ Intestinal Involvement in Kawasaki DiseaseThe Journal of PediatricsVol. 202PreviewTo describe a case of Kawasaki disease with intestinal involvement and to analyze other published reports to define clinical characteristics, diagnostic issues, and therapeutic approaches of gastrointestinal involvement in Kawasaki disease. Full-Text PDF

Utilization of Coronary Artery to Aorta for the Early Detection of Kawasaki Disease

Timely diagnosis of coronary involvement is paramount in Kawasaki disease (KD) as it can be associated with long-term morbidity. However, echocardiographic measurements of coronary artery dilation in KD are inconsistent and not proficient for all abnormal arteries. The purpose of this study was to investigate more valuable indices and determine their sensitivity and specificity for early diagnosis of coronary involvement in KD. We performed this retrospective study in 218 children. All patients underwent laboratory and echocardiographic evaluations upon admission. We measured the size of the left main coronary artery (LMA), left anterior descending coronary artery (LAD), right coronary artery (RCA), and aorta (Ao), and calculated the LMA/Ao, LAD/Ao, and RCA/Ao ratios. We also calculated the cut-off values of each index using receiver operating characteristic curves. LMA, LAD, and RCA measurements did not correlate with white blood cell count, platelet count, erythrocyte sedimentation rate, C-reactive protein level, or brain natriuretic peptide level. The LMA measurement was associated with hemoglobin, hematocrit, and iron saturation. LAD/Ao was correlated with white blood cell and platelet counts (P < 0.05), whereas RCA/Ao was correlated with ferritin level (P < 0.05). The cut-off value of LMA/Ao was 0.2, with a sensitivity of 85% and specificity of 70%. Individual coronary artery/Ao ratios might provide helpful insight for detection of coronary abnormality in KD in the acute phase. Further investigation is essential to clarify prompt early diagnosis of coronary involvement in KD.

Intestinal Involvement in Kawasaki Disease

To describe a case of Kawasaki disease with intestinal involvement and to analyze other published reports to define clinical characteristics, diagnostic issues, and therapeutic approaches of gastrointestinal involvement in Kawasaki disease. A computerized search without language restriction was conducted using PubMed and SCOPUS. An article was considered eligible for inclusion in the systematic review if it reported data on patient(s) with intestinal involvement in Kawasaki disease. Our case was also included in the analysis. Thirty-three articles reporting 48 cases of Kawasaki disease with intestinal involvement were considered. Fever, abdominal pain, and vomiting were the most frequent symptoms observed and typical Kawasaki disease signs and symptoms appeared after intestinal complaints in all cases. Plain radiographs, ultrasonography, and computed tomography showed pseudo-obstruction as the most frequent sign of gastrointestinal involvement; 25 patients underwent surgery. Cardiac involvement was documented in 21 cases. All but three patients received medical treatment with immunoglobulin intravenous or aspirin. The outcome was good in 28 patients; 7 patients showed persistence of coronary artery abnormalities; 1 patient developed cyanosis, and later, left hand and forefoot gangrene; 3 patients died. The diagnosis and treatment of Kawasaki disease might be delayed if intestinal symptoms appear before the characteristic clinical features of Kawasaki disease, thus, increasing the risk of cardiac complications. Furthermore, patients may undergo unnecessary invasive procedures. Pediatricians and pediatric surgeons, therefore, should consider Kawasaki disease among diagnoses in children with fever, abdominal symptoms, and radiologic findings of pseudo-obstruction.

Risk of coronary artery involvement in Kawasaki disease

Kawasaki disease refers to systemic vasculitis with risk of coronary artery disease. Our objective is to identify risk factors associated with coronary artery disease in patients with complete and incomplete Kawasaki disease. Descriptive, retrospective study conducted in patients diagnosed with Kawasaki disease in a tertiary-care hospital between 2008 and 2014. The American Heart Association diagnostic criteria were used to define complete and incomplete Kawasaki disease. Thirty-one children were diagnosed with Kawasaki disease; 24 met the criteria for the complete form, and 7, for the incomplete form of this condition. Five had coronary artery disease. One of them had incomplete Kawasaki disease (1/7= 14.3%), and the remaining four had the complete form (4/24= 16.7%). No significant differences were found between both groups (p= 1.0). Patients with coronary artery involvement had a higher C-reactive protein level (median: 16.2 mg/dL versus 8.4 mg/dL, p= 0.047) and lower albuminemia (median: 3.2 mg/dL versus 3.99 mg/dL, p= 0.002). The risk of coronary artery involvement in incomplete Kawasaki disease is similar to that in complete Kawasaki disease; therefore, in patients with the incomplete form, immunoglobulin therapy should not be delayed. In our population, C-reactive protein and albumin levels were related to a higher risk of coronary artery involvement.

Coronary artery involvement in Kawasaki disease

Coronary artery involvement in Kawasaki disease

Bilateral optic neuritis in a patient with Kawasaki disease

We describe a 7-year-old male with a history of fever, headache, bilateral non-purulent conjunctivitis, and photophobia. Patient was initially treated for suspected bacterial meningitis with intravenous antibiotics. Due to lack of clinical response, negative cultures, and evolving signs of Kawasaki disease, he was treated with one dose of intravenous immunoglobulin. Patient improved except for the persistence of photophobia and bilateral conjunctival injection. Eye examination showed bilateral optic neuritis and a visual acuity of 20/80 in both eyes. Follow up eye examination after 6 weeks showed normal visual acuity, resolution of conjuctival injection, photophobia, and optic neuritis. This case highlights the posterior segment involvement in Kawasaki disease and the need for careful eye examination in patients with Kawasaki disease who have prolonged photophobia and conjunctival injection.

Abstract 144: Lung Parenchymal Consolidation during typical Kawasaki Disease

Introduction: Kawasaki disease (KD) is a systemic vasculitis of childhood throughout the body. We report a patient who complicated by severe pneumonia of lung parenchymal consolidation (LPC) during typical KD clinical course. Case reports: A 7-yr-old male who had a past history of KD at 3-yr-old, visited a general practitioner with fever, sore throat and neck pain. Three days later, after being treated with CTRX/TBPMPI, he was transferred to our hospital because of bilateral lymph nodes swelling and a generalized erythema and rash. On admission he was still febrile and had bilateral conjunctival injections, cervical and axillary lymphadenopathy and markedly reddened pharynx, without edema. We diagnosed him as KD and treated him with aspirin and IVIG at a dose of 2.0g/kg, but the condition worsened; he developed dyspnea and dry cough. Crackles were noted on rt-lung auscultation, along with rt-basal dullness to percussion of the thorax. Chest radiography and a subsequent computed tomography scan revealed disseminated patchy infiltrates and ground-glass alterations with massive pleural effusions on rt-lung, and enlarged axillary and mediastinal lymph nodes. Some kinds of microbiology (including Mycoplasma) and virology (Epstein-Barr virus, cytomegalovirus, parvovirus B19), from all sampled sites (pleural effusion, blood, pharyngeal swab and cerebrospinal fluid), were negative for pathogenic specimens. So we treated him as intractable KD with second-IVIG and m-PSL, which led to the quick resolution of fever and progressive amelioration of pneumonia. Echocardiography showed normal ventricular size and function without coronary artery lesion (CAL). Thereafter, he was finally afebrile. At the first follow-up visit, 1 month after discharge, he had fully recovered. Clinically, echocardiographically and radiographically, no residues were noted. Conclusion: Several manifestations of broncho-pulmonary involvement in KD have been described. But there are very few case of LPC. We should consider the possibility that LPC were secondary to pulmonary arteritis.

Abstract 224: Repeat Coronary Artery Bypass Grafting in an Adult Case of Kawasaki Disease

Background: The incidence of giant coronary artery aneurysm as a complication of Kawasaki disease is approximately 0.35%. Kawasaki disease was first described in 1967. However, only a few studies have reported on adult cases of cardiovascular sequelae in Kawasaki disease. Adult cases of coronary stenosis due to Kawasaki disease shows a predilection for the age range of late teens to the twenties; elderly onset, such as around the age of 60 years, is rare. Case report: A 62-year-old woman with a history of coronary artery bypass grafting (two-vessel bypass) was diagnosed with a coronary artery aneurysm and concomitant Kawasaki disease at another hospital at the age of 48 years. A reoperation was recommended owing to re-strangulation, but the patient opted for progress observation. At the age of 53 years, the patient experienced respiratory pain during laborious work, and coronary angiography was indicated. We therefore performed coronary artery bypass grafting (four-vessel bypass) for there to base Kawasaki disease, calcification of advanced, restenosis, coronary flow was observed. The postoperative course was good, and the patient has remained without restenosis for 9 years after the surgery. Conclusion: The progress of patients with a history of Kawasaki disease in adulthood has not been established; these patients therefore require periodical follow-up examinations. Moreover, because coronary artery involvement in Kawasaki disease is a risk factor for myocardial infarction, careful attention should be paid to lifestyle-related diseases.

Abstract 28: African American Race is Not Protective Against Coronary Artery Involvement in Kawasaki Disease

It has been previously reported that African American race may be protective against coronary artery aneurysm development in Kawasaki Disease (KD). We aimed to test this with our own cohort of KD patients from a large pediatric cardiology practice. Data from 250 subjects diagnosed with KD and followed as outpatients with surveillance echocardiography over a two-year period were analyzed. Twelve patients were excluded due to incomplete records or an unconfirmed diagnosis. Race designated by parent was recorded. Charts were reviewed for any coronary involvement (ectasia or aneurysm) and coronary Z-score greater than 2.5 at the time of diagnosis and at subsequent follow-up visits. Odds rations were calculated comparing each racial group to others for any coronary involvement and for coronary Z-score &gt; 2.5. Of 238 included patients, 44.5% were African American, 37.4% were non-Hispanic white, 10.5% were Hispanic, and 7.6% identified with other racial designations. Approximately 21.9% of African American patients had any coronary involvement and 9.5% had a coronary Z-score &gt; 2.5. Approximately 21.4% of non-Hispanic whites had any coronary involvement and 13.5% of non-Hispanic whites had a coronary Z-score &gt; 2.5. Twenty-eight percent of Hispanic patients had any coronary involvement and 12% had a coronary Z-score &gt; 2.5%. Of patients that identified with other racial designations, 38.9% had coronary involvement and 22.2% had a coronary Z-score &gt; 2.5. No statistically significant odds ratios were identified. Relative to reference group (non-Hispanic whites) African American patients had nearly identical rates of 1) any coronary involvement, or 2) coronary Z-score &gt; 2.5. KD occurs commonly in African-American children. Given equal risk for late coronary sequelae vigilance and strict adherence to consensus guidelines is essential.

Can neutrophil-to-lymphocyte ratio predict cardiac involvement in kawasaki disease?

Kawasaki disease (KD) is an acute self-limited vasculitis, in which 1/3 of patients develop coronary artery lesions (CAL) if untreated. Although studies have explored potential biomarkers to predict patients with KD who are at risk of CAL, no useful single marker exists. The blood neutrophil to lymphocyte ratio (NLR) is identified as a potentially useful marker of clinical outcome in inflammatory diseases.

Hepatobiliary risk factors for clinical outcome of Kawasaki disease in children

BackgroundKawasaki disease (KD) is an acute febrile vasculitis that causes coronary artery abnormality (CAA) as a complication. In some patients, an association has been noted between elevated liver enzymes or an abnormal gallbladder (GB) and hepatobiliary involvement in KD. In this study, we aimed to evaluate clinical, laboratory, and ultrasonographic (USG) risk factors of hepatobiliary involvement for the intravenous immunoglobulin (IVIG) resistance and the development of CAA in children with KD.MethodsFrom March 2004 through January 2013, clinical features, laboratory data, echocardiographic findings, and USG findings were retrospectively reviewed regarding the response to IVIG treatment and coronary artery complications in 67 children with KD. Acute acalculous cholecystitis (AAC) was diagnosed based on USG criteria.ResultsAmong all factors, only the prothrombin time international normalized ratio was significantly different between the IVIG-response and IVIG-resistance groups (p = 0.024). CAA was statistically more frequent in the AAC group (n = 24) than in the non-AAC group (n = 43) (23.3% vs. 58.3%, p = 0.019). Among the laboratory factors, segmented neutrophil percentage, total bilirubin level, and C-reactive protein were significant in children with CAA (p = 0.014, p = 0.009, and p = 0.010). Abnormal GB findings on USG were significantly more frequent in children with CAA than in those without CAA (p = 0.007; OR = 4.620; 95% confidence interval [CI]: 1.574–13.558). GB distension on USG was the only significant risk factor for CAA (p = 0.001; OR = 7.288; 95% CI: 2.243–23.681) by using multiple logistic regression analysis.ConclusionFor children in the acute phase of KD, USG findings of the GB, especially GB distension, may be an important risk factor for CAA as a complication.

Cardiovascular involvement in Kawasaki disease in Algerian children: Our experience

Cardiovascular involvement in Kawasaki disease in Algerian children: Our experience

Can Coronary Artery Involvement in Kawasaki Disease be Predicted?

Background: Coronary artery involvement is seen in approximately 15–20% of children with Kawasaki disease. There is conflicting literature regarding the clinical and laboratory findings associated with coronary artery involvement. In this retrospective study, we attempt identification of predictive factors for coronary artery involvement at our institute and review the existing literature. Methods and results: A review of 203 patients (65% males) with Kawasaki disease was performed, of whom 33 (16.3%) had coronary artery involvement. High erythrocyte sedimentation rate, high platelet count, low hematocrit, low albumin levels, and refractory Kawasaki disease showed significant association with coronary artery involvement. High erythrocyte sedimentation rate and refractory Kawasaki disease were found to be independent predictors of coronary artery involvement. Review of literature suggested a wide range of coronary involvement (<5% to >60%), and highly conflicting clinical and laboratory associations. Conclusion: It remains difficult to accurately determine risk of coronary artery involvement, although some laboratory markers may provide information that is helpful for parental counseling and clinical follow up. Future identification of novel biomarkers and host predispositions may further our understanding of coronary artery risks and help personalize therapy for Kawasaki disease.

Facial Nerve Palsy and Kawasaki Disease

Kawasaki Disease (KD) is a vasculitic disease and can affect any organ system in the body. The development of coronary artery aneurysms is the most common and life threatening complication of KD and makes this disease the leading cause of acquired heart disease in children in the developed world. Facial nerve palsy has been reported as a possible marker of more severe disease and increased risk of coronary artery involvement in KD. Herein, the authors report an 8-mo-old infant who had left sided facial nerve palsy and multiple coronary aneurysms associated with KD.

Kidney and Urinary Tract Involvement in Kawasaki Disease

Kawasaki disease (KD) is a systemic vasculitis and can develop multiple organ injuries including kidney and urinary tract involvement. These disorders include pyuria, prerenal acute kidney injury (AKI), renal AKI caused by tubulointerstitial nephritis (TIN), hemolytic uremic syndrome (HUS), and immune-complex mediated nephropathy, renal AKI associated with either Kawasaki disease shock syndrome or unknown causes, acute nephritic syndrome (ANS), nephrotic syndrome (NS), renal tubular abnormalities, renal abnormalities in imaging studies, and renal artery lesions (aneurysms and stenosis). Pyuria is common in KD and originates from the urethra and/or the kidney. TIN with AKI and renal tubular abnormalities probably result from renal parenchymal inflammation caused by T-cell activation. HUS and renal artery lesions are caused by vascular endothelial injuries resulting from vasculitis. Some patients with ANS have immunological abnormalities associated with immune-complex formation. Nephromegaly and renal parenchymal inflammatory foci are detected frequently in patients with KD by renal ultrasonography and renal scintigraphy, respectively. Although the precise pathogenesis of KD is not completely understood, renal vasculitis, immune-complex mediated kidney injuries, or T-cell immune-regulatory abnormalities have been proposed as possible mechanisms for the development of kidney and urinary tract injuries.

Risk Factors of Coronary Involvement in Kawasaki Disease in Recent Outbreak in South Iran, Shiraz.

Background: During the past year, Kawasaki disease (KD) had an outbreak with a high inci-dence of coronary involvement in Shiraz, Iran. This study focuses on risk factors and their correlation with aneurysm formation. Methods: All patients who were admitted with the diagnosis of KD in the pediatric units of Shiraz, Iran between 2009 and 2010 were included in this study. Data analysis was performed on demographic as well as clinical features, laboratory data, echocardiography, and electrocardiogram as well as treatment details. Results: The population under study included 45 patients, 25 males (55.6%) and 20 females (44.4%), with the mean age of 3.52 ± 2.77 years. Aneurysm was seen in 10 cases (22.2%) with the male to female ratio of 9:1. The most important risk factor of aneurysm formation was the male gender with the odds ratio of 10.6. Age more than 8 years was a very specific but non-sensitive predictor of aneurysm formation (sensitivity: 40% and specificity: 100%). Platelet counts more than 424Aƒâ€”103 was the most sensitive predictor of aneurysm formation (sensitivity: 90% and specificity: 57.1%). Fever duration more than 10 days had the sensitivity of 60% and the specificity of 88.6% for developing coronary aneurysm. No significant difference was seen in other clinical symptoms and laboratory data including complete blood count, liver function test, renal function test, electrolyte, and ECG data. Conclusion: Male gender, older age, prolong fever before treatment, and higher platelet counts were the risk factors of coronary aneurysm in KD. More education for early diagnosis of Kawa-saki disease in older patients is highly recommended.

Cardiac involvement in Kawasaki disease in Pakistani children

Introduction:Coronary artery involvement is the most dreaded long-term complication of Kawasaki disease. Our aim was to look at the pattern of cardiovascular involvement in Pakistani children admitted with Kawasaki disease.Methods:This study included children admitted with Kawasaki disease at the Aga Khan University Hospital Karachi over a period of 14 years from January 1997 to December 2010. Information gathered included patient demographics, clinical features, investigations, echocardiographic findings, treatment and follow-up. Those with coronary artery involvement on initial echocardiogram remained on long-term follow-up with clinical examination and echocardiogram.Results:A total of 56 patients were admitted. (Mean age at diagnosis 33 ± 30 months, age range 2 months to 9 years). 18% of patients had incomplete features. Twenty-five percent (14/56) patients presented after 10 days of fever. Cardiac examination was normal except for tachycardia. Abnormal coronary arteries were seen in 23 patients (41%) - left main coronary artery in 23 (41%), left anterior descending and right main coronary artery in 20 (36%), circumflex branch in 17 (30%). Risk factors for cardiac involvement were male sex, fever >10 days duration at the time of initial presentation and neutrophil percentage >75% in the initial white blood cell counts. Fifty four of 56 cases received intravenous immunoglobulin (IVIG), Seventy-five percent of the patients received IVIG within 10 days of illness. Mean duration of follow-up was 2.5 years. Eight percent of the patients still continue to have abnormal coronaries. There was no mortality.Conclusions:A higher incidence of coronary artery involvement was found in our study. Presentation after 10 days of illness increases the risk of coronary artery involvement. High index of suspicion among the general pediatricians about the disease can possibly be helpful for early referral and treatment.

Severe Global Inflammatory Involvement of Ocular Segments and Optic Disc Swelling in a 12-Year-Old Girl with Kawasaki Disease

Pediatric Kawasaki ocular involvement is dominated by bulbar conjunctival injection and mild, self-limited anterior uveitis. Posterior segment involvement is rare. Case Report. Despite early efficient treatment including aspirin and intravenous immunoglobulins, a 12-year-old girl developed a severe bilateral global inflammatory ocular involvement including punctuated keratitis, retrodescemetic precipitates, anterior uveitis, vitritis, and bilateral optic disc swelling with papillitis. This is the first description of severe bilateral global inflammatory involvement of the eyes in Kawasaki disease (KD). Usually subclinical and self-limited, eye involvement in KD can lead to severe visual impairment. Inflammation of both anterior and posterior segments does not seem to respond to KD-specific treatment and could justify a specific ophthalmologic therapeutic approach.

Normalization of echocardiographically derived paediatric cardiac dimensions to body surface area: time for a standardized approach

The quantification of cardiac dimensions derived from echocardiography is important in paediatric cardiac practice. Evaluation of the size and growth of cardiac chambers, valves, and great vessels plays a key role in the management of congenital heart disease, from the initial decision-making in the neonatal period to the nature and timing of subsequent interventions. It may also be important in the assessment and risk stratification of children with ‘acquired’ heart disease such as hypertrophic cardiomyopathy or coronary artery involvement in Kawasaki disease. Body size and cardiac dimensions change dramatically during normal growth and development. Therefore, it is necessary to place in context the measured size of a given cardiac structure by correcting for body size, through the process of normalization. The paper by Neilan et al. 1 in this issue of the Journal is a timely addition to the literature on normalization in paediatric populations, and raises a number of important issues.