Atypical Kawasaki Disease Research Articles

Prolonged Fever and Intravenous Immunoglobulin Resistance in Kawasaki Disease: Should Macrophage Activation Syndrome Be Considered?

Introduction: Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of Kawasaki disease (KD). Typically, MAS presents as the exacerbation of KD, manifested by prolonged fever and resistance to treatment. Early and accurate diagnosis might be challenging due to overlapping features of MAS and intravenous immunoglobin (IVIG)-resistant KD and lack of validated diagnostic criteria. Case Presentation: In this article, we report four cases of KD with prolonged fever that were eventually diagnosed and treated as MAS. Three cases were boys and one infant was a girl. The mean age of the cases was 29 months. Three cases had typical and one case had atypical KD. Two cases had abnormal findings in echocardiography and three cases had hepatomegaly. In laboratory evaluation, all of them had thrombocytopenia, increased liver enzymes, and hyperferritinemia. All the patients were treated with intravenous high-dose methylprednisolone (pulse therapy) after IVIG therapy, two cases with oral cyclosporine, and one case with infliximab. There was no mortality in our cases, and in long-term follow-up, all the patients had normal clinical, laboratory, and echocardiography findings. Conclusions: KD resistant to IVIG and sustained fever have high diagnostic values in MAS with underlying KD.

Vascular imaging findings with high-pitch low-dose dual-source CT in atypical Kawasaki disease.

Determining the presence of aneurysms, thrombosis, and stenosis is very important for the diagnosis of atypical Kawasaki disease (AKD) and in the follow-up of AKD patients with aneurysms. We aimed to demonstrate high-pitch low-dose dual-source computed tomography (CT) angiography findings in pediatric patients with AKD. Over a 5-year period, high-pitch low-dose CT angiography was performed to determine vascular aneurysms or occlusions in 17 patients who had suspected AKD. The patients ranged from 2 months of age to 11.3 years, with a mean age of 3 years. The American Heart Association's criteria were used to diagnose AKD. We did not detect any vascular problems in 6 of the patients, and they were not included in our study. Arterial aneurysms were present in 11 patients (aged 2 months to 11.3 years; mean age, 4.2 years; 7 males). In one patient, there was also a thrombus at an arterial aneurysm. Coronary artery aneurysms were detected in 7 patients and systemic artery aneurysms were detected in 7 patients. Three patients had both systemic and coronary aneurysms. Our results suggest that high-pitch low-dose dual-source CT can detect all types of aneurysms, stenosis and occlusions of vessels in patients with AKD who were not previously diagnosed. This useful, easy, robust and fast technique may be preferred to diagnose AKD.

Recurrent Peeling of Skin Following Post-Streptococcal Atypical Kawasaki Disease: A Case Report and Literature Review

Kawasaki disease (KD) is a systemic vasculitis occurring due to collective immune mechanisms in a genetically susceptible individual, triggered by infections, though the actual cause is yet to be determined. KD is the most common cause of coronary heart disease in children. Typical diagnostic criteria are fever more than 4 days plus four of clinical criteria of cervical lymphadenopathy, skin rashes, mucus membrane redness and lip changes, non-purulent conjunctivitis, peripheral changes in palms and feet. In atypical cases three criteria with fever are enough to commence treatment and echocardiograph early. An often-reported association with KD is streptococcal infections. Skin peeling occurs in both conditions, however in streptococcal infections it occurs early while late during convalescence stage in KD except peeling of the genitocrural area that may occurs early. Skin peeling in the streptococcal infection is related to exotoxin while the cause of the peeling in KD is not well known; but it is likely related to the immunological pathogenesis. Another type of skin peeling that occurs recurrently following the initial attack of KD in many months is of an unknown reason. The presentation in this report is exceptional; it is for the first time reporting association of atypical KD presented with post streptococcal complications and vitamin D deficiency followed by the skin re-peeling 6 months after the patients fully resolved. Moreover, it is for the first time reporting nail changes, painful micturition due to urethral involvement and mucus membrane erythema that all resolved without treatment. The cause possibly is related to innate immune mechanism post infections and possible role for vitamin D deficiency paving the way for the post-group A streptococcal (GAS) vasculitis which is an immune response that later acted to trigger the skin peeling. Patient initially presented with significant peeling of streptococcal infections and features of KD responding to monoclonal antibody. This case highlights that clinicians need to know about recurrent peeling following KD in many months and to make the parents aware about its harmless course. It is the first report of association of post streptococcal atypical KD vasculitis, vitamin D deficiency, skin harmless post-KD skin peeling, nail changes, mucus membrane erythema and painful micturition due to urethral involvement. Int J Clin Pediatr. 2019;8(1):12-18 doi: https://doi.org/10.14740/ijcp319

PERIODIC FEVER WITH APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENTITIS (PFAPA) SYNDROME AND GLOMERULONEPHRITIS – AN UNCOMMON ASSOCIATION

Introduction Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a diagnosis of exclusion after thorough evaluation for alternate causes of periodic fevers. The diagnosis can also be more challenging when there are atypical presentations. We present a case of a young child with PFAPA and hematuria. Case Description Fifteen-month old female presented to care twice for prolonged fever and was treated for atypical Kawasaki disease with IVIG. Fevers associated with adenitis and pharyngitis continued every 8-10 weeks along with persistent microscopic hematuria. Genetic evaluation did not reveal mutations in genes associated with periodic fever syndromes. Fevers, but not hematuria, was responsive to prednisone; she was diagnosed with PFAPA. Evaluation from nephrology showed normal C3/C4, negative ANA, with urine analysis showing 11-50 RBC/hpf, normal urine protein-to-creatinine ratio, and normal calcium-to-creatinine ratio. She underwent kidney biopsy, which demonstrated focal segmental mesangial hypercellularity, mesangial matrix expansion, focal foot process effacement and villous transformation, consistent with glomerulonephritis. Discussion PFAPA is characterized by periodic fevers, aphthous ulcers, pharyngitis, and cervical lymphadenopathy, but other symptoms have been described such as abdominal pain, arthralgias, and headache1. We identified one other report of PFAPA with hematuria in a child whom was found to have IgA nephropathy on kidney biopsy2. Our case demonstrates a rare presentation of PFAPA with glomerulonephritis.

Pitfalls in the diagnosis and management of Kawasaki disease: An update for the pediatric dermatologist

Kawasaki disease is easily diagnosed when it presents in its complete form, but because not all characteristic symptoms are always present at the same time, and the diagnosis of incomplete and atypical Kawasaki disease is often challenging, a delay in diagnosis or misdiagnosis often occurs. We present the diagnostic approach to Kawasaki disease with common pitfalls and explain how to avoid them. We also describe current practice and new trends in treatment.

Abstract O-59: KAWASAKI DISEASE SHOCK SYNDROME (KDSS) IN CHILDREN FROM 16 LATIN AMERICAN (LA) COUNTRIES: A 5-YEAR RETROSPECTIVE MULTICENTER STUDY OF THE REKAMLATINA NETWORK (JANUARY 2009-DECEMBER 2013)

Aims & Objectives: KD is the leading cause of pediatric acquired cardiac disease in developed countries. KDSS occurs approximately in 5–7% of KD cases; however, its recognition is difficult even among experienced clinicians. We describe the first retrospective multinational multicenter study of KDSS in LA children. Methods Retrospective descriptive study of pts with a discharge diagnosis of classic or incomplete (atypical) KD who met KDSS criteria at 39 main referral hospitals from 16 LA countries. Study period: January-1st-2009 to December-31st-2013. Results Among 976 enrolled KD pts, 8(0.81%) developed a KDSS. Classic and incomplete (atypical) KD was diagnosed in 6(75%) and 2(25%) pts, respectively. 5(62.5%) were female pts. 5(62.5%) pts were <60 months of age. Median length of hospitalization was 14(8–35) days. Median length of fever at admission was 6(4–8) days. In 3(37.5%) pts, sepsis/shock but not KDSS was the initial admission diagnoses. Antibiotics were given in all 8 pts prior to both final KD and KDSS diagnoses. On admission, 5(62.5%) pts looked poorly perfused, 5(62.5%) had thrombocytopenia, and 6(75%) hypoalbuminemia. IVIG, aspirin and steroids were given in 8(100%), 7(87.5%), and 5(62.5%) pts respectively. A second dose of IVIG was required in 4(50%) pts. Baseline echocardiograms showed: coronary artery dilations and/or aneurysms, 2(25.0%) pts; pericardial effusion, 2(25.0%); valvular insufficiency, 2(25.0%). No deaths occurred. Conclusions Our incidence of KDSS is lower than reports from developed countries. However, difficulties in diagnosing KD and KDSS, misdiagnosis with sepsis/shock, and the retrospective nature probably reveal underestimates. KDSS cases were associated with prolonged hospitalizations, high rates of antibiotic use, and IVIG-resistance.

Sudden Child Death due to Thrombotic Giant Coronary Artery Aneurysms Complicated by Atypical Kawasaki Disease: An Autopsy Case.

Sudden Child Death due to Thrombotic Giant Coronary Artery Aneurysms Complicated by Atypical Kawasaki Disease: An Autopsy Case.

Atypical Kawasaki Disease in a 4 Years Old Child with Mumps

Kawasaki disease is an acute febrile condition seen in children. However, it is also well recognized that some patients do not fulfill the classic diagnostic criteria for the diagnosis of Kawasaki disease. The incomplete form of Kawasaki disease is termed as 'Incomplete KD' or 'Atypical KD'. This is a case of 4 years old child with fever and mumps. He had bilateral cervical adenitis. Patient failed to respond to IV antibiotics fulfilled the criteria of incomplete Kawasaki disease. The child was managed with high dose aspirin until the child was afebrile for 48 hours. Kawasaki disease is a common vasculitis in children. Atypical cases might be missed if there is concomitant viral illness. Hence the identification and management of Kawasaki disease is paramount to decrease the mortality related to the cardiac disease. Keywords: bilateral cervical adenitis; fever and mumps; failed to respond IV antibiotics; incomplete kawasaki disease.

Kawasaki Disease Complicated by Peripheral Gangrene in a Case of Inherited Thrombophilia

Kawasaki disease is a febrile vasculitis targeting medium sized arteries, causing coronary artery aneurysm without appropriate treatment. Many children do not fulfill the criteria and due to its serious complications, the American Heart Association (AHA) published an algorithm for atypical Kawasaki disease based on laboratory tests and echocardiography. Peripheral ischemia is a rare complication with harmful sequalae. Here we reports a case of a 14-month-old patient, who was diagnosed of atypical Kawasaki disease, developed peripheral ischemia and gangrene of the second and third right hand fingers, and was found to have factor V Leiden heterozygous mutation. Int J Clin Pediatr. 2018;7(3):43-45 doi: https://doi.org/10.14740/ijcp306w

Kawasaki disease: a comprehensive review

Kawasaki disease is an acute systemic vasculitis that was first reported in 1961. Over the last 5 decades multiple papers have been published to further understand this disease. The diagnosis of Kawasaki disease is made based on the clinical findings. Atypical Kawasaki disease includes patients who do not meet all the criteria for diagnosis. The main complication of Kawasaki disease is coronary aneurysm, and the treatment is intravenous immunoglobulin and aspirin. A second dose of immunoglobulin is given if the patient does not improve, and several other treatment options have been proposed over the last few years as second and third line options.

Macrophage Activation Syndrome in a Patient with Systemic-Onset Juvenile Idiopathic Arthritis Initially Treated for Atypical Kawasaki Disease : A Case-Based Review

Systemic Onset-Juvenile Idiopathic Arthritis | Atypical Kawasaki | Macrophage Activating Syndrome | Coronary Artery Dilatation | Corticosteroids | Arthrite Juvénile Idiopathique Systémique | Atypique Maladie de Kawasaki | Syndrome dʼActivation Macrophagique | Dilatation des Artères Coronaires | Corticoïdes

Manifestaciones neurológicas en la enfermedad de Kawasaki atípica

Kawasaki disease (KD) is a type of systemic vasculitis of unknown etiology. Atypical Kawasaki disease is defined as that where there are signs and symptoms not corresponding to the classical criteria for this nosological entity. Children with atypical Kawasaki disease may present with acute abdominal symptoms, meningeal irritation, pneumonia or renal failure. We describe 4 children with ages ranging from 2 to 12 years who had atypical Kawasaki disease, with neurological and gastrointestinal symptoms as part of the systemic presentation of the disease. Treatment consisted of immunoglobulin and corticosteroids with good evolution. KD is a systemic vasculitis that can involve many territories. Atypical manifestations can mislead the clinician and delay diagnosis. Pediatricians and sub-specialists should be aware of these neurological manifestations in order to provide adequate and opportune treatment.

Joint glucocorticoid clinical analysis of initial treatment of Kawasaki disease

Objective To investigate the advantages of glucocorticoid combined with traditional methods and to explore glucocorticoid use in appropriate time in the whole course of the disease and the appropriate dose. Methods With reference to Kawasaki disease(KD)diagnostic criteria, 363 cases of patients with KD were enrolled, and divided into conventional treatment group and combined hormone group randomly.Combined hormone group according to clinical manifestations can be divided into sub groups as typical KD and atypical KD group.We analyzed respectively before and after 1 week treatment in two groups of children with the onset of age, gender, heating duration after treatment, IVIG treatment time, hospitalization days and total hospitalization expenses, tips and course of acute stage, recovery period the relevant indexes to the test, and coronary artery lesion(CAL)in one month. Results Thermal process after treatment, hospitalization duration and hospitalization expenses decreased significantly in combined hormone group compared with conventional treatment group.The difference between the two, in the clinical laboratory indexes and CAL have no obvious difference.In different types of KD, the thermal process after treatment, hospitalization days and hospitalization expenses, have decreased significantly in typical KD sub group when treating with combined hormone therapy comparing with hormone therapy atypical sub group.Other clinical laboratory indexes and CAL have no obvious differences in compared between the sub groups. Conclusion Combined hormone therapy for KD patients, plays an important role in shortening the thermal process after treatment and reducing the hospitalization expense. Key words: Kawasaki disease; Glucocorticoids; Intravenous gamma globulin

Super-Giant Coronary Aneurysm Secondary to Atypical Kawasaki Disease in an 11-Year Old

Super-Giant Coronary Aneurysm Secondary to Atypical Kawasaki Disease in an 11-Year Old

Acute myocardial infarction with a giant left main aneurysm in atypical Kawasaki disease

Acute myocardial infarction with a giant left main aneurysm in atypical Kawasaki disease

Syndrome d’activation macrophagique et maladie de Kawasaki : à propos de quatre observations

Macrophage activation syndrome (MAS) is a rarely reported complication of Kawasaki disease (KD). It must be sought during KD with unusual clinical signs, such as enlargement of the liver or spleen, cytopenia (including thrombocytopenia), and elevated serum triglycerides. Here, we report four cases from a single center. The first is the description of a case of KD occurring in an 11-month-old child 15 days after the occurrence of infectious spondylitis. For the second, acute myocarditis heralded KD in a 5-year-old child. A 15-year-old boy had multiorgan failure with shock. The last case describes an infant with atypical KD. All had hepatosplenomegaly, thrombocytopenia, and increased serum ferritin. For all of them, hemophagocytosis was present on the bone marrow smears. The condition rapidly evolved favorably with intravenous immunoglobulins or steroids. The extensive microbiological work-up was unrevealing. None had any coronary sequelae after the episode. KD should be added to the list of inflammatory conditions that may be complicated by MAS that modifies the presentation mimicking toxic shock or infection-associated activation syndrome and makes the diagnosis difficult. This association does not appear to have a prognostic impact on the course of the disease in the literature, confirmed by the analysis of this small series.

Kawasaki disease – experience of Pediatric University Hospital, Sofia, Bulgaria, 1993–2014. Part I: clinical manifestations

ABSTRACTKawasaki disease (KD) is a vasculitis syndrome causing coronaritis in young children. As a result of the vascular damage, coronary lesions (CL), ectasia and aneurysms form in 20%–25% of the untreated children. The assessment of KD is difficult and challenging because of the lack of specific diagnostic or laboratory criteria. This is a retrospective study of 107 patients for a period of 21 years (1993–2014). In the cohort, 30.8% of patients had CL (19.6% had coronary aneurysms and 11.2% had significant coronary dilatations). The number of CL was high compared to that reported in international studies, although 45% of children were treated by modern protocols. In an attempt to analyse the reasons for the high coronary risk, the aim of this study was to investigate the clinical aspects of the disease and to establish the diagnostic problems causing diagnosis delay, which is subsequently related to increased coronary risk. The high incidence of the observed CL was associated with the failure of recognizing the disease, delayed diagnosis and, subsequently, lack of correct treatment. The analysis of the clinical presentation indicated significant correlation of gastrointestinal syndrome with typical and atypical KD. The incidence of the gastrointestinal syndrome correlated with the typical KD symptoms (p = 0.030), suggesting that it could be considered as a further diagnostic criterion.

Echocardiographic evaluation of complete and incomplete Kawasaki disease in terms of coronary and non coronary cardiac involvement

Background &amp; Objectives: With increasing incidence of Kawasaki disease and its associated complications, the need of the hour is early diagnosis. Incomplete Kawasaki is often diagnosed late and is frequently associated with morbidities and mortalities. Our study aims to emphasize the importance of echocardiography in detection of incomplete Kawasaki disease.Materials &amp; Methods: A total of 150 children, clinically diagnosed as Kawasaki disease were evaluated echocardiographically and involvement of coronary artery and associated cardiac involvements were studied.Results: Amongst the incomplete Kawasaki disease patients 76% were from extremes of ages and 19.2% of them had coronary involvement. Myocarditis(38.1%) was the commonest non-coronary findings.Conclusion: Atypical Kawasaki disease is more common in extremes of ages and echocardiography helps in detecting atypical disease at the earliest, thus facilitating prompt treatment and reducing complications.

Difficult diagnosis of atypical kawasaki disease in an infant younger than six months: a case report

BackgroundKawasaki disease (KD) is an acute inflammatory vasculitis of unknown origin.Case presentationWe report the case of a 5-month-old child with an atypical form of KD, characterized by undulating symptoms, who developed an aneurysm of the right coronary artery and an ectasia of the left anterior descending coronary artery.ConclusionThis case report underlines the difficulties in recognizing incomplete forms of the illness in young infants, who are at higher risk of cardiac complications.

Atypical Kawasaki Disease in 2-Months Old Infant Presenting with Aseptic Meningitis

The diagnosis of incomplete or atypical Kawasaki disease (KD) is challenging. Children presenting with febrile illnesses may have few of the classical diagnostic criteria, yet develop clinical, laboratory and echocardiographic findings compatible with the condition. Early recognition and prompt treatment of KD is essential for better outcomes. This is particularly the case for infants who are at a greater risk of developing complications such as coronary artery aneurysms. This may be partly attributed to late diagnosis or initiation of therapy. This report discusses the case of a febrile infant with atypical Kawasaki disease presenting with aseptic meningitis and coronary artery aneurysm. The case highlights the importance of considering atypical Kawasaki disease in any infant with prolonged fever (more than seven days), following exclusion of infectious causes and in the absence of classical diagnostic criteria.