Diagnosis and treatment of incomplete Kawasaki disease in children / 中国当代儿科杂志

Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion. This article reviews the concept, epidemiological features, diagnosis, treatment, and follow-up management of IKD, in order to deepen the understanding of IKD among clinical workers and help to improve the clinical diagnosis and treatment of KD in China.

Clinical application of plasma exchange combined with continuous veno-venous hemofiltration dialysis in children with refractory Kawasaki disease shock syndrome / 中国当代儿科杂志

OBJECTIVES@#To study the role of plasma exchange combined with continuous blood purification in the treatment of refractory Kawasaki disease shock syndrome (KDSS).@*METHODS@#A total of 35 children with KDSS who were hospitalized in the Department of Pediatric Intensive Care Unit, Hunan Children's Hospital, from January 2019 to August 2022 were included as subjects. According to whether plasma exchange combined with continuous veno-venous hemofiltration dialysis was performed, they were divided into a purification group with 12 patients and a conventional group with 23 patients. The two groups were compared in terms of clinical data, laboratory markers, and prognosis.@*RESULTS@#Compared with the conventional group, the purification group had significantly shorter time to recovery from shock and length of hospital stay in the pediatric intensive care unit, as well as a significantly lower number of organs involved during the course of the disease (P<0.05). After treatment, the purification group had significant reductions in the levels of interleukin-6, tumor necrosis factor-α, heparin-binding protein, and brain natriuretic peptide (P<0.05), while the conventional group had significant increases in these indices after treatment (P<0.05). After treatment, the children in the purification group tended to have reductions in stroke volume variation, thoracic fluid content, and systemic vascular resistance and an increase in cardiac output over the time of treatment.@*CONCLUSIONS@#Plasma exchange combined with continuous veno-venous hemofiltration dialysis for the treatment of KDSS can alleviate inflammation, maintain fluid balance inside and outside blood vessels, and shorten the course of disease, the duration of shock and the length of hospital stay in the pediatric intensive care unit.

Consenso sobre el tratamiento del síndrome inflamatorio multisistémico asociado a COVID-19 / Consensus on treatment of multisystemic inflammatory syndrome associated with COVID-19

La pandemia ocasionada por el nuevo coronavirus (SARS-CoV-2), declarada por la Organización Mundial de la Salud OMS) en marzo de 2020, afecta a un reducido número de pacientes pediátricos, quienes presentan, en su mayoría, compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos, comenzó a observarse un aumento de casos definidos como síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por el nuevo coronavirus (COVID-19) (KL-C) que evolucionan al shock y requieren internación en la unidad de cuidados intensivos.Los cuadros de SIM-C y los KL-C se caracterizan por fiebre, signos de inflamación, síntomas gastrointestinales y disfunción cardiovascular; las formas graves de presentación tienen mayor incidencia de hipotensión y/o shock. En el laboratorio se observan marcadores de inflamación, hipercoagulabilidad y daño miocárdico. El tratamiento farmacológico de primera línea consiste en la administración de inmunoglobulina por vía intravenosa más ácido acetilsalicílico por vía oral.Se recomienda un abordaje multidisciplinario para un diagnóstico certero y un tratamiento temprano y eficaz para disminuir la morbimortalidad.

The pandemic caused by the SARS-CoV-2 virus declared by the WHO in March 11th 2020, affects a small number of pediatric patients, who mostly present mild respiratory compromise and favorable evolution.However began to be observed in previously healthy children, an increase in cases defined as "Multisystemic Inflammatory Syndrome" (MIS-C) or "Kawasaki-like" post-COVID 19 (KL-C) that evolve to shock and require hospitalization in the Pediatric Intensive Care Unit.MIS-C and KL-C are characterized by fever; signs of inflammation, gastrointestinal symptoms, and cardiovascular dysfunction, associated with sever forms of presentation with higher incidence of hypotension and/or shock. In the laboratory, markers of inflammation, hypercoagulability and myocardial damage are observed. First-line drug treatment consists of intravenous immunoglobulin plus oral acetylsalicylic acid.A multidisciplinary approach is recommended for an accurate diagnosis and an early and effective treatment, in order to reduce morbidity and mortality.

Clinical-epidemiological relation between sars-cov-2 and kawasaki disease: an integrative literature / Relação clínico-epidemiológica entre sars-cov-2 e doença de kawasaki: uma revisão integrativa da literatura

ABSTRACT Objective: To analyze the current scientific literature to document, in an integrative review, the main findings that correlate Kawasaki disease (KD) to COVID-19. Data sources: The search was carried out in June 2020 in the following databases: Biblioteca Virtual em Saúde (BVS), periódico da CAPES and U.S National Library of Medicine (PubMed). The combination of descriptors used was [(COVID-19 OR SARS-CoV-2) AND (Kawasaki disease)], and the inclusion criteria stipulated were studies published from January 2019 to June 2020, without restriction of language or location, and available online in full. News, editorials, comments, and letters, as well as duplicates and articles that did not answer the guiding question were excluded. Data synthesis: A total of 97 articles were identified, of which seven comprised this review. The association of KD to the new coronavirus appears to trigger a severe clinical condition of vasculitis. Different from the usual, in this inflammatory syndrome, patients are older, and prevalence is higher in children from African or Caribbean ancestry; clinical and laboratory manifestations are also atypical, with a predominance of abdominal complaints and exaggerated elevation of inflammatory markers. In addition, there was a greater report of rare complications and greater resistance to the recommended treatment for KD. Conclusions: Pediatric COVID-19 and its potential association to severe KD, still unfamiliar to health professionals, reinforces the importance of testing patients with vasculitis for the new coronavirus and the need to wage high surveillance and preparation of the health system during the current pandemic.

RESUMO Objetivo: Analisar a literatura científica atual a fim de documentar, por meio de revisão integrativa, os principais achados que associam a doença de Kawasaki (DK) à doença do coronavírus (COVID-19). Fonte de dados: A busca ocorreu em junho de 2020, nas bases de dados: Biblioteca Virtual em Saúde (BVS), periódico da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) e U.S. National Library of Medicine (PubMed). Os descritores utilizados foram [(COVID-19 OR SARS-CoV-2) AND (Kawasaki Disease)], e os critérios de inclusão estipulados compreenderam estudos publicados de janeiro de 2019 a junho de 2020, sem restrição de idioma ou localização e disponíveis online integralmente. Foram excluídos notícias, editoriais, comentários e cartas de apresentação, assim como duplicatas e artigos que não respondiam à pergunta norteadora. Síntese dos dados: Identificaram-se 97 artigos, dos quais sete compuseram esta revisão. A associação da DK com o novo coronavírus parece desencadear um quadro de vasculite grave. Diferentemente do usual, nessa síndrome inflamatória, os pacientes são mais velhos e a descendência prevalente é africana ou caribenha; as manifestações clínicas e laboratoriais também são atípicas, com predomínio de queixas abdominais e elevação exagerada de marcadores inflamatórios. Além disso, houve maior relato de complicações raras e maior resistência ao tratamento preconizado para DK. Conclusões: A COVID-19 pediátrica e sua potencial associação com uma DK grave, ainda pouco conhecida pelos profissionais da saúde, reforçam a importância da testagem de pacientes com vasculite para o novo coronavírus e a necessidade de empreender alta vigilância e preparação do sistema de saúde durante a atual pandemia.

Interpretation of the JCS/JSCS 2020 guideline on diagnosis and management of cardiovascular sequelae in Kawasaki disease / 中国当代儿科杂志

Kawasaki disease is the main cause of acquired heart disease in children. The cardiovascular sequelae of Kawasaki disease, such as coronary artery lesion and giant coronary aneurysm, have a great impact on children's physical and mental health. The Japanese Circulatory Society and the Japanese Society of Cardiac Surgery jointly released the JCS/JSCS 2020 guideline on diagnosis and management of cardiovascular sequelae in Kawasaki disease in July, 2020, which systematically introduces the advances in the diagnosis and management of cardiovascular sequelae of Kawasaki disease. The article gives an interpretation in the severity evaluation of Kawasaki disease and diagnosis, treatment and long-term management of cardiovascular sequelae in the guideline.

Recomendación para la sospecha, manejo y seguimiento del compromiso cardiovascular en pacientes con Síndrome Inflamatorio Multisistémico en Pediatría asociado a COVID-19 (PIMS-TC). Declaración de Posición de la Sociedad Chilena de Pediatría (SOCHIPE) y Sociedad Chilena de Cardiología y Cirugía Cardiovascular (SOCHICAR) / Recommendation for the recognition, management and follow up of cardiovascular compromise in patients with Pediatric Multisystemic Inflammatory Syndrome associated with COVID-19 (PIMS-CT). Position statement of Chilean Scientific Societies

Desde la aparición de la pandemia por SARS-CoV-2, la población pediátrica ha sido menos afectada por la enfermedad tanto en frecuencia como en severidad. Sin embargo, desde abril de este año se han reportado casos de presentación y gravedad variables, caracterizados por fenómenos inflamato rios que afectan múltiples órganos, condición denominada Síndrome Inflamatorio Multisistémico Pediátrico (PIMS). La literatura describe frecuente compromiso cardíaco, hasta en un 80%. Este se caracteriza por injuria miocárdica con significativa elevación de biomarcadores: Troponinas séricas I/T, BNP o NT-ProBNP, unido a diversos grados de disfunción ventricular, pericarditis, valvulitis y arritmias. Además, se ha evidenciado la presencia de compromiso coronario el cual puede ocurrir hasta en un 23% de los casos, en un rango que va desde dilataciones hasta aneurismas. El seguimien to cardiológico hospitalizado y ambulatorio se ha sistematizado en base a los fenotipos clínicos de presentación: injuria miocárdica (miocarditis, valvulitis, pericarditis), shock (habitualmente de tipo "vasopléjico"), manifestaciones tipo Enfermedad de Kawasaki y aquellos casos PIMS que no cumplen con la clínica de los tres precedentes. Este último grupo es el que representa el mayor desafío en el cor to, mediano y seguimiento a largo plazo. Por esta razón se requiere un equipo multidisciplinario para su manejo. Considerando la alta frecuencia del compromiso cardíaco en el PIMS y la importancia de lograr un consenso en su manejo y seguimiento, se presentan estas recomendaciones según el estado actual del conocimiento de esta patología recientemente descrita.

Since the onset of the SARS-CoV-2 pandemic, the pediatric population has been less affected by the disease both in frequency and severity. However, since April cases of variable presentation and severity characterized by inflammatory phenomena that affect multiple organs have been reported, a condition called Multisystem Inflammatory Syndrome in Children (MIS-C). The literature has reported frequent cardiac involvement, up to 80%. This is characterized by myocardial injury with a significant increase of biomarkers such as serum troponins I and T, BNP, or NT-ProBNP coupled with varying degrees of ventricular dysfunction, pericarditis, valvulitis, and arrhythmias. Coronary compromise has also been described, which can occur in up to 23% of cases, and ranges from dila tations to aneurysms. Inpatient and outpatient cardiology follow-up has been systematized based on the clinical phenotypes such as myocardial injury (myocarditis, valvulitis, pericarditis), shock (usua lly vasoplegic), Kawasaki disease-type manifestations, and those MIS-C that do not comply with the clinic of the previous three. This last group represents the main challenge in the short-, medium- and long-term follow-up, therefore, it is necessary a multidisciplinary team for managing these patients. Considering the high frequency of cardiac compromise in MIS-C, and the importance of reaching a consensus regarding its management and follow-up, we present these recommendations according to the current state of knowledge regarding this recently described pathology.

Actualización en enfermedad de kawasaki: diagnóstico, estudio y tratamiento / Kawasaki disease update: diagnosis, study and treatment

La enfermedad de Kawasaki (EK) es una vasculitis aguda de vaso mediano que afecta principalmente a niños menores de 5 años, que de no ser tratada, se asocia al desarrollo de aneurismas de las arterias coronarias en aproximadamente el 25% de los casos. Típicamente la EK se presenta con fiebre, cambios mucocutáneos y linfadenopatía. Sin embargo, EK es una enfermedad excepcional en la que las formas incompletas de la enfermedad son muy comunes y a menudo asociación sintomatologías atípicas. Éstas pueden crear un desafío diagnóstico para los tratantes y retrasar el inicio de la terapia. No existe un gold standard para el diagnóstico de EK pero hay estudios de laboratorio y hallazgos ecocardiográficos que permiten apoyar el diagnóstico en casos incompletos. El estudio con ecocardiograma debe realizarse lo más pronto posible cuando se sospecha el diagnóstico, pero no debe retrasar el inicio de tratamiento. El objetivo de la terapia en EK es disminuir el riesgo de desarrollar aneurismas de las arterias coronarias y de esta forma, la morbimortalidad asociada a dicha condición. El propósito de esta revisión es conocer las características clínicas y las posibles formas de presentación de esta patología, además del tratamiento actual.

Kawasaki disease (KD) is an acute vasculitis of the medium vessel that mainly affects children under 5 years old, which if it's not treated, is associated with the development of coronary artery aneurysms in approximately 25% of all cases. Typically, KD presents with fever, mucocutaneous changes and lymphadenopathy. However, EK is an exceptional disease in which incomplete forms of the disease are very common and atypical presentations often occur. These presentations may create a diagnostic puzzle for pediatricians and may delay the start of therapy. There is no specific study for the diagnosis of KD but there are laboratory studies and findings in ECG that support the diagnosis in atypical cases. The echocardiogram study should be performed as soon as possible when the diagnosis is suspected, but should not delay the treatment. The goal of treatment in KD is to reduce the risk of developing aneurysms of the coronary arteries and thereby decrease the morbidity and mortality associated with this condition.

Severe clinical spectrum with high mortality in pediatric patients with COVID-19 and multisystem inflammatory syndrome

OBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p<0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p<0.001), vasoactive agent use (83% vs. 3%, p<0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p<0.001), and death (67% vs. 3%, p<0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p<0.001), aspirin therapy (50% vs. 0%, p<0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39-526.79; p<0.0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.

Características clínico-epidemiológicas y predictores de complicaciones coronarias en niños de Argentina con enfermedad de Kawasaki / Clinical- epidemiological characteristics and predictors of coronary complications in children of Argentina with Kawasaki disease

Resumen Introducción: La enfermedad de Kawasaki (EK) es una vasculitis sistémica aguda con riesgo de desarrollar aneurismas coronarios. Objetivos: Describir características clínico-epidemiológicas en niños con diagnóstico de EK en Argentina. Analizar factores de riesgo para el desarrollo de complicaciones coronarias (CC). Población y Métodos: Estudio multicéntrico, retrospectivo, transversal, observacional y analítico. Incluyó pacientes bajo 18 años de edad, con diagnóstico de EK en hospitales de Argentina, entre el 1 de enero de 2010 y el 31 de diciembre de 2013. Resultados: N = 193 sujetos. Edad: mediana: 29 meses. Tasa promedio total país 5 casos/10.000 egresos hospitalarios. Presentaron CC 15,5%. Mayor riesgo de CC: Mayor cantidad de días de fiebre al momento de colocación del tratamiento (p = 0,0033); Aumento de: frecuencia cardíaca (p = 0,0021), eritrosedimentación (VSG) (p = 0,005), proteína C reactiva (PCR) (p < 0,0001), leucocitosis (p = 0,0006), neutrofilia (p = 0,0021); Disminución de hematocrito (p = 0,0007) y hemoglobina (p < 0,0001). Asociación con CC: alteraciones cardiológicas no coronarias (ORv10.818); PCR mayor de 68 mg/L (OR = 11.596); leucocitos mayores a 20.000/mm3 (OR= 4.316); y VSG mayor de 64 mm/1° hora (OR = 4.267). Conclusión: La forma de presentación más frecuente fue EK completa, el riesgo de CC fue mayor en varones, menores de 5 años de edad, los factores de riesgo (clínicos y de laboratorio) fueron semejantes a los descritos en la bibliografía.

Background: Kawasaki disease (EK) is an acute systemic vasculitis with a risk of developing coronary aneurysms. Aim: To describe the clinical and epidemiological characteristics of children with EK in Argentina and to analyse the risk factors for the development of coronary's complications (CC). Methods: Multicenter, retrospective, cross-sectional, observational and analytical study. It included patients younger than 18 years of age diagnosed with EK in hospitals in Argentina, between January the 1st, 2010 and December the 31th, 2013. Results: N = 193 subjects. Age: medium: 29 months. Total incidence 5 cases / 10,000 hospital discharges. CC was observed in 15.5% of patients. Increased risk factors for CC: Elevated number of days with fever at the time of treatment placement (p = 0.0033); Increased of: heart frequency (p = 0.0021), erythrosedimentation (ESR) (p = 0.005), C-reactive protein (CRP) (p < 0.0001), leukocytes (p = 0.0006), neutrophils (p = 0.0021); Decreased of hematocrit (p = 0.0007) and hemoglobin (p < 0.0001).Association with CC: non-coronary cardiological alterations (OR = 10,818); PCR greater than 68 mg /L (OR = 11,596); leukocytes greater than 20,000 / mm3 (OR = 4.316); and ESR greater than 64 mm / 1 hour (OR = 4.267). Conclusion: The most frequent form of presentation was complete EK, the risk of CC was higher in males, younger than 5 years old, the risk factors (clinical and laboratory) were similar to those described in the literature.

Enfermedad de Kawasaki. Revisión de la literatura / Kawasaki Disease: a Review of the Literature

La Enfermedad de Kawasaki (EK) es una vasculitis sistémica febril, con predilección por las arterias de pequeño y mediano calibre, cuya complicación más frecuente es la dilatación aneurismática de las arterias coronarias. Actualmente es la primera causa de cardiopatía adquirida en la infancia en los países desarrollados. El propósito de esta revisión es conocer las características clínicas y las posibles formas de presentación de esta patología, además del tratamiento actual .

Kawasaki disease (KD) is a febrile typeof systemic vasculitis with a predilectionfor small-and medium-sized arteries.The most frequent complication is aneuritic coronary artery dilation. It is currently the most common cause of acquired cardiopathy in childhood in developed countries. The purposeof this review is to high light the clinical...

Revisión de la enfermedad de Kawasaki en México, desde la perspectiva de las publicaciones médicas (enero de 1977 a mayo de 2012) / A review of Kawasaki disease, a perspective from the articles published in Mexico since January 1977 to May 2012

La enfermedad de Kawasaki fue descrita en 1967 por Tomisu Kawasaki. Afecta principalmente a los niños entre uno y 5 años, y se manifiesta con fiebre y vasculitis de vasos medianos que pueden conducir a diversas complicaciones cardiovasculares, tales como aneurismas en las arterias coronarias, miocarditis, daño valvular, derrame pericárdico e infarto del miocardio; eventualmente involucra otros órganos. La etiología aun no es bien conocida, como tampoco lo son los mecanismos patogénicos exactos, pero existen notables avances. Cuando los signos y síntomas clínicos son identificados tempranamente y los niños reciben tratamiento con gammaglobulina y aspirina, la evolución suele ser satisfactoria y pueden evitarse las afecciones coronarias y otras complicaciones. La enfermedad de Kawasaki no se registra frecuentemente en México.

Kawasaki disease was described in 1967 by Tomisu Kawasaki. It affects children aged between one and 5 years, and it evolves with fever and small vessel vasculitis, which leads to cardiovascular complications, including coronary aneurisms, myocarditis, valve injuries, pericardial effusion and myocardial infarction; eventually involving many others organs. The etiology actually is not well known, as the exactly pathogenic mechanisms; however, now there are important advances. If the clinical signs and symptoms are identify early and the children received treatment with aspirin and intravenous immunoglobulin, the patients evolves without sequels. The Kawasaki disease is an infrequent disease in Mexico.

Síndrome de kawasaki: presentación de un caso poco común / Kawasaki syndrome: presentation of an uncommon case

La enfermedad de Kawasaki es un proceso vasculítico multisistémico cuya etiología es poco conocida. La presentación clínica es florida y su evolución está condicionada al inicio temprano de la terapia específica con gammaglobulinas, de allí la importancia de un diagnóstico precoz. Habitualmente estos casos cursan con dilatación del lecho arterial coronario siendo excepcional la aparición de alteraciones a nivel de arterias de mayor calibre, menos común aneurismas a nivel cerebral, que confiere un peor pronóstico para estos pacientes. Presentamos el caso de lactante de 3 meses de edad con aneurisma de ambas arterias coronarias, arteria subclavia izquierda, cerebral media derecha y ambas Iliacas, y obstrucción de las mismas.

Kawasaki disease, a multisystem vasculitis of unknown etiology can present in various ways. It is crucial to make an early diagnosis, and consequently give gamma globulin in order to abort its insidious evolution and not infrequently fatal outcome. Aneurysms and obstruction of the coronary arteries are the most characteristic presentations. Other large vessels may be involved, with the cerebral circulation being affected least often but having the worst prognosis. We present a clinical case of a 3 month old breast fed infant with aneurysms and obstructions in both coronary arteries, the left subclavian, right middle cerebral, and both iliac arteries.

[ case report: an atypical Kawasaki syndrome following aseptic meningitis in a 9-month old infant]

Kawasaki syndrome is an acute, sometimes fatal vasculitis of young children. The ethiology of the illness is unknown, but its clinical and epidemiologic features are most consistent with an infectious cause. We treated a 9-month-old infant with diagnosis of bacterial meningitis. He received wide spectrum antibiotics. He was clinically improved but in 8th day of admission he again developed pharyngitis, diarrhea and high grade fever. He has had raising of erythrocyte sedimentations rate [ESR] and thrombocytosis. According to these clinical manifestation with impression of atypical Kawasaki disease, treatment was started for him and after 24 hours his fever subsided and other clinical signs relieved. On the basis of this case report in the course of bacterial meningitis if the patient developed elevated ESR and fever, Kawasaki syndrome with the other complications of meningitis should be considered

Giant coronary artery aneurysms in a young infant with Kawasaki, disease

We report the case of a young infant with prolonged fever and delayed diagnosis of Kawasaki disease which was complicated with development of multiple large coronary aneurysms

Kawasaki disease: a retrospective study

The aim of this study was to appraise/review the criteria of diagnosis of Kawasaki Disease [KD], cardiac status and the management. A cross-sectional, retrospective and descriptive study. Pediatric Department, Salmaniya Medical Complex. A retrospective review of children with diagnosis of KD between June 1992 and August 2002. Medical records of 34 children were reviewed. The median age at diagnosis was 31 +/- 22.2 months. Male to Female ratio was 1.8:1. Thirty children were Bahraini while the rest were: two Indians, one Pakistani and one Filipino. All patients fulfilled the criteria for diagnosis of KD. Heart complications were recorded in sixteen patients [47%]. One child presented with myocarditis but fifteen patients showed coronary artery dilatation [CAD]. Children with CAD received aspirin therapy. The majority of the patients [33/34] received IVIG; seventeen patients [51%] had intravenous immune globulin [IVIG] as a single infusion. All children fulfilled the criteria for diagnosing KD. Fifteen patients [44%] presented with CAD but none of them had aneurysm. Aspirin and IVIG were the main treatment. The majority of the patients with KD had resolution of CAD in less than six months, no patient had a relapse or developed heart complications in the follow up period

Assessment of IVIG treatment in children and its complications [Babol; 1999-2004]

IVIG is used in patients incapable of producing antibodies and in autoimmune disorders. IVIG infusion rarely causes undesirable reactions due to the speed of infusion. So, this study was performed to determine the indications and complications of IVIG infusion. This descriptive study was performed on 265 patients, hospitalized in Amirkola pediatric hospital, needing IVIG infusions, from October 1999 to June 2004. Data was analyzed by SPSS. T-test, chi square and fisher exact test were used to compare drug complications based on age and speed of infusion in two genders. In this research, 265 patients with 871 infusions were studied. One hundred and thirty one patients [49.4%] were male and 134 [50.6%] were female. Two patients [0.7%] had autoimmune hemolytic anemia, one patient [0.3%] had ataxia telangectasia, 9 patients [3.4%] had refractory seizure, 2 patients [0.7%] had guillain-barre syndrome, 83 patients [31.3%] had immune thrombocytopenic purpura, 46 patients [17.3%] had kawasaki, 51 patients [19.2%] had immunodeficiency and 72 patients [27.1%] were premature neonates. Among the studied patients, 17 infusions [1.95%] had complications, which were mild in 14 patients [82.5%], moderate in 2 patients [11.7%] and severe in 1 patient [5.8%]. Complication was the same in both genders, and it had a significant difference with the speed of drug infusion [P<0.05]. The most common cause of IVIG infusion is immune thrombocytopenic purpura. Complications are usually mild and had relationship with the speed of drug infusion

Enfermedad de Kawasaki en un lactante de dos meses / Kawasaki disease in a two months old infant

La enfermedad de Kawasaki (EK) es un síndrome vasculítico multisistémico febril agudo, autolimitado, de etiología incierta. Se manifiesta frecuentemente en niños menores de cinco años de edad, siendo poco común en menores de tres meses. El diagnóstico se establece a partir de un cuadro clínico compatible, asociado a alteraciones sugestivas en los exámenes complementarios. El tratamiento consiste en la administración de gammaglobulina hiperinmune y ácido acetilsalicílico dentro de los primeros diez días del inicio de la sintomatología. Se presenta una paciente de dos meses de edad que cumple todos los criterios diagnósticos de la enfermedad. El tratamiento se realiza en forma precoz, siendo su evolución favorable, sin secuelas cardiovasculares hasta el momento actual. Si bien esta enfermedad es poco frecuente, se destaca la importancia de pensar en ella, aún en lactantes pequeños, dado que la instauración temprana del tratamiento puede disminuir la incidencia de aneurismas coronarios.

Enfermedad Kawasaki / Kawasaki disease

La enfermedad de Kawasaki es una enfermedad febril aguda de la infancia (1) de etiología desconocida. La hipótesis más aceptada es que ésta puede producirse por una respuesta inmunológica exagerada o aberrante contra un agente infeccioso o antígeno que aún no se ha podido identificar. (1,9) No existe un examen de laboratorio concreto que permita plantear el diagnóstico por lo que el mismo debe realizarse basado en la clínica que presenta el paciente. (1,9,18) Esta enfermedad fue descrita por primera vez en Japón por el Dr. Tomisaku Kawasaki con el nombre del Síndrome febril linfonodular-mococutáneo. (9,20) En estas primeras descripciones él define los signos clínicos que constituyen la base del diagnóstico. Sin embargo, con los años venideros se pone de relieve su gravedad y se reconoce el aumento de la frecuencia de los casos atípicos o incompletos de este síndrome que deben estudiarse con el fin de instaurar un tratamiento precoz para tratar de evitar principalmente las complicaciones cardiovasculares que puedan causar la muerte del paciente. (18,19) Desafortunadamente, el diagnóstico se basa en signos y síntomas que también pueden ocurrir en otras enfermedades por lo que el diagnóstico puede ser particularmente difícil en casos atípicos. (5,7,11,12,16,21,22) Dicha enfermedad afecta principalmente a niños menores de 9 años con una mayor prevalencia entre los 18 y 24 meses de edad. El 50 por ciento de los casos se han reportado en menores de 2 años y el 80 por ciento en menores de 4 a 5 años. (9) Es más frecuente en el sexo masculino con una proporción hombre / mujer de 1.5:1. (9,13) A continuación se describe un caso de un niño de 5 años en donde queda claramente explícita la gama de diagnósticos por las que puede pasar un niño en esta enfermedad tanto por baja frecuencia de estos casos como por la evolución natural de la enfermedad.