ABSTRACT
La enfermedad de Kawasaki (EK) es la principal causa de cardiopatía adquirida en menores de cinco años. Nuestro objetivo fue conocer las características clínicas, el compromiso coronario y la evolución de pacientes atendidos en nuestra institución. Se revisó una serie de casos desde 2001 hasta 2018. Se incluyeron 63 pacientes, 58 % varones; la mediana de edad fue 2,6 años. La mediana de días de fiebre al diagnóstico fue 5,5 días. El 33 % presentó la forma incompleta y se detectó compromiso coronario en el 20 %. El 60 % de los pacientes con afectación coronaria presentaron EK incompleta versus el 28 % de presentación incompleta en los pacientes sin compromiso coronario (p 0,06). No se observaron diferencias en datos de laboratorio entre los grupos según el compromiso coronario. En conclusión, 33 % presentó EK incompleta y el 20 %, afectación coronaria. Hubo una tendencia de mayor riesgo para daño coronario en la forma incompleta.
Kawasaki disease (KD) is considered the leading cause of acquired heart disease in children younger than 5 years. Our objective was to know the clinical characteristics, coronary involvement, and course of patients seen at our facility. A case series from 2001 to 2018 was reviewed. Sixty-three patients were included; their median age was 2.6 years; 58% were males. The median duration of fever at the time of diagnosis was 5.5 days. The incomplete form was observed in 33% and coronary involvement, in 20%. Among patients with coronary involvement, 60% had incomplete KD versus 28% among those without coronary involvement (p: 0.06). No differences were observed between groups in laboratory data based on coronary involvement. To conclude, 33% had incomplete KD and 20%, coronary involvement. There was a trend to a higher risk for coronary artery damage in the incomplete form of KD.
Subject(s)
Humans , Child, Preschool , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/epidemiology , Retrospective Studies , Immunoglobulins, Intravenous , Fever , Hospitals, GeneralABSTRACT
Introducción. El tamaño del recién nacido se asocia a condiciones intrauterinas. El potencial genético se expresa más tarde; la canalización del crecimiento se describe clásicamente hasta los 24 meses. Objetivo. Describir la canalización del crecimiento entre los 2 y los 5 años en niños aparentemente sanos con talla baja a los 2 años. Población y métodos. Estudio de cohorte retrospectiva. Se incluyeron niños seguidos en un hospital universitario de comunidad entre 2003 y 2019, con puntaje Z de talla menor a -2 DE para edad y sexo a los 2 años. Se excluyeron los nacidos prematuros, con bajo peso y con enfermedades crónicas. Se evaluó la trayectoria de crecimiento. Se definió canalización como la adquisición de talla normal para la población general. Resultados. Se incluyeron 64 niños, de los cuales 37 (58 %) presentaron canalización del crecimiento a los 5 años (20 a los 3 años, 8 a los 4 años, y 9 a los 5 años). La velocidad de crecimiento a los 3 y a los 5 años fue significativamente mayor en los que canalizaron en comparación con los que no lo hicieron; hubo una tendencia similar a los 4 años. De los 27 niños con talla baja a los 5 años, 25 tuvieron al menos un registro de velocidad de crecimiento anual menor al percentil 25. Conclusiones. La mayoría de los niños aparentemente sanos con baja talla a los 2 años alcanzan una talla normal a los 5 años. La velocidad de crecimiento anual permite detectar a los niños con riesgo de no canalizar.
Introduction. Newborn size is associated with intrauterine conditions. Genetic potential is expressed later; the canalization of growth is typically described up to 24 months of age. Objective. To describe the canalization of growth between 2 and 5 years of age in apparently healthy children with short stature at age 2 years. Population and methods. Retrospective, cohort study. Children seen at a community teaching hospital between 2003 and 2019, who had a Z-score for height below -2 SDs for age and sex at age 2 years were included. Infants born preterm, with a low birth weight, and chronic conditions were excluded. Growth patterns were assessed. Canalization was defined as reaching a normal stature for the general population. Results. Sixty-four children were included; 37 (58%) showed canalization of growth at 5 years old (20 at 3 years, 8 at 4 years, and 9 at 5 years). The growth rate at 3 and 5 years of age was significantly higher among those who showed canalization compared to those who did not; a similar trend was observed at 4 years of age. Among 27 children with short stature at 5 years of age, 25 had at least 1 annual growth velocity below the 25th centile. Conclusions. Most apparently healthy children with short stature at 2 years old reached a normal stature at 5 years old. The annual growth velocity allows to detect children at risk of not showing canalization.
Subject(s)
Humans , Child, Preschool , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/epidemiology , Immunoglobulins, Intravenous , Fever , Hospitals, GeneralABSTRACT
Abstract The coronavirus disease (COVID-19), that assumed pandemic proportions in March 2020, mainly affects the respiratory tract, causing severe interstitial pneumonia in adults. Worldwide data indicate that COVID-19 tends to be more benign in children, which is evidenced by a high incidence of asymptomatic or mild upper airways' infection cases in this population. However, recent studies have been associating Kawasaki-like symptoms as a nonclassical presentation of coronavirus disease in pediatrics. It is suggested that the intense cytokine cascade, promoted by the SARS-CoV-2 infection, can trigger a multisystem inflammatory response as an atypical Kawasaki form in genetically predisposed individuals. In this context, patients may develop more severe clinical features with a greater predisposition to myocardial involvement, Macrophage Activation Syndrome, and Kawasaki Disease Shock Syndrome. Despite critical conditions, patients usually respond to conventional treatment of Kawasaki Disease with intravenous immunoglobulin. This article intends to provide an approach to the association between Kawasaki-Like Syndrome and COVID-19.
Subject(s)
Humans , Male , Female , Child , COVID-19/complications , Mucocutaneous Lymph Node Syndrome/complications , COVID-19/diagnosis , COVID-19/drug therapy , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/drug therapyABSTRACT
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.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Systemic Inflammatory Response Syndrome/therapy , COVID-19/therapy , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Aspirin/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Systemic Inflammatory Response Syndrome/complications , Systemic Inflammatory Response Syndrome/diagnosis , Critical Care , Diagnosis, Differential , COVID-19/complications , COVID-19/diagnosis , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapyABSTRACT
Abstract A 32-year-old man diagnosed with Kawasaki disease at the age of three years presented with coronary artery aneurysm (CAA). The aneurysm increased in size, and the patient was referred to our hospital for surgery. Preoperative computed tomography scan showed a super-giant right CAA and giant left CAAs; surgery was performed. The super-giant right CAA was resected, and the ostium of the right coronary artery was closed; then, coronary artery bypass grafting was performed. The left CAAs were not treated surgically because the risk of rupture was low. Here, we describe the successful surgical treatment of a right super-giant CAA.
Subject(s)
Humans , Male , Child, Preschool , Adult , Coronary Aneurysm/surgery , Coronary Aneurysm/etiology , Coronary Aneurysm/diagnostic imaging , Mucocutaneous Lymph Node Syndrome/complications , Tomography, X-Ray Computed , Coronary Artery Bypass , Coronary Vessels/surgery , Coronary Vessels/diagnostic imagingABSTRACT
Kawasaki disease (KD) can be a primary cause of an acute coronary syndrome in young adults, with or without a history of KD during childhood. We report a 31year old female, admitted for acute chest pain. She had negative T waves on DIII and AVF leads of the electrocardiogram. Troponins were positive. A cardiac magnetic resonance was compatible with an acute inferior myocardial infarction. In the coronary angiography she had a calcified complete occlusion of the proximal right coronary artery. The left coronary artery had no visible lesions. A computed angiogram showed an extensive calcified and thrombotic aneurysm of the right coronary artery, compatible with Kawasaki disease (KD). The patient was discharged seven days after admission in good conditions.
Subject(s)
Humans , Female , Adult , Young Adult , Coronary Aneurysm/etiology , Coronary Aneurysm/diagnostic imaging , Acute Coronary Syndrome , Mucocutaneous Lymph Node Syndrome/complications , Coronary Angiography , ElectrocardiographyABSTRACT
RESUMO Apresentação de um caso de infecção por Influenza B e doença de Kawasaki em adolescente ocorrido durante a pandemia da COVID-19. Adolescente asmática evoluiu com febre e síndrome gripal por 7 dias e deu entrada com quadro de insuficiência respiratória aguda, necessitando de intubação orotraqueal. Evoluiu também com instabilidade hemodinâmica respondedora ao uso de droga vasoativa. Foram introduzidas antibioticoterapia e medidas de suporte. Apresentou melhora hemodinâmica e respiratória progressiva, porém mantinha febre e alteração de provas inflamatórias. Durante internação, evoluiu com conjuntivite não purulenta bilateral, descamação de mão e pés, língua em framboesa e linfonodomegalia cervical, recebendo diagnóstico de doença de Kawasaki. Recebeu gamaglobulina e, por conta de quadro clínico refratário, foi administrado também corticoide, evoluindo afebril 24 horas após. Não apresentou alterações coronarianas. O único agente isolado foi Influenza B, mesmo realizando painel viral e investigação para COVID-19 com reação em cadeia da polimerase e sorologia. Durante internação, apresentou tromboembolismo pulmonar, e, em investigação de coagulopatias, foi diagnosticada com mutação em heterozigose de fator V de Leiden. Há uma potencial associação entre doença de Kawasaki e infecção por Influenza B ou outros vírus, como o coronavírus e, por isso, esses diagnósticos devem ser investigados nos pacientes pediátricos, incluindo adolescentes, com quadros febris prolongados.
ABSTRACT We report a case of Influenza B infection and Kawasaki disease in an adolescent, diagnosed during the COVID-19 pandemic. An asthmatic female adolescent presented with fever and flu-like symptoms for 7 days and was admitted with acute respiratory failure requiring mechanical ventilation. She progressed with hemodynamic instability responsive to vasoactive drugs. Antibiotic therapy and support measures were introduced, showing progressive hemodynamics and respiratory improvement, however with persistent fever and increased inflammatory markers. During the hospitalization, she developed bilateral non-purulent conjunctivitis, hand and feet desquamation, strawberry tongue, and cervical adenopathy, and was diagnosed with Kawasaki disease. She was prescribed intravenous immunoglobulin and, due to the refractory clinical conditions, corticosteroid therapy was added; 24 hours later, the patient was afebrile. No coronary changes were found. A full viral panel including COVID-19 C-reactive protein and serology could only isolate the Influenza B virus. During the hospitalization, she was diagnosed with pulmonary thromboembolism; coagulopathies were investigated, and she was diagnosed with heterozygous factor V Leiden mutation. There is a potential association between Kawasaki disease and infection with Influenza B or with other viruses such as coronavirus. Therefore, this association should be considered in pediatric patients, adolescents included, with prolonged febrile conditions.
Subject(s)
Humans , Female , Adolescent , Respiratory Insufficiency/diagnosis , Influenza, Human/diagnosis , COVID-19 , Mucocutaneous Lymph Node Syndrome/diagnosis , Influenza B virus/isolation & purification , Respiration, Artificial , Respiratory Insufficiency/etiology , Influenza, Human/therapy , Hospitalization , Mucocutaneous Lymph Node Syndrome/complicationsABSTRACT
Introducción. La enfermedad de Kawasaki (EK) es una vasculitis sistémica inespecífica que suele presentarse en los niños; la lesión de las arterias coronarias (LAC) es la complicación más grave.Objetivos. Nuestro objetivo fue investigar los factores de riesgo de LAC en niños con EK.Materiales y métodos. Se incluyó a niños con EK según los criterios diagnósticos, hospitalizados entre enero de 2014 y diciembre de 2017. Se realizaron análisis univariado y multivariado de regresión logística para investigar las relaciones entre LAC y género, edad, diagnóstico clínico, velocidad de sedimentación globular (VSG), recuento de trombocitos, concentración de hemoglobina, concentración de proteína C-reactiva, recuento de leucocitos, momento de inicio de la administración de inmunoglobulina intravenosa (IgIV) y duración de la fiebre.Resultados. Se dividió a los 982 niños con EK en un grupo con LAC (n = 104) y otro sin LAC (n = 878), según una ecocardiografía Doppler color. La tasa de incidencia de LAC fue del 10,6 % (104/982). En el análisis univariado, se observó una diferencia significativa entre ambos grupos en cuanto al género, la VSG, el recuento de trombocitos, el momento de inicio de la administración de IgIV y la duración de la fiebre (p < 0,05). Según el análisis multivariado de regresión logística, el sexo masculino, una VSG elevada y la administración tardía de IgIV fueron factores de riesgo independientes de EK complicada con LAC.Conclusiones. El sexo masculino, una VSG elevada y la administración tardía de IgIV fueron factores de riesgo independientes de EK complicada con LAC.
Introduction. Kawasaki disease (KD) is a non-specific systemic vasculitic disease that frequently occurs among children, and coronary artery lesion (CAL) is the most serious complication.Objectives. We aimed to study the risk factors for CAL in children with KD.Materials and methods. KD children in accordance with diagnostic criteria, who were hospitalized from January 2014 to December 2017, were selected as subjects. Univariate and multivariate logistic regression analyses were conducted to explore the relationships between CAL and gender, age, clinical diagnosis, erythrocyte sedimentation rate (ESR), platelet count, hemoglobin level, C reactive protein level, white blood cell count, initiation time of IVIG administration and duration of fever.Results. The enrolled 982 KD children were divided into a CAL group (n = 104) and an NCAL group (n = 878) according to cardiac color Doppler ultrasonography. The incidence rate of CAL was 10.6 % (104/982). Univariate analysis showed that the two groups had significantly different gender, ESR, platelet count, initiation time of IVIG administration and duration of fever (P < 0.05). Multivariate logistic regression analysis revealed that male gender, elevated ESR and delayed use of IVIG were independent risk factors for KD complicated with CAL.Conclusions:Male gender, increased ESR and delayed use of IVIG were independent risk factors for KD complicated with CA
Subject(s)
Humans , Male , Female , Child , Coronary Artery Disease/epidemiology , Mucocutaneous Lymph Node Syndrome/complications , Logistic Models , Risk Factors , Coronary Vessels/injuries , Mucocutaneous Lymph Node Syndrome/diagnosisABSTRACT
SUMMARY OBJECTIVE To present scientific evidence based on a systematic literature review (PRISMA) evaluating the association of Kawasaki Disease (DK) and COVID-19 in children. METHODS For the selection of studies, a combination based on the Medical Subject Heading Terms (MeSH) was used. The Medline (Pubmed), LILACS, SciELO, COCHRANE, and BIREME databases were used. The search period for the articles comprised the last 10 years (2010 to 2020). RESULTS 840 articles with potential for inclusion were retrieved, one of which met the inclusion criteria and the guiding question that consisted of evaluating the association of Kawasaki disease and COVID-19 in children. CONCLUSION A significant increase in the incidence of Kawasaki-type diseases after the onset of the epidemic has been reported, suggesting an association between the COVID-19 epidemic and the high incidence of a severe form of KD. However, further studies are needed to conduct an investigation of the association between these two diseases.
RESUMO OBJETIVO Apresentar evidências científicas com base em revisão sistemática da literatura (Prisma) avaliando a associação da Doença de Kawasaki (DK) e COVID-19 em crianças. MÉTODOS Para a seleção dos estudos foi utilizada a combinação baseada no Medical Subject Heading Terms (MeSH). Foram utilizadas as bases de dados Medline (PubMed), Lilacs, SciELO, Cochrane e Bireme.O período de busca dos artigos compreendeu os últimos dez anos (2010 a 2020). RESULTADOS Foram recuperados 840 artigos com potencial de inclusão, sendo que um respondeu aos critérios de inclusão e à pergunta norteadora que consistiu em avaliar a associação da Doença de Kawasaki e COVID-19 em crianças. CONCLUSÃO Um aumento significativo na incidência de doenças do tipo Kawasaki após o início da epidemia já foi relatado, sugerindo a associação entre a epidemia de COVID-19 e a elevada incidência de uma forma grave da DK. Contudo, mais estudos são necessários para conduzir a investigação da associação entre essas duas doenças.
Subject(s)
Humans , Child , Pneumonia, Viral/complications , Coronavirus Infections/complications , Systemic Inflammatory Response Syndrome/virology , Betacoronavirus/isolation & purification , Mucocutaneous Lymph Node Syndrome/virology , Pneumonia, Viral/immunology , Pneumonia, Viral/epidemiology , Coronavirus Infections , Coronavirus Infections/immunology , Coronavirus Infections/epidemiology , Pandemics , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/epidemiologySubject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Coronavirus Infections , Mucocutaneous Lymph Node Syndrome/complicationsSubject(s)
Humans , Male , Child , Delayed Diagnosis , Mucocutaneous Lymph Node Syndrome/complications , Echocardiography/methods , Aspirin , Conjunctivitis , Coronary Vessels , Exanthema , AneurysmABSTRACT
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.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Coronary Disease/etiology , Coronary Disease/epidemiology , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/epidemiology , Argentina/epidemiology , Seasons , Blood Sedimentation , C-Reactive Protein/analysis , Incidence , Cross-Sectional Studies , Retrospective Studies , Risk Factors , Immunoglobulins, Intravenous/therapeutic use , Statistics, Nonparametric , Risk Assessment , Mucocutaneous Lymph Node Syndrome/therapyABSTRACT
Abstract We report a case of a 23-year-old man who was diagnosed with Kawasaki disease that progressed to a coronary aneurysm in the left main coronary artery (LMA). He had suffered from acute coronary syndrome and then underwent an emergent percutaneous coronary angioplasty, in which a polyurethane-covered stent was placed inside the aneurysm. The stent was thrombosed one year later, despite the patient had been treated with anticoagulant and antiplatelet therapy. Emergency percutaneous intervention was then performed. LMA was reopened and stent malposition was observed. Therefore, urgent coronary bypass grafting was performed in which a high degree of competitive flow was observed through the reopened stent. LMA was ligated at the inflow of the aneurysm, resulting in an improvement of graft flow. Left main ligature has not been previously reported.
Subject(s)
Humans , Male , Young Adult , Coronary Aneurysm/surgery , Coronary Aneurysm/etiology , Coronary Artery Bypass/methods , Mucocutaneous Lymph Node Syndrome/surgery , Mucocutaneous Lymph Node Syndrome/complications , Coronary Aneurysm/diagnostic imaging , Treatment Outcome , Acute Coronary Syndrome/surgery , Computed Tomography Angiography/methods , Ligation , Mammary Arteries/surgeryABSTRACT
Resumen: Introducción: La enfermedad de Kawasaki (EK) es una vasculitis sistémica aguda de pequeños y medianos vasos, que se presenta principalmente en niños. Se manifiesta como síndrome febril aunado a datos de vasculitis, y puede causar anormalidades en las arterias coronarias en el 25% de los pacientes no tratados. El objetivo de este estudio fue describir el comportamiento clínico y la identificación de factores de riesgo para complicaciones cardiovasculares en pacientes pediátricos con EK atendidos en un hospital de segundo nivel del noroeste de México. Métodos: Bajo un diseño de serie de casos, se estudiaron pacientes pediátricos con diagnóstico de EK. Se midieron variables clínicas, de laboratorio y presencia de complicaciones cardiacas, y se estimó la probabilidad de riesgo con razón de momios (RM) y asociación con prueba de ji al cuadrado. Resultados: Se incluyeron 12 pacientes y predominó el sexo femenino; la edad media de presentación de la EK fue de 2 años. La presentación clínica fue completa en el 100% de los casos y hubo además manifestaciones atípicas. El 50% de los pacientes estudiados presentaron complicaciones cardiovasculares; la más común fue afección coronaria (33%). Las variables sexo masculino, edad menor de 2 años y anemia tuvieron RM de 5.5 y 10 de presentar complicaciones cardiovasculares. Conclusiones: Las complicaciones cardiovasculares de la EK son frecuentes (más del 30% de los pacientes). Las variables sexo masculino, edad menor de 2 años y anemia incrementaron la probabilidad de riesgo para la presencia de complicaciones cardiovasculares.
Abstract: Background: Kawasaki disease (KD) is an acute systemic vasculitis of small and medium vessels, which occurs primarily in children; it manifests itself as a febrile syndrome coupled with vasculitis data and can cause coronary artery abnormalities in 25% of untreated patients. The objective of this study was to describe the clinical behavior and to identify risk factors for cardiovascular complications in pediatric patients with KD, in a second level hospital in Northwestern Mexico. Methods: Under a case series design, we studied pediatric patients with diagnosis of KD. We measured clinical variables, laboratory values and the presence of cardiac complications; the probability of risk was determined with odds ratio (OR) and the association with chi squared test. Results: 12 patients were included, and the female gender predominated; the mean age of presentation of KD was 2 years. The clinical presentation was complete in 100 % of the cases and patients also presented atypical manifestations. 50% of the patients studied had cardiovascular complications, the most common of which was coronary disease (33%). The variables male gender, age under 2 years and anemia reported OR of 5.5 and 10 to present cardiovascular complications. Conclusions: Cardiovascular complications of KD are frequent (more than 30%). Male gender, age under 2 years and anemia increase the probability of risk for the presence of cardiovascular complications.
Subject(s)
Child, Preschool , Female , Humans , Infant , Male , Cardiovascular Diseases/etiology , Coronary Disease/etiology , Mucocutaneous Lymph Node Syndrome/complications , Cardiovascular Diseases/epidemiology , Sex Factors , Risk Factors , Age Factors , Coronary Disease/epidemiology , Coronary Vessel Anomalies/etiology , Anemia/complications , MexicoABSTRACT
La enfermedad de Kawasaki (EK) es una vasculitis autoinmunitaria sistémica que afecta a los vasos pequeños y medianos. La complicación principal de la enfermedad de Kawasaki es el aneurisma de las arterias coronarias, cuyo riesgo es más alto si se retrasa el diagnóstico y el tratamiento. Si bien hasta la fecha se han presentado casos de EK completa e incompleta en diferentes tipos de enfermedades por inmunodeficiencia, no se ha informado acerca de la evolución clínica de la EK en pacientes con hipogammaglobulinemia (HG). En este artículo, se presenta un caso de diagnóstico de EK incompleta en un niño con HG transitoria de la infancia. También se resumen casos previamente informados de EK e inmunodeficiencia. En el caso de una inmunodeficiencia, las infecciones recurrentes pueden ocultar la EK, lo que retrasa el diagnóstico y aumenta el riesgo de complicaciones. En pacientes inmunodeficientes, debe tenerse en cuenta la posibilidad de EK cuando la fiebre es prolongada.
Kawasaki Disease (KD) is a systemic autoimmune vasculitis that affects small and medium sized vessels. Main complication of Kawasaki Disease is coronary artery aneurism, which has higher risk in case of delayed diagnosis and treatment. Although, complete and incomplete KD cases in different types of immune deficiency diseases have been presented up to date, clinical course of KD in patients with hypogammaglobulinemia (HG) has not been reported. Herein, a case diagnosed as incomplete KD in a child with transient HG of infancy has been reported. Previously reported cases with KD and immunedeficiency have also been summarized. Recurrent infections in case of immunedeficiency may mask KD disease resulting in delay in diagnosis and increased risk of complication. KD should be kept in mind in immunedeficient patients in case of prolonged fever.
Subject(s)
Humans , Male , Child, Preschool , Agammaglobulinemia/complications , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Diagnosis, Differential , Delayed DiagnosisSubject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Pregnancy Complications, Cardiovascular/diagnostic imaging , Coronary Aneurysm/diagnostic imaging , Mucocutaneous Lymph Node Syndrome/complications , Pregnancy Complications, Cardiovascular/etiology , Severity of Illness Index , Coronary Aneurysm/etiology , Pregnancy Outcome , Echocardiography , Computed Tomography AngiographyABSTRACT
Resumen Objetivo: Describir las manifestaciones cardiacas en la etapa aguda de la enfermedad de Kawasaki en pacientes atendidos en un hospital de tercer nivel de la Ciudad de México, México. Métodos: Estudio retrospectivo, descriptivo en pacientes con diagnóstico de enfermedad de Kawasaki de agosto de 1995 a diciembre del 2016 en el Instituto Nacional de Pediatría, México. Se estudio la demografía de los pacientes, características clínicas, tratamiento empleado y desarrollo de complicaciones cardiacas en la etapa aguda de la enfermedad. Resultados: Se estudiaron 508 casos de enfermedad de Kawasaki. La edad media al diagnóstico fue de 37.64 ± 35.56 meses. Predominio de pacientes masculinos del 65.4%, con una relación masculino/femenino de 1.88:1. La mayoría de los casos (79.2%) tuvo una presentación completa. La gammaglobulina intravenosa fue administrada en 92.4% de los casos. Veintiocho pacientes (5.5%) desarrollaron arritmias, se presentaron cambios en el segmento ST en 29 pacientes (5.6%) y 5 pacientes desarrollaron isquemia miocárdica. En el ecocardiograma inicial, 51 pacientes (9.9%) presentaron datos de miocarditis, 72 pacien- tes (14%) datos de pericarditis y 77 casos tuvieron derrame pericárdico (15%). Se detectaron alteraciones en las arterias coronarias en 169 casos (32.9%). Cuatro pacientes fallecieron en la etapa aguda de la enfermedad por complicaciones cardiacas de la enfermedad de Kawasaki. Conclusiones: En México cada vez existen más casos de enfermedad de Kawasaki con un alto porcentaje de manifestaciones cardiacas al diagnóstico. Se requiere de un mayor conocimiento de la enfermedad en México, para poder establecer cuál es la evolución cardiológica de los pacientes en el país.
Abstract Objectives: To describe the cardiac manifestations in the acute phase of patients with Kawasaki disease treated in a third level Children's hospital in Mexico City, Mexico. Methods: A cross-sectional study was conducted in patients with a diagnosis of Kawasaki disease treated in this hospital from August 1995 to December 2016. Information included patient demographics, clinical features, treatment used, electrocardiographic findings, extra-coronary echocardiographic findings, and the development of coronary artery aneurysms in the acute phase of the disease. Results: The study included 508 cases of Kawasaki disease, with a mean age at diagnosis of 37.64 ± 35.56 months (range from 2 to 200 months). Almost two-thirds (65.4%) of the patients were male, with a male/female ratio of 1.88:1. Complete Kawasaki disease was diagnosed in 79.2% of cases. Almost all cases (92.4%) received intravenous immunoglobulin. Twenty-eight patients (5.5%) developed arrhythmias, ST changes developed in 29 patients (5.6%), and 5 patients presented with ischaemic changes. In the initial echocardiographic evaluation, 51 patients (9.9%) were diagnosed with myocar- ditis, 72 patients (14.0%) with pericarditis and 77 cases (15.0%) developed pericardial effusion. Coronary artery anomalies were detected in 169 cases (32.9%). 32 cases were diagnosed as giant coronary aneurysms. Four patients died from cardiac complications in the acute phase of the disease. Conclusions: There has been an increase in the diagnosis of Kawasaki disease in Mexico. They presented with more cardiac complications than reported in literature. An increased knowledge of Kawasaki disease is required in Mexico in order to establish the cardiac outcomes of this group of patients.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Coronary Aneurysm/etiology , Coronary Artery Disease/etiology , Heart Diseases/etiology , Mucocutaneous Lymph Node Syndrome/complications , Coronary Aneurysm/epidemiology , Coronary Artery Disease/epidemiology , Echocardiography , Cross-Sectional Studies , Retrospective Studies , Immunoglobulins, Intravenous/administration & dosage , Heart Diseases/epidemiology , Hospitals, Pediatric , Mexico/epidemiology , Mucocutaneous Lymph Node Syndrome/drug therapySubject(s)
Humans , Male , Middle Aged , Coronary Aneurysm/etiology , Mucocutaneous Lymph Node Syndrome/complications , Severity of Illness Index , Coronary Aneurysm/surgery , Coronary Aneurysm/diagnosis , Diagnosis, Differential , Asymptomatic Diseases , Mucocutaneous Lymph Node Syndrome/surgery , Mucocutaneous Lymph Node Syndrome/diagnosisABSTRACT
Resumen Objetivo La enfermedad de Kawasaki (EK) es una vasculitis sistémica cuya complicación más grave es la formación de lesiones coronarias, las cuales pueden llevar a infarto del miocardio y muerte súbita. El estudio ecocardiográfico es parte del seguimiento obligado de los pacientes con EK. La deformación sistólica longitudinal (DSL) medida mediante speckle tracking es una herramienta precisa para evaluar la función de la fibra miocárdica (longitudinal) del ventrículo izquierdo. No se ha establecido la utilidad en la práctica clínica de la DSL en niños con antecedente de EK. El objetivo de este estudio fue analizar si la presencia de lesiones coronarias condiciona alteraciones en la DSL segmentaria y su correspondencia con el territorio coronario en donde se encuentra la lesión. Método Serie de casos. Se realizó un estudio ecocardiográfico completo y la evaluación de la DSL a niños con antecedente de EK al menos 6 meses después de la fase aguda. Resultados Se estudiaron 9 pacientes. La mediana de edad fue de 6 años (mínimo 2 y máximo 17). El 56% era de sexo masculino. El 77% presentó aneurismas coronarios. La DSL resultó alterada en el 56% de la muestra estudiada. Dentro de los pacientes que presentaron una DSL anormal, todos mostraron aneurismas coronarios y lesiones estenóticas u oclusivas demostradas mediante cateterismo de arterias coronarias, además de alteraciones de la perfusión miocárdica en estudio de Medicina Nuclear. Conclusiones En la muestra estudiada, los pacientes en quienes se encontró una DSL anormal, resultaron tener lesiones coronarias que condicionaban isquemia o infarto.
Abstract Objective Kawasaki disease (KD) is a systemic vasculitis that affects young children. Coronary artery aneurisms, ectasia and stenosis are its main complications and may lead to ischemic heart disease or sudden death. Echocardiography evaluation it's mandatory in all patients with history of KD. Left ventricular longitudinal systolic strain (LVLSS) measured by speckle tracking it's an accurate tool to evaluate global and segmental left ventricle mechanics. Clinical utility of LVLSS in children with KD hasn't been established. The goal of this study was to analyse if the presence of coronary lesions alters segmental LVLSS and if there is a relationship with the affected coronary territory. Method Case series. A complete transthoracic echocardiography with LVLSS was performed in children with history of KD with at least 6 months after the acute phase. Results Nine patients where studied, with a median age of 6 years (minimum 2 and maximum 17). A percentage of 56 were male, and 77% had coronary aneurisms. An abnormal LVLSS was found in 56% of the population studied. All of the patients that had an abnormal LVLSS had coronary aneurisms with stenosis or complete occlusion confirmed by invasive coronary angiography and abnormal Nuclear Medicine perfusion scans. Conclusions On the population studied, all patients with an abnormal LVLSS had obstructive coronary lesions and ischemic heart disease.