Growth Trajectories Over the First Year of Life Among Early-Treated Infants with Human Immunodeficiency Virus and Infants Who are Human Immunodeficiency Virus-Exposed Uninfected.

OBJECTIVE: To investigate the role of early antiretroviral therapy (ART) on growth trajectories of infants with human immunodeficiency virus (IHIV) in the first year of life. STUDY DESIGN: As part of a clinical trial of early ART in Johannesburg, South Africa (2015-2018), 116 IHIV diagnosed within 48 hours of birth were started on ART as soon as possible, and 80 uninfected infants born to mothers living with HIV (IHEU) were enrolled. Both groups were followed prospectively from birth through 48 weeks and growth parameters collected. The groups were compared and risk factors for poor growth investigated, in the full cohort and among IHIV separately. RESULTS: IHIV had lower mean weight-for-age Z-scores (WAZ) than IHEU at 4 and 8 weeks (-1.17 [SE:0.14] vs -0.72 [0.14], P = .035 and -1.23 [0.15] vs -0.67 [0.14], P = .012). Although there was some closing of the gap over time, means remained lower in IHIV through 48 weeks. In length-for-age Z-scores (LAZ), differences widened over time and IHIV had lower Z-scores by 48 weeks (-1.41 [0.15] vs -0.80 [0.18], P = .011). Deficits in WAZ and LAZ in IHIV vs IHEU were most marked among girls. IHIV with pre-ART viral load ≥1000 copies/ml had significantly lower weight-for-length and mid-upper arm circumference Z-scores across all time points through 48 weeks. CONCLUSIONS: IHIV on early ART had deficits in WAZ over the first 8 weeks of life and lower LAZ at 48 weeks than IHEU. Among IHIV, higher pre-ART viral load was associated with worse anthropometric indicators through 48 weeks.

Improving emergency department care of infants with acute bronchiolitis by reducing the use of unrecommended drugs: a quality-of-care initiative in a Spanish autonomous community. / Iniciativa de mejora para reducir el uso de fármacos no recomendados en el manejo de lactantes con bronquiolitis aguda en los servicios de urgencias de una comunidad autónoma.

OBJECTIVES: To evaluate the impact of a quality-of-care improvement program implemented in emergency departments (EDs) in a Spanish autonomous community with the aim of reducing the use of unrecommended drugs when treating infants for acute bronchiolitis. MATERIAL AND METHODS: Before-after quasi-experimental intervention study. We retrospectively included infants aged 12 months or less who were treated for acute bronchiolitis in 24 Spanish national health system hospital EDs in December during 2 epidemic periods: in 2018, before implementing the program, and in 2019, after implementation. Data collected included epidemiologic information, clinical and care details, and clinical course. The program consisted of providing informative material and training sessions before the epidemic period started. RESULTS: A total of 7717 episodes (4007 in 2018 and 2710 in 2019) were identified. Epidemiologic and clinical characteristics did not differ between the 2 periods. ED use of the following treatments decreased between the 2 periods: salbutamol, from 29.4% (95% CI, 28.8%-30.8%) in 2018 to 10.6% (95% CI, 9.6%-11.6%) in 2019; epinephrine from 6.0% (95% CI, 5.3%-6.8%) to 0.9% (95% CI, 0.7%-1.3%); and hypertonic saline solution fell from 8.2% (95% CI, 7.3%-9.1%) to 2.1% (95% CI, 1.7%-2.6%) (P.001, all comparisons). Prescriptions for salbutamol on discharge fell from 38.7% (95% CI, 36.9%-40.4%) to 10.6% (95% CI, 9.6%-11.6%) (P.001). Admissions and readmissions did not change, and the median time (interquartile range) spent in the ED fell from 81 (44-138) minutes to 66 (37-127) minutes (P.001). CONCLUSION: The quality-of-care improvement initiative was able to decrease the number of unrecommended therapeutic interventions for acute bronchiolitis. However, we identified great variations between EDs, suggesting that training and assessment of impact should continue.

OBJETIVO: Evaluar el impacto de una iniciativa de mejora realizada en los servicios de urgencias (SU) de una comunidad autónoma para reducir el uso de fármacos no recomendados en lactantes con bronquiolitis aguda (BA). METODO: Estudio cuasi-experimental analítico del tipo "antes y después de una intervención". Se incluyeron de forma retrospectiva todas las BA en niños # 12 meses atendidas en los SU de 24 hospitales públicos durante el mes de diciembre de dos periodos epidémicos: 2018 (preintervención) y 2019 (postintervención). Se recogieron variables epidemiológicas, clínicas, asistenciales y evolutivas. La intervención consistió en difundir material informativo y realizar actividades formativas previas al periodo epidémico. RESULTADOS: Se incluyeron 7.717 episodios (2018: 4.007 y 2019: 3.710). No existieron diferencias en las características epidemiológicas y clínicas. El empleo de salbutamol en los SU descendió del 29,4% [intervalo de confianza del 95% (IC 95%): 28,8-30,8] en 2018 al 10,6% (IC 95%: 9,6-11,6) en 2019 (p 0,001), el de adrenalina del 6,0% (IC 95%: 5,3-6,8) al 0,9% (IC 95%: 0,7-1,3) y el de suero salino hipertónico del 8,2% (IC 95%: 7,3-9,1) al 2,1% (IC 95%: 1,7-2,6) (p 0,001). La prescripción al alta de salbutamol se redujo del 38,7% (IC 95%: 36,9-40,4) al 10,6% (IC 95%: 9,6-11,6) (p 0,001). La tasa de ingreso y la tasa de readmisión no cambiaron y la mediana de tiempo de estancia en los SU se redujo 81 minutos [rango intercuartil (RIC) 44-138] a 66 (RIQ: 37-127) (p 0,001). CONCLUSIONES: La iniciativa de mejora ha conseguido disminuir la tasa de intervenciones terapéuticas no indicadas en BA. Sin embargo, existe una gran variabilidad entre los diferentes SU por lo que la estrategia y la medición de su impacto deben mantenerse en el tiempo.

Iniciativa de mejora para reducir el uso de fármacos no recomendados en el manejo de lactantes con bronquiolitis aguda en los servicios de urgencias de una comunidad autónoma / Improving emergency department care of infants with acute bronchiolitis by reducing the use of unrecommended drugs: a quality-of-care initiative in a Spanish autonomous community

Objetivo. Evaluar el impacto de una iniciativa de mejora realizada en los servicios de urgencias (SU) de una comunidad autónoma para reducir el uso de fármacos no recomendados en lactantes con bronquiolitis aguda (BA).Método. Estudio cuasi-experimental analítico del tipo “antes y después de una intervención”. Se incluyeron de forma retrospectiva todas las BA en niños # 12 meses atendidas en los SU de 24 hospitales públicos durante el mes de diciembre de dos periodos epidémicos: 2018 (preintervención) y 2019 (postintervención). Se recogieron variables epidemiológicas, clínicas, asistenciales y evolutivas. La intervención consistió en difundir material informativo y realizar actividades formativas previas al periodo epidémico.Resultados. Se incluyeron 7.717 episodios (2018: 4.007 y 2019: 3.710). No existieron diferencias en las características epidemiológicas y clínicas. El empleo de salbutamol en los SU descendió del 29,4% [intervalo de confianza de 95% (IC 95%): 28,8-30,8] en 2018 al 10,6% (IC 95%: 9,6-11,6) en 2019 (p < 0,001), el de adrenalina del 6,0% (IC 95%: 5,3-6,8) al 0,9% (IC 95%: 0,7-1,3) y el de suero salino hipertónico del 8,2% (IC 95%: 7,3-9,1) al 2,1% (IC 95%: 1,7-2,6) (p < 0,001). La prescripción al alta de salbutamol se redujo del 38,7% (IC 95%: 36,9-40,4) al 10,6% (IC 95%: 9,6-11,6) (p < 0,001). La tasa de ingreso y la tasa de readmisión no cambiaron y la mediana de tiempo de estancia en los SU se redujo 81 minutos [rango intercuartil (RIC) 44-138] a 66 (RIQ: 37-127) (p < 0,001).Conclusiones. La iniciativa de mejora ha conseguido disminuir la tasa de intervenciones terapéuticas no indicadas en BA. Sin embargo, existe una gran variabilidad entre los diferentes SU por lo que la estrategia y la medición de su impacto deben mantenerse en el tiempo. (AU)

Objective. To evaluate the impact of a quality-of-care improvement program implemented in emergency departments (EDs) in a Spanish autonomous community with the aim of reducing the use of unrecommended drugs when treating infants for acute bronchiolitis. Methods. Before-after quasi-experimental intervention study. We retrospectively included infants aged 12 months or less who were treated for acute bronchiolitis in 24 Spanish national health system hospital EDs in December during 2 epidemic periods: in 2018, before implementing the program, and in 2019, after implementation. Data collected included epidemiologic information, clinical and care details, and clinical course. The program consisted of providing informative material and training sessions before the epidemic period started. Results. A total of 7717 episodes (4007 in 2018 and 2710 in 2019) were identified. Epidemiologic and clinical characteristics did not differ between the 2 periods. ED use of the following treatments decreased between the 2 periods: salbutamol, from 29.4% (95% CI, 28.8%-30.8%) in 2018 to 10.6% (95% CI, 9.6%-11.6%) in 2019; epinephrine from 6.0% (95% CI, 5.3%-6.8%) to 0.9% (95% CI, 0.7%-1.3%); and hypertonic saline solution fell from 8.2% (95% CI, 7.3%-9.1%) to 2.1% (95% CI, 1.7%-2.6%) (P<.001, all comparisons). Prescriptions for salbutamol on discharge fell from 38.7% (95% CI, 36.9%-40.4%) to 10.6% (95% CI, 9.6%-11.6%) (P<.001). Admissions and readmissions did not change, and the median time (interquartile range) spent in the ED fell from 81 (44-138) minutes to 66 (37-127) minutes (P<.001). Conclusions. The quality-of-care improvement initiative was able to decrease the number of unrecommended therapeutic interventions for acute bronchiolitis. However, we identified great variations between EDs, suggesting that training and assessment of impact should continue. (AU)

Risk scores for Kawasaki disease, a management tool developed by the KAWA-RACE cohort.

INTRODUCTION/OBJECTIVES: Asian scores developed to predict unresponsiveness to intravenous immunoglobulin (IVIG) or development of coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) are not appropriate in Western populations. The purpose of this study is to develop 2 scores, to predict unresponsiveness to IVIG and development of CAA, appropriate for Spanish population. METHOD: Data of 625 Spanish children with KD collected retrospectively (2011-2016) were used to identify variables to develop the 2 scores of interest: unresponsiveness to IVIG and development of CAA. A statistical model selected best variables to create the scores, and scores were validated with data from 98 patients collected prospectively. RESULTS: From 625 patients of the retrospective cohort, final analysis was performed in 439 subjects: 37 developed CAA, and 212 were unresponsive to IVIG. For the score to predict CAA, a cutoff ≥ 8 was considered for high risk, considering a score system with a different weight for each of the eight variables. External validation showed a sensitivity of 22% and a specificity of 75%. The score to predict unresponsiveness to IVIG established a cutoff ≥ 8 for high risk, considering a score system with a different weight for each of the nine variables. External validation showed a sensitivity of 78% and a specificity of 50%. CONCLUSIONS: Two risk scores for KD were developed from Spanish population, to predict development of CAA and unresponsiveness to IVIG; validation in other cohorts could help to implement these tools in the management of KD in other Western populations.

Ventricular Repolarization Parameters and Coronary Involvement in Kawasaki Disease.

OBJECTIVES: To evaluate electrocardiogram markers to predict coronary involvement in patients with Kawasaki disease by assessing measures of ventricular repolarization parameters on the 12-lead electrocardiogram. STUDY DESIGN: This cross-sectional study included 180 Spanish and Japanese patients ≤14 years of age with Kawasaki disease, with or without coronary involvement, from 2011 to 2016. We manually measured the Tp-Te/QT ratio and QTc interval (with Bazett's formula) in 12-lead electrocardiogram in the acute and recovery period and explored their potential association with coronary involvement. RESULTS: No association was found between Tp-Te/QT ratio obtained manually in V5 and V6 leads and coronary involvement in the acute (V5:0.25 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .80; V6:0.24 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .86) or the recovery (V5: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.19-0.25], P = .68; V6: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.17-0.25], P = .50) period. By contrast, QTc in V5 and V6 was significantly lower in patients with Kawasaki disease and coronary involvement in the acute period (V5: 378 ms [IQR, 364-395 ms] vs 390 ms [IQR, 371-411 ms], P = .04; V6: 377 ms [IQR, 364-392 ms] vs 390 ms [IQR, 371-410 ms], P = .01). A QTc interval of <385 ms in lead V6 was associated with a 2.5-fold increased risk of coronary involvement (OR, 2.5; 95% CI, 1.2-5.3; P = .02). CONCLUSIONS: Manually measured QTc interval may be a marker of coronary disease in the acute period of Kawasaki disease.

Prevalence and Clinical Characteristics of SARS-CoV-2 Confirmed and Negative Kawasaki Disease Patients During the Pandemic in Spain.

Introduction: COVID-19 has a less severe course in children. In April 2020, some children presented with signs of multisystem inflammation with clinical signs overlapping with Kawasaki disease (KD), most of them requiring admission to the pediatric intensive care unit (PICU). This study aimed to describe the prevalence and clinical characteristics of KD SARS-CoV-2 confirmed and negative patients during the pandemic in Spain. Material and Methods: Medical data of KD patients from January 1, 2018 until May 30, 2020 was collected from the KAWA-RACE study group. We compared the KD cases diagnosed during the COVID-19 period (March 1-May 30, 2020) that were either SARS-CoV-2 confirmed (CoV+) or negative (CoV-) to those from the same period during 2018 and 2019 (PreCoV). Results: One hundred and twenty-four cases were collected. There was a significant increase in cases and PICU admissions in 2020 (P-trend = 0.001 and 0.0004, respectively). CoV+ patients were significantly older (7.5 vs. 2.5 yr) and mainly non-Caucasian (64 vs. 29%), had incomplete KD presentation (73 vs. 32%), lower leucocyte (9.5 vs. 15.5 × 109) and platelet count (174 vs. 423 × 109/L), higher inflammatory markers (C-Reactive Protein 18.5vs. 10.9 mg/dl) and terminal segment of the natriuretic atrial peptide (4,766 vs. 505 pg/ml), less aneurysm development (3.8 vs. 11.1%), and more myocardial dysfunction (30.8 vs. 1.6%) than PreCoV patients. Respiratory symptoms were not increased during the COVID-19 period. Conclusion: The KD CoV+ patients mostly meet pediatric inflammatory multisystem syndrome temporally associated with COVID-19/multisystem inflammatory syndrome in children criteria. Whether this is a novel entity or the same disease on different ends of the spectrum is yet to be clarified.

Epidemiological and clinical features of Kawasaki disease in Spain over 5 years and risk factors for aneurysm development. (2011-2016): KAWA-RACE study group.

BACKGROUND: Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology affecting mainly children less than 5 years of age. Risk factors for cardiac involvement and resistance to treatment are insufficiently studied in non-Japanese children. OBJECTIVE: This study aimed to investigate the epidemiology, clinical features and risk factors for resistance to treatment and coronary artery lesions (CAL) in KD in Spain. METHODS: Retrospective study (May 2011-June 2016) of all patients less than 16 years of age diagnosed with KD included in KAWA-RACE network (84 Spanish hospitals). RESULTS: A total of 625 cases were analyzed, 63% were males, 79% under 5 year-olds and 16.8% younger than 12 months. On echocardiographic examination CAL were the most frequent findings (23%) being ectasia the most common (12%). Coronary aneurysms were diagnosed in 9.6%, reaching 20% in infants under 12 months (p<0.001). A total of 97% of the patients received intravenous immunoglobulin (IVIG) with a median number of days from fever onset to IVIG administration of 7.2. A second dose was given to 15.7% and steroids to 14.5% patients. Only 1.4% patients received infliximab. No deaths were reported. A multivariate analysis identified anemia, hypoalbuminemia, hyponatremia, higher creatinine and procalcitonin as independent risk factors for treatment failure and length under 103 cm, hemoglobin < 10.2 mg/dL, platelets > 900,000 cells/mm3, maximum temperature < 39.5°C, total duration of fever > 10 days and fever before treatment ≥ 8 days as independent risk factors for developing coronary aneurysms. CONCLUSIONS: In our population, children under 12 months develop coronary aneurysms more frequently and children with KD with anemia and leukocytosis have high risk of cardiac involvement. Adding steroids early should be considered in those patients, especially if the treatment is not started before 8 days of fever. A score applicable to non-Japanese children able to predict the risk of aneurysm development and IVIG resistance is necessary.

Infecciones previas o coincidentes con la sospecha de enfermedad de Kawasaki ¿debemos cambiar nuestra actitud? / Previous or coincident infections with suspected Kawasaki disease. Should we change our approach?

Introducción: La enfermedad de Kawasaki (EK) es una vasculitis multisistémica asociada a lesiones en las arterias coronarias. Las infecciones podrían ser un desencadenante de la inflamación. Nuestro objetivo fue describir la presencia de infecciones en los niños con EK y analizar las características clínicas y la presencia de alteraciones coronarias en estos casos. Pacientes y métodos: Análisis retrospectivo de los pacientes incluidos en la red KAWA-RACE entre 2011 y 2016. Se estudió tanto a los pacientes que tuvieron una identificación microbiológica confirmada (IMC) en el periodo agudo como a los que presentaron antecedente de infección previa reciente (IPR) las 4 semanas anteriores. Resultados: Se incluyó a un total de 621 niños, de los cuales 101 (16,3%) tuvieron una IMC y 107 (17,2%) una IPR. Encontramos una significativa menor afectación ecocardiográfica en el grupo de IPR respecto a los niños sin infección previa (23 vs. 35%; p 0,01), con menor proporción no significativa de las alteraciones coronarias globales (16 vs. 25%; p 0,054). Sin embargo, no se detectaron diferencias en la proporción de aneurismas en ninguno de los 2 grupos (IMC o IPR) respecto al resto de los pacientes sin infecciones asociadas. Conclusiones: En nuestro estudio no encontramos diferencias en la incidencia de aneurismas coronarios en niños con y sin IMC o IPR, por lo que ante la sospecha de EK debe iniciarse siempre tratamiento, aunque se tenga infección confirmada microbiológicamente

Introduction: Kawasaki disease (KD) is a multisystem vasculitis associated with coronary artery abnormalities. Infections could be a trigger of the inflammation. The main aim of this study was to describe the presence of infections in children with KD, and to analyse the clinical characteristics and the presence of coronary abnormalities in these cases. Patients and methods: A retrospective study was performed within the Kawasaki Diseases Network (KAWA-RACE (2011-2016). An analysis was performed that included patients with positive microbiological findings (PMF) during the acute phase, as well as those with a previous recent infection (PRI) during the 4 weeks preceding KD diagnosis. Results: The study included total of 621 children with KD, with PMF being found in 101 (16.3%) patients, and a PRI in 107 (17.2%). Significantly less echocardiographic abnormalities were found in the in the group with a PRI, when compared to those without a PRI (23 vs. 35%, P = .01) and also a lower proportion of overall coronary artery lesions (16 vs. 25%, P = .054). No significant differences were found in the proportion of aneurysms in either of these groups (PRI or PMF) when compared to those without infection. Conclusions: In the present study, no differences were found in the incidence of coronary aneurysms in either of the groups, with or without PRI or PMF. Therefore, if KD is suspected, appropriate treatment should be started despite having a confirmed infection

[Previous or coincident infections with suspected Kawasaki disease. Should we change our approach?] / Infecciones previas o coincidentes con la sospecha de enfermedad de Kawasaki ¿debemos cambiar nuestra actitud?

INTRODUCTION: Kawasaki disease (KD) is a multisystem vasculitis associated with coronary artery abnormalities. Infections could be a trigger of the inflammation. The main aim of this study was to describe the presence of infections in children with KD, and to analyse the clinical characteristics and the presence of coronary abnormalities in these cases. PATIENTS AND METHODS: A retrospective study was performed within the Kawasaki Diseases Network (KAWA-RACE (2011-2016). An analysis was performed that included patients with positive microbiological findings (PMF) during the acute phase, as well as those with a previous recent infection (PRI) during the 4 weeks preceding KD diagnosis. RESULTS: The study included total of 621 children with KD, with PMF being found in 101 (16.3%) patients, and a PRI in 107 (17.2%). Significantly less echocardiographic abnormalities were found in the in the group with a PRI, when compared to those without a PRI (23 vs. 35%, P=.01) and also a lower proportion of overall coronary artery lesions (16 vs. 25%, P=.054). No significant differences were found in the proportion of aneurysms in either of these groups (PRI or PMF) when compared to those without infection. CONCLUSIONS: In the present study, no differences were found in the incidence of coronary aneurysms in either of the groups, with or without PRI or PMF. Therefore, if KD is suspected, appropriate treatment should be started despite having a confirmed infection.

Consenso nacional sobre diagnóstico, tratamiento y seguimiento cardiológico de la enfermedad de Kawasaki / National consensus on the cardiological treatment and follow-up of Kawasaki disease

La enfermedad de Kawasaki es una vasculitis aguda autolimitada que afecta a vasos de pequeño y mediano calibre y es la causa más común de enfermedad cardiaca adquirida en niños en nuestro medio. Hasta un 25% de pacientes no tratados desarrollan aneurismas coronarios. Se sospecha que un agente infeccioso puede ser el desencadenante de la enfermedad, pero aún se desconoce el agente causal. En base a la evidencia previa, se proponen recomendaciones para el diagnóstico, tratamiento de la enfermedad aguda y manejo a largo plazo de estos pacientes, con el fin de unificar criterios. El diagnóstico debe ser rápido, basado en algoritmos de fácil manejo y con el apoyo de pruebas complementarias. Este documento recoge la indicación de las técnicas de imagen disponibles, así como la planificación de las revisiones cardiológicas en función de la afectación inicial. La inmunoglobulina intravenosa es la base del tratamiento inicial. El papel de los corticoides aún es controvertido, pero cada vez hay más estudios que avalan su uso como tratamiento adyuvante. Un equipo multidisciplinar ha elaborado un esquema con diferentes pautas de tratamiento en función de los factores de riesgo al diagnóstico, situación clínica del paciente y respuesta al tratamiento previo, incluyendo indicaciones sobre tromboprofilaxis en pacientes con afectación coronaria. La estratificación del riesgo para el tratamiento a largo plazo es esencial, así como las recomendaciones acerca del proceder en función de la afectación cardiológica inicial y su evolución. Los pacientes con aneurismas coronarios requieren un seguimiento cardiológico continuo e ininterrumpido de por vida

Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patient's clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life

[National consensus on the cardiological treatment and follow-up of Kawasaki disease]. / Consenso nacional sobre diagnóstico, tratamiento y seguimiento cardiológico de la enfermedad de Kawasaki.

Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patient's clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life.

Congenital ADAMTS13 deficiency: a rare mimicker of immune thrombocytopenic purpura.

Congenital ADAMTS13 deficiency is a rare disease that leads to recurrent episodes of thrombotic thrombocytopenic purpura. We report a case that mimicked a recurring immune thrombocytopenic purpura in a child. Mild cases of ADAMTS13 deficiency may be initially confused with immune thrombocytopenic purpura if hemolytic anemia is not severe and renal or neurological symptoms are not present. Fresh frozen plasma is the treatment of choice in acute thrombotic thrombocytopenic purpura in ADAMTS13-deficient patients. The best long-term treatment for slightly symptomatic cases remains to be elucidated. Recombinant human ADAMTS13 factor will be a promising option when commercially available.