Assessing pulmonary hypertensive vascular disease in childhood. Data from the Spanish registry.

RATIONALE: There is a lack of knowledge regarding the epidemiology, clinical characterization, and survival in pediatric pulmonary hypertension. OBJECTIVES: To describe the epidemiology, outcomes, and risk factors for mortality in pediatric pulmonary hypertension in Spain. METHODS: We analyzed data from the Spanish Registry for Pediatric Pulmonary Hypertension. From January 2009 to June 2012, a total of 225 patients diagnosed with pulmonary hypertension in 1998 or after were collected from 21 referral and nonreferral centers. We included all Nice etiologies, estimated incidence and prevalence of pulmonary hypertension in the Spanish pediatric population, and analyzed risk factors for mortality (Nice etiologic group, clinical and hemodynamic variables). Patients were classified as follows: group I, pulmonary arterial hypertension (n = 142; 61%); group II, left heart disease (n = 31; 14%); group III, respiratory disease (n = 41; 18%); group IV, thromboembolic pulmonary hypertension (n = 2; 1%); or group V, mostly inherited metabolic diseases (n = 10; 4.5%). Of the patients studied, 31% had multifactorial pulmonary hypertension. MEASUREMENTS AND MAIN RESULTS: Mean age at diagnosis was 4.3 ± 4.9 years (50% < 2 yr). Survival rates at 1 and 3 years were 80 and 74% for the whole cohort, and 89 and 85% for patients with pulmonary arterial hypertension. Independent risk factors for mortality included an etiologic group other than pulmonary arterial hypertension (P < 0.001), age at diagnosis younger than 2 years old (P < 0.001), advanced functional class at diagnosis (P < 0.001), and high right atrial pressure at diagnosis (P = 0.002). CONCLUSIONS: In moderate to severe pediatric pulmonary hypertension, the prognosis is better in pulmonary arterial hypertension than in other Nice categories. In pediatric pulmonary hypertension age at diagnosis younger than 2 years is a risk factor for mortality, in addition to the previously established risk factors.

[Suspected child abuse in paediatric emergency service]. / Sospecha de maltrato infantil en urgencias pediátricas.

OBJECTIVE: To describe the epidemiology of child abuse in an emergency department of a tertiary paediatric hospital. METHODS: Descriptive and retrospective study from January 2008 to January 2006 including patients less than sixteen years of age who were suspected of being abused during the examination in the emergency department. RESULTS: Child maltreatment was 0.07% of all paediatric emergencies (45% physical abuse, 35% sexual abuse and 20% neglect). Mean age of 6 years old, with no gender differences. 86% were suspected of maltreatment. An adult living with the child was suspected in 67% of cases. Social and judicial procedures were activated. A total of 24 children were admitted, 14 under medical criteria and the rest in order to protect the child; 2 had serious neurological consequences and one died. Eight patients were discharged to social service care centres. CONCLUSIONS: We believe it is necessary to improve the pediatrician's knowledge of child abuse and to create specialized units.

[Non-compacted cardiomyopathy: clinical characteristics, evolution and prognostic data in childhood. Results of a multicenter study]. / Miocardiopatía no compactada: características clínicas, evolutivas y pronósticas en edad pediátrica. Resultados de un estudio multicéntrico.

INTRODUCTION: Non-compaction of the ventricular myocardium (NCVM) is a rare congenital heart disease. Heightened awareness has resulted in increased detection of the morphological features of NCVM in routine clinical practice. PATIENTS AND METHODS: Multicentre study including paediatric patients affected by NCVM according to the echocardiographic criteria of Chin and Jenni. RESULTS: A total of 29 patients were included, 15 female and 14 male, the median age at diagnosis was 5 years and 7 months (birth to 17 years). Sixteen patients (55%) presented as an isolated lesion, 8 (27.5%) had a ventricular septal defect, one of them associated with aortic coarctation, 3 (10%) had an inborn error of metabolism, 1 (3.5%) had Juvenile Idiopathic Arthritis and 1 (3.5%) has a syndrome being studied. The location of the trabeculae has been predominantly at the apex, but also affected the left ventricle free wall in 11 patients (40%) and right ventricle in 2 (7%). No complications were present in 12 patients (41%), with cardiac failure in 12 patients (41%), an implantable cardioverter defibrillator was placed for ventricular arrhythmias in 2 patients (7%), stroke, 1 patient (3,5%) and death, 2 patients (7%), both of them less than 6 months of age (P<.05). Median follow up was 12 months (2 months to 8 years). Current treatment includes carvedilol, ACEI's and ASA, and one patient is waiting for a cardiac transplantation. CONCLUSIONS: Early onset of symptoms is associated with a poor prognosis. Clinical and prognostic heterogeneity is described.

Miocardiopatía no compactada: características clínicas, evolutivas y pronósticas en edad pediátrica. Resultados de un estudio multicéntrico / Non-compacted cardiomyopathy: clinical characteristics, evolution and prognostic data in childhood. Results of a multicentre study

Introducción y objetivos: La miocardiopatía no compactada es una enfermedad congénita infrecuente pero su diagnóstico se está incrementando últimamente coincidiendo con un mejor conocimiento de la entidad. Pacientes y métodos: Estudio multicéntrico que incluye pacientes pediátricos diagnosticados de miocardiopatía no compactada según los criterios ecocardiográficos de Chin y Jenni. Resultados: Se incluyó a un total de 29 pacientes, 15 niñas y 14 niños, con una edad mediana al diagnóstico de 5,6 años (0-17). Dieciséis pacientes (55%) tienen una lesión aislada, 8 (27,5%) comunicación interventricular asociada (uno de ellos con coartación de aorta), 3 (10%) error innato del metabolismo, 1 (3,5%) artritis idiopática juvenil y 1 otros. La localización de las trabéculas ha sido predominantemente en el ápex, afectando también en 11 casos (40%) la pared libre del ventrículo izquierdo y en 2 (7%) el ventrículo derecho. La evolución ha sido buena en 12 pacientes (41%), insuficiencia cardiaca congestiva 12 (41%), arritmias ventriculares precisando implante de desfibrilador automático 2 (7%), accidente vascular cerebral 1 (3,5%) y fallecimiento 2 (7%), ambos menores de 6 meses de vida (p<0,05). El tiempo mediano de seguimiento ha sido de 12 meses (2 meses a 8 años). El tratamiento se basa en combinación farmacológica y un paciente está en lista de trasplante cardíaco. Conclusiones: Parece existir una relación entre el inicio precoz de la sintomatología y un peor pronóstico. Se describe una gran heterogeneidad clínica, evolutiva y pronóstica(AU)

Introduction: Non-compaction of the ventricular myocardium (NCVM) is a rare congenital heart disease. Heightened awareness has resulted in increased detection of the morphological features of NCVM in routine clinical practice. Patients and methods: Multicentre study including paediatric patients affected by NCVM according to the echocardiographic criteria of Chin and Jenni. Results: A total of 29 patients were included, 15 female and 14 male, the median age at diagnosis was 5 years and 7 months (birth to 17 years). Sixteen patients (55%) presented as an isolated lesion, 8 (27.5%) had a ventricular septal defect, one of them associated with aortic coarctation, 3 (10%) had an inborn error of metabolism, 1 (3.5%) had Juvenile Idiopathic Arthritis and 1 (3.5%) has a syndrome being studied. The location of the trabeculae has been predominantly at the apex, but also affected the left ventricle free wall in 11 patients (40%) and right ventricle in 2 (7%). No complications were present in 12 patients (41%), with cardiac failure in 12 patients (41%), an implantable cardioverter defibrillator was placed for ventricular arrhythmias in 2 patients (7%), stroke, 1 patient (3,5%) and death, 2 patients (7%), both of them less than 6 months of age (P<0.05). Median follow up was 12 months (2 months to 8 years). Current treatment includes carvedilol, ACEI‘s and ASA, and one patient is waiting for a cardiac transplantation. Conclusions: Early onset of symptoms is associated with a poor prognosis. Clinical and prognostic heterogeneity is described(AU)

Sospecha de maltrato infantil en urgencias pediátricas / Suspected child abuse in paediatric emergency service

Objetivo: Describir la epidemiología del maltrato infantil en el servicio de urgencias de un hospital pediátrico de tercer nivel. Metodología: Estudio descriptivo retrospectivo, desde enero de 2008 a enero de 2006. Se incluyeron pacientes menores de 16 años atendidos en urgencias en los que se sospechó maltrato. Resultados: De un total de 96.419 urgencias, 71 casos (0,07%) fueron por un potencial maltrato (el 45% físico, el 35% sexual y el 20% por negligencia y abandono). La edad media fue de 6 años, sin diferencias entre sexos. El 86% de los casos consultó por sospecha de maltrato. En un 67% de los casos, el posible maltratador convivía en el domicilio con el menor. Se activó la vía sociojurídica en todos los casos. Ingresaron 24 pacientes, 14 por criterio médico y el resto para protección del menor. Dos pacientes quedaron con secuelas neurológicas graves y uno falleció. Se derivó a 8 pacientes a un centro de acogida. Conclusiones: Son necesarias tanto la sensibilización y la formación del personal sanitario para la detección del maltrato como la creación de unidades especializadas para el tratamiento y el seguimiento (AU)

Objective: To describe the epidemiology of child abuse in an emergency department of a tertiary paediatric hospital. Methods: Descriptive and retrospective study from January 2008 to January 2006 including patients less than sixteen years of age who were suspected of being abused during the examination in the emergency department. Results: Child maltreatment was 0.07% of all paediatric emergencies (45% physical abuse, 35% sexual abuse and 20% neglect). Mean age of 6 years old, with no gender differences. 86% were suspected of maltreatment. An adult living with the child was suspected in 67% of cases. Social and judicial procedures were activated. A total of 24 children were admitted, 14 under medical criteria and the rest in order to protect the child; 2 had serious neurological consequences and one died. Eight patients were discharged to social service care centres (AU)