Converting an infant with borderline left heart structures to biventricular circulation after an initial hybrid Norwood approach: a case report.

Background: Hybrid approach in hypoplastic left heart syndrome (HLHS) implies placement of bilateral pulmonary artery bands and ductal stenting. This approach serves as a less invasive surgical-interventional alternative to the Norwood procedure with reported equivalent survival. Potential benefits include deferrable surgical risks and in some borderline left ventricle patients, when combined with a restrictive inter-atrial communication, it offers a chance for biventricular circulation. Case summary: Term newborn with total anomalous pulmonary venous connection (TAPVC), hypoplastic left heart, small aortic arch, and atrial septal defect (ASD) who was multi-disciplinary deemed eligible for a hybrid palliation performed on day six of life after TAPVC surgical correction. Subsequently, percutaneous ductal stent implantation was performed (two Sinus Superflex-DS stents) on day 11 of life. Magnetic resonance imaging (MRI) showed a hypoplastic non-apex-forming left ventricle (LV) [left ventricular end-diastolic volume index (LVEDVI) 15 mL/m2]. On day 27th of life, the ASD was closed surgically with a fenestrated patch. At 2 months of age, control cardiac MRI showed striking LV growth (LVEDVI 37 mL/m2). Herein an interventional hybrid take-down was performed with dilatation of both pulmonary arterial bands and closure of the ductal stent with an Amplatzer Vascular plug II, thus achieving biventricular circulation. Discussion: Hybrid approach has proven as a safe alternative to the Norwood procedure for HLHS. This case illustrates the importance of multi-disciplinary care in high-risk two ventricular patients by means of LV rehabilitation. Further studies are needed to systematically define and understand the intricacies of potential candidates for staged biventricular repair initially managed with hybrid approach.

Comparing infant and toddler sleep patterns prior to and during the first wave of home confinement due to COVID-19 in Spain.

Recent literature has shown that sleep patterns are shaped during the first years of life, playing a relevant role in children's functioning. We focused on comparing sleep patterns in infants and toddlers in Spain before and during COVID-19 home confinement to assess the immediate impact on sleep patterns. We compared data from two cross-sectional surveys from parents of 1658 children three to 36 months of age from Spain. One conducted before COVID-19 (2017-2018, n = 1380) and another during COVID-19 pandemic (March-May of 2020, n = 254). We used an internet self-administrated questionnaire using the Brief Infant Sleep Questionnaire (BISQ) criteria in both surveys. During confinement, infants and toddlers went to sleep later (median bedtime 21:30 before confinement vs. 21:36 during confinement (p = 0.004)). More infants and toddlers showed longer sleep latencies (> 30 min) during confinement median 33.9% versus 12.3% (p < 0.001). Based on the recommended BISQ criteria, we observed an increase in poor sleepers meeting at least one criterion of inadequate patterns during confinement (p < 0.001). Parental perception of a child's sleep as problematics were 39.4% and 44.1% (adjusted p = 0.363) before and under lockdown, respectively. CONCLUSION: Home confinement generally affected infant's and toddler's sleep patterns negatively; however, parents did not report worse perception of sleep quality of their children. Follow-up studies can help understand the potential long-term effects of the COVID-19 pandemic on sleep patterns. WHAT IS KNOWN: • Adequate sleep patterns in infants and toddlers are relevant as they are linked to proper and long-term social-emotional development as well as adequate daytime functioning. • Regarding sleep patterns in paediatrics during the COVID pandemic, recent literature has described an increase in total daily sleep time as well as more exposure to screens in children and adolescents, providing evidence of immediate collateral consequences of the COVID-19 outbreak. WHAT IS NEW: • Comparing sleep patterns in two samples of infants and toddlers in Spain before and during COVID-confinement, we found later bedtimes as well as a significant increase in infants' and toddlers' sleep latency by >30 min during confinement. • Parental self-reported questionnaire during COVID-19 home confinement reported an overall worsening of their children's sleep based on the BISQ criteria.

Smartphone and Tablet Usage during COVID-19 Pandemic Confinement in Children under 48 Months in Barcelona (Spain).

BACKGROUND: Total lockdown due to COVID-19 pandemic might have potentially increased screen time in children. This study aims to describe the smartphone and tablets usage in children under 48 months living in Barcelona during the COVID-19 confinement. METHODS: Cross-sectional study using a non-probabilistic sample of parents with children under 48 months living in Barcelona (Spain) during COVID-19 confinement (n = 313). We calculated percentages of exposure to smartphones and tablets. Moreover, for those children were exposed, we calculated unadjusted and adjusted Geometric Mean Ratios (GMR) of daily smartphones and tablets usage and their 95% confidence intervals (95% CI) trough Generalized Linear Models with Gamma family and link log. Associations were adjusted for potential confounders. RESULTS: During COVID-19 confinement, 67.5% of children under 48 months were daily exposed to smartphones and tablets. Further, those children who were exposed during meals, as well as before going to bed, spend longer durations using them, aGMR = 2.38 (95% CI 1.73, 3.34) and aGMR = 1.95 (95% CI 1.34, 2.91) respectively. CONCLUSION: Two out of three children under 48 months living in Barcelona were daily exposed to smartphones and tablets during total lockdown due to COVID-19. Taking this findings into account cohort studies are needed to assess any change in the screen time patterns due to total confinement in order to allow the Government help families, particularly those more vulnerable, in a possible pandemic resurgence.

How do infants and toddlers sleep in Spain? A cross-sectional study.

There is limited information on sleep patterns among infants and toddlers in Spain. The aim of this study was to assess sleep patterns in children three to 36 months of age in Spain. A cross-sectional study was conducted between February 2017 and February 2018. Sociodemographic data and sleep variables were collected using an expanded version of the validated Spanish version of the brief infant sleep questionnaire. A total of 1,404 parental reports on children (725 males; 679 females) with a mean age of 18.8 ± 9.5 months were collected. Parents who perceived their child's sleep as problematic (39% of our sample) reported fewer sleep hours (median 9 versus 10 h), more night awakenings (median 2 versus 1), and longer periods of nocturnal awakenings (median 0.5 versus 0.08 min) (p < 0.001). Parental presence at the time of sleep onset and later and irregular bedtime routines were significantly associated with a reduction in total sleep time, longer sleep latency, and disruptive night awakenings (p < 0.001). These findings highlight the need for further studies to assess how to improve sleep patterns as a relevant modifiable lifestyle factor.Conclusion: A substantial percentage of the population perceived that their children slept poorly, which was evident in a variety of sleep patterns, including sleep duration and sleep quality. What is known: • Previous research has established that sleep difficulties among pediatric population affect up to 30% of all children and up to 20-30% of infants and toddlers. • A positive relationship between less parental bedtime involvement and sleep consolidation in infants and toddlers has been established. What is new: • More than a third of Spanish parents perceived their infants and toddlers sleep as problematic and their children reportedly have shorter night sleep hours, more night awakenings, and longer periods of nocturnal awakenings. • Later and irregular parental bedtime routines were associated with worst infants and toddlers sleep.

Nen de 5 anys amb augment supraclavicular de teixits tous / No disponible

No disponible

Neonatal lethal hypophosphatasia: A case report and review of literature.

RATIONALE: Hypophosphatasia (HPP) is a very rare metabolic bone disease caused by loss-of-function mutations in the ALPL gene encoding the tissue nonspecific alkaline phosphatase. The severe neonatal form is considered lethal but insights into manifestations of the disease can help to increase our knowledge of the natural history for an early initiation of treatment and improvement of survival. PATIENT CONCERNS: We report the case of a newborn in which his fetal imaging showed findings of skeletal dysplasia disorder, considering initially achondroplasia as a potential diagnosis. DIAGNOSIS: A definitive diagnosis compatible with perinatal lethal HPP was established in the 1st days due to the presentation at birth with thoracic and pulmonary hypoplasia, bone hypomineralization, and undetectable alkaline phosphatase. The genetic analysis identified a new heterozygous c.413G>C mutation and another 1 c.473-2G>C previously described in the ALPL gene. OUTCOMES: The patient died on the 4th day by clinical course complicated without having started enzyme replacement therapy (ERT). Retrospectively, previous analyzes of the parents already showed both a decreased alkaline phosphatase. LESSONS: This report highlights the importance of prenatal differential diagnosis of bone dysplasia with the key biochemical marker of alkaline phosphatase in the parents. Substitutive ERT administered very soon after birth, seems to change the prognosis in these patients with neonatal HPP.

Adaptación y estudio de propiedades métricas de un cuestionario de valoración del sueño para lactantes y preescolares / Adaptation and study of the measurement properties of a sleep questionnaire for infants and pre-school children

INTRODUCCIÓN: Aunque las alteraciones del sueño en lactantes y preescolares son comunes, no se dispone de un instrumento en español adecuadamente validado para su estudio. El Brief Infant Sleep Questionnaire (BISQ) es un cuestionario multidimensional bien establecido con este fin en el ámbito internacional. OBJETIVOS: Adaptar el BISQ al español y analizar su fiabilidad y validez. Explorar su viabilidad en el contexto asistencial y de investigación. Participantes y métodos: Se incluyeron niños de 3 a 30 meses de edad. La adaptación al español (BISQ-E) se efectuó mediante técnica bilingüe de retrotraducción y consenso, siguiendo las recomendaciones internacionales. Se evaluó la fiabilidad mediante análisis de los resultados del test-retest del BISQ-E y de la concordancia de las respuestas a 2formas de administración (autoadministración y entrevista clínica). La validez de constructo se estableció analizando su correlación con un diario de sueño. RESULTADOS: Participaron un total de 87 familias/niños. La correlación global test-retest en 60 sujetos fue de r = 0,848 (p < 0,001). El índice de kappa entre las 2 formas de administración fue de 0,939 (IC 95%: 0,858-1,00; p < 0,001).La correlación BISQ-E-diario de sueño se analizó en 27 familias/niños y fue estadísticamente significativa para las variables estudiadas: hora de acostarse (r = 0,731), horas de sueño nocturno (r = 0,726), horas de sueño diurno r = 0,867) y número de despertares nocturnos (r = 0,888) (p < 0,001). CONCLUSIONES: La adaptación española del BISQ presenta unas características de validez y fiabilidad adecuadas para la evaluación del sueño en lactantes y preescolares. Su utilización como instrumento clínico o para estudios de investigación clínico-epidemiológica resulta factible

INTRODUCTION: Although sleep disturbances in infants and toddlers are common, there is no suitable validated tool in Spanish to evaluate sleep disorders. The Brief Infant Sleep Questionnaire (BISQ) is a well-established multidimensional questionnaire widely used internationally. OBJECTIVES: To adapt the BISQ questionnaire to Spanish and analyse its reliability and validity. To explore its feasibility, both in the clinical context, and in epidemiological research. METHODS: Participants were parents of children between 3 and 30 months of age. The adaptation to Spanish (BISQ-E) was achieved by using both back translation and consensus, following the international guidelines.Reliability was determined by means of test-retest and measurement of agreement (Kappa value) between 2 forms of administration of the questionnaire (self-administration and clinical interview). Construct validity was established by analysing its correlation with a sleep diary. RESULTS: A total of 87 families/children participated. The test-retest reliability undertaken in 60 subjects was excellent; r = 0.848 (P<0.001). The kappa value was 0.939 (95% CI: 0.858-1.00, P<0.001).The agreement between BISQ-E and the sleep diary was analysed in 27 families/children, with statistically significant values being obtained for the following variables: bedtime (r = 0.731), hours of night sleep (r = 0.726), hours of daytime sleep (r = 0.867), and number of nocturnal awakenings (r = 0.888) (P < 0.001). CONCLUSIONS: The Spanish adaptation of the BISQ shows overall adequate validity and reliability for the evaluation of sleep in infants and pre-school children. Its use as a clinical tool, or for clinical-epidemiological research studies, is feasible

Knowledge and attitudes toward thirdhand smoke among parents with children under 3 years in Spain.

INTRODUCTION: The knowledge and beliefs about what is thirdhand smoke (THS) are limited. Our objective is to characterize the knowledge and beliefs about THS in parents of children under 3 years old in Spain. METHODS: A cross-sectional study (n = 1406 parents) was conducted online in 2017. We collected information about the knowledge of THS given later, written information with the definition of THS, and asking about beliefs of the effects of THS on children's health. RESULTS: A total of 27% of the respondents had heard about THS. We only found significant differences among smoking status, being the smokers who declare higher knowledge about THS. A total of 86% of the respondents believed that THS is harmful to their children with statistically significant differences according to educational level, higher among parents with a university degree (ORa = 2.6), and according to the previous knowledge on THS (ORa = 2.1). CONCLUSIONS: This is the first study in Europe to describe the knowledge and belief of THS. Around 3 out of 10 parents have heard about THS and more than 8 out of 10 parents believed that THS is harmful to their children. Currently, they were not aware of THS but after providing brief information about it, most of them agreed that THS exposure is harmful to their children.

[Adaptation and study of the measurement properties of a sleep questionnaire for infants and pre-school children]. / Adaptación y estudio de propiedades métricas de un cuestionario de valoración del sueño para lactantes y preescolares.

INTRODUCTION: Although sleep disturbances in infants and toddlers are common, there is no suitable validated tool in Spanish to evaluate sleep disorders. The Brief Infant Sleep Questionnaire (BISQ) is a well-established multidimensional questionnaire widely used internationally. OBJECTIVES: To adapt the BISQ questionnaire to Spanish and analyse its reliability and validity. To explore its feasibility, both in the clinical context, and in epidemiological research. METHODS: Participants were parents of children between 3 and 30 months of age. The adaptation to Spanish (BISQ-E) was achieved by using both back translation and consensus, following the international guidelines.Reliability was determined by means of test-retest and measurement of agreement (Kappa value) between 2forms of administration of the questionnaire (self-administration and clinical interview). Construct validity was established by analysing its correlation with a sleep diary. RESULTS: A total of 87 families/children participated. The test-retest reliability undertaken in 60 subjects was excellent; r = 0.848 (P<0.001). The kappa value was 0.939 (95% CI: 0.858-1.00, P<0.001).The agreement between BISQ-E and the sleep diary was analysed in 27 families/children, with statistically significant values being obtained for the following variables: bedtime (r = 0.731), hours of night sleep (r = 0.726), hours of daytime sleep (r = 0.867), and number of nocturnal awakenings (r = 0.888) (P < 0.001). CONCLUSIONS: The Spanish adaptation of the BISQ shows overall adequate validity and reliability for the evaluation of sleep in infants and pre-school children. Its use as a clinical tool, or for clinical-epidemiological research studies, is feasible.