Efectos del tratamiento con andrógenos sobre la neurocognición en adolescentes transgénero de mujer a hombre / Androgen treatment effects on neurocognition in female-to-male transgender adolescents

Introducción Se ha planteado la hipótesis de que la neurocognición en personas transgénero durante el tratamiento hormonal cruzado podría aproximarse a la del género subjetivo. Sin embargo, la investigación sobre este tema ha producido resultados inconsistentes y, hasta donde sabemos, ningún estudio ha investigado los cambios neurocognitivos en adolescentes transgénero de mujer a hombre (FM) tratados con andrógenos. Sujetos y métodos Quince adolescentes transgénero FM (14-17 años) se sometieron a pruebas neuropsicológicas para examinar los efectos de los andrógenos en sus habilidades visuoespaciales, memoria verbal, velocidad de procesamiento y funciones ejecutivas. Utilizamos un diseño longitudinal en el que se evaluó a 10 participantes dos veces, antes y después de recibir, durante 12 meses, tratamiento con testosterona. Este grupo también se comparó con cinco adolescentes transgénero FM sin tratamiento con andrógenos. Resultados Los participantes evaluados antes y después de 12 meses de tratamiento con andrógenos mejoraron significativamente en velocidad de procesamiento en una tarea visuoespacial (prueba de la figura compleja de Rey-Osterrieth) y en una tarea visual (Stroop), en una tarea de memoria verbal (test de aprendizaje verbal España-Complutense) y en interferencia (Stroop), y exhibieron un menor control de la impulsividad (test de percepción de diferencias revisado). Los adolescentes que recibieron tratamiento con andrógenos mostraron un peor control de la impulsividad cognitiva que los adolescentes que no recibieron tratamiento con andrógenos. Conclusiones Los resultados indican que los andrógenos influyen en la memoria verbal, la interferencia cognitiva, el control de la impulsividad y la velocidad de procesamiento. (AU)

INTRODUCTION It has been hypothesized that cognitive and memory-related brain function in transgender during cross-sex hormonal treatment might be activated towards that of the subjective gender. However, research on this topic has produced inconsistent results, and to the best of our knowledge no studies have investigated neurocognitive changes in androgen-treated female-to-male (FM) transgender adolescents. SUBJECTS AND METHODS A total of 15 FM transgender adolescents (14-17 years) underwent neuropsychological testing in order to examine the effects of androgen on visuo-spacial abilities, verbal memory language, processing speed and executive functions. We used a longitudinal design in which 10 participants were tested twice, before and after receiving 12 months of testosterone treatment. This group was also compared with 5 FM transgender adolescents off-androgen treatment. RESULTS Participants tested before and after 12 months of androgen treatment improved significantly on processing speed in a visuo-spatial (Rey-Osterrieth complex figure test) and in a visuo-oral task (Stroop), their performance on a verbal memory task (TAVEC) and on interference (Stroop) and they exhibited lower impulsivity control (CARAS-R). On-androgen treatment adolescents exhibited worse cognitive impulsivity control than off-androgen treatment adolescents. CONCLUSIONS The results indicate that androgen has an influence on immediate verbal memory, cognitive interference, impulsivity control and processing speed. (AU)

Androgen treatment effects on neurocognition in female-to-male transgender adolescents. / Efectos del tratamiento con andrógenos sobre la neurocognición en adolescentes transgénero de mujer a hombre.

INTRODUCTION: It has been hypothesized that cognitive and memory-related brain function in transgender during cross-sex hormonal treatment might be activated towards that of the subjective gender. However, research on this topic has produced inconsistent results, and to the best of our knowledge no studies have investigated neurocognitive changes in androgen-treated female-to-male (FM) transgender adolescents. SUBJECTS AND METHODS: A total of 15 FM transgender adolescents (14-17 years) underwent neuropsychological testing in order to examine the effects of androgen on visuo-spacial abilities, verbal memory language, processing speed and executive functions. We used a longitudinal design in which 10 participants were tested twice, before and after receiving 12 months of testosterone treatment. This group was also compared with 5 FM transgender adolescents off-androgen treatment. RESULTS: Participants tested before and after 12 months of androgen treatment improved significantly on processing speed in a visuo-spatial (Rey-Osterrieth complex figure test) and in a visuo-oral task (Stroop), their performance on a verbal memory task (TAVEC) and on interference (Stroop) and they exhibited lower impulsivity control (CARAS-R). On-androgen treatment adolescents exhibited worse cognitive impulsivity control than off-androgen treatment adolescents. CONCLUSIONS: The results indicate that androgen has an influence on immediate verbal memory, cognitive interference, impulsivity control and processing speed.

TITLE: Efectos del tratamiento con andrógenos sobre la neurocognición en adolescentes transgénero de mujer a hombre.Introducción. Se ha planteado la hipótesis de que la neurocognición en personas transgénero durante el tratamiento hormonal cruzado podría aproximarse a la del género subjetivo. Sin embargo, la investigación sobre este tema ha producido resultados inconsistentes y, hasta donde sabemos, ningún estudio ha investigado los cambios neurocognitivos en adolescentes transgénero de mujer a hombre (FM) tratados con andrógenos. Sujetos y métodos. Quince adolescentes transgénero FM (14-17 años) se sometieron a pruebas neuropsicológicas para examinar los efectos de los andrógenos en sus habilidades visuoespaciales, memoria verbal, velocidad de procesamiento y funciones ejecutivas. Utilizamos un diseño longitudinal en el que se evaluó a 10 participantes dos veces, antes y después de recibir, durante 12 meses, tratamiento con testosterona. Este grupo también se comparó con cinco adolescentes transgénero FM sin tratamiento con andrógenos. Resultados. Los participantes evaluados antes y después de 12 meses de tratamiento con andrógenos mejoraron significativamente en velocidad de procesamiento en una tarea visuoespacial (prueba de la figura compleja de Rey-Osterrieth) y en una tarea visual (Stroop), en una tarea de memoria verbal (test de aprendizaje verbal España-Complutense) y en interferencia (Stroop), y exhibieron un menor control de la impulsividad (test de percepción de diferencias revisado). Los adolescentes que recibieron tratamiento con andrógenos mostraron un peor control de la impulsividad cognitiva que los adolescentes que no recibieron tratamiento con andrógenos. Conclusiones. Los resultados indican que los andrógenos influyen en la memoria verbal, la interferencia cognitiva, el control de la impulsividad y la velocidad de procesamiento.

Estado nutricional y prevalencia de disfagia en parálisis cerebral infantil. Utilidad del cribado mediante la escala Eating and Drinking Ability Classification System y su relación con el grado de afectación funcional según el Gross Motor Function Classification System / Nutritional status and prevalence of dysphagia in cerebral palsy: Usefulness of the Eating and Drinking Ability Classification System scale and correlation with the degree of motor impairment according to the Gross Motor Function Classification System

Introducción y objetivos: La enfermedad digestiva es una de las comorbilidades más frecuentes en niños con parálisis cerebral infantil (PCI). Nuestro objetivo es analizar el estado nutricional de los pacientes con PCI, la prevalencia de disfagia según la afectación motriz (GMFCS) y su repercusión en la calidad de vida.Material y métodosEstudio descriptivo transversal y abierto en pacientes con PCI seguidos en un hospital terciario de la Comunidad de Madrid mediante una entrevista estructurada y la clasificación de la disfagia según la escala Eating and Drinking Ability Classification System (EDACS). Recogimos datos demográficos y antropométricos y relacionamos el nivel de disfagia con el nivel funcional según el Gross Motor Function Classification System (GMFCS).ResultadosLa muestra incluyó 44 pacientes (65,9% varones), con una edad media de 9,34 ± 5 años y un IMC de 18,5 ± 4,9. El 43% tenía limitaciones en seguridad y/o eficiencia (EDACS > II). El porcentaje de pacientes afectados fue mayor cuanto más extensa desde el punto de vista topográfico fue la PCI (tetraparesia 60%), más variada la semiología clínica (87% en formas mixtas) y peor el nivel funcional (100% en GMFCS V). La repercusión nutricional aumentó con puntuaciones más altas en EDACS y GMFCS.ConclusionesPresentamos el primer estudio sobre la utilidad de la escala EDACS en una muestra representativa de niños y adolescentes españoles con PCI. Los resultados deben hacernos reflexionar sobre la importancia del cribado de disfagia en estos pacientes, independientemente del grado de afectación motriz y la necesidad de una intervención precoz para evitar sus principales consecuencias: desnutrición (hipocrecimiento, déficit de micronutrientes, osteopenia, etc.), microaspiraciones o infecciones de repetición que empeoran el estado neurológico. (AU)

Introduction and objectives: Digestive disorders are one of the most common comorbidities among children with cerebral palsy (CP). The aim of this study is to examine the nutritional status of patients with CP, the prevalence of dysphagia by degree of motor impairment, and the impact of digestive disorders on quality of life.Material and methodsWe conducted a descriptive, cross-sectional, open-label study of outpatients with CP from a tertiary hospital in the Region of Madrid using a structured interview, classifying dysphagia using the Eating and Drinking Ability Classification System (EDACS). We gathered demographical and anthropometric data, and analysed the correlation between severity of dysphagia and functional status as measured with the Gross Motor Function Classification System (GMFCS).ResultsOur sample included 44 patients (65.9% boys), with a mean (standard deviation) age of 9.34 (5) years and a mean BMI of 18.5 (4.9). Forty-three percent presented safety and efficiency limitations (EDACS level > II). Safety and efficiency limitations were associated with more extensive motor involvement (60% had tetraparesis), more varied clinical manifestations (87% had mixed forms) and poorer functional capacity (100% on GMFCS V). The impact on nutritional status increased with higher EDACS and GMFCS scores.ConclusionsThis is the first study into the usefulness of the EDACS scale in a representative sample of Spanish children and adolescents with CP. Our findings underscore the importance of screening for dysphagia in these patients, regardless of the level of motor impairment, and the need for early treatment to prevent the potential consequences: malnutrition (impaired growth, micronutrient deficiencies, osteopaenia, etc.), microaspiration, or recurrent infections that may worsen patients’ neurological status. (AU)

Nutritional status and prevalence of dysphagia in cerebral palsy: usefulness of the Eating and Drinking Ability Classification System scale and correlation with the degree of motor impairment according to the Gross Motor Function Classification System.

INTRODUCTION AND OBJECTIVES: Digestive disorders are one of the most common comorbidities among children with cerebral palsy (CP). The aim of this study is to examine the nutritional status of patients with CP, the prevalence of dysphagia by degree of motor impairment, and the impact of digestive disorders on quality of life. MATERIAL AND METHODS: We conducted a descriptive, cross-sectional, open-label study of out-patients with CP from a tertiary hospital in the Region of Madrid using a structured interview, classifying dysphagia using the Eating and Drinking Ability Classification System (EDACS). We gathered demographical and anthropometric data, and analysed the correlation between severity of dysphagia and functional status as measured with the Gross Motor Function Classification System (GMFCS). RESULTS: Our sample included 44 patients (65.9% boys), with a mean (standard deviation) age of 9.34 (5) years and a mean BMI of 18.5 (4.9). Forty-three percent presented safety and efficiency limitations (EDACS level > II). Safety and efficiency limitations were associated with more extensive motor involvement (60% had tetraparesis), more varied clinical manifestations (87% had mixed forms) and poorer functional capacity (100% on GMFCS V). The impact on nutritional status increased with higher EDACS and GMFCS scores. CONCLUSIONS: This is the first study into the usefulness of the EDACS scale in a representative sample of Spanish children and adolescents with CP. Our findings underscore the importance of screening for dysphagia in these patients, regardless of the level of motor impairment, and the need for early treatment to prevent the potential consequences: malnutrition (impaired growth, micronutrient deficiencies, osteopaenia, etc.), microaspiration, or recurrent infections that may worsen patients' neurological status.

Expanding the Phenotypic Spectrum of Alazami Syndrome: Two Unrelated Spanish Families.

Alazami syndrome is a rare disorder with an autosomal recessive inheritance caused by pathogenic biallelic variants in the LARP7 gene. Clinically, it is mainly characterized by short stature, intellectual disability, and dysmorphic facial features. However, the phenotype is not yet well-defined because less than 50 cases have been described to date. Here, we report three new patients from two unrelated Spanish families who, in addition to the defined features of Alazami syndrome, also exhibit unique features that broaden the phenotypic spectrum of the syndrome. Moreover, we describe the novel frameshift variant c.690_699delins27 in the LARP7 gene, in which loss of function is a known mechanism of Alazami syndrome.

Outcomes from a Spanish Expanded Access Program on cannabidiol treatment in pediatric and adult patients with epilepsy.

AIM: To evaluate the effectiveness and tolerability of cannabidiol (CBD) in patients with developmental and epileptic encephalopathies, including Dravet syndrome (DS), and Lennox-Gastaut syndrome (LGS), in a Spanish Expanded Access Program (EAP). METHODS: This was a multicenter, retrospective, observational study of patients treated with purified CBD in 14 hospitals across Spain. Patients with (1) written informed consent and (2) at least 6 months follow-up before the closure of the database were included. Primary effectiveness endpoints included reductions (100 %, ≥75 %, ≥50 %, ≥25 %, or 0 %) or worsening in seizure frequency (all seizure types and most disabling seizures) at 1-, 3-, 6-, and 12-month visits and at the last visit, and median relative seizure reduction between baseline and last visit. Secondary effectiveness endpoints included retention rate, reduction in seizure severity, status epilepticus, healthcare utilization, and quality of life. Primary safety endpoints included rates of adverse events (AEs) and AEs leading to discontinuation. RESULTS: One hundred and two patients (DS 12 %; LGS 59 %; other epilepsy syndromes 29 %) with a mean age of 15.9 years were enrolled. Patients were highly refractory to antiseizure medications (ASMs); mean number of prior failed ASMs was 7.5 (SD 3.7). The mean CBD dose was 13.0 mg/kg/day at the last visit. The proportion of patients with ≥50 % reduction in the total number of seizures from baseline was 44.9 % at 6 months and 38.9 % at 12 months. The median number of total seizures per month reduced by 47.6 % from baseline to the last visit. At 12 months, seizure severity was lower in 33/54 patients (61.1 %) and unchanged in 17/54 patients (31.5 %). Quality of life, based on the CAVE scale, increased from a mean score of 17.9 ± 4.7 (n = 54) at baseline to 21.7 ± 5.5 (n = 51) at the last patient visit (21.2 % improvement). The mean treatment retention time was 10.3 months. There were no statistically significant changes in the number of status epilepticus episodes, but lower healthcare utilization was observed. Adverse events occurred in sixty-eight patients (66.7 %), and the most common were somnolence (34.3 %) and diarrhea (12.7 %). Cannabidiol was discontinued exclusively due to AEs in 7.8 % of patients, increasing to 25.5 % when both lack of efficacy and AEs were considered together. CONCLUSIONS: Cannabidiol demonstrated promising effectiveness and tolerability in patients with developmental and epileptic encephalopathies taking part in a Spanish EAP.

New therapeutic applications of ozenoxacin in superficial skin infections.

In recent years, the incidence of community- acquired methicillin-resistant S. aureus skin infections (CA-MARSA) has increased in pediatric population without associated risk factors. Ozenoxacin 10mg/g is a topical quinolone that has shown high activity on strains of S. aureus, S. pyogenes and other Gram-positive bacteria sensitive and resistant to methicillin, other quinolones, mupirocin and fusidic acid. Ozenoxacin 10mg/g cream was applied twice a day for 5 days in pediatric patients with superficial skin infections other than non-bullous impetigo where oral antibiotics were not needed. Therapeutic success was achieved in 93.7% of the patients after 5 days of treatment, with a 98.2% decrease in the mean SIRS scale of symptoms. No adverse reaction was reported during treatment. Given the achieved effectiveness, safety, and adherence of the treatment, we believe that pediatricians should consider this topical antibiotic for the treatment of other superficial skin infections, without limiting its use to non-bullous impetigo.

Utilidad de la telemedicina en la atención pediátrica urgente durante la pandemia por COVID-19 / Usefulness of telemedicine in urgent pediatric care during the COVID-19 pandemic

Introducción: nuestro sistema sanitario ha sufrido una reorganización sin precedentes priorizando la atención de los pacientes con sintomatología COVID-19. El uso de telemedicina se presenta como una alternativa útil en la era pos-COVID. El objetivo del estudio fue valorar la utilidad del servicio de mensajería de Twitter como herramienta de telemedicina para el cribado de patología urgente. Material y métodos: estudio descriptivo, retrospectivo y transversal de un programa de telemedicina desarrollado por un equipo de especialistas en Pediatría y sus Áreas Específicas durante el estado de alarma. Se recogieron datos demográficos, número y motivos de consultas según signos, síntomas y su forma de presentación (texto, foto o vídeo). Se analizó el número de consultas resueltas, derivaciones y el grado de satisfacción. Resultados: se atendió un total de 182 consultas realizadas en su mayoría por mujeres (71%), durante las primeras semanas del confinamiento (70%). El 100% fueron mensajes de texto, acompañados casi en un tercio de los casos de material audiovisual (27,2% fotos, 4,6% vídeos). La edad media de los pacientes atendidos fue de 2,72 ± 2,74 y los principales motivos de consulta: fiebre, exantemas y dificultad respiratoria. El 18,13% tuvo relación con la COVID-19, y solamente el 8,24% fue derivado. Conclusiones: aunque la telemedicina no puede reemplazar la valoración presencial y todavía existen limitaciones técnicas y legales, nuestros resultados sugieren que podría ser una alternativa prometedora para mejorar el acceso, reducir los tiempos de triaje, coordinar los recursos disponibles, y disminuir el riesgo de contagio y saturación de las instalaciones sanitarias (AU)

Introduction: our healthcare system has undergone an unprecedented reorganization, prioritizing the care of patients with COVID-19 symptoms. Telemedicine has emerged as a useful alternative in the post-COVID era. The aim of the study was to assess the usefulness of the Twitter® messaging service as a telemedicine tool for the screening of urgent pathology.Material and methods: cross-sectional, retrospective and descriptive study of a telemedicine programme developed by a team of specialists in paediatrics and its subspecialities during the state of alarm. We collected demographic data and the number and reasons for consultations based on the presenting signs and symptoms and how they were conveyed (text, photo and/or video). We analysed the number of resolved concerns, referrals and the degree of user satisfaction.Results: the service managed a total of 182 consultations, mostly made by women (71%) and during the first weeks of the survey (70%). All consultations included text, accompanied in almost 1/3 of the cases by audiovisual content (27.2% photo, 4.6% video). The average age of the managed patients was 2.72 ± 2.74 years and the main reasons for consultation were fever, exanthema and respiratory difficulty. Of all consultations, 18.13% were related to COVID-19, and only 8.24% led to referral.Conclusions: although telemedicine cannot replace face-to-face assessment and there are still technical and legal limitations, our results suggest that it could be a promising alternative to improve access, reduce triage times, coordinate available resources, and decrease the risk of contagion and the saturation of health care facilities. (AU)

Impacto del tratamiento con metilfenidato sobre las propiedades funcionales y estructurales del ventrículo izquierdo a medio plazo en el trastorno por déficit de atención e hiperactividad / The impact of methylphenidate treatment on the functional and structural properties of the left ventricle: A medium-term prospective study

Introducción: El metilfenidato (MTF) es un psicoestimulante que aumenta frecuencia cardiaca (FC) y presión arterial (PA), lo cual a largo plazo podría modificar la geometría del ventrículo izquierdo (VI) y alterar sus propiedades funcionales, principalmente la diastólica. Material y métodos: Estudio prospectivo, longitudinal y comparativo tipo caso-caso en niños y adolescentes sanos diagnosticados de trastorno por déficit de atención e hiperactividad (TDAH) tratados con MTF durante 3 años. Se valoró pre y postratamiento PAS/PAD, geometría ventricular, función sistólica y diastólica. Resultados: Incluimos a 112 pacientes, completando 73. El 75,3% varones, entre 4-15 años (9±2,6), con índice de masa corporal de 18,27±3,75 y una dosis media de MTF de 0,9±0,17mg/kg/día. Objetivamos aumento de PAS/PAD de 3,7±9mmHg (p = 0,004) y 2±11,5mmHg, respectivamente. No tuvimos ningún evento cardiovascular grave, cambios estructurales ni variación en los parámetros de función sistólica estudiados. Sin embargo, encontramos un aumento discreto, pero progresivo y significativo del tiempo de relajación isovolumétrica del VI (p = 0,046) y del tiempo de desaceleración (p = 0,016) indicativos de alteración en la relajación. No observamos variación en los parámetros relacionados con la distensibilidad ni con las presiones diastólicas tempranas y ningún paciente cumplió criterios de disfunción diastólica. Conclusiones: El incremento de PAS/PAD y las alteraciones de la relajación del VI objetivadas podrían ser un indicador precoz de una posible disfunción diastólica y riesgo cardiovascular a largo plazo.(AU)

Introduction: Although methylphenidate (MPH) used for treatment of Attention deficit hyperactivity disorder (ADHD) are considered safe in healthy children and adolescents in the short and medium term, there is a widespread concern about long-term cardiovascular safety. Material and methods: Interventional, prospective, longitudinal and comparative study with a crossover design to evaluate the cardiovascular impact of the treatment with MPH in healthy children and adolescents diagnosed with ADHD. A protocol for the cardiovascular evaluation was established at a basal point, after the first and the second year of the beginning with treatment based on the monitoring of Blood pressure (BP) and echocardiographic follow-up of the systolic and diastolic functions, and structural cardiac properties. Results: 73 patients completed the study, with an average age of 9+/- 2.6 years, 75.3% were male and the majority were thin (64.4%). We found an increase in Systolic and Diastolic BP of 3.7±9mmHg (P).004) and 2±11,5mmHg respectively. There were no severe cardiovascular events. We didn’t find any echocardiographic alterations namely on the structural properties or parameters of systolic function. Regarding diastolic function, a significant increase in the isovolumic relaxation time (IVRT) (P=.046) and deceleration time (P=.016) was observed. However, no significant alterations in the parameters related to distensibility of the LV neither in the early diastolic pressure were found. Conclusion: Further studies are needed to evaluate the impact of psychostimulants as a modifiable long-term Cardiovascular Risk Factor.(AU)

The impact of methylphenidate treatment on the functional and structural properties of the left ventricle: A medium-term prospective study.

INTRODUCTION: Although methylphenidate (MPH) used for treatment of attention deficit hyperactivity disorder (ADHD) are considered safe in healthy children and adolescents in the short and medium term, there is a widespread concern about long-term cardiovascular safety. MATERIAL AND METHODS: Interventional, prospective, longitudinal and comparative study with a crossover design to evaluate the cardiovascular impact of the treatment with MPH in healthy children and adolescents diagnosed with ADHD. A protocol for the cardiovascular evaluation was established at a basal point, after the first and the second year of the beginning with treatment based on the monitoring of blood pressure (BP) and echocardiographic follow-up of the systolic and diastolic functions, and structural cardiac properties. RESULTS: 73 patients completed the study, with an average age of 9 ±â€¯2.6 years, 75.3% were male and the majority were thin (64.4%). We found an increase in Systolic and Diastolic BP of 3.7 ±â€¯9 mmHg (P = 0.004) and 2 ±â€¯11,5 mmHg respectively. There were no severe cardiovascular events. We didn't find any echocardiographic alterations namely on the structural properties or parameters of systolic function. Regarding diastolic function, a significant increase in the isovo-lumic relaxation time (IVRT) (P = 0.046) and deceleration time (P = 0.016) was observed. However, no significant alterations in the parameters related to distensibility of the LV neither in the early diastolic pressure were found. CONCLUSION: Further studies are needed to evaluate the impact of psychostimulants as a modifiable long-term Cardiovascular Risk Factor.

Effects of a Novel Infant Formula on Weight Gain, Body Composition, Safety and Tolerability to Infants: The INNOVA 2020 Study.

Exclusive breastfeeding is recommended for the first six months of life to promote adequate infant growth and development, and to reduce infant morbidity and mortality. However, whenever some mothers are not able to breastfeed their infants, infant formulas mimicking human milk are needed, and the safety and efficacy of each formula should be tested. Here, we report the results of a multicenter, randomized, blinded, controlled clinical trial that aimed to evaluate a novel starting formula on weight gain and body composition of infants up to 6 and 12 months, as well as safety and tolerability. For the intervention period, infants were divided into three groups: group 1 received formula 1 (Nutribén® Innova 1 (Alter Farmacia S.A., Madrid, Spain) or INN (n = 70)), with a lower amount of protein, a lower casein to whey protein ratio by increasing the content of α-lactalbumin, and a double amount of docosahexaenoic acid/arachidonic acid than the standard formula; it also contained a thermally inactivated postbiotic (Bifidobacterium animalis subsp. lactis, BPL1TM HT). Group 2 received the standard formula or formula 2 (Nutriben® Natal (Alter Farmacia S.A., Madrid, Spain) or STD (n = 70)) and the third group was exclusively breastfed for exploratory analysis and used as a reference (BFD group (n = 70)). During the study, visits were made at 21 days and 2, 4, 6, and 12 months of age. Weight gain was higher in both formula groups than in the BFD group at 6 and 12 months, whereas no differences were found between STD and INN groups either at 6 or at 12 months. Likewise, body mass index was higher in infants fed the two formulas compared with the BFD group. Regarding body composition, length, head circumference and tricipital/subscapular skinfolds were alike between groups. The INN formula was considered safe as weight gain and body composition were within the normal limits, according to WHO standards. The BFD group exhibited more liquid consistency in the stools compared to both formula groups. All groups showed similar digestive tolerance and infant behavior. However, a higher frequency of gastrointestinal symptoms was reported by the STD formula group (n = 291), followed by the INN formula (n = 282), and the BFD groups (n = 227). There were fewer respiratory, thoracic, and mediastinal disorders among BFD children. Additionally, infants receiving the INN formula experienced significantly fewer general disorders and disturbances than those receiving the STD formula. Indeed, atopic dermatitis, bronchitis, and bronchiolitis were significantly more prevalent among infants who were fed the STD formula compared to those fed the INN formula or breastfed. To evaluate whether there were significant differences between formula treatments, beyond growth parameters, it would seem necessary to examine more precise health biomarkers and to carry out long-term longitudinal studies.

Foramina parietalia permagna: evaluación clínico radiológica de una familia española con mutación no descrita en el gen ALX4 / Foramina parietalia permagna: Clinical radiological evaluation of a Spanish family with an undescribed mutation in the Alx4 gene

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Chorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting.

BACKGROUND: Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF. CASE PRESENTATION: A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs. CONCLUSIONS: SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.

[The impact of methylphenidate treatment on the functional and structural properties of the left ventricle: A medium-term prospective study]. / Impacto del tratamiento con metilfenidato sobre las propiedades funcionales y estructurales del ventrículo izquierdo a medio plazo en el trastorno por déficit de atención e hiperactividad.

INTRODUCTION: Although methylphenidate (MPH) used for treatment of Attention deficit hyperactivity disorder (ADHD) are considered safe in healthy children and adolescents in the short and medium term, there is a widespread concern about long-term cardiovascular safety. MATERIAL AND METHODS: Interventional, prospective, longitudinal and comparative study with a crossover design to evaluate the cardiovascular impact of the treatment with MPH in healthy children and adolescents diagnosed with ADHD. A protocol for the cardiovascular evaluation was established at a basal point, after the first and the second year of the beginning with treatment based on the monitoring of Blood pressure (BP) and echocardiographic follow-up of the systolic and diastolic functions, and structural cardiac properties. RESULTS: 73 patients completed the study, with an average age of 9+/- 2.6 years, 75.3% were male and the majority were thin (64.4%). We found an increase in Systolic and Diastolic BP of 3.7±9mmHg (P).004) and 2±11,5mmHg respectively. There were no severe cardiovascular events. We didn't find any echocardiographic alterations namely on the structural properties or parameters of systolic function. Regarding diastolic function, a significant increase in the isovolumic relaxation time (IVRT) (P=.046) and deceleration time (P=.016) was observed. However, no significant alterations in the parameters related to distensibility of the LV neither in the early diastolic pressure were found. CONCLUSION: Further studies are needed to evaluate the impact of psychostimulants as a modifiable long-term Cardiovascular Risk Factor.

Single stick access using a VA-ECMO arterial return cannula for coronary intervention in cardiogenic shock.

Use of veno-arterial extracorporeal membrane oxygenation (VA-ECMO) is growing exponentially for cardiogenic shock and cardiac arrest, and many of these patients require percutaneous coronary intervention (PCI). In some cases, radial arterial access may not feasible among patients with peripheral vascular disease or if larger diameter guide catheters are required. Further, VA-ECMO is commonly used in combination with an intra-aortic balloon pump or Impella, thereby limiting vascular access options and increasing the risk of vascular complications including bleeding and limb ischemia. For these reasons, new approaches to perform PCI without the need for an additional arterial puncture are required. We describe a case of a 70-year-old man with cardiogenic shock referred for high-risk PCI while supported with VA-ECMO and an Impella CP and illustrate a novel method for single-stick access for PCI through the return cannula of the VA-ECMO circuit.

Impacto psicológico del confinamiento en población infantil y cómo mitigar sus efectos: revisión rápida de la evidencia / Sychological impact of lockdown (confinement) on young children and how to mitigate its effects: rapid review of the evidence

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Nutritional status and prevalence of dysphagia in cerebral palsy: Usefulness of the Eating and Drinking Ability Classification System scale and correlation with the degree of motor impairment according to the Gross Motor Function Classification System. / Estado nutricional y prevalencia de disfagia en parálisis cerebral infantil. Utilidad del cribado mediante la escala Eating and Drinking Ability Classification System y su relación con el grado de afectación funcional según el Gross Motor Function Classification System.

INTRODUCTION AND OBJECTIVES: Digestive disorders are one of the most common comorbidities among children with cerebral palsy (CP). The aim of this study is to examine the nutritional status of patients with CP, the prevalence of dysphagia by degree of motor impairment, and the impact of digestive disorders on quality of life. MATERIAL AND METHODS: We conducted a descriptive, cross-sectional, open-label study of outpatients with CP from a tertiary hospital in the Region of Madrid using a structured interview, classifying dysphagia using the Eating and Drinking Ability Classification System (EDACS). We gathered demographical and anthropometric data, and analysed the correlation between severity of dysphagia and functional status as measured with the Gross Motor Function Classification System (GMFCS). RESULTS: Our sample included 44 patients (65.9% boys), with a mean (standard deviation) age of 9.34 (5) years and a mean BMI of 18.5 (4.9). Forty-three percent presented safety and efficiency limitations (EDACS level>II). Safety and efficiency limitations were associated with more extensive motor involvement (60% had tetraparesis), more varied clinical manifestations (87% had mixed forms) and poorer functional capacity (100% on GMFCS V). The impact on nutritional status increased with higher EDACS and GMFCS scores. CONCLUSIONS: This is the first study into the usefulness of the EDACS scale in a representative sample of Spanish children and adolescents with CP. Our findings underscore the importance of screening for dysphagia in these patients, regardless of the level of motor impairment, and the need for early treatment to prevent the potential consequences: malnutrition (impaired growth, micronutrient deficiencies, osteopaenia, etc.), microaspiration, or recurrent infections that may worsen patients' neurological status.