Quality of life in adolescents with narcolepsy type 1- a transversal study in a tertiary hospital.

PURPOSE: - Narcolepsy type 1 (NT1) is a rare chronic sleep disorder, usually arising by adolescence that negatively impacts quality of life. It is characterized by excessive daytime sleepiness (EDS), cataplexy, hypnagogic/hypnopompic hallucinations, sleep paralysis and sleep fragmentation. The goals of this work were to characterize NT1 adolescents regarding sleep characteristics, health-related quality of life (HRQoL) and future life perspectives and later to compare this group with a control group of healthy adolescents (HA). METHODS: - Transversal descriptive/analytical study including NT1 patients followed in a sleep center of a tertiary hospital and 23 HA. Data were collected through an online survey, fulfilled by the participants, including four sections: demographics; questionnaire evaluating sleep and EDS; questionnaire evaluating HRQoL; inquiry regarding future perspectives. An extra section for the NT1 group only, comprising questions about the characterization of narcolepsy, was included. RESULTS: 22 NT1 adolescents were included, with a median age of 15.0 years-old. Beyond EDS, all had presented cataplexy - 19 still reported it. Twenty patients took psychostimulants regularly for EDS, while 13 patients took venlafaxine or fluoxetine for cataplexy. Nineteen adolescents took regular naps and 19 maintained psychological appointments. Self-reported sleep quality was similar between groups (p = 0.112). EDS was identified in seven NT1 patients and none in the control group. HRQOL was significantly lower in NT1 patients only for the physical well-being domain (p = 0.001). Regarding future perspectives, results were similar, except for a lower probability of getting a driver's license in NT1 patients, despite no statistical significance (p = 0.104). DISCUSSION: Daytime sleepiness is difficult to control in NT1, despite specialized treatment. HRQoL was similar between groups in all domains except for the physical well-being. Despite good adherence to pharmacological and non-pharmacological treatments (namely psychological therapy) that account for these good results, the physical well-being domain is difficult to manage.

A rare case of tongue biting during sleep in childhood.

Bleeding tongue-biting episodes during sleep are a rare and alarming situation that can negatively impact the child's and parents' sleep, affecting their quality of life. Although highly suggestive of epilepsy, a differential diagnosis should be made with sleep-related movement disorders such as bruxism, hypnic myoclonus, facio-mandibular myoclonus, and geniospasm when this hypothesis is excluded. The clinical history, electroencephalogram, and video-polysomnography are essential for diagnostic assessment. Treatment with clonazepam can be necessary in the presence of frequent tongue biting that causes severe injuries and sleep disturbance. This study reports the challenging case of managing and diagnosing a 2-year-old boy with recurrent tongue biting during sleep since he was 12 months old, causing bleeding lacerations, frequent awakenings, and significant sleep impairment with daytime consequences for him and his family. CITATION: Cascais I, Ashworth J, Ribeiro L, Freitas J, Rios M. A rare case of tongue biting during sleep in childhood. J Clin Sleep Med. 2024;20(4):653-656.

Relación entre los síntomas de déficit de atención/hiperactividad y el sueño en preescolares / Association between attention-deficit/hyperactivity symptoms and sleep in preschoolers

Introducción: Los problemas de sueño son frecuentes en niños con trastorno por déficit de atención/hiperactividad (TDAH). Algunos autores han tratado de caracterizar los hábitos de sueño pediátricos en Portugal, pero ninguno se ha centrado en los niños en edad preescolar ni ha intentado correlacionarlos con el TDAH. El objetivo fue evaluar la prevalencia de los síntomas del TDAH en niños en edad preescolar y estudiar su asociación con los hábitos de sueño. Material y métodos: Estudio transversal mediante la administración de un cuestionario a una muestra aleatoria de cuidadores de niños matriculados en guarderías en Oporto. Se recogieron datos de características sociodemográficas, consumo de televisión y actividades al aire libre. Los síntomas del TDAH y los hábitos de sueño fueron evaluados mediante las versiones portuguesas del Conners’ Parents Rating Scale-Revised y el Children's Sleep Habits Questionnaire (CSHQ-PT), respectivamente. Resultados: El estudio incluyó 381 preescolares (50,90% varones). Se encontraron niveles altos de síntomas de TDAH en el 13,10%, con una prevalencia mayor en las niñas (14,40% vs. 11,85%; p=0,276). El 45,70% tenían una puntuación total en el CSQH-PT superior a 48, que es el punto de corte establecido para el cribado de los trastornos del sueño en la población portuguesa. Se encontró una asociación significativa entre niveles altos de síntomas de TDAH y un nivel educativo materno más bajo (p<0,001), una menor duración del sueño (p=0,049) y mayores puntuaciones en las subescalas de parasomnias (p=0,019) y de trastornos respiratorios del sueño (p=0,002). (AU)

Introduction: Sleep problems are frequent in children with attention-deficit/hyperactivity disorder (ADHD). Some authors have tried to characterize paediatric sleep habits in Portugal, but none has focused on preschool-age children nor attempted to establish their association with ADHD. We aimed to assess the prevalence of ADHD symptoms in preschool-age children and to study their association with sleep habits. Material and methods: We conducted a cross-sectional study. We distributed questionnaires to a random sample of caregivers of children enrolled in early childhood education centres in Porto. We collected data on sociodemographic characteristics, television watching and outdoor activities. We assessed ADHD symptoms and sleep habits with the Portuguese versions of the Conners’ Parents Rating Scale, Revised and the Children's Sleep Habits Questionnaire (CSHQ-PT), respectively. Results: The study included 381 preschoolers (50.90% male). We found high scores for ADHD symptoms in 13.10%, with a higher prevalence in girls (14.40% vs. 11.85%; P=.276). In the CSHQ-PT, 45.70% of participants had a mean total score greater than 48, which is the cut-off point applied in the screening of sleep disturbances in the Portuguese population. There was a significant association between high scores for ADHD symptoms and a lower maternal education level (P<.001), a shorter sleep duration (P=.049), and higher scores on parasomnias (P=.019) and sleep disordered breathing (P=.002) in CSHQ-PT subscales. (AU)

Association between attention-deficit/hyperactivity symptoms and sleep in preschoolers.

INTRODUCTION: Sleep problems are frequent in children with attention-deficit/hyperactivity disorder (ADHD). Some authors have tried to characterize paediatric sleep habits in Portugal, but none has focused on preschool-age children nor attempted to establish their association with ADHD. We aimed to assess the prevalence of ADHD symptoms in preschool-age children and to study their association with sleep habits. MATERIAL AND METHODS: We conducted a cross-sectional study. We distributed questionnaires to a random sample of caregivers of children enrolled in early childhood education centres in Porto. We collected data on sociodemographic characteristics, television watching and outdoor activities. We assessed ADHD symptoms and sleep habits with the Portuguese versions of the Conners' Parents Rating Scale, Revised and the Children's Sleep Habits Questionnaire (CSHQ-PT), respectively. RESULTS: The study included 381 preschoolers (50.90% male). We found high scores for ADHD symptoms in 13.10%, with a higher prevalence in girls (14.40% vs. 11.85%; P = 0.276). In the CSHQ-PT, 45.70% of participants had a mean total score greater than 48, which is the cut-off point applied in the screening of sleep disturbances in the Portuguese population. There was a significant association between high scores for ADHD symptoms and a lower maternal education level (P < 0.001), a shorter sleep duration (P = 0.049), and higher scores on parasomnias (P = 0.019) and sleep disordered breathing (P = 0.002) in CSHQ-PT subscales. CONCLUSIONS: ADHD and sleep disorders are common in preschoolers, in Porto, and this study suggests some clinical correlations between them. Since these interactions are complex and far from being elucidated, further studies are paramount to provide guidance for prevention and managing strategies in younger children at risk for ADHD.

First and second waves of coronavirus disease-19: A comparative study in hospitalized patients in Reus, Spain.

Many countries have seen a two-wave pattern in reported cases of coronavirus disease-19 during the 2020 pandemic, with a first wave during spring followed by the current second wave in late summer and autumn. Empirical data show that the characteristics of the effects of the virus do vary between the two periods. Differences in age range and severity of the disease have been reported, although the comparative characteristics of the two waves still remain largely unknown. Those characteristics are compared in this study using data from two equal periods of 3 and a half months. The first period, between 15th March and 30th June, corresponding to the entire first wave, and the second, between 1st July and 15th October, corresponding to part of the second wave, still present at the time of writing this article. Two hundred and four patients were hospitalized during the first period, and 264 during the second period. Patients in the second wave were younger and the duration of hospitalization and case fatality rate were lower than those in the first wave. In the second wave, there were more children, and pregnant and post-partum women. The most frequent signs and symptoms in both waves were fever, dyspnea, pneumonia, and cough, and the most relevant comorbidities were cardiovascular diseases, type 2 diabetes mellitus, and chronic neurological diseases. Patients from the second wave more frequently presented renal and gastrointestinal symptoms, were more often treated with non-invasive mechanical ventilation and corticoids, and less often with invasive mechanical ventilation, conventional oxygen therapy and anticoagulants. Several differences in mortality risk factors were also observed. These results might help to understand the characteristics of the second wave and the behaviour and danger of SARS-CoV-2 in the Mediterranean area and in Western Europe. Further studies are needed to confirm our findings.

Prolonged use of nimotuzumab in children with central nervous system tumors: safety and feasibility.

Primary brain tumors constitute the most frequent solid tumor of childhood. High expression of the epidermal growth factor receptor (EGFR) protein has been associated with tumor progression and enhanced tumorigenicity in adult and children gliomas. Nimotuzumab is a humanized antibody that targets the EGFR and has proven efficacy in adult and children gliomas. To provide a new therapeutic option for patients with active, poor prognosis central nervous system (CNS) tumors and to evaluate the feasibility and safety of long-term nimotuzumab therapy in children with diverse CNS tumors, an expanded access program was launched at the Juan Manuel Marquez hospital. Patients were required to be 18 or younger and have one CNS tumor: low-grade glioma (LGG) or high-grade glioma (HGG), brainstem glioma (BSG), ependymoma or primitive neuroectodermal tumor (PNET), and a Lansky or Karnofsky performance status ≥40. Treatment consisted of weekly nimotuzumab administered at 150 mg/m(2) for 12 weeks, continuing every 14 days in the absence of severe condition worsening or unacceptable toxicity. Nimotuzumab was administered alone or in combination with radiotherapy, chemotherapy, or both, depending on the tumor type, stage, and previous treatment. Eighty-eight patients, 39 with BSG, 25 with HGG, 9 with progressive LGG, 9 with anaplastic ependymomas, and 6 with other tumor types, including PNET, neuroblastoma, meduloblastoma, and thalamic tumors, were treated with the antibody. The mean number of nimotuzumab doses was 36, from 1 to 108. The most frequent adverse events were mild to moderate skin rash, mucositis, vomiting, seizures, hypothermia, hyperthermia, and paleness. One patient had a grade 3 mucositis, while the other had a grade 3 bleeding on surgery. Sixteen children stopped treatment after at least 2 years with stable disease, partial or complete response. All children were able to maintain the best response achieved on treatment after a 3-year interruption. In summary, this study shows the feasibility of very prolonged administration of nimotuzumab together with the lack of rebound effect after treatment cessation.

Volkmann ischemic contracture in a newborn.

Congenital Volkmann ischemic contracture is a very rare condition in which a neonate presents skin, muscular and nerve lesions due to increased intracompartment pressure and subsequent ischemia, probably due to extrinsic intrauterine compression. In this age group, there are only about 50 reported cases and a specific cause is unknown. The authors describe the case of a newborn who presented with bullous and ulcerated skin lesions and nerve palsy of his forearm at birth, evolving to subcutaneous and muscular necrosis and contracture. Two surgeries were performed and the baby began a daily physiotherapy program that resulted in aesthetical improvement and recovery of his hand and forearm mobility. Early recognition of this rare entity and subsequent emergency fasciotomy are the best ways to improve prognosis.

Conocimientos y percepción del riesgo de las infecciones de transmisión sexual en profesores generales integrales / Knowledges and perceptions of the integral general professors on sexually transmitted diseases risk

INTRODUCCIÓN: las infecciones de transmisión sexual constituyen peligros importantes para la salud humana en el nuevo milenio, y confluyen en la adolescencia, por lo que los jóvenes son los más vulnerables porque son los que se exponen con más frecuencia a los factores o conductas de riesgo. OBJETIVO: explorar el nivel de conocimientos básicos acerca de las infecciones de transmisión sexual en profesores generales integrales. MÉTODOS: estudio prospectivo, descriptivo de corte transversal, en profesores generales integrales de 2 secundarias básicas, cuyas edades oscilaban entre 17 y 24 años, que laboran en el municipio 10 de Octubre, Ciudad de La Habana, en el curso escolar 2006-2007. De un universo de 90 profesores se escogieron 50 por una fórmula estadística para considerarla muestra probabilística. Se realizó una encuesta validada, que llenaron previo consentimiento informado. Los datos de la encuesta se introdujeron en una base de datos y luego se expusieron en forma de tablas. RESULTADOS: aún persiste falta de conocimientos básicos por parte de los encuestados acerca de los síntomas y vías de transmisión, la percepción de riesgo y la vulnerabilidad de la mujer a estas infecciones. Se evidencia que casi más de las tres cuartas partes, aunque identifican los criterios de riesgo para adquirirlas, todavía presentan concepciones erróneas. Las tres cuartas partes de nuestros encuestados no reconocen que los portadores pueden transmitir el VIH. CONCLUSIONES: los profesores generales integrales presentan dificultades al identificar algunas de las infecciones de transmisión sexual, persisten errores con respecto a la vía de transmisión, dificultades en precisar los síntomas clínicos de estas enfermedades, problemas en percibir las conductas de riesgo y poco dominio sobre la transmisión de la enfermedad(AU)

INTRODUCTION: sexually transmitted diseases are significant dangers for human health in the new millennium and converge in adolescence that is why the young people arte the more vulnerable by their more frequent exposition to risk factors and behaviors. AIM: to explore at basic knowledges level of integral general professors on sexually transmitted diseases from two high schools aged between 17 and 24 working in 10 de Octubre municipality, Ciudad de La Habana during 2006-2007 school course. From an universe 90 professors we selected 50 according to a statistic formula as probabilistic sample. We applied a validated survey with a previous informed consent. Survey data were entered in a database and then were exposed in table models. RESULTS: still there is a lack of basic knowledges by polled person on symptoms and transmission routes, the risk perception, and female vulnerability to these diseases. It is demonstrate that the three-quarter, although they identify the risk criteria of acquisition, yet they had mistaken concepts. The three-quarter of our polled persons don't recognize that carriers may to transmit the HIV. CONCLUSIONS: general integral professors have difficulties to identify some sexually transmitted diseases; there are errors as regard to transmission route, difficulties to specify exactly the clinical symptoms of these diseases, the problems to notice the risked behaviors, and not much control of disease transmission(AU)

Conocimientos y percepción del riesgo de las infecciones de transmisión sexual en profesores generales integrales / Knowledges and perceptions of the integral general professors on sexually transmitted diseases risk

INTRODUCCIÓN: las infecciones de transmisión sexual constituyen peligros importantes para la salud humana en el nuevo milenio, y confluyen en la adolescencia, por lo que los jóvenes son los más vulnerables porque son los que se exponen con más frecuencia a los factores o conductas de riesgo. OBJETIVO: explorar el nivel de conocimientos básicos acerca de las infecciones de transmisión sexual en profesores generales integrales. MÉTODOS: estudio prospectivo, descriptivo de corte transversal, en profesores generales integrales de 2 secundarias básicas, cuyas edades oscilaban entre 17 y 24 años, que laboran en el municipio 10 de Octubre, Ciudad de La Habana, en el curso escolar 2006-2007. De un universo de 90 profesores se escogieron 50 por una fórmula estadística para considerarla muestra probabilística. Se realizó una encuesta validada, que llenaron previo consentimiento informado. Los datos de la encuesta se introdujeron en una base de datos y luego se expusieron en forma de tablas. RESULTADOS: aún persiste falta de conocimientos básicos por parte de los encuestados acerca de los síntomas y vías de transmisión, la percepción de riesgo y la vulnerabilidad de la mujer a estas infecciones. Se evidencia que casi más de las tres cuartas partes, aunque identifican los criterios de riesgo para adquirirlas, todavía presentan concepciones erróneas. Las tres cuartas partes de nuestros encuestados no reconocen que los portadores pueden transmitir el VIH. CONCLUSIONES: los profesores generales integrales presentan dificultades al identificar algunas de las infecciones de transmisión sexual, persisten errores con respecto a la vía de transmisión, dificultades en precisar los síntomas clínicos de estas enfermedades, problemas en percibir las conductas de riesgo y poco dominio sobre la transmisión de la enfermedad...

INTRODUCTION: sexually transmitted diseases are significant dangers for human health in the new millennium and converge in adolescence that is why the young people arte the more vulnerable by their more frequent exposition to risk factors and behaviors. AIM: to explore at basic knowledges level of integral general professors on sexually transmitted diseases from two high schools aged between 17 and 24 working in 10 de Octubre municipality, Ciudad de La Habana during 2006-2007 school course. From an universe 90 professors we selected 50 according to a statistic formula as probabilistic sample. We applied a validated survey with a previous informed consent. Survey data were entered in a database and then were exposed in table models. RESULTS: still there is a lack of basic knowledges by polled person on symptoms and transmission routes, the risk perception, and female vulnerability to these diseases. It is demonstrate that the three-quarter, although they identify the risk criteria of acquisition, yet they had mistaken concepts. The three-quarter of our polled persons don't recognize that carriers may to transmit the HIV. CONCLUSIONS: general integral professors have difficulties to identify some sexually transmitted diseases; there are errors as regard to transmission route, difficulties to specify exactly the clinical symptoms of these diseases, the problems to notice the risked behaviors, and not much control of disease transmission...