ABSTRACT
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Subject(s)
Humans , Child , Humanization of Assistance , Critical Care/methods , Child, Hospitalized/psychology , Intensive Care Units, Pediatric/organization & administration , Visitors to Patients , Professional-Family Relations , Continuity of Patient Care/organization & administration , Hospital-Patient RelationsABSTRACT
Introducción: La ventilación mecánica domiciliaria (VMD) es una técnica cada vez más frecuente en el niño. Existen pocos estudios que hayan analizado las características y necesidades de los niños sometidos a esta técnica. Material y métodos: Estudio descriptivo observacional transversal multicéntrico de pacientes entre un mes y 16 años dependientes de ventilación mecánica domiciliaria. Resultados: Se estudiaron 163 pacientes de 17 hospitales españoles con una edad media de 7,6 años. La causa más frecuente de VMD fueron los trastornos neuromusculares. El inicio de la VMD fue a una edad media de 4,6 años. Un 71,3% recibieron ventilación no invasiva. Los pacientes con ventilación invasiva tenían menor edad, menor edad de inicio de la VMD y mayor tiempo de uso diario. El 80,9% precisaban VM solo durante el sueño, y un 11,7% durante todo el día. Únicamente un 3,4% de los pacientes tiene asistencia sanitaria externa como ayuda a la familia. Un 48,2% es controlado en consultas específicas de VMD o consultas multidisciplinares. Un 72,1% de los pacientes está escolarizado (recibiendo enseñanza adaptada un 42,3%). Solo un 47,8% de los pacientes escolarizados cuentan con cuidadores específicos en su centro escolar. Conclusiones: La VMD en niños se utiliza en un grupo muy heterogéneo de pacientes iniciándose en un importante porcentaje en los primeros 3 años de vida. A pesar de que un significativo porcentaje de pacientes tiene una gran dependencia de la VMD pocas familias cuentan con ayudas específicas tanto a nivel escolar como en el domicilio, y el seguimiento sanitario es heterogéneo y poco coordinado(AU)
Introduction: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. Materials and methods: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. Results: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. Conclusions: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated(AU)
Subject(s)
Humans , Male , Female , Child , Respiration, Artificial , Assisted Living Facilities/methods , Respiratory Insufficiency/therapy , Tracheostomy , Neuromuscular Diseases/complicationsABSTRACT
INTRODUCTION: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. MATERIALS AND METHODS: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. RESULTS: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. CONCLUSIONS: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated.
Subject(s)
Home Care Services , Respiration, Artificial , Adolescent , Child , Child, Preschool , Cross-Sectional Studies , Female , Humans , Infant , Male , SpainABSTRACT
La distrofia torácica asfixiante, o síndrome de Jeune, es una displasia ósea de herencia autosómica recesiva, con expresión fenotípica variable. El diagnóstico es fundamentalmente clínico y radiológico. Se caracteriza por la presencia de un tórax estrecho y acampanado, polidactilia, costillas horizontalizadas y huesos iliacos cortos, con una alteración típica del techo acetabular en tridente, y suele asociar otras complicaciones: nefrocalcinosis, hepatopatía colestásica, anomalías pancreáticas y retinianas. La distrofia torácica produce hipoplasia pulmonar secundaria e insuficiencia respiratoria restrictiva que puede ser mortal en etapas precoces de la vida. Aunque la existencia de malformaciones espinales no es un hallazgo frecuente, en este caso, el paciente presentaba una malformación de C1 que producía compresión medular. Se realizó una descompresión quirúrgica y, posteriormente, la evolución del paciente ha sido muy favorable. Concluimos que debe realizarse siempre el cribado de malformaciones espinales en pacientes afectados de este síndrome, ya que su tratamiento puede contribuir significativamente a mejorar su pronóstico y calidad de vida(AU)
Asphyxiating thoracic dystrophy-Jeune syndrome is an autosomal recessive disease. It is part of a group of skeletal dysplasias with great clinical variability. Diagnosis is based on clinical and radiographic findings: the syndrome is characterized by a small, narrow chest, variable limb shortness, postaxial polydactyl, horizontally oriented ribs, and short iliac bones with a typical trident appearance of the acetabula. Renal, hepatic, pancreatic and ocular complications may occur later in life. As result of the thoracic dystrophy a lung hypoplasia is produced and a lethal respiratory distress during neonatal and infancy period may occur as well as multiple respiratory infections. Despite spine malformations are not frequently associated, we present a patient who had C1 malformation with cord compression. Surgical cord decompression was done and probably was determinant in his course. We concluded that the screening of spinal malformation in these patients should be always done because it treatment could improve their prognosis and quality of life(AU)
Subject(s)
Humans , Male , Infant, Newborn , Bone Diseases, Developmental/complications , Chromosome Aberrations , Muscle Hypotonia/complications , Respiratory Insufficiency/complications , Spine/abnormalitiesSubject(s)
Renal Insufficiency/complications , Respiratory Insufficiency/complications , Respiratory Insufficiency/pathology , Stevens-Johnson Syndrome/complications , Ureter/pathology , Child, Preschool , Fatal Outcome , Female , Humans , Lung/pathology , Respiration, Artificial , Respiratory Insufficiency/therapyABSTRACT
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Subject(s)
Child, Preschool , Infant, Newborn , Female , Humans , Stevens-Johnson Syndrome , Ureter , Renal Insufficiency , Fatal Outcome , Respiration, Artificial , Respiratory Insufficiency , Hyperglycinemia, Nonketotic , LungABSTRACT
We report a 1-year-old girl with acute abdominal pain. Clinical examination revealed distended abdomen with increased intestinal peristalsis without other pathologic signs. Ultrasound of the abdomen showed a small bowel intussusception. Seven days later, after a new episode of sudden acute abdominal pain, ultrasound revealed a new bowel intussusception. Ig A and Ig G antigliadin-antibodies were elevated and intestinal biopsy revealed total villous atrophy. After being placed on a gluten-free diet the girl was free of colicky complaints and on repeated ultrasound there was no sign of intussusception. With recurrent intussusception, celiac disease should be considered as a cause.
