Body composition and nutritional status changes in adolescents with anorexia nervosa.

INTRODUCTION: Nutritional status assessment in anorexia nervosa (AN) includes the evaluation and monitoring of body composition throughout the treatment period. The gold standard for the study of body composition is dual-energy X-ray absorptiometry (DEXA), although electrical bioimpedance (BIA) is a more accessible, cheaper and faster method that does not involve exposure to radiation. MATERIAL AND METHODS: We recruited 33 female adolescents with AN (age, 11.7-16.3 years) by consecutive sampling. We collected data on clinical, anthropometric and laboratory variables. Patients were assessed with BIA and DEXA at inclusion in the study and at the end of the study, with a mean duration of follow-up of 1 year, during the nutritional rehabilitation phase. RESULTS: There was significant improvement in nutritional status, reflected by the body composition obtained by anthropometric measurements and BIA. The phase angle increased significantly during the follow-up. Greater weight loss was associated with the presence of secondary amenorrhoea and decreased bone mineral density in the spine. CONCLUSIONS: Electrical BIA is a useful tool for assessment and monitoring of nutritional status in paediatric patients with AN. Dual-energy X-ray absorptiometry continues to be essential to assess bone mineral density. The role of hormones such as leptin remains to be elucidated.

Translation and validation of the Spanish version of the Pedi-EAT-10 questionnaire for screening of dysphagia.

INTRODUCTION: The Pedi-EAT-10 is a quick and simple validated tool for screening for dysphagia in the paediatric age group. The objective of our study was to translate and adapt the scale to Spanish and assess its psychometric properties, level of difficulty and speed of completion. PATIENTS AND METHODS: Following the forward and back translation and the approval by the research team of the Spanish version of the Pedi-EAT-10, we carried out a prospective study in a group of patients with dysphagia and a group of children who were healthy or had minor disease. Their legal guardians completed the questionnaire and reported the duration and difficulty of the test. RESULTS: The study included 87 cases of dysphagia and 91 controls. The Cronbach alpha for internal consistency was 0.87. Most correlations between single item scores and the total scale score were greater than 0.65 (P < .001). The Pedi-EAT-10 scores were significantly higher in patients with dysphagia in every age group (P < .001), evincing a high sensibility and specificity for the screening of dysphagia. In the control group, the mean time taken to complete the questionnaire was 2.18 ± 1.98 min, and all participants found it easy. CONCLUSIONS: We verified the validity, reliability and internal consistency of the Spanish version of the Pedi-EAT-10. It is an easy and quick instrument that can be used for screening of dysphagia in paediatric clinical practice.

Switching to Glycerol Phenylbutyrate in 48 Patients with Urea Cycle Disorders: Clinical Experience in Spain.

Background and objectives: Glycerol phenylbutyrate (GPB) has demonstrated safety and efficacy in patients with urea cycle disorders (UCDs) by means of its clinical trial program, but there are limited data in clinical practice. In order to analyze the efficacy and safety of GPB in clinical practice, here we present a national Spanish experience after direct switching from another nitrogen scavenger to GPB. Methods: This observational, retrospective, multicenter study was performed in 48 UCD patients (age 11.7 ± 8.2 years) switching to GPB in 13 centers from nine Spanish regions. Clinical, biochemical, and nutritional data were collected at three different times: prior to GPB introduction, at first follow-up assessment, and after one year of GPB treatment. Number of related adverse effects and hyperammonemic crisis 12 months before and after GPB introduction were recorded. Results: GPB was administered at a 247.8 ± 102.1 mg/kg/day dose, compared to 262.6 ± 126.1 mg/kg/day of previous scavenger (46/48 Na-phenylbutyrate). At first follow-up (79 ± 59 days), a statistically significant reduction in ammonia (from 40.2 ± 17.3 to 32.6 ± 13.9 µmol/L, p < 0.001) and glutamine levels (from 791.4 ± 289.8 to 648.6 ± 247.41 µmol/L, p < 0.001) was observed. After one year of GPB treatment (411 ± 92 days), we observed an improved metabolic control (maintenance of ammonia and glutamine reduction, with improved branched chain amino acids profile), and a reduction in hyperammonemic crisis rate (from 0.3 ± 0.7 to less than 0.1 ± 0.3 crisis/patients/year, p = 0.02) and related adverse effects (RAE, from 0.5 to less than 0.1 RAEs/patients/year p < 0.001). Conclusions: This study demonstrates the safety of direct switching from other nitrogen scavengers to GPB in clinical practice, which improves efficacy, metabolic control, and RAE compared to previous treatments.

[EATING DISORDERS IN PEDIATRIC AGE: A BOOM PATHOLOGY]. / TRASTORNOS DE LA CONDUCTA ALIMENTARIA EN LA EDAD PEDIÁTRICA: UNA PATOLOGÍA EN AUGE.

AIMS: to determine the characteristics of pediatric patients suffering from eating disorders that were hospitalized at Hospital Universitario de Canarias. MATERIALS AND METHODS: a retrospective study in a cohort of pediatric patients diagnosed with eating disorders and admitted in our area was developed during the last seven years. RESULTS: out of 35 patients in our study, 85.7 % were women, onset average age 13.5. 77.1% of the cases were diagnosed as anorexia nervosa- restrictive type. The most frequent analytical alterations, detected when patients were in hospital, consisted in a drop in plasma levels in retinol binding protein (RBP)- in 57.6% of the cases- and D hypovitaminosis- in 46.9 % of them: the use of high - calories supplements was required in 71.4% of patients during hospitalization. The average weight gain was higher when the body mass index (BMI) was smaller at patient's admission to hospital (p = 0,006). Conclussions: eating disorders are increasing in pediatric age: puberty is a special vulnerable period for its development, as well as medical complications secondary to malnutrition. Admission to hospital is an essential tool for handling many cases; taking the necessary monitoring leading to a weight increase, preventing complications in nutritional support and tackling the underlying psychopathology. Diagnosis and a precocious treatment are crucial to avoid an excessive weight loss and more complications.

Objetivos: determinar las características de los pacientes en edad pediátrica afectados de trastornos de la conducta alimentaria que requirieron ingreso en el Servicio de Pediatría del Complejo Hospitalario Universitario de Canarias (CHUC). Material y método: se realizó un estudio retrospectivo de una cohorte de pacientes pediátricos diagnosticados de trastornos de la conducta alimentaria e ingresados en planta de hospitalización pediátrica durante los últimos siete años. Resultados: de los 35 pacientes de la muestra, un 85,7% fueron mujeres, con una edad media al debut de 13,5 años. En el 77,1% de los casos el diagnóstico principal fue el de anorexia nerviosa de tipo restrictivo. Las alteraciones analíticas más frecuentes detectadas en el momento del ingreso consistieron en descenso de los niveles plasmáticos de proteína fijadora del retinol (RBP), presente en el 57,6% de los casos, e hipovitaminosis D, que en esta muestra estaba presente en el 46,9% de los casos. Se requirió el uso de suplementos hipercalóricos en el 71,4% de los pacientes durante la hospitalización. La ganancia ponderal media durante la estancia fue mayor cuanto menor era el índice de masa corporal (IMC) al ingreso (p = 0,006). Conclusiones: los TCA son enfermedades con incidencia creciente en la edad pediátrica. La pubertad constituye un momento de especial vulnerabilidad para el desarrollo de los TCA (así como de complicaciones médicas secundarias a la desnutrición). En muchos casos el ingreso hospitalario constituye una herramienta necesaria para un correcto manejo, instaurándose las medidas de control necesarias para la recuperación ponderal, la prevención de complicaciones del soporte nutricional y el abordaje de la psicopatología subyacente. El diagnóstico y tratamiento precoz resultan cruciales para evitar una excesiva pérdida ponderal y mayor incidencia de complicaciones.