Medición ecográfica de parámetros anatómicos de la vía aérea superior en adultos / Ultrasound measurement of anatomical parameters of the upper airway in adults

Antecedentes: La mayoría de los trabajos sobre anatomía ecográfica de la vía aérea (VA) se limitan a la descripción morfológica. Presentamos un estudio cuyo objetivo es ofrecer datos objetivos de medidas de normalidad. Materiales y métodos: Estudio observacional para describir con ecografía las características de la VA superior en adultos sin criterios clínicos de VA difícil (VAD) y comparación con modelos anatómicos de disección en cadáver. Resultados: Se incluyó a 45 voluntarios (27 varones y 18 mujeres) y 3 modelos de cadáver. La calidad de la exploración fue muy buena/buena en el 93% de los casos. Mediciones: diámetro traqueal (1,3±0,3cm), cuerda vocal (1,6±0,5cm), membrana tirocricoidea (0,94±0,32cm), membrana cricotraqueal (0,3±0,09cm), grosor de los músculos del suelo de la boca (MSB) (1,5±0,26cm), tejido subcutáneo de la grasa submandibular y MSB (2,11±0,34cm), distancia hioides-mandíbula (5,35±0,69cm), distancia paladar-suelo de la boca (4,92±0,5cm), distancia paladar-borde anterior de la mandíbula (5,51±0,7cm), ángulo de los ejes bucal y faríngeo (114±14). Diferencias observadas: el diámetro traqueal fue mayor en hombres (H: 1,4±0,3 vs. M: 1,2±0,2cm; p=0,014). La altura mostró una correlación significativa con el diámetro traqueal (R: 0,501; p <0,001), la longitud de la cuerda vocal (R: 0,363; p=0,016) y el grosor MSB (R=0,299; p=0,046), así como con las distancias hioides-mandíbula (R: 0,556; p<0,001) y mandíbula-paladar (R: 0,362; p=0,015). Conclusiones: La ecografía permite evaluar la anatomía de la VA superior y calcular distancias entre estructuras anatómicas. En nuestro estudio se definen estas distancias en voluntarios adultos sin criterios clínicos de VAD

Background: Most of the works on ultrasound airway anatomy are limited to a morphological description. A study was conducted in order to provide an objective normal range of measurements. Materials and methods: Observational study to describe the ultrasound characteristics of the upper airway in adults without clinical difficult airway criteria, compared to cadaver dissection anatomical models. Results: The study included 45 volunteers (27 men and 18 women), and 3fresh cadavers. The quality of the examination was very good/good in 93% of the cases. Measurements: tracheal diameter (1.3±0.3cm), vocal cord (1.6±0.5cm), cricothyroid membrane (0.94±0.32cm), cricotracheal membrane (0.3±0.09cm), thickness of the muscles in the floor of the mouth (MFM) (1.5±0.26cm), sub-mandibular subcutaneous fat plus MFM (2.11±0.34cm), hyoid-mandible distance (5.35±0.69cm), palate-floor of the mouth distance (4.92±0.5cm), palate-anterior border of the mandible (5.51±0.7cm), and palate-pharynx angle (114±14). Observed differences: Males had a larger tracheal diameter than females (M: 1.4±0.3 vs. F: 1.2±0.2cm, p=0.014). Subject height showed a significant correlation with the tracheal diameter (R: 0.501, p<0.001), as well as the length of the vocal cord (R: 0.363, p=0.016), the thickness of MFM (R=0.299, p=0.046) as well as the hyoid-mandible (R: 0.556; p<0.001) and palate-mandible distances (R: 0.362; p=0.015). Conclusions: Ultrasound allows the anatomy of upper airway to be evaluated, as well as to calculate distances between the anatomical structures. The study defines these distances in adult volunteers without clinical difficult airway criteria

Ultrasound measurement of anatomical parameters of the upper airway in adults. / Medición ecográfica de parámetros anatómicos de la vía aérea superior en adultos.

BACKGROUND: Most of the works on ultrasound airway anatomy are limited to a morphological description. A study was conducted in order to provide an objective normal range of measurements. MATERIALS AND METHODS: Observational study to describe the ultrasound characteristics of the upper airway in adults without clinical difficult airway criteria, compared to cadaver dissection anatomical models. RESULTS: The study included 45 volunteers (27 men and 18 women), and 3fresh cadavers. The quality of the examination was very good/good in 93% of the cases. MEASUREMENTS: tracheal diameter (1.3±0.3cm), vocal cord (1.6±0.5cm), cricothyroid membrane (0.94±0.32cm), cricotracheal membrane (0.3±0.09cm), thickness of the muscles in the floor of the mouth (MFM) (1.5±0.26cm), sub-mandibular subcutaneous fat plus MFM (2.11±0.34cm), hyoid-mandible distance (5.35±0.69cm), palate-floor of the mouth distance (4.92±0.5cm), palate-anterior border of the mandible (5.51±0.7cm), and palate-pharynx angle (114±14). Observed differences: Males had a larger tracheal diameter than females (M: 1.4±0.3 vs. F: 1.2±0.2cm, p=0.014). Subject height showed a significant correlation with the tracheal diameter (R: 0.501, p<0.001), as well as the length of the vocal cord (R: 0.363, p=0.016), the thickness of MFM (R=0.299, p=0.046) as well as the hyoid-mandible (R: 0.556; p<0.001) and palate-mandible distances (R: 0.362; p=0.015). CONCLUSIONS: Ultrasound allows the anatomy of upper airway to be evaluated, as well as to calculate distances between the anatomical structures. The study defines these distances in adult volunteers without clinical difficult airway criteria.

Analgesia multimodal domiciliaria con metadona en perfusión intravenosa mediante bomba elastomérica en cirugía mayor ambulatoria / Outpatient intravenous multimodal elastomeric pump with methadone in ambulatory surgery

Antecedentes y objetivo: La analgesia en cirugía mayor ambulatoria (CMA) necesita evolucionar paralelamente a la complejidad quirúrgica. Diseñamos un estudio para intentar mejorar la analgesia en cirugía dolorosa mediante elastómera intravenosa. Como novedad, se incluyó metadona. Pacientes y métodos: Estudio observacional en pacientes ASA I-II, intervenidos en CMA de cirugías con dolor postoperatorio moderado-severo. Se administró analgesia durante 48h mediante elastómera intravenosa de metadona, tramadol, dexketoprofeno y ondansetrón a dosis bajas, y paracetamol oral. A las 24 y 48h se evaluó la eficacia analgésica en reposo y en movimiento (Escala Visual Analógica [EVA], Escala de Andersen y Test de Lattinen), la necesidad de analgesia de rescate y los efectos adversos. Resultados: Se incluyeron 73 pacientes: un 37% de ellos intervenidos de cirugía de pared abdominal, un 30% de hemorroidectomías y un 33% de cirugía del periné. La mediana en reposo a las 24 y 48h fue EVA=0, y en movimiento, fue de 3 a las 24h y de 2 a las 48h. En la Escala de Andersen, a las 24h el 89% presentó puntuación ≤1. En el Test de Lattinen, el 90% presentó una puntuación ≤6. Precisó rescate el 30%. Dos pacientes presentaron vómitos a las 24 y 48h. Se observaron problemas menores con el catéter y la elastómera en el 8% de los pacientes. Conclusiones: La analgesia multimodal con metadona intravenosa administrada mediante bomba elastómera para cirugía ambulatoria dolorosa es eficaz y segura. Sin embargo, es necesaria vigilancia para identificar disfunciones del dispositivo

Background and objective: Analgesia in Ambulatory Surgery (AS) needs to evolve in parallel with surgical complexity. We designed a study to try to improve analgesia in painful surgery using an intravenous elastomeric pump. As a novelty, methadone was included. Patients and methods: An observational study, physical status ASA I-II, underwent ambulatory surgeries with moderate-severe postoperative pain. Analgesia was administered for 48h by an intravenous multimodal elastomeric pump (methadone, tramadol, dexketoprofen and ondansetron at low doses). Visual Analogue Scale (VAS) at rest and movement were evaluated at 24 and 48h. Andersen Scale, Lattinen Test, rescue analgesia and side-effects were recorded at 24h after surgery. Results: We included 73 patients: 37% abdominal wall surgery, 30% hemorrhoidectomies and 33% perineal surgery. Median VAS score at rest and movement were 0 and 3 at 24h, and 0 and 2 at 48h. At 24h, Andersen's Scale score was ≤1 in 89%, and Lattinen Test ≤6 in 90% of patients. Rescue medication was administered in 30% of patients. Two patients had vomiting at 24 and 48h. Minor catheter and pump dysfunctions were observed in 8% of patients. Conclusions: Multimodal analgesia with intravenous methadone administered by elastomeric perfusion at home is effective and safe. However, monitoring is needed to diagnosis dysfunction of devices

Outpatient intravenous multimodal elastomeric pump with methadone in ambulatory surgery. / Analgesia multimodal domiciliaria con metadona en perfusión intravenosa mediante bomba elastomérica en cirugía mayor ambulatoria.

BACKGROUND AND OBJECTIVE: Analgesia in Ambulatory Surgery (AS) needs to evolve in parallel with surgical complexity. We designed a study to try to improve analgesia in painful surgery using an intravenous elastomeric pump. As a novelty, methadone was included. PATIENTS AND METHODS: An observational study, physical status ASA I-II, underwent ambulatory surgeries with moderate-severe postoperative pain. Analgesia was administered for 48h by an intravenous multimodal elastomeric pump (methadone, tramadol, dexketoprofen and ondansetron at low doses). Visual Analogue Scale (VAS) at rest and movement were evaluated at 24 and 48h. Andersen Scale, Lattinen Test, rescue analgesia and side-effects were recorded at 24h after surgery. RESULTS: We included 73 patients: 37% abdominal wall surgery, 30% hemorrhoidectomies and 33% perineal surgery. Median VAS score at rest and movement were 0 and 3 at 24h, and 0 and 2 at 48h. At 24h, Andersen's Scale score was ≤1 in 89%, and Lattinen Test ≤6 in 90% of patients. Rescue medication was administered in 30% of patients. Two patients had vomiting at 24 and 48h. Minor catheter and pump dysfunctions were observed in 8% of patients. CONCLUSIONS: Multimodal analgesia with intravenous methadone administered by elastomeric perfusion at home is effective and safe. However, monitoring is needed to diagnosis dysfunction of devices.

Hemorragia coroidea expulsiva espontánea. Caso clínico / Spontaneous expulsive choroidal hemorrhage. Case report

CASO CLÍNICO: Se presenta el caso de una paciente de 85 años de edad, procedente de un centro geriátrico, que acudió a urgencias con un cuadro de hemorragia ocular masiva aparentemente de forma espontánea en ojo izquierdo (OI). No había constancia de antecedentes oftalmológicos en su historial clínico. La paciente refería haber sido operada de catarata en ambos ojos hacía más de 25 años y llevar varios años sin visión por el OI. DISCUSIÓN: La hemorragia coroidea expulsiva espontánea (HCEE) no quirúrgica es una entidad clínica devastadora muy rara. Los factores predisponentes implicados en su desarrollo incluyen: edad avanzada, enfermedades vasculares (arterioesclerosis sobre todo), glaucoma y daño corneal severo

CLINICAL CASE: A case is presented of an 85-year-old woman living in a geriatric residence, who was admitted to the emergency department of our hospital with a spontaneous expulsive choroidal hemorrhage in her left eye. There was no a history of ophthalmic disease, and the patient only reported having intracapsular cataract surgery in both eyes 25 years ago, and that she also became blind in her left eye in the past few years. DISCUSSION: Non-surgical spontaneous expulsive choroidal hemorrhage is a very rare and disastrous clinical event. The predisposing factors involved are: advancing age, vascular illness (especially atherosclerosis), glaucoma, and severe corneal damage

[Spontaneous expulsive choroidal hemorrhage. Case report]. / Hemorragia coroidea expulsiva espontánea. Caso clínico.

CLINICAL CASE: A case is presented of an 85-year-old woman living in a geriatric residence, who was admitted to the emergency department of our hospital with a spontaneous expulsive choroidal hemorrhage in her left eye. There was no a history of ophthalmic disease, and the patient only reported having intracapsular cataract surgery in both eyes 25 years ago, and that she also became blind in her left eye in the past few years. DISCUSSION: Non-surgical spontaneous expulsive choroidal hemorrhage is a very rare and disastrous clinical event. The predisposing factors involved are: advancing age, vascular illness (especially atherosclerosis), glaucoma, and severe corneal damage.

Factors predicting distress among parents/caregivers of children with neurological disease and home enteral nutrition.

BACKGROUND & AIMS: Caregivers of children with chronic diseases included in a home enteral nutrition (HEN) programme are at risk of experiencing a feeling of burden, high level of anxiety and psychological distress. The aims of this study were: first, to examine the prevalence of symptoms of anxiety-depression in caregivers of children with neurological diseases requiring HEN by gastrostomy tube (GT); second, to compare the characteristics of caregivers with high or low risk of exhibiting symptoms of anxiety-depression; and third, to investigate possible associations to child disease severity and nutrition support mode. METHODS: A cross-sectional observational study was performed in 58 caregivers of children (31 boys, aged 0.3-18 years) with neurological diseases and GT feeding. The characteristics of caregivers with high or low risk of presenting symptoms of anxiety-depression were compared regarding the following variables: socio-demographic characteristics, the primary caregiver's intrapsychic factors, anthropometric parameters of the child, length of HEN, type of nutrients delivered by GT and infusion regime. RESULTS: All primary caregivers were mothers. Fifty-three per cent of them showed high risk of exhibiting symptoms of anxiety-depression. Mothers with high or low risk of presenting symptoms of anxiety-depression were comparable in age and family socio-economic status. They were also similar in terms of age, anthropometric conditions and length of HEN in their children.No differences were found between the two groups of mothers according to the level of the child's motor function impairment, type of nutrients delivered by GT and infusion regime. Higher levels of psychological distress and perception of burden overload were found in mothers with high risk of exhibiting symptoms of anxiety-depression. CONCLUSIONS: This study found a high prevalence of symptoms of anxiety-depression, perception of burden overload and psychological distress in caregivers of children with HEN. Thus, greater practical and emotional support is required for these families.

Analysis of the Spanish national registry for pediatric home enteral nutrition (NEPAD): implementation rates and observed trends during the past 8 years.

BACKGROUND/OBJECTIVES: The home enteral nutrition (HEN) provides nutritional support to children with chronic diseases who are nutritionally compromised and allows them to be discharged more quickly from hospitals. In 2003, a web-based registry (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition -NEPAD-) was created with the objective of gathering information about pediatric HEN practices in Spain. AIM: The aim of this study was to report the implementation of the NEPAD (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition) registry of pediatric HEN in Spain and to analyze data evolution trends from 2003 to 2010. SUBJECTS/METHODS: The data from the Spanish NEPAD registry were analyzed according to the following variables: demographic data, diagnosis, indication for HEN, nutritional support regime and administration route. RESULTS: Over the study period, 952 patients (1048 episodes) from 20 Spanish hospitals were included in the NEPAD registry. The most frequent indication for HEN was decreased oral intake (64%), and neurological disease was the most prevalent illness. HEN was delivered via a nasogastric tube in 573 episodes (54.7%), by gastrostomy in 375 episodes (35.8%), oral feeding in 77 episodes (7.3%) and by jejunal access in 23 episodes (2.2%). Significant differences in the mode of administration were observed based on the pathology of the child (χ(2), P<0.0001). The cyclic feeding was the most widely used technique for the administration of HEN. Most of the patients used a pump and a polymeric formula. Transition to oral feeding was the primary reason for discontinuation of this type of support. CONCLUSIONS: Since the NEPAD registry was established in Spain, the number of documented patients has increased more than 25-fold. Many children with chronic illness benefit from HEN, mainly those suffering from neurological diseases.

Psychometric properties of the structured Satisfaction Questionnaire with Gastrostomy Feeding (SAGA-8) for caregivers of children with gastrostomy tube nutritional support.

BACKGROUND: To analyse the psychometric properties of the structured Satisfaction Questionnaire with Gastrostomy Feeding (SAGA-8) in parents/caregivers of children with home enteral nutrition (HEN) by gastrostomy tube (GT). METHODS: Eighty-six caregivers (mothers) of paediatric patients with HEN by GT were recruited. Patients suffered from neurological disease (61.6%) and other chronic diseases. The SAGA-8 scale, a structured questionnaire to explore satisfaction with HEN by GT, and the Caregiver Burden Inventory (Zarit) were completed. The discriminating power of each of the SAGA-8 items, internal consistency and external validity were evaluated. An exploratory factor analysis and Kaiser-Meyer-Olkin (KMO) was performed as well. RESULTS: Eighty-four percent of the families expressed high satisfaction with GT feeding. All eight items of SAGA-8 gave additional information. The exploratory factor analysis revealed that a significant part of the items' variability could be explained by two independent factors: Factor 1 (direct benefit), which compiled the variables related to the perception of children's overall improvement by GT feeding; Factor 2 (indirect benefit), which grouped the variables related to a decrease in respiratory infections, feeding time and institutional support. Results from KMO (0.628) indicated the high adequacy of the items assessed in the factorial analysis. Moreover, the questionnaire presented high internal consistency (0.76), and the external validation analysis confirmed the correlation between SAGA-8 and Zarit, thereby emphasising the approptiate use of the SAGA-8 to detect carers' satisfaction. CONCLUSIONS: The SAGA-8 questionnaire has a high discriminatory power to assess the degree of satisfaction experienced by parents/caregivers of children with HEN by GT and, subsequently, the patients' wellbeing.

Estudio epidemiológico de las enfermedades metabólicas con homocistinuria en España / Epidemiological study of the metabolic diseases with homocystinuria in Spain

Objetivos: Conocer la prevalencia en España de los diferentes errores congénitos del metabolismo que presentan homocistinuria y establecer las medidas oportunas para garantizar su prevención, diagnóstico y tratamiento, en aquellos casos posibles. Material y métodos: En abril 2009 se realizó una encuesta nacional de carácter transversal mediante cuestionario enviado a 35 centros, en los que se atiende a pacientes infantiles y adultos. La finalidad de la encuesta era establecer la prevalencia en ese momento recogiendo el histórico de pacientes que cada centro tuviera documentados. Resultados: A través de los cuestionarios respondidos por 25 médicos de 16 centros, se han identificado 75 pacientes: 41 defectos de transulfuración (uno fallecido), 27 de remetilación (6 fallecidos) y 7 sin diagnóstico etiológico definitivo. La edad de diagnóstico muestra una amplia variación, en 18 casos había más de un hermano afectado. Las manifestaciones clínicas más graves inciden en el grupo de los pacientes afectados de trastornos de la remetilación. Destaca el alto porcentaje de déficit cognitivo, seguido de la patología de cristalino; casi la mitad de los pacientes presentan trastornos neurológicos, es elevada la afectación vascular en los adultos con deficiencia de CBS; las opciones terapéuticas más utilizadas han sido el ácido fólico, la hidroxicobalamina y la betaína. Conclusiones: A la vista de estos resultados, y en especial del escaso número de deficiencias de CBS detectadas, se concluye la necesidad de implantar el cribado neonatal para la homocistinuria clásica y asegurar la puesta en marcha del proceso diagnóstico oportuno en todos los pacientes de riesgo(AU)

Objectives: To determine the prevalence of homocystinuria in Spain and to establish the measures and mechanisms to ensure its prevention, diagnosis and treatment. Material and methods: A national cross-sectional survey was conducted by means of a questionnaire sent to 35 hospitals in which children and adult patients are treated. Results: Using the questionnaires submitted by 25 physicians from 16 centres, 75 patients were identified: 41 transsulphuration defects (one deceased), 27 remethylation (six deaths) and 7 without a syndromic diagnosis. The age at diagnosis varied widely, and 18 cases had more than one sibling affected. The more severe clinical manifestations involved the patients with remethylation defects. There was a high percentage of cognitive impairment, followed by lens diseases. Almost half of the patients had neurological disorders. There was increased vascular involvement in CBS-deficient adults. The therapeutic options most used were, folic acid, hydroxycobalamin and betaine. Conclusions: In view of these results and especially the small number of CBS deficiencies detected, we conclude that there is a need to introduce newborn screening for classical homocystinuria and ensure implementation of an appropriate diagnostic workup in all patients at risk(AU)

[Epidemiological study of the metabolic diseases with homocystinuria in Spain]. / Estudio epidemiológico de las enfermedades metabólicas con homocistinuria en España.

OBJECTIVES: To determine the prevalence of homocystinuria in Spain and to establish the measures and mechanisms to ensure its prevention, diagnosis and treatment. MATERIAL AND METHODS: A national cross-sectional survey was conducted by means of a questionnaire sent to 35 hospitals in which children and adult patients are treated. RESULTS: Using the questionnaires submitted by 25 physicians from 16 centres, 75 patients were identified: 41 transsulphuration defects (one deceased), 27 remethylation (six deaths) and 7 without a syndromic diagnosis. The age at diagnosis varied widely, and 18 cases had more than one sibling affected. The more severe clinical manifestations involved the patients with remethylation defects. There was a high percentage of cognitive impairment, followed by lens diseases. Almost half of the patients had neurological disorders. There was increased vascular involvement in CBS-deficient adults. The therapeutic options most used were, folic acid, hydroxycobalamin and betaine. CONCLUSIONS: In view of these results and especially the small number of CBS deficiencies detected, we conclude that there is a need to introduce newborn screening for classical homocystinuria and ensure implementation of an appropriate diagnostic workup in all patients at risk.

Consensus on paediatric enteral nutrition access: a document approved by SENPE/SEGHNP/ANECIPN/SECP.

Standardization of clinical procedures has become a desirable objective in contemporary medical practice. To this effect, the Spanish Society of Parenteral and Enteral Nutrition (SENPE) has endeavoured to create clinical practice guidelines and/or documents of consensus as well as quality standards in artificial nutrition. As a result, the SENPE´s Standardization Team has put together the "Document of Consensus in Enteral Access for Paediatric Nutritional Support" supported by the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP), the National Association of Pediatric and Neonatal Intensive Care Nursery (ANECIPN), and the Spanish Society of Pediatric Surgery (SECP). The present publication is a reduced version of our work; the complete document will be published as a monographic issue. It analyzes enteral access options in the pediatric patient, reviews the levels of evidence and provides the team-members' experience. Similarly, it details general and specific indications for pediatric enteral support, current techniques, care guidelines, methods of administration and complications of each enteral access. The data published by the American Society for Parenteral and Enteral Nutrition (ASPEN) and several European Societies has also been incorporated.

Long-chain polyunsaturated fatty acid concentration in patients with inborn errors of metabolism.

INTRODUCTION: Long-chain polyunsaturated fatty acid (LCPUFA) can be provided by diet (fatty fish, eggs, viscera and human milk) or synthetised from essential fatty acids linoleic and α-linolenic acids through the microsomal pathway. However, endogenous LCPUFA synthesis is rather low, especially for docosahexaenoic (DHA), and seems insufficient to achieve normal DHA values in individuals devoid of preformed dietary supply. Inborn errors of metabolism (IEMs) are therefore diseases with a special risk for LCPUFA deficient status. AIM: Our aim was to evaluate LCPUFA status in 132 patients with different IEMs. METHODS: We performed a cross-sectional study of plasma and erythrocyte LCPUFA composition of 63 patients with IEMs treated with protein-restricted diets compared with data from 69 patients with IEMs on protein-unrestricted diets, and 43 own reference values. RESULTS: Erythrocyte and plasma DHA and arachidonic acid concentrations were significantly decreased in patients treated with protein-restriction compared with those on protein-unrestricted diets and with our reference values (p < 0.0001). In the protein-restricted group, 45% of patients showed decreased erythrocyte and plasma DHA values (only 7% and 10%, respectively in the protein-unrestricted group) (p < 0.0001). Erythrocyte and plasma DHA values correlated with the natural protein intake in patients on protein-restriction (r = 0.257; p = 0.045; r = 0.313; p = 0.014, respectively). CONCLUSION: Plasma and erythrocyte DHA concentrations are decreased in patients with IEMs treated with protein restriction. DHA concentration correlates with the patients' protein intake. Supplementation of patients with LCPUFA would have a beneficial influence on their nutritional status.

Cuidados de las vías de acceso en nutrición enteral / No disponible

No disponible

Long-chain polyunsaturated fatty acid concentration in patients with inborn errors of metabolism / Concentración de ácidos grasos poliinsaturados de cadena larga en pacientes con errores innatos del metabolismo

Introduction: Long-chain polyunsaturated fatty acid (LCPUFA) can be provided by diet (fatty fish, eggs, viscera and human milk) or synthetised from essential fatty acids linoleic and α-linolenic acids through the microsomal pathway. However, endogenous LCPUFA synthesis is rather low, especially for docosahexaenoic (DHA), and seems insufficient to achieve normal DHA values in individuals devoid of preformed dietary supply. Inborn errors of metabolism (IEMs) are therefore diseases with a special risk for LCPUFA deficient status. Aim: Our aim was to evaluate LCPUFA status in 132 patients with different IEMs. Methods: We performed a cross-sectional study of plasma and erythrocyte LCPUFA composition of 63 patients with IEMs treated with protein-restricted diets compared with data from 69 patients with IEMs on protein-unrestricted diets, and 43 own reference values. Results: Erythrocyte and plasma DHA and arachidonic acid concentrations were significantly decreased in patients treated with protein-restriction compared with those on protein-unrestricted diets and with our reference values (p < 0.0001). In the protein-restricted group, 45% of patients showed decreased erythrocyte and plasma DHA values (only 7% and 10%, respectively in the protein-unrestricted group) (p < 0.0001). Erythrocyte and plasma DHA values correlated with the natural protein intake in patients on protein-restriction (r = 0.257; p = 0.045; r = 0.313; p = 0.014, respectively). Conclusion: Plasma and erythrocyte DHA concentrations are decreased in patients with IEMs treated with protein restriction. DHA concentration correlates with the patients' protein intake. Supplementation of patients with LCPUFA would have a beneficial influence on their nutritional status (AU)

Introducción: Los ácidos grasos poliinsaturados de cadena larga (LCPUFA) pueden ser suministrados por la dieta o sintetizados a partir de los ácidos grasos esenciales, linoleico y α-linolénico. La síntesis endógena de LCPUFA es escasa, especialmente la de ácido docosahe-xaenoico (DHA), e insuficiente para alcanzar los valores normales de DHA en individuos que carecen de un suministro dietético de dichos ácidos preformados. Por ello, los errores innatos del metabolismo (IEM) son enfermedades con riesgo especial de deficiencia de LCPUFAs. Objetivos: Evaluar el estado de LCPUFA en 132 pacientes con diferentes IEMs. Métodos: Estudio transversal de LCPUFA en plasma y eritrocitos de 63 pacientes con IEMs tratados con dieta restringida en proteínas comparados con 69 pacientes con IEMs con una dieta libre y 43 valores de referencia. Resultados: Las concentraciones de DHA y ácido ara-quidónico en plasma y eritrocitos se hallaron disminuidas en pacientes con restricción proteica comparados con pacientes con dieta libre y valores de referencia (p < 0,0001). El 45% de pacientes con restricción proteica mostró un descenso de DHA en plasma y eritrocitos (solo un 7% y un 10%, respectivamente en aquellos con dieta libre) (p < 0,0001). Los valores de DHA en eritrocitos y plasma correlacionaron con la ingesta de proteínas naturales en pacientes con restricción proteica (r = 0,257; p = 0,045; r = 0,313; p = 0,014, respectivamente). Conclusión: Las concentraciones de DHA en plasma y eritrocitos se hallaron descendidas en pacientes con IEMs con restricción proteica, correlacionando con la ingesta proteica de los pacientes. La suplementación de dichos pacientes con LCPUFA podría ser beneficiosa para su estado nutricional (AU)

Home parenteral nutritrion in children: procedures, experiences and reflections / La nutrición parenteral pediátrica domiciliaria: procedimientos, experiencias y reflexiones

This document summarizes the issues raised in a think tank meeting held by professionals with expertise in pediatric Home Parenteral Nutrition. This nutritional technology enables patients to return home to their family and social environment, improves their quality of life and decreases health-care costs; however, it is complex and requires an experienced nutritional support team. Patient selection is normally made according to their underlying disease, the estimated duration of support and family and social characteristics. The patient’s family must agree to take on caregiver’s responsibilities and should be able to perform treatment safely and effectively after receiving proper training from the nutritional support team. Close monitoring must be carried out to ensure tolerance and effectiveness of nutritional support, there by avoiding complications. This nutritional treatment achieves, in most cases, recovery and intestinal adaptation in varying periods of time. In certain diseases, and when home parenteral nutrition becomes complicated, intestinal transplant may be recommendable, so referral to rehabilitation units and Intestinal Transplantation should be made early on (AU)

El presente documento resume los aspectos abordados en una Jornada de puesta en común con la participación de profesionales con experiencia en nutrición parenteral domiciliaria pediátrica. Este tratamiento permite el retorno de los pacientes a su medio familiar y social, mejora su calidad de vida y disminuye los costes sanitarios pero es complejo y requiere un equipo de soporte nutricional experimentado. La selección del paciente se realizará en función de su enfermedad de base, la duración estimada del soporte y las características familiares y sociales. La familia del paciente ha de querer hacerse cargo de su cuidado y debe ser capaz de realizar el tratamiento de forma segura y eficaz tras recibir la formación adecuada por el equipo de soporte nutricional. El seguimiento ha de efectuarse de forma estrecha para asegurar la tolerancia y eficacia del soporte, evitando las complicaciones. Este tratamiento nutricional consigue, en la mayoría de los casos, la recuperación y adaptación intestinal en periodos variables de tiempo. En ciertas patologías y cuando la nutrición parenteral domiciliaria se complica puede estar indicado el trasplante intestinal, por lo que la remisión a las Unidades de Rehabilitación Intestinal y Trasplante debe hacerse de forma precoz (AU)

Current status of pediatric home enteral nutrition in Spain: The importance of the NEPAD register / Estado actual de la nutrición enteral pediátrica en España: la importancia del registro NEPAD

Home enteral nutrition (HEN) is a type of enteral nutrition (EN) which is becoming progressively more widespread in pediatrics due to the benefits it affords to patients, their families and to reducing hospital costs. However, the true extent of its use is unknown in Spain as the data-base set up for this purpose is still underused (Registro de Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria -NEPAD-). More thorough registration of patients in the NEPAD online register will provide information about the characteristics of HEN in Spain: prevalence, diagnosis, the population sector being administered HEN, complications and developments. Likewise, forecast and planning of the necessary resources could be made while those in use could be analysed (AU)

La nutrición enteral domiciliaria (NED) es una modalidad de nutrición enteral (NE) con una progresiva extensión en pediatría por los beneficios que supone para el paciente, sus familiares y la disminución de los costes hospitalarios. Sin embargo, se desconoce su verdadero alcance en España ya que el registro creado a tal efecto está aún infrautilizado (Registro de Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria -NEPAD-). La inclusión exhaustiva de pacientes en NEPAD permitiría conocer las características de la NED en España: prevalencia, indicaciones, población a la que se le está administrando, complicaciones y evolución. Así mismo se podría realizar una previsión y planificación de los recursos que se precisan y analizar los que se están utilizando (AU)

Quality of dietary control in phenylketonuric patients and its relationship with general intelligence / Calidad del control de la dieta en pacientes fenilcetonúricos y su relación con la inteligencia general

Objectives: Assessment of the quality of dietary treatment of phenylketonuria (PKU) patients and investigation of its relationship with the general intelligence of the patients. Methods: Cross-sectional and longitudinal study of 105 PKU treated patients. The index of dietary control (IDC) was calculated as the phenylalanine (Phe) data reduction in half-year medians and the mean of all medians throughout the patient's life. We calculated four different IDCs related to age: IDC-A (< 6 years), IDC-B (6-12 years), IDC-C (13-18 years) and IDC-D (> 18 years). To evaluate the fluctuation of Phe values we calculated the standard error of the estimate of the regression of Phe concentration over age. Development quotient was calculated with the Brunet-Lezine test (< 4 years). Intelligence quotient was evaluated with the Kaufman Bit Intelligence Test (K-Bit), Wechsler Intelligence Scale for Children-Revised (WISC-R) and Wechsler Adult Intelligence Scale Third Edition (WAIS III). Results: Cross-sectional study: The IDC in age groups were significantly different and so were the number of patients with good, acceptable and poor IDC related to age (p < 0.001). Sampling frequency was good in 72, acceptable in 23 and poor in 10 patients. The general intelligence (101 ± 10) correlated negatively with the IDC (p < 0.0001) and Phe fluctuations (p < 0.004). Longitudinal study: Significant differences were observed between the IDC through the patients' lifetime except in the adolescent/adult period. Conclusions: 85% of PKU patients showed good/acceptable quality of dietary control. General intelligence correlates with the IDC at all ages, which highlights the importance of good control to achieve good prognosis (AU)

Objetivos: Evaluación de la calidad del control dietético en pacientes con fenilcetonuria (PKU) e investigación de su relacióncon el nivel de inteligencia. Métodos: Estudio transversal y longitudinal de 105 pacientes PKU en tratamiento dietético. El índice de control de la dieta (IDC) se ha calculado como la reducción de los valores de fenilalanina (Phe) a las medianas de cada 6 meses y la media de todas las medianas a lo largo de la vida del paciente. Se han calculado cuatro diferentes IDC según la edad: IDC-A (< 6 años), IDC-B (6-12 años), IDC-C (13-18 años) and IDC-D (> 18 años). Para evaluar las fluctuaciones de los valores de Phe hemos calculado el error estándar de la regresión estimada de la concentración de Phe según la edad. El índice de inteligencia se ha evaluado mediante el test de Brunet-Lezine (< 4 años) y el coeficiente de inteligencia mediante Kaufman Bit Intelligence Test (K-Bit), Wechsler Intelligence Scale for Children-Revised (WISC-R) y Wechsler Adult Intelligence Scale Third Edition (WAIS III). Resultados: Estudio transversal: El IDC en los diferentes grupos de edad es significativamente diferente y también lo son el número de pacientes con un IDC bueno, aceptable y malo en relación con la edad (p < 0,001). La frecuencia de controles de Phe fue buena en 72 pacientes, aceptable en 23 y mala en 10. La inteligencia general (101 ± 10) se correlaciona negativamente con el IDC (p < 0,0001) y con las fluctuaciones de Phe (p < 0,004). Estudio longitudinal: Se han observado diferencias significativas entre ICD a lo largo de la vida de los pacientes a excepción del período adolescencia/edad adulta. Conclusiones: El 85% de pacientes PKU mostraron una calidad del control de la dieta buena/aceptable. Los niveles de inteligencia general se correlacionan con el IDC en todas las edades, lo que muestra la importancia del buen control de la dieta para lograr un buen pronóstico (AU)