Evaluation of the first 5 years of a glutaric aciduria type I neonatal screening programme in Asturias.

INTRODUCTION: . Neonatal screening of glutaric aciduria type 1 (GA-1) has brought radical changes in the course and outcomes of this disease. This study analyses the outcomes of the first 5 years (2015-2019) of the AGA1 neonatal screening programme in our autonomous community. MATERIAL: . We conducted an observational, descriptive and retrospective study. All neonates born between January 1, 2015 and December 31, 2019 that participated in the neonatal screening programme were included in the study. The glutarylcarnitine (C5DC) concentration in dry blood spot samples was measured by means of tandem mass spectrometry applying a cut-off point of 0.25 µmol/L. RESULTS: . A total of 30 120 newborns underwent screening. We found differences in the C5DC concentration based on gestational age, type of feeding and hours of life at sample collection. These differences were not relevant for screening purposes. There were no differences between neonates with weights smaller and greater than 1500 g. Screening identified 2 affected patients and there were 3 false positives. There were no false negatives. The diagnosis was confirmed by genetic testing. Patients have been in treatment since diagnosis and have not developed encephalopathic crises in the first 4 years of life. CONCLUSIONS: . Screening allowed early diagnosis of two cases of GA-1 in the first 5 years since its introduction in our autonomous community. Although there were differences in C5DC levels based on gestational age, type of feeding and hours of life at blood extraction, they were not relevant for screening.

Spanish Pediatricians' Positions Regarding Prevention, Diagnosis, Nutritional Management, and Challenges in Cow's Milk Protein Allergy.

Management of cow's milk protein allergy (CMPA) can vary depending on the experience and area of expertise of the clinician responsible for the patient's follow-up, which may or may not align with the recently published literature. To analyze the perspectives of Spanish pediatricians on this topic, a survey was conducted. The survey aimed to determine the current opinions and attitudes of 222 primary care and hospital pediatricians toward CMPA prevention and nutritional management. Participating pediatricians completed the questionnaire, providing insights into their daily clinical practices, including access to testing, attitudes with respect to various aspects of CMPA diagnosis, prevention, oral food challenges, and treatment. The findings revealed that pediatricians generally agree on the use of extensively hydrolyzed formulas (eHFs) to prevent CMPA in high-risk atopic children, despite limited evidence supporting the widespread use of this practice. However, consensus was lacking regarding the utility of formulas with prebiotics and probiotics for expediting tolerance development. In most cases, pediatricians preferred eHFs for the nutritional management of CMPA, followed by hydrolyzed rice formulas (HRFs), with amino-acid-based formulas (AAFs) being the third option. Certain issues remained controversial among pediatricians, such as prevention methods, symptom assessment, and the role of probiotics. These variations in management approaches reflect the influence of clinician experience and area of expertise, underscoring the need for standardized guidelines in this field.

Seguridad y eficacia de una nueva pauta de suplementación en pacientes con fibrosis quística e insuficiencia de vitamina D / Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency

Objetivos: Basándonos en los documentos de consenso europeo y americano de fibrosis quística (FQ) se propone un incremento de la suplementación de vitamina D (VD) en pacientes con FQ y niveles insuficientes. El objetivo de nuestro estudio fue conocer la seguridad y la eficacia de este nuevo protocolo. Material y métodos: Estudio multicéntrico, experimental no aleatorizado ni controlado. A los pacientes con niveles insuficientes (<30ng/ml) se les administró dosis crecientes de VD (entre 800 y 10.000UI/día). Se realizó seguimiento durante 12 meses analizando estatus vitamínico, nutricional, función pulmonar y metabolismo fosfocálcico. Análisis estadístico: pruebas t para datos apareados y regresión logística con análisis multivariable. Resultados: Un total de 30 pacientes entre 1 y 39 años (mediana 9,1) completaron el estudio. Se retiraron 2 por niveles de 25 OH VD>100ng/ml a los 3 meses sin encontrarse signos clínicos ni analíticos de hipercalcemia. Tras 12 meses se observó un incremento de 7,6ng/ml (IC 95% 4,6-10ng/ml) de los niveles medios de 25 OH VD. El 37% alcanzaron niveles ≥30ng/ml, un 13% <20ng/ml y un 50% entre 20 y 30ng/ml. No se observó asociación de la mejoría de los niveles de VD con la función pulmonar. Conclusiones: Con el protocolo propuesto se consigue un incremento de los niveles séricos de VD y una disminución del porcentaje de pacientes con insuficiencia de la misma, aunque todavía muy lejos de alcanzar los porcentajes de suficiencia recomendados para esta entidad. (AU)

Objectives: Based on the European and American cystic fibrosis (CF) consensus recommendations, an increase in vitamin D (VD) supplementation in patients with CF and insufficient or deficient levels was proposed. The objective of our study was to determine the safety and efficacy of this new protocol. Material and methods: Multicentre nonrandomized uncontrolled experimental study. Patients with insufficient levels (<30ng/mL) received increasing doses of VD (between 800 and 10,000IU/day). Patients were followed up for 12 months, during which their vitamin and nutritional status, pulmonary function and calcium and phosphate metabolism were assessed. Statistical analysis: t test for paired data and multivariate logistic regression analysis. Results: Thirty patients aged 1–39 years (median, 9.1) completed the follow-up. Two patients were dropped from the study on account of 25-OH VD levels greater than 100ng/mL at 3 months without clinical or laboratory signs of hypercalcaemia. At 12 months, we observed an increase of 7.6ng/mL (95% CI, 4.6–10ng/mL) in the mean 25-OH VD level and an improvement in vitamin status: 37% achieved levels of 30ng/mL or greater, 50% levels between 20 and 30ng/mL and 13% remained with levels of less than 20ng/mL. We found no association between improved VD levels and pulmonary function. Conclusions: The proposed protocol achieved an increase in serum VD levels and a decrease in the percentage of patients with VD insufficiency, although it was still far from reaching the percentages of sufficiency recommended for this entity. (AU)

Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency.

OBJECTIVES: Based on the European and American Cystic Fibrosis (CF) consensus recommendations, an increase in vitamin D (VD) supplementation in patients with CF and insufficient or defficient levels was proposed. The objective of our study was to determine the safety and efficacy of this new protocol. MATERIAL AND METHODS: Multicentre nonrandomized uncontrolled experimental study. Patients with insufficient levels (<30 ng/mL) received increasing doses of VD (between 800 and 10 000 IU/day). Patients were followed up for 12 months, during which their vitamin and nutritional status, pulmonary function and calcium and phosphate metabolism were assessed. STATISTICAL ANALYSIS: t test for paired data and multivariate logistic regression analysis. RESULTS: Thirty patients aged 1-39 years (median, 9.1) completed the follow-up. Two patients were dropped from the study on account of 25-OH VD levels greater than 100 ng/mL at 3 months without clinical or laboratory signs of hypercalcaemia. At 12 months, we observed an increase of 7.6 ng/mL (95% CI, 4.6-10 ng/mL) in the mean 25-OH VD level and an improvement in vitamin status: 37% achieved levels of 30 ng/mL or greater, 50% levels between 20 and 30 ng/mL and 13% remained with levels of less than 20 ng/mL. We found no association between improved VD levels and pulmonary function. CONCLUSIONS: The proposed protocol achieved an increase in serum VD levels and a decrease in the percentage of patients with VD insufficiency, although it was still far from reaching the percentages of sufficiency recommended for this entity.

[Cardiovascular risk and insulin resistance in childhood leukemia survivors]. / Riesgo cardiovascular y resistencia insulínica en supervivientes de leucemia infantil.

Introduction: Material and methods: a retrospective study of childhood acute leukemia survivors. Survivors with a diagnosis of leukemia before 16 years of age in a tertiary hospital, during the period of 1998-2018, were selected, who had completed their treatment at least two years earlier. We examined: blood adipokine levels and carbohydrate metabolism, body composition by bioimpedance, and carotid status by ultrasound. Somatometric measures were also taken. Results: the registry showed 82 children diagnosed with acute leukemia, aged between 6 and 16 years. Only 22 met the criteria to be included in the study. Results reveled that 32 % of the sample met the criteria for overweight-obesity, and 36 % had high insulin resistance indexes (IR). Leptin levels were higher in women (15.45 vs. 3.25; p = 0.044) and in obese and overweight subjects, as was the leptin/adiponectin ratio, which rises in the presence of IR (2.52 vs. 0.45; p = 0.037). We observed an increase in carotid intima-media thickness in relation to BMI (0.008; CI, -0.002 to 0.013; p = 0.007) without any association with an increase in fat mass in these patients (0.204; CI, -0.043 to 0.451; p = 0.101). Conclusions: childhood leukemia survivors have a high cardiovascular risk, characterized by an increase in IR, not associated with an increase in fat mass. This risk could justify the implementation of preventive actions in these long-lived patients.

Introducción: Material y métodos: estudio retrospectivo de supervivientes de leucemia aguda en edad infantil. Se seleccionaron aquellos supervivientes con diagnóstico de leucemia antes de los 16 años de edad, en un hospital de tercer nivel y durante el período 1998-2018, que hubieran finalizado su tratamiento como mínimo dos años antes. Se analizaron: niveles de adipokinas y metabolismo hidrocarbonado en sangre, composición corporal mediante bioimpedancia y evaluación ecográfica carotídea. Se tomaron además datos somatométricos. Resultados: de 82 niños con diagnóstico de leucemia aguda, con edades comprendidas entre 6 y 16 años, incluidos en el registro, solamente 22 cumplieron los criterios para ser incluídos en el estudio. Entre los resultados destaca que el 32 % de la muestra cumplían los criterios de sobrepeso-obesidad y el 36 % presentaban índices de resistencia insulínica (RI) elevados. Los niveles de leptina fueron más elevados en las mujeres (15,45 vs. 3,25; p = 0,044) y en los individuos con obesidad o sobrepeso, así como la ratio leptina/adiponectina, que se eleva en presencia de RI (2,52 vs. 0,45; p = 0,037). Se observó un incremento del grosor mediointimal carotídeo en relación con el IMC (0,008; IC: -0,002 a 0,013; p = 0,007) sin asociarse a un aumento de masa grasa en estos pacientes (0,204; IC: -0,043 a 0,451; p = 0,101). Conclusiones: los pacientes supervivientes de leucemia en la edad infantil tienen un riesgo cardiovascular elevado, caracterizado por un aumento de la RI no asociado a aumento de la masa grasa. Este riesgo podría justificar la implementación de medidas preventivas en estos pacientes, cada vez más longevos.

Riesgo cardiovascular y resistencia insulínica en supervivientes de leucemia infantil / Cardiovascular risk and insulin resistance in childhood leukemia survivors

Material y métodos: estudio retrospectivo de supervivientes de leucemia aguda en edad infantil. Se seleccionaron aquellos supervivientes con diagnóstico de leucemia antes de los 16 años de edad, en un hospital de tercer nivel y durante el período 1998-2018, que hubieran finalizado su tratamiento como mínimo dos años antes. Se analizaron: niveles de adipokinas y metabolismo hidrocarbonado en sangre, composición corporal mediante bioimpedancia y evaluación ecográfica carotídea. Se tomaron además datos somatométricos. Resultados: de 82 niños con diagnóstico de leucemia aguda, con edades comprendidas entre 6 y 16 años, incluidos en el registro, solamente 22 cumplieron los criterios para ser incluídos en el estudio. Entre los resultados destaca que el 32 % de la muestra cumplían los criterios de sobrepeso-obesidad y el 36 % presentaban índices de resistencia insulínica (RI) elevados. Los niveles de leptina fueron más elevados en las mujeres (15,45 vs. 3,25; p = 0,044) y en los individuos con obesidad o sobrepeso, así como la ratio leptina/adiponectina, que se eleva en presencia de RI (2,52 vs. 0,45; p = 0,037). Se observó un incremento del grosor mediointimal carotídeo en relación con el IMC (0,008; IC: -0,002 a 0,013; p = 0,007) sin asociarse a un aumento de masa grasa en estos pacientes (0,204; IC: -0,043 a 0,451; p = 0,101). Conclusiones: los pacientes supervivientes de leucemia en la edad infantil tienen un riesgo cardiovascular elevado, caracterizado por un aumento de la RI no asociado a aumento de la masa grasa. Este riesgo podría justificar la implementación de medidas preventivas en estos pacientes, cada vez más longevos. (AU)

Material and methods: a retrospective study of childhood acute leukemia survivors. Survivors with a diagnosis of leukemia before 16 years of age in a tertiary hospital, during the period of 1998-2018, were selected, who had completed their treatment at least two years earlier. We examined: blood adipokine levels and carbohydrate metabolism, body composition by bioimpedance, and carotid status by ultrasound. Somatometric measures were also taken. Results: the registry showed 82 children diagnosed with acute leukemia, aged between 6 and 16 years. Only 22 met the criteria to be included in the study. Results reveled that 32 % of the sample met the criteria for overweight-obesity, and 36 % had high insulin resistance indexes (IR). Leptin levels were higher in women (15.45 vs. 3.25; p = 0.044) and in obese and overweight subjects, as was the leptin/adiponectin ratio, which rises in the presence of IR (2.52 vs. 0.45; p = 0.037). We observed an increase in carotid intima-media thickness in relation to BMI (0.008; CI, -0.002 to 0.013; p = 0.007) without any association with an increase in fat mass in these patients (0.204; CI, -0.043 to 0.451; p = 0.101). Conclusions: childhood leukemia survivors have a high cardiovascular risk, characterized by an increase in IR, not associated with an increase in fat mass. This risk could justify the implementation of preventive actions in these long-lived patients. (AU)

Consensus document on the primary prevention of cow's milk protein allergy in infants aged less than 7 days.

INTRODUCTION: Cow's milk protein allergy (CMPA) is the most frequent food allergy in the first year of life. There is no clear consensus regarding its prevention. A recommendation to avoid CMP in the first week of life as a preventive measure in all infants, regardless of their atopic risk, has recently been published. The purpose of this document is to issue a recommendation on the use of extensively hydrolyzed CMP formulas in the first week of life for the primary prevention of CMPA. METHODS: A group of experts was formed with members proposed by the Spanish Association of Pediatrics (AEP), the Spanish Society of Clinical Immunology and Allergology and Pediatric Asthma (SEICAAP), the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) and the Spanish Society of Neonatology (SENEO). The group conducted a critical review of the evidence on the subject published in the last 10 years. RESULTS: The search yielded 72 studies, of which 66 were rejected for not meeting the inclusion criteria. The final review included 6 documents: 3 clinical trials and 3 systematic reviews, 2 of them with meta-analysis. There was no evidence of a statistically significant reduction in the incidence of CMPA in the infants who received hypoallergenic formulae or exclusive breastfeeding. CONCLUSION: Based on the current evidence, it is not possible to draw clear conclusions about the effect of avoiding CMP in the first week of life for prevention of CMPA. Although there are data that suggest a certain beneficial effect of avoiding CMPA in atopic risk infants, these results are not conclusive enough to extend the recommendation to the general population.

Documento de consenso en la prevención primaria de alergia a proteínas de leche de vaca en lactantes menores de 7 días de vida / Consensus document on the primary prevention of cow's milk protein allergy in infants aged less than 7days

Introducción: La alergia a las proteínas de la leche de vaca (APLV) es la alergia alimentaria más frecuente en el primer año de vida. No existe un consenso claro respecto a su prevención. Recientemente se ha publicado la recomendación de evitar estas proteínas en la primera semana de vida como medida de prevención en todos los niños, con independencia de su riesgo atópico. El objetivo de este documento es emitir una recomendación sobre el uso de fórmulas extensamente hidrolizadas de PLV en la primera semana de vida para la prevención primaria de la APLV. Métodos: Se constituyó un grupo de expertos propuestos por la Asociación Española de Pediatría (AEP), la Sociedad Española de Inmunología Clínica y Alergología y Asma Pediátrica (SEICAAP), la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP) y la Sociedad Española de Neonatología (SENEO). Se realizó una revisión crítica de la evidencia publicada en los últimos 10 años sobre el tema. Resultados: Se seleccionaron 72 estudios, de los cuales 66 fueron rechazados por no cumplir los criterios de inclusión. Se incluyeron en la revisión 6 documentos: 3 ensayos clínicos y 3 revisiones sistemáticas, 2de ellas con metaanálisis. No se observó una reducción estadísticamente significativa en la incidencia de APLV en los grupos de lactantes que recibieron fórmulas hipoalergénicas ni lactancia materna exclusiva. Conclusión: Con base en las evidencias existentes en la actualidad, no se pueden establecer conclusiones claras acerca del efecto de evitar las PLV durante la primera semana de vida en la prevención de la APLV. A pesar de existir datos que pudieran orientar a un cierto efecto beneficioso de su evitación en niños con riesgo atópico, estos resultados no son concluyentes ni generalizables a lactantes sin dicho riesgo. (AU)

Introduction: Cow's milk protein allergy (CMPA) is the most frequent food allergy in the first year of life. There is no clear consensus regarding its prevention. A recommendation to avoid CMP in the first week of life as a preventive measure in all infants, regardless of their atopic risk, has recently been published. The purpose of this document is to issue a recommendation on the use of extensively hydrolyzed CMP formulas in the first week of life for the primary prevention of CMPA. Methods: A group of experts was formed with members proposed by the Spanish Association of Pediatrics (AEP), the Spanish Society of Clinical Immunology and Allergology and Pediatric Asthma (SEICAAP), the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) and the Spanish Society of Neonatology (SENEO). The group conducted a critical review of the evidence on the subject published in the last 10 years. Results: The search yielded 72 studies, of which 66 were rejected for not meeting the inclusion criteria. The final review included 6 documents: 3 clinical trials and 3 systematic reviews, 2 of them with meta-analysis. There was no evidence of a statistically significant reduction in the incidence of CMPA in the infants who received hypoallergenic formulae or exclusive breastfeeding. Conclusion: Based on the current evidence, it is not possible to draw clear conclusions about the effect of avoiding CMP in the first week of life for prevention of CMPA. Although there are data that suggest a certain beneficial effect of avoiding CMPA in atopic risk infants, these results are not conclusive enough to extend the recommendation to the general population. (AU)

Vitamin D Status in Pediatric and Young Adult Cystic Fibrosis Patients. Are the New Recommendations Effective?

INTRODUCTION: In recent years, guidelines for vitamin D supplementation have been updated and prophylactic recommended doses have been increased in patients with cystic fibrosis (CF). OBJECTIVE: To evaluate safety and efficacy of these new recommendations. RESULTS: Two cohorts of pancreatic insufficient CF patients were compared before (cohort 1: 179 patients) and after (cohort 2: 71 patients) American CF Foundation and European CF Society recommendations were published. Cohort 2 patients received higher Vitamin D doses: 1509 (1306-1711 95% CI) vs 1084 (983-1184 95% CI) IU/Day (p < 0.001), had higher 25 OH vitamin D levels: 30.6 (27.9-33.26 95% CI) vs. 27.4 (25.9-28.8 95% CI) ng/mL (p = 0.028), and had a lower prevalence of insufficient vitamin D levels (<30 ng/mL): 48% vs 65% (p = 0.011). Adjusted by confounding factors, patients in cohort 1 had a higher risk of vitamin D insufficiency: OR 2.23 (1.09-4.57 95% CI) (p = 0.028). CONCLUSION: After the implementation of new guidelines, CF patients received higher doses of vitamin D and a risk of vitamin D insufficiency decreased. Despite this, almost a third of CF patients still do not reach sufficient serum calcidiol levels.

Satisfacción de las consultas telefónicas realizadas durante el confinamiento por COVID-19 / Satisfaction in telephone consultations during confinement due to COVID-19

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Análisis de la calidad de vida relacionada con la salud en pacientes celiacos / Analysis of health-related quality life in celiac patients

Introducción: el tratamiento de la enfermedad celiaca es una dieta sin gluten de por vida, lo cual puede repercutir en la calidad de vida (CV) de los pacientes. Objetivos: nuestro objetivo fue evaluar la CV de una muestra de niños celiacos y estudiar los factores que pueden influir en la misma. Material y métodos: estudio observacional descriptivo. Se estudió la CV con el cuestionario Celiac Disease Dux Questionnaire (CDDUX). Se estudió la adherencia con el cuestionario Celiac Dietary Adherence Test (CDAT) y la determinación de péptidos inmunogénicos del gluten (GIP) en heces. Se recogieron datos sociodemográficos y clínicos, y se elaboró una encuesta ad hoc. Resultados: se incluyeron 80 pacientes. La mediana del CDDUX fue de 44,04 puntos (CV "neutra"); la de la subescala "comunicación" fue de 58,3 ("neutra"), la de "tener EC" fue de 25 ("mala") y la de "dieta" fue de 41,6 puntos ("neutra"). La CV fue peor en los pacientes con familiares celiacos ("mala" frente a "neutra", p = 0,02) y en aquellos insatisfechos con las características somatosensoriales y el precio de los alimentos sin gluten ("mala" frente a "neutra", p = 0,02). Los insatisfechos con la textura de estos alimentos tenían peor CV ("mala" frente a "neutra", p = 0,009). Los que consideraban comer fuera de casa como factor inductor de transgresiones referían una CV "mala" y los que no, una "neutra" (p = 0,03). Conclusiones: los pacientes celiacos tienen una CV neutra. El hecho de tener familiares con enfermedad celiaca, la insatisfacción con los alimentos sin gluten y el considerar un factor inductor de transgresiones el comer fuera de casa se relacionaron con una peor calidad de vida. (AU)

Introduction: the treatment of celiac disease is gluten-free diet for life. This can impact the quality of life (QoL) of patients. Objectives: the objective was to evaluate the QoL and the factors with an impact on QoL in a sample of celiac children. Methods and materials: a descriptive observational study. QoL was evaluated using the Celiac Disease Dux Questionnaire (CDDUX). Adherence to gluten-free diet was assessed with the Celiac Dietary Adherence Test (CDAT) and the presence of gluten immunogenic peptides (GIP) in the feces. Sociodemographic and clinical data were collected, and an ad-hoc survey was developed. Results: eighty patients were included. Median CDDUX score was 44.04 points (QoL: "neutral"). Subscale scores included: "communication", 58.3 points ("neutral"); "having CD", 25 points ("Bad"); and "diet", 41.6 points ("neutral"). QoL was worse among patients with celiac relatives (the result of the survey was "bad" vs. "neutral" with p = 0.02) and among those who found unsatisfactory the somatosensory characteristics and the price of gluten-free food (the result of the survey was "bad" vs. "neutral" with p = 0.02). Those who found unsatisfactory the texture of these food reported a worse QoL ("bad" vs. "neutral", p = 0.009). Those who reported eating outside the home as a transgression inducer reported a "bad" QoL; those who did not, reported a "neutral" QoL (p = 0.03). Conclusions: celiac patients report a "neutral" QoL. A poorer QoL was related to having celiac relatives, finding gluten-free food unsatisfactory, and considering eating outside the home as an inducer. (AU)

Study of adherence to the gluten-free diet in coeliac patients.

INTRODUCTION: The following of a strict gluten-free diet (GFD) is essential in the control of coeliac disease. The aim of this study was to determine the adherence to a GFD in coeliac patients and to evaluate the factors that could influence this adherence. MATERIAL AND METHODS: A descriptive observational study was carried out, in which gluten immunogenic peptides (GIP) were determined in faeces using a semi-quantitative method, and the Coeliac Dietary Adherence Test was completed. Sociodemographic and clinical details were collected, and an ad hoc questionnaire was prepared. RESULTS: Of the 80 patients included, 92.5% were adherent according to the GIP and 86.3% according to Coeliac Dietary Adherence Test (acceptable agreement; Kappa: 0.31, P = .004). The large majority (83.3%) of patients with positive GIP gave negative anti-transglutaminase antibodies in the latest determination. Current age and time of onset were significantly associated with adherence. Those with a positive GIP had a mean age of 5 years more (P = .0001) and were 52 months more on a GFD (P = .025). One quarter of those surveyed considered the diet difficult to follow. Just under two-thirds (60%) considered that the variability in the eating site was an important factor in leading to infringements, with children's parties being the main area where they occurred (66.7%). The lack of variety (61.4%) and the increased cost (98.6%) of gluten-free foods is highlighted. CONCLUSIONS: The adherence to the GFD is generally good. The analysis of GIP helps to detect non-adherent patients that would pass unnoticed in other circumstances. Measures must be established in order to maintain good long-term adherence, taking into account the risk factors and difficulties detected.

Estudio de la adherencia a la dieta sin gluten en pacientes celiacos / Study of adherence to the gluten-free diet in coeliac patients

Introducción: La realización estricta de una dieta sin gluten (DSG) es fundamental para el control de la enfermedad celiaca. El objetivo del estudio fue analizar la adherencia a la DSG en celiacos y evaluar factores que pudieran influir en la misma. Material y métodos: Estudio observacional descriptivo. Se realizó una determinación de péptidos inmunogénicos del gluten (GIP) en heces con método semicuantitativo y se cumplimentó el cuestionario Celiac Dietary Adherence Test. Se recogieron datos sociodemográficos, clínicos y se elaboró una encuesta ad hoc. Resultados: Se incluyeron 80 pacientes. El 92,5% eran adherentes mediante GIP y 86,3% con Celiac Dietary Adherence Test (concordancia aceptable; Kappa: 0,31, p=0,004). El 83,3% de los pacientes con GIP positivos tenía la última determinación de anticuerpos antitransglutaminasa negativos. La edad actual y el tiempo de evolución se asociaron significativamente con la adherencia. Aquellos con GIP positivos tenían de media 5 años más (p=0,0001) y llevaban 52 meses más de DSG (p=0,025). Una cuarta parte de los encuestados consideraba difícil realizar la dieta. El 60% consideraba que la variabilidad en el lugar de comida era importante para inducir transgresiones, siendo las fiestas infantiles el principal lugar donde sucedían (66,7%). Se destaca la escasa variedad (61,4%) y el elevado coste (98,6%) de los alimentos sin gluten. Conclusiones: La adherencia a la DSG es en general, buena. El análisis de GIP permitió detectar a pacientes no adherentes que en otras circunstancias pasarían desapercibidos. Se deben establecer medidas para mantener una buena adhesión de manera prolongada, considerando los factores de riesgo y dificultades detectados. (AU)

Introduction: The following of a strict gluten-free diet (GFD) is essential in the control of coeliac disease. The aim of this study was to determine the adherence to a GFD in coeliac patients and to evaluate the factors that could influence this adherence. Material and methods: A descriptive observational study was carried out, in which gluten immunogenic peptides (GIP) were determined in faeces using a semi-quantitative method, and the Coeliac Dietary Adherence Test was completed. Sociodemographic and clinical details were collected, and an ad hoc questionnaire was prepared. Results: Of the 80 patients included, 92.5% were adherent according to the GIP and 86.3% according to Coeliac Dietary Adherence Test (acceptable agreement; Kappa: 0.31, P=.004). The large majority (83.3%) of patients with positive GIP gave negative anti-transglutaminase antibodies in the latest determination. Current age and time of onset were significantly associated with adherence. Those with a positive GIP had a mean age of 5 years more (P=.0001) and were 52 months more on a GFD (P=.025). One quarter of those surveyed considered the diet difficult to follow. Just under two-thirds (60%) considered that the variability in the eating site was an important factor in leading to infringements, with children's parties being the main area where they occurred (66.7%). The lack of variety (61.4%) and the increased cost (98.6%) of gluten-free foods is highlighted. Conclusions: The adherence to the GFD is generally good. The analysis of GIP helps to detect non-adherent patients that would pass unnoticed in other circumstances. Measures must be established in order to maintain good long-term adherence, taking into account the risk factors and difficulties detected. (AU)

[Analysis of health-related quality life in celiac patients]. / Análisis de la calidad de vida relacionada con la salud en pacientes celiacos.

INTRODUCTION: Introduction: treatment of celiac disease is gluten-free diet for life. This can impact the quality of life (QoL) of patients. Objectives:the aim of this study was to evaluate the QoL and the factors with an impact on QoL in a sample of children with celiac disease. Methods and materials: a descriptive observational study. QoL was evaluated using the Celiac Disease Dux Questionnaire (CDDUX). Adherence to gluten-free diet was assessed with the Celiac Dietary Adherence Test (CDAT) and the presence of gluten immunogenic peptides (GIP) in the stools. Sociodemographic and clinical data were collected, and an ad-hoc survey was developed. Results: eighty patients were included. Median CDDUX score was 44.04 points (QoL: "neutral"). Subscale scores included: "communication", 58.3 points ("neutral"); "having CD", 25 points ("Bad"); and "diet", 41.6 points ("neutral"). QoL was worse among patients with celiac relatives (the result of the survey was "bad" vs. "neutral" with p = 0.02) and among those who found unsatisfactory the somatosensory characteristics and the price of gluten-free food (the result of the survey was "bad" vs. "neutral" with p = 0.02). Those who found unsatisfactory the texture of these food reported a worse QoL ("bad" vs. "neutral", p = 0.009). Those who reported eating outside the home as a transgression inducer reported a "bad" QoL; those who did not, reported a "neutral" QoL (p = 0.03). Conclusions: celiac patients report a "neutral" QoL. A poorer QoL was related to having celiac relatives, finding gluten-free food unsatisfactory, and considering eating outside the home as an inducer factor for transgressions.

INTRODUCCIÓN: Introducción: el tratamiento de la enfermedad celiaca es una dieta sin gluten de por vida, lo cual puede repercutir en la calidad de vida (CV) de los pacientes. Objetivos: nuestro objetivo fue evaluar la CV de una muestra de niños celiacos y estudiar los factores que pueden influir en la misma. Material y métodos: estudio observacional descriptivo. Se estudió la CV con el cuestionario Celiac Disease Dux Questionnaire (CDDUX). Se estudió la adherencia con el cuestionario Celiac Dietary Adherence Test (CDAT) y la determinación de péptidos inmunogénicos del gluten (GIP) en heces. Se recogieron datos sociodemográficos y clínicos, y se elaboró una encuesta ad hoc. Resultados: se incluyeron 80 pacientes. La mediana del CDDUX fue de 44,04 puntos (CV "neutra"); la de la subescala "comunicación" fue de 58,3 ("neutra"), la de "tener EC" fue de 25 ("mala") y la de "dieta" fue de 41,6 puntos ("neutra"). La CV fue peor en los pacientes con familiares celiacos ("mala" frente a "neutra", p = 0,02) y en aquellos insatisfechos con las características somatosensoriales y el precio de los alimentos sin gluten ("mala" frente a "neutra", p = 0,02). Los insatisfechos con la textura de estos alimentos tenían peor CV ("mala" frente a "neutra", p = 0,009). Los que consideraban comer fuera de casa como factor inductor de transgresiones referían una CV "mala" y los que no, una "neutra" (p = 0,03). Conclusiones: los pacientes celiacos tienen una CV neutra. El hecho de tener familiares con enfermedad celiaca, la insatisfacción con los alimentos sin gluten y el considerar un factor inductor de transgresiones el comer fuera de casa se relacionaron con una peor calidad de vida.

Gluten-Free Diet: Nutritional Strategies to Improve Eating Habits in Children with Celiac Disease: A Prospective, Single-arm Intervention Study.

BACKGROUND: Elimination of gluten-containing cereals and consumption of ultra-processed gluten-free foods might cause an unbalanced diet, deficient in fiber and rich in sugar and fat, circumstances that may predispose celiac children to chronic constipation. AIM: to evaluate if counseling with a registered dietitian (RD) was capable of improving eating and bowel habits in a celiac pediatric population. METHODS: Dietetic, lipid profile and stool modifications were analyzed, comparing baseline assessments with those twelve months after receiving heathy eating and nutrition education sessions. At both time points, 3-day food records, a bowel habit record and a lipid panel were conducted. Calculated relative intake of macro- and micro-nutrients were compared with current recommendations by the European Food Safety Authority (EFSA). Student's paired t-test, McNemar test, Mandasky test and Pearson correlation tests were used. RESULTS: Seventy-two subjects (58.3% girls) with a mean (standard deviation (SD)) age of 10.2 (3.4) years were included. Baseline diets were imbalanced in macronutrient composition. Significant improvements were observed in their compliance with dietary reference values (DRVs), where 50% of the subjects met fat requirements after the education and 67% and 49% with those of carbohydrates and fiber, respectively (p < 0.001). Celiac children decreased red meat and ultra-processed foods consumption (p < 0.001) and increased fruits and vegetables intake (p < 0.001), leading to a reduction in saturated fat (p < 0.001) and sugar intake (p < 0.001). Furthermore, 92% of the patients achieved a normal bowel habit, including absence of hard stools in 80% of children constipated at baseline (p < 0.001). CONCLUSIONS: RD-led nutrition education is able to improve eating patterns in children with celiac disease (CD).