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BACKGROUND & AIMS: Different nutritional screening tools have been proposed in childhood cancer, but none has shown convincing predictive capacity so far. The "nutrition screening tool for childhood cancer (SCAN)" has been specifically designed for this population and provides an easy-to-use, promising approach to identify patients at risk of malnutrition. We aim to: 1. Validate the SCAN tool prospectively in identifying malnourished patients or those who required nutritional support 2. Validate the SCAN tool prospectively in predicting toxicities or outcome. 3. Compare performance of a pediatric screening tool (STRONGKIDS) with SCAN. METHODS: Children in our center with a new diagnosis of cancer from August 2018 to May 2019 were offered to participate in the study. Measurements (SCAN questionnaire, weight, height, body-mass index (BMI), and mid upper-arm circumference (MUAC)) were taken at diagnosis and at regular intervals throughout therapy. The last measurement was taken 6 months after finishing the intensive treatment phase. SCAN score at diagnosis was validated prospectively against variables of interest. RESULTS: A total of 49 patients were recruited. When considering malnutrition during therapy the SCAN tool showed a sensitivity of 37.5% and negative predictive value (NPV) of 81%. Patients who required nutritional support were identified with a sensitivity of 50% and NPV of 62%. The SCAN tool was not able to predict increased toxicities, risk of relapse or decreased survival. The pediatric screening tool STRONGKIDS was unable to discriminate nutritional risk and labeled all 49 patients (100%) as medium or high-risk. Applying SCAN periodically during therapy increased sensitivity for identifying malnutrition to 87.5%. CONCLUSIONS: In our study, applying the SCAN tool at diagnosis showed low sensitivity in identifying patients who go on to develop malnutrition during therapy. However, patients labeled as "not at risk" were unlikely to need nutritional support in the form of nasogastric tube or total parenteral nutrition. Using SCAN throughout therapy could be helpful in building awareness for malnutrition and successfully discriminates between patients who need further support and those who don't.
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AIM: Ketogenic dietary therapies (KDT) produce anticonvulsant and neuroprotective effects, reduce seizures and improve the cognitive state in patients with epilepsy. Our purpose was to evaluate the effects of KDT in children with refractory epilepsy (effectiveness, side effects, impact on nutritional status and growth). METHODS: A retrospective and prospective observational descriptive study was conducted in a Spanish tertiary hospital (January 2000 to December 2018). One hundred sixty pediatric patients with epilepsy were treated with KDT (82 males; mean age 5 years 9 months). Seizures, anti-epileptic drugs, anthropometric measures, side effects, and laboratory assessment were monitored baseline and at 3, 6, 12 and 24 months after the onset of KDT. RESULTS: In these time intervals, the seizure-free patients were: 13.7, 12.5, 14.4 and 10.6%, respectively, and a reduction of seizures ≥ 50% was achieved in 41.9, 37.5, 28.7 and 16.2%. Side effects were frequent, especially digestive disorders, hypercalciuria, hypoglycemia, hepatic dysfunction and dyslipidemia. Prealbumin, retinol binding protein, vitamin A and magnesium decreased significantly. Height was affected, especially in children below 2 years. CONCLUSIONS: KDT are effective for refractory epilepsy in children. However, adverse effects are frequent, and it may affect nutritional status and growth.
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Dieta Cetogénica , Epilepsia Refractaria , Epilepsia , Niño , Preescolar , Dieta Cetogénica/efectos adversos , Humanos , Masculino , Estudios Retrospectivos , ConvulsionesRESUMEN
Introducción: Las terapias dietéticas cetogénicas (TDC) tienen efecto neuroprotector y anticonvulsivante, reducen las crisis epilépticas y mejoran el estado cognitivo en pacientes epilépticos. Nuestro propósito fue evaluar los efectos de las TDC en niños con epilepsia refractaria (eficacia, efectos secundarios, impacto en el estado nutricional y crecimiento).Métodos: Se realizó un estudio observacional descriptivo retrospectivo y prospectivo en un hospital terciario español (enero de 2000-diciembre de 2018). Ciento sesenta pacientes pediátricos con epilepsia fueron tratados con TDC (82 varones; edad media 5 años 9 meses). Las convulsiones, los fármacos antiepilépticos, la antropometría, los efectos secundarios y los parámetros analíticos se controlaron al inicio del tratamiento y a los 3, 6, 12 y 24 meses.Resultados: En estos intervalos los pacientes libres de crisis fueron: 13,7%, 12,5%, 14,4% y 10,6%, respectivamente, lográndose una reducción de las convulsiones≥50% en el 41,9%, 37,5%, 28,7% y 16,2%. Los efectos secundarios fueron frecuentes, especialmente trastornos digestivos, hipercalciuria, hipoglucemia, disfunción hepática y dislipidemia. La prealbúmina, la proteína de unión al retinol, la vitamina A y el magnesio disminuyeron significativamente. La talla se vio afectada, especialmente en niños menores de 2 años.Conclusiones: Las TDC son efectivas para la epilepsia refractaria infantil. Sin embargo, los efectos adversos son frecuentes y pueden afectar al estado nutricional y al crecimiento. (AU)
Aim: Ketogenic dietary therapies (KDT) produce anticonvulsant and neuroprotective effects, reduce seizures and improve the cognitive state in patients with epilepsy. Our purpose was to evaluate the effects of KDT in children with refractory epilepsy (effectiveness, side effects, impact on nutritional status and growth).Methods: A retrospective and prospective observational descriptive study was conducted in a Spanish tertiary hospital (January 2000 to December 2018). One hundred sixty pediatric patients with epilepsy were treated with KDT (82 males; mean age 5 years 9 months). Seizures, anti-epileptic drugs, anthropometric measures, side effects, and laboratory assessment were monitored baseline and at 3, 6, 12 and 24 months after the onset of KDT.Results: In these time intervals, the seizure-free patients were: 13.7, 12.5, 14.4 and 10.6%, respectively, and a reduction of seizures≥50% was achieved in 41.9, 37.5, 28.7 and 16.2%. Side effects were frequent, especially digestive disorders, hypercalciuria, hypoglycemia, hepatic dysfunction and dyslipidemia. Prealbumin, retinol binding protein, vitamin A and magnesium decreased significantly. Height was affected, especially in children below 2 years.Conclusions: KDT are effective for refractory epilepsy in children. However, adverse effects are frequent, and it may affect nutritional status and growth.(AU)
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Humanos , Preescolar , Niño , Dieta Cetogénica , Epilepsia Refractaria , Anticonvulsivantes , Fármacos Neuroprotectores , Epidemiología Descriptiva , Estudios Retrospectivos , Estudios Prospectivos , EspañaRESUMEN
No standardized approach towards nutritional screening and assessment of pediatric oncology patients has been established. The nutrition screening tool for childhood cancer (SCAN) has been previously published as an effective screening method. This is an observational cross-sectional study to assess the validity and reliability of the SCAN tool, compare it to the detection of undernutrition using standard measures of assessment, and determine the overall prevalence of malnutrition and micronutrients alterations in our cohort. We included children newly diagnosed with cancer in a pediatric tertiary hospital in Madrid, Spain from August 2018 to May 2019. The following measurements were performed: SCAN questionnaire, anthropometric measurements, nutritional markers in blood, and micronutrient levels. A total of 49 patients were included. 22 patients (45%) were at risk of malnutrition according to the SCAN questionnaire. Four patients (8%) could be diagnosed with moderate undernutrition. These undernourished patients were distributed homogeneously among at-risk and not at-risk populations identified by the SCAN tool. Several micronutrient deficiencies were identified. We conclude that the SCAN questionnaire is an easy-to-use tool for everyday clinical practice. By not including anthropometric measurements it misses patients considered to be malnourished. Future data might help clarify if it is an effective tool in predicting a higher nutritional risk during the entire treatment course.
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Desnutrición , Neoplasias , Niño , Estudios Transversales , Detección Precoz del Cáncer , Humanos , Desnutrición/diagnóstico , Desnutrición/epidemiología , Desnutrición/etiología , Micronutrientes , Neoplasias/complicaciones , Evaluación Nutricional , Estado Nutricional , Reproducibilidad de los ResultadosRESUMEN
AIM: Ketogenic dietary therapies (KDT) produce anticonvulsant and neuroprotective effects, reduce seizures and improve the cognitive state in patients with epilepsy. Our purpose was to evaluate the effects of KDT in children with refractory epilepsy (effectiveness, side effects, impact on nutritional status and growth). METHODS: A retrospective and prospective observational descriptive study was conducted in a Spanish tertiary hospital (January 2000 to December 2018). One hundred sixty pediatric patients with epilepsy were treated with KDT (82 males; mean age 5 years 9 months). Seizures, anti-epileptic drugs, anthropometric measures, side effects, and laboratory assessment were monitored baseline and at 3, 6, 12 and 24 months after the onset of KDT. RESULTS: In these time intervals, the seizure-free patients were: 13.7, 12.5, 14.4 and 10.6%, respectively, and a reduction of seizures≥50% was achieved in 41.9, 37.5, 28.7 and 16.2%. Side effects were frequent, especially digestive disorders, hypercalciuria, hypoglycemia, hepatic dysfunction and dyslipidemia. Prealbumin, retinol binding protein, vitamin A and magnesium decreased significantly. Height was affected, especially in children below 2 years. CONCLUSIONS: KDT are effective for refractory epilepsy in children. However, adverse effects are frequent, and it may affect nutritional status and growth.
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BACKGROUND: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is caused by mutations in the SLC2A1 gene and produces seizures, neurodevelopmental impairment, and movement disorders. Ketogenic dietary therapies (KDT) are the gold standard treatment. Similar symptoms may appear in SLC2A1 negative patients. The purpose is to evaluate the effectiveness of KDT in children with GLUT1DS suspected SLC2A1 (+) and (-), side effects (SE), and the impact on patients nutritional status. METHODS: An observational descriptive study was conducted to describe 18 children (January 2009-August 2020). SLC2A1 analysis, seizures, movement disorder, anti-epileptic drugs (AEDS), anthropometry, SE, and laboratory assessment were monitored baseline and at 3, 6, 12, and 24 months after the onset of KDT. RESULTS: 6/18 were SLC2A1(+) and 13/18 had seizures. In these groups, the age for debut of symptoms was higher. The mean time from debut to KDT onset was higher in SLC2A1(+). The modified Atkins diet (MAD) was used in 12 (5 SLC2A1(+)). Movement disorder improved (4/5), and a reduction in seizures >50% compared to baseline was achieved in more than half of the epileptic children throughout the follow-up. No differences in effectiveness were found according to the type of KDT. Early SE occurred in 33%. Long-term SE occurred in 10, 5, 7, and 5 children throughout the follow-up. The most frequent SE were constipation, hypercalciuria, and hyperlipidaemia. No differences in growth were found according to the SLC2A1 mutation or type of KDT. CONCLUSIONS: CKD and MAD were effective for SLC2A1 positive and negative patients in our cohort. SE were frequent, but mild. Permanent monitoring should be made to identify SE and nutritional deficits.
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Errores Innatos del Metabolismo de los Carbohidratos/dietoterapia , Errores Innatos del Metabolismo de los Carbohidratos/genética , Dieta Rica en Proteínas y Pobre en Hidratos de Carbono/métodos , Dieta Cetogénica/métodos , Transportador de Glucosa de Tipo 1/análisis , Proteínas de Transporte de Monosacáridos/deficiencia , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Proteínas de Transporte de Monosacáridos/genética , Trastornos del Movimiento/dietoterapia , Trastornos del Movimiento/genética , Mutación , Convulsiones/dietoterapia , Convulsiones/genética , Resultado del TratamientoRESUMEN
BACKGROUND: The ketogenic diet (KD) is an effective treatment against drug-resistant epilepsy in children. The KD is a diet rich in fats that produces anticonvulsant and neuroprotective effects that reduces seizures and improves the cognitive state. Nevertheless, it can produce side effects that sometimes can be serious. Further, the effect on growth is quite controversial when used for an extended period of time. The aim of this paper was to assess the effectiveness, side effects, and repercussions in the development of children who have been treated with a KD for more than 2 years. METHODS: Observational descriptive study of 26 pediatric patients on a KD, with data collection at baseline, at 3, 6, and 12 months, and then once a year. Number of seizures, type of seizures, anti-seizure drugs, anthropometry, side effects, and alterations in laboratory assessment were monitored. RESULTS: In every assessment, about 60%-75% of the patients experienced a reduction in number of seizures of over 90%, and at least 50% experienced side effects, of which digestive issues, alteration in the lipid metabolism, and hypercalciuria were the most common. The KD significantly affected height after 2 years of treatment. CONCLUSIONS: The KD is an effective treatment for drug-resistant epilepsy. Its side effects, although common, are very mild; therefore, this constitutes a very safe treatment for children of all ages. More studies are needed to identify and prevent potential causes of growth retardation in children on the KD.