RESUMEN
BACKGROUND: There appears little consensus concerning protein requirements in phenylketonuria (PKU). METHODS: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. RESULTS: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). CONCLUSIONS: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.
Asunto(s)
Aminoácidos/administración & dosificación , Caseínas/administración & dosificación , Proteínas en la Dieta/administración & dosificación , Suplementos Dietéticos , Fragmentos de Péptidos/administración & dosificación , Fenilcetonurias/dietoterapia , Adulto , Niño , Preescolar , Europa (Continente) , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Fenilalanina , Encuestas y Cuestionarios , Turquía , Organización Mundial de la SaludRESUMEN
Se revisan las necesidades de agua en la infancia, así como las características de los tipos de agua para el consumo humano. Se indica la concentración ideal de sodio, calcio, nitratos y flúor en las aguas de bebida para los niños según su edad. Se revisa el papel de los zumos y las bebidas de refresco como factores de riesgo de obesidad y sus comorbilidades. Se recuerda que el agua y la leche son las dos bebidas fundamentales de los niños y adolescentes. Finalmente, se revisa la importancia de una buena hidratación en caso de realizar ejercicio físico (AU)
The needs of water in childhood, as well as the characteristics of the types of water for human consumption, are reviewed. The ideal concentration of sodium, calcium, nitrates and fluoride in the water that is drank by children according to their age group. The role of juices and soda drinks is also reviewed as a risk factor of obesity and its comorbidities. It is recalled that water and milk are two fundamental drinks in children and adolescents. Finally, the importance of a good hydration if doing exercise is recalled (AU)
Asunto(s)
Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Adolescente , Agua Potable/uso terapéutico , Agua/administración & dosificación , Agua Potable/efectos adversos , Agua Potable/métodos , Zumos , Comorbilidad , Factores de Riesgo , Obesidad/epidemiología , Obesidad/inducido químicamente , Obesidad/fisiopatología , Obesidad Mórbida/epidemiología , Obesidad Mórbida/prevención & controlAsunto(s)
Técnicas Cosméticas/efectos adversos , Infecciones por Mycobacterium no Tuberculosas/microbiología , Micobacterias no Tuberculosas/aislamiento & purificación , Infecciones de los Tejidos Blandos/microbiología , Adulto , Antiinfecciosos/uso terapéutico , Quimioterapia Combinada , Femenino , Humanos , Inyecciones Subcutáneas/efectos adversos , Persona de Mediana Edad , Infecciones de los Tejidos Blandos/patología , Resultado del TratamientoRESUMEN
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Asunto(s)
Humanos , Niño , Ácidos Linoleicos Conjugados/uso terapéutico , Obesidad/dietoterapia , Sobrepeso/dietoterapia , Suplementos Dietéticos , Obesidad/epidemiología , Sobrepeso/epidemiología , Alimentos Integrales , Conducta AlimentariaRESUMEN
The serum of a patient with subacute cerebellar dysfunction was used to probe a cDNA expression library and isolate two genes: Zic1 (zinc-finger of the cerebellum) and Zic4. The patient had intrathecal synthesis of Zic antibodies, suggesting that the Zic proteins were autoantigens of the neurologic disorder. The Zic proteins are involved in cerebellar development and are reported as being preferentially expressed by medulloblastomas. It was found that the expression of Zic proteins is enriched in, but not limited to, medulloblastomas and primitive neuroectodermal tumors.
Asunto(s)
Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/patología , Enfermedades Cerebelosas/patología , Cerebelo/patología , Degeneración Nerviosa/patología , Dedos de Zinc/inmunología , Anciano , Autoanticuerpos/análisis , Autoanticuerpos/líquido cefalorraquídeo , Autoanticuerpos/inmunología , Western Blotting , Neoplasias Cerebelosas/inmunología , Neoplasias Cerebelosas/patología , ADN Complementario/genética , Humanos , Inmunohistoquímica , Imagen por Resonancia Magnética , Masculino , Meduloblastoma/inmunología , Meduloblastoma/patología , Tumores Neuroectodérmicos/inmunología , Tumores Neuroectodérmicos/patologíaRESUMEN
Lambert-Eaton myasthenic syndrome is a paraneoplastic neuromuscular disorder in which an immune response directed against a small-cell lung tumor crossreacts with antigens in the neuromuscular junction. To isolate and characterize the antigens, we screened a human fetal brain expression library with a high-titer serum from a patient with Lambert-Eaton myasthenic syndrome. This screening resulted in the isolation of a complementary DNA clone encoding an antigen we call myasthenic syndrome antigen B (MysB). Approximately 43% (3 of 7) of Lambert-Eaton myasthenic syndrome sera specifically recognized MysB fusion protein, whereas none of 34 control sera did. The predicted amino acid sequence of this clone shows a high degree of homology to the beta subunit of calcium channel complexes. The MysB pre-messenger RNA is alternatively spliced to yield 3 forms of the protein differing in the domain between two highly conserved alpha-helical segments.