Fenilquetonuria de diagnóstico neonatal y lactancia materna

Phenylketonuria (PKU) is due to of a defect in the phenylalanine hydroxylase gene (12q22-24.1) leading to hyperphenylalaninemia. Treatment consists in a low phenylalanine (Phe) diet. Aim: To evaluate the evolution of early diagnosed PKU children, receiving direct breast feeding, and a special formul...

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Autores principales:	Cornejo E,Verónica, Manríquez E,Viviana, Colombo C,Marta, Mabe S,Paulina, Jiménez M,Mónica, De la Parra C,Alicia, Valiente G,Alf, Raimann B,Erna
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2003
Materias:	Breast feeding Phenylalanine hydroxylase Phenylketonuria
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872003001100008
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spelling	oai:scielo:S0034-988720030011000082004-01-27Fenilquetonuria de diagnóstico neonatal y lactancia maternaCornejo E,VerónicaManríquez E,VivianaColombo C,MartaMabe S,PaulinaJiménez M,MónicaDe la Parra C,AliciaValiente G,AlfRaimann B,Erna Breast feeding Phenylalanine hydroxylase Phenylketonuria Phenylketonuria (PKU) is due to of a defect in the phenylalanine hydroxylase gene (12q22-24.1) leading to hyperphenylalaninemia. Treatment consists in a low phenylalanine (Phe) diet. Aim: To evaluate the evolution of early diagnosed PKU children, receiving direct breast feeding, and a special formula without Phe, during their first six months of life. Patients and methods: Nineteen PKU children diagnosed in the neonatal period (19.29±13.8 days of age), treated with breast feeding and formula without Phe since diagnosis, were studied. Intake of calories, proteins and dietary Phe were quantified. Blood Phe, nutritional status and psychomotor development were also measured. Results: The diet that these children received during the 6 months period of study, had a mean of 127±19.9 Kcal/kg/day, 1.95±0.3 g protein/kg/day and 35.3±9.5 mg Phe/kg/day. Fifteen children maintained the blood level of Phe under 8 mg/dl, considered an excellent metabolic control. Only 4 cases had intermittently high levels, between 10-12 mg/dl. At 6 months of age, 74% of the children maintained breast feeding as the only source of Phe. Sixty three percent had a normal nutritional status, 5.2% were at nutritional risk and 31.6% were overweight. Eighty one percent had a normal mental development. Conclusions: The use of direct breast feeding allows a good metabolic control and improves growth and development of early diagnosed PKU children (Rev Méd Chile 2003; 131: 1280-87)info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.131 n.11 20032003-11-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872003001100008es10.4067/S0034-98872003001100008
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Breast feeding Phenylalanine hydroxylase Phenylketonuria
spellingShingle	Breast feeding Phenylalanine hydroxylase Phenylketonuria Cornejo E,Verónica Manríquez E,Viviana Colombo C,Marta Mabe S,Paulina Jiménez M,Mónica De la Parra C,Alicia Valiente G,Alf Raimann B,Erna Fenilquetonuria de diagnóstico neonatal y lactancia materna
description	Phenylketonuria (PKU) is due to of a defect in the phenylalanine hydroxylase gene (12q22-24.1) leading to hyperphenylalaninemia. Treatment consists in a low phenylalanine (Phe) diet. Aim: To evaluate the evolution of early diagnosed PKU children, receiving direct breast feeding, and a special formula without Phe, during their first six months of life. Patients and methods: Nineteen PKU children diagnosed in the neonatal period (19.29±13.8 days of age), treated with breast feeding and formula without Phe since diagnosis, were studied. Intake of calories, proteins and dietary Phe were quantified. Blood Phe, nutritional status and psychomotor development were also measured. Results: The diet that these children received during the 6 months period of study, had a mean of 127±19.9 Kcal/kg/day, 1.95±0.3 g protein/kg/day and 35.3±9.5 mg Phe/kg/day. Fifteen children maintained the blood level of Phe under 8 mg/dl, considered an excellent metabolic control. Only 4 cases had intermittently high levels, between 10-12 mg/dl. At 6 months of age, 74% of the children maintained breast feeding as the only source of Phe. Sixty three percent had a normal nutritional status, 5.2% were at nutritional risk and 31.6% were overweight. Eighty one percent had a normal mental development. Conclusions: The use of direct breast feeding allows a good metabolic control and improves growth and development of early diagnosed PKU children (Rev Méd Chile 2003; 131: 1280-87)
author	Cornejo E,Verónica Manríquez E,Viviana Colombo C,Marta Mabe S,Paulina Jiménez M,Mónica De la Parra C,Alicia Valiente G,Alf Raimann B,Erna
author_facet	Cornejo E,Verónica Manríquez E,Viviana Colombo C,Marta Mabe S,Paulina Jiménez M,Mónica De la Parra C,Alicia Valiente G,Alf Raimann B,Erna
author_sort	Cornejo E,Verónica
title	Fenilquetonuria de diagnóstico neonatal y lactancia materna
title_short	Fenilquetonuria de diagnóstico neonatal y lactancia materna
title_full	Fenilquetonuria de diagnóstico neonatal y lactancia materna
title_fullStr	Fenilquetonuria de diagnóstico neonatal y lactancia materna
title_full_unstemmed	Fenilquetonuria de diagnóstico neonatal y lactancia materna
title_sort	fenilquetonuria de diagnóstico neonatal y lactancia materna
publisher	Sociedad Médica de Santiago
publishDate	2003
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872003001100008
work_keys_str_mv	AT cornejoeveronica fenilquetonuriadediagnosticoneonatalylactanciamaterna AT manriquezeviviana fenilquetonuriadediagnosticoneonatalylactanciamaterna AT colombocmarta fenilquetonuriadediagnosticoneonatalylactanciamaterna AT mabespaulina fenilquetonuriadediagnosticoneonatalylactanciamaterna AT jimenezmmonica fenilquetonuriadediagnosticoneonatalylactanciamaterna AT delaparracalicia fenilquetonuriadediagnosticoneonatalylactanciamaterna AT valientegalf fenilquetonuriadediagnosticoneonatalylactanciamaterna AT raimannberna fenilquetonuriadediagnosticoneonatalylactanciamaterna
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Fenilquetonuria de diagnóstico neonatal y lactancia materna

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