Your browser doesn't support javascript.
loading
Fenilquetonuria de diagnóstico neonatal y lactancia materna / Phenylketonuria diagnosed during the neonatal period and breast feeding
Cornejo E, Verónica; Manríquez E, Viviana; Colombo C, Marta; Mabe S, Paulina; Jiménez M, Mónica; Parra C, Alicia De la; Valiente G, Alf; Raimann B, Erna.
  • Cornejo E, Verónica; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Manríquez E, Viviana; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Colombo C, Marta; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Mabe S, Paulina; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Jiménez M, Mónica; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Parra C, Alicia De la; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Valiente G, Alf; Universidad de Chile. Unidad de Nutrición Clínica. CL
  • Raimann B, Erna; Universidad de Chile. Unidad de Nutrición Clínica. CL
Rev. méd. Chile ; 131(11): 1280-1287, nov. 2003. tab, graf
Article in Spanish | LILACS | ID: lil-358947
Responsible library: CL12.1
RESUMO
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).
Subject(s)
Full text: Available Index: LILACS (Americas) Main subject: Phenylalanine Hydroxylase / Phenylketonurias / Breast Feeding Type of study: Prevalence study Limits: Female / Humans / Infant / Male / Infant, Newborn Language: Spanish Journal: Rev. méd. Chile Journal subject: Medicine Year: 2003 Type: Article Affiliation country: Chile Institution/Affiliation country: Universidad de Chile/CL

Similar

MEDLINE

...
LILACS

LIS

Full text: Available Index: LILACS (Americas) Main subject: Phenylalanine Hydroxylase / Phenylketonurias / Breast Feeding Type of study: Prevalence study Limits: Female / Humans / Infant / Male / Infant, Newborn Language: Spanish Journal: Rev. méd. Chile Journal subject: Medicine Year: 2003 Type: Article Affiliation country: Chile Institution/Affiliation country: Universidad de Chile/CL