Breast feeding in IMD

MacDonald, Anita; Depondt, Eliane; Evans, Sharon; Daly, Anne; Hendriksz, Christian J.; Saudubray, J. M.

doi:10.1007/s10545-006-0332-x

Cited by 31 publications

(39 citation statements)

References 7 publications

(2 reference statements)

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“…[6][7][8][9][13][14][15] Even in the presence of certain risk factors for definitive weaning, which are inevitable in this situation, we consider that the breastfeeding duration of the children with PKU was very good and that it was comparable to the mean durations described in the literature, both for children with PKU and for Brazilian children without PKU. 13,16,17 Demirkol et al, 13 McCabe et al 10 and Motzfeldt et al 6 found mean breastfeeding duration in phenylketonuric patients of 6.1, 8.9 and 7.0 months, respectively.…”

Section: Discussionmentioning

confidence: 99%

O aleitamento materno no tratamento de crianças com fenilcetonúria

Kanufre¹,

Starling²,

Leão³

et al. 2007

J. Pediatr. (Rio J.)

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Objective: To evaluate the effect of breastmilk as a source of phenylalanine (phe) on levels of this amino acid and on growth in phenylketonuric infants. Methods:The study recruited 35 breastfed phenylketonuric infants and compared their results with those of 35 infants fed on commercial, milk-based formula. The groups were paired for sex and age at weaning from breastfeeding.Data were analyzed up until cessation of breastmilk or for 12 months' follow-up. The breastfed group were given a "special formula" free of phe, by bottle every 3 hours, and breastmilk at will during the intervals. Levels of phe in the blood, collected weekly up to 6 months and fortnightly up to 1 year de age, were analyzed while breastfeeding continued.The two groups were compared in terms of the time taken for the levels of phe in blood to return to normal after treatment was started, using the Wilcoxon test. Anthropometric data were compared with Student's t paired test in the form of z scores. The phe assays were analyzed throughout breastfeeding. Results:The median time taken for phe levels to return to normal was 8 days for the breastfed group and 7 days for the control group. The phe assay results were normal in 87% of tests for the breastfed group and in 74.4% for the control group. The majority of children in both groups exhibited a z score > -2 on anthropometric examination. Conclusions:Continuation of breastfeeding, during the treatment, proved adequate for metabolic control and growth in children with phenylketonuria.J Pediatr (Rio J). 2007;83(5):447-452: Breastfeeding, phenylketonuria, phenylalanine.

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Section: Discussionmentioning

confidence: 99%

O aleitamento materno no tratamento de crianças com fenilcetonúria

Kanufre¹,

Starling²,

Leão³

et al. 2007

J. Pediatr. (Rio J.)

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“…An international survey reported five infants with CBS deficiency who had received breast-feeds in combination with a Met-free AA infant formula (MacDonald et al 2006a, b). The principles are the same as for breast-feeding in PKU (Francis and Smith 1981).…”

Section: Recommendationsmentioning

confidence: 99%

“…The WHO/FAO/UNU (2007) recommendations can be used to guide the total protein requirements (WHO et al 2007) but PKU research suggests that higher protein intakes than the WHO recommendations are required to compensate for the sub-optimal bio-availability of L-AAs in current preparations (MacDonald et al 2006a, b). It is recommended that the L-AA supplement is split into three to four doses throughout the day to maximise nitrogen retention and achieve appropriate growth (Acosta 2010).…”

Section: Recommendationsmentioning

confidence: 99%

Guidelines for the diagnosis and management of cystathionine beta‐synthase deficiency

Morris

Kožich

Santra

et al. 2016

J of Inher Metab Disea

262

360

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Cystathionine beta-synthase (CBS) deficiency is a rare inherited disorder in the methionine catabolic pathway, in which the impaired synthesis of cystathionine leads to accumulation of homocysteine. Patients can present to many different specialists and diagnosis is often delayed. Severely affected patients usually present in childhood with ectopia lentis, learning difficulties and skeletal abnormalities. These patients generally require treatment with a low-methionine diet and/or betaine. In contrast, mildly affected patients are likely to present as adults with thromboembolism and to respond to treatment with pyridoxine. In this article, we present recommendations for the diagnosis and management of CBS deficiency, based on a systematic review of the literature. Unfortunately, the quality of the evidence is poor, as it often is for rare diseases. We strongly recommend measuring the plasma total homocysteine concentrations in any patient whose clinical features suggest the diagnosis. Our recommendations may help to standardise testing for pyridoxine responsiveness. Current evidence suggests that patients are unlikely to develop complications if the plasma total homocysteine concentration is maintained below 120 μmol/L. Nevertheless, we recommend keeping the concentration below 100 μmol/L because levels fluctuate and the complications associated with high levels are so serious.

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“…. Entretanto, para que a utilização do LM tenha êxito no tratamento da PKU, é necessário que os pacientes tenham consultas freqüentes no serviço de referência, possibilitando ajustes dietéticos constantes e controle metabólico adequado, evitando níveis indesejáveis de phe no sangue [6][7][8][9][13][14][15] . Mesmo na presença de alguns fatores de risco, inevitá-veis nesta situação, para o desmame definitivo, consideramos que a duração da amamentação das crianças com PKU foi muito boa e semelhante aos tempos médios descritos na literatura, tanto para crianças com PKU quanto para crianças brasileiras sem PKU 13,16,17 .…”

Section: Métodosunclassified

Breastfeeding in the treatment of children with phenylketonuria

Kanufre

Starling²,

Leão³

et al. 2007

J Pediatr (Rio J)

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ResumoObjetivo: Avaliar o efeito do leite materno como fonte de fenilalanina (phe) nos níveis sangüíneos desse aminoácido e no crescimento de fenilcetonúricos. Métodos:Foram estudados 35 fenilcetonúricos que mantiveram leite materno, e os resultados foram comparados com os de 35 lactentes que usaram fórmula láctea comercial. Os grupos foram pareados por sexo e por idade à suspensão do aleitamento materno. Os dados foram analisados até a suspensão do leite materno ou durante 12 meses de acompanhamento. O grupo amamentado recebeu "fórmula especial" isenta em phe, em mamadeira a cada 3 horas, e leite materno em livre demanda nos intervalos. Os níveis sangüíneos de phe, coletados semanalmente até 6 meses e quinzenalmente até 1 ano de idade, foram analisados durante a amamentação. Foram comparados o tempo necessário para adequação dos níveis sangüíneos de phe, após o início do tratamento, utilizando o teste de Wilcoxon e os dados antropométricos, pelo teste t de Student pareado, utilizando o escore z. As dosagens de phe foram analisadas durante a amamentação.Resultados: O tempo mediano para adequação dos níveis de phe no sangue foi de 8 dias para o grupo amamentado e de 7 dias para o grupo controle. As dosagens de phe estavam adequadas em 87% das vezes para o grupo amamentado e em 74,4% para o grupo controle. Na avaliação antropométrica, a maioria das crianças, de ambos os grupos, apresentou escore z > -2. Conclusão:A manutenção do aleitamento materno, durante o tratamento, mostrou-se adequada no controle metabólico e no crescimento das crianças fenilcetonúricas. J Pediatr (Rio J). 2007;83(5):447-452:Aleitamento materno, fenilcetonúria, fenilalanina. AbstractObjective: To evaluate the effect of breastmilk as a source of phenylalanine (phe) on levels of this amino acid and on growth in phenylketonuric infants. Methods:The study recruited 35 breastfed phenylketonuric infants and compared their results with those of 35 infants fed on commercial, milk-based formula. The groups were paired for sex and age at weaning from breastfeeding. Data were analyzed up until cessation of breastmilk or for 12 months' follow-up. The breastfed group were given a "special formula" free of phe, by bottle every 3 hours, and breastmilk at will during the intervals. Levels of phe in the blood, collected weekly up to 6 months and fortnightly up to 1 year de age, were analyzed while breastfeeding continued. The two groups were compared in terms of the time taken for the levels of phe in blood to return to normal after treatment was started, using the Wilcoxon test. Anthropometric data were compared with Student's t paired test in the form of z scores. The phe assays were analyzed throughout breastfeeding. Results:The median time taken for phe levels to return to normal was 8 days for the breastfed group and 7 days for the control group. The phe assay results were normal in 87% of tests for the breastfed group and in 74.4% for the control group. The majority of children in both groups exhibited a z score > -2 on anthropometric examination. Conc...

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Breast feeding in IMD

Cited by 31 publications

References 7 publications

O aleitamento materno no tratamento de crianças com fenilcetonúria

O aleitamento materno no tratamento de crianças com fenilcetonúria

Guidelines for the diagnosis and management of cystathionine beta‐synthase deficiency

Breastfeeding in the treatment of children with phenylketonuria

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