Last update Nov. 23, 2019

Maternal Phenylketonuria

Compatible

Safe substance and/or breastfeeding is the best option.

An autosomal recessive congenital disease in which there is an enzymatic deficit that prevents the metabolization of the essential amino acid phenylalanine to the amino acid tyrosine, producing an accumulation of phenylalanine in the body that, untreated, damages the central nervous system causing mental deficiency.
The treatment consists of restricting from one’s diet foods that contain large amounts of phenylalanine, especially pulses, soya beans, meat, fish, eggs, cereals (except rice and corn), dairy products and the sweetener aspartame.

This restriction, which is fundamental in the initial stage of life, is recommended to be life-long.
During pregnancy, women with phenylketonuria should have stricter control of their phenylalaninemia levels through diet in order to prevent embryopathy syndrome due to phenylalanine in their babies (Manta 2018, MotherToBaby 2017 and 2015, Lawrence 2016 p.591, Murphy 2015, Purnell 2001, Campistol 1999, Matalon 1986, Bradburn 1985).

Mothers with phenylketonuria breastfed their healthy children without problems (Purnell 2001, Fox-Bacon 1997, Matalon 1986, Bradburn 1985).

Milk from mothers with phenylketonuria controlled by diet has normal levels of phenylalanine (Matalon 1986).

Breastfeeding mothers suffering from phenylketonuria can breastfeed their healthy babies without problems, who are not affected by phenylketonuria (MotherToBaby 2017 and 2015, Lawrence 2016 p.591, Lawrence 2013, Purnell 2001, Matalon 1986).

There is little or no relationship between plasma levels of phenylalanine and those of prolactin in patients who have the illness under control (Juhász 2016, Carlson 1992).


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Suggestions made at e-lactancia are done by APILAM team of health professionals, and are based on updated scientific publications. It is not intended to replace the relationship you have with your doctor but to compound it. The pharmaceutical industry contraindicates breastfeeding, mistakenly and without scientific reasons, in most of the drug data sheets.

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Other names

Maternal Phenylketonuria in other languages or writings:

Group

Maternal Phenylketonuria belongs to this group or family:

References

  1. Manta-Vogli PD, Schulpis KH, Dotsikas Y, Loukas YL. The significant role of amino acids during pregnancy: nutritional support. J Matern Fetal Neonatal Med. 2018 Jul 30:1-7. Abstract
  2. MotherToBaby. Maternal PKU. Fact Sheet 2017 Full text (link to original source) Full text (in our servers)
  3. Lawrence RA, Lawrence RM. Breastfeeding. A guide for the medical profession. Eighth Edition. Philadelphia: Elsevier; 2016
  4. Juhász E, Kiss E, Simonova E, Patócs A, Reismann P. Serum prolactin as a biomarker for the study of intracerebral dopamine effect in adult patients with phenylketonuria: a cross-sectional monocentric study. Eur J Med Res. 2016 May 11;21(1):22. Abstract
  5. Murphy E. Medical Problems in Obstetrics: Inherited Metabolic Disease. Best Pract Res Clin Obstet Gynaecol. 2015 Jul;29(5):707-20. Abstract
  6. MotherToBaby. Fenilcetonuria materna. Hoja informativa. 2015 Full text (link to original source) Full text (in our servers)
  7. Lawrence RM. Circumstances when breastfeeding is contraindicated. Pediatr Clin North Am. 2013 Feb;60(1):295-318. Abstract
  8. Purnell H. Phenylketonuria and maternal phenylketonuria. Breastfeed Rev. 2001 Jul;9(2):19-21. Review. Abstract
  9. Campistol J, Arellano M, Poo P, Escofet C, Pérez P, Vilaseca M.A. Embriopatía por fenilcetonuria materna. Una causa de retardo mental poco diagnosticada. Revisión de 8 observaciones. (Maternal Phenylketonuria. An under-diagnosed cause of Mental Retardation. A report of 8 cases). An Esp Pediatr 1999;51:139-142. 1999 Full text (link to original source) Full text (in our servers)
  10. Fox-Bacon C, McCamman S, Therou L, Moore W, Kipp DE. Maternal PKU and breastfeeding: case report of identical twin mothers. Clin Pediatr (Phila). 1997 Sep;36(9):539-42. No abstract available. Abstract
  11. Carlson HE, Hyman DB, Bauman C, Koch R. Prolactin responses to phenylalanine and tyrosine in phenylketonuria. Metabolism. 1992 May;41(5):518-21. Abstract
  12. Matalon R, Michals K, Gleason L. Maternal PKU: strategies for dietary treatment and monitoring compliance. Ann N Y Acad Sci. 1986;477:223-30. Abstract
  13. Bradburn NC, Wappner RS, Lemons JA, Meyer BA, Roberts RS. Lactation and phenylketonuria. Am J Perinatol. 1985 Apr;2(2):138-41. Abstract
  14. Valdivieso F, Maties M, Ugarte M, Mayor F. Increased free phenylalanine in the milk of a phenylketonuric mother. Biochem Med. 1973 Apr;7(2):341-2. No abstract available. Abstract

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