PKU meal plans are different for each baby and can vary over time depending on how much phenylalanine your baby can tolerate. Health care providers at a medical center or clinic that has a special program to treat PKU can help you create a meal plan for your baby.
Your child will follows the PKU meal plan through their whole life. If they eventually get pregnant, they follow their meal plan throughout pregnancy.
Most pregnant and parenting people who have PKU can have healthy pregnancies and healthy babies. The medicine is more likely to work in people with mild or special forms of PKU. They still need regular blood tests to check phenylalanine levels. See also: Genetic counseling , Newborn screening.
Get expert tips and resources from March of Dimes and CDC to increase your chance of having a healthy, fully-term pregnancy and baby.
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Mission stories Spotlights Impact Stories. PKU Phenylketonuria in your baby. For each of the eligible adult PKU patients, ten controls were drawn from the InGef research database via direct, exact matching, without replacement on age and sex.
Non-PKU controls no PKU diagnosis code in the enrollment period were required to be continuously enrolled in the database during the outcomes observation period, except for patients who died.
The 50 most common comorbidities among the overall adult PKU cohort, the early-diagnosed PKU cohort, and the late-diagnosed PKU cohort in were identified and rank-ordered using ICDGM codes and the prevalence of those comorbidities compared with the matched control group. The most commonly prescribed concomitant medications in were identified using 7-digit Anatomical Therapeutic Chemical ATC codes and pharmaceutical central numbers PZN and prescribing levels compared with the matched control group.
The CCI is a weighted index that takes into account the number and the seriousness of comorbid diseases by assigning points for certain illnesses. Phenylalanine hydroxylase deficiency: diagnosis and management guideline. Genet Med. Phenylalanine hydroxylase gene locus-specific database [Internet]. Accessed June 12, Fluctuations in phenylalanine concentrations in phenylketonuria: a review of possible relationships with outcomes.
Mol Genet Metab. The complete European guidelines on phenylketonuria: diagnosis and treatment. Orphanet J Rare Dis. Article Google Scholar. Rationale for the German recommendations for phenylalanine level control in phenylketonuria Eur J Pediatr. The response of patients with phenylketonuria and elevated serum phenylalanine to treatment with oral sapropterin dihydrochloride 6R-tetrahydrobiopterin : a phase II, multicentre, open-label, screening study.
J Inherit Metab Dis. Efficacy of sapropterin dihydrochloride in increasing phenylalanine tolerance in children with phenylketonuria: a phase III, randomized, double-blind, placebo-controlled study.
J Pediatr. Quality of life in noncompliant adults with phenylketonuria after resumption of the diet. Pegvaliase for the treatment of phenylketonuria: results of a long-term phase 3 clinical trial program PRISM. How practical are recommendations for dietary control in phenylketonuria? Vitamin and mineral status in patients with hyperphenylalaninemia.
The micronutrient status of patients with phenylketonuria on dietary treatment: an ongoing challenge. Ann Nutr Metab. The metabolic syndrome - a new worldwide definition.
Monatsschr Kinderheilkd. Google Scholar. Arterial stiffness assessment in patients with phenylketonuria. Fish-free diet in patients with phenylketonuria is not associated with early atherosclerotic changes and enhanced platelet activation. PLoS One. Lipid profile status and other related factors in patients with hyperphenylalaninaemia.
Plasma cholesterol in adults with phenylketonuria. Comparison of atherogenic risk factors among poorly controlled and well-controlled adolescent phenylketonuria patients.
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Cell Mol Neurobiol. Phenylalanine induces oxidative stress and decreases the viability of rat astrocytes: possible relevance for the pathophysiology of neurodegeneration in phenylketonuria.
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Am J Epidemiol. Download references. The analyses were performed in collaboration with Prof. Kohlscheen, J. Altevers, C. You can also search for this author in PubMed Google Scholar. All authors interpreted the data. KK and PL were responsible for drafting an initial outline of the manuscript for review by all authors prior to development of a first draft.
All authors provided critical review, revision of drafts, and approval of the the final manuscript. Correspondence to F. Back to Health A to Z. Our bodies break down the protein in foods, such as meat and fish, into amino acids, which are the "building blocks" of protein.
These amino acids are then used to make our own proteins. Any amino acids that are not needed are broken down further and removed from the body.
People with PKU cannot break down the amino acid phenylalanine, which then builds up in their blood and brain. This can lead to brain damage.
At around 5 days old, babies are offered newborn blood spot screening to test for PKU and many other conditions. This involves pricking your baby's heel to collect drops of blood to test.
If PKU is confirmed, treatment will be given straight away to reduce the risk of serious complications. Treatment includes a special diet and regular blood tests. With early diagnosis and the correct treatment, most children with PKU are able to live healthy lives. Without treatment, PKU can damage the brain and nervous system, which can lead to learning disabilities.
The main treatment for PKU is a low-protein diet that completely avoids high-protein foods such as meat, eggs and dairy products and controls the intake of many other foods, such as potatoes and cereals. In addition, people with PKU must take an amino acid supplement to ensure they're getting all the nutrients required for normal growth and good health.
There are also several low-protein versions of common foods such as flour, rice and pasta specifically designed for people with PKU and related conditions to incorporate into their diets. Many of these are available on prescription. If a high phenylalanine level is confirmed, a baby will immediately be started on a low-protein diet and amino acid supplements. Phenylalanine levels are regularly monitored by collecting blood from a finger prick on to a special card and sending it to a laboratory.
A dietitian will create a detailed dietary plan for your child that can be revised as your child grows and their needs change. As long as a person with PKU sticks to a low-protein diet throughout childhood, and their phenylalanine levels stay within certain limits, they'll remain well and their natural intelligence will be unaffected.
People with PKU must also avoid food products that contain aspartame, as it's converted into phenylalanine in the body.
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