Can phenylketonuria eat corn?

Written by Zeng Hai Jiang

Pediatrics

Updated on October 26, 2024

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People with phenylketonuria can eat corn. Phenylketonuria is a hereditary metabolic disease that can be treated through dietary control. Once diagnosed with phenylketonuria, one must stop a natural diet and switch to a low-phenylalanine diet. When the concentration of phenylalanine in the blood drops to an ideal level, natural foods can be gradually reintroduced in small amounts, but the diet must still adhere to low-protein and low-phenylalanine standards. Corn mainly consists of starch and does not contain phenylalanine, so individuals with phenylketonuria can eat corn.

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Written by Zeng Hai Jiang

Pediatrics

44sec

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Does phenylketonuria definitely cause intellectual disabilities when one grows up?

Phenylketonuria is a common amino acid metabolic disorder that is due to a deficiency of phenylalanine hydroxylase in the phenylalanine metabolic pathway, leading to metabolic disorder of phenylalanine in the liver. Phenylketonuria is the first genetic metabolic disorder that can be controlled and treated through diet. Once diagnosed, natural diet should be discontinued for the patient, and a low-phenylalanine diet should be administered. If phenylketonuria is diagnosed and treated early, intelligence can be normal. However, if not controlled, it can lead to delayed growth and development, especially in terms of intellectual development.

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Written by Zeng Hai Jiang

Pediatrics

1min 2sec

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Does phenylketonuria require lifelong dietary control?

Theoretically, phenylketonuria requires lifelong dietary control. Phenylketonuria is caused by a deficiency in phenylalanine hydroxylase in the metabolic pathway of phenylalanine, leading to disordered metabolism of phenylalanine in the liver. Phenylketonuria is the first genetic metabolic disease that can be managed through diet. Since natural foods all contain certain amounts of phenylalanine, once diagnosed, affected children should cease consuming natural diets and should be treated with a low-phenylalanine diet. Treatment with a low-phenylalanine formula should continue at least until the age of 12. When the concentration of phenylalanine in the blood is controlled at an ideal level, natural foods can gradually be reintroduced in small amounts. The reintroduction of food should still follow the principles of low protein and low phenylalanine. The types and amounts of food that each child can add vary individually and are related to the severity of the enzyme deficiency.

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Written by Tong Peng

Pediatrics

1min 5sec

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Does phenylketonuria have a distinctive facial appearance?

Phenylketonuria is characterized by a specific facial appearance, primarily presenting with pale skin and yellow hair. Some affected children may develop eczema due to a disruption in melanin synthesis, which results in a distinctive skin color. Additionally, there are neuropsychiatric abnormalities, such as intellectual disability, increased excitability, and heightened muscle tone. The urine of patients has a plastic-like smell, largely because phenylketonuria is a hereditary metabolic disease. Newborns exhibit hyperphenylalaninemia, but as harmful metabolites of phenylalanine are not present in high concentrations before feeding begins, initial conditions at birth are often normal. However, as diet is introduced and metabolite concentrations increase, specific clinical symptoms, including severe cases of epileptic seizures manifested as infantile spasms, nodding seizures, or other forms, become evident. Therefore, dietary management is essential in treatment.

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Written by Zeng Hai Jiang

Pediatrics

40sec

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Can phenylketonuria be detected before a newborn is born?

Phenylketonuria can be screened prenatally before the birth of a newborn. The procedure involves genetic screening using amniotic fluid drawn through amniocentesis between the 16th and 20th week of pregnancy. Phenylketonuria is an autosomal recessive hereditary disease, greatly associated with chromosomal abnormalities. If chromosomal abnormalities are detected through amniocentesis, indicating the presence of pathogenic genes, it confirms that the fetus will be born with phenylketonuria. If both parents carry the relevant genes, prenatal screening is still necessary to rule out the risk of the disease in the fetus.

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Written by Yan Xin Liang

Pediatrics

55sec

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Phenylketonuria Test Methods

The examination methods for phenylketonuria primarily include newborn screenings usually performed three to seven days after birth by drawing a blood sample from the heel and placing drops on specialized blood collection filter paper. Once dried, the sample is sent to a screening laboratory to determine the concentration of phenylalanine. If the phenylalanine concentration exceeds the cutoff value, further examinations and confirmatory tests are necessary, with the confirmatory test involving the measurement of phenylalanine concentration. Another method involves the analysis of urinary pterin profile, mainly used to differentiate dihydrobiopterin reductase deficiency. Additionally, gene analysis can be applied to detect mutations in genes such as phenylalanine hydroxylase, aiding in genetic diagnoses and prenatal diagnoses.