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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Clinical symptoms of phenylketonuria

The clinical symptoms of phenylketonuria generally include normal appearance at birth, with symptoms usually appearing between three to six months, and becoming more pronounced by the age of one. The most noticeable initial symptoms involve the nervous system; delayed intellectual development is prominent, with intelligence often below normal, along with behavioral abnormalities such as hyperactivity, depression, restlessness, and withdrawal. There could be minor epileptic seizures, and in some cases, increased muscle tone or exaggerated tendon reflexes. Regarding the skin, several months after birth, due to insufficient melanin synthesis, the child’s hair changes from black to yellow, the skin appears pale, and eczema is also relatively common. Additionally, the urine and sweat contain higher levels of phenylacetic acid, which can result in a distinctive mouse-like urine odor.

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Pediatrics

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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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Pediatrics

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Phenylketonuria is a disease.

Phenylketonuria is an autosomal recessive genetic disorder resulting from mutations in the phenylalanine hydroxylase gene, leading to reduced enzyme activity and the accumulation of phenylalanine and its metabolic products in the body, thereby causing this disease. Phenylketonuria is the most common congenital amino acid metabolic disorder, clinically characterized by intellectual developmental delays, light skin and hair pigmentation, and a mouse-like urine odor. The incidence of the disease varies by race and region, with an incidence rate in China of about 1 in 11,000. Phenylalanine is an essential amino acid for the human body, but due to reduced activity of phenylalanine hydroxylase, phenylalanine cannot be converted into tyrosine, leading to extremely high concentrations of phenylalanine in the blood, cerebrospinal fluid, and tissues. Abnormal metabolism produces a large amount of phenylpyruvic acid, phenylacetic acid, phenyllactic acid, and p-hydroxyphenylacetic acid, along with high concentrations of phenylalanine and its metabolic products, causing brain damage and corresponding symptoms.

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Pediatrics

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Can phenylketonuria be treated with surgery?

Phenylketonuria (PKU) in children cannot be treated surgically. PKU is an autosomal recessive genetic disorder caused by a deficiency in the enzyme needed for the metabolic pathway of phenylalanine. Typically, there are no obvious symptoms in newborns, although some children may experience non-specific symptoms such as vomiting and feeding difficulties. If no relevant treatment is pursued, the child may gradually show signs of developmental delays in movement and intellect, and the hair might change from black to yellow while the skin tends to be paler. As the child grows older, the intellectual delays become more pronounced, and in severe cases, they might develop cerebral palsy. This disease can be treated in its early stages through dietary methods, and the younger the age at which treatment begins, the better the outcomes.

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Does phenylketonuria easily cause a cold?

Phenylketonuria does not easily cause a cold, as a cold is a type of infectious disease mainly caused by various pathogen infections and stimuli, leading to symptoms such as cough, fever, and runny nose. Phenylketonuria is a common amino acid metabolism disorder caused by a deficiency of the enzyme required for phenylalanine to convert into tyrosine, resulting in excessive excretion of phenylalanine in urine. This disease is a recessive hereditary disorder, which can lead to intellectual disability, neuropsychiatric symptoms, and pigment loss. Due to the lack of melanin, affected children often present with yellow hair, pale skin and sclera, and their urine has a mousy odor. They may also suffer from eczema, vomiting, and diarrhea. Phenylketonuria is one of the few treatable hereditary metabolic diseases, so it generally does not cause symptoms of a cold.