Metabolism Test: Trivia Questions! Quiz

Methionine is not a DNA nucleotide because it is an amino acid, not a nucleotide. Nucleotides are the building blocks of DNA and consist of a sugar molecule, a phosphate group, and a nitrogenous base. Thymine, cytosine, and adenosine are all nitrogenous bases that are found in DNA nucleotides. Methionine, on the other hand, is an amino acid that is used in protein synthesis.

Without diet therapy, levels of blood and brain phenylalanine are actually increased in PKU, not decreased. PKU is a genetic disorder that causes a deficiency in the enzyme needed to break down phenylalanine, an amino acid found in protein-containing foods. Without proper diet therapy, phenylalanine builds up in the blood and can lead to intellectual disability and other neurological problems. Therefore, the correct answer is False.

In autosomal recessive disorders, both parents must carry a copy of the "bad" gene for their child to be affected. Each parent has a 50% chance of passing on the "bad" gene, so the probability of both parents passing it on is 50% multiplied by 50%, which equals 25%. Therefore, there is a 25% chance that a child will be affected with the disorder when both parents carry the "bad" gene.

PKU (Phenylketonuria) is a genetic disorder that affects the body's ability to process phenylalanine, an amino acid found in protein-rich foods. Following a PKU diet is crucial for managing the condition. This diet involves avoiding foods that are high in phenylalanine, such as meat, fish, eggs, dairy products, and certain grains. Since PKU is a lifelong condition, it is recommended for patients to follow the PKU diet for life to prevent the buildup of phenylalanine in the body, which can lead to intellectual disabilities and other health problems.

Autosomal recessive inheritance is the correct answer because most metabolic diseases are caused by mutations in genes that are located on autosomal chromosomes, which are non-sex chromosomes. In autosomal recessive inheritance, an individual must inherit two copies of the mutated gene, one from each parent, in order to develop the disease. This means that both parents must be carriers of the mutated gene.

During an acute episode associated with illness or prolonged fasting in long-chain fatty acid oxidation disorders, excessive ketone production would not be seen. This is because these disorders impair the body's ability to break down long-chain fatty acids for energy, leading to a decreased production of ketones. The other symptoms mentioned, such as hypoglycemia, vomiting, and lethargy, can be seen in these disorders due to the inability to properly utilize fatty acids for energy.

The given answer, "All of the above is possible," suggests that patients can calculate their phenylalanine intake using any of the three options mentioned in the question: exchanges of 15 mg phenylalanine, every mg of phenylalanine, or grams of protein. This means that patients have the flexibility to choose the method that works best for them in monitoring their phenylalanine intake.

Patients with amino acid disorders other than PKU present acutely ill. This means that individuals with these disorders experience sudden and severe symptoms or illness. Unlike PKU, which may have more gradual or mild symptoms, other amino acid disorders can cause more immediate and severe health issues. Therefore, the statement is true.

Chromatinization is not a mechanism for genetic transmission because it refers to the process of condensing and packaging DNA into a compact structure called chromatin, which is essential for the regulation of gene expression. However, it does not involve the actual transmission of genetic information from one generation to the next. In contrast, autosomal dominant, autosomal recessive, and X-linked are all mechanisms of genetic transmission that describe how specific traits or disorders are inherited from parents to offspring.

PKU (Phenylketonuria) is a genetic disorder that affects the body's ability to process an amino acid called phenylalanine (PHE). Individuals with PKU need to limit their intake of PHE to prevent its buildup in the body, as high levels can cause brain damage. The given statement is true because, on average, PKU patients can tolerate around 300 mg of PHE or 6 grams of protein per day. This restricted diet helps maintain PHE levels within a safe range and prevents complications associated with PKU.

In tyrosinemia, the offending amino acids are phenylalanine and tyrosine. This means that these two amino acids are present in higher than normal levels in the body, leading to the symptoms and complications associated with tyrosinemia. Phenylalanine is an essential amino acid that is normally converted into tyrosine, but in tyrosinemia, this conversion is impaired, causing an accumulation of both phenylalanine and tyrosine. This can result in liver and kidney damage, as well as neurological problems.

Monogen is not indicated for MCADD (Medium-Chain Acyl-CoA Dehydrogenase Deficiency) diets. MCADD is a condition where the body is unable to break down certain fats for energy. The main treatment for MCADD involves a carefully controlled diet that is low in fats and high in carbohydrates. Monogen, on the other hand, is a specialized formula designed for individuals with fat malabsorption or other conditions that require a high-calorie, low-fat diet. Therefore, it is not suitable for MCADD diets.

The principle of reducing the amount of "product" that is blocked in the pathway is not a principle in the management of patients with inborn errors of metabolism. In these cases, the focus is on reducing the amount of substrate (through diet and catabolism) and catabolism that can add to the substrate. Additionally, supplementation with coenzymes may be necessary in some cases. However, the concept of reducing the amount of "product" that is blocked in the pathway is not mentioned as a principle in the management of these patients.

Walnut oil provides the best N6-N3 ratio among the given options. This means that it contains a higher proportion of omega-3 fatty acids (N3) compared to omega-6 fatty acids (N6). Omega-3 fatty acids are considered beneficial for health as they have anti-inflammatory properties and are important for brain function. On the other hand, excessive consumption of omega-6 fatty acids can lead to inflammation and other health issues. Therefore, walnut oil is a better source for maintaining a balanced ratio of these essential fatty acids.

Plasma reflects the current intake of EFAs because it is the liquid component of blood that carries various substances, including nutrients, throughout the body. Since plasma is in direct contact with the cells and tissues, it provides a more accurate representation of the recent intake of essential fatty acids (EFAs) compared to whole blood, which includes both plasma and cellular components. Therefore, monitoring EFAs through plasma would give a better indication of the current levels of intake.

The correct answer is 50%. This means that on a non-PKU diet, the average amount of protein coming from medical food is 50%. This suggests that half of the protein intake is derived from medical food, indicating a significant contribution to the overall protein consumption.

Periflex Junior contains 25 grams of PE per 100 grams of powder, while Periflex Advance contains 35 grams of PE per 100 grams of powder.

The new Periflex Junior contains 25g of protein per 100g.

Periflex Infant and Phenex 1 have the same calorie to protein ratio of 32:1. This means that for every 32 calories in both formulas, there is 1 gram of protein. Therefore, the statement is true.

The "Lana-" in the brand name "Lanaflex" most likely comes from the term "Large Neutral Amino Acids." This is supported by the fact that the other options do not include the term "Lana-" and are not directly related to amino acids. Therefore, "Large Neutral Amino Acids" is the most logical explanation for the origin of "Lana-" in the brand name "Lanaflex."

The correct answer is XPhe Maxamum Drink. This is a phenylalanine-free, ready-to-drink medical food.

Periflex Advance does not provide fewer calories from medical food compared to XPhe Maximum, which means it does not allow for greater flexibility in other food choices. Therefore, the correct answer is False.

Periflex Infant contains 0.2g of DHA and 0.35g of ARA per 100g of the product.

Abbott-Ross is the correct answer because they are the manufacturer of the PKU formula Phenex-1. The other companies listed, Cambrooke, Mead Johnson, and Vitaflo, do not manufacture this specific formula.

The study suggests that using a combination of flax oil and walnut oil as the essential fatty acid (EFA) source leads to fewer abnormal acylcarnitines being produced in fibroblasts. This implies that these two oils have a positive effect on the production of acylcarnitines, potentially indicating a beneficial impact on overall cellular health.

The given statement states that Applied Nutrition has 41% off Metabolic Market Share and is the current PKU Infant Formula Market Leader. However, there is no direct correlation between having a 41% off market share and being the market leader. Therefore, the statement is false.

The correct answer is 13 g PE. This means that there are 13 grams of protein per 100 grams in the new Periflex Infant with DHA & ARA.

Periflex Junior is designed to meet the nutritional needs of children between the ages of 1 and 8 years. This age range is crucial for growth and development, and it is important to provide them with adequate nutrition during this period. Periflex Junior is specifically formulated to provide the necessary nutrients for children within this age group, ensuring their optimal health and well-being.

Carnitine is not always necessary in any of the mentioned disorders (LCHADD, VLCADD, MCADD, SCADD). While these disorders may involve defects in the metabolism of fatty acids, the need for Carnitine supplementation varies depending on the specific case and the severity of the disorder. Therefore, none of the mentioned disorders require Carnitine supplementation as a general rule.

Phlexy 10 is the correct answer because it is a Nutricia metabolic product that does not contain vitamins and minerals. The other options, Periflex, Maxamum, Maxamaid, and Milupa, all contain vitamins and minerals.

The statement is false because EFA deficiency actually increases the production of oleic acid. Oleic acid is a monounsaturated fatty acid that can be produced by the body in the absence of essential fatty acids (EFAs). When there is a deficiency in EFAs, the body compensates by increasing the production of oleic acid. Therefore, oleic acid production is not reduced, but rather increased, with EFA deficiency.

An LCHADD patient's diet should not include a reduction in MCT (Medium-chain triglycerides). Medium-chain triglycerides are easily absorbed and utilized by the body for energy, making them an important component of the diet for LCHADD patients. LCHADD (Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency) is a metabolic disorder that affects the body's ability to break down long-chain fatty acids. Therefore, reducing total fat and LCT (Long-chain triglycerides) is necessary for these patients, but MCT should not be reduced.

LCHADD (Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency) is a disorder that can lead to retinitis pigmentosa as a long-term complication. This condition affects the breakdown of fats for energy, leading to a buildup of harmful substances in the body. Over time, this buildup can cause damage to the retina, resulting in retinitis pigmentosa, which is characterized by progressive vision loss and impairment.

PhenylAde is a product that is high in saturated fat. The given answer of 98% suggests that almost all of the total saturated fat comes from PhenylAde. This means that the majority of the saturated fat consumed is from PhenylAde, making it a significant contributor to the total saturated fat intake.

The correct answer is Loprofin low protein foods. This is because the question asks about an extension to a current product line in the US, and Loprofin low protein foods is a product line that already exists. It is reasonable to assume that the extension mentioned in the question would be related to this existing product line.

During illness, it is not recommended to give a 2-year-old child with LCHADD 2 to 3 oz of Pedialyte every 3 hours. This could potentially overload their system and worsen their condition. If the child is vomiting and doesn't seem to stop, it is important to seek medical attention rather than waiting until the next day. Additionally, if the child is sleepy, it is crucial to monitor their condition and seek medical advice if necessary, rather than assuming they just need rest. Therefore, options b and c are not recommended during illness for a child with LCHADD.