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A Person Diagnosed With Phenylketonuria (pku) Should Avoid Products Containing

Artificial Sweeteners And Diabetes

Artificial Sweeteners And Diabetes

Is it possible to eat sweets when you have diabetes? The answer is "yes." But when you’re trying to satisfy your sweet tooth, it can be hard to know what to reach for at the grocery store (sugar-free this or low-calorie that). So, use this primer to help you choose wisely. The Sweet Facts When you’re comparing sweeteners, keep these things in mind: Sugars are naturally occurring carbohydrates. These include brown sugar, cane sugar, confectioners’ sugar, fructose, honey, and molasses. They have calories and raise your blood glucose levels (the level of sugar in your blood). Reduced-calorie sweeteners are sugar alcohols. You might know these by names like isomalt, maltitol, mannitol, sorbitol, and xylitol. You'll often find them in sugar-free candy and gum. They have about half the calories of sugars and can raise your blood sugar levels, although not as much as other carbohydrates. Artificial sweeteners are considered "free foods." They were designed in a lab, have no calories, and do not raise your blood sugar levels. Types of Artificial Sweeteners Artificial low-calorie sweeteners include: Saccharin (Sweet'N Low, Sugar Twin). You can use it in both hot and cold foods. Avoid this sweetener if you are pregnant or breastfeeding. Aspartame (NutraSweet, Equal). You can use it in both cold and warm foods. It may lose some sweetness at high temperatures. People who have a condition called phenylketonuria should avoid this sweetener. Acesulfame potassium or ace-K (Sweet One, Swiss Sweet, Sunett). You can use it in both cold and hot foods, including in baking and cooking. Sucralose (Splenda). You can use it in hot and cold foods, including in baking and cooking. Processed foods often contain it. Advantame can be used in baked goods, soft drinks and other non-alcoholic bev Continue reading >>

Phenylketonuria

Phenylketonuria

Phenylketonuria (PKU) is a rare but potentially serious inherited disorder. Our bodies break down protein in foods like 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 aren't needed are broken down further and removed from the body. People with PKU can't break down the amino acid phenylalanine, which then builds up in the blood and brain. This can lead to brain damage. Diagnosing PKU At around 5 days old, babies are offered newborn blood spot screening to check if they have PKU or a number of 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, the majority of children with PKU are able to live healthy lives. About 1 in 10,000 babies born in the UK has PKU. Symptoms of PKU PKU doesn't usually cause any symptoms if treatment is started early. Without treatment, PKU can damage the brain and nervous system, which can lead to learning disabilities. Other symptoms of untreated PKU include: behavioural difficulties – such as frequent temper tantrums and episodes of self-harm fairer skin, hair and eyes than siblings without the condition (phenylalanine is involved in the body's production of melanin, the pigment responsible for skin and hair color) recurrent vomiting jerking movements in arms and legs musty smell on the breath, skin and urine Treating PKU Diet 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 oth Continue reading >>

Phenylketonuria (pku) - Aspartame

Phenylketonuria (pku) - Aspartame

Phenylketonuria (PKU) is a rare, inherited metabolic disease that results in mental retardation and other neurological problems when treatment is not started within the first few weeks of life. When a very strict diet is begun early and well-maintained, effected children can expect normal development and a normal life span. Elevations of blood phenylalanine are dangerous for those with PKU, and require treatment and modification. A child with a level less than 6 mg/dl does not need to be on a special diet and is not risk for mental retardation, but should avoid aspartame at all cost due to the 50% phenylalanine levels. Scientists have been able to show that carriers had higher phenylalanine levels in their urine than non-carriers. Phenylalanine Hydroxylase The disease arises from the absence of a single enzyme (phenylalanine hydroxylase). This enzyme normally converts the essential amino acid, phenylalanine, to another amino acid, tyrosine. Failure of the conversion to take place results in a buildup of phenylalanine. Through a mechanism that is not well understood, the excess phenylalanine is toxic to the central nervous system and causes the severe problems normally associated with PKU. Not every child has the same degree of enzyme deficiency, however; some have enough enzyme activity that the diet can be quite liberal, while others must have the very strict diet. The nature of the diet for an individual child must be determined by an experienced PKU treatment program. Phenylketonuria is carried through a "recessive" gene. The incidence of carriers in the general population is approximately one in fifty people. Although PKU affects only one out of every approximately 10,000 babies born in the US, there are several hundred babies diagnosed and put on a strict diet each Continue reading >>

Dealing With Phenylketonuria (pku)

Dealing With Phenylketonuria (pku)

PKU: An Amino Acid Disorder Amino acid disorders incidence excluding Phenylketonuria (PKU) in West Midland (1 in 5354) according to the study conducted in year (Sanderson et al, 2006). In British Columbia Amino acid disorder in Newborns is 24 in 100,000 births and 1/ 4,200 (Applegarth et al, January 2000) Prevalence The occurrence of PKU varies among ethnic groups and geographic regions worldwide. Local Prevalence In a study using newborn screening data from West Midlands region of England, PKU prevalence was not found to be significantly different among Pakistani and white children (∼ 0.7/10,000 vs ∼ 0.8/10,000), although the gene frequency was much lower in the Pakistani population (Hutchesson, 1998). Global Prevalence Babies in the United States are screened for PKU shortly after birth. The condition is uncommon in this country, only affecting about 1 in 10,000 to 15,000 newborns each year. PKU is more common in individuals with Irish, northern European, Turkish, or Native American ancestry. It is less common in people of African, Japanese, or Ashkenazi Jewish backgrounds. The incidence of PKU is approximately 1 in 12,000 Caucasians. It is less common in the African-American population, with an incidence of approximately 1 in 50,000 based on newborn screening data from the state of Maryland. PKU is rare in Finland and Japan (National Institutes of Health Consensus Development Panel). The overall birth prevalence of PKU in European, Chinese and Korean populations is approximately 1/10,000 (Pubmed: Hardelid et al. 2008) To www.rightdiagnosis.com, approximately 1 in 10,000 or 0.01% or 27,200 people in USA can counteract phenylketonuria. The estimate number per area ratio in Pakistan is 15,919 in 159, 196, 3362 as compared to that of 29,365 in 293, 655, 4051 of USA a Continue reading >>

Phenylketonuria

Phenylketonuria

Phenylketonuria (PKU) is inherited, which means it is passed down through families. Both parents must pass on a nonworking copy of the gene in order for a baby to have the condition. Babies with PKU are missing an enzyme called phenylalanine hydroxylase, which is needed to break down the essential amino acid phenylalanine. Phenylalanine is found in foods that contain protein. Without the enzyme, levels of phenylalanine build up in the body. This buildup can harm the central nervous system and cause brain damage. Continue reading >>

Phenylketonuria (pku)

Phenylketonuria (pku)

What Is It? Phenylketonuria (PKU) is a rare genetic (inherited) disorder that can cause abnormal mental and physical development if not detected promptly and treated appropriately. Normally, when a person eats foods that contain protein, special chemicals called enzymes break down these proteins into amino acids. The amino acids are then broken down further by other enzymes. A person with PKU does not have enough of the specific enzyme that breaks down the amino acid phenylalanine. Therefore, any phenylalanine in the food someone eats cannot be digested properly and collects in the body. Too much phenylalanine in the body causes problems with the brain and other organs. Damage from a buildup of phenylalanine can begin within the first month of life and, if undetected and/or untreated, PKU results in severe mental retardation, hyperactivity, and seizures. PKU affects about 1 baby in every 10,000 or 15,000 births. To be born with PKU, a baby has to have inherited the PKU gene from both parents. Most often, the parents do not know that they carry the gene. Symptoms Newborn babies with PKU do not show any symptoms. At about 3 to 6 months, babies with untreated PKU will begin to be less active. They show little interest in everything around them. By 1 year, the child may have severe developmental delays. Children with PKU have lower levels of melanin, the substance that gives color to hair and skin. That's because when phenylalanine is broken down, one of its products is used to make melanin. As a result, children with PKU often will have pale skin, blond hair and blue eyes. Dry skin; eczema; and a "musty" odor resulting from the buildup of phenylalanine in hair, skin and urine are also common. Other signs and symptoms may include irritability, muscle stiffness, seizures, a Continue reading >>

Diet Intervention Guidelines For Adults With Untreated Pku

Diet Intervention Guidelines For Adults With Untreated Pku

by Barbara E. Dolan, RN, MSN Nurse Counselor for Genetics, Redwood Coast Regional Center, Ukiah, CA Richard Koch, MD Division of Medical Genetics, Children’s Hospital of Los Angeles and The Department of Pediatrics, University of Southern California, School of Medicine, Los Angeles, CA Christina Bekins, MS, RD Nutrition Consultant, Calistoga, CA Virginia Schuett, MS, RD Nutritionist and Director, National PKU News, Seattle, WA Acknowledgments The authors applaud the “outreach clinic model” staffed by specialists including Richard Goldwasser, MD, psychiatrist. We wish to thank Kathleen Schmidt Yule, RN, MS, UCS, NBS Program; Kathleen Dall, RD, Scientific Hospital Supplies, Inc.; The Inherited Metabolic Disease Clinic, Denver, CO; and Steven Yannicelli, MMSc, RD and Phyllis Acosta, PhD, RD, Ross Laboratories for their interest and guidance in the beginning months of this project. We are grateful for the interest, confidence and love of adult care providers and their families, and for their willingness to collaborate and share with us. Note: Before applying any of the information contained in these guidelines to treatment of PKU, you must consult with a PKU specialist. The guidelines do not give advice or recommendations for individuals, whose unique medical, nutritional, and other needs must be considered. Contents Introduction Overcoming Obstacles to Treatment: Obtaining Information, Guidance, and SupportStarting and Maintaining the Low PHE Diet for Adults Other Treatment Considerations Diet Resources Appendix Sample Menus (will be available at a later date on the PKU News web site) Introduction Purpose and Approach of the Guidelines The purpose of these guidelines is to present an organized way to start and maintain a phenylalanine (PHE)-restricted diet for a prev Continue reading >>

Pku (phenylketonuria) In Your Baby

Pku (phenylketonuria) In Your Baby

Phenylketonuria (also called PKU) is a condition in which your body can’t break down an amino acid called phenylalanine. Amino acids help build protein in your body. Without treatment, phenylalanine builds up in the blood and causes health problems. In the United States, about 1 in 10,000 to 15,000 babies is born with PKU each year. The illness happens in all ethnic groups. But it’s more common in people who are Native American and Northern European than those who are African-American, Ashkenazi Jewish or Japanese. PKU is inherited. This means it’s passed from parent to child through genes. A gene is a part of your body’s cells that stores instructions for the way your body grows and works. Genes come in pairs—you get one of each pair from each parent. Sometimes the instructions in genes change. This is called a gene change or a mutation. Parents can pass gene changes to their children. Sometimes a gene change can cause a gene to not work correctly. Sometimes it can cause birth defects or other health conditions. A birth defect is a health condition that is present in a baby at birth. Your baby has to inherit a gene change for PKU from both parents to have PKU. If she inherits the gene from just one parent, she has the gene change for PKU, but she doesn’t have PKU. When this happens, your baby is called a PKU carrier. A PKU carrier has the gene change but doesn’t have PKU. How do you know if your baby has PKU? All babies have a newborn screening test for PKU. Newborn screening checks for serious but rare conditions at birth. It includes blood, hearing and heart screening. With newborn screening, PKU can be found and treated early so babies can grow up healthy. Before your baby leaves the hospital, his health care provider takes a few drops of blood from hi Continue reading >>

Meal Plan / Pku Diet For Children With Phenylketonuria

Meal Plan / Pku Diet For Children With Phenylketonuria

Phenylketonuria (PKU) is a rare inherited condition in which there is a build up of the amino acid phenylalanine in the body. PKU is tested for at birth of newborns by measuring phenylalanine levels in the heel-prick blood test. All babies should have this test as it allows treatment to start early in life. High levels of phenylalanine are toxic to the brain, and will affect brain development, and the PKU sufferer will develop learning and behavioural difficulties. However, PKU is a treatable condition. Phenylalanine is found in the protein part of the food we eat and the treatment is a low protein diet. This means that high protein foods such as meat, cheese, poultry, eggs and milk are not permitted. Instead the diet is supplemented with specially formulated protein sources which contain no phenylalanine. This diet is very effective at lowering levels of phenylalanine and allows normal growth and development of the child. Basic principles of the diet Treatment consists of a diet containing only the amount of phenylalanine which is essential for growth and development. Meat, fish, cheese, eggs and nuts are rich in protein and therefore phenylalanine so they are not allowed. Other foods which contain some protein such as potato, milk and cereals are given in small measured quantities so that the blood phenylalanine, which is measured regularly, is kept within safe limits. These measured foods are spread out between the day's meals. The quantities allowed will vary from person to person. Most fruit, some vegetables and salads can be taken in normal quantities but avoid excess use. Sugar, jam, syrups, and fats such as butter, lard and cooking oil can be used freely. There are many manufactured foods which are low in protein and are available on prescription for the person Continue reading >>

What Is The Diet For Pku?

What Is The Diet For Pku?

What is Included in a Low Phenylalanine Food Pattern? The diet for PKU consists of a phenylalanine-free medical formula and carefully measured amounts of fruits, vegetables, bread, pasta, and cereals. Many people who follow a low phenylalanine (phe) food pattern eat special low protein breads and pastas. They are nearly free of phe, allow greater freedom in food choices, and provide energy and variety in the food pattern. What is Not Included in a Low Phenylalanine Food Pattern? Foods that contain large amounts of phe must be eliminated from a low phe diet. These foods are high protein foods, such as milk, dairy products, meat, fish, chicken, eggs, beans, and nuts. These foods cause high blood phe levels for people with PKU. Aim for healthy food choices This target is an easy way to visualize the foods allowed on the diet for PKU. The phenylalanine-free formula, such as Phenyl-Free*, is the center of the target diet. As the foods get further away from the bull's-eye, they are higher in phenylalanine. The foods outside the target are not included in the low-phenylalanine meal plan. How Can This Food Pattern be Enough? It is not unusual for someone who follows a low phe diet to have 2 kinds of vegetables and a baked potato for dinner. However, if these foods were the only foods a person consumed, his or her diet would be lacking protein, vitamins, and minerals. That is where the special formula comes in. A special phenylalanine-free formula, such as Phenyl-Free*, contains protein, vitamins, minerals and energy (calories) with no phenylalanine. With formula, a person with PKU gets plenty of protein, without the side effects of the high phe content of most foods. The phenylalanine-free formula is the most important part of the diet for PKU. How Long Must a Person With PKU F Continue reading >>

Phenylalanine Hydroxylase Deficiency: Diagnosis And Management Guideline

Phenylalanine Hydroxylase Deficiency: Diagnosis And Management Guideline

Phenylalanine hydroxylase deficiency, traditionally known as phenylketonuria, results in the accumulation of phenylalanine in the blood of affected individuals and was the first inborn error of metabolism to be identified through population screening. Early identification and treatment prevent the most dramatic clinical sequelae of the disorder, but new neurodevelopmental and psychological problems have emerged in individuals treated from birth. The additional unanticipated recognition of a toxic effect of elevated maternal phenylalanine on fetal development has added to a general call in the field for treatment for life. Two major conferences sponsored by the National Institutes of Health held >10 years apart reviewed the state of knowledge in the field of phenylalanine hydroxylase deficiency, but there are no generally accepted recommendations for therapy. The purpose of this guideline is to review the strength of the medical literature relative to the treatment of phenylalanine hydroxylase deficiency and to develop recommendations for diagnosis and therapy of this disorder. Evidence review from the original National Institutes of Health consensus conference and a recent update by the Agency for Healthcare Research and Quality was used to address key questions in the diagnosis and treatment of phenylalanine hydroxylase deficiency by a working group established by the American College of Medical Genetics and Genomics. The group met by phone and in person over the course of a year to review these reports, develop recommendations, and identify key gaps in our knowledge of this disorder. Above all, treatment of phenylalanine hydroxylase deficiency must be life long, with a goal of maintaining blood phenylalanine in the range of 120–360 µmol/l. Treatment has predominant Continue reading >>

Metabolic Disorders: Pku, Msud, And Nutrition

Metabolic Disorders: Pku, Msud, And Nutrition

What are metabolic disorders? Metabolic disorders are genetic diseases that interfere with the body's ability to process specific substances. Often, the body is missing an enzyme that is needed to process a certain type of amino acid. As a result, these acids can build up in the body causing health problems. Many metabolic disorders need special dietary therapy. Dietary therapy is a way of limiting the intake of the substance the body does not process well by avoiding certain foods and eating a special diet. People with metabolic disorders need ongoing counseling and monitoring by a team of physicians, nurses, genetic counselors, social workers, and dietitians for improved health and longevity. PKU and MSUD Two common diet-affected metabolic disorders are PKU or phenylketonuria, and Maple Syrup Urine Disease (MSUD). Both conditions involve deficiency in an enzyme that breaks down an amino acid into another compound. This means that the essential amino acid tends to build up in the body, causing problems. Phenylketonuria (PKU) PKU is a complete absence or deficiency in phenylalanine hydroxylase (PAH), an enzyme involved in metabolizing or converting the amino acid phenylalanine into tyrosine, another amino acid. PAH deficiency results in high levels of blood phenylalanine and an accumulation of phenylketones in the urine. Partial deficiency of the enzyme results in hyperphenylalaninemia. In this condition, the child has elevated blood phenylalanine, although it is not quite as high as when there is a complete absence of PAH. In hyperphenylalaninemia, phenylketones do not accumulate. Unless it is recognized and treated soon after birth, PKU will cause brain damage. In the United States, PKU is most common in white and Native Americans and less common in African American, Continue reading >>

Foods To Avoid On A Phenylketonuria Diet

Foods To Avoid On A Phenylketonuria Diet

Pheylketonuria (PKU) is a genetic disorder in which the body is not able to process an amino acid called phenylalanine, according to MedlinePlus.com. High levels of phenylalanine can damage the brain and lead to mental retardation. All babies born in the U.S. must have a screening test for PKU. Phenylalanine is mostly found in high-protein foods. High-protein foods should be avoided by patients with PKU. Video of the Day Fish, Poultry, Red Meat and Dairy Products Brain damage can be prevented if patients with PKU are treated with a special diet that is low in phenylalanine. In the PKU diet, animal protein is avoided because it contains the highest amounts of phenylalanine. Examples of animal protein to be avoided include red meat, fish, poultry and milk and milk products. PKU patients are to eat low-protein foods such as low-protein breads, pastas and cereals. Babies with PKU are often fed a special formula containing high protein and low phenylalanine. People with PKU who are on this special diet from birth or shortly thereafter develop normally and often have no symptoms of PKU, according to MedlinePlus.com. Dried Beans and Peas and Nuts Patients with PKU should avoid vegetable protein such as dried beans, peas and nuts as they contain high amounts of phenylalanine, according to MedlinePlus. Examples of nuts to avoid include almonds, hazelnuts, peanuts, chestnuts, pistachios, walnuts, cashewnuts, pecans and macadamias. PKU patients are usually advised to eat a lot of fruits and vegetables because they contain little phenylalanine. Patients with PKU should avoid products containing the artificial sweetener aspartame, according to MedlinePlus. When digested, aspartame produces amounts of phenylalanine considered dangerous to PKU patients. Examples of products that may c Continue reading >>

Aspartame

Aspartame

Sweeteners and all other food additives are tightly regulated and may only be used once their safety has been rigorously assessed. Aspartame was first approved in the UK in 1982 following the review of its safety by the UK's Committee on Toxicity, Consumer Products and the Environment (COT), a committee of independent experts that advises the Government on the safety of food chemicals. This was reaffirmed in 1988 by the European Commission's former Scientific Committee on Food (SCF). Following the publication of a number of anecdotal reports, which cast doubt on the safety of this sweetener, the SCF reviewed more than 500 papers published in the scientific literature between 1988 and 2001 on the safety of aspartame, including studies supporting the safety of aspartame and others pointing to potential adverse effects, which concluded, in 2002, that there was no evidence to suggest a need to revise the outcome of their earlier risk assessment or the Acceptable Daily Intake (ADI) previously established for aspartame of 40 milligrams per kilogram of body weight per day (40 mg/kg bw/day). A study published by the Ramazzini Foundation in Bologna, Italy, in July 2005 claimed to have shown that rats given dosages of aspartame equivalent to the Acceptable Daily Intake (ADI) may develop tumours. EFSA assessed the study and raised a number of concerns regarding it. They concluded, that ‘there is no need to further review the safety of aspartame nor to revise the previously established ADI'. The FSA supported the conclusions of EFSA's review but reiterated that all approvals of food additives should be kept under review as and when new scientific information becomes available. Indeed, as part of its systematic re-evaluation of all food additives, EFSA has re-evaluated the safety Continue reading >>

Diabetes: Sugar Substitutes & Nonnutritive Sweeteners

Diabetes: Sugar Substitutes & Nonnutritive Sweeteners

What are nonnutritive sweeteners? Nonnutritive sweeteners are substances that are used instead of sugars (i.e., sucrose, corn syrup, honey, agave nectar) to sweeten foods, beverages and other products, such as oral care products and certain medications. Nonnutritive sweeteners (also called sugar substitutes or artificial sweeteners) contain few or no calories or nutrients. Artificial sweeteners may be derived from plants or herbs, or even sugar itself. They have a greater intensity of sweetness compared with sugar, so smaller quantities are needed for flavoring foods and beverages. Some artificial sweeteners are not metabolized, meaning that they pass through the digestive tract essentially unchanged. The seven nonnutritive sweeteners that have been approved by the U.S. Food and Drug Administration (FDA) are aspartame, acesulfame potassium, luo han guo (monk) fruit extract, neotame, saccharin, stevia and sucralose. Aspartame (Equal® or NutraSweet®) is about 200 times sweeter than table sugar. Aspartame is used as a tabletop sweetener found in a wide variety of foods and beverages, including cereals, yogurt, frozen and gelatin desserts, candy, sugar-free gum, juices, diet sodas, and many other products. It is also used in drugs such as vitamin supplements and laxatives. Acesulfame potassium (Sunett® and Sweet One®) is generally used in combination with other non-nutritive sweeteners and is frequently found in sugar-free sodas. Neotame is also used in low-calorie foods and beverages, but to a lesser extent than other sweeteners. It is about 7,000 to 13,000 times sweeter than sugar. It is a derivative of the amino acids phenylalanine and aspartic acid. Saccharin (Sweet ‘n Low®, Sweet Twin®, and Sugar Twin®) is the oldest artificial sweetener on the market. It was Continue reading >>

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