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Phenylketonuria (PKU) is an inborn error of metabolism that results in decreased metabolism of the amino acid phenylalanine. Untreated PKU can lead to intellectual disability, seizures, behavioral problems, and mental disorders. It may also result in a musty smell and lighter skin. A baby born to a mother who has poorly treated PKU may have heart problems, a small head, and low birth weight.
Phenylalanine is an essential α-amino acid with the formula C
9H
11NO
2. It can be viewed as a benzyl group substituted for the methyl group of alanine, or a phenyl group in place of a terminal hydrogen of alanine. This essential amino acid is classified as neutral, and nonpolar because of the inert and hydrophobic nature of the benzyl side chain. The L-isomer is used to biochemically form proteins coded for by DNA. Phenylalanine is a precursor for tyrosine, the monoamine neurotransmitters dopamine, norepinephrine (noradrenaline), and epinephrine (adrenaline), and the skin pigment melanin. It is encoded by the codons UUU and UUC.
Infant formula, baby formula, or simply formula ; or baby milk or infant milk, is a manufactured food designed and marketed for feeding to babies and infants under 12 months of age, usually prepared for bottle-feeding or cup-feeding from powder or liquid. The U.S. Federal Food, Drug, and Cosmetic Act (FFDCA) defines infant formula as "a food which purports to be or is represented for special dietary use solely as a food for infants by reason of its simulation of human milk or its suitability as a complete or partial substitute for human milk".
A baby bottle, nursing bottle, or feeding bottle is a bottle with a teat attached to it, which creates the ability to drink via suckling. It is typically used by infants and young children, or if someone cannot drink from a cup, for feeding oneself or being fed. It can also be used to feed non-human mammals.
Newborn screening (NBS) is a public health program of screening in infants shortly after birth for conditions that are treatable, but not clinically evident in the newborn period. The goal is to identify infants at risk for these conditions early enough to confirm the diagnosis and provide intervention that will alter the clinical course of the disease and prevent or ameliorate the clinical manifestations. NBS started with the discovery that the amino acid disorder phenylketonuria (PKU) could be treated by dietary adjustment, and that early intervention was required for the best outcome. Infants with PKU appear normal at birth, but are unable to metabolize the essential amino acid phenylalanine, resulting in irreversible intellectual disability. In the 1960s, Robert Guthrie developed a simple method using a bacterial inhibition assay that could detect high levels of phenylalanine in blood shortly after a baby was born. Guthrie also pioneered the collection of blood on filter paper which could be easily transported, recognizing the need for a simple system if the screening was going to be done on a large scale. Newborn screening around the world is still done using similar filter paper. NBS was first introduced as a public health program in the United States in the early 1960s, and has expanded to countries around the world.
Baby food is any soft easily consumed food other than breastmilk or infant formula that is made specifically for human babies between four and six months and two years old. The food comes in many varieties and flavors that are purchased ready-made from producers, or it may be table food eaten by the family that has been mashed or otherwise broken down.
Tetrahydrobiopterin (BH4, THB), also known as sapropterin (INN), is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), epinephrine (adrenaline), and is a cofactor for the production of nitric oxide (NO) by the nitric oxide synthases. Chemically, its structure is that of a (dihydropteridine reductase) reduced pteridine derivative (quinonoid dihydrobiopterin).
Pablum is a processed cereal for infants originally marketed and co-created by the Mead Johnson Company in 1931. The product was tested on aboriginal children in Canadian residential schools, and then further developed at The Hospital for Sick Children in Toronto, Ontario, to combat infant malnutrition.
Baby colic, also known as infantile colic, is defined as episodes of crying for more than three hours a day, for more than three days a week, for three weeks in an otherwise healthy child. Often crying occurs in the evening. It typically does not result in long-term problems. The crying can result in frustration of the parents, depression following delivery, excess visits to the doctor, and child abuse.
The BRAT diet is a restrictive diet that was once recommended for people, particularly children, with gastrointestinal distress like vomiting, diarrhea, or gastroenteritis. Evidence, however, does not support a benefit. It is no longer generally recommended as it is unnecessarily restrictive. The diet was first discussed in 1926.
Neurodevelopmental disorders are a group of disorders that affect the development of the nervous system, leading to abnormal brain function which may affect emotion, learning ability, self-control, and memory. The effects of neurodevelopmental disorders tend to last for a person's lifetime.
Proteins are essential nutrients for the human body. They are one of the building blocks of body tissue and can also serve as a fuel source. As a fuel, proteins provide as much energy density as carbohydrates: 4 kcal per gram; in contrast, lipids provide 9 kcal per gram. The most important aspect and defining characteristic of protein from a nutritional standpoint is its amino acid composition.
Baby-led weaning is an approach to adding complementary foods to a baby's diet of breast milk or formula. BLW facilitates oral motor development and strongly focuses on the family meal, while maintaining eating as a positive, interactive experience. Baby-led weaning allows babies to control their solid food consumption by "self-feeding" from the start of their experience with food. The term weaning does not imply giving up breast milk or formula, but simply indicates the introduction of foods other than breast milk or formula.
Breastfeeding, or nursing, is the process by which human breast milk is fed to a child. Breast milk may be from the breast, or may be expressed by hand or pumped and fed to the infant. The World Health Organization (WHO) recommends that breastfeeding begin within the first hour of a baby's life and continue as often and as much as the baby wants. Health organizations, including the WHO, recommend breastfeeding exclusively for six months. This means that no other foods or drinks, other than vitamin D, are typically given. WHO recommends exclusive breastfeeding for the first 6 months of life, followed by continued breastfeeding with appropriate complementary foods for up to 2 years and beyond. Of the 135 million babies born every year, only 42% are breastfed within the first hour of life, only 38% of mothers practice exclusive breastfeeding during the first six months, and 58% of mothers continue breastfeeding up to the age of two years and beyond.
Nutricia is a specialized medical nutrition company. It is part of the Medical Nutrition Division of Groupe Danone, Paris. Nutricia manufactures products used in the management of severe allergic and gastrointestinal disorders, metabolic conditions as well as other conditions requiring nutritional therapy, including intractable epilepsy.
Hyperphenylalaninemia is a medical condition characterized by mildly or strongly elevated concentrations of the amino acid phenylalanine in the blood. Phenylketonuria (PKU) can result in severe hyperphenylalaninemia. Phenylalanine concentrations ([phe]) are routinely screened in newborns by the neonatal heel prick, which takes a few drops of blood from the heel of the infant. Standard [phe] concentrations in unaffected persons are about 60µM: [phe] concentrations in persons with untreated phenylketonuria may be many times that, which indicate that the child is at risk for severe intellectual disability. Phenylketonuria is classed as an autosomal recessive condition: in heterozygous form, [phe] shows a moderate elevation, perhaps two-fold over that of unaffected homozygotes, which is classified as hyperphenylalaninemia.
Vegan nutrition refers to the nutritional and human health aspects of vegan diets. A well-planned, balanced vegan diet is suitable to meet all recommendations for nutrients in every stage of human life. Vegan diets tend to be higher in dietary fiber, magnesium, folic acid, vitamin C, vitamin E, iron, and phytochemicals; and lower in calories, saturated fat, cholesterol, long-chain omega-3 fatty acids, vitamin D, calcium, zinc, and vitamin B12.
The Graz tube weaning model is a method that supports parents, caregivers and professionals to help and empower medically fragile children with early and post-traumatic eating behavior disorders, particularly tube dependency.
Infant feeding is the practice of feeding infants. Breast milk provides the best nutrition when compared to infant formula. Infants are usually introduced to solid foods at around four to six months of age.
Fish protein powder (FPP) describes a food grade powder product designated primarily for human consumption applications. It differs significantly from fish meal products which are designated for animal feed applications. Fish protein powders have various sanitary processing, purity and functional characteristics which establish them as human food ingredients. Production plants registered for the USA market are located in Peru and France.
References
- 1 2 "Infant formula, MEAD JOHNSON, LOFENALAC, with iron, prepared from powder". Calorie Counter. Retrieved 16 April 2011.
- ↑ "LOFENALAC®". RxMed: Pharmaceutical Information. Retrieved 16 April 2011.
- ↑ Gregory, Arnall Golden (3 July 2013). "What kind of food am I? Medical food history, enforcement and future". Lexology. Retrieved 19 November 2016.
- ↑ "Medical foods for the nutritional support of infant/toddler metabolic diseases". SureChem. 1996-08-27. Retrieved 16 April 2011.
Patent Number: 5550146
- ↑ Marlow, Dorothy R. (1969). Textbook of pediatric nursing (3rd ed.). Philadelphia: W. B. Saunders Co. p. 345. ISBN 0-7216-6097-5.
- ↑ Roth, Ruth A.; Townsend, Carolynn E. (2003). Nutrition and diet therapy. Cengage Learning. p. 236. ISBN 978-0-7668-3567-2 . Retrieved 19 April 2011.
- ↑ Stanfield, Peggy; Hui, Y. H. (2009-05-06). Nutrition and Diet Therapy: Self-Instructional Approaches. Jones & Bartlett Learning. pp. 396, 398. ISBN 978-0-7637-6137-0 . Retrieved 19 April 2011.
- ↑ Brubacher, Joyce (April 17, 1995). "Formula companies respond". MSUD Family Support Group. Archived from the original on 3 March 2016. Retrieved 16 April 2011.
- ↑ Pillitteri, Adele; Nettina, Sandra M. (2003). Maternal & Child Health Nursing (4th ed.). Hagerstwon, MD: Lippincott Williams & Wilkins. p. 1483. ISBN 0-7817-3628-5.