Diet Therapy Diseases of Infancy and Childhood PDF 2024/2025

10/15/2024 Al Balqa Applied University Faculty of Agriculture and Technology Nutrition and Food Processing Diet Therapy Diseases of Infancy and Childhood (30402352)...

10/15/2024 Al Balqa Applied University Faculty of Agriculture and Technology Nutrition and Food Processing Diet Therapy Diseases of Infancy and Childhood (30402352) Dr. Reem M. “Al Haj Ahmad” 2024/2025 1. The Infantile Colic: Infantile colic is a condition occurring in the second or third week of life. The key features of infantile colic are:  Periods of inconsolable crying, irritability, and screaming that last for 3 hours or more WITHOUT an apparent cause. This condition leads to 10% - 20% of pediatric provider visits during the first few weeks of their lives. 1  10/15/2024 1. The Infantile Colic: Infants with colic often show symptoms in the evening, displaying red-faced appearances, and abdominal tension. Traditional soothing methods typically prove ineffective during this phase. Healthcare providers establish a clinical diagnosis of infantile colic through a history and physical examination after eliminating potential pathological causes. 1. The Infantile Colic: The etiology of colic is still unknown, however it could be attributed to: 1. Incorrect feeding techniques; overfeeding, underfeeding, insufficient burping, and change of baby formula. 2. Maternal diets that include cow's milk may contribute to infant distress in breastfed infants with colic. 3. Gases result from milk fermentation. 2  10/15/2024 1. The Infantile Colic: Treatment: NO universal guidelines for treatment are available. Healthcare professionals recommend a trial of feeding a bottle-fed baby vertically, burping the baby frequently, and using a bottle with a collapsible bag to diminish the amount of swallowed air. 1. The Infantile Colic: Examples of soothing techniques: taking a ride in the car or stroller, using a pacifier, gently rocking, using a swing or front carrier, doing a gentle abdominal massage, playing soft white noise in the background, reducing stimulation, having a warm bath, and swaddling. 3  10/15/2024 2. Vomiting (Simple Functional Vomiting): Spitting up or regurgitation, usually happens in the first few months. Etiology: Gastric distension due to over feeding. Giving a hot formula. If a child is not burped.  Air swallowing during feeding or crying, this is because the child is less possible to expel air because they are in a horizontal (flat) position, this is especially in bottle-fed babies. 2. Vomiting (Simple Functional Vomiting): Treatment Giving the child the formula without exaggeration in over feeding. Give warm food. Burping the child 4  10/15/2024 3. Constipation: Usually occurs at the end of the first year of age. Etiology: Psychological reason. The type of the diet (e.g. Fiber-free diet, solid additives may cause constipation). Less active child. 3. Constipation: Treatment: Give sufficient amount of fluids with enough fiber. Give high amount of fruit and vegetables. We do not encourage the use of laxatives unless prescribed by the physician. 5  10/15/2024 4. Infantile Diarrhea: The stool become fluid resulting in: 1. Dehydration 2. Loss of body fluids and electrolytes 3. Depletion of mineral stores 4. Infantile Diarrhea: Etiology: Bacterial infection. This needs treatment, it might be dangerous when K ions and electrolytes are depleted or when acidosis occurs. Nutritional cause (e.g. when child is given too much fiber). 6  10/15/2024 4. Infantile Diarrhea: Treatment Using antibiotic if the causative agent is bacterial. Use formula rich in electrolyte to replace the electrolyte loss.  In chronic diarrhea oral dehydration solutions might be given which contain NaCl, KCl, acetate, lactate, and bicarbonate. Use certain gels or pectins to solidify the feces. Low residue diet (low fiber diet). 5. Cleft lip and Palate: A birth defect that occurs when the lip and/or the roof of the mouth does not form or close properly, resulting in a cleft lip and/or cleft palate. The most commonly occurring birth defects. Causes are unknown, it maybe associated with genetic and environmental factors. 7  10/15/2024 5. Cleft lip and Palate: Cleft palate: when the root of the mouth contains an opening into the nose. Cleft lip: it contains an opening in the upper lip that may extend into the nose. The opening maybe in one side, both sides or in the middle. 5. Cleft lip and Palate: 8  10/15/2024 5. Cleft lip and Palate: Signs and Symptoms: Swallowing difficulty Abnormal swallow study showing aspiration and/or oral/pharyngeal dysphagia. Inadequate oral intake. Frequent respiratory infections/pneumonias. Coughing/choking with foods/liquids. Breastfeeding Inability to form proper latch. difficulty Inadequate oral intake. Underweight Inadequate energy intake. Increased energy expenditure. 5. Cleft lip and Palate: Treatment: Surgical treatment is to repair the defect for good cosmetic and functional results. Nonsurgical treatment addresses how to feed the infant until the cleft is repaired and how to optimize growth and nutrition and promote successful surgical treatment. 9  10/15/2024 5. Cleft lip and Palate: 6. Inborn Errors of Metabolism (IEM): There are three major nutrients that are not metabolized correctly: 1. Protein (amino acids/organic acids/urea cycle) 2. Carbohydrate (glucose/galactose/fructose) 3. Fat (fatty acids/transport/electron transport chain) 10  10/15/2024 6. Inborn Errors of Metabolism (IEM): Dietary management requires an understanding of both; (1) normal nutritional requirements and (2) the basic biochemical defect. Dietary therapy should be started as early as possible to protect the brain from the toxic effects of the substrate and its by products. In most situations, dietary therapy has to be continued throughout life. Frequent measurements of plasma amino acids and other metabolites is essential for monitoring deficiencies and control of the disorder. 6. Inborn Errors of Metabolism (IEM): The principle strategies of management are: 1. Dietary reduction of substrates, frequently protein or amino acids, associated with the formation of the toxic metabolites, for example, phenylalanine in PKU. 2. Replacement of essential nutrients that are deficient as a result of the metabolic block, for example, tyrosine in PKU. 11  10/15/2024 6. Inborn Errors of Metabolism (IEM): 3. Co-factor therapy: Pharmacological doses (up to 100 times the nutrient requirement) of specific vitamins to induce non-functional enzyme activity. 4. Enhancement of excretion or product utilization to form non-toxic metabolites. 6.1. Phenylketonuria (PKU): Most common disease of the inborn errors of metabolism (IEM). The aromatic amino acid, phenylalanine, is essential for protein synthesis and also over half of it is converted by phenylalanine hydroxylase to tyrosine, another important amino acid. 12  10/15/2024 6.1. Phenylketonuria (PKU): Etiology: Phenylalanine is not metabolized to Tyrosine because of a deficiency or inactivity of phenylalanine hydroxylase (PAH). Autosomal recessive (i.e. both parents carry traits to pass it on). When PAH is not functioning, phenylalanine passes into the urine as phenylketonuria. 6.1. Phenylketonuria (PKU): 13  10/15/2024 6.1. Phenylketonuria (PKU): Thyroxine Melanin Epinephrine Hyperphenylalaninemias: (1) Phenylalanine hydroxylase deficiency. (2) Dihydropteridine reductase deficiency. (3) Biopterin synthetase. 6.1. Phenylketonuria (PKU): 14  10/15/2024 6.1. Phenylketonuria (PKU): 6.1. Phenylketonuria (PKU): Diagnosis Should be done as early as possible because mental growth is developed in the early months to approximately 2 years. A blood sample is taken from the heel of the baby: Normal = 1 – 3 mg/dl PKU > 8 mg/dl To ensure that blood concentrations of phenylalanine and tyrosine are close to normal, blood tests are performed periodically. 15  10/15/2024 6.1. Phenylketonuria (PKU): Dinitrophenyl hydrazine test (DNPH): 1 ml of urine in a test tube, then an indicator or chemical reacting with it: Pale yellow (orange): negative test Bright yellow: positive test A blood sample is taken from the heel of the baby: Normal = 1 – 3 mg/dl PKU > 8 mg/dl To ensure that blood concentrations of phenylalanine and tyrosine are 6.1. Phenylketonuria (PKU): Symptoms: Mental retardation Behavioral or social problems Seizures, tremors or jerking movements in the arms and legs Hyperactivity Stunted growth Skin rashes (eczema) Small head size (microcephaly) 16  10/15/2024 6.1. Phenylketonuria (PKU): A musty odor in the child's breath, skin or urine, caused by too much phenylalanine in the body. Fair skin and blue eyes, because phenylalanine cannot transform into melanin — the pigment responsible for hair and skin tone. 6.1. Phenylketonuria (PKU): Medical Nutrition Intervention (MNI) See attachment Nutritional treatment involves restricting the substrate (Phe) and supplementing the product (Tyr). Blood Phe concentration must be checked regularly, depending on the age and health status of the child, to be sure it remains within the range of 2 to 6 mg/dL or 120 to 360 μmol/L. Phe-containing foods are offered as tolerated as long as the blood concentration of Phe remains in the range of good biochemical control. 17  10/15/2024 6.1. Phenylketonuria (PKU): The child’s rate of growth and mental development must be monitored carefully. 1. Formula Dietary therapy is planned around the use of a formula/medical food with Phe removed from the protein. It provides a major portion of the daily protein and energy needs for affected infants, children, and adults. 6.1. Phenylketonuria (PKU): The protein source in the formula/medical food is L-amino acids, with the critical amino acid (i.e. Phe) omitted. Carbohydrate sources are corn syrup solids, modified tapioca starch, sucrose, and hydrolyzed cornstarch. Fat is provided by a variety of oils. Some formula/medical foods contain no fat or carbohydrate, so these components must be provided from other sources. 18  10/15/2024 6.1. Phenylketonuria (PKU): Phe-free formula is supplemented with regular infant formula or breastmilk during infancy and cow’s milk in early childhood to provide high-biologic value protein, nonessential amino acids, and sufficient Phe to meet the individualized requirements of the growing child. The optimal amount of protein substitute depends on the individual’s age and enzyme activity; thus it must be prescribed individually. 6.1. Phenylketonuria (PKU): The Phe-free formula and milk mixture often provides approximately 90% of the protein and 80% of the energy needed by infants and toddlers. A method for calculating the appropriate quantities of a low-Phe food pattern is in the attachment document. To support metabolic control effectively, formula/medical foods must be consumed in three or four nearly equal portions throughout the day. 19  10/15/2024 6.1. Phenylketonuria (PKU): 2. Maternal PKU A pregnant woman with elevated blood Phe concentrations endangers her fetus because of the active transport of amino acids across the placenta. Babies whose mothers have elevated blood Phe concentrations have an increased occurrence of cardiac defects, restricted growth, microcephaly, and intellectual disability. 6.1. Phenylketonuria (PKU): Strict control of maternal Phe levels before conception and throughout pregnancy offers the best opportunity for normal fetal development. 20  10/15/2024 6.1. Phenylketonuria (PKU): 3. Low-phenylalanine foods: Foods of moderate- or low-Phe content are used as a supplement to the formula or medical food mixture. These foods are offered at the appropriate ages to support developmental readiness and to meet energy needs. See attachment. 6.2. Maple syrup disease (MSUD): Maple syrup urine disease (MSUD), or branched-chain ketoaciduria, results from a defect in enzymatic activity. It is not very common (1:216000). Infants appear normal at birth, but by 4 or 5 days of age they demonstrate poor feeding, vomiting, lethargy, and periodic hypertonia. A characteristic sweet, malty odor from the urine and perspiration can be noted toward the end of the first week of life. 21  10/15/2024 6.2. Maple syrup disease (MSUD): Etiology: It is an autosomal recessive gene causing a defect in the synthesis of the Branched Chain Ketoacid Dehydrogenase enzymes. 6.2. Maple syrup disease (MSUD): 22  10/15/2024 6.2. Maple syrup disease (MSUD): The decarboxylation defect of MSUD prevents metabolism of the branched-chain amino acids (BCAAs) leucine, isoleucine, and valine. Leucine tends to be more problematic than the others. The reason why leucine metabolism is significantly more abnormal than that of the other two BCAAs is still not well known. 6.2. Maple syrup disease (MSUD): Symptoms Avoiding food, vomiting Feeding difficulties Lethargy Seizures Urine that smells like maple syrup 23  10/15/2024 6.2. Maple syrup disease (MSUD): Possible Complications Coma Death Neurological damage 6.2. Maple syrup disease (MSUD): Medical Nutrition Intervention (MNI) The goal of therapy is to maintain plasma leucine concentrations of 100– 200 μmol/L for infants and children