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Health Differences Across the Lifespan 2 Study Guide Assessment: Many infants with PKU appear healthy at birth; if treatment is not started to lower phenylalanine level immediately, an infant’s IQ can drop as many as 10 points within the first month and will continue to decline. Signs and symptoms arise over time, such as failure thrive, vomiting, irritability, and urine with a musty odor. The child may also experience myoclonic or grand mal seizures. All fifty states require newborn testing. Testing information includes: • Infants should ingest adequate protein (usually 24 hours of normal feedings of breast milk or formula) prior to test being performed. • Heel blood should be used for specimen; the sample should be collected after the first 48 hours, but no later than 7 days, after birth. • A normal level is under 2 mg/dL; if the level is elevated, a repeat test is performed to validate original results. A level higher than 15 mg/dL is considered dangerous. Interventions: allow phenylalanines in the diet based on the weight of the child, usually at a level of 20-30 mg/kg body weight or amount prescribed by physician; place child on a protein restricted diet; encourage use of mature breast milk or modified protein (p phenylalanine free, hydrolysate formula) as a source of infant nutrition; use special protein foods that are free of phenylalanine for life, which is very important before the age of 6 years (because of the impact on IQ); provide emotional support for family and suggest genetic counseling because each child born to this couple has a one in four chance of having PKU Actions/Uses Bomocriptine (Parlodel) Suppresses GH level in children with GH excess in combination with octreotide Lanreotide (Somatuline Depot) Suppresses GH level in patients with acromegaly who have not responded to radiation therapy or are unable to tolerate surgery Pegvisomant (Somavert) GH receptor antagonist Ocreotide (Sandostatin) Suppresses intestinal peptide hormones, insulin, glucagon, and growth hormone Somatropin (Humatrope) Used as GH replacement therapy Mecasermin (Increlax, Iplex) Recombinant DNA insulin like growth factor with same actions as GH; used for growth failure in children only (before bone epiphyses close) 4.4 Drug Treatments Drugs that Affect Growth Hormone (GH): Generic (Trade Name)

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