Peds Test # 3 Endocrine Flashcards
(T/F) The Endocrine System is less developed at birth than any other body system.
True
What is the impact of the fact that hormonal control of many functions is lacking until 12-18 months of age?
Infants might manifest imbalances in concentration of fluids, electrolytes, amino acids, glucose, and trace substances.
Describe the etiology and pathophysiology of Phenylketonuria (PKU)?
PKU is an autosomal recessive disorder marked by a deficiency in phenylalanine hydroxylase which is the enzyme that breaks down phenylalanine into tyrosine. phenylalanine accumulates ion the blood after ingesting protein containing phenylalanine.
What are 7 SxS of Infant PKU which are often evident by 3 months old?
1) Vomiting
2) Failure to gain weight
3) Musty urine odor
4) Eczema
5) Hypertonia
6) Hyperactive behavior/irritable
7) Seizures
What are the 3 SxS of PKU in older children?
1) Hypopigmentation of hair, skin and irises
2) Blond with light blue eyes
3) Longterm mental retardation
How is PKU diagnosed?
Mandatory Newborn Screen for every infant - False negatives may occur if tests are done before the 3rd day of life.
When treatment for PKU started?
As soon as it is diagnosed
What are 7 methods used in the Tx of PKU?
1) Restrict Phenylalanine (Use phenylalanine-free supplements and milk).
2) Limit protein foods (main food source should be veges, fruits and starches).
3) Avoid aspartame (nutria sweet) because it contains phenylalanine
What are the 3 criteria used when suspecting Growth Hormone (GH) Deficiency?
1) Consistently poor growth (< 5cm/yr)
2) Growth rate more than 2 standard deviations below the mean for age
3) Downward deviation from the previous growth curve
Growth Hormone Deficiency is usually idiopathic, however, when it is not idiopathic, what is usually the underlying cause?
Usually caused by the anterior pituitary not secreting sufficient Growth Hormone because of a malformation or tumor.
What are the 7 SxS of Growth Hormone Deficiency?
1) < 5% height on growth chart
2) Immature of cherubic faces
3) Delayed puberty
4) Hypoglycemia
5) ⬇ Muscle mass
6) ⬆ Adiposity
7) Micropenis
What 5 methods are used to manage Growth Hormone Deficiency?
1) GH replacement therapy
2) Monitor growth
3) Monitor Psychosocial issues
4) Early treatment ⬆ effectiveness
5) Continue Tx until acceptable height has been achieved or X-ray confirms growth plate fusion.
What is the average age of onset of puberty in girls and boys?
1) Girls - 10 yrs old
2) Boys - 11 yrs old
What are the 4 characteristics of Precocious Puberty?
1) Onset before 8yrs for girls and 9 yrs for boys
2) Premature secondary sexual sexual characteristics
3) Accelerated growth rate
4) Advanced bone maturation
What are the 3 psychosocial aspects of Precocious Puberty that a nurse should be aware of?
1) The child appears older than their chronological age but should be treated according according to their chronological age.
2) The child is at an increased risk for abuse
3) Precocios Pubierty may present in infancy or childhood
