Endocrine Flashcards
1
Q
Pediactric differences in the endocrine system
A
- less developed at birth than any other system
- hormonal control is lacking until 12-18 mos
- infants manifest imbalances: fluids/ lytes/ amino acids/ glucose etc.
2
Q
PKU- Phenylketonuria
A
- autosomal recessive
- deficiency of phenlalanine hydroxylase
- can adversely affect the CNS
3
Q
Infant PKU s/s
A
- often evident by 3 mos
- digestive problems: vomiting
- failure to gain weight
- urine odor (musty)
- eczema
- hypertonia
- hyperactive behavior, irritable
4
Q
Children PKU s/s
A
- hypopigmentation or hair, skin, irisis
- blonde with light blue eyes
- mental retardation in long-term consequences
5
Q
Diagnostics
A
- mandatory NBS (newborn screening)
- false negatives may occur in tests done before 3rd day of life
- repeat test if done before 3rd day
6
Q
PKU Treatment
A
- start ASAP
- Diet: restrict phenylaline
- Infants: low phenylaline formula
- protein restricted solids
- Main food source: vegetables, fruits, starches
- phenylaline-free protein substances
7
Q
Older child treatment PKU
A
- phenyl-free milk
- avoid high-protein foods: milk, dairy products, eggs
- encourage foods low in phenylaline: veg, fruits, juices, cereals, breads, starches
- Avoid aspartame- contains pheylaline
8
Q
Growth Hormone (GH) Deficiency
A
- consistently poor growth (<5cm/yr)
- growth rate more than two standard deviations below the mean for age
- downward deviation from the previous growth curve
9
Q
GH Deficiency Etiology
A
- Anterior pituitary secretes insufficient GH
- most often cause: idiopathic
- may be underlying cause: -pituitary malformation
- pituitary or hypothalamus tumors
10
Q
GH Deficiency Clinical Manifestations
A
- < muscle mass
- relatively increased adiposity
- micropenis
11
Q
GH Deficiency Management
A
- treat underlying cause if identified
- GH replacement therapy
- Monitor growth
- early tx increases effectiveness
- continue tx until: Acceptable height achieved and x-ray confirms growth plate fusion
12
Q
Puberty
A
- Sexual maturation- may take 2-3 yrs
- occurs when gonads secrete increased amounts of estrogen and testosterone
- average onset 10 yrs girls and 11 yrs boys
- the hypothalamus produces gonadotropin releasing hormone, this stimulates the anterior pituitary to increase production of LH and FSH
13
Q
Precocious puberty definition
A
- onset before 8 yrs girls 9 yrs boys
- premature secondary sexual characteristics
- accelerated growth rate
- advanced bone maturation
14
Q
The child w/ precocious puberty
A
- child appears older than chronologic age and should be treated according to true age
- child is often teased by other children
- child is at increased risk for abuse
- may be present in infancy or cchildhood
15
Q
Precocious puberty Dx
A
GnRH stimulation test
radiographic studies
