Endocrine disorders and Centile charts and assessment of child growth II Flashcards
When should the cranial sutures close? [1]
After 18 months
Name two types of ISS
constitutional growth delay
familial short stature
Describe how the growth of an individual with familiar short stature occurs
How does their height compare to their parents?
- Normal birth weight and length - first two years of life linear growth velocity slows as they near their genetically determined centile (normal in first but will slow towards second)
- Once target centile is reached the child resumes normal linear growth to the growth curve (not going up and down)
- Grows along growth centile: final height is short but appropriate for family
Describe how growth of an individual with constitutional growth delay occurs?[2]
How does their height compare to their parents?
- Delay in bone age
- Similar to familial short stature to start with: below the 5th centile, but have delay in skeletal maturation AND puberty: occurs later
- No necessarily short parents
- Growth continues beyond the time when for an average child stops growing
- Final height is normal
- Often a Fx of late growth spurt
Describe the release pattern of GH [1]
GH is stimulated by the release of ? [1]
Pulsatile
GHRH –> GH –> IGF-1
What stimulates the release of GH? [3]
When is GH released? [3]
Sleep, exercise and hypoglycaemia
Release peaks during deep sleep, 3hrs after a meal and after exercise
Growth hormone deficiency characterised by? [3]
Growth hormone deficiency characterised by:
- decreased growth velocity
- delayed skeletal maturation
- in the absence of other explanations
**Normal birth weight and only slighly reduced length **
Normal growth: but after 7 / 8 years growth plateaus (regains after GH treatment)
What can cause GH deficiency? [7]
What is the most common? [1]
- Isolated
- Coexist with other anterior pituitary deficiencies
- Congenital (septo-optic dysplasia or ectopic posterior pituitary as anterior part may also be missing or underdeveloped)
- Genetic (GH or GHRH gene mutation)
- Acquired (craniopharyngioma, germinoma, histocytosis or cranial irradiation)
- Idiopathic is the most common 1:4000
Why does GH deficiency suggest that GH is only a minor contributor to intrauterine growth? [1]
Children with GHD have normal birth weight and only slightly reduced length
If you have short stature or decreased growth rate - what would screening studies try and rule out / discover? [2]
If no findings were found for ^, what would you diagnose? [1]
Growth Hormone Deficiency
Another underlying disease
No findings of GHD: Idiopathic short stature
What type of drug is oxandrolone and what does it help to treat? [1]
Androgen anoblic steroid: can improve height in Tuner syndrome; but not for ISS
What does giving GH to do people with ISS? (remember they dont have GHD)
Baseline IGF-1 for ISS is low; after treatment increases
GH is useful treatment for ISS
What treatment can you give for ISS? [1]
Growth Hormone
When does puberty begin in boys [1] and girls [1]
Puberty starts in 95%:
Girls between 8 -13 years
Boys between 9-14 years
What is the physiological mechanism behind puberty starting [2]
GnRH produced and stimulates LH and FSH release from AP
What are the 5 stages of James Tanner puberty?
Tanner stage 1 = pre-puberty
Tanner stage 2 = start of puberty
Tanner stage 5 = sexual maturity
What is unilineal autosomal dominant delayed puberty? [1]
What is bilineal autosomal dominant delayed puberty [1]
If one / both parents are effected from delayed puberty [1]
What is bilineal by extension delayed puberty?
Bilineal tracing descent through both maternal and paternal lines
Probs autosomal recessive
