Paeds endocrine (short stature in children) Flashcards
What is the decimal age?
Expressed as a decimal fraction in units of years
It’s calculated as the number of days / 365.25. The extra 0.25 is to account for the leap year
What is growth velocity?
Height or weight velocity is a variable derived from the measurement of height or weight at different times and represents the increase in height or weight during a fixed period
There is a dip pre-puberty, and a spurt during puberty
How is genetic potential height calculated?
Determined by the corrected mid-parental height method
Average height difference between men and women is 13cm
So 6.5cm in added to the average parental height for men, and 6.5cm in subtracted for women
What is bone age?
X-rays are taken of the bones to determine the maturity of the child’s skeletal system
Useful in evaluating growth and puberty disorders, deciding when to start therapy for hypogonadism, and to monitor growth hormone therapy
What is the bone age used for?
- Used to diagnose Freeman-Sheldon syndrome (FSS) and Chronic granulomatous disease (CGD)
- interpreting hormone levels in puberty
- Diagnosis of precocious puberty or hyperandrogenism
What is Freeman-Sheldon syndrome (FSS)?
Main feature: small mouth and pursed lips
Affects the development of the bones, joints, head, and face
What is Chronic granulomatous disease (CGD)?
Susceptibility to repeated bacterial and fungal infections
Also associated with the development of granulomatous lesions of the skin, lungs, bones and lymph nodes formed by collections of inflammatory white blood cells
How does Growth hormone deficiency present in children?
- Slowing down of height velocity after the age of 3
- Shorter than other children
- May be chubby
Treated with Growth hormone supplements
When is it too late to prescribe growth hormone treatment?
Once the child has completed growth and the growth hormone are fused
What is the US/LS ratio?
Upper segment/ lower segment ratio
Calculated by dividing the US by the LS
- Lower segment (LS): Measure from the symphysis pubis to the floor
- Upper segment (US): Subtract the LS from the height
What are some causes of growth failure?
- Familial short stature
- Constitution delay in growth and maturation (delayed puberty)
- Malnutrition
- Chronic disease/ systemic disorders
- Psychosocial dwarfism
- Chromosomal abnormalities
- Noonan syndrome
- Russell-Silver syndrome (RSS)
- Prader-Willi syndrome (PWS)
- Target tissue defects
- Bone and cartilage disorders
- Hypothyroidism
- Growth hormone insensitivity
- Glucocorticoid excess
What is psychosocial dwarfism?
Caused by deprivation, emotional stress and/or neglect
Characterised by symptoms of delayed motor and intellectual development, abnormal eating and drinking habits, enuresis and encopresis, aggressiveness and a pathological family structure
What is Noonan syndrome?
A genetic condition that stops typical development in various parts of the body
Causes fussy eaters, behave immaturely compared to children of a similar age, have problems with attention and have difficulty recognising or describing their or other people’s emotions
What is Russell-Silver syndrome (RSS)?
A growth disorder characterised by intrauterine growth restriction (IUGR), poor growth after birth, a relatively large head size, a triangular facial appearance, a prominent forehead (looking from the side of the face), body asymmetry and significant feeding difficulties
What is Prader-Willi syndrome (PWS)?
A genetic multisystem causing lethargy, diminished muscle tone (hypotonia), a weak suck and feeding difficulties with poor weight gain and growth and other hormone deficiency
Also causes intellectual disability
