Growth and development + paediatric endocrine problems Flashcards
Measurements to assess growth
Height
Length (i.e. measuring them lying down) - from 0-2yrs
Sitting height - measuring this then subtracting from total height); done in people with unusual body proportions between top and bottom half
Head circumference
Weight
How is the potential height of a child calculated from their parents
By obtaining the mid-parental height
- if male, add together the father’s and the mother’s height, divide this by two then add 7cm to the total (still normal if +/- 10cm)
- if female, add together the father’s and the mother’s height, divide this by two then subtract 7cm from the total (Still normal if +/-8.5cm)
What is bone age an indicator of
Skeletal maturation: how much growth has taken place and how much there is left.
Name the method that’s used to stage puberty
+ describe the stages (5)
TANNER METHOD
- Breast development (stage 1-5) (females only)
- Genital development (stage 1-5) (males only)
- Pubic hair (stage 1-5)
- Axillary hair (stage 1-3)
- Testicular volume (2-20ml)
Reasons for delayed bone age (4)
Constitutional delay of growth
Growth hormone deficiency
Hypothyroidism
Malnutrition/chronic illness
Reasons for advanced bone age (6)
Tall stature Premature adrenarche Overweight Early puberty Congenital Adrenal Hyperplasia Overgrowth syndromes
Name 2 normal variations of growth
Familial short stature (FSS)
-associated with normal skeletal maturation
Constitutional growth delay (CGD)
-delayed bone age but eventually will catch up
Be aware of the main patterns of growth disorders
Short and thin
Short and fat,
Short and dysmorphic
Prepubertal sizes of testicles are estimated to be of what testicular volume
Pubertal sizes of testicles are estimated to be of what testicular volume
Adult sizes of testicles are estimated to be of what testicular volume
Prepubertal sizes - 1–3 ml,
pubertal sizes - 4 ml and up
adult sizes - 12-25ml
Know the history and examination features to be identified when assessing growth disorders
Birth weight
PMH/ FH/ SH
Growth charts
Examination: Dysmorphic features Current height/weight Bone age Pubertal assessment
Define normal growth
progression of changes in height, weight, and head circumference that are compatible with established standards for a given population
Factors influencing height (7)
Age Sex Race Nutrition Parental height Puberty Growth disorder
Growth is driven by what in infantile years of life (0-2)
+ is growth rapid or not in this 2 years
NUTRITION, not growth hormone
rapid growth
Growth in childhood years (2-12) is largely driven by what hormones
+ is growth rapid or not in these childhood years
Growth hormone
Thyroxine
Not as rapid growth as infantile years
Growth in pubertal years of life (12+) is largely driven by what
Sex steroids that cause an increase in growth hormone secretion:
- testosterone in boys
- oestrogen in girls
Growth acceleration is limited towards the end of puberty by what
fusion of the epiphyseal plates (due to oestrogen in both sexes)
Do males/females grow faster at start of puberty
Females
Males grow fastest in mid-puberty
What is menarche + around what age does it start
Start of menstruation, i.e. first period
13
Tanner pubertal stage indicating start of puberty
- males (2)
- females
To help: TANNER METHOD -Breast development (stage 1-5) (females) -Genital development (stage 1-5) (males) -Pubic hair (stage 1-5) -Axillary hair (stage 1-3) -Testicular volume (2-20ml)
Males - G2 (scrotum and testes begin to enlarge) and T3-4ml
Females - B2 (breast budding)
Men end up taller than women by around 12.5cm because of what 3 factors
Pubertal growth spurt starts 2 years later than in girls (14 yr compared to 12 yr)
Pubertal growth spurt is more intense in boys
Boys are slightly bigger than girls during childhood
Pubertal growth spurt age in
- males
- females
14 (12-16)
12 (10-14)
-EARLIER in females
Effect of obesity on childhood growth
Usually taller than those the same age
Non-pathological causes of short stature (3)
Familial
Constitutional delay - short initially but eventually will catch up, however will be in lower half of target range
Small for gestational age (SGA)
Pathological causes of short stature (6)
Undernutrition
Chronic illness (juvenile idiopathic arthritis, IBD)
Iatrogenic (Steroids)
Psychosocial factors
Hormonal (growth hormone deficiency, hypothyroidism)
Genetic syndromes (turner, prader willi)
Genetic syndromes that affect growth (4)
Turner syndrome (–> short stature in females)
Prader willi syndrome (–> short stature in males)
Noonan’s syndrome (–> short stature in both sexes)
Achondroplasia (–> short limbs –> disproportionate short stature)
Clinical features of growth hormone deficiency (hypopituitarism) in newborns (3)
+ other clinical features in general (3)
hypoglycaemia,
prolonged jaundice,
micropenis
Excess subcutaneous tissue
Mid-face hypoplasia
Delayed skeletal maturation
3 most common characteristics of Noonan syndrome
unusual facial features
short stature (restricted growth)
heart defects present at birth (congenital heart disease)
What is achondroplasia + clinical features
Genetic bone growth disorder that causes disproportionate dwarfism
Normal sized trunk but short legs and arms
-mainly affects the upper arms and thighs
What is turner syndrome + clinical features (5)
Chromosomal abnormality involving a complete/ partial absence of the second sex chromosome –> 45, X
Short stature Underdeveloped ovaries (gonadal dysgenesis) Webbed neck Delayed puberty Congenital heart defects
What is prader willi syndrome + clinical features (4)
Chromosomal abnormality of chromosome 15
Excessive appetite –> overweight
Short stature
Intellectual impairment
Weak muscles (hypotonia)
What is Noonan’s syndrome + clinical features (4)
Autosomal dominant inherited disorder
short stature,
facial dysmorphisms,
chest deformity,
congenital heart disease
It’s common for males to have delayed puberty at what age and above + especially what group of boys does this occur in
> 14
Those with constitutional delay of growth and puberty (CDGP)
Causes of delayed puberty (3)
Constitutional delay of growth and puberty (CDGP) - mainly boys
Gonadal dysgenesis (e.g. turner syndrome)
Chronic disease (e.g. crohn’s)
What is thelarche
onset/appearance of breast development in girls - usually occurring at start of puberty
What is infantile thelarche/ premature thelarche
Early sexual, specifically breast, development in females
Incomplete form of pubertal development without any other signs
Is a NORMAL VARIANT of pubertal development
What is true/ central precocious puberty
+ what investigation needs to be done
Early puberty signs that’s thought to be linked with problems in the hypothalamus/pituitary gland (hence CENTRAL)
Hypothalamus starts releasing gonadotropin-releasing hormone at an earlier age than usual
- -> early breast/testicular development
- -> advanced bone age
MRI - TO EXLUDE PITUITARY TUMOUR
What is premature pubarche
Normal variant of pubertal development in which pubic and/or axillary hair develops early
What is adrenarche
early stage in sexual maturation where adrenal gland starts secreting increased levels of androgens which promote development of pubic hair, oily skin, oily hair, body odour
Cause of central precocious puberty in girls v boys
Usually idiopathic in girls
Boys more likely to have a trigger like brain tumour
What is precocious pseudopuberty
+ condition needs to be excluded
Partial pubertal development that results from gonadotropin-independent (i.e. independent of luteinizing hormone or follicle stimulating hormone) production of testosterone in a prepubertal boy
so testosterone secretion is abnormal
MUST EXCLUDE CAH
What to assess in an obese child (4)
Weight
BMI
Height
Waist circumference
If child is obese AND short, is this normal
No
Obese children are usually taller
Pathological causes of obesity in children (4)
Genetic syndrome, e.g. prader willi
Hypothalamic-pituitary pathology
Endocrine disorders
Drugs, e.g. insulin, steroids
Complications of obesity (6)
Fatty liver disease Gallstones Reproductive dysfunction Nutritional deficiencies Sleep apnoea Heart disease
Drugs that can increase weight (4)
Inuslin
Steroids
Antithyroid drugs
Sodium valproate
Genetic syndromes that cause obesity
Prader willi
Down’s syndrome
Endocrine disorders that cause weight gain/obesity (5)
Hypothyroidism Growth hormone deficiency Glucorticoid excess Hypothalamic lesion, e.g. tumour, trauma (--> loss of appetite control) Insulinoma
Treatment of obesity (3)
diet change
Exercise
Psychological support
Clinical features of hypopituitarism in adults (5) + children (4)
Children
- dwarfism/short stature
- slow growth
- overweight
- delayed puberty
Adults
- premature ageing
- fatigue
- infertility
- reduced sex drive
- decreased body hair
What is congenital adrenal hyperplasia
A group of autosomal recessive inherited enzyme deficiencies
- missing the enzyme that stimulates the adrenal glands to release corticosteroids, e.g. cortisol
What condition is associated with females being born with virilised (male looking) genitalia
Congenital adrenal hyperplasia
Clinical features of congenital adrenal hyperplasia (CAH) (5)
Dehydration –> hyponatraemia
hypoglycaemia
Weight loss
Vomiting
Ambiguous genitalia
How to immediately test for diabetes in children
Finger prick capillary glucose test
If >11mmol/l then suspicious, phone specialist for same day review
Typical 4 symptoms of type 1 diabetes
Thirsty
Fatigue
Weight loss
Peeing more
Tools to assess growth in childhood
Height/ length/ weight Growth Charts and plotting MPH and Target centiles Growth velocity Bone age Pubertal assessment
IGF-1 is a marker of what hormone
GH
What simple medical instrument can be used to assess testicular volume
Orchidometer
Puberty is considered early or delayed at what age
- males
- females
Males <9 (rare), >14
Females <8, >13 (rare)
Commonest cause of acquired hypothyroidism in kids
+ symptoms/signs
Hashimoto’s thyroiditis
Symptoms/signs
- lack of HEIGHT gain
- pubertal delay
