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PaediatricsEndocrine problems - key factsWojciech Cymes
Plan
GROWTH CHARTS
short stature
puberty problems
hypothyroidism
CAH
diabetes
Growth Charts
UK-WHO growth charts, 0-18 years
children of the same age and maturity who have shown optimum growth
based on WHO Child Growth Standards
Hospital chart
THE Red Book
Weight and Height changes in time head circumference up to 1yo
NB correction for prematurity up to 2 yo
0.4, 2, 9, 25, 50, 75, 91, 98, 99.6 centiles
Short Stature
Causes
constitutional delay
pubertal delay
GH deficiency
Cushing's syndrome/ disease
skeletal disorder
chronic illness
malabsorption
congenital heart disease
CF
DM
immunodeficiency
hypopituitarism
hypothyroidism
Turner's syndrome
Emotional problems
Short Stature
Height below 0.4th centile
Need ≥2 measurements 6 months apart growth velocity important
height vs. mid-parental height (F+M)/2
trace the centile
+/- 10cm boy, +/- 8cm girls
History
childhood illness
FHx of skeletal disorders
Examination
growth charts!!!
dysmorphic features
weight
stage of puberty
+ 7cm (boys)
-7 cm (girls)
Short Stature
Investigations bone age
karyotype
TFTs
GH and IGF-1
coeliac screen
inflammatory markers
Management GH replacement
IGF-1 replacement if GH resistance
LOOK AT EXAMPLES IN LISSAUER
Puberty
defined sequence of changes leading to full sexual development
Female:
breast development (8.5-12.5 years)
pubic hair growth
Rapid height spurt
menarche
Male:
Testicular enlargement
pubic hair growth
height spurt
©www.uwgb.edu
Delayed puberty
Boys > girls
Absence of pubertal development at 14 in girls and 15 in boys
Causes constitutional delay most common
hypogonadotropic hypogonadism
CF
anorexia
hypothyroidism
hypopituitarism
hypergonadotroipc hypogonadism
Turner's or Kleinefelter's syndromes
steroid enzyme deficiencies
gonadal damage
Delayed puberty
Assessment pubertal staging
growth staging
chronic systemic disorders
karyotype in girls
Management treat the cause if possible
reassure!
accelerate growth and puberty if necessary
oxandrolone and later testosterone for boys
oestradiol for girls
psychologist's help
©hardballtalk.nbcsports.com
Precocious puberty
Girls > boys
in girls often premature onset of normal puberty
Gonadotropin dependent
idiopathic
CNS abnormalities
hypothyroidism
Gonadotropin independent
CAH
granulosa cell tumours / Leydig cell tumours
exogenous sex steroids
Precocious puberty
Management treat underlying cause if possible
reduce rate of skeletal maturation if necessary
early growth spurt -> early growth cessation -> lower adult height
GnRH analogues for gonadtropin-dependent
androgen / oestrogen inhibitors or antagonists for gonadotropin-independent
Turner's syndrome
45 X0
most cases lead to early miscarriage
risk not affected by maternal age
Clinical features:
short stature (NB may be the only one)
webbed neck
cubitus valgus
widely spaced nipples
delayed puberty and ovarian dysgenesis
normal intelectual function
Management
GH replacement
oestrogen replacement at puberty time
©www.pinterest.com
Congenital adrenal hyperplasia
AR 21-hydroxylase deficiency
Presentation virilisation of female infants
enlarged penis in male infants (rarely identified)
salt-losing crisis at 1-3 weeks of age
vomiting, weight loss, collapse
tall and muscular built
precocious pubarche
Management glucocorticoid (to allow normal growth) ± mineralocorticoid replacement
monitor growth, skeletal maturity and androgens - avoid over-replacement
additional hormone replacement at times of stress
±corrective surgery in females
Hypothyroidism
Congenital 1:4000
untreated leads to cretinism
causes
thyroid development anomalies
iodine deficiencies
TSH deficiency
usually picked up on Guthrie test before symptomatic
lifelong thyroxine replacement
Acquired short stature
slipped upper femoral epiphysis
school work deterioration
other features as in adults
Type 1 diabetes mellitus
Autoimmune condition
Classic triad: polydipsia
polyuria
weight loss
Treat by replacing insulin MDT approach
sick day rules important to avoid DKA
education
monitoring and injection technique
basic pathology
diet©www.afb.org
Diabetic ketoacidosis
Relative deficiency of insulin
Can be provoked by eg infection importance of sick day rules
Triad of: acidosis
ketonaemia
hyperglycaemia
Emergency treatment rehydrate
fixed infusion rate insulin until ketones normalise
replace glucose and potassium as needed
SENIOR HELP NEEDED
identify reasons, educate!
Thank you!
Remember the GROWTH CHART!
This sessions won't grow any better without your feedback!
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