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

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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

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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

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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!

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