Transcript PAEDIATRIC_ENDOCRINOLOGY_AND_GROWTH

PAEDIATRIC
ENDOCRINOLOGY
DR NOMAN AHMAD
CORK UNIVERSITY HOSPITAL
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Presentation Outline
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Paediatric endocrinology scope
Physiology of endocrine system
Normal growth
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Prerequisites
Parameters
Short stature evaluation
Congenital hypothyroidism
Congenital Adrenal Hyperplasia
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Paediatric Endocrinology Scope
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Regulation of normal growth
Maintenance of body metabolism
Stress management
Fluid and electrolyte balance
Bone mineral homeostasis
Sex differentiation
Puberty
Glucose metabolism
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Pituitary Gland
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Pituitary Gland
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Pituitary Gland
Pituitary Gland
Adenohypophysis
Anterior lobe
Somatotrophs
Growth hormone
Neurohypophsis
Middle Lobe
MSH & Endorphins
Posterior Lobe
AVP
Oxytocin
Thyrotrophs
TSH
Lactotrophs
Prolactin
Gonadotrophs
LH & FSH
Corticotrophs
ACTH
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Hypothalamic-Pituitary GH-IGF1 Axis
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Growth Hormone Secretion
IGF1
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Hypothalamic-Pituitary-Thyroid Axis
TSH
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Hypothalamic-Pituitary Adrenal Axis
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Cortisol Production
8.00 AM Cortisol
Or
ACTH stimulation test
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Renin-Angiotensin-Aldosterone
ELECTROLYTES
BLOOD
PRESSURE
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Hypothalamic-Pituitary Gonadal Axis
LH FSH
GnRH Stimulation
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Bone Mineral Metabolism
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Glucose Metabolism
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Insulin
Glucagon
Growth hormone
Glucocorticoids
Catecholamines
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Normal Growth
And
Evaluation of Short Stature
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Normal Growth
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Normal Growth
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Normal Growth
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Normal Growth
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Growth represents general health of
a child
Growth is analysed with
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Percentile
SDS
Height velocity
Weight for height
Mid parental height
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What does a child need to grow?
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Food (money)
Hormones
Good genes
A good start (intrauterine)
Good general health
Love
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Important Growth Factors
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Prenatal
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Postnatal
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Insulin
IGF-1 and IGF-2
Growth hormone and IGF-1
Thyroxin
Puberty
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Gonadal hormones
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Constitutional Delay in Growth and
Adolescence (CDGA)
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Late bloomers
Slowing in growth and weight in
first 3 years
Normal growth rate
Delayed bone age
Positive family history
Normal final height
Common in boys
Benefit with gonadal steroids
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Familial Short Stature
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Normal intrauterine growth
Linear growth cross percentiles
downward in first 2 years or during
puberty
Bone age is not delayed
Final height is short and consistent
with mid parental height or family
history
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Pathological Short Stature
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Absolute height < 3rd percentile
Abnormal height velocity
Height SDS ->2.5 SDS
Weight to height relationship
Upper lower segment ratio
Arm span(> 6 cm)
Mid parental height
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Measurements
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Mid Parental Height
Target Height is MPH ± 10 cm
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Boys
Father Ht. +Mother Ht. + 13
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Girls
Father Ht. + Mother Ht – 13
2
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Upper to lower segment ratio
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Lower segment: upper end of
symphysis pubis to floor
Upper segment: Height – LS
U/L decline from birth to puberty
Slight increase at puberty
Precocious puberty inc. U/L
Delayed puberty dec. U/L
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Upper to lower segment ratio
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Measurements
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Weight
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BMI
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Growth Velocity
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Arm span
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Causes of Short Stature
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Genetic
IUGR or SGA
Chromosomal
Nutritional
Chronic Illness
Endocrine
Bone Dysplasia
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Causes of Short Stature
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Short and obese
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Short and thin
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Hormone deficiency
Syndrome
BMI
Constitutional
Malnutrition
Systemic disease
Tall and obese
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Exogenous obesity
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Endocrine Causes
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Growth hormone deficiency or
resistance
Hypothyroidism
Cushing syndrome
Precocious puberty
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Diagnostic Evaluation
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FBC
Electrolytes
ESR
BUN, creatinine
Bone profile
LFT
Glucose
Coeliac screen
Urinalysis
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Bone age
IGF-1
Free T4 and TSH
Growth hormone
24 hrs. urinary
cortisol
Dexamethasone
suppression test
Karyotype
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Congenital Hypothyroidism
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Congenital Hypothyroidism
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1:2000 to 1:4000 live births
F:M 2:1
Most common treatable cause of
mental retardation
Thyroid dysgenesis
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Ectopy (2/3), hypoplasia, agenesis
Hormone dysgenesis
TSH (heel prick)
Isotope scan
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Isotope Scan
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Congenital Adrenal Hyperplasia
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CAH is disorder of adrenal cortex
21 hydroxylase deficiency
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Cortisol deficiency
± Aldosterone deficiency
Androgen excess
Girls present with virilization
Boys present with salt losing crisis
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Congenital Adrenal Hyperplasia
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