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PAEDIATRIC ENDOCRINOLOGY

DR NOMAN AHMADCORK UNIVERSITY HOSPITAL

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Presentation Outline

Paediatric endocrinology scope Physiology of endocrine system Normal growth

Prerequisites Parameters

Short stature evaluation Congenital hypothyroidism Congenital Adrenal Hyperplasia

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Paediatric Endocrinology Scope

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

Adenohypophysis Neurohypophsis

Anterior lobe Middle Lobe

Somatotrophs

Thyrotrophs

Lactotrophs

Gonadotrophs

Corticotrophs

Growth hormone

TSH

Prolactin

LH & FSH

ACTH

MSH & Endorphins

Posterior Lobe

AVP

Oxytocin

Pituitary Gland

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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 FSHGnRH Stimulation

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Bone Mineral Metabolism

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Glucose Metabolism

Insulin Glucagon Growth hormone Glucocorticoids Catecholamines

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Normal GrowthAnd

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

Growth represents general health of a child

Growth is analysed with Percentile SDS Height velocity Weight for height Mid parental height

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What does a child need to grow?

Food (money) Hormones Good genes A good start (intrauterine) Good general health Love

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Important Growth Factors

Prenatal Insulin IGF-1 and IGF-2

Postnatal Growth hormone and IGF-1 Thyroxin

Puberty Gonadal hormones

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Constitutional Delay in Growth and Adolescence (CDGA)

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

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

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 Boys Father Ht. +Mother Ht. + 13

2 Girls

Father Ht. + Mother Ht – 13 2

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Upper to lower segment ratio

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

Weight

BMI

Growth Velocity

Arm span

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Causes of Short Stature

Genetic IUGR or SGA Chromosomal Nutritional Chronic Illness Endocrine Bone Dysplasia

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Causes of Short Stature

Short and obese Hormone deficiency Syndrome

Short and thin Constitutional Malnutrition Systemic disease

Tall and obese Exogenous obesity

BMI

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Endocrine Causes

Growth hormone deficiency or resistance

Hypothyroidism Cushing syndrome Precocious puberty

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Diagnostic Evaluation

FBC Electrolytes ESR BUN, creatinine Bone profile LFT Glucose Coeliac screen Urinalysis

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

1:2000 to 1:4000 live births F:M 2:1 Most common treatable cause of

mental retardation Thyroid dysgenesis

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

CAH is disorder of adrenal cortex 21 hydroxylase deficiency

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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