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PAEDIATRIC ENDOCRINOLOGY

PAEDIATRIC ENDOCRINOLOGY. DR NOMAN AHMAD CORK UNIVERSITY HOSPITAL. 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

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  1. PAEDIATRIC ENDOCRINOLOGY DR NOMAN AHMADCORK UNIVERSITY HOSPITAL

  2. Presentation Outline • Paediatric endocrinology scope • Physiology of endocrine system • Normal growth • Prerequisites • Parameters • Short stature evaluation • Congenital hypothyroidism • Congenital Adrenal Hyperplasia

  3. 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

  4. Pituitary Gland

  5. Pituitary Gland

  6. Pituitary Gland

  7. Hypothalamic-Pituitary GH-IGF1 Axis

  8. Growth Hormone Secretion IGF1

  9. Hypothalamic-Pituitary-Thyroid Axis TSH

  10. Hypothalamic-Pituitary Adrenal Axis

  11. Cortisol Production 8.00 AM Cortisol Or ACTH stimulation test

  12. Renin-Angiotensin-Aldosterone ELECTROLYTES BLOOD PRESSURE

  13. Hypothalamic-Pituitary Gonadal Axis LH FSH GnRH Stimulation

  14. Bone Mineral Metabolism

  15. Glucose Metabolism • Insulin • Glucagon • Growth hormone • Glucocorticoids • Catecholamines

  16. Normal GrowthAndEvaluation of Short Stature

  17. Normal Growth

  18. Normal Growth

  19. Normal Growth

  20. Normal Growth • Growth represents general health of a child • Growth is analysed with • Percentile • SDS • Height velocity • Weight for height • Mid parental height

  21. What does a child need to grow? • Food (money) • Hormones • Good genes • A good start (intrauterine) • Good general health • Love

  22. Important Growth Factors • Prenatal • Insulin • IGF-1 and IGF-2 • Postnatal • Growth hormone and IGF-1 • Thyroxin • Puberty • Gonadal hormones

  23. 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

  24. 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

  25. 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

  26. Measurements

  27. Mid Parental Height Target Height is MPH ± 10 cm • Boys Father Ht. +Mother Ht. + 13 2 • Girls Father Ht. + Mother Ht – 13 2

  28. 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

  29. Upper to lower segment ratio

  30. Measurements • Weight • BMI • Growth Velocity • Arm span

  31. Causes of Short Stature • Genetic • IUGR or SGA • Chromosomal • Nutritional • Chronic Illness • Endocrine • Bone Dysplasia

  32. Causes of Short Stature • Short and obese • Hormone deficiency • Syndrome • Short and thin • Constitutional • Malnutrition • Systemic disease • Tall and obese • Exogenous obesity BMI

  33. Endocrine Causes • Growth hormone deficiency or resistance • Hypothyroidism • Cushing syndrome • Precocious puberty

  34. 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 Diagnostic Evaluation

  35. Congenital Hypothyroidism

  36. 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

  37. Isotope Scan

  38. 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

  39. Congenital Adrenal Hyperplasia

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