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Some aspects of paediatric endocrinology

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low growth hormone peaks in GH stimulation test. Causes of GHD ... Psychosocial deprivation. Cranial irradiation. Secondary tumours of hypothalamus or pituitary ... – PowerPoint PPT presentation

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Title: Some aspects of paediatric endocrinology


1
Some aspects of paediatric endocrinology
  • Luczay
  • 15/09/2008

2
Parathyroid gland
3
GhRH GnRH TRH CRH ADH
GH FSH, LH TSH ACTH PRL
4
SHORT STATURE
15 organic diseases 2 skeletal dysplasia 1
Turner syndrome 1 hypothyroidism 1 GHD
80 FSS PSD
5
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6
GHD
  • micropenis, hypoglycaemia in newborns
  • delay in dentation
  • progressive growth delay
  • bone age retardation
  • low growth hormone peaks in GH stimulation test

7
Causes of GHD 1.
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9
Diabetes insipidus
  • ADH defficiency
  • polyuria, polidipsia
  • enuresis
  • fever in infancy
  • Hypernatraemia, low urine osmolarity

10
Parathyroid gland
thyroxine, trijodthyronine
11
Patology of the thyroid gland IHypothyroidism
  • Congenital hypothyroidism
  • 1/4000 live birth
  • floppy infant, thick, protruding tongue,
  • poor feeding, constipation,
  • prolonged jaundice
  • Mass screenig TSH ?
  • L-thyroxine supplementation

12
Patology of the thyroid gland IHypothyroidism II.
  • Hashimoto thyreoiditis
  • goiter
  • Autoantibody positivity
  • Inhomogen ultrasound structure
  • Lympocytic infiltration in histology
  • fatigue, constipation, short stature
  • TSH?, fT4?
  • L-thyroxine supplementation

13
Patology of the thyroid gland IHyperthyroidism
II.
  • Graves-Basedow thyroiditis

exophtalmus
tachycardia, weight loss, diarrhoea
TSH?, fT4, fT3?
methimasol, surgery, iodine isotope treatment
14
Parathyroid gland
PTH
15
Pathology of the parathyroid gland
  • Hypoparathyroidism
  • se Ca?, seizure, osteoporosis
  • Hyperparahyroidism adenoma, hyperplasia
  • se Ca?, nephrolithiasis,

16
Parathyroid gland
glucocorticoids cortisol mineralocorticoids
aldosteron androgens androstendione
17
Steroid biosynthesis
P450c21
18
Congenital adrenal hyperplasia
  • 95 21-OH deficiency
  • ¾ salt waisting form- in girls - ambigous
    genitalia

- early pubic hair puberty in boys
19
Parathyroid gland
testosterone
20
47,XXY Klinefelter delayed puberty gynaecomastia m
ental retardation
21
Parathyroid gland
estrogen progesterone
22
Turner syndrome
  • 1/2500 girls/year
  • Lymphoedema of the hands and/or feet
  • Pterygium colli, short stature, facial dysmorphy
  • Coarctation of the aorta, renal malformation
  • Sexual infantilism (streak gonad)
  • Karyotype 45,X

23
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