short stature dr olcay evliyaoğlu. definition height lower than 3 % in growth charts(

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Short stature Short stature Dr Olcay Evliyaoğlu Dr Olcay Evliyaoğlu

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  • Slide 1
  • Short stature Dr Olcay Evliyaolu
  • Slide 2
  • Definition Height lower than 3 % in growth charts(
  • Differential diagnosis of normal and pathological short stature diopathic normal variant 6 years >4,5 cm/ year) Parallel to growth curves Pathologic -3SD GV is subnormal(
  • Pathological short stature Term, BW2500gr Postnatal Proportional Disproportional Skeletal dysplasia Dysmofic synd Systemic dis yes no Endocrine short stature TFT Hypothyroidism TFT euthyroidism Growth hormone axis
  • Slide 16
  • Management of a child with short stature Physical examination and anthropometric mesurements Somatic abnormalities, proportion of the body Signs of chronic systemic disease Height, sitting height, upper and lower segments, arm span measurement, weight, bm Growth velocity Bone age Target height Predicted adult height according to bone age
  • Slide 17
  • Height should be given as standart deviation score (SDS) (z-score) SDS = measured height mean height according to gender and age / SDS
  • Slide 18
  • Target height Girls (cm)= (mat height+pat height)-13 2 Boys (cm)=(mat height+pat height)-13 2
  • Slide 19
  • TH + 8-10cm is what expected
  • Slide 20
  • In the first 6 months of life knee later left wrist radiography In skeletal dysplasias and in some dysmorphic syndromes despite short stature bone age may not be delayed.
  • Slide 21
  • Laboratuary Total blood count Urine density, urine evaluation Kidney and liver function tests Blood gases Electrolytes Calcium, phosphorus, alkaline phosphatase. Celiac, antiendomysial, antigliadin autoantibodies Creatine kinase (CPK) TFT
  • Slide 22
  • Postnatal Proportional short stature No systemic disease Euthyroid Growth velocity < 4,5-5 cm/y Delay in bone age >2 years GH tests
  • Slide 23
  • Evaluation of growth hormone axis No response to 2 pharm stim Disturbed secretion in sleep Bioinactive GH IGF-1 N or low 20 % increase in IGF-1 levels in IGF1 generation test GH resistance GH normal or increased Low IGF-1 levels in IGF-1 generation test GH deficiency Neurosecretory dysfunction
  • Slide 24
  • Growth hormone Most of the cells in the pituitary are somatotrophs. One chain alpha-helical nonglicolized polipeptid. 191 aminoasits and 2 intramolecular disulfide connection. 22kDa molecular weight 75% 20kDa 10-25% N-asetile and desamine forms or oligomers
  • Slide 25
  • Human growth hormone (hGH) Nonpulsatile secretion in infants GH secretion pulses and amplitude is decreased and tonic secretion disappears untill puberty. In puberty GH secretion amplitude increase (gonodal steroids effect on GHRH) Secretion of GH decrease with aging but is secreted in whole life.
  • Slide 26
  • Growth homone effects ncrease protein synthesis, positive nitrogen balance Lypolysis in fat tissue Decrease carbonhydrate utilization and insulin sensivity increase blood glucose
  • Slide 27
  • GH effect Binded to GH-binding protein (GHBP) (at least 50%) GHBP,is the extracelluar part of GH-R.
  • Slide 28
  • IGF-1 levels are decreased GH deficiency HypothyroidismMalnutrition Chronic diseases
  • Slide 29
  • IGFs bind to binding proteins (IGFBP). t Transport to target tissue Modifiy the relation between IGF and its rec 6 different IGFBP are cloned IGFBP-3 90%, depends on IGF-1
  • Slide 30
  • IGF-1 rec Structurally similiar to insulin rec (2 alfa,2beta subunits)
  • Slide 31
  • GH IGFs (somatomedins) Similar to proinsulin Effect on extracellular growth is mediated by IGF-1 (70aa polipeptid)
  • Slide 32
  • Factors that increase growth hormone secretion GHRH Arginine, leucin Alpha adrenergic agonistler (alfa 2 adrenargic) Beta adrenergic antagonists Dopamine, acetylcholine HypoglycemiaSleepingExercise
  • Slide 33
  • Factors that decrease growth hormone secretion HyperglycemiaObesity ncrease in free fatty acids Glucocorticoid excess Hypothyroidism ncrease in adrenargic tonus Psychosocial deprivition
  • Slide 34
  • Growth hormoned stimulation tests Pharmacologic stimulation GH response < 5 ng/ml complete GH def GH response 5-10 ng/ml partial GH def GH response >10ng/ml normal At least no response to 2 stimuli
  • Slide 35
  • Growth hormone deficiency Congenital growth hormone deficiency incidence1:4000 -1:10,000 live birth Stunded growth
  • Slide 36
  • Growth hormone deficiency Mild deficiency Symptoms after the first 6 months of life. After the disappearence of maternal hormones Normal birth length Growth velocity decrease gradually Bone age delays Periabdominal fat tissue increase Less muscle mass Teeth development is delayed Thin hair, delay in nail growing
  • Slide 37
  • Growth hormone deficiency Severe deficiency Newborn period Hypoglycemia Conjugated hyperbilirubinemia Micropenis (multiple pituitary hormone deficiency)
  • Slide 38
  • Slide 39
  • Slide 40
  • Treatment Konstitutional growth and pubertal delay Boys : Testesterone enantate 50-100mg/ months IM for 3-4 months Girls: Ethynylestradiol 10-20g/ day for 3-4 months GH deficiency GH 0,2mg/kg/ week, in the management of pediatric endocrinologist