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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  • Short stature Dr Olcay Evliyaolu
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  • 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
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  • 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
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  • Height should be given as standart deviation score (SDS) (z-score) SDS = measured height mean height according to gender and age / SDS
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  • Target height Girls (cm)= (mat height+pat height)-13 2 Boys (cm)=(mat height+pat height)-13 2
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  • TH + 8-10cm is what expected
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  • 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.
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  • 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
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  • Postnatal Proportional short stature No systemic disease Euthyroid Growth velocity < 4,5-5 cm/y Delay in bone age >2 years GH tests
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  • 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
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  • 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
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  • 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.
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  • Growth homone effects ncrease protein synthesis, positive nitrogen balance Lypolysis in fat tissue Decrease carbonhydrate utilization and insulin sensivity increase blood glucose
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  • GH effect Binded to GH-binding protein (GHBP) (at least 50%) GHBP,is the extracelluar part of GH-R.
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  • IGF-1 levels are decreased GH deficiency HypothyroidismMalnutrition Chronic diseases
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  • 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
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  • IGF-1 rec Structurally similiar to insulin rec (2 alfa,2beta subunits)
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  • GH IGFs (somatomedins) Similar to proinsulin Effect on extracellular growth is mediated by IGF-1 (70aa polipeptid)
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  • Factors that increase growth hormone secretion GHRH Arginine, leucin Alpha adrenergic agonistler (alfa 2 adrenargic) Beta adrenergic antagonists Dopamine, acetylcholine HypoglycemiaSleepingExercise
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  • Factors that decrease growth hormone secretion HyperglycemiaObesity ncrease in free fatty acids Glucocorticoid excess Hypothyroidism ncrease in adrenargic tonus Psychosocial deprivition
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  • 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
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  • Growth hormone deficiency Congenital growth hormone deficiency incidence1:4000 -1:10,000 live birth Stunded growth
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  • 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
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  • Growth hormone deficiency Severe deficiency Newborn period Hypoglycemia Conjugated hyperbilirubinemia Micropenis (multiple pituitary hormone deficiency)
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  • 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