七年制医学ppt课件 儿科 19生长激素缺乏症.pptVIP

Growth hormone deficiency 生长激素缺乏症 (pituitary dwarfism) 垂体性侏儒;GHD occurs when anterior pituitary synthetize and secrete growth hormone insufficient partly or completely, lead to the disorders of growth and development，the length / heights are 3rd percentile rank or 2SD below the mean Disease incidence 2/10000~2.5/10000 ;human GH is a protein with 191 amino acides, single chain, molecular wieght: 22KD;Basic function: GH can promote anabolic metabolism: promote protein synthesis，stimulate hepatic glycogen decompose and lipoclasis →muscles, tissue, organ cells increase and proliferate→childen’s growth and development. Special function： GH promote skeleton grow: promote epiphyseal cartilage cell proliferate, bony matrix deposite → epiphyseal plate thicken→bone longitudinal grow→body height increase.;Regulation of growth hormone secretion;Secretion mode square pulse-like, day-night rhythm, reach the peak in sleep Ⅲ or Ⅳ stage Influence factor sleep, motion, starvation, drug（insuline, L-dopa, arginine, clonidine hydrochloridum may increase the GH secretion; 1. Idiopathic hypopituitarism: main cause neurotransmitter-neurohormone function pathway defect　　　　　 　　　（dopamine, 5-HT, monamine, ACH）↓ 　　　 ↓ 　 hypothalamus secrete GHRH↓ 　　　 ↓ 　　 anterior pituitary secrete GH↓;2. Secondary hypopituitarism tumor, infection, craniocerebral trauma, brain radiation injury 3.Hereditag pituitary maldevelopment rare, 5% , familial GH1 gene defect 4. Other cause pneuma (temporality): thymo-denudated dwarfism GH receptor deficiency：African Pygmy people;African Pygmy people;Pathopoiesis frequency data statistics: 325 GHD patients Idiopathic : 254 cases 78％ Secondary : 62 cases 19％ Heritage: 9 cases 3％;;10.2-yr-old;18 year old;10 year old; 1. Serum GH determination Sc