《儿科学》课程教学课件（PPT讲稿）16 Growth Hormone Deficiency

Growth hormone deficiency(pituitary dwarfism)

Growth hormone deficiency (pituitary dwarfism)

O utlin eGHD occurs when anterior pituitary synthetize andsecrete growth hormone insufficient partly orcompletely, lead to the disorders of growth anddevelopment , the length / heights are less than 3rdpercentile or 2SD below the mean normalrange.Disease incidence:2/10000~2.5/10000

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 less than 3rd percentile or 2SDbelow the mean normalrange. Disease incidence: 2/10000~2.5/10000 O u tline

Y00000XDBooooooohuman GH isa protein with 19l amino acides,single chain, molecular wieght: 22KD

h u m a n G H is a protein with 191 amino acides, single chain, molecular wieght: 22KD

Physiologic functionBasic function:GHpromotes anabolic metabolism:Promoting protein synthesis, stimulating hepaticglycogen decompose and lipoclasis-muscles, tissue, organ cells proliferation1childen growth and development

Basic function: GH promotes anabolicmetabolism: Promoting protein synthesis, stimulating hepatic glycogen decompose and lipoclasis ↓ muscles, tissue, organ cells proliferation ↓ childen growth and development Physiologic function

Physiologic function: Specialfunction :GH promotes skeleton growing:Promote epiphyseal cartilage cellsproliferating, and bony matrix deposition1epiphysealplate thickening1bone longitudinalgrowing, height increasing

Physiologic function • Special function： G H promotes skeleton growing: Pr o mote epiphyseal cartilage cells proliferating, and bony matrix deposition ↓ epiphysealplate thickening ↓ bone longitudinal growing, height increasing

Regulationof growth hormone secretionHypothalamusgrowth hormone releasinggrowth hormone releaseinginhibiting hormonehormone(-)):（）(GHRH)(+)(GHIH)--Pituitaryrowth hormone( +) promotion()inhibitionLiver - negative feedbackpositive feedbackIGF1

（ +）promotion （一）inhibition negative feedback positive feedback Regulation of growth hormone secretion Hypothalamus Pituitary Liver growth hormone releasing growth hormone releaseing hormone inhibiting hormone （一）（一） （GHRH） ( +） （－） (GHIH） IGF1 growth hormone （+）

Secretion modesquare pulse-like, day-night rhythm, reachingthe peak insleep II or IV stageInfluence factorsleep,motion,starvation,drugs(includinginsuline, L-dopa, arginine,clonidine mayincrease the GHsecretion

Secretion mode square pulse-like, day-night rhythm, reaching the peak in sleep Ⅲ or Ⅳ stage Influence factor sleep,motion,starvation,drugs (including insuline, L-dopa, arginine, clonidine may increase the GHsecretion

Etiology1. Idiopathic hypopituitarism: main causeNeurotransmitter-neurohormone functionpathway abnormalitydopamine, 5-HT(hydroxytryptamine),monamine, acetylcholine(乙烯胆碱）↓hypothalamus secrete GHRHI+anterior pituitary secrete GHI

1. Idiopathichypopituitarism: main cause Neurotransmitter-neurohormone function pathway abnormality dopamine, 5-HT(hydroxytryptamine), monamine, acetylcholine(乙烯胆碱） ↓ ↓ hypothalamus secrete GHRH↓ ↓ anterior pituitary secrete GH↓ Etiology

Etiology2. Secondary hypopituitarismCraniocerebral tumor, infection, trauma, brainradiation injury3. Hereditary pituitary maldevelopmentrare, 5% , familialGHl gene defect4. Other cause-temporality growth retardation:thymo-denudationdwarfism情感剥脱性矮小-GHreceptor deficiency : AfricanPygmy people

2. Secondary hypopituitarism Craniocerebral tumor, infection, trauma, brain radiation injury 3. Hereditary pituitary maldevelopment rare, 5% , familial GH1 genedefect 4. Other cause －temporality growth retardation: thymo-denudation dwarfism 情感剥脱性矮小 －GH receptor deficiency：African Pygmypeople Etiology

AfricanPygmypeople

African Pygmypeople