七年制医学课件 儿科 19生长激素缺乏症

Growthhormonedeficiency生长激素缺乏症 pituitarydwarfism 垂体性侏儒 GHDoccurswhenanteriorpituitarysynthetizeandsecretegrowthhormoneinsufficientpartlyorcompletely leadtothedisordersofgrowthanddevelopment thelength heightsare3rdpercentilerankor2SDbelowthemeanDiseaseincidence2 10000 2 5 10000 Outline humanGHisaproteinwith191aminoacides singlechain molecularwieght 22KD Basicfunction GHcanpromoteanabolicmetabolism promoteproteinsynthesis stimulatehepaticglycogendecomposeandlipoclasis muscles tissue organcellsincreaseandproliferate childen sgrowthanddevelopment Specialfunction GHpromoteskeletongrow promoteepiphysealcartilagecellproliferate bonymatrixdeposite epiphysealplatethicken bonelongitudinalgrow bodyheightincrease Physiologicfunction Secretionmodesquarepulse like day nightrhythm reachthepeakinsleep or stageInfluencefactorsleep motion starvation drug insuline L dopa arginine clonidinehydrochloridummayincreasetheGHsecretion 1 Idiopathichypopituitarism maincauseneurotransmitter neurohormonefunctionpathwaydefect dopamine 5 HT monamine ACH hypothalamussecreteGHRH anteriorpituitarysecreteGH Etiopathogenisis 2 Secondaryhypopituitarismtumor infection craniocerebraltrauma brainradiationinjury3 Hereditagpituitarymaldevelopmentrare 5 familialGH1genedefect4 Othercausepneuma temporality thymo denudateddwarfismGHreceptordeficiency AfricanPygmypeople Etiopathogenisis AfricanPygmypeople Pathopoiesisfrequencydatastatistics 325GHDpatientsIdiopathic 254cases78 Secondary 62cases19 Heritage 9cases3 growthretardationmicrosomia growthvelocityslow 4cmperyear bodytyperegularity unusualfaciesimmatureexpression thefaceisshortandbroad skinexquisite subcutaneousfatplentifulintelligenceintelligenceage actualagedelayedpubertaspubertydelayedafter16yearsold Clinicalmanifestations 10 2 yr old 142cm 110cm 18yearold 10yearold 1 SerumGHdeterminationScreeningtest sleeptest3 yr oldresults GH 10ug LexcludeGHDGH 10ug Lfurtherformaltest Laboratoryfindings GHsecretionfunctionaltest Formaltest 确诊试验 ResulttwoormorepharmacologicprovocativetestpeaklevelofGHlessthan5ug L completeGHdeficiencypeaklevelofGHaround5 10ug L partialGHdeficiencypeaklevelofGHmorethan10ug L noGHdeficiency 2 Boneage BA BAdelayedmorethan2years3 OthertestSerumlevelofIGF1 0 5u mlHeadCT MRI excludeintracranialtumor encephalodysplasia diagnosticcriteriaheightbelow2SDor3rdpercentilerankgrowthratelessthan4cm yrnormalintelligenceBAdelayedmorethan2yearIntwopharmacologicprovocativetest post stimulusserumGHvalueslessthan10ug L Diagnosis Constitutionaldelayedadolescence 体质生长延迟 Familialdwarfism 家族性矮小 Intrauterinegrowthretardation 宫内发育迟缓 Turner ssyndrome 先天性卵巢发育不全 Differentialdiagnosis Constitutionaldelayedadolescencemoreboysthangirls heightnearthe3rdpercentile growthvelocity 4cm yr Boneagedelayed1 2year delayedpuberty parentsoftenhavethesamepasthistory Eventuallycanreachnormalstature Differentialdiagnosis Familialdwarfishnessparentsareshortofstature nearthe3rdpercentileforheightgrowthvelocity 4cm yrnormalintelligenceandsexdevelopmentnormalboneageGHlevelisnormal Differentialdiagnosis IntrauterinegrowthretardationSizeandeightatbirthbelowthe10thpercentilerankthanthemeangrowthvelocityisnearthenormallowerlimitunusualfacies thinandsmall symmetrical broadforehead micromandibleNormalboneageoralittledelayGHlevelisnormal Differentialdiagnosis Turner ssyndromeMicrosomia symmetrical nondevelopmentofsecondarysexlowhairline shortneck shieldchest farbreastdistance cubitusvalgusnormalintelligenceoralittledelaynormalboneageoralittledelaysonographyofcavitaspelvis uterustrabs like nondevelopmentofovarieschromosomalabnormality 45 Xor45 X 46 XX Differentialdiagnosis 130cm Turner ssyndrome 12yearsold GHtherapyDrugrecombinantsomatropin r hGH Dose0 1u kg dHqn6 7 weekCourseoftreatment tillosteoepiphysisagglutinatedCurativeeffect bestartedassoonaspossible tohavethegreatesteffectonmatureheight Treatment AnabolichormoneDrug testosteronederivate durabolin stanozolol Effect promoteproteinsynthesis promotegrow Disadvantage makeearlierfusionofepiphysis lowerthefinalheightIndic morethan10 yrold BAdelayedmorethan3yearsDose durabolin1mg kgim1 2 wk 10 ceaseforhalfyear ifBAstilldelayedmorethan3years continuethesecondcourseoftreatmentResult enhancethegrowthabout10cm yr finalheightisabout150 160c