身材矮小的鉴别诊断和处理

2002202124(200092)1hGHmRMA(shortstature),2,,(GH)GH,,-GH-IGFGHGH,191,,GH15min30minGH5,17q222455’3’hGH-NhCS-LhCS-AhGH-VhCS-B-3’5DNA,54hGH-N,4,(1)GH3GH,GH,12,GH60g/L,30130g/L,GH,30g/L,GH,,,,5g/L1GH,,2468GHGH,,:1.:,GH,,,,,;,1.StrachanDP.Theepidemiologyofchildhoodasthma.JAllergyClinImmunol,1999,54(Suppl49)7-11.2.,..,2001,24(3)181-183.3.RichardGH,HomaYA,AllanBB,etal.Theassociationbetweenrespiratoryvirusesandsymptomsin2weekoldinfantsathighriskforasthmaandallergy.JPediatr,2001,138(6)831-836.4.BjorkstemB,Theenvironmentalinfluenceonchildhoodasth2ma.JAllergyClinImmunol,1999,54(Suppl49)17-23.5.,,..,2001,24(5)295-297.6.HananiaNA,DavidWA,KestenS,etal.Factorsassociatedwithemergencydepartmentdependenceofpatientswithasthma.Chest,1997,111(2)290-295.7.RedingtonAE,HowarthPH.Airwaywallremodelinginasth2ma.Thorax,1997,52310-312.8.().,1998,36(12)747-751.9.,..1,,1999,230-410.10.,,..1,,200187-306.©1995-2005TsinghuaTongfangOpticalDiscCo.,Ltd.Allrightsreserved.1252002202(IGF1),,,,,GHGH(1-25D)2.:GH,GH,GHIGF,IGF,GH,IGF3.:GH,GH,,30min2hGH,GH,GH,,GH4.:GH,GHGH,GHGH,GH5.:GH,GH,,,--,6.:GHIGF1GH,70,12,690%IGF1,IGF1IGF1(IGFBPs),,IGF-GH-IGF-GH-IGF,2GH(GHRH)(SS)GHRHGH,mRNA,GHGHRH,,GHRHGHRHSSGHSS14,GHRH,,GH,,;GH(GHRPs),GH,GHRHGHGHRP-6GHRP,Hexarelin,GH,,,,,,,,,1,;,,,,,,,;,;;,1POUIFI(Pit-1)PROP-1GHRHGHRHGH-NGH(Laron)IGF-1IGF1(GH)IGF-1IGF1SHOX(PHOG)Leri-Weill,Turner,2-GH-IGFGH©1995-2005TsinghuaTongfangOpticalDiscCo.,Ltd.Allrightsreserved.2002202126,,,X,,,(FT3FT4TSH)(),IGH1IGFBP3GH-IGFLHRHHCG(2)GHGH,(pituitaryshortstature),-GH-IGF,:1.GH::GH(growthhormoneneurosecretorydysfunction,GH2ND);GH:;GH:GH2.GH:GH;GH;GH;GH;GH3.IGF1:IGF1;IGF1;IGF14.IGF1:IGF1;IGF1;IGF1;IGF1;IGF1GH,,5,23,,,,,,,,,,,5cm,2cm3cm,-2SD2SD,,,,,;ACTH,;,,2.5kg(intrauterinegrowthretardation,IU2GR):IUGR,,(Russell-Silver)IUGR,,;,,,,,IUGRGH,IUGR,GH10g/mlRussell-Silver,,,:,,,;;,,,,;,;,,,,Wilm’s(constitutionaldelayofgrowthandpuberty),1/3,,GH,,,,,,(101191023),1618,1416,,,2GHLaronD()(Turner)21-©1995-2005TsinghuaTongfangOpticalDiscCo.,Ltd.Allrightsreserved.12720022023GH(min)4g/kg0.15mg/m2,0306090minGH6090-,GHRHL-10mg/kg0.5g/1.73m2,,6090,GHRH0.5g/kg,30g,30min,306090120min6090-,0.05U/kg,,15304560min1530,GH50%,20,(idiopathicshortstature),,,,GH(),,-2.2(0.6)SD,,,GH,,GH,GH(nutritionalgrowthretardation),,,/(),,GHD,,GHIGF1,IGF1,,(psychosocialshortstature),,,-GH-IGF,GH,GH,,;,,,IGF1ACTH,X,,,,,X,GH,2SD,GHGH,,,,GH,(3)8,GH5g/L,GH;GH(5.19.9)g/LGH;GH10g/LIGF1GHIGF1,GH-IGF-IGF1GH,IGF1,rhGHIGF1,IGF1,GH,IGF1,GH3(IGFBP3)IG2FBP3IGF1,95%IGF1IGF2BP3,,IGF1GH,GHGHIGFBP3(MRI)MRI,,GH,27GH,,96%,44%,37%,20%,,TurnerGHGH80DNArhGH,GHGHGH-2SD:©1995-2005TsinghuaTongfangOpticalDiscCo.,Ltd.Allrightsreserved.2002202128(67)(),,,,,50%,70%80%,,(24),,(video-EEG),,,,,CTMRI,SPECT,PET,,(2001919);;;;GH,;;,(0.5-0.7)U/kg,0.1U/kg612,,,0.05U/kg,0.2U/(kg24h)GH20,4cm/9.2cm13.7cm/,12cmGH,2h4h,20h40h0.5ml1.0ml,,,,GHGH,,,,,,,,GH1.:GH,,,23,,12.:,1%4%3.(T4):T4,,7,T445%,4.:,rhGH,rhGH,TurnerrhGH,1.0U/kg,,14,2.5cmGHGH,GH,,,,,,1.BinderG,SchwarzeCP,RankeMB.Identificationofshortsta-turebySHOXdefectsandtherapeuticeffectofrecombinanthumangrowthhormone.JClinEndocrinolMetab,2000,85245-249.2.LucaFD,ArgenteJ,CavalloL,etal.Managementofpubertyinconstitutionaldelayofgrowthandpuberty.JPediatrEn2docrinolMetab,2001,14953-957.3.PasquinoAM,AlbaneseA,BozzolaM,etal.Idiopathicshortsta-ture.JPediatrEndocrinolMetab,2001,14967-974.4.RosenfeldRG.Disordersofgrowthhormoneandinsulin-likegrowthfactorsecretionandaction.In:MarkAS,ed.PediatricEndocrinology.Philadelphia:W.B.SaundersCompany,1996117-170.5.ShaletSM,ToogoodA,RahimA,etal.Thediagnosisofgrow-thhormonedeficiencyinchildrenandadult.EndocrineReviews,1998;19(2)202-223.6...,1998,5281.©1995-2005TsinghuaTongfangOpticalDiscCo.,Ltd.Allrightsreserved.