16!"#$%&'!"# ()*+, 387 $%&'!"(multiplepituitaryhormone deficiency,mphd)# 375/1 10 5, V 2/1 10 5[1] $%&'!", #V,# GH! ",T$%&'!"#9C 2+&'A( A-, (growthhor

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1 : (2016) Basic& ClinicalMedicine March2016 Vol.36 No.3 临床园地 16!"#$% &'!"# ()*+,,,,,,,,!"#,$%&,' (-./ / , ) : 78!"(GHD)#$%&'!"9(MPHD)#: (GH) ()*!" ;+, 16!"#$%&'!"# #$ <=>?@ 16 MPHD#,A-BCDE9 9 D DE 13FDGHE 6 IJ KLMNOO 10 (110±35)P,QRST4U LDopaGHVW 5 GHXY(014±017)ng/mL,GH((011±002)IU/kg (T IGF1Z[QR \] #% ^_`ab &'cjt, GH!"# MPHDA!"T, GH( QR,3V &':$%&'!";&'E; ;( :R58 ()*:A Thecharacteristicsandefectsofgrowthhormonetreatmentof16 multiplepituitaryhormonedeficiencypatientswithgrowthhormonedeficiency HOUJiatong,YANGHongbo,WANGLinjie,CHENShi,XUKe,WANGShuchang,LIUZhihui, GONGFengying,ZHUHuijuan,PANHui (Dept.ofEndocrinology,KeyLaboratoryofEndocrinologyofMinistryofHealth,PUMCHospital,CAMS&PUMC Beijing100730,China) Abstract:Objective Tostudytheclinicalfeatureandtherapeuticefectsof16patientsdiagnosedwithmultiple pituitaryhormonedeficiencywithgrowthhormonedeficiency(ghd).methods Clinicaldataof16caseswereret rospectivelystudied.results 16MPHDwithGHDpatientswereincluded,containing9hypothyroidism,13hypog onadismand6acthdeficiency.tenofthemhadbreechorfootpresentation.themeanboneagewas(110± 35)years,delayedfromchronologicalage,peakGHofLDopaprovocationtestwas(014±017)ng/mL.GH dosewas(011±002)iu/kg.afterghreplacementtherapy,theserumconcentrationofigf1andgrowthveloc ityincreased.conclusions GHreplacementtherapyiscrucialonceafteroneisdiagnosedofMPHDexcludingthe hypothalamusandpituitarylesions.otherpituitaryhormonesmustbereplacedsuficientlyformphd patients. Thereisnosevereadverseevent. Keywords:multiplepituitaryhormonedeficiency;hypopituitary;growthhormone;replacementtherapy : : (correspondingauthor):panhui @163.com;huijuanzhu@hotmail.com

2 16!"#$%&'!"# ()*+, 387 $%&'!"(multiplepituitaryhormone deficiency,mphd)# 375/1 10 5, V 2/1 10 5[1] $%&'!", #V,# GH! ",T$%&'!"#9C 2+&'A( A-, (growthhormone,gh) (foli clestimulatinghormone,fsh)' (lutein izinghormone,lh)!"# [2],FDG H (adrenocorticotrophichormone,acth) BCD (thyroidstimulatinghormone,tsh)#! " `ab&'cj # MPHD, GH, (,cjh]3v, V$%&'!"9(, GH BCD : # (! " 9 (GH defi ciency,ghd)#, GH (,GH:,:' + [3] GHD# MPHD (,<= V MPHD GH()*: 78,@ ( 1 +,-!" /# MPHD#, 1:F >?: GHD#$%&'!", ',?V,!"# GH $%&(,&' MRIcJ'# GH [4] :1) ;2) Z [ < 7cm/(3P:`);<5cm/(3P ~"); <6cm/();3)ST4U;4) ( VW5XY <10ng/L;5))*+, -. 1(IGF1)/0 1` A#:BCDE:BC D (FT4),2BD345(FT3) TSH6 FD# [5] :7F 8~98,)*-GH9 301μg/dL( 5~25μg/dL),:#3,;:GH9!"# * ACTHY,; VFDGHE D#:)*,LH FSH 12./!" < =>?@ 'H VABC IGF1 BCD D FD# &' MRI:( GHVW5D* VABC: Tanner+,E GP(GreulichandPyle) MRI( 1) 'H3F (rhgh)# (,("GH?IJKL,MNF3 (KLO rhgh(", A!",BCD # (T 3 6 1,PQK8BRF 'H,S)TUE E VW5X YH ) ) BCDE+, -. 1 (IGF1) ( 6 12T X,RF GP+, 13 E SPSS170Z+, [ #\[+, tt5(]+^) Kolmogor ovsmirnovt5(_]+^),"t E`3a+,;[`Eb ct5 ]+^`` ±a(x±s)#,_]+^`:-j`[25+j`, 75+J`] 2 #$ <=>?@ / MPHD#,A- 15(9375%), 1 (625%), (191±47)P, (110±35)P,A-BCDE9 9, D DE 13,FDGH E 6 <-3SD,(505±

3 388 Basic&ClinicalMedicine (3) MPHDmultiplepituitaryhormonedeficiency 1 MPHD56789: Fig1 TheprogresofinclusionandexclusionofMPHD 20)cm,'H(321±041)kg,O 10,V 625%(10 /16),A-KL M 7(70%),IJ 3(30%) &' MRI GH("Z[(375± 121)cm/ 22 ;<=>? IGF1(263±06)ng/mL( Y 111~ 551ng/mL) [6] LDopaGH V W 5 GH X Y (014±017)ng/mL ) (bloodglucose,bg) (480±061)mmol/L,Na(141±3)mmol/L,K (448±035)mmol/L,Ca(242±007)mmol/L 5 (UA)(258±61)μmol/L, (Cr)(506± 96)μmol/L,LH 001[000,022]mIU/mL,FSH 095[034,266]mIU/mL BCD F D/!",BCD () 375μg/d,T FT3 (278±044)pg/mL,FT4(092±025)pg/mL, TSH094[062,246]μIU/mL,# 5mg/d,T ACTH(2136±891)pg/mL 16#TMNJKLO T,".(01-015)IU/(kg d), rhgh(,<=-(011± 001)IU/kg )* IGF1:( 3T (146± 136)ng/mL,( 6T(171±132)ng/mL,( 12T(245±158)ng/mL /`3 #a+,t,igf1("qr\](p< 005)( 2) Z[:3(1142±373)cm/,6(1055±273)cm/,12(1007± 237)cm/,("QRF( 3) ( 1T, -267SD,"F (P<005), P<001comparedwithbaseline; # P<005compared with3m; Δ P<005comparedwith6m 2 CDE IGF1#$ Fig2 TheIGF1levelatbaselineandfolowups

4 16!"#$%&'!"# ()*+, 389 P<001, P<005, P<001comparedwith baseline 3 CDEF Fig3 Thegrowthrateatbaselineandfolowups 24 GHIJ <=-,16 1,) Y(512±047)mmol/L, 1BCD E, 25μg/d,BCDE, ( T ' FT3 (353±083)pg/mL,FT4 (106±029)pg/mL,TSH(234±177)μIU/mL, "T\[L \# `,V:\ V 3 K% &'F'3#+D', VV3 <=Q &'# 50%" +, GH FSH LH!"#,ACTH TSH#!",<=D*A MPHD$V,<=D* MPHD +_,"& '" \ + #. Prop1 Pit1 Hesx1 [7],T+IJ! <= 16-, O 10 GHD,#( rhgh ( [8]!"# MPHD rhgh(t9c]3,\]v V# (# 3 MRI#T,:^_`ab &'/,AcJ V MPHD GHD#(9(,GH BCD : #( YL#,BCD ("L #GH (,,:VFD <=-,(T#Z[ IGF1# Y("QR\],\[a',,GH ( IGF1\] [9] '( ",4 MPHD!",,/!"# T, 8# GH(#/, GH# #F$ # T^_ IGF1+#,<= \]&'1V# "$- $.?#`R, GH (\]# [10],# MRI# TQ \]&'1V# GH(#!:" MPHD # )#(,#!",T #(,$#8,)* QR LM: [1]FernandezRodriguezE,LopezRatonM,AndujarP,etal. Epidemiology,mortalityrateandsurvivalinahomogeneous populationofhypopituitarypatients.[j] ClinEndocrinol (Oxf),2013,78: [2]KaracaZ,TanriverdiF,UnluhizarciK,etal.Pregnancy andpituitarydisorders.[j]eurjendocrinol,2010,162:

5 390 Basic&ClinicalMedicine (3) [3]LosaM,BeckPeccozP,AimaretiG,etal.Characteristics andoutcomesofitaliapatientsfromtheobservational,mul ticentre,hypopituitarycontrolandcomplicationsstudy(hy poccs)accordingtotertilesofgrowthhormonepeakcon centrationfolowingstimulationtestingatstudyentry.[j] ClinEndocrinol(Oxf),2015,83: [4]Poyrazog lu 爦,Akg ayt,arslanog lui,etal.curentprac ticeindiagnosisandtreatmentofgrowthhormonedeficiency inchildhood:asurveyfromturkey.[j]jclinrespediatr Endocrinol,2015,7:3744. [5]ConsensusstatementformanagementofAddisonsdisease [J].JInternMed,2014,275: [6]%&&,0,',.)*+,.1+,.D3#Y<= [J]./(,2009,27: [7]DataniMT.Growthhormonedeficiencyandcombinedpitu itaryhormonedeficiency:doesthegenotypemater?[j]. ClinEndocrinol(Oxf),2005,63: [8]KawaMP,StecewiczI,PiecykK,etal.Efectsofgrowth hormone therapeutic supplementation on hematopoietic stem/progenitorcelsinchildrenwithgrowthhormonedefi ciency:focusonproliferationanddiferentiationcapabilities [J].Endocrine,2015,50: [9]AppelmanDijkstraNM,ClaesenKMJA,RoelfsemaF,et al.longterm efectsofrecombinanthumangh replace mentinadultswithghdeficiency:asystematicreview[j]. EurJEndocrinol,2013,169:R114. [10]BurmanP,MatsonAF,JohannsonG,etal.Deathsa mongadultpatientswithhypopituitarism:hypocortisolism duringacutestres,anddenovomalignantbraintumors contributetoanincreasedmortality[j].jclinendocrinol Metab,2013,98: 新闻点击 NOPQ 据美国 WebMD 医学新闻网 ( ) 报道, 花太多时间坐着会增加女性癌症的概率, 但对男性来说似乎没有类似的效果主导美国癌症协会癌症预防研究的 AlpaPatel 博士表示, 花较多闲暇时间坐着与女性整体癌症风险较高有关, 尤其是多发性骨髓瘤乳腺癌以及卵巢癌等 ; 但坐着的时间与男性癌症风险没有关联性这篇研究针对超过名男女从 1992 年追踪至 2009 年, 参与者开始时都没有癌症在这段期间, 近名参与者罹患癌症专家们因为坐着似乎会增加女性癌症概率而感到困惑, 研究团队指出, 久坐只代表每天运动比较少这篇研究并未设计证明因果关系, 然而, 因为美国人花很多时间坐着, 即使坐着与增加癌症风险稍微有关, 对公共卫生来说都有重大的涵义这篇研究结果刊载最近一期的癌症流行病学生物标记与预防 (CancerEpidemiology,Biomarkers&Prevention) RS XTUVWXFY 据美国 WebMD 医学新闻网 ( ) 报道, 最新研究声称, 定期做乳房 X 线摄影筛检乳腺癌可能造成普遍过度诊断, 让一些女性治疗不会造成生病或死亡的肿瘤研究人员在分析了美国卫生官员搜集的县级数据后发现, 医生们倾向于在拍摄乳房 X 线摄影的地方发现许多小肿瘤与癌前病灶然而, 哈佛大学得茅斯学院的研究人员指出, 乳房 X 线摄影比例增加的那些地区, 乳腺癌死亡率似乎没有下降研究人员表示, 最简单的解释就是普遍过度诊断增加了小癌症的发生率, 而没有改变死亡率但癌症专家表示, 这些研究结果不应该让女性认为乳房 X 线摄影是不需要或有潜在危害的之前的研究显示, 乳房 X 线摄影让 40 岁以上的女性乳腺癌死亡率至少降低 20% 然而, 波士顿达那法柏癌症机构的乳房肿瘤计划资深内科医生 HaroldBurstein 博士表示, 这篇最新研究结果显示, 有些妇女或许可以不要这么频繁地做筛检这篇研究提醒医生与妇女们, 在决定每年做一次乳房 X 线摄影之前, 值得花更长的时间讨论能不能做到正确诊断该研究结果刊载在 JAMA 内科医学杂志

Microsoft Word - Z1I07A0-17.doc

Microsoft Word - Z1I07A0-17.doc 國文科文 / 林士敦老師 1 前言一 95 課綱發表後,40 篇選文對大學入學考試產生巨大影響這點從 98 99 年兩份試卷中可以看出不言可喻, 熟讀 40 篇是必然的結論二在測驗目標中, 大考中心已經明示指考所要檢測的內容與能力, 因此, 準