甲狀腺機能亢進(jìn)癥

Hyperthyroidism中山大學(xué)附屬第一醫(yī)院內(nèi)分泌科肖海鵬RegulationofThyroidHormonesFigure4-3.Basicelementsintheregulationofthyroidfunction.TRHisanecessarytonicstimulustoTSHsynthesisandrelease.TRHsynthesisisregulateddirectlybythyroidhormones.T4isthepredominantsecretoryproductofthethyroidgland,withperipheraldeiodinationofT4toT3intheliverandkidneysupplyingroughly80%ofthecirculatingT3.BothcirculatingT3andT4directlyinhibitTSHsynthesisandreleaseindependently;T4viaitsrapidconversiontoT3.SRIH=somatostatin.非甲狀腺功能亢進(jìn)類型⒈亞急性肉芽腫性甲狀腺炎（亞急性甲狀腺炎）⒉亞急性淋巴細(xì)胞性甲狀腺炎（無痛性甲狀腺炎）⒊慢性淋巴細(xì)胞性甲狀腺炎（橋本甲狀腺炎、萎縮性甲狀腺炎）⒋產(chǎn)后甲狀腺炎（PPT）⒌外源甲狀腺激素替代⒍異位甲狀腺激素產(chǎn)生（卵巢甲狀腺腫等）Graves病病因與發(fā)病機制

免疫功能異常體液免疫TRAb:TSAbTSBAbTGITPOAbTgAbNIS眶后成纖維細(xì)胞抗體眼外肌自身抗體細(xì)胞免疫病因與發(fā)病機制

遺傳因素家族史HLA相關(guān)感染因素精神因素Figure7-15PossiblesequenceandclinicaloutcomeinAITD,indicatingtheinterrelationofenvirenmentalandgeneticfactors,anddependenceoftheclinicalpictureonthetypeofimmuneresponse.甲狀腺毒癥心血管系統(tǒng)消化系統(tǒng)肌肉骨骼系統(tǒng)甲亢性周期性麻痹甲亢性肌病伴重癥肌無力皮膚造血系統(tǒng)生殖系統(tǒng)Thyroidacropachy眼征單純性突眼

輕度突眼(<18mm)Stellwag征瞬目減少凝視上瞼攣縮眼裂增寬(Darymple征)VonGraefe征Joffroy征Mobius征浸潤性突眼

PalpebraledemaWideningofpalpepralFissuresLidretractionParalysisofrightRectusmuscleParalysisofupwardGazeontherightFigure12-3.EndstageinsevereinvolvementofextraocularmusclesinophthalmopathyGraves病眼征的分級標(biāo)準(zhǔn)（美國甲狀腺學(xué)會ATA)級別眼部表現(xiàn)0無癥狀和體征無癥狀，體征有上瞼攣縮、

Stellwag征、vonGraefe征等2有癥狀和體征，軟組織受累3突眼（﹥18mm)

眼外肌受累角膜受累視力喪失（視神經(jīng)受累）

甲狀腺危象誘因:感染手術(shù)放射碘治療創(chuàng)傷嚴(yán)重藥物反應(yīng)心梗臨床表現(xiàn)高熱（39以上）心率140次/分以上房顫或房撲大汗淋漓厭食惡心嘔吐腹瀉煩躁不安、休克、譫妄、昏迷Figure10-9.Congestiveheartfailureinducedinanotherwisehealthyyoungwoman(a),whichreceded(b),andreturnedtonormal(c),duringandaftertherapy.淡漠型甲亢T3或T4型甲亢亞臨床型甲亢妊娠期甲亢脛前黏液性水腫甲狀腺功能正常型Graves眼病脛前粘液性水腫Figure12-6.Acaseofseverepretibialmyxedemashowingthecoarsened,nodular,infiltrated,pigmentedlesionsonthelowerextremities.實驗室及其他檢查

甲狀腺功能檢查

TT3、TT4ReverseT3FT3、FT4TSH

123I或131I攝取率TRH興奮試驗T3或T4抑制試驗

影響TBG的因素TBG增加TBG降低

妊娠雄激素雌激素糖皮質(zhì)激素急性肝炎或慢活肝低蛋白血癥藥物先天因素先天因素Figure3c-1.Pathwaysofthyroidhormonemetabolism.Figure6-8.TheeffectofserumTSHassaysensitivityonthediscriminationofeuthyroidsubject(Euth)fromthosewiththyrotoxicosis(Toxic).(FromC.Spencer,ClinicalDiagnostics,EastmanKodakCo.,1992).明確病因的檢查TSI或TRAbTPOAbThyroidScanFigure13-1.Hotnoduleinrightlobeofthyroid.Notethatuptakeofradioactivityinthecontralaterallobeissuppressed.Figure18-3.Scintiscansofthyroid.Thescanontheleftisnormal.

Atypical

scanofa"cold"thyroidnodulefailingtoaccumulateiodideisotopeisshownontheright.

Incidentally,apyramidallobeisalsoseenonthisscan,whichmightsuggestthepresenceofHashimoto'sThyroiditis.Figure6-6.ThyroidScans.Normalthyroidimagedwith123I.Coldnoduleintherightlobeimagedby99mTc.Elderlywomanwithobviousmultinodulargoiterandthecorrespondingradioiodidescanontheright.Figure17-5.(A)Crosssectionofmultinodulargoiter.(B)Grossradioautographofthethyroidinparta.Observethevariationin131Iuptakeindifferentareas.影像學(xué)檢查B超CTMRI(b)Inthistransverseviewtheenlargedmusclesareseen(appearingdarkagainstthelightfatsignal)andtheexophthalmosisapparrent.Figure10-5.(a)ThisMRIimagefromapatientwithGraves'ophthalmopathyprovidesacoronalviewoftheeyes.

Inthisdepictionthemusclesappearwhite,andareenormouslyenlarged,especiallyinthelefteye.診斷高代謝癥狀和體征甲狀腺腫大伴或不伴血管雜音FT4增高、TSH降低浸潤性突眼脛前粘液性水腫TRAb(TSI)鑒別診斷甲亢病因鑒別131IUptakeandscanB超單純性甲狀腺腫嗜鉻細(xì)胞瘤神經(jīng)官能癥更年期綜合征抑郁癥其他：結(jié)核、腫瘤、糖尿病、慢性結(jié)腸炎、心臟病、眶內(nèi)腫瘤甲亢的治療一般治療甲亢的治療抗甲狀腺藥物放射碘(RAI)治療手術(shù)治療藥物治療(ATD)種類與機理

硫脲類：甲硫氧嘧啶(methylthiouracil,MTU)丙硫氧嘧啶(propylthiouracil,PTU)咪唑類：甲硫咪唑(methimazole,MM他巴唑)卡比馬唑(carbimazole,CMZ甲亢平)機理:抑制甲狀腺激素的合成免疫抑制外周T4向T3轉(zhuǎn)化

適應(yīng)證病情輕甲狀腺輕中度腫大青少年（20歲以下），孕婦，年邁體弱有嚴(yán)重肝腎疾病不能手術(shù)術(shù)前準(zhǔn)備，術(shù)后復(fù)發(fā)131I治療前后輔助治療劑量與療程初治期:MTU/PTU300mg-450mg/dMMI/CMZ30mg–40mg/d甲亢癥狀緩解，T3、T4恢復(fù)正常減量期:每2–4周減一次，每次MTU/PTU50mg-100mgMMI/CMZ5mg–10mg甲亢癥狀完全消失，體征明顯好轉(zhuǎn)維持期:MTU/PTU50mg-100mg/dMMI/CMZ5mg–10mg/d(18個月)

不良反應(yīng)

粒細(xì)胞減少或缺乏

藥疹

膽汁淤積性黃疸、血管神經(jīng)性水腫、中毒性肝炎停藥指征癥狀消失、甲狀腺腫減輕或消失療程18個月T3、T4、FT3、FT4、TSH均正常TSI轉(zhuǎn)陰T3抑制試驗恢復(fù)正常其他藥物治療

碘劑術(shù)前準(zhǔn)備甲亢危象B-阻斷劑131I適應(yīng)證中度甲亢年齡25歲以上甲亢藥物過敏、長期無效、或治療后復(fù)發(fā)心、肝、腎疾病不宜手術(shù)、術(shù)后復(fù)發(fā)、或不愿手術(shù)131I禁忌證妊娠、哺乳婦年齡25歲以下嚴(yán)重心、肝、腎疾病或活動性結(jié)核白細(xì)胞低于3109/L，或中性粒細(xì)胞低于1.5109/L重癥浸潤性突眼甲狀腺危象甲狀腺不能吸碘者131I劑量與療程：ATD停藥3-5天,戒碘2~4W80μCi/g甲狀腺組織半年后仍未緩解，進(jìn)行第二次治療131I并發(fā)癥:甲減(一過性和永久性)甲狀腺炎(131I治療后7~10d)誘發(fā)甲亢危象突眼加重手術(shù)適應(yīng)證中重度甲亢長期服藥無效,不愿服藥,或停藥復(fù)發(fā)巨大甲狀腺,有壓迫癥狀胸骨后甲