SISTIM ENDOKRIN
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Transcript of SISTIM ENDOKRIN
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SISTIM ENDOKRINDr. Hj. RESMI KARTINI, MS
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Homeostasis seluler diatur :Sistim saraf Sistim endokrin terutama hipotalamus ( pituitari )Dan sel neuroendokrin APUD
HormonKelenjar endokrin target organinteraksi melalui reseptor
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Reseptor - reseptorSurface membrane receptorsHormon polipeptide ( pituitary )KatekolaminCytoplasmic reseptorSteroidNukleusIntranuclear reseptorThyroid hormonSitoplasma
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HIPOTALAMUS
KONTROL
KELENJAR ENDOKRIN PERIFERFEED BACK MECHANISMPITUITARY HORMON STIMULATORY INHIBITORY
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Pituitary gland
ANT:adenohipofisis Rathkes PouchPost : Neuro Hipofisis Floor Ventr IIIPost - Vasopresin ( ADH ) -OxytocinAnt -Asidofil somatrroph GH mammotroph PrL -Basofil Corticotroph Proopiomesamocortin acth,msh, endorfin,lipotropin Tirotrof TSH Glikoprotein Gonadotrop: FSH,LH -Khromofob
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PATOLOGIPrimer : Lobus Ant PostAdenohipofisis Hiper / Hipopituit 1.Tropic hormon / Hipopituitary : 75 % destruksi -tumor non fungsional -Infeksi -Hipotal (jarang)
2.Local effect - rare - Sela tursika besar --- Xray,CT,MRI Expansile lesion ( neoplasma )
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- Defek Expanding dis.pit Chiasma opt / N. II Bitemporal hemianopsia visual impairment- Sangat jarang Tek intrakranial( Headache, nausea, vomitus Neoplasma )
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HiperpituitariAdenomaKarsinoma (Hipotalamus Stimulasi >> Pituiti)----- jarangAdenoma MonoclonalSingle hormon Manifestasi klinikPlurihormonalPrimitif / Pluri potent cells
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Morfologi
Mikro adenoma ( < 10 mm )Mikro adenoma ( > 10 mm )Mikro adenoma : 40 % unselected autopsi. single ,multipel multiple foci hiperplMakro adenoma : Ekpansi dpt erosi tulangSella enkapsul poorly diliputi retikulin chiasma optikum syaraf kranialDasar otak,sinus cavernosus, tlg sfenoid
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Mikroskopis :
Uniform sel poligonal tersusun sheet,cord,nests.Stroma vascularisasi bertambahNecrosis ischemik,psammoma bodiesHemoragik pituit apoplexyMitosis /pleomorfik ? Malignancy metastasis
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PITUITARY ADENOMA
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PITUITARY ADENOMA
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Somatotropic ADENOMASSomatotro. acidophilic macro adenoma GH Akromegali Gigantisme jarangHiperplasiaAkromegali Adult Megali :akral,Kepala, tgn, Kaki,jaw,lidah Soft tissue
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Gigantisme : ChildrenSel matur uniform --- granulatedSebagian pleomorfik asidofil1/3 BSM Prl bimorfusPrl granul pada sel neoplastik tidak terdapat pada darah
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ProlactinomasSparsely granulatedHiperplasia jarangHiperprolactinemi lesi hipotal drug impair dopaminergic trans mission (metildopa,reserpin) estrogen terapi Hipogonad laki 2 --- impoten,infertil peremp --- galactorrhea amenorrhea
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2/3 macro adenoma granulated acidophilic cells1/3 Micro adenomaKalsifikasiSel uniform sela tursika
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Corticotroph tumors
basophilik mikroadenoma ACTH ------ Hipersekresi kortisol adrenalHiperplasia / multiple micro adenoma cushings diseasePro opio melanocortincrookes hyaline changeskhromofobic less well defined cortisol excessSilent tumor besar induce local changes
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Other functioning AdenomasGonadotroph tumor 6%Laki 2 FSH LH hipogonadPerempuan Gonadotropin sekresi - wl pun LH dapat dideteksi serum BesarMikros : Laki-laki Pleom, wanita :uniform small cell
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CARCINOMA
DiferensiasiDiagnosis Matastase : KGB Bone Hati dll
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Disorders associated with HypopituitarismGerm cell TKraniofaringiomaGliomaKetiga diatas lesions of hypothal,ant hipofis hipopituitary 75 % destruksi klinikTumor klinik : Diab-insipidus Growth Acceleration Delayed puberty
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90% hipopituitproses destruksi adeno hipophisisTU: Nonsecretory adenomas Sheehans pit.necrosis Empty sella tursikaNeoplasma .metas.Pituit apopleksi chemorDisruption blood supply by sistemic arteritis / or trSinus venosus cavernosusDistruksi inflamasi lob ant by sarcoidosis / inf,surgical / radiation ablation metab.dis
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NON SECRETORY CHROMOPHOBE PITUIT ADENOMA
25 -30 %Klinik efek lokal - Kelainan lapangan pandangan - Sakit kepala - hipotensi --- one of target endoc organ UNDER pituit control ( HIPOTIROID HIPOGONADISM )
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MORFOLOGIBesar silent bertahun-tahunNull cell adenomaOncocytoma sparsely granules lessionImunohistokimia FSH, / hormon, LH
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SHEEHANS SYNDROME / NECROSIS PITUIT POST PARTUMHamil pituitary besar Hemorr vasospasmenekrosis.iskemik/ infark lobus anterior Shock
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DICSickle cell anemiaTr. Sinus cavernosus arteritis temporal, traumatic injury pembuluh darah, DM (lama)
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95-99 % destruksi lobus anterior defisiensi gonadotropic laktasi menurun (puerperium)Defisiensi TSH atau hipotiroidACTH (insufisiensi adrenocortical)Infark : soft, pale fibrosis
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EMPTY SELLA SYNDROMEHernia arachnoid deffect diafragma sellaTekanan CSF atropi pituitary empty sella
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SINDROMA PITUITARY ANTERIORJarangTerutama lesi hipotalamus suprasellerDisfungsi ADH defisiensi diabets insipidusArginin vasopresin:oksitosin kontraksi uterus partus stim glandula lactating
Neoplastik : tumor supraseller, metas Ca, abses, meningitis, TBC, sarcoidosis (inflamasi hipotalamus-hipofisial)Surgical radiasi hipotalamus-hipofisialSevere head injuryIdiopatik
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Hypothalamik suprasellar tumors Neoplasma jrg GLIOMA / Craniopharyngioma
Hipofungsipituitary anteriorHiperfungsiDiabetes insipidusCraniopharyngiomaDari sisa rathkes pouchPada sella, suprasellar Anak, dewasa mudabenign
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MORFOLOGI3-4 cm, solid encapsule, kistik multilokuler, kalsifikasiMikroskopis :
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ADENO HIPOFISIAL HIPOFUNGSILorain levi syndromeGH / hormon lain HipoglikemiagonadotropinKraniofaringiomaFrohlichs syndromeObesitasPerkembangan sex arrestMental sub normal,Growthtumor
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Prepubertas--- growth pituitary dwarfism sexual retard
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HIPOGONADISMAmenoreLoss of axilla and pubicSterillityOvarium atropi, genital atropiTestis atropi, sterillityLoss of axilla, pubicHipotiroid TSHHipoadrenal ACTHPanhipopituit Simmonds
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TSHIodine in diet iodine+tirosin T3 sekresi abs stimulasi monoiodo oleh TSH oleh TSH andbowel diiodotirosinT4 blood
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TIROIDTirotoksikosis (hipertiroidism)HipotiroidismDifus / fokal enlargement (Goiter) tidak ada hubungan antara lesi morfol dan klinik
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TIROTOKSIKOSISHipertiroidism, Free T3 , T4 Hiperfungsi tiroid (Graves disease) hipertiroidismNon hiperaktif kelenjar tirotoksikosisSindrom :NervousnessBB (nafsu makan baik)PalpitasiHeat intoleranceRapid pulseWarm skinFatigabilityEmosi labilKelemahan ototPerubahan haidBMRTremor tanganPerubahan mataKelenjar tiroid membesar
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MORFOLOGIVasodilatasi : Periferheat lossSirkulasi hiperdinamik general kulit : warm, moist, flushedhipertiroidperubahan mataGraves disease---proptosis perubahan imunologiInflamasi sekunder jaringan retroorbital jantung TakhikardiPalpitasiKardiomegaliAritmia, fibrilasi supraventrikuler
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GRAVES DISEASE
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THYROTOXIC CARDIOMIOPATHYFokus infiltrasi limfosit, eosinofil, interstisial, fibrosis ringan, fatty changesManifestasi lain: Otot skelet : atropi, fatty infiltrasi, infiltrasi limfosit fokal Hati : minimal fatty changes, fibrosis periportal, infiltrasi limfositOsteoporosis general lymphoid hyperplasia dg lymphadenopathy
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Graves Disease< 40 th (85%)HipertiroidDiffuse toxic hyperplasiaHiperfungsi tiroid Pemberian iodine >> pada kelainan (T3 / T4)tiroid Jod basedows disease
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HIPERFUNGSI TIROIDT3 T4 sirkulasi tirotoksikosisTerdiri dari :Graves diseaseToxic adenomaToxic noduler goiterKlinik :ExophtalmusProminent tiroidBMR Skin warm, sweaty : heat intoleranceWeakness,hiperkinesia, emotional instabilityBBGlucose tolerance, glukosuriaNadi , Cardial arrhythmia and failure ( orang tua )TSH
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TIROIDITISTiroiditis interstitialPalpation tiroidSuperior / inferior tiroidjarang direct traumaticImunologi Staf, strep, salmonella, enterobacter, M tbc, jamur (candida, asper, mucor)
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MorfologiKelenjar besarHasimotoSub acute granulomatous tiroiditisSub acute limfositic tiroiditisRiedel struma
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HASHIMOTOS THYROIDITIS/ STRUMA LYMPHOMATOSA/ LYMPHADENOID GOITER : Goitrous hipotirNon endemic goter (children)Autoimun diseaseBerlangsung lama hipotiroid hipertiroidhashitoxicosisHashimoto Grave
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ETIOLOGIAutoimunDefek fungsi tiroid spesifikSupressor T cellAntibodies tiroid peroksidase (antimicrosomal antibodies)AB Spesifik untuk tiroglobulinTSH reseptor
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MorfologiKelenjar besar, diffuse, asimetris, kapsul intak, coklat noduler, Rubbery tissueMikroskopis :Perjalanan penyakit : usia pertengahanHipotiroidismAtropiStadium dini metabolisme normal, fungsi tiroid, TSH, T3 T4 Prognosa baik: 1-200 lymphomaGoitrous hipotirNon endemic goter (children)Autoimun disease lymphoma
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HASHIMOTOS THYROIDITIS
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HASHIMOTOS THYROIDITIS
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Riedels fibrous thyroiditis /Riedel strumaCausa unknownAtropi/hipertropi, fibrosis,firm,abu 2 caKadang : Retroperitoneal fibrosis( multi fokal fibro sclerosis )Mikros : fibrosis , infiltrasi limposit scant,fol tiroid distorsi
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Sub acuta granulomatous(de QuervainS )Thyroiditisde Quervains Thy Self limited, inf granule / Giant cell / Gran.thy dekade 2-5 : --- 3: 1Unknown virus onset didahului infeksi virus (mumps,measles,influ,adeno vi,coksackie vi,echo vi) Klinik : demam,tir >>> nyeriMORFOLOGI :Tir > > > /
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MORFOLOGI
Tir > > / Fokal asimetrisOpt gland tkena irreg : lokalisasi firm,kuning putih ruberryHistologi :Patchy LamaProses inflam,micro absesLanjut folikel rusak / agregat sel sukar ? sel dari folikel makropagmultinuclear giant cell (koloid) fibrosis gran thyroiditis
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SUB ACUTE GRANULOMATOUS THYROIDITIS (DE QUERVAINS THYROIDITIS)
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SUBACUTE LYMPHOCYTIC (PAINLESS) THYROIDITIS? GOITROUS HYPERTHYROIDFokus infiltrasi limfositJaringan fibrous interstisialKel tir >> dan Hipertiroid Amerika utara 15 % graveGoitrous >> tdk terdeteksi self limiting disease hipotiroid? T3 dan T4
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GRAVES DISEASEHipertiroid hiperfungsi difuse hiperplasia goiterInfiltratif ophtalmopathy, dermopathyOpthalmopathy :Lid lagUpper lid retractionEdem periorbitalStareOtot mata lemahproptosisDermopathy:Edem pinggung kaki, tungkaiPlaque like / nodulerLokal myxedema(-) Tirotoksikosis diffuse toxic goiterUSA 12-20 %
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ETIOLOGI DAN PATOGENESISAUTOIMUNIg G antibodi TSH reseptorThyroid stimulating AB (TSAb)Thyroid stimulating Ig (TSI) HashimotoPada pdrt Graves dpt an.pernisiosa, rheumatoid arthritisMORFOLOGI :Diffuse hyperplasia80-90 grKapsul intactDiameter parenkim lunak daging
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HISTOLOGICell>> vask Pseudopapil mushroom, folikelKoloid , pucatStroma jaringan limfoid >>, folikel >>general lymphoid hypertrophyPERJALANAN PENYAKIT Muda tirotoksikosis tir > simetrisUptake JOD131 Proptosis >> injury cornea, ulserasi, mata (-)
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DIFFUSE AND MULTINODULAR GOITERKerusakan output hipertiroid kompensasi kad TSH Hipertropi, hiperplasia epitel folikel Diffuse/ multi nodulerTir > hor < eutiroid1. diffuse non toxic (simple goiter)2. multinod goiter
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Diffuse non toxic (simple) goiterPembesaran diffuseHiper/hipofungsiENDEMIC GOITERSimple goiter, lokalisasi geografik 10 % populasi pegununganIntake yod hor tir TSH hipertir, hiperplasiaCa, flour goiterMakanan dan sayur
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NON ENDEMIC / SPORADIC SIMPLE GOITER 8 : 1Pubertas / Dws mudaTSH Kehamilan estrogen TBG 1. defek transport jod T3 T42. defek organifikasi feed back inhibition TSH3.defek dehalogenaseDefek iodotirosin coupling
- MORFOLOGIStadium hiperplastik, colloid involutionHiperplastikKel >> sedang (100-150 gr)Difuse, simetris, hiperemik Histologi : epitel fol kolumner, generasi fol ,
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Multi noduler goitersimple goiter multi nod goitersporadik endemiknon toxictirotoxicosis (toxic multi nod goiter) plummers diseaseJarang hipotiroidTiroid membesar DD/neoplastikOlder ok. komplikasi Sel tiroid normal-heterogen perbedaan potensiProliferatifFolikel membesar ruptur hemoragik jar. Parut kalsifikasi
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Morfologi1. nod hiperplasi folikel, folikel dg koloid banyak2. irreguler scarring3. Hemoragik fokal, deposit hemosiderin4. kalsifikasi fokal pd jar. Parut5. mikrokista
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Perjalanan PenyakitKlinik penting:Ukuran, lokalisasi massaFungsi abnormal biasa : tirotoksikosisPerbedaan dg neoplasmaCVS pd org tua: fibrilasi atrium, takikardi, heart failure kosmetik, disfagi, stridor inspirasi, penekanan v,kava sup : distensi vena leher dan ekstremitas atas, edem palpebra / konjungtiva, batuk dan sinkope
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Hemoragik tiroid >>, nyeri dan serakHiperfungsi toksik noduler goiterUptake jod 131 , T3 T4 Akumulasi radioaktif tersebar ke dalam tiroidJarang menyebabkan noduler hiperfungsiTiroid >> 2000gr 1 atau lebih lobus menekan trakea, Oesofagus, dan dibelakang sternum intrathoracic plunging goiter
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MorfologiMultinod heterogenNod batad tidak tegasJar. Parut tampak sebagian encapsul Penamaan yg salah adenomatous goiter dan multiple coloid adenomatous goiterDD/: neoplasma sukarCT scanUSGMRIBiopsi aspirasiResiko Ca namun data (-)
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NODULER GOITER
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NODULER GOITER
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HipotiroidismHipometabolismeHipotiroid1. selama fase perkembangan, invasi, kretinisme physical, mental retard2. older children/adult myxedemaAkumulasi hidrofilik mukopolisakaridPada jar penyokong edem dan kulit tebal
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KausaUSA hipotiroid, idiopatik primertiroiditis autoimun atropik15-60% Sustansi ------------TSH reseptor bloking auto ABdapat menghilang spontanTerapiPost tiroidektomi >>
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CretinismJarangRetardasi fisik dan intelektualPada saat lahir tergantung dr hormonal efeknya terlihat setelah bbrp minggu-bulan2Klinik : kulit kering,kasar Widely set eyes peri orbital bengkak hidung lebar,flattened lidah besarJod -pada perkembangan fetal Agenesis tiroid Defek biosintese kongenital Pertumbuhan skelet terganggu, perkembangan otak Endemik cretinism endemik goiterSporadic cretinism cretinism sporadik atropi
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MYXEDEMAOlder child diantara kretin dan dewasa dg hipotiroidAdult pelan2 Aktifitas fisik dan mental lambatBicara, intelek Edem preorbitaKulit tebal, kasar, kering, lidah >>Facial feature thikenedLetargik keringat banyak, konstipasi, fungsi motorik => Sebagai kretinMikros : miofiber edem, striae hilang
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TUMORSNODUSA, adult, 2-4%, nod daerah endemik goiter,90%adenoma, karsinoma 25-35kasus/milionSolitary mass nodules, multinucleated goiter, kista, pembesaran asimetrisNod soliter neoplastikwarm/hot nodul benignaPenderita> non neoplastik
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ADENOMADari epitel folikel ad. FolikulerMikros : fetal,embrional,simple, colloid, mikro & makrofolMorfologi :DD/ sukar dg multinod goiterBerkapsul, mendesak sekitarHomogen, 3-10 cmHitam abu2, soft fleshyHemoragis, fibrosis sentralNekrosis sentral -- kista
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Mikroskopis :TrabeculerFetal stroma miksomstousColloidSpindel / atipikalhurthlePerjalanan penyakit :Sloly increase insizeUkuran tertentuCepat besar, nyeri, hemoragisJarang T3 dan T4 tinggicold nodule dapat hot
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ADENOMA FOLLIKULER
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ADENOMA FOLLIKULER
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Malignant tumors USA 1991 1000 , 2/3 perempuan, >1% penyebab Papil Ca 75-85%Fol Ca 10-20%Med Ca 5%Anaplastik Ca jarangPatogenesis :1. Radiasi head and neckPembesaran tonsil,timus,acne, irradiasi pd dekade 2 carcinogenik4-9%infant rad Ca 20 th, 70% bom di jepang2. hashimoto limfomaNon toxic goiter
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Papillary CaKhas :Hipokhromatik empty nucleidevoid of nucleus (orphann annie eyes)Inti grooveInklusi intranukl eosinofilikpsammomaEncapsulated variantFollikulerTall cell
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PAPPILARY CARCINOMA
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Follicular CarcinomaPerjalanan peny :Foll Ca tumbuh lambat lanjut irreg,multinodD/ ditegakkan metas (+)/(-)Prognosis :Ukuran primer?Invasi kapsul +/_Anaplasia?Larger invesive Ca5 th surv rate 30%10 th surv rate 20 %
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Anaplastic Ca5 %Older, daerah endemik goiterHistologi:Spindle cell Ca dapat fol, papil CaGiant cellSmall cell jarangD/ massa cepat >>Lokal invasive (trakhea)
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Medullary CarcinomaNeuroendokrin neoplasma sel parafol:Calcitonin Stroma amiloid20-25% ~ MEN syndrMorfologi:Tumor tersebar 1 lobusNodules kedua lobusFamilial MEN syndr10 th surv rate 90 % sedangkan MEN syndr 10 th surv rate 30-50%
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MEDULLARY CARCINOMA