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va tang nOng dO hormon testosteron trong mau. DOi vai FSH, no kich thich to bao sertoli bai tiet cac chAt sink tinh trang, Sing died to bao nay ding fiat ra hormon la inhibin, IA mot gl[r]
(1)Chu'o'ng
HOA SINH LAM SANG BENH LY TUYEN NOI TIET MUG TIEU
1 Trinh bay Ave d6 lieu hoa ngtroc truc dtroi dai-tuyn yen-tuyen 2 Liet ke cluvc she thay doi cac xet nghiem hoa sinh twang giap 3 Liet ke Ave su• thay doi cac xet nghiem h6a sinh nhuvrc
DPJ CU'O'NG
Co the song la met kh6i th6ng nhat hoan chinh, vai tre chuyen hoa cac chat co the la do'cac to chic va ca quan dam nhiem Cac chirc nang cua cac co quan, be phen co the duct dieu hoa boi hai he th6ng N than kinh va he nei tiet
He th6ng than kinh: &rig vai tre dan truyen tin hieu hay th6ng tin di qua met he th6ng co dinh ve eau trite
He th6ng nei tiet: g6m nhieu hormon the tuyen dee biet bai tiet va dirge van chuyen mau nhtr nhung thong tin di dOng de tat &Ong len cac co quan Hai N thong c6 m6i lien quan rat tinh vi Vi du catecholamin la nhiing chat dan truyen than kinh a mot to chirc nhtrng lai la hormon o to chic khac
Tuyen nei tiet la cac tuyen khong e6 6ng dan xuat, cac hormon cua no tham trgc tier, qua N mao mach thong vao mau Hau c6 nguon gee tir cac to bao Welt me, ngoE o tinh hoan (san xuat testosteron) Aid buong tang (san xuat estrogen) c6 nguon gee tir to chirc lien ket, he th6ng than kinh tuyen yen va cac to bao tiet c6 nguon gee than kinh Cac tuyen nei Wet nam rat rat co the nhtr tuyen yen, tuyen giap, tuyen can giap, tuyen thuong then, tuyen tay nei tiet, tuyen sinh due Ngodi cac co quan khde ding co chirc nang nei tiet nhu viing duai doi, cac dau ten clang eila cac sqi than kinh, 'Ong tieu h6a, then
T6 chirc dich CO lien quan met thiet den cac he thong nei tiet Tin hieu tir he than kinh trung uong dtroc truyen qua met boat he tir dieu chinh toi to chirc dich cuoi clang
‘Viing duoi doi la trung tam phihop chirc nang th6ng nei tiet N6 nhen va
(2)I-16A SINN LAM SANG
yen, mot s6 dugc goi la "yeu to giai phong" c6,tac dung kich thich tien yen tiet mot hormon Wang *rig, so khac goi la "yeu to ire che i c the tien yen tiet hormon Khi dugc kich thich, tien yen se tiet hormon vao mau de van chuyen tai cac tuyen n6i tiet khac nhu v6 thugng than, tuyen giap, tinh hoan, buong trimg, tuyen nOi tiet
Cac hormon vimg duoi d6i dugc san xudt vai mot lugng rat nho
1 DANH GIA CHCPC NANG TUYEN YEN
Hinh 6.1 Tuyen yen
Tuyen yen tuyen nOi tiet nh6, kich thuac cm, lugng 0,5 — g, ndm a day nao Tuyen yen c6 hai
(3)CHUO'NG ► HOA SINE/ LAM SANG BUNN LY TUYEN NOI TIET
Cac hormon c6 nhfing dac di6m sau:
- V6 eau frac, la nhang polypeptid Wang d6i dai (ACTH c6 39 acid amin) - Ve tac dung:
+ Kich thich cac tuyen not tit san xuat hormon ttrang frng
+ Die‘u hoa chuyen hoa glucid, lipid, protein va din giai cua nhieu to chirc, ca,
mo gan
- Co ch6 hoat dOng cda hormon tin yen la tao phirc hcrp H-R o mang bdo ttrang va hoat hoa adenylatcyclase (AC)
- Su bai tit va tong hop hormon tin yen not Chung dtrov ki6m soat bed h6 th6ng feed-back
Thily sau hay hau yen hay yen thAn kinh khong san xuat ma chi china hormon Id anti-diuretic hormon (ADH) goi la arginin-vasopressin (AVP) va oxytocin
1.1 ChCpc nang sinh ly thOy tru'o'c tuy6n yen
HAu h6t cac hormon cua thiiy truerc tuy6n yen du c6 tac dung kich thich t6 bao tuy6n dich nhu tuyen giap, vo thugng than, buong thing, tinh hoan va tuyen vu Rieng co GH tao tac dung tren hdu h6t t6 bao o cac mo ca th6
1.1.1 Growth Hormon (GH)
Hinh 6.2 Hormon GH cua they tru&c tuyen yen
(4)HOA SINH LAM SANG
phut trien se luting to bao, va da'c biet la gay biet h6a mot s6 to bao nhu to bao xuang va to bao co, sau truemg xuang ngimg phut hien, cac mo khac van tiep tuc phut trien
Tac dung chuyn hoa
Chuyen hoa protein: GH lam tang mire tong hop protein tat ca to bao co the bang cac tac dung:
- Tang v'a'n chuyen acid amin qua mang to bao, lam tang neng de acid amin not bao tuo thuan loi cho tong hop protein
- Lam tang dich ma ARNm de tong hop protein
Tang chop ADN nhan de too ARNm, qua gidp thitc day sir tong hop protein va phut trien ca the ,
- Giam hoa protein va cac acid amin to bao N6 huy &Ong mot luang,lan acid boo tu dr cac to chirc de cung cap cho nhu cau nang luting co the
Chuyen hoa lipid: huy dOng acid boo tir cac to chirc lam tang acid boo to mau, tang sir dung acid boo cho nang luting Nhu vay ca the clanh glucose va protein cho so phut trien Khi tang qua nhieu GH se tang sir dung acid boo sinh nhieu the ceton va viec huy dOng nhieu acid boo ve gan ding gay chimg gan nhiem
Chuyen hod glucid:
- Giam tieu ding glucose cho nang lugng tang huy dOng acid boo nhu trinh bay a tren
- Tang du till glucose duai dung glycogen to bao: glucose khong tieu di cho nang luting se vao to bao
- Tang glucose mau: glucose vao to bao clued tac dung ciia GH nhung tac dung chi keo dui 30 phut den gia, sau to bao da nhan glucose qua mire se khong nhan them lam tang glucose mau (c6 the den > 50% so vai binh thuemg) va c6 the xuat hien glucose nieu goi la dui thao dueing tuyen yen
- Tang bai tiet insulin hai tac dung: GH lam tang clueing huyet dong thai kich thich truc tiep to bao beta dia tuyen toy tiet insulin Su kich thich doi qua mire gay kiet quo kha nang tiet insulin dm to bao beta gay dui thao clueing toy that stx Vi vay GH dirge coi la nguyen nhan gay benh dui thao clueing
Hog dOng cua GH can c6 su phei hop dm insulin va glucid, neu thi'eu insulin va glucid, GH se khong gay phut trien co the
Kich thich sun va phut trien xuang
Tac dung rO rang nhAt cua GH la tang phut trien xuong, bao gem: (1) tang gift protein cho to bao sun va to bao sinh )(mug, lam xuong phut trien, (2) tang mire sinh san cua to bao nay, (3) chuyen to bao sun to bao sinh xuong, nhu the se to xuong mei Co hai co the phut trien xuong:
(5)CHU'O'NG ► HOA SINH LAM SANG BENH LY TUYEN NOI TIET
xuong lam keo dai than )(strong va day dau xuong xa D6ng thai ban than sun dau xuong ding citroc sir dung clan de sau tu6i twang thanh, dau xuong not vai than xtrong va xuong khong dai them duce nua GH kich thich tat ca cac qua trinh nay, nhtmg dau xuong not vai than xuong thi GH khong the lam dal xuong duce nita
Cac to bao tao xuong nam a chung quanh xuong va mat s6 h6 xuong, chimg tao xtrcmg mai tren be mat dm xuong cit Wong thi cac to bao hay xuong hity di cac xuong cu, mac d6 tao xuong 16n hon hity thi xuong ngdy cang day len GH kich thich manh tao cot bao lam xuong to va qua trinh din suot dai, dac biet la cac xuong mang
GH kich thich gan va mot so to chirc tao nhieu protein nho goi la somatomedin,
no c6 tac dung manh lam tang sty phat trien xuong CO somatomedin &roc phan lap,
quan nhat la somatomedin-C goi la Insulin-like growth factor 1(IGF-1), nemg dm no trong,huyet tuong c6 quan mat thiet vai mac bai tiet GH Ngugi to cho rang tac dung chit yeu caa GH th6ng qua somatomedin hon la true tiep len xuong va cac mo khac
Didu boa bai tiet GH
Bai tiet GH thay cf6i timg,phirt va tang cac trirang hop nhin doi, giam tram protein, giam dtrang huyet, Ming d6 acid beo mau giam thap, tap luyen, lao dgng, chan thuong Hac biet no tang gig dau sau ngit say
N6ng de GH binh thugng huyet tuong a ngtrai lan tir 1,6 den 3ng/ml, tre em va Med trtrang khoang 6ng/ml Khi nhin dOi keo dai, du tilt dm protein va glucid dia co the bi giam, neog c/6 GH tang len den 5Ong/ml
Vimg cluai d6i bai tiet hai hormon de dieu hoa bai tiet GH la growth-hormon-re-leasing hormon (GHRH) la polypeptid c6 44 acid amin, kich thich tiet GH va growth hormon-inhibiting hormon (GHIH) c6 14 acid amin, irc cite giai ph6ng GH
Su dieu boa bai tiet GH phirc tap, ma sir dieu hea lau dai la tinh trang dinh dual-1g, chit yeu la ham luting protein to bao, cang thieu protein cang kich thich bai tiet GH
Cac bat tlaco'ng bai tiet GH
Bai tiet qua mire GH goi la cuang tuyan yen va giant hoac khong bai fiat GH goi la suy tuyen yen
Thieu GH:
(6)HOA SINN LAM SANG
Nguai suy tuyen yen chi thieurGH don thuen, c6 the duoc dieu tri khoi hoan toan, GH có tac dung cai thin cac roi loan chuyen hoa khac, vi no co chirc nang chuyen h6a kha rung
Suy tuyen yen,tren ngued lan hay gap u chen ep hay tac mach mau tuyen yen, thuemg la s6c tun hoan sau sinh (h6i chirng Sheehan)
Suy tuyen yen se den den met chirc nang Binh due, suy giap gay phis niem, tang can,
giam san xuat hormon glucocorticoid tuyen vO thuong then
ngurori giam hay met kha nang bai tiet GH, qua trinh gia se phat trien nhanh Nhur vey gia la mot qua trinh giam dan six bai tiet GH cua tuyen yen tithe
Tang tiet GH:
Benh kh6ng 16 (gigantism) thuang u tuyen yen a to bao somatotroph, hay can goi to bao ua acid gay nen Vic san xuat qua In& GH lam cho tat ca ma ca the Oat trien nhanh, va neu xay truck tu6i day thi chua dung den xucmg thi co the phat trien nhanh ve chieu cao, ngued khong 16, có the cao ten 2m Nguai kh6ng to bi tang throng huyet va to bao beta bi thodi hoa lam viec qua mix duai su kich thich cua duang huyet cao va kich thich true tiep cua GH Khi u tien trien qua mix c6 the den den suy yen
Benh to dau chi (acromegaly): neu u tuyen yen a to bao somatotrop, xay sau tuoi truang Xucmg khong dai dirge nhien van day len, to dec biet cac,xuang det tao nen hinh clang dec biet g6m ham va tran nho tnxac Cac ma mem van tiep tuc phat trien lam mlai to gap doi binh thuremg, ngon tay, chan to va cac co quan nhu gan, then ding 16n dan
1.1.2 Adrenocorticotropic Hormon (ACTH)
La met polypeptid c6 39 acid amin
Tac dung: Kich thich to bao lap 136 va Wed cua vo thuong then, no lam tang bai tang sinh va phi dai cac to bao
Dieu Ma bai tiet: Chu yeu kich thich cua corticotropin-releasing hormon (CRH) yang duai doi, la met polypeptid c6 41 acid amin Ngoai c6 stress tinh than va the xac, chi vai philt den den su tang bai -het ACTH va cortisol den 20 lan
Nhip ngdy dem: su tiet CRH xay theo chu kST ngay-dem lam cho su tiet ACTH clang tuang tu Nhip tiet khoang 15 phat/len, dat dinh lfic gia sang va thap nhat khoang 22 gia den gia
(7)CHU'O'NG 0- HOA SINH LAM SANG BENH L).1 TUYEN NOI TIET
Cac stress tinh than lam tang nhanh stx hal tiet ACTH N6 lam tang hoat gong cila he limbic, dac biet la vtlng hanh nhan hai ma, r6i ca hai viing truyen thong tin den viing duai doi
Dieu hoa ngugc cua cortisol len yang dirai doi, tuyen yen: n'ang de cortisol mau tang len no tac dung dieu hoa ngugc am tinh len yang duai doi lam giam bai tiet CRH va len tuyen yen lam giam ACTH Ca hai tac dung ginp gift on dinh n6ng de cortisol mau
1.1.3 Prolactin (PRL)
La met hormon to bao tra acid dm tien yen net ra, c6 cau true la chai don poly-peptid c6 198 acid amin, luting phan tir' 23 kDa
Tac clung
,
Kich thich tuyen bai tiet sita, nhung chi ea tac dung that su tuyen vu da dirge Oat trien day du clued tac dung cila estrogen va progesteron Do chu kST bung trimg binh thiremg prolactin duet bai tiet it, va khong c6 the dung kich thich tuyen vu bai tiet sua Trong thai Icr co thai, prolactin dirge bai tier tang len tir' tuan thir nam, va tiep tuc tang den thai ky sinh con, Mc no tang den 10-20 lan so vai binh thuong Ben canh thai cung tiet met luting hormon somatomammotropin c6 tac dung sinh sua nhe, nhu vay hai hormon phihop de gay bai tiet sfra Tuy nhien truck sinh tuyen vu van chua bai tiet sita vi hai hormon estrogen va progesteron ire che sir bai tiet sua cila tuyen
Ngay sau sinh, ca hai hormon estrogen va progesteron thai tiet ngung dot
nget, cho pile") tac dung sinh sita prolactin Sir bai -het sib can sir phoi,hop cila met so
hormon khac nhu GH, insulin, cortisol va thyroxin, nhung hormon can thiet de cung cap acid amin, acid beo, glucose va calci can cho sir tao sita
Di'du hda bai tiet prolactin
Vai tuan sau sinh, muc bai tiet prolactin ve binh thixemg nhu luc chua c6 thai Tuy nhien cho bu, nhirng kich thich co' hoc se truyen den viing doi cila nguai me, thong qua prolactin releasing hormon (PRH) den tuyen yen trirac gay tang tiet prolactin, mire bai tiet tang dr 10-20 lan, va keo dai khoang gia, cai tri sir bai tiet sita cho dot bu tie') theo Ngung cho bu, tuyen yen cling ngirng bai tiet prolactin va tuyen vu ngirng bai tiet scra Su san xuat sita al the keo dai nhieu nam, neu tre van tiep tuc bu, nhung mire tao sua se giam dang ke sau 7-9 thang
Trong thai gian cho bu phan lon ba me khong co chu ky bu6ng,trang va sir rung
tr(rng nen kh6ng c6 thai tiep., Ca che la tre milt xung clang truyen len vung clued
(8)C) Hormon bung trCmg
Estrogen
Progesteron
D) Noi mac tix cung
FS
I I I i 1 i
B) Bu6ng ft-Ong Rung truing Hoang the
• • GoCNO Nang•
A) Hormon tuyen yen
Vimg duai d6i tiet prolactin-inhibiting hormon (PIH) lam irc the su bai tiet prolactin cua tien yen, cc') the giam den 10 lan Dopamin va cac chat dopaminergic ding c6 the dung irc che tiet PRL
1.1.4 Follicle Stimulating Hormon (FSH) va Luteinizing Hormon (LH)
Hinh 6.3 Dieu hba bai tiet FSH va LH
Ca hai deu la glycoproteinsclugc tiet cac to bao ua base cila tuyen yen, chiing
tac dOng den to chic dich bung trCmg va tinh hoan
Tac clung
Bung trung: FSH kich thich mot s6 nang &mg trixong thanh, di mot truCmg nhanh nhat, chin va xay hien tugng rung‘trimg LH kich thich t6 bao hat, lop vo oda to bao se tiet hormon buong trnng la estrogen Tinh hodn: FSH kich thich to bao sertoli 6ng sinh tinh, lam cho cac to bao phat trien va bai tiet cac chat sinh tinh trung Chat ding v6i hormon tes-tosteron co tac dung dinh &fang manh tren ong sinh tinh, lam to bao mam cua
(9)CHU'ONG IN.- HOA SINH LAM SANG BENH LY TUYEN NOI TIET
trang LH kich thich to bao ke Leydig bai tiet testosteron, mire bai tiet testosteron phu thuOc vao n6ng dO LH mau
Di& hoa bai tiet FSH va LH
Bai tiet FSH va LH cua tuyen yen truac la hormon yang dual doi gonadotropin-re-leasing hormon (GnRH) hay goi la luteinizing-hormon- regonadotropin-re-leasing hormon (LHRH) dam nhiem, la mot decapeptid
GnRH dugc bai tiet cach quang den giO, moi lAn chi bai tiet khoang it phut Cuing dO kich thich cua hormon phu thuOc vao hai yeu to: tan so va lugng GnRH dugc bai tiet a moi chu kST Su bai tiet a tuyen yen truac ding c6 chu lcSr theo chu IcSf cua bai tiet GnRH Do lien quan chat the nhu the nen GnRH dugc goi la hormon giai phOng LH viet tat la LHRH Trong d6 su bai tiet FSH chi thay doi nhe theo GnRH, no thay doi chum sau nhieu
frc the ngugc cua hormon sinh due len Wing doi-tuyen yen: co' the dieu hoe ngugc am tinh cua testosteron la no dugc bai tiet nhieu, se tac dung ix the \Tung clued doi, lam giam bai tiet GnRH lam giam bai tiet LH dm tuy'en yen Testosteron tac dung live tiep len to bao yen truac lam giam bai tiet LH Ngugc lai testosteron mau
giam se cho phdp yang duai doi bai tiet mot lugng han GnRH clan den tang tiet LH
va tang nOng dO hormon testosteron mau
DOi vai FSH, no kich thich to bao sertoli bai tiet cac chAt sink tinh trang, Sing died to bao ding fiat hormon la inhibin, IA mot glycoprotein Khi ong,sinh tinh san xuat qua nhieu tinh trimg, thi bao sertoli cling san xuat nhieu inhibin Chat c6 tac dung dieu hoa ngugc am tinh, ire the lai tuyen yen truac, lam giam su bai tiet FSH, d6 lam giam san xuat tinh trung N6 cling c6 tac dung uc the nhe len yang doi lam giam n6ng dO GnRH
Tac dung dieu hoa ngugc cua estrogen va progesteron len tuyen yen: LH c6 tac dung tri hoang the va kich thich hoang the tiet estrogen va progesteron Khi ming di) hai hormon tang cao mau se c6 tac dung *c che ngugc lam tuyen yen truac giam bai tiet LH va yang duai doi lam giam bai tiet GnRH, nhien mac clO it ham so vai 111C che ngugc len tien yen va chu yeu la no lam giam tan so nhip Wet GnRH
Cac t6 bao hat dm hoang the ding tiet inhibin, tac dung *c the manh su bai tiet FSH cua tuyen yen Ira uc the LH vai mire ciO it hon Khi LH N uc the thi hoang the bi teo di
(10)HOA SINN LAM SANG
lai cua nang trimg, chit yeti la cac to bao hat chuyen hoang the, LH tiep tuc tri hoang the
1.1.5 Thyroid Stimulating Hormon (TSH)
La mot glycoprotein lugng phan tit 28 kDa, co quan dich la tuyen giap
Tac clung:
- Tac dung quan nhdt cita TSH la giai phong T3, T4 vao mau, no gift') phan giai thyroglobulin, tach T3, T4 khoi globulin va ph6ng thich chimg vao mau khoang 30 phut
- Kich thich to bao giap tang kich think va tang san xudt hormon giap
Tang se lugng to bao tuyen Ara chuyen clang to bao tir hinh sang hinh tru
Die'u boa bai ilk:
Tiet TSH dugc dieu,hoa Thyrotropin-releasing hormon (TRH) dm \rang duai
deli TRH tac dung true tip len to bao tuyen yen lam tang giai phong TSH
Lanh kich thich yang &PM deli bai tiet TRH lam tang tiet TSH cua tien yen Cam xitc gay tang bai tiet TRH va TSH, kich dOng hoac lo tang kich thich manh 118 giao cam gay giam hal tiet TRH va TSH, c6 le tang than nhi8t va tang chuyen h6a lam *c the v-Ung duai deli
Dieu hoa ngugc: bai tiet TSH cua thiiy tilt& tuyen yen se giam c6 su tang hormon giap mau Hormon giap tang 1,75 lan so vai binh thuong thi TSH giam tad khong Ca the gifip tri nOng dO hang dinh hormon giap ca the
1.2 Chirc 'fang sinh 19 they sau tuy6n yen Arginin vasopressin (AVP)
Cys — Tyr — Phe — Gin —Asn Cys Pro Arg — Gly-N H2
Oxytocin (OT)
Cys Tyr Ile Gln Asn Cys Pro Leu Gly-N H2 Hinh 6.4 C6u tr0o cua ADH
Thiry sau tuyen yen khong bai tiet hormon, ma cac,te bao tuyen yen sau chi
trq gifip nhung tan cling than kinh vang dugi xuong Nhiing tan cling than
(11)CHU'O'NG 0- HOA SINH LAM SANG BENH LY TUYEN NOI TIET
arginin8-vasopressin (AVP) va hormon thin hai la oxytocin Hai hormon dugc tong hop bgi nhan ten thi (chit yeu tong hop ADH, chi mot it oxytocin) va nhan canh nao that (chi yeu tong hop oxytocin, chi mot it ADH) Sau dtrgc tong hop chung ket hop vai protein mang la neurophysin, chuyen den dau tan cling than kinh n'am a hau yen Khi xung citing than kinh tuyen tlr nhan tren thi va nhan canh nao that xuong vimg hau yen, hormon &roc bai Wet ter cac hat nam a tan cling than kinh, qua co the xuat bao va dugc hap thu \Tao matt bai cac mao mach ben canh Ca hai neurophysin va hormon dugc ph6ng thich &mg thai, nhung gin long leo nen neurophysin tach thi hormon va khong c6 tic dung se bi thodi hoa
1.2.1 Anti diuretic hormon (ADH) Mc dung
Khang lgi ni'eu: ADH lam tang tinh tham cua 'Ong xa va 6ng gop doi vai nuac lam cho phan lon nuac dugc tai hap thu tra lai co the, nuac thai dual clang nuac tieu it, giup co dac nuac tieu.,Thieu ADH Ong g6p than hau nhtr kh6ng doi vai nuac, nuackhOng dugc tai hap thu se thai clang nuac lieu, lam nuac tieu loang, giam ty (b'enh dal thao nhat)
ADH mau cao c6 tic dung co lieu dOng mach cila toan co the, lam tang ap suit dOng mach, nen ADH dugc goi la vasopressin
Di'du hoa bai tiet ADH
- Tham thau: ap suit tham thau cao, no qua Ong mach den yang °huh d6i tic dung den cac thu the tham thau a day, tao tin hi'eu than kinh ttrang trig truyen theo sal than kinh den hau yen va tang tiet ADH, mdc tang c6 the den 20 lan so vai binh thtremg Ngtrgc lai suit than' thau mau giam, vimg dugi doi ngimg phat xung va ngimg tiet ADH Thai gian ban huy ADH chi 15-20 phitt, do n6ng dO ADH mau thay d6i nhieu chi it phdt
- The tich Man hoan cling anh liming den bai tiet ADH, the tich mau giam chi 15 -25% thi ADH c6 the tang 50 lin so binh thuang Ca che la cac thu the sic cang a tam nhY se nhan biet hru luting tuan hoan, xung deeg se truyen den nao de dieu hoa bai tiet ADH Tuan hoan giam se tang tiet ADH va ngtroc lai
1.2.2 Oxytocin
(12)HOA SINH LAM SANG
1.3 13§nh ly tultin yen
1.3.1 Suy tuydn ydn &war
La thieu but chirc nang tuyen yen tn.rdrc lam anh huOng den cac hormon tuyen yen Phan loai:
Suy tuyen yen toan phan: c6 phi hop vai suy thily sau tao nen benh canh suy toan b6 tuyen yen
Suy tuyen yen tirng phan: c6 loai
- Don dOc: chi mot loaf hormon bi thieu hut - Mei hop: it nhat hai hormon bi thieu
1.3.2 U tuydn yen
La u to bao mo tuyen yen hay u tir cac di tich pilot thai tuyen yen (tai Rathke)
Phan loci:
- Lanh tlnh tire cac adenom (u tuyen) tuyen yen: c6 hai nhom la (1) kh6ng bieu hien not tiet, loai chiem 20% va (2) Well hien not tiet hay goi adenom chirc nang Loaf adenom chirc nang 14i chia nh6m: nguyen phat la adenom tiet met hay,nhieu hormon tuyen yen va thir phat la suy cac tuyen ngoai bien, vi du suy tuyen giap lau khong dieu tri se gay adenom tuyen yen tang blest TSH - Ac tinh hay u throng mO: dang nguyen phat rat hiem, throng la thin Oat ttr K vu,
K phoi, loan san nhu u SO halt hay hiem han nhu u quad, u day song
Bieru hien bang hai koi chung:
H6i chimg not tiet:
Throng la bieu hien d'au ti'en bang tinh suy hay cu6ng tuyen yen hay c6 hOi
chimg than kinh - 6i
- Adenom kitting tiet hormon: cac hormon throng la gfoi han Binh throng Dang tier' trien chant va hay tai phat sau dfeu tri
- Adenom tuyen yen tiet hormon (u tuyen chic nang): try theo hormon &roc tiet LH, ACTH
Nguyen Oat: benh Cushing Thu Oat: h6i chimg Nelson
LH-FSH not chung la hien' c6 the nguyen phat, thir Oat gap sau suy sinh due lau khong duoc dieu tri
(13)CHUO'NG 61 HOA SINH LAM SANG BENH LY TUYEN NOI TIET
Prolactin: la adenom tuyen yen tiet qua mire PRL, la loci ademon tuyen yen pile Nen nhat chiem 40% tong s6 adenom tuyen yen
TSH
Nguyen phat: cac hiem
Thir phat: suy giap lau khong dieu tri tat
- U thucmg m6: thir phat hay gap han nguyen phat (rat hiem), di can vao tuyen
yen gap 25% tru6ng hop K vu, thuemg khong c6 bieu hien not tiet met vai
truemg hop gay dal than nhat va sau la suy yen
Hei chUng khoi u: kich thuac va huang phat trien khei u ma c6 Neu hien khac
1.4 Them chirc nang tuy6n yen
Tuan theo met so nguyen tac - Khdo sat hormon tuyen yen:
Dinh lucmg tinh: can chU3'7thay doi n'eng de theo nhip sinh hoc Dinh lucmg deng: cac nghiem phap fic the hoac kich thich
- Kith() sat dap Ung‘ngoai vi: kich thich tuyen dich true tip hoac ca quan thgc hien bang hormon tuyen yen thich hap
- Khdo sat hieu (mg cua hormon: dinh lucmg hormon tuyen dich Trong bai chUng toi trinh bay chn yeu la khdo sat hormon tuyen yen
1.4.1 Tham d6 chav nang U67 yen
1.4.1.1 Chur nang tit GH
GH &rex tiet bed tien yen theo xung nhip, viec bai,tiet anh huemg bari nhieu yeu to nhu nhip dem, van deng, stress, dinh du6ng, nong glucose mau, va chiu sly lieu khien cua cac hormon vimg doi, IGF-1 cua gan, Do do, c6 su thing lap gifta ranh gi6i binh thuong va benh ly ve Deng de GH, met xet nghiem nong de GH ngau nhien khong giup ich cho chan loan, ca a nguai binh thu6ng De danh gia tinh trang lam sang lien quan den tiet GH nguai to khuyen phai phoi hop cac xet nghiem khac (vi du IGF-1, TSH, ff4, fTs) va vai cac nghiem phap khich thich hay irc the fiat GH
Ky thuat: tucmg to cac hormon tuyen yen khac
Mau dung huyet (co the &mg huyet tucmg theo yeu cau cua nha san xu't thuOc thir), de &Ong to nhien va tach huyet De a 4°C duac 48 gier, lau hem phai tril a -20°C Cho phep long den lan
Tri se tham khao: mk thu thap tit 214 nguai truemg cho mau tir mot nghien
(14)HOP, SINN LAM SANG
Bang 6.1 Wing trung binh GH
DOi tugng N Trung binh Trung vi 5th-95th
0,75 0,05 0,21
0,06 — 6,88 0,02 — 1,23 0,02 - 4,77 Dcm vi thuerng dung la ng/ml va p,IU/ml, he so chuy6n d6i: ng/ml x3µ1U/ml MOt so nguang nhu sau dang dugc ap dung:
Binh thugng < ng/ml nguoi truer/1g lay mau kw gig sang
Neing clO,GH rat nhay voi cac kich thich, ten ding mot ngugi nghiem phap phai thuc hien nhieu lan hoac phoi hop it hai nghiem phap truck ket luan: arginin-insu-lin, glucagon-propranolol hay betaxolol
Khi danh gia phai xet den su giam tiet tueii hoac có cac thieu but not tiet phihop Dinh luting IGF-1 tirc somatomedin-C:
La cach danh gia, gian tiep sg tiet GH, (lac biet có su khong thOng nhat giita ket qua Binh luting GH va lam sang nhu hOi chimg Laron
MOt so nghiem phap lien quan - Nghiem phap kich thich:
Chi dinh: có nghi ng6 thieu GH nhu tre phat trien cham va lun ham tre cimg trang lira hormon giap binh thuong (suy giap ding lam chum phat trien), X-quang thiremg quy thyxuang chum phat hien O ngugi trirang bieu hien bang met mai, giam dam xuang va rei loan lipid Tuy nhien can nh6 la thieu GH la benh hiem va benh a ngugi 16n thugng la có tien can thieu GH tir nho hoac benh dm doi - tuyen yen, do can có chi dinh cila bac s'Sr chuyen khoa sau da loai trir cac benh gay benh canh Wong ter va Binh luting cac hormon lien quan
Thuc hien: mau man tinh mach dirge lay sau nhin an 10-12 gig (qua dem), sau tiem chat kich thich tiet GH, cac mau mau se clugc lay each khoang 30 phut gi6 de Binh luting GH Cac chat gay kich thich:
+ Deng van -adrenergic: dimg clonidin mg (100 g/m2 tre)_uOng Dap img xay sau 60 phut Ca the la kich thich tiet GH-RH
+ Doi van -adrenergic: propranolol hoac betaxolol, lieu 30-40 mg uemg Dung phi hop vai glucagon de tang six marsh, co the ging &mg van -adrenergic
+ Gay throng huyet bang insulin dirgng tinh mach, lieu 0,05 — 0,1 clan vi/kg Can theo doi chat the lam sang va duorng huyet, có the phihop vai arginin Ca the thong qua N adrenergic
(15)CHU'O'NG ► HOA SINN LAM SANG BENH LY TUYEN NOI TIET
+ Glucagon: 0,03 mg/kg tiem tinh mach, throng phi hop vai propranolol + GH-RH: µg/kg tiem tinh mach, dung nap tot
K6t qua: duac xem la binh thuang ne'u dinh GH = ng/ml (21 gIU/m1) sau lam nghiem phap kich thich
nghia: nong de GH khong dap irng (thap hon mire no nen co) vai nghiem phap kich thich, nong de IGF-1 thap a doi tuong c6 trieu chimg thieu GH se gifip ket luan benh nhan thiL GH va quye't dinh dieu tri
- Nghiem phap ire die:
Chi dinh: nghi nga hei chimg khong le (gigantism) er tre em hoac to dau chi (acro-megaly) a nguai tn.rang thanh, nghi u tuyen yen, phoi hop vai nong de IGF-1 danh gia di'eu tri benh 1)'T tuye'n yen va theo del tai phat
Cach tin hanh Prong tir nghiem phap kich thich, cac chat ix ch6 gem: + Tang dueng huy& bang Ong 75 g glucose
+ Somatostatin 100 gg tiem clued da
Ket qua: binh thtremg nong de GH < ng/ml (< uIU/m1) sau lam nghiem phap ire che
Y nghia: nong de GH sau irc che van cao hon mUc le no nen c6 (nghiem phap throng tinh) a nguai CO bieu hien hei chimg khong le hoac to dau chi kern theo IGF-1 tang giup chan doan xac dinh benh Ne'u nghiem phap duong tinh or nguai dang theo doi benh sau di6'u tri thi c6 the la truemg hop tang tiet GH tai phat
1.4.1.2 ChCfc nang tiet ACTH
Lay mau: rat can than vi no rat nhay vai six thay del protein Do can lay mau dung ca.ch nhu bao quan lanh, cheng dong EDTA
Cac nghiem phap lien quan:
Nghiem phap i c che bang dexamethason: dexamethason la met steroid nhan tao, c6 kha nang ire che tiet ACTH sinh ly tai tuy6n yen, hau qua la cortisol va cac corticosteroid se bi
Nghiem phap kich thich:
+ Nghiem phap metyrapon chat irc che khau cuei tong hop cortisol lam chat giam di va Nu qua lam tang tiet ACTH (gian tiep) cua tuyen yen + Nghiem phap kich thich bang CRH (corticotropin releasing hormon
(16)Tuoi sinh san Man kinh
HOA SINH LAM SANG
nghia: tang tiet ACTH gap thieu but CRH cita viing doi va kitting Wet ACTH gap suy tuyen yen Nghiem phap giup phan biet benh Cushing (tang tiet ACTH va cortisol) \Teri cac truerng hop u lac ch76 tiet ACTH va 116i chirng Cushing tuyen thuemg than (ACTH va cortisol khong dap Aug)
Chit 3'7: dap irng thay d6i theo gio ngay, bu6i chieu ACTH va cortisol tang nhieu hon
+ Nghiem phap kich thich bang Lysin-vasopressin (LVP): LVP la d6ng van cua AVP c6 tac dung nhu CRF/CRH, kich thich tiet ACTH va cortisol Hien nghiem phap khong diing vi dap Aug kern \Ta nhieu tac dung phu
Tri so tham khao: mau huyet ley vao buoi sang a 252 ngued kit& manh tien hanh dinh lugng PRL bang phuong phap CLIA
Bang 6.2 Ntrig cit) trung binh ACTH
Dun vi chuyen d6i: ng/ml = 21,2 mIU/L Nghiem phap lien quan:
It có gia tri chan doan nhung lai c6 gia tri dieu tri Nghiem phap kich thich bang TRH: neu la adenom prolactin thi chi tang nhe lam nghiem phap, ngugc lai se biing no da tiet prolactin phan *rig
Nghiem pita') dimg danh gia mire dO suy tuyen yen, su thieu but prolactin thuemg xay muon
Neu prolactin binh thuOng hay chi tang nhe sau kich thich thi cac bieu hien suy tuyen yen la Mi
MOt s6 nghiem phap khac nhu ire the thu the dopaminergic (sulpirid, domperidon), gay kich thich tiet PRL, bien d6 kich thich giam cac twang hop khoi u Nghiem phap xac dinh tinh nhay cam voi dopaminergic nhu parlodel va hieu qua dieu tri Test vOi clan xuat dopamin (L-DOPA 500mg u6ng)
1.4.1.4 Chirc nang tiet FSH, LH
Dinh lugng FSH va LH
Phuang phap: RIA, ELISA, CLIA, FPIA
Noi chung cac the N may sinh boa mien dich tu dOng hien deu co the dinh him
(17)LH (n=183) FSH (n =251)
KT: CILA/Liaison - Nang
- Phong noan - Hoang the - Man kinh
* Khong dung hormon *Ming hormon thay the
3,5 - 9,2 mIU/m1 4,7 - 25,9 mIU/m 1,7 - 5,6 mIU/m1 13,9 103,1 mIU/
1,7 - 96,8 mIU/m
1,9 - 9,2 mIU/ m1 6,1 -49,1 mIU/m1
1,3 -10,8 mIU/ml
1,3 - 11,8 mIU/m1
Nam Ulml
15,4 - 53,3 mIU/ml 0,7 - 52,7 mIU/m1
CHU'O'NG Ow HOA SINH LAM SANG BENH LY TUYEN NOI TIET
(FSH) thay d'6i My may va kyr thuat Lay mau va tilt:
Mau huyet hoac huyet tuang có ch6ng &Ong EDTA dugc dimg, mau phai ly tam va thch huyet (huyet tuong) Dung ngdy, neu khong phai de lanh ngay, de 4°C dugc 24 gig, lau hon phai trir - 20°C Khong dugc &Ong lap lai va phai • deu truck thin
1\1-611g dg thay d6i theo chu kST kinh nguyet va thai kST nen in& chit y ban Juan Gia tri tham khao: d6i tugng nguoi khoe manh, thy mau bu6i sang
Bang 6.3 N6ng d6 trung binh FSH va LH
Nghiem phap kich thich bang Gn-RH/LH-RH danh gia kha Wang hoat dOng cua tuye'n yen va cho phap phan biet six thieu but la & doi hay tuyen yen Sau‘kich thich neu tuyen yen dap (frig binh thuong hay chi giam nhe thi nguyen nhan doi, neu khong dap (mg, ca sau nhac lai thi nguyen nhan thugc tuyen yen Dap img cna LH doi vgi Gn-RH thay doi then chu lcST kinh nguyet,,dap img giai doan hoang the gap doi giai doan nang Dap Ung tit FSH khong thay doi theo IcST kinh
Tian hanh: 100 µg Gn-RH dugc tiem tinh mach hay clued da Mau mau n'6ng dg FSH, LH dugc lay vao cac thoi diem 0, 15, 30, 45 Va 60 phi&
Ngdy vai cac xet nghiem dinh lugng FSH, LH có dg nhay cao, de clang xac dinh cac mitt ni5ng dg thap (dO' nhay phan tich tot) lam cho nghiem phap it dugc &mg
1.4.1.5 Chipc nang ti6t TSH
Dinh lugng hormon TSH: ngdy nlig irng dung ky thu4t cap khang the don clang dugc chon loc giup tang dg nhay cua xet nghiem dinh lugng TSH (test TSH sieu nhay), nhg ma nghiem phap TRH it can thiet hon
Nghiem phap kich thich bang TRH:
Chi dinh: TSH giap g gigi han clued hoac lam sang nghi ngg suy giap
(18)HOA SINN LAM SANG
Cach thgc hien: tiem tinh mach 200g TRH (protirelin) se lam tang n6ng dO TSH, t6i da sau 30 phut va trey v6 mire ban dau phut thin 120
Then vai ngtr6i c6 tin can hen, b6nh phi tic man tinh, suy tim cung luting thap, c6 thai
)".(- nghia: ginp chan loan suy giap c6 nguyen nhan tuyen yen hay d6i Ngay hau nhu khong dung nghi6m phap vi protirelin khong throe san xuat
1.4.2 Tham d6 chav nang thily sau tuyen yen
Dinh luting AVP huy& tuang:
- Mau tinh mach dung ch6ng dong heparin hay EDTA gift 4°C
- N6ng dO binh thuang thap 1- ng/ml, ti& AVP thay d6i theo nhip bai tit nen dinh luting don dOc it c6 gia tn
Dinh luting AVP nuac
- DUng nuac tieu 24 gi6, d6 lanh 4-10°C Nhtroc di6m la cac true:mg hop benh 1ST c6 luting ntrac tieu qua nhi6u (thir6ng > 2,5 lit) se lam giam n'ong dO AVP xuong du6i dO nhay cua xet nghi6m
M6t s6 nghi6m phap lien quan:
Nghiesm phap kich thich nhin uong: nage Bich la tang dO tham thau huyet tuang gay kich thich tit AVP Dinh luting AVP mau, AVP nirac tieu c6 th6 dinh luting kern theo Khi nhin u6ng tit 6-8 gi6 (hoec den mat 3% luting co the) se lam tang thai AVP qua duong nieu va giam luting mrac tieu, tang dO tham than
Nghi6m phap phai dirge thgc hi6n 136nh vin bai bac si (c6 nai den hed phai c6 chuyen gia lam nghi6m phap nay) Trong qua trinh thgc hi6n phai theo dOi can neng, than nhi6t, huyet ap va cac dau hi6u mat nuac cap Tinh tang mat nuac hay xay som ngir6i dai thao nhat
Chi dinh: nghi ng6 dai thao nhat (tieu chuan "yang" cho chin doan) va giup phan bit nguyen nhan cua dai thao nhat (do Ply& yen hay then) Chu ST chi dinh kha han ch6 va khong can tin hanh tren cac ct6i tuong c6 bi6u hi6n tuang d6i re, vi du da niesu ten b6nh nhan tin can c6 pheu thuet \Tung sang SO hay chan thuong so nao kem theo tang natri mau va tang ap lgc tham thau mau thi khong lam nghi6m phap
Khong nen chi dinh ngu6i suy then, tang natri mau, tang ap lgc tham than man, dai than duemg khong ki6rn soar, giam the tich tuan hoan, suy tuyen yen phoi hop
Ti6n hanh: lay man mau vao cac gi6 0, hay va gi6 (1(6t thiic nghi6m phap) d6 lanh Nuac tieu tuoi dirge lay mizii gi6 va gift lanh Th6 tich nu& tieu, ap lgc tham thau mau va ni6u throe
(19)THOY TRAI TUYEN
GIAP
THOY PHAI TUYEN
GIAP
CHU'O'NG OA- HOA SINH LAM SANG BENH LY TUYEN NOI TIET
- Nghiem phat kich thich vai AVP (desmopressin)
Dimg phan biet nguyen nhan dai than nhat trung acing hay than
Tiem duoi_da 2gg desmopressin, ap luc tham thau mau ya nuoec lieu, the tich nu& tieu &roc mai gia gig
Ket qua: neu nguyen nhan trung uang se lam giam the tich nu& tieu ya tang ap Arc tham thau > 50% Ngugc lai neu than thi it có dap Ung, the tich nuac lieu khong doi, dp luc tham thau khong tang hay tang < 50%
- Nghiem phap Ire the tit AVP: tang ganh were
Benh nhan dugc huang dan nhin an lir chieu horn truck, tieing nuac nhu binh thuemg Sang cho benh nhan di tieu het, cho uong nuac loc 20m1/kg 20 philt Lay nuac tieu mai gig gia giir lanh Lay mau mau lac bat dau, gitta ya cu'Oi nghiem phap
Nghiem phap dUng hoac diphenylhydantoin tiem tinh mach da bi be)
2 DANH GIA CHOC NANG TUYEN GIAP
2.1 Chivc n5ng sinh ly tuy-en giap
2.1.1 So' luvc cgu frac tuyen giap
Hinh 6.5 Ca the hoc tuyen giap
Tuyen giap la mot tuyen not tiet quan cua ca the
,Tuyen giap (TG) nam clued hai ben ya phia truac quan
(20)HOA SINN LAM SANG
Moi thity dai tir - 8cm, rOng - 4cm, day - 2,5cm Hai thity can nang binh thuong khoang: 40 - 42g Khi tuy6n giap phi dai tao buOu giap
Hinh 6.6 CuA tao mo hoc tuyen giap
COu tao mo h9c: TG duoc tao bed hai loai t6 bao: t6 bao nang va t6 bao quanh nang
T6 bao nang la don vi tong hop va bai tiet hormon tuyen giap: thyroxin va triiodothy-ronin MOi nang có hinh cau &rang kinh to 100 -300 Rm, bao boc mot chat keo (chat tiet) gom chit yeu thyroglobulin, duce tiet bai tuye'n giap Thyroglobulin la mot glycopro-tein china iod va la co chat quan cho su gan iod vao tyrosin qua trinh tong hop hormon giap Ngoai chat keo china mot luting nho thyroalbumin china iod
Thanh cua nang tuyen la nhang t6 bao bi6u mo hinh tru, bai ti& chat ti& vao king nang
Hormon giap dirge hap thu trO lai qua to bap bieu mo tuyen giap vao mau va phan phi khap co the
T6 bao quanh nang (holac t6 bao C): t6 bao C khu tit gifta cac nang ho4c dinh lien vai cac nang T6 bao C san xuat calcitonin, mot hormon peptid co vai trO sty di6u hoa calci a co the
2.1.2 Chin ming sinh ly ctia MAI giap