Suy Giap Bam Sinh

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<ul><li> 1. SUY GIP BM SINH </li></ul> <p> 2. c im m phi - gii phuMm gip Pht trin tun 3 Di chuyn Tun 9 thai, c v tr vhnh dng c nh Pht trin bt thng: - tuyn gip lc ch - u nang gip 3. c im m phi - gii phu 4. C IM SINH L TUYN GIP BO THAI &amp; S SINH Thyroglobulin : tun 4 Tuyn gip hot ng tun 10 : T3, T4 TRH h i : tun 8 TSH tuyn yn : tun 10. Trong thi k u ca thai k: Tuyn gip khng ph thuc trc : Thai nhi v s/v tuyn yn:c T3, T4 Thyroglobulin xut hin trc TSH 5. Hot ng ca trc h i- tuyn yn - gip: - TSH tng cao s sinh suy gip - Bu gip tr c m dng thuc khng gip Hin tng tng TSH sau sinh: - TSH t ngt tng cao ln n 10- 15 ln mu rn - Cao nht l 30 pht sau sinh - T ngy th 3 sau sinh, TSH n nh - tui dy th nh lng TSH sng lc SGBS &gt; 3-5 ngy tui Hot ng HMG: Pht trin v trng thnh t bo no- T bo no c nhiu gen chu s iu khin ca hormone gip- Tng hp cc Protein ca Myelin v Neurone- Tng sinh ca cc ui gai v si trc, to ra cc sinap 6. Hormone tuyn gip v hng ro nhau thaiHormone T4 ca m bo v no thai nhi. hormone gip m c th truyn qua nhau thai Cc thai nhi khng c tuyn gip hon ton: - T3, T4 30% sau sinh bin mt sau 2 tun m SG thiu Iode thiu ht HMG thai nhi khi m tuyngip cha hot ng gy bnh n a phng Iode, TSI , IgG t m mc bnh Basedow qua cnhau thai 7. Tng hp hormone gipGiai on bt gi Iod ti tuyn gip T bo nang gip bt gi Iod bng mt c ch ch ng - bm iodur. Nng Iod ti tuyn gip cao gp 40 ln Iod trong huyt tng . Hu c ho Iod Iod nguyn t (I-) st nhp vo phn t TG khi c enzym peroxydase v nc oxy gi (H2O2) C nh 1 nguyn t Iod vo TG s cho Monoiodothyrosin(MIT)2 nguyn t Iod vo TG s cho Diiodothyrosin (DIT) . 8. Tng hp hormone gip Kt i cc Iodothyrosin (IT), hnh thnh cc Iodothyronin (ITRN) Nh xc tc ca enzym peroxydase, Kt i trong phn t TG gia 2 DIT -&gt; tetraiodothyronin(T4) Kt i gia 1 DIT v 1 MIT s to ra triiodothyronin (T3) T3 vT4 cn gi tn chung l Thyronin hay l (ITRN). Cc thuc khng gip c ch enzym peroxydase s c ch qu trnh oxy ho iodur v kt i cc IT. 9. Tng hp hormone gipGii phng cc ITv ITRN, MIT, DIT, T3 v T4 c d tr trong cc phn t TGNh qu trnh thu phn protein TG do tc dng ca enzym peptitdase. T3 v T4 c tit vo mu, Kh iod ho cc IT v ti s dng iodurCc IT c gii phng t TG b mt iod, do tc dng ca enzym Desiodase. Iod c gii phng li c t bo gip bt gi tham gia vo qu trnh sinh tng hp hormone gip mi. 10. Tc dng sinh l ca cc hormone gip Tc dng ln h thn kinh v tng trng - S pht trin ca no thi k bo thai ph thuc vohormone gip- Hormone gip kim sot cc gen iu khin tng hpprotein ca myelin v neuron. - Trong thi k s sinh, s kim sot ca hormone gipcng tng. - Hormone gip tc ng ti s pht trin v trng thnhca t bo no: s di c, tng sinh ca cc ui gai, sitrc, to ra cc sinap v cc bao myelin. Suy gip bm sinh, nu khng iu tr kp thi, chmpht trin tinh thn tr tu nng n. 11. Tc dng sinh l ca cc hormone gip Hormon gip v tng trng Hormone gip v hormone GH tng bit ho t bo, tng tc d pht trin ca c th. iu ho s pht trin ca xng, tc ng ln qutrnh to ra v trng thnh ca sn. Khi thiu hormone gip, cc im ct ho xut hinchm, c th chm pht trin . Trong suy gip bm sinh, nu khng iu tr kp thi, tr va b ln va n n 12. Tc dng sinh l ca cc hormone gip Tc ng ln chuyn ho Vitamin - Hormone gip thc y tng hp mt s Co-enzym t cc vitamin - Vitamin A c tng hp t Caroten. - Do , trong suy gip s tng hp vitamin A bgim i - Tng - Caroten lm cho tr c da mu vngsp. 13. Nguyn nhn suy gip bm sinh Tn sut mc bnh 1:4000 Ri lon cu to tuyn gip: 80-90% Khng c tuyn gip (athyreose): 30-45% Gim sn /lc ch tuyn gip: 40% Ri lon tng hp hormone gip: ( 1/40 000) Thiu hormone TRH / TSHSuy gip th pht Th th khng p ng vi hormone gip Thiu Iode gy bu gip suy gip: n a phng Ph nim M dng thuc khng gip, tia x, ha cht 14. Yu t nguy c ca SGBS Ma Gii: n Gen: HLA AW24 Khng c tuyn gip: Ttf1, Ttf2 v Pax8 , t bin PAX8 Ri lon tng hp HMG: ( th Pendred) t bin PDS gene (7q31) Lc ch- lon sn:khng c yu t di truyn c t mi trng Nhim trng : 15. Biu hin lm sng 16. Biu hin lm sng Ng nhiu, th To bn ko di Vng da ko di Khc khn Ph quanh mt. Li to Thp trc v sau rng 17. Biu hin lm sng Mt th, mi tt, mi mt dy Da kh Tc kh, d gy, tc mai mc thp trn v thi dng Rm lng lng Gim trng lc c Bng chng to Rn li, thot v rn 18. Biu hin lm sng 19. CHN ON SUY GIP S SINH o TSH h thng Dng giy thm lc vo ngy 3 (filter paper, day 3) Ly mu gt TSH cao:&gt; 50 mIU, o T3,T4, TSH TM v iu tr ngay L Levothyroxine TSH 30-50 mIU: XN ln 2 TSH TSH 5 im: nghi ngh XN TSH Scintigraphie T99: v tr v hnh thi TG nh gi tui xng : XQ gi 21. IU TR SUY GIP BM SINH Ngay khi chn on sng lc Mun cn hn khng iu tr sut i iu chnh liu thuc theo tui v cn nng Liu lng LT s sinh trung bnh :5 g/kg/ngy 22. THEO DI IU TR SUY GIP BM SINH Khm li hng tun cho n khi TSH bnh thng Khm li theo thng trong nm u Khm li mi 2 thng trong nm th 2 v 3 Khmm li mi 3 6 thng trong cc nm sau o TSH, FT4 Khm ton th : chiu cao, tr tu, tui xng. 23. Tin lngChn on sm v iu tr ng Chng trnh sng lc </p>