Y Hpc TP. H o Chi Minh » T$p 20 * S£( 1 * 2016 Nghien cihz Y hpc
TINH TRANG SUY GIAP 6 TRE MAC H O I CHXJNG THAN HU*
KHANG CORTICOID
Hodng Vinh An*, Vd Huy Tru**
TOMTAT
Muc tieu nghien cdu: Ddc diem lam sdng, can Idm sdng dia Unh trtmg suy gidp (SG) vd suy gidp die&i Idm sdng (SGDLS) a tre mdc hdi chimg than hu khdng corticmd (HCTHKC) tai Bmh viin Nhi dong 1.
Phmmg phdp n^en diu: Md td hdng loat ca.
Kei qud:C6 27 tru&ng hap HCTHKC tai Benh viin Nhi ddng 1, TP. Ho Chi Minh tie 01/06/2014 dm 30/04/2015. Ti le SGDLS Id 22,2%, ti le SG Id 11,1%. Benh nhan HCTHKC Ihong co triiu chieng SG. Gid tri trung vi cua TSH mdu 3,44 plU/ml, khoang tie phan vi 1,67 - 5,76 plU/ml, ndng do T4 mdu trung binh 5,26 ± 3,02 pg/dl, nong do FT4 mdu trung binh 1,23 ± 0,42 ng/dl. Tieu protdn nhieu liin quan giam T4 vd tang TSH mdu (>6,3pTWml). Benh nhdn SG co FT4 mdu giam (<0.8 ng/ml) nhung TSH mdu binh thu&ng (0,6 - 6,3 pJU/ml). Co moi tuang quan giiea nong do T4, FT4, TSH vd albumin mdu.
Kei ludniSG vd SGDLS a binh nhdn HCTHKC liin quan tieu protdn nhim. Binh nhdn bi SGDLS khong trim chdng, chi tang TSH vd giam T4 mdu. Binh nhdn bi SG bim hien giam FT4 mau nhimg TSH mdu binh thudng. Sau 3 thdng dieii trj HCTHKC, tinh trang roi loan chiec ndng tuym gidp cai thien.
Til khod: Hoi chieng th^ hu khdng corticoid, suy gidp, suy gidp dudi l&m sdng.
ABSTRACT
HYPOTHYROIDISM IN CHUDKEN WTTH STEROID RESISTANT NEPHROTIC SYNDROME Hoang Vinh An, Vu Huy Tru* Y Hoc TP. Ho Chi Minh * Vol. 20 - No 1 - 2016:81 - 85 Objectives: The clinical and laboratory characteristics of hypothyroidism and subclinical hypothyroidism in children with sterioid resistant nephrotic syndrome.
Methods: Case series study.
Results: From June 1", 2014 to April 30^, 2015, there were twmty - seven childrm with sterioid resistant nephrotic syndrome in Children's hospital 1, Ho Chi Minh dty. The prevalence of subclinical hypothyroidism was 22.2%, the prevalence cf hypothyroidism was 13.3%. Children vnth sterioid resistant nephrotic syndrome didn't have symptom ofhypothyrmdism. The median serum TSH value was 3.44 pIU/ml,interquartile range was 1.67 - 5.76 plU/ml; the mean serum T4 concentration was 5.26 ± 3.02 pg/dl and the mean serum FT4 concentration was 1.23 ± 0.42 ng/dl. Massive protdnuria related to law T4 and high TSH. Children vnth hypothyrmdism had low serum FT4 (<0.8 ng/ml) but normal serum TSH concentration (0.6 - 6.3 pIU/ml).There was correlation betwem serum concentration qfT4, FT4, TSH and albumin.
Ccmclusions: Hypothyrmdism and subclinical hypothyrmdism in childrm with steroid resistant nephrotic syndrome related to massive protdnuria. (Jiildrm with subclinical hypothyroidism didn't have symptoms of hypothyroidism but had high serum TSH and law serum T4. Children with hypothyroidism had low serum FT4 but normal serum TSH. Three months after the initial of treatment of steroid resistant nephrotic syndrome, thyroid dysfunction had improved.
Keywords: Steroid resistant nephrotic syndrcmie, hypothyroidism, sid)clinical hypothyroidism.
* B§nh Vi?n Nhi Ddng 1, TP.HCM **Bp mon Nhi, Dgi hoc Y IXrcrc TP Ho Chi Minh Tdc gid liin lac: BS. Hoang Vinh An DT: 0976276468 Email: [email protected]
N h i Khoa 81
N g h i e n c m i Y hpc Y H p c TP. H o Chi M i n h * T a p 20 * Sfi 1 * 2016 D A T V A N D E
Hpi chiing than hu (HCIT^ la mot trong nhiing hoi chiing benh cau than thuong gap nhat a tre em, da so nguyen phat va co sang thuong toi thieu.
Khoang 10% deh 20% tre HCTH nguyen phat khang corticoidf^y. Tre mac HCTH, dac biet la HCTH khang corticoid (HCTHKC) bi nhieu bien chung, trong do co giam honnon tuyan giap. Da so tre HCTH chi suy giap duoi lam sang (SGDLS), chiic nang t u y a i giap tro ve binh thuong khi giam tieu protein, tuy nhien mpt so' truong hop bi suy giap that su (SG). Tinh trang SG va SGDLS deu anh huong den hoat dgng a i a co theP^^^.
Tren the gjoi, cac nghien a i u ve chiic nang tuyoi giap 6 benh nhan HCTH va HCTHKC con it Tai Viet Nam, chting toi thay chua co n ^ e n ciiu nao ve chiic nang tuyai giap 6 tre mac HCTHKC Ben canh do, benh nhan HCTHKC nguy CO cao bi SG nhung chua dupe theo doi chiic nang tuyoi giap dinh ky. Vi vay, chiing toi ti&i hanh nghien a i u nay vdi cac muc tieu:
Xac dinh ti le SG, SGDIS tai thoi diem tre moi dupe chan doan HCTHKC va sau 3 tiiang dieu tri.
Mo ta cac dac diem ve dich te, lam sang, can lam sang lien quan tinh trang SG, SGD15.
Xac dinh moi lien quan giiia nong dp T4, FT4, TSH mau vdi tinh trgng tieu protein, albumin mau.
P H i r O N G PHAP N G H I E N CLfU Thiel ke nghien cmi
Mo ta hang loat ca Doi t u o n g n g h i e n cmi
Benh nhan tii 1 tuoi deh 15 tuoi, nhap Khoa Than, Benh vien Nhi dong 1 trong khoang thoi gian hi 01/06/2014 deh 30/04/2015, duoc chah doan HCTHKC.
Tieu chi dtra vao
Benh nhan da dupe chain doan HCTH dua
vao cac tieu chuan saui'J;
- P h u
- Tieu protein nguang than hu: Protein nieu 24 gio >50 mg/kg/ngay (>40 mg/m2/gi6,
>1 g/m2/ngay) hoac ti 1# protein/creatinin trong m l u nuoc tieu bai ky >2 mg/mg (>2G0 mg/mmol) hoac que thu mxac tieu co protein
>a4- hoac >300 mg/dl.) - Giam albumin mau <2,5 g/dl.
- Tang cholesterol mau >200 mg/dl hay >5,18 mmol/L
Chah doan HCTHKC dira vao tieu chuSi benh nhan da dirpc chan doan HCTH, da dieu tri Prednisone 2 mg/kg/ngay (toi da 60 mg/ngay) tea thieu 8 tuan nhung khong dat lui benh hoan toan (ti le protein/creatinine trong mau nuoc tieii bat ky <0,2 mgAng (<20 mg/mmol) hoac protein nieu (-) hay vet tr&i que thu nxxac tieu trong 3 ngay lien tiep)''^.
Tieu chi loai ra
- HCTH do nguyen nhan thii phat (viem gan sieu vi B man tmh, sot ret. Lupus ban do he thong, Henoch-Schonlein, lymphoma...).
- B&ih nhan da duoc chan doan SG hoac gai y SG mac phai tnroc khi khdi phat HCTH
- Gia <finh benh nhan khong dong y tham gia nghien ciiu.
l i e u chuan chan doan s a y g;ap d u o i lam sang
FT4 binh thuang theo tuoi, TSH tang theo tuoi, khOTig trieu ehiing lam sangfl2».
Tieu chuan chan doan soy giap
FT4 gjim theo tuoi, TSH tang, giam hoac binh thuang theo tuoi, eo hoac khong trieu chiing lam sang^'^.
Bang 1: Nong do harmon tuym gidp iheo tuoM Tuii
l - 5 t u 6 l 6-10tu6i 11-15tuSi
TSHUUM) 0.6-6.3 o.e-6.3 0,6-6,3
ntngMI) 7,3-16 6,4-13 5.5-12
rmtmm
0,8-2.0 0.8-2.0 0,8-2,0
82 C h u y e n D e Sire K h o e S i n h S i n - Ba M e Tre Em
Y H p c TP. H o C h i M i n h * T^p 20 * So 1 * 2016 N g h i e n ciru Y hpc KET Q U A V A BAN LUAN
Tix 01/06/2014 dSi 30/04/2015, eo 27 b | n h nhan HCTHKC tu 2 tuoi deh 15 tuoi dupe dua vao nghien a h i , trong dd, 19 b&ih nhan dupe chiing t6i khAo sat t ^ 2 thoi diem: mdi chah doan HCTHKC va sau 3 thang dieu tri 8 benh nMn dupe khao sat 6 thcri diem liie mdi chan doan HCTHKC. Ket qua ghi n h | n dupe nhu sau:
D^c diem dich te
Bdngl: Dac diim dich te lien quan tinh trang SGDLS
Ti le suy gjap va suy giap d u o i lam sang Khi mdi dupe chan doan HCTHKC, 6 truong hop SGDLS chiran ti le 22,2%, 3 truong hpp SG chiran h le 11,1%. T\ le SGDLS ciia chung toi thap hon ket qua nghien ciiu ciia Kapoor la 3(W^. Sau 3 tiiang, chung toi khong thay truong hpp nao SG va SGDLS.
Yeutd Tuoi khoi b^nti HCTH Tu6i Qitqfc ciiln floSn HCTHKC
Tuydn gi&p binh tttuvng (n = 18) 7,83 ±4,58 10,06 ±4,11 Nam/nii | 11/7
SGDl^(n = 6) 5,17 ±3,70 6,00 ±4.29 3/3
- P 0,212*
0,050*
0,065***
*PhepJa^T mdu ddcl&p,***Phepkxm Fisher Nhom benh nhan bieu hien SGDLS eo tuoi dupe chah doan HCTHKC la 6,00 ± 4,29 tuSi, nhd hon tu6i dupe c h ^ d o ^ HCTHKC trung binh eiia nhom binh thuong (p= 0,05). Khong cd su lien quan giua gioi tinh va tinh trang SGDLS
Tneu chiing thuc Ihe
Bdng3: Triiu chieng thuc th^liin quan SG
D | c di^m lam sang Trieu chiing ca ndng
Benh nhan trong nghien ciiu eiia ehiing toi khong CO trieu chiing co nang ciia tinh trang SG nhu tao bon, sp lanh, da kho, tdc thua de rung, met mdi, kem linh hoat. Ket qua nay tuong tu vdi nghien ciiu ciia Kapoor^^.
Y«uU Chilu cao thSp
Mach chdm Tang huyet Sp Giim phSn X9 gSn cc
Giam tn/cng 1 ^ ca
Tuy4n gidp binh t h u ^ g (n = 18) 5/18 (27.8%)
0/18 9/18 (50%)
0/18 0/18
SGDL.S(n = e) 0/6 (0%)
0/6 4/6 (66,6%)
oie 0/6
P 0,280"*
0,380"
*** Ph^ kiSn Filter
Tat ca benh nhan HCTHKC trong m i u nghien ctiu cua chung toi cd tan so mgch, phan xa gan co va truang luc eo binh thuong. Chiing toi chua ghi nhan moi lien quan gjiia tinh trang SGD15 vdi trieu chiing chieu eao thap va tang huyet ap.
D^c diem can lam sang
Nong dp albumin mau co gia trj trung vj 1,57 g/dUhoang tii phan vj 1,27 - 2,70 g/dl.
Nong dp cholesterol mau trung binh 1^2 ± 6,13 mmol/l-
Nong dp TSH mau cd gia tri trung vi 3,44 plU/ml, khoang tii phan vi 1,67 ~ 5,76 plU/ml.
Nong dp T4 mau trung binh 5,26 ± 3,02 fig/dl Nong dp FT4 mau trung binh 1,23 ± 0,42 ng/dl.
Can Idm sdng lien quan SGDLS
Nhdm SGDLS cd nong dp albumin mau thap hon nhung cholesterol mau cao hon so vdi nhom tuy&i giap binh thucmg (p <0,05). Ket qua nay goi y SGDLS lien quan tinh trang tieu protein nhieu gay mat albumin, cac enzyme thuy phan lipid, eae protein van chuy&i hormcm tuyai giap
N h i K h o a 83
Nghifin cthi Y hpc Y Hpc TP. Ho Chi Minh * T|p 20 * S£( 1 =*
qua nude tieu, lam giam nong dp hormon tuy&i giap dSn den tang TSH. Chiing toi chua ghi nhan mdi U^n quan gjiia dO Ipc cSu thin vk SGDLS (p= 0,665).
Nhdm SGDLS cd nong dp albumin mau thap hon nhung cholesterol mau cao hon so vdi nhdm tuyrai gjap binh thuong (p <0,05). Ket qua nay Bang 4: C&n l&m sdng lien quan SGDLS
gpi -y SGDLS lien quan tinh ti:ang tieii protein nhieu gay mat albumin, cac enzyme thuy phan lipid, cac protein v3n chuyai hormon t u y ^ gjap qua nudic tieu, lam gjam nong dphormon tuyoi gjap dan d m tang TSH. Chung ^ i chua g ^ n h | n moi lien quan giua dp Ipc cau thgn va SGDLS (p= 0,665).
vSutS; •;.••?,. , . /\lbumrn mdu (g/dl) Cholestercl mdu (mmol/l)
GiamioccSuth^n TSH (ulU/m?
T4(Mg/dl) n"4 (ng/dl)
Tuyin gidp binh t h i f ^ g (n = 18) 2,28 (1,35-2,97) 10,68(8.52-15,36)
7/18 (38,9%) 1,81(1.60-3.60)
6.4012.77 1.38 ±0.40
SGDl£(n = 6) 1.25(1.06-1.63) 19.35(14.55-24.41)
3B(50%) 6.93(6.64-8.90)
3.01*1.84 1,04±0,18
IH5
0.020"
0.009"
0.665"*
0.001"
0.018*
0.056*
• Phep h&n T mSu dSc Idp, ** Phep Idim bAann - Whitney, '•
So vdi nhdm ehiic nang tuym giap Irinh thuong, nhdm SGDLS co nong do TSH mau eao hon (p= 0,001), nong d6 T4 mau th^p hon (p=
0,018), tuy nhien nong dp FT4 mau khdng cd su khae biet (p= 0,056). Nhu ehiing ta da biet, gan nhu toan bp honnon tuyen giap (99%) khi tiet vao mau dupe gan ket vdi eae protein van chuyen nhu TBG, TBPA va albumin, chi mot phan nho (1%) ton tai duoi dang tu do la FT3 va FT4'5'"'i. Cd the thay, hormon tuy&i giap mat qua nude tieu chii ye\x dudi d ^ g T4 gan k^t vdi protein v$n chuyen gay gjam nong dp T4 mau.
Theo eo che dieii hoa ngirpe, co the se tang san xuat TSH de duy tri 6n djnh nSng d6 honnon tuyen giap trong mau gay nen tinh trang SGDLS.
Tucmg quan TSH, T4, FT4 vd albumin mdu N&ig dp T4 va albumin mau co tuong quan thuan, kha chat (R= 0,60, p= 0,001). Fhuong tiinh du bao: Nong dp T4 (fig/dl) = 1,91 x nmig do albumin mau (g/dl) +1,48.
Nong dp FT4 va albumin mau co tucmg quan thuan, kha ehgt (R= 0,53, p= 0,005). Phuong trinh du bao: Nong dp FT4 (ng/dl) = 0,23 x nong dp albumin mau (g/dl) + 0,77.
Nong dp TSH va aB^umin mau cd tuong quan nghjch, miic dp vira (R= -0,^, p = 0,(B7). Hiuong tiinh du bao: Nor^ do TSH (fjIU/ml) = -1,26 x nong do albumin mau (g/dl) + 6,31.
Phep Jdmt Fisher
Dac tfiem cua b a b e n h n h a n suy gjap B?nh nhan thii nhat la nany, khdi benh HCTH tii nam 6 tu^i, duoc cheffi doan HCTHKC mu6n vao nam 14 tuoi. Benh nhan nay co diicu eao t h ^ so vdi tuoi va tang huyet ap tren 13m sang. Benh nhan cd tinh trang tieu protein nhieu, gjam albumin mau va tang cholesterol mau, gjam dp Ipe cau than, ket qua mo hoc la xo hoa cau than khu tni ttoig phan. Nong dp FT4 trong mau benh nhSn gj^un nhe 0,75 ng/dl nhung noog dp TSH 1,71 filLf/ml lai nam trong gidi han binh thuong tiieo hioi (0,6 ~ 6,3 ^ilU/ml).
Benh nhan thii hai ia nam, khdi b&nh HCTH tCr nam 2 tuSi, dupe chan doan HCTHKC mupn vao nam 3 tuoi. B^nh nhan nay cd chi^u cao binh thiKmg so vdi tuoi va tang h u y ^ ap tren lam sang. Ket qua xet n g h i ^ cho thay, benh nhan c6 tinh trang tieu protein nhieu, giam albumin mau va tang cholesterol mau, gjam dp Ipc cau thin, ket qua mo hpc la sang thuong toi thieu. Nong dd FT4 trong mau benh nhan gjam nhe 0,68 ng/dl nhimg nong dp TSH 5,03 [olU/ml nam trong gjoi han binh fhuong theo tuoi 0,6 |aIQ/ml deh 6,3 ^.tIU/Inl
Benh nhan thii ba la nii, khoi benh HCTH tir nam 2 tuoi, sau 2 tiiang dupe chim doan khang corticoid sdm. Benh nhan eo chieu cao binh thuong theo tuoi, tien tang huyet ap. Benh nhan
84 C h u y e n Oi Sure Khoe S i n h S a n - Ba M ? T r e Em
Y Hpc TP. Ho Chi Minh * Tap 20 * So 1 * 2016 Nghien ciiu Y hpc CO tinh trgng tieu protein nhieu, giam FT4 0,6
ng/dl nhung TSH 3,99 |x[U/ml trong gjcH hgn binh thudng flieo tuoi (0,6 - 6,3 ^lU/ml). DO Ipc cau t h ^ binh thucmg, ket qu^ mo hpc la sang thucmg toi fhieu.
Ca ba b?nh nhan SG deu rm loan huyet ap, hai truong hpp tang huyet ap va mpt trudng hpp tien tang huyet ap gpi y SG cd moi hen he vdi tang huyet ap mau. Hai trong ba benh nhan SG gieim dp loc cau than, ket cpia nay tuong d&ig vdi bao cao cua Iglesi^ la SG se anh hucmg len th$n, lam gjam dp loc cau than, giam chiic nang ong than nhu chiic nang pha loang nuoc tieu hay tai hap thu natri, tang ti^ reninRCa ba tnrong hpp deu giam T4, Fr4 va albumin mau kem tieu protein nhieii. Ket qua nay eiing eo eho n h | n djnh, giam hormon tuyan giap trong HCTH lien quan tinh trang tieu protein nhieu va keo dai'*''^.
Tuy nhien, ea ba benh nhan SG trong nghien eiiu ciia ehiing toi deu cd nong dp TSH mdu trong gioi han binh thuong theo tuoi (0,6 - 6,3 fjIU/ml). Nhu vay, tinh trang suy giap 6 b^nh nhan HCTHKC eo hen quan deax vimg ha doi va tuyai y&v dan den hien tupng TSH mau khong tang tiong khi Fr4 gjam. Nguyen nhan TSH khong tang phu hpp vdi tmh trang SG co th^ do mat TSH qua nude heaf^, dong (hdi vi?c sii dung corticoid keo dai 6 cac benh nhan HCTHKC gay giam tiet TRH gjam kich thich tuyai yen ti^
TSH vao mauP-^.
KETLUAN
SG va SGDLS a benh nhan HCTHKC li&i quan tinh tiang tieu protein nhieii va keo dai.
Cac b^nh hhsn SGDLS khong bieu hi#n trieu chiing, chi thay doi tien xet nghiem vdi tang TSH va giam T4 mau. Mot so benh nhan cd tinh trang SG bieu hien bang gjam FT4 mau, tuy nhien nong dp TSH mau binh thirong. Tinh tiang SG anh hudng d s i tar^ huyet ap va giam dp Ipc cau tiian 0" benh nhan HCTHKC. Cae roi loJin chiic nang t u y m gjap cai thien sau 3 thang dieu tri HCTHKC, tuy nhien can phai theo doi
lau dai de danh gia them. De ngju nghien a i u vdi sfif lupng Ion benh nhan de kMo sat tinh ti-gng SG, SGDLS 6 be bi HCTH v^ HCTHKC Can lam thSm xet nghiem djnh lupng nong dp TSH, T4, FT4 cac protein vgn ehuy«i trong mau va nudie tieu 24 gia. Can danh gia them cac y&u to lien quan tinh brang SG, SGDLS 6 tr^ HCTH nhu thai gian sii dung thuoc, lieu tich luy ciia corticoid va Cydosporine.
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Ngdy nhdn bai bdo- 20/11/2015 Ngdy phdn biin nhan xet bdi bdo: 22/11/2015 Ngdy bdi bdo duoc dang: 20/01/2016
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