Vietnam Journal of Physiology 19(1), 3/2015 ISSN: 1 8 5 9 - 2 3 7 6 TlNH TRjBiNG LT S A T T R E N B E N H N H A N THALASSEMIA TAI BENH VIEN DA KHOA
TRUNG U-ONG T H A I NGUYEN NAM 2013
Nguyen Thu Hanh^'*, Nguyen Kieu G i a n g \ Pham Thj Thu K h u y e n \ Vi Thj Phuang Lan' T 6 M T A T : Mqc tieu nghien cuv nhim xac dinh ty le cic thi benh Thalassemia va dinh gii tinh trang if sit tren bgnh nhan Thalassemia tai Binh vien Da khoa Trung Wang ThSi Nguyin nam 2013. Ddi tuang vi phuang phip nghien euv: Nghien cim md ta cSt ngang, chqn miu cd chu dich. ed miu thuan tign, 48 bgnh nhin thiiu mau huyit tin duqc chin doan xie djnh la Thalassemia didu trj tqi khoa Nhi, Binh vign Da khoa Trung Wang Thii Nguyin trong thai gian nghiin cwu tir 1/1/2013 ddn 31/10/2013. Kit qui cho thiygip 70,8 % la 0-Thal, 25,0 % p-Thal phdi hqp HbE vi 4,2 % a-Thal. tyle binh nhw nhau a nam va nu; din tie it ngudi chiem 64,6%. Benh nhan din vien diiu tri chO yeu la tinh Thii Nguyin. trong dd huyen Ddng Hy ehiem ty le cao (39.6 %). Ndng do Hemoglobin luc vio vien thip, trung binh li 54,36 + 16,57g/l. B$nh nhan nghien cwu co ndng do sSt huyet thanh cao, trung binh la 26,3 + 18.2 pmol. Ndng do Feritin cao, trung binh la 1586,5 + 985,8 ng/ml. Cd 52,1 % bgnh nhan u sat (cd ndng do feritin > 1000 ng/ml).
Tip khda: Thalassemia, ir sit. feritin huyit thanh, binh Hb di truyin. thidu mau.
I . D A T V A N D t
Thalassemia la mfit hgi chung benh hemoglobin di truyin (bgnh Hemoglobin) do thieu hyt tdng hgp mgt hay nhieu chufii polypeptide trong globin cua hemoglobin.
Trigu chdng iam sang cua benh Thalassemia r i t da dang eo t h i thiiu mau r l l nang phai truyin mau thudng xuyen hogc CO t h i chf thilu mau nhg. Phuang phap dieu trj chii ydu cho benh nhan Thalassemia la truydn mau va thai s i t Vdi benh nhan t h i nang va t h i trung gian thudng gay ra tinh trang u sat lam tdn thuang te bao, mo va d i n d i n cac biln chdng nhu cham phat trien, suy tim, dai thao dudng,... [11,14]. Tinh trgng u s i t dugc chan doan chii yeu dya tren cac xet nghigm can lam sang nhu sinh thiit gan, chgp MRI gan de djnh luang dy tru s i t tgi gan hay xet nghigm s i t huydt thanh, tuy ' Dgi hgc Y dugc Thai Nguyen
* Tac gia chju trach nhiem chinh:
Nguyen Thu Hanh Email: hanhh htm®yahoo com Ngay nhan b^i. 31/10/2014 Ngdy nhan b^i phan bien 12/2/2015 Ngay chip nh§n dang: 8/3/2015
nhien, xet nghigm dugc su dyng rgng rai hign nay la djnh lugng nfing do Fentin huyit thanh [8,14] Tren thd gidi va d Vigt Nam da co nhieu cong trinh nghien cdu ve tinh trang u s i t d bgnh nhan Thalassemia, cac nghien cuu nay cung chi ro vai tro quan trgng ciia dieu tri thai s i t n h i m giam toi da tinh trgng u sat tgi mfi va giam cac bien chung do u sat, g6p phan nang cao chit luang cupc sdng va keo dai tuoi tho cho benh nhan Thalassemia. Bgnh vign Da khoa Trung Lfang Thai Nguyen la benh vign idn d khu vuc, thu nhan benh nhan tu cac tinh mien nui phi'a Bac, ty le benh nhan den didu tn bgnh co xu hudng gia tang. Tuy nhien, trudc day do chua co may dign di huyit s i c td de chan doan xac djnh t h i bgnh Thalassemia n6n benh nhan vao vign thudng vdi chin doan la thieu mau huyit tan. Benh nhan den chii y i u dieu tn bing truyin mau la chinh, thai sat chua duac chu trgng. Do vay, chiing tfii t i l n hanh nghien edu d l tai nay nhim muc tieu:
Vietnam Joumal of Physiology 19(1). 3/2015 ISSN: 1 8 5 9 - 2 3 7 6 1.Xac djnh tyle cie thi bgnh Thalassemia
gip tai khoa Nhi. Benh vign Da khoa Trung Wuang Thii Nguyin nSm 2013.
2 .Dinh gii tinh trang ir sSt cua bgnh nhan Thalassemia diiu trj Bgnh viin Da khoa Trung Wong Thai Nguyin nam 2013.
2. D 6 I nrqrHG vA PHifONG PHAP NGHIEN COU
2.1. Doi tu>gnig nghien c d u : Bfinh nhan thidu mau huydt tan dugc chin doSn xae djnh la Thalassemia dieu trj tai khoa Nhi bgnh vign Da khoa Trung U'ong Thai Nguyfin trong thdi gian nghien cdu t u 1/1/2013 d i n 31/10/2013.
Chan doan xae i^nh Thalassemia dya vao tieu chuin sau:
- Tieu chuan c h i n dodn Thalassemia [5], [12]:
+ Lam sang: Cd eac trigu chdng thieu mau tan mau mgn tinh.
+ Xet nghigm: Thay ddi thanh phan Hb dac thu theo tung the b^nh. dien di co Hb b i t thudng:
+ Tieu chuan logi trir ddi t u g n g nghien c d u :
+ B§nh nhan mac bgnh nhilm trimg kem theo tgi thdi diem nghien cuu.
+ Binh nhan thidu mau huyet tan khong do bfinh ly huyit sac td.
2.2 Phu-cng phdp nghidn c d u - NghiSn cdu mo ta e i t ngang, chgn m l u cd chii dich, ed m i u thuan tifin.
- Cae eh! tiSu nghien edu dugc thu thgp theo mdt m i u thdng nhdt.
2.3. c a c ky thugt va ti§u c h u i n danh gia
c a c ky thugt xet nghiSm dugc thyc hien theo quy trinh dang dugc ap dyng tgi phong xat nghigm huyet hgc, Tmng tam Huyet hgc truydn mau va khoa Sinh hda Benh vign Da khoa Trung U'ong Thai Nguyen.
2.4. Xir ly so ligu
- Cac sd lifiu thu thgp dugc x d ly theo phuang phap thdng ke Y hgc tren ehuang trinh SPSS 16.0, Excell.
3. K^T QUA NGHIEN CCTU V A B A N LUAN
Qua nghien cuu 48 bgnh nhan thilu mau huyet tan didu trj tgi khoa Nhi, Bgnh vign Da khoa Trung U'ong Thai Nguyen chung toi thu dugc mdt sd k i t qua nhu sau:
3.1 .Dgc d i l m bgnh nhdn nghien c d u va the bgnh
3 . 1 1 Phan toai tienh nhan nghien cuu theo thS binh
Bieu dd 1. Phin logi theo the bgnh cua b§nh nhan nghien edu
Vietnam Joumal of Physiology 19(1), 3/2015 ISSN: 1 8 5 9 - 2 3 7 6 Bleu dd 1 eho t h i y trong 48 bgnh nhan
Thalassemia ty Id p-Thal la 70,8%, p- Thal/HbE la 25,0% va a-Thal la 4,2%. Ket qua nghiSn edu cDa diUng toi dya tren nghien edu cac bgnh nhi nen ty le the bgnh P-Thal cao han so vdi cac tSc gia
khac nhu Phiing Thj Hong Hgnh (2009), Nguyin Quang H i o (2010), Hoang Thj Hdng (2011), Pham Thj Thu KhuySn (2012) [1], [2], [3], [6].
3.1.2.Phan bd ve d'a dw va dan tdc &
benh nhan nghien cwu.
^ -^ . ^ ' ^ . ^ -^ Jl^ > ^ ^
Bidu do 2. Phan bd dia du nghifin cdu Bang 1. Phan bd dan tgc cua bgnh nhan nghien cuu
D i n tdc Cao Lan Kinh NCing S^n chii Sdn Diu Tay Tong
n 4 17
8 2 7 10 48
%
8.3 35.4 16.6 4.2 14.6 20.8 100 Bidu dd 2 cho thay bgnh nhan ggp d
h l u h i t eae huy$n trong tinh trong do huygn Ddng Hy chiem ty Ifi cao nhdt 39.6% sau do la Vo Nhai, Phu Binh, Djnh H6a, huydn Phd Yen khong ggp trudng hgp nao. Tinh khac den b§nh vien dieu trj la Lang San, chiem ty la 8.3%. Nghien cdu ciia chung toi thue hien d Thai Nguyen, la mfit tinh miln niii, dan tgc it
ngudi chiem d i n hon 40% dan sd, do vay ty le bgnh nhan la dan tgc it ngudi tang han so vdi cac nghien cuu trong nudc (64,6%) Cac dan tgc it ngudi ggp trong nghien cuu ciia chung toi la Tay, Niing, San diu, Cao Lan va dan ^ San chi [2], [4].
3.1.3. D^ diem ve gioi tinh & tienh nhin nghien cUv
Vietnam Journal of Physiology 19(1). 3/2015 ISSN: 1 8 5 9 - 2 3 7 6
Bieu dd 3. Dgc diem ve gidi tinh eiia bgnh nhan nghign cuu Trong so 48 bgnh nhan nghien edu co
25 bgnh nhan nam chilm ty ig 52.1 %. 23 bgnh nhan nu chilm ty Ig 47,9 %.
Thaslassemia la benh di truyen khong lien quan den gidi tinh, trong nghien cuu ciia chimg toi ty ig nam/ nti' la 1.09/1. Kdt qua
nghifin cuu eua chiing toi cijng phu hgp vdi k i t qua nghien cuu cua cac tac gia cho thay khong co sy chenh Ifieh vugt trgi giua 2 gidi v l tJ Ifi m i c bgnh [2], [3], [6].
3.1.4. D0C diem huyet hgc mau ngoai
Bang 2. IVIpt so chi so dong hong cau khi vao vi^n va the benh
T h i benh
a - Thai p - Thai Thal/HbE Chung
Hemoglobin (g/l)
65.8+11.5 51.2 + 23.1 56.73 + 25.7 54.36+16.57
H/ICV (fl)
89.1+2.2 62.5 + 4.6 65.7 + 3.2 64.8 ± 5.9
RDW (%)
19.5 + 3.1 25.2 + 5.7 26.1 +4.3 22.5 + 6.4 Bang 2 eho thiy, dgc didm thieu mau
cua cac benh nhan Thalassemia la thieu mau hdng cau nho nhugc sic, dg phan bd kich thudc hdng cau r i t khong ddng d i u .
Trong nghien cdu eua chiing toi, benh nhan thudng den vien khi ndng dfi Hemoglobin giam nang hoac r i t ngng, ndng do huyit s i c td trung binh cua cac benh nhan khi vao vign la 54,36 + 16.57
g/l trong d6 lugng huyet sac td thip nhat la 19 g/l va cao nhdt gap trong nghien cdu cua chung toi la 73 g/l.
3.2. Tinh trang u sat d benh nhan nghiin cdu
Chiing toi tien hanh dinh lugng s i t va Ferritin huyet thanh cho 48 benh nhan, thu dugc k i t qua nhu sau:
Vietnam Journal of Physiology 19(1). 3/2015 ISSN: 1 8 5 9 - 2 3 7 6 3.2.1 Nong do sat va feritin huyet thanh
trung binh
Bang 3. Nong do s i t va feritin huydt thanh trung binh d b$nh nhan nghien cuu Xet nghiem
s i t huyit thanh (pmol) Ferritin (ng/ml)
Gia trj trung binh 26,3 + 18.2 1486 5 ±985.8
Bang 4: Phan bd benh nhan theo eac phan muc ciia ndng dp s i t va fierntin huyet thanh Nong do sat huy^t thanh (Mmol)
Phan mu'c ThSp Nam < 11 N C K 7 Binh thu'dng Nam: 1 5 - 2 7 N u ' : 7 - 2 6 Tang Nam' >27 Nii >26
n 0
21
27
%
0
43.8
56.2
Nang dp Ferritin huyet thanh (ng/ml) Ph§n mijc
Thap Nam < 30 N & < 1 5 Binh thuang Nam:30 - 300 NO: 1 5 - 3 0 0 Tang 300-1000
Ll'sat> 1000 n 0
8
15 25
%
0
16.7
31.2 52.1 Theo ket qua nghien cuu ciia ehiJng
toi (bang 3), ferritin huyit thanh tang, trung binh la 1486,5 + 985,8 ng/ml, trong dfi luang ferritin tang tr^n lOOOng/ml chilm ty Ig 52,1% (bang 4). Khi ferritin tang tren lOOOng/ml co the gay ra cac dgc tfnh do d sat, co rat nhidu bidn chung xay ra tren ddi tugng benh nhan nay [14].
Theo khuyin cao cua hiep hfii Thalassemia qudc te viec thai s i t d nhung bgnh nhan phu thugc vao truydn mau la r i t can thiet va can dugc lam thudng quy khi ndng do ferntin tang tren lOOOng/ml. Do vgy, can co nhung nghien cdu di sau phan tich va co nhung giai
phap thiit thyc nham giam cac biln chung ciia hien tugng u s i t cho bgnh nhan.
K i t qua nghien cuu ciia chiing toi thip hon so vdi nghien cuu ciia Hoang Thi H6ng, Nguyin Quang Hao, Phgm Thi Thu Khuyen khi nghien cuu tren benh nhan Thalassemia didu trj tai Vien Huyet hgc - Truydn mau Trung U'ong [1], [2], [3]. Dieu nay co the ly giai do benh nhan didu tn tai Vien Huydt hgc - Truyen mau Trung Lfang da phin la ngudi idn, thdi gian phat hien benh da lau, qua trinh truyin mau keo dai
Vietnam Journal of Physiology 19(1). 3/2015 ISSN: 1 8 5 9 - 2 3 7 6 3.2.2 Phan bd ndng do ferritin huyet thanh theo the b^nh.
Bleu do 4. Phan bo mdc feritin theo the bgnh d the bgnh a - Thai, ndng dfi feritin
huyet fliartfi tang, chii ydu g ^ d mde dudi 1000 ngfrnl.
Cs the banh p-Thal, ndng dd feritin huydt thanh binh thudng chiem ty Ig 20,6%: day chii ydu la nhung bgnh nhan l i n d i u vao vign. Nong dg feritin tang dudi 1000ng/ml chiem ty Ig 26.4%. Mdc dfi d s i t nhg (1000 - 2500ng/dl) la 41,2%
va muc do d s i t ngng (> 2500ng/dl) la 11,8% fren tdng sd cac bgnh nhan p-Thal.
6 the bgnh p-Thal/HbE, ndng dd fentin huyit thanh tang tren lOOOng/mt chiem ty le 58,4%. Mdc dg d sat nhe (1000 - 2500ng/dl) la 33.3% va mdc dg d sat ngng (> 2500ng/dl) la 25,1% tren tdng sd cac b$nh nhan p-Thal/HbE. Sy khae bigt vd ndng do femtin huyet thanh theo the bfinh ed y nghTa thdng ke vdi p < 0,05.
Dieu nay cho thay vide d i l u tri thai sat eho benh nhan cdn dugc chii trgng.
4. K^T LUAN
Qua nghien edu 48 benh nhan Thalassemia didu trj tat khoa Nhi Benh vi§n Da khoa Trung LTang Thai Nguyen chung toi nJt ra mgt sd ket lugn sau.
- Ggp 70.8 % la p-Thal va 25,0 % p-Thal phdi hgp HbE va gap 4.2 % a-Thal, ty 1$
nhu nhau d nam va nu, din tfie it ngudi
chidm 64,6%. Bgnh nhan ddn vifin didu tri chii y i u la tinh Thai Nguyfin, trong do huyen Ddng Hy chiem ty (g cao (39,6 %).
- Nong dg Hemoglobin luc vao vien t h i p , tamg binh la 54,36 + 16.57g/l - Banh nhan nghien cdu co ndng dg sat huydt thanh cao, tnjng binh la 26,3 + 18,2pmol.
- Ndng dg Feritin cao, trung binh la 1486.5 + 985,8 ng/ml.
- C6 52,1 % benh nhan u s ^ (co ndng 66 feritin > 1000 ng/ml)
TAt LI^U THAM K H A O 1. Nguydn Thi Thu Ha (2009) "Nghiin ciru mqt sd bien ddi miu ngoai vi va chuyin hda sat trin mdt sd bgnh ly thidu miW, Lugn van Thac sT y hgc, Dgi hoc y khoa Ha Ndi
2. Hoang Thj Hdng {ZOnyNghiin ciru tinh trang w sSt eua binh nhin Thalassemia dwqc diiu tri tqi Vign HH- TM trung uang", Luan van tdt nghigp bac sy ngi tru Dgi hgc y khoa Ha Ndi.
3. N g u y i n Quang Hao (2010), "Buac diu nghien ciru dieu tri binh nhin Thalassemiaassemia ngudi Idn tai Vign HHTMTW, Lugn van tdt nghi§p bac sy nfii tnJ Dgi hgc y khoa Thai Nguyen
Vietnam Journal of Physiology 19(1). 3/2015 ISSN: 1 8 5 9 - 2 3 7 6 4. Nguyen Cdng Khanh, D u v n g Ba
Tryc (1990) " ^-Thalassemiaassemia/
Hemoglobin E d Viet Nam" Tropical Pediatrics. 36, 3 6 - 4 3 .
5. Nguyen Cong Khanh (2008), "Binh hemoglobin", Huyit hgc lam sang nhi khoa.
Nha xuat ban Y hgc, 124-164.
6. Pham Thj Thu Khuyen (2012) 'Nghien cuv die diem lam sing, xet nghigm a bgnh nhan tqi Viqn HHTMTW', Luan van Thge sT y hgc, Dgi hoe y khoa Ha Nfii
7. Nguyen Nghidm Lugt (2006), 'Chuyen hoa sit vi rdi loan chuydn hoi sit". Bai giang hoa sinh sau dgi hgc
8. Nguyen Ha Thanh (2010). 'Chuydn hoa sit vi thiiu miu thiiu sSt", Bai giang huyet hpe truyen mau, NXB Y hgc Ha Ndi.
208-213
9. Beutler E, Hoffbrand AV, Cook JD (2003). Iron deficiency and over/oad.Hematology (Am Soc Hematol Educ Program). ;40-61
10. Bernard G. Foget, Alan R., Cohen (2005), Thalassemiaassemia syndromes. Hematology, Basic Principles and Pratiee, 557 - 590.
11. Bernard D (2006) "Iron overioad in 77ja/assem/aassem/a" .Thalassemiaassemia Intemational Frederation magazine. Issue No.49, pp 24-25.
12. Quirolo K and VIchinsky E (2004), 'Thalassemiaassaemia Syndrome", Nelson Textbook of Pediatric, 17"^ Edition. Volume 2, Saunders, pp 1630-1634.
13. World Health Organization (2009). "Woridwide prevalence of anaemia 1993 2005 WHO global database on anaemia". Worldwide prevalence of anaemia report 1993 - 2005 accessed, May 5'" 2009.
14. Wormw^ood M (2001) Iran deficieney anemia and iron overload' Dacis and Lewis Practical Heamatology:
Laboratory Method. 115-128, SUMMARY
IDENTIFY THE TYPE OF THALASSEMIA AND ESTIMATE IRON STASIS STATUS ON THALASSEMIA PATIENTS IN THE CENTRAL HOSPITAL OF THAI NGUYEN IN 2013 Nguyen Thu Hanh^', Nguyen Kieu G i a n g \ Pham Thi Thu K h u y e n \
Vi Thi Phuong Lan^
^Thai Nguyen Medical Pharmaceutical University This study was conducted in order to indentify the type of thalassemia and estimate iron stasis status on thalassemia patients
Subjects and methods: Cross sectional study, 48 patients were diagnosed exactly a Thalassemia in Pediatrics department of the Central Hospital of Thai Nguyen from 1/1/2013 to 31/10/2013.
Results and conclusion: The prevalence of ^-Thai was 70.8%, ^-Thal combine HbE was 25.0% and a-Thal was 4.2%, male = female (male 52.1%,, female 47,9%), the prevalence of Thalassemia was hight in the ethnic minotory (64,6%). The patients come from Thai Nguyen province, Dong Hy district had a high percentage of Thalassemia (39.6%). Hemoglobin concentration was low, average concentration of Hb was 54.36
±16,57 gA. Iron serum eoneentration was hight, average concentration of iron serum was 26,3 ± 18,2pmot, feritin serum was hight, average concentration of feritin serum was 1586,5 ± 985,8 ng/ml. there were 52,1% patients with iron stasis status, feritin serum.
Keywords: Thalassemia, iron stasis, feritin serum, anemia.
