Y HOC VIET NAM THANG 2 - SO 2/2012
DAC DIEIVI CAN LAM SANG CUA BENH NHAN B| HOI CHUNG THAN HIT TIEN PHAT KHANG THUOC CORTICOSTEROID
Nguyen Thj Quynh Hu'dng*, Nguyen Thj Yen*
TOM
T A TMuc tieu: Nghien cifu dac diem lam sang ciia benh nhan bj hdi chifng than hU tien phat (HCTH TP) khang corticosteroid. Phu'dng phap nghien cufu:
Nghien cifu thuan tap vdi 64 benh nhan dUdc chan doan la HCTHTP khang corticosteroid (Cst) va 68 benh nhan bj HCTHTP nhay cam Cst nhap vien tai khoa Than Tiet nieu benh vien Nhi Trung L/dng tir 1/1/2008 den 31/12/2010. Ket qua: nam (62,5%) va nU (37,5%). Ty le namlnH la 1,67. Tudi trung binh ciia HCTH khang Cst la 6,64±4,3 tuoi. HCTH khang Cst gap phii nang (29,7%) nhieu hdn HCTH nhay cam Cst (8,8%) vdi p<0,05. Cd chUdng gap trong HCTH khang Cst (82,8%) nhieu hdn HCTH ntiay cam Cst (55,9%) (p< 0,05). Tang huyet ap gap trong HCTH khang Cst (81,2%) cao hdn han HCTH nhay cam Cst (22,1%) vdi p<0,01. Tang huyet ap d HCTH khang Cst nang hdn, HCTH khang Cst ed tang huyet ap de doa (18,8%) cao hdn HCTH nhay cam Cst (0%). HCTH khang Cst CO tdi 40,6% benh nhan ed dai mau trong khi HCTH nhay cam Cst ehi cd 5,9% vdi p<0,05. Ty le bi cac bien chifng eiia nhdm HCTH khang Cst (83%)) eao hdn han nhom HCTH nhay cam Cst (23,5%) vdi p<0,05.
Hay gap nhat la viem dUdng hd hap chiem 39,1%, ty le tif vong eiia HCTH khang Cst la 6,3%. Ket luan:
HCTH khang Cst gap d nam nhieu hdn nu', cac trieu chiiTig phii, tang huyet ap, dai mau deu gap nhieu va nang hdn d HCTH khang Cst so vdi HCTH nhay cam Cst.
Keywords: Hdi chifng than hU khang corticosteroid, phii, dai mau, eao huyet ap.
SUMMARY
Clinical characteristics of patients with corticostero- resistant idiopathologic nephrotic syndrome
Objective: To study the clinical features of patients with eortieostero-resistant idiopathologic nephrotic syndrome (CRNS). Methods: The study cohort of 64 patients diagnosed CRNS and 68 patients were hospitalized corticostero -sensitive nephrotic syndrome (CSNS) at the Department of Uro- Nephrology of National Hospital of Paediatrics from 1/1/2008 to 31/12/2010. Results: Males (62.5%) and females (37.5%). The rate of male/female was 1.67.
The average age of the CRNS was 6.64 ± 4.3 years.
CRNS had more severe oedema (29.7%) than CSNS (8.8%) with p <0.05. Ascitis was seen higher in CRNS (82.8%) than CSNS (55.9%) (p <0.05). Hypertension was frequently in CRNS (81.2%) than CSNS (22.1%)
with p <0.01. Hypertension in CRNS was heavier than CSNS with severe hypertension (18.8%) vs CSNS (0%). 40.6% CRNS presented a hematuria but this rate only 5.9% in CSNS with p<0.05. Rate of complications of CRNS (83%) was higher than CSNS (23.5%) with p<0.05. Most common complication was respiratory tract infections ( 39.1%), the mortality rate of CRNS was 6.3%. Conclusion: CRNS was frequently in men than women, the symptoms of oedema, hypertension, hematuria were more seen and more severe in CRNS to compare with CSNS.
Keywords: corticosteroid-resistant nephrotic syndrome, oedema, hematuria, hypertension.
L
OAT VANof
46,6 % sd benh nhan eiia khoa Than-tiet nieu benh vien Nhi trung Udng la hdi chifng than hu tien phat (HCTHTP), la benh eau than hay gap nhat d tre em [2] tuy nhien trieu chifng lam sang lai rat ngheo nan. Tien lUpng eiia nhu'ng benh nhan bj HCTHTP khang Cst thUdng rat xau cd khoang gan 5 0 % sd benh nhan nay se bj suy than giai doan cudi ddi hdi phai Ipe mau hoac ghep than. Nghien cifu ve trieu chifng lam sang van ludn la van de dat ra vdi eae nha than hpe ndi chung va than nhi ndi rieng chinh vi vay de tai 6Mc tien hanh nham: Nghien cdu dac diem lam sang cua benh nhan bi HCTHTP khang corticosteroid tai khoa Than-Tiit nieu benh vien Nhi trung ddng tCt 1/1/2008 din 31/12/2010.
II. o d l TUpNG VA PHUONG P H A P NGHIEN CCfU 2 . 1 . D o i t u ' d n g n g h i e n cu'u
2.1.1 Nhdm benh
Gdm 54 benh nhan dddc chan doan la HCTHTP khang Cst nhap vien tai khoa Than Tiet nieu benh vien Nhi Trung U'dng trong thdi gian 3 nam tif 1/1/2008 den 31/12/2010.
Tieu chuan chan doan HCTH: Protein nieu
>i 50 mg/kg/24 gid, albumine mau < 25 g/l, Protid mau < 56 g/l (tieu chuan ciia ISKDC International study of Kidney Diseases in Children) [ 6 ] .
Tieu chuan chan doan HCTH khang corticosteroid: sau khi diing prednisolon lieu 2 mg/kg/ngay trong 4 tuan, sau dd 3 lan truyen
methylprednisolon 1000 mg/l,73m7'18 gid ma protein nieu van ~- 50 mg/kg/24gid [6].
Tieu chuain loai trd:
Nhii'ng benh nhan khdng dieu trj day dii theo phac dd.
HCTH bam sinh (tdc la HCTH khdi benh d dp tudi < 3 thang)
HCTH thd phat (tim thay nguyen nhan vf du lupus ban dd he thdng, ban xuat huyet dj irng, ong ddt, ngd doe...)
2.1.2 Nhom chdng: Gdm 68 benh nhan 6\i0c chan doan HCTH the nhay cam Cst nhap vien trong khoang thdi gian nghien cdu. Dd la eae bdnh nhan HCTH sau 4 tuan dieu trj prednisolon lieu tan edng (2mg/kg/ngay) thi protein nieu am ti'nh [6].
2.2. Phu'dng phap nghien cu'u:
Nghien cifu thuan tap vdi c5 mau eiia moi nhdm dupe tfnh theo edng thifc sau:
n >
1,96^
m x p x ( l - p ) Thay sd vao ta cd n >. 62
-1- Lam sang: can, do chieu cao, do huyet ap (do toi thieu 2 lan va vdi cd bang huyet ap phii hpp vdi tirng tre) va phan loai eao HA theo tudi va gidi va 6\idc do tdi thieu 3 lan, do sd lUdng nUdc tieu 24 gid, mau sac, kham phii, phan loai mifc dp phii theo ISKDC.
Danh gia mifc dp phii khi vao vien: so sanh can nang khi vao vien vdi can nang liic het phii [6]:
-I- Khdng phii: kham lam sang khdng thay phii, can nang khdng thay dd'i.
+ Phil nhe: benh nhan phii mat va chan, can nang tang nhd hdn 10% trpng lUdng ed the.
+ Phu vLi^: benh nhan phii rd, thoat nhin da thay, can nang tang td 10- 20% trpng lUPng ed the.
-I- Phil nang: can nang tang tren 20% trpng lupng cd the kem thee tran djch da mang nhU mang bung, mang phd'i, phii ha nang (d tre trai)
Mifc dp tang huyet ap theo chieu cao va gidi [3]: Binh thUdng: <97,5 dp bach phan;
Tang huyet ap gidi han: TiT 97,5 dp bach phan den97,5 dp bach phan -i- lOmmHg; Tang huyet ap chac chan: Td 97,5 dp bach phan + lOmmHg den 97,5 dp bach phan -t- 30mmHg; Tang huyet ap de dpa: > 97,5 dp bach phan (percentile) -i- 30mmHg.
Danh gia tinh trang dai mau:
+ Dai mau dai the: la tinh trang nUde tieu cd mau ma bang mat thUdng thay mau dd mau khdng ddng, mau nUde rifa thit...
+ Dai mau vi the: chan doan nhd lam xet nghiem thay cd hdng eau trong nUdc tieu.
Cac benh nhiem triing: nhiem triing dUdng hd hap neu cd ho, sot, nghe phdi cd ran phe quan, ran am nhd hat...Nhiem triing dUdng tieu neu cd dau hieu rdi loan tieu tien, sdt, bach a u nieu xuat hien trong nUdc tieu, cay nUdc tieu giuS ddng vi khuan nieu > lOVml, viem phiic mac neu benh nhan cd ndn, dau bung, rdi loan tieu hoa, kham bung co phan ifng thanh bung hay cam ifng phuc mac, chpc dich mang bung ed te bao, rivalta dUdng ti'nh, cay co the thay vi khuan...
Cac xet nghiem can lam sang:
Creatinin mau lam theo phUdng phap Jaffe kinetic (khdng khif tap) tren may HITACHI 717 tai khca sinh hda.
* Mifc Ipc cau than tfnh theo edng thifc Swcharzt
h(cm)*k Clearance creatinine =
-I- He so k: Tre sd sinh < 2.5 kg: k =29,1;
Tre sd sinh > 2.5 kg: k = 39,7
Tre < 2 tudi: 40; Tre t d 2-21 tudi nu': k = 48,6; Tre nam 2-12 tud'i: k =48,6.
Tre nam 12-21 tudi: k = 61,7.
* Chan doan giai doan suy than theo dp thanh thai Creatinine [5]:
* Ure: dUdc lam bang phUdng phap Enzym UV, may Olympus AU.400 tai khoa sinh hda.
* Protid mau, albumin mau dUdc thUc hien may Olympus AU.400 tai khoa sinh hda benh vien
Cre mau(pmol/l)
nhi Trung Udng. Prdtid tdan phan mau va Albumin mau dUpc phan tfch thanh giam 6 ngudng than hU (Protid toan phan mau < 56 g/l va > 56 g/l)
* Lay nude tieu 24h: NUdc tieu dUdc lay trong 24 gid va diing nUdc chdng thoi bang dung djch HgCI, benh nhan dUdc can va do the tfch nUdc tieu ciia ngay hdm thu thap nUdc tieu.
Protein nieu djnh lUdng: dUdc pha vdi dung djch b-ichloracetic 5%, do dp due bang may quang phd T60 ciia My va du'dc thi/c hien tai khoa sinh hoa.
Y HOC VIET NAM THANG 2 - SO 2/2012
Protein nieu 24h (mg/kg/24h) = Protein nieu 24h (mg) x V(lit) V: The tich nUde tieu thu thap 24 gid.P: Trpng lUpng benh nhan ngay lam xet nghiem.
* DUa vao protein nieu de ehia ket qua dieu trj thanh 3 loai [6]:
-I- Thuyen giam hoan toan: benh nhan het phii, protein nieu am ti'nh hoac vet (xet nghiem trong 3 ngay lien).
+ Thuyen giam mot phan: protein nieu giam, edn < 50mg/kg/24h.
-1- That bar. protein nieu van eao >
50mg/kg/24h.
HCTH dddc gpi la tai phat neu trUde dd benh nhan da thuyen giam nay xuat hien lai protein nieu [6].
* Te bao nieu: soi tUdi tai khoa huyet hpc bang kfnh hien vi vat kinh 10 va 40. Tif <5 Hong cau/vi trudng: Rai rac; Tir 5-10 Hdng cau/vi
3.2. Phan bo benh theo tuoi va gidi
trudng: (+), Td 10-20 Hdng cau/vi trudng: (++), Tif >20 Hong cau/vi trUdng: (++-I-).
2.3. Xii ly so lieu: Sd lieu dddc xii ly bang phan mem SPSS 16, Sir dung cac test thdng ke: T- test, x^ ANOVA test, Fisher test... Tinh yeu td lien quan bang ty suat chenh OR vdi dp tin cay 95% Q.
III.KET QUA NGHIEN CQU
3.1. Tan suat gap HCTH khang corticosteroid: Tif 01/01/2008 den 31/12/2010 chiing tdi thu thap dddc 614 benh nhan bj HCTH nhap vien dieu tri tai khoa Than bdnh vien Nhi Trung UPng, trong dd cd 64 benh nhan bj HCTH tien phat khang corticosteroid, ehiem ty le 10,4%.
i
^ D
Bieu dd 1. Phan bo benh theo tudi va gidi
Nhan xet: nam chiem 62,5% va nii' chiem 37,5%. Ty le nam/nii' la 1,67. HCTH khang thudc gap nhieu nhat d dp tudi tif 2-10 tudi. Tudi trung binh ciia nhdm HCTH khang thudc la 6,64±4,3 tud'i.
Trong dd thap nhat la 13 thang tud'i, eao nhat la 17 tudi.
3.3. Trieu chu'ng l a m sang cua Bang 1 : Oac diem phii ciia HCTH Dac diem phu
Khdng phii Phil nhe Phil vira Phil nang Tdng
Khdnq cd chUdng Co cd chudng Tdnq
HCTH n 3 20 22 19 64 11 53 64
hdi chu'ng than hu' tien phat khang corticosteroids khang corticosteroids (Cst)
khang Cst Tyle 4,7%
31,2%
34,4%
29,7%
100%
17,2%
82,8%
100%
HCTH nhai n
0 31 31 6 68 30 38 68
/ c a m Cst Tyle
0%
45,6%) 45,6%
8,8%
100%
44,1%
55,9%
100%
P 0,004
0,0012
Nhan xet: Ca HCTH khang Cst gap phii nang (29,7%) nhieu hPn HCTH nhay cam Cst (8,8%) vdi p<0,05."
Cd chu'dng gap ti-ong HCTH khang Cst (82,8%) nhieu hdn han HCTH nhay cam Cst (55,9%) (p< 0,05).
Bang 2: Trieu chufng huyet ap va dai mau ciia HCTH khang^st Trieu chu'ng huyet ap va dai mau
Huyet ap binh thuVliuj
Tang huyet fang huyi-t ap yidi han ap L l T a n g huyol ap x,)( djnh
T3jig huyet ap do doa jrdn£
7 ong Khung dai mau
Dai mau Tong
Nhan xet Tang huyet ap gap trong HCTH khang Cst (81,2%) eao hdn han HCTH nhay earn Cst (22,1%) vdi p<0,01. Trong dd mu'c dp nang eiia tang huyet ap trong HCTH khang Cst eung nang hdn (18,8% HCTH khang Cst ed tang huyet ap de doa trong khi HCTH nhay cam Cst ty le nay la 0%)
HCTH khang Cst ed tdi 40,6% benh nhan cd dai mau trong khi HCTH nhay cam Cst ehi ed 5,9%
vdi p<0,05.
ig Cst Tyl^(o/o)
18,8 20,2 42,2 18/8 81,2 100 59,4 40,6 100
Nh n
"53 4 0 15 68 64 4 68
ay cam Cst Tyl^C/o)
77,9 16,2 5,9
0 22,1
100 94,1 5,9 100
p
0,0014
0,001
Bang 3: Dac diem ve cae bien chu'ng eiia HCTH khang Cst Bien chu'ng
Viem dudng hd hap Viem dudng tiet nieu Viem phue mac tien phat Viem md te bao
Sdc giam the tich Bd mat Cushing Viem da day cap Tir vong
Tong edng
Nhan xet Ty le bi cac bien ch
Khang steroid(n=64) SoBN
25 4 6 1 1 10
2 4 53 Lfng eiia nhd
Tyle 39,1%
6,3%
9,4%
1,6%
1,6%
15,6%
3,1%
6,3%
83%
Tl HCTH kha
Nhay cam SoBN
5 2 2 0 0 1 6 0 16 ng Cst ( 8 3 %
steroid(n=68) Tyle 7,4%
2,9%
2,9%
0%
0%
1,5%
8,8%
0%
23,5%
) cao hdn han n P
0,0011
hdm HCTH nhay cam Cst (23,5%) vdi p<0,05. Hay gap nhat la viem dudng he hap chiem 39,1%, tiep theo la bp mat Cushing (15,6%). Ty le tif vong ciia HCTH khang Cst la 6,3% trong khi HCTH nhay cam Cst ty le nay la 0%.
IV. BAN LUAN
Ty le HCTH khang Cst trong nghien cifu cua chung tdi la 10,4%. Tud'i trung binh eiia eae benh nhan HCTH khang thude trong nghien cifu ciia chung tdi la 6,64±4,3 tudi (thap nhat la 13 thang tud'i, cao nhat la 17 tud'i). Ket qua nay cung gidng cac nghien cifu khac [1].
HCTH khang Cst gap chii yeu d tre trai, cd 40 benh nhan nam (chiem ty le 62,5%) va 24 benh nhan nu' (chiem ty le 37,5%). Ty le nam/nu' la 1,6. Ket qua nay cung tu'dng tU nhu' cae nghien cifu khae [1 ] .
Phil la dau hieu thudng gap nhat trong HCTH. Trong nghien cifu eiia ehiing tdi HCTH khang Cst ed ty le phii liie vao vien chiem 95,3%
(3 benh nhan khdng phii ciia nhdm nay deu la nhu'ng benh nhan dang du'dc dieu trj Prednisolon tru'dc luc vao vien tren 6 tuan), ngUdc lai, HCTH
nhay cam Cst thi 100% benh nhan luc vao vien la cd phii. Benh nhan bj HCTH khang Cst cd ty le benh nhan bi phu nang cao hdn va ty le cd chu'dng cao hdn benh nhan bj HCTH nhay cam Cst. Nhan xet ve trieu chifng phii, tac gia khac cung chd rang phii xuat hien d 95-100% benh nhan bi HCTH, nhuTig benh nhan bj HCTH khang Cst thi phii nSng hdn benh nhan bj HCTH nhay cam Cst.
81,2 % benh nhan bi HCTH khang Cst co tang huyet ap va tang huyet ap de dpa chiem tdi 18,8% trong khi dd ty le tang huyet ap d benh nhan bj HCTH nhay cam Cst lai chi ed 22,1% va khdng cd benh nhan nao bj tang huyet ap de doa. Ket qua nay cung tUPng tU nhU nghien cifu khae.
Oai mau dai the la mdt trieu chifng ft xuat hien trong HCTH. Nd thu'dng gap trong cac the
64
Y HOC VIET NAM THANG 2 - SO 2/2012 HCTH ket hpp. Trong nghien cifu eiia ehiing tdi d
benh nhan bj HCTH khang Cst ed 40,6% benh nhan bj dai mau trong khi HCTH nhay cam Cst ty le nay chf gap 5,9%. Ket qua nay eung tUdng tU nhu cac nghien cifu khae. Theo Habib va edng sU nhdm tdn thUdng tdi thieu va nhdm td'n thUdng xd hda cue bd rat it ed dai mau dai the, edn nhdm viem cau than tang sinh mang lai cd tdi 20% ed dai mau dai the va d nhdm nay ty le benh nhan bi HCTH khang Cst eao hPn [4].
Ty le gap bien chifng d nhdm khang Cst len tdi 83% trong khi dd d nhdm nhay cam Cst con sd nay ehi la 23,5%. Trong nhdm khang Cst ty le eae bien chifng eao hPn han nhdm nhay cam Cst (p<0,01). Cae bien chifng hay gap nhat la viem du'dng hd hap (39,1%), viem phiic mac tien phat (9,4%)...Trong hdm khang Cst ed 4 benh nhan tif vong (6,3%) trong qua trinh dieu trj deu do suy than. Theo Kevin D. McBryde [7] nguyen nhan chfnh gay tif vong d HCTH ndi chung va HCTH khang thudc ndi rieng la do nhiem triing, tif vong do suy than chi chiem du'dl 2 0 % cac trUdng hdp va thu'dng xuat hien mudn sau khdi phat benh.
Sif khac biet ve nguyen nhan gay tir vong cd the do thdi gian theo ddi cac benh nhan trong nghien cifu ciia ehiing tdi edn ngan.
V. KET LUAN
HCTH khang Cst gap d nam nhieu hdn nii'.
Cae trieu chifng: phii, tang huyet ap, dai mau deu gap vdi ty le nhieu va nang hdn d HCTH khang Cst so vdi HCTH nhay cam Cst.
TAI UEU J H A M KHAO
1. Nguyen Ngoc Sang (1987), " Nhan xet v'e dac diem lam sang va sinh hda qua 52 trUdng hdp HCTHTP the khang corticoid d tre em", Luan van tdt nghiep bac sy ndi trii cac benh vien, TrUdng Dai hoc Y Ha Ndi.
2. Le Nam Tra, Tran Oinh Long, Oo Bich Hang (1994), Tinh hinh benh than, tiet nieu ciia tre em dUdc dieu trj tai Vien Nhi 1981 - 1990", Ky yeu cdng trinh nhi khoa ciia Vien Nhi, Tr. 161-162.
3. Andre JL, Deschamps JP, Gueguen R.(1980)
"Arterial blood pressure in 17067 children and adolescents. Variation with age and height". Ar.
Fr. Pediatr, vol 37: 477-82.
4. Habib R, Kleinknecht C (1971), "The primary nephrotic syndrome of childhood, in Pathology Annual", edited by Sommers SC, New York, Appleton-Century-Crofts, pp. 417.
5. Hogg RJ, Furth S, Lemley KV, et al. (2003).
"National Kidney Foundation's Kidney Disease Outcomes Quality Initiative dinical practice guidelines for chronic kidney disease in children and adolescents:
evaluation, dassification, and stratification.'' Pediatric, vol 111 (No 6): pp 1416-21.
6. Kashim M.S., Ngo L.Y., Lajin L e t al.(1996)
"Consensus statement: Management of idiopathic nephrotic syndrome in childhood", A report of the International Study of Kidney Disease in Children, www.acadmed.orq.my/view file.cfm
7. Kevin D. McBryde, MD, David B. Kershaw, MD, and William E. Smoyer, MD (2001). "Pediatric Steroid-Resistant Nephrotic Syndrome", Current Problems in Pediatric, Vol. 31(9): 275-307.
NGHIEN Cl)U CAN NGUYEN GAY VIEIVI PHOI UEN QUAN THOf MAY TAI KHOA CAP CllfU VA HOI SlJfC TICH ClTC BENH VIEN BACH MAI
Tran Hu'u Thong*, N g u y i n Oat Anh*, Oang Quoc T u a n '
TOM
TATOat van de: Viem phdi do thd may la bien chifng thudng gap, chiem 25-50% sd benh nhan thd may, lam tang thdi gian nam vien, ehi phf va tang ti le tif vong.Muc tieu nghien cufu: Can nguyen vi sinh gay viem phdi lien thd may. Odi tu'dng va phu'dng phap nghien cufu: tif 9/2009 den 8/2011cd 11 bn dii tieu chuan dUdc dUa vao nghien cifu. Ket qua: cd 30 bn viem phdi lien quan thd may. Vi khuan gay viem phdi lien quan thd may gap nhieu nhat la
Acinetobacter, ehiem 40%; tiep den la Pseudomonas aeruginosa, ehiem 26.7%, eae vi khuan khac la Klebsiella pneumoniae, Pseudomonas aeruginosa va Staphylococcus aureus. Vi khuan gay viem phd'i mudn chii yeu la Acinetobacter va Pseudomonas aeruginosa, khang vdi nhieu loai khang sinh, ty le khang cao nhat ddi vdi Ampicillin-i-Sulbactam, Amikacin, Ceftazidime va Ciprofloxacin. Ket luan: can nguyen vi sinh chii yeu gay viem phd'i lien quan thd may la Acinetobacter va Pseudomonas aeruginosa, khang vdi nhieu loai khang sinh thudng diing hien nay.
