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HI CHNG THN H TR EM

PGS.TS. Trn Th Mng Hip

MC TIU: 1. nh nghi c Hi chng thn h (HCTH) 2. Lit k c cc triu chng lm sng, CLS v bin chng ca HCTH v cn 3. Nu c cc nguyn tc iu tr HCTH v cn

nh ngha HCTH: HCTH l tnh trng:

1/ Tiu m: m niu /24 gi > 50mg/kg/ngy Protin (mg)/creatinine (mg) > 3, trn 1 mu nc tiu

2/ Gim Albumin/mu : Albumin/mu < 30 g/l

Mt s khi nim : . HCTH n thun c nh ngha khi khng c tiu mu, cao huyt p, suy thn . Hi chng vim thn cp c nh ngha khi c tiu mu i th, cao huyt p,

suy thn cp.

HCTH c th gp trong nhiu bnh cu thn khc nhau, bi ging ny ch cp phn: HCTH v cn (bnh thn h).

HCTH V CN (BNH THN H)

1. LM SNG:

HCTH v cn chim 90% cc HCTH tr em < 15 tui, 1 5 tui (70%), thng tr trai nhiu hn tr gi (Trai/gi : 3 / 1)

Bnh khi pht t ngt, him khi tnh c pht hin, vi triu chng : . Ph (do gi mui-nc) mi mt, chn, mc c, hng khi nm. Ph trng, mm, khng au, n khng mt . Bng bng, kh th, trn dch mng phi, tim, ph bu . Sc gim th tch: HA kp, au bng, tay chn lnh . C th khi pht bng triu chng nhim trng.

Ngoi ra, triu chng au bng bnh nhn HCTH c th do: . Vim phc mc . Tc tnh mch thn hoc ng mch phi . Gim cung lng tun hon

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. Vim ty cp

. Lot d dy (do corticoid)

2. SINH HC: . Tiu m > 50mg/kg/ngy (Tiu chn lc albumin) . Albumin mu < 25g/ l

. Tiu mu vi th: 20% cc trng hp

. Gim Natri niu

. Protid mu < 50 g/ l, Tng 2 globulin, gim gammaglobulin

. Tng cholestrol mu, tng triglycerid mu

. Natri mu bnh thng hoc gim

. Tng kali mu nu suy thn

. H calci mu ton phn

. Tng Hct, tng tiu cu, tng fibrinogen, yu t V, VII, VIII, X, XIII, gim anti- thrombine III

3. C CH BNH SINH: C ch do thay i tnh thm mng y cu thn: ngi ta ghi nhn vai tr ca tc bo (podocyte), in tch m (anion) trn mng y gi li protein. Trong HCTH: in tch m gim, cc chn tc bo b xa v dnh li vi nhau.

C ch cn do ri lon h thng min dch (vai tr ca cytokine, TB lympho) v c th do s hin din ca cc yu t lu hnh.

Ngoi ra, v c a, HCTH c lin quan n nhm HLA DR 7

4. CH NH SINH THIT THN: Ch nh sinh thit thn trong cc trng hp sau y: . HCTH km tiu mu i th . Cao huyt p . Gim b th . Tui < 1 hoc > 11 . Triu chng ngoi thn ca bnh ton th . Khng p ng vi iu tr corticoid

5. GII PHU BNH: . Sang thng di knh hin vi thng:

- Sang thng ti thiu (85%) - X hyalin ha mt phn cu thn khu tr (10%),

(FSGS: Focal segmental glomerulosclerosis, HSF: hyalinose segmentaire et

focale)

- Tng sinh trung m lan ta (5%) . Sang thng di knh hin vi in t: cc t bo c chn b dnh li vi nhau . Tin lng (theo gii phu bnh):

Sang thng ti thiu: 90% p ng vi corticoid, tin lng tt

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X hyalin ha, tng sinh trung m: thng khng corticoid

6. BIN CHNG: 1. Ri lon thng bng nc, in gii 2. Suy thn cp: . Gim lc do gim th tch tun hon . Nghn tnh mch thn 2 bn do cc mu ng . Vim thn k (sau dng furosemide) 3. Nhim trng: Vim phc mc do ph cu, Nhim trng Gram m (Hemophilus), vim mng no, vim phi, vim m t bo do t cu Bnh nhn d b nhim trung do: lng IgG gim, suy gim min dch, iu tr Corticoid, thuc c ch min dch 4. Tc mch: Do tng ng, gim th tch tun hon, gy ra bi:

- bt ng, nhim trng, - tng tiu cu: s lng, ngng kt, - tng fibrinogen, yu t V, VII, VIII, X, XIII, gim anti-thrombine III

Yu t thc y: do tim chch ng mch quay, i, t KT tnh mch 5. Gim cung lng tun hon: Lm sng: au bng, h huyt p, tng Hct, suy thn, nght mch Yu t thc y:

- Ti pht nng - Nhim trng - Li tiu - Chc d mng bng - Tiu chy

6. Ri lon tng trng: . Chm pht trin chiu cao c ghi nhn do: - thot mt s hormone qua nc tiu - suy gip: do thot protein mang Iod

. SDD do thiu m

7. IU TR: Chn on bnh d dng nn khng cn sinh thit thn v iu tr ngay bng Corticoid.

S p ng vi t u gip tin lng bnh. Tin lng tt khi bnh p ng hay nhy vi Corticoid. Tin lng xu khi khng vi Corticoid.

7.1. HCTH ln u: 7.1.1. Tn cng: Prednison 60 mg/m2/ ngy, ti a 60mg/ ngy, trong 4 tun. Nu m niu vn (+): Methylprednisolone 1000mg/1,73 m2/48 gi TTM 3 liu. 7.1.2. Nu m niu tr v (-): nhy corticoid

Cng c : (8 tun k) dng Prednison 60mg/m2 cch ngy Duy tr : (6 tun k) gim liu Prednison 15mg/m2 /2 tun

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Tun 13-14 : 1,5 mg/kg/cch ngy Tun 15-16 : 1 mg/kg/cch ngy Tun 17-18 : 0,5 mg/kg/cch ngy ri ngng thuc

7.1.3. Nu sau iu tr tn cng, m niu cn (+): khng Corticoid Xem phn iu tr HCTH khng Corticoid.

7.2. HCTH ti pht:

HCTH ti pht khi m niu 50mg/kg/24 gi hay (++) vi que th 3 ngy lin tip mt trng hp hi chng thn h iu tr khi trc . Trong trng hp ny:

Ch vi ngy, kim tra m niu 24 gi iu tr tch cc cc nhim trng Nu chc chn ti pht : tn cng li bng phc ring

7.2.1. Hi chng thn h ti pht xa ( 3 thng sau ngng thuc) iu tr li nh trong mc 7.1.1

7.2.2. Hi chng thn h ti pht thng xuyn ( 3 thng sau ngng thuc) hoc HCTH ph thuc corticoid (ti pht khi gim liu)

Prednison 60 mg/m2/ ngy n ngy 3-4 sau khi m niu (-)

Prednison 60 mg/m2/ cch ngy x 8 tun

Sau gim liu v dng li liu c bn bng liu ang dng khi bnh nhn b ti pht cng thm 0,25mg/kg/cch ngy v duy tr liu ny trong 12 18 thng. 7.2.3. HCTH l thuc corticoid liu c bn > 0,5mg/kg/cch ngy v c du hiu ng c thuc (chm pht trin th cht, long xng, cao huyt p, tiu ng, ri lon tm thn) cn c phi hp vi mt trong cc thuc c ch min dch sau y: Mycophenolate Mofetil (MMF):

600mg/m2/ngy (2 ln/ngy), tng n 1200mg/m2/ngy (2 ln/ngy).

Gi Corticoid liu 1-1,5mg/kg/ngy sau gim dn v duy tr vi liu 0,1-0,3mg/kg/cch ngy.

Cyclophosphamide (Endoxan): 3mg/kg/ngy x 8 tun (tng liu 168mg/kg). Trnh dng tay b thuc phn liu (gy c da khi tip xc trc tip): c th lm trn liu thuc ung theo vin 50mg v ko di thi gian iu tr Endoxan (nu cn). Tc dng ph: vim bng quang xut huyt, c ch ty xng, gim bch cu ht, hi u, thiu tinh v tinh trng nam, suy bung trng n, nguy c pht trin bnh c tnh. Theo di huyt mi tun. Nu bch cu a nhn : 1000-1500/mm3 th gim na liu. Nu bch cu a nhn

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Ciclosporin A (Neoral):

Ch nh: HCTH ph thuc corticoid hoc sau khi s dng nhm Alkyls. Khi u 150mg/m2/ngy (hoc 6mg/kg/ngy) chia lm 2 ln, ti a 200mg/m

2/ngy. Gi nng Ciclosporin A (CsA): 50-125ng/ml. Prednison c dng cch nht, song song v gim liu mi tun ngng hn sau 1-2 thng.

Thi gian iu tr: 1-3 nm, nu khng c ti pht, gim liu dn trong 3 thng. Nu c ti pht khi ang iu tr CsA, thm corticoid liu thp. Trc khi iu tr, nh gi chc nng thn, sinh thit thn. Sinh thit thn li sau 2 nm iu tr v khi thay i chc nng thn. Tc dng ph: ph i nu rng, rm lng, tng HA, tng K+ mu, gim Mg++, tng creatinine mu v c thn. Theo di:

- Ion mu, ur, creatinine mu, m mu, albumine mu v thanh thi creatinine.

- Nng CsA mu vo cc ngy 7, 15, 30 v sau mi thng 1 ln. Ch gim liu Ciclosporin A 20-50% nu: Creatinine mu tng v thanh lc creatinin mu gim 30% so tr s ban u, tng Kali mu v nng Ciclosporin A mu > 200ng/ml. Nu bt thng vn cn tn ti sau 15 ngy gim liu, th ngng iu tr.

Khi ti pht di Ciclosporin A th c th dng Corticoid cch nht vi liu cao hn cng vi Ciclosporin, hoc Cyclophosphamide ln 2, hoc Tacrolimus.

Hin nay, Levamisol c s dng cc nc pht trin trc khi dng cc thuc c ch min dch khc. Levamisol hin nay cha c tm thy trn th trng ti Vit Nam.

Cc tc dng ph ca Corticoid bao gm: Tng huyt p Ri lon th gic Chm pht trin Ri lon tm l Hi chng Cushing Lon dng xng Nhim trng nng

7.3. HCTH khng Corticoid: cn sinh thit thn trc khi iu tr. Nu l sang thng ti thiu, tng sinh trung m, x ha cu thn khu tr v tng vng m khng phi l mt dng khc ca vim cu thn, Ciclosporin A v Prednison s c s dng. Nu tht bi, c th dng Cyclophosphamide, Tacrolimus hoc MMF v c ch men chuyn.

7.4. iu tr h tr, triu chng v bin chng:

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Ch n : ch hn ch mui, nc trong giai on ph nhiu v Natri mu gim. Nhim trng : Vim phc mc nguyn pht: Cephalosporin th h 3 v Aminosid trong 48 gi Vim m t bo: Oxacillin hoc Vancomycine v Aminosid trong 48 gi. Gim th tch: Albumin 20% 0,5 - 1g/kg (khng qu 1g/kg), TTM trong 2 gi. Khi ph nhiu v km triu chng nng, hoc tn thng da nng: Albumin 0,5 1g/kg TTM trong 4 gi + Furosemide 1-2mg/kg TM, gia lc truyn albumin. Nga tc mch: iu tr gim th tch, nhim trng, cho bnh nhn i li, vn ng nh, trnh t catheter.. D phng bng Anti-Vit K, Aspirine, khi albumin/mu 6g/l hoc anti thrombine III < 70%. H tr khi dng corticoid : Canxi 250 500mg/ngy Vitamin D (Sterogyl): 400 800 n v/ngy

Thuc bo v nim mc d dy Khng cn b sung Kali nu Kali mu bnh thng

7.5. Theo di:

Cn nng, chiu cao, que nc tiu Chng nga y Hc vn, vn ng th lc

Quan im chung: Trong giai on u nn cho bnh nhn nhp vin. Cn gim s ln nhp vin v gii thch r nguy c ti pht. Khng cho tr ngng i hc. Tin lng tt i vi dng p ng vi Corticoid.

TI LIU THAM KHO:

1. Niaudet P (2014). Available at: http://www.uptodate.com/contents/etiology-clinical-manifestations-and-

diagnosis-of-nephrotic-syndrome-in-children, last updated: Feb 28, 2014

2. Niaudet P (2014). Treatment of idiopathic nephrotic syndrome in children. Available at: http://www.uptodate.com/contents/treatment-of-idiopathic-

nephrotic-syndrome-in-children, last updated: July 09, 2014.

3. Nguyn ng Bo Minh, Trn Th Mng Hip (2012). Kho st s pht trin th cht v cc du n sinh hc v chuyn ha xng trong hi chng thn h l thuc Corticosteroid tr em. Y Hc TP H Ch Minh, Tp 16, ph bn s 4, 233-239

4. Nguyn Trng Tm, Vu Tiu H, Trn Th Mng Hip (2004). Kho st kin thc hnh vi chm sc ti nh ca m bnh nhi hi chng thn h iu tr ngoi tr ti bnh vin Nhi ng 2. Y Hc TP H Ch Minh, Tp 8, s 2, 36-38

5. Deschnes G (2012). Hmaturie. In: Bourrillon A (Eds). Pdiatrie pour le praticien, pp 613- 614. Elsevier Masson, Paris

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6. Niaudet P. Syndrome nphrotique chez lenfant. EMC (Elsevier Masson SAS, Paris), Nphrologie, 18-064-C-15, Pdiatrie. Maladies infectieuses,4-083-L-

10,2008.