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  • C IM S TO MU v MU NGOI BIN TR EM

  • MC TIU

    1. Trnh by c c im s to mu trong thi k bo thai v sau khi sinh.

    2. Vn dng c nhng c im mu ngoi bin tr em phn tch xt nghim cng thc mu ngoi bin.

  • S TO MU TR EM TRONG BO THAI

    S to mu phi thai c thc hin nhiu b phn (hnh thnh, bit ha dn t m gia cng s hnh thnh v pht trin thai nhi) v c t rt sm. T tun th 3: ti non hong T tun th 5 v ch yu thi k gia thai: gan (ch yu HC, t BC v TC) T tun th 6, mnh dn t thng th 4-5: ty xng T thng th 3, 4: lch (ch yu lympho, t HC)

    - T thng th 5,6: hch lympho, 1 phn tuyn c

  • SAU KHI SINH: Ty xng l c quan to mu ch yu

    - To mu mnh - s pht trin nhanh c th: tr nh (tt c ty xng)-ty , tr ln v ngi ln ( ct sng, sn, chu, c, s, b vai, n v 1 phn u x di)- ty vng . - Khng n nh, c kh nng hi phc. - D b lon sn khi b bnh mu v c quan to mu.

  • C IM MU NGOI BIN TR EMHNG CU: S lng HC thay i ty theo tui

    S sinh thng 4.5-6 x1012/l, ht thi k s sinh 4-4.5x1012/l6-12 thng : 3.2-3.5x1012/l ( TM sinh l)trn 1 tui : n nh dn > 4.0 x 1012/l HC li: - tr ss 1-3 ngy 8-10% , ss 4 ngy 0.5-2%

    - Ngoi ss 0.5-1% Nguyn HC c th thy tr ss: non thng: 3 - 6%, thng:1-4%

  • Hb: tr mi sinh 17-19g/dl

    6-12 thng 10-12 g/dlTr trn 1 tui, Hb li tng dn, trn 3-6 tui n nh t 12 - 14g/dl.- Th tch trung bnh HC (MCV): 80-100fl, s sinh >100 fl- Hb trung bnh HC ( MCH) : s sinh 36 pg, 6-12 thng 28 pg, trn 1 tui khong 30 pg.- Nng Hb trung bnh HC ( MCHC) : 30-33 g/dl

  • Thnh phn Hb:

    TuiHb A1(%)Hb A2(%)Hb F(%)

    S sinh20-400,03-0,660-802 thng40-700,9-1,630-604 thng80-901,8-2,910-206 thng93-972,0-3,01,0-5,01 tui972,0-3,00,4-2,05 tui v ngi ln 972,0-3,00,4-2,0

  • BCH CU- S lng bch cu thay i nhiu theo tuiMi sinh 10 - 100x109/l 7-15 ngy sau sinh 10 - 12x109/l Trn 1 tui, s lng bch cu mi n nh, trung bnh t 6-8x109/l. CTBC cng thay i nhiu theo tui: BCTT, lympho (hnh v), cc BC khc t thay i nh BCAT 1-2%, BCAK 0.1-1%, BCMN 4-8%.

  • Cng thc BC

    60 65%45%20 30%5-7 ngy9-10 thng5-7 tuiBCLPBCTT

  • TIU CUS lng tiu cu ni chung t thay i.Tr s sinh, s lng tiu cu t 100- 400x109/l.Ngoi tui s sinh, s lng tiu cu t 150-300x109/l.KHI LNG MUTr s sinh khong 14% trng lng c th.Tr ln khong 7-8% trng lng c th.

  • CC YU T NG MULc mi sinh, cc yu t II, VII, X, IX, XI, XII u thp hn bnh thng. T l prothrombin ch bng 6520,4%, thp nht vo ngy th 3-4 sau sinh, t mc bnh thng sau 1 tun (80-100%).

  • Case study 1Tr nam 6 tui, vo vin v thiu mu. CTM ngoi bin nh sau:- Hb 6.1 g/dl RBC 3.4 T/l (MCH 65 fl, MCH 22.5 pg, MCHC 28 g/dl)- BC 7.5 G/l (BCTT 62%, BCAX 1.4%, BCAK 0.6%, MN 8%, LP 28%)- TC 253 G/l C: Thiu mu nhc sc HC nh do thiu st / Polyp T xch ma.

  • Case study 2Tr n 13 thng, vo vin v thiu mu, vng da, lch to. Xt nghim in di HST cho thy:HbA1 = 34%HbA2 = 3,8%HbF = 62,2 % C: Beta Thalassemia

  • CHM SC TR THIU MU

  • Mc tiu: (1) Trnh by y cc nguyn nhn thiu mu tr em.(2) Trnh by y cc triu chng ca thiu mu(3) Theo di, x tr v chm sc y v chnh xc bnh nhi b thiu mu.

  • nh nghaThiu mu l tnh trng gim Hb hay khi hng cu trong mt n v th tch mu, Hb hay khi hng cu thp hn gii hn di ca ngi cng la tui.

    - Theo WHO, thiu mu khi Hb di gii hn sau:+ S sinh: < 140 g/L + T 6 thng n 6 tui: < 110 g/L+ T 6 n 14 tui: < 120 g/L+ Trng thnh: Nam < 130 g/L N < 120 g/L N c thai < 110 g/L.

  • 3 nhm nguyn nhn thiu mu: Thiu mu do gim sinh Thiu mu do tan mu Thiu mu do chy mu

  • 1. Thiu mu do gim sinh+ Thiu mu do thiu yu t to mu:. Thiu mu do thiu st. Thiu mu do thiu acid folic, vitamin B12.. Thiu mu do thiu protein. Thiu mu do s dng st km.+ Thiu mu do gim sn v bt sn tu:. Gim sinh nguyn HC n thun. Suy tu ton b bm sinh hay mc phi. Thm nhim tu: Bch cu cp, cc ung th di cn+ Cc nguyn nhn khc: suy thn mn, thiu nng gip, nhim khun mn tnh, bnh collagen.

  • 2. Thiu mu do tan muTan mu do bt thng ti hng cu, di truyn:+ Bt thng v hemoglobin: alpha-thalassemia, beta-thalassemia, bnh HbE, HbS, HbC, HbD + Bt thng mng hng cu: bnh hng cu nh hnh cu, hng cu hnh thoi....+ Thiu ht enzym hng cu: Thiu G6PD, thiu pyruvat-kinase, thiu glutathion reductaseTan mu do nguyn nhn ngoi hng cu, mc phi:+ Tan mu min dch: bt thng nhm mu m-con ABO, Rh, t min.+ St rt, nhim khun mu.+ Nhim c mt s thuc, ho cht, nc rn, nm c.+ Cng lch

  • 3. Thiu mu do chy muChy mu cp:+ Chn thng+ Xut huyt tiu ho+ Xut huyt no-mng no.+ Ri lon qu trnh cm mu.Chy mu mn tnh+ Giun mc+ Lot d dy hnh t trng.+ Tr, sa trc trng.

  • TRIU CHNG Biu hin ca thiu mu

    - Da xanh, nim mc nht- Mt mi, hoa mt chng mt, tr ln i hc th hin km tp trung, lc hc gim st.- Bnh nhi c th b suy tim do thiu mu nng Khi thiu mu do mt mu nhanh v nhiu, bnh nhi c th b sc do gim khi lng tun hon.

  • =>Ty theo nng hemoglobin, thiu mu c coi l:+ Nh khi Hb: 90-110 g/l+ Va khi Hb: 60-90 g/l+ Nng khiHbdi 60g/l2. Cc triu chng khc:Ty theo nguyn nhn m c thm cc triu chng ca cc bnh c s

  • Theo di, x tr v chm sci vi bnh nhn thiu mu nng:- Cho bnh nhi ngh ngi ti ging, th oxy, nm u thp.- ng k v truyn mu theo y lnh ca BS. Ch khi truyn mu cn phi theo di st cc phn ng bt li v tai bin ca truyn mu.

    i vi cc bnh nhi suy tim do thiu mu, sc do thiu mu v mt mu. X tr nh thiu mu nng.

    - Thc hin cc y lnh BS x tr nh: truyn mu, truyn dch, thuc iu tr, theo di mch, nhp tim, HA, nhp th nh k, nc tiu 24 gi.

  • Mt s x tr v chm sc c bit theo nguyn nhn- Thiu mu thiu st - Thiu mu do ty xng : xut huyt, nhim trng, au xng khp- Thiu mu do xut huyt- Thiu mu do tan mu: cp / mn tnh

  • CHM SC TR XUT HUYT

  • Mc tiu(1) Lit k y cc nguyn nhn xut huyt.(2) Trnh by y TCLS ca bnh nhn xut huyt.(3) Theo di, x tr v chm sc y v chnh xc mt tr b xut huyt.

  • 4 nhm nguyn nhn xut huyt theo c ch bnh sinh nh sau:1.Xut huyt do tn thng thnh mch 2. Xut huyt do nguyn nhn tiu cu 3. Xut huyt nguyn nhn do huyt tng 4. Xut huyt do nguyn nhn phi hp

  • Xut huyt do tn thng thnh mch - Do tng tnh thm thnh mch: thiu vit C.- Gim sc bn thnh mch:+ NK: NKH do no m cu, t cu, Dengue XH, st rt, Toxoplasma, Rickettsia.+ Nhim c: thuc (Aspirin), ha cht, ur huyt cao, nc rn.+ Huyt p cao.+ i tho ng.- Schonlein-Henoch (Vim mao mch d ng).- D dng thnh mch: bnh Rendu-Osler

  • Xut huyt do nguyn nhn TCRi lon v s lng:- Gim s lng TC: < 100.000/mm3 Tng s lng TC > 800.000/ mm3

    Ri lon v cht lng:

  • GIM S LNG TCNguyn nhn ngoi bin:- NK: VK ( NKH Gr (-), no m cu, vim ni tm mc ); VR: st xut huyt, si, rubeola, Herpes, CMV.- MD: Gim TC ng MD tr s sinh, sau truyn mu; XHGTC t min (ITP); cc bnh t min.- Cng lch.- Do tiu th nhiu: ng mu ni qun ri rc (DIC).- Bnh u mch mu ln.Nguyn nhn ti ty: suy ty (BS, MF), thm nhim ty (BCC, cc UT di cn ty), xm ln ty (x ty, xng ha ) Di truyn: khng c MTC bm sinh, ri lon sinh MTC (Wiskott-Aldrich).

  • TNG S LNG TCTin pht

    - Th pht: BC kinh, hi chng tng sinh ty, sau ct lch

  • Do ri lon v cht lnga) Di truyn:Suy nhc tiu cu (Glanzmann),Lon dng tiu cu (Jean-Bernard-Solier)Bnh v ht tiu cu.Thiu yu t 3 tiu cub) Mc phi:Thuc: mt s thuc chng vim, khng sinh...Ur huyt caoBnh h to mu: u ty lan ta

  • Xut huyt nguyn nhn do huyt tng. Ri lon sinh Thromboplastin ni sinhHemophilia A (thiu yt VIII), Hemophilia B (thiu yu t IX), Thiu yu t XII, C cht chng ng trong mu..Ri lon sinh ThrombinThiu yu t II (Prothrombin), Thiu yu t V (Owren), Thiu yu t VII (Alexander),Thiu yu t X (Stuart).=> bm sinh hay mc phi ( thiu vitamin K, suy gan )..Ri lon sinh Fibrin- Bt thng tng hp: bm sinh hay mc phi.- Tiu hy qu mc:+ Tiu th nhiu: DIC.+ Tiu Fibrin: hi chng tiu Fibrin cp.

  • Xut huyt do nguyn nhn phi hpBnh von-WilerbrandBnh mu c tnhBnh gan, thn, ung th ton th. Ri lon globulin mu gy xut huyt

  • Triu chng lm sng1. Hnh thi, v tr xut huyta) Di da: chm, nt hay mng bm mu di da.b) Nim mc: Mi ming (chy mu cam, chn rng hay li). Tiu ha: nn mu, a phn en hay a mu ti. TN- SD: i mu, a kinh hay rong kinh.c) C-khp: T mu c: sng, au cc c b t mu.T mu khp: sng au khp, hn ch V =>ti pht nhiu ln m iu tr PHCN khng tt: teo c, cng khp.d) Chy mu phi: kh th, ho mu, tro mu qua NKQe) Chy mu N-MN: au u, nn vt, HCMN (+), lit thn kinh s hay lit chi.

  • 2. Cc triu chng l hu qu xut huyt:- Thiu mu: cc mc khc nhau ty theo mc chy mu.- Gim khi lng tun hon (do chy mu nhiu v nhanh): mch nhanh, HA h thm ch khng o c.3. Cc triu chng khc: Ty theo nguyn nhn xut huyt khc nhau m c cc TC ca cc bnh c s.

  • Theo di, x tr v chm sc TD, nh gi mc chy mu: quan trng! Din bin mc thiu mu trn lm sng v nng Hb mu Theo di st mch, HA cht ch trong tng khong thi gian nht nh => pht hin kp thi gim khi lng tun hon

    Pht hin y cc v tr chy mu : di da, XHTH, SD-TN, chy mu phi, no mng no, c, khp, vt m...

  • Chm sc v x tr Cp cu ban u- Pht hin c thiu mu nhiu hay c gim KLTH => bo bc s truyn mu v chng sc.- XH N-MN c ri lon nhp th, c cn ngng th => lp tc bo bc s v chun b dng c thng kh h tr. - Cm mu ti ch: bng tm Adrenalin, nht microcell, Gelaspon l mi trc, bng p cho BN khi ch bc s n gii quyt.

  • X tr tip theo- Thc hin cc y lnh c hiu theo nguyn nhn ca BS: - TD st cc phn ng bt li khi truyn mu v ch phm mu- Chy mu khp gi khp t th c nng, iu tr phc hi chc nng theo y lnh ca bc s phng bin chng teo c cng khp - Ch v sinh c nhn bnh nhi c bit v sinh rng ming khi c chy mu chn rng

  • X tr tip theo (tip)- T vn cho cha m bnh nhi hemophilia cch hn ch chn thng v cc nguy c chy mu cho tr trong lao ng, hc tp v sinh hot.- Hng dn cha m bnh nhn bit khi no cn ti khm v truyn cc yu t ng mu thiu ht nh k phng chy mu cng nh khi tr cn phi tin hnh cc can thip chy mu. - Hng dn cha m bnh nhi bit cch cho con ung thuc ng khi iu tr ngoi tr