THIU MU

Ths.Bs. V Nguyn Th 1
 MC TIU

Trnh by c nguyn nhn ca thiu mu

Nu c triu chng, iu tr bnh thiu mu

2
SINH L TO MU

Stem cell

Proerythroblast

Early
erythroblast

Intermediate
erythroblast

Reticulocyte

RBC
3
4
 SINH L HNG CU
TNG HP HEMOGLOBIN

VC oxy
Duy tr cu hnh HC

HbF Bo thai Ngi

HbA dimer kp
HEMOGLOBIN
(2, 2) trng thnh
(2, 2)
HbA2
22

* HEMOGLOBIN (HB)
Hb = Hem + Globin
Hem = Fe2+ + protoporphyrin IX
Globin = 2 chui alpha + 2 chui beta
 NH NGHA

THIU MU
ANEMIA
S gim s lng hng cu hoc gim nng
Huyt sc t ngoi vi thiu oxy CC cho m tb
Thiu mu Hb < so ngi cng tui, gii, mi trng sng

H/C THIU MU

C NHIU NGUY NHN TM NN iU TR

NHIU TR/C C CH BNH SINH THIU MU
6
NGUYN NHN
THIU MU

GiM SINH
HNG CU

THIU NGUYN LIU GiM SINH TY XNG

THIU ST SUY TY
THIU VITAMIN B12 THIU ERYTHRPOIETIN
 NGUYN NHN
hng cu
THIU MU
TN HUYT

hng cu hnh bia

HNG CU V - B.L H/C

RL MEN HNG CU
BT THNG RL CU TO Hb
Thiu G6PD-Nst X
MNG H/C Thalasemia RL gen
tn huyt do
-BM SINH iu ha Hb HbF
+ thuc SR
HC hnh trn HC hnh lim:
+ PAS
HC hnh bu dc RL Hb aa6/
+ Sulfamid
HC hnh lim
+ Vitamin C
 THIU MU

TN HUYT HNG CU V - B.L H/C

+ D hp t: thiu mu nh
+ ng hp t: rt nng, t vong
Valin
Glutamat
HbS
Di truyn

HbA HbS

B ly oxy
Trng hp si xon kt tinh

HbA HbS
Tan trong vi mch
9
9
 NGUYN NHN
THIU MU

TN HUYT

HNG CU V - B.L NGOI H/C

(KT >< HC, CHT C, NT, CNG LCH)

Truyn nhm nhm mu, nhm O nguy him

Khc bit Rh m v con
Gim HC do thuc c ch t min + tham gia b th
Thiu mu tn huyt t min
Nhim khun, virus, KST
Nhim c: Phenol, benzen, quinin, sulfamid

10
NGUYN NHN
THIU MU

CHY MU

CHY MU - CP TNH CHY MU - MN TNH

Chn thng Tr
Phu thut Lot d dy
Xut huyt ni KST ng rut
 PHN LOI
THIU MU

3 CCH
PHN LOI

TH TCH TRUNG BNH

THEO NGUYN NHN C CH BNH SINH
HNG CU

HNG CU + TY SINH HC
+ NH + HY HOI HC
+ BNH THNG
+ TO
12
PHN LOI
TH TCH TRUNG BNH
THIU MU HNG CU

13
TRIU CHNG
LS THIU MU CP
Thn kinh: au u, hoa mt,
chng mt, ngt thong qua

H hp: kh th,
nhp th nhanh Tim: nhp nhanh,
ATTT ( )

HA M: gim mt mu nhiu
(>1,5 lt) try tim mch

C v khp: Da xanh, nim nht,

mi c, i li kh khn lng bn tay trng bch

Xut huyt nng hoc chn thng ngoi V mu cp oxy m c th

TRIU CHNG
LS THIU MU MN
Thn kinh: au u, hoa mt,
chng mt, tai,
ngt thong qua

H hp: kh th khi gng sc,

nhp th nhanh Tim: nhp nhanh, ATTT ( )
lu di - suy tim or cn au TN

RL tiu ha: n km,

i ngoi phn lng or to bn Mng tay kh, mt bng,
d gy, mng khum

RL sinh dc: RL kinh nguyt, rong kinh, Da xanh Xy ra t t,

or mt KN tnh dc nam nim mc mi mt di li nht
15
TRIU CHNG
LS THIU MU TN HUYT CP

Thiu mu t ngt
St cao, rt run

au bng

Vng da, nim: tng nhanh v r rt

Lch to: trong cn tan mu

Nc tiu sm mu, phn vng

Trong cn tan mu nng: c th suy thn, suy tim cp

16
TRIU CHNG
CN LM SNG THIU MU

DUNG TCH HNG CU Hct

Gim di mc bnh thng

NNG HEMOGLOBIN Hb
Gim di mc bnh thng

S LNG HNG CU RDW

Gim di mc bnh thng

17
 10 THNG S C HC
* RBC: s lng HC

* HGB: nng Hb

* Hct : dung tch hng cu

* MCV: th tch trung bnh HC

* MCH: lng Hb trung bnh trong mt HC

* MCHC: nng Hb trung bnh trong 100 ml HC

* RDW: phn b th tch HC

* WBC: s lng BC

* Diff : phn loi BC

* PLT : s lng TC 18
 THIU MU DO THIU ST
IRON DEFICIENCY ANEMIA

Fe2+ cn heme v ezym khc

75 90% trong Hb: 1ml khi HC-1mg Fe2+
Huyt tng: transferrin. Gan ferritin
Thiu mu mn TB hemosiderin (d tr)
hng cu nh,
nhc sc do thiu st

THIU MU - THIU ST

+ Thc n hp thu: DD, TT, trn R non

+ Hp thu Fe = mt 1mg/ngy
19
 PHN B ST TRONG C TH
Duodenum Dietary iron
(average, 1 - 2 mg
Utilization Utilization
per day)

Total Iron Binding Capacity

Plasma
transferrin (TIBC)
(3 mg)
Bone
Muscle marrow
(myoglobin) Circulating (300 mg)
(300 mg) erythrocytes
Storage
iron (hemoglobin)
(Ferritin) (1,800 mg)

Sloughed mucosal cells

Desquamation/Menstruation
Other blood loss
(average, 1 - 2 mg per day) Reticuloendothelial
Liver
(1,000 mg) macrophages
Iron loss (600 mg)

Tng s lng st trong c th 3000-4000 mg 20

 THIU MU THIU ST
NGUYN NHN

NGUYN NHN HON CNH C TH

Thiu dinh dng Ch dinh dng khng hp l

Gim hp thu Sau ct d dy

Tng nhu cu C thai
Cho con b
Cho mu
Mt mu Kinh nguyt ko di
DD rut
Giun mc, giun trn
Bnh i ra Hb

21
 THIU MU DO THIU ST
IRON DEFICIENCY ANEMIA

Thai v cho con b

Fe tng nhu cu: 2 5mg/ngy/thuc
Kinh nguyt
Rong kinh, lng nhiu
Do: nhu cu > hp thu
Thiu st

THIU MU - THIU ST
N

Ct d dy
Dinh dng km NL
DO GIM HP THU DD- R ko di: chy mu r r
22
TRIU CHNG
LS THIU MU THIU ST

Thiu mu mn tnh nh v va (mt, kh th - gng sc, nhanh)

thch n nhng thc n l

Giai on u: biu hin gim d tr st

Ferritin HT: thp
KN gn Fe ton phn HT (total iron binding capacity)
MCV: bnh thng

G. sau: d tr st thiu ht trm trng

Ferritin HT: thp < 30 g/dL

MCV, MCH, MCHC: gim
Nng: HC thay i kch thc khng u, bin dng
23
 IU TR
THIU MU THIU ST

Tm nguyn nhn thiu st loi b NN

B sung st
Thc n
Ung: vin st sulfat
200 300mg/ngy/3 6 thng

24
 THIU MU DO THIU VIT B12

B12 gn yu t ni hp thu vo mu
+ Cui hi trng Recepor (B12-YTN)
Cobalamin 90% - H.tng nh transcobalamin I,II,III
Ngun II v/c B12 GAN d tr
Thc phm ng vt
Hp thu rut
5 g/ngy

THIU MU - THIU B12

25
HP THU VITAMIN B12

26
 THIU MU DO THIU VIT B12
NGUYN NHN

Vitamin B12 c protein ng vt n chay thiu B12

PT d dy rut thiu vitamin B12

Ct on d dy thiu yu t ni
Ct b on hi trng mt hp thu vitamin B12

Him gp
Nhim sn dy c (diphyllobothrium latum): dng B12
Bnh Crohn: hy hi trng gim hp thu vitamin B12

NN chnh thiu B12

Thiu mu c tnh
Bnh RLDT t min vng Scandinave hay Bc u 27
THIU MU DO THIU VIT B12
NGUYN NHN

* Cc NN gy thiu vitamin B12

NGUYN NHN HON CNH C TH

1. Ch n khng c protein V n chay hon ton

2. Gim yu t ni ti Ct on d dy
Thiu mu c tnh

3. Cnh tranh gn B12 trong rut Bnh sn dy c

4. Gim hp thu B12 rut Phu thut ct b hi trng

Bnh Crohn

5. Thiu transcobalamin II (him)

28
TRIU CHNG
THIU VITAMIN B12

LM SNG
Tiu ha: chn n, tiu chy
Thn kinh: d cm Tt dy TK ngoi bin
Nng hn: tn thng ty or suy CN no

XT NGHIM
Thiu mu nng HC to (khng l), u sc
Pht mu ngoi bin: HC to, khng u, a hnh thi
Ty : sinh dng HC, kt HC ln bt thng
nh lng B12 HT < 100 pg/ml (bt 130 150 pg/ml)
29
SCHILLING
TEST

30
 IU TR
THIU MU THIU B12

VTAMIN B12: UNG TIM, LU DI SUT I

31
 THIU MU - THIU ACID FOLIC
Folate deficiency anemia

QU V RAU
(L CHANH V RAU XANH)
NHU CU: 50 100 g/ngy
QUAN TRNG
TO HNG CU

ACID FOLIC

32
 THIU MU - THIU ACID FOLIC
NGUYN NHN
* NN l CH N khng , t rau qu ti, nu qu chn
* Hp thu ton b rut t khi b thiu thiu do dng thuc
* Thai, bnh tan mu, bnh trc da: nhu cu x 5 10 ln
NGUYN NHN HON CNH C TH
1. Ch n thiu i, ch n khng hp l

2. Gim hp thu Tiu chy

Thuc phenyltoin, sulfaxalazin, bactrim

3. Tng thi tr Lc mu ngoi thn

4. Tng nhu cu Thiu mu tn huyt mn

C thai , bnh trc da

5. TD acid folic b c ch Thuc methotraxat, pyrimethamine (k.di)

Trimethoprim, co-trimoxazole 33
TRIU CHNG
THIU ACID FOLIC

LM SNG
Ging thiu vitamin B12
Thn kinh: khng c tn thng

XT NGHIM

Thiu mu HC to (khng l) ging vitamin B12

nh lng B12 HT: bnh thng
Lng folat < 9 nmol/L

34
IU TR
THIU MU THIU ACID FOLIC

ACID FOLIC: UNG p ng iu tr nhanh

35
 THIU MU TAN HUYT T MIN
Autoimmune Hemolytic Anemia

B bt gi li

KN bin dng

Fc Ct on

Tn huyt
BNH MC PHI Kuffer
T KT IgG
Gn mng HC THIU MU
C3b
TN HUYT Hot ha
T MIN b th

36
IgG
THIU MU TAN HUYT T MIN
Autoimmune Hemolytic Anemia

37
THIU MU TAN HUYT T MIN
NGUYN NHN

* a s KRNN

* Mt s trng hp

+ Lupus ban h thng

+ Bnh bch cu mn
+ U lympho ton thn

38
TRIU CHNG
THIU MU T MIN

LM SNG
Thiu mu xut hin nhanh, t ngt
C th e da tnh mng
Vng da, lch to
XT NGHIM
Thiu mu: mc va or nng
Huyt : HC li v HC hnh bi. Tn mu nng - HC nhn
Bilirubin mu:
Test combs (+): t KT IgG mng HC

39
 TEST COOMBS

1. Nguyn tc; tim khang th trn b mt hng cu hay tim khang

th lu hanh trong huyt tng.
2. Phn loai:
Coombs trc tip: T khang th trn b mt hng cu
Coombs gian tip: T khang th lu hanh trong huyt
tng.
3. Ky thut:
Ra hng cu BN nhiu ln loai cac protein trn mang.
U hng cu BN vi khang huyt thanh tho a clone co th
lin kt vi IgG va b th (C3).

40
41
 TEST COOMBS

Kt qua Coombs trc tip dng

1. Co khang th trn b mt hng cu. Hay co b th.
2. inh danh khang th khi Coombs trc tip dng
Phng phap Coombs mi trng co nhiu nhit
370C, 200C hay 40C
Test dng tinh 37 0: la IgG
Test dng tinh nhit lanh: la b th, cho bit co
khang th hoat ng mi trng lanh.Trong trng
hp nay cn phn bit IgM hay IgG Donath-Landsteiner.

42
 IU TR
THIU MU T MIN

PRENISOLON: C u tin
khng hiu qu or ti pht liu cao ct lch

THUC UCMD: C
khng p ng prenisolon v ct lch

THIU MU TRM TRNG

TRUYN MU V TD CHC CH

43
 THIU MU SUY TY
Bone marrow failure
SUY TY
L tn thng Tb gc
ton b cc dng tb
mu ngoi bin
TT C TB MU
T TB GC-TY

SUY TY

44
THIU MU SUY TY
NGUYN NHN

* Tia x, ha tr liu,
do thuc (Chloramphenicol, phenylbutazol, methotrexat)

* Mt s bnh

+ Lupus ban h thng: tb gc b c ch t KT IgG

+ Suy ty KRNN c ch t min

45
TRIU CHNG
THIU MU SUY TY

LM SNG 3 HI CHNG
Thiu mu do gim HC
Nhim khun do gim BC
XHDD v nim mc do gim TC

XT NGHIM

Thiu mu: nng

Huyt : gim c 3 dng. Trong g u ch 1 or 2 dng
Sinh thit ty: Ty ngho tb, khng thy tb bt thng

46
 IU TR
THIU MU SUY TY

TRUYN KHI HC V TC

KHNG SINH: C
C nhim khun

GiM NEUTROPHIL (++): dng GM-CSF KT N v nhim khun

GHP TY, GHP TB GC: c iu kin

SUY TY LUPUS: prenisolon

47
 IU TR
THIU MU SUY TY

HNG CU LNG

Hng cu lng: mu ton phn bng bt 80-90% plasma

Dung dch bo dng cha dextrose, adenine v mannitol cho php d tr
c 42 ngy
Hng cu: 4o C, 1 n v hng cu lng = 250 ml, c Hct 70%, s lm tng
hemoglobine ln 1 g/dL hay 3% Hct.
Hng cu lng khng cha yu t ng mu, nn sau khi truyn nhanh
khong 5 n v hng cu lng th phi truyn plasma ti ng lnh
u im: hng cu lng so vi mu ton phn l gim nguy c qu ti th
tch, gim lng citrate, ammonia v cc acid hu c, gim nguy c bnh
min dch (allo immunization) nh cha t khng nguyn
Hng cu lng lm tng nhanh kh nng chuyn ch oxygen BN b chy
mu cp hay mn
48
THIU MU BNH MN
NGUYN NHN

* Vim or nhim khun mn, ung th, bnh gan

gim nh i sng HC ty sinh HC khng b

* Bnh thn mn tnh

+ Do gim erythropoietin
+ ng cht c trong mu i sng HC

49
TRIU CHNG
THIU MU BNH MN

LM SNG

TR/C bnh mn tnh km thiu mu

XT NGHIM

St HT: thp
Kh nng gn st ton phn HT thp
Ferritin HT: bt or
Hct: , MCV bnh thng

50
 IU TR
THIU MU BNH MN TNH

TRUYN KHI HC KHI C THIU MU NNG

ERYTHROPOIETIN TNG HP
Tim di da

51
XUT HUYT

52
 MC TIU

Trnh by nguyn nhn, triu chng, PP iu tr

mt s bnh l xut huyt

53
 HI CHNG XUT HUYT

Xut huyt (Bleeding disorders)

L hi chng biu hin tnh trng mu thot ra khi thnh mch
Do v mch or khng do v mch
 NGUYN NHN
HI CHNG XUT HUYT

NN
XUT HUYT

TN THNG DO DO BNH
THNH MCH TIU CU HUYT TNG

55
 NGUYN NHN
HI CHNG XUT HUYT

TN THNG
THNH MCH

Thiu vitamin C, PP
Nhim khun: si, NK huyt do no m cu
Mt s bnh mn tnh: lao, T, x gan
Bnh Scholein Henoch: d ng TT thnh mch
 Vitamin C

C ch tc dng
Acid ascorbic b oxy ha cho acid dehydroascorbic; y l phn ng oxy ha kh thun
nghch, qua Vitamin C tc dng nh mt ng yu t (cofactor), tham gia vo nhiu phn
ng ha sinh trong c th, nh:
Hydroxyl ha,
Amid ha;
Lm d dng s chuyn prolin, lysin sang hydroxyprolin v hydroxylysin (trong tng hp
collagen);
Gip chuyn acid folic thnh acid folinic trong tng hp carnitin;
Tham gia xc tc oxy ha thuc qua microsom (cytochrom P450) gan;
Gip dopamin hydroxyl ho thnh nor-adrenalin;
Gip d hp thu st do kh Fe3+ thnh Fe2+ d dy, ri d hp th rut.
m, Vitamin C gip tng hp collagen, proteoglycan v cc thnh phn hu c khc
rng, xng, ni m mao mch.

57
 NGUYN NHN
HI CHNG XUT HUYT

DO
TIU CU

BNH TIU CU BM SINH

Bnh tiu cu suy nhc Glanzmann

+ Bnh DT gen ln trn Nst thng
+ SL tiu cu bnh thng RL tp trung tiu cu

Bnh Willerbrand
+ Bnh DT tnh tri
+ Thi gian chy mu ko di, co cc mu bnh thng

58
 NGUYN NHN
HI CHNG XUT HUYT

DO
TIU CU

GIM TIU CU TH PHT

Suy ty
+ Gim TC : do nhim c thuc or ha cht, cht c

Ty b ln t
+ Ty b ln t: do thm nhim tb c tnh
SL mu TC TC ngoi bin
+ Gp: Leukemia cp. Leukemis th ty mn g chuyn cp

59
Platelet functional disorders

60
 NGUYN NHN
HI CHNG XUT HUYT

DO
TIU CU

GIM TIU CU TH PHT

D ng
+ Thuc c th sinh KT >< TC
Tng hy TC
+ Cng lch th pht

Nhim khun
+ St XH, NK huyt

61
 NGUYN NHN
HI CHNG XUT HUYT

DO
TIU CU

XUT HUYT GIM TIU CU T MIN

62
 NGUYN NHN
HI CHNG XUT HUYT

DO BNH HUYT TNG

THIU HT YU T TO THNH THROMBOPLASTIN

Bnh Hemophyllia
+ Lin quan thiu yu t VIII, IX
+ Bnh DT Nst X trai
Thiu prothrombin v proconvertin
+ Tn thng gan, mt, tt ng tiu ha. Thuc khng ng
khng prothrombin
Bnh tiu si huyt (tiu fibrinogen v fibrin)
+ Sau PT: phi, sn ph khoa, NT huyt, nhim c
+ Hy qu mc YTMu v TC v gp sp gin ha fibrin
+ NN: hy hoi c quan Streptokinase, urokinase
CH (plasminogen plasmin) v gin ha fibrinogen
c ch to thromboplastin v CN TC H/C XH nng 63
 G TO CC MU NG

* G 1: to prothrombinase
* G 2: chuyn prothrombin thrombin
* G 3: chuyn Fibrinogen fibrin

Tan cc mu, vai tr plasmin

Plasminogen (hh: tPA, thrombin,XIIa,lysosym,urokinase)
plasmin: tan cc mu (hy fibrin)
C ch chng ng c th:
Nga tc mch, qa trnh lin so

64
TRIU CHNG
XUT HUYT
XH t nhin hay va chm, th thut rt nh (tim, nh rng)
XUT HUYT DI DA
Bt k v tr no, SL nhiu t ty mc nng nh
mu sc thay i theo thi gian: tm vng xanh mt
Kch thc XH
+ Chm XH: nh < 1mm
+ Nt XH: 1 10 mm
+ mng XH: 1 10 cm
+ m XH: tp hp chm-nt-mng XH
+ Khi t mu: cc di da
XUT HUYT NIM MC
Li, rng li: chm, khi mu t or chy mu
XUT HUYT CC TNG
T cung, d dy, thn tit niu, gan, lch, phi. ti pht 65
TRIU CHNG
CLS - XUT HUYT

XT NGHIM
1 Nghim php dy tht: nh gi sc bn thnh mch
(+) sc bn thnh mch

2 nh gi tiu cu:
+ Thi gian mu chy
+ Thi gian co cc mu
+ Thi gian tiu th prothrombin
+ m SL tiu cu

nh gi YT ng mu
3
+ Thi gian ng mu
+ Thi gian Howell
+ Thi gian Quick v t l phc prothrombin
+ nh lng - fibrinogen
XH GIM TIU CU T MIN
Xut huyt gim tiu cu t min
(Immunologic thrombocytopenic purpura)
L bnh t min
T KT IgG gn TC

Fc b TB lch gn TC hy TC

67
 IU TR
XH GIM TC TM

Khi u prednisolon liu cao i lc TB lch

gim liu dn

CT LCH
Bin php tt nht, 80% BN c tin trin tt

THUC UCMD: khng p ng prednisolon v ct lch

Cyclophosphamid, vinblastin, azathioprin

TRUYN
GAMAGLOBULIN

68
TRIU CHNG
XUT HUYT GIM TC TM

LM SNG
Gp: tr, n > nam (2:1)

TR/C XH
+ Chy mu cam, ming
+ Rong kinh
+ Vt bm tm or chm XH di da
+ Lch to ()

XT NGHIM
+ SL TC: gim < 100.000/mm3
+ Dng tb khc bnh thng
+ Huyt : bt tr TC hi to
69
 BNH A CHY MU
HEMOPHILIA

Bnh a chy mu Hemophilia

l bnh bm sinh do DT Nst X

+ Thiu yu t VIII:
Hemophilia A
+ Thiu yu t IX:
Hemophilia B

70
TRIU CHNG
HEMOPHILIA

LM SNG
Bnh cng nng XH cng sm
Sau: Chn thng d nh
t t cm. Ti pht ni nht nh
V tr - XH
+ Vt thng ngoi da
+ Hc t nhin (mi, rng, li)
+ Khi mu t di da, vm hng, h mt, c mng
+ khp ()
+ CC: tim mng, chc d khp
XT NGHIM xc nh, phn loi v theo di iu tr
Thi gian mu chy ko di (8 10)
Thi gian Howell mu b ko di (130 215
Thi gian tiu th prothrombin b rt ngn 71
72
 TRIU CHNG
XT NGHIM MU

Thi gian Howell (thi gian phc hi Calci)

Nguyn l: mu khi mch mu b ng.
giai on hot ho yu t X (yu t IX v VIIIa hot ho X) - cn ion Ca2+
Ko c Ca2+ khng to nn fibrin citrat - chng ng mu (ta cc ion Ca)
Thi gian Howell: thi gian phc hi calci - thi gian t khi cho calci
(Ca[sup]++[/sup]) vo huyt tng chng ng bng citrat cho n khi
cc ng c hnh thnh
Bnh thng: 1,5 2,5 pht
Ko di: Thiu ht yu t ng mu ng ni sinh (thng nht l VIII, IX)
Gim s lng hay cht lng tiu cu
iu tr heparin

Ch s INR (international normalized ratio):

c dng theo di iu tr khng ng vi warfarin.
73
INR= (PT ca BN/PT chng). Gii hn ca iu tr warfarin: INR=2-4
 TRIU CHNG
XT NGHIM MU

Thi gian Quick (PT hay TQ)

Dng Thromboplastin chit xut cho vo mu phc hi calcium kho st

kt qu, c qui thnh t lt Prothrombin. Bnh thng >60%
Kho st yu t I,II,V,VII,X
TQ c th ko di do suy gan, tc mt hoc do thuc khng Vit K
nn thng c theo di iu tr chng ng v khng Vit K

Thi gian Thromboplastin (aPTT)

nh gi cc yu t ng mu theo con ng ni sinh (kininogen TLPT cao,

prekallikrein, XII, XI, IX, VIII) v con ng chung (fibrinogen, II, V, X).
aPTT ko di: c s thiu ht hay c ch bt c yu t ng mu no, tr VII
Dng theo di qu trnh iu tr bng heparin.

74
 Cc XN nh gi qu trnh to phc
Prothrombinase

Thi gian
Howell
Thi gian
PTT Quick

aPTT (PT)
PT%

75
 IU TR
HEMOPHILIA

TRUYN YU T NG MU
VIII hemophilia A IX Hemophila B

76
 THIU VITAMIN K
VITAMIN K DEFICIENCY DISORDERS

VITAMIN K ng vai tr quan trng QT NG MU

- ng yu t thc y hot ha yu t X, II

CUNG CP:
thc n
ni sinh QT vi khun rut

NGUYN NHN:
Dinh dng km
Hp thu km
Khng sinh ba bi: lon khun c li cho vitamin K
77
TRIU CHNG
THIU VITAMIN K

LM SNG
Gp: Sau PT khng n ung c v dng khng sinh ko di

TRIU CHNG
+ Khng c trng
+ XH bt k ch no

XT NGHIM
+ Thi gian prothrombin ko di
+ Ch s huyt hc: bnh thng

78
 IU TR
THIU VITAMIN K

VITAMIN K TIM DI DA
p ng nhanh

79
 BNH SCHOLEIN HENOCH
SCHOLEIN HENOCH PURPURA

XUT HUYT DO HIN TNG

D NG GY TN THNG THNH MCH
Cha c hiu tng tn
vim mch mu qua trung gian min dch, km
lng ng IgA.
Lin quan nhim trng nhng NN vn cha r
Min dch, yu t gen v yu t mi trng c l
ng vai tr bnh sinh.
c trng: vim mch mu, lng ng bch cu
(neutrophils v monocyte) km phc hp min
dch IgA trong cc c quan b nh hng
Tng IgA trong mu

Yu t gia nh: him (sinh i, h hng, )

Gen: HLA B35, DR4
Khim khuyt gen C4 c tn sut mc bnh cao
80
TRIU CHNG
SCHOLEIN HENOCH

LM SNG
Gp: ngi tr v tr em. N > nam
TRIU CHNG
+ XH di da (++): chm , khng dnh nhau chi
XH trc cng chn, gn khp v i xng 2 bn
+ TR/C khc: au lng, RLTH, au c, au khp, st nh

CN LM SNG
+ XN - mu: trong gii hn bnh thng
+ Sc bn thnh mch gim, tng tnh thm v tt mao mch
81
 IU TR
SCHOLEIN HENOCH

PREDNISOLON
ban u, c p ng tt

82
 NG KINH

83
http://tailieu.vn/doc/bai-giang-chan-doan-va-dieu-tri-dong-kinh-1787707.html
 MC TIU

Trnh by nguyn nhn v phn loi bnh NG KINH

M t cc triu chng lm sng ca NG KINH

Trnh by PP iu tr ca BNH NG KINH

84
 NH NGHA
NG KINH
EPILEPSY

NG KINH l s ri lon tng cn chc nng ca TKTW do

S phng in T NGT QU MC NG THI
Ca cc NEURON

CN NGN VI GIY N 1 PHT

NH HNH T NGT RL TKTW MT THC
85
NGUYN NHN
NG KINH

HI CHNG NG KINH amitriptyline.

* Tai bin hay triu chng ca RL chc nng no tm thi

* NO b kch thch cn ng kinh n c

* TC NHN KCH THCH

Thuc chng trm cm 3 vng
Nhim trng no hoc vim no
Tng p lc ni s, CTSN, AS mnh
RLCH: mt cn bng ng huyt, h canxi mu,
RL pH or p sut thm thu mu, thiu pyridoxin

86
 NGUYN NHN
NG KINH

BNH NG KINH (Epilepsy)

* NHIU NN TUI KHI PHT LIN QUAN N NN

* D TT BM SINH V SANG CHN TK CHU SINH:

ng kinh tr s sinh v tr nh
* BT THNG CU TRC NO
U no
D dng mch mu, XH di nhn, teo no
CTSN
Nhim khun: cp VMN, vim no
(virus, giang mai, kn sn)
Nghin ru
87
Khng tm thy NN bnh ng kinh nguyn pht
 PHN BIT
CN V BNH - NG KINH

Cn ng kinh (Seizures) Bnh ng kinh (Epilepsy)

L hu qu ca bnh l cp Cn khng c yu t khi
tnh v tm thi (yu t khi pht
pht: st, h ng huyt )
Ti pht thng xuyn (trn 2
Khng ti pht nu cn cn) v phi iu tr lu di
nguyn c gii quyt
C th hoc khng th tm
Th d: co git do st, hi thy nguyn nhn
chng ngng thuc an thn,
chn thng s no

88
 PHN LOI

NG KINH

NG KINH CC B (Seizures)

NG KINH
Thy trn, nh, D on tn thng c hiu
chm, thi dng K
Vng no?
CC B

LS NG KINH
Tr/c vn ng, cm gic
Gic quan, thc vt
V or tm tr khng c tc 2 bn
Lin quan CN TK ring bit

89
 PHN LOI

NG KINH

TON B TIN PHT

Phng lc qu mc
V lan rng tb no or
Di v ca 2 bn cu TON B
TIN PHT

Khi u
Mt thc
TT nhm c ln cn i 2 bn
+ K rung git co cng (cn ln)
+ Vng thc (cn nh)
90
PHN LOI

NG KINH

TON B HA TH PHT

TON B
TH PHT
S phng lc ca cc tb thn kinh
Lc u 1 vng
lan ta nhiu vng c 2 bn cu

91
 C ch c bn ca ng kinh

Dng Ca2+

Thay i in th
m knh Na +

Dng Na+

in th lp i
lp li
92
Cc cht dn truyn thn kinh

93
94
 tn hiu in kch thch hay c ch (EPSP hay IPSP).

C CH BNH SINH
NG KINH

GiM NGNG KH CC
MNG NEURON Mt cn bng [CHT DT - TGTK]

Kch thch c ch

kh cc qu mc
Glutamate GABA
 TRIU CHNG
LM SNG

Khi pht: t ngt, khng lin quan t th v cng vic

Mt s kch thch cn c trng

VD: AS cng mnh, stress, sa st tm thn

96
 TRIU CHNG
LM SNG

NG KINH CN LN

* Thng gp nht din bin 5 giai on

Khng d nhn bit

TIN TRIU
Tr/c khng c hiu c xy ra vi gi trc :
au u, thay i kh sc, th
TR/C Tr/c c hiu hn
BO TRC Nga hay ngi thy mi l, au thng v
(vi giy, vi pht trc cn)
Co tht ton thn c c trn v c vn. 30 1
CO CNG
Hu qu: mt thng bng, t ng, co tht c HH
(la ht ngn khng t ch, tm ti), 97
Cn co cng-co git (tonic clonic) 98
 TRIU CHNG
LM SNG

NG KINH CN LN

Hng lot cc cn co git, 2 3 hn m su

CO GIT
Mt thc, si bt mp, cn mi, li
i tiu tin khng t ch, chn thng
Th gin, lit mm v ng ngc i vo gic ng
SAU CN Sau vi gi BN tnh dy: au u, ln ln,
khng nh nhng g xy ra

99
 TRIU CHNG
LM SNG

CN VNG THC

RL thc, cn or mt TLC
C TRNG Cn thc vt (i dm)
Khi pht v kt thc t ngt
Absence seizures
Nhm ng gt hay sa st trong hc tp
GP TR EM
Vi cn mi ngy ( 100 cn) tr chm ln

Bt u tr nh ht 20 tui
DiN TiN
cn vng thc thay bng cc cn ton th khc
100
 TRIU CHNG
LM SNG

CN NG KINH CC B

Cn ng kinh bit chnh xc v tr kch thch

C IM Nhiu th K cc b khc nhau, cn vi pht
K thy thi dng hay gp nht # 50% KCB
Tr/c TK a dng
TRIU CHNG Tm thn, mt ngn ng, RL tm thn, o gic
chong v ngt 101
 TRIU CHNG
LM SNG

CN K LIN TC TRNG THI K

Ch cn ng kinh ko di khng t kt thc

C IM or xy ra lin tip khong ngn K khng dt

X TR CP CU TI BNH ViN

102
 TRIU CHNG
CN LM SNG

IN NO - ECG
QUAN TRNG NHT ElectroEncephaloGraphy
C nghi ng cn ng kinh
GIP THY THUC CT scanner no, MRI
CHN ON PHN BiT
PHT HiN TT KHU TR NO XC NH
PHN BiT Tn thng ni s
Cn ton th t V
Cn cc b or Tm NN PT ?
Cn ton th th pht
HUYT HC, SINH
NH GI
Kt qu iu tr HA, VI SINH
103
Toxoplasmosis no BN - HIV 104
105
 TIN TRIN V BIN CHNG

Khng tin trin (khng xu i)

C IM XU HNG Tr em khi ln thot bnh
CHUNG Ngi ln t khi (KRNN)
Ngng thuc t ngt xut hin cn

K v iu tr ko di
khng gy tn hi tr tu
THN KINH
Cc cn lin tip khng kim sot
thiu mu no tn hi no

Kh khn v mt tm l x hi
NH HNG (hc tp, gii tr, quan h XH), gii hn BN
Trm cm nguy c t t gp 5 ln
(K thi dng hay phi hp bnh tm thn
- Tm thn phn lit)
106
 IU TR
IU TR - KHNG THUC

Sinh hot iu , trnh cng thng, gic ng tt m bo

Trnh cng vic c th gy nguy him cho BN
khi ln cn t ngt:
Lm vic cao
Li tu, xe
Tuyt i king ru

107
108
 IU TR
IU TR - THUC

NGUYN TC IU TR

La chn loi thuc ph hp vi tnh trng bnh

Liu thuc ty vo th bnh, loi cn, th trng BN

Tt nht o [thuc] hiu chnh liu

Thuc: u n, ng v liu hng ngy

Theo di: LS, ADR thuc (LS v sinh ha) chnh liu ph hp

109
 IU TR
IU TR - THUC

C CH TC DNG

c ch knh Na+

Tng cng GABA: tng m knh Cl-, tng s ln m knh Cl-,

c ch GABA transaminase

c ch knh Ca+

Tng tnh thm K+

110
 S ra i ca cc thuc chng ng kinh
1857 Sodium bromide
1912 Phenobarbital ( Gardenal )
1938 Phenytoin ( Dilantin )
1946 Trimethadone
1953 Primidone ( Mysoline )
1954 Methsuximide
1958 Ethosuximide ( Zarontin )
1960 Benzodiazepines
1974 Carbamazepine ( Tegretol )
1978 Valproic acid ( Depakine )
1993 Felbamate ( Felbatol )
1994 Gabapentin ( Neurontin )
1995 Lamotrigine ( Lamictal )
1996 Fosphenytoin (Cerebyx )
1997 Topiramate ( Topamax )
1997 Tiagabine ( Gabitril )
1998 Vigabatrine ( Sabril )
2000 Oxcarbazepine ( Trileptal )
2000 Levetiracetam ( Keppra )
.
111
 S ra i ca cc thuc chng ng kinh

Valproic acid ( Depakine )

Carbamazepine ( Tegretol )
Phenytoin ( Dilantin )
Phenobarbital ( Gardenal )
Sodium bromide

112
 IU TR

THUC
NG KINH

CARBAMAZEPIN do c ch knh Na+

PHENYLTOIN c ch knh Na+
PHENOBARBITALT ln GABA, Chn knh Ca+
ETHOSUXIMID tt trn cn vng thc
DIPHENYL HYDANTOIN
PRIMIDON
CLONAZEPAM
VALPROAT NATRI
c ch knh Na+, tng GABA, gim dng Ca2+
114
 Chn la thuc theo chi ph iu tr

115
Chi ph iu tr
 IU TR
NGOI KHOA

ng kinh cc b do
tn thng cc b v no
V tr ca ng kinh:
Vng c th phu thut c
Nguyn nhn iu tr PT
U mng no, u no
D dng mch mu no
mu t - CT or do XH

116
 Toxicity of Phenytoin
Ch nh: cn cc b n gin hay
phc tp, cn co cng co git
P- p450 Interaction p450 Tng tc
H- Hypersensitivity, hyperglycemia, hirsutism,
hypoprothrombinemia Qu mn, tnggim ng huyt, rm lng,
prothombin mu
E- Enlarged gums nu ph
N- Nystagmus rung git nhn cu
Y- Yellow discoloration of skin b vng da
T- Teratogenic gy qui thai
O- Osteomalacia long xng
I- Interferes with metabolism of folate Cn tr s trao i cht ca folate
N- Neuropathies bnh thn kinh
117
118