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29 Cards in this Set

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mucocutanous bleeding (post surgical –tonsillectomy/dental extraction, ecchymosis, menorrhagia-poor fibrin clot, + FH of bleeding; dental exraction bleeding; # 1 bleeding diz
-? dx
dx Nl- Hi PTT; Hi BT
(intrinsic pathway)-PT NL -low vWF; low ristocetin cofacor;-Factor VIII nL- low
-Von Willebrands***
Defic of GP IIB/IIIa,
-impaired plt aggregation ; Severe bleeding
Glanzman’s Thromabsthenia
Def of GP1b -severe bleeding/ GI bleeding Giant plt
-Hi BT
Bernard Soulier Syndrome
Uremia,
Meds: aspirin – irreversible-inhibits plt ; NSAIDS - transient
Plt dysfunx
d/o DNA repair; affects all cell lines;
-see plt decrease first Short, renal probs, radial ray anomolies
Fanconi Anemia
Severe low plt @ birth—tx: Bone marrow
Cong amegakarysocityic thmoboctyponeia
2-4 yo; post infx, self limitied; Isolated thrombocytopenia; -petechiae, purpura, epistaxis, gingival bleeding;
- chronic > 6 mo;
ITP
only one cell line down; low plt. what's tx?
ITP,
Tx: IVIG; steroids
? what are vit K dependent cofactors?
2, 7, 9, 10, prot C/S
- what deficiency leads to prolonged PT and PTT;
-?
-vitamin K deficiency
examples of causes leads to prolonged PT/PTT ?
causes of defieincy: dieeray insufficiency; atnbx, malabsorption; warfaring, INH, rifampin, Phb
what's normal PT ?
PT 9-13
what's normal value of PTT?
-what's normal value in NB?
23-32/
NB: 23-45
which factors are intrinsic and which one ~ with intrinsic: PT or PTT?
12, 11, 9, 10; 8, ~ vWF;
-intrinsic ~ PTT (PiTT)
which factors are extrinsic and which one ~ with : PT or PTT?
PT (PeT); ; 7, 10
NB w/ Bleeding, GI, intracnatial; purpura, oozing, bleeding w/ circs; h/o maternal ingest of vit K antagonists (anticonvulsants) or failure to give Vit K @ birth
hemorrgic diz of NB
Deep soft tissue/joint bleeding; NL PT; Hi PTT in a boy; due to what? what's tx ? what's genetics?
Hemophilia 8,9; -x linked;
low 8 or 9;
Tx: Replace factor; cryo; FFP; DDAVP; aminocaproic acid
Vit K dependent-warfarin sensitive;
-Homozygous: neonatal purpura fulminans;
-heterozygous: neonatal purpura fulminans : massive microthrombosis, skin necrosis, DIC
~ wat def?
PROTEIN C /S DEFIENCY – factor 2, 7, 9, 10
Aq’d: DIC, liver dysfxn, nephritic syndrome; IBD; L –asparginase;
-when heparin fails to anticoagulate
Antithrombin III deficiency
tx of ANtithrombin III def?
warfarin
mutation → ? this becoming resistant to cleavagee by Protein C;
-late onset thrombosis; OCPs can confer APC resistance
Factor V leiden: activated protein C resistance:
G20210A increases prthrombin conc
-DVT; promotoes enhanced thrombin generation
Hyperthrombinenemia- prothrombin gene mutation
Defect in converting homocysteine to methione;
-defect in ? what gene?
-increased risk of areterial DVT; Hi levels of homocysteine; what's tx?
MTHF gene;
Tx: folate
prolongs PTT,but not a bleeding disorder!!!;
-h/o mom w/ recurrent miscarriages;
-inflamm/autoimmune disease, infx, meds; recurrent DVT; stroke in young pts; recurrent pregnancy loss
Antiphospholipd syndrome-lupus like anticoagulant
Binds to ATIII and inhibits thrombin, 9, 10, 11, 12;
what do you follow? Pt/or PTT?
-how do you reverse?
Heaprin/LMWH
-follow PTT?
-reverse w/ Vit K
Inhibits gamma carboxylatino of F 2, 7, 9, 10, Prot C; vit K dependent factors
- follow w/ PT
- what's it reversible with ?
warfarin;; reversible w/ Vit K
10 yo boy w/ RLQ pain, palpable mass in RLQ, pain on mcburney’s point; hip externally rotated in flexed position;
Hemophilia A or b; send for factor levels
deep delayed bleeding, males, Hi PTT, low Factor 8; Nl vWF; Nl ristocetin cofactor
hemophilia
superficial immediate bleeding; males/females; Nl or hi PTT, nl or low F 8; No or low v WF **; decreased ristocetin cofactor
vWD