UW 5 Flashcards
Hemophilias (clasification)
Deficiency:
A=VIII (most common) XR
B= IX. XR
C= X. AR
Hemophilia diagnosis
PPT is prolonged. PT and BT normal
Best initial test: Patients blood + normal plasma = correction on PTT
Most accurate: Obtain specific factor deficiency. Look for 8, 9, 10, 11 and 12
Treatment of hemophilias
Depends on severity of defect
if <1% of normal: inmidiate factor transfusion
if >5% (mild) or 1%-5% (moderate): desmopresin===> factor 8 release
Prophylactic application of clotting factor concentrates is the basis of modern treatment for severe hemophilia A.
Functions of wWF (3)
- Bring platelets to exposed endothelium
- Transport/bind factor VIII
- Aggregate platelets
Labs of vWF deficiency and diagnosis
Increased bleeding time. PTT (normal or elevated) normal PT
Ristocetin cofactor essay (messures vWF capacity to aggregate platelets)
Treatment of vWF deficiency
Mild
Severe
Prevention
Desmopressin for mild cases
Factor VIII concentrate or vWF for severe cases
Control bleeding with OCPs, avoid ASAs and NSAIDs and platelet function inhibitor
Pathophysiology Factor V Leiden
Factor V is resistant to cleavage by protein C
Increased risk for DVT and PE is 5 fold if heterozygous and 50fold if homozygous
Young, white patient with personal or family hx of DVT/PE in unusual locations
Heparin-induced thrombocytopenia
Platalet agregation and a >30% decrease of platelet count after the administration of heparin (most common with unfractionated)
5-10 after. Can present with necrosis at injection site
Venous (most common) and arterial thromboembolism can occur
Diagnostic tests for:
Factor V leiden
Heparin-induced thrombocytopenia
Antiphospholipid sd
a. APC resistance test
b. Platelet factor 4 antibody or serotonin release assay
c. lupus anticoaguland and anticardiolipin
Managment of thrombophilias
Heparin-Warfarin for 3-6 months on 1st time event
Lifelong anticoagulation for >2 events
Vena cava filter if anticoagulation is contraidicated
Management for Heparin-induced thrombocytopenia
Stop heparin
Direct thrombin inhibitors (fondaparinux, argatroban and bivaluridin)
Switch to Warfarin
Labs in DIC
Cause by Fibrin deposition in small blood vessels. Present with both thrombosis and hemorrhages
Elevated PT and PTT
Low platelets
Elevated D-dimer
Eleveted fibrin, low fibrinogen
Presentation of TTP and HUS
LMNOP
Low platelet count
Microangipathic hemolytic anemia (jaundice, pruritus)
Neurological sings (seizures, stroke, confusion, decrease vision)
Obsolete renal function (AKI)
Pirexia
HUS does not present with neurologic sings and is more common in children with E. coli infection O157:H7
Common associations with Thrombotic trombocytopenic purpura (8)
SLE Malignancy Pregnancy Cyclosporine, quinidine, clopidogrel, ticlopidine AIDS
Labs in TTP and HUS
Low platelets, low hemoglobin, increased indirect bilirrubin
Normal clotting/bleeding labs
Schystocytes on smear
Pathophysiology of TTP and HUS
deficiency in vWF cleavage enzyme (ADAMTS-13)
Abnormaly large vWF, microthrobi formation that hemolysis (microangipatic hemolytic anemia)
Pathophysiology of Idiopathic thrombocytopenic purpura
IgG against Platelets.
Platelets destroyed in the spleen
Bone marrow increases production of megakaryocytes
Common associations with Idiopathic thrombocytopenic purpura
HIV, Hep C
SLE, lymphoma, leukemia
Presentation of Idiopathic thrombocytopenic purpura
Acute: abrubt onsent bleeding following a viral illnes with self limiting purpura. Common in children 2-6 yoa
Chronic: Insidious onsent of thrombocytopenia. Fluctuating bleeding, purpura, epistaxis. Adults 20-40 yoa
Treatment of Idiopathic thrombocytopenic purpura
Platelet count
>30.000 and no bleeding: no treatment
<30.000 or severe bleeding: steroid or inmunoglobulin
If treatment fails consider:
splenectomy +/- rituximab +/- thrombopoietin
DO NOT GIVE PLATELETS
Management of secondary amenorrhea
pg 361 firs aid
Neuroleptic malignant sd.
side effect of typical antypsychotics
fever, muscle rigidity, autonomic instability, elevated CK and WBC, delirium
Antipsychotics and Lewi body dementia
High sesnitivity to medication side effects
Worsen parkinsonism, confusion and autonomic instability (eg. orthostatic hypotension)
Antipsychotics side effects
Extrapyramidal: dystonia, akathisia, dyskinesia, tardive dyskenesia, Neuroleptic malignant syndrome
Anticholinergic: sedetaion
Anti alpha1: orthostatic hypotension
Endocrine: prolactinemia, galactorrhea, oligomenorrhea
Muscarinic block: dry mouth, constipation
Cardio: QT prolongation
Bupropion MOA, SE, name others in same class
Serotonine/dopamine reuptake inhibitor
Lower seizure threshold. Does not cause weight gain. Contraindicated in patients with eating and seizure disorders
Trazadone, (highly sedating, priapism)
Mirtazapine (sedation, weight gain)
PTSD presentation and treatment
Intrusive: remembering, nightmares, flashbacks
Avoidance of selected stimuli
Changes in cognition or mood (lack of interest, detachment, negative mood
Sleep disorders, irritability, decreased sleep
Persistently increase in arousal
