investigations for childhood bleeding disorder
DESCRIPTION
this is jst a little effort to find a easy strategy for diagnosis of childhood bleeding disorderTRANSCRIPT
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Approach to the patient presenting with symptoms of bleeding disorder
Contents of History , Physical examination and lab investigation
Step by step investigation procedure
Discussion topics
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Evaluation of the Patient
* History* Physical Examination* Laboratory Evaluation
* Genetic screening test
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For whom the History is Important?
1) Asymptomatic pt. who will undergo a
surgical/invasive procedure
2) Individuals presenting with a personal and/or family H/O of bleeding disorder,
abnormal laboratory tests or concern about bleeding symptoms
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History
Site of Bleeding
- Purpura, epistaxis- Bleeding into muscle and joint
- Recurrent bleeds at single site
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History
Are you a bleeder? – surgical challenges– accidents & injuries–dental extractions– Easy bruising
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History
Does it sound genetic?
• Duration of bleeding history• family history–examine pedigree–determine inheritance
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History
- Liver disease - Renal disease - Malignancies - Drug therapy - Poor nutrition & pre-maturity
(Vitamin K or C)
Medical History
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• If the answers are negative,• no evaluation required.
• But if the answers are positive,• proceed with • physical examination and laboratory tests
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Physical examination
Ecchymoses, hematomas, petechiae etc.
Liver disease (jaundice), Splenomegaly,
Signs of anemia Joint & skin laxity (Ehlers-Danlos syndrome),
Telangiectasia (hereditary hemorrhagic telangiectasia),
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Laboratory Assessment
• Guided by history• Screening tests - Full blood count
- Blood film examination– Platelet count (150-400 x 109/L)
– Bleeding time (< 8 min)
– aPTT (26-36 sec)
– PT (09-12 sec)
– Fibrinogen conc. (1.5-4 g/L)
– Thrombin time (11-15 sec)
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Specific Laboratory Tests
• Mixing studies– Normal plasma & patient's plasma mixed by 1:1– incubated 2 hours at 37o C– perform clotting assay as usual
If, Corrected – Factor deficiency , But if, Uncorrected – Circulating anticoagulant present.
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2nd part …
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The full blood countRBC CountHb PCVErythrocyte Indices (MCV, MCH, MCHC)TC , DC WBC
Peripheral blood film
Platelet count
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Bleeding time
BT , Platelet count , Thrombocytopenia Quantitative disorder of Platelet
possibly,
Idiopathic thrombocytopenic purpura
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Bleeding time
if, BT , platelet count normal,
Thrombasthenia
Qualitative disorder of Platelet
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Quick Review
aPTT PT
T T
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aPTT
aPTT --- PT, TT, Platelet Count- all normal
* Factor deficiency (factor VIII,IX,XI)* vWD* Inhibitors* Heparin therapy
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aPTT
Prolong aPTT, Haemophilia (A or B) von Willebrand disease (vWD)
To differentiate between these 2, we can also do BT. As, Haemophilia : BT normal
vWD : BT
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Confirmation of vWD
H/O mucocutaneous bleedingQuantitative assay for vWF antigenDetermination of vWF structureTesting for vWF activity (ristocetin
cofactor)
Differentiating Haemophilia A or BFactor assay: Factor VIII & factor IX
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PT- aPTT, TT, PC – normal
* Factor VII deficiency early liver disease, early vitamin K deficiency * Oral anticoagulant therapy warfarin therapy
PT
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Both aPTT & PT
aPTT, PT – both Platelet count – normal
Vit-K deficiencfyLiver diseaseWarfarinHeparin
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aPTT, PT, TT all
aPTT, PT, TT all PBF : Red cell fragments platelet count
** DIC
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Only TT
aPTT, PT – normal TT –
Heparin therapy excessDysfibrinogenemia Afibrinogenemia
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• When coagulation screening tests are normal but there is bleeding , suggests
Abnormality of, Vasculature and Integument
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Genetic Screening Test
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Take Home Message The key to diagnosis is the history, physical examination combined with laboratory investigation & genetic screening test. A doctor may order PT, aPTT, full blood count to see whether or not the patient is anemic, how many platelets he has, and to evaluate which pathways may be involved.
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Evaluation of the patient
Thank You For Your Patience Attention