APPROACH TO A

BLEEDING CHILD
SAMSON NADEW(MD)

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Bleeding…
 OBJECTIVES

-DESCRIBE THE NORMAL HEMOSTATIS IN CHILDREN

-EVALUATE CLINICALLY A BLEEDING DISORDER IN

CHILDREN

-INVESTIGATE A BLEEDING DISORDER USING

LABORATORY TOOLS

- MANAGE A POSSIBLE BLEEDING DISORDER

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BLEEDING D/O IN CHILDREN…
 Introduction
Normal hemostasis
Hemostasis is the active process that clots blood in
areas of blood vessel injury, yet simultaneously
limits the clot size only to the areas of injury.
-Over time, the clot is lysed by the fibrinolytic
system, and normal blood flow is restored.
If clotting is impaired, hemorrhage occurs. If clotting
is excessive, thrombotic complications ensue.
-The hemostatic response needs to be rapid and
regulated and localized.
-The main components of the hemostatic process
are the vessel wall, platelets, coagulation proteins,
anticoagulant proteins, and fibrinolytic system.
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Bleeding…
 The classic hemostasis has 5 stages:

1. Vascular response-vessel spasm

2. Formation of platelet plug-Platelet adhesion and
aggregation
3. Formation of fibrin plug- Clot stabilization
4. Limitation of clot to the site of injury
5. Reestablishing vascular patency( fibrinolysis and
vascular healing

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Bleeding…
1. Vascular response-vessel spasm (in seconds).
- Local and humoral mechanism-TXA2 released from

platelets
- Constrics the vessel and reduce blood flow

2. Formation of platelet plug

- As platelet comes in contact with vessel wall-

adhere and aggregate

- Adhesion requires VWF and platelet receptor sites

- Bridges of fibrinogen attach platelets to each other

Aggreg. Agents- ADP,TXA2,thrombin

-thrombin converts fibrinogen to fibrin which
consolidates the plug

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Bleeding…
3. Formation of fibrin plug
- occurs as a result of IPW( slower-occurs in vascular
system) and EPW(faster-in tissues)
- Terminal steps are the same i.e. interaction of

thrombin and fibrinogen to form fibrin

- Both pathways are needed for normal hemostasis

but bleeding due to defect of EPW is less severe.

-coag process involves about 30 substances-pro and
anticoagulants
- Ca⁺⁺ (factor V) is needed in all steps except the 1st
two

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Bleeding…
4. Limitation of clot to the site of injury
- Clot retraction requires large no of platelets – pulls

the broken edges of vessels

5. Reestablishing vascular patency( fibrinolysis and

vascular healing- allows blood to flow
Plasminogen is a proenzyme for fibrinolysis is
activated to plasmin
- Plasmin digests the fibrin strands

- inactivated by plasmin inhibitor

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Bleeding…
 PATHOLOGY.
 Congenital deficiency of an individual procoagulant protein
leads to a bleeding disorder, whereas deficiency of an
anticoagulant (clotting factor inhibitor) predisposes the
patient to excessive thrombosis.
 A primary illness (sepsis) and its secondary effects (shock
and acidosis) activate coagulation and fibrinolysis and impair
the host's ability to restore normal hemostatic function.
 When sepsis triggers DIC clotting factors and anticoagulant
proteins are consumed, leaving the hemostatic system prone
to bleeding or clotting.
 newborn infants and patients with severe liver disease have
synthetic deficiencies of both procoagulant and
anticoagulant proteins. Such dysregulation causes the
patient to be predisposed to both hemorrhage and
thrombosis with mild or moderate triggers .

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Bleeding…
Clinical and Laboratory Evaluation of Hemostasis
HISTORY:
- determine the site or sites of bleeding, the severity

and duration of hemorrhage, and the age at onset.

- Was the bleeding spontaneous, or did it occur after

trauma?
- a previous personal or family history of similar

problems?
- Did the symptoms correlate with the degree of injury

or trauma? Does bruising occur spontaneously?

- Are there lumps with bruises with minimal trauma?

- If the patient had previous surgery or significant

dental procedures, was there any increased

bleeding?
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Bleeding…
 After surgery of the mucosal surfaces, s.a.
tonsillectomy or dental extractions, the absence of
bleeding usually rules out a hereditary bleeding
disorder.
 Delayed or slow healing of superficial injuries may
suggest a hereditary bleeding disorder.
 careful menstrual history- common bleeding
disorders, such as von Willebrand disease (VWD), have
a fairly high prevalence.
 Women with mild VWD who have a moderate history
of bruising frequently have a reduction of symptoms
during pregnancy or after administration of OCP.
 aspirin and other NSAIDs, may inhibit platelet
function and increase bleeding symptoms in patients
with a low platelet count or abnormal hemostasis.
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Bleeding…
 Outside the neonatal period, thrombotic disorders
are relatively rare until adulthood.
 In the neonate, physiologic deficiencies of
procoagulants and anticoagulants cause the
hemostatic mechanism to be dysregulated, and
clinical events can lead to either hemorrhage or
thrombosis.
 If a child or teenager presents with DVT or
pulmonary emboli, a detailed family history must
be obtained to evaluate for DVT, pulmonary
emboli, MI, or stroke in other family members.
 Even with no family hx- consider hereditary dx

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Bleeding…
 PHYSICAL EXAMINATION.
- mucous membranes or skin (mucocutaneous bleeding)
or the muscles and joints (deep bleeding)?
- Check for the presence of petechiae, ecchymoses,
hematomas, hemarthroses, or mucous membrane
bleeding.
 Patients with defects in platelet–blood vessel wall

interaction (VWD or platelet function defects) usually

have mucocutaneous bleeding-epistaxis, menorrhagia,
petechiae, ecchymoses, occasional hematomas, and less
commonly, hematuria and gastrointestinal bleeding.
 Individuals with a clotting factor deficiency, such as

hemophilia (factor VIII or factor IX deficiency), have

symptoms of deep bleeding into muscles and joints,
with extensive ecchymoses and hematoma formation.

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Bleeding…
 Patients with mild VWD or other mild bleeding
disorders may have no abnormal findings on physical
examination.
 Individuals with disorders of the collagen matrix and
vessel wall may have loose joints and lax skin
associated with easy bruising (Ehlers-Danlos
syndrome).

 for thrombotic disorders-asked about swollen, warm,

tender extremities or internal organs (venous
thrombosis), unexplained dyspnea or persistent
“pneumonia,” especially in the absence of fever
(pulmonary emboli).
 Arterial thrombi usually cause an acute illness such as
stroke, MI, or a painful, white, cold extremity.
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Bleeding…
 LABORATORY TESTS.
 do a platelet count, PT, and partial thromboplastin time (PTT).

 If the results are normal, a thrombin time to evaluate fibrinogen

function and VWF testing should be considered.

 In individuals with abnormal screening test results, further

specific factor work-up should be undertaken.

Bleeding Time.
 Bleeding time assesses the function of platelets and their

interaction with the vascular wall.

 A blood pressure cuff is applied to the upper arm and inflated to

40 mm Hg for children and adults, lower in yound children. After

an incision is made –blot from the margin of the incision at 30
sec intervals until bleeding ceases.
 bleeding usually stops within 4–8 min.

 has interlaboratory & interobservant variations.

 If abnormal with platelet count of >100,000/mm3 consider

platelet dysfunction

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Bleeding…
 Platelet Count.(N= 150,000-450000/microlitre)
Patients with a platelet count of >50,000/mm3rarely have
significant clinical bleeding.
Thrombocytosis in children is usually reactive-not
associated with bleeding or thrombotic complications.
PROTHROMBIN TIME
PT measures the activation of clotting by tissue factor
(thromboplastin) in the presence of calcium. An isolated
abnormal prothrombin time (PT) suggests F VII
deficiency.
normal -10-13sec
PT has been standardized using the International
Normalized Ratio (INR) so that values can be compared
from 1 laboratory or instrument to another.

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Bleeding…
 Partial Thromboplastin Time.
 Evaluates the intrinsic pathway -involves the initial

activation of factor XII, which is activated by

prekallikrein and high molecular weight kininogen.
In the clinical laboratory, factor XII is activated using
a surface (silica or glass) or a contact activator, such
as ellagic acid.
-It does not measure factor VII, factor XIII, or
anticoagulants.
a prolonged activated partial thromboplastin time
(aPTT) indicates most commonly hemophilia or FXI
deficiency.
The prolongation of both PT and aPTT suggests
deficiency of FV, FX, FII, or fibrinogen abnormalities.

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Bleeding…
 Thrombin Time.

 Thrombin time measures the final step in the clotting

cascade, in which fibrinogen is converted to fibrin. The

normal thrombin time varies between laboratories, but is
usually 11–15 sec.
 Prolongation of thrombin time occurs with reduced

fibrinogen levels, with dysfunctional fibrinogen, or with the

use of substances that interfere with fibrin polymerization,
such as heparin or fibrin split products.
 If heparin contamination is a potential cause of prolonged

thrombin time, a reptilase time is usually ordered.

Reptilase Time.
 Reptilase time uses snake venom to clot fibrinogen.

reptilase time is not sensitive to heparin and is prolonged

only by reduced or dysfunctional fibrinogen and fibrin split
products.

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Bleeding…
 Mixing Studies.
 If there is unexplained prolongation of PT, PTT, or
thrombin time, a mixing study is usually performed.
 Normal plasma is added to the patient's plasma, and
PT or PTT is repeated. Correction of PT or PTT by 1:1
mixing with normal plasma suggests deficiency of a
clotting factor, because a 50% level of individual
clotting proteins is sufficient to produce normal PT or
PTT. If the clotting time is not corrected or only
partially corrected, an inhibitor is usually present.
 An inhibitor of clotting is-like heparin or an antibody
directed against a specific clotting factor or the
phospholipid used in clotting tests.

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Bleeding…
 Differential Diagnosis
 Disorders of Platelets
 Thrombocytopenia.-Platelet counts less than
150,000/mm3 .
 Mucocutaneous bleeding is the hallmark of platelet
disorders.
 The risk of bleeding correlates imperfectly with the
platelet count.
 Children with platelet counts less than 20,000/mm3 are
at risk for spontaneous bleeding.
 factors such as the age of the platelets (young, large
platelets) and the presence of inhibitors of platelet
function, such as antibodies, drugs (especially aspirin),
FDP, and toxins formed in the presence of hepatic or
renal disease.

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Bleeding…
The etiology of thrombocytopenia may be
organized into disorders of (1) decreased platelet
production, (2) increased destruction, and (3)
sequestration.
Thrombocytopenia Resulting from Decreased Platelet
Production.

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Bleeding…
 Disorders of Clotting Factors .
 The genes for factor VIII and factor IX are on the X chromosome,

whereas virtually all the other clotting factors are coded on autosomal
chromosomes.
 Factor VIII and factor IX deficiencies are the most common severe

inherited bleeding disorders.

 von Willebrand disease is the most common congenital bleeding

disorder.
 Hemophilia

Hemophilia A (factor VIII deficiency) occurs in 1 in 5000 males.

Hemophilia B (factor IX deficiency) occurs in approximately 1 in
25,000.In mild hemophilia (>5% factor VIII or factor IX), significant
trauma is necessary to induce bleeding; spontaneous bleeding does
not occur.
Mild hemophilia may go undiagnosed for many years, whereas severe
hemophilia manifests in infancy when the child reaches the toddler
stage.
In severe hemophilia, spontaneous bleeding occurs, usually in the
muscles or joints (hemarthroses). .

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Bleeding…
 von Willebrand Disease.
 von Willebrand disease is a common disorder
(found in 1% of the population) caused by a
deficiency of vWF.
 von Willebrand disease usually is inherited as an
AD trait and rarely as an autosomal recessive trait.
 Approximately 80% of patients with von Willebrand
disease have classic (type 1) disease (i.e., a mild to
moderate deficiency of vWF).
 Mucocutaneous bleeding, epistaxis, gingival
bleeding, cutaneous bruising, and menorrhagia
occur in patients with von Willebrand disease

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Bleeding…
 Disseminated Intravascular Coagulation.
DIC is a disorder in which a severely ill patient sustains
widespread activation of the coagulation mechanism,
usually associated with shock.
Bleeding and clotting manifestations may be present.
-decline in platelets and fibrinogen associated with elevated
PT, PTT, and high levels of FDP.
- In a severely ill patient, the sudden occurrence of bleeding

from a venipuncture or incision site, gastrointestinal or

pulmonary hemorrhage, petechiae, or ecchymosis or
evidence of peripheral gangrene or thrombosis suggests
the diagnosis of DIC.
- treat the disorder inducing the DIC first;

- correct hypoxia, acidosis, and poor perfusion; and replace

depleted blood clotting factors, platelets, and anticoagulant

proteins by transfusion.

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