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15. Diseases Affecting Neonate & Children > 15.8.3 Bleeding Disorders In Chidren > Flashcards

15.8.3 Bleeding Disorders In Chidren Flashcards

1
Q

Define haemostasis

A
  • cessation/stop bleeding
  • Process of keeping blood within the vessels
  • by repairing damaged vessels,
  • without compromising the flow of the blood
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2
Q

Major components of hemostasis

A

In video
Vascular injury
⬇️ collagen + tissue factor
Vasoconstriction
Platelet activation
Coagulation Cascade
Antithrombotic control mechanism

Slide 4

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3
Q

Defective hemostasis bleeding

A
  • no vessel constriction
  • no platelet plug
  • no fibrin clot
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4
Q

Coagulation cascade

A

Slide 7

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5
Q

Where can the problem be?

A

Blood vessels
- not intact
- inflam
- not normal
- eg Vaculitis (Henoch Schönlein purpura)

Platelets
- no platelet plug = bleeding
- 1. Decreased number (immune thrombocytopenic purpura -> not idiopathic but immune mediated)
- 2. Abnormal function (Glanzmann Thrombasthenia)

Clotting factors
- no fibrin clot = bleeding
- 1. Deficient (Haemophila A & B)

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6
Q

Blood vessels

A

Vasculitis
➢Inflammation of blood vessels
- e.g. Henoch Schönlein Purpura
- IgA mediated vasculitis of small vessels
- Triad = purpura/arthritis/abdominal pain
- Palpable purpura
- Typically lower limbs & buttocks
- Occasionally GIT bleed
- IgA nephropathy: proteinuria

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7
Q

Platelets

A
  • Superficial bleeding
  • Mucocutaneous bleeding pattern:
    ➡️Epistaxis, gingival bleeding, haematuria,
    ➡️Petechiae, purpura, ecchymoses
  • May stop with pressure
  • never has haemarthroses
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8
Q

Clotting factors

A
  • Most often after trauma
    • Can be spontaneous as well (severely decreased levels)
  • Deep bleeding pattern:
    • Haematomas
    ➡️Bleed into deep tissues: muscles
    • Haemarthroses (classic)
    ➡️Bleed in joint
  • Can also have epistaxis, haematuria, ecchymoses, gingival bleeding
  • Pressure usually insufficient to stop bleeding

Coagulation disorders
Slide 13

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9
Q

History: ask right questions

A
  • Recent infections eg Viral infections (*ITP)
  • Severity
  • Frequency
  • Triggers egtrauma
  • Ask about the “potential bleeding points” in a child’s life:
    • Haematomas/bleeding at birth (especially vit K)
    • Bleeding from umbilical stump
    • Haematoma after immunisations (rare)
    • Prolonged bleeding with teething
  • Any bleeding with previous surgery/procedures
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10
Q

History: age

A
  • Age at start of symptoms
  • Haemorrhage in neonatal period – suspect congenital cause strongly In older patient congenital or acquired

Infancy
- CONGENITAL
- Neonatal intracranial haemorrhage due to Vitamin K deficiency
- Post circumcision
- Umbilical stump bleeding : Factor XIII deficiency, Fibrinogen deficiency
- Wiskott Aldrich syndrome: X-linked recessive immunodeficiency disorder: thrombocytopenia, eczema, recurrent infections
- Start bearing weight→inherited coagulation disorders eg Haemophilia

Older child
- CONGENITAL
- Von Willebrand Disease= Most common congenital bleeding disorder!
-Inherited coagulation disorders eg Haemophilia A (A more common than B), Haemophilia B
- Platelet dysfunction (rare) eg Glanzmann Thrombocytopenia, Bernard Soulier Syndrome

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11
Q

History: gender

A

Boys
- X-linked recessive
- eg Haemophilia A
- Haemophilia B
- Wiskott Aldrich Syndrome

Boys and girls
- Autosomal recessive/dominant: Von Willebrand Disease (VWD)
- Autosomal recessive: Haemophilia C
Acquired:
- Immune Thombocytopenic Purpura (ITP)
- Disseminated Intravascular Coagulopathy (DIC)

Ethnicity
- Jewish population more common : Haemophilia C
- Clotting factor XI deficiency
- Autosomal recessive
- Boys and girls

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12
Q

History: family history

A
  • Males with bleeding/ haemarthroses/ death
  • Mother/female relatives:
    ➡️postpartum bleeding, need for blood transfusion, iron supplementation, menorrhagia
    ➡️Know how to identify menorrhagia
  • Inheritance patterns
  • Spontaneous mutations
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13
Q

Screening tool for ID women with menorrhagia for testing and evaluation for underlying bleeding disorders

A
  • duration of menses was greater than or equal to 7 days
  • flooding or bleeding through a tampon or napkin in 2 hrs or less with most periods

Slide 19

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14
Q

What bleeding pattern is present?

A

Mucocutaneous (superficial)
- = Platelets/ Von Willebrand types 1 & 2
- Petechiae
- Purpura
- Ecchymoses
- Mouth/nose bleeds
- Haematuria
- Rarely intracranial

Clotting factor deficiency (deep)
- = Clotting factor deficiencies / Von Willebrand type 3
- Haematomas (Soft tissue/muscle)
- Haemarthroses
- Ecchymoses
- Mouth/nose bleeds
- Haematuria
- Intracranial
- GIT

Slides 21 +22

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15
Q

Other history taking

A

Medication that may cause bleeding
- Aspirin
- Warfarin
- Non Steroid Anti inflammatory Medication eg Ibuprofen, diclofenac

Diet
- Vitamin K (intestinal flora, green leavy food) deficiency
- Vitamin C deficiency (scurvy) Perifollicular haemorrhage

Medic Alert

Underlying disease
- eg acute or chronic liver disease

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16
Q

Clinical examination

A

Observations
- Fever, tachycardia, tachypnea, hypotension

Anthropometry
- Fanconi anaemia – 75% short stature

Pallor
- Blood loss
- Part of bone marrow failure
- Haemolysis

Lymphadenopathy
Dysmorphic features

Skin:
- petechiae, ecchymoses, vasculitis, café au lait spots, blueberry muffin nodules, other masses/nodules

Abdomen
- Hepatosplenomegaly
- Abdominal mass (neuroblastoma, rhabdomyosarcoma, lymphoma)

CVS
- If pallor present, is the child symptomatic of the anaemia
- Most sensitive sign = resting tachycardia in the absence of fever, NOT a murmur!

Resp
- Respiratory distress
- Signs of superior vena cava syndrome
- Pleural effusion
- Consolidation/mass

CNS
- Level of consciousness
- Meningism
- Focal signs

17
Q

Child with bleeding clinical examination

A

Bleeding symptoms and signs:
- Mucocutaneous bleeding: petechiae, purpura, ecchimoses
- Soft tissue bleeds: Haematoma
- Joint bleeds: Haemarthrosis

  • Other symptoms and signs:
  • Fatigue
  • Weight Loss
  • Pallor
  • Lymphadenopathy
  • Hepatosplenomegaly
18
Q

Congenital bleeding disorder
Common ways of presentation

A

Known family history
- But family members do not always disclose
- Usually autosomal recessive; X-linked but may also be autosomal dominant (VWD)
- NO family history does not exclude congenital cause (spontaneous mutation)

Neonatal period:
- Intracranial or other sites of bleeding at birth
- Haematoma after IM (intramuscular) vitamin K
- Bleeding from umbilical stump
- Excessive bleeding after circumcision in 1st week of life

Haematoma after vaccinations (uncommon)
Easy bruising (parents sometimes accused of abuse)
Poor wound healing (uncommon)

Bleeding episodes
- Epistaxis
- Gingival bleeding
- Haematuria
- Haematomas
- Haemarthroses

Excessive bleeding after trauma or procedures
- Tear of tongue frenulum leads to ++bleeding (eg when drinking bottle)
- Dental or other procedures

19
Q

Von Willebrand disease

A
  • Most common inherited bleeding disorder
  • Mucosal bleeds, Epistaxis
  • Boys and girls
  • Older child, adolescent ,post surgery/procedure bleeding
  • *Type 3 may behave like Haemophilia
20
Q

Haemophilia A & B

A
  • Inherited, X-linked recessive
  • Spontaneous mutations
  • Males (females extremely rare)
  • Haemophilia A = Clotting factor VIII deficiency (more common)
  • Haemophilia B= Clotting factor IX deficiency
    (Christmas disease)
  • Haemarthrosis = bleeding in joint
  • Haematomas=bleeding in soft tissue
  • Can also have mucosal bleeding eg Haematuria,epistaxis
  • Inhibitors (measured in Bethesda units)
    ➡️Inhibitors are neutralising antibodies against the deficient clotting factor that limit the effectiveness of Factor infusions
21
Q

Common ways of presentation of an acquired bleeding disorder

A

DIC/ Malignancy/ Other
- Acutely ill/unwell child
- Infection
- Petechiae/purpura
- Ecchymoses
- Bleeding from venipuncture sites
- Bleeding from mouth/nose/GIT/other sites
- Ischaemia (peripheral)
- Lymphadenopathy
- Hepatosplenomegaly
- Other

Immune Thrombocytopenic Purpura
- Well child
- Petechiae and purpura
- Ecchymoses
- Bleeding from mouth/nose
- Less commonly other sites
- Often following recent (2-3 weeks prior to rash) viral infection or (rarely) vaccination
- NO lymphadenopathy
- NO hepatosplenomegaly

22
Q

Immune thrombocytopenic purpura

A
  • Most common thrombocytopenia in children
  • Age:2-5years
  • Boys and girls
  • Well child
  • Post viral infection (2-3 weeks prior to rash)
  • Petechiae, purpura, ecchymoses
  • Epistaxis,oralbleeding
  • Severe bleeding eg intracranial bleeding uncommon (<1%)
  • NO lymphadenopathy
  • NO hepatosplenomegaly
23
Q

Important

A
  • Patients with clotting factor deficiencies can have haematuria, epistaxis and mouth bleeds, but NOT petechiae/purpura
  • Patients with platelet disorders DO NOT get muscle/joint bleeds
  • Patients with VWD can have superficial and/or deep bleeds depending on subtype
24
Q

Disseminated intravascular coagulopathy (DIC)

A
  • Acquired Bleeding disorder
  • Complication of Underlying conditions eg
    • Septicemia
    • Viraemia
    • Shock
    • Hypoxia
    • Burns
    • Malignancy (Acute Promyelocytic Leukaemia)
    • Post operative

Pathogenesis Slide 39

25
Q

Vit K deficiency

A

Acquired bleeding disorder
Vitamin K
- Required by liver for activation of Factors II, VII,
IX, X
9 (+1) = 10)
( To remember: 2 + • Natural K vitamins synthesised by Intestinal flora
7 =
• Fat soluble, require bile for absorption, not stored in body
Vit K deficiency
Vitamin K deficiency
• liver synthesises abnormal proteins nl PIVKAs, which are inactive and may cause inhibition of coagulation Neonate at risk.
Other causes: obstructive jaundice, malabsorption syndromes, broadntibiotics long tem use,oral anticoagulants , diet deficient aan vitK
Haemorrhagic disease of newborn (HDN)
Early HDN: Bleeding hours to 1 week after birth : due to Maternal medication eg Anti-convulsant Classic HDN : Bleeding 1-3 weeks : due to missed Vit K at birth
LateHDN :Bleeding1-3monthsoflife (higherriskofintracranialbleeding)
Clinical: melaena, haematemesis, umbilical cord bleeding

26
Q

Liver disease

A

Slide 43

15. Diseases Affecting Neonate & Children (54 decks)

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