BLEEDING DISORDERS IN CHILDREN

Question 1

NORMAL PHYSIOLOGY OF HAEMOSTASIS

Causes of bleeding disorders
Disorder of __________;_________ and ___________
 Abnormalities of ___________

Answer 1

coagulation

Inherited & Acquired

platelets

Question 2

PLATELETS

Usually ______um in size

Average count is _______-________/mm3

One-third of platelets are in the _______ while 2/3rd are in the _________

Average life span is __________

Answer 2

1-4

150000-400000

Spleen; blood stream

7-10

Question 3

INVESTIGATIONS

List 6
Antinuclear antibodies Bone marrow studies

Answer 3

Full blood count
Blood group
Coomb’s test
Bleeding time
Coagulation tests : PT, APTT, Fibrinogen level
Liver function test

Question 4

Difference between coagulation disorder and platelet disorder

Petechiae
Deep dissecting Haemorrhage
Superficial ecchymoses

Answer 4

Rare ; Characteristic

Characteristic; rare

Common, large and solitary ; small and multiple

Question 5

Difference between coagulation disorder and platelet disorder

Haemathrosis
Delayed bleeding
Sex of patient
Positive family history

Answer 5

Characteristic; rare

Common; rare

Male; Female

Common; rare

Question 6

IMMUNE THROMBOCYTOPENIC PURPURA

Clinical syndrome characterized by

•___________
•Shortened ____________
•Presence of ______________ in plasma
•Presence of _____________ in the bone marrow

Answer 6

Thrombocytopenia

platelet survival

antiplatelet antibody

megakaryocytes

Question 7

ACUTE VS CHRONIC ITP

Age
Sex
Seasonal distribution
Autoimmune conditions

Answer 7

2-6 years; adults commonly

Equal; females

April; none

Uncommon; common

Question 8

ACUTE VS CHRONIC ITP

Platelet count

Eosinophilia /lymphocytosis

Duration

Answer 8

<20,000/mm3; 40,000-80,000/mm3

Common; rare

2-6 weeks ; Months to Years

Question 9

WHAT TO READ?
Predisposing factors
Clinical features
Infantile ITP
What are the laboratory findings?

Answer 9

Ise oyaaaaa

Question 10

HEMOPHILIA A (CLASSICAL HEMOPHILIA)
• Factor ______ deficiency
• ____-linked
• 1:30,000 births
• (Positive or Negative?) family history in 1/3rd of cases
•Female offspring of affected males are ‘________________’
•Classified based on _________________

Answer 10

VIII; X-

Negative

obligate carriers
baseline factor VIII levels

Question 11

Hemophilia A

• Bleeding occurs in the _________ period
•________________ bleeding
•____________ occur with ambulation
•Hallmark is __________ into __________
•Spontaneous __________ can occur

Answer 11

neonatal

Post circumcision ; Hematomas

hemarthrosis; large joints

hematuria

Question 12

Von Willebrand Disease
Autosomal inherited congenital bleeding disorder due to;
Type 1: —————
Type 2: —————
Type 3: —————

Answer 12

Deficiency
Dysfunction
Complete absence of
VON WILLEBRAND FACTOR

Question 13

vWF is synthesized by ____________ and ______________ and stored in _________ and ____________

Answer 13

megakaryocytes & endothelial cells

platelets & endothelial cells

Question 14

Haemophilia A

Spontaneous Haemorrhage into the brain and neck can occur and is usually life threatening

<1% : severe (___________)

1-5%: moderate(____________a)

5-30%: mild (_____________)

Answer 14

Jointbleeds

Minor trauma

Major trauma

Question 15

Haemophilia A

Spontaneous Haemorrhage into the brain and neck can occur and is usually life threatening

<1% : severe (___________)

1-5%: moderate(____________a)

5-30%: mild (_____________)

Answer 15

Jointbleeds

Minor trauma

Major trauma

Question 16

Haemophilia A
_______ APTT

Answer 16

PROLONGED

Question 17

HAEMOPHILIA A

target factor 8 level = ???

Answer 17

Baseline factor 8 X body weight/2

Question 18

Mild haemophilia may respond to ________,________, or __________

Answer 18

DDAVP, FFP, Cryoprecipitate

Question 19

VwF VS haemophilia A

Symptoms
Sexual distribution
Abnormal protein
History

Answer 19

Bruising, bleeding, epistaxis ; Muscle, joint bleed

Males = Females ; Males

vWF ; F VIII

Abnormal ; Abnormal

Question 20

VwF VS haemophilia A

Bleeding time
PTT
Factor VIII

Answer 20

Often abnormal ; Usually normal
Normal or prolonged; Prolonged
Borderline or decreased ; Decreased or absent

Question 21

HEMORRHAGIC DISEASE OF THE NEWBORN

Vitamin K is required for __________ of vitamin K dependent coagulation factors (___,___,____,____)

Answer 21

Carboxylation

2,7,9,10

Question 22

Neonates are at risks of HDN due to :
•Lack of _________
•Reduced capacity of the ______
•Prematurity
•__________

Answer 22

Gut Flora; liver

Breastfeeding

Question 23

Early HDN
 Occurs within ________
 Presents with ________/________
 Usually associated with ________( warfarin, antiTB )

Answer 23

24hrs

ICH/Hematoma

maternal drugs

Question 24

Classical HDN
 Occurs within ________
 Occurs in ________
 ________ bleedS/ GI bleeds

Answer 24

1-7 days
exclusively breastfed ; Umbilical cord

Question 25

Late HDN
 Occurs at ________
 Can present with ________
 Often associated with ________ disease
Investigation
 ________ PT/APTT  ________ platelet

Answer 25

3 – 8 weeks; ICH

cholestatic liver disease

Isolated High ; Normal

Question 26

Treatment of HDN
• IV/SC ________ 1mg (corrects
PT/APTT within ________)
• If life threatening haemorrhage, give ________ 10-15mls/kg

Answer 26

Vitamin K

4-6 hrs

FFP

Question 27

Prevention OF HDN
???????????????

Answer 27

🌚 so 🌚