2. Introduction
•
Neonates are susceptible to bleeding for
various reasons
Immaturity of the haemostatic system
because of quantitative and qualitative
deficiency of coagulation factors
Maternal disease and drugs
Birth trauma
Other conditions - sepsis and asphyxia
3. Clinical presentation
•
Bleeding in neonates may present with
Oozing from the umbilical stump
Cephalhaematoma
Bruising , Petechiae
Bleeding from peripheral venipuncture or
procedure sites
Bleeding following circumcision
Intracranial haemorrhage
Bleeding from mucous membranes
Unexplained anemia and hypotension
4. Etiology
A.Deficiency of clotting factors:
1.Transitory deficienciesDeficiency of vitamin K dependent
C.F- II, VII, IX, X.
Deficiency of anticoagulant proteins
C & S.
5. Causes:
a. Total parenteral nutrition or antibiotics
b. Lack of administration of vitamin K .
c. Drug intake in pregnancy
eg.i. Phenytoin, Phenobarbital, Salicylates .
(Interferes with the synthesis of vit. K
dependent c.f. )
ii. Calmodulin compounds
6. • The incidence among babies born to mothers
on these drugs have varied between 6-12%*.
In a recent series on children born to mothers
on anticonvulsants, abnormal PT was
documented in 14 out of 105 babies (13%)
, no overt bleeding was observed*.
7. 2. Disturbances of clotting
- Related to DIC due to
infection, shock, anoxia, NEC, renal vein
thrombosis, use of IV canula.
3. Inherited abnormalities of C.F.
a. X-Linked recessive diseasesi. Hemophilia-A : Factor VIII deficiency.
ii. Hemophilia-B : Factor IX deficiency.
8. b. Autosomal dominant diseases:
i. Von Willebrand disease – Deficiency of
VWF which is a carrier of factor VIII & as a
platelet aggregation agent.
c. Autosomal recessive diseases:
i. Severe factor VII & factor XIII deficiency –
intracranial hemorrhage in neonates
ii. Factor XI deficiency –
unpredictable bleeding during
surgery/trauma.
9. iii. VWD Type III
B. Platelet problems:
1. Qualitative disorders:
- Glanzman’s thrombasthenia.
- Bernard-Soulier syndrome
- Platelet type VWD
10. 2. Quantitive disorders:
- Immune thrombocytopenia
- Matrnal Preeclampsia, HELLP syndrome
or severe uteroplacental insuffuciency.
- DIC due to infection or asphyxia.
- Inherited marrow failure syndromes :
Fanconi anemia & congenital
amegakaryocytic thrombocytopenia
13. Diagnostic workup
• HISTORY: A detailed history and examination
essential in the assessment of bleeding neonate
History includes
• Maternal diseases as ITP, preeclampsia .
• Maternal exposure to drugs as
aspirin, anticonvulsants, rifampicin and isoniazid
• Family history of bleeding disorders
• Previous affected sibling
14. B. Examination:
First diagnose whether the infant is Sick or Well
1. Sick infant:
- DIC
- Bacterial/ viral infections.
2. Well infant:
- Vit K deficiency
- Isolated C.F. deficiencies
- Immune thrombocytopenia
- Maternal blood in infant’s GIT.
15. 3. Patchiae, ecchymosis, mucosal
bleeding: Platelet problem
4. Large bruises: DIC, C.F
deficiencies, liver diseases
5. Enlarged spleen : Possible congenital
infections or erythroblastosis.
6. Jaundice : Sepsis, liver
diseases, resorption of large hematoma.
16. C. Laboratory tests:
1. Apt test :
- To rule out maternal blood in infant’s
GIT
- Done in otherwise well infant with
only GI bleeding.
2. PBS :
- DIC- fragmented RBCs
- Congenital macrothrombocytopenias –
large platelets.
17. 3. PT
4. APTT
5. D-Dimer assays: Measure fibrin
degradation products in DIC & Liver
diseases causing defective clearing of
fibrin split products.
6. Specific factor assays & Von
Willebrand assay: For patients with +
ve family h/o.
18. Laboratory findings
Laboratory Studies
Other useful tests
DIC
Platelets PT
Likely Diagnosis
Fibrinogen, FDP, Sepsis
screen
Platelet consumption
(NEC, Renal vein thrombosis,
marrow infiltration, Sepsis)
LFT, Albumin
APTT
SICK INFANTS
N
N
Liver disease
N
N
N
N
Compromised vascular integrity
(hypoxia, prematurity, acidosis)
19. Laboratory Studies
Platelets PT
Likely Diagnosis
Other useful tests
Immune thrombocytopenia
Bone marrow hypoplasia
Maternal platelet count,
Platelet antigen typing,
Bone marrow, Fibrinogen,
FDP, Factor VII & IX assays
APTT
HEALTHY INFANTS
N
N
N
N
N
Vitamin K Deficiency
N
Heriditory C.F. deficiencies
N
Bleeding d/t local factors,
Plt function anomalies,
Factor XIII deficiency(rare)
N
Platelet aggregometry
Urea clot solubility
20. Treatment Of Bleeding
A. Inj Vitamin K1 (Aquaminophyton)
- 1 mg IV or IM if not given at birth.
- Infants on TPN
- Infants on Antibiotics > 2 weeks: at
least 0.5mg Vit K weekly.
- Preferred rather than FFP for prolonged
PT & PTT, FFP should be reserved for
emergencies.
21. B. FFP:
- 10ml/kg IV for active bleeding
- Repeated 8-12 hrly as needed.
- Replaces C.F. immediately.
C. Platelets:
- 1 Unit of platelet raises count by
50,000-100,000/mm3 in a 3kg
newborn.
- Platelet count slowly decreases if stores
3-5 days.
22. D. Fresh whole blood:
- 10ml/kg
- Can be repeated after 6-8 hrs as needed.
E. Clotting factor concetrates
- Severe VWD :
- VWF containing plasma derived factor VIII
concetrate.
- Known deficiency of factor VIII or IX :
Recombinent DNA derived factor VIII and
IX concetrate
23. F. Disorders due to problems other than hemostatic
proteins :
- Rule out the underlying possibilities
- eg. Infection, Liver rupture, catheter, NEC.
G. T/t of specific disorders :
1. DIC :
- Treat the underlying cause i.e. sepsis, NEC
- Make sure that Vit K1 has been given.
24. - Platelets/ FFP to keep platelet counts > 50,000/ml
and to stop bleeding.
- If bleeding persists,
i. Exchange transfusion with fresh whole blood
/Packed RBC/Platelets/FFP
ii. Continuous transfusion with platelets, packed
RBCs or FFP as needed.
iii. For hypofibrinogenemia : Cryoprecipitate
(10ml/kg)
25. VKDB
•
•
•
•
Early , Classic, and Late forms
Early VKDB – in first day
Severe bleeding – GI and ICH
Cause – Maternal drug intake
Phenytoin, phenobarb,
ATT, warfarin
26. VKDB
Classical form: 2-7 days of age
• 0.25-1.7% of all babies
• Cause – not received prophylaxis
on breast feeds, sterile gut, lack of
placental transfer
Late form : 2-8 weeks of age
• Boys > girls, 5-10/1 lac
• Well , breastfed, term baby
• Liver disease
• Malabsorption
27. Management of VKDB
• Prolonged PT , APTT (if severe)
• Normal platelets and fibrinogen
• Factor assays of vit K dependent
factors
• Treatment – 1mg iv or sc
• FFP in severe cases
28. Prophylaxis of VKDB
• Early VKDB- single IM inj of vit K at
birth and oral Vit K to mother for
last 4 weeks
• Classical and Late forms –
IM Vit K at birth
oral Vit K at 0 , 4 days and 4 weeks
In preterms – Weekly iv Vit K
29. Hemophilia in the Newborn
• Factor VIII or XI deficiency
– A good family history goes a long way
30. Hemophilia A
Most common inherited clotting factor def
X linked recessive, 1 in 4000 males
1/3rd of cases present in newborn period
ICH(25%), cephalhematoma(10-15%)
Post circumcision bleed is characteristic
Family history – absent in 30%
Inv – prolonged APTT, normal PT, normal
platelets.
• Factor VIIIc assay level <2% severe, 2-10%
moderate, >10% mild
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31. Hemophilia B
•
•
•
•
•
•
XLR
Deficiency of Factor IX
Less common than the classical form
Prolonged APTT and low Factor IX
Rx- 100u/k iv OD , to raise levels to 100%
Avoid lumbar punctures, IM injections
32. Thrombocytopenia
•
•
•
•
•
Less than 150,000/uL
Incidence in newborns: 1-5%
Incidence in NICU – 15-30%
In VLBW and preterms – 50%
Causes of thrombocytopenia in newborn:
Neonatal megakaryocytes are smaller
Inadequate production of thrombopoietin
33. Causes of thrombocytopenia
• Immune-mediated
• Associated with infection - Bacterial or Nonbacterial
• Drug-Related
• Increased peripheral consumption of platelets –
Disseminated Intravascular Coagulation,
Necrotizing enterocolitis, hypersplenism
• Genetic and Congenital Anomalies
• Miscellaneous – asphyxia, IUGR, PIH, GDM
36. Immune Thrombocytopenia
• Neonatal allo-immune thrombocytopenia
(NAIT)
• Incidental thrombocytopenia of
pregnancy or Gestational
thrombocytopenia
• Autoimmune thrombocytopenic purpura
37. Neonatal allo-immune
thrombocytopenia (NAIT )
•
•
•
•
•
•
Incompatibility between mother and baby
Similar to Rh disease
Antibodies against HPA – 1 (most common)
In utero bleed can occur
Manifests with first pregnancy in 50%
Postnatal : petechiae, purpura
ICH in 10% with sequelae
38. NAIT
• Management – fetal blood sampling and
platelet transfusion or maternal IVIG
• If previous sibling had a significant bleed
• Caesarian section
• In newborn – maternal platelets or HPA
compatible platelets
• IVIG 1gm/k for 2 days or 0.5g/k for 4 days