2. Etiology
A.Deficiency of clotting factors:
1.Transitory deficiencies-
Deficiency of vitamin K dependent
C.F- II, VII, IX, X.
Deficiency of anticoagulant proteins
C & S.
3. 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 : interfare
with synthesis of vit K
dependeat C.F.
4. • 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*.
5. 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 diseases-
i. Hemophilia-A : Factor VIII deficiency.
ii. Hemophilia-B : Factor IX deficiency.
6. 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.
7. iii. VWD Type III
B. Platelet problems:
1. Qualitative disorders:
- Glanzman’s thrombasthenia.
- Bernard-Soulier syndrome
- Platelet type VWD
8. 2. Quantitive disorders:
- Immune thrombocytipenia
- Matrnal Preeclampsia, HELLP syndrome
or severe uteroplacental insuffuciency.
- DIC due to infection or asphyxia.
- Inherited marrow failure syndromes :
Fanconi anemia & congenital
amegakaryocytic thrombocytopenia
11. Diagnostic workup
A.History
- Family h/o bleeding disorders
- Maternal medications
- Pregnancy & birth history
- Maternal h/o infant with bleeding disorder
- Any medications, procedures, anomalies in
infant
12. 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.
13. 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.
14. 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.
15. 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.
16.
17.
18. Laboratory findings
Laboratory Studies Likely Diagnosis Other useful tests
Platelets PT APTT
SICK INFANTS
DIC, sepsis, hypoxia, acidosis, cold Fibrinogen, FDP, Sepsis
stress screen
Platelet consumption LFT, Albumin
(NEC, Renal vein thrombosis,
N N marrow infiltration, Sepsis)
Liver disease
N
Compromised vascular integrity
N N N (hypoxia, prematurity, acidosis)
19. Laboratory Studies Likely Diagnosis Other useful tests
Platelets PT APTT
HEALTHY INFANTS
Immune thrombocytopenia Maternal platelet count,
N N Bone marrow hypoplasia Platelet antigen typing,
Bone marrow, Fibrinogen,
FDP, Factor VII & IX assays
N Vitamin K Deficiency
N N Heriditory C.F. deficiencies B.T.
Bleeding d/t local factors, Platelet aggregometry
N N N Plt function anomalies, Urea clot solubility
Factor XIII deficiency(rare)
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
50,000-10,000/mm3.
- 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. 2. Haemorrhagic disease of newborn
- Incidance is 1:200 neonates (Not given Vit-K).
- For active bleeding : 10ml/kg FFP & Inj
Vitamin K 1mg IV .
- If mother is on t/t with Phenytoin, primidone,
Methoximide or Phenobarbital, the infant
may be deficient in vit K
.Inj Vit K 10mg IM 24 hours before delivery
. Newborn is monitored for signs of bleeding,
PT, APTT.
26. 3. Delayed Hemorrhagic disease of
newborn:
- Occurs at 4-12 weeks of age
- Not very common in infants who received Vit
K at birth.
- Exclusively breast feeding infant
- Infant on t/t with broad spectrum antibiotics
- Infant with malabsorption
T/t: Vitamin K1- 1mg/week orally for first 3
months of life.
27. Referances
*. Sutor AH, von Kries R, Marlies Conelissen EA,
Mcninch, Andrew M. VitaminK Deficiency
Bleeding (VKDB) in infancy.Thrombosis and
Haemostasis 1999;81: 456-461.
* Narang A. Hemorrhagic Disease of Newborn.
Indian Pediatr 1989, 26:523-524. 4. von Kries
R, Hanawa Y. Neonatal vitamin K
prophylaxis.Thrombosis and Haemostasis
1993, 69:293-295.
