HAEMORRHAGIC DISORDERS IN

NEWBORNPRESENTED BY :

VRUTI PATEL,

HAEMORRHAGIC DISEASES

OF THE NEWBORN

It is a syndrome characterized by spontaneous internal or external bleeding. In neonates, there is decreased activity of several clotting factors and diminished platelet function.

CAUSES

• Abnormalities of clotting factors

• Deficiencies of vitamin k dependent factors – II, VII, IX, X. it usually occurs between D2 and D5 and common in preterm and breast fed babies. Deficiency of protein C is also responsible

• Drugs – received by mother during pregnancy – phenytoin, coumarin compounds, salicylates (affects vit K function).

• DIC – due to infection, Anoxia, shock.

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.

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.

III. VWD Type III

B. Platelet problems:

1. Qualitative disorders:- Glanzman’s thrombasthenia.- Bernard-Soulier syndrome- Platelet type VWD

2. Quantitive disorders:

- Immune thrombocytipenia

- Matrnal Preeclampsia, HELLP syndrome or severe uteroplacental insuffuciency.

- DIC due to infection or asphyxia.

- Inherited bone marrow failure syndromes : Fanconi anemia & congenital amegakaryocytic thrombocytopenia

- Congenital leukemia

- Inherited thrombocytopenia syndromes : gray platelet syndrome

- Macrothrombocytopenias : May-Hegglin syndr.

- Platelet consumption in clots/ vascular disorders eg. Vascular malformations, NEC.

C. Vascular origin:

- Pulmonary haemorrhage

- A-V malformations

- CNS haemorrhage

- Hemangiomas.

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

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.

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.

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.

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.

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.

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.

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

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.

- 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)