Haemorrhagic disease of newborn

Haemorrhagic disease of newborn by Dr. Rabi Dhakal

1. Haemorrhagic Disease of Newborn Dr.Rabi Dhakal

2. Introduction Any haemorraghic manifestation due to deficiency of vitamin K dependent clotting factors is known as Haemorraghic disease of newborn (HDN). Coagulation factors II, VII, IX , X and other Gla-proteins ( protein C, protein S, protein Z) also depend on the presence of vitamin K for their activity.

3. Vitamin K The following forms of vitamin K are known: K  1 : Phylloquinone is predominantly found in green leafy vegetables, vegetable oils, and dairy products. Vitamin K given to neonates as a prophylactic agent is an aqueous, colloidal solution of vitamin K  1 . K  2 : Menaquinone is synthesized by gut flora. K  3 : Menadione is a synthetic, water soluble form. Vitamin K is an essential cofactor for γ-glutamyl carboxylase enzymatic activity that catalyses the γ-carboxylation of specific glutamic acid residues in a subclass of proteins.

4. Contd….

5. Vitamin K in Newborn New born babies are predisposed to develop vitamin K deficiency. Minimal transplacental passage of vitamin K Limited hepatic storage of vitamin k in newborn Low concentration of vitamin k in breast milk Absence of the bacterial intestinal flora normally responsible for the synthesis of vitamin K

6. Haemorrhagic Disease of Newborn Haemorrhagic disease of the newborn resulting from severe transient deficiencies in vitamin K–dependent factors is characterized by bleeding that tends to be gastrointestinal, nasal, subgaleal, intracranial, or post-circumcision . Classification: Early-HDN Classical-HDN Late-HDN

7. Early-HDN Onset: 0-24 hr Incidence:Rare Site: Cephalohematoma, Subgaleal, Intracranial, Gastrointestinal, Umbilicus, Intra-abdominal. Etiology: Maternal drugs (phenobarbital, phenytoin, warfarin, rifampin, isoniazid) that interfere with vitamin K. Risk factor: Inherited coagulopathy.

8. Classical-HDN Onset : 2-7 days Incidence: ≈2% if infant not given vitamin K. Site : Gastrointestinal, Ear-nose-throat-mucosal, Intracranial, Circumcision, Cutaneous, Injection sites. Etiology: Vitamin K deficiency, Breast-feeding.

9. Late-HDN Onset: 1-6  mo Incidence: Dependent on primary disease. Site:I ntracranial, Gastrointestinal, Cutaneous, Ear-nose-throat-mucosal, Injection sites, Thoracic. Etiology: Cholestasis—malabsorption of vitamin K (biliary atresia, cystic fibrosis, hepatitis ) Risk factor: Abetalipoprotein deficiency, Idiopathic in Asian breast-fed infants, Warfarin ingestion.

10. Presentation G astrointestinal haemorrhage Prolonged bleeding after circumcision E pistaxis Ecchymosis (bruising) I ntracranial haemorrhage Bleeding from umbilicus Cephalohematoma…..

11. Investigation Coagulation profile:Prothrombin time (PT), activated partial thromboplastin time (aPTT), fibrinogen levels, and a platelet count should be included in the initial workup for vitamin K deficiency bleeding in a newborn. A prolonged PT is usually the first laboratory test result to be abnormal in vitamin K deficiency bleeding. Normal aPTT, fibrinogen levels and a platelet count . Factor assay. Imaging study:CT scan ,MRI,USG CBC LFT Stool for occult blood ERCP Liver biopsy

12. DDX Alloimmune Thrombocytopenia Consumption Coagulopathy Hepatobiliary Disease Maternal Isoimmune Thrombocytopenia Pediatric Von Willebrand Disease Uncommon Coagulopathies Swallowed blood syndrome

13. Management Intramuscular administration of 1 mg of vitamin K at the time of birth prevents the decrease in vitamin K–dependent factors in full-term infants, but it is not uniformly effective in the prophylaxis of haemorrhagic disease of the newborn, particularly in breast-fed and in premature infants . The disease may be effectively treated with a slow intravenous infusion of 1-5 mg of vitamin K 1. Serious bleeding, particularly in premature infants or those with liver disease, may require a transfusion of fresh frozen plasma or whole blood . Hematomas, melena, and post-circumcision and umbilical cord bleeding may be present; only 5-35% of cases of factor VIII and IX deficiency become clinically apparent in the newborn period. Treatment of the rare congenital deficiencies of coagulation factors requires fresh frozen plasma or specific factor replacement.

14. Surgical Care Normally , vitamin K deficiency bleeding infants do not require surgical care but in rare cases, an infant may need neurosurgical evaluation and treatment. Other conditions, such as those associated with short bowel syndrome and hepatobiliary disease may require surgical evaluation

15. Complication Intracranial hemorrhage is the primary serious complication of vitamin K deficiency bleeding. Complications of treatment include anaphylactoid like reactions during intravenous (IV) vitamin K administration, hyperbilirubinemia or hemolytic anemia after high doses of vitamin K, and hematomas at the site of injection, if administered IM.

16. Prevention Early-HDN: Administrations of vitamin K to infant at birth or to mother (20 mg) before birth. Classical-HDN:Parenteral vitamin K at birth. Late-HDN:Parenteral and high-dose oral vitamin K during periods of malabsorption or cholestasis.

17. Prognosis In the absence of intracranial haemorrhage, the prognosis for vitamin K deficiency bleeding in an otherwise healthy infant is excellent. Prognosis after intracranial haemorrhage depends on the extent and location of the haemorrhage. Long-term sequelae of intracranial hemorrhage may include motor and intellectual deficits.

18. References Nelson Textbook of Pediatrics Manual of Neonatal Care-Cloherty Newborn care-Meharban Singh Medscape

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