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.
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
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:Intracranial, 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.
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
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 K1.
▪ 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.
