HEMORRHAGIC DISEASE OF NEWBORN

14/2/2014 ::13 ربيع الثاني 1435 Ped.lec Dr.Emad Alhadeethi
HEMORRHAGIC DISEASE OF NEWBORN
(Vitamin K Deficiency)
Vit K deficiency is the most common cause of hemorrhagic disease in the newborn. It result in deficiency of the clotting factors; 2, 7, 9 & 10. The site of bleeding can be any site in body, especially the umbilical stump.
Currently, the following 3 forms of vitamin K are known:
K1 ( 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 K1.
K2 ( Menaquinone ) is synthesized by gut flora.
K3 ( Menadione ) is a synthetic, water soluble form that is no longer used medically because of its ability to produce hemolytic anemia.

Pathophysiology:

Transplacental transfer of vitamin K is very limited during pregnancy, and the storage of vitamin K in neonatal liver is also limited.
The newborn infant’s intestinal tract is relatively sterile and takes some time to colonize with bacteria, which may have a role in synthesizing vitamin K2 (menaquinones).
Once the infantile gut is colonized with bacterial flora, the microbial production of vitamin K2 results in a lower risk of infantile vitamin K deficiency bleeding.
Bacteroides fragilis are more significant in producing human vitamin K in the intestine than Escherichia coli.
Breast milk is a poor source of vitamin K (breast milk levels are 1-4 μ g/L). The recommended dietary intake of vitamin K is 1 μ g/kg/d.
Exclusively breastfed infants have intestinal colonization with lactobacilli that do not synthesize vitamin K2.
Formula-fed infants have higher concentrations of vitamin K1.

Etiology:

It can be divided according to the onset of bleeding as follows:-


Early onset;
* 1st 24 hr after birth.
* It occurs if the mother has been treated with drugs that interfere with vit K function e.g. warfarin, phenobarbital, phenytoin, rifampin, isoniazid.
* Site of bleeding :
Cephalohematoma, subgaleal, intracranial, gastrointestinal, umbilicus and intra-abdominal.
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Classic disease (most common);
* Usually manifested after the 2nd - 3rd day & gradually improve by 7-10 day of life.
* Causes include:
1- lack of free vit K from the mother during pregnancy.
2- absence of the bacterial intestinal flora (which normally responsible for the synthesis of vit K).
3- breast feeding (because breast milk is deficient in vit K).
*Sites of bleeding :
Intracranial,Gastrointestinal, Ear-Nose-Throat, circumcision, cutaneous, thoracic and injection sites.
Late onset;
* During 1-6 months of life.
* It usually due to vit K malabsorption by Cholestasis e.g. biliary atresia, neonatal hepatitis... etc.
* Sites of bleeding :
Gastrointestinal, Ear-Nose-Throat, intracranial, circumcision, cutaneous and injection sites.

Investigations:

PT & PTT are prolonged (especially PT).
Decrease levels of vit K–dependent factors.
Increase PIVKA (protein induced in vitamin K absence) is a sensitive marker for vit K status; this protein represent the precursor of vit K–dependent factors.

Differential Diagnosis:

Neonatal Thrombocytopenic Purpura
Hemophilia A & B.
Von Willibrand disease.
DIC, usually in sick preterm infant.
Ecchymoses & bruising in the infant skin (especially preterm), or may occur after traumatic delivery.
Petechiae or bluish suffusion limited to the face (or any presenting part) as a result of venous congestion during delivery; it usually resolve within 2-3 wk.
Swallowed blood syndrome; it is bloody stool in the 2nd or 3rd day of life due to swallowing of maternal blood during delivery or may be due to fissure in the maternal nipple during feeding.

Treament:

If there is clinical bleeding, give vit K1 ( philloquinone ) 1-5 mg slow IV infusion with improvement in coagulation defects and cessation of bleeding noted within few hours.
Serious bleeding, particularly in premature infants or those with liver disease, may require a transfusion of fresh frozen plasma or whole blood.

Prevention:

1 mg of vit K by IM injection at birth prevents the decrease in Vit K-dependant factors in full-term infants, but it is not uniformly effective in the prophylaxis of hemorrhagic disease of the newborn in premature infants.


Mortality and Morbidity:
Intracranial hemorrhage is uncommon in classic vitamin K deficiency bleeding but can be observed in more than 50% of infants with late-onset vitamin K deficiency bleeding. Intracranial hemorrhage is responsible for nearly all mortality and long-term sequelae due to vitamin K deficiency bleeding.