- The infant presented with bleeding from the umbilical stump at 27 days of age. Coagulation studies showed prolonged PT and PTT with low coagulation factors II, VII, IX and X. She was diagnosed with vitamin K deficiency bleeding disorder (VKDBD), also known as hemorrhagic disease of the newborn (HDN). VKDBD is a potentially life-threatening condition caused by low vitamin K levels in newborns. She was treated with fresh frozen plasma, vitamin K, and transfusions which normalized her coagulation studies.

1. CASE STUDY

2. • Baby A was a term female infant born via uncomplicated vaginal delivery.
• Maternal serologies were unremarkable.
• she did not receive any prophylaxis or vaccine after delivery.
• She was discharged to home and was reported to have an uneventful
course until, at 27 days of age, she developed severe bleeding from her
umbilical stump.
• The parents used direct pressure but were unable to stop the bleeding.
• At this time, her parents called her primary care physician and were
directed to take her to the emergency department.

3. • She presented at the emergency department with profuse bleeding
from the umbilical stump.
• Parents denied history of bruising, fever, respiratory distress, feeding
intolerance, or changes in voiding, stooling, or activity.
• Parents denied family history of hemophilia or coagulopathy.
• On admission, her complete blood cell count with differential was
unremarkable with hemoglobin of 14.1 g/dL.

4. • Coagulation studies showed prolonged prothrombin time (PT) and
partial thromboplastin time (PTT) without evidence of disseminated
intravascular coagulation (Table 1).
• Blood cultures were obtained and she was started on ampicillin
and cefotaxime.
• With concerns for coagulopathy and her persistent bleeding, and
after 2 unsuccessful attempts to stop the bleeding from her umbilical
stump by cauterization, she was transferred to a medical center with
a hematology service.

6. Related to DIC..

7. • During a review of systems with hematology, the parents stated a
history of small nose bleeds occurring over the past 24 hours and
noted that she also appeared paler in color at that time.
• On examination, her pressure dressing to her umbilicus was
saturated with blood.
• She was hemodynamically stable and breathing comfortably on room
air with normal oxygen saturations.

8. • Laboratory values at her admission showed a hemoglobin of
10.7 g/dL, prolonged PT and PTT (Table 1),and low coagulation
factors II, VII, IX, and X (Table 2).
• She was transfused with 10 mL/kg fresh-frozen plasma (FFP) and
given 5-mg vitamin K by mouth.

10. • About 2 hours after arrival at the emergency department, she
developed compensated hemorrhagic shock with an elevated heart
rate of 160 to 170 beats per minute, normal blood pressures, well
perfused extremities, and capillary refill at 3 seconds.
• She was noted to have oxygen saturations of 93% on room air and
was placed on a nasal cannula to maintain adequate oxygen
saturations.
• A normal saline bolus of 10 mL/kg was given and 10 mL/kg
packed red blood cells was ordered.

11. • She was transferred to the neonatal intensive care unit (NICU) for
further management at which time she received the packed red blood
cell transfusion.
• Shortly after being admitted to the NICU, her hemorrhaging subsided.
• Coagulation studies were obtained at approximately 4 hours after FFP
administration and were normalized (Table 2).

13. Summary
• PT >100 sec
• PTT >200 sec
• INR 16
• Fibrinogen 250 mg/dL
• Factor II assay <10 (Low) – Normal range 27%-64%
• Factor VII assay <10 (Low) – Normal range 28%-78%
• Factor IX assay <1 (Low) – Normal range 15%-50%
• Factor X assay <10 (Low) – Normal range 21%-65%
• Factor V, VIII, XI, XII – within normal range.

14. • Diagnosis..
It’s VKDBD.

15. • Hemorrhagic Disease of the Newborn (HDN), now known Vitamin K
Deficiency Bleeding Disorder (VKDBD).
• Hemorrhagic disease of the newborn is a rare bleeding problem that
can occur after birth.
• It’s a potentially life-threatening condition.
• Vitamin K plays a key role in blood clotting. Because vitamin K is not
efficiently passed on from mother to baby in utero, most babies are
born with low stores of this vitamin in their system.

16. • Forms of vitamin K:
– Vitamin K1 (phylloquinon) – plant origin
– Vitamin K2 (menaquinon) – normally produced by bacteria in the
large intestine
• K1 a K2 are used differently in the body
• K1 – used mainly for blood clothing
• K2 – important in non-coagulation actions - as in metabolism and
bone mineralization, in cell growth, metabolism of blood vessel
walls cells.

17. Types of VKDB
VKDB is categorized according to the timing of first symptoms:
- early onset occurs within 24 hours of birth
- classic onset occurs within two to seven days
-late onset occurs within two weeks to six months
Newborns routinely receive vitamin K injection (0.5 -1 mg vitamin
K) or 2 mg orally.

18. Signs
• Gastrointestinal haemorrhage
• Prolonged bleeding after circumcision
• Epistaxis
• Ecchymosis (bruising)
• Intracranial haemorrhage
• Bleeding from umbilicus
• Cephalohematoma

19. Vitamin K Dependent Proteins
• factor II (prothrombin)
• factor VII
• factor IX
• factor X (Stuart factor)
• protein C & protein S
• Protein Z

20. • 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

21. http://health.allrefer.com/health/nutrition.html

22. Investigation
• Coagulation profile: (PT), (aPTT), fibrinogen levels, and a platelet
count.
– A prolonged PT is usually the first laboratory test result to be
abnormal in vitamin K deficiency bleeding.
• Factor assay.
• CBC
• Stool for occult blood
- Others

23. Management
▪ Intramuscular administration of 1 mg of vitamin K at the time of birth
- not effective in the prophylaxis of haemorrhagic disease of the newborn,
particularly 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.
▪ specific factor replacement.