CASE STUDY Ahmad Adel A.

Maternal serologies were unremarkable. 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. Maternal serologies; [TORCH] Toxoplasmosis, Other (syphilis, varicella-zoster, parvovirus B19), Rubella, Cytomegalovirus (CMV), and Herpes infections.

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.

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.

Related to DIC..

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.

Laboratory values at her admission showed a hemoglobin of 10 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.

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.

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).

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.

Diagnosis.. It’s VKDBD.

Vitamin K Deficiency Bleeding Disorder (VKDBD), - also known as (Hemorrhagic disease of the newborn). **Not to be confused with Hemolytic disease of the newborn (HDN). It’s a coagulation disturbance in newborn infants due to vitamin K deficiency. As a consequence of vitamin K deficiency there is an impaired production of coagulation factors II, VII, IX, X, protein C and protein S by the liver, resulting in excessive bleeding (hemorrhage). It’s a potentially life-threatening condition. 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.

Forms of vitamin K – Vitamin K1 (phylloquinon) – plant origin. – Vitamin K2 (menaquinon) – normally produced by bacteria in the large intestine. – Vitamin K3 - is no longer used medically because of its ability to produce hemolytic anemia.

comparison of Vitamin K1 and K2 K1 a K2 are used differently in the body K1 – Blood clotting - prevents excessive bleeding. K2 – important in non-coagulation actions - mineralization Helps direct calcium to the right places (into bones and teeth and out of soft issues like arteries and kidneys) Deficiency symptoms; K1 – Bruises, Increased bleeding time K2 – Weak bones, Arterial calcification https://innovixlabs.com/blogs/insights/vitamin-k1-vs-k2-a-side-by-side-comparison

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.

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

Vitamin K Dependent Proteins factor II (prothrombin) factor VII factor IX factor X (Stuart factor) protein C & protein S Protein Z 20

Newborns- Why Vitamin K Levels Are Low? – The movement of vitamin K through the placenta is difficult. It means that a developing baby does not receive sufficient vitamin K. – Babies who do not receive preventive vitamin K in an injection at birth – Breast milk contains low levels of vitamin K **breast milk contains less vitamin K than cow's milk formula – Good gut bacteria in human bodies produce vitamin K. But this is not present in the body of the newborns – Maternal use of antiseizure or anticoagulant drugs

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

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

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.