Vitamin K deficiency bleeding

Last updated
Vitamin K deficiency bleeding
Other namesHaemorrhagic disease of the newborn
Phylloquinone structure.svg
Vitamin K 1
Specialty Pediatrics   OOjs UI icon edit-ltr-progressive.svg
Symptoms Bleeding
Usual onsetBirth to 2 months of age
TypesEarly, Classical, Late
CausesVitamin K deficiency
PreventionVitamin K supplementation after birth

Vitamin K deficiency bleeding (VKDB) of the newborn, previously known as haemorrhagic disease of the newborn, [1] is a rare form of bleeding disorder that affects newborns and young infants due to low stores of vitamin K at birth. [2] It commonly presents with intracranial haemorrhage with the risk of brain damage or death. [3]

Contents

Newborn infants have low stores of vitamin K, and human breast milk has low concentrations of the vitamin. This combination can lead to vitamin K deficiency and later onset bleeding. Vitamin K deficiency leads to the risk of blood coagulation problems due to impaired production of clotting factors II, VII, IX, X, protein C and protein S by the liver. More rarely VKDB can be caused by maternal medicines causing vitamin K deficiency in the newborn. [2]

VKDB can largely be prevented by prophylactic supplementation of vitamin K, which is typically given shortly after birth by intramuscular injection. Most national health organisations recommend routine vitamin K supplementation after birth. [2] Widespread use of this has made this a rare disease.

Classification

VKDB is classified as early, classical or late depending on when it first starts with each having somewhat different types of bleeding and underlying cause:

Classification of vitamin K deficiency in the newborn (VKDB) [2]
SyndromeTime of onsetCommon sites of bleedingPotential causes
EarlyFirst 24 hoursScalp, skin, brain, chest, abdomenMaternal medications
Classical1-7 daysGut, umbilicus, skin, nose, circumcision Idiopathic, breast feeding
LateAfter day 8Brain, skin, gutIdiopathic, breast feeding, cholestasis

Signs and symptoms

VKDB presents typically in the first month of life with bleeding which can be from various locations. Late onset VKDB presents with bleeding into the brain (intracranial haemorrhage) in more than half of cases. [2]

Causes

Newborns are relatively vitamin K deficient for a variety of reasons: They have low vitamin K stores at birth as vitamin K passes the placenta poorly. Levels of vitamin K in human breast milk are low. Gut flora, that in adults produces vitamin K, has not yet developed. [2] Early VKDB is rare and caused by maternal medications that interact with vitamin K such as warfarin, phenytoin, or rifampicin. [2] Classical VKDB is more common and caused by the relative deficiency at birth with inadequate vitamin K intake. This is often termed idiopathic as no one cause is found.[ citation needed ] Late VKDB presents after day 8 and up to 6 months of age, coinciding with the typical age for exclusive breast feeding due to the low levels of vitamin K in human breast milk. Many of these infants have poor vitamin K absorption due to cholestasis which compounds low intake. [2]

Diagnosis

Bleeding in an infant without vitamin K supplementation with elevated prothrombin time (PT) that is corrected by vitamin K administration is typically sufficient to make the diagnosis. Confirmation, or investigation of minor deficiency, can be performed by testing proteins produced in the absence of vitamin K, the most established assay being for PIVKA-II. [2]

Prevention

Late onset VKDB is nearly completely prevented by early supplementation of vitamin K which is typically given to newborns shortly after birth. [2] [4] [5] The most effective method of administration is by intramuscular injection shortly after birth but it can be given orally in three doses over the first month. [2] [6]

It is not possible to reliably distinguish which infants are at high risk of late VKDB and the potential consequences are high, as such most national health organisations recommend routine supplementation in the first 24 hours of life. [2]

Controversy

Controversy arose in the early 1990s regarding routine supplementation, when two studies suggested a relationship between parenteral administration of vitamin K and childhood cancer. [7] However, both studies have been discredited on the basis of poor methodology and small sample sizes, and a review of the evidence published in 2000 by Ross and Davies found no link between the two. [8]

Treatment

Treatment of established bleeding depends on the location but includes vitamin K1 (phylloquinone; phytomenadione; phytonadione) administration which restores the prothrombin time rapidly. Severe bleeding may require blood products such as fresh frozen plasma (FFP), a prothrombin complex concentrate (PCC). [2]

Related Research Articles

<span class="mw-page-title-main">Rickets</span> Childhood bone disorder

Rickets is a condition that results in weak or soft bones in children, and is caused by either dietary deficiency or genetic causes. Symptoms include bowed legs, stunted growth, bone pain, large forehead, and trouble sleeping. Complications may include bone deformities, bone pseudofractures and fractures, muscle spasms, or an abnormally curved spine.

<span class="mw-page-title-main">Vitamin K</span> Fat-soluble vitamers

Vitamin K is a family of structurally similar, fat-soluble vitamers found in foods and marketed as dietary supplements. The human body requires vitamin K for post-synthesis modification of certain proteins that are required for blood coagulation or for controlling binding of calcium in bones and other tissues. The complete synthesis involves final modification of these so-called "Gla proteins" by the enzyme gamma-glutamyl carboxylase that uses vitamin K as a cofactor.

<span class="mw-page-title-main">Shaken baby syndrome</span> Medical condition

Shaken baby syndrome (SBS), also known as abusive head trauma (AHT), is a medical condition in children younger than five years old, generally caused by blunt trauma, vigorous shaking, or a combination of both. SBS is the leading cause of fatal head injuries in children under two, with a risk of death of about 25%. The most common symptoms include retinal bleeds, multiple fractures of the long bones, and subdural hematomas.

<span class="mw-page-title-main">Breast milk</span> Milk produced by the mammary glands in the breast of a human female

Breast milk or mother's milk is milk produced by the mammary glands in the breast of human females. Breast milk is the primary source of nutrition for newborn infants, comprising fats, proteins, carbohydrates, and a varying composition of minerals and vitamins. Breast milk also contains substances that help protect an infant against infection and inflammation, such as symbiotic bacteria and other microorganisms and immunoglobulin A, whilst also contributing to the healthy development of the infant's immune system and gut microbiome.

<span class="mw-page-title-main">Neonatal jaundice</span> Medical condition

Neonatal jaundice is a yellowish discoloration of the white part of the eyes and skin in a newborn baby due to high bilirubin levels. Other symptoms may include excess sleepiness or poor feeding. Complications may include seizures, cerebral palsy, or kernicterus.

<span class="mw-page-title-main">Nutrition and pregnancy</span> Nutrient intake and dietary planning undertaken before, during and after pregnancy

Nutrition and pregnancy refers to the nutrient intake, and dietary planning that is undertaken before, during and after pregnancy. Nutrition of the fetus begins at conception. For this reason, the nutrition of the mother is important from before conception as well as throughout pregnancy and breastfeeding. An ever-increasing number of studies have shown that the nutrition of the mother will have an effect on the child, up to and including the risk for cancer, cardiovascular disease, hypertension and diabetes throughout life.

<span class="mw-page-title-main">Phytomenadione</span> Chemical compound

Phytomenadione, also known as vitamin K1 or phylloquinone, is a vitamin found in food and used as a dietary supplement. It is on the World Health Organization's List of Essential Medicines.

<span class="mw-page-title-main">Hypoprothrombinemia</span> Medical condition

Hypoprothrombinemia is a rare blood disorder in which a deficiency in immunoreactive prothrombin, produced in the liver, results in an impaired blood clotting reaction, leading to an increased physiological risk for spontaneous bleeding. This condition can be observed in the gastrointestinal system, cranial vault, and superficial integumentary system, affecting both the male and female population. Prothrombin is a critical protein that is involved in the process of hemostasis, as well as illustrating procoagulant activities. This condition is characterized as an autosomal recessive inheritance congenital coagulation disorder affecting 1 per 2,000,000 of the population, worldwide, but is also attributed as acquired.

<span class="mw-page-title-main">Factor X deficiency</span> Medical condition

Factor X deficiency is a bleeding disorder characterized by a lack in the production of factor X (FX), an enzyme protein that causes blood to clot in the coagulation cascade. Produced in the liver FX when activated cleaves prothrombin to generate thrombin in the intrinsic pathway of coagulation. This process is vitamin K dependent and enhanced by activated factor V.

<span class="mw-page-title-main">Folate deficiency</span> Abnormally low level of folate (vitamin B9) in the body

Folate deficiency, also known as vitamin B9 deficiency, is a low level of folate and derivatives in the body. This may result in a type of anemia in which red blood cells become abnormally large and is a late finding in folate deficiency and folate deficiency anemia is the term given for this medical condition. Signs of folate deficiency are often subtle. Symptoms may include feeling tired, heart palpitations, shortness of breath, feeling faint, open sores on the tongue, loss of appetite, changes in the color of the skin or hair, irritability, and behavioral changes. Temporary reversible infertility may occur. Folate deficiency anemia during pregnancy may give rise to the birth of low weight birth premature infants and infants with neural tube defects.

Neonatal alloimmune thrombocytopenia is a disease that affects babies in which the platelet count is decreased because the mother's immune system attacks her fetus' or newborn's platelets. A low platelet count increases the risk of bleeding in the fetus and newborn. If the bleeding occurs in the brain, there may be long-term effects.

<span class="mw-page-title-main">Breastfeeding</span> Feeding of babies or young children with milk from a womans breast

Breastfeeding, or nursing, is the process by which human breast milk is fed to a child. Breast milk may be from the breast, or may be pumped and fed to the infant. The World Health Organization (WHO) recommends that breastfeeding begin within the first hour of a baby's life and continue as often and as much as the baby wants. Health organizations, including the WHO, recommend breastfeeding exclusively for six months. This means that no other foods or drinks, other than vitamin D, are typically given. The WHO recommends exclusive breastfeeding for the first 6 months of life, followed by continued breastfeeding with appropriate complementary foods for up to 2 years and beyond. Of the 135 million babies born every year, only 42% are breastfed within the first hour of life, only 38% of mothers practice exclusive breastfeeding during the first six months, and 58% of mothers continue breastfeeding up to the age of two years and beyond.

Vitamin K deficiency results from insufficient dietary vitamin K1 or vitamin K2 or both.

<span class="mw-page-title-main">Vitamin K reaction</span> Medical condition

Vitamin K reactions are adverse side effects that may occur after injection with vitamin K. The liver utilizes vitamin K to produce coagulation factors that help the body form blood clots which prevent excessive bleeding. Vitamin K injections are administered to newborns as a preventative measure to reduce the risk of hemorrhagic disease of the newborn (HDN).

<span class="mw-page-title-main">Congenital cytomegalovirus infection</span> Medical condition

Congenital cytomegalovirus (cCMV) is cytomegalovirus (CMV) infection in a newborn baby. Most have no symptoms. Some affected babies are small. Other signs and symptoms include a rash, jaundice, hepatomegaly, retinitis, and seizures. It may lead to loss of hearing or vision, developmental disability, or a small head.

Infant feeding is the practice of feeding infants. Breast milk provides the best nutrition when compared to infant formula. Infants are usually introduced to solid foods at around four to six months of age.

Neonatal hypocalcemia is an abnormal clinical and laboratory hypocalcemia condition that is frequently observed in infants. It is commonly presented within the first 72 hours of a newborn's life. Neonatal hypocalcemia can cause seizures in infants requiring a calcium infusion until homeostasis is achieved; allowing for positive clinical outcomes within weeks of treatment.

<span class="mw-page-title-main">Neonatal infection</span> Human disease

Neonatal infections are infections of the neonate (newborn) acquired during prenatal development or within the first four weeks of life. Neonatal infections may be contracted by mother to child transmission, in the birth canal during childbirth, or after birth. Neonatal infections may present soon after delivery, or take several weeks to show symptoms. Some neonatal infections such as HIV, hepatitis B, and malaria do not become apparent until much later. Signs and symptoms of infection may include respiratory distress, temperature instability, irritability, poor feeding, failure to thrive, persistent crying and skin rashes.

The postpartum physiological changes are those expected changes that occur in the woman's body after childbirth, in the postpartum period. These changes mark the beginning of the return of pre-pregnancy physiology and of breastfeeding. Most of the time these postnatal changes are normal and can be managed with medication and comfort measures, but in a few situations complications may develop. Postpartum physiological changes may be different for women delivering by cesarean section. Other postpartum changes, may indicate developing complications such as, postpartum bleeding, engorged breasts, postpartum infections.

<span class="mw-page-title-main">4-Amino-2-methyl-1-naphthol</span> Chemical compound

4-Amino-2-methyl-1-naphthol is a menadione analog. Its water-soluble hydrochloride (HCl) salt is often called vitamin K5. The HCl salt has been used as a medicine for vitamin K deficiency under tradenames such as Synkamin, which was sold by Parke-Davis, but has since been discontinued.

References

  1. Sutor, Anton; von Kries, Rüdiger; Cornelissen, Marlies; McNinch, Andrew; Andrew, Maureen (9 December 2017). "Vitamin K Deficiency Bleeding (VKDB) in Infancy". Thrombosis and Haemostasis. 81 (3): 456–461. doi:10.1055/s-0037-1614494. S2CID   77702558.
  2. 1 2 3 4 5 6 7 8 9 10 11 12 13 Shearer, Martin J. (March 2009). "Vitamin K deficiency bleeding (VKDB) in early infancy". Blood Reviews. 23 (2): 49–59. doi:10.1016/j.blre.2008.06.001. PMID   18804903.
  3. Volpe, Joseph J (2017-10-06). Volpe's neurology of the newborn (Sixth ed.). Elsevier. ISBN   978-0-323-42876-7 . Retrieved 9 March 2020.
  4. American Academy of Pediatrics Committee on Fetus Newborn (July 2003). "Controversies concerning vitamin K and the newborn. American Academy of Pediatrics Committee on Fetus and Newborn" (PDF). Pediatrics. 112 (1 Pt 1): 191–2. doi: 10.1542/peds.112.1.191 . PMID   12837888.
  5. Logan S, Gilbert R (1998). "Vitamin K For Newborn Babies" (PDF). Department of Health. Archived from the original (PDF) on 7 January 2013. Retrieved 12 Oct 2014.
  6. "Postnatal care up to 8 weeks after birth". www.nice.org.uk. NICE . Retrieved 9 March 2020.
  7. Parker L, Cole M, Craft AW, Hey EN (January 1998). "Neonatal vitamin K administration and childhood cancer in the north of England: retrospective case-control study". BMJ. 316 (7126): 189–93. doi:10.1136/bmj.316.7126.189. PMC   2665412 . PMID   9468683.
  8. McMillan DD, et al. (Canadian Paediatric Society, Fetus and Newborn Committee) (1997). "Routine administration of vitamin K to newborns". Paediatrics & Child Health. 2 (6): 429–431. doi:10.1093/pch/2.6.429. PMC   7745636 . PMID   33372508.