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Hemorrhagic Disease of the Newborn

Hemorrhagic Disease of Newborn Due to Vitamin K Deficiency

Hemorrhagic disease of the newborn, also known as vitamin K deficiency bleeding affects term and preterm infants and causes unexpected blood loss at various sites, including cerebral bleeding, with potentially severe consequences. Traumatic birth and hemophilia are predisposing factors.


Presentation

While assessing hemorrhagic disease of the newborn, the physician should first inquire about maternal conditions, medication and feeding preferences, in order to determine a deficiency of vitamin K during the pregnancy. In the infant, the classical signs may present during the 2nd and 7th day. The illness is classified based on the timing the initial blood loss event is observed [1]. In early-onset, bleeding starts during the first 24 hours of life. This is the case if mothers used anticonvulsant or antituberculosis drugs during gestation. The severe type may be partly prevented by administering vitamin K to the mother during the last 2 to 4 weeks of pregnancy.

Affected children exhibit gastrointestinal, brain, skin or mucous membrane bleeding or blood loss from the umbilical stump. Late-onset symptoms usually appear between 2nd and 12th week. However, they can be noticed till 6 months of age. It affects breastfed babies who failed to receive prophylactic vitamin K at birth and have undiagnosed cholestasis leading to vitamin K malabsorption [2] and may also cause intracranial hemorrhage [3]. This manifests as apnea, seizures, and shock, while other parenchymal organs blood loss may be difficult to detect by clinical examination. However, if suspicion arises, urgent imaging evaluation is required. Cases have been reported in Europe [4] and Australia [5] [6].

Infants may report with blood-stained sputum, respiratory distress, irritability or melena. If the hemorrhage is significant, the baby will be tachycardic and the blood pressure will be low. The late form of the illness may also be a manifestation of several underlying conditions, such as cystic fibrosis, diarrhea, hepatitis, alpha 1-antitrypsin deficiency, Alagille syndrome, galactosemia, and abetalipoproteinemia.

Pathologist
  • We present a single fatal case of late-onset HDN with illustration of hematologic assays that can be performed to assist the pathologist in making the correct diagnosis of HDN.[ncbi.nlm.nih.gov]
Developmental Delay
  • However, he suffered significant developmental delay with failure to thrive due to poor swallow function. His parents declined further invasive therapy, and he died at home before 1 year of life.[thefreelibrary.com]
Easy Bruising
  • An unrelated infant presented at 5 months of age with diarrhea and easy bruising despite IM vitamin K at birth. These cases illustrate the morbidity associated with late HDN.[ohsu.pure.elsevier.com]
Aspiration
  • […] associated hepatic damage Infections Viability, survivability and long term sequelae of prematurity Viability Survivability in prematurity Severe long term sequelae in babies born prematurely Birth asphyxia Hypoxic-ischemic encephalopathy (HIE) Meconium aspiration[atlases.muni.cz]
Colic
  • He was discharged home with the diagnosis of a possible viral infection versus onset of colic. The patient remained fussy throughout the day and then refused to nurse despite an interval of 5 hours between feeds.[thefreelibrary.com]
Palatal Petechiae
  • At that time, he was noted to have palatal petechiae and a rapid strep was performed which was negative. He was discharged home with the diagnosis of a possible viral infection versus onset of colic.[thefreelibrary.com]
Hypotension
  • Other potential problems include transmission of infectious disease, anaphylactoid reactions, and alterations in serum ionized calcium with hypotension. (22) Recombinant factor VIIa (rFVIIa, NovoSeven, Novo Nordisk, Princeton, NJ) is a novel hemostatic[thefreelibrary.com]
Scleral Icterus
  • The infant remained irritable over the ensuing 48 hours and the day before admission to our institution, the report from the family's pediatrician was that the only abnormal physical finding was scleral icterus.[thefreelibrary.com]
Petechiae
  • At that time, he was noted to have palatal petechiae and a rapid strep was performed which was negative. He was discharged home with the diagnosis of a possible viral infection versus onset of colic.[thefreelibrary.com]
Intracranial Hemorrhage
  • Isolated intracranial hemorrhage is a common mode of presentation.[ncbi.nlm.nih.gov]
  • A qualitative assessment of platelets concerning mass, as well as their total number is a reliable predictor for intracranial hemorrhage in gram-negative sepsis newborns.[symptoma.com]
  • The first case is a previously healthy breastfed male who received one dose of oral vitamin K at birth and developed an intracranial hemorrhage 5 weeks later.[ohsu.pure.elsevier.com]
  • At 4 weeks she began bleeding at the umbilicus and 4 days after she suffered an intracranial hemorrhage.[link.springer.com]
Screaming
  • The patient had been seen earlier that day at an urgent care clinic because he was screaming, fussy, and acting differently than normal. At that time, he was noted to have palatal petechiae and a rapid strep was performed which was negative.[thefreelibrary.com]
Encephalopathy
  • Total parenteral nutrition associated hepatic damage Infections Viability, survivability and long term sequelae of prematurity Viability Survivability in prematurity Severe long term sequelae in babies born prematurely Birth asphyxia Hypoxic-ischemic encephalopathy[atlases.muni.cz]

Workup

When facing a child with evident bleeding, the physician should order a complete coagulation panel, including a complete cell blood count, fibrinogen level, fibrin degradation products, prothrombin time, activated partial thromboplastin time and thrombin clotting time, as well as the international normalized ratio (INR). These tests have orientation values and only point to the existence of a vitamin K deficiency, they do not directly establish the diagnosis. The prothrombin time will be increased, while fibrinogen and platelets are normal. A more useful test is represented by protein induced by vitamin K antagonism, that will be increased, but it is not widely available. Thrombocytopenia or an increased activated partial thromboplastin time are not consistent with hemorrhagic disease of the newborn diagnosis, but rather with antiplatelet antibody maternal transmission by breastfeeding, for instance [7]. Fibrin degradation products are increased in liver diseases, after blood transfusion or in disseminated intravascular coagulation in children. An INR 1.4 or less excludes vitamin K deficiency. Factor VIII may be congenitally low or decreased due to disseminated intravascular coagulation.

A qualitative assessment of platelets concerning mass, as well as their total number is a reliable predictor for intracranial hemorrhage in gram-negative sepsis newborns [8]. If suspected, liver pathology can be demonstrated using classical biochemistry tests and imaging methods like endoscopic retrograde cholangiopancreatography. A liver biopsy is occasionally needed in order to exclude biliary atresia or inherited metabolic liver diseases [9]. In case neurologic symptoms are present and intracranial hemorrhage is suspected, a magnetic resonance imaging cranio- cerebral scan should be performed. Intrathoracic bleeding is ruled out using radiographs and ultrasound examination.

Treatment

  • This case highlighted the good prognosis of an uncommon complication when prompt diagnosis and appropriate treatment are instituted. The importance of vitamin K prophylaxis to all newborns is emphasized.[ncbi.nlm.nih.gov]
  • Treatment for vitamin K deficiency bleeding: Specific treatment for VKDB will be determined by your baby's physician based on: your baby's gestational age, overall health, and medical history extent of the disease your baby's tolerance for specific medications[medcentral.org]
  • Vitamin K deficiency is rare in adults, except in syndromes with poor fat absorption, in liver disease, or during treatment with certain anticoagulant drugs, which interfere with vitamin K metabolism.[britannica.com]

Prognosis

  • This case highlighted the good prognosis of an uncommon complication when prompt diagnosis and appropriate treatment are instituted. The importance of vitamin K prophylaxis to all newborns is emphasized.[ncbi.nlm.nih.gov]
  • 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.[slideshare.net]
  • (MCA) Doppler time average mean velocity (TAMV): may occur before precipitation of fetal hydrops 1 increase in fetal middle cerebral arterial (MCA) Doppler peak systolic velocity (PSV): may occur before precipitation of fetal hydrops 2 Treatment and prognosis[radiopaedia.org]
  • Prognosis The prognosis is good for most affected babies. Intracranial haemorrhage and late VKDB account for the mortality associated with VKDB [ 11 ]. Prevention All forms of VKDB are now far less common due to understanding of the aetiology.[patient.info]

Etiology

  • Emergency physicians may encounter presentations of bleeding in the neonate that have multifactorial etiologies.[ncbi.nlm.nih.gov]
  • Etiology Vitamin K is required for production of coagulation factors II., VII., IX. and X. in the liver Newborns are relatively vitamin K deficient because of low vitamin K stores at birth low levels of vitamin K in the breast milk sterility of the intestine[atlases.muni.cz]
  • Head Trauma 234 Sepsis and Septic Shock 242 Nonpolio Enteroviruses 332 Respiratory Syncytial Virus 334 Rhinoviruses 336 Roseola and Human Herpesviruses 337 Rubella 338 Rubeola 339 VaricellaZoster Virus 342 Viral Gastroenteritis 344 Disorders of Unknown Etiology[books.google.com]
  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Etiologies and risk factors of three types of hemorrhagic diseases of newborn have been shown in Table 1.[alliedacademies.org]

Epidemiology

  • Robison, Epidemiology of Leukemia in Childhood, Oncology of Infancy and Childhood, 10.1016/B978-1-4160-3431-5.00001-7, (1-25), (2009). Martin J. Shearer, Vitamin K deficiency bleeding (VKDB) in early infancy, Blood Reviews, 23, 2, (49), (2009).[doi.org]
  • Epidemiology [ 2 ] In the UK, VKDB is very rare with most cases occurring in breastfed babies whose parents have refused prophylaxis. The incidence internationally of early VKDB of at-risk neonates (see Risk factors, below) ranges from 6-12%.[patient.info]
  • A few case reports are available of late HDN in babies even after receiving injection vitamin K. 14 An epidemiological study from Germany by von Kries 15 showed a failure rate (occurrence of late HDN) of 0.25 per 100,000 infants after IM administration[pjms.com.pk]
  • Our purpose was to describe the epidemiological, diagnostic, and common causes of new-bornhaemorrhagic syndrome in paediatric emergency medical department of the Rabat Children's Hospital.[panafrican-med-journal.com]
Sex distribution
Age distribution

Prevention

  • Our conclusions are that physicians must be alert to mild bleeding in newborns and that prophylaxis with 1 mg of intramuscular vitamin K at birth may be insufficient to prevent late HDN.[ncbi.nlm.nih.gov]
  • Prevention of vitamin K deficiency in newborns. Br J Haematology. 1999104430-437.[powershow.com]

References

Article

  1. Pichler E, Pichler L. The neonatal coagulation system and the vitamin K deficiency bleeding - a mini review. Wien Med Wochenschr. 2008;158(13-14):385-95.
  2. Shearer MJ. Vitamin K deficiency bleeding (VKDB) in early infancy. Blood Rev. 2009;23(2):49-59.
  3. Pereira SP, Shearer MJ, Williams R, et al. Intestinal absorption of mixed micellar phylloquinone (vitamin K1) is unreliable in infants with conjugated hyperbilirubinaemia: implications for oral prophylaxis of vitamin K deficiency bleeding. Arch Dis Child Fetal Neonatal Ed. 2003;88:F113–18.
  4. von Kries R, Göbel U. Oral vitamin K prophylaxis and late haemorrhagic disease of the newborn. Lancet. 1994;343:352.
  5. Loughan PM, McDougall PN. The efficacy of oral vitamin K1: Implications for future prophylaxis to prevent haemorrhagic disease of the newborn. J Paediatr Child Health. 1993;29:171-6.
  6. Loughnan PM, McDougall PN. Epidemiology of late onset haemorrhagic disease: A pooled data analysis. J Paediatr Child Health. 1993;29:177-81.
  7. Hauschner H, Rosenberg N, Seligsohn U, et al. Persistent neonatal thrombocytopenia can be caused by IgA antiplatelet antibodies in breast milk of immune thrombocytopenic mothers. Blood. 2015;126(5):661-4.
  8. Mitsiakos G, Pana ZD, Chatziioannidis I, et al. Platelet mass predicts intracranial hemorrhage in neonates with gram-negative sepsis. J Pediatr Hematol Oncol. 2015;37 (7):519-23.
  9. Bellini S. What parents need to know about vitamin K administration at birth. Nurs Womens Health. 2015;19(3):261-5.

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Last updated: 2019-06-28 09:28