Late-onset vitamin K deficiency presenting as haemorrhagic shock and severe multi-system organ failure.

Vitamin K is an essential dietary cofactor required for the synthesis of active forms of vitamin K-dependent procoagulant proteins. Vitamin K deficiency, particularly late-onset deficiency occurring between 1 week and 6 months of age, can cause a life-threatening bleeding disorder. An exclusively breastfed, full-term, 6-week-old infant male presented with severe haemorrhagic shock and multi-system organ failure related to caregiver refusal of intramuscular vitamin K after birth. Coagulation studies were normalised within 8 hours of intramuscular vitamin K administration. An increasing number of caregivers are refusing intramuscular vitamin K which has led to a rise in the incidence of vitamin K deficiency bleeding. Health policy organisations around the world emphasise the benefits of intramuscular vitamin K and risks of refusal, particularly in exclusively breastfed infants who are at higher risk due to low vitamin K levels in breast milk. This case highlights the multi-system severity of this life-threatening yet preventable disorder.

Late-onset vitamin K deficiency bleeding in an extremely preterm infant fed an exclusively human milk-based diet.

All newborns need extra phylloquinone (vitamin K1; K1) to prevent vitamin K deficiency bleeding (VKDB). In preterm babies, the main sources are prophylactic K1 given at birth and parenteral and/or enteral feeding thereafter. Preterm babies are at risk of late-onset VKDB if ongoing K1 supplementation is inadequate. For extremely preterm infants fed an exclusive human milk diet, the low K1 content of human milk may predispose them to vitamin K deficiency. Human milk fortification with either bovine milk-derived fortifier or human milk-based fortifier (HMF) made from pooled donor milk is a widely used strategy to improve the micronutrient and growth status of preterm infants. However, the K1 content of HMF is markedly lower than that of bovine-based preparations. We present an unusual case of late-onset VKDB in an extremely preterm infant who received an exclusive human milk diet and HMF and quantify total K1 intake prior to the bleeding.

Vitamin K insufficiency and the prophylaxis strategy in term healthy infants: A multicentre study.

BACKGROUND/AIM: Late vitamin K deficiency bleeding (VKDB) during early infancy is a serious problem worldwide. Vitamin K (VK) deficiency commonly occurs in newborns who are exclusively breastfed. Protein Induced by VK Absence (PIVKA-II) has been identified as an early indicator of subclinical VK deficiency in neonates, surpassing prothrombin time. To assess PIVKA-II levels at 48 h, 1 and 3 months of age in full-term newborns who were exclusively breastfed and received varying VKDB prophylaxis regimens. METHODS: A prospective observational study was conducted in four hospitals, enrolling 105 newborns. PIVKA-II levels were measured using a sandwich-type enzyme-linked immunosorbent assay. RESULTS: At 48 h of age, there was no significant difference in PIVKA-II concentrations between newborns who received intramuscular administration of 1 mg of phylloquinone (VK1) and those who received oral administration of 2 mg of VK1 at birth. At 1 and 3 months of life, infants who received any supplementation regimen between 2 and 14 weeks exhibited significantly lower PIVKA-II concentrations compared to infants who received only 1 mg of intramuscular VK1 at birth. The prophylaxis involving a dose of 1 mg of intramuscular VK1 at birth followed by oral administration of 150 µg/day of VK1 from the 2nd to the 14th week of life showed the lowest PIVKA-II blood concentrations. CONCLUSIONS: Oral supplementation of VK1 after discharge significantly reduced PIVKA-II concentrations in exclusively breastfed term infants. These findings suggest the importance of oral VK1 supplementation in exclusively breastfed infants during their first 3 months of life to avoid the risk of VK insufficiency.

Take the Shot: A Review of Vitamin K Deficiency.

Vitamin K is essential for the process of coagulation. In its absence, severe and sometimes fatal bleeding events can occur, especially in newborns. Vitamin K prophylaxis at birth has been shown to prevent morbidity and mortality associated with vitamin K deficiency bleeding (VKDB) and is recommended by multiple organizations including the American Academy of Pediatrics and the World Health Organization. Pediatricians should feel comfortable explaining the risks and benefits of vitamin K prophylaxis to families and should be equipped to recognize signs of VKDB, especially given increasing rates of parental refusal. This article aims to improve understanding of VKDB, including prevention, early recognition, and treatment. [Pediatr Ann. 2023;52(2):e42-e45.].

Attention to diseases that re-emerge due to refusal of vitamin K: an infant case.

Intramuscular vitamin K injection is recommended for all newborns to prevent bleeding. However, the number of parents who reject vitamin K is at an increase. We present a 1-month girl who presented with haemorrhagic shock due to extraordinary intra-thoracic bleeding. The patient was treated with thoracentesis and blood transfusion. Parents were informed the about the benefits of Vitamin K and they were convinced to continue a routine immunization programme.

Neonatologists and vitamin K hesitancy.

Hemorrhagic disease of the newborn, more aptly termed "Vitamin K Deficiency Bleeding (VKDB)," has long been recognized as a cause of significant morbidity and mortality in early infancy. A single intramuscular dose of vitamin K administered at birth has virtually eliminated VKDB, and this prophylactic regimen has been recommended by the American Academy of Pediatrics since 1961. Although most newborns in the United States receive vitamin K at birth, a growing number of parents are hesitant about this intervention, citing concerns about harm from the injection, preservatives contained in the medication, and clashes with personal belief systems. Ultimately, there is distrust in the medical establishment as many of these parents also opt out of newborn ophthalmic prophylaxis and importantly, childhood vaccinations, establishing a dangerous public health precedent that will lead to resurgence of vaccine-preventable diseases. Various shared decision making models and educational efforts can assist newborn health care professionals in addressing these parental concerns so that VKDB can be effectively prevented in all newborns.

Hemorrhagic Disease of the Newborn: A Case Series Illustrating Preventable Harm.

Newborns are susceptible to postnatal Vitamin K deficiencies from limited placental transfer, gastrointestinal absorption, and bioavailability in breast milk and formula preparations. For over 50 years, the American Academy of Pediatrics has recommended prophylactic vitamin K to prevent hemorrhagic disease in newborns. Yet, public skepticism contributes to increasing refusal rates. We present three cases of vitamin K-dependent bleeding following parental refusal of postnatal prophylaxis. Two patients experienced intracranial hemorrhage with resultant neurological devastation and mortality, respectively. The third child presented with symptomatic hematuria. Perinatal providers must partner with families and advocate vitamin K prophylaxis to limit unnecessary morbidity and mortality.

Gastrointestinal bleeding due to idiopathic early onset of vitamin K deficiency bleeding in a girl baby 50 min after birth: a rare case.

BACKGROUND: The incidence of early-onset vitamin K deficiency bleeding (VKDB) in at-risk neonates who did not receive vitamin K supplementation varied from 6 to 12%. This case report aims to show that VKDB can occur abruptly after birth despite vitamin K1 1 mg IM being given immediately after birth. CASE PRESENTATION: A term female baby was born through vaginal delivery of a 28 years old mother, G1P0A0, 39-40 weeks gestation with normal APGAR score, and birth weight was 3445 g, birth length was 52 cm. During pregnancy, the mother did not take any drugs except vitamins. There are no abnormalities on the baby's physical examination. The anus is patent. Immediately after birth, the baby received a vitamin K1 1 mg intramuscularly. Abruptly, 50 min after delivery, there was meconium with lots of fresh blood. Laboratory results showed hemoglobin, 19.6 g/dL; leukocytes, 25,010/uL; platelets, 390,000/uL, with increased PT and aPTT. A peripheral blood smear showed a normal blood morphology. When 7 h old, the baby had much hematochezia. Laboratory results showed decreased hemoglobin to 17.5 g/dL and increased PT, aPTT, and INR. No abnormalities were found on the babygram and abdominal ultrasound. The working diagnosis was gastrointestinal bleeding due to idiopathic early-onset VKDB. The baby received vitamin K1 2 mg IM, Fresh Frozen Plasma, and a Packed Red Cells transfusion. The patient returned home in good clinical condition. CONCLUSION: Vitamin K1 1 mg IM prophylaxis should be given immediately after birth to prevent early-onset VKDB. In addition, pregnant women who receive drugs that interfere with vitamin K metabolism (anti-epileptic drugs, anti-tuberculosis drugs, vitamin K antagonist drugs) should be given prophylactic vitamin K1, 20 mg/d orally, for at least two weeks before the expected time of delivery.

Intracranial Hemorrhage Secondary to Vitamin K Deficiency Bleeding in a Newborn.

We present a case of a 6-week-old infant who presented with seizure-like activity. Workup revealed abnormal coagulation and imaging confirmed intracranial hemorrhage. Parental refusal of vitamin K treatment at birth suggested vitamin K deficiency bleeding (VKDB) in this newborn. Though VKDB is rare in developed countries, rates have been rising which coincides with an increasing trend of parental refusal of vitamin K prophylaxis at birth.

Midwives' Perspectives about Vitamin K Prophylaxis Against Vitamin K Deficiency Bleeding of the Newborn.

BACKGROUND: The American Academy of Pediatrics recommends one intramuscular (IM) vitamin K injection at birth to prevent Vitamin K Deficiency Bleeding of the Newborn (VKDB). Among factors associated with IM vitamin K refusal, investigators have reported an increased frequency of IM vitamin K refusal among parents who select midwife-assisted deliveries. Reasons behind this association are unclear. METHODS: To understand the perspectives of midwives on IM vitamin K prophylaxis and approach to counseling parents using qualitative methodology, we conducted in-depth semi-structured interviews of midwives associated with 3 tertiary academic medical centers and surrounding communities in Connecticut, Iowa and Michigan. We used the grounded theory approach and the constant comparative method until saturation was reached. RESULTS: We interviewed 19 white female midwives from different training pathways. Participants who were Certified Nurse Midwives (CNMs) routinely recommended IM vitamin K prophylaxis and Certified Professional Midwives (CPMs) took a more neutral approach. The following 4 themes emerged: (1) Emphasis on an educational approach to counseling that supports parents' decision-making authority; (2) Low-intervention philosophy in the midwifery model of care attracts certain parents; (3) Need for relationship building between midwives and pediatricians and (4) Opportunities for the future. CONCLUSIONS: Midwives in our study perceived that the midwifery model of care, the focus on physiologic birth and prioritizing parents' decision-making autonomy appears to attract a sub-set of expectant parents with certain belief systems who question interventions such as IM vitamin K prophylaxis. There are opportunities for better collaboration between midwives and pediatricians.

Vitamin K and the Newborn Infant.

Intramuscular administration of vitamin K for prevention of vitamin K deficiency bleeding (VKDB) has been a standard of care since the American Academy of Pediatrics recommended it in 1961. Despite the success of prevention of VKDB with vitamin K administration, the incidence of VKDB appears to be on the rise. This increase in incidence of VKDB is attributable to parental refusal as well as lowered efficacy of alternate methods of administration. The aim of this statement is to discuss the current knowledge of prevention of VKDB with respect to the term and preterm infant and address parental concerns regarding vitamin K administration.

Parto domiciliario y ausencia de profilaxis con vitamina K / Planned home birth and absence of vitamin K prophylaxis

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Belgian Consensus Recommendations to Prevent Vitamin K Deficiency Bleeding in the Term and Preterm Infant.

Neonatal vitamin K prophylaxis is essential to prevent vitamin K deficiency bleeding (VKDB) with a clear benefit compared to placebo. Various routes (intramuscular (IM), oral, intravenous (IV)) and dosing regimens were explored. A literature review was conducted to compare vitamin K regimens on VKDB incidence. Simultaneously, information on practices was collected from Belgian pediatric and neonatal departments. Based on the review and these practices, a consensus was developed and voted on by all co-authors and heads of pediatric departments. Today, practices vary. In line with literature, the advised prophylactic regimen is 1 or 2 mg IM vitamin K once at birth. In the case of parental refusal, healthcare providers should inform parents of the slightly inferior alternative (2 mg oral vitamin K at birth, followed by 1 or 2 mg oral weekly for 3 months when breastfed). We recommend 1 mg IM in preterm <32 weeks, and the same alternative in the case of parental refusal. When IM is perceived impossible in preterm <32 weeks, 0.5 mg IV once is recommended, with a single additional IM 1 mg dose when IV lipids are discontinued. This recommendation is a step towards harmonizing vitamin K prophylaxis in all newborns.

Late vitamin K deficiency bleeding in infants: five-year prospective study

Abstract Objective: To study the presenting clinical and demographic features, risk factors, and outcome of infants with late vitamin K deficiency bleeding. Methods: Over a 5-year study period, the presenting clinical features and outcome of all 47 infants observed aged less than 6 months, who were diagnosed with late-onset primary and secondary VKDB by detailed history, physical examination, and laboratory findings were evaluated. Confirmed primary late VKDB was diagnosed when no cause other than breastfeeding could be found, while in the secondary subtype additional risk factors compromising the vitamin K effect were diagnosed. Results: Secondary late VKDB (83%, 39 patients) was more common than the primary subtype. The mean age of patients was 10.50 ± 5.75 and 9.74 ± 6.04 weeks in primary and secondary VKDB subtypes, respectively, and the age of infants did not have a significant difference (> 0.05). The male to female ratio was 2.13:1. The residency, place and mode of delivery, gestational age, and types of feeding of patients did not have a significant difference between VKDB subtypes. The skin and gastrointestinal tract (GIT) (40.4%) followed by intracranial hemorrhage (ICH) (32%), were common sites of bleeding. Neurological complications were seen in 21% of patients; however, lethality was 23%, and the outcome of patients did not have a significant difference (p > 0.05) between VKDB subtypes. Conclusion: Secondary late VKDB is more common than the primary subtypes, and late VKDB is still a serious disease in developing countries, including Iraq, when vitamin K prophylaxis isn't routinely used at birth.

Vitamin K prophylaxis in newborns.

We looked at existing recommendations and supporting evidence on the effectiveness of vitamin K given after birth in preventing the haemorrhagic disease of the newborn (HDN).We conducted a literature search up to the 10th of December 2019 by using key terms and manual search in selected sources. We summarized the recommendations and the strength of the recommendation when and as reported by the authors. We summarized the main findings of systematic reviews with the certainty of the evidence as reported.All newborns should receive vitamin K prophylaxis, as it has been proven that oral and intramuscular prophylactic vitamin K given after birth are effective for preventing classical HDN. There are no randomized trials looking at the efficacy of vitamin K supplement on late HDN. There are no randomized trials comparing the oral and intramuscular route of administration of prophylactic vitamin K in newborns. From older trials and surveillance data, it seems that there is no significant difference between the intramuscular and the oral regimens for preventing classical and late HDN, provided that the oral regimen is duly completed. Evidence assessing vitamin K prophylaxis in preterm infants is scarce.