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.

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.

Intracranial haemorrhage associated with Vitamin K deficiency in Egyptian infants.

AIM: Intracranial haemorrhage (ICH) in infancy is a rare life-threatening event. The aim of this review is to highlight the association of ICH and potentially preventable vitamin K deficiency and to describe risk factors, presentation and outcome. METHODS: Original published data on ICH related to vitamin K deficiency during 2008-2012 were extracted from records of participating centres in Egypt (Cairo and Delta region). Full data on 70 infants (0-24 weeks) have been reported in three publications. RESULTS: The first study involved premature infants where ICH was potentially preventable with administration of parenteral vitamin K prophylactic doses to mothers ahead of imminent preterm delivery. The other 2 studies involved term newborns and infants. ICH due to early or classic vitamin K deficiency was reported in nine patients while 44 were due to late vitamin K deficiency. Main risk factors for late onset were exclusive breastfeeding, persistent diarrhoea and/or prolonged antibiotic therapy. CONCLUSION: Vitamin K deficiency bleeding is a relatively frequent problem underlying ICH in infancy. Prophylactic vitamin K to mothers when anticipating preterm labour or a vitamin K boost in exclusively breast-fed infants with prolonged antibiotic usage and, or, persistent diarrhoea might have an impact on prevention and outcome.

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

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 ±â€¯5.75 and 9.74 ±â€¯6.04 weeks in primary and secondary VKDB subtypes, respectively, and the age of infants did not have a significant difference (p > 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.

Spontaneous intracranial haemorrhage in children-intensive care needs and predictors of in-hospital mortality: a 10-year single-centre experience.

PURPOSE: Spontaneous intracranial haemorrhage (SICH) in children, although uncommon, is associated with significant mortality and morbidity. Paediatric data is however limited. MATERIAL AND METHODS: Case records of 105 children with SICH, > 1 month to 12 years, admitted to a tertiary level PICU of a teaching and referral hospital between January 2009 and May 2018 were analysed retrospectively. In-hospital mortality was the primary outcome. Variables between survivors and non-survivors were compared to  determine predictors of mortality. RESULTS: The median (IQR) age of subjects was 6 (2.25, 70) months. Common clinical features were altered sensorium (n = 87, 82.9%), seizures (n = 73, 69.5%), pallor (n = 66, 62.9%) and bulging anterior fontanelle (n = 52, 49.5%). Median (IQR) Glasgow Coma Scale (GCS) at admission was 10 (6, 13) with herniation noted in 27 (25.7%) children. Vitamin K deficiency bleeding (VKDB) and arteriovenous malformation (AVM) were the most common etiology for bleeding among infants and older children respectively. The most common site of bleeding was intracerebral (n = 47, 44.8%) followed by subdural (n = 26; 24.8%). Sixteen (15.2%) children died during hospital stay. On univariate analysis, GCS < 8, Pediatric Risk of Mortality score (PRISM III) > 20, need for intubation, thiopentone coma for refractory intracranial pressure (ICP) and progression to shock and acute kidney injury (AKI) predicted mortality. Seizures were favourably associated with survival. Age, site of bleeding, etiology or type of management for raised ICP (conservative versus decompressive craniectomy) did not affect the outcome. On multivariable analysis, progression to AKI (OR 5.86; 95% CI, 1.53-22.4; p 0.01) predicted poor outcome. Seizures, however, were associated with better odds for survival (OR 0.12; 95% CI, 0.03-0.47; p 0.002). CONCLUSIONS: VKDB and AVM were the common etiologies among infants and older children respectively. Age, site, etiology of bleeding and type of management did not affect outcome. Severe decompensation at presentation, thiopentone for refractory ICP and progression to multiorgan dysfunction determined mortality.

Vitamin K deficiency bleeding in an apparently healthy newborn infant: the compelling need for evidence-based recommendation.

BACKGROUND: Vitamin K is a key point for guarantee normal blood clotting and its level in newborns is commonly low, so a supplementation after delivery is mandatory. Vitamin K prophylaxis in newborns is still an open field of debate: many types of protocol have been proposed in different years and Countries, and sometimes with great variability inside the same Nation (for instance, in Italy a national consensus is not available, so different protocols are employed). Recommendations include different protocols for healthy newborns born at term, but the unpreventable presence of bleeding favouring factors (i.e. blood vessels malformations) or limiting intestinal absorption of liposoluble vitamins (i.e. cholestasis), which could be unrecognized or subclinical in the perinatal period, rises some concerning about the most precautionary route of administration and the timing of further doses after the first one given at birth. The purpose of this report is to underline the most recent evidences available in literature and to arise a debate about this topic, in order to stimulate the production of evidence-based guidelines concerning the prophylaxis with vitamin K1 in newborn infants, considering that many bleeding risk factors are not recognizable at birth. CASE PRESENTATION: We are hereby presenting an emblematic case concerning the risk of intracranial bleeding in an apparently healthy newborn: the described infant did not show any pathological elements in pregnancy history or perinatal life which suggest a possible increased risk of bleeding and the needing of a particular approach in the administration of vitamin K1, but at the end of the first week of life presented an intracranial bleeding with neurological symptoms that required treatment for vitamin K deficiency. CONCLUSIONS: Univocal recommendations about vitamin K prophylaxis are not available and the contrast between oral and intramuscular routes persists unsolved. The difficulty to certainly identify an infant eligible for oral administration of vitamin K1 at birth suggests that the intramuscular route should be preferred. How to prosecute the supplementation in the first months of life is still an open topic of debate.

The Presentation of a Fussy Infant With Bruising: Late-Onset Vitamin K Deficiency Bleeding.

Vitamin K plays an integral role in the clotting cascade. Deficiency, specifically in vulnerable neonates with insufficient stores, can lead to spontaneous bleeding and devastating effects. In this case, we report a young infant with late-onset vitamin K deficiency bleeding who did not receive vitamin K prophylaxis after birth. Initially presenting with bruising and fussiness, the patient was later found to have intracerebral hemorrhage with midline shift and uncal herniation. The infant was not a surgical candidate and died shortly thereafter. Laboratory studies confirmed the diagnosis of late-onset vitamin K deficiency bleeding as the cause of hemorrhage and death.

Prophylactic Dosing of Vitamin K to Prevent Bleeding.

BACKGROUND AND OBJECTIVES: Based on a high incidence of Vitamin K deficiency bleeding (VKDB) in breastfed infants with thus far unrecognized cholestasis, such as biliary atresia (BA), the Dutch regimen to prevent VKDB in breastfed infants was changed from a daily oral dosage of 25 µg to 150 µg vitamin K. Infants continued to receive 1 mg of vitamin K orally at birth. We compared the efficacy of the 150-µg regimen with the 25-µg regimen and with the Danish regimen of a single intramuscular (IM) dose of 2 mg vitamin K at birth. METHODS: Data were retrieved from the national BA registries: 25 µg group (Netherlands, January 1991 to February 2011); 150 µg group (Netherlands, March 2011 to January 2015); and IM 2 mg group (Denmark, July 2000 to November 2014). We compared the incidence of VKDB in the groups. RESULTS: VKDB occurred in 45 of 55 (82%) infants of the 25 µg group, in 9 of 11 (82%) of the 150 µg group, but in only 1 of 25 (4%) of the IM 2 mg group (P < .001). Forty percent of all infants of the 25 µg group had an intracranial hemorrhage as presenting symptom, compared with 27% of the infants of the 150 µg group (P = .43). Intracranial hemorrhage was not observed in the IM 2 mg group (0%; P < .001). CONCLUSIONS: A vitamin K prophylactic regimen of 1 mg of vitamin K orally at birth followed by a daily oral dosage of either 25 or 150 µg fails to prevent VKDB in breastfed infants with still unrecognized BA. The data support 2 mg vitamin K IM at birth as prophylaxis against VKDB.

Prothrombin Complex Concentrate for Intracerebral Hemorrhage Secondary to Vitamin K Deficiency Bleeding in a 6-Week-Old Child.

Four-factor prothrombin complex concentrate is approved for use of life-threatening bleeding secondary to vitamin K antagonism in adults. We describe the use of four-factor prothrombin complex concentrate for hemostasis in a 6-week-old child with life-threatening vitamin K dependent-bleeding who never received vitamin K prophylaxis at birth.

Another Disease Re-emerges Due to Parental Shot Refusal: Case Report of a Fussy Infant with Blood in Stool.

BACKGROUND: Infants may present to the emergency department (ED) with vague complaints worrisome to parents and may initially appear well, despite serious underlying pathology. Whereas sepsis and nonaccidental trauma are high on most providers' diagnostic considerations, we report a case representative of a worrisome trend secondary to the refusal of parenteral vitamin K at birth leading to significant neurologic sequelae. CASE REPORT: A 10-week-old boy presented to the ED with gradual increase in fussiness for 2 weeks and new onset of blood flecks in the stool on the day of presentation. Careful physical examination revealed a pale-appearing infant, leading to diagnostic evaluation demonstrating profound anemia and intracranial bleeding. The patient was diagnosed with late-onset vitamin K-deficient bleeding (VKDB) secondary to parental refusal of the vitamin K shot at birth. Why Should Emergency Physicians be Aware of This? Emergency Medicine providers need to add this serious treatable disease into their diagnostic consideration for fussy infants, infants with unexplained bruising or bleeding, or infants with new-onset seizures. Rapid identification of VKDB can lead to prompt treatment and halt the rapid progression of symptoms. Emergency Medicine providers should ask all parents if their infant received parenteral vitamin K in the newborn period, especially if they are exclusively breastfed or born out of the hospital.

Vitamin K deficiency bleeding leading to the diagnosis of Crohn's disease.

We report the case of a 45 year old man who came to Emergency Room of Polyclinic for sudden onset of localized ecchymosis and widespread hematomas. He was subjected to blood count and first level investigations to assess coagulation. Based on the results, second level investigations were performed. Endoscopy of the gastrointestinal tract with histological examination revealed a diagnosis of Crohn's disease. Vitamin K deficiency causes the formation of vitamin K-dependent clotting factors that cannot perform their pro-coagulant action. Consequently, patients present with hemorrhagic manifestations. Clinical and laboratory features observed in this patient show that the deficiency of vitamin K-dependent coagulation factors may reveal a complex clinical condition such as an inflammatory bowel disease.

Intracranial hemorrhage in infants as a serious, and preventable consequence of late form of vitamin K deficiency: a selfie picture of Turkey, strategies for tomorrow.

PURPOSE: Vitamin K deficiency bleeding is one of the most common causes of acquired hemostatic disorder in early infancy. Although vitamin K is practiced routinely after every birth in Turkey, children with type of vitamin K deficiency bleedings (L-VKDB) can be encountered. We aimed to evaluate the clinical features of the children with L-VKDB reported from Turkey. METHODS: Between 1995 and 2013, 48 studies reporting 534 children with L-VKDB were evaluated in this study. RESULTS: Of the 534 reported children (178 girls, 356 boys), 486 (91 %) were extremely breastfed. The most common bleeding sites were intracranial hemorrhage, gastrointestinal, and umbilical in 414 (77.4 %), 33 (6.2 %), and 33 (6.2 %) children, respectively, and 35 (6.6 %) children had been diagnosed incidentally without any bleeding. The etiology of 399 (74.7 %) children were classified as idiopathic, whereas 135 (25.3 %) were secondary. Intramuscular vitamin K was administered in 248 (46.4 %), not administered in 228 (42.7 %), and the administration of vitamin K were not determined in 58 (10.9 %) children. The outcomes of Turkish cohort showed that 111 (20.8) children died, 257 (48.1 %) cases developed neurologic deficit (mainly epilepsy and psychomotor retardation), and only 166 (31.1 %) patients recovered without squeal. CONCLUSIONS: The compliance of prophylactic measures in Turkey does not seem to be satisfactory. As a further measure of tomorrow, we vigorously emphasize that a national surveillance program may be initiated. An additional intramuscular dose or oral supplementation of vitamin K especially for exclusively breast-fed infants may reduce this catastrophic problem in our country.

Intracranial haemorrhage is linked to late onset vitamin K deficiency in infants aged 2-24 weeks.

AIM: Late vitamin K deficiency bleeding (VKDB) can be serious and manifest as early onset intracranial haemorrhage (ICH). This study aimed to determine the frequency of ICH in relation to vitamin K deficiency and the outcome in infants aged two to 24 weeks. METHOD: A hospital-based study was conducted in two main tertiary hospitals in Cairo, Egypt, from May 2011 to May 2012 with 40 patients with ICH and 50 age-matched controls without ICH. RESULTS: Forty patients with ICH were recruited, 19 were excluded for clinical reasons and the remaining 21 had a significantly low vitamin K level. Exclusive breast feeding (81% of patients), diarrhoea lasting more than 1 week (38.1%) and antibiotic consumption within a week before the development of ICH (57.1%) were more common in the patients than in the control group (p value>0.05, <0.01 and <0.01, respectively). CONCLUSION: A high frequency of ICH due to late VKDB was reported in Egyptian infants aged two to 24 weeks, with poorer outcomes than international studies. A national survey is required to evaluate the timing and protective value of a second booster vitamin K dose to reduce ICH, especially in high-risk patients in this age group.

Spinal haematoma after removal of a thoracic epidural catheter in a patient with coagulopathy resulting from unexpected vitamin K deficiency.

Postoperative epidural analgesia is effective and widely utilised after major abdominal surgery. Spinal haematoma is a rare and devastating complication after epidural analgesia. Well-established risk factors for the development of spinal haematoma after neuraxial procedures have been documented. We present the case of a patient with normal pre-operative coagulation parameters who developed a spinal haematoma more than 24 h after removal of an epidural catheter; she had been without oral intake for only 4 days during which time she developed vitamin K-deficient coagulopathy. Clinicians should consider pre-operative screening of coagulation (International Normalised Ratio), or giving vitamin K supplementation, before performing neuraxial procedures in patients who are at risk of developing vitamin K deficiency or coagulopathy in the peri-operative period.