Late Vitamin K Deficient Bleeding in 2 Young Infants--Renaissance of a Preventable Disease.

INTRODUCTION: Late vitamin K deficiency bleeding in young infants is a rare disorder which occurs almost exclusively in breast-fed infants who did not receive proper vitamin K prophylaxis at birth and who might additionally suffer from cholestasis. Its impact on morbidity is high since in 50% of the cases it presents with intracranial hemorrhage with a mortality rate of 20% and life-long neurologic sequelae in 30% of the affected infants. CASE REPORTS: 2 male infants were both admitted to our unit at the age of 5 weeks with subdural hematoma with midline shift due to late vitamin K deficiency bleeding. Both infants did not receive the recommended Vitamin K prophylaxis in Germany. One patient presented with cholestatic jaundice on admission as an additional risk factor. DISCUSSION: Parents who in the apparent best interest for their children refuse the recommended and well established vitamin K prophylaxis at birth leading to the reappearance of late vitamin K deficiency bleeding. These parents also tend to refuse routine immunizations of childhood in later life, which not only have an impact on their own child but might bear a risk for the whole community. CONCLUSION: It is the responsibility of health-care takers to show increased awareness to the growing number of parents refusing vitamin K prophylaxis at birth and educate them properly about the devastating consequences of late vitamin K deficiency bleeding.

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.

Evaluation of risk factors in patients with vitamin K-dependent coagulopathy presumed to be caused by exposure to brodifacoum.

BACKGROUND/AIMS: Recently, many cases of vitamin K-dependent coagulopathy of unknown origin have been reported. Such patients lack any relevant family history and have no systemic disease, raising suspicion of superwarfarin intoxication. We evaluated individual risk factors causing coagulopathy and hemorrhagic symptoms in patients with suspected superwarfarin intoxication. In addition, we determined how to effectively treat vitamin K-dependent coagulopathy caused by suspected superwarfarin intoxication. METHODS: Seven patients with suspected superwarfarin intoxication who lacked any definitive history of rodenticide ingestion were included. Thirty-one patients initially diagnosed with rodenticide poisoning were also included. We performed a retrospective chart review of all subjects and examined clinical data including patient demographics and medical histories. RESULTS: Patients initially diagnosed with rodenticide poisoning were divided into two groups, one of which had a laboratory abnormality (prothrombin time [PT] > 13 seconds) and another group with PTs in the normal range. There was no significant difference between the two groups in any of age, gender, the extent of chronic alcohol consumption, the causative rodenticide, psychiatric problems, ingestion of drugs interacting with warfarin, the extent of intoxication, or the type of ingestion attempt. The albumin level of the former group was significantly lower than that of the latter group (p = 0.014). Furthermore, a significant difference between the two groups was evident in terms of simultaneous ingestion of rodenticide and alcohol (p = 0.023). CONCLUSIONS: Most patients with superwarfarin poisoning did not exhibit any complication. When such complications were evident, they were associated with serum albumin level and coingestion of rodenticide and alcohol.

Early vitamin K deficiency bleeding in a neonate associated with maternal Crohn's disease.

We report herein a case of early vitamin K deficiency bleeding (VKDB) in a neonate associated with maternal Crohn's disease. A female neonate was born at 37 weeks' gestation and weighed 2778 g. She developed broad purpura on her back on day 1. Laboratory data showed anemia, prolonged coagulation time and elevated protein induced by vitamin K absence or antagonist-II. Early VKDB has not been reported in a neonate born from mother with active Crohn's disease. It is essential to give vitamin K selectively as soon as possible after birth to prevent early VKDB in neonates.

Neonatal hematology.

Neonatal hematology is a complex and dynamic process in the pediatric population. Surgeons frequently encounter hematologic issues regarding hemostasis, inflammation, and wound healing. This publication provides a surgeon-directed review of hematopoiesis in the newborn, as well as an overview of the current understanding of their hemostatic profile under normal and pathologic conditions.

Intracranial hemorrhage due to vitamin K deficiency in infants: a clinical study.

The hospital records of 30 infants with a diagnosis of intracranial hemorrhage (ICH) due to late onset of vitamin K deficiency, seen during a 5-year period (2001-2005) were retrospectively evaluated. Signs and symptoms of the patients were convulsions (80%), poor sucking (50%), irritability (40%), vomiting (47%), acute diarrhea (33%), and fever (40%). On physical examination there were bulging or full fontanel in 19 patients (63%), collapsed fontanel in one (3%), diminished or absent neonatal reflexes in 11 (37%), pallor in 14 (47%), and cyanosis in one (3%) patient. Gastrointestinal disorder, skin hemorrhagic findings, and epistaxis each were noted in two (7%) patients. All the infants had prolonged prothrombin time (PT) and seven had prolonged activated partial thromboplastin time (APTT), both of which were corrected by the administration of vitamin K. All the infants had ICH, with the most common being intraparenchymal hemorrhage, followed by multiple type ICH (27%). Neurosurgical intervention was performed in five patients (17%). The overall case fatality rate was 33%. In conclusion, we would like to stress that ICH due to vitamin K deficiency in infants is still an important health problem in Turkey resulting in high mortality rate.

Hemorragia suprarrenal neonatal. Consideraciones diagnósticas y de actitud clínica / Neonatal adrenal hemorrhage. Diagnostic considerations and clinical attitude

Se presenta una serie de 3 casos diagnosticados en nuestro Servicio de hemorragia suprarrenal unilateral. En dos de los casos el diagnóstico es en período neonatal y en el tercer caso se sospecha de forma retrospectiva, por encontrar en una radiografía abdominal calcificaciones suprarrenales. En los dos recién nacidos, la causa de ingreso es ictericia, acompañada en uno de ellos de masa abdominal. Los dos casos son varones sin antecedentes familiares de interés, procedentes de una primera gestación a término y sin incidencias. Parto prolongado en ambos, siendo instrumental en el primero. El período neonatal inmediato, somatometría y exploraciones complementarias al ingreso son normales. En ambos niños se realiza ecografía abdominal en la que se detecta masa suprarrenal con áreas anecoicas, compatible con hemorragia suprarrenal derecha. En ambos casos se mantiene una actitud expectante, con observación clínica estricta y centrada en la aparición de signos de insuficiencia suprarrenal (hipotensión, hipoglucemia, hipercaliemia, hiponatremia, acidosis, convulsiones, coma), hemorragia masiva o signos de indicación quirúrgica. La determinación de hormonas adrenales y el ionograma en suero y orina son normales. Los dos niños permanecen asintomáticos, no precisando otro tratamiento que fototerapia. Se realizan controles ecográficos seriados, que constituyen la base de la confirmación del diagnóstico y del diagnóstico diferencial con otras entidades como el neuroblastoma quístico, quiste cortical o absceso adrenal. Hemos querido añadir un tercer caso de diagnóstico retrospectivo probable. Es un varón de 8 años con calcificaciones suprarrenales derechas de hallazgo casual, sugerentes de antigua hemorragia suprarrenal, que presentó en período neonatal ingreso por ictericia sin sospecha de hemorragia suprarrenal. Los tres casos clínicos nos permiten revisar una entidad relativamente frecuente en el recién nacido, que cursa habitualmente de forma unilateral y asintomática, si bien en ocasiones puede producir un importante compromiso clínico con insuficiencia suprarrenal aguda, hemorragia masiva o muerte neonatal, obligando a un tratamiento precoz y enérgico (AU)

Three cases diagnosed at our Service of unilateral adrenal hemorrhage are presented. The diagnosis was at neonatal period in two cases and the third case should be suspected later by abdominal radiography revealed an adrenal calcifications. The first and second newborn was admitted to hospital by jaundice and the abdominal examination revealed a palpable mass in the first. Both cases are men. Families’ histories were no pertinent; they have been born at term after a normal pregnancy. The childbirth course was complicated by prolonged labor; it was Instrumental childbirth in the first. The neonatal period, anthropometric and laboratory studies were normal. In both children abdominal ultrasound revealed adrenal mass with anechoic areas, it is suggesting adrenal hemorrhage. In both cases was conservative man-agement, and clinical observation in the appearance of signs of adrenal insufficiency (hypotension, hypoglycemia, hyperkaliema, hyponatremia, acidose, convulsions, and comma), massive hemorrhage or signs of surgical indication. The hormonal determination, levels electrolytes blond and urine were normal. Both young they remain asymptomatic, treatment was phototherapy and serial ultrasounds. Follow up ultrasound is needed for diagnosis and differential diagnosis with neuroblastoma, cortical cyst or adrenal abscess. A third case the diagnosis was made later incidentally, suggestive adrenal calcifications of old adrenal hemorrhage. He was 8 years old boy. He was admitted to hospital by jaundice at newborn period. The three clinical cases allow to review an usually common disorder in the newborn period, that attends habitually an asyntomatic form; however sometimes it can produce an important clinical commitment with acute adrenal insufficiency, Massive adrenal hemorrhage or neonative death, forcing a precocious and energetic treatment (AU)

Intracranial hemorrhage due to late hemorrhagic disease in two siblings.

Deficiency of vitamin K predisposes to early, classic or late hemorrhagic disease of the newborn (HDN); late HDN may be associated with serious and life-threatening intracranial hemorrhage. Late HDN is characterized by intracranial bleeding in infants aged 1 week to 6 months due to severe vitamin K deficiency, occurring particularly in exclusively breastfed infants. Late HDN is still an important cause of mortality and morbidity in developing countries where vitamin K prophylaxis is not routinely practiced. In this study, we report on two siblings with intracranial bleeding who were fully breastfed without a routine supplementation of vitamin K. Vitamin K should be given to all newborns as a single, intramuscular dose of 1 mg.

Hemorragia intraventricular en el recién nacido prematuro / Intraventricular hemorrage in preterm neonates

El presente trabajo es producto de una revisión de la patogénesis, incidencia, tratamiento y pronóstico de la hemorragia intraventricular en el recién nacido prematuro. En las publicaciones revisadas, que incluyen series importantes de seguimiento, esta patología se presenta en 25% de los infantes menores de 1500 gramos de peso, en los que tanto la morbilidad como la mortalidad es mayor que los recién nacidos normales, ya que el desarrollo de la hemorragia intraventricular, puede producir alteraciones de flujo sanguíneo cerebral en la inmadura matriz germinal y en la red microvascular. En consecuencia la prevención de la hemorragia intraventricular está directamente relacionada con su patogénesis. Se pregona que el uso de esteroides tipo dexametazona en dosis bajas en el período prenatal y bajas dosis de endometacina en el período postnatal, pueden dar mejor neuroprotección. El tratamiento quirúrgico es excepcional y tiene indicaciones bien precisas, cuando se demuestra certeramente una hidrocefalia progresiva tardía. Por lo tanto en los neonatos prematuros con hemorragia intraventricular el mejor tratamiento actual, es el usar un sistema de seguimiento médico y farmacológico...

Hemorragia intraventricular en el recién nacido prematuro / Intraventricular hemorrage in preterm neonates

El presente trabajo es producto de una revisión de la patogénesis, incidencia, tratamiento y pronóstico de la hemorragia intraventricular en el recién nacido prematuro. En las publicaciones revisadas, que incluyen series importantes de seguimiento, esta patología se presenta en 25% de los infantes menores de 1500 gramos de peso, en los que tanto la morbilidad como la mortalidad es mayor que los recién nacidos normales, ya que el desarrollo de la hemorragia intraventricular, puede producir alteraciones de flujo sanguíneo cerebral en la inmadura matriz germinal y en la red microvascular. En consecuencia la prevención de la hemorragia intraventricular está directamente relacionada con su patogénesis. Se pregona que el uso de esteroides tipo dexametazona en dosis bajas en el período prenatal y bajas dosis de endometacina en el período postnatal, pueden dar mejor neuroprotección. El tratamiento quirúrgico es excepcional y tiene indicaciones bien precisas, cuando se demuestra certeramente una hidrocefalia progresiva tardía. Por lo tanto en los neonatos prematuros con hemorragia intraventricular el mejor tratamiento actual, es el usar un sistema de seguimiento médico y farmacológico...(AU)

Late hemorrhagic disease of newborn.

The clinical features of 14 infants diagnosed with late hemorrhagic disease of newborn (LHDN), of which 10 did not receive vitamin K prophylaxis, are presented. All infants were exclusively breast-fed and 12 did not have any underlying illness to explain the abnormal coagulation profile. The common presenting symptoms were seizures (71%), vomiting (57%), poor feeding (50%) and altered sensorium (36%). Physical examination shared pallor in all infants and a bulging anterior fontanel in 64%. Intracranial bleed was the predominant manifestation (93%), with CT scan showing intracranial bleed in 78%. Eight infants (57%) succumbed to their illness, while 36%had neurological sequelae. Since LHDN leads to significant morbidity and mortality, it should be prevented by providing vitamin K prophylaxis to all newborns.

Intracranial hemorrhage in late hemorrhagic disease of the newborn.

This study was conducted to evaluate the clinical profile and outcome in late hemorrhagic disease of the newborn (HDN) with particular reference to intracranial hemorrhage. Infants (n = 42) presenting with late HDN from January 1998 to December 2001 were studied. Majority (76%) were in the age group of 1-3 months. All were term babies on exclusive breast-feeding and none received vitamin K at birth. 71% patients presented with intracranial hemorrhage, commonest site being intracerebral and multiple ICH. Visible external bleeding was noted in 1/3rd of patients only. Three patients expired. Late HDN is still an important cause of mortality and morbidity in developing countries where vitamin K prophylaxis is not routinely practiced. Isolated intracranial hemorrhage is a common mode of presentation.

[Neonatal hemorrhagic syndromes]. / Syndromes hémorragiques du nouveau-né.

PURPOSE: The purpose of our study was to clarify the frequency of these causes. PATIENTS AND METHODS: Retrospective study using reports of newborns in the neonatal unit in Sousse (Tunisia) from 1991 to 1996, hospitalized for hemorrhagic syndrome defined by bleeding, exteriorized or not, whatever its importance, severity, causes and the associated clinical and biological disorders. Isolated meningeal hemorrhages, limited cutaneo-mucous hemorrhages (conjunctival hemorrhages, bruises), and genital crises of the newborn, were excluded. RESULTS: One hundred and fifty-five hemorrhagic syndromes were observed from 7,128 newborn infants (2.17% of hospitalization). Sex ratio was 1.42. Prematurity rate was 35.7%. The Apgar score was < 7 at one minute in 40.7% of cases. Disorders associated with hemorrhagic syndromes were observed in 118 newborn infants (76.1%) with a predominance of neonatal infections (35.6%). The etiology of neonatal hemorrhages was specified in 93% of cases: newborn hemorrhagic disease (27.7%), disseminated intravascular coagulation (27.1%), isolated thrombocytopenia (9%), digestive lesions (13.5%), and obstetrical trauma (2.6%). CONCLUSION: The frequency of the newborns hemorrhagic syndromes underlines the need for its systematic prevention by vitamin K in the antenatal period to the mother and after birth to the newborn.

Severe HDN due to anti-Ce that required exchange tranfusion.

BACKGROUND: Rh system antibodies are commonly encountered in blood bank practice as well as during pregnancy. Nevertheless, no examples of anti-Ce (RH7) have been reported as a cause of HDN that requires exchange transfusion. CASE REPORT: A 38-year-old woman in her fourth pregnancy was typed as blood group O D+, C-, c+, E+, e-. Anti-C and anti-e were detected in her serum during a routine prenatal work-up. Further evaluation, including flow cytometric analysis, revealed the presence of a strong anti-Ce and a weak anti-e. Her partner was typed as group A D+, C+, c-, E-, e+. A seemingly healthy male infant was delivered at 40 weeks of gestation. The infant's RBCs were typed as group O D-, C+, c+, E+, e+ with a positive DAT (titer 128). Twenty-five hours after birth, the baby had to be transferred to the neonatal intensive care unit because of rapidly rising total serum bilirubin. Despite intensive treatment, including double phototherapy, albumin infusion, and the administration of furosemide and IVIG, the total serum bilirubin level increased during the following day and exchange transfusion with 2 units of type O D-, C-, c+, E+, e- had to be performed; this resulted in a prompt decrease in total serum bilirubin without relapse. CONCLUSION: Anti-Ce caused severe HDN requiring exchange transfusion. This highlights the need for a close follow-up throughout pregnancy if unexpected RBC antibodies are present, to permit the provision of compatible blood in case of a rare antibody.

Vitamin K deficiency bleeding (VKDB) in infancy. ISTH Pediatric/Perinatal Subcommittee. International Society on Thrombosis and Haemostasis.

TERMINOLOGY: Replace the term "Hemorrhagic Disease of the Newborn" (HDN) by "Vitamin K Deficiency Bleeding" (VKDB), as neonatal bleeding is often not due to VK-deficiency and VKDB may occur after the 4-week neonatal period. DEFINITION: VKDB is bleeding due to inadequate activity of VK-dependent coagulation factors (II, VII, IX, X), correctable by VK replacement. DIAGNOSIS: In a bleeding infant a prolonged PT together with a normal fibrinogen level and platelet count is almost diagnostic of VKDB; rapid correction of the PT and/or cessation of bleeding after VK administration are confirmative. WARNING SIGNS: The incidence of intracranial VKDB can be reduced by early recognition of the signs of predisposing conditions (prolonged jaundice, failure to thrive) and by prompt investigation of "warning bleeds". CLASSIFICATION: VKDB can be classified by age of onset into early (<24 h), classical (days 1-7) and late (>1 week <6 months), and by etiology into idiopathic and secondary. In secondary VKDB, in addition to breast feeding, other predisposing factors are apparent, such as poor intake or absorption of VK. VK-PROPHYLAXIS: BENEFITS: Oral and intramuscular VK (one dose of 1 mg) protect equally well against classical VKDB but intramuscular VK is more effective in preventing late VKDB. The efficacy of oral prophylaxis is increased with a triple rather than single dose and by using doses of 2 mg vitamin K rather than 1 mg. Protection from oral doses repeated daily or weekly may be as high as from i.m. VK. VK-PROPHYLAXIS: RISKS: VK is involved in carboxylation of both the coagulation proteins and a variety of other proteins. Because of potential risks associated with extremely high levels of VK and the possibility of injection injury, intramuscular VK has been questioned as the routine prophylaxis of choice. Protection against bleeding should be achievable with lower peak VK levels by using repeated (daily or weekly) small oral doses rather than by using one i.m. dose. BREAST FEEDING MOTHERS TAKING COUMARINS: Breast feeding should not be denied. Supervision by pediatrician is prudent. Weekly oral supplement of 1 mg VK to the infant and occasional monitoring of PT are advisable. CONCLUSION: VKDB as defined is a rare but serious bleeding disorder (high incidence of intracranial bleeding) which can be prevented by either one i.m. or multiple oral VK doses.

Uso da vitamina K no periodo neonatal: parenteral x oral / Vitamina K in the newborn infant: parenteral x oral use

A vitamina K e amplamente utlizada logo apos o nascimento, para a prevençäo da doença hemorragica do recém-nascido. Esta patologia se apresenta sob varias formas: precoce, "clássica" e tardia, sendo esta última a de maior morbidade, por provocar hemorragias intracranianas. A vitamina K pode ser administrada por via parenteral ou oral, cada qual com suas vantagens e desvantagens. Ao se optar pela forma oral e necessario adotar um esquema de multiplas doses, para que a profilaxia da forma tardia seja eficaz