Hemorragia digestiva en el contexto de una deficiencia aislada del factor VII: reporte de caso / Gastrointestinal bleeding in the context of isolated factor VII deficiency: case report

Introducción: Se conoce poco de la forma adquirida del déficit del factor VII y son pocos los casos reportados en la literatura. Objetivo: Presentar el caso de una paciente con déficit aislado del factor VII, en el contexto de una hemorragia digestiva baja. Presentación del caso: Mujer peruana de 82 años que acude a emergencia por presentar hemorragia digestiva baja. Durante los exámenes de rutina se le detecta tiempo de protrombina prolongado y déficit aislado de factor VII. No se evidencia mecanismos patológicos de deficiencia de vitamina K o malabsorción, terapia anticoagulante con antagonistas de la vitamina K, hiperfibrinolisis o coagulación intravascular diseminada. Respondió al tratamiento con plasma fresco congelado y los resultados normales de la prueba hematológica realizada a la hermana, alejan la posible etiología hereditaria. Conclusión: Este caso peruano de déficit aislado del factor VII, en el contexto de una hemorragia digestiva baja, permite sumar información a la escasa evidencia Latinoamericana(AU)

Introduction: Little is known about the acquired form of factor VII deficiency and few cases are reported in the literature. Objective: To present a case of a patient with an isolated deficit of factor VII, in the context of low gastrointestinal bleeding. Presentation of the case: 82-year-old Peruvian woman who comes to the emergency room for presenting with lower GI bleeding. Prolonged prothrombin time and isolated factor VII deficiency are detected during routine examinations. There were no evidence of pathological mechanisms of vitamin K deficiency or malabsorption, anticoagulant therapy with vitamin K antagonists, hyperfibrinolysis, or disseminated intravascular coagulation. She responded to the treatment with fresh frozen plasma and the normal results of the hematological test carried out on the sister remove the possible hereditary etiology. Conclusion: This Peruvian case of isolated factor VII deficit, in the context of low gastrointestinal bleeding, allows adding information to the limited Latin American evidence(AU)

Coagulopatía asociada a síndrome de malabsorción por enfermedad celíaca / Coagulopathy associated with celiac disease malabsorption syndrome

This article describes a 19-y-old patient with abdominal pain and signs of malnutrition. She had been treated previously with an antibiotic for chronic diarrhea. Laboratory analyses showed the presence mild hypoalbuminemia, and considerably prolonged prothrombin time. Tests revealed that hemostasis improved after the patient received fresh frozen plasma and vitamin k. A coagulation profile showed a decrease in clotting factors V, VII, IX, and fibrinogen. Positive serology (immunoglobulin A antitissue transglutaminase and immunoglobulin A antiendomysial antibodies) and small bowel mucosal histopathology confirmed the presence of celiac disease (CD). The girl recovered completely after she was put on a gluten-free diet. Vitamin K­deficiency is a rare complication that occurs in celiac disease manifestations. In addition to antibiotic therapy, treatment with other drugs that influence vitamin K resorption and metabolism may increase the risk of bleeding in patients with CD with hypoprothrombinemia.

Prevalencia de deficiencia de vitamina K y factores asociados en pacientes con fibrosis quística sin aporte suplementario / Prevalence of vitamin K deficiency and associated factors in non-supplemented cystic fibrosis patients

Introducción. La deficiencia de vitamina K es prevalente en pacientes con fibrosis quística (FQ) aun con aporte suplementario. Se desconocen factores de riesgo fiables para determinar su ocurrencia. Nuestro objetivo fue evaluar la prevalencia de deficiencia de vitamina K y factores asociados en los pacientes con FQ que no recibían aporte suplementario. Métodos. Se determinaron protrombina inducida por ausencia de vitamina K (PIVKA-II) y osteocalcina infracarboxilada (OCic). Se evaluó el estado clínico y su relación con la deficiencia de vitamina K. El análisis estadístico incluyó prueba de Mann-Whitney, ANOVA o Kruskal-Wallis, prueba χ² o prueba de Fisher-Freeman-Halton y regresión logística múltiple lineal y escalonada hacia adelante. Resultados. Se incluyeron 79 pacientes con FQ de entre 0,4-25,3 años. Se observaron valores anómalos de PIVKA-II y OCic en 56 (70,9%) y 45 (57,0%) pacientes. Los pacientes con PIVKA-II elevada eran significativamente mayores (p = 0,0184) y tenían puntajes Z de peso corporal (p= 0,0297) inferiores a los pacientes que tenían concentraciones normales. No se hallaron diferencias entre los pacientes con OCic normal o patológica. Se notificaron valores anómalos de PIVKA-II y OCic más frecuentemente en pacientes con dos mutaciones graves en el gen CFTR y con un estado nutricional malo/deficiente. Los análisis de regresión múltiple lineal y de regresión múltiple escalonada hacia adelante no revelaron factores predictivos sólidos para determinar la deficiencia de vitamina K. Conclusión. La deficiencia de vitamina K es altamente prevalente durante la evolución natural de la fibrosis quística. No se hallaron determinantes clínicos fiables para precisar su ocurrencia.

Introduction. Vitamin K deficiency is highly prevalent in cystic fibrosis (CF) patients despite supplementation. Moreover, no reliable risk factors for its occurrence are known. The aim was to assess the prevalence of vitamin K deficiency and associated factors in non-supplemented CF patients. Methods. Prothrombin concentration induced by vitamin K absence (PIVKA-II) and the undercarboxylated osteocalcin percentage (u-OC) were determined. In all patients clinical status was assessed and its relation to vitamin K deficiency determined. The following tests were used for statistical analysis: Mann-Whitney test, ANOVA test or the Kruskal Wallis test, the chi-squared test or the Fisher-Freeman-Halton test, and multiple linear and multiple forward stepwise logistic regression analysis. Results. The study group comprised 79 CF patients aged 0.4-25.3 years. PIVKA-II and u-OC were abnormal in 56 (70.9%) and 45 (57.0%) patients. Patients with elevated PIVKA-II were significantly older (p= 0.0184) and had lower Z-score values for body weight (p= 0.0297) than those with normal concentrations. Patients with normal or pathological u-OC percentage did not differ. Abnormal PIVKA-II and u-OC were reported more frequently in subjects with two severe CFTR mutations and with worse/poor nutritional status. Multiple linear and forward stepwise regression analyses did not reveal strong predictive factors of vitamin K deficiency. Conclusion. Vitamin K deficiency is highly prevalent in the natural course of cystic fibrosis. There are no reliable clinical determinants of its occurrence.

A Case of Acquired Transient Vitamin K Deficiency in a Teenage Girl / 임상소아혈액종양

A 13-year old girl visited emergency medical center presenting with nasal bleeding and gross hematuria. She had no growth retardation, nor history of abnormal bleeding. Her initial blood test results showed normal platelet counts, normal liver enzyme level but prolonged prothrombin time and activated partial thromboplastin time. On admission, she showed massive but intermittent bleeding until the 15th hospital day. Evaluation including coagulation factor assay was done and the results were compatible with vitamin K deficiency. She was treated with vitamin K intramuscular injection 7 times and intermittent transfusion of red blood cells, platelets and fresh frozen plasma. After that, all of her blood test results returned to normal levels including coagulation tests concomitent with resolving symptoms. In that there were no proof of underlying diseases that can cause vitamin K deficiency, she was diagnosed as idiopathic transient vitamin K deficiency.

Hemorrhagic Diathesis as the Presenting Symptom of Neonatal Cholestasis / 대한소아소화기영양학회지

A 4-week-old infant presented with a coagulation disorder resulting from a vitamin K deficiency. The vitamin K deficiency was caused by neonatal cholestasis due to biliary atresia. Jaundice, hepatomegaly and pale stools are the predominant presenting symptoms of biliary atresia, none of which were recognized in our patient before admission. However, the patient presented with bleeding caused by vitamin K deficiency. She was fully breastfed and had received adequate doses of vitamin K at birth and from the age of 1 week. In case of a hemorrhagic diathesis due to neonatal cholestasis, timely identification of treatable underlying disorders, in particular biliary atresia, is important because an early surgical intervention results in a better prognosis. Meticulous history taking and a thorough physical exam can be decisive for an early diagnosis and subsequent intervention.

A Case of Acquired Transient Vitamin K Deficiency in a Teenage Girl / 임상소아혈액종양

A 13-year old girl visited emergency medical center presenting with nasal bleeding and gross hematuria. She had no growth retardation, nor history of abnormal bleeding. Her initial blood test results showed normal platelet counts, normal liver enzyme level but prolonged prothrombin time and activated partial thromboplastin time. On admission, she showed massive but intermittent bleeding until the 15th hospital day. Evaluation including coagulation factor assay was done and the results were compatible with vitamin K deficiency. She was treated with vitamin K intramuscular injection 7 times and intermittent transfusion of red blood cells, platelets and fresh frozen plasma. After that, all of her blood test results returned to normal levels including coagulation tests concomitent with resolving symptoms. In that there were no proof of underlying diseases that can cause vitamin K deficiency, she was diagnosed as idiopathic transient vitamin K deficiency.

Distúrbios da hemostasia: doenças hemorrágicas / Disorders of homeostasis: bleeding disorders

As doenças hemorrágicas abrangem diversas condições clínicas, sendo caracterizadas por hemorragias de gravidade variável em diferentes locais do corpo. Podem ser de causa hereditária ou adquirida, relacionadas a doenças hematológicas ou a outras condições sistêmicas. Para o diagnóstico e tratamento adequados dessas doenças éfundamental a realização de anamnese detalhada e de testes laboratoriais, que podem ser complexos. Neste artigo serão abordadas as principais condições hemorrágicas, classificadas em doenças vasculares/doenças plaquetárias, coagulopatias e doenças hemorrágicas secundárias a doenças sistêmicas e uso de anticoagulantes.

The bleeding disorders include several clinical conditions, being characterized by bleeding of varying severity in different body sites. They can be either inherited or acquired disease - related to hematological diseases or other systemic conditions. For the diagnosis and treatment of these diseases, it is essential to conduct a detailed clinical history and laboratory tests, which may be complex. This article deals with the major hemorrhagic conditions, classified as vascular diseases/platelet diseases, coagulopathy and bleeding disorders secondary to systemic diseases and use of anticoagulants.

Relationship between vitamin K status, bone mineral density, and hs-CRP in young Korean women

Vitamin K intake has been reported as an essential factor for bone formation. The current study was conducted under the hypothesis that insufficient vitamin K intake would affect inflammatory markers and bone mineral density in young adult women. The study was a cross-sectional design that included 75 women in their 20s. Physical assessments, bone mineral density measurements, 24-hr dietary recalls, and biochemical assessments for high sensitivity C-reactive protein (hs-CRP) and percentages of undercarboxylated osteocalcin (%ucOC) were performed. An analysis of vitamin K nutritional status was performed comparing first, second, and third tertiles of intake based on %ucOC in plasma. Vitamin K intake levels in the first, second, and third tertiles were 94.88 +/- 51.48 microg, 73.85 +/- 45.15 microg, and 62.58 +/- 39.92 microg, respectively (P < 0.05). The T-scores of the first and third tertiles were 1.06 and -0.03, respectively, indicating that bone mineral density was significantly lower in the group with lower vitamin K intake (P < 0.05). There was a tendency for different serum hs-CRP concentrations between the first (0.04 +/- 0.02) and third tertiles (0.11 +/- 0.18), however this was not statistically significant. Regression analysis was performed to identify the correlations between vitamin K nutritional status, inflammatory markers, and bone mineral density after adjusting for age and BMI. Serum hs-CRP concentrations were positively correlated with vitamin K deficiency status (P < 0.05). And bone mineral density, which was represented by speed, was negatively correlated with vitamin K deficiency status (P < 0.05). In conclusion, status of vitamin K affects inflammatory status and bone formation. Therefore, sufficient intake of vitamin K is required to secure peak bone mass in young adult women.

Risk factors of acquired prothrombin complex deficiency syndrome: a case-control study.

BACKGROUND: Idiopathic vitamin K deficiency in infancy or acquired prothrombin complex deficiency (APCD) is a serious bleeding disorders in infants. It leads to a high mortality rate and permanent neurological sequele among the survivors. A low vitamin K intake by infants is suggested to have a major role in the pathogenesis. To reduce the incidence of this syndrome, its risk factors have to be identified. OBJECTIVE: To determine the risk factors of the acquired prothrombin complex deficiency syndrome in the early infantile period. MATERIAL AND METHOD: A case-control study was conducted in 20 cases and 60 age- and sex-matched controls who were admitted to the Queen Sirikit National Institute of Child Health in Bangkok during August 1991 to August 1993. Feeding type, maternal history of herb-liquor extracts (herbal medicine) use and no history of vitamin K1 prophylactics at birth were identified to be risk factors of the syndrome. All subjects were fed by breast milk with or without formula milk. None of the subjects fed by formula milk were in the case group (Chi-square for trend = 14.77, p = 0.001). RUSULTS: The rate of a maternal history of herb-liquor extracts use in the case group was significantly higher than that of the control group (p = 0.03). Vitamin K2MK4 level in breast milk obtained from the mothers of the infants with maternal history of herb-liquor extracts use was lower than that obtained from the mothers of the infants without maternal history of herb-liquor extracts use (p = 0.03). No infant with history of intramuscular K1 prophylactics was in the case group. Three out of eight infants with history of oral vitamin K1 regimen were cases. Although vitamin K1 and K2MK4 level in breast milk obtained from the cases' mothers were significantly lower than that obtained from the controls' mothers (p = 0.015 and p = 0.003 respectively), there was an overlapping of vitamin K levels among these two groups. CONCLUSION: This study demonstrated that vitamin K in breast milk has a main role in the pathogenesis of this disease. Herb-liquor extracts may be a cause of the APCD syndrome. Intramuscular vitamin K1 prophylactics should be routinely given to all newborn babies who will receive breast feeding. Effectiveness of oral vitamin K1 prophylactics regimen must be studied urgently.

Clinical presentation of late haemorrhagic disease of newborn

To observe the clinical presentation of late haemorrhagic disease of the newborn [LHDNB], and clinical improvement after the administration of vitamin K1. This is a prospective descriptive study. All the children older than seven days who presented with bleeding were admitted in pediatrics ward of Isra University Hyderabad from April 2006 to April 2007 were included. Data collection was done by means of detailed proforma. Analysis was done on SPSS version 11. Thirty five cases were included. Commonest site of bleeding was subcutaneous followed by oral and injection site. Mean age of late haemorrhagic disease of newborn was 109 days and minimum age of presentation was 28 days. Common clinical presentations were irritability, convulsions, poor reflexes and poor feeding. Mostly recovery was within 24 hours after vit K. Late HDN results in severe hemorrhage especially hemorrhage in the central nervous system. Administration of Vitamin K [1mg, IM] at birth can present these severe complications

Binder Syndrome Infant Born from Mother with Cholelithiasis / 대한소아내분비학회지

Binder syndrome is a maxillonasal dysostosis characterized by midface and nasal hypoplasia. It is sometimes associated with short terminal phalanges of fingers and toes and transient radiological features of chondrodysplasia punctata. It is associated with vitamin K deficiency during pregnancy. We describe here a baby with Binder syndrome who was born from mother with cholelithiasis during pregnancy.

Trastornos de coagulación en el cirrótico / Coagulation disorders in cirrhosis

The liver plays a central role in the clotting process. In this organ are sintetizated the major part of the coagulation factors. Historically, was considered that alteration in liver function causes important bleeding disorders. However, actual evidence is not in agreement with this asseveration. Decreased synthesis of clotting and inhibitor factors, decrease clearance of activated factors, quantitative and qualitative platelet defects, hyperfibrinolysis and intravascular coagulation are some of the defects observed in liver diseases. Thrombotic events, even if rare in cirrhotic patients, occur manly in the portal and mesenteric veins. The aim of the present work is to review the present evidence in coagulation disorders and liver disease.

El hígado participa de manera importante en el proceso de la coagulación. En él se sintetizan la mayor parte de los factores pro- y anticoagulantes. De manera histórica se ha considerado que las alteraciones en la función de este órgano provoca trastornos predisponentes para eventos de sangrado. La evidencia actual pone en tela de juicio esta aseveración. En los casos de hepatopatía se hacen evidentes alteraciones en el número y funcionamiento de las plaquetas, disminución de la síntesis de factores de la coagulación, disfibrinogenemia, alteraciones en la fibrinólisis, deficiencia de vitamina K y cambios similares a los ocurridos en la coagulación intravascular diseminada (CID). El presente trabajo está dirigido a revisar los conocimientos actuales respecto a las alteraciones de la coagulación presentes en los pacientes con hepatopatías.

Late hemorrhagic disease of newborn [HDN]

To assess the clinical profile and risk factors of patients with late hemorrhagic disease of newborn. The study was conducted at the Department of Pediatric, Bahawal Victoria Hospital, Bahawalpur from June 2004 to May 2006 and Sheikh Zayed Hospital Rahim Yar Khan from June 2006 to May 2007. All the infants above the age of 7 days admitted in Pediatric unit with vitamin-K deficiency bleeding were evaluated. Thirty two infants the criteria of late HDN; 24 [75%] were male and 8 [25%] female. Majority of the babies i.e. 24 [75%] were in the age group 4 to 12 weeks. All were born at term and were on exclusive breastfeeding. The place of delivery was home in 18 [56%] while the remaining 14 [44%] were born in hospital/private clinics. Prophylactic vitamin-K was not given to any of the infants at birth or later. Majority of the infants 24 [75%] presented with neurological features, Intracranial hemorrhage is the commonest presentation of late HDN carrying significant morbidity and subsequent mortality. As there is ample evidence that late hemorrhagic disease of the newborn is prevalent because of lack of vitamin-K prophylaxis at birth hence prophylactic vitamin-K administration at birth is a deadly desirable intervention to avoid this potentially preventable life threatening condition

Evaluation of prevention effectiveness of intracranial hemorrhage with vitamin K injection for newborns

Background: Intracranial hemorrhage (lCH) is a common disease with high rate of mortality and neurological sequel. Objectives:to estimate preventive effectiveness of intracranial hemorrhage by vitamin K1 and K3 for newborns just after birth". Subjects and methods: An epidemiological, randomized control study with intervention was conducted. The study included all children under 3 months with and without ICH during period 1/1/2000 -30/6/2004 living in Hatay province. Among them all the newborns during period 1/6/2002 - 30/6/2004 were injected either 1 mg vitamin K1 or K3. Results: Children injected with vitamin K in the intervention period were followed up in three months and evaluated by a united process. Effects of intervention were calculated by preventive value. 62.057/82069 newborns were injected vitamin K1 or Vitamin K3 (25.725 newborns were injected Vitamin K1; 36.332 newborns were injected vitamin K3). Single dose 1M reduced the rate of ICH in infants: The morbidity changed from 3.3\ufffd?to 0.82\ufffd? the prevention value was 96%. There were no complications and side effects of vitamin K injections reported. Conclusions: Injection of vitamin K1 and K3 have similar preventive effectiveness. Vitamin K injection 1M with recommended dose was safe for newborns.\r\n', u' \r\n', u'\r\n', u'