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1.
Dent Clin North Am ; 64(2): 411-434, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32111278

RESUMO

The oral health care provider sees a significant number of patients in his or her practice who suffer from systemic diseases affecting the ability to clot. These medical issues can be acquired or inherited bleeding dyscrasias requiring pharmacologic therapy during the perioperative period. Patients with inherited or acquired bleeding disorders require careful attention with respect to the assessment of bleeding risk. This article develops algorithms to manage acquired and inherited bleeding dyscrasias. These approaches include a discussion of the epidemiology of bleeding disorders in surgical patients, mechanism of hemostasis, and strategies for patient management based on the etiology of bleeding disorder.


Assuntos
Hemofilia A , Doenças de von Willebrand , Feminino , Humanos
2.
Subcell Biochem ; 94: 437-464, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32189311

RESUMO

Von Willebrand factor (VWF) and coagulation factor VIII (FVIII) circulate as a complex in plasma and have a major role in the hemostatic system. VWF has a dual role in hemostasis. It promotes platelet adhesion by anchoring the platelets to the subendothelial matrix of damaged vessels and it protects FVIII from proteolytic degradation. Moreover, VWF is an acute phase protein that has multiple roles in vascular inflammation and is massively secreted from Weibel-Palade bodies upon endothelial cell activation. Activated FVIII on the other hand, together with coagulation factor IX forms the tenase complex, an essential feature of the propagation phase of coagulation on the surface of activated platelets. VWF deficiency, either quantitative or qualitative, results in von Willebrand disease (VWD), the most common bleeding disorder. The deficiency of FVIII is responsible for Hemophilia A, an X-linked bleeding disorder. Here, we provide an overview on the role of the VWF-FVIII interaction in vascular physiology.


Assuntos
Fenômenos Fisiológicos Cardiovasculares , Fator VIII/metabolismo , Fator de von Willebrand/metabolismo , Fator VIII/química , Hemofilia A/metabolismo , Humanos , Doenças de von Willebrand/metabolismo , Fator de von Willebrand/química
3.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 28(1): 235-241, 2020 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-32027283

RESUMO

OBJECTIVE: To establish a novel flow cytometric immunobead array (FCIA) for detecting plasma von Willebrand factor antigen (vWF:Ag), and to analyze the clinical value of FCIA in predicting the prognosis of patients with ischemic stroke (IS). METHODS: Anti-human vWF monoclonal antibody SZ29 IgG was coated on microspheres overnight, the diluted plasma was added after blocking, then incubated with FITC-conjugated sheep-anti-human vWF IgG polyclonal antibody, and finally detected by flow cytometry. The plasma vWF in 21 case of von Willebrand disease (vWD) and 105 controls (CTL) were detected by FCIA and ELISA, so as to carry out methodological assessment. Plasma vWF:Ag of 61 IS patients was detected by FCIA and the data of prognosis followed-up for 2-year were collected. RESULTS: The linear fitting of FCIA was good (R2=0.99) without significant difference between FCIA and ELISA. The Bland-Altman bias was 1.12% with 95% limits of agreement that spanned from -45.06% to 47.30%, and the slope of the linear regression was 0.97 (r=0.86, P<0.01). Importantly, the FCIA method was faster than ELISA, and superior to the ELISA in the detection of low levels of vWF:Ag. The levels of vWF:Ag, vWF:GPIbR and vWF:CB in IS patients were significantly higher than those in healthy controls (Z=8.36, 8.71, 6.22, respectively, P<0.01). CONCLUSION: The FCIA for detecting plasma vWF:Ag is not only an effective supplement to ELISA, but also the efficiency is faster and more sensitive, thus improves the diagnosis of type 3 vWD. Elevated levels of vWF: Ag in IS patients indicate the poor recovery of daily activities and prognosis.


Assuntos
Doenças de von Willebrand , Animais , Anticorpos , Citometria de Fluxo , Humanos , Ovinos , Acidente Vascular Cerebral , Fator de von Willebrand
4.
Am J Hematol ; 95(1): 10-17, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31612544

RESUMO

There are limited observational studies among children diagnosed with von Willebrand Disease (VWD). We analyzed differences in bleeding characteristics by sex and type of VWD using the largest reported surveillance database of children with VWD (n = 2712), ages 2 to 12 years old. We found that the mean ages of first bleed and diagnosis were lowest among children with type 3 VWD. It was even lower among boys than girls among all VWD types, with statistically significant difference among children with type 1 or type 3 VWD. Children with type 3 VWD also reported higher proportions of ever having a bleed compared to other VWD types, with statistically higher proportions of boys compared to girls reporting ever having a bleed with type 1 and type 2 VWD. A similar pattern was observed with the use of treatment product, showing higher usage among type 3 VWD, and among boys than girls with type 1 and type 2 VWD. While there were no differences in life quality or in well-being status by sex, children with type 3 VWD showed a greater need for mobility assistance compared to children with type 1 and type 2 VWD. In an adjusted analysis among children with type 1 VWD, boys showed a significant association of ever bleeding [hazard ratio 1.4; P-value <.001)] compared to girls. Understanding phenotypic bleeding characteristics, well-being status, treatment, and higher risk groups for bleeding among pre-adolescent children with VWD will aid physicians in efforts to educate families about bleeding symptoms.


Assuntos
Monitoramento Epidemiológico , Hemorragia/etiologia , Terapêutica/estatística & dados numéricos , Doenças de von Willebrand/patologia , Doenças de von Willebrand/terapia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Fatores Sexuais , Doença de von Willebrand Tipo 1/epidemiologia , Doença de von Willebrand Tipo 1/patologia , Doença de von Willebrand Tipo 1/terapia , Doença de von Willebrand Tipo 2/epidemiologia , Doença de von Willebrand Tipo 2/patologia , Doença de von Willebrand Tipo 2/terapia , Doença de von Willebrand Tipo 3/epidemiologia , Doença de von Willebrand Tipo 3/patologia , Doença de von Willebrand Tipo 3/terapia , Doenças de von Willebrand/classificação , Doenças de von Willebrand/epidemiologia
6.
Clin Appl Thromb Hemost ; 25: 1076029619873976, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31496264

RESUMO

Patients with von Willebrand disease (VWD) often require treatment with supplemental von Willebrand factor (VWF) prior to procedures or to treat bleeding. Commercial VWF concentrates and more recently recombinant human VWF (rVWF) have replaced cryoprecipitate as the mainstay of therapy. In comparison with cryoprecipitate, the VWF content and multimer distribution under current manufacturing processes of these commercial products has not been reported. We measured the factor VIII (FVIII:C), VWF antigen (VWF:Ag), VWF collagen-binding activity (VWF:CB), VWF platelet-binding activity by GPIbM enzyme-linked immunosorbent assay (VWF:GPIbM), and percentage of high-molecular-weight (HMWM) VWF in 3 pools of group A and O cryoprecipitate, 3 vials of VWF concentrate (Humate-P), and 1 lot of rVWF (Vonvendi). We found that both group O and group A cryoprecipitate have significantly higher ratios of VWF:GPIbM activity and FVIII:C activity relative to VWF:Ag and have better preservation of HMWM than Humate-P. Although not compared statistically, rVWF appears to have more HMWM VWF and a higher ratio of VWF:GPIbM to VWF:Ag than Humate-P and cryoprecipitate. The estimated acquisition cost for our hospital for treating one major bleeding episode was more than 4-fold higher with Humate-P and 7- to 10-fold higher with rVWF than with cryoprecipitate.


Assuntos
Doenças de von Willebrand/tratamento farmacológico , Fator de von Willebrand/análise , Ensaio de Imunoadsorção Enzimática/métodos , Fator VIII/análise , Fibrinogênio/análise , Hemorragia/tratamento farmacológico , Hemorragia/economia , Humanos , Proteínas Recombinantes/análise , Doenças de von Willebrand/economia
7.
Comput Methods Programs Biomed ; 179: 104989, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31443861

RESUMO

BACKGROUND AND OBJECTIVE: Von Willebrand disease (VWD) is one of the most severe inherited bleeding disorder in humans, and it is associated with a qualitative and/or quantitative deficiency of von Willebrand factor, a multimeric glycoprotein fundamental in the coagulation process. At present, the diagnosis of VWD is extremely challenging and mostly based on clinical experience. Kinetic models have been recently proposed and applied to help in the diagnosis and characterization of VWD, but the complexity of these models is such that they requires long and stressful clinical tests, such as the desmopressin response test (DDAVP), to achieve a satisfactory estimation of the individual haemostatic parameters. The goal of this paper is to design a minimal set of clinical tests for the identification of akinetic model to decrease the required time and effort for the characterization and diagnosis of VWD. METHODS: A model proposed in the literature is used as a building block to develop a new model, where response surface methodologies have been applied to determine a set of explicit correlations linkingkinetic model parameters to basal clinical trials data. Model-based design of experiments techniques are then used to devise optimally informative tests for model validation which are shorter and easier to implement. RESULTS: Results show an excellent agreement between the original model for VWD and the new proposed model on representing healthy and VWD subjects. The application of experimental design techniques for model validation shows the possibility to drastically reduce the duration of DDAVP tests from 24 h-3 h by exploiting complementary information from basal clinical tests. CONCLUSIONS: Basal clinical tests can be used alongside a time-reduced DDAVP test to validate pharmacokinetic models for a quantitative characterisation of subjects affected by VWD and for a quicker and easier diagnosis of the disease.


Assuntos
Doenças de von Willebrand/diagnóstico , Estudos de Casos e Controles , Protocolos Clínicos , Ensaios Clínicos como Assunto/métodos , Ensaios Clínicos como Assunto/estatística & dados numéricos , Simulação por Computador , Desamino Arginina Vasopressina/administração & dosagem , Diagnóstico por Computador/métodos , Diagnóstico por Computador/estatística & dados numéricos , Humanos , Funções Verossimilhança , Modelos Biológicos , Fatores de Tempo , Doenças de von Willebrand/sangue , Fator de von Willebrand/análise
8.
Blood Coagul Fibrinolysis ; 30(7): 361-363, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31464689

RESUMO

: von Willebrand disease (VWD) type 2B is a rare bleeding disorder, presenting with moderate-to-severe lifelong bleeding. We present the case of a 61-year-old woman who was misdiagnosed as immune thrombocytopenic purpura during her three pregnancies resulting in a delayed diagnosis of VWD type 2B. This genetically confirmed diagnosis resulted in testing and the establishment of the diagnosis in her otherwise asymptomatic adult son as well. VWD may not be diagnosed till beyond mid adulthood in women with thrombocytopenia previously attributed to pregnancy and should be considered as a differential in female patients developing thrombocytopenia less than 100 × 10/µl with an increased bleeding assessment tool score.


Assuntos
Diagnóstico Tardio , Doença de von Willebrand Tipo 2/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Gravidez , Trombocitopenia/diagnóstico , Doenças de von Willebrand/diagnóstico
10.
Int J Lab Hematol ; 41(5): 664-670, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31271527

RESUMO

INTRODUCTION: Chromogenic substrate assay (CSA) reagents Revohem™ FVIII and Revohem™ FIX are now available as in vitro diagnostic reagents for autoanalysers in Japan. In this study, we evaluated the performance of these reagents in the CS-5100 automated coagulation analyser. METHODS: We assessed within-run and between-day imprecision, on-board stability and frozen-storage stability of Revohem FVIII and FIX. Sensitivity to lupus anticoagulant (LA) was examined using LA-positive patient plasma. Correlations were analysed using plasma samples from normal individuals and patients with haemophilia A (HA) or B (HB) or von Willebrand disease (VWD). RESULTS: Imprecision was <2% for Revohem FVIII and <6.5% for Revohem FIX. On-board storage of Revohem FVIII resulted in a <10% decrease in FVIII levels from baseline at 24 hours, whereas Revohem FIX showed a >10% decrease at 8 hours. Revohem FVIII showed good stability while frozen for 22 days. Although Revohem FIX showed degradation due to freeze-thawing, a new calibration improved stability up to 22 days. Interference from LA was not observed with Revohem FVIII or FIX. The FVIII CSA-CSA correlation was excellent in normal (r = 0.9924), HA (r = 0.9945) and VWD (r = 0.9914). The FVIII CSA-OSA correlation was good in normal (r = 0.8468) and excellent in HA (r = 0.975) and VWD (r = 0.9936). The FIX CSA-OSA correlation was fair in normal (r = 0.4791) and excellent in HB (r = 0.9501). CONCLUSION: Revohem FVIII and FIX both showed excellent performance in the CS-5100 analyser. These reagents could be useful in routine laboratory testing for diagnosing and treating haemophilia.


Assuntos
Automação Laboratorial/instrumentação , Testes de Coagulação Sanguínea/instrumentação , Compostos Cromogênicos/metabolismo , Fator IX/metabolismo , Fator VIII/metabolismo , Automação Laboratorial/métodos , Testes de Coagulação Sanguínea/métodos , Hemofilia A/sangue , Hemofilia A/diagnóstico , Hemofilia A/metabolismo , Hemofilia B/sangue , Hemofilia B/diagnóstico , Hemofilia B/metabolismo , Humanos , Inibidor de Coagulação do Lúpus/sangue , Inibidor de Coagulação do Lúpus/metabolismo , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Doenças de von Willebrand/sangue , Doenças de von Willebrand/diagnóstico , Doenças de von Willebrand/metabolismo
11.
Blood Coagul Fibrinolysis ; 30(5): 239-242, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31157683

RESUMO

: Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic condition that poses both a diagnostic and a therapeutic challenge. Here we report a singular case of AVWS with two associated conditions, small lymphocytic lymphoma (SLL) and Sjögren's syndrome. The patient presented with recurrent and severe digestive bleeding that forced us to raise a curative attempt of AVWS. A first immunosuppressive therapy with immunoglobulins was unsuccessful and it was later decided to treat lymphoproliferative entity with bendamustine and rituximab effectively achieving SLL and AVWS remission. On the basis of our case and through literature review, we discuss potential strategies to achieve AVWS remission when it appears in the setting of several causative associated conditions.


Assuntos
Leucemia Linfocítica Crônica de Células B/complicações , Síndrome de Sjogren/complicações , Doenças de von Willebrand/complicações , Idoso , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Cloridrato de Bendamustina/uso terapêutico , Feminino , Hemorragia/etiologia , Hemorragia/terapia , Humanos , Imunoglobulinas/uso terapêutico , Leucemia Linfocítica Crônica de Células B/terapia , Rituximab/uso terapêutico , Síndrome de Sjogren/terapia , Doenças de von Willebrand/terapia
13.
Ann Lab Med ; 39(6): 545-551, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31240882

RESUMO

BACKGROUND: von Willebrand disease (VWD), characterized by quantitative or qualitative defects of von Willebrand factor (VWF), is the most common inheritable bleeding disorder. Data regarding the genetic background of VWD in Korean patients is limited. To our knowledge, this is the first comprehensive molecular genetic investigation of Korean patients with VWD. METHODS: Twenty-two unrelated patients with VWD were recruited from August 2014 to December 2017 (age range 28 months-64 years; male:female ratio 1.2:1). Fifteen patients had type 1, six had type 2, and one had type 3 VWD. Blood samples were collected for coagulation analyses and molecular genetic analyses from each patient. Direct sequencing of all exons, flanking intronic sequences, and the promoter of VWF was performed. In patients without sequence variants, multiplex ligation-dependent probe amplification (MLPA) was performed to detect dosage variants. We adapted the American College of Medical Genetics and Genomics guidelines for variant interpretation and considered variants of uncertain significance, likely pathogenic variants, and pathogenic variants as putative disease-causing variants. RESULTS: VWF variants were identified in 15 patients (68%): 14 patients with a single heterozygous variant and one patient with two heterozygous variants. The variants consisted of 13 missense variants, one small insertion, and one splicing variant. Four variants were novel: p.S764Efs*16, p.C889R, p.C1130Y, and p.W2193C. MLPA analysis in seven patients without reportable variants revealed no dosage variants. CONCLUSIONS: This study revealed the spectrum of VWF variants, including novel ones, and limited diagnostic utility of MLPA analyses in Korean patients with VWD.


Assuntos
Grupo com Ancestrais do Continente Asiático/genética , Técnicas de Amplificação de Ácido Nucleico/métodos , Doenças de von Willebrand/diagnóstico , Fator de von Willebrand/genética , Adolescente , Adulto , Criança , Pré-Escolar , DNA/química , DNA/genética , DNA/metabolismo , Feminino , Heterozigoto , Humanos , Masculino , Pessoa de Meia-Idade , Mutagênese Insercional , Mutação de Sentido Incorreto , Polimorfismo de Nucleotídeo Único , Processamento de RNA , República da Coreia , Adulto Jovem , Doenças de von Willebrand/genética , Fator de von Willebrand/metabolismo
14.
Recurso na Internet em Inglês | LIS - Localizador de Informação em Saúde, LIS-bvsms | ID: lis-LISBR1.1-46558

RESUMO

The World Federation of Hemophilia improves and sustains care for people with inherited bleeding disorders around the world.


Assuntos
Hemofilia A , Transtornos da Coagulação Sanguínea , Hemofilia B , Deficiência do Fator XI , Doenças de von Willebrand
15.
Zhonghua Xue Ye Xue Za Zhi ; 40(4): 312-316, 2019 Apr 14.
Artigo em Chinês | MEDLINE | ID: mdl-31104443

RESUMO

Objective: To assess the significance of DDAVP use in the diagnosis and treatment of VWD. Methods: An analysis of 15 VWD cases who referred to Hematology Division of First affiliated Hospital of Soochow University and treated with DDAVP from March 2016 to August 2018 was conducted. Efficacy and treatment response of DDAVP were monitored by observations of changes in factor Ⅷ procoagulant (FⅧ∶C) and von Willebrand Factor (VWF) related indicators before and 2 h after DDAVP injection. Results: Of 15 cases with VWD, 7 males and 8 females with a median age of 23 (6-46) years, 7 of 9 type I VWD patients achieved complete response (CR) , 1 type 2A VWD case CR, 5 type 3 VWD ones no response (NR) . The VWF multimer analysis in 5 patients combined with other plasma VWF values were in accordance with the known diagnosis. Conclusions: DDAVP was effective in most type 1 patients, and ineffective in some type 2 and almost all type 3 cases. It was helpful for diagnosis and subsequent treatment planning.


Assuntos
Hemostáticos , Adolescente , Adulto , Criança , Desamino Arginina Vasopressina , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Doenças de von Willebrand , Fator de von Willebrand
17.
Acta Haematol ; 142(2): 71-78, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31085919

RESUMO

INTRODUCTION: Women with von Willebrand disease (VWD) are at a higher risk of bleeding, which might affect the health of mother and child during pregnancy and the intra- and postpartum periods. This retrospective cohort study evaluates changes in the coagulation parameters von Willebrand factor antigen (VWF:Ag), von Willebrand ristocetin cofactor (VWF:RCo), and Factor VIII activity (FVIII:C) during pregnancy in patients with VWD. In total, 44 pregnancies of 38 patients were assessed (VWD type 1 n = 32, type 2A n = 3, type 2B n = 1, type 2 subtype unidentified n = 2). The patients' median age at childbirth was 32 years (range 22-40). RESULTS: A significant increase in coagulation parameters was found in patients with VWD type 1 (VWF:Ag, VWF:RCo, and FVIII:C p = 0.000). In the third trimester, VWF:Ag and FVIII:C normalized in all patients with VWD type 1; in 3 patients VWF:RCo remained below the normal range. Patients with VWD type 2 showed a significant increase of VWF:Ag (p = 0.003) and FVIII:C (p = 0.011), and a non-significant increase of VWF:RCo (p = 0.097). In 4 of 9 pregnancies of patients with VWD type 2, all surveyed coagulation parameters normalized until the third trimester. CONCLUSION: For the majority of the observed patients, the von Willebrand parameters increased during pregnancy.


Assuntos
Coagulação Sanguínea , Fator VIII/metabolismo , Complicações Hematológicas na Gravidez/sangue , Terceiro Trimestre da Gravidez/sangue , Doenças de von Willebrand/sangue , Fator de von Willebrand/metabolismo , Adulto , Feminino , Humanos , Gravidez , Estudos Retrospectivos
18.
Haemophilia ; 25(3): 463-467, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31144420

RESUMO

BACKGROUND: Haemophilia and von Willebrand disease (VWD) are common inherited bleeding disorders. Although patients with haemophilia or VWD have a high risk of hepatitis virus infection and hepatocellular carcinoma (HCC), little is known about the safety of liver resection in these patients. METHODS: From 2006 to 2016, there were seven hepatectomies with haemophilia A and three hepatectomies with VWD for malignant liver tumours at tertiary care hospitals in Japan and Switzerland. To evaluate the safety of hepatectomy in the blood coagulation disorder group (BD group), short-term outcomes in these patients were compared with 20 hepatectomies (non-BD group) for HCC, matched to a 2:1, operative procedure, period and background liver. RESULTS: Ten liver resections were performed in patients with haemophilia or VWD with administration of recombinant FVIII or VWF concentrate. Comparison of the BD vs non-BD group revealed no significant differences in the operative time (327 vs 407 minutes, P = 0.359), estimated blood loss (730 vs 820 mL, P = 0.748), red blood cell transfusion rate (10.0% vs 5.0%, P = 0.605), major complication rate (Clavien-Dindo grade III or IV) (10.0% vs 5.0%, P = 0.605) or mortality rate (0% vs 0%, P > 0.999). Additionally, the length of the postoperative hospital stay was similar between the two groups (13 vs 14 days, P = 0.296). CONCLUSION: Liver resection for treatment of HCC in patients with haemophilia or VWD can be safely performed through an appropriate perioperative administration protocol of coagulation factors.


Assuntos
Fatores de Coagulação Sanguínea/metabolismo , Hemofilia A/metabolismo , Hemofilia A/cirurgia , Hepatectomia/efeitos adversos , Segurança , Doenças de von Willebrand/metabolismo , Doenças de von Willebrand/cirurgia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
19.
Tidsskr Nor Laegeforen ; 139(8)2019 May 07.
Artigo em Norueguês | MEDLINE | ID: mdl-31062561

RESUMO

BACKGROUND: As a result of good medical treatment, the life expectancy of patients with severe haemophilia is now approaching normal. This implies a growing need for treatment of lifestyle- and age-related disease. This article describes surgery in patients with severe haemophilia in the period 1997-2014. MATERIAL AND METHOD: Data were retrieved from the registry linked to the national treatment service for surgery, intervention and advanced diagnostics for haemophilia. The patients were categorised according to type of haemophilia and type of intervention in orthopaedic and non-orthopaedic surgical procedures. RESULTS: A total of 825 surgical procedures were undertaken in 286 patients. The number of procedures increased from 21 in 1997 to 66 in 2014. This increase was associated with non-orthopaedic interventions: altogether 4 such procedures were undertaken in 1997 and 45 in 2014. The number of orthopaedic interventions varied somewhat from year to year, but no clear trend was evident. INTERPRETATION: With increased life expectancy, we are seeing a growing need for treatment of diseases that are not causally related to haemophilia. Doctors with little experience of patients with severe haemophilia will need to deal with lifestyle- and age-related disease in this patient group.


Assuntos
Hemofilia A/cirurgia , Hemofilia B/cirurgia , Procedimentos Cirúrgicos Operatórios/estatística & dados numéricos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Envelhecimento , Criança , Deficiência do Fator VII/epidemiologia , Deficiência do Fator VII/cirurgia , Hemofilia A/epidemiologia , Hemofilia B/epidemiologia , Humanos , Pessoa de Meia-Idade , Procedimentos Ortopédicos/estatística & dados numéricos , Sistema de Registros , Adulto Jovem , Doenças de von Willebrand/epidemiologia , Doenças de von Willebrand/cirurgia
20.
Urology ; 130: 211-212, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31051167

RESUMO

We describe a rare case of a large renal arteriovenous malformation in a patient with von Willebrand disease. Initial attempts at technically challenging embolization failed requiring a nephrectomy. Extra-intestinal vascular malformations are rare in von Willebrand disease. However, there is more recent evidence of von Willebrand factor's regulatory role in angiogenesis and vascular malformations.


Assuntos
Anormalidades Múltiplas/cirurgia , Malformações Arteriovenosas/complicações , Artéria Renal/anormalidades , Veias Renais/anormalidades , Doenças de von Willebrand/complicações , Malformações Arteriovenosas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Renal/cirurgia , Veias Renais/cirurgia , Doenças de von Willebrand/cirurgia
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