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  1. Article ; Online: From the Discovery of ADAMTS13 to Current Understanding of Its Role in Health and Disease.

    Woods, Adriana Inés / Paiva, Juvenal / Dos Santos, Celia / Alberto, María Fabiana / Sánchez-Luceros, Analía

    Seminars in thrombosis and hemostasis

    2022  Volume 49, Issue 3, Page(s) 284–294

    Abstract: ADAMTS13 (a disintegrin-like metalloprotease domain with thrombospondin type 1 motif, member 13) is a protease of crucial importance in the regulation of the size of von Willebrand factor multimers. Very low ADAMTS13 activity levels result in thrombotic ... ...

    Abstract ADAMTS13 (a disintegrin-like metalloprotease domain with thrombospondin type 1 motif, member 13) is a protease of crucial importance in the regulation of the size of von Willebrand factor multimers. Very low ADAMTS13 activity levels result in thrombotic thrombocytopenic purpura, a rare and life-threatening disease. The mechanisms involved can either be acquired (immune-mediated thrombotic thrombocytopenic purpura [iTTP]) or congenital (cTTP, Upshaw-Schulman syndrome) caused by the autosomal recessive inheritance of disease-causing variants (DCVs) located along the
    MeSH term(s) Humans ; ADAM Proteins/genetics ; ADAMTS13 Protein/genetics ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Purpura, Thrombotic Thrombocytopenic/genetics ; Purpura, Thrombotic Thrombocytopenic/therapy ; von Willebrand Factor
    Chemical Substances ADAM Proteins (EC 3.4.24.-) ; ADAMTS13 Protein (EC 3.4.24.87) ; ADAMTS13 protein, human (EC 3.4.24.87) ; von Willebrand Factor
    Language English
    Publishing date 2022-11-11
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0042-1758059
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Clinical relevance of genetic variants in the von Willebrand factor according to in-silico methods.

    Woods, Adriana Inés / Primrose, Débora Marina / Paiva, Juvenal / Blanco, Alicia Noemí / Alberto, María Fabiana / Sánchez-Luceros, Analía

    American journal of medical genetics. Part A

    2023  Volume 194, Issue 3, Page(s) e63430

    Abstract: Clinical interpretation of genetic variants in the context of the patient's phenotype is a time-consuming and costly process. In-silico analysis using in-silico prediction tools, and molecular modeling have been developed to predict the influence of ... ...

    Abstract Clinical interpretation of genetic variants in the context of the patient's phenotype is a time-consuming and costly process. In-silico analysis using in-silico prediction tools, and molecular modeling have been developed to predict the influence of genetic variants on the quality and/or quantity of the resulting translated protein, and in this way, to alert clinicians of disease likelihood in the absence of previous evidence. Our objectives were to evaluate the success rate of the in-silico analysis in predicting the disease-causing variants as pathogenic and the single-nucleotide variants as neutral, and to establish the reliability of in-silico analysis for determining pathogenicity or neutrality of von Willebrand factor gene-associated genetic variants. Using in-silico analysis, we studied pathogenicity in 31 disease-causing variants, and neutrality in 61 single-nucleotide variants from patients previously diagnosed as type 2 von Willebrand disease. Disease-causing variants and non-synonymous single-nucleotide variants were explored by in-silico tools that analyze the amino acidic sequence. Intronic and synonymous single-nucleotide variants were analyzed by in-silico methods that evaluate the nucleotidic sequence. We found a consistent agreement between predictions achieved by in-silico prediction tools and molecular modeling, both for defining the pathogenicity of disease-causing variants and the neutrality of single-nucleotide variants. Based on our results, the in-silico analysis would help to define the pathogenicity or neutrality in novel genetic variants observed in patients with clinical and laboratory phenotypes suggestive of von Willebrand disease.
    MeSH term(s) Humans ; von Willebrand Factor/genetics ; von Willebrand Factor/metabolism ; Clinical Relevance ; Reproducibility of Results ; von Willebrand Diseases/diagnosis ; von Willebrand Diseases/genetics ; Nucleotides
    Chemical Substances von Willebrand Factor ; Nucleotides
    Language English
    Publishing date 2023-10-23
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2108614-X
    ISSN 1552-4833 ; 0148-7299 ; 1552-4825
    ISSN (online) 1552-4833
    ISSN 0148-7299 ; 1552-4825
    DOI 10.1002/ajmg.a.63430
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Type 2A and 2M von Willebrand Disease: Differences in Phenotypic Parameters According to the Affected Domain by Disease-Causing Variants and Assessment of Pathophysiological Mechanisms.

    Woods, Adriana Inés / Paiva, Juvenal / Primrose, Débora Marina / Blanco, Alicia Noemí / Sánchez-Luceros, Analía

    Seminars in thrombosis and hemostasis

    2021  Volume 47, Issue 7, Page(s) 862–874

    Abstract: Type 2A and 2M von Willebrand disease (VWD) broadly show similar phenotypic parameters, but involve different pathophysiological mechanisms. This report presents the clinical and laboratory profiles of type 2A and type 2M patients genotypically diagnosed ...

    Abstract Type 2A and 2M von Willebrand disease (VWD) broadly show similar phenotypic parameters, but involve different pathophysiological mechanisms. This report presents the clinical and laboratory profiles of type 2A and type 2M patients genotypically diagnosed at one large center. Higher bleeding score values and a higher incidence of major bleeding episodes were observed in type 2A compared with type 2M, potentially reflective of the absence of large and intermediate von Willebrand factor (VWF) multimers in 2A. In type 2A, most of disease-causing variants (DCVs) appeared to be responsible for increased VWF clearance and DCV clustered in the VWF-A1 domain resulted in more severe clinical profiles. In type 2M, DCV in the VWF-A1 domain showed different laboratory patterns, related to either reduced synthesis or shortened VWF survival, and DCV in the VWF-A2 domain showed patterns related mainly to shortened survival. VWF-type 1 collagen binding/Ag (C1B/Ag) showed different patterns according to DCV location: in type 2A VWD, C1B/Ag was much lower when DCVs were located in the VWF-A2 domain. In type 2M with DCV in the VWF-A1domain, C1B/Ag was normal, but with DCV in the VWF-A2 domain, C1B/Ag was low. The higher frequency of major bleeding in VWD 2M patients with DCV in the VWF-A2 domain than that with DCV in the VWF-A1 domain could be a summative effect of abnormal C1B/Ag, on top of the reduced VWF-GPIb binding. In silico modeling suggests that DCV impairing the VWF-A2 domain somehow modulates collagen binding to the VWF-A3 domain. Concomitant normal FVIII:C/Ag and VWFpp/Ag, mainly in type 2M VWD, suggest that other nonidentified pathophysiological mechanisms, neither related to synthesis/retention nor survival of VWF, would be responsible for the presenting phenotype.
    MeSH term(s) Hemorrhage ; Humans ; Phenotype ; von Willebrand Disease, Type 2/genetics ; von Willebrand Diseases/genetics ; von Willebrand Factor/genetics
    Chemical Substances von Willebrand Factor
    Language English
    Publishing date 2021-06-15
    Publishing country United States
    Document type Journal Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0041-1726097
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: From the Discovery of ADAMTS13 to Current Understanding of Its Role in Health and Disease

    Woods, Adriana Inés / Paiva, Juvenal / Dos Santos, Celia / Alberto, María Fabiana / Sánchez-Luceros, Analía

    Seminars in Thrombosis and Hemostasis

    (Celebrating 50 Years of Seminars in Thrombosis and Hemostasis–Part II)

    2022  Volume 49, Issue 03, Page(s) 284–294

    Abstract: ADAMTS13 (a disintegrin-like metalloprotease domain with thrombospondin type 1 motif, member 13) is a protease of crucial importance in the regulation of the size of von Willebrand factor multimers. Very low ADAMTS13 activity levels result in thrombotic ... ...

    Series title Celebrating 50 Years of Seminars in Thrombosis and Hemostasis–Part II
    Abstract ADAMTS13 (a disintegrin-like metalloprotease domain with thrombospondin type 1 motif, member 13) is a protease of crucial importance in the regulation of the size of von Willebrand factor multimers. Very low ADAMTS13 activity levels result in thrombotic thrombocytopenic purpura, a rare and life-threatening disease. The mechanisms involved can either be acquired (immune-mediated thrombotic thrombocytopenic purpura [iTTP]) or congenital (cTTP, Upshaw–Schulman syndrome) caused by the autosomal recessive inheritance of disease-causing variants (DCVs) located along the ADAMTS13 gene, which is located in chromosome 9q34. Apart from its role in TTP, and as a regulator of microthrombosis, ADAMTS13 has begun to be identified as a prognostic and/or diagnostic marker of other diseases, such as those related to inflammatory processes, liver damage, metastasis of malignancies, sepsis, and different disorders related to angiogenesis. Since its first description almost 100 years ago, the improvement of laboratory tests and the description of novel DCVs along the ADAMTS13 gene have contributed to a better and faster diagnosis of patients under critical conditions. The ability of ADAMTS13 to dissolve platelet aggregates in vitro and its antithrombotic properties makes recombinant human ADAMTS13 treatment a potential therapeutic approach targeting not only patients with cTTP but also other medical conditions.
    Keywords ADAMTS13 ; thrombotic thrombocytopenic purpura ; Upshaw–Schulman syndrome ; genotypic studies ; phenotypic studies
    Language English
    Publishing date 2022-11-11
    Publisher Thieme Medical Publishers, Inc.
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0042-1758059
    Database Thieme publisher's database

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  5. Article: Type 2A and 2M von Willebrand Disease: Differences in Phenotypic Parameters According to the Affected Domain by Disease-Causing Variants and Assessment of Pathophysiological Mechanisms

    Woods, Adriana Inés / Paiva, Juvenal / Primrose, Débora Marina / Blanco, Alicia Noemí / Sánchez-Luceros, Analía

    Seminars in Thrombosis and Hemostasis

    (Editorial Compilation X)

    2021  Volume 47, Issue 07, Page(s) 862–874

    Abstract: Type 2A and 2M von Willebrand disease (VWD) broadly show similar phenotypic parameters, but involve different pathophysiological mechanisms. This report presents the clinical and laboratory profiles of type 2A and type 2M patients genotypically diagnosed ...

    Series title Editorial Compilation X
    Abstract Type 2A and 2M von Willebrand disease (VWD) broadly show similar phenotypic parameters, but involve different pathophysiological mechanisms. This report presents the clinical and laboratory profiles of type 2A and type 2M patients genotypically diagnosed at one large center. Higher bleeding score values and a higher incidence of major bleeding episodes were observed in type 2A compared with type 2M, potentially reflective of the absence of large and intermediate von Willebrand factor (VWF) multimers in 2A. In type 2A, most of disease-causing variants (DCVs) appeared to be responsible for increased VWF clearance and DCV clustered in the VWF-A1 domain resulted in more severe clinical profiles. In type 2M, DCV in the VWF-A1 domain showed different laboratory patterns, related to either reduced synthesis or shortened VWF survival, and DCV in the VWF-A2 domain showed patterns related mainly to shortened survival. VWF-type 1 collagen binding/Ag (C1B/Ag) showed different patterns according to DCV location: in type 2A VWD, C1B/Ag was much lower when DCVs were located in the VWF-A2 domain. In type 2M with DCV in the VWF-A1domain, C1B/Ag was normal, but with DCV in the VWF-A2 domain, C1B/Ag was low. The higher frequency of major bleeding in VWD 2M patients with DCV in the VWF-A2 domain than that with DCV in the VWF-A1 domain could be a summative effect of abnormal C1B/Ag, on top of the reduced VWF-GPIb binding. In silico modeling suggests that DCV impairing the VWF-A2 domain somehow modulates collagen binding to the VWF-A3 domain. Concomitant normal FVIII:C/Ag and VWFpp/Ag, mainly in type 2M VWD, suggest that other nonidentified pathophysiological mechanisms, neither related to synthesis/retention nor survival of VWF, would be responsible for the presenting phenotype.
    Keywords disease-causing variants ; phenotype–genotype ; von Willebrand disease ; von Willebrand factor ; collagen binding
    Language English
    Publishing date 2021-06-15
    Publisher Thieme Medical Publishers, Inc.
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0041-1726097
    Database Thieme publisher's database

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  6. Article ; Online: Type 2N von Willebrand disease: Is it always a recessive trait?

    Woods, Adriana Inés / Rossetti, Liliana Carmen / Paiva, Juvenal / De Brasi, Carlos Daniel / Romero, María Lucila / Casinelli, María Marta / Blanco, Alicia Noemí / Sánchez-Luceros, Analía

    Thrombosis research

    2020  Volume 198, Page(s) 49–51

    MeSH term(s) Humans ; Phenotype ; von Willebrand Disease, Type 2 ; von Willebrand Diseases/diagnosis ; von Willebrand Diseases/genetics ; von Willebrand Factor/genetics
    Chemical Substances von Willebrand Factor
    Language English
    Publishing date 2020-11-28
    Publishing country United States
    Document type Letter ; Research Support, Non-U.S. Gov't
    ZDB-ID 121852-9
    ISSN 1879-2472 ; 0049-3848
    ISSN (online) 1879-2472
    ISSN 0049-3848
    DOI 10.1016/j.thromres.2020.11.029
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Phenotypic Parameters in Genotypically Selected Type 2B von Willebrand Disease Patients: A Large, Single-Center Experience Including a New Novel Mutation.

    Woods, Adriana Ines / Kempfer, Ana Catalina / Paiva, Juvenal / Sanchez-Luceros, Analia / Bermejo, Emilse / Chuit, Roberto / Alberto, Maria Fabiana / Blanco, Alicia Noemi / Lazzari, Maria Angela

    Seminars in thrombosis and hemostasis

    2017  Volume 43, Issue 1, Page(s) 92–100

    Abstract: von Willebrand disease type 2B (VWD2B) expresses gain-of-function mutations that enhance binding of an individual's von Willebrand factor (VWF) to its platelet ligand, glycoprotein Ib (GPIb), and which are usually identified by increased ristocetin- ... ...

    Abstract von Willebrand disease type 2B (VWD2B) expresses gain-of-function mutations that enhance binding of an individual's von Willebrand factor (VWF) to its platelet ligand, glycoprotein Ib (GPIb), and which are usually identified by increased ristocetin-induced platelet aggregation (RIPA). We describe here the phenotypic profile of 38 genotypically selected VWD2B-affected family members (AFMs) belonging to 19 unrelated families. Major bleeding was observed in 68.4% of AFMs (previous to their diagnosis and registered by lifetime interviews), with a total of 46 episodes (1.21/patient), and was found to be highly related to the individual bleeding score and presence of thrombocytopenia, but otherwise unrelated to other laboratory parameters. Excessive muco-cutaneous bleeding symptoms were often reported, the most frequent of which comprised menorrhagia, epistaxis, easy bruising, and bleeding after teeth extraction/in oral cavity. Eight unaffected family members were also studied. The prevalence of VWD2B within families was 0.826, and the penetrance of mutations was complete, making it mandatory to study entire family sets to complete diagnostic profiles. Seven heterozygous missense mutations were found, the most common being p.V1316M. In the p.R1308C group, 75% of the AFMs showed absence of RIPA at 0.5 mg/mL, 66.6% of whom had VWF:RCo < 10 IU/dL, and 50% of whom had VWF:CB < 10 IU/dL. In the p.S1310F group, none of the AFMs had VWF:RCo/VWF:Ag < 0.6 (RCo/Ag), but 100% had VWF:CB/VWF:Ag < 0.6/(CB/Ag). Patients with p.P1266L and p.R1304V were characterized as atypical VWD2B. Two de novo mutations were found in four AFMs belonging to two families. We also describe a novel mutation: p.Y1258C. Of our patients, 70.5% had O blood group. In conclusion, a normal RCo/Ag and a negative RIPA at 0.5 mg/mL do not necessarily rule out a diagnosis of VWD2B.
    MeSH term(s) Female ; Genotype ; Humans ; Male ; Mutation ; von Willebrand Disease, Type 2/genetics ; von Willebrand Diseases/genetics ; von Willebrand Factor/genetics
    Chemical Substances von Willebrand Factor
    Language English
    Publishing date 2017-02
    Publishing country United States
    Document type Journal Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0036-1597293
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article: Phenotypic Parameters in Genotypically Selected Type 2B von Willebrand Disease Patients: A Large, Single-Center Experience Including a New Novel Mutation

    Woods, Adriana Ines / Kempfer, Ana Catalina / Paiva, Juvenal / Sanchez-Luceros, Analia / Bermejo, Emilse / Chuit, Roberto / Alberto, Maria Fabiana / Blanco, Alicia Noemi / Lazzari, Maria Angela

    Seminars in Thrombosis and Hemostasis

    (Editorial Compilation III)

    2016  Volume 43, Issue 01, Page(s) 92–100

    Abstract: von Willebrand disease type 2B (VWD2B) expresses gain-of-function mutations that enhance binding of an individual's von Willebrand factor (VWF) to its platelet ligand, glycoprotein Ib (GPIb), and which are usually identified by increased ristocetin- ... ...

    Series title Editorial Compilation III
    Abstract von Willebrand disease type 2B (VWD2B) expresses gain-of-function mutations that enhance binding of an individual's von Willebrand factor (VWF) to its platelet ligand, glycoprotein Ib (GPIb), and which are usually identified by increased ristocetin-induced platelet aggregation (RIPA). We describe here the phenotypic profile of 38 genotypically selected VWD2B-affected family members (AFMs) belonging to 19 unrelated families. Major bleeding was observed in 68.4% of AFMs (previous to their diagnosis and registered by lifetime interviews), with a total of 46 episodes (1.21/patient), and was found to be highly related to the individual bleeding score and presence of thrombocytopenia, but otherwise unrelated to other laboratory parameters. Excessive muco-cutaneous bleeding symptoms were often reported, the most frequent of which comprised menorrhagia, epistaxis, easy bruising, and bleeding after teeth extraction/in oral cavity. Eight unaffected family members were also studied. The prevalence of VWD2B within families was 0.826, and the penetrance of mutations was complete, making it mandatory to study entire family sets to complete diagnostic profiles. Seven heterozygous missense mutations were found, the most common being p.V1316M. In the p.R1308C group, 75% of the AFMs showed absence of RIPA at 0.5 mg/mL, 66.6% of whom had VWF:RCo < 10 IU/dL, and 50% of whom had VWF:CB < 10 IU/dL. In the p.S1310F group, none of the AFMs had VWF:RCo/VWF:Ag < 0.6 (RCo/Ag), but 100% had VWF:CB/VWF:Ag < 0.6/(CB/Ag). Patients with p.P1266L and p.R1304V were characterized as atypical VWD2B. Two de novo mutations were found in four AFMs belonging to two families. We also describe a novel mutation: p.Y1258C. Of our patients, 70.5% had O blood group. In conclusion, a normal RCo/Ag and a negative RIPA at 0.5 mg/mL do not necessarily rule out a diagnosis of VWD2B.
    Keywords bleeding ; mutation ; RIPA ; von Willebrand disease type 2B ; von Willebrand factor
    Language English
    Publishing date 2016-12-15
    Publisher Thieme Medical Publishers
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0036-1597293
    Database Thieme publisher's database

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  9. Article: Major haemorrhage related to surgery in patients with type 1 and possible type 1 von Willebrand disease.

    Woods, Adriana Inés / Blanco, Alicia Noemí / Chuit, Roberto / Meschengieser, Susana Sara / Kempfer, Ana Catalina / Farías, Cristina Elena / Lazzari, María Angela

    Thrombosis and haemostasis

    2008  Volume 100, Issue 5, Page(s) 797–802

    Abstract: Patients with von Willebrand disease (VWD) frequently bleed under a challenge. The aim of our study was to identify predictive markers of perioperative major haemorrhage in type 1 (VWF:RCo = 15-30 IU dl(-1)) and possible type 1 (VWF:RCo = 31-49 IU dl(-1)) ...

    Abstract Patients with von Willebrand disease (VWD) frequently bleed under a challenge. The aim of our study was to identify predictive markers of perioperative major haemorrhage in type 1 (VWF:RCo = 15-30 IU dl(-1)) and possible type 1 (VWF:RCo = 31-49 IU dl(-1)) VWD patients. We recorded perioperative bleeding complications previous to diagnosis and laboratory parameters in 311 patients with 498 surgical procedures. The patients were grouped according to the absence (A) or presence (B) of perioperative major haemorrhages. Eighty-one patients (26%) and 87 surgical procedures (17.5%) presented major haemorrhages associated with surgeries. There was no difference between the percentage of type 1 and possible type 1 VWD patients who had major haemorrhages (32.6% and 24.8% respectively; p = ns). No difference in the prevalence of O blood group, age, gender, positive family history and laboratory test results (FVIII and VWF) was observed, independent of the haemorrhagic tendency. Bleeding after tooth extraction was the most frequent clinical feature observed in patients with perioperative major haemorrhages. The bleeding score and the number of bleeding sites (> or = 3) were not predictors of major haemorrhage associated with surgery. Caesarean section and adenotonsillectomy showed the highest frequency of major haemorrhages (24.6% and 22.3%, respectively). In conclusion, type 1 and possible type 1 VWD patients showed similar incidence of perioperative major haemorrhages. Laboratory tests and positive family history did not prove to be effective at predicting major haemorrhages in patients that had either type 1 or possible type 1 VWD. The history of bleeding after tooth extraction could define risk factors of major haemorrhage.
    MeSH term(s) Adenoidectomy/adverse effects ; Adolescent ; Adult ; Aged ; Biomarkers/blood ; Blood Loss, Surgical/prevention & control ; Cesarean Section/adverse effects ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Postoperative Hemorrhage/blood ; Postoperative Hemorrhage/etiology ; Postoperative Hemorrhage/prevention & control ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Surgical Procedures, Operative/adverse effects ; Tonsillectomy/adverse effects ; Tooth Extraction/adverse effects ; Young Adult ; von Willebrand Diseases/blood ; von Willebrand Diseases/complications ; von Willebrand Diseases/therapy
    Chemical Substances Biomarkers
    Language English
    Publishing date 2008-11
    Publishing country Germany
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 518294-3
    ISSN 0340-6245
    ISSN 0340-6245
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article: Major haemorrhage related to surgery in patients with type 1 and possible type 1 von Willebrand disease

    Woods, Adriana Inés / Blanco, Alicia Noemí / Chuit, Roberto / Meschengieser, Susana Sara / Kempfer, Ana Catalina / Farías, Cristina Elena / Lazzari, María Angela

    Thrombosis and Haemostasis

    2008  Volume 100, Issue 05, Page(s) 797–802

    Abstract: Patients with von Willebrand disease (VWD) frequently bleed under a challenge. The aim of our study was to identify predictive markers of perioperative major haemorrhage in type 1 (VWF:RCo = 15–30 IU dl -1 ) and possible type 1 (VWF:RCo = 31–49 IU dl -1 ) ...

    Abstract Patients with von Willebrand disease (VWD) frequently bleed under a challenge. The aim of our study was to identify predictive markers of perioperative major haemorrhage in type 1 (VWF:RCo = 15–30 IU dl -1 ) and possible type 1 (VWF:RCo = 31–49 IU dl -1 )VWD patients. We recorded perioperative bleeding complications previous to diagnosis and laboratory parameters in 311 patients with 498 surgical procedures. The patients were grouped according to the absence (A) or presence (B) of perioperative major haemorrhages. Eighty-one patients (26%) and 87 surgical procedures (17.5%) presented major haemorrhages associated with surgeries. There was no difference between the percentage of type 1 and possible type 1 VWD patients who had major haemorrhages (32.6% and 24.8% respectively; p=ns). No difference in the prevalence of O blood group, age, gender, positive family history and laboratory test results (FVIII and VWF) was observed, independent of the haemorrhagic tendency. Bleeding after tooth extraction was the most frequent clinical feature observed in patients with perioperative major haemorrhages. The bleeding score and the number of bleeding sites (≥3) were not predictors of major haemorrhage associated with surgery. Caesarean section and adenotonsillectomy showed the highest frequency of major haemorrhages (24.6% and 22.3%, respectively). In conclusion, type 1 and possible type 1VWD patients showed similar incidence of perioperative major haemorrhages. Laboratory tests and positive family history did not prove to be effective at predicting major haemorrhages in patients that had either type 1 or possible type 1 VWD. The history of bleeding after tooth extraction could define risk factors of major haemorrhage.
    Keywords Type 1 VWD ; surgery ; major haemorrhage ; laboratory and clinical predictive markers
    Language English
    Publishing date 2008-01-01
    Publisher Schattauer GmbH
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 518294-3
    ISSN 2567-689X ; 0340-6245
    ISSN (online) 2567-689X
    ISSN 0340-6245
    DOI 10.1160/TH-07-12-0757
    Database Thieme publisher's database

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