Hemophilia orthopedic management with emphasis on developing countries.

Abstract:

:The articular problems of patients with hemophilia begin in infancy when minor injuries result in hemarthroses. Early continuous prophylaxis (from cradle to college) is of paramount importance because the immature skeleton is very sensitive to the complications of hemophilia; severe structural deficiencies may develop quickly. If continuous prophylaxis is not feasible owing to the expense of the clotting material or lack of venous access, then a major hemarthrosis must be treated aggressively to prevent the development of synovitis, recurrent joint bleeds, and ultimately end-stage arthritis. Once synovitis has developed, which bleeding will make inevitable, the aim should be to treat it as soon as possible. There are conservative (synoviorthesis) and operative (open or arthroscopic synovectomy) methods. Between the second and fourth decades, many patients with hemophilia develop articular complications. Once this has occurred, treatment modalities such as alignment osteotomies, joint debridement, arthrodesis, and arthroplasties are possible. Should surgery be required, one should consider multiple procedures.

journal_name

Semin Thromb Hemost

authors

Rodriguez-Merchan EC,Heim M

doi

10.1055/s-2005-922223

subject

Has Abstract

pub_date

2005-11-01 00:00:00

pages

518-26

issue

5

eissn

0094-6176

issn

1098-9064

journal_volume

31

pub_type

杂志文章,评审
  • Coronavirus (COVID-19), Coagulation, and Exercise: Interactions That May Influence Health Outcomes.

    abstract::The proinflammatory cytokine storm associated with coronavirus disease 2019 (COVID-19) negatively affects the hematological system, leading to coagulation activation and endothelial dysfunction and thereby increasing the risk of venous and arterial thrombosis. Coagulopathy has been reported as associated with mortalit...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-0040-1715094

    authors: Zadow EK,Wundersitz DWT,Hughes DL,Adams MJ,Kingsley MIC,Blacklock HA,Wu SSX,Benson AC,Dutheil F,Gordon BA

    更新日期:2020-10-01 00:00:00

  • Endothelial heterogeneity associated with regional athero-susceptibility and adaptation to disturbed blood flow in vivo.

    abstract::Endothelial phenotypic heterogeneity plays an important role in the susceptibility of the cardiovascular system to disease. Arteries and heart valves are susceptible to chronic inflammatory disease in regions of blood flow disturbance that implicates hemodynamic forces and transport characteristics as prominent influe...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-0030-1253449

    authors: Davies PF,Civelek M,Fang Y,Guerraty MA,Passerini AG

    更新日期:2010-04-01 00:00:00

  • Structure and function studies of factor XIIIa by x-ray crystallography.

    abstract::The three-dimensional structures of several forms of the factor XIII A subunit have been determined using single crystal x-ray diffraction methods. Our crystallographic studies have provided the first detailed structural view of the factor XIII A subunit and information that is useful for understanding transglutaminas...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-2007-999035

    authors: Yee VC,Le Trong I,Bishop PD,Pedersen LC,Stenkamp RE,Teller DC

    更新日期:1996-01-01 00:00:00

  • CD36 and macrophage scavenger receptor a modulate foam cell formation via inhibition of lipid-laden platelet phagocytosis.

    abstract::CD34 (+) progenitor cells are a promising source of regeneration in atherosclerosis or ischemic heart disease. However, as recently published, CD34(+) progenitor cells have the potential to differentiate not only into endothelial cells but also into foam cells upon interaction with platelets. The mechanism of platelet...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0030-1251499

    authors: Seizer P,Schiemann S,Merz T,Daub K,Bigalke B,Stellos K,Müller I,Stöckle C,Müller K,Gawaz M,May AE

    更新日期:2010-03-01 00:00:00

  • Different models of inheritance in selected genes in patients with sticky platelet syndrome and fetal loss.

    abstract:INTRODUCTION:The aim of this study was to evaluate the genetic variability of selected single nucleotide polymorphisms (SNPs) within GAS6 and PEAR1 genes and explore the association between selected SNPs and risk for fetal loss in women with sticky platelet syndrome (SPS). MATERIALS AND METHODS:We examined 23 female p...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0034-1395351

    authors: Sokol J,Biringer K,Skerenova M,Stasko J,Kubisz P,Danko J

    更新日期:2015-04-01 00:00:00

  • Aspects of the laboratory identification of von Willebrand disease in women.

    abstract::The increased prevalence of the laboratory diagnosis of von Willebrand disease (vWD) in women presenting with menorrhagia has raised concerns regarding certain specifics in vWD testing in women, including when vWD testing should be done in relation to menses and whether testing should be done while the patient is not ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2006-947861

    authors: Kouides PA

    更新日期:2006-07-01 00:00:00

  • Standardization of the INR: how good is your laboratory's INR and can it be improved?

    abstract::The prothrombin time (PT) assay is the most clinically ordered coagulation test and is most often used for monitoring of vitamin K antagonist (VKA) therapy (e.g., warfarin), where results are expressed as an international normalized ratio (INR). The INR is in essence the patient's PT "mathematically adjusted" to a sta...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0028-1104538

    authors: Favaloro EJ,Adcock DM

    更新日期:2008-10-01 00:00:00

  • Stimulation of cell surface plasminogen activation by heparin and related polyionic substances.

    abstract::The functional operation of the cell surface pro-u-PA and plasminogen activating system has previously been shown to depend on the assembly of u-PA receptors, plasminogen binding sites, and their respective ligands at the focal adhesions of cell extensions. We now show that additional factors operate that affect the p...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-2007-1002610

    authors: Stephens RW,Pöllänen J,Tapiovaara H,Woodrow G,Vaheri A

    更新日期:1991-07-01 00:00:00

  • Travel, venous thromboembolism, and thrombophilia.

    abstract::Current evidence indicates that prolonged air travel predisposes to venous thrombosis and pulmonary embolism. An effect is seen once travel duration exceeds 6 to 9 hours and becomes obvious in long-haul passengers traveling for 12 or more hours. A recent records linkage study found that increase in thrombosis rate amo...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2005-863810

    authors: Gallus AS

    更新日期:2005-02-01 00:00:00

  • New-Onset Atrial Fibrillation and Adverse In-Hospital Outcome in Patients with Acute Pulmonary Embolism.

    abstract::Atrial fibrillation (AF) can be secondary to acute pulmonary embolism (PE). This study aimed to investigate the prognostic impact of new-onset AF on patients with acute PE. In this study, 4,288 consecutive patients who were diagnosed with acute PE were retrospectively screened. In total, 77 patients with acute PE and ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0040-1718397

    authors: Tang RB,Jing YY,Xu ZY,Dong JZ,Du X,Wu JH,Yu RH,Long DY,Ning M,Sang CH,Jiang CX,Bai R,Liu N,Wen SN,Li SN,Chen X,Huang ST,Cui YK,Ma CS

    更新日期:2020-11-01 00:00:00

  • Protective effects of DX-9065a, an orally active, novel synthesized and selective inhibitor of factor Xa, against thromboplastin-induced experimental disseminated intravascular coagulation in rats.

    abstract::We investigated the protective effects of DX-9065a, an orally active, newly synthesized, and specific inhibitor of factor Xa, against experimental disseminated intravascular coagulation (DIC) in rats. Experimental DIC was induced by a 4 hour sustained infusion of thromboplastin at a dose of 2.5 mg/kg. The rats were or...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-2007-999016

    authors: Yamazaki M,Asakura H,Aoshima K,Saito M,Jokaji H,Uotani C,Kumabashiri I,Morishita E,Ikeda T,Matsuda T

    更新日期:1996-01-01 00:00:00

  • Regulation of thrombosis and hemostasis by antithrombin.

    abstract::In this article, three cases, with antithrombin (AT) abnormality "Toyama" (type IIb), AT abnormality "Aomori" (type IIa), and congenital AT deficiency (type I) with pregnancy and delivery managed with administration of both AT concentrates and low-molecular-weight heparin, are described. Additionally, a case of AT-pro...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-996136

    authors: Sakuragawa N

    更新日期:1997-01-01 00:00:00

  • Coagulation factor deficiencies and pregnancy loss.

    abstract::Of all the coagulation factor deficiencies, only deficiency of factor XIII (FXIII) and fibrinogen are associated with pregnancy loss. FXIII deficiency and a complete or partial deficiency of fibrinogen are associated with bleeding since childhood, impaired wound repair, and recurrent spontaneous abortions. Both FXIII ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2003-38832

    authors: Inbal A,Muszbek L

    更新日期:2003-04-01 00:00:00

  • Von Willebrand disease in children: diagnosis and management of a pediatric cohort in one single center in Argentina.

    abstract::Clinical and laboratory data of children with von Willebrand disease (VWD) types have been derived from retrospective studies and small case series. This article reports on the clinical and laboratory data of a large pediatric cohort in one single Argentinian center. The biological and clinical responses to desmopress...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0031-1281043

    authors: Bonduel M,Frontroth JP,Hepner M,Sciuccati G,Feliu-Torres A,Pieroni G

    更新日期:2011-07-01 00:00:00

  • Protective Effects of Statins Administration in European and North American Patients Infected with COVID-19: A Meta-analysis.

    abstract::Severe acute respiratory syndrome coronavirus 2 has spread rapidly throughout the world, becoming an overwhelming global health emergency. The array of injuries caused by this virus is broad and not limited to the respiratory system, but encompassing also extensive endothelial and systemic tissue damage. Since statins...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0040-1722307

    authors: Onorato D,Pucci M,Carpene G,Henry BM,Sanchis-Gomar F,Lippi G

    更新日期:2021-01-22 00:00:00

  • Multiple roles of tissue plasminogen activator in schizophrenia pathophysiology.

    abstract::Schizophrenia, a disabling mental disorder, is characterized by brain atrophy, especially in the superior temporal gyrus and the medial temporal lobe, which includes the hippocampus and the amygdala. The model that better explains brain atrophy includes a trigger and a predisposing condition. The trigger is exemplifie...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-0033-1357505

    authors: Hoirisch-Clapauch S,Nardi AE

    更新日期:2013-11-01 00:00:00

  • The influence of beta2-glycoprotein I on tissue factor activity.

    abstract::The beta2-glycoprotein I (beta2-GPI) is known for its procoagulant as well as anticoagulant properties. The influence of beta2-GPI on tissue factor (TF) activity was investigated by chromogenic assays for both factor Xa generation and factor VIIa activity. When 1.36 mg/mL beta2-GPI was added to phospholipids prior to ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-2007-995844

    authors: Ieko M,Yasukouchi T,Sawada KI,Koike T

    更新日期:1998-01-01 00:00:00

  • Developmental Hemostasis and Management of Central Venous Catheter Thrombosis in Neonates.

    abstract::Neonatal hemostasis differs qualitatively, but in particular quantitatively, from hemostasis in older children and adults. Nevertheless, hemostasis in healthy neonates is functionally stable with no tendency to bleeding or thrombotic complications. In sick neonates, however, risk factors may disrupt this equilibrium a...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0036-1592299

    authors: van Ommen CH,Sol JJ

    更新日期:2016-10-01 00:00:00

  • Extensive Computed Tomography versus Limited Screening for Detection of Occult Cancer in Unprovoked Venous Thromboembolism: A Multicenter, Controlled, Randomized Clinical Trial.

    abstract::Patients with unprovoked venous thromboembolism (VTE) may harbor occult cancer. Whether an extensive diagnostic work-up for cancer has additional value over a more limited screening for detection of underlying malignancy in these patients is controversial. We performed a randomized multicenter trial to assess if in pa...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1055/s-0036-1592335

    authors: Prandoni P,Bernardi E,Valle FD,Visonà A,Tropeano PF,Bova C,Bucherini E,Islam MS,Piccioli A

    更新日期:2016-11-01 00:00:00

  • The putative mechanism of thrombosis in antiphospholipid syndrome: impairment of the protein C and the fibrinolytic systems by monoclonal anticardiolipin antibodies.

    abstract::The mechanism of thrombosis in patients with antiphospholipid syndrome is not clear. To investigate it, we examined the effect of monoclonal anticardiolipin (aCL) antibodies and beta2-glycoprotein I (beta2-GPI), which is required for formation of the aCL epitopes, on activated protein C (APC) and on fibrinolytic activ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-994958

    authors: Ieko M,Sawada KI,Koike T,Notoya A,Mukai M,Kohno M,Wada N,Itoh T,Yoshioka N

    更新日期:1999-01-01 00:00:00

  • Exposure to high altitude: a risk factor for venous thromboembolism?

    abstract::There are several genetic and acquired risk factors for venous thromboembolism. Exposure to high altitude (HA), either during air travel, ascension of mountains, or while engaging in sports activities, has been observed to result in a hypercoagulable state, thus predisposing to thromboembolic events. Although several ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-0032-1301413

    authors: Gupta N,Ashraf MZ

    更新日期:2012-03-01 00:00:00

  • Diagnosis of venous thromboembolism in children.

    abstract::Venous thromboembolic events (VTE) in children are mostly related to central venous lines (CVL), and are located in the central upper venous system. The incidence of VTE in children with CVL is significant. However, the majority of CVL-related VTE do not present with typical symptoms or are not recognized due to under...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2003-42588

    authors: Male C,Kuhle S,Mitchell L

    更新日期:2003-08-01 00:00:00

  • The regulation of the factor VII-dependent coagulation pathway: rationale for the effectiveness of recombinant factor VIIa in refractory bleeding disorders.

    abstract::We have explored the molecular basis of the clinical therapeutic effect of factor VIIa in hemophilia A using empirical reconstituted in vitro thrombin generation models. Tissue factor acts as a receptor and activator of preexistent but virtually inactive two-chain plasma factor VIIa. However, most of the factor VII ci...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-2000-8454

    authors: van't Veer C,Mann KG

    更新日期:2000-01-01 00:00:00

  • How to Assess Fibrinogen Levels and Fibrin Clot Properties in Clinical Practice?

    abstract::Fibrin formed from fibrinogen is the main component of thrombi. Clot structure is characterized by fiber thickness and pore size, which differs within a given clot and between individuals. Plasma clot architecture is largely determined by the quantity and quality of fibrinogen. Plasma fibrinogen concentrations are mos...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-0036-1579636

    authors: Undas A

    更新日期:2016-06-01 00:00:00

  • Outcome of Major Hemorrhage at a Major Cardiothoracic Center in Patients with Activated Major Hemorrhage Protocol versus Nonactivated Protocol.

    abstract::This study aimed to determine the impact of major hemorrhage (MH) protocol (MHP) activation on blood administration and patient outcome at a UK major cardiothoracic center. MH was defined in patients (> 16 years) as those who received > 5 units of red blood cells (RBCs) in < 4 hours, or > 10 units in 24 hours. Data we...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0040-1718869

    authors: Chang KW,Owen S,Gaspar M,Laffan M,Arachchillage DRJ

    更新日期:2021-02-01 00:00:00

  • Description of an in vitro platelet function analyzer--PFA-100.

    abstract::A new in vitro system for the detection of platelet dysfunction, PFA-100, has been developed. It provides a quantitative measure of platelet function in anticoagulated whole blood. The system comprises a microprocessor-controlled instrument and a disposable test cartridge containing a biologically active membrane. The...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0032-1313612

    authors: Kundu SK,Heilmann EJ,Sio R,Garcia C,Davidson RM,Ostgaard RA

    更新日期:1995-01-01 00:00:00

  • Coacute heparin: a new simple monotest for monitoring heparin treatment.

    abstract::Anti-factor Xa methods have been generally accepted for the monitoring of heparin treatment mainly due to their sensitivity to LMWH and excellent performance on automated equipment. When such equipment is not available, as in small laboratories or on the night shift, there is a need for a simple manual method. In the ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:

    authors: Friberger P,Krig E,Eriksson-Skoog L,Svensson S,Isacson C

    更新日期:1993-01-01 00:00:00

  • Biologic tolerance of two different low molecular weight heparins.

    abstract::Heparin preparations have been used for prophylaxis and treatment of deep vein thrombosis for many years. Several biologic effects of heparin are known. Since 1978, there have been several reports about reversible elevation in serum values of AST and ALT in patients and healthy volunteers given heparin in small and hi...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:10.1055/s-2007-1002654

    authors: Christiansen HM,Lassen MR,Borris LC,Sørensen JV,Rahr HB,Jørgensen LN,Jørgensen PW,Hauch O

    更新日期:1991-10-01 00:00:00

  • Diagnostic efficacy of a simple radioimmunoassay test for fibrinogen/fibrin fragments containing the B beta 15-42 sequence.

    abstract::A simple RIA method for B beta 15-42 RPs has been evaluated in our laboratory to investigate experimental and clinical fibrinolytic states. The assay utilizes bentonite precipitation to remove cross-reacting fibrinogen. Due to the heterogeneity in molecular weights of the B beta RPs, the results are expressed as nanog...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章

    doi:10.1055/s-2007-1004430

    authors: Walenga JM,Fareed J,Mariani G,Messmore HL Jr,Bick RL,Emanuele RM

    更新日期:1984-10-01 00:00:00

  • Role of plasminogen activator inhibitor-1 in senescence and aging.

    abstract::The average age of the US population continues to increase. Age is the most important determinant of disease and disability in humans, but the fundamental mechanisms of aging remain largely unknown. Many age-related diseases are associated with an impaired fibrinolytic system. Elevated plasminogen activator inhibitor-...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审

    doi:10.1055/s-0034-1387883

    authors: Eren M,Boe AE,Klyachko EA,Vaughan DE

    更新日期:2014-09-01 00:00:00