The Experts below are selected from a list of 21510 Experts worldwide ranked by ideXlab platform
Katrien Devreese - One of the best experts on this subject based on the ideXlab platform.
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lupus anticoagulant hypoprothrombinemia syndrome report of two cases and review of the literature
Lupus, 2015Co-Authors: Sylvie Mulliez, F De Keyser, C Verbist, Anna Vantilborgh, Walter Wijns, I Beukinga, Katrien DevreeseAbstract:Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare Acquired Disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.
Hans Forstl - One of the best experts on this subject based on the ideXlab platform.
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double blind placebo controlled provocation study in patients with subjective multiple chemical sensitivity mcs and matched control subjects
Clinical Toxicology, 2008Co-Authors: Susanne Bornschein, Hans Forstl, Constanze Hausteiner, Horst Rommelt, Dennis Nowak, Thomas ZilkerAbstract:Introduction. Multiple Chemical Sensitivity (MCS) is an Acquired Disorder with recurrent symptoms referable to multiple organ systems. No widely accepted test of physiologic function correlates with symptoms and it has not been recognized as a distinct entity by the scientific community. Few double-blind placebo-controlled studies have been done. The objectives of this study were to test two hypotheses: that patients with MCS can distinguish reliably between solvents and placebo, and that there are significant differences in objective biological and neuropsychological parameters between solvent and placebo exposures. Methods. Twenty patients with MCS and 17 controls underwent six exposure sessions (solvent mixture and clean air in random order, double-blind) in a challenge chamber. Positive reactions were defined as subjective perception of being exposed to solvents, blood pressure or heart rate change of ≥ 10%, rash or clinical signs of hypoxia, or symptom severity rise after exposure. Results. No differences between the groups with regard to sensitivity, specificity, and accuracy were found. Cognitive performance was not influenced by solvent exposure, and did not differ between the groups. There was no difference between the groups in serum cortisol levels measured before and after exposures. Conclusion. The hypotheses were not confirmed.
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psychiatric and somatic Disorders and multiple chemical sensitivity mcs in 264 environmental patients
Psychological Medicine, 2002Co-Authors: Susanne Bornschein, Thomas Zilker, Constanze Hausteiner, Hans ForstlAbstract:Background. An increasing number of individuals with diverse health complaints are currently seeking help in the field of environmental medicine. Multiple chemical sensitivity (MCS) or idiopathic environmental intolerances (IEI) is defined as an Acquired Disorder with multiple recurrent symptoms associated with environmental chemicals in low concentrations that are well tolerated by the majority of people. Their symptoms are not explained by any known psychiatric or somatic Disorder. Method. Within a 2-year period we examined 264 of 267 consecutive patients prospectively presenting to a university based out-patient department for environmental medicine. Patients underwent routine medical examination, toxicological analysis and the structured clinical interview for DSM-IV psychiatric Disorders (SCID). Results. Seventy-five per cent of the patients met DSM-IV criteria for at least one psychiatric Disorder and 35% of all patients suffered from somatoform Disorders. Other frequent diagnoses were affective and anxiety Disorders, and dependence or substance abuse. In 39% a psychiatric Disorder, in 23% a somatic condition and in 19% a combination of the two were considered to provide sufficient explanation of the symptoms. Toxic chemicals were regarded as the most probable cause in only five cases. The suspected diagnosis of MCS/IEI could not be sustained in the vast majority of cases. Conclusion. This investigation confirms previous findings that psychiatric morbidity is high in patients presenting to specialized centres for environmental medicine. Somatoform Disorders are the leading diagnostic category, and there is reason to believe that certain ‘environmental’ or MCS patients form a special subgroup of somatoform Disorders. In most cases, symptoms can be explained by well-defined psychiatric and medical conditions other than MCS, which need specific treatment. Further studies should focus on provocation testing in order to find positive criteria for MCS and on therapeutic approaches that consider psychiatric aspects.
Sylvie Mulliez - One of the best experts on this subject based on the ideXlab platform.
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lupus anticoagulant hypoprothrombinemia syndrome report of two cases and review of the literature
Lupus, 2015Co-Authors: Sylvie Mulliez, F De Keyser, C Verbist, Anna Vantilborgh, Walter Wijns, I Beukinga, Katrien DevreeseAbstract:Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare Acquired Disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.
Barbara P. Yawn - One of the best experts on this subject based on the ideXlab platform.
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clinical and laboratory diagnosis of von willebrand disease a synopsis of the 2008 nhlbi nih guidelines
American Journal of Hematology, 2009Co-Authors: William L. Nichols, Margaret E. Rick, Mae B. Hultin, Evan J Sadler, Barbara P. Yawn, Thomas L. Ortel, Robert R Montgomery, Andra H. James, Marilyn J MancojohnsonAbstract:Von Willebrand factor (VWF) mediates blood platelet adhesion and accumulation at sites of blood vessel injury, and also carries coagulation factor VIII (FVIII) that is important for generating procoagulant activity. Von Willebrand disease (VWD), the most common inherited bleeding Disorder, affects males and females, and reflects deficiency or defects of VWF that may also cause decreased FVIII. It may also occur less commonly as an Acquired Disorder (Acquired von Willebrand syndrome). This article briefly summarizes selected features of the March 2008 evidence-based clinical and laboratory diagnostic recommendations from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel for assessment for VWD or other bleeding Disorders or risks. Management of VWD is also addressed in the NHLBI guidelines, but is not summarized here. The VWD guidelines are available at the NHLBI Web site (http://www.nhlbi.nih.gov/guidelines/ vwd). Am. J. Hematol. 84:366-370, 2009. (C) 2009 Wiley-Liss, Inc.
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von willebrand disease vwd evidence based diagnosis and management guidelines the national heart lung and blood institute nhlbi expert panel report usa 1
Haemophilia, 2008Co-Authors: William L. Nichols, Margaret E. Rick, Mae B. Hultin, Marilyn J Mancojohnson, Thomas L. Ortel, Robert R Montgomery, Andra H. James, J E Sadler, M Weinstein, Barbara P. YawnAbstract:Summary. von Willebrand disease (VWD) is a commonly encountered inherited bleeding Disorder affecting both males and females, causing mucous membrane and skin bleeding symptoms, and bleeding with surgical or other haemostatic challenges. VWD may be disproportionately symptomatic in women of child-bearing age. It may also occur less frequently as an Acquired Disorder (Acquired von Willebrand syndrome). VWD is caused by deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates platelet haemostatic function and stabilizes blood coagulation factor VIII. The pathophysiology, classification, diagnosis and management of VWD are relatively complex, but understanding them is important for proper diagnosis and management of patients with VWD. These evidence-based guidelines for diagnosis and management of VWD from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel (USA) review relevant publications, summarize current understanding of VWD pathophysiology and classification, and present consensus diagnostic and management recommendations based on analysis of the literature and expert opinion. They also suggest an approach for clinical and laboratory evaluation of individuals with bleeding symptoms, history of bleeding or conditions associated with increased bleeding risk. This document summarizes needs for further research in VWF, VWD and bleeding Disorders, including clinical research to obtain more objective information about bleeding symptoms, advancements in diagnostic and therapeutic tools, and enhancement in the education and training of clinicians and scientists in bleeding and thrombotic Disorders. The NHLBI Web site (http://www.nhlbi.nih.gov/guidelines/vwd) has a more detailed document, a synopsis of these recommendations, and patient education information.
Susanne Bornschein - One of the best experts on this subject based on the ideXlab platform.
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double blind placebo controlled provocation study in patients with subjective multiple chemical sensitivity mcs and matched control subjects
Clinical Toxicology, 2008Co-Authors: Susanne Bornschein, Hans Forstl, Constanze Hausteiner, Horst Rommelt, Dennis Nowak, Thomas ZilkerAbstract:Introduction. Multiple Chemical Sensitivity (MCS) is an Acquired Disorder with recurrent symptoms referable to multiple organ systems. No widely accepted test of physiologic function correlates with symptoms and it has not been recognized as a distinct entity by the scientific community. Few double-blind placebo-controlled studies have been done. The objectives of this study were to test two hypotheses: that patients with MCS can distinguish reliably between solvents and placebo, and that there are significant differences in objective biological and neuropsychological parameters between solvent and placebo exposures. Methods. Twenty patients with MCS and 17 controls underwent six exposure sessions (solvent mixture and clean air in random order, double-blind) in a challenge chamber. Positive reactions were defined as subjective perception of being exposed to solvents, blood pressure or heart rate change of ≥ 10%, rash or clinical signs of hypoxia, or symptom severity rise after exposure. Results. No differences between the groups with regard to sensitivity, specificity, and accuracy were found. Cognitive performance was not influenced by solvent exposure, and did not differ between the groups. There was no difference between the groups in serum cortisol levels measured before and after exposures. Conclusion. The hypotheses were not confirmed.
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psychiatric and somatic Disorders and multiple chemical sensitivity mcs in 264 environmental patients
Psychological Medicine, 2002Co-Authors: Susanne Bornschein, Thomas Zilker, Constanze Hausteiner, Hans ForstlAbstract:Background. An increasing number of individuals with diverse health complaints are currently seeking help in the field of environmental medicine. Multiple chemical sensitivity (MCS) or idiopathic environmental intolerances (IEI) is defined as an Acquired Disorder with multiple recurrent symptoms associated with environmental chemicals in low concentrations that are well tolerated by the majority of people. Their symptoms are not explained by any known psychiatric or somatic Disorder. Method. Within a 2-year period we examined 264 of 267 consecutive patients prospectively presenting to a university based out-patient department for environmental medicine. Patients underwent routine medical examination, toxicological analysis and the structured clinical interview for DSM-IV psychiatric Disorders (SCID). Results. Seventy-five per cent of the patients met DSM-IV criteria for at least one psychiatric Disorder and 35% of all patients suffered from somatoform Disorders. Other frequent diagnoses were affective and anxiety Disorders, and dependence or substance abuse. In 39% a psychiatric Disorder, in 23% a somatic condition and in 19% a combination of the two were considered to provide sufficient explanation of the symptoms. Toxic chemicals were regarded as the most probable cause in only five cases. The suspected diagnosis of MCS/IEI could not be sustained in the vast majority of cases. Conclusion. This investigation confirms previous findings that psychiatric morbidity is high in patients presenting to specialized centres for environmental medicine. Somatoform Disorders are the leading diagnostic category, and there is reason to believe that certain ‘environmental’ or MCS patients form a special subgroup of somatoform Disorders. In most cases, symptoms can be explained by well-defined psychiatric and medical conditions other than MCS, which need specific treatment. Further studies should focus on provocation testing in order to find positive criteria for MCS and on therapeutic approaches that consider psychiatric aspects.
