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Hereditary Angioedema in Sweden

Hereditary Angioedema in Sweden PDF Author: Patrik Nordenfelt
Publisher: Linköping University Electronic Press
ISBN: 9176854302
Category :
Languages : en
Pages : 116

Book Description
Background: Hereditary angioedema (HAE) due to C1-inhibitor deficiency, type I and II, is a rare disease with an estimated prevalence of 1/50,000. Angioedema in the larynx can be life threatening and angioedema in the abdomen and skin can give severe and disabling pain. Data on patients with HAE in Sweden were scarce before our study. Aim: To study the prevalence of HAE, and to investigate clinical manifestations, treatments, and Health-Related Quality of Life (HR-QoL) in adults and children in Sweden. Method: In studies, I and II, all patients received a written questionnaire followed by a phone interview with questions about clinical manifestations, medication, sick leave and QoL. In study III the patients completed EuroQol 5 Dimensions 5 Levels (EQ-5D-5L) questionnaires for both the attack-free state (EQ5D today), and the last HAE attack (EQ5D attack). Questions were also asked about sick-leave. In study IV all adults received questionnaires with EQ-5D-5L and RAND-36, Angioedema Quality of Life instrument (AE-QoL), and Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication. Results: We identified 146 patients, 110 adults and 36 children with HAE, type I (n=136) or II (n=10), giving a minimal HAE prevalence of 1.54/100,000. For adults, the median age at onset of symptoms was 12 years and median age at diagnosis was 22 years. Median age at onset of symptoms for children was 4 years and at diagnosis 3 years. During the previous year, 47% of adults experienced at least 12 attacks, 21% 4-11 attacks, 11% 1-3 attacks, while 22% were asymptomatic. For children, the corresponding figures were about the same. The median number of attacks in those having attacks was 14 in adults and 6 in children last year. Adult females reported on average 19 attacks the previous year versus nine for males. Irrespective of location nine out of 10 reported pain. Trigger factors were experienced in 95 % of adults and 74 % of children. Plasma-derived C1-inhibitor concentrate (pdC1INH) had a very good effect on acute attacks. Long-term prophylaxis with androgens and pdC1INH reduced the annual attack frequency by more than 50 %. Of the children’s parents, 73% had been on parental leave to care for the child due to HAE symptoms. Health and QoL were generally rated as good. In study III 103 of 139 responded and reported an EQ5D today score that was significantly higher than the EQ5D attack score. Attack frequency had a negative effect on EQ5D today. Children had significantly higher EQ-5D-5L than adults. Forty four percent had been absent from work or school during the latest attack. In study IV 64 of 133 adults responded. The most affected HR-QoL dimensions in EQ-5D-5L were pain/discomfort and anxiety/depression, in RAND-36 energy/fatigue, general health, health transition, pain, and in AE-QoL fears/shame and fatigue/mood. Females had significantly lower HR-QoL in RAND-36 for general health and energy/fatigue. There was an association between AAS and EQ-5D-5L/RAND-36 (except physical function) /AEQoL. There was no significant difference in HR-QoL in patients with and without prophylactic medication. Conclusion: The minimal prevalence of HAE type I and II in Sweden is 1.54/100,000. Median age at onset was 12 years. Adult females had twice as many attacks as males, adults had also twice as many attacks as children. For acute treatment, pdC1INH had a very good effect. For long term prophylaxis, androgens and pdC1INH had good effect. The most affected HR-QoL dimensions in EQ-5D-5L were pain/discomfort and anxiety/ depression, in RAND-36 energy/fatigue, general health, health transition and pain, and in AE-QoL fears/shame and fatigue/mood. Children reported better HR-QoL than adults. AE-QoL is more disease-specific in HAE than the generic instruments EQ-5D-5L and RAND-36. However, the latter highlights the pain aspect, whereas AE-QoL does not. Patients with high disease activity should thus be considered for more intensive treatment to improve their HR-QoL.

Hereditary Angioedema in Sweden

Hereditary Angioedema in Sweden PDF Author: Patrik Nordenfelt
Publisher: Linköping University Electronic Press
ISBN: 9176854302
Category :
Languages : en
Pages : 116

Book Description
Background: Hereditary angioedema (HAE) due to C1-inhibitor deficiency, type I and II, is a rare disease with an estimated prevalence of 1/50,000. Angioedema in the larynx can be life threatening and angioedema in the abdomen and skin can give severe and disabling pain. Data on patients with HAE in Sweden were scarce before our study. Aim: To study the prevalence of HAE, and to investigate clinical manifestations, treatments, and Health-Related Quality of Life (HR-QoL) in adults and children in Sweden. Method: In studies, I and II, all patients received a written questionnaire followed by a phone interview with questions about clinical manifestations, medication, sick leave and QoL. In study III the patients completed EuroQol 5 Dimensions 5 Levels (EQ-5D-5L) questionnaires for both the attack-free state (EQ5D today), and the last HAE attack (EQ5D attack). Questions were also asked about sick-leave. In study IV all adults received questionnaires with EQ-5D-5L and RAND-36, Angioedema Quality of Life instrument (AE-QoL), and Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication. Results: We identified 146 patients, 110 adults and 36 children with HAE, type I (n=136) or II (n=10), giving a minimal HAE prevalence of 1.54/100,000. For adults, the median age at onset of symptoms was 12 years and median age at diagnosis was 22 years. Median age at onset of symptoms for children was 4 years and at diagnosis 3 years. During the previous year, 47% of adults experienced at least 12 attacks, 21% 4-11 attacks, 11% 1-3 attacks, while 22% were asymptomatic. For children, the corresponding figures were about the same. The median number of attacks in those having attacks was 14 in adults and 6 in children last year. Adult females reported on average 19 attacks the previous year versus nine for males. Irrespective of location nine out of 10 reported pain. Trigger factors were experienced in 95 % of adults and 74 % of children. Plasma-derived C1-inhibitor concentrate (pdC1INH) had a very good effect on acute attacks. Long-term prophylaxis with androgens and pdC1INH reduced the annual attack frequency by more than 50 %. Of the children’s parents, 73% had been on parental leave to care for the child due to HAE symptoms. Health and QoL were generally rated as good. In study III 103 of 139 responded and reported an EQ5D today score that was significantly higher than the EQ5D attack score. Attack frequency had a negative effect on EQ5D today. Children had significantly higher EQ-5D-5L than adults. Forty four percent had been absent from work or school during the latest attack. In study IV 64 of 133 adults responded. The most affected HR-QoL dimensions in EQ-5D-5L were pain/discomfort and anxiety/depression, in RAND-36 energy/fatigue, general health, health transition, pain, and in AE-QoL fears/shame and fatigue/mood. Females had significantly lower HR-QoL in RAND-36 for general health and energy/fatigue. There was an association between AAS and EQ-5D-5L/RAND-36 (except physical function) /AEQoL. There was no significant difference in HR-QoL in patients with and without prophylactic medication. Conclusion: The minimal prevalence of HAE type I and II in Sweden is 1.54/100,000. Median age at onset was 12 years. Adult females had twice as many attacks as males, adults had also twice as many attacks as children. For acute treatment, pdC1INH had a very good effect. For long term prophylaxis, androgens and pdC1INH had good effect. The most affected HR-QoL dimensions in EQ-5D-5L were pain/discomfort and anxiety/ depression, in RAND-36 energy/fatigue, general health, health transition and pain, and in AE-QoL fears/shame and fatigue/mood. Children reported better HR-QoL than adults. AE-QoL is more disease-specific in HAE than the generic instruments EQ-5D-5L and RAND-36. However, the latter highlights the pain aspect, whereas AE-QoL does not. Patients with high disease activity should thus be considered for more intensive treatment to improve their HR-QoL.

Urticaria and Angioedema

Urticaria and Angioedema PDF Author: Selda Pelin Kartal
Publisher: BoD – Books on Demand
ISBN: 9535131672
Category : Medical
Languages : en
Pages : 252

Book Description
This book contains the latest advances and scientific knowledge from the leading experts in urticaria and angioedema. The book consists of 15 chapters in which urticaria classification, urticaria etiopathogenesis, urticaria clinics, urticarial syndromes, angioedemas, diagnosis, pathogenesis and pathophysiology of urticaria, and treatment options are discussed. This book also emphasizes on the various laboratory tests necessary for urticarias. One chapter of the book is devoted to comorbidities in chronic spontaneous urticaria. Another chapter is related with pathophysiology and treatment of hereditary angioedema. We are grateful to all the contributors and leading experts for their valuable chapters, which provide an in-depth view of all aspects of the content, backed with the most current literature in the field. We hope that this book will provide interesting knowledge and serve as a comprehensive guide to many physicians dealing with urticaria and angioedemas in their clinical practice.

Healthcare and Culture

Healthcare and Culture PDF Author: Maria Francesca Freda
Publisher: IAP
ISBN: 168123646X
Category : Psychology
Languages : en
Pages : 337

Book Description
The book deals with current issues, pertinent every healthcare relationship. Changes in medicine as well as some constant aspects over time arise within a cultural ground and generate new questions and issues that are not only purely medical, but also bioethical, social, political, economic and psychological of course. On the one hand, changes in medicine generate new questions for society, on the other hand, the society poses new questions to the medicine, new challenges, and in some cases they can conflict with consolidated models and practices. Never the progress of Western medicine and its therapeutic practices have been as significant as in the last decades but the increase of specific competence and effectiveness of medical treatments are not linearly translated into an increase of consensus, dialogue and alliance between medicine and society. How does psychology take on a position of interlocutor towards medicine and its transformations? How does Cultural Psychology, Health Psychology, Clinical Psychology confront themselves with the processes of meaning making generated by medicine? The interest of the book is aimed to grasp the construction of processes of cultural, relational and subjective meaning in the dialogical encounter between medicine and society, between doctor and patient. The book intends to focus in particular on two specific plans: on the one hand, to present a reflection and analysis on contemporary medicine and its on?going transformations of the healthcare relationship; on the other hand, to present and discuss experiences of intervention and possible models of intervention addressed to healthcare and doctor?patient relationships during its crucial steps (consultation, formulation and communication of diagnosis, therapy, conclusion). The book’s purposes are aimed to discuss crucial and current issues on the borders between medicine and psychology: consensus and sharing, decision?making and autonomy, subjectivity and narration, emotions and affectivity, medical semeiotics and cultural semiotics, training of physicians, and epistemological, theoretical and methodological issues.

Clinical Allergy and Asthma Management in Adolescents and Young Adults

Clinical Allergy and Asthma Management in Adolescents and Young Adults PDF Author: Ravindran Chetambath
Publisher: CRC Press
ISBN: 100044922X
Category : Medical
Languages : en
Pages : 533

Book Description
Key Features • Covers allergic diseases in general and asthma in detail, providing a comprehensive coverage of clinical features, diagnosis and management, using a case-based approach • Creates awareness of the fundamentals of clinical allergy, helping physicians make wise choices of medical and surgical management, especially in resource limited settings • Features insight from international authors with clinical experience in diagnosis and treatment of asthma in the specific age group of adolescents and young adults.

Hereditary Angioedemas: New Insights for the Healthcare Professional: 2012 Edition

Hereditary Angioedemas: New Insights for the Healthcare Professional: 2012 Edition PDF Author:
Publisher: ScholarlyEditions
ISBN: 1464988234
Category : Medical
Languages : en
Pages : 17

Book Description
Hereditary Angioedemas: New Insights for the Healthcare Professional / 2012 Edition is a ScholarlyPaper™ that delivers timely, authoritative, and intensively focused information about Hereditary Angioedemas in a compact format. The editors have built Hereditary Angioedemas: New Insights for the Healthcare Professional / 2012 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Hereditary Angioedemas in this eBook to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Hereditary Angioedemas: New Insights for the Healthcare Professional / 2012 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.

Angioedema, An Issue of Immunology and Allergy Clinics of North America, E-Book

Angioedema, An Issue of Immunology and Allergy Clinics of North America, E-Book PDF Author: Marc Riedl
Publisher: Elsevier Health Sciences
ISBN: 0323532381
Category : Medical
Languages : en
Pages :

Book Description
This issue of Immunology and Allergy Clinics, guest edited by Mariana Castells, is devoted to Drug Hypersensitivity and Desensitizations. Articles in this issue include: Principles and Practice of Drug Desensitization; Incidence and Prevalence of Drug Hypersensitivity; Drug-Induced Anaphylaxis; Penicillin and Beta Lactam Hypersensitivity; Platins Chemotherapy Hypersensitivity: Prevalence and Management; Monoclonal Antibodies Hypersensitivity: Prevalence and Management; Injectable Drugs and MoAbs Reactions and Management with Desensitization; Delayed Cutaneous Reactions to Antibiotics, Management with Desensitization; Management of Children with Hypersensitivity to Antibiotics and MoAbs; Taxenes Hypersensitivity and Management; Progesterone Autoimmune Dermatitis; Severe Delayed Drug Reaction: Role of Genetics and Viral Infections; andAspirin and NSAIDS Hypersensitivity and Management.

Angioedema, An Issue of Immunology and Allergy Clinics

Angioedema, An Issue of Immunology and Allergy Clinics PDF Author: Bruce L. Zuraw
Publisher: Elsevier Health Sciences
ISBN: 032324226X
Category : Medical
Languages : en
Pages : 146

Book Description
Guest edited by Jonathan Dillman and Ethan Smith, this issue of MRI Clinics covers pediatric MRI imaging of lower body structures, such as the liver, kidneys, urinary tract, bowel, pancreas and pelvis. Certain pathologies such as anorectal malformations, Crohn disease, inflammatory bowel disease and oncologic imaging are also discussed.

Genetics Abstracts

Genetics Abstracts PDF Author:
Publisher:
ISBN:
Category : Genetics
Languages : en
Pages : 764

Book Description


Pediatric Allergy

Pediatric Allergy PDF Author: Donald Y. M. Leung
Publisher: Elsevier Health Sciences
ISBN: 0323674631
Category : Medical
Languages : en
Pages : 577

Book Description
With complete, concise coverage of prevention, indications, diagnosis, and treatment, Pediatric Allergy, 4th Edition, is your go-to resource for current, clinically-focused content in the rapidly changing field of allergies and immune-mediated diseases in children. This highly regarded reference fully covers immunology and origins of allergy, growth and development, pregnancy, infancy, and early, middle, and late childhood—all with a new, practical, clinical focus. You’ll find authoritative coverage of the full range of allergies in pediatric patients, from asthma and other common conditions to rare or uncommon allergies, providing a one-stop resource for clinicians across specialties who care for children. Focuses on the clinical application of science to the bedside, including clinical pearls discussing best practice approaches and guidelines. Integrates the latest research on the origins of allergy in early life, including pregnancy, the neonate, and infants. Includes new, evidence-based management guidelines for various diseases, including food allergy, atopic dermatitis, asthma and immunodeficiency. Provides new content on the prevention of allergies; asthma, eczema, and food allergies in young children; and extensive current guidelines and new therapies. Offers up-to-date information on key areas such as food challenges, skin testing, IgE diagnostic labs, immunotherapy, clinical immunology diagnostic testing, molecular diagnostics, and lung function testing. Highlights current guidance from a new team of leading experts with 150 anatomic schematics, clinical photos, and algorithms—many new to this edition. Covers gene therapy, stem-cell therapy, and a host of immunodeficiency diseases found in children with allergies. Discusses targeted biologics for severe allergic diseases, defects of innate immunity, rheumatic diseases of childhood, and inflammatory disorders.

Pediatric Allergy: Principles and Practice E-Book

Pediatric Allergy: Principles and Practice E-Book PDF Author: Stanley J. Szefler
Publisher: Elsevier Health Sciences
ISBN: 0323339468
Category : Medical
Languages : en
Pages : 727

Book Description
The third edition of Pediatric Allergy continues this title's steadfast tradition of providing comprehensive, authoritative guidance on the day-to-day diagnosis and management of pediatric allergic and immunologic diseases. You'll have the most up-to-date research at hand thanks to an easily accessible full-color format that highlights a host of new chapters, extensive updates, and clinically focused coverage. Whether you're a student, resident, pediatrician or allergist, you'll appreciate this user-friendly and versatile source for providing optimal care! Includes diagnostic tests available for asthma, upper respiratory allergy, and more. Equips you with an understanding of the immune mechanisms underlying allergic diseases. Features coverage of drug allergies and cross-reactivity. Highlights clinical pearls discussing the best approaches to the care and treatment of pediatric patients. Appendices listing common food allergies and autoantibodies in autoimmune diseases make for quick reference to essential material. Expert Consult eBook version included with purchase. This enhanced eBook experience allows you to search all of the text, figures, images, and references from the book on a variety of devices. Revised asthma section examines current asthma guidelines; school-centered asthma programs; exercise-induced asthma; and new directions in asthma therapy. Includes the most current knowledge relating to emerging asthma within young children, medication adherence, and the impact of infection on the natural history of asthma. New information on gene therapy, stem-cell therapy, and a host of new immunodeficiency diseases helps you obtain the best results from the therapeutics for pediatric allergic and immunologic diseases. Features brand-new chapters on immunopathology; diagnostics and management; potential immunotherapeutic strategies for treating food allergies; current status of immunotherapy for food allergy; and biologic therapies. Focused coverage of today's hot topics in pediatric allergy includes the use of targeted biologics to treat specific activation pathways leading to severe allergic diseases; defects of innate immunity; rheumatic diseases of childhood; and inflammatory disorders. Discusses new studies examining potential etiologies for the increase in food allergy and examines potential immunotherapeutic strategies for treating food allergies. New evidence-based principles of medical care help you make the best use of available medications for your patients.