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Diagnosis and Management of Marfan Syndrome
This book has been written in response to the many excellent questions posed by our patients and their care teams, questions which deserve the best-informed and up to date answers provided by our experts in each of the many health areas affected by Marfan syndrome. The aim of this text is to provide a summary of the present day understanding of diagnosis, management and best medical and surgical treatment of infants, children and adults with Marfan syndrome. The authors cover the lifelong problems from birth to old age, in each affected system. Forty percent of this information is the result of new careful research based on a well-defined longitudinally studied UK patient population.
Diagnosis and Management of Marfan Syndrome
This book has been written in response to the many excellent questions posed by our patients and their care teams, questions which deserve the best-informed and up to date answers provided by our experts in each of the many health areas affected by Marfan syndrome. The aim of this text is to provide a summary of the present day understanding of diagnosis, management and best medical and surgical treatment of infants, children and adults with Marfan syndrome. The authors cover the lifelong problems from birth to old age, in each affected system. Forty percent of this information is the result of new careful research based on a well-defined longitudinally studied UK patient population.
Marfan Syndrome
Historical Introduction The Marfan Syndrome: From Clinical Delineation to Mutational Characterization, a Semiautobiographic Account VictorA. McKusick l n 1876, E. Williams, an ophthalmologistin Cincinnati, Ohio, described ectopia lentis in a brother and sister who were exceptionally tall and had been loosejointed from birth. I Although there is a Williams syndrome that has aortic manifestations (supravalvar aortic stenosis), the name Williams was never associated with the disorder we now call Marfan syndrome. The reason is clear: Williamswas geographically removed from the leading medical centers and published in the Transactions of the American Ophthalmological Society; surely his report at...
Cardiovascular Aspects of Marfan Syndrome
Marfan Syndrome is a hereditary disorder of connective tissue which primarily affects the eyes, skeleton, blood vessels, and various structures of the heart. Since 1943, when the cardiovascular manifestations of Marfan Syndrome were first described, understanding of its nature, diagnostic tools, and surgical treatment have been remarkably improved. Although the specific surgical procedures have reached an advanced standard and general acceptance, many questions remain unanswered and have given rise to ongoing controversy. Cardiovascular Aspects of Marfan Syndrome presents the advances in understanding the cause and pathogenesis of Marfan Syndrome and its current specific surgical therapy.
Aortopathy
This is the first textbook to focus on Aortopathy, a new clinical concept for a form of vasculopathy. The first section of the book starts from discussing general concept and history of Aortopathy, and then deals with its pathophysiology, manifestation, intrinsic factor, clinical implication, management and prevention. The second part closely looks at various disorders of the Aortopathy such as bicuspid aortic valve and coarctation of aorta. The book editors have published a lot of works on the topic and have been collecting relating data in the field of congenital heart disease for the past 20 years, thus present the book with confidence. The topic - an association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction - is getting more and more attention among cardiovascular physicians. This is the first book to refer for cardiologists, pediatric cardiologists, surgeons, ACHD specialists, etc. to acquire thorough knowledge on Aortopathy.
The Marfan Syndrome
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A Handbook of Eisenmenger Syndrome and Marfan Syndrome
A Handbook of Eisenmenger Syndrome and Marfan Syndrome deals with two entirely different diseases – Eisenmenger syndrome, a cyanotic heart disease, and Marfan syndrome, a multisystem disorder of connective tissue. The features of Eisenmenger syndrome have been well described in Paul Wood’s case series, and it is difficult to find a better description than his till date. However, strategies on managing it have changed a lot in recent years, and this book incorporates the classical description by Paul Wood as well as the newer advances in its management in great details. Marfan syndrome due to its multisystem manifestation is not well represented in cardiology textbooks. An attempt has been made to describe Marfan syndrome in a simple but comprehensive manner. A short chapter on ‘Straight Back Syndrome’ is also included in this handbook. Medical students, especially students of medicine and cardiology, will find this handbook very useful.
The Marfan Syndrome
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Cassidy and Allanson's Management of Genetic Syndromes
MANAGEMENT OF GENETIC SYNDROMES THE MOST RECENT UPDATE TO ONE OF THE MOST ESSENTIAL REFERENCES ON MEDICAL GENETICS Cassidy and Allanson’s Management of Genetic Syndromes, Fourth Edition is the latest version of a classic text in medical genetics. With newly covered disorders and cutting-edge, up-to-date information, this resource remains the most crucial reference on the management of genetic syndromes in the field of medical genetics for students, clinicians, caregivers, and researchers. The fourth edition includes current information on the identification of genetic syndromes (including newly developed diagnostic criteria), the genetic basis (including diagnostic testing), and the routin...
Aneurysms-Osteoarthritis Syndrome
Aneurysms-Osteoarthritis Syndrome: SMAD3 Gene Mutations is a first-of-its-kind compilation of the genetic discovery, research, and care associated with AOS. With the field of genetically triggered aortopathies growing, this important reference will compile the newest discoveries in this field, allowing cardiologists, cardio-thoracic surgeons, clinical geneticists, vascular surgeons, orthopedic surgeons, and researchers to gain the knowledge they need without having to gather the data from various sources. Coverage includes genotype and phenotype correlations, the functional role of SMAD3, and insights into the role of TGFbeta signaling in aortic disease. The book will increase knowledge about AOS, providing awareness and better patient care for this aggressive disease. - Covers Aneurysms-Osteoarthritis Syndrome, from genetic discovery to patient care - Contains clinical management guidance on optimal cardiovascular treatments and surgery - Explains the autosomal dominant syndromes caused by mutations in the SMAD3 gene - Identifies the key features of this syndrome, including arterial aneurysms and tortuosity, early onset arthritis, and mild craniofacial features
