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Books > Science & Mathematics > Biology, life sciences > Cellular biology
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Progress in Heritable Soft Connective Tissue Diseases (Paperback, 2nd ed. 2021)
Loot Price: R2,903
Discovery Miles 29 030
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Progress in Heritable Soft Connective Tissue Diseases (Paperback, 2nd ed. 2021)
Series: Advances in Experimental Medicine and Biology, 1348
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This volume represents a substantially revised and updated 2nd
edition of a reference handbook on major structural components of
soft connective tissues and a whole slew of heritable diseases of
soft connective tissues. The number of clearly identifiable and
distinct disorders has grown somewhat since the 1st edition in
2014, e.g., Ehlers-Danlos syndrome has now 13 entities. A brand new
syndrome, Meester Loeys syndrome carrying the name of Bart Loeys
was added as a companion to Loeys-Dietz syndrome. Numerous
variations of cutis laxa and joint mobility disorders have been
discovered taking advantage of recent advancements in genetic
analysis. We have acquired better understanding of pathogenesis and
biochemical changes in some other, more established entities, such
as Marfan and collagen VI myopathies where better management and
possible treatment are on the horizon. Even in the case of
connective tissue diseases in domestic animals some progress has
been made. All these updates were contributed by a group of
distinguished and preeminent physicians and scientists, all of them
not just working in the field but making new discoveries described
by them. Readers will notice that seemingly there is an overlap
among many of these disorders. And indeed, many of them, if not
most are interconnected because of the prominent roles of TGF , of
fibrillin microfibrils and collagen fibril assembly (and other
molecules) playing in connective tissues physiology, and by
extension in pathogenesis of many disorders described in the book.
What I found particularly helpful that author(s) of each chapter
bring their own perspective even when described closely related
mechanism of the disease. These observations should help with
diagnosis and management of such cases. The first chapters are more
general, concentrating more on the physiology, structure and
biochemistry of normal soft tissues. That should help in better
understanding of the pathophysiology. Last but not least, the
chapters are very readable, more like detective stories than dry
description of genetic/biochemical defects. I do hope that basic
scientists and clinicians with similar and diverse interests alike
will appreciate this volume and will be inspired by it to develop
their research in the field.
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