Neurofibromatosis : diagnosis, management and clinical outcomes / [E-Book]

Includes index.

NEUROFIBROMATOSIS: DIAGNOSIS, MANAGEMENT AND CLINICAL OUTCOMES; NEUROFIBROMATOSIS: DIAGNOSIS, MANAGEMENT AND CLINICAL OUTCOMES; Library of Congress Cataloging-in-Publication Data; Contents; Preface; Chapter 1: Cervical Pseudoaneurysm Associated with Neurofibromatosis Type 1; Abstract; Introduction; Litterature Review; Discussion; Conclusion; References; Chapter 2: Hematopoietic Lineages Cooperate with Osteoblasts in the Initiation and Progression of Neurofibromatosis Type 1 Associated Skeletal Deficits.

Herman B. Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN, USAbstract; I. Introduction; Neurofibromatosis Type 1 (NF1); Skeletal Deficits in NF1; Osteopenia/Osteoporosis; Spinal Deformities; Pseudarthrosis; Brief Chapter Overview; II. Skeletal Homeostasis and Hematopoiesis Are Interdependent; Hematopoietic Derived Myeloid Cells/Osteoclasts Are the Primary Mediators of Bone Resorption; Osteoblast -- Osteoclast Cross Talk in Bone Homeostasis; III. Nf1 Gene Dose Pivotally Regulates Myeloid Cell Fate and Function.

IV. Nf1 Haploinsufficient Hematopoietic Cells Cooperate to Engender Multiple NF1 Skeletal Anomalies in MiceModeling NF1 Skeletal Dysplasia in the Mouse; Critical Role for Nf1 Haploinsufficient Hematopoietic Cells in the Genesis of NF1 Osseous Deficits; V. Therapeutic Implications in the Treatment of NF1 Skeletal Disease; Conclusion; References; Chapter 3: Cerebrovascular Complications of Neurofibromatosis Type I; Abstract; Introduction; NF1 Vasculopathies Overview; Moya Moya Syndrome and Vascular Stenosis; Aneurysm; Arteriovenous Fistulas; Arteries Compressed or Invaded by; Neural Tumors.

Outcomes and Proposed Approaches to NF1 VasculopathyFuture Directions; References; Chapter 4: Targeting the Tumor Microenvironment for the Treatment of Plexiform Neurofibromas in Patients with Neurofibromatosis Type 1; Herman B Wells Center for Pediatric Research, Departments of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, US; Abstract; 1. Tumor Microenvironments; 2. NF1 Genetics and Manifestations; 3. Modeling Neurofibromas in the Mouse; 4. Microenvironment Interactions Perpetuate Neurofibroma Progression.

5. SCF/C-Kit Activated Nf1+/- Mast Cells Pivotally Underpin Plexiform Neuribroma Genesis6. Pharmacologic SCF/c-kit Inhibition in the Treatment of Plexiform Neurofibromas; Conclusion; References; Blank Page; Index.

Neurofibromatosis Type 1 (NF1) is a hereditary neurocutaneous tumor disorder that owes many of its most common features to abnormalities in neural crest-derived cells. NF1 may cause dysplasia in various tissues, even in some tissues that are non-neural crest-derived (e.g. bone). While common manifestations of NF1 include caf�e-au-lait spots and neurofibromas, vasculopathies are less common yet noteworthy complications of NF1. NF1 vasculopathies can involve vessels supplying various organs. Cerebrovascular abnormalities associated with NF1 have been sporadically described in the literature; thes.

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