scholarly journals A murine model of neurofibromatosis type 1 tibial pseudarthrosis featuring proliferative fibrous tissue and osteoclast-like cells

2011 ◽  
Vol 27 (1) ◽  
pp. 68-78 ◽  
Author(s):  
Jad El-Hoss ◽  
Kate Sullivan ◽  
Tegan Cheng ◽  
Nicole YC Yu ◽  
Justin D Bobyn ◽  
...  
Keyword(s):  
Murine Model ◽  
Neurofibromatosis Type 1 ◽  
Fibrous Tissue ◽  
Neurofibromatosis Type

scholarly journals Dystrophic Spinal Deformities in a Neurofibromatosis Type 1 Murine Model

PLoS ONE ◽  
2015 ◽  
Vol 10 (3) ◽  
pp. e0119093 ◽  
Author(s):  
Steven D. Rhodes ◽  
Wei Zhang ◽  
Dalong Yang ◽  
Hao Yang ◽  
Shi Chen ◽  
...  
Keyword(s):  
Murine Model ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Spinal Deformities

scholarly journals Primary osteopathy of vertebrae in a neurofibromatosis type 1 murine model

Bone ◽  
2011 ◽  
Vol 48 (6) ◽  
pp. 1378-1387 ◽  
Author(s):  
Wei Zhang ◽  
Steven D. Rhodes ◽  
Liming Zhao ◽  
Yongzheng He ◽  
Yingze Zhang ◽  
...  
Keyword(s):  
Murine Model ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type

Hamartoma Involving the Pseudarthrosis Site in Patients with Neurofibromatosis Type 1

2005 ◽  
Vol 8 (2) ◽  
pp. 190-196 ◽  
Author(s):  
Rocío P. Mariaud-Schmidt ◽  
Sergio Rosales-Quintana ◽  
Emilio Bitar ◽  
Daniel Fajardo ◽  
Gilberto Chiapa-Robles ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Fibrous Tissue ◽  
Neurofibromatosis Type ◽  
Clinical Effects ◽  
Long Bones ◽  
Male And Female ◽  
Congenital Pseudarthrosis ◽  
First Time ◽  
Exact Origin

Congenital pseudarthrosis is a rare disease with variable clinical effects. The disease remains 1 of the most controversial pediatric entities in terms of etiopathogenesis, therapy, and prognosis. Between 0.5% and 2.2% of patients with neurofibromatosis demonstrate pseudarthrosis in any of the long bones. The exact origin of the lesion is even unclear; although several attempts have been made to determine the type of tissue involving the pseudarthrosis site, only fibrous tissue has been documented in different reports. We present 2 unrelated Mexican patients (male and female) with familial neurofibromatosis and congenital pseudarthrosis of the tibia and fibula. Histochemical and immunostain studies after surgical resection of the affected ends from the pseudarthrosis site of both patients showed a picture compatible with hamartoma. This is the first time when histologic evidence of hamartomatous tissue involving the pseudarthrosis site is presented.

scholarly journals A case of appendiceal ganglioneuroma in neurofibromatosis type 1

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Tadaaki Shimizu ◽  
Nao Hondo ◽  
Yusuke Miyagawa ◽  
Masato Kitazawa ◽  
Futoshi Muranaka ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Neurofibromatosis Type 1 ◽  
Histopathological Examination ◽  
Submucosal Tumor ◽  
Fibrous Tissue ◽  
Neurofibromatosis Type ◽  
Inherited Disease ◽  
Negative Resection Margin ◽  
Neurogenic Tumors

Abstract Background Neurofibromatosis type 1 is an autosomal dominant inherited disease associated with multiple skin neurofibromas or other neurogenic tumors, such as nodular plexiform neurinoma or cerebrospinal tumor. Gastrointestinal stromal tumors are often complicated in patients with neurofibromatosis type 1, although involvement of the appendix is rare, and there have been few reports of appendiceal ganglioneuroma. Case presentation The patient was a 29-year-old man diagnosed with neurofibromatosis type 1 based on physical findings and his family history. During the follow-up of neurofibromatosis, computed tomography was performed to detect neurological tumors, such as neurofibromas in the brain, spinal cord, and gastrointestinal tract. Computed tomography showed a markedly thickened appendix wall, and an appendiceal tumor was suspected. Laparoscopic appendectomy was performed, and a 50 × 35 mm appendiceal submucosal tumor was resected with a negative resection margin. At histopathological examination, the tumor was diagnosed as ganglioneuroma; it showed short spindle-shaped cells and ganglion cells diffusely infiltrated into the proper muscle layer and fibrous tissue that grew around nerve cells. The patient was discharged on the 5th postoperative day without postoperative complications and was doing well at 13 months following the operation. Conclusions Gastrointestinal stromal tumor and neurofibroma are the most common gastrointestinal tumors associated with neurofibromatosis type 1, but ganglioneuroma of the appendix is rare. Appendiceal neurogenic tumors should be considered in patients with neurofibromatosis type 1, and surgical resection is necessary because of the risk of malignancy.

Psychological disturbance and sleep disorders in children with neurofibromatosis type 1

2005 ◽  
Vol 47 (4) ◽  
pp. 237-242 ◽  
Author(s):  
Hilary Johnson ◽  
Luci Wiggs ◽  
Gregory Stores ◽  
Susan M Huson
Keyword(s):  
Sleep Disorders ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Psychological Disturbance

Social information processing of children with Neurofibromatosis Type 1

2008 ◽  
Author(s):  
Jonathan M. Kurss ◽  
Anna E. Craig ◽  
Jennifer Reiter-Purtill ◽  
Kathryn Vannatta ◽  
Cynthia Gerhardt
Keyword(s):  
Information Processing ◽  
Neurofibromatosis Type 1 ◽  
Social Information Processing ◽  
Social Information ◽  
Neurofibromatosis Type

Lovastatin improves impaired LTP-like plasticity in patients with Neurofibromatosis Type 1

2011 ◽  
Vol 42 (01) ◽  
Author(s):  
F. Mainberger ◽  
N. Jung ◽  
M. Zenker ◽  
I. Delvendahl ◽  
U. Wahlländer ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type

Intima-Type Vasculopathy in Neurofibromatosis Type 1

2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
J. Keppler ◽  
A. Fiedler
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type
Sign in / Sign up

Export Citation Format

Share Document