نتایج جستجو برای: craniosynostosis

تعداد نتایج: 2854  

Journal: :AJNR. American journal of neuroradiology 1994
M W Vannier T K Pilgram J L Marsh B B Kraemer S C Rayne M H Gado C J Moran W H McAlister G D Shackelford R A Hardesty

PURPOSE To measure diagnostic performance and preference of two three-dimensional CT reconstruction modalities (voxel-gradient and surface-projection) displayed two ways (conventional and unwrapped) in craniosynostosis confirmed by surgical inspection and histologic analysis of resected sutures. METHODS High-resolution 2-mm contiguous CT sections were obtained and three-dimensional reconstruc...

Journal: :Orphanet Journal of Rare Diseases 2006
Annick Vogels Jean-Pierre Fryns

Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three...

2017
Dongzhe Song Fugui Zhang Russell R Reid Jixing Ye Qiang Wei Junyi Liao Yulong Zou Jiaming Fan Chao Ma Xue Hu Xiangyang Qu Liqun Chen Li Li Yichun Yu Xinyi Yu Zhicai Zhang Chen Zhao Zongyue Zeng Ruyi Zhang Shujuan Yan Tingting Wu Xingye Wu Yi Shu Jiayan Lei Yasha Li Wenwen Zhang Jia Wang Michael J Lee Jennifer Moriatis Wolf Dingming Huang Tong-Chuan He

The cranial suture complex is a heterogeneous tissue consisting of osteogenic progenitor cells and mesenchymal stem cells (MSCs) from bone marrow and suture mesenchyme. The fusion of cranial sutures is a highly coordinated and tightly regulated process during development. Craniosynostosis is a congenital malformation caused by premature fusion of cranial sutures. While the progenitor cells deri...

Journal: :Endocrine reviews 2000
Z Vajo C A Francomano D J Wilkin

Achondroplasia, the most common form of short-limbed dwarfism in humans, occurs between 1 in 15,000 and 40,000 live births. More than 90% of cases are sporadic and there is, on average, an increased paternal age at the time of conception of affected individuals. More then 97% of persons with achondroplasia have a Gly380Arg mutation in the transmembrane domain of the fibroblast growth factor rec...

Journal: :Journal of medical genetics 1997
W Reardon D Wilkes P Rutland L J Pulleyn S Malcolm J C Dean R D Evans B M Jones R Hayward C M Hall N C Nevin M Baraister R M Winter

Several mutations involving the fibroblast growth factor receptor (FGFR) gene family have been identified in association with phenotypically distinct forms of craniosynostosis. One such point mutation, resulting in the substitution of proline by arginine in a critical region of the linker region between the first and second immunoglobulin-like domains, is associated with highly specific phenoty...

Journal: :Journal of Medical Genetics 1998

Journal: :Archives of Disease in Childhood 1987

Journal: : 2023

Objective: In our study, it is aimed to examine the effect of surgery on brain perfusion with ASL sequence MRI taken before and 12 weeks after in cases non-syndromic craniosynostosis treated clinic. Materials Methods: The study was conducted between 28.12.2021-16.09.2022 10 patients who evaluated preoperatively surgically at Neurosurgery Clinic Ankara City Hospital. Perfusion performed postoper...

Journal: :Journal of visual communication in medicine 2005
William A Peña Anne Slavotinek Snehlata Oberoi

Saethre-Chotzen syndrome (acrocephalosyndactyly type III) is a craniosynostosis syndrome inherited in an autosomal dominant manner. Although similar to the other craniosynostosis syndromes in its clinical presentation, this syndrome is caused by a mutation in the TWIST1 gene. The TWIST1 gene product is a transcription factor containing a basic helix-loop-helix (bHLH) domain important in the dev...

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