نتایج جستجو برای: thanatophoric

تعداد نتایج: 254  

Journal: :Journal of prenatal medicine 2008
Adekunle Y Abdulkadir Kabiru Isyaku Akintade Dare Sulaiman G Abdullahi Sule K Idris Abdulkadir M Tabari

OBJECTIVES Thanatophoric dysplasia (TD), the most common of the congenital lethal skeletal dysplasias occurs sporadically in 1/64,000-100,000 live births. To the best of our knowledge, the in utero attitudes and behaviours of such babies with serial ultrasound scans have not been previously described. We present the in utero third trimester sonographic behaviors of TD in a 22-year-old primigra...

Journal: :Human molecular genetics 2009
Takehiko Matsushita William R Wilcox Yuk Yu Chan Aya Kawanami Hülya Bükülmez Gener Balmes Pavel Krejci Pertchoui B Mekikian Kazuyuki Otani Isakichi Yamaura Matthew L Warman David Givol Shunichi Murakami

Activating mutations in FGFR3 cause achondroplasia and thanatophoric dysplasia, the most common human skeletal dysplasias. In these disorders, spinal canal and foramen magnum stenosis can cause serious neurologic complications. Here, we provide evidence that FGFR3 and MAPK signaling in chondrocytes promote synchondrosis closure and fusion of ossification centers. We observed premature synchondr...

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...

2014
Kaicun Wang

Global mean surface air temperature (Ta) has been reported to have risen by 0.74°C over the last 100 years. However, the definition of mean Ta is still a subject of debate. The most defensible definition might be the integral of the continuous temperature measurements over a day (Td0). However, for technological and historical reasons, mean Ta over land have been taken to be the average of the ...

Journal: :Journal of Atherosclerosis and Thrombosis 2007

Journal: :International Journal of Reproduction, Contraception, Obstetrics and Gynecology 2016

2015
Selvi Gülaşı Aytuğ Atıcı Yalçın Çelik

Thanatophoric dysplasia (TD) is a lethal form of skeletal dysplasia with short-limb dwarfism. Two types distinguished with their radiological characteristics have been defined clinically. The femur is curved in type 1, while it is straight in type 2. TD is known to be due to a mutation in the fibroblast growth factor receptor 3 (FGFR3) gene. We report a male patient who showed clinical findings...

2014
Inji Cho Jae-Yoon Shim Gu-Hwan Kim Han-Wook Yoo Eun Jung Lee Hye-Sung Won Pil Ryang Lee Ahm Kim

Thanatophoric dysplasia (TD) is caused by mutation of the gene that encodes fibroblast growth factor 3 (FGFR3). Owing to the poor prognosis for TD, prenatal diagnosis is critical to optimal perinatal management. We report here a case of TD in twin pregnancy, which was prenatally diagnosed by DNA analysis following amniocentesis at 15 weeks, and was managed by selective fetal termination. Prenat...

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