نتایج جستجو برای: ductal plate malformation

تعداد نتایج: 106447  

Journal: :Journal of Veterinary Medical Science 2016

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2006
Hee Jin Chang So Young Jin Chanil Park Young Nyun Park Ja June Jang Cheol Keun Park Yeon Lim Suh Eunsil Yu Dae Young Kang Han Ik Bae

Mesenchymal hamartoma (MH) of the liver is an uncommon benign lesion related to ductal plate malformation. It is usually cystic and mainly composed of myxoid mesenchymal tissue with tortuous or cystic bile ducts. In order to characterize the clinicopathological features of MH, the Korean Gastrointestinal Pathology Study Group collected a total of 17 MH cases diagnosed in 7 hospitals from 1992 t...

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Journal: :Annals of hepatology 2009
Parviz Tabatabaie Gholam-Hossein Fallahi Fatemeh Farahmand Kambiz Eftekhari Maedeh Ahmadi Faezeh Ahmadi Fatemeh Bazvand Nima Rezaei

Congenital choledochal cyst is malformation of the biliary ductal system, which rarely occur. We describe here a 4-month old boy, who was referred to our center with respiratory distress and low level consciousness. In physical examination, a mass was detected in right upper quadrant of abdomen. Sonographic examination indicated a cystic structure representing the choledochal cyst. Further eval...

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Journal: :International journal of clinical and experimental pathology 2013
Tadashi Terada

In the current WHO blue book, combined hepatocellular-cholangiocarcinoma (C-HCC-CC) was classified into two types; classical type and type with stem cell features. The latter is extremely rare, and is subcategorized into the following three subtypes; typical subtype, intermediate cell subtype, and cholangiocellular subtype. Recently, intrahepatic cholangiocarcinoma (ICC) with features of ductal...

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Journal: :Archives of disease in childhood 1994
H E Hughes S J Davies

The incidence of congenital heart defects in patients with Kabuki syndrome is estimated to be about 30%. To date, no specific type of heart malformation is known to be associated with the syndrome. A further 20 unselected children with Kabuki syndrome are presented. The incidence of heart abnormalities in these children is almost twice that previously reported (55%) and juxta-ductal coarctation...

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2016
Andjenie Madhuban Freek van den Heuvel Margriet van Stuijvenberg

The authors report the case of a neonate presenting with signs of a congenital cardiac disease. Echocardiography showed a structural normal heart, right-to-left ductal flow, a dilated superior caval vein, and reversed diastolic flow in the proximal descending aorta. Brain magnetic resonance imaging showed a vein of Galen arteriovenous malformation. This highlights the importance of considering ...

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Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2003
Yves Jossin Masuhara Ogawa Christine Metin Fadel Tissir André M Goffinet

During development, most cortical neurons migrate to the cortical plate (CP) radially. CP development is abnormal in reeler and other mutant mice with defective Reelin signaling. Reelin is secreted by Cajal-Retzius cells and binds to the very low density lipoprotein receptor and apolipoprotein E receptor type 2 receptors on the surface of CP cells, inducing tyrosine phosphorylation of the intra...

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Journal: :Journal of medical genetics 2010
D Doherty M A Parisi L S Finn M Gunay-Aygun M Al-Mateen D Bates C Clericuzio H Demir M Dorschner A J van Essen W A Gahl M Gentile N T Gorden A Hikida D Knutzen H Ozyurek I Phelps P Rosenthal A Verloes H Weigand P F Chance W B Dobyns I A Glass

OBJECTIVE To identify genetic causes of COACH syndrome BACKGROUND COACH syndrome is a rare autosomal recessive disorder characterised by Cerebellar vermis hypoplasia, Oligophrenia (developmental delay/mental retardation), Ataxia, Coloboma, and Hepatic fibrosis. The vermis hypoplasia falls in a spectrum of mid-hindbrain malformation called the molar tooth sign (MTS), making COACH a Joubert syn...

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Journal: :Comparative Hepatology 2009

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Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2006
Xun Cheng Ching-mei Hsu D Spencer Currle Jia Sheng Hu A James Barkovich Edwin S Monuki

The roof plate is a well known signaling center in CNS development, but its roles in the developing telencephalon and the common holoprosencephaly (HPE) malformation have been uncertain. Using cellular ablations in mice, we show that roof plate cell loss causes failed midline induction and HPE in the dorsal telencephalon. This morphologic phenotype is accompanied by selective deficits in midlin...

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