Results 21 to 30 of about 4,442 (232)

Identification of PKD1L1 Gene Variants in Children with the Biliary Atresia Splenic Malformation Syndrome [PDF]

, 2019
Biliary atresia (BA) is the most common cause of end‐stage liver disease in children and the primary indication for pediatric liver transplantation, yet underlying etiologies remain unknown.
Alonso, Estella M, Berauer, John‐Paul, Bezerra, Jorge A, Bull, Laura N, Chopra, Pankaj, Cutler, David J, Finegold, Milton J, Gibbs, Richard A, Guthery, Stephen L, Harpavat, Sanjiv, Hegde, Madhuri R, Kamath, Binita M, Karpen, Saul J, Loomes, Kathleen M, Magee, John C, Mezina, Anya I, Molleston, Jean P, Moore, Barry, Murray, Karen F, Muzny, Donna M, Network, for the Childhood Liver Disease Research, Okou, David T, Perlmutter, David H, Rajagopalan, Ramakrishnan, Rosenthal, Philip, Russo, Pierre, Sabo, Aniko, Sherker, Averell H, Sokol, Ronald J, Spinner, Nancy B, Squires, Robert H, Thompson, Richard J, Wang, Kasper S, Yandell, Mark   +33 more
core   +2 more sources

Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report

The Egyptian Journal of Radiology and Nuclear Medicine, 2022
Background The “heterotaxy syndrome” also called “situs ambiguus” is a rare condition in which the internal organs of the chest and abdomen are abnormally arranged.
Mahrukh Ali, Noman Khan, Jehanzeb Shahid, Samana Zainab Shah, Aimun Raees   +4 more
doaj   +1 more source

Inflammation, Active Fibroplasia, and End-stage Fibrosis in 172 Biliary Atresia Remnants Correlate Poorly With Age at Kasai Portoenterostomy, Visceral Heterotaxy, and Outcome [PDF]

, 2018
Published histologic studies of the hilar plate or entire biliary remnant at the time of Kasai portoenterostomy (KHPE) have not provided deep insight into the pathogenesis of biliary atresia, relation to age at surgery, prognosis or the basis for ...
Arva, Bove, Chen, Davenport, Davenport, De Jong, Feldman, Gautier, Gautier, Gautier, Harpavat, Harpavat, Humphrey, Kolb, Lobeck, Mack, Magalhaes, Mirkin, Nakamura, Petersen, Russo, Sarinet, Schwarz, Shen, Song, Stahlschmidt, Superina, Tan, Tanaka, Volpert, Wong, Yam-Puc, Zheng   +32 more
core   +1 more source

Heterotaxy syndrome

World Journal for Pediatric and Congenital Heart Surgery, 2015
A session dedicated to heterotaxy syndrome was included in the program of the Tenth International Conference of the Pediatric Cardiac Intensive Care Society in Miami, Florida in December 2014. An invited panel of experts reviewed the anatomic considerations, surgical considerations, noncardiac issues, and long-term outcomes in this challenging group ...
Sarah A, Teele, Jeffrey P, Jacobs, William L, Border, Nikhil K, Chanani   +3 more
openaire   +3 more sources

Acute appendicitis in a patient with heterotaxy syndrome

Journal of Pediatric Surgery Case Reports, 2018
Heterotaxy syndrome, also known as situs ambiguous, is a congenital condition that results in an abnormal distribution of visceral organs in the chest and abdomen. Heterotaxy syndrome has generally been categorized into two forms: a left-atrial isomerism
Mariam Selevany, Manrique Guerrero, John A. Perrone, Maryam Hussain   +3 more
doaj   +1 more source

Heterotaxy syndrome: This is the left, right?

South African Journal of Radiology, 2015
Heterotaxy syndrome is a rare and complex disorder of the chest and abdominal organ arrangements, and presents a diagnostic challenge to the radiologist.
Lili Huang, Belinda J. Mitchell, Savvas Andronikou, Zarina I. Lockhat, Farhana Suleman   +4 more
doaj   +1 more source

Outcome of prenatally diagnosed fetal heterotaxy: A systematic review and meta-analysis. [PDF]

, 2017
OBJECTIVES: To assess the perinatal outcomes of fetuses affected by heterotaxy. METHODS: Medline, Embase and Cinhal were searched. Only studies reporting a prenatal diagnosis of isomerism were included.
Abuhamad, Atkinson, Bardin, Baschat, Berg, Bronshtein, Brueckner, Carvalho, Collins, Degenhardt, Ditchfield, Dreux, Egger, Escobar-Diaz, Friedberg, Gaur, Gottschalk, Henderson, Ho, Hoefstaetter, Hunter, Icardo, Jacobs, Kennedy, Kheiri, Kothari, Ladd, Lee, Leeflang, Lim, Lin, Loomba, Loomba, Manzoli, McKiernan, Nakada, Nemec, NHS Centre for Reviews and Dissemination, Ozkutlu, Paladini, Pasquini, Peoples, Pepes, Phoon, Pockett, Shen, Shen, Taketazu, Tan, Tan, Varela-Fascinetto, Yan   +51 more
core   +1 more source

Heterotaxy Syndrome In A Middle-Aged Pakistani Male

Journal of Rawalpindi Medical College, 2023
Background: Heterotaxy syndrome (or situs ambiguous) is an extremely rare disorder in which the viscera are arranged in an abnormally asymmetrical pattern around the midline along with cardiac and spleen abnormalities.
Seema Kanwal, Ahsan Tameez ud Din, Mudassir Shafiq, Farzana Kausar, Asim Tameez Ud Din   +4 more
doaj   +1 more source

Ccdc11 is a novel centriolar satellite protein essential for ciliogenesis and establishment of left-right asymmetry [PDF]

, 2016
The establishment of left–right (L-R) asymmetry in vertebrates is dependent on the sensory and motile functions of cilia during embryogenesis. Mutations in CCDC11 disrupt L-R asymmetry and cause congenital heart disease in humans, yet the molecular and ...
Betleja, Ewelina, John, Emily, Mahjoub, Moe R, Mitchell, Brian J, Moresco, James J, Silva, Erica, Spear, Philip, Yates, John R, III, Zhang, Siwei   +8 more
core   +2 more sources

Thoracoscopic oesophago-oesophagostomy in the prone position for oesophageal stenosis caused by dilated azygos vein in polysplenia-associated heterotaxy

Journal of Minimal Access Surgery, 2021
Background: Heterotaxy syndrome is associated with a plethora of cardiovascular and other multi-system anomalies with a high childhood mortality. A dilated azygos vein as part of the polysplenia variant of heterotaxy syndrome may cause oesophageal ...
Rajesh Bhojwani, Nikhil Jain
doaj   +1 more source