Results 21 to 30 of about 5,571 (281)

Heterotaxy: fluctuat nec mergitur [PDF]

Cardiology in the Young, 2021
AbstractThe International Pediatric and Congenital Cardiac Code (IPCCC) states thatvisceral heterotaxyis defined as “a congenital malformation in which the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, in congenital cardiology, heterotaxy syndrome does not include patients with
Lucile Houyel, Anne Moreau de Bellaing, Damien Bonnet   +2 more
openaire   +2 more sources

Heterotaxy Syndrome with Polysplenia, Fused Adrenal Glands, and Diabetes Mellitus

Clinical Medicine Insights: Cardiology, 2022
Heterotaxy syndrome is a rare congenital heart disease with a disarrangement of the heart and abdominal organs. We present a young African female with features of heart failure, diffuse irregular cardiac murmurs, and palpable, tender epigastric mass.
Abid M Sadiq, Adnan M Sadiq
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

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

Noncompaction cardiomyopathy and heterotaxy syndrome [PDF]

, 2017
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD).
Martinez, Hugo R., Parent, John J., Schamberger, Marcus S., Ware, Stephanie M.   +3 more
core   +1 more source

A catheter-based interventional strategy redirects hepatic vein flows after Fontan procedure in left isomerism to treat severe hypoxemia

Annals of Pediatric Cardiology, 2022
Pulmonary arteriovenous malformations after the Kawashima procedure causing severe hypoxemia are treated by Fontan surgery that redirects hepatic venous blood to the pulmonary circulation.
Kothandam Sivakumar
doaj   +1 more source

Discordant Post-natal Patterns in Fetuses With Heterotaxy Syndrome: A Retrospective Single-Centre Series on Outcome After Fetal Diagnosis

Frontiers in Pediatrics, 2022
ObjectiveCardiac and extra-cardiac anomalies in 46 pre-natally diagnosed cases of heterotaxy were compared to post-natal anatomical patterns in order to reveal discordant findings.
Elisabeth Seidl-Mlczoch, Gregor Kasprian, Erwin Kitzmueller, Daniel Zimpfer, Irene Steiner, Victoria Jowett, Marlene Stuempflen, Alice Wielandner, Barbara Ulm, Ina Michel-Behnke   +9 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

Heterotaxy

Journal of Marine Medical Society, 2022
Heterotaxy syndrome is an uncommon disease, with an incidence ranging from 1 in 6000 to 1 in 20,000 live births.[1] This syndrome is characterized by major cardiac vascular malformations, congenital asplenia, polysplenia, and abnormal arrangement of the chest and abdominal organs.
openaire   +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