Results 21 to 30 of about 2,415 (196)

Long‐term clinical and socioeconomic outcomes of children with biliary atresia

JGH Open, Volume 7, Issue 12, Page 841-847, December 2023., 2023
With improved medical facilities, more children of biliary atresia are surviving with their native livers and require lifelong follow‐up by adult services for better outcome. Abstract Background Biliary atresia (BA) is rare liver disease of unknown etiology, and is a major indication for liver transplant (LT).
Javaid Sadiq, Carla Lloyd, James Hodson, Maria Trapero Marugan, James Ferguson, Khalid Sharif, Darius F Mirza, Gideon Hirschfield, Deirdre Kelly   +8 more
wiley   +1 more source

Atrioventricular canal defect and genetic syndromes: the unifying role of sonic hedgehog [PDF]

, 2019
The atrioventricular canal defect (AVCD) is a congenital heart defect (CHD) frequently associated with extracardiac anomalies (75%). Previous observations from a personal series of patients with AVCD and "polydactyly syndromes" showed that the distinct ...
Baban, A, Calcagni, G, Dallapiccola, B, De Luca, A, Dentici, M L, Digilio, M C, Marino, B, Pugnaloni, F, Tartaglia, M, Versacci, P   +9 more
core   +1 more source

Further examination of behavior during extinction‐based treatment of pediatric food refusal

Behavioral Interventions, Volume 38, Issue 4, Page 1-28, November 2023., 2023
Abstract Researchers have demonstrated the effectiveness of extinction‐based treatments to reduce challenging behavior. Although bursts and temporary increases in emotional responding may occur in some basic and applied studies, recent studies on their prevalence have shown that side effects are far from ubiquitous.
Christopher W. Engler, Vivian F. Ibañez, Kathryn M. Peterson, Ashley S. Andersen   +3 more
wiley   +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

Biliary atresia and posterior fossa bleed: Chance or causality. A case report and review of the literature

Clinical Case Reports, Volume 11, Issue 11, November 2023., 2023
Biliary atresia Key Clinical Message A newborn with a rare form of biliary atresia had posterior fossa bleed and subarachnoid hemorrhage despite vitamin K prophylaxis, indicating biliary atresia is a causality rather than chance. Abstract Biliary atresia frequently causes surgical jaundice, resulting in delayed vitamin K deficiency.
Susmin Karki, Vikash Chand, Asmita Parajuli, Sushil Kumar Shilpakar, Prakash Regmi, Kabi Raj Bhusal   +5 more
wiley   +1 more source

Polysplenia syndrome with complex heart disease and jejunal atresia with malrotation in neonate: A case report

Clinical Case Reports, 2020
Polysplenia is heterotaxy syndrome or bilateral left‐sidedness. We report a case of polysplenia syndrome in order to draw attention to this rare syndrome that must be excluded in an infant presenting with congenital heart disease and intestinal ...
Roya Arif Huseynova, Latifa A. Bin Mahmoud, Adli Abdelrahim, Majeed A. Alroiedy, Ogtay Huseynov   +4 more
doaj   +1 more source

Polysplenia and other anatomical variants of the spleen

Journal of Education, Health and Sport, 2022
The anatomy, physiology and embryology of the spleen are essential fields of study for the determination of congenital varieties as well as the pathological processes occurring in this organ.The aim of this study is to summarize the current knowledge on
Agnieszka Kopystecka, Justyna Lipińska, Izabela Kopeć, Łukasz Lipiński, Magdalena Woźniak, Grzegorz Jędrzejewski   +5 more
doaj   +1 more source

Fetal heterotaxy with tricuspid atresia, pulmonary atresia, and isomerism of the right atrial appendages at 22 weeks. [PDF]

, 2013
We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex ...
Richardson, Randy R, Silverman, Norman H, Solomon, Julia E, Stock, John H   +3 more
core   +2 more sources

Exome sequencing findings in children with annular pancreas

Molecular Genetics &Genomic Medicine, Volume 11, Issue 10, October 2023., 2023
Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Several theories currently exist. We conducted exome sequencing in 115 affected infants and identified missense variants in two genes IQGAP1 and NRCAM.
Georgia Pitsava, Nathan Pankratz, John Lane, Wei Yang, Shannon Rigler, Gary M. Shaw, James L. Mills   +6 more
wiley   +1 more source

Polysplenia syndrome with situs ambiguous, common mesentery, and IVC interruption discovered incidentally in an adult

Radiology Case Reports, 2019
Polysplenia syndrome associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary malformations. Polysplenia has been described mainly in childhood owing to critical anatomic malformations related to ...
Hajar El Mortaji, Kenza Elatiqi, Hanane El Hammaoui, Soumaya Alj   +3 more
doaj   +1 more source