Results 61 to 70 of about 226,924 (326)

Differential diagnosis of neonatal cholestasis by genetic testing: A case report

Journal of Pediatric Surgery Case Reports, 2023
Introduction: Alagille syndrome (ALGS) is a cholestasis disorder with multiple organ dysfunction, including heart and kidney. The causative genes of ALGS are JAG1 and NOTCH2.
Sakura Kawahara, Kazuo Imagawa, Shusuke Takeuchi, Tomoya Iwasaki, Yuki Okada, Yuri Nakamura, Shogo Saito, Takato Sasaki, Kouji Masumoto, Hidetoshi Takada   +9 more
doaj   +1 more source

Cyclosporine Absorption Following Orthotopic Liver Transplantation [PDF]

, 1986
Blood concentrations of cyclosporine were determined in adult and pediatric patients following orthotopic liver transplantation to quantitate cyclosporine blood clearance and oral absorption.
Beveridge, Burckart, Burckart, Calne, Calne, Holt, Kahan, Kennedy, Malatack, Miles, Ptachcinski, Ptachcinski, Ptachcinski, Sawchuk, Starzl, Starzl, Starzl, Venkataramanan, Venkataramanan, Venkataramanan, Wood   +20 more
core   +2 more sources

Liver transplantation before 1 year of age [PDF]

, 1987
Since 1981, 20 infants younger than 1 year of age received 26 orthotopic liver transplants. Immunosuppression was with cyclosporine and corticosteroids. Thirteen (65%) of the reciplents were discharged from the hospital.
Ascher, Baburao Koneru, Carlos O. Esquivel, Frederick Karrer, Gartner, Gordon, Iwatsuki, Kasai, Leonard Makowka, Robert D. Gordon, Rosenthal, Satoru Todo, Shunzaburo Iwatsuki, Starzl, Starzl, Starzl, Starzl, Thomas E. Starzl, Wallis J. Marsh   +18 more
core   +1 more source

Overview of Biliary Atresia

대한영상의학회지, 2022
Biliary atresia is a progressive, idiopathic, obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. It is the most common indication for liver transplantation in children.
Tae Yeon Jeon
doaj   +1 more source

Development and Validation of a Machine Learning–Based Prediction Model for Detection of Biliary Atresia

Gastro Hep Advances, 2023
Background and Aims: Biliary atresia is a rare and devastating bile duct disease that occurs during the neonatal period. Timely identification and prompt surgical intervention is critical for improving the outcome.
Ho Jung Choi, Yeong Eun Kim, Jung-Man Namgoong, Inki Kim, Jun Sung Park, Woo Im Baek, Byong Sop Lee, Hee Mang Yoon, Young Ah Cho, Jin Seong Lee, Jung Ok Shim, Seak Hee Oh, Jin Soo Moon, Jae Sung Ko, Dae Yeon Kim, Kyung Mo Kim   +15 more
doaj   +1 more source

Obliterative cholangiopathy in acquired cystic biliary atresia type III after cyst perforation: a case report

BMC Pediatrics, 2018
Background In biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration.
Tsugumichi Koshinaga, Kensuke Ohashi, Kakou Ono, Hide Kaneda, Takeshi Furuya   +4 more
doaj   +1 more source

Association of biliary atresia with jejuno-ileal atresia: Case report

Journal of Pediatric Surgery Case Reports, 2023
Background: Jejuno‐ileal atresia (JIA) is a congenital anomaly characterized clinically by bilious vomiting and abdominal distension. The incidence of JIA is approximately between 1 in 5000 live births in different parts of the world.
Amru Abulkhair, Adel Albiety, Petros Valioulis, Khalid Eibani, Eshraq Omar, Amjad Tala't, Reem Mosalli, Omar Mansour   +7 more
doaj   +1 more source

Variants in AKR1D1 and Infant Mortality: Should Bile Acid Screening be a Routine Part of Newborn Screening?

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Biallelic pathogenic variants in AKR1D1 cause Δ4‐3‐oxosteroid 5β‐reductase deficiency, disrupt bile acid synthesis, and result in Congenital Bile Acid Synthesis defect type 2 (CBAS2). CBAS2 presents in infancy with cholestasis, coagulopathy, and failure to thrive.
Jade Hudson, Stephanie Hyunh, Bojana Rakic, Cornelius Boerkoel   +3 more
wiley   +1 more source

Embryonic Biliary Atresia in a Very-low-birth-weight Premature Infant

Journal of the Formosan Medical Association, 2007
Two major forms of biliary atresia, the embryonic and perinatal type, are considered to have different pathogeneses and distinct prognoses. Embryonic biliary atresia is associated with worse prognosis.
Hung-Wen Chen, Wen-Ming Hsu, Mei-Hwei Chang, Chien-Yi Chen, Hung-Chieh Chou, Po-Nien Tsao, Wu-Shiun Hsieh   +6 more
doaj   +1 more source

Biliary Atresia

Seminars in Pediatric Surgery, 2000
Although the prognosis of biliary atresia has been dramatically improved in the era of liver transplantation, the Kasai operation is still the first line of surgical treatment. Successful hepatic portoenterostomy depends on early diagnosis and surgery, adequate surgical technique, prevention of cholangitis, and precise postoperative management.
M, Nio, R, Ohi
openaire   +2 more sources