Results 21 to 30 of about 15,555 (178)

The bile duct ligated rat : a relevant model to study muscle mass loss in cirrhosis [PDF]

, 2016
Muscle mass loss and hepatic encephalopathy (complex neuropsychiatric disorder) are serious complications of chronic liver disease (cirrhosis) which impact negatively on clinical outcome and quality of life and increase mortality.
Bosoi, Cristina R., Bémeur, Chantal, Deutz, Nicolaas E. P., Macedo de Oliveira, Mariana, Ochoa-Sanchez, Rafael, Rose, Christopher, Ten Have, Gabriella A., Tremblay, Mélanie   +7 more
core   +1 more source

Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy. [PDF]

, 2014
Hyperammonemia is less severe in arginase 1 deficiency compared with other urea cycle defects. Affected patients manifest hyperargininemia and infrequent episodes of hyperammonemia. Patients typically suffer from neurological impairment with cortical and
Cantero, G, Cantero-Nieto, G, Cederbaum, SD, Chan, E, Hu, C, Lipshutz, GS, Park, H, Tai, DS, Yudkoff, M   +8 more
core   +2 more sources

Hyperornithinemia, Hyperammonemia, and Homocitrullinuria Syndrome Causing Severe Neonatal Hyperammonemia [PDF]

, 2018
Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome (OMIM 238970) is an autosomal recessive disorder that is caused by a deficiency of mitochondrial ornithine transporter 1, resulting in dysfunction of the urea cycle. HHH is the rarest of the urea cycle disorders, reported in fewer than 100 patients.
Katherine Taylor, Wild, Rebecca D, Ganetzky, Marc, Yudkoff, Lynne, Ierardi-Curto   +3 more
openaire   +2 more sources

Bariatric Surgery Causing Hyperammonemia [PDF]

Cureus, 2019
Bariatric surgery is recognized as a highly effective therapy for obesity but it does carry a risk of short term and long term complications since it results in a permanent alteration of the patient's anatomy. We present a case of 45-year-old female presented with a macular rash on extremities and facial rash from a rehabilitation center after having ...
Krishnan, Prathik, Ramadas, Poornima, Landsberg, David   +2 more
openaire   +2 more sources

Restoring Ureagenesis in Hepatocytes by CRISPR/Cas9-mediated Genomic Addition to Arginase-deficient Induced Pluripotent Stem Cells. [PDF]

, 2016
Urea cycle disorders are incurable enzymopathies that affect nitrogen metabolism and typically lead to hyperammonemia. Arginase deficiency results from a mutation in Arg1, the enzyme regulating the final step of ureagenesis and typically results in ...
Angarita, Stephanie Ak, Byrne, James A, Cederbaum, Stephen D, Chang, Katherine M, Gilmore, W Blake, Hermann, Kip, Lam, Alex K, Lee, Patrick C, Lipshutz, Gerald S, Pyle, April D, Schoenberg, Benjamen E, Tang, Jonathan K, Truong, Brian, Vega-Crespo, Agustin, Wininger, Austin E   +14 more
core   +3 more sources

Orthotopic liver transplantation for urea cycle enzyme deficiency [PDF]

, 1992
Hyperammonemia, abnormalities in plasma amino acids and abnormalities of standard liver functions were corrected by orthotopic liver transplantation in a 14‐day‐old boy with carbamyl phosphate synthetase‐I deficiency and in a 35‐yr‐old man with ...
Bachmann, Broelsch, Brusilow, Brusiow, Featherston, Green, Largilliere, Moore, Nuzum, Roth, Saheki, Starzl, Starzl, Voorhies, Windmueller, Wood, Wood   +16 more
core   +1 more source

New Aspects of Thromboangiitis obliterans (von Winiwarter-Buerger's Disease) [PDF]

, 1984
The existence of thromboangiitis obliterans as a clinical entity has been a matter of debate for many years. In contrast to other immunovasculitides there is no organ involvement while peripheral vessels are affected.
Berlit, Peter, Kessler, C., Krause, Klaus-Henning, Reuther, R.   +3 more
core   +1 more source

Asparaginase-associated hyperammonemia [PDF]

Haematologica
Asparaginase is an essential drug in the treatment of acute lymphoblastic leukemia, and discontinuation of asparaginase therapy due to clinical toxicity or silent inactivation may lead to reduced event-free survival. Common toxicities include hypersensitivity reactions, acute pancreatitis, thrombosis, hepatotoxicity, and hyperlipidemia.
Raja, Raheel Altaf, Als-Nielsen, Bodil, Lund, Allan M., Vilstrup, Hendrik, Dalhoff, Kim Peder, Schmiegelow, Kjeld   +5 more
openaire   +3 more sources

Potent Liver‐Tropic mRNA Lipid Nanoparticles: ApoE‐Mediated Delivery Through a Low‐Density Lipoprotein Receptor Independent Uptake Mechanism

Advanced Materials, EarlyView.
Helper and ionizable lipids play a crucial role in determining ApoE binding and subsequent liver tropism and LDLR‐mediated uptake. Ionizable lipids primarily govern the LDLR‐independent uptake pathway. This complementary interplay between lipid components ultimately governs LNP delivery performance and therapeutic efficacy in the liver.
Ashish Sarode, Christian Ortiz, Tadeh Derstepanian, Natalia Vargas‐Montoya, Priyal Patel, Nikita Khadse, Saikat Manna, Ryan Landis, Joseph Skaleski, Lianne Boeglin, Hongfeng Deng, Anusha Dias, Hong Wang, Debora Barreiros Petropolis, Barak Yahalom, Shrirang Karve, Frank DeRosa   +16 more
wiley   +1 more source

Systemic oxidative stress is implicated in the pathogenesis of brain edema in rats with chronic liver failure [PDF]

, 2012
Chronic liver failure leads to hyperammonemia, a central component in the pathogenesis of hepatic encephalopathy (HE); however, a correlation between blood ammonia levels and HE severity remains controversial. It is believed oxidative stress plays a role
Bosoi, Cristina R., Huynh, Jimmy, Jiang, Wenlei, Parent-Robitaille, Christian, Rose, Christopher, Tremblay, Mélanie, Yang, Xiaoling   +6 more
core   +1 more source