Zobrazeno 1 - 10
of 1 280
pro vyhledávání: '"Herbert. L. Bonkovsky"'
Publikováno v:
JIMD Reports, Vol 64, Iss 2, Pp 146-149 (2023)
Abstract A 47‐year‐old woman with acute intermittent porphyria (AIP) has had recurring symptoms after achieving biochemical normalization of her urinary 5‐aminolevulinic acid (ALA), porphobilinogen (PBG), and total porphyrins with givosiran. Sh
Externí odkaz:
https://doaj.org/article/d0391af819fd4e309bf8952d45148000
Autor:
Cynthia Levy, Karl E. Anderson, Manisha Balwani, Herbert L. Bonkovsky, Amy Dickey, Siobán Keel, Brendan Mcguire, Roy Smith, Manish Thapar, Bruce Wang, William Savage
Publikováno v:
HemaSphere, Vol 7, p e904346e (2023)
Externí odkaz:
https://doaj.org/article/f2e13231cb1848a0a6e7f2207e468328
Publikováno v:
Pharmaceuticals, Vol 17, Iss 1, p 31 (2023)
Erythropoietic protoporphyria (EPP) is a genetic disorder stemming from reduced ferrochelatase expression, the final enzyme in the pathway of heme biosynthesis. A closely related condition, X-linked protoporphyria (XLP), bears similar clinical featur
Externí odkaz:
https://doaj.org/article/b27547bc45c64dba972240cb304b0998
Publikováno v:
Molecular Genetics and Metabolism Reports, Vol 34, Iss , Pp 100946- (2023)
A 39-year-old woman with biochemically and clinically active acute intermittent porphyria (AIP) developed moderately severe liver injury after receiving her second dose of givosiran. Serologic evaluation ruled out hepatitis caused by viral, autoimmun
Externí odkaz:
https://doaj.org/article/eda8bca7f5cc4c3ca34adcc7e9ee6de6
Autor:
Manisha Balwani, Hetanshi Naik, Jessica R. Overbey, Herbert L. Bonkovsky, D. Montgomery Bissell, Bruce Wang, John D. Phillips, Robert J. Desnick, Karl E. Anderson
Publikováno v:
Molecular Genetics and Metabolism Reports, Vol 33, Iss , Pp 100939- (2022)
The use of iron supplementation for anemia in erythropoietic protoporphyria (EPP) is controversial with both benefit and deterioration reported in single case reports. There is no systematic study to evaluate the benefits or risks of iron supplementa
Externí odkaz:
https://doaj.org/article/46347898d81d43eaac3445c801bbcb85
Publikováno v:
Molecular Genetics and Metabolism Reports, Vol 33, Iss , Pp 100915- (2022)
Elagolix is an FDA-approved treatment for moderate-to-severe pain associated with endometriosis but has been associated with increased acute porphyric attacks in women with the acute hepatic porphyrias (AHPs). A fluorescence-based screening assay for
Externí odkaz:
https://doaj.org/article/51c972137d8d4258a83f26ff9db5bf77
Publikováno v:
Frontiers in Pharmacology, Vol 13 (2022)
Eslicarbazepine acetate, a third-generation antiepileptic drug (AED), has shown improved clinical response and safety in comparison to older generation AEDs for patients with partial-onset seizures. It is currently not known whether eslicarbazepine a
Externí odkaz:
https://doaj.org/article/7bd8e5f27e1e47b09717a79a9102f91c
Publikováno v:
Pharmacology Research & Perspectives, Vol 10, Iss 3, Pp n/a-n/a (2022)
Abstract Compounds that induce 5‐aminolevulinic acid [ALA] synthase‐1 and/or cytochromes P‐450 may induce acute porphyric attacks in patients with the acute hepatic porphyrias [AHPs]. Currently, there is no simple, robust model used to assess a
Externí odkaz:
https://doaj.org/article/5ec12b435a7642d281de8de263bf3914
Autor:
Hellen A. Oketch-Rabah, Amy L. Roe, Cynthia V. Rider, Herbert L. Bonkovsky, Gabriel I. Giancaspro, Victor Navarro, Mary F. Paine, Joseph M. Betz, Robin J. Marles, Steven Casper, Bill Gurley, Scott A. Jordan, Kan He, Mahendra P. Kapoor, Theertham P. Rao, Averell H. Sherker, Robert J. Fontana, Simona Rossi, Raj Vuppalanchi, Leonard B. Seeff, Andrew Stolz, Jawad Ahmad, Christopher Koh, Jose Serrano, Tieraona Low Dog, Richard Ko
Publikováno v:
Toxicology Reports, Vol 7, Iss , Pp 386-402 (2020)
As part of the United States Pharmacopeia’s ongoing review of dietary supplement safety data, a new comprehensive systematic review on green tea extracts (GTE) has been completed. GTEs may contain hepatotoxic solvent residues, pesticide residues, p
Externí odkaz:
https://doaj.org/article/d2d9065cabf74b6d8673dea045edc21e
Autor:
Hetanshi Naik, Jessica R. Overbey, Robert J. Desnick, Karl E. Anderson, D. Montgomery Bissell, Joseph Bloomer, Herbert L. Bonkovsky, John D. Phillips, Bruce Wang, Ashwani Singal, Manisha Balwani
Publikováno v:
JIMD Reports, Vol 50, Iss 1, Pp 9-19 (2019)
Abstract Background Erythropoietic protoporphyria (EPP) and X‐linked Protoporphyria (XLP) are rare photodermatoses presenting with severe phototoxicity. Although anecdotally, providers who treat EPP patients acknowledge their life‐altering effect
Externí odkaz:
https://doaj.org/article/8ff7d0c724624c68a6b8a8340f7f3b3d