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Human iPSC-derived hepatocyte system models cholestasis with tight junction protein 2 deficiency.
Li CZ, Ogawa H, Ng SS, Chen X, Kishimoto E, Sakabe K, Fukami A, Hu YC, Mayhew CN, Hellmann J, Miethke A, Tasnova NL, Blackford SJI, Tang ZM, Syanda AM, Ma L, Xiao F, Sambrotta M, Tavabie O, Soares F, Baker O, Danovi D, Hayashi H, Thompson RJ, Rashid ST, Asai A. Li CZ, et al. Among authors: miethke a. JHEP Rep. 2022 Feb 1;4(4):100446. doi: 10.1016/j.jhepr.2022.100446. eCollection 2022 Apr. JHEP Rep. 2022. PMID: 35284810 Free PMC article.
Maralixibat for the treatment of PFIC: Long-term, IBAT inhibition in an open-label, Phase 2 study.
Loomes KM, Squires RH, Kelly D, Rajwal S, Soufi N, Lachaux A, Jankowska I, Mack C, Setchell KDR, Karthikeyan P, Kennedy C, Dorenbaum A, Desai NK, Garner W, Jaecklin T, Vig P, Miethke A, Thompson RJ. Loomes KM, et al. Among authors: miethke a. Hepatol Commun. 2022 Sep;6(9):2379-2390. doi: 10.1002/hep4.1980. Epub 2022 May 4. Hepatol Commun. 2022. PMID: 35507739 Free PMC article. Clinical Trial.
Impact of long-term administration of maralixibat on children with cholestasis secondary to Alagille syndrome.
Shneider BL, Spino CA, Kamath BM, Magee JC, Ignacio RV, Huang S, Horslen SP, Molleston JP, Miethke AG, Kohli R, Leung DH, Jensen MK, Loomes KM, Karpen SJ, Mack C, Rosenthal P, Squires RH, Baker A, Rajwal S, Kelly D, Sokol RJ, Thompson RJ; for ChiLDReN and UK IMAGO/IMAGINE Investigators. Shneider BL, et al. Among authors: miethke ag. Hepatol Commun. 2022 Aug;6(8):1922-1933. doi: 10.1002/hep4.1992. Epub 2022 Jun 7. Hepatol Commun. 2022. PMID: 35672955 Free PMC article.
Maralixibat in progressive familial intrahepatic cholestasis (MARCH-PFIC): a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial.
Miethke AG, Moukarzel A, Porta G, Covarrubias Esquer J, Czubkowski P, Ordonez F, Mosca A, Aqul AA, Squires RH, Sokal E, D'Agostino D, Baumann U, D'Antiga L, Kasi N, Laborde N, Arikan C, Lin CH, Gilmour S, Mittal N, Chiou FK, Horslen SP, Huber WD, Jaecklin T, Nunes T, Lascau A, Longpre L, Mogul DB, Garner W, Vig P, Hupertz VF, Gonzalez-Peralta RP, Ekong U, Hartley J, Laverdure N, Ovchinsky N, Thompson RJ. Miethke AG, et al. Lancet Gastroenterol Hepatol. 2024 May 6:S2468-1253(24)00080-3. doi: 10.1016/S2468-1253(24)00080-3. Online ahead of print. Lancet Gastroenterol Hepatol. 2024. PMID: 38723644
Customized Postoperative Therapy Improves Bile Drainage in Biliary Atresia: A Single Center Preliminary Report.
Pandurangi S, Kim S, Asai A, Bondoc A, Balistreri W, Campbell K, Miethke A, Peters A, Rogers M, Taylor A, Attia SL, Gibbons T, Mullapudi B, Sheridan R, Tiao G, Bezerra JA. Pandurangi S, et al. Among authors: miethke a. J Pediatr Surg. 2023 Aug;58(8):1483-1488. doi: 10.1016/j.jpedsurg.2022.10.050. Epub 2022 Nov 5. J Pediatr Surg. 2023. PMID: 36496264 Free PMC article.
Longitudinal Outcomes in Young Patients with Alpha-1-Antitrypsin Deficiency with Native Liver Reveal that Neonatal Cholestasis is a Poor Predictor of Future Portal Hypertension.
Teckman J, Rosenthal P, Hawthorne K, Spino C, Bass LM, Murray KF, Kerkar N, Magee JC, Karpen S, Heubi JE, Molleston JP, Squires RH, Kamath BM, Guthery SL, Loomes KM, Sherker AH, Sokol RJ; ChiLDReN (Childhood Liver Disease Research Network). Teckman J, et al. J Pediatr. 2020 Dec;227:81-86.e4. doi: 10.1016/j.jpeds.2020.07.031. Epub 2020 Jul 11. J Pediatr. 2020. PMID: 32663593 Free PMC article.
86 results