Miethke Lab

Publications

Kudira, R; Yang, ZF; Osuji, I; Damen, MSMA; Yang Vom Hofe, A; Singh, M; Karns, R; Bariya, P; Pfuhler, L; Mullen, M; Subramanian, S; Salomonis, N; Miraldi, ER; Miethke, AG. Bile acids engage the SIPR-STAT3 signaling axis to modulate regulatory T cell responses in fibrosing cholangiopathies. Journal of Hepatology. 2025; 83(5):1128-1141.

White, SM; Wondergem, AP; Breet, I; Dittmaier, M; Bell, K; Richmond, CM; Hardikar, W; Bhatia, K; Quinlan, C; Orchard, D; Miethke, AG; Gregersen, PA; Phillips, E; Luijsterburg, MS. A new multisystem ERCC1-hepatorenal syndrome: insights from a clinical cohort, molecular pathogenesis, and management guidelines. European Journal of Human Genetics. 2025; 33(10):1252-1263.

Luo, Y; Fraser, L; Jezykowski, J; Gupta, NA; Miethke, AG; Taylor, SA; Alonso, EM; Horslen, S; Kohli, R; Molleston, JP; Rosenthal, P; Valentino, P; Sokol, RJ; Mack, CL. Interleukin 8-CXCR2-mediated neutrophil extracellular trap formation in biliary atresia associated with neutrophil extracellular trap-induced stellate cell activation. Hepatology. 2025; 82(3):552-565.

Schwartz, TS; Mouzaki, M; Berklite, L; Lopez-Nunez, OF; Miethke, A; Xanthakos, SA; Vitale, DS. Pediatric endoscopic ultrasound-guided liver biopsy: 3-year experience. Journal of Pediatric Gastroenterology and Nutrition. 2025; 80(5):920-925.

Debnath, P; Ata, NKA; Cao, JY; Lala, SV; Malik, A; Riedesel, EL; Schooler, GR; Shet, NS; Spence, LH; Stanescu, AL; Khendek, L; Miethke, AG; Trout, AT; Dillman, JR. Magnetic resonance cholangiopancreatography for suspected cholangiopathy in children and young adults: a multi-reader agreement study. Pediatric Radiology. 2025; 55(3):479-487.

D'antiga, L; Thompson, RJ; Horslen, SP; Mogul, DB; Nunes, T; Garner, W; Vig, P; Miethke, AG. The relationship between serum bile acids and event-free survival following the use of maralixibat for progressive familial intrahepatic cholestasis: data from MARCH/MARCH-ON. Digestive and Liver Disease. 2025; 57:s67-s68.

Khendek, L; Castro-Rojas, C; Nelson, C; Alquraish, M; Karns, R; Kasten, J; Teng, X; Miethke, AG; Taylor, AE. Quantitative fibrosis identifies biliary tract involvement and is associated with outcomes in pediatric autoimmune liver disease. Hepatology Communications. 2025; 9(1).

Shneider, BL; Goodrich, NP; Ye, W; Molleston, JP; Leung, DH; Sokol, RJ; Cavallo, L; Wang, K; Valentino, PL; Teckman, JH; Jensen, MK; Bezerra, JA; Bass, LM; Magee, JC. Prospective Multicenter Longitudinal Measurement of Liver Stiffness in School-Age Children With Cholestatic Liver Disease. Gastro Hep Advances. 2025; 4(10):100788.

Fleifil, Y; Gulati, R; Jennings, K; Miethke, A; Bondoc, A; Tiao, G; Geller, JI; Karns, R; Timchenko, L; Timchenko, N. DNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways. Cancers. 2024; 17(1).

Rudman-Melnick, V; Vanhoutte, D; Stowers, K; Sargent, M; Adam, M; Ma, Q; Perl, AKT; Miethke, AG; Burg, A; Shi, T; Hildeman, DA; Woodle, ESS; Kofron, JM; Devarajan, P. Gucy1α1 specifically marks kidney, heart, lung and liver fibroblasts. Scientific Reports. 2024; 14(1):29307.

Dillman, JR; Trout, AT; Taylor, AE; Khendek, L; Kasten, JL; Sheridan, RM; Sharma, D; Karns, RA; Castro-Rojas, C; Zhang, B; Miethke, AG. Association Between MR Elastography Liver Stiffness and Histologic Liver Fibrosis in Children and Young Adults With Autoimmune Liver Disease. American Journal of Roentgenology. 2024; 223(1):e2431108.

Miethke, AG; Moukarzel, A; Porta, G; Covarrubias Esquer, J; Czubkowski, P; Ordonez, F; Mosca, A; Aqul, AA; Squires, RH; Sokal, E; Hartley, J; Laverdure, N; Ovchinsky, N; Thompson, RJ. Maralixibat in progressive familial intrahepatic cholestasis (MARCH-PFIC): a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial. The Lancet Gastroenterology and Hepatology. 2024; 9(7):620-631.

Wang, YM; Kaj-Carbaidwala, B; Lane, A; Agarwal, S; Beier, F; Bertuch, A; Borovsky, KA; Brennan, SK; Calado, RT; Catto, LFB; Simonetto, DA; Ziegler, DS; Miethke, AG; Myers, KC. Liver disease and transplantation in telomere biology disorders: An international multicenter cohort. Hepatology Communications. 2024; 8(7).

Schwartz, TS; Mouzaki, M; Berklite, L; Lopez-Nunez, O; Miethke, A; Xanthakos, S; Vitale, DS. Su2029 INTERVENTIONAL RADIOLOGY VS. ENDOSCOPIC ULTRASOUND GUIDED LIVER BIOPSIES IN PEDIATRIC MASLD. Gastroenterology. 2024; 166(5):s-898.

Kocoshis, TS; Kalfa, TA; Miethke, AG; Balistreri, WF; Seu, KG; Slaughter, CG; Singh, R; Mullen, M; Kocoshis, SA. Red cell abnormalities characterized by ektacytometry in children with cholestasis. Pediatric Research. 2024; 95(4):1035-1040.

Pandurangi, S; Malik, A; Owens, J; Valencia, CA; Miethke, AG. Deleterious variants in TNFAIP3 are associated with type II and seronegative pediatric autoimmune hepatitis. Journal of Hepatology. 2024; 80(1):e26-e28.

Van Hove, JLK; Friederich, MW; Strode, DK; Van Hove, RA; Miller, KR; Sharma, R; Shah, H; Estrella, J; Gabel, L; Horslen, S; Valentino, PL; Magee, JC; Sundaram, SS; Sokol, RJ. Protein biomarkers GDF15 and FGF21 to differentiate mitochondrial hepatopathies from other pediatric liver diseases. Hepatology Communications. 2024; 8(1).

Coppola, T; Hughes, H; Finch, P; Hess, J; Wu, S; Prada, C; Miethke, AG; Zhang, W; Kalfa, TA. Case Report: Childhood Erythrocytosis due to Hypermanganesemia Caused by Homozygous SLC30A10 Mutation. Frontiers in Hematology. 2024; 3.

Boster, JM; Goodrich, NP; Spino, C; Loomes, KM; Alonso, EM; Kamath, BM; Sokol, RJ; Karpen, S; Miethke, A; Shneider, BL; Valentino, PL; Teckman, JH; Hangartner, TN; Sundaram, SS. Sarcopenia is associated with osteopenia and impaired quality of life in children with genetic intrahepatic cholestatic liver disease. Hepatology Communications. 2023; 7(11).

Pandurangi, S; Kim, S; Asai, A; Bondoc, A; Balistreri, W; Campbell, K; Miethke, A; Peters, A; Rogers, M; Taylor, A; Mullapudi, B; Sheridan, R; Tiao, G; Bezerra, JA. Customized Postoperative Therapy Improves Bile Drainage in Biliary Atresia: A Single Center Preliminary Report. Journal of Pediatric Surgery. 2023; 58(8):1483-1488.

Peters, AL; Kim, S; Mourya, R; Asai, A; Taylor, A; Rogers, M; Campbell, K; Fei, L; Miethke, A; Balistreri, WF; Bezerra, JA. Recent Increase in Incidence of Severe Acute Hepatitis of Unknown Etiology in Children is Associated with Infection with Adenovirus and Other Nonhepatotropic Viruses. Journal of Pediatrics. 2023; 259:113439.

Khan, M; Klepper, C; Orkin, S; Arce-Clachar, AC; Bramlage, K; Fei, L; Miethke, A; Kohli, R; Xanthakos, S; Mouzaki, M. Presence of Alpha 1 Antitrypsin Risk Variants is Not Associated With Histologic Severity of Pediatric NAFLD. Journal of Pediatric Gastroenterology and Nutrition. 2023; 77(2):166-170.

Trout, AT; Taylor, AE; Kasten, JL; Castro-Rojas, C; Mahalingam, N; Miethke, AG; Dillman, JR. Corrected T1 Mapping in Children and Young Adults With Autoimmune Liver Disease: Correlation With Histology. American Journal of Roentgenology. 2023; 220(6):901-902.

Mahalingam, N; Trout, AT; Zhang, B; Castro-Rojas, C; Miethke, AG; Dillman, JR. Longitudinal changes in quantitative magnetic resonance imaging metrics in children and young adults with autoimmune liver disease. Abdominal Radiology. 2023; 48(6):1933-1944.

Squires, JE; Miethke, AG; Valencia, CA; Hawthorne, K; Henn, L; Van Hove, JLK; Squires, RH; Bove, K; Horslen, S; Kohli, R; Wang, KS; Friederich, MW; Magee, JC; Sokol, RJ. Clinical spectrum and genetic causes of mitochondrial hepatopathy phenotype in children. Hepatology Communications. 2023; 7(6).

Kudira, R; Pasula, S; Vom Hofe, AY; Pfuhler, L; Singh, M; Kasten, J; Bernieh, A; Kostrub, C; Vig, P; Troutman, T; Miethke, A. FRI-350 Genetic predisposition for liver inflammation and response to anti-cholestatic therapy in experimental sclerosing cholangitis. Journal of Hepatology. 2023; 78:s417-s418.

Leung, DH; Devaraj, S; Goodrich, NP; Chen, X; Rajapakshe, D; Ye, W; Andreev, V; Minard, CG; Guffey, D; Molleston, JP; Magee, J; Merion, R; Spino, C; Ye, W. Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease. Hepatology. 2023; 77(2):530-545.

Shi, T; Malik, A; Yang Vom Hofe, A; Matuschek, L; Mullen, M; Lages, CS; Kudira, R; Singh, R; Zhang, W; Setchell, KDR; Hildeman, D; Pasare, C; Wagner, B; Miethke, AG. Farnesoid X receptor antagonizes macrophage-dependent licensing of effector T lymphocytes and progression of sclerosing cholangitis. Science Translational Medicine. 2022; 14(675):eabi4354.

Rogers, ME; Delman, A; Campbell, K; Miethke, A; Tiao, G; Mullapudi, B; Bondoc, A. Children undergoing early liver re-transplantation for primary non-function have improved survival. Pediatric Transplantation. 2022; 26(7):e14347.

Sharma, A; Kudira, R; Wang, J; Miethke, A; Gandhi, CR. Differential recruitment of monocyte-derived macrophages in control and stellate cell-depleted mice during recurrent carbon tetrachloride-induced acute liver injury. Journal of Cellular Physiology. 2022; 237(11):4215-4225.

Bass, LM; Ye, W; Hawthorne, K; Leung, DH; Murray, KF; Molleston, JP; Romero, R; Karpen, S; Rosenthal, P; Loomes, KM; Kyle Jensen, M; Sokol, RJ; Magee, JC; Shneider, BL. Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia. Hepatology. 2022; 76(3):712-726.

Loomes, KM; Squires, RH; Kelly, D; Rajwal, S; Soufi, N; Lachaux, A; Jankowska, I; Mack, C; Setchell, KDR; Karthikeyan, P; Jaecklin, T; Vig, P; Miethke, A; Thompson, RJ. Maralixibat for the treatment of PFIC: Long-term, IBAT inhibition in an open-label, Phase 2 study. Hepatology Communications. 2022; 6(9):2379-2390.

Shneider, BL; Spino, CA; Kamath, BM; Magee, JC; Ignacio, RV; Huang, S; Horslen, SP; Molleston, JP; Miethke, AG; Kohli, R; Rajwal, S; Kelly, D; Sokol, RJ; Thompson, RJ. Impact of long-term administration of maralixibat on children with cholestasis secondary to Alagille syndrome. Hepatology Communications. 2022; 6(8):1922-1933.

Mccrary, J; Trout, AT; Mahalingam, N; Singh, R; Rojas, CC; Miethke, AG; Dillman, JR. Associations Between Quantitative MRI Metrics and Clinical Risk Scores in Children and Young Adults With Autoimmune Liver Disease. American Journal of Roentgenology. 2022; 219(1):142-150.

Bondoc, A; Barker, M; Mullapudi, B; Miethke, A; Tiao, G. Pediatric Living Donor Liver Transplantation Outcomes-Is it Time for Outcome Optimization of Technical Aspects of Transplantation Using Learning Networks? Liver Transplantation. 2022; 28(6):928-930.

Kaj-Carbaidwala, B; Bernieh, A; Russi, AE; Miethke, AG; Abu-El-Haija, M; Trout, AT; Gupta, A; Ricci, KW; Nathan, JD. Total pancreatectomy with islet autotransplantation in a child with pancreatic kaposiform hemangioendothelioma. Journal of Pediatric Surgery Case Reports. 2022; 81:102266.

Li, CZ; Ogawa, H; Ng, SS; Chen, X; Kishimoto, E; Sakabe, K; Fukami, A; Hu, Y-C; Mayhew, CN; Hellmann, J; Hayashi, H; Thompson, RJ; Rashid, ST; Asai, A. Human iPSC-derived hepatocyte system models cholestasis with tight junction protein 2 deficiency. JHEP Reports. 2022; 4(4):100446.

Amevor, AA; Yodoshi, T; Trout, AT; Dillman, JR; Singh, R; Jarvis, R; Fei, L; Liu, C; Taylor, A; Miethke, A; Mouzaki, M. Sarcopenia is highly prevalent in children with autoimmune liver diseases and is linked to visceral fat and parent-perceived general health. Liver International. 2022; 42(2):394-401.

Mahalingam, N; Trout, AT; Gandhi, DB; Sahay, RD; Singh, R; Miethke, AG; Dillman, JR. Associations between MRI T1 mapping, liver stiffness, quantitative MRCP, and laboratory biomarkers in children and young adults with autoimmune liver disease. Abdominal Radiology. 2022; 47(2):672-683.

Baker, EK; Ulm, EA; Belonis, A; Brightman, DS; Hallinan, BE; Leslie, ND; Miethke, AG; Vawter-Lee, M; Wu, Y; Pena, LDM. Clinically available testing options resulting in diagnosis in post-exome clinic at one medical center. Frontiers in Genetics. 2022; 13:887698.