Miethke Lab

Publications

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. 2025; 17:83.

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:e0594.

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

Heekin, S; Lopez-Nunez, O; Smith, J; Denson, N; Denson, L; Miethke, A; Dillman, J; Miraldi, E; Kofron, JM; Dhaliwal, J; Erdman, L. (2024) Trade-Offs in Deep Learning Model Loss and Configuration for Sparse Histological Segmentation: A Case Study in Pediatric Ileal Histology. Institute of Electrical and Electronics Engineers (IEEE). 00:1-8.

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:e2431108.

Wang, YZ M; Kaj-Carbaidwala, B; Lane, A; Agarwal, S; Beier, F; Bertuch, A; Borovsky, KA; Brennan, SK; Calado, RT; Catto, LF B; Simonetto, DA; Ziegler, DS; Miethke, AG; Myers, KC. Liver disease and transplantation in telomere biology disorders: An international multicenter cohort. Hepatology Communications. 2024; 8:e0462.

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; 9:620-631.

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:s-898.

Coppola, T; Hughes, H; Finch, PT; Hess, JA; Wu, S; Prada, CE; Miethke, AG; Zhang, W; Kalfa, TA. Case report: Childhood erythrocytosis due to hypermanganesemia caused by homozygous SLC30A10 mutation. 2024; 3:1331485.

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; 95:1035-1040.

Van Hove, JL K; 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:e0361.

Pandurangi, S; Malik, A; Owens, J; Valencia, CA; Miethke, AG; Marsh, R; Chiang, S; Yang, L; Hopkin, R; Kasten, J; Huggins, J; Gutierrez Sanchez, LH; Castro-Rojas, C; Alquaraish, M. Deleterious variants in TNFAIP3 are associated with type II and seronegative pediatric autoimmune hepatitis. Journal of Hepatology. 2024; 80:e26-e28.

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:e0293.

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; 77:166-170.

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. The Journal of Pediatrics. 2023; 259:113439.

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; 58:1483-1488.

Squires, JE; Miethke, AG; Alexander Valencia, C; Hawthorne, K; Henn, L; Van Hove, JL K; 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:e0139.

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; 220:901-902.

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; 48:1933-1944.

Leung, DH; Devaraj, S; Goodrich, NP; Chen, X; Rajapakshe, D; Ye, W; Andreev, V; Minard, CG; Guffey, D; Molleston, JP; Lowichik, A; Book, L; Meyers, R; Hall, T. Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease. Hepatology. 2023; 77:530-545.

Shi, T; Malik, A; vom Hofe, AY; Matuschek, L; Mullen, M; Lages, CS; Kudira, R; Singh, R; Zhang, W; Setchell, KD R; 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:eabi4354.

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; 237:4215-4225.

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:e14347.

Loomes, KM; Squires, RH; Kelly, D; Rajwal, S; Soufi, N; Lachaux, A; Jankowska, I; Mack, C; Setchell, KD R; 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; 6:2379-2390.

Bass, LM; Ye, W; Hawthorne, K; Leung, DH; Murray, KF; Molleston, JP; Romero, R; Karpen, S; Rosenthal, P; Loomes, KM; Wang, L; Soufi, N; Gupta, N; Vos, MB. Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia. Hepatology. 2022; 76:712-726.

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; 6:1922-1933.

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

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; 219:142-150.

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; 28:928-930.

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, YC; 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; 4:100446.

Amevor, AA; Yodoshi, T; Trout, AT; Dillman, J; 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; 42:394-401.

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; 47:672-683.

Kudira, R; Sharma, BK; Mullen, M; Mohanty, SK; Donnelly, B; Tiao, GM; Miethke, A. Isolation and Culturing Primary Chaolangiocytes from Mouse Liver. Bio-protocol. 2021; 11:e4192.

Schultz, K; Divanovic, A; Towe, C; Miethke, A; Wusik, K; Hammill, A; Brunner, H. Clinicopathologic Conference: A Four-Year-Old Child With Digital Clubbing. Arthritis Care and Research. 2021; 73:1379-1386.

Gandhi, DB; Pednekar, A; Braimah, AB; Dudley, J; Tkach, JA; Trout, AT; Miethke, AG; Franck, MD; Heilman, JA; Dzyubak, B; Lake, DS; Dillman, JR. Assessment of agreement between manual and automated processing of liver MR elastography for shear stiffness estimation in children and young adults with autoimmune liver disease. Abdominal Radiology. 2021; 46:3927-3934.

Pham, DH; Kudira, R; Xu, L; Valencia, CA; Ellis, JL; Shi, T; Evason, KJ; Osuji, I; Matuschek, N; Pfuhler, L; Zhang, K; Wali, S; Yin, C; Miethke, A. Deleterious Variants in ABCC12 are Detected in Idiopathic Chronic Cholestasis and Cause Intrahepatic Bile Duct Loss in Model Organisms. Gastroenterology. 2021; 161:287-300.e16.

El-Matary, W; Guthery, SL; Amir, AZ; DiGuglielmo, M; Draijer, LG; Furuya, KN; Gupta, N; Hochberg, JT; Horslen, S; Kerkar, N; Soufi, N; Valentino, PL; Zizzo, A; Deneau, MR. Colorectal Dysplasia and Cancer in Pediatric-Onset Ulcerative Colitis Associated With Primary Sclerosing Cholangitis. Clinical Gastroenterology and Hepatology. 2021; 19:1067-1070.e2.

Deneau, MR; Mack, C; Perito, ER; Ricciuto, A; Valentino, PL; Amin, M; Amir, AZ; Aumar, M; Auth, M; Broderick, A; Woynarowski, M; Zerofsky, M; Zizzo, A; Guthery, SL. The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children. Hepatology. 2021; 73:1074-1087.

Deneau, MR; Mack, C; Mogul, D; Perito, ER; Valentino, PL; Amir, AZ; DiGuglielmo, M; Draijer, LG; El-Matary, W; Furuya, KN; Singh, R; Vitola, B; Zizzo, A; Guthery, SL. Oral Vancomycin, Ursodeoxycholic Acid, or No Therapy for Pediatric Primary Sclerosing Cholangitis: A Matched Analysis. Hepatology. 2021; 73:1061-1073.

Abu Ata, N; Dillman, JR; Gandhi, DB; Dudley, JA; Trout, AT; Miethke, AG. Association between liver diffusion-weighted imaging apparent diffusion coefficient values and other measures of liver disease in pediatric autoimmune liver disease patients. Abdominal Radiology. 2021; 46:197-204.

Leonis, MA; Miethke, AG; Fei, L; Maynor, S; Chapin, CA; Bleesing, JJ H; Alonso, EM; Squires, RH. Four Biomarkers Linked to Activation of Cluster of Differentiation 8-Positive Lymphocytes Predict Clinical Outcomes in Pediatric Acute Liver Failure. Hepatology. 2021; 73:233-246.