Review
Medicine, General & Internal
Mark Davenport, Ancuta Muntean, Nedim Hadzic
Summary: Biliary atresia is a biliary tract obstructive condition characterized by persistent jaundice and pale stools. It may have diverse causative factors and pathogenic mechanisms, with some cases showing distinguishable variants with higher degree of etiological homogeneity, while others may be related to synchronous viral infections. In most cases, known as isolated BA, the etiology and pathogenesis remain unclear.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Multidisciplinary Sciences
Wison Laochareonsuk, Komwit Surachat, Piyawan Chiengkriwate, Surasak Sangkhathat
Summary: Cholestatic jaundice is a common neonatal condition, and biliary atresia (BA) is a correctable cholangiopathy. This study aimed to explore the pathogenesis of BA by studying genetic variations and found a correlation between different pathways of cholestatic diseases and BA.
Review
Pediatrics
Li-Na Wu, Zhi-Jun Zhu, Li-Ying Sun
Summary: Biliary Atresia is a common cause of neonatal cholestasis and a primary indication for Liver Transplantation. Genetic variants play a crucial role in the development of this condition. This review summarizes and analyzes the genetic variants of Biliary Atresia from a pathological perspective, providing a foundation for further research and treatment.
FRONTIERS IN PEDIATRICS
(2022)
Article
Medicine, General & Internal
Yongliang Wang, Hongtao Yuan, Maojun Zhao, Li Fang
Summary: This study aimed to identify gene expression signature in biliary atresia (BA) using weighted gene co-expression network analysis. The results revealed a strong positive correlation between a specific gene module (brown module) and BA. Functional enrichment analysis showed that the module genes were involved in biological processes, metabolic pathways, and cell adhesion molecules. The combination of keratin 7 (KRT7) and C-X-C motif chemokine ligand 8 (CXCL8) was identified as a potential diagnostic model for BA.
Article
Gastroenterology & Hepatology
Yuan Li, Tian-Yu Li, Qiao Qi, Min-Ting Zhang, Ming-Xin Tong, Peng-Jun Su, Zhi-Bo Zhang
Summary: This study found that the poliovirus receptor (PVR) plays an important role in the pathogenesis of biliary atresia (BA) by regulating NK cell-mediated bile duct injury. PVR may serve as a biomarker for BA.
LIVER INTERNATIONAL
(2022)
Article
Gastroenterology & Hepatology
Celia Short, Allen Zhong, Jiabo Xu, Elaa Mahdi, Alison Glazier, Nicolas Malkoff, Nicolas Noriega, Theresa Yeo, Kinji Asahina, Kasper S. Wang
Summary: This study found that Prom1-expressing hepatic progenitor cells (HPCs) expand in biliary atresia (BA), giving rise to cholangiocytes within biliary ductular reactions. The TWEAK/FN14 signaling pathway mediates HPC proliferation, leading to fibrogenesis in BA.
Review
Biochemistry & Molecular Biology
Helen Y. Chen, Umaimainthan Palendira, Carl G. Feng
Summary: The development of multiplex tissue imaging has allowed for the visualization of numerous markers on a single tissue section, enabling the study of cell-cell interactions and tissue microenvironment. This technology has facilitated the identification of novel biomarkers and therapeutic targets in diseases, considering the spatial context of the cellular environment.
COMPUTATIONAL AND STRUCTURAL BIOTECHNOLOGY JOURNAL
(2022)
Review
Allergy
Atsushi Kato, Anju T. Peters, Whitney W. Stevens, Robert P. Schleimer, Bruce K. Tan, Robert C. Kern
Summary: The study of chronic rhinosinusitis (CRS) has shifted from phenotype-based classification to endotype-based classification, identifying three main endotypes that orchestrate the expression of different gene sets. The development of improved methods for endotyping disease is expanding the use of biological therapies, targeting Type 2 inflammation and potentially other inflammatory endotypes in the future.
Article
Gastroenterology & Hepatology
Sujit K. Mohanty, Bryan Donnelly, Haley Temple, Ana Ortiz-Perez, Sarah Mowery, Inna Lobeck, Phylicia Dupree, Holly M. Poling, Monica McNeal, Reena Mourya, Todd Jenkins, Ruchi Bansal, Jorge Bezerra, Greg Tiao
Summary: Biliary atresia (BA) is a devastating cholangiopathy of infancy. Research has shown that HMGB1 plays a role in virus-induced BA pathogenesis and could be a target for therapeutic interventions.
Article
Pediatrics
Fang-Min Liao, Kai-Chi Chang, Jia-Feng Wu, Huey-Ling Chen, Yen-Hsuan Ni, Mei-Hwei Chang
Summary: This study aimed to determine the sensitivity and specificity of elevated direct bilirubin (D-bil) levels and the direct-to-total bilirubin (D/T) ratio for detecting biliary atresia (BA) in high-risk infants. The findings showed that D-bil >= 1.0 mg/dL was better for detecting BA in infants aged 3 to 60 days than the D/T ratio. However, a more definitive cutoff point is required for newborns aged <3 days.
Review
Biochemistry & Molecular Biology
Lin He, Patrick Ho Yu Chung, Vincent Chi Hang Lui, Clara Sze Man Tang, Paul Kwong Hang Tam
Summary: This study reviewed the clinical characteristics of different subtypes of biliary atresia and revealed the molecular mechanisms of their development. By highlighting the differences among these subtypes, it contributes to the development of specific management protocols for each subtype.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Medicine, General & Internal
Jing Ye, Dengming Lai, Dan Cao, Linhua Tan, Lei Hu, Hua Zha, Jiezuan Yang, Qiang Shu
Summary: The study characterized the TRBV profiles in infants with BA and healthy donors using HTS, revealing lower diversity indices in the BA group compared to the HD group. Significant differences in neutrophil levels, NLR, and LDH were observed between the BA and HD groups, indicating potential biomarkers for the differential diagnosis of BA.
FRONTIERS IN MEDICINE
(2021)
Review
Pediatrics
Laura A. Conrad, Michael D. Cabana, Deepa Rastogi
Summary: Asthma, a complex and heterogeneous disease, has seen recent advancements in understanding its pathobiology, emphasizing the need for more nuanced definitions. Key biomarkers distinguishing childhood asthma subtypes have been summarized, along with a proposed approach to endotyping asthma for clinical care and personalized medicine research in the future.
PEDIATRIC RESEARCH
(2021)
Article
Gastroenterology & Hepatology
Julie Osborn, Reena Mourya, Unmesha Thanekar, Weizhe Su, Lin Fei, Pranavkumar Shivakumar, Jorge A. Bezerra
Summary: Through large-scale proteomics, we identified SEMA6B, SFRP3, COMMD7, BMX, and VCAM1 as serum biomarkers highly associated with clinical portal hypertension in children with biliary atresia. Immunostaining of the new protein biomarkers showed increased expression in hepatic endothelial cells, cholangiocytes, and immune cells within portal triads in BA livers with clinical PHT compared to healthy livers.
HEPATOLOGY COMMUNICATIONS
(2022)
Article
Biochemistry & Molecular Biology
Meng-Meng Cui, Yi-Ming Gong, Wei-Hua Pan, Hao-Yue Pei, Mei-Rong Bai, Huan-Lei Song, Xin-Ru Han, Wen-Jie Wu, Wen-Wen Yu, Bei-Lin Gu, Wei Cai, Ying Zhou, Xun Chu
Summary: This study identified an association between the ADD3 gene and the risk of non-syndromic biliary atresia (BA), as well as abnormal expression of ADD3 in cholangiocytes and hepatocytes. Variants in the HLA gene region did not reach genome-wide significance, suggesting a minimal role in the genetic predisposition of BA. There were no significant differences in genetic risk profiles between BA patients with cytomegalovirus (CMV) IgM positivity and CMV IgM negativity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Jose Gomez-Tames, Akihiro Asai, Akimasa Hirata
CLINICAL NEUROPHYSIOLOGY
(2020)
Article
Gastroenterology & Hepatology
Tadahiro Shinozawa, Masaki Kimura, Yuqi Cai, Norikazu Saiki, Yosuke Yoneyama, Rie Ouchi, Hiroyuki Koike, Mari Maezawa, Ran-Ran Zhang, Andrew Dunn, Autumn Ferguson, Shodai Togo, Kyle Lewis, Wendy L. Thompson, Akihiro Asai, Takanori Takebe
Summary: This study established a screening model based on human liver organoids (HLO) for analyzing drug-induced liver injury (DILI) pathology, providing unique advantages at the organoid resolution. Through this model, a multi-readout organoid analysis was successfully developed to measure viability, cholestatic and/or mitochondrial toxicity, showing high predictive values for marketed drugs.
Article
Cell & Tissue Engineering
Hisamitsu Hayashi, Shuhei Osaka, Kokoro Sakabe, Aiko Fukami, Eriko Kishimoto, Eitaro Aihara, Yusuke Sabu, Ayumu Mizutani, Hiroyuki Kusuhara, Nakayuki Naritaka, Wujuan Zhang, Stacey S. Huppert, Masahide Sakabe, Takahisa Nakamura, Yueh-Chiang Hu, Christopher Mayhew, Kenneth Setchell, Takanori Takebe, Akihiro Asai
Summary: The study established a model of BSEP-deficient hepatocytes using human induced pluripotent stem cells, revealing that BSEP deficiency leads to impaired apical export of bile acids but maintains a low intracellular bile acid concentration through inducing basolateral export. The findings suggest potential therapeutic targets for reducing bile acid accumulation in patients with BSEP deficiency.
Article
Neurosciences
Jose Gomez-Tames, Akihiro Asai, Akimasa Hirata
Summary: Research on temporal interference (TI) stimulation using computational techniques in a neural cortical model of a mouse head showed that carrier frequency affects the threshold, beat frequency has little effect on the threshold, and the intensity ratio of the alternating currents changes the location of the responding nerve. Regions with high modulation depth and low minimum envelope corresponded with the activation region found through neural computation.
FRONTIERS IN NEUROSCIENCE
(2021)
Article
Multidisciplinary Sciences
Masahito Masato, Yasuyoshi Miyata, Hiroki Kurata, Hidenori Ito, Kensuke Mitsunari, Akihiro Asai, Yuichiro Nakamura, Kyohei Araki, Yuta Mukae, Tsuyoshi Matsuda, Junki Harada, Tomohiro Matsuo, Kojiro Ohba, Hideki Sakai
Summary: In this study using a mouse model of prostate cancer, it was found that feeding with the EP1 receptor antagonist delayed cancer progression by increasing apoptosis, resulting in a lower incidence of cancer and a lower percentage of poorly differentiated cancer cells in the experimental group compared to the control group at different time points. The results suggest that the EP1 receptor antagonist may be a novel chemopreventive agent for prostate cancer.
SCIENTIFIC REPORTS
(2021)
Article
Gastroenterology & Hepatology
Akihiro Asai, Jia-Feng Wu, Kasper S. Wang, Atsuyuki Yamataka, Masaki Nio, Da-Jyun Su, Celia Short, Koichi Tsuboi, Takanori Ochi, Hideyuki Sasaki, Ryuji Okubo, Toshifumi Yodoshi, Kenichiro Konishi, Michael E. Rogers, Gregory M. Tiao
Summary: The outcomes of patients with cystic biliary atresia are closely related to the anatomy of the hepatic ducts and the choice of biliary reconstruction surgery. In patients with healthy proximal hepatic ducts, hepatic-cyst-jejunostomy leads to favorable clinical outcomes.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2022)
Article
Cell Biology
Tomoaki Hishida, Mako Yamamoto, Yuriko Hishida-Nozaki, Changwei Shao, Ling Huang, Chao Wang, Kensaku Shojima, Yuan Xue, Yuqing Hang, Maxim Shokhirev, Sebastian Memczak, Sanjeeb Kumar Sahu, Fumiyuki Hatanaka, Ruben Rabadan Ros, Matthew B. Maxwell, Jasmine Chavez, Yanjiao Shao, Hsin-Kai Liao, Paloma Martinez-Redondo, Isabel Guillen-Guillen, Reyna Hernandez-Benitez, Concepcion Rodriguez Esteban, Jing Qu, Michael C. Holmes, Fei Yi, Raymond D. Hickey, Pedro Guillen Garcia, Estrella Nunez Delicado, Antoni Castells, Josep M. Campistol, Yang Yu, Diana C. Hargreaves, Akihiro Asai, Pradeep Reddy, Guang-Hui Liu, Juan Carlos Izpisua Belmonte
Summary: Mammals have limited regenerative capacity, but inducing specific factor expression can enhance liver regeneration. This study provides a new avenue for tissue regeneration.
Article
Urology & Nephrology
Tsubasa Kondo, Toshifumi Tsurusaki, Akihiro Asai, Kuniko Abe
Summary: BCG intravesical instillation therapy is the most effective adjuvant treatment for bladder cancer after TURBT, but it can lead to severe local side effects such as bladder and ureteral necrosis.
UROLOGY CASE REPORTS
(2022)
Article
Pediatrics
Sindhu Pandurangi, Seung Kim, Akihiro Asai, Alexander Bondoc, William Balistreri, Kathleen Campbell, Alexander Miethke, Anna Peters, Michael Rogers, Amy Taylor, Suzanna Labib Attia, Troy Gibbons, Bhargava Mullapudi, Rachel Sheridan, Greg Tiao, Jorge A. Bezerra
Summary: This study aimed to evaluate the efficacy of personalized use of antibiotics and corticosteroids in patients with biliary atresia after surgery. The results showed that the personalized medication group had a significantly higher success rate of bile drainage compared to the conventional treatment group, and also maintained more native livers at 2 years. Therefore, personalized medication may help improve postoperative bile drainage.
JOURNAL OF PEDIATRIC SURGERY
(2023)
Review
Endocrinology & Metabolism
Rebecca L. Koch, Claudia Soler-Alfonso, Bridget T. Kiely, Akihiro Asai, Ariana L. Smith, Deeksha S. Bali, Peter B. Kang, Andrew P. Landstrom, H. Orhan Akman, T. Andrew Burrow, Jennifer L. Orthmann-Murphy, Deberah S. Goldman, Surekha Pendyal, Areeg H. El-Gharbawy, Stephanie L. Austin, Laura E. Case, Raphael Schiffmann, Michio Hirano, Priya S. Kishnani
Summary: Glycogen storage disease type IV (GSD IV) is an ultra-rare autosomal recessive disorder characterized by impaired glycogen synthesis and accumulation of poorly branched glycogen. The clinical manifestations can vary greatly and include hepatic, cardiac, muscular, and neurologic symptoms. Adult polyglucosan body disease (APBD) is a neurodegenerative form of GSD IV. Currently, there are no consensus guidelines for diagnosis and management, leading to misdiagnosis and lack of standardized care. To address this, experts developed recommendations for diagnosis and management, including imaging, assessments, laboratory investigations, and long-term follow-up care.
MOLECULAR GENETICS AND METABOLISM
(2023)
Editorial Material
Pediatrics
Hiroo Uchida, Gregory M. M. Tiao, Pranavkumar Shivakumar, Kenneth K. Y. Wong, Akihiro Asai, Hizuru Amano
FRONTIERS IN PEDIATRICS
(2023)
Article
Gastroenterology & Hepatology
Chao Zheng Li, Hiromi Ogawa, Soon Seng Ng, Xindi Chen, Eriko Kishimoto, Kokoro Sakabe, Aiko Fukami, Yueh-Chiang Hu, Christopher N. Mayhew, Jennifer Hellmann, Alexander Miethke, Nahrin L. Tasnova, Samuel J. Blackford, Zu Ming Tang, Adam M. Syanda, Liang Ma, Fang Xiao, Melissa Sambrotta, Oliver Tavabie, Filipa Soares, Oliver Baker, Davide Danovi, Hisamitsu Hayashi, Richard J. Thompson, S. Tamir Rashid, Akihiro Asai
Summary: The study established a disease model using patient-specific induced pluripotent stem cells (iPSC) and CRISPR genome-editing, which can recapitulate the phenotypes of patients with TJP2 deficiency.
Article
Genetics & Heredity
Kathryn Clarkston, Joy Lee, Sarah Donoghue, Heidi Peters, Hernan Eiroa, Amit A. Shah, Kathleen Loomes, Jessica Wen, Mark Oliver, Winita Hardikar, Carlos E. Prada, Akihiro Asai
Summary: This study describes 10 females with ornithine transcarbamylase (OTC) deficiency and liver dysfunction, showing a unique pattern of hepatocyte injury where initial hyperammonemia and coagulopathy is followed by a delayed peak in aminotransferase levels. Five patients eventually underwent liver transplant for recurrent metabolic crises. Further investigations into the pathophysiology of liver dysfunction in OTC-deficient patients are needed to develop therapies and prevent the need for liver transplant.
AMERICAN JOURNAL OF MEDICAL GENETICS PART A
(2021)
Meeting Abstract
Gastroenterology & Hepatology
Akihiro Asai, Toshifumi Yodoshi, Gregory M. Tiao, Elaa Mandi, Kasper S. Wang, Kohichi Tuboi, Hideyuki Sasaki, Masaki Nio, Ryuji Okubo, Takanori Ochi, Atsuyuki Yamataka, Da-Jyun Su, Jia-Feng Wu
Meeting Abstract
Gastroenterology & Hepatology
Akihiro Asai, Eriko Kishimoto, Kokoro Sakabe, Joseph Shiley, Christopher Mayhew, Yue-Chiang Hu