Current Understanding in the Clinical Characteristics and Molecular Mechanisms in Different Subtypes of Biliary Atresia
Abstract
:1. Introduction
2. Search Strategy and Inclusion Criteria
3. Clinical Characteristics
3.1. Phenotypes and Clinical Manifestations of All BA Subtypes
3.2. Surgical Treatment for All BA
3.3. Clinical Characteristics of Different BA Subtypes
4. Etiologic and Pathogenic Mechanisms in Different Subtypes of BA
4.1. Mechanisms of Ciliary Dysgenesis
4.2. Mechanisms of Epithelial Injury
4.3. Mechanisms of Duct Obstruction
5. Development of Biomarkers to Improve Clinical Managements
6. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
Abbreviations
References
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Clinical Subtype | Distribution Frequency | Characteristic Manifestation | References |
---|---|---|---|
IBA | 67–89% | Isolated duct obliteration | [7,10,11,12,13,14,15,16] |
SBA | 4.9–10% | Combination with other defects | [7,10,22,23,24,25] |
CBA | 5–22.4% | A cyst at the portal hepatis | [7,10,22,23,24,25] |
CMVBA | 5–9.5% | Cytomegalovirus-IgM positive | [7,21] |
Gene | 3’-UTR Position/Rs Number(Minor Allele) | Population | Risk | Functions | Ref. |
---|---|---|---|---|---|
ADD3 | rs17095355 (T) | Chinese | Increased | Cytoskeletal organization | [55,56] |
rs17095355 (T) | Thai | Increased | [57] | ||
rs17095355 (T) | Thai | Increased | [67] | ||
rs7099604 (G) | Caucasian | Increased | [58] | ||
GPC1 | rs6707262 (C) rs6750380 (C) | Chinese | Increased | Growth factors and cytokines | [55] |
rs2292832 (C) | Chinese | Decreased | [59] | ||
ARF6 | rs3126184 (T) | Chinese | No association | Cytoskeletal organization | [55] |
rs3126184 (T) rs10140366 (C) | Caucasian | Increased | [60] | ||
EFEMP1 | 4 SNPs | Chinese | No association | Cell proliferation | [55] |
rs10865291 (A) rs6761893 (T) rs727878 (T) | Caucasian | Increased | [80] | ||
USF2 | rs916145 (C) | Chinese | No association | Gene regulation | [61] |
rs916145 (C) | Taiwanese | Increased | [66] | ||
CD14 | -159 C/T (T) | Taiwanese | Increased | Immunoregulation | [63] |
miR-499 | rs3746444 (G) | Egyptian | Increased | Cell adhesion | [64] |
rs3746444 (G) | Chinese | Increased | [65] | ||
VEGFA | rs3025039 (T) | Taiwanese | Decreased | Vasculogenesis, cell proliferation, and cell transmigration | [68] |
rs3025039 (T) | Chinese | No association | [69] | ||
rs3025039 (T) | Chinese | Decreased | [70] | ||
PDGFA | rs9690350 (C) | Chinese | Decreased | Vasculogenesis, cell proliferation, and cell transmigration | [71] |
ADIPOQ | rs1501299 (T) | Thai | Decreased | Anti-inflammation | [74] |
ITGB2 | rs1160263 (T) | Chinese | Decreased | Immunoregulation | [75] |
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He, L.; Chung, P.H.Y.; Lui, V.C.H.; Tang, C.S.M.; Tam, P.K.H. Current Understanding in the Clinical Characteristics and Molecular Mechanisms in Different Subtypes of Biliary Atresia. Int. J. Mol. Sci. 2022, 23, 4841. https://doi.org/10.3390/ijms23094841
He L, Chung PHY, Lui VCH, Tang CSM, Tam PKH. Current Understanding in the Clinical Characteristics and Molecular Mechanisms in Different Subtypes of Biliary Atresia. International Journal of Molecular Sciences. 2022; 23(9):4841. https://doi.org/10.3390/ijms23094841
Chicago/Turabian StyleHe, Lin, Patrick Ho Yu Chung, Vincent Chi Hang Lui, Clara Sze Man Tang, and Paul Kwong Hang Tam. 2022. "Current Understanding in the Clinical Characteristics and Molecular Mechanisms in Different Subtypes of Biliary Atresia" International Journal of Molecular Sciences 23, no. 9: 4841. https://doi.org/10.3390/ijms23094841