Background: Mitochondrial hepatopathies (MHs) are primary mitochondrial genetic disorders that can present as childhood liver disease. No recognized biomarkers discriminate MH from other childhood liver diseases. The protein biomarkers growth differentiation factor 15 (GDF15) and fibroblast growth factor 21 (FGF21) differentiate mitochondrial myopathies from other myopathies. We evaluated these biomarkers to determine if they discriminate MH from other liver diseases in children.
Methods: Serum biomarkers were measured in 36 children with MH (17 had a genetic diagnosis); 38 each with biliary atresia, α1-antitrypsin deficiency, and Alagille syndrome; 20 with NASH; and 186 controls.
Results: GDF15 levels compared to controls were mildly elevated in patients with α1-antitrypsin deficiency, Alagille syndrome, and biliary atresia-young subgroup, but markedly elevated in MH (p<0.001). FGF21 levels were mildly elevated in NASH and markedly elevated in MH (p<0.001). Both biomarkers were higher in patients with MH with a known genetic cause but were similar in acute and chronic presentations. Both markers had a strong performance to identify MH with a molecular diagnosis with the AUC for GDF15 0.93±0.04 and for FGF21 0.90±0.06. Simultaneous elevation of both markers >98th percentile of controls identified genetically confirmed MH with a sensitivity of 88% and specificity of 96%. In MH, independent predictors of survival without requiring liver transplantation were international normalized ratio and either GDF15 or FGF21 levels, with levels <2000 ng/L predicting survival without liver transplantation (p<0.01).
Conclusions: GDF15 and FGF21 are significantly higher in children with MH compared to other childhood liver diseases and controls and, when combined, were predictive of MH and had prognostic implications.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10781130 | PMC |
| http://dx.doi.org/10.1097/HC9.0000000000000361 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Palmitoylation-dependent association with Annexin II directs hepatitis E virus ORF3 sorting into vesicles and quasi-enveloped virions.
Proc Natl Acad Sci U S A
January 2025
Division of Livestock Infectious Diseases, State Key Laboratory for Animal Disease Control and Prevention, Harbin Veterinary Research Institute, Chinese Academy of Agricultural Sciences, Harbin 150069, China.
Historically considered to be nonenveloped, hepatitis E virus (HEV), an important zoonotic pathogen, has recently been discovered to egress from infected cells as quasi-enveloped virions. These quasi-enveloped virions circulating in the blood are resistant to neutralizing antibodies, thereby facilitating the stealthy spread of infection. Despite abundant evidence of the essential role of the HEV-encoded ORF3 protein in quasi-enveloped virus formation, the underlying mechanism remains unclear.
View Article and Find Full Text PDFBacillus coagulans alleviates hepatic injury caused by Klebsiella pneumoniae in rabbits.
PLoS One
January 2025
College of Animal Science and Technology, Henan University of Science and Technology, Luoyang, China.
Background: As an opportunistic bacterial pathogen, Klebsiella pneumoniae (KP) is prone to causing a spectrum of diseases in rabbits when their immune system is compromised, which poses a threat to rabbit breeding industry. Bacillus coagulans (BC), recognized as an effective probiotic, confers a variety of benefits including anti-inflammatory and antioxidant properties.
Aim: The purpose of this study was to investigate whether dietary BC can effectively alleviate hepatic injury caused by KP.
Background & Aims: This systematic literature review of qualitative findings aims to identify the perceived barriers and enablers for hepatocellular carcinoma (HCC) surveillance from patient and clinician perspectives.
Methods: A systematic search of databases using key term combinations with the following inclusion criteria: 1) qualitative and quantitative (survey) studies exploring barriers and enablers of HCC surveillance, and 2) qualitative and quantitative (survey) studies exploring barriers and enablers of enagagement in clinical care for patients with cirrhosis and/or viral hepatitis.
Results: The search returned 445 citations: 371 did not meet the study criteria and were excluded.
Hyperprolactinemia complicated with peliosis hepatis: One case report and review of literature.
Medicine (Baltimore)
January 2025
Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Rationale: Peliosis hepatis (PH) is a rare disease with few clinical reports and complex etiology. However, there have been no reports of hyperprolactinemia (HPRL) leading to PH at present. This paper, through case reports, expands the understanding of the etiology of PH and the pathological damage effect of prolactin (PRL).
View Article and Find Full Text PDFAll-positive HBsAg, HBsAb, HBeAg, HBeAb, and HBcAb in a patient with hepatitis B: A case report.
Medicine (Baltimore)
January 2025
Department of Clinical Immunology, Nanjing Kingmed Clinical Laboratory, Nanjing, Jiangsu, China.
Rationale: Mass vaccination, low cost of immunoglobulins, and new drugs led to the emergence of new, unusual patterns of hepatitis B serum markers. This study reported a rare case of hepatitis B with all 5 positive serum markers, including HBsAg, HBsAb, HBeAg, HBeAb, and HBcAb.
Patient Concerns: A 30-year-old female patient was admitted due to abnormal liver function.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!