ArticleJournal of Korean medical science2020
Development of Hepatocellular Carcinoma in Patients with Glycogen Storage Disease: a Single Center Retrospective Study.
Article in Journal of Korean medical science, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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16 citing papers in PubMed, 38 citations in OpenAlex.
- Continuous Glucose Monitoring-Driven Personalization of Cornstarch Therapy in Glycogen Storage Disease: A Retrospective Analysis.Yonsei medical journal · 2026Article
- Clinical Outcomes and Management in Late Diagnosed Siblings Affected With Attenuated GSD Ib.JIMD reports · 2026Article
- Correlation of biochemical and imaging markers with hepatic adenoma in patients with glycogen storage disease: a retrospective single-center study.Orphanet journal of rare diseases · 2026Article
- Clinical characteristics of 50 hepatocellular adenoma patients among 164 cases of glycogen storage disease type Ia.European journal of pediatrics · 2025Article
- Efficacy of magnetic resonance imaging in managing glycogen storage disease.Orphanet journal of rare diseases · 2025Article
- MRI features of hepatocellular adenomas in children: clinical and radiological review.Pediatric radiology · 2025Article
- Transcriptomic analysis of Paraoxonase 1 expression in hepatocellular carcinoma and its potential impact on tumor immunity.Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico · 2025Article
- Hepatocellular adenoma update: diagnosis, molecular classification, and clinical course.The British journal of radiology · 2024Review
- A case study of a liver transplant-treated patient with glycogen storage disease type Ia presenting with multiple inflammatory hepatic adenomas: an analysis of clinicopathologic and genetic data.BMC medical genomics · 2024Article
- A novelMolecular genetics and metabolism reports · 2024Article
- Systematic analysis of Mendelian disease-associated gene variants reveals new classes of cancer-predisposing genes.Genome medicine · 2023Article
- Review
- Clinical and biochemical footprints of inherited metabolic diseases. VIII. Neoplasias.Molecular genetics and metabolism · 2022Review
- Pediatric Primary Hepatic Tumors: Diagnostic Considerations.Diagnostics (Basel, Switzerland) · 2021Review
- "Bull's eye" appearance of hepatocellular adenomas in patients with glycogen storage disease type I - atypical magnetic resonance imaging findings: Two case reports.World journal of clinical cases · 2021Article
- Hepatocellular carcinoma (HCC): Epidemiology, etiology and molecular classification.Advances in cancer research · 2021Review
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Authors and funding
6 authors at 1 institution in 1 country.
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Abstract
backgroundGlycogen storage disease (GSD) is an inherited disorder leading to abnormal glucose metabolism and glycogen accumulation, and is associated with various complications including hepatic adenoma and hepatocellular carcinoma. The aim of this study was to analyze the risk factors for hepatic adenoma and its malignant change, and the hepatocellular carcinoma-free survival rate in patients with GSD who developed adenoma.
methodsA total of 72 patients with GSD who were enrolled from March 1982 to September 2013 at Seoul National University Children's Hospital were retrospectively analyzed, and the median follow-up period was 19.2 years.
resultsThirty-two patients (44.4%) developed hepatic adenoma at an age range of 7.9-26.3 years (median, 14.3 years). Among the 32 patients with hepatic adenoma, 4 patients (12.5%) developed hepatocellular carcinoma on an average interval of 6.7 years between the diagnosis of adenoma and the development of hepatocellular carcinoma. GSD type I and portacaval shunt operation were found to be the risk factors for hepatic adenoma development. The hepatocellular carcinoma-free survival rate at 10 years from adenoma development was 82%.
conclusionThe present study found that portacaval shunt operation increases the risk of development of hepatic adenoma in GSD patients, especially in GSD type I. The hepatic adenoma in GSD patients has a potential of malignant transformation, which should be keep in mind in follow-up process of the disease.
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