• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.26 no.4
• /
• pp.201-212
• /
• 2023

Purpose: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. Methods: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded. Results: The study included 24 patients (58.3% male; median age [range]: 29 [0.5-204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%). Conclusion: Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.

• Journal of Preventive Medicine and Public Health
• /
• v.32 no.4
• /
• pp.452-458
• /
• 1999

Objectives: The authors conducted the study to evaluate bias when potentially diseased subjects were included in cohort members while analyzing risk factors of chronic liver diseases. Methods: Total of 14,529 subjects were followed up for the incidence of liver diseases from January 1993 to June 1997. We have used databases of insurance company with medical records, cancer registry, and death certificate data to identify 102 incident cases. The cohort members were classified into potentially diseased group(n=2,217) when they were HBsAg positive, serum GPT levels higher than 40 units, or had or has liver diseases in baseline surveys. Cox's model were used for potentially diseased group, other members, and total subjects, respectively. Results: The risk factors profiles were similar for total and potentially diseased subjects: HBsAg positivity, history of acute liver disease, and recent quittance of smoking or drinking increased the risk. while intake of pork and coffee decreased it. For the potentially diseased, obesity showed marginally significant protective effect. Analysis of subjects excluding the potentially diseased showed distinct profiles: obesity increased the risk, while quitting smoking or drinking had no association. For these intake of raw liver or processed fish or soybean paste stew increased risk; HBsAg positivity, higher levels of liver enzymes and history of acute liver diseases increased the risk. Conclusions: The results suggested the potential bias in risk ratio estimates when potentially diseased subjects were included in cohort study on chronic liver diseases, especially for lifestyles possibly modified after disease onset. The analytic strategy excluding potentially diseased subjects was considered appropriate for identifying risk factors for chronic liver diseases.

• Health Policy and Management
• /
• v.31 no.3
• /
• pp.301-311
• /
• 2021

Background: This study purposed to analyze the relationship between the local extinction index and medical service uses of chronic diseases. The local extinction index is an indicator of the demographic structure and population aging of the region. Methods: The 2014-2018 statistics of National Health Insurance Corporation and Korean Statistical Information Service data were used for the analysis. First, descriptive statistics were used to analyze the general status of research variables. Second, a panel analysis was performed to analyze the relationship between the local extinction index and medical service uses of chronic diseases (hypertension, diabetes mellitus, periodontal disease, arthritis, mental health, epidemic disease, liver disease). Medical service uses were measured by the number of visits/inpatient days and medical charges of seven chronic diseases. Results: Panel analysis results showed that higher local extinction risks (meaning lower local extinction index) had a positive relationship with the number of visits/inpatient days and medical charges of chronic diseases. But the relationships were varied when the seven chronic diseases were analyzed separately. Conclusion: This study showed a significant relationship between the local demographic structure and medical service uses of chronic disease. Analyzing the local demographic structure will be an essential prerequisite step for implementing appropriate regional health care policies.

• The Korean Journal of Nuclear Medicine
• /
• v.21 no.1
• /
• pp.9-16
• /
• 1987

It is well-known that hepatic scintigraphv have been found to be less sensitive and specific in the detection of the diffuse hepatocellular diseases than that of the space-occupying lesions. To obtain the higher diagnostic specificity and sensitivity, we, using the computer quantitation, have attempted to analyze hepatic and extrahepatic $^{99m}Tc-tin$ colloid uptake patterns in various diffuse hepatocellular diseases retrospectively. The studied groups consisted of 116 cases of normal, 67 cases of acute hepatitis, 112 cases of chronic hepatitis, 61 cases of liver cirrhosis, 47 cases of fatty liver, 12 cases of hepatoma and 9 cases of metastasis, making total 424 cases. Scintigraphic imagings were obtained in the anterior, right lateral and posterior projections using high-resolution collimation, and simultaneously these gamma data were acquisited into the computer system. Both large region of interest (ROI) using light pen and ROI computer program were placed over right lobe, left lobe of liver, spleen and cardiac blood pool. Total counts in ROI were divided by the number of pixels in the ROI, and mean count rate per pixels calculated. Mean right-lobe counts were divded by mean-left lobe counts to determine right-to-left hepatic lobe ratio and mean spleen counts were divided by mean liver counts to determine spleen to liver ratio. The results were as follows. 1) Of 424 cases, 292 were male and 132 were female. The majority of age distribution was in $30\sim49$ (54.5%). 2) Inter-observer between two independant operators and inter-method between drawing by light-pen and ROI computer program variations were not significant. 3) The uptake count values (per pixel) determined at each area in normal group were $106.53{\pm}18.35$ in right lobe, $79.00{\pm}13.82$ in left lobe, $17.52{\pm}8.31$ in spleen and $8.09{\pm}3.43$ in cardiac blood pool. 4) In liver cirrhosis, right lobe uptake was decreased but spleen and cardiac blood pool uptakes were increased (p<0.01). 5) Right-to-left hepatic lobe uptake ratio was $1.37{\pm}0.24$ in normal group and significantly low in chronic hepatitis, liver cirrhosis and fatty liver, and more or less low in acute hepatitis. 6) Spleen-to-right hepatic lobe uptake ratio was $0.17{\pm}0.09$ in normal group and high in chronic hepatitis and liver cirrhosis. 7) The computer-quantitation of hepatic and extrahepatic uptake patterns thought to be sensitive and useful method in the interpretation of liver scintigram.

• IMMUNE NETWORK
• /
• v.16 no.3
• /
• pp.147-158
• /
• 2016

The liver lies at the intersection of multiple metabolic pathways and consequently plays a central role in lipid metabolism. Pathological disturbances in hepatic lipid metabolism are characteristic of chronic metabolic diseases, such as obesity-mediated insulin resistance, which can result in nonalcoholic fatty liver disease (NAFLD). Tissue damage induced in NAFLD activates and recruits liver-resident and non-resident immune cells, resulting in nonalcoholic steatohepatitis (NASH). Importantly, NASH is associated with an increased risk of significant clinical sequelae such as cirrhosis, cardiovascular diseases, and malignancies. In this review, we describe the immunopathogenesis of NASH by defining the known functions of immune cells in the progression and resolution of disease.

• Genomics & Informatics
• /
• v.21 no.4
• /
• pp.45.1-45.11
• /
• 2023

Nonalcoholic fatty liver disease (NAFLD) is a common type of chronic liver disease, with severity levels ranging from nonalcoholic fatty liver to nonalcoholic steatohepatitis (NASH). The extent of liver fibrosis indicates the severity of NASH and the risk of liver cancer. However, the mechanism underlying NASH development, which is important for early screening and intervention, remains unclear. Weighted gene co-expression network analysis (WGCNA) is a useful method for identifying hub genes and screening specific targets for diseases. In this study, we utilized an mRNA dataset of the liver tissues of patients with NASH and conducted WGCNA for various stages of liver fibrosis. Subsequently, we employed two additional mRNA datasets for validation purposes. Gene set enrichment analysis (GSEA) was conducted to analyze gene function enrichment. Through WGCNA and subsequent analyses, complemented by validation using two additional datasets, we identified five genes (BICC1, C7, EFEMP1, LUM, and STMN2) as hub genes. GSEA analysis indicated that gene sets associated with liver metabolism and cholesterol homeostasis were uniformly downregulated. BICC1, C7, EFEMP1, LUM, and STMN2 were identified as hub genes of NASH, and were all related to liver metabolism, NAFLD, NASH, and related diseases. These hub genes might serve as potential targets for the early screening and treatment of NASH.

• Genomics & Informatics
• /
• v.6 no.4
• /
• pp.192-201
• /
• 2008

Erythropoietin-producing human hepatocellular carcinoma receptor B1 (EPHB1) is a member of the Eph family of receptor tyrosine kinases that mediate vascular system development. Eph receptor overexpression has been observed in various cancers and is related to the malignant transformation, metastasis, and differentiation of cancers, including hepatocellular carcinoma (HCC). Eph receptors regulate cell migration and attachment to the extracellular matrix by modulating integrin activity. EphrinB1, the ligand of EPHB1, has been shown to regulate HCC carcinogenesis. Here, we sought to determine whether EPHB1 polymorphisms are associated with hepatitis B virus (HBV)-infected liver diseases, including chronic liver disease (CLD) and HCC. We genotyped 26 EPHB1 single nucleotide polymorphisms (SNPs) in 399 Korean CLD, HCC, and LD (CLD+HCC) cases and seroconverted controls (HBV clearance, CLE) using the GoldenGate assay. Two SNPs (rs6793828 and rs11717042) and 1 haplotype that were composed of these SNPs were associated with an increased risk for CLD, HCC, and LD (CLD+HCC) compared with CLE. Haplotypes that could be associated with HBV-infected liver diseases by affecting downstream signaling were located in the Eph tyrosine kinase domain of EPHB1. Therefore, we suggest that EPHB1 SNPs, haplotypes, and diplotypes may be genetic markers for the progression of HBV-associated acute hepatitis to CLD and HCC.

• Journal of Korean Biological Nursing Science
• /
• v.8 no.2
• /
• pp.41-59
• /
• 2006

Chronic liver diseases and hepatic cancer have been reported as 10% of cause of death in Koreans. Regardless of various causes, chronic liver disease accompanies commonly hepatic fibrosis. But still the mechanism of hepatic fibrosis remains poorly understood. Using the dimethylnitrosamine(DMN)-induced hepatic fibrosis rat model, We performed to evaluate the possible therapeutic effect of RIP(extracts of Phellodendron amurense and Patrinia scabiosaefolia) and to investigate the changes in referential connective tissue proteins($TGF-{\beta}_1$, ${\alpha}$-smooth muscle actin, and vimentin) as a marker of fibrogenesis. For these purposes, liver tissues were stained with H & E, and Azan staining for estimation of developing fibrosis. In the DMN-treated rat liver tissue, fibrosis were developed forming incomplete septal fibrosis. Whereas, in the RIP-treated rat liver tissues, the fibrosis were decreased recovering to normal morphology. The expressions of $TGF-{\beta}_1$, ${\alpha}$-smooth muscle actin($\alpha-SMA$), and vimetin were increased in the DMN-treated rat liver tissues, but decreased in the various areas of RIP-treated rat liver tissues. According to these results, RIP could be a possible therapeutic agent to reduce hepatic fibrosis, and the $TGF-{\beta}_1$, ${\alpha}$-SMA, and vimentin could be possible indicative markers of hepatic fibrosis development and recovery.

• The Journal of the Korean life insurance medical association
• /
• v.7 no.1
• /
• pp.31-35
• /
• 1988

• 대한핵의학회:학술대회논문집
• /
• 1971.11a
• /
• pp.84.2-85
• /
• 1971