• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.2
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.

• Biotechnology and Bioprocess Engineering:BBE
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• v.11 no.2
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• pp.173-177
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• 2006

In this study, a novel strategy was developed for the highly selective immobilization of proteins, using the polyhydroxyalkanoate (PHA) depolymerase substrate binding domain (SBD) as an active binding domain. In order to determine the appropriacy of this method for immunodiagnostic assays, the single-chain antibody (ScFv) against the hepatitis B virus (HBV) preS2 surface protein and the severe acute respiratory syndrome coronavirus (SARS-CoV) envelope protein (SCVe) were fused to the SBD, then directly immobilized on PH A-coated slides via microspotting. The fluorescence-labeled HBV antigen and the antibody against SCVe were then utilized to examine specific interactions on the PHA-coated surfaces. Fluorescence signals were detected only at the spotted positions, thereby indicating a high degree of affinity and selectivity for their corresponding antigens/antibodies. Furthermore, we detected small amounts of ScFv-SBD (2.7 ng/mL) and SCVe-SBD fusion proteins (0.6ng/mL). Therefore, this microarray platform technology, using PHA and SBD, appears generally appropriate for immunodiagnosis, with no special requirements with regard to synthetic or chemical modification of the biomolecules or the solid surface.

• Journal of Environmental Health Sciences
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• v.36 no.2
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• pp.155-162
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• 2010

Aflatoxin $B_1$, a known human carcinogen, is the member of aflatoxin subfamily that is most frequently found in contaminated foods. Epidemiological studies have suggested that aflatoxins may be associated with human liver cancer and acute hepatitis. Recently it was reported that the traditional medical herbs sold in domestic markets are contaminated with aflatoxins. Long-term administration of these contaminated medicines could result in adverse health effects. Therefore, it is important to evaluate the levels of exposure to aflatoxin in people who ingest traditional herbal medicines. Blood samples were collected, before and after the herbal medicine intake, from 151 subjects who visited the hospital. The metabolite of aflatoxin $B_1$ in blood, aflatoxin $B_1$-albumin (aflatoxin $B_1$-lysine), is reportedly an appropriate internal exposure indicator, and its levels in the collected bloods were therefore analyzed using a liquid chromatography-mass spectrometry. The analytical method of aflatoxin $B_1$-lysine in blood was firstly optimized in Korea and the levels were detected below quantification limits (2 pg/mg albumin) in this study population. Consequently, the exposure levels of aflatoxin $B_1$ by ingestion of herbal medicines were low but it is important to monitor routinely due to the possibility of risk on the aflatoxin exposure.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.5
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• pp.430-438
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• 2020

Purpose: We investigated the clinical features and factors affecting the choice of treatment modality and the course of pediatric gallstone (GS) disease. Methods: We retrospectively analyzed the medical records of 65 patients diagnosed with GS using imaging studies between January 2009 and December 2017 were included. Results: This study included 65 patients (33 boys and 32 girls; mean age, 8.5±5.3 years; range, 0.2-18 years) who primarily presented with abdominal pain (34%), jaundice (18%), and vomiting (8%). Idiopathic GS occurred in 36 patients (55.4%). The risk factors for GS included antibiotic use, obesity, hemolytic disease, and chemotherapy in 8 (12.3%), 7 (10.8%), 6 (9.2%), and 4 patients (6.2%), respectively. We observed multiple stones (including sandy stones) in 31 patients (47.7%), a single stone in 17 (26.2%), and several stones in 17 (26.2%). GS with a diameter of <5 mm occurred in 45 patients (69.2%). Comorbidities included hepatitis, choledocholithiasis, cholecystitis, and acute pancreatitis in 20 (30.8%), 11 (16.9%), 11 (16.9%), and 4 patients (6.2%), respectively. Ursodeoxycholic acid (UDCA) was administered to 54 patients (83.1%), leading to stone dissolution in 22 patients (33.8%) within 6 months. Cholecystectomy was performed in 18 patients (27.7%) (mean age, 11.9±5.1 years). Most patients treated surgically had multiple stones (83%) and stones measuring <5 mm in size (89%), and 66.7% of patients had cholesterol stones. Conclusion: Cholecystectomy is feasible in patients with small-sized or large numbers of GS and those with persistent abdominal pain and/or jaundice. UDCA administration with close follow-up is recommended in patients with uncomplicated GS.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.28-32
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• 2007

Purpose : Human angiotensin converting enzyme (ACE) gene shows an insertion/deletion polymorphism in 16 intron, and three genotypes are determined by whether a 287 bp fragment of the DNA is present or not; II, ID and DD genotype. DD genotype has been suggested as a risk factor of chronic nephrotic disease such as IgA nephropathy and diabetic nephropathy, various cardiovascular diseases and several other diseases. ACE activity increases in acute hepatitis, chronic persistent hepatitis, chronic active hepatitis and cirrhosis. On the other hand, patients with fatty livers have normal ACE activity. This study was designed to find out the relation between polymorphsims of the ACE genes and neonatal hyperbilirubinemia in Koreans. Methods : The genomic DNA was isolated from 110 full-term Korean neonates who had hyperbilirubinemia with no obvious causes (serum bilirubin$${\geq_-}12mg/dL$$) and 164 neonates of a control population (serum bilirubin <12 mg/dL). We performed polymerase chain reaction (PCR) to see the allele of the ACE gene. Electrophoresis was done in the PCR products in 1.5 percent agarose gel, and then DNA patterns were directly visualized under ethidium bromide staining. Results : ACE genotypes in the hyperbilirubinemia group are as follows; 26.36 percent for II, 53.64 percent for ID, 20.00 percent for DD, 0.532 for I allele and 0.468 for D allele. These distributions were not significantly different from those in the control group; 24.39 percent for II, 51.83 percent for DI, 23.78 percent for DD, 0.503 for I allele and 0.497 for D allele. Conclusion : In this study, ACE gene polymorphism was detected in the neonatal hyperbilirubinemia and control group. The most frequent genotype was ID. Our results indicate that the ACE gene polymorphism is not associated with the prevalence of neonatal hyperbilirubinemia in Koreans.

• Molecules and Cells
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• v.42 no.12
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• pp.893-905
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• 2019

Mitochondria are highly dynamic organelles that constantly undergo fission and fusion processes that closely related to their function. Disruption of mitochondrial dynamics has been demonstrated in acute kidney injury (AKI), which could eventually result in cell injury and death. Previously, we reported that augmenter of liver regeneration (ALR) alleviates renal tubular epithelial cell injury. Here, we gained further insights into whether the renoprotective roles of ALR are associated with mitochondrial dynamics. Changes in mitochondrial dynamics were examined in experimental models of renal ischemia-reperfusion (IR). In a model of hypoxia-reoxygenation (HR) injury in vitro, dynamin-related protein 1 (Drp1) and mitochondrial fission process protein 1 (MTFP1), two key proteins of mitochondrial fission, were downregulated in the Lv-ALR + HR group. ALR overexpression additionally had an impact on phosphorylation of Drp1 Ser637 during AKI. The inner membrane fusion protein, Optic Atrophy 1 (OPA1), was significantly increased whereas levels of outer membrane fusion proteins Mitofusin-1 and -2 (Mfn1, Mfn2) were not affected in the Lv-ALR + HR group, compared with the control group. Furthermore, the mTOR/4E-BP1 signaling pathway was highly activated in the Lv-ALR + HR group. ALR overexpression led to suppression of HR-induced apoptosis. Our collective findings indicate that ALR gene transfection alleviates mitochondrial injury, possibly through inhibiting fission and promoting fusion of the mitochondrial inner membrane, both of which contribute to reduction of HK-2 cell apoptosis. Additionally, fission processes are potentially mediated by promoting tubular cell survival through activating the mTOR/4E-BP1 signaling pathway.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.28-35
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• 2007

Purpose: The aim of this study was to evaluate the clinical usefulness of ultrasound examination of children performed by a pediatrician. Methods: One thousand children who presented with symptoms of a gastrointestinal disorder and underwent abdominal ultrasound evaluation in the Department of Pediatrics, between January 2003 and June 2006, were included in this study. We analyzed the patient's medical records and ultrasound results retrospectively. Results: Among the 1,000 patients, 58.4% were male and 41.6% were female. The mean age of the patients was $4.7{\pm}4.0$ years. The main reasons for ultrasound were abdominal pain (43.9%), vomiting (17.3%), elevated liver enzymes (11.8%), and jaundice (9.8%). Abnormal ultrasound findings were present in 57.9% of cases. The major abnormal findings were mesenteric lymphadenitis (29.2%), fatty liver (12.1%), hepatitis (6.4%), hepatosplenomegaly (6.2%), and acute appendicitis (4.8%). The time interval between the initial medical evaluation and the ultrasound evaluation was within 24 hours in most cases (78.5%). The main findings in children with abdominal pain were mesenteric lymphadenitis (32.6%), fatty liver (5.9%), intussusception (2.7%), and acute appendicitis (2.7%). The main findings in children with vomiting were mesenteric lymphadenitis (12.7%), hypertrophic pyloric stenosis (10.4%), and acute appendicitis (3.5%). The major ultrasound findings in children with urinary tract diseases were hydronephrosis (45.4%), urolithiasis (21.5%) and cystic renal disease (18.1%). Conclusion: Ultrasound examination played an important role as a non-invasive and prompt screening examination for detection of abdominal diseases. Ultrasound was an important tool for pediatricians to determine timely information for patient management.

• Korean Journal of Microbiology
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• v.45 no.2
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• pp.105-111
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• 2009

Norovirus (NV) with a variety of genotypes, a member of the family Caliciviridae, causes acute nonbacterial gastroenteritis in humans. We determined the nucleotide sequence of three open reading frames (ORFs) of a NV Korean strain and characterized the genetic relationship with others. The Korean strain designated Hu/NLV/Gunpo/2006/KO was isolated from the stool specimen of a 2-year-old female suffering from gastroenteritis. By performing reverse transcription and PCR amplification, three overlapping cDNAs were synthesized and used for direct sequencing. We found that like other NVs, this strain contains three ORFs: ORF1, 5,100 bp; ORF2, 1,647 bp; ORF3, 765 bp. Of 35 NVs, ORF1 had a level of genetic diversity lower than ORF2 and ORF3, of which the C-termini of the ORF2 and ORF3 showed a relatively high degree of genetic diversity. Phylogenetic analyses indicated that the Korean strain belonged to genogroup II, with Saitama U1, Gifu'96, Mc37, and Vietnam 026 being formed a single genetic cluster. The nucleotide sequence information of three ORFs of a NV Korean isolate will be useful not only for the development of a diagnostic tool and understanding of genetic relationship, but also provide important basic information for the functional analysis of their gene products.

• Korean Journal of Plant Resources
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• v.26 no.1
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• pp.36-43
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• 2013

Aralia elata Seem. have a typical traditional significance among the wild herbs traditionally. Edible shoots of A. elata will augment consumer's interest due to its high value functional value, eco-friendly and pesticide-free produce. A. elata's root, fruit and bark are used as material of hypoglycemic agent and medicine for diabetes, kidney trouble, acute hepatitis, rheumation arthritis, stomach cancer and gastroenteric trouble. Flavonoid glycoside compound which is separated from A. elata's shoot shows high antioxidative activity. Also, root's identified active materials of antimicrobial was reported to be produced as food preservative and handy antimicrobial. Therefore, this research investigated quantitative morphological characteristics of leaves, spine and bud in naturally dominated and introducted A. elata in south Korea and then considered its principal compound analysis(PCA) and classification analysis(CA) among the 6 improved cultivars and 19 clones. PCA results showed that it show 76% accumulated explanation from four PC. The A. elata clones were classified into five groups; the first group of 15 clones including Yeongok, the second group of 5 clones including Yeoju, the third group of Bonghwa, Ulleung, the fourth group of Yongmunsa, Boseong and the fifth group of Singu. The object of this study will give us invaluable information about breeding by selection of A. elata in south Korea.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.34 no.5
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• pp.332-336
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• 2012

Purpose: The third molar extraction is one of the mostly performed procedures in the department of oral and maxillofacial surgery. In most of dental clinic or hospital, the third molar extraction used to be frequently performed in an office-based surgery, and most patients did not have specific medical history with young ages. Medical history taking are dependent on the only way by asking to the patients about their individual conditions. Therefore, as the specialists of the oral and maxillofacial surgery in the field of dentistry, we suggest a new policy that the preoperative lab must be performed routinely before extraction of the third molar. Methods: This study is based on 1,096 patients who have been managed with third molar extractions, from March 2008 to September 2011 by a single surgeon. The preoperative lab, including complete blood count, coagulation panel, chemistry and serology, was performed before any surgical procedures. The results were informed to the patients regardless of their abnormalities, and any abnormalities related to the surgical procedures, such as platelet count and coagulation factors, were checked and corrected safely. Results: Through the preoperative blood test, systemic diseases that the patients had not recognized before, such as anemia, leukopenia, fatty liver and chronic renal disease, were identified. Patients with acute or chronic leukemia, Hepatitis B, and HIV positive, were also detected as a small number. Also, the possibilities of the cross-infection between dentists and patients or between patients and patients, and any other emergency situations can be prevented; as well as the public health condition can be improved, too. The patients were satisfied with low cost preventive blood test and high quality of medical services. Conclusion: Therefore, routine medical lab testing, including history taking are needed before an office-based minor surgery, such as third molar extractions, and these results were suggested as a new policy in the field of dentistry.