• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.8-11
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• 2021

Glutaric aciduria type 1 (GA1; OMIM #231670) is a rare autosomal recessive-inherited neurometabolic disorder caused by the deficiency of glutaryl-CoA dehydrogenase (GCDH), which is encoded by the GCDH gene. It results in the accumulation of glutaric acid (GA), 3-hydroxyglutaric acid (3-OH-GA), glutaconic acid, and glutarylcarnitine (C5DC). These metabolites are considered to damage the striatum through an excitotoxic mechanism. The treatments of GA1 known to date are metabolic maintenance treatment based on a low-lysine diet and emergency treatment during acute illness. However, treatment after the onset of neurological symptoms has limited effectiveness and is associated with poor outcomes, and the effect of treatment and disease course after treatment are not good. After the implementation of newborn screening, the incidence of acute encephalopathic crisis fell to 10%-20% with early diagnosis, preventative dietary management, and aggressive medical intervention during acute episodes. Recently, several cohort studies have been published on the natural course and treatment of GA1 patients. This mini review will cover the clinical symptoms, natural history, and treatment of GA1 through a literature review.

• Research in Plant Disease
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• v.28 no.2
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• pp.57-60
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• 2022

Leaves of Chionanthus retusus were found to be damaged by leaf spot disease associated with a fungus in Iksan, Korea. Leaf spots were angular to irregular, vein-limited, scattered, 1-8 mm diameter, brownish-gray to dark brown when dry, with heavy fructification. The pathogen causes premature defoliation of C. retusus plant and was identified as Pseudocercospora chionanthi-retusi based on morphological and molecular-phylogenetic analyses. The phylogenetic tree was constructed using multi-locus DNA sequence data of partial actin (actA), partial translation elongation factor 1-alfa (tef1), partial DNA-directed RNA polymerase II second largest subunit (rpb2) genes, and internal transcribed spacer regions. Current study provides detail morphological description of P. chionanthi-retusi on C. retusus in Korea, with supports of phylogenetic analysis and pathogenicity test.

• The Korean Journal of Mycology
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• v.50 no.2
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• pp.131-136
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• 2022

Cornus officinalis plants that grow in several locations in Korea have been found to be infected with leaf spot disease. Symptoms include necrotic lesions, which are angular, irregularly shaped, vein-limited, and dark brown, on both sides of the leaves. The causal agent of the disease was identified to be Pseudocercospora cornicola based on the morphological characteristics of the fungus and molecular phylogenetic analysis of the obtained multi-locus DNA sequence data. This is the first report investigating P. cornicola found on C. officinalis in Korea.

• Journal of Veterinary Science
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• v.24 no.4
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• pp.57.1-57.8
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• 2023

Siewert-Kartagener's syndrome, a type of primary ciliary dyskinesia, is a complex disease comprising situs inversus, rhinosinusitis, and bronchiectasis. Situs inversus totalis is a condition in which all organs in the thoracic and abdominal cavities are reversed. Furthermore, primary ciliary dyskinesia, an autosomal genetic disease, may coexist with situs inversus totalis. Reports on Siewert-Kartagener's syndrome in veterinary medicine are limited. We report a rare case of primary ciliary dyskinesia with Siewert-Kartagener's syndrome in a dog, concurrently infected with canine distemper virus and type-2 adenovirus. This case highlights that situs inversus totalis can cause primary ciliary dyskinesia, and concurrent infections are possible.

• Korean Journal of Radiology
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• v.23 no.1
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• pp.13-29
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• 2022

Nonalcoholic fatty liver disease, characterized by excessive accumulation of fat in the liver, is the most common chronic liver disease worldwide. The current standard for the detection of hepatic steatosis is liver biopsy; however, it is limited by invasiveness and sampling errors. Accordingly, MR spectroscopy and proton density fat fraction obtained with MRI have been accepted as non-invasive modalities for quantifying hepatic steatosis. Recently, various quantitative ultrasonography techniques have been developed and validated for the quantification of hepatic steatosis. These techniques measure various acoustic parameters, including attenuation coefficient, backscatter coefficient and speckle statistics, speed of sound, and shear wave elastography metrics. In this article, we introduce several representative quantitative ultrasonography techniques and their diagnostic value for the detection of hepatic steatosis.

• Microbiology and Biotechnology Letters
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• v.52 no.3
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• pp.221-232
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• 2024

Zoonotic diseases are rare but the transmission of disease to humans may cause serious illness. Nipah virus (NiV) is a bat-borne zoonotic pathogen, which can cause severe encephalitis and respiratory distress. The transmission of Nipah virus from bats to humans was first reported in Malaysia in 1998. Different strains of NiV show different epidemiological and clinical features. Few of the strains are highly lethal and can spread to the community resulting in a global threat. However, the availability of effective management or prophylactic measures are only limited. Thus, it is essential to contain such outbreaks by implementing proper infection control and surveillance measures. Many serological and molecular diagnostic techniques have been developed for diagnosis of this infection. This review mainly focuses on the epidemiology, transmission of Nipah virus, pathogenesis and management of NiV infection. The review also throws light on the immune response of NiV in humans and the role of One Health approach in prevention and control of NiV infection.

• Journal of Chest Surgery
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• v.13 no.3
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• pp.229-232
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• 1980

Approximately 5 percent of infective endocarditis are limited to the right side of the heart, the tricuspid valve being the usual site of involvement. Usually there is no underlying cardiac disease, and the vegetations occur on previously normal tricuspid leaflets. This paper reports a case of bacterial endocarditis involving the bio-tricuspid valve in a patient with tetralogy of Fallot, and who required prosthetic valve replacement in addition to surgical therapy for the congenital lesions.

• Proceedings of the Korean Society of Toxicology Conference
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• 2003.10b
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• pp.185-185
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• 2003

In-vitro expanded CNS precursors provide a potentially unlimited source of dopamine (DA) neurons for the experimental treatment in Parkinson's disease. An efficient dopaminergic differentiation from CNS precursors in vitro is limited to mesencephalic precursors isolated from early embryonic ages (embryonic day 11.5 (E11.5)-E12.5).(omitted)

• Neonatal Medicine
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• v.28 no.3
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• pp.139-142
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• 2021

Macrophage activation syndrome (MAS) is a potentially life-threatening complication in many autoimmune diseases. Early recognition and intervention are essential for a favorable outcome. Neonatal lupus, an acquired autoimmune disease in neonates caused by the transplacental passage of maternal autoantibodies, is rare and usually self-limited. Herein, we report a case of MAS in a patient with neonatal lupus, which improved with intravenous immunoglobulin.

• Korean Journal of Food Science and Technology
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• v.51 no.6
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• pp.558-564
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• 2019

Hypertriglyceridemia is one of the metabolic syndrome that is often observed as a result of lipid abnormalities. It is associated with other lipids, metabolic disorders, cardiovascular disease and liver disease. Korean red ginseng is known to affect obesity, dyslipidemia, liver disease and liver function, but the mechanism of its effect is not clear. This study examined the beneficial effects of hypertriglyceridemia and the mechanism of action of Jinan red ginseng extract (JRG) in hepatoma cells. To measure the levels of triglyceride accumulation, we studied the expression of proteins and mRNAs related to lipidogenesis in hepatoma cells (Huh7 and HepG2). JRG decreases the lipidogenic markers, peroxisome proliferator-activated receptor γ (PPARγ), CCAAT-enhancer-binding proteins α (C/EBPα) and C/EBPβ which are major regulators of triglyceride synthesis in hepatoma cells. We also found that JRG reduced sterol regulatory element binding proteins 1c (SREBP-1c), C/EBPα and C/EBPβ by regulating liver X receptor (LXR) and stearoyl CoA desaturase (SCD) expressions. In addition, the first-limited step of synthesis triglyceride (TG), glycerol-3-phosphate (G3P) is decreased by JRG. These results suggest that the anti-hypertriglyceride effect of JRG in hepatoma cells could be accompanied with the inhibition of lipidogenic transcription factors by regulating LXR and SCD expression.