• Journal of mucopolysaccharidosis and rare diseases
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• v.3 no.1
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• pp.33-33
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• 2017

• 대한소아신장학회:학술대회논문집
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• 2006.10a
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• pp.6-6
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• 2006

• Clinical and Experimental Pediatrics
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• v.64 no.11
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• pp.599-601
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• 2021

• Experimental and Molecular Medicine
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• v.50 no.11
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• pp.5.1-5.14
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• 2018

Oxidative stress-induced mitochondrial dysfunction is implicated in the pathogenesis of intervertebral disc degeneration (IVDD). Sirtuin 3 (SIRT3), a sirtuin family protein located in mitochondria, is essential for mitochondrial homeostasis; however, the role of SIRT3 in the process of IVDD has remained elusive. Here, we explored the expression of SIRT3 in IVDD in vivo and in vitro; we also explored the role of SIRT3 in senescence, apoptosis, and mitochondrial homeostasis under oxidative stress. We subsequently activated SIRT3 using honokiol to evaluate its therapeutic potential for IVDD. We assessed SIRT3 expression in degenerative nucleus pulposus (NP) tissues and oxidative stress-induced nucleus pulposus cells (NPCs). SIRT3 was knocked down by lentivirus and activated by honokiol to determine its role in oxidative stress-induced NPCs. The mechanism by which honokiol affected SIRT3 regulation was investigated in vitro, and the therapeutic potential of honokiol was assessed in vitro and in vivo. We found that the expression of SIRT3 decreased with IVDD, and SIRT3 knockdown reduced the tolerance of NPCs to oxidative stress. Honokiol ($10{\mu}M$) improved the viability of NPCs under oxidative stress and promoted their properties of anti-oxidation, mitochondrial dynamics and mitophagy in a SIRT3-dependent manner. Furthermore, honokiol activated SIRT3 through the AMPK-PGC-$1{\alpha}$ signaling pathway. Moreover, honokiol treatment ameliorated IVDD in rats. Our study indicated that SIRT3 is involved in IVDD and showed the potential of the SIRT3 agonist honokiol for the treatment of IVDD.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.249-256
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• 2006

Diarrhea-associated hemolytic uremic syndrome(D+ HUS) is induced by enterohemorrhagic Escherichia coli(EHEC) and is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The disease is usually transmitted by meat and water contaminated by excreta of domestic animals. We report a son and his mother with diarrhea-associated hemolytic uremic syndrome that spread within the family.

• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.358-361
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• 2015

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.

• Advances in pediatric surgery
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• v.18 no.1
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• pp.18-23
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• 2012

Totally implantable venous devices (TIVD)는 장기간 항암 화학 요법이나 총 정맥 영양, 수액치료가 필요한 환자에서 많이 사용된다. TIVD는 매우 유용하고 안전한 장치이지만, 이와 관련된 다양한 합병증이 보고 되어 왔다. 저자들은 카테터의 혈관 내 유착에 의해 TIVD의 제거가 불완전하게 된 2예를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• v.22 no.2
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• 대한소아신장학회:학술대회논문집
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• 2007.04a
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• pp.9-11
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• 2007

• Human Ecology Research
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• v.61 no.2
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• pp.169-182
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• 2023

This study aimed to investigate the mediating effect of the screen media time of young children on the relationship between mothers' reasons for allowing young children to use screen media and their children's ability to regulate screen media. The participants consisted of 614 children aged 5-6 (312 boys and 302 girls), and all the survey questions were answered by their mothers. Descriptive statistics, Pearson's correlation, structural equation modeling, and bootstrapping analysis were employed to analyze the data using SPSS 25.0 and Mplus 8.6. The results were as follows. First, mothers' educational and ritualized reasons for allowing young children to use screen media and children's screen media time directly affected young children's ability to regulate screen media. Second, children's screen media time mediated the relationship between mothers' educational and ritualized reasons for allowing their children to use screen media and children's ability to regulate screen media. When mothers permitted their children to use screen media for educational purposes, their children were likely to spend less time on screen media usage which, in turn, increased their ability to regulate screen media. Conversely, the more mothers allowed their children to use screen media for ceremonial purposes, the more time their children spent on screen media and the less ability they had to regulate screen media usage. These outcomes suggest that, to improve children's screen media regulation ability, mothers must not routinely allow their children to use screen media and should manage their children's screen media time.