• Pediatric Infection and Vaccine
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• 제18권1호
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• pp.15-22
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• 2011

Invasive Candida infections (ICI) have become the third most common cause of late-onset infection among premature infants in the neonatal intensive care unit (NICU). Risk factors include birth weight less than 1,000 g, exposure to more than two antimicrobials, third generation cephalosporin exposure, parenteral nutrition including lipid emulsion, central venous catheter, and abdominal surgery. Candida colonization of the skin and gastrointestinal tract is an important first step in the pathogenesis of invasive disease. Strict infection control measures against the infection should be done in the NICU. The following practices are likely to contribute to reducing the rate of ICI: (1) restriction of broad-spectrum antibiotics, antacids and steroid; (2) introduction of early feeding and promoting breast milk. Fluconazole prophylaxis may be an effective control measure to prevent Candida colonization and infections in individual units with high incidence of fungal infection. In addition, there is a need of further data including the development of resistant strains and the effect on long-term neurodevelopmental outcomes of infants exposed to drugs before the initiation of routine application of antifungal prophylaxis in the NICU.

• Clinical and Experimental Pediatrics
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• 제60권3호
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• pp.55-63
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• 2017

Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic lesions such as focal segmental glomerulosclerosis or diffuse mesangial sclerosis. This paper provides an update on the current knowledge of podocyte genes involved in the development of hereditary nephrotic syndrome and, thereby, reviews genotype-phenotype correlations to propose an approach for appropriate mutational screening based on clinical aspects.

• Journal of Oral Medicine and Pain
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• 제43권1호
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• pp.16-20
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• 2018

Graft-versus-host disease (GVHD) is frequent complications of hematopoietic stem cell transplantation. In the chronic GVHD (cGVHD), the oral cavity is the most commonly affected region. The clinical manifestations include erythema, ulceration, lichenoid-hyperkeratotic change in oral mucosa, dry mouth, and limitation of mouth opening. The initial treatment strategy of oral cGVHD patients is topical corticosteroid therapy in various formulation. However, corticosteroid resistance appears in some patients. We report a case of a 25-year-old male patient with oral cGVHD, who has resistance to topical corticosteroid medication, treated with 0.03% tacrolimus ointment and low-dose doxycycline. The patient showed subjective and objective improvement without side effect.

• Journal of Yeungnam Medical Science
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• 제35권2호
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• pp.222-226
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• 2018

Recurrent pericarditis is rare in children and is considered idiopathic in most cases. Its course is chronic, and preventing recurrences is important for the patient's quality of life. Although a treatment strategy in pediatric recurrent pericarditis has not yet been established, non-steroidal anti-inflammatory drugs (NSAIDs) are the most common treatment for management of this condition, followed by corticosteroids, colchicine, immunosuppressive agents, immunoglobulins, and interleukin-$1{\beta}$ receptor antagonists (e.g. anakinra). Herein, we report a case of recurrent pericarditis with pericardial effusion in a 5-year-old child who presented with fever and epigastric pain. He responded poorly to NSAIDs and corticosteroid therapy, but was successfully treated with colchicine.

• Childhood Kidney Diseases
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• 제25권1호
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• pp.14-21
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• 2021

Chronic kidney disease, the presence of structural and functional abnormalities in the kidneys, is associated with a lower quality of life and increased morbidity and mortality in children. Genetic etiologies account for a substantial proportion of pediatric chronic kidney disease. With recent advances in genetic testing techniques, an increasing number of genetic causes of kidney disease continue to be found. Genetic testing is recommended in children with steroid-resistant nephrotic syndrome, congenital malformations of the kidney and urinary tract, cystic disease, or kidney disease with extrarenal manifestations. Diagnostic yields differ according to the category of clinical diagnosis and the choice of test. Here, we review the characteristics of genetic testing modalities and the implications of genetic testing in clinical genetic diagnostics.

• Journal of Chest Surgery
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• 제30권10호
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• pp.1040-1043
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• 1997

식도암 .수술후 발생한 bronchiolitis obliterans organizing pneumonia환자 1례를 보고하고자 한다. 이 환자는 수술후 4일째부터 미열, 마른기침, 경미한 호흡곤란을 호소하였다 이때 촬영한 단순 흉부 사진과 흉부 컴퓨터 촬영상 폐양측에 반성(Patchy)침습소견이 나타났다. 고식적 치료에도 불구하고 경도의 백혈구 증다증과 함께 호흡기 증상은 더욱 악화되어 호흡부전의 소견을 보였다. 개흉적 폐생검을 실시하였으며, 병리조직검사 상 BOOP라는 진단을 얻었다. 수주간에 걸친 부신피질호르몬치료후 임상적, 생리학적, 그리고 방사선검사에서 많은 호전을 보였다. 이에 우리는 개흉술후 발생될 수 있는 급성호흡부전증에서 BOOP도 그 원인이 될 수 있음을 알았다. 이 경우 부신피질호르몬치료로 좋은 효과를 얻을 수 있으므로 개흉술후 발생되는 급성호 흡부전의 경우 가능한 조속히 조직검사를 시행할 팔요가 있음을 알 수 있었다.

• Clinical and Experimental Pediatrics
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• 제64권7호
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• pp.355-363
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• 2021

Background: Nephrotic syndrome (NS) is a common renal disorder in children attributed to podocyte injury. However, children with the same diagnosis have markedly variable treatment responses, clinical courses, and outcomes, suggesting molecular heterogeneity. Purpose: This study aimed to explore the molecular responses of podocytes to nephrotic plasma to identify specific genes and signaling pathways differentiating various clinical NS groups as well as biological processes that drive injury in normal podocytes. Methods: Transcriptome profiles from immortalized human podocyte cell line exposed to the plasma of 8 subjects (steroid-sensitive nephrotic syndrome [SSNS], n=4; steroid-resistant nephrotic syndrome [SRNS], n=2; and healthy adult individuals [control], n=2) were generated using microarray analysis. Results: Unsupervised hierarchical clustering of global gene expression data was broadly correlated with the clinical classification of NS. Differential gene expression (DGE) analysis of diseased groups (SSNS or SRNS) versus healthy controls identified 105 genes (58 up-regulated, 47 down-regulated) in SSNS and 139 genes (78 up-regulated, 61 down-regulated) in SRNS with 55 common to SSNS and SRNS, while the rest were unique (50 in SSNS, 84 genes in SRNS). Pathway analysis of the significant (P≤0.05, -1≤ log2 FC ≥1) differentially expressed genes identified the transforming growth factor-β and Janus kinase-signal transducer and activator of transcription pathways to be involved in both SSNS and SRNS. DGE analysis of SSNS versus SRNS identified 2,350 genes with values of P≤0.05, and a heatmap of corresponding expression values of these genes in each subject showed clear differences in SSNS and SRNS. Conclusion: Our study observations indicate that, although podocyte injury follows similar pathways in different clinical subgroups, the pathways are modulated differently as evidenced by the heatmap. Such transcriptome profiling with a larger cohort can stratify patients into intrinsic subtypes and provide insight into the molecular mechanisms of podocyte injury.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.123-129
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• 1997

목적 : 소아의 스테로이드 저항성 신증후군은 예후가 불량함에도 불구하고 지금까지 효과적인 치료방법이 없는 병이었으나 1990년 Mendoza등이 Methylprednisolone 충격 요법이 스테로이드 저항성 신증후군에서 효과적이라는 것을 보고하였다. 하지만 2년 뒤 Waldo 등은 Methylprednisolone 충격 요법이 Mendoza 등의 보고와는 다르게 스테로이드 저항성 신증후군에 효과가 적었다고 보고하여 이에 저자는 한국 소아에서 스테로이드 저항성 신증후군이 발생한 경우 Methylprednisolone 충격 요법의 효과를 알아보고 위의 보고의 서로 다른 결과를 확인하고자 본 연구를 시행하였다. 방법 : 1990년부터 1995년까지 만 5년간 서울대병원 소아과에 스테로이드 저항성 신증후군으로 입원한 소아를 대상으로 Methylprednisolone 충격 요법을 시행하여 $30{\pm}11$개월동안 추적 관찰하였다. 결과 : 1) Methylprednisolone 충격 요법 치료로 신증후군이 완해가 유도된 환아는 20명중 9명으로 45%이었다. 2) Methylprednisolone 충격 요법 치료후 신증후군의 완해가 유지된 환아는 20명중 9명으로 45%이었다. 3) Methylprednisolone 충격 요법에 반응이 없었던 환아중 만성 신부전으로 이행된 경우는 5례로서 25%이었다. 결론 : Methylprednisolone 충격 요법은 스테로이드 저항성 신증후군 소아의 45%에서 신증후군의 완해를 유도하고 유지시키는 효과적인 치료 방법이었다.

• 한국식물생명공학회:학술대회논문집
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• 한국식물생명공학회 2002년도 추계학술대회
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• pp.69-75
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• 2002

Sterols play two major roles in plants: a bulk component in biological membranes and precursors of plant steroid hormones. Physiological effects of plant steroids, brassinosteroids (BRs), include cell elongation, cell division, stress tolerance, and senescence acceleration. Arabidopsis mutants that carry genetic defects in BR biosynthesis or its signaling display characteristic phenotypes, such as short robust inflorescences, dark-green round leaves, and sterility. Currently there are more than 100 dwarf mutants representing 7 genetic loci in Arabidopsis. Mutants of 6 loci, dwf1/dim1/cbb1, cpd/dwf3, dwf4, dwf5, det2/dwf6, dwf7 are rescued by exogenous application of BRs, whereas bri1/dwf2 shares phenotypes with the above 6 loci but are resistant to BRs. These suggest that the 6 loci are defective in BR biosynthesis, and the one locus is in BR signaling. Biochemical analyses, such as intermediate feeding tests, examining the levels of endogenous BR, and molecular cloning of the genes revealed that dwf7, dwf5, and dwf1 are defective in the three consecutive steps of sterol biosynthesis, from episterol to campesterol via 5-dehydroepisterol. Similarly, det2/dwf6, dwf4, and cpd/dwf3 were shown to be blocked in $D^4$ reduction, 22a-hydroxylation, and 23 a-hydroxylation, respectively. A signaling mutant bri1/dwf2 carries mutations in a Leucine-rich repeat receptor kinase. Interestingly, the bri1 mutant was shown to accumulate significant amount of BRs, suggesting that signaling and biosynthesis are dynamically coupled in Arabidopsis. Thus It is likely that transgenic plants over-expressing the rate-limiting step enzyme DWF4 as well as blocking its use by BRI1 could dramatically increase the biosynthetic yield of BRs. When applied industrially, BRs will boost new sector of plant biotechnology because of its potential use as a precursor of human steroid hormones, a novel lead compound for cholesterol-lowering effects, and a various application in plant protection.

• 한국식물생명공학회:학술대회논문집
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• 한국식물생명공학회 2002년도 춘계학술대회
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• pp.69-75
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• 2002

Sterols play two major roles in plants: a bulk component in biological membranes and precursors of plant steroid hormones. Physiological effects of plant steroids, brassinosteroids (BRs), include cell elongation, cell division, stress tolerance, and senescence acceleration. Arabidopsis mutants that carry genetic defects in BR biosynthesis or its signaling display characteristic phenotypes, such as short robust inflorescences, dark-green round leaves, and sterility. Currently there are more than 100 dwarf mutants representing 7 genetic loci in Arabidopsis. Mutants of 6 loci, dwf1/dim1/cbb1, cpd/dwf3, dwf4, dwf5, det2/dwf6, dwf7 are rescued by exogenous application of BRs, whereas bri1/dwf2 shares phenotypes with the above 6 loci but are resistant to BRs. These suggest that the 6 loci are defective in BR biosynthesis, and the one locus is in BR signaling. Biochemical analyses, such as intermediate feeding tests, examining the levels of endogenous BR, and molecular cloning of the genes revealed that dwf7, dwf5, and dwf1 are defective in the three consecutive steps of sterol biosynthesis, from episterol to campesterol via 5-dehydroepisterol. Similarly, det2/dwf6, dwf4, and cpd/dwf3 were Shown to be blocked in $D^4$ reduction, 22a-hydroxylation, and 23 a-hydroxylation, respectively. A signaling mutant bri1/dwf2 carries mutations in a Leucine-rich repeat receptor kinase. Interestingly, the bri1 mutant was shown to accumulate significant amount of BRs, suggesting that signaling and biosynthesis are dynamically coupled in Arabidopsis. Thus it is likely that transgenic plants over-expressing the rate-limiting step enzyme DWF4 as well as blocking its use by BRI1 could dramatically increase the biosynthetic yield of BRs. When applied industrially, BRs will boost new sector of plant biotechnology because of its potential use as a precursor of human steroid hormones, a novel lead compound for cholesterol-lowering effects, and a various application in plant protection.