• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.4
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• pp.207-218
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• 2013

Long term outcomes after liver transplantation are major determinants of quality of life and of the value of this heroic treatment. As short term outcomes are excellent, our community is turning to take a harder look at long term outcomes. The purpose of this paper is to review these outcomes, and highlight proposed treatments, as well as pressing topics needing to be studied. A systemic review of the English literature was carried in PubMed, covering all papers addressing long term outcomes in pediatric liver transplant from 2000-2013. Late outcomes after pediatric liver transplant affect the liver graft in the form of chronic liver dysfunction. The causes include rejection particularly humoral rejection, but also de novo autoimmune hepatitis, and recurrent disease. The metabolic syndrome is a major factor in long term cardiovascular complication risk. Secondary infections, kidney dysfunction and malignancy remain a reality of those patients. There is growing evidence of late cognitive and executive function delays affecting daily life productivity as well as likely adherence. Finally, despite a good health status, quality of life measures are comparable to those of children with chronic diseases. Long term outcomes are the new frontier in pediatric liver transplantation. Much is needed to improve graft survival, but also to avoid systemic morbidities from long term immunosuppression. Quality of life is a new inclusive measure that will require interventions and innovative approaches respectful not only on the patients but also of their social circle.

• Journal of Yeungnam Medical Science
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• v.37 no.4
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• pp.269-276
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• 2020

Fibrosis is characterized by excessive accumulation of extracellular matrix components. The fibrotic process ultimately leads to organ dysfunction and failure in chronic inflammatory and metabolic diseases such as pulmonary fibrosis, advanced kidney disease, and liver cirrhosis. Idiopathic pulmonary fibrosis (IPF) is a common form of progressive and chronic interstitial lung disease of unknown etiology. Pathophysiologically, the parenchyma of the lung alveoli, interstitium, and capillary endothelium becomes scarred and stiff, which makes breathing difficult because the lungs have to work harder to transfer oxygen and carbon dioxide between the alveolar space and bloodstream. The transforming growth factor beta (TGF-β) signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis and scarring of the lung tissue. Recent clinical trials focused on the development of pharmacological agents that either directly or indirectly target kinases for the treatment of IPF. Therefore, to develop therapeutic targets for pulmonary fibrosis, it is essential to understand the key factors involved in the pathogenesis of pulmonary fibrosis and the underlying signaling pathway. The objective of this review is to discuss the role of kinase signaling cascades in the regulation of either TGF-β-dependent or other signaling pathways, including Rho-associated coiled-coil kinase, c-jun N-terminal kinase, extracellular signal-regulated kinase 5, and p90 ribosomal S6 kinase pathways, and potential therapeutic targets in IPF.

• Journal of the East Asian Society of Dietary Life
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• v.11 no.1
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• pp.1-10
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• 2001

Since the Industrial Revolution, people have enjoyed an abundant life, owing to the technological innovations of science. However, because of changes in the environment or polution to the environment, it has brought on many chronic diseases. Especially, even though it may be a small amount, if cadmium gets into a human body, because its biological half life is long, it fatally causes a kidney disease and damage to human organisms. It was reported that Metallothioneins(MT), a substance that is closely related with a free radical that comes from environmental pollution and the course of which cadmium, which causes chronic addition in the body, is formed, along with harmful metals, make the toxicity reduce. MT compound led into the body by cadmium indirectly functions as an antioxidation, supplied adequate amount of vitamin E, and suppresses the accumulation of cadmium in heart, liver and blood. Cadmium content found in Korean soil according to the test conducted from 1992 to 1996 was 0.02~0.03mg/kg in cereals, pulses, and potatoes. Free radical scavenger, finding solution for metal substance that comes from environmental pollution from food and natural substances implicates a better future for the study of food science.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.164-175
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• 2001

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)

• Journal of Appropriate Technology
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• v.6 no.2
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• pp.163-173
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• 2020

For the successful execution of an ODA project, it is necessary to know what areas are weak and necessary to the country of demand exactly. The health sector is also a top priority in most of developing countries. This study was carried out to introduce non-communicable disease (NCD) in Fiji for ODA projects planning. The major causes of death in Fiji in 2016 are diabetes, ischemic heart disease, cerebrovascular disease, chronic kidney disease, lower respiratory infect, asthma in ranking. The major causes of death in Korea in same year are cancer, ischemic heart diseases, cerebrovascular diseases, pneumonia, suicide, diabetes in the order of ranking. The chronic disease as non-communicable disease (NCD) has been increasing continuously due to changes in lifestyle and consumption patterns and population aging in prevalence rate. This global trend is also apparent in Fiji and Korea, reflected in increasing mortality and personal costs for the treatment and management of NCD. The need for a sustained comprehensive treatment tailored for individual patients has suggested from many studies and the development of a systematic program to manage NCD patients to provide such care have been recommended. The Fiji government developed Non-communicable Diseases Strategic Plan 2015-2019 and has tried to reduce the prevalence rate of non-communicable diseases by factors. The WHO global action plan guiding national-level NCD policies requires an NCD prevention and control model at the community level, presenting strategic goals and detailed options for the introduction and application of the approach to communities. It is necessary to develop an NCD prevention and control model, consisting of a strategy of community intervention, education for students and NCD patients, and the legal enactment of NCD that adequately meets the needs of community members.

• Clinical and Experimental Pediatrics
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• v.55 no.4
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• pp.136-142
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• 2012

Purpose: Urinary excretion of N acetyl-beta-D glucosaminidase (NAG) and ${\beta}_2$-microglobulin (${\beta}_2$-M) was increased in the presence of proximal tubular damage. Based on these urinary materials, we investigated the ability of expecting renal function in chronic glomerular diseases. In this study, we evaluated the relationship between glomerular filtration rate (GFR) urinary NAG, and urinary ${\beta}_2$-M. Methods: We evaluated 52 children with chronic kidney disease at the Chung-Ang University Hospital between January 2003 and August 2009. We investigated the 24-hour urinalysis and hematologic values in all 52 patients. Serum creatinine, creatinine clearance (Ccr), serum cystatin C, urinary ${\beta}_2$-M and urinary NAG were measured. Results: Out of 52 patients, there were 13 children with minimal change in disease, 3 children with focal segmental glomerulosclerosis, 17 children with immunoglobulin A nephropathy, 15 children with Henoch-Sch$\ddot{o}$nlein purpua nephritis, 3 children with poststreptococcal glomerulonephritis, and 1 child with thin glomerular basement membrane disease. In these patients, there were significant correlation between the Ccr and urinary NAG (r=-0.817; $P$ <0.01), and between the GFR (as determined by Schwartz method) and urinary NAG (r=-0.821; $P$ <0.01). In addition, there was a significant correlation between the GFR (as determined by Bokencamp method) and urinary NAG (r=-0.858; $P$ <0.01). Conclusion: In our study, there was a significant correlation between the GFR and urinary NAG, but there was no correlation between the GFR and urinary ${\beta}_2$-M, suggesting that the GFR can be predicted by urinary NAG in patients with chronic glomerular disease.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.227-231
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• 1999

Hemolytic uremic syndrome is a clinical syndrome with various etiology and pathogenesis. And pneumococcal neuraminidase has been known to play a pathogenetic role in some cases with this syndrome. We experienced two children with hemolytic uremic syndrome complicated by pneumococcal infection. One was 21-month-old girl with pneumococcal pneumonia, and the other was 7-month-old girl with pneumococcal meningitis and sepsis. Both of them showed typical clinical manifestations of hemolytic uremic syndrome with prolonged anuria during the course of pneumococcal infection. The renal functions of both cases did not recovered after resolution of acute hemolytic episode and chronic renal failure developed.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.217-220
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• 1999

Kimura disease is a chronic benign disorder, primarily seen in asians male during the second and third decades of life, which presents itself as a tumour like lesion with a predilection for the head and neck region. There is high prevalence of associated renal disease. We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.7-21
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• 2019

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.35-39
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• 2017

Azathioprine is commonly used as immunosuppressive therapy for various inflammatory diseases including chronic glomerulonephritis. Myelosuppression is a common side effect of azathioprine, resulting in the need for dose reduction. However, severe pancytopenia or alopecia is not often encountered. Here, we report a case of severe myelosuppression, and alopecia totalis that occurred after azathioprine treatment in a patient with IgA nephropathy. A 10-year-old boy with IgA nephropathy was treated with oral deflazacort and later with azathioprine. After 4 weeks, the patient complained of hair loss, and despite a dose reduction in azathioprine, he developed bone marrow suppression and alopecia totalis in two weeks. The blood indices and alopecia of the patient had returned to normal after azathioprine withdrawal and 3 consecutive doses of granulocyte colony-stimulating factor. We suggest that physicians remain vigilant to the side effects of azathioprine. Unusual hair loss after azathioprine treatment might suggest a defect in the metabolism of the drug, warranting the discontinuation of azathioprine to prevent more severe side effects.