• Korean Journal of Clinical Pharmacy
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• 제32권2호
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• pp.125-132
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• 2022

Background: Fixed-dose combinations have the advantage of improving patient compliance, but may increase the risk of duplicate prescriptions. As the use of fixed-dose combination antihypertensives increases, it is necessary to investigate the current status of class duplication prescriptions (CDP) in patients taking fixed-dose combination antihypertensives in Korea and to identify factors associated with CDP. Methods: We conducted a retrospective observational study using nationally representative claim data. Hypertensive patients aged 20 years or older taking fixed-dose combination antihypertensives were extracted. Among these patients, patients with CDP were identified. A chi-square test was applied to determine the differences between patients with CDP and non-CDP. The associated factors of CDP were identified through multiple logistic regression. Results: Of the 74,165 patients who were prescribed fixed-dose combination antihypertensives, 426 patients (0.6%) with CDP were identified. The most common antihypertensive class associated with CDP was calcium channel blockers (194 patients, 45.5%), followed by angiotensin II receptor blockers (136 patients, 31.9%). Patients aged 75 years or older (odds ratio [OR] 1.83, 95% confidence interval [CI] 1.02-3.52), chronic kidney disease (OR 4.45, 95% CI 2.15-8.25), chronic heart failure (OR 2.71, 95% CI 1.93-3.72), coronary artery disease (OR 2.22, 95% CI 1.60-3.03) and Medical Aid/Patriots and Veterans Insurance (OR 1.49, 95% CI 1.04-2.07) were significantly associated with increased CDP. Conclusions: The factors associated with CDP were the elderly, comorbidities, and low socioeconomic status. Since CDP can result in negative clinical outcomes, active intervention by the pharmacist is warranted.

• Journal of The Korean Society of Inherited Metabolic disease
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• 제18권3호
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• pp.87-94
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• 2018

We present the case of long-term observation of a patient with chronic kidney disease (CKD) caused by advanced focal segmental glomerulosclerosis (FSGS) resulting from underlying congenital chloride diarrhea (CLD). A 20-year-old woman was admitted for prolonged proteinuria despite conservative treatment for CLD. She was diagnosed with CLD and started taking KCl salt supplementation from the time of birth. Mild proteinuria was first found at 12 years of age, which progressed to moderate proteinuria at 16 years of age. At 16 years of age, CKD stage 2 with FSGS was diagnosed based on the initial assessment of the glomerular filtration rate (GFR) and kidney histology. On admission, we re-assessed her renal function, histology and genetic analysis. GFR had deteriorated to CKD stage 4 and renal histology revealed an advanced FSGS combined with tubulointerstitial fibrosis. A homozygous mutation in the SLC26A3 gene (c.2063-1G>T) was found by diagnostic exome sequencing and may have been inherited from both parents. CLD patients can be more vulnerable to renal injury, which may also cause progression of renal failure. Therefore, even if there is an early diagnosis and adequate salt supplementation, close monitoring of renal function and tailored treatment should be emphasized for renal protection and favorable CLD prognosis.

• Asian Pacific Journal of Cancer Prevention
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• 제14권3호
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• pp.1597-1599
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• 2013

Since renal failure causes decrease in tumor marker excretion, use of these markers in cancer care and treatment in patients with renal insufficiency or hemodialysis is controversial. The aim of this study was to investigate differences of serum levels of tumor markers CA15-3, AFP, CA19-9 and CEA in patients with impaired renal function. A total of 100 patients referred to the Tabriz Immam Reza and Amiralmomenin hospital from June 2010 to November 2011 were selected for study. Subjects were divided to 3 groups of healthy, dialysis and renal failure but non hemodialysis cases, the last category being re-grouped based on creatinine clearance. No significant relationship between different groups in serum levels of CEA (P=0.99) and CA19-9 (P=0.29) tumor markers was found. A significant correlation was observed between serum levels of AFP (P<0.001) and CA15-3 (P<0.001) and also a tendency between creatinine clearance and CEA (r=0.05, P=0.625). Creatinine clearance significantly correlated with AFP (P<0.001, r=0.53) and CA15-3 (p=0.00, r=-0.412), but not CA19-9 (P=0.089, r=-0.171). According to results of this study it appears that use of tumor markers in patients with impaired renal function should be performed with special precautions.

• Childhood Kidney Diseases
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• 제12권1호
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• pp.99-104
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• 2008

Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.

• Journal of The Korean Society of Inherited Metabolic disease
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• 제15권1호
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• pp.9-17
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• 2015

Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

• Journal of Nutrition and Health
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• 제53권1호
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• pp.1-12
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• 2020

Purpose: Diabetic nephropathy is one of the most important diabetic complications prompted by chronic hyperglycemia, characterized by glomerulosclerosis, tubular fibrosis, and it eventually causes kidney failure. Nobiletin is a polymethoxyflavone present in tangerine and other citrus peels, and has anti-cancer and anti-inflammatory effects. This study investigated the effects of nobiletin on glomerular fibrosis through inhibition of the transforming growth factor (TGF)-β1-Src-caveolin-1 pathway. Methods: Human renal mesangial cells (HRMC) were incubated in media containing 33 mM glucose with or without 1-20 uM nobiletin for 3 day. The cellular expression levels of fibrogenic collagen IV, fibronectin, connective tissue growth factor (CTGF), TGF-β1, Src and caveolin-1 were all examined. In addition, TGF-β1, Src and caveolin-1 proteins were screened to reveal the relationship among TGF-β1-Src-caveolin-1 signaling in glomerular fibrosis. Results: High glucose promoted the production of collagen IV, fibronectin and CTGF in HRMC, which was inhibited in a dose dependent manner by 1-20 uM nobiletin. The Western blot data showed that high glucose elevated the expression of TGF-β1, Src, caveolin-1 and Rho GTPase. When nobiletin was treated to the HRMC exposed to high glucose, the expression of TGF-β1-Src-caveolin-1 was dampened. Finally, TGF-β1-Src-caveolin-1 signaling pathway was activated in high glucose-exposed HRMC, and such activation was encumbered by nobiletin. Conclusion: These result demonstrated that nobiletin blunted high glucose-induced extracellular matrix accumulation via inhibition of the TGF-β1-Src-caveolin-1 related intracellular signaling pathway. Nobiletin may be a potent renoprotective agent to counteract diabetes-associated glomerular fibrosis that leads to kidney failure.

• Journal of Korean Academy of Fundamentals of Nursing
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• 제6권1호
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• pp.78-95
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• 1999

The grafting of a kidney has been found to be the best medical treatment for patients who have renal insufficiency failure, but the patients still have experienced much trouble and apprehension. This study was done to further nursing theory developing for patients who have has a kidney graft from another person. The research method followed grounded theory methodology of Strauss and Corbin. The subjects were three female and four male patients. This study done befween Oct. 1997 and Mar. 1998. All of the subjects were interviewed by the author. Interview were done by the long interview technique and observation. In the process of data analysis, 'heart-boiling' was found to be the core phenomenon. The results were composed to 101 concepts. These concepts were grouped into nineteen categories, and then to twelve categories. There were 12 super-class categories as follows ; 'pain', 'heart-boiling', 'experience of dialysis', 'term of admission to a hospital', 'support of other person', 'dependence on God', 'direction', 'negative reaction', 'positive reaction', 'comfortable', 'lacking', 'acceptance'. In this process, 14 hypotheses were derived from the categories as follows ; (1) The more experience with dialysis that the patients have, the stronger the heart-boiling will tend to be. (2) The less experience with dialysis the patients have, the weaker the heart-boiling will tend to be. (3) The longer admission to hospital the patients have, the stronger the heart-boiling will be. (4) The shorter the admission to hospital the patients have, the weaker the heart-boiling will be. (5) The weaker the intense-grief is, the more positive the reaction to heart-boiling the patients wll have. (6) The stronger the intense-grief is, the more negative the reaction to heart-boiling the patients will have. (7) The stronger the support of other persons that the patients have, the more positive the reaction to heart-boiling the patients will have. (8) The weaker the support of other person that the patients have, the more negative the reaction to heart-boiling the patients will have. (9) The stronger the dependence on God that the patients have, the mure positive reaction to heart-boiling the patients will have. (10) The weaker the dependence on God that the patients have, the more negative reaction to heart-boiling the patients will have. (11) The more positive thoughts that the patients have, the more positive reaction to heart-boiling the patinets will have. (12) The more negative thoughts that the patients have, the more negative reaction to heart-boiling the patients will have. (13) The more positive reaction the patients have, the more free from heart-boiling the patients tend to be. (14) The more negative reaction the patients have, the less free from heart-boiling the patients tend to be. From the analysis of observed data and comparing each class, I concluded that there are four formula relation types between reaction of patients and heart-boiling. (1) If patients have the experience of dialysis, have a long term admission to hospital, are strong in heart-boiling, depend on God, have positive thoughts and another's strong support, they experience release by positive reaction to the intense-grief. (2) If patients have the experience of dialysis, have a short term admission to hospital, are weak in heart-boiling, do not depend on God, have negative thoughts, and have few supports from others, they experience attachment to heart-boiling though a negative reaction. (3) If patients have the experience of dialysis, have a long term admission to hospital, are strong in heart-boiling, do not depend on God, and have negative thoughts, they experience attachment to heart-boiling through negative reaction in spite of support from another. (4) If patients have the experience of dialysis, have a long term admission into hospital, are strong in heart-boiling and satisfaction is low, but they have positive thoughts, then they experience acceptance and harmony through the positive reaction to heart-boiling. The results of this study are expected to help the way nurses care for patients who have had a kidney graft from another.

• Journal of The Korean Society of Inherited Metabolic disease
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• 제17권3호
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Childhood Kidney Diseases
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• 제4권2호
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• pp.92-101
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• 2000

Purpose : We analyzed the demogaphic data md clinical course of Korean children with chronic renal failure (CRF) observed between 1990 and 1999. Patients and Methods : Questionnaires were mailed to all children's hospitals ail through the country. We asked for primary renal disease age and serum creatinine levels at first presentation with CRF and end-stage renal disease (ESRD), and modes of renal replacement therapy (RRT). Results : 401 children (254 boys, 147 girls) with CRF, defined as a permanent increase of serum creatinine above 1.2 mg/dl for at least 3 months or until death, were identified. This represents an incidence of 3.68 per million child population per year. Of these patients, 22$\%$ on younger than 5 years, 28$\%$ 5 to 10 years and 50$\%$ 10 to 15 year. Eight five $\%$ of the patients could be classified with a primary renal disease. The most frequent cause is glomerulonephritis (36$\%$), followed by chronic pyelonephritis (21$\%$), renal hrpo/dylplasia (9$\%$), and hereditary nephropathies (7$\%$). Reflux nephropathy (16$\%$) was the most common single cause of CRF. ESRD was reached in 70$\%$ of all patient. 99.3$\%$ of these started RRT. Hemodialysis (HD, 42$\%$), peritoneal dialysis (PD, 35$\%$) and transplantation (TP, 23$\%$) were performed as the initial mode of RRT. A total of 161 TPs were performed (159 first grafts, 2 second grafts). A total of 32 patients died. The main causes of death were dialysis related complication in HD patients and infections in PD patients. Survival rate on any form of RRT was 88.7$\%$ during the mean follow-up period of 37 months. Conclusion Major efforts should be directed toward earlier diagnosis and treatment of reflux nephropathy to prevent occurrence of Of. Dialysis and TP have now become well accepted forms of treatment in Korean children with ESRD.

• Childhood Kidney Diseases
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• 제12권1호
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• pp.38-46
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• 2008

Purpose: Growth failure is a common problem in chronic renal failure(CRF). We studied the effect of growth hormone(GH) treatment and the factors influencing growth on chronic peritoneal dialysis patients. Methods: Seventeen patients who were treated with peritoneal dialysis and GH for more than one year were enrolled. Factors influencing growth such as age, height at start of GH treatment, total Kt/Vurea, residual renal Kt/Vurea, hemoglobin, albumin, BUN, creatinine, total $CO_2$, calcium, phosphate and iPTH during GH treatment were compared between the growth group (increase in height-standard deviation score(Ht-SDS) after one year of GH treatment, n=l1) and poor growth group(no increase in Ht-SDS after one year of GH treatment, n=6). Results: The mean age at the start of dialysis was 7.7${\pm}$5.2 years and the mean age at the start of GH treatment was 8.5${\pm}$4.8 years. In the growth group, Ht-SDS at start of GH treatment was smaller(-1.72${\pm}$1.00 vs. -0.77${\pm}$0.88, P=0.048) and residual renal Kt/Vurea was better (1.54${\pm}$0.51 vs. 0.15${\pm}$0.26, P=0.02) than the poor growth group. After three years of GH treatment, Ht-SDS of the growth group was better than the poor growth group. Conclusion: GH treatment in children with peritoneal dialysis was more effective on patients who had more severe growth retardation. The reservation of residual renal function was important for improvement of effect of GH treatment. And the growth response during the first year of GH treatment may be predicted as the indicator for long-term response.