• Childhood Kidney Diseases
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• v.10 no.2
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• pp.219-227
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• 2006

Purpose : We aim to identify the clinical and demographic characteristics in children who underwent renal transplantation(RTx) and to evaluate the influence on growth of RTx in children. Methods : We reviewed 17 medical records of chronic renal failure patients who underwent RTx from April 1992 and June 2004 at Busan Paik Hospital. Age and sex distribution, cause of disease, donor analysis, patient and graft survival rate, and the status of growth after RTx were analysed by retrospective study. Results : Eighteen RTx were performed in 17 patients(8 boys, 9 girls). The mean age at the time of RTx was $15.8{\pm}3.5$ years and the mean duration of dialysis therapy before RTx was $22.4{\pm}18.0$ months. The 1 year and 5 year patient survival rate were each 100%, and the 1 year and 5 year graft survival rate were 88%, 36% respectively. The most common cause of graft failure was chronic rejection. The mean final height of male patients was $162.8{\pm}10.0$ cm(143.0-172.5 cm) and of female patients was $154.5{\pm}12.1$ cm(135.8-160.0 cm). The mean height standard deviation score(Ht SDS) increased after RTx from -1.95 to -1.53 but the increment rate was not statistically significant. Similar changes were noted in individual patient analysis. Also there was no significant difference between the living-related donors and cadaveric donors. Conclusion : Our data shows that even successful RTx rarely results in full growth rehabilitation. To overcome retarded growth in children with chronic renal failure, appropriate combined management of metabolic and nutritional problems, correction of anemia, proper use of recombinant growth hormone therapy, early renal transplantation and shortening of the duration of dialysis would be necessary.

• Childhood Kidney Diseases
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• v.4 no.2
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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.

• The Korean Journal of Nuclear Medicine
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• v.33 no.1
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• pp.49-56
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• 1999

Purpose: Heart to liver ratio on T1-201 per rectal scintigraphy (shunt index) is known to be useful in the assessment of portal systemic shunt. We assessed T1-201 uptake pattern and early liver/heart uptake rate of T1-201 and correlated with shunt index in patients with chronic active hepatitis (CAH) and liver cirrhosis (LC). Materials and Methods: Fifty eight patients with biopsy-proven chronic liver disease (35 with CAH, 23 with LC) underwent T1-201 per rectum scintigraphy after instillation of 18.5 MBq of T1-201 into the upper rectum. We evaluated hepatic uptake (type 1 : homogeneous, 2: inhomogeneous segmental, 3: inhomogeneous nonsegmental) and extrahepatic uptake of spleen, heart and kidney (grade 0: no uptake, 1: less than liver, 2: equal to liver, 3: greater than liver). We measured the early liver/heart uptake rate (the slope of the liver to heart uptake ratio for 10 min) and shunt index (heart to liver uptake ratio). T1-201 uptake pattern and early liver/heart uptake rate of T1-201 was correlated with the pathologic diagnosis and shunt index. Results: Hepatic uptake patterns of type 1 and 2 were dominant in CAH (CAH: 27/35, LC. 8/23), and type 3 in LC (CAH: 8/35, LC: 15/23)(p<0.005). The grades of extrahepatic uptake were higher in LC than in CAH (spleen: p<0.001, other soft tissue: p<0.005). The early liver/heart uptake rate of CAH ($0.110{\pm}0.111$) was significantly higher than that of LC ($0.014{\pm}0.090$)(p<0.001). The sensitivity and specificity of the early liver/heart uptake rate were 77.7% and 67.7% in differentiating LC from CAH. There was negative correlation between early liver/heart uptake rate and shunt index (r=-0.3347, p<0.01). Conclusion: Hepatic and extrahepatic uptake pattern and early liver/heart uptake rate on T1-201 per rectum scintigraphy are useful in the assessment of portal systemic shunt in patients with chronic liver disease.

• Journal of Chest Surgery
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• v.50 no.2
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• pp.86-93
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• 2017

Background: The influence of lifestyle diseases on postoperative complications and long-term survival in patients with non-small cell lung cancer (NSCLC) is unclear. The aim of this study was to determine whether lifestyle diseases were significant risk factors of perioperative and long-term surgical outcomes in elderly patients with stage I NSCLC. Methods: Between December 1995 and November 2013, 110 patients aged 65 years or older who underwent surgical resection of stage I NSCLC at Dong-A University Hospital were retrospectively studied. We assessed the presence of the following lifestyle diseases as risk factors for postoperative complications and long-term mortality: diabetes, hypertension, chronic obstructive pulmonary disease, stroke, and ischemic heart disease. Results: The mean age of the patients was 71 years (range, 65 to 82 years). Forty-six patients (41.8%) had hypertension, making it the most common lifestyle disease, followed by diabetes (n=23, 20.9%). The in-hospital mortality rate was 0.9% (n=1). The 3-year and 5-year survival rates were 78% and 64%, respectively. Postoperative complications developed in 32 patients (29.1%), including 7 (6.4%) with prolonged air leakage, 6 (5.5%) with atrial fibrillation, 5 (4.5%) with delirium and atelectasis, and 3 (2.7%) with acute kidney injury and pneumonia. Univariate and multivariate analyses showed that the presence of a lifestyle disease was the only independent risk factor for postoperative complications. In survival analysis, univariate analysis showed that age, smoking, body mass index, extent of resection, and pathologic stage were associated with impaired survival. Multivariate analysis revealed that resection type (hazard ratio [HR], 2.20; 95% confidence interval [CI], 1.08 to 4.49; p=0.030) and pathologic stage (HR, 1.89; 95% CI, 1.02 to 3.49; p=0.043) had independent adverse impacts on survival. Conclusion: This study demonstrated that the presence of a lifestyle disease was a significant prognostic factor for postoperative complications, but not of survival, in elderly patients with stage I NSCLC. Therefore, postoperative complications may be influenced by the presence of a lifestyle disease.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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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.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.48-56
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• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• Journal of Life Science
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• v.19 no.10
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• pp.1438-1443
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• 2009

Renal fibrosis is a final common manifestation of every type of chronic kidney disease. Plasminogen activator inhibitor (PAI)-1 is induced by lipopolysaccharide (LPS) and is known to play an essential role in the progress of renal fibrosis. In this paper, we found that an isoprenoid antibiotic, ascofuranone (AF), suppresses expression of profibrotic factors, PAI-1 and promoter activity of PAI-1 induced by LPS in rat kidney fibroblast cells. We therefore investigated signaling pathway mediated inhibitory effects of LPS-induced PAI-1 by AF in rNRK-49F cells. PAI-1 expression is suppressed by treatment with kinase inhibitors for MEK-1/2, as it isin inhibition of PAI-1 expression by AF, and AF inhibits phosphorylation of ERK-1/2. This study suggest that AF suppresses expression of PAI-1 through the inhibition of an ERK-1/2-dependent signal transduction pathway. The data indicates the possibility that AF can be used to prevent the development and progression of renal fibrosis.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.17-23
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• 1997

After the first description of IgA nephropathy by Berger in 1968, the prognosis of this disease was considered favourable. However recent studies have revealed that IgA nephropathy result in end stage renal desease in 25-30% by 20 years. Heavy proteinuria, hypertension, histological high class are regarded as poor prognostic factors. In 1996, Yagame et al reported the new histopathologic grading with a strong correlation between the grading, heavy proteinuria, high s-Cr level and renal survival. The aims of this study are to determine whether the pathological grading and other clinical parameters could contribute to predicting the outcome of this disease eventhough pediatric patients. Seventy nine patients (59 males, 20 females) with IgA nephropathy were examined. Patients were 2.08-15.17 years of age ($9.85{\pm}2.83$). The mean follow-up duration were $27{\pm}28$ months. Six of seventy nine patients progressed to chronic renal failure during the follow-up periods. High 24h urinary protein excretion at diagnosis were significantly higher in chronic renal failure patients (p<0.05). Hypertension at diagnosis were the significant associated factors in progression of chronic renal failure (p<0.05). Histological changes of IgA nephropathy in light microscopy were classified into five classes by WHO classification, four grades in Yagame's gradings. Among the seventy nine patients, 24 were as class 1, 30 as class 2, 23 as class 3; 4 as class 4, 0 as class 5 by WHO classification. 23 were classified grade 1, 31 as grade 2, 24 as grade 3, 1 as grade 4 by Yagame's grading. Among six patients who progressed to chronic renal failure, 1 clssified as class 1, 1 as class 2, 3 as class 3, 1 as class 4, 0 as class 5 by WHO Classification. 1 patients were classified as grade 1, 1 as grade 2, 3 as grade 3, 1 as grade 4 by Yagame's grading. (p>0.05) In conclusion, hypertension and heavy proteinuria at initial presentation were significantly associated with progression of chronic renal failure. The classification of WHO & Yagame's grading has no significant association with the progression of chronic renal failure in pediatric patients.

• Journal of Veterinary Clinics
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• v.29 no.6
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• pp.435-440
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• 2012

This study was designed to evaluate the clinical efficacy and safety of Rhubarb extracts ($Rubenal^{(R)}$) in dogs with chronic renal failure (CRF). Client-owned 40 dogs with CRF graded International renal interest Society (IRIS) II-III were enrolled in this study. The dogs were equally allocated and blindly administered with $Rubenal^{(R)}$ or placebo. The following items were evaluated at day 0, 30, 90 and 180: body condition score (BCS), clinical score (appetite, polydipsia/polyuria, quality of life score), hemogram (WBC, RBC, PCV), serum biochemistry (ALT/AST, ALP, Creatinine/BUN, total protein, albumin), serum electrolyte (Na, K, Cl, Ca, P), systolic blood pressure, urinalysis (UPC, USG) and IRIS stage. In this study, we found that the $Rubenal^{(R)}$ preparation was well tolerated by dogs and induced no adverse effects. Statistically significant improvements were observed in clinical score (quality of life score by vet and clients), serum BUN and creatinine levels, serum phosphorus concentration, level of proteinuria, and the IRIS score of CRF in dogs after 6 month of treatment of $Rubenal^{(R)}$. Those findings suggested that the Rhubarb extracts can improve the clinical signs of CRF (i.e. azotemia, hypertension, proteinuria, hyperphosphoremia) and the quality of life (i.e. BCS, clinical score) and can retard the progression of CRF in dogs. Therefore the Rhubarb extracts can be a good supplementary drug for treating dogs with subclinical and clinical renal diseases. However, care should be taken for interpreting our result, because this study is not double-blinded controlled study but pilot study.

• The Korean Journal of Nuclear Medicine
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• v.15 no.1
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• pp.27-32
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• 1981

HBsAg. was identified in the urine of the patients positive for serum HBsAg. by Tripatzis in 1970. In 1977, Hourani et al reported the incidence of HBsAg. in urine was about 52% in the patients positive for serum HBsAg. with hemodialysis treatment due to chronic renal failure. A series of studies on the HBsAg. in urine has revealed the urine of the patients positive for serum HBsAg. to be important source of infection. But there's much room to debate on the relationship of HBsAg. in urine with infectivity and the exact mechanism of urinary emergence of HBsAg. The authors detected HBsAg. in serum and urine by employing sandwitch solid-phase rad ioimmunoassay, and performed urinalysis, liver function test and renal function evaluation. Percutanous liver and/or kidney biopsis were done. Among 38 renal disease patients, 9 cases (23.4%) were shown to be positive for serum HBsAg. and 5 cases (55.5%) among above 9 patients positive for urine HBsAg.. 56 cases (67.4%) of 83 liver disease patients revealed positive for serum HBsAg. but only 11 cases (13.2%) among the 56 cases positive fo urine HBsAg. All 10 renal and liver disease patients revealed positive serum HBsAg., and among the 9 cases (90%) positive for urine HBsAg.. In the 25 patients positive for urine HBsAg. all of 5 renal patients and 9 renal and liver patients had hematuria or/and proteinuria above 2 positive for albumin. But in the 11 liver patients 6 cases (55.1%) were normal findings. And there's no significant difference in cpm of urine HBsAg. between the patient positive for serum HBsAg. and negative, and in cpm of serum HBsAg. between liver and renal disease patients. But there's statistical significance in cm of urine HBsAg. between renal and liver diseases.