• Childhood Kidney Diseases
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• v.18 no.2
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• pp.128-131
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• 2014

Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.

• Journal of Chest Surgery
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• v.26 no.6
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• pp.475-482
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• 1993

90 patients[75 men and 15 women] with the thoracic disease underwent video-assisted thoracic surgery[VATS] during the period March 1992 to February 1993. The thoracic diseases were classified into two groups of spontaneous pneumothorax and general thoracic patients and they were 66 and 24, respectively.The mean size of the tumor resected was 4.3 $\pm$ 2.0 cm x 3.3 $\pm$ 1.1 cm x 2.7 $\pm$ 1.0 cm. The mean time of anesthesia and operation were 90.0 $\pm$ 19.9 min and 43.7 $\pm$ 13.1 min in spontaneous pneumothorax group and 123.3 $\pm$ 40.3 min and 62.8 $\pm$ 32.2 min in general thoracic group. The mean period of postoperative chest tube drainage and hospital stay were 5.0$\pm$ 5.5 days and 6.6 $\pm$ 7.4 days in spontaneous pneumothorax group and 3.5$\pm$ 1.6 days and 9.5 $\pm$ 6.1 days in general thoracic group. The indications of VATS were 71 pleural disease[78.9%: 66 spontaneous pneumothorax; 3 pleural effusions ; 1 pleural paragonimus westermanii cyst; 1 malignant pleural tumor with metastasis to the lung], 9 mediastinal disease[10.0%: 5 benign neurogenic tumor; 2 pericardial cyst; 1 benign cystic teratoma; 1 undifferentiated carcinoma], 8 pulmonary parenchymal disease[8.9%: 3 infectious disease ; 3 interstitial disease ; 2 malignant tumor ], and 2 traumatic cases of exploration and removal of hematoma[2.2%]. The applicated objectives of VATS were diagnostic[ 7 ], therapeutic[ 67 ] and both[ 16 ] and the performed procedures were pleurodesis[ 66 ], wedge resection of lung[ 59 ], parietal pleurectomy[ 11 ], removal of benign tumor[ 9 ], excision and/or biopsy of tumor[ 4 ], pleural biopsy and aspiration of pleural fluid[ 3 ] and exploration of hemothorax and removal of hematoma in traumatic 2 patients. The complication rate was 24.2%[ 16/66 ] in the spontaneous pneumothorax group and 8.3%[ 2/24 ] in the general thoracic group and so overally 20.0%[ 18/90 ]. The mortality within postoperative 30 days was 2.2%[ 2/90 ], including 1 acute renal failure and 1 respiratory failure due to rapid progression of pneumonia. The conversion rate to open thoracotomy during VATS was 5.6%[ 5/90 ], including 2 immediate postoperative massive air leakage, 1 giant bullae, 1 malignant pleural tumor with metastasis to lung and 1 pulmonary malignancy. The successful cure rate of VATS was 75.8%[ 50/66 ] in the spontaneous pneumothorax group and 76.5%[ 13/17 ] in the general thoracic group and the successful diagnostic rate was 100%[ 7/7 ]. In conclusion, although prospective trials should be progressed to define the precise role of VATS, the VATS carries a low morbidity and mortality and high diagnostic and therapeutic success rate and now can be effectively applicated to the surgical treatment of the extensive thoracic disease.

• Pediatric Infection and Vaccine
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• v.24 no.3
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• pp.141-145
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• 2017

Purpose: Clinical and laboratory features of two Kawasaki disease (KD) groups were evaluated; the patient with pyuria and those without pyuria. Methods: From January 2015 to December 2016, the medical records of 140 (86 males and 54 females) inpatients with KD were retro-spectively analyzed. Results: Forty-eight KD patients (34.3%) presented with pyuria. KD patients with pyuria showed a higher level of C-reactive protein (CRP) and a higher proportion of elevated liver enzymes than those without pyuria. There were no differences in the proportions of unresponsiveness to intravenous immunoglobulin and coronary artery lesions between the two groups. Six KD patients (12.5%) with pyuria underwent a renal imaging study to rule out the possibility of a urinary tract infections. Thirty-two KD patients (66.7%) with pyuria received treatment with antibiotics in addition to the standard treatment for KD. Conclusions: KD patients with pyuria showed a higher level of CRP and elevated levels of liver enzymes than those without pyuria. These findings suggest that KD patients with pyuria have more severe systemic inflammation than those without pyuria.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.1-8
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• 2003

Purpose : Focal segmental glomerulosclerosis(FSGS) is known to have a poor prognosis and a poor response to treatment. We performed a combination therapy of plasmapheresis, steroid pulse and immunoglobulin in 4 patients with relapsed steroid dependent(SD) or steroid resistant(SR) FSGS after steroid pulse therapy. Materials and Methods : 8 cycles of plasmapheresis were performed in 4 patients with biopsy proven FSGS who had relapsed after steroid pulse therapy from March 1988 to July 2002. Clinical findings and treatment courses were reviewed retrospectively. Results : Among the 4 patients, there were 3 males and 1 female. After 8 cycles of plasmapheresis, clinical remissions were obtained. Two of the four patients had two relapses and received 2 more cycles of plasmapheresis which resulted in remissions. One of these patients had two further relapses and was treated with oral steroid resulting in clinical remission. Three patients have maintained normal serum creatinine level and glomerular filtration rates during the follow-up period of 10 years, and the other 1 patient for 5 months. Conclusion : A combination therapy of plasmapheresis, steroid pulse and immunoglobulin led to a complete remission in patients with FSGS who were SD or SR and was effective in maintaining normal renal function.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.68-73
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• 2004

Both Gitelman syndrome and Bartter syndrome are autosomal recessively inherited renal tubular disorders characterized by hypokalemic metabolic alkalosis, salt wasting and normal to low blood pressure. Gitelman syndrome is caused by mutations in the thiazide-sensitive Na-Cl cotransporter (NCCT) and distinguished from Bartter syndrome, which is associated with mutations of several genes, by the presence of hypomagnesemia and hypocalciuria. In most of the patients with Gitelman syndrome, the disease manifests with transient episodes of muscular weakness and tetany in the adult period, but, often, is asymptomatic. We report here an 11 years-old female with Gitelman syndrome who presented with aggravation of epileptic seizure. The diagnostic work-up showed typical clinical features of metabolic alkalosis, hypokalemia, hypomagnesemia and hypocalciuria. We also identified a heterozygote mutation($^{642}$CGC(Arg)>TGC(Cys)) and an abnormal splicing in the SLC12A3 gene encoding NCCT.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.95-101
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• 2012

Purpose: Recently, many evidence-based guidelines for the management of urinary tract infection (UTI) have been developed because of the importance of proper management. However, there is a lack of data regarding how pediatricians manage UTIs in Korea. Therefore, we surveyed pediatricians to determine whether they manage UTIs in an appropriate manner. Methods: A postal questionnaire survey of 78 pediatricians practicing in Daegu city was performed. Subjects were asked about diagnosis, imaging studies, treatment, and prevention of UTIs. Results: Most of the respondents (94.8%) performed urinalysis to diagnose UTI in febrile children with an unknown fever focus. However, many preferred inaccurate collection methods, such as bagged urine collection, and did not obtain urine cultures. The most frequently performed imaging modality was renal-bladder ultrasonogram. Orally administered antibiotics were preferred unless admission was needed. After diagnosis of UTI, the pediatricians usually provided information to caregivers about the disease itself and supplementary treatment. Of the respondents, only 28.6% had their own guidelines for management of vesicoureteral reflux. Conclusion: Most pediatricians suspected UTI in febrile children with an unknown focus appropriately. Nevertheless, the fact that many pediatricians preferred inaccurate urine collection methods and did not perform sufficient imaging studies to detect associated abnormalities likely resulted in overtreatment due to false-positive diagnosis of UTI and a low probability of ruling out genitourinary anatomical problems. To improve the quality of management of UTI, pediatricians should follow scientific and evidence-based guidelines.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.202-212
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• 2008

Purpose : The aim of this study was to develop a quality of life scale for children on chronic peritoneal dialysis(QOL-CPD). Methods : Thirty children on chronic PD at Seoul National University Children's Hospital participated. A healthy control group included 47 elementary school children. Other patients groups are 32 children from the department of pediatric orthopedics and 28 children from the department of child psychiatry. The age range of all children was 7 to 16 years. Preliminary items of the QOL-CPD were developed and administered along with the Korean version of the Children's Depression Inventory(CDI) to all children. Results : The final QOL-CPD was constructed by excluding those items with a factor loading of less than .20, and the principal axis factor analysis was performed again. The QOLCPD demonstrated a good internal consistency with a value of .87. The dialysis and childpsychiatric groups showed significantly lower QOL scores compared to the healthy control group. In addition, the dialysis and child-psychiatric groups showed greater difficulties on physical and academic functions. For the CDI, the PD group showed a mild level of depression. Conclusion : The results of this study demonstrate the clinical utility of a newly developed self-report QOL scale specific for children on chronic PD.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.49-54
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• 2008

Background: Aortic diseases tend to involve the entire aorta. Hence, there is the constant possibility of the need for a secondary operation at the remnant aorta. This study analyzed our cases of secondary aortic surgery in order to determine its characteristics and problems. Material and Method: Between April 2003 and June 2007, 12 patients (6 male and 6 female) underwent thoracoabdominal aortic replacement as a secondary aortic operation. Their clinical courses were analyzed. Four of the patients underwent lower thoracobadominal aortic replacement under the normothermic femorofemoral bypass, and the others underwent an entire thoracobdominal aortic replacement under deep hypothermic circulatory arrest. Result: There was no death or paraplegia. As local complications, there were 3 cases of wound infection and 2 cases of an immediate reoperation caused by bleeding and one case of delayed wound. revision for a contaminated perigraft hematoma. As a systemic complication, there was one case of renal insufficiency, which required hemodialysis and one case of respiratory insufficiency that needed prolonged ventilator care. The mean admission period was $30{\pm}21$ days. All the patients were followed up for $626{\pm}542$ days without reoperation or other problems. Conclusion: Using properly selected patients and a careful approach, thoracoabdominal aortic replacement can be performed safely as a secondary aortic surgery.

• Applied Microscopy
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• v.27 no.4
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• pp.433-452
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• 1997

Mongolian gerbil (Meriones unguiculatus) has been as an animal model for studing the neurological diseases such as stroke and epilepsy because of the congenital incompleteries in Willis circle, as well as the investigation of water metabolism because of the long time-survival in the condition of water-deprived desert condition, compared with other species animals. In order to accomplish the this research, first of all another divided the laboratory animals 5 groups of which each group include the 5 animals. In this study were investigated the histological structure in the kidney, measured the plasma osmolalities at the time of sacrifice of indivisual animals, and the body weights every day during water-deprived. The results obtained in this study were summarized as followings: 1. The body weights and decreasing rates of the body weight in water-deprived mongolian gerbil groups were continuosly decreased. 2. The plasma osmolalities were increased from the 5th water-deprived day, after then the gradually increasing reached nearly its equilibrium state at the 10th water-deprived day. 3. The urine volumes were abruptly decreased from the 2th water-deprived day, after then the gradually decreasing patterns were reached nearly its equilibrium state at the 10th day, and stopped the 11th day. 4. In the light microscopical observation of the kidney, glomerular capillary loop thickening, mesangial matrix increasing, sclerosis, glomerular cystic atrophy, interstitial fibrosis, tubular dilatation, mononuclear interstitial inflammation, interstitial mineralization, and hyperplasia of the collecting duct epithelium in the cortex area, were observed from the 10th water deprived day, and the lesions were gradually severe changed as the time lapse. 5. In the electron microscopical findings of the kidney, the degenerative changes of endothelial cell, podocyte and mesangial cell in glomeruli were initially observed on the 10th water-deprived day as well as the degeneration of microvilli and intracellular organelle in the renal tubules.

• Korean Journal of Clinical Pharmacy
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• v.21 no.4
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• pp.364-375
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• 2011

Given that single blockade with angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) can achieve only partial and undurable suppression of the Renin Angiotensin System (RAS), it has been hypothesized that dual blockage would be more beneficial in the management of blood pressure (BP) reduction and prevention of progressive chronic kidney disease (CKD) than either agent alone. Thus, it has been suggested that the combination of an ACEI and an ARB might provide renal benefits to hypertensive patients over and above BP reduction. However, this might also expose patients to additive or synergistic side effects. We attempted to conduct a systematic review to evaluate the benefits and harms of combination therapy in hypertensive patients with or without kidney diseases. MEDLINE and KoreaMed were searched for relevant randomized clinical trials in adult hypertensive patients with or without diabetes (restricted to 1997, limited to trials published in English). Results were summarized using the random-effects model, and between-studies heterogeneity was estimated with $I^2$. A final analysis of ten trials (23,928 patients) revealed that the combination of an ACEI and an ARB reduced blood pressure (SBP/DBP) by 3.95/2.02 mmHg (95% confidence interval [CI], -4.38 to -3.53/-2.33 to -1.71) compared with ACEI monotherapy, and 2.83/2.64 mmHg (95% CI, -3.25 to -2.41/-4.95 to -0.33) compared with ARB monotherapy. Eight trials (391 patients) demonstrated a significant reduction in 24h-proteinuria (weighted mean difference, 0.16 g/day, 95% CI, -0.26-0.05), but they did not translate into an improvement in GFR. Tests for heterogeneity showed no difference in effect among the studies. The combination therapy reduced proteinuria by 30% (95% CI, 23% to 37%) and 39% (95% CI, 31% to 48%) compared with ACEI monotherapy and ARB monotherapy, respectively. However, in patients who had proteinuria more than 0.5 g/day, the combination therapy failed to show significant reduction in urinary protein excretion. The current cumulative evidence suggests that diabetic patients with proteinuria on dual RAS blockade have an increase risk of adverse events such as hyperkalemia, hypotension, and so on, compared with ACEI or ARB alone. It is, therefore, proposed that the combination therapy should not be routinely used for the treatment of hypertension with or without compelling indications.