• Childhood Kidney Diseases
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• v.6 no.2
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• pp.266-271
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• 2002

There are several diseases characterized by neurologic abnormalities and renal disease. Joubert syndrome is one of them. Joubert syndrome Is a relatively rare autosomal recessive syndrome. The most significant and constant neurologic finding is hypoplasia of the cerebellar vermis. Joubert syndrome is associated with hypotonia, retinal dystrophy, abnormal eye movement, delayed development, abnormal respiratory pattern (neonatal episodic tachypnea or apnea) and nephronophthisis. We report a boy with Joubert syndrome associated with nephrocalcinosis and agenesis of the cerebellar vermis. This patient had also abnormal eye movement, hypotonia, abnormal respiratory pattern, delayed development and chronic renal failure.

• The Journal of Internal Korean Medicine
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• v.21 no.4
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• pp.683-686
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• 2000

Vesicoureteral reflux(VUR) is a state that urine regurge from bladder to ureter and kidney because of congenital, structural, functional abnormality of ureterovesical junction and lower urinary tract than bladder. It may be the primary cause of recurrent urinary tract infection(UTI) in chindhood, If urine regurge with UTI, it can cause renal damage, leading to scar formation, hypertension, chronic renal failure, But upper complications can be prevented by early diagnosis and proper treatment of VUR and UTI, so clinician must focus on them in treatment of VUR. We had experienced a case of recurrent UTI with VUR regardless of consistent antibiotics therapy in 7 years old boy, Chief complain was urinary frequency, The symptom of urinary frequency was successfully treated by herb medicine(Gamijihwag-tang), So, we report this case with a brief review of related literatures.

• Journal of Chest Surgery
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• v.53 no.3
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• pp.121-126
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• 2020

Background: The aim of this study was to analyze the clinical outcomes of autogenous brachiocephalic arteriovenous fistulas and to investigate the factors associated with 1-year patency after initiation of hemodialysis. Methods: We retrospectively reviewed the medical records of 41 patients who underwent surgery to create an autogenous brachiocephalic arteriovenous fistula between January 2015 and December 2017, received hemodialysis at the same hospital for longer than 1 year, and were monitored for their vascular access status. Intraoperative flow was measured using transit-time ultrasonography. Results: The 1-year primary and secondary patency rates were 61% (n=25) and 87.8% (n=36), respectively. The functional group (subjects who required no intervention to maintain patency within the first year after hemodialysis initiation) displayed a significantly higher median intraoperative flow rate (450 mL/min) than the non-functional group (subjects who required intervention at least once regardless of 1-year patency) (275 mL/min) (p=0.038). Based on a receiver operating characteristic curve analysis, all patients were additionally subdivided into a high-flow group (>240 mL/min) and a low-flow group (≤240 mL/min). The high-flow group included a significantly greater number of functional brachiocephalic arteriovenous fistulas than the low-flow group (74.2% vs. 20%, respectively; p=0.007). Conclusion: Transit-time flow, as measured with intraoperative transit-time ultrasonography, was associated with patency without the need for intervention at 1 year after initiation of hemodialysis.

• Journal of the Korean Dietetic Association
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• v.24 no.2
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• pp.117-140
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• 2018

The number of patients is increasing and their mean age is also increasing. Proper dietary adjustments are necessary to prevent protein-calorie malnutrition or complications but it is difficult for dialysis patients to adapt to diet therapy due to stress or anorexia. Education does not consider the individual characteristics, knowledge, dietary inhabit education demands, and initial education. The purpose of this study was to identify dialysis patient's nutrition knowledge and, dietary practice and compare those with nutrition education or counseling demands for providing basic data of desirable nutrition management. The data were collected by a survey consisting of the general characteristics, disease related characteristics, nutrition education and counsel characteristics, level of nutrition knowledge, diet therapy, and nutrition education and counsel demands from the 28th March to 22th July 2017. The total number of subjects were 33 patients among dialysis patients at two tertiary medical institutions and an artificial kidney room at a private hospital in Incheon Gyeonggi. The data collected were analyzed statistically using the SPSS program 23.0, followed by further analyses using frequency analysis, one-way ANOVA, cross analysis, and correlation analysis. The results of the dialysis patients showed that younger (P<0.05), female (P<0.05), abnormal high school diploma (P<0.001) groups had high nutrition scores. In addition, dietary practice and nutrition education and counsel demands showed a positive correlation (P<0.05, P<0.01). In particular, females were higher than males in nutrition knowledge, dietary practice, nutrition education, and counseling demand scores.

• Journal of Chest Surgery
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• v.56 no.6
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• pp.435-444
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• 2023

Background: Edwards Intuity is recognized as a relatively contraindicated bioprosthesis for bicuspid aortic valve disease. This study compared the early echocardiographic and clinical outcomes of rapid-deployment aortic valve replacement for bicuspid versus tricuspid aortic valves. Methods: Of 278 patients who underwent rapid-deployment aortic valve replacement using Intuity at Seoul National University Hospital, 252 patients were enrolled after excluding those with pure aortic regurgitation, prosthetic valve failure, endocarditis, and quadricuspid valves. The bicuspid and tricuspid groups included 147 and 105 patients, respectively. Early outcomes and the incidence of paravalvular leak were compared between the groups. A subgroup analysis compared the outcomes for type 0 versus type 1 or 2 bicuspid valves. Results: The bicuspid group had more male and younger patients. Comorbidities, including diabetes mellitus, hypertension, chronic kidney disease, and coronary artery disease, were less prevalent in the bicuspid group. Early echocardiographic evaluations demonstrated that the incidence of ≥mild paravalvular leak did not differ significantly between the groups (5.5% vs. 1.0% in the bicuspid vs. tricuspid groups, p=0.09), and the early clinical outcomes were also comparable between the groups. In the subgroup analysis between type 0 and type 1 or 2 bicuspid valves, the incidence of mild or greater paravalvular leak (2.4% vs. 6.7% in type 0 vs. type 1 or 2, p=0.34) and clinical outcomes were comparable. Conclusion: Rapid-deployment aortic valve replacement for bicuspid aortic valves demonstrated comparable early echocardiographic and clinical outcomes to those for tricuspid aortic valves, and the outcomes were also satisfactory for type 0 bicuspid aortic valves.

• Journal of Chest Surgery
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• v.57 no.2
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• pp.178-183
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• 2024

Background: The superficial veins are commonly used in conventional autogenous arteriovenous fistulas and the placement of prosthetic grafts. When they are unsuitable, however, the use of the deep veins (venae comitantes) is generally considered to be a reasonable alternative. This study conducted a comparative analysis of clinical outcomes for arteriovenous grafts between 2 groups based on the type of venous outflow: superficial veins or venae comitantes. Methods: In total, 151 patients who underwent arteriovenous grafts from November 2005 to March 2022 were retrospectively analyzed. The patients were divided into 2 groups: group A (superficial veins, n=89) and group B (venae comitantes, n=62). The primary, secondary patency, and complication rates were analyzed in each group. A propensity score-matched analysis was performed. Results: In total, 55 well-balanced pairs were matched. Kaplan-Meier analysis revealed no significant differences in the primary patency rate between the 2 groups at 1-year, 3-year and 5-year intervals (group A, 54.7%, 35.9%, 25.4% vs. group B, 47.9%, 16.8%, 12.6%; p=0.14), but there was a difference in the secondary patency rate (group A, 98.2%, 95.3%, 86.5% vs. group B, 87.3%, 76.8%, 67.6%; p=0.0095). The rates of complications, simple percutaneous transluminal angioplasty, and stent insertion were comparable between the groups. Conclusion: Although this study demonstrated not particularly favorable secondary patency rates in the venae comitantes group, the venae comitantes may still be a viable option for patients with unsuitable superficial veins because there were no significant differences in the primary patency and complication rates between the 2 groups.

• Journal of Yeungnam Medical Science
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• v.40 no.4
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• pp.381-387
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• 2023

Background: Frailty is defined as a condition of being weak and delicate, and it represents a state of high vulnerability to adverse health outcomes. Recent studies have suggested that the cingulate gyrus is associated with frailty in the elderly population. However, few imaging studies have explored the relationship between frailty and the cingulate gyrus in patients with end-stage renal disease (ESRD) undergoing hemodialysis. Methods: Eighteen right-handed patients with ESRD undergoing hemodialysis were enrolled in the study. We used the FreeSurfer software package to estimate the cortical thickness of the regions of interest, including the rostral anterior, caudal anterior, isthmus, and posterior cingulate gyri. The Beck Depression Inventory, Beck Anxiety Inventory, and laboratory tests were also conducted. Results: The cortical thickness of the right rostral anterior cingulate gyrus (ACG) was significantly correlated with the Fried frailty index, age, and creatinine level. Multiple regression analysis indicated that the cortical thickness of the right rostral ACG was associated with frailty after controlling for age and creatinine level. Conclusion: Our results indicate that the cortical thickness of the rostral ACG may be associated with frailty in patients with ESRD on hemodialysis and that the rostral ACG may play a role in the frailty mechanism of this population.

• Korean Circulation Journal
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• v.53 no.8
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• pp.550-562
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• 2023

Background and Objectives: To identify the factors associated with adverse outcomes following surgery for functional insufficiency of the mitral valve (MV) or tricuspid valve (TV) associated with atrial fibrillation (AF). Methods: We evaluated 100 patients (age, 66.5±10.0 years; 47 males) who consecutively underwent surgery for functional insufficiency of the MV or TV associated with AF between January 2000 and December 2020 at our center. The primary outcome was a composite endpoint of all-cause death, valve reoperation, congestive heart failure (CHF) requiring rehospitalization, and stroke. Results: During follow-up (532 patients-years [PYs]), adverse events included death in 16 (3.0%/yr), MV reoperation in 1 (0.2%/yr), CHF in 14 (2.6%/yr), and stroke in 5 (0.9%/yr) patients, demonstrating a 5-year rate of freedom from the primary endpoint of 69.5%. The rate of postoperative AF was high even in those who underwent AF ablation (n=92), with cumulative rates of 48.1% at 1 year and 60.2% at 5 years. In multivariable analyses, the primary outcome was significantly associated with age (adjusted hazard ratio [aHR], 1.06; 95% confidence interval [CI], 1.02-1.10; p=0.005), chronic kidney disease (aHR, 7.76; 95% CI, 2.28-26.38; p=0.001), left atrial appendage exclusion (aHR, 0.35; 95% CI, 0.16-1.78; p=0.010), and postoperative AF as a time-varying covariate (aHR, 3.33; 95% CI, 1.50-7.40; p=0.003). Conclusion: Among patients undergoing surgery for functional atrioventricular insufficiency associated with AF, a significant proportion showed recurrence of AF over time after concomitant AF ablation, which was significantly associated with poor clinical outcomes.

• Korean Circulation Journal
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• v.54 no.3
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• pp.113-123
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• 2024

Background and Objectives: Previous studies have reported an association between impaired renal function and poor outcomes after radiofrequency catheter ablation in patients with atrial fibrillation (AF). However, outcomes of cryoballoon ablation (CBA) in patients with renal insufficiency are not fully elucidated. This study aimed to compare outcomes of CBA in AF patients with chronic kidney disease (CKD) versus those without CKD and to assess changes in renal function over 12 months following CBA. Methods: A total of 839 patients (65.1% with non-paroxysmal AF [PAF]) who underwent de novo CBA were prospectively enrolled. We divided patients into two groups based on creatinine clearance rate (CCr) and performed intracardiac echocardiography (ICE)-guided contrast agent-free CBA. Results: In comparison with patients without CKD (CCr >50, n=722), those with CKD (CCr ≤50, n=117) were older and predominantly female, had a lower body mass index, and showed a higher prevalence of heart failure and hypertension. Mean CHA₂DS₂-VAS score was significantly higher in CKD group than in non-CKD group. Procedure-related complications were not significantly different between two groups. During a mean follow-up period of 25.4±11.9 months, clinical recurrence occurred in 182 patients (21.7%) and not significantly different between two groups. In multivariate analysis, non-PAF and left atrial size were independent predictors of AF recurrence. CCr levels significantly improved over 12 months after CBA in CKD group. Conclusions: ICE-guided contrast-agent-free CBA showed comparable long-term clinical outcomes without increasing procedure-related complications and improvement of renal function over 12 months following CBA in AF patients with CKD.

• 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.