• 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 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.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

• Childhood Kidney Diseases
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• v.12 no.1
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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.

• 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.14 no.1
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• pp.42-50
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• 2010

Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.464-470
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• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.212-218
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• 2011

Purpose: The diagnosis of acute pyelonephritis (APN) is often difficult, as its clinical and biological manifestations are non-specific in children. If not treated quickly and adequately, however, APN may cause irreversible renal damage, possibly leading to hypertension and chronic renal failure. We were suspecting the diagnostic value of $^{99m}Tc$-dimercaptosuccinic acid (DMSA) scan by experiences and so compared the results of DMSA scan to those of multi-detector row computed tomography (MDCT). Methods: We retrospectively selected and analyzed 81 patients who were diagnosed as APN by MDCT during evaluation of their acute abdomen in emergency room and then received DMSA scan also for the diagnostic work-up of APN after admission. We evaluated the results of imaging studies and compared the diagnostic value of each method by age groups, <2 years (n=45) and ${\geq}$2 years (n=36). Results: Among total 81 patients with MDCT-proven APN, DMSA scan was diagnostic only in 55 children (68%), while the remaining 26 children (32%) showed false negative normal findings. These 26 patients were predominantly male and most of them, 19 (73.1%) were <2 years of age. Conclusion: DMSA scan holds obvious limitation compared to MDCT in depicting acute inflammatory lesions of kidney in children with APN, especially in early childhood less than 2 years of age. MDCT showed hidden lesions of APN, those were undetectable through DMSA scan in children.

• 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.

• 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.