• Childhood Kidney Diseases
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• v.13 no.2
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• pp.130-137
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• 2009

Purpose : Stem cell transplantation (SCT) has gained worldwide acceptance as a treatment for hematologic disorders. This study was performed to evaluate the clinical characteristics and outcomes of the acute kidney injury after SCT in children. Methods : The records of 53 patients who were treated with SCT at the pediatric department of Yeungnam University Hospital between January, 1996 and April, 2009 were used as subjects. Their were divided into two groups ; 'Early renal insufficiency' (ERI, n=18) and 'Non-early renal insufficiency' (NERI, n=35). ERI had greater than 25% of drop in GFR after SCT. Results: Total 53 patients were analyzed. In cord blood SCT (n=11), ERI was 4 (36.4%) and NERI was 7 (63.6%). In bone marrow SCT (n=16), ERI was 8 (50.0%) and NERI was 8 (50.5%). In autologous peripheral blood SCT (n=26), ERI was 6 (23.1%) and NERI was 20 (76.9%). There is no difference in both groups according to kinds of SCT. GVHD was developed in 22 patients, and there is no difference in each group. Twenty two of 53 patients died. ERI was 12 (66.7%) and NERI was 10 (28.6%). Acute renal failure is most important cause of the deaths. Conclusion : Out of 53 pediatric patients who were treated with SCT, 18 patients had greater than 25% of drop in GFR. There is no difference in both groups according to kinds of SCT. GVHD was found in 22 patients and there is no relation between GVHD development and acute kideney injury.

• Journal of Chest Surgery
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• v.32 no.8
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• pp.715-721
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• 1999

Background:The number of old patients receiving coronary artery bypass grafting(CABG) is increasing. With the more recent advances in operative techniques, the age at which CABG is indicated has also increased. This study evaluated the risk factors associated with the hospital mortality and the morbidity following CABG in elderly patients. Material and Method: Between March 1991 and June 1998, we retrospectively reviewed 45 consecutive patients aged 65 years or older who underwent CABG. We compared the data with the results of 179 patients under the age 65 years operated during the same period. Result: Mean age was 68${\pm}$1.41 years(range 65 to 74 years). Emergency surgery was required in 4, and elective surgery in 41 patients. The mean number of distal anastomosis per patient was 3.62 ${\pm}$0.81 and mean aortic cross-cramp time was 69.84${\pm}$18.5 minutes. Thirty patients had Canadian class III or IV preoperatively, but 43 patients had class I or II postoperatively. The left ventricular ejection fraction increased significantly from 54.23${\pm}$10.62% preoperatively to 58.14${\pm}$9.88% postoperatively(p<0.05). Postoperative complication was pneumonia in 2 patients, acute renal failure in 2 patients, sternal wound infection in 1 patient, and postoperative myocardial infarction in 1 patient. There were two postoperative deaths. The causes of deaths were low output syndrome in one patient, and sepsis due to pneumonia in the other patient. The hospital mortality was higher in the elderly group(4.4 versus 2.86%) but was not statistically significant(p>0.05). Incremental risk factors for hospital deaths in the elderly were emergent operation, preoperative PTCA, postoperative use of IABP and postoperative ARF(p<0.05). The duration of hospital stay after operation was significantly longer for the elderly group than the younger group(19.27${\pm}$12.51 vs 15.55${\pm}$6.99 days; p＜ 0.05). Follow-up was complete for 34 of the hospital survivors and ranged from 1 to 73 months(mean: 23.58${\pm}$19.56 months). There was no late mortality of cardiac origin. Conclusion: Age is an important factor in selecting optimal management for elderly patients with coronary compromise, but age alone should not dictate the choice of therapy. Coronary artery bypass surgery in the elderly is associated with acceptable early mortality and excellent long-term results.

• Journal of Chest Surgery
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• v.35 no.10
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• pp.705-711
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• 2002

The advantages of mitral valve reconstruction have been well established and so mitral valve reconstruction is now considered as the procedure of choice to correct mitral valve disease. This is the report of intermediate-term results of 38 cases that performed mitral valve reconstruction for valve insufficiency(the total number of mitral valve reconstruction were 49 cases, but 11 cases that performed mitral valve replacement due to incomplete reconstruction were excluded). Material and Method : From March 1991 to March 2001, 38 patients underwent mitral valve repair due to mitral valve regurgitation with or without stenosis. Mean age was 47.6$\pm$14.7 years(range 15 to 70 years) : 11 were men and 27 were women. The causes of mitral valve regurgitation were degenerative in 14, rheumatic in 21, infective in 2 and the other was congenital. Result : According to the Carpentier's pathologic classification of mitral valve regurgitation, 3 were type 1 , 16 were type II and 19 were type III. Surgical procedures were annuloplasty 15, commissurotomy 19, leaflet resection and annular plication 9, chordae shortening 11, chordae transfer 5, new chordae formation 2, papillary muscle splitting 2 and vegetectomy 2. These procedures were combined in most patients. There were 2 early death and the causes of death were respiratory failure, renal failure and sepsis. There was no late death. Valve replacement was done in 6 patients after repair due to valve insufficiency or stenosis 3 weeks, 1, 3, 51, 69, 84months later respectively. These patients have been followed up from 1 to 116 months(mean 43.0 months). The mean functional class(NYHA) was 2.36 pre-operatively and improved to 1.70. Conclusion : In most cases of mitral valve regurgitation, mitral valve reconstruction when technically feasible is effective operation that can achieve stable functional results and low surgical and late mortality.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.998-1003
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• 2005

Purpose : We evaluated the occurrence of renal scarring in children with unilateral vesicoureteral reflux(VUR), and the relationships between renal scar formation and risk factors such as VUR, duration of fever, acute-phase reactant, age, and sex. Methods : We retrospectively analyzed the data of 35 children newly diagnosed with unilateral vesicoureteral reflux after urinary tract infection(UTI) in Wallace Memorial Baptist Hospital between January 1996 and December 2004. Ultrasonography, Erythrocyte sedimentation rate(ESR), and C-reactive protein(CRP) were performed initially. Voiding cystourethrography(VCUG) was performed 1 to 3 weeks after treatment with UTI. $^{99m}Tc$-dimercaptosuccinic acid(DMSA) scan was performed 4 to 6 months after treatment. Results : Scintigraphic renal damage was present in 29 percent of the refluxing and in 3 percent of the nonrefluxing kidneys(P<0.05). The severity of VUR was significantly correlated with renal scar formation(P<0.05). The duration of fever before treatmen($5.0{\pm}1.3$ vs $2.6{\pm}1.3$) and prolonged fever of over 5 days were significantly different between renal scar group and non-renal scar group(P<0.05). ESR($56.3{\pm}23.8$ vs $27.9{\pm}18.1mm/hr$, P<0.05) and CRP($12.8{\pm}7.3$ vs $3.9{\pm}3.8mg/dL$, P<0.05) at the diagnosis of UTI in the renal scar group were higher, compared to those of the non-renal scar group. There were no significant differences in age and sex between the two groups. Conclusion : The presence and grade of VUR, the duration of fever before treatment, prolonged fever over 5 days, ESR, and CRP were risk factors for renal scarring, irrespective of age and sex. Diagnosis and management of VUR, in children with UTI, is important to prevent renal scars.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.464-472
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• 2009

Background: We analyzed the clinical results and the factors for survival of patients who underwent extracorporeal life support system after adult cardiovascular surgery. Material and Method: We retrospectively reviewed the medical record of 44 patients (1.6% of the total adult cardiovascular surgical cases) who underwent the use of a ventricular assisted device or extracorporeal membrane oxygenation from January 2002 to August 2008. There were 32 (72.7%) males and their mean age was 61.7$\pm$14.9(range: 20$\sim$73) years old. The mean duration of extracorporeal life support system was 5.3$\pm$3.0 (range: 1$\sim$12) days. Result: Of these 44 patients, 24 (54.5%) patients were successfully weaned from the extracorporeal device. Eighteen (40.9%) survivors were able to be discharged from the hospital. Complications were noted in 38 patients (86.4%). An emergency operation, no usage of a concomitant intraaortic balloon pump and major complications during use of the extra corporeal life support system such as bleeding, flow instability and renal failure were identified as significant risk factors for poor survival on univariated analysis. Owing to educational support and a continuous renal replacement therapy system, the clinical outcomes of these patients have improved since 2006. On multivariated analysis, renal failure and bleeding during extracorporeal life support were significant risk factors for poor survival. Conclusion: Although using. extracorporeal life support systems after adult cardiovascular surgery revealed acceptable clinical results, determining the optimal treatment strategy and further well designed larger studies are needed to improve the survival rate of patients who undergo extracorporeal life support after adult cardiovascular surgery.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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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.

• Journal of Yeungnam Medical Science
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• v.16 no.2
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• pp.309-317
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• 1999

Background: Left ventricular diastolic filling is an important determinant for maintenance of cardiac output during hemodialysis. Few investigators have studied the influence of hemodialysis on diastolic function. To evaluate the change of left ventricular systolic and diastolic function. we performed M-mode and Doppler echocardiographic studies before and after hemodialysis. Methods: The study population consisted of 30 patients(15 patients were male, mean age $45{\pm}10$ years) with CRF on maintenance hemodialysis. They have normal left ventricular systolic function(Fractional shortening>30%) and no evidence of valvular heart disease or regional wall motion abnormalities. The ejection fraction (EF) was measured using M-mode echocardiography and Doppler indices such as peak E velocity, peak A velocity, isovolumetric relaxation time(IVRT), deceleration time(DT). and left ventricular ejection time(LVET) obtained from Doppler echocardiography. The index of myocardial performance (IMP) was calculated from each of the Doppler velocity indices. Results: The weight reduction after hemodialysis was $2.1{\pm}1.0kg$(p<0.0001), After hemodialysis, there was some decrease in blood pressure(p<0.05), but no significant change in heart rate, EF and fractional shortening, mean VCF, peak A velocity, and DT. And significant reduction in peak E velocity, E/A ratio(p<0.0001. p<0.001), and significant increase in IVRT and IMP(p<0.05, p<0.0001) were noted. Conclusion: In conclusion, preload reduction is the main mechanism that accounts for changes in Doppler diastolic indices after hemodialysis. And an increased IMP suggests that diastolic function may be aggravated after hemodialysis, and that implies impaired left ventricular filling and disturbed left ventricular compliance.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.46-52
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• 1997

Urinary tract infection (UTI) in children has been known to be a cause of renal damage, leading to scar formation, hypertension and renal failure. And vesico-ureteral reflex (VUR), frequently accompanying UTI in young children, has been incriminated as the main factor causing scar formation. This retrospective study has been undertaken to see the relationship among UTI, VUR and renal scar formation. Study population consisted of 291 children (boy 134, girl 42) with UTI, who have been admitted to the Pediatric Department of Kyungpook University Hospital during 6 1/2 year period from January 1990 to June 1996. VUR was diagnosed by VCUG and renal scar by ultrasonogram, DMSA scan (or DMSA SPECT) and IVP. The following result were obtained. Sexual difference showed male predominance (male to female, 134:42) below 1 year of age, and female predominance (male to female, 11:35) over 5 years of age were rioted. VUR has been found in 64 children (22%) and the degree of reflux, classfied by the method proposed by 'International Reflux Study in Children', were as follows ; Grade I : 4.0%, Grade II : 3.0%, Grade III : 2.7%, Grade IV : 5.8% and Grade V : 6.2%. There was no sexual difference E.coli was the most predominant infecting agent occurring in 167 children (57%), and end-stage renal failure was diagnosed at the time of first admission in 5 children with Grade V VUR. Renal scar has been noted in 49 out of 582 kidneys (8.4%), and the incidence of scar foramation according to the degree of VUR were as follow ; Grade 0 (No reflux) : 1.2%, Grade I : 6.7%, Grade II 27.3%, Grade III 29.4%, Grade IV : 57.1%, and Grade V : 100%. In summary, present study shows that renal scar formation in UTI has close correlation with the severity of VUR occurring more frequently in severe reflux, so that early diagnosis and proper treatment of UTI and VUR is of paramount importance in preventing renal damage in children with UTI.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.31-37
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• 1997

The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.72-79
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• 1999

Renal scarring associated with vesico-ureteral reflux(VUR) is one of the major causes of end stage renal failure and renal hypertension in children. Urinary ${\beta}_2$-microglobulin(MG) has been suggested as a potential marker for presence of renal tubular damage. This study was designed to evaluate the significance of random urine ${\beta}_2$-MG as a predictor of presence of vesico-ureteral reflux in children with urinary tract infection(UTI). 57 children with urinary tract infection were studied. Patients were devided into two groups; 35($78.9\%$) children have UTI without VUR and 12($21.1\%$) children have UTI and VUR. Beta2-MG and creatinine in random urine sample was measured to decide the excretion ratio(${\beta}_2$-MG/creatinine). Among the 57 children with UTI, 44 children were confirmed by urine culture study and 13 children suspected by compatible clinical feature. Random urine ${\beta}_2$-MG of VUR group ($2.2{\pm}5.91$ mg/L) were significantly higher than that of simple UTI group($0.19{\pm}0.16mg/L$)(P=0.03). The ${\beta}_2$-MG/creatinine ratio of VUR group($32.41{\pm}25.7$) were significantly higher than that of simple UTI group($3.93{\pm}3.44$)(P=0.007). In conclusion, random urine ${\beta}_2$-MG and excretion ratio deserved early predictor of presence of VUR in children with UTI. And this method was more simple and inexpensive than the method of measuring ${\beta}_2$-MG with 24 hour urine collection, so might be a useful screening test for VUR in children with UTI.