• Childhood Kidney Diseases
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• v.8 no.2
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• pp.195-204
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• 2004

Purpose: Hypophosphatemic rickets is a hereditary disease, characterized by hypophosphatemia due to renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin $D_3$, rachitic bone deformities and impaired growth. The purpose of this study is to provide clinical profiles of patients with hypophosphatemic rickets in our hospital. Methods: Between July 1983 and February 2004, 56 patients were diagnosed as having hypophosphatemic rickets. The medical records of these patients were reviewed retrospectively. Clinical manifestations, family histories, laboratory data, treatment outcomes were described. Results: Fifty six patients were enrolled in this study. The average age at symptom onset and diagnosis were 20 months and 5 years respectively. Fourteen patients had family histories. The main clinical manifestations were bow legs and short stature. There was a significant negative correlation between the ages and the height z-scores at the time of diagnosis(r=-0.47, P=0.005). Initial laboratory data showed normocalcemia, hypophosphatemia, elevated serum alkaline phosphatase, decreased tubular reabsorption of phosphate and a normal range of 1,25-dihydroxyvitamin $D_3$ Radiographic examinations of bone revealed fraying, widening and cupping of the metaphyseal ends. Treatment consisted of Joulie solution and vitamin D metabolites, and resulted in improved biochemical and radiographic findings. However, height z-scores remained essentially unchanged(P=0.224). Complications of treatment were frequently observed, including hyperparathyroidism, nephrocalcinosis, and hypercalciuria. Sixteen patients had corrective osteotomy and 4 of them underwent leg lengthening together. Conclusion: There was a gap of several years between the onset of symptoms and the diagnosis. Early treatment seems to be essential to growth. For the earlier treatment, the offsprings of affected parents should be followed up closely.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.561-565
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• 2003

Purpose : Urinary tract infection(UTI) is the most common bacterial infectious disease that may induce severe renal injury unless early diagnosis and appropriate treatment are performed. If recurrent UTI is prevented, renal injury can be also reduced. Therefore, we studied the risk factors of recurrent UTI in children. Methods : We performed a retrospective study of 168 children(58 girls and 110 boys) who were treated for UTI in the Department of Pediatrics, Korea University Medical Center, during 2000-2001. Among 168 children, 93 children were followed up for more than six months. For the detection of recurrence of UTI, we performed monthly routine urine cultures and physical examinations. Results : The total rate of recurrence was 32.3%. The recurrent rate in boys and girls were 37.1% and 17.4%, respectively(P<0.05). The most common causative bacteria in the first onset and in recurrence were Escherichia coli. There was a significant difference in the onset age of UTI between boys with recurrence($4.8{\pm}1.0months$) and without recurrence($16.5{\pm}3.8months$)(P<0.01). In 77% of cases, urinary tract infection recurred within six months of the first infection. The time of the first recurrence after UTI was $3.7{\pm}0.6months$ in boys and $14{\pm}8.2months$ in girls(P<0.01). The number of recurrences showed a significant difference between the group under the age of one year($0.69{\pm}0.8/year$) and those above the age of one year($0.16{\pm}0.4/year$)(P<0.05). There was no difference in the recurrent rate between those with structural abnormality and those with normal anatomy. Conclusion : Monthly routine urine cultures are efficient in detecting recurrent UTI in children. Because the male sex and young age especially less than one year of age are risk factors for increased recurrence rate of UTI, these children should be followed-up with urine cultures.

• Tuberculosis and Respiratory Diseases
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• v.66 no.2
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• pp.116-121
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• 2009

Background: Tsutsugamushi, leptospirosis and hemorrhagic fever with renal syndrome (HFRS) are the prevalent diseases among the acute febrile illnesses in Korea. Pulmonary involvement in the patients with these diseases remains poorly recognized in endemic regions, and this is despite reports of recent outbreaks and epidemic episodes. Pulmonary involvement and a higher CRP level as clinical manifestations show a more severe form of infection. The aim of this study is to analyze the correlation of pulmonary involvement and the CRP level in patients with acute febrile illnesses. Methods: We retrospectively reviewed the clinical records of 105 patients who were diagnosed with tsutsugamushi, HFRS and leptospirosis from January 2002 to May 2008 in Chuncheon Sacred Heart Hospital. The radiographic images were retrospectively analyzed by two radiologists. We analyzed the pulmonary complications of the patients with these febrile diseases and we checked the CRP level at admission. Results: The study included 105 patients who were diagnosed with febrile diseases. Of these patients, 32 patients had hantaan, 10 patients had leptospirosis and 63 patients had tsutsugamushi disease. 42 (40%) patients had pulmonary complications, 20 patients had pulmonary edema, 20 patients had pleural effusion and 2 patients had interstitial pneumonitis. The patients with pulmonary involvement showed a more severe form of infection and a higher CRP level than that of those patients without pulmonary involvement (p=0.0073). Conclusion: Pulmonary involvement in patients with acute febrile diseases might be correlated with a higher CRP level. Identification of this factor on admission might provide useful selection criteria for the patients who need early intensive care.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.280-287
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• 2007

Purpose : Urinary lithiasis is uncommon in children, however, it may lead to chronic renal insufficiency and even end stage renal disease. The etiology of stone formation in children is largely unknown; although the most common causes are known to be associated with congenital anomalies of the genito-urinary(G-U) tract, urinary tract infections(UTI), and metabolic diseases. Methods : A total of 73 children(male:female=42:31, mean age $6.6{\pm}5.3$ years) presented with urinary lithiasis between Sep. 1998 and Jul. 2007 at Seoul National University Children's Hospital. The medical records were reviewed retrospectively. Results : The most common presenting symptoms were gross hematuria(28/73, 38%) and flank or abdominal pain(23/73, 32%). The stones were located in the upper urinary tract in 48 patients(66%), in the bladder in 18(24%), and in both the bladder and upper urinary tract in 2 (3%). Congenital anomalies of the G-U tract with/without UTI were detected in 30 children (41%), hypercalciuria with/without hypercalcemia in 15(20%), and other metabolic diseases in 8(11%). In 17 patients(23%), no underlying cause of stone formation was detected. The majority of stones were infected stones(24/36, 67%), which were followed by calcium stones(8/36, 22%), uric acid stones(3/36, 8%). and cystine stones(1/36, 3%). Thirty-four patients(46%) underwent surgical procedures and/or extracorporeal shockwave lithotripsy for stone removal, and 13(18%) passed stones spontaneously with/without medical management. Stones recurred in 6 patients(8%): 4 with neurogenic bladder augmented by ileocystoplasty, 1 with cystinuria, and 1 with unknown etiology. Conclusion : The common causes of urinary lithiasis in children were congenital anomalies of the G-U tract with/without UTI and metabolic disorders including hypercalciuria/hypercalcemia. For the management of stones, minimally invasive procedures should be chosen on the basis of accompanying symptoms and the composition, locations and etiology of stones.

• Pediatric Infection and Vaccine
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• v.16 no.2
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• pp.162-166
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• 2009

Purpose : Urinary tract infection (UTI) is one of the most common bacterial infectious disease in childhood. Renal scarring is an important complication of UTIs. Known risk factors for renal scarring are younger age, anatomic defects, delayed treatment, and causative pathogens other than Escherichia coli. The aim of this study was to compare the characteristics of clinical and laboratory features of UTI with E. coli to those with non-E. coli in infants. Methods : We reviewed the medical records of 1,120 infants under 12 months of age who had been admitted for UTIs between January 1998 and December 2007. All patients who were diagnosed with UTIs were divided into two groups (E. coli and non-E. coli UTIs). Results : Three hundred twenty-four of 1,120 cases met the inclusion criteria. The number of E. coli and non-E. coli UTIs was 273 (84.3%) and 51 (15.7%), respectively. As compared to the non-E. coli UTI group, the E. coli UTI group was younger (3.59 vs. 4.47 months, P =0.008), a longer duration of pyuria (3.96 vs. 3.06 days, P =0.01), higher peripheral white blood cell counts (13.89 vs. $12.13{\times}10^3/mm^3$, P =0.043), and lower rates of high degree (III-V) vesico-ureteral reflux (P =0.005). Conclusion : UTIs with E. coli might have more severe clinical features and a lower prevalence of high grade vesicoureteral reflux than UTIs with non-E. coli. However, no difference was noted in the clinical response to antibiotic therapy between the two groups.

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.123-131
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• 2005

Background: The number of elderly patients undergoing coronary artery bypass grafting (CABG) is increasing. Elderly patients are at increased risk for a variety of perioperative complications and mortality. We identified determinants of operative complications and mortality in elderly patients undergoing CABG. Material and Method: Between January 1995 and July 2003, 91 patients older than 75 years underwent isolated CABG at Asan Medical Center. There were 67 men and 24 women with mean age of $77.0\pm2.4$ years. Thirty clinical or hemodynamic variables hypothesized as predictors of operative mortality were evaluated. Result: CABG was performed under emergency conditions in 5 patients. The internal thoracic artery was used in 85 patients and 10 patients received both internal thoracic arteries. The mean number of distal anastomosis was 3.7 per patient. Operative mortality was $3.3\%$. Twenty-two patients had at least one major postoperative complication. Low cardiac output syndrome was the most common complication, followed by reoperation for bleeding, pulmonary dysfunction, perioperative myocardial infarction, stroke, acute renal failure, ventricular arrhythmia, upper gastrointestinal bleeding, infection, and delayed sternal closure. None were the predictors of mortality. Renal failure, peripheral vascular disease, emergency operation, recent myocardial infarction, congestive heart failure, New York Heart Association (HYHA) class III or IV, Canadian Cardiovascular Society (CCS) angina scale III or IV, and low left ventricle ejection fraction below $40\%$ were univariate predictors of overall complications. Actuarial probability of survival was $94.9\%,\;89.8\%,\;and\;83.5\%$ at postoperative 1, 3 and 5 years respectively. During the follow-up period $93.3\%$ of patients were in NYHA class I, or II and $91.1\%$ were free from angina. Conclusion: Although operative complication is increased, CABG can be performed with an acceptable operative mortality and excellent late results in patients older than 75 years.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.123-129
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• 1999

Purpose: Acute poststreptococcal glomerulonephritis(APSGN) is a renal disease which is characterized by glomerular proliferation and inflammatory changes due to immune reaction. Although the 95% of patients with APSGN seems to recover fully and present as benign course, the remaining patients show poor prognosis. Therefore comparative retrograde study between APSGN with and without nephrotic syndrome was done to find out the any prognostic indicator to predict the outcome in patients with APSGN. Methods: We had retrospectively analyzed seventy-one patients who were diagnosed as APSGN clinically from Mar.1989 to Feb.1999 in Yonsei university medical center. Sixty-four of the patients was APSGN without nephrotic syndrome(Group A) and seven patients were in APSGN with nephrotic syndrome(Group B). Results: Patients who were diagnosed as APSGN with nephrotic syndrome were seven(9.9%) out of seventy-one. In the comparative study, sex ratio was 1:1 in group A and 1.9: 1 in group B, onset mean age was $8.9{\pm}2.6$ in group A and $8.8{\pm}2.6$ in group B. Following clinical profiles were compared but there were no significant difference between these two groups: WBC count($9413{\pm}2964\;vs\;9368{\pm}2650(/mm^3)$), hemoglobin($10.6{\pm}1.2\;vs\;10.0{\pm}0.9(gm/dL)$), ASO($746.1{\pm}640.7\;vs\;614.9{\pm}475.9(IU/ml)$), $C_3(20.1{\pm}17.0\;vs\;16.9{\pm}13.1(mg/dL)$), $C_4(22.8{\pm}9.5\;vs\;22.6{\pm}6.9(mg/dL)$), BUN($25.8{\pm}26.1\;vs\;28.1{\pm}14.5(mg/dL)$), creatinin($0.8{\pm}0.3\;vs\;0.8{\pm}0.3(mg/dL)$), $C_{cr}(80.6{\pm}28.8{\pm}62.4{\pm}31.4(ml/min/1.73\;m^2$)), the duration of edma, gross hematuria, and hypertension. However, we found that there were a significant difference in the duration of proteinuria($1.95{\pm}2.27\;vs\;13.3{\pm}21.1(months)$)(P<0.05), decreased $C_3$ duration($1.9{\pm}2.9\;vs\;7.3{\pm}5.0(weeks)$)(P<0.05) and especially it was proloned according to the amount of early urine protein excretion. Conclusion: Our study showed markedly prolonged duration of proteinuria and decreased $C_3$ duration in patients with APSGN with nephrotic syndrome. We were not able to find the definite prognostic factor that will guide the outcome of patients with APSGN accompaning nephrotic syndrome, but above findings seemed to represent as a relative indication of the outcome of the disease. All patients recovered completely and we did not experience any cases that progressed into the renal failure.

• Journal of Chest Surgery
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• v.39 no.5 s.262
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• pp.354-358
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• 2006

Background: Pulsatile pumps for extracorporeal circulation have been known to be better for tissue perfusion than non-pulsatile pumps but be detrimental to blood corpuscles. This study is intended to examine the risks and benefits of $T-PLS^{TM}$ through the comparison of clinical effects of $T-PLS^{TM}$ (pulsatile pump) and $Bio-pump^{TM}$ (non-pulsatile pump) used for coronary bypass surgery. Material and Method: The comparison was made on 40 patients who had coronary bypass using $T-PLS^{TM}\;and\;Bio-pump^{TM}$ (20 patients for each) from April 2003 to June 2005. All of the surgeries were operated on pump beating coronary artery bypass graft using cardiopulmonary extra-corporeal circulation. Risk factors before surgery and the condition during surgery and the results were compared. Result: There was no significant difference in age, gender ratio, and risk factors before surgery such as history of diabetes, hypertension, smoking, obstructive pulmonary disease, coronary infarction, and renal failure between the two groups. Surgery duration, hours of heart-lung machine operation, used shunt and grafted coronary branch were little different between the two groups. The two groups had a similar level of systolic arterial pressure, diastolic arterial pressure and mean arterial pressure, but pulse pressure was measured higher in the group with $T-PLS^{TM}\;(46{\pm}15\;mmHg\;in\;T-PLS^{TM}\;vs\;35{\pm}13\;mmHg\;in\;Bio-pump^{TM},\;p<0.05)$. The $T-PLS^{TM}$-operated patients tended to produce more urine volume during surgery, but the difference was not statistically significant $(9.7{\pm}3.9\;cc/min\;in\;T-PLS^{TM}\;vs\;8.9{\pm}3.6\;cc/min\;in\;Bio-pump^{TM},\;p=0.20)$. There was no significant difference in mean duration of respirator usage and 24-hour blood loss after surgery between the two groups. Plasma free Hb was measured lower in the group with $T-PLS^{TM}\;(24.5{\pm}21.7\;mg/dL\;in\;T-PLS^{TM}\;versus\;46.8{\pm}23.0mg/dL\;in\;Bio-pump^{TM},\;p<0.05)$. There was no significant difference in coronary infarction, arrhythmia, renal failure and morbidity rate of cerebrovascular disease. There was a case of death after surgery (death rate of 5%) in the group tested with $T-PLS^{TM}$, but the death rate was not statistically significant. Conclusion: Coronary bypass was operated with $T-PLS^{TM}$ (Pulsatile flow pump) using a heart-lung machine. There was no unexpected event caused by mechanical error during surgery, and the clinical process of the surgery was the same as the surgery for which $Bio-pump^{TM}$ was used. In addition, $T-PLS^{TM}$ used surgery was found to be less detrimental to blood corpuscles than the pulsatile flow has been known to be. Authors of this study could confirm the safety of $T-PLS^{TM}$.

• Archives of Plastic Surgery
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• v.34 no.5
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• pp.659-662
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• 2007

Purpose: Multiple skin leiomyoma and uterine myoma bearing autosomal dominant traits are benign smooth muscle tumors which originate in skin or female uterus. Skin leiomyoma occurs after gene mutation originating from arrector pili muscle of hair follicle where its clinical manifestations vary significantly from person to person. Our department hereby reports the histological findings and genetic evaluations of this very rare disease. Methods: A 57-year-old woman presented in our institute with multiple tumors in the left and central parts of her back that started to appear since 19 years ago. The patient was diagnosed as having uterine myoma 15 years ago and underwent hysterectomy. Biopsy has been done on the specimen, and genomic DNA was separated from Fumarate hydratase gene for it to go through PCR amplification. The results of PCR amplification were aligned by sequencer. Results: According to the results of biopsy, tumor cells were spindle-shaped and were aligned in a bundle where there was no dysplasia or mitosis. Moreover, these cells had abundant eosinophilic cytoplasm with elongated nucleus, and benign leiomyoma that showed positive reactions to SMA stain were found. In genetic examination, mutations such as heterozygous single nucleotide substitutions were found in alignments of amplified DNA. Conclusion: Multiple skin leiomyoma and uterine myoma are relatively uncommon diseases that are transmitted through autosomally dominant traits from genetic mutations. When a patient's chief complaint lies upon skin-colored or brown masses that occur in multiples appearing in the trunk or extremities with characteristic clinical symptoms and histological findings, and when the patient's family history is acknowledged such as skin or uterine leiomyoma or renal tumor, necessary genetic examination on multiple skin leiomyoma and uterine myoma could be done, and thereby precise diagnosis could also be made.

• Journal of Chest Surgery
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• v.43 no.6
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• pp.642-647
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• 2010

Background: It is very important to obtain vascular access that resists repeated punctures and maintains an adequate blood flow for performing hemodialysis in patients with ESRD. This study was designed to identify the risk factors that may influence the patency rate of arteriovenous fistula (AVF) using perforating vein on antecubital fossa. Material and Method: We analyzed 205 cases of AVF in 195 patients who underwent hemodialysis access surgery on antecubital fossa in our hospital from May 2006 to December 2009. Result: The patency rate of AVF from 6 months after surgery using perforating vein was 75.91%. The risk factors that influence the patency rate was age. There was no statistic difference between used vessels. Conclusion: The patency of the AVF using perforating vein on antecubital fossa was comparable. The condition of sex and location and presence or absence of diabetes and hypertension and other cardio-neurovascular disease did not make statistically significant effect on the AVF patency rate. The age was an independent risk factor for patency rate.