• The Monthly Diabetes
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• s.261
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• pp.18-21
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• 2011

만성신부전증 환자 절반가량에서 당뇨병이 원인인 것으로 드러나면서 당뇨병성 신증에 대한 심각성이 제기되고 있다. 당뇨병에 의한 말기신부전은 생명을 이어가려면 투석과 신장이식이 불가피한 질병으로 일상에 불편을 가져오는 것은 물론 결국 사망에 이르게 하는 치명적인 질환이다. 중앙대학교 병원 신장내과 유석희 교수는 "당뇨로 인한 반성신부전은 다른 원인으로 인해 발생하는 신부전보다 생존율이 낮다"며 "당뇨병이 오래된 환자일수록 신장합병증이 발병률이 높아지는데, 당뇨병환자가 늘 혈당관리에만 매달리다보면 신장기능이 나빠지는 신호를 놓칠 수 있다"고 전했다.

• The Monthly Diabetes
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• s.261
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• pp.27-29
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• 2011

• Journal of Chest Surgery
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• v.34 no.4
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• pp.338-344
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• 2001

배경: 말기 신부전 환자에서 심장병, 특히 관상 동맥 질환의 이환이 늘어나면서 관상동맥 우회수술의 대상 환자가 지속적으로 증가하고 있다. 이들 환자군은 수술후 유병률과 사망률이 매우 높고 고위험군으로 알려져 있다. 대상 및 방법: 1996년 3월부터 2000년 5우러까지 서울 중앙 병원 흉부외과에서 술전 말기 신부전증으로 진단 받은 후 관상 동맥 우회수술을 시행받은 환자 25명을 대상으로 의무 기록을 중심의 후향적 분석을 하였다. 술전 위험 인자 및 술전 신기능, 수술 결과, 술후 경과, 수술후 합병증, 사망률 및 생존률 등을 분석하였다. 결과: 술전 평균 크레아티닌 청소율은 12.7$\pm$5ml/mim였고 술전 평균 혈중 크레아티닌 치는 6.2$\pm$3mg/dl(1.7-14.4)였다. 술전 투석을 시행중인 환자는 11례(44%)였고 술전 투석을 시행하지 않았던 14례(56%)중 8례(8/14, 57.1%)는 수술전후로 새로이 투석이 필요하였다. 술전 혈액 투석 중이었던 9례중 2례에서 수술후 복막 투석으로 전환하였다. 수술 사망률은 2례(8%)로 흡인성 폐렴과 종격동염으로 1례, 그리고 수술후 출형과 종격도염으로 1례가 사망하였다. 수술후 합병증은 14명(56%)의 환자에서 발생하여 매우 높은 발생율을 보였다. 만기사항은 2례(8%)에서 발생하였으며 사망원인은 카테터에 의한 복막염이었다. 생존 환자의 4년 생존률은 82$\pm$13% 였다. 결론: 말기 신부전 환자에서의 관상동맥 우회수술을 비교적 만족스러운 범위의 수술 사망률(8%)을 보였으나 합병증 발생률이 매우 높고 합병증 발생 시 사망률이 매우 높아 수술주위 감염 예방과 세심한 환자 관리가 필요하다.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.92-101
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• 2000

Purpose : We analyzed the demogaphic data md clinical course of Korean children with chronic renal failure (CRF) observed between 1990 and 1999. Patients and Methods : Questionnaires were mailed to all children's hospitals ail through the country. We asked for primary renal disease age and serum creatinine levels at first presentation with CRF and end-stage renal disease (ESRD), and modes of renal replacement therapy (RRT). Results : 401 children (254 boys, 147 girls) with CRF, defined as a permanent increase of serum creatinine above 1.2 mg/dl for at least 3 months or until death, were identified. This represents an incidence of 3.68 per million child population per year. Of these patients, 22$\%$ on younger than 5 years, 28$\%$ 5 to 10 years and 50$\%$ 10 to 15 year. Eight five $\%$ of the patients could be classified with a primary renal disease. The most frequent cause is glomerulonephritis (36$\%$), followed by chronic pyelonephritis (21$\%$), renal hrpo/dylplasia (9$\%$), and hereditary nephropathies (7$\%$). Reflux nephropathy (16$\%$) was the most common single cause of CRF. ESRD was reached in 70$\%$ of all patient. 99.3$\%$ of these started RRT. Hemodialysis (HD, 42$\%$), peritoneal dialysis (PD, 35$\%$) and transplantation (TP, 23$\%$) were performed as the initial mode of RRT. A total of 161 TPs were performed (159 first grafts, 2 second grafts). A total of 32 patients died. The main causes of death were dialysis related complication in HD patients and infections in PD patients. Survival rate on any form of RRT was 88.7$\%$ during the mean follow-up period of 37 months. Conclusion Major efforts should be directed toward earlier diagnosis and treatment of reflux nephropathy to prevent occurrence of Of. Dialysis and TP have now become well accepted forms of treatment in Korean children with ESRD.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.29-35
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• 2020

Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.

• The Monthly Diabetes
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• s.261
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• pp.22-23
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• 2011

당뇨병은 전 세계적으로 급격하게 증가하면서 심각한 대혈관 및 미세 혈관 합병증들의 원인이 되고 있으며 대다수 선진국에서 당뇨병은 만성 신질환 및 말기 신부전의 가장 중요한 원인으로 알려져 있다. 당뇨병성 신증은 단백뇨를 특징으로 하며 30% 정도의 환자에서는 혈뇨도 관찰된다. 그래서 당뇨병성 신증의 발병 검진은 미세알부민뇨 검사로 하는 것이 가장 이상적이며 제 1형 당뇨병환자는 일반적으로 당뇨병 발병 5년 이후에 하며 위험인자가 있는 환자는 더 조기에 한다. 제 2형 당뇨병환자는 당뇨병 진단 시점부터 매년 하는 것을 원칙으로 한다.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.588-590
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• 1999

It is now generalized to perform cardiac surgery in the patients with end-stage renal disease. The growing number of patients with chronic renal failure wake us up to the need to prepare for proper management. There are not only the prevalence of coronary artery disease, but also a great amount of valve dysfunction exist in this group. Peritoneal dialysis may be one of the obstacles for cardiopulmonary bypass but it is not a great hindrance in cardiac surgery with careful preparation and well organized perioperative care. The author has performed mitral valve replacement in a 33-year-old anuric female patient with chronic renal failure and severe mitral insufficiency. Preoperatively, the patient was kept in adequate fluid and electrolyte balance using peritoneal dialysis. Peritoneal dialysis continued and regulated according to the laboratory data in this patient during and after the surgery. She recovered well showing an uneventful course and was discharged on postoperative 1 th day.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.136-143
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• 2000

Purpose : The hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia thrombocytopenia, and acute renal failure. It is ole of tile most common cause of acute renal failure in children but few reports are available in Korea. Thus we investigated the 23 patients diagnosed as HUS during last 14 years. Method : We retrospectively investigated the etiologic factor, clinical manifestations laboratory findings, treatment modalities, and final outcomes of the patients. Then patients were divided into two groups according to outcome, md comparison was performed. Group A(8) comprised patients who progressed to end-stage renal disease or expired. Group B(15) comprised patients who completely recovered after dialysis treatment. Result The number of patients aged less than 4 years were 17; between 5 and 10 were 4 and more than 10 were 2. The gende ratio was M:F=2 : 1. The etiologic factors were as follows: acute gastroenteritis in 14 patients including 4 bloody diarrhea, upper respiratory tract infection in 7 patients, and 1 patient with herbal mediation. The overall mortality rate was 22$\%$: 2 patients died of US complications, 2 patients died of sepsis, and 1 patient died of pulmonary hemorrhage. Group A (Hb 4.8${\pm}$1.2 g/dL) showed lower value in hemoglobin than group B (Hb 6.3${\pm}$1.7 g/dL) during hospital stay (P< 0.05), And the time interval between tile disease onset and dialysis treatment was significantly longer in group A ($11.9{\pm}9.1\;days\;vs\;2.8{\pm}2.1\;days$) (P< 0.05). Conclusion : Overall mortality rate was 22$\%$. Low hemoglobin value and the prolonged time interval between the disease onset and dialysis treatment were related with poor prognosis. So early diagnosis and appropriate intensive care including dialysis treatment is essential to achieve better outcome in children.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.176-188
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• 2009

Purpose : Peritoneal dialysis (PD) is the major form of dialysis in use for infants and children with end-stage renal disease (ESRD). The aim of this study was to gain insight into the current status of children on PD in Korea. Methods : In May 2008, questionnaires were sent to the pediatric nephrologists via e-mail. Four centers replied and those data were reviewed. Results : A total of 103 patients were included in this study. Male to female ratio was 1.6:1. Mean age was $11.5{\pm}4.9$ years (0-19 years). Primary renal diseases diagnosed were as follows: primary glomerular disease (34%), chronic pyelonephritis-reflux nephropathy (14.6%), systemic disease (9.7%), renal hypoplasia/dysplasia (8.7%), heredofamilial disease (6.8%), vascular disease (3.9%), drug-induced nephropathy (1.0%), and unknown (12.6%). PD modalities were as follows: CAPD (42.7%), CCPD (27.2%), NIPD (11.7%), and Hybrid (18.4%). Weekly total Kt/V was $2.1{\pm}0.7$ (0.3-4.1). Results of peritoneal equilibrium test were as follows: low 36.8%, low average 31.6%, high average 19.7%, and high 11.8%. Z-score for weight was $-1.00{\pm}1.20$ (-4.54~+2.50). Z-score for height was $-1.55{\pm}1.65$ (-9.42~+1.87). Growth hormone was administered in 24.3% of patients. Anti-hypertensive drugs were administered in 64.0% of patients. Laboratory findings were as follows: hemoglobin $10.5{\pm}1.4$ g/dL, calcium $9.7{\pm}0.7$ mg/dL, phosphorus $5.4{\pm}1.4$ mg/dL, and parathyroid hormone $324.2{\pm}342.8$ pg/mL. Conclusion : Primary glomerular disease was the most common cause of ESRD. CAPD was the most prevalent PD modality. Low and low average peritoneal transport type were common. Growth disturbance were noted in many patients. Some patients had hypertension even with anti- hypertensive drugs. Calcium-phosphorus levels were maintained adequately, but many patients had secondary hyperparathyroidism.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.232-239
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• 2002

Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. Results : In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases( 7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. Conclusion : Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.