• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• Pediatric Infection and Vaccine
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• v.24 no.2
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• pp.112-116
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• 2017

Kawasaki disease (KD) is an acute febrile mucocutaneous lymph node syndrome that commonly presents with stable hemodynamic status during the acute phase. An 8-year-old boy initially presented with severe hypotension and acute kidney injury. He was placed in the intensive care unit and was diagnosed with KD. Observed clinical features were defined as KD shock syndrome. His coronary artery was dilated during the subacute phase. Furthermore, he was given anti-hypertensive medications, owing to hypertension as an unusual complication of KD. We knew the importance of monitoring for blood pressure considering vasculitis as an aspect of the main pathogenesis of KD.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.205-213
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• 2004

Purpose: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. Methods: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospital from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. Results: The male to female ratio was 1.6:1 and they were $9.8\pm2.6$ years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. Conclusion: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.

• 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.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.127-131
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• 2013

Urinomas can occur after renal trauma or perforation of the collecting system during an endosurgical procedure. However, spontaneous urinomas are very rare. Here we report a case of a spontaneous perinephric urinoma following the removal of a Foley catheter in an 18-year-old girl with acute kidney injury caused by septic shock. The patient had been treated for septic shock, acute kidney injury, and acute respiratory distress syndrome, and had a Foley catheter in place for seven days. After Foley catheter removal, the patient complained of consistent voiding difficulty. An abdominal computed tomography scan showed a large amount of left perinephric fluid, and the aspirated fluid included urothelial cells, confirming the diagnosis of a urinoma. The urinoma was successfully treated by insertion of a double-J stent into the left ureter. This report discusses the available literature on urinomas, and their clinical features, diagnosis, and treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.28-35
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• 2007

Purpose: The aim of this study was to evaluate the clinical usefulness of ultrasound examination of children performed by a pediatrician. Methods: One thousand children who presented with symptoms of a gastrointestinal disorder and underwent abdominal ultrasound evaluation in the Department of Pediatrics, between January 2003 and June 2006, were included in this study. We analyzed the patient's medical records and ultrasound results retrospectively. Results: Among the 1,000 patients, 58.4% were male and 41.6% were female. The mean age of the patients was $4.7{\pm}4.0$ years. The main reasons for ultrasound were abdominal pain (43.9%), vomiting (17.3%), elevated liver enzymes (11.8%), and jaundice (9.8%). Abnormal ultrasound findings were present in 57.9% of cases. The major abnormal findings were mesenteric lymphadenitis (29.2%), fatty liver (12.1%), hepatitis (6.4%), hepatosplenomegaly (6.2%), and acute appendicitis (4.8%). The time interval between the initial medical evaluation and the ultrasound evaluation was within 24 hours in most cases (78.5%). The main findings in children with abdominal pain were mesenteric lymphadenitis (32.6%), fatty liver (5.9%), intussusception (2.7%), and acute appendicitis (2.7%). The main findings in children with vomiting were mesenteric lymphadenitis (12.7%), hypertrophic pyloric stenosis (10.4%), and acute appendicitis (3.5%). The major ultrasound findings in children with urinary tract diseases were hydronephrosis (45.4%), urolithiasis (21.5%) and cystic renal disease (18.1%). Conclusion: Ultrasound examination played an important role as a non-invasive and prompt screening examination for detection of abdominal diseases. Ultrasound was an important tool for pediatricians to determine timely information for patient management.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.7 no.2
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• pp.103-106
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• 2011

다운 증후군(Down syndrome, Trisomy 21)은 가장 흔하게 나타나는 증후군으로 대략 1/1000의 확률로 태어난다. 세 개의 유전적 유형이 있지만, 95% 정도가 3개의 21번 염색체를 갖는 비분리 염색체 유형이다. 주된 구강 증상으로는 거대설, 균열설, 과잉치, 결손치, 왜소치, 유치와 영구치의 맹출지연 그리고 이에 따른 부정교합 등이 있다. 75%의 환자에서는 어린 나이에 치주질환에 이환되기 쉬우며, 치아우식증에는 낮은 이환율을 보인다는 보고가 있다. 급성 림프구성 백혈병(Acute Lymphoblastic Leukemia)은 주로 어린이에서 나타나며, 특히 다운 증후군 환자에서의 발병율은 정상인에 비해 20배 높다. 치은비대와 출혈의 양상이 주로 나타나며, 간혹 상악골과 구개골에 종괴가 관찰 되기도 한다. 본 증례에서는 서울대학교치과병원 소아치과에 내원한 환자 중 급성림프구성 백혈병을 앓고 있는 다운 증후군 환아가 있어 이를 보고하고자 한다.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.111-119
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• 2000

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a common pediatric discase presenting most frequently with skin, gastrointestinal, joint and renal manifestations. The prognosis of HSP is mainly determined by the involvement of the kidney, but prognostic markers have not been established. We evaluated the patients who have HSP nephritis with nephrotic syndrome. Method : Clinical manifestations and laboratory findings were observed and analyzed in 34 cases with HSP which were manifested by nephrotic syndrome hospitalized at Kyung Hee university Hospital during the period from Jan. 1990 to Dec. 1998. Results : 1) Male to female ratio was 1.3:1, and mean age at onset was 8.3 year. 2) Mean duration from symptom onset to renal biopsy was 10.5 weeks. 3) Proportion of patients presenting with acute nephritis was 32.4$\%$, gross hematuria 17.6$\%$, microscopic hematuria 50$\%$. 4) The findings of renal biopsy were 20 cases of grade II, 11 cases of grade III, 2 cases of grade I, 1 case of grade IV according to classification by ISKDC. 5) Patients with grade I were recovered with no residual defect, but patients with grade IV shows active renal disease(states C). Conclusion : Among the 디le patients with Henoch-$Sch{\ddot{o}}nlein$ purpura accompanying nephrotic syndrome, more aggressive treatment might be needed in patients showing crescents formation on renal biopsy. A prospective study will be needed to explore the progression of this disease.

• 월간산업보건
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• s.90
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• pp.22-25
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• 1995

신장은 급성 혹은 만성 신장 기능부전 혹은 말기 신장질환을 야기할 수 있는 작업장내의 여러 화학물질에 폭로되어 있다. 이러한 물질들은 효과에 있어 상이하며 4개의 주요 형태로 분류할 수 있다. 중금속, 유기화합물, 살충제들, 다른 생물이물질 등, 신독성은 산업의학에서 독자적인 소견으로는 나타나지 않고 독성 폭로의 다른 전신적 증상과 함께 나타난다. 신장은 흔히 독성 물질의 공격 목표가 된다. 비교적 크기가 작음에도, 심박출량의 25%를 받아 다량의 독성 물질에 폭로된다. 이러한 기능때문에, 신장에서 삼투압 경사가 발생하여 - 주로 수질에서 - 신장은 다른 기관에서 발견되는 것보다 훨씬 높은 수준으로 독성물질을 농축한다. 신장은 소변을 신성화할 수 있기 때문에, 다른 조직에서 발견되지 않는 이온형태의 여러 용질이 발생한다. 이러한 여러 요인들로 신장이 여러 독성물질에 어떻게 영향을 받는지 설명할 수 있다.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.123-127
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• 2014

Erythema multiforme (EM) is an acute mucocutaneous disorder involving the skin, mouth, eyes, and genital organs. It is classified into EM minor and EM major according to the involvement of the mucosal membrane. Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) belong to EM major. Compared to EM minor, SJS presents with more severe and progressive symptoms, and has a higher mortality rate. Corticosteroids are used in the treatment of EM. We report three cases of EM (two cases of EM minor and one case of SJS) that developed during treatment with oral corticosteroid (deflazacort; $Calcort^{(R)}$) in children with nephrotic syndrome.