• 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.6 no.1
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• pp.37-47
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• 2002

Purpose: We reviewed the clinical manifestations, responsiveness to treatment, and prognosis in children with nephrotic syndrome. Methods: Medical records of 159 children with idiopathic nephrotic syndrome who were admitted to the pediatric department of Chonbuk National University Hospital from January 1979 to December 2000 w ere retrospectively reviewed. Results: There were 32 females and 127 males. The most common age group was between 3 and 5 years of age among the 159 children with nephrotic syndrome. Generalized edema ($75.5\%$), scrotal edema ($20.1\%$), upper respiratory infection ($19.5\%$), and ascites ($28.3\%$) were frequently observed. After the initial steroid therapy, diuresis occurred within tile first two weeks in 138 children, and proteinuria disappeared within the first two weeks in 105 children. Among 159 patients who received initial daily steroid therapy, 110 children were in complete remission, 29 children were in partial remission and 20 children were in poor response state. Hematuria, hypertension and elevated serum creatinine were more frequently observed in the partial and the poor response groups than in the complete remission group. Among 107 children who were followed up for more than one year, 78 children were in complete remission and 55 children were relapsed within the first one year after steroid therapy. Renal biopsy was undertaken in 76 children and 53 children had minimal change nephrotic syndronm. Conclusion: Our study showed that illost children with idiopathic nephrotic syndrome have a good responsiveness to steroid therapy and even most children show frequent relapse during 1st year after remission, long term prognosis is excellent.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.80-87
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• 1999

Purposes : Renal involvement is a potentially serious complication of systemic lupus erythematosus (SLE). There have been only few studies of lupus nephritis in pediatric age. In this study, the clinical manifestations, pathologic findings, response to treatment, and clinical course of lupus nephritis in children were analyzed. And the results will provide basic data for future nation-wide prospective multi-center study. Methods . The medical records of 46 children clinically and pathologically diagnosed to have lupus nephritis at Seoul National University Children's Hospital during 1986 to 1997 were analyzed retrospectively. Results : 1) The median age of diagnosis of lupus nephritis was 12.8 years ($2\;years\~\;15year$ 8months), and the sex ratio was 1:2.5. 2) FANA($85.7\%$), anti-ds-DNA antibody ($78.0\%$), and malar rash ($60.8\%$) were the most common findings among the classification criteria by ARA Decreased C3 was detected in $88.9\%$ of patients. 3) Hematuria ($87.0\%$) was the most common renal symptom, and WHO class IV lupus nephritis was identified in 41 cases by renal biopsy. 4) In most of patients, the disease activity was controlled relatively well with a single or combined therapy of prednisolone, azathioprine, or cyclophosphamide. The response revealed no difference according to the mode of treatment. 5) Infection, especially of Varicella-Zoster virus and candida, was the most common complication during the disease course. Conclusion : The renal involvement was noted in $87.0\%$ of childhood SLE, and $89.1\%$ of renal lesions was WHO class IV lupus nephritis known to associated with poor long-term prognosis. So, aggressive treatment using immunosuppressants in the early disease course may be helpful to increase long-term prognosis of lupus nephritis. A prospective multi-center study is necessary to analyze the therapeutic efficacy of various treatment modalities.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.136-143
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• 1997

Purpose : To evaluate the prevalence and clinical manifestations of various glomerulonephritis(GN) in children with asymptomatic urinary abnormalities, a clinicopathological analysis of 134 biopsied cases which were subdivided into 3 groups of proteinuria with hematuria, isolated hematuria and isolated proteinuria was done. Methods : We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 134 cases with asymptomatic urinary abnormalities diagnosed by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results : 1) The proportion of children with asymptomatic urinary abnormalities was 43.2% of all biosied cases. 2) Among these, primary GN were 95 cases and secondary GN were 39 cases, it's ratio was 2.44:1. As a whole, the most common pathologic diagnosis was IgA nephropathy(IgAN, 26.9%), which was followed by $Henoch-Sch\"{o}nlein$ purpura nephritis(HSPN, 17.9%), minimal change lesion(MC, 17.2%), thin GBM disease(12.7%), Hepatitis B associated glomerulonephritis(HBGN, 6.0%), poststreptococcal glomerulonephritis(PSAGN, 3.0%), mesangial proliferative glomerulonephritis(MesPGN, 2.2%), membranoproliferative glomerulonephritis (MPGN, 2.2%), Alport syndrome (1.5%) and Fibrillary nephritis(0.7%). 3) In proteinuria with hematuria, the most common pathologic diagnosis was IgAN(34.6%), which was followed by HSPN(19%), MC(17.7%), thin GBM disease(8.9%), HBGN(6.3%), PSAGN(3.6%), MesPGN(1.2%), MPGN(1.2%) and Alport syndrome(1.2%). 4) Major causes of isolated hematuria were thin GBM disease(19.6%), IgAN(17.6%), HSPN(17.6%), MC(11.8%). 5) Isolated proteinuria was due to of 3 cases of MC and 1 case of HBGN. Conclusion : The prevalence of glomerulonephritis with asymptomatic urinary abnormalities in children were 43.2% of all biopsed cases. When these children were subdivided into 3 groups, proteinuria with hematuria was accounted 58.9%(79 cases) and then isolated hematuria was 38.1%(51 cases), isolated proteinuria was only 3%(4 cases) respectively. The most common pathologic diagnosis was IgA nephropathy in patient with proteinuria and hematuria, and thin GBM disease in patient with isolated hematuria.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.150-156
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• 2003

Purpose : Efforts to predict the clinicopathological outcome of IgA nephropathy have been made but have yielded conflicting results and have not helped in deciding the appropriate timing of the renal biopsy. In this study, we reviewed the predictive factors of clinicopathological outcome for finding out the criteria of renal biopsy timing of IgA nephropathy. Methods : Forty children diagnosed with biopsy proven IgA nephropathy at Wonju Christian Hospital were studied retrospectively, based on medical records. Results : Among 39 patients, 2 children progressed to higher serum creatinine level. One of them reached to the end stage renal disease within 2 year 7 months. According to WHO histopathological classification, there were 15 cases of class I, 14 cases of class II, 7 cases of class III, and 3 cases of class IV. In the mild histological classes(class I, II), gross hematuria was shown in 23 out of 29 children(P=0.02). In the severe histological classes(class III, IV), gross hematuria was noted in 4 out of 10(P>0.05). The tubulointerstitial changes were grade 1 in 24 cases, grade 2 in 4 cases, grade 3 in 8 cases, and grade 4 in 3 cases. With an increase in the tubulointerstitial grade, the 24 hour urine protein/albumin ratio increased. Serum creatinine less than 0.79 mg/dL could predict the lower grade(grade 1 and 2) of tubulointerstitial changes. But serum creatinine greater than 1.13 mg/dL could predict the higher grade(grade 3 and 4) of tubulointerstitial changes. In children with gross hematuria(n=27), serum creatinine was lower(0.78 vs 1.09 mg/dL, P=0.027), serum IgA was higher(316.3 vs 198.8 mg/dL), and the cases of lower WHO classification(I and II) were more common(23 vs 4, P=0.029) than the children with microscopic hematuria. Conclusion : Serum creatinine less than 0.79 mg/dL, macroscopic hematuria, and higher 24 hour urine protein/albumin ratio would predict the lower grade glomerulo tubulointerstitial lesion in IgA nephropathy and could be used as the criteria delaying the renal biopsy.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1287-1295
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• 2006

Purpose : The importance of staphylococcal infections in NICU has been emphasized in terms of increased resistant strains and increased incidence of morbidity and mortality. In this study, we inrestignted the clinical characteristics and risk factors for staphylococcal infections, and looked into sensitivity trends of antibiotics in the era of a high rate of methicillin-resistant staphylococcus aureus (MRSA) in our society. Methods : This study included 240 neonates with positive blood, urine and other sites cultures for staphylococci who were admitted to NICU of Hanil General Hospital and Kyunghee University Hospital from January 2000 to December 2004. The analyses included clinical characteristics of staphylococcal infections and the relationship of incidence rate among various factors, including invasive procedures. Results : For 5 years, 3,593 patients were hospitalized in the NICU and 7,481 specimens were cultured from blood, urine, and other sites. During the study period, staphylococci were isolated from 240 patients, of whom 88 patients had MRSA, 41 patients methcillin sensitive staphylococcus aureus (MSSA), 63 patients S. epidermidis, 48 patients coagulase-negative staphylococcus (CNS) except S. epidermidis infections. The risk factors associated with staphylococcal infections were less than 37 weeks of gestational age, less than 7 on a 5 minutes Apgar score, receiving TPN, applied mechanical ventilation, use of central venous catheters and other tubes. The sensitivity to vancomycin was 100 percent. A relatively high sensitivity against teicoplanin, trimethoprim-sulfamethoxazole, chloramphenicol, clindamycin and low sensitivity against gentamicin and erythromycin were shown. Conclusion : To reduce staphylococcal infections in NICU, we need to monitor and manage premature neonates from the beginning of the birth process and to avoid as many invasive procedures as possible in NICU. Considering MRSA, control of preceding factors and early use of appropriate antibiotics is expected to reduce the morbidity and mortality caused by MRSA infections.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.74-78
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• 2007

Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. Results : The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). Conclusion : Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.946-952
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• 2005

Purpose : Vancomycin-resistant enterococci(VRE) are now nosocomial pathogens in Korea. But little is known about the prevalence of stool colonization with VRE in neonates in Korea. So we studied the prevalence and risk factors of VRE colonization in the Neonatal Intensive Care Unit(NICU). Methods : From January 2000 to December 2004, the medical records of 294 neonates(127 cases of VRE group and 167 cases of non-VRE group, according to the results of stool culture) were reviewed retrospectively. We studied the annual prevalence of VRE and risk factors of VRE colonization in neonates. Results : From 2000 to 2003, the prevalence rate of VRE in NICU increased. After preventing VRE transmission, the prevalence rate of VRE has decreased. Conclusion : VRE colonization increased recently. Risk factors of VRE colonization were prematurity, lower birth weight, longer hospitalization and use of vancomycin or 3rd generation cephalosporin, compared with the non-VRE group. To prevent VRE transmission among newborns, aggressive infection control strategies by NICU staffs must be implemented immediately for all babies.

• Journal of Chest Surgery
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• v.31 no.3
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• pp.315-318
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• 1998

Primary hemangioperiycytoma is quite rare tumor of the lung and arising from pericyte in external layer of capillaries. Most cases are developed in 4th and 5th decade, are asymptomatic, and have malignant otential. On chest radiography, primary pulmonary hemangiopericytoma shows lobulated, well demarcated, homogeneous soft tissue density. Microscopically, it consisits of numerous vascular spaces of variable size and shape separated by aggregates of tightly packed oval to spindle-shaped cells. Treatment of choice is surgical excision. We report a case of primary pulmonary hemangiopericytoma in a 16-year-old man who had well demarcated homogeneous mass in the superior segment of left lower lobe, but had no symptom. He had undergone left lower lobectomy. He has been followed up for 8 months but has no sign of relapse or metastasis yet.

• Korean Journal of Head & Neck Oncology
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• v.29 no.1
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• pp.18-21
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• 2013

랑게르한스 세포 조직구증은 골수에서 유래하는 랑게르한스 세포 조직구의 이상 증식에 의해 발병하는 희귀한 질병으로 알려져 있다. 비록 모든 장기에서 발생 할 수 있으나 갑상선을 침범하는 경우는 매우 드물다. 18세 남자가 5달전부터 점점 커지는 갑상선 종괴를 주소로 내원하여 세침흡인 세포검사, 총샘검, 경부 전산화단층촬영을 시행하였다. 세침흡인 세포검사에서 악성신생물이 의심되었고, 총생검에서 랑게르한스 세포 조직구증으로 나타났다. 경부 전산화단층촬영에서는 우측 갑상선에서 윤곽이 잘 구분되는 저음영의 종괴와 우측 기관 주위 림프절의 종대가 관찰되었다. 갑상선 전절제술과 우측 중앙 선택적 경부 림프절 청소술이 시행되었다. 랑게르한스 세포 조직구증이 갑상선을 침범하는 경우는 드물지만 갑상선 비대가 있는 환자가 뇌하수체 기능부전의 증상이나 뼈와 폐의 침범과 관련된 증상을 호소한다면 갑상선의 랑게르한스 세포 조직구증 침범을 고려해야 한다. 또한, 다른 장기의 랑게르한스 세포 조직구증을 치료한 과거력이 있는 경우는 갑상선 종괴를 감별 진단하는데 있어 랑게르한스 세포 조직구증을 고려해야 한다.