• Title/Summary/Keyword: Alport 증후군

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A Study on the Association of Thin Glomerular Basement Membrane Abnormality with Minimal Change Nephrotic Syndrome (사구체 기저막 비박화 소견을 보인 미세변화 신증후군에 관한 고찰)

  • Kim, Chang-Woo;Cho, Min-Hyun;Ko, Cheol-Woo;Koo, Ja-Hoon;Kwak, Jung-Sik
    • Childhood Kidney Diseases
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    • v.6 no.1
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    • pp.48-55
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    • 2002
  • Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

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Clinicopathological Analysis of Glomerulonephritis in Children (소아 사구체신염의 임상 및 병리학적 분석)

  • Park Gwang-Yong;Yoon Hye-Kyoung;Chung Woo-Yeong
    • Childhood Kidney Diseases
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    • v.1 no.1
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    • pp.4-12
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    • 1997
  • Purpose: To evaluate the prevalence and clinical manifestations of various glomerulonephritis (GN) in children, a clinicopathological anlysis of 310 biopsied cases were performed. Method: We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 310 cases diagnosed as glomerulonephritis by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results: 1) Male to female ratio was 1.54:1 and the range of age was from 13 months to 15 years 10 months. 2) Among these, 217 (70.0%) patients were belong to primary GN and 93 (30.0%) patients were belong to secondary GN. As a whole, the most common pathologic diagnosis was minimal change lesion (MC, 32.6%), which was followed by IgA nephropathy (IgAN, 15.8%), $Henoch-Sch\"{o}nlein$ purpura nephritis (HSPN, 13.5%), Poststreptococcal glomerulonephritis (PSAGN, 8.1%). 3) Clinical manifestations of patients were asymptomatic urinary abnormality (43.2%), nephrotic syndrome (41.0%), acute glomerulonephritis (14.2%), chronic glomerulonephritis (1.0%), rapidly progressive glomerulonephritis (0.6%). 4) In primary GN, the most common pathologic diagnosis was MC (46.5%), IgAN (22.6%), thin glomerular basement membrane (GBM) disease (7.8%), membranoproliferative glomerulonephritis (MPGN, 5.5%), mesangial proliferative glomerulonephritis (MesPGN,4.6%), focal segmental glomerulosclerosis (FSGS, 4.6%), membranous nephropathy (MN, 0.9%), sclerosing glomerulonephritis (SCGN, 0.9%), crescentic glomerulonephritis (CreGN, 0.5%) and non-specific glomerulonephritis (NonspGN, 6.0%). 5) Major causes of secondary GN were HSPN (45.2%), PSAGN (26.9%), hepatitis B associated glomerulonephritis (HBGN, 17.2%), lupus nephritis (LN, 6.5%), Alport syndrome (2.2%), hemolytic uremic syndrome (1.0%), fibrillary glomerulonephritis (1.0%) in descending order. Conclusions: There are some differences of the results of clinicopathological stuidies of glomerulonephritis in children because of its different indications of renal biopsy, pathologic classification of renal disease and methods of analysis among investigators. In order to establish more reliable data of incidence and classification of childhood glomerulonephritis in Korea, multicenter cooperative study were necessary.

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Arthroscopic treatment of septic arthritis of the knee in adults (성인의 화농성 슬관절염의 관절경적 치료)

  • Kyung Hee-Soo;Ihn Joo-Chul;Oh Chang-Wug;Kim Sung-Jung;Kim Joon-Woo
    • Journal of the Korean Arthroscopy Society
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    • v.6 no.1
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    • pp.21-24
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    • 2002
  • Purpose : The purpose was to assess the result of arthroscopic management of the septic arthritis on the knee in compromised patients. Materials and Methods : Fourteen patients with septic knee were analyzed. The mean age was 55 years and the mean follow-up period was 14.6 months. Underlying diseases included 4 cases of diabetes, and history of direct acupuncture in 4 cases. Clinical stage of septic arthritis was judged by $G\ddot{a}chter's$ classification, which was determined by arthroscopic findings. After arthroscopic irrigation and debridement, we observed the results of laboratory data and improvement of clinical findings. Results : Causative organism was identified in 7 cases and no organism was detected in the remaining 7 cases. Stage I was 1, stage II 8, stage III 4, and stage IV 1, respectively. Eleven of 14 cases were improved by one stage operation. Two cases of stage III were recurred and additional arthroscopic management was done. In 1 case of stage IV, symptom was not improved and needed arthrotomy. The result was unsatisfactory in patients with stage III and IV. Serum erythrocyte sedimentation rate and C-reactive protein were normalized after 29.3 and 20.8 days following the operation, respectively. Clinical symptoms disappeared average 2 days following the operation. Conclusion : Arthroscopic management of acute septic arthritis of the knee would be an effective and satisfactory treatment modality in that its postoperative pain and complications are minimal, and it can be done with ease repeatedly.

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Asymptomatic Primary Hematuria in Children (소아의 무증상성 일차성 혈뇨)

  • Cho Min-Hyun;Jang You-Cheol;Kim Young-Cheol;Koo Ja-Hoon;Ko Cheol-Woo
    • Childhood Kidney Diseases
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    • v.8 no.2
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    • pp.166-175
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    • 2004
  • Purpose: Present study has been undertaken to determine the distribution of various renal diseases causing asymptomatic hematuria in children and to evaluate the benefit of doing renal biopsy in these children. Methods: Study population consisted of 146 children with asymptomatic primary hematuria who had been admitted to the pediatric departmen of Kyungpook National University Hospital for the past 4 years from 1999 to 2002. In 122 out of 146 cases, renal biopsy was performed percutaneously and in 24 out of 146 cases, diagnosed as idiopathic hypercalciuria, oral calcium loading test was performed. Results: The age$(mean{\pm}SD)$ at onset or discovery of hematuria of the 146 children in-cluded in this study was $8.0\pm3.2$ years and the proportion of boys and girls was 54.8% and 45.2%, respectively. In 76 out of 146 cases(52%), asymptomatic hematuria was first diagnosed by school urinalysis screening. The proportion of histopathologic findings based on 122 biopsies was as follows : Thin Glomerular Basement Membrane(TGBM) 73 cases(50%): IgA nephropathy 20 cases(14%): Alport syndrome 6 cases(4%), Membranous Glomerulonephropathy(MGN) 4 cases(3%): Membranoproliferative Glomerulonephritis(MPGN) 2 cases(1%); IgA nephropathy with TGBM 3 cases(2%): 'normal' glomeruli 14 cases(10%) Twenty four cases (16%) were diagnosed as idiopathic hypercalciuria. During follow-up periods, 15% of 146 cases became hematuria-free and renal function did not deteriorate in any cases. Conclusion: Unless hematuric children manifest poor prognostic indicators for renal survival, we would recommend long term regular follow-up prior to a renal biopsy.

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A Histological and Clinical Study of the Children with Thin Glomerular Basement Membrane (사구체 기저막 비박화를 보인 소아들의 조직학적 및 임상적 고찰)

  • Kim Young-Chol;Lee Dong-Won;Cho Min-Hyun;Kwak Jung-Sik;Ko Cheol-Woo
    • Childhood Kidney Diseases
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    • v.9 no.1
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    • pp.31-37
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    • 2005
  • Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

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