• Title/Summary/Keyword: 만성 사구체 신염

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A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children (소아 급속 진행성 사구체 신염의 임상-병리학적 고찰)

  • Cho Hee-Yeon;Chung Dae-Lim;Kang Ju-Hyung;Ha Il-Soo;Choi Yong;Cheong Hae-Il
    • Childhood Kidney Diseases
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    • v.8 no.2
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    • pp.176-185
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    • 2004
  • Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

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A Case of Acute Poststreptococcal Glomerulonephritis Associated with Chronic Granulomatous Disease (만성 육아종 질환에 동반된 급성 연쇄상 구균 감염 후 사구체 신염 1례)

  • Kim Hyung-Tae;Seo Jung-Wook;Kim Han-Seong;Lee Chong-Guk
    • Childhood Kidney Diseases
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    • v.7 no.2
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    • pp.217-222
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    • 2003
  • Chronic granulomatous disease(CGD) is a rare inherited disorder of phagocytic cells which results in a susceptibility to infections by catalase-positive bacteria and fungi, as well as granuloma formation. And acute poststreptococcal glomerulonephritis(APSGN) is one of the most common glomerular lesions of gross hematuria in children. We experienced a case of APSGN accompanied with CGD presenting with a liver granuloma.

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A Case of Type II Membranoproliferative Glomerulonephritis Detected by School Urinary Screening Tests (학교 신체검진 상 우연히 발견된 제2형 막증식성 사구체신염 1예)

  • Kim, Hyung-Joong;Kwak, Byung-Ok;Bae, Jae-Uk;Kim, Kyo-Sun;Lim, So-Dug
    • Childhood Kidney Diseases
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    • v.14 no.1
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    • pp.79-83
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    • 2010
  • Type II membranoproliferative glomerulonephritis (MPGN) is characterized by thickening of the glomerular basement membrane owing to electron-dense deposits on electron microscopy. We experienced a case of type II MPGN in a child presenting with proteinuria, hematuria on school urinary screening tests. He had been treated with losartan and enalapril. This is the first case report of type II MPGN detected by school urinary screening tests in Korea. Thus we report a case of 10-years-old male with type II MPGN with a review of brief literature.

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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Angiotensinogen M235T Polymorphism in Children with $Henoch-Sch\"{o}nlein$ Purpura Nephritis ($Henoch-Sch\"{o}nlein$ Purpura 신염에서 Angiotensinogen M235T 유전자 다형성)

  • Ha Chang-Woo;Joo Hee-Jung;Park Ji-Kyoung;Chung Woo-Yeong
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.10-17
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    • 2004
  • Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angioten-sinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. Methods : The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. Results : The M235T polymorphism of AGT gene frequency was MM 75%, MT : 25%, TT : 0% in HSP and MM : 64%, MT : 36%, TT : 0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 $mg/m^2/day$) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. Conclusion : We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

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Glomerular Filtration Rate Test Methods and Guidelines (Glomerular Filtration Rate 검사방법 및 가이드라인)

  • Park, Min-Ho;Lee, Ha-Young;Ryu, Hwa-Jin;Yoo, Tae-Min;Noh, Gyeong-Woon
    • The Korean Journal of Nuclear Medicine Technology
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    • v.22 no.2
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    • pp.97-100
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    • 2018
  • Purpose The glomerular filtration rate (GFR) test is an important indicator of glomerular filtration and has been used to test renal function and the extent of its function. The GFR test is performed by intravenous injection of radioactive medicines made of $^{51}Cr$-EDTA, and blood concentration is measured by taking blood according to the elapsed time. also, PET-CT, bone scan, transfusion and so on will affect the outcome. Therefore, we will improve the quality of the test by providing guidelines for the GFR test for more accurate testing. Materials and Methods 5 mL of physiological saline solution and 2 mL of $^{51}Cr$-EDTA solution are used to make 5 mL of the radiopharmaceutical solution to be injected into the patient. First, the syringe weight is measured before the injection, and then the radioactive medicine is injected into the patient's vein and the syringe weight is measured after the injection. Blood sampling is performed twice in total. In adults, blood is collected 3 hours / 5 hours after injection and in children 2 hours / 5 hours after injection. The blood sample is centrifuged at 3300 rpm for 5 minutes. Standard solution is prepared by filling diluent water up to the scale indicated in the 200-mL volumetric flask, discarding $500{\mu}L$, injecting $500{\mu}L$ of GFR reagent and mixing well. $500{\mu}L$ each of the standard solution is dispensed into two test tubes, and $500{\mu}L$ of each of the plasma samples collected in time is dispensed into two test tubes and measured with a Cobra Counter. Results At present, the reference range applied in this study is $119.5{\pm}30.3ml/min/1.73m2$ for males and $125.2{\pm}28.2ml/min/1.73m^2$ for females. Conclusion The GFR test is conducted using radioactive medical products. GFR testing is performed as a scheduled test, but PET-CT, dialysis and transfusion, which may affect GFR testing, may be scheduled during GFR testing. Therefore, we could get accurate GFR test results by notifying the ward and department beforehand when booking.

耐 마모 코우팅 생산을 위한 CVD와 PVD 기술

  • O, Seung-Tak
    • Journal of the Korean institute of surface engineering
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    • v.20 no.1
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    • pp.15-24
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    • 1987
  • 超硬合金과 高速度鋼에 TiC, TiN 그리고 $Al_2O_3$의 耐 마모 코우팅시에 사용되는 CVD와 PVD 기술을 M E Sjostrand와 A G Thelin 등이 본 논문에서 논의하였다. 또한 CVD와 PVD 의 특별한 장점과 응용영역에 대하여도 토의하였다. 수년동안 CVD 기술은 금속질화물, 금속탄화물 그리고 금속산화물 등의 耐 마모 코우팅을 생산하는데 성공적으로 사용되어 왔다. Munster와 Ruppert에서는 1950년대 초기에 TiC와 TiN의 CVD 공정을 연구했는데 이런 코우팅 재료의 높은 화학적 안정성과 더불어 高硬鋼라는 독특한 성질로 인해 그 주요 영역이 명백해졌다. 1968년에 처음으로 CVD법에 의한 보호막 코우팅의 대규모 응용이 이루어졌는데 그것은 超硬合金 절삭공구의 코우팅이었다. (그림1 참고) 이것은 硬금속산업에 있어서 가장 중요한 발전단계였다. TiC, TiN 그리고 $Al_2O_3$로 코우팅된 공구의 생산은 1970년대에 빠른 성장을 보였으며 오늘날 사용되는 절삭날의 50% 이상을 점유하고 있다. (그림2 참고) TiC와 TiN은 현재 이용되고 있는 모든 耐 마모 코우팅 중에서 독보적인 위치를 차지하고 있다. 그 이유는 생산과정이 비교적 단순하기 때문이다. 지난 5년동안 절삭공구와 總形바이트의 코우팅에 대한 PVD기술의 응용이 폭발적으로 증가했다. 증착기술인 이들 CVD와 PVD 각각은 자체의 독특한 장점이 있으므로 그 응용영역은 증착조건, 즉 기지금속의 접착성과 증착온도 그리고 증착속도등에 따라 매우 다를 것이다. PVD 공정으로 인해 고속도 공구강이 급속도로 발전되었고 더우기 PVD공정은 500$^{\circ}C$ 이하의 증착온도에서 brazed carbide 공구의 코우팅을 가능하게 하였다. 따라서 두 증착기술은 서로 상반적이라기 보다는 상호보완적인 것이라고 생각하는 편이 더 좋다.TEMPLA에 비해 CLB의 수가 15.58% 감소되었다. 높은 활성을 나타내었다. 정제된 효소의 최적온도는 40$^{\circ}C$이었으며 20~50$^{\circ}C$에서 비슷한 활성을 나타내었고, 30$^{\circ}C$에서는 60분동안 효소활성이 거의 상실되지 않았다. 정제된 효소는 ethanol과 chloroform 처리에는 안정하였으나 12mM AT 와 0.1mM $NaN_3$ 및 1mM KCN에 의해 90% 이상의 활성이 억제되었다.이에 근거하여 서울시 학생들($7{\sim}18$세)의 만성신부전증 유병률은 1백만명당 5.7명으로 추정되었다. 결론 : 서울시내 학생들 중 11세, 14세, 17세 3개 군에서 한 번 검사로 확인된 무증상 단백뇨의 유병률은 0.28%(약 2.8명/1,000명)이었고 이들중 약 5%만이 3차검사에서 신질환이 의심되었으며 이에 따른 신질환 유병률은 1만명당 1.4명이었다. $7{\sim}18$세 연령층에서 무증상으로 발생하는 사구체 신질환 중에는 IgA 신병증의 유병률이 가장 높아 1만명당 0.64명으로 추정되었고 만성신부전증의 유병률은 1백만명당 5.7명으로 추정되었다. 집단뇨 검사를 통해 확인되는 신질환은 대부분 사구체 질환이기 때문에 집단뇨검사의 의의는 좀더 연구가 필요할 것으로 생각되었다. 오히려 증상을 동반하는 경우보다 빈도가 증가한다는 사실은 집단뇨 검사에서 소변의 이상소견이 발견되어 신장 조직검사를 실시할 경우 혈청 $C_3$치의 감소 여부에 관계없이 MPGN도 진단적 고려 대상이 되어야 한다고 생각한다.신장 조직검사를 시행한 결과 진행성 경과를 취할 수 있는 막 증식성 사구체 신염과 매우 희귀한 증례인 신유전분증 등으로 진단됨으로써 지속성 단백뇨의 경우 정확 진단적 접근이 필수적임을 알 수 있다. 기립성

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Prognosis of Acute Poststreptococcal Glomerulonephritis (APSGN) in Children (소아 연구균 감염 후 급성 사구체 신염의 예후)

  • Shin, Yun-Hye;Lee, Ji-Yeon;Pai, Ki-Soo
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.137-142
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    • 2005
  • Purpose : Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A$\beta$-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. Methods : Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A $\beta$-hemolytic streptococcal infection were evaluated. Results : Six(12.5$\%$) patients showed elevation of serum creatinine level but there was no patient with Persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9$\%$). Hematuria disappeared within 6 months(79$\%$) and proteinuria within 6 months(100$\%$) from the disease onset. Conclusion : Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4$\%$) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequoia. (J Korean Soc Pediatr Nephrol 2005;9:137-142)

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Clinicopathologic Features and HBsAg and HBeAg Expressions in Hepatitis B Virus-associated Glomerulopathy (B형 간염 바이러스 감염과 연관된 사구체신염의 임상병리학적 특성과 신 조직내에서 HBs Ag 과 HBe Ag의 발현)

  • Jung Soo-Jin;Kim Young-Joo;Yoon Hye-Kyoung;Chung Woo-Yeong;Kim Young-Hoon;Kim Su-Yung
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.50-59
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    • 1998
  • Purpose : Hepatitis B virus (HBV) infection has been involved in several forms of immune-related glomerulopathy but the pathogenic role of HBV infection is not clear. To evaluate the clinicopathological features of HBV-associated glomerulopathy, a clinicopathological analysis and immunohistochemical stain for HBs Ag and HBe Ag were done. Methods : Clinicopathological features of HBV-associated glomerulopathy were analyzed with renal biopsies in 28 HBsAg seropositive patients from April 1990 to February 1997 at Pusan Paik Hospital, and immunohistochemical evaluation for HBsAg and HBeAg was done in renal tissues. Light microscopic, immunofluorescent and electron microscopic examination and immunohistochemical staining for HBsAg (DAKO) and HBeAg (BIONIKE) of renal tissue were performed. Result ; 1. The age distribution was 6 to 73 years old, and eight were children and 20 were adults. Male : female ratio was 3:1. Nineteen (67.9%) and 21 (75.0%) of 28 cases showed hematuria and proteinuria, respectively at the time of biopsy. Sixteen (57.2%) of them had nephrotic syndrome. 2. Liver function test was performed in 11 patients and seven (63.6%) of them showed increased AST and ALT levels. Liver biopsy was done in three patients and revealed findings of chronic active hepatitis. 3. HBV-associated glomerulopathy was membranous glomerulonephritis (MGN) in 10 (35.7%), mesangiopathy in 8 (28.6%), membranoproliferative glomerulonephritis (MPGN) in 7 (25.0%) and minimal change disease in 3 (10.7%) out of 28 cases. 4. Ultrastructurally HBV-associated MGN showed conspicuous subepithelial deposits with intramembranous, mesangial and subendothelial deposits and proliferation of mesangial cells and matrix, which were suggestive of MPGN. In HBV-associated MPGN, intramembranous and subepithelial deposits were scattered. 5. Immunohistochemical staining revealed no expression for HBsAg, but positive reaction for HBeAg along capillary wall in 8 cases (28.6%), of which 3 cases were checked for serum HBeAg, all showed positivity. Conclusion : HBV-associated glomerulopathy showed a wide morphologic spectrum and overlapping ultrastructural features in MGN and MPGN, and the activity of hepatitis B virus may be related to the development of HBV-associated glomerulopathy but further studies are recommended to confirm this relationship.

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