• Title/Summary/Keyword: Glomerular Basement Membrane

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Clinical and Pathologic Analysis of Thin Glomerular Basement Membrane Disease in Children (소아 비박형 기저막신증의 임상 및 병리학적 분석)

  • Ko Myoung Jin;Yang Tae Jin;Kim Young Ju;Chung Woo Yeong
    • Childhood Kidney Diseases
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    • v.5 no.1
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    • pp.1-8
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    • 2001
  • Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

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The Transmission Electron Microscopic Study on the Alteration of Filtration Barrier in Aged Rat Kidney (흰쥐 콩팥여과관문의 노화 변화에 관한 투과전자현미경적 연구)

  • Lee, Se-Jung;Lim, Hyoung-Soo;Lim, Do-Seon;Hwang, Douk-Ho
    • Applied Microscopy
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    • v.38 no.2
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    • pp.107-115
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    • 2008
  • The filtration barrier of kidney consists of endothelial cell, glomerular capillary, glomerular basement membrane, mesangial matrix, and podocyte. In aged rats, the morphological changes were shown in various parts, including the glomerulus. These changes were thickening of basement membrane and mesangial matrix, crescent formation of glomerular capillary, deformity of foot processes, glomerular sclerosis and obsolescence. But these glomerular morphologies are partial images or few serial images analysis. In this study, we examined the morphological alteration of glomerulus in the young and aged rats by light microscopy, transmission electron microscopy and three dimensional reconstruction. We were found in aged rat glomerulus, expansion of urinary space and mesangial matrix, thickening and degrading of glomerular basement membrane, decreasing in podocyte foot processes, fragmentation of podocytic nucleus membrane. These observations indicate that may provide useful data for investigating the pathogenesis of age-related dysfunction of kidney.

Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome : Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis (비박형 사구체 기저막 질환이 동반된 중복 자궁 일측성 폐쇄질 및 동측 신장 무형성 증후군 ( Herlyn - Werner- Wunderlich syndrome ) 1례)

  • Kim, Myoung-Soo;Park, Yong-Jun;Park, Young-Jun;Park, Noh-Hyuck;Song, Ji-Sun;Kim, Pyung-Kil
    • Childhood Kidney Diseases
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    • v.11 no.2
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    • pp.299-305
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    • 2007
  • Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.

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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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A Comparative Analysis of the Clinical and Pathological features of IgA Nephropathy and Thin Glomerular Basement Membrane Disease (IgA 신병증과 비박형 기저막 신증의 임상 및 병리학적 비교 분석 - 사구체 기저막의 비박화를 중심으로 -)

  • Chi, Geun-Ha;Ha, Chang-Woo;Kim, Young-Ju;Yoon, Hye-Kyung;Chung, Woo-Yeong
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.147-155
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    • 2001
  • Purpose : IgA nephropathy(IgAN) and thin glomerular basement membrane disease(TGBMD) are common glomerular diseases that cause hematuria in childhood. IgAN has characteristics of IgA deposit as the sole or predominantly localized to the mesangium Recently, it has been reported that thinning of glomerular basement membrane(GBM) is commonly accompanied with precipitation of electron dense deposits in IgAN. We performed this study to examine the frequency of thinning of GBM among children with IgAN and to analysis tile correlation between urinary abnormalities and GBM thickness and furthermore to conduct comparative analysis of the clinical and pathological features of IgAN and TGBMD. Methods : This study summarizes data collected from Department of Pediatrics, Busan Paik Hospital, Inje Medical College. Data include 51 cases who were diagnosed as IgAN from 1995 to 2000, and 26 cases who were diagnosed as TGBMD from 1990 to 2000 by percutaneous renal biopsy. Results : Males accounted for 29/51($56.9\%$) patients with IgAN and 8/26($30.8\%$) of those with TGBMD. The clinical and laboratory features between IgAN and TGBMD were significantly different regarding the incidence of proteinuria(IgAN vs TGBMD: $43.1\%\;vs\;3.8\%$, p=0.001), the incidence of co-appearance of proteinuria with hematuria ($41.2\%\;vs\;3.8\%$, p=0.001), total amount of protein in 24 hours collected urine ($808{\pm}\;mg\;vs\;251{\pm}200.7\;mg$, p=0.001) and the incidence of proteinuria more than 1 gm in 24 hours collected urine ($23.5\%\;vs\;3.8\%$, p=0.01). On the contrary, there were no significant differences in the levels of serum albumin, creatinine, BUN, and Ccr between two groups. The mean thickness of GBM in patients with IgAN was $293.0{\pm}79.2\;nm$(139.7-461.9 nm) and $180.9{\pm}35.8\;nm$(110.5-229.5 nm) in patients with TGBMD. The mean GBM thickness revealed significantly thinner in TGBMD compared than those with IgAN (P=0.0001). The frequency of thickness being less than 250 nm was $37.4{\pm}34.4\%$ in IgAN and $93.0{\pm}7.0\%$ in TGBMD (P=0.0001). But there were no correlations between urinary abnormalities and GBM thickness in patients with IgAN. Conclusion : The thinning of GBM would be one of the common pathological findings in IgAN Moreover, there is no significant correlations between urinary abnormalities and GBM thickness in patients with IgAN, However, patients with IgAN tend to have significantly higher possibilities of proteinuria, co-appearance of proteinuria with hematuria and higher total amount of protein in 24 hours collected urine compared those with TGBMD. These differences might be play all important role as progressive prognostic indicators in patients with IgAN. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

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Genetic Basis of Steroid Resistant Nephrotic Syndrome

  • Park, Eujin
    • Childhood Kidney Diseases
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    • v.23 no.2
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    • pp.86-92
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    • 2019
  • Steroid-resistant nephrotic syndrome (SRNS) has long been a challenge for clinicians due to its poor responsiveness to immunosuppressants, and rapid progression to end-stage renal disease. Identifying a monogenic cause for SRNS may lead to a better understanding of podocyte structure and function in the glomerular filtration barrier. This review focuses on genes associated with slit diaphragm, actin cytoskeleton, transcription factors, nucleus, glomerular basement membrane, mitochondria, and other proteins that affect podocyte biology.

Long-term Prognosis of thin Glomerular Basement Membrane Nephropathy in Children: A Retrospective Single Center Study

  • Lim, Myung Hee;Bae, Hee Jung;Jang, Kyung Mi;Park, Yong Hoon
    • Childhood Kidney Diseases
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    • v.21 no.2
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    • pp.41-46
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    • 2017
  • Purpose: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. Methods: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. Results: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was $7.18{\pm}2.64$ years, and mean time from onset of disease until a renal biopsy was performed was $2.48{\pm}2.10$ years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. Concluson: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

Detection of Mercury in Kidney, Liver, Spleen and Cerebellum of the Mouse by Autometallography (오토메탈로그라피에 의한 마우스의 신장, 간장, 비장, 및 소뇌에 축적된 수은의 검출)

  • 조현욱;김명훈;황규영;이성태
    • Toxicological Research
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    • v.13 no.4
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    • pp.401-408
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    • 1997
  • Adult male ICR mice were exposed to methylmercuric chloride (CH$_3$HgCI) through drinking water for 80 days. The distribution of mercury in the kidney, liver, spleen and cerebellum of the mouse was examined according to a autometallographic silver-enhancement technique based on a physical development process which renders mercury deposit visible. Grains of mercury traces were located in the proximal convoluted tubules. Lesser staining of the grains was seen in the collecting tubules of medulla. The glomerular basement membrane was void. In the liver, mercury accumulations were present primarily in the hepatocytes around portal area containing interlobular bile duct, artery and portal vein. Also grains of mercury traces were accumulated in the white pulp of the spleen and Purkinje cell layer of the cerebellum.

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Diffuse Alveolar Hemorrhage

  • Park, Moo Suk
    • Tuberculosis and Respiratory Diseases
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    • v.74 no.4
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    • pp.151-162
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    • 2013
  • Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.

Pathology of C3 Glomerulopathy

  • Shin, Su-Jin;Seong, Yoonje;Lim, Beom Jin
    • Childhood Kidney Diseases
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    • v.23 no.2
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    • pp.93-99
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    • 2019
  • C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.