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  1. Article ; Online: Complement System and C4d expression in cases of Membranous nephropathy.

    Custódio, Fabiano Bichuette / Silva, Crislaine Aparecida da / Helmo, Fernanda Rodrigues / Machado, Juliana Reis / Reis, Marlene Antônia Dos

    Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia

    2018  Volume 39, Issue 4, Page(s) 370–375

    Abstract: Introduction: Membranous nephropathy (MN) is one of the major causes of nephrotic syndrome. The complement system plays a key role in the pathophysiology of MN.: Objectives: To identify the complement pathway possibly activated in MN cases and ... ...

    Abstract Introduction: Membranous nephropathy (MN) is one of the major causes of nephrotic syndrome. The complement system plays a key role in the pathophysiology of MN.
    Objectives: To identify the complement pathway possibly activated in MN cases and correlate the presence of C4d with more severe clinical and histological markers.
    Methods: Sixty nine cases from renal biopsy with membranous nephropathy were investigated. The presence of C1q was analyzed by direct immunofluorescence; and expression of C4d by immunohistochemistry. Clinical and epidemiological data were obtained upon biopsy request.
    Results: The presence of focal segmental glomerulosclerosis, global glomerulosclerosis, vascular lesions and tubulointerstitial fibrosis were collected by anatomopathological report. C4d(+) was found in 58 (84%), and C1q(+) was found in 12 (17%) of the cases. Twelve patients had C4d(+)/C1q(+), 46 had C4d(+)/C1q(-), and 11 patients had C4d(-)/C1q(-), probably indicating the activation of the classical, lectin and alternative pathways, respectively.
    Conclusion: C4d was associated with increased interstitial fibrosis, but not with clinical markers of poor prognosis. Through the deposition of C4d and C1q we demonstrated that all complement pathways may be involved in MN, highlighting the lectin pathway. The presence of C4d has been associated with severe tubulointerstitial lesions, but not with clinical markers, or can be taken as a universal marker of all cases of MN.
    MeSH term(s) Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biomarkers ; Complement Activation ; Complement C4b/biosynthesis ; Complement System Proteins/biosynthesis ; Female ; Glomerulonephritis, Membranous/immunology ; Humans ; Male ; Middle Aged ; Peptide Fragments/biosynthesis ; Young Adult
    Chemical Substances Biomarkers ; Peptide Fragments ; Complement C4b (80295-50-7) ; complement C4d (80295-52-9) ; Complement System Proteins (9007-36-7)
    Language Portuguese
    Publishing date 2018-02-01
    Publishing country Brazil
    Document type Journal Article
    ZDB-ID 2057873-8
    ISSN 2175-8239 ; 0101-2800
    ISSN (online) 2175-8239
    ISSN 0101-2800
    DOI 10.5935/0101-2800.20170068
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Extended hemodialysis in acute kidney injury.

    Custodio, Fabiano Bichuette / de Lima, Emerson Quintino

    Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia

    2013  Volume 35, Issue 2, Page(s) 142–146

    Abstract: About 10% of patients in the intensive care unit which develop acute renal failure will depend on renal replacement therapy. Although there are no data showing reduction in mortality when compared with intermittent therapy, continuous therapies provide ... ...

    Abstract About 10% of patients in the intensive care unit which develop acute renal failure will depend on renal replacement therapy. Although there are no data showing reduction in mortality when compared with intermittent therapy, continuous therapies provide higher cumulative doses of dialysis and greater hemodynamic stability. However, have high costs and are not available in many centers. In this context the Extended Hemodialysis gaining ground in clinical practice because it combines the hemodynamic tolerability, slow and sustained solute control and effective doses of continuous dialysis therapies associated with reduced costs and logistics facilities of intermittent therapy.
    MeSH term(s) Acute Kidney Injury/therapy ; Humans ; Renal Dialysis/methods
    Language Portuguese
    Publishing date 2013-04
    Publishing country Brazil
    Document type Journal Article
    ZDB-ID 2057873-8
    ISSN 2175-8239 ; 0101-2800
    ISSN (online) 2175-8239
    ISSN 0101-2800
    DOI 10.5935/0101-2800.20130023
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Focal and Segmental Glomerulosclerosis and Membranous Nephropathy overlapping in a patient with Nephrotic Syndrome: a case report.

    Silva, Crislaine Aparecida da / Custódio, Fabiano Bichuette / Monteiro, Maria Luíza Gonçalves Dos Reis / Araújo, Stanley de Almeida / Araújo, Liliane Silvano / Côrrea, Rosana Rosa Miranda / Reis, Marlene Antônia Dos / Machado, Juliana Reis

    Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia

    2019  Volume 42, Issue 1, Page(s) 113–117

    Abstract: Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary.: Case ... ...

    Abstract Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary.
    Case presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II.
    Conclusions: In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.
    MeSH term(s) Adolescent ; Angiotensin-Converting Enzyme Inhibitors/therapeutic use ; Biopsy ; Female ; Glomerulonephritis, Membranous/complications ; Glomerulonephritis, Membranous/diagnosis ; Glomerulonephritis, Membranous/drug therapy ; Glomerulonephritis, Membranous/pathology ; Glomerulosclerosis, Focal Segmental/complications ; Glomerulosclerosis, Focal Segmental/diagnosis ; Glomerulosclerosis, Focal Segmental/drug therapy ; Glomerulosclerosis, Focal Segmental/pathology ; Humans ; Kidney/pathology ; Nephrotic Syndrome/complications ; Nephrotic Syndrome/diagnosis ; Nephrotic Syndrome/drug therapy ; Treatment Outcome
    Chemical Substances Angiotensin-Converting Enzyme Inhibitors
    Language English
    Publishing date 2019-02-25
    Publishing country Brazil
    Document type Case Reports ; Journal Article
    ZDB-ID 2057873-8
    ISSN 2175-8239 ; 0101-2800
    ISSN (online) 2175-8239
    ISSN 0101-2800
    DOI 10.1590/2175-8239-jbn-2018-0239
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Ultrastructural deposits appearing as "zebra bodies" in renal biopsy: Fabry disease?- comparative case reports.

    de Menezes Neves, Precil Diego Miranda / Machado, Juliana Reis / Custódio, Fabiano Bichuette / Dos Reis Monteiro, Maria Luíza Gonçalves / Iwamoto, Shigueo / Freire, Marlene / Ferreira, Marisa França / Dos Reis, Marlene Antônia

    BMC nephrology

    2017  Volume 18, Issue 1, Page(s) 157

    Abstract: Background: Fabry Disease (FD) is a genetic disorder caused by alpha-galactosidase A deficiency. Certain drugs, such as hydroxychloroquine, can produce renal deposits that mimic morphological findings seen in FD, characterizing a type of drug-induced ... ...

    Abstract Background: Fabry Disease (FD) is a genetic disorder caused by alpha-galactosidase A deficiency. Certain drugs, such as hydroxychloroquine, can produce renal deposits that mimic morphological findings seen in FD, characterizing a type of drug-induced renal phospholipidosis.
    Case presentation: Case 1: A 28-year-old female patient with systemic lupus erythematosus who had been using hydroxychloroquine for 14 months presented subnephrotic proteinuria. Renal biopsy showed deposits compatible with FD. Neither activity analysis of alpha-galactosidase A nor genetic analysis were available and were not performed. These deposits were not detected in a subsequent renal biopsy three years after withdrawal of the medication, characterizing a possible hydroxychloroquine-induced renal phospholipidosis. Case 2: A 29-year-old male patient presented with acroparesthesia, angiokeratomas, cornea verticillata and subnephrotic proteinuria. Deposits compatible with FD were detected upon renal biopsy. The evaluation of alpha-galactosidase A showed no activity in both blood and leukocytes. Genetic analysis identified an M284 T mutation in exon 6, and such mutation was also found in other family members.
    Conclusion: Clinical investigation is necessary in suspected cases of Fabry Disease upon renal biopsy in order to confirm diagnosis. Drug-induced renal phospholipidosis should be considered in differential diagnosis in cases with intracellular osmiophilic, lamellar inclusions in electron microscopy.
    Language English
    Publishing date 2017-05-12
    Publishing country England
    Document type Journal Article
    ZDB-ID 2041348-8
    ISSN 1471-2369 ; 1471-2369
    ISSN (online) 1471-2369
    ISSN 1471-2369
    DOI 10.1186/s12882-017-0571-0
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Diagnosis of congenital and infantile nephrotic syndromes in renal biopsies in Minas Gerais, Brazil: Six case reports.

    Rocha, Laura Penna / Custódio, Fabiano Bichuette / Machado, Juliana Reis / de Morais Pereira, Lívia Helena / Monteiro, Maria Luiza Gonçalves Dos Reis / Laterza, Valéria Lima / Dos Reis, Marlene Antônia / Corrêa, Rosana Rosa Miranda

    Ultrastructural pathology

    2016  Volume 40, Issue 6, Page(s) 311–316

    Abstract: Congenital or infantile nephrotic syndromes (CNS/INS) correspond to a heterogeneous group of rare diseases in which glomerular renal dysfunction and proteinuria are prominent. The aim of this study is to present six cases of possible CNS/INS with ... ...

    Abstract Congenital or infantile nephrotic syndromes (CNS/INS) correspond to a heterogeneous group of rare diseases in which glomerular renal dysfunction and proteinuria are prominent. The aim of this study is to present six cases of possible CNS/INS with diagnoses based on clinical findings and especially histological, ultrastructural, and immunohistochemical characteristics of renal biopsies. Four cases are presented with diffuse mesangial sclerosis, one of them possibly part of Denys-Drash syndrome and two cases with CNS probably of the Finnish type in patients between 3 months old and 13 years old. The study focuses on the late evolution of Denys-Drash syndrome to end-stage renal disease in a 13-year-old patient and the diagnosis of diffuse mesangial sclerosis in an 8-year-old patient. Thus, it contributes to a better epidemiological characterization of these syndromes, demonstrating cases of CNS/INS in infrequent age groups.
    Language English
    Publishing date 2016-11
    Publishing country England
    Document type Journal Article
    ZDB-ID 603269-2
    ISSN 1521-0758 ; 0191-3123
    ISSN (online) 1521-0758
    ISSN 0191-3123
    DOI 10.1080/01913123.2016.1221868
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: A novel single amino acid deletion impairs fibronectin function and causes familial glomerulopathy with fibronectin deposits: case report of a family.

    Dos Reis Monteiro, Maria Luíza Gonçalves / Custódio, Fabiano Bichuette / de Menezes Neves, Precil Diego Miranda / Ferreira, Frederico Moraes / Watanabe, Elieser Hitoshi / Lerário, Antônio Marcondes / de Araújo, Liliane Silvano / Balbo, Bruno Eduardo Pedroso / Pinto, Vívian Christine Dourado / Barbosa, Lívia Maria Gruli / de Paiva Marques, Vilmar / Machado, Juliana Reis / Reis, Marlene Antônia / Onuchic, Luiz Fernando

    BMC nephrology

    2019  Volume 20, Issue 1, Page(s) 322

    Abstract: Background: Glomerulopathy with fibronectin deposits is an autosomal dominant disease associated with proteinuria, hematuria, hypertension and renal function decline. Forty percent of the cases are caused by mutations in FN1, the gene that encodes ... ...

    Abstract Background: Glomerulopathy with fibronectin deposits is an autosomal dominant disease associated with proteinuria, hematuria, hypertension and renal function decline. Forty percent of the cases are caused by mutations in FN1, the gene that encodes fibronectin.
    Case presentation: This report describes two cases of Glomerulopathy with fibronectin deposits, involving a 47-year-old father and a 14-year-old son. The renal biopsies showed glomeruli with endocapillary hypercellularity and large amounts of mesangial and subendothelial eosinophilic deposits. Immunohistochemistry for fibronectin was markedly positive. Whole exome sequencing identified a novel FN1 mutation that leads to an amino-acid deletion in both patients (Ile1988del), a variant that required primary amino-acid sequence analysis for assessment of pathogenicity. Our primary sequence analyses revealed that Ile1988 is very highly conserved among relative sequences and is positioned in a C-terminal FN3 domain containing heparin- and fibulin-1-binding sites. This mutation was predicted as deleterious and molecular mechanics simulations support that it can change the tertiary structure and affect the complex folding and its molecular functionality.
    Conclusion: The current report not only documents the occurrence of two GFND cases in an affected family and deeply characterizes its anatomopathological features but also identifies a novel pathogenic mutation in FN1, analyzes its structural and functional implications, and supports its pathogenicity.
    MeSH term(s) Adolescent ; Fibronectins/genetics ; Glomerulonephritis, Membranoproliferative/genetics ; Glomerulonephritis, Membranoproliferative/pathology ; Humans ; Kidney Glomerulus/pathology ; Male ; Middle Aged ; Mutation ; Pedigree ; Sequence Analysis, Protein
    Chemical Substances FN1 protein, human ; Fibronectins
    Language English
    Publishing date 2019-08-16
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 2041348-8
    ISSN 1471-2369 ; 1471-2369
    ISSN (online) 1471-2369
    ISSN 1471-2369
    DOI 10.1186/s12882-019-1507-7
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Clinical and epidemiological prevalence of glomerulopathies elderly in the city of Uberaba - MG.

    Oliveira, Lethícia Borges / Cobo, Eliangela de Castro / Machado, Juliana Reis / Custódio, Fabiano Bichuette / da Silva, Marcos Vinícius / de Oliveira, Flávia Aparecida / Correa, Rosana Rosa Miranda / dos Reis, Marlene Antônia

    Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia

    2015  Volume 37, Issue 2, Page(s) 166–170

    Abstract: Introduction: Currently, the elderly population of Brazil is suffering significant increase. Aging is a physiological process that causes changes in various organs, including the kidney. A kidney biopsy is of paramount importance to clarify the ... ...

    Abstract Introduction: Currently, the elderly population of Brazil is suffering significant increase. Aging is a physiological process that causes changes in various organs, including the kidney. A kidney biopsy is of paramount importance to clarify the morphological changes of these entities.
    Objectives: The aim of this work was to conduct a clinical epidemiological analysis of elderly patients and evaluate the prevalence of major glomerulopathies that affect.
    Methods: This is a retrospective and descriptive, with a review of 104 reports of renal biopsies of elderly aged over 60 years, performed in the Nefropatologia Federal University of Triângulo Mineiro (UFTM), between periods January 1996 and December 2010. Patients were grouped according to clinical syndrome.
    Results: We reviewed 104 biopsies of elderly patients. Of these, 52.94% were male. The Hypertension was found in 50.54% of patients. The clinical syndrome was the predominant nephrotic syndrome (42.17%). Most disease was glomerular origin. The glomerulopathy was the most prevalent (34.07%).
    Discussion/conclusion: Through this review, we noted that the nephrotic syndrome was the main clinical syndrome and Podocytophaties glomerulopathies were more prevalent in the group of elderly patients undergoing renal biopsy. The analysis of renal biopsies of elderly patients is of paramount importance, since knowledge of the clinical manifestations of major glomerulopathies that affect this group, to assist in establishing the diagnosis and therapeutic management.
    MeSH term(s) Aged ; Brazil/epidemiology ; Female ; Humans ; Kidney Diseases/epidemiology ; Kidney Glomerulus ; Male ; Prevalence ; Retrospective Studies ; Urban Health
    Language Portuguese
    Publishing date 2015-04
    Publishing country Brazil
    Document type Journal Article
    ZDB-ID 2057873-8
    ISSN 2175-8239 ; 0101-2800
    ISSN (online) 2175-8239
    ISSN 0101-2800
    DOI 10.5935/0101-2800.20150027
    Database MEDical Literature Analysis and Retrieval System OnLINE

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