LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 43

Search options

  1. Article ; Online: Diagnostic Challenges and Emerging Pathogeneses of Selected Glomerulopathies.

    Andeen, Nicole K / Hou, Jean

    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

    2024  , Page(s) 10935266241237656

    Abstract: Recent progress in glomerular immune complex and complement-mediated diseases have refined diagnostic categories and informed mechanistic understanding of disease development in pediatric patients. Herein, we discuss selected advances in 3 categories. ... ...

    Abstract Recent progress in glomerular immune complex and complement-mediated diseases have refined diagnostic categories and informed mechanistic understanding of disease development in pediatric patients. Herein, we discuss selected advances in 3 categories. First, membranous nephropathy antigens are increasingly utilized to characterize disease in pediatric patients and include phospholipase A2 receptor (PLA2R), Semaphorin 3B (Sema3B), neural epidermal growth factor-like 1 (NELL1), and protocadherin FAT1, as well as the lupus membranous-associated antigens exostosin 1/2 (EXT1/2), neural cell adhesion molecule 1 (NCAM1), and transforming growth factor beta receptor 3 (TGFBR3). Second, we examine advances in techniques for paraffin and light chain immunofluorescence (IF), including the former's function as a salvage technique and their necessity for diagnosis in adolescent cases of membranous-like glomerulopathy with masked IgG kappa deposits (MGMID) and proliferative glomerulonephritis with monotypic Ig deposits (PGNMID), respectively. Finally, progress in understanding the roles of complement in pediatric glomerular disease is reviewed, with specific attention to overlapping clinical, histologic, and genetic or functional alternative complement pathway (AP) abnormalities among C3 glomerulopathy (C3G), infection-related and post-infectious GN, "atypical" post-infectious GN, immune complex mediated membranoproliferative glomerulonephritis (IC-MPGN), and atypical hemolytic uremic syndrome (aHUS).
    Language English
    Publishing date 2024-04-05
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1463498-3
    ISSN 1615-5742 ; 1093-5266
    ISSN (online) 1615-5742
    ISSN 1093-5266
    DOI 10.1177/10935266241237656
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 5063: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  2. Article ; Online: Fabry Disease Presenting as End-Stage Kidney Disease.

    Pahl, Madeleine V / Hou, Jean

    Glomerular diseases

    2023  Volume 3, Issue 1, Page(s) 189–196

    Abstract: Background: Fabry disease (FD) is an X-linked disorder due to a pathogenic variant of the : Summary: A 40-year-old man with a history of seizures presented with increased serum creatinine, nephrotic rage proteinuria, and new-onset hypertension. A ... ...

    Abstract Background: Fabry disease (FD) is an X-linked disorder due to a pathogenic variant of the
    Summary: A 40-year-old man with a history of seizures presented with increased serum creatinine, nephrotic rage proteinuria, and new-onset hypertension. A renal biopsy revealed numerous, whorled, and lamellated cytoplasmic inclusions in podocytes, glomerular peritubular capillary endothelial cells, mesangial cells, arterial myocytes, and interstitial macrophages. Ultrastructural analysis confirmed the presence of glycosphingolipid inclusions and enlarged lysosomes packed with multi-lamellated structures ("zebra" bodies). The findings were suggestive of a lysosomal storage disorder, and testing for alpha-galactosidase A levels revealed near-absent enzyme activity, confirming the diagnosis of advanced FD.
    Key messages: The diagnosis of FD can be challenging as the manifestations of the disease are nonspecific, and patients can present early with classical symptoms or late with non-classical patterns of involvement. We will discuss strategies to identify the disorder early by reviewing the classical and non-classical presentations and further outline currently available and potential future treatment options.
    Language English
    Publishing date 2023-08-16
    Publishing country Switzerland
    Document type Case Reports
    ISSN 2673-3633
    ISSN (online) 2673-3633
    DOI 10.1159/000533502
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

    Inter-library loan at ZB MED

    Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

  3. Article ; Online: Characterization of Membranous Nephropathy with Microspherular Deposits.

    Ren, Kevin Yi Mi / Hou, Jean

    Glomerular diseases

    2023  Volume 3, Issue 1, Page(s) 197–210

    Abstract: Introduction: Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome in the USA. The typical ultrastructural finding is of global uniformly dense subepithelial electron-dense immune complex deposits along glomerular basement membranes. ...

    Abstract Introduction: Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome in the USA. The typical ultrastructural finding is of global uniformly dense subepithelial electron-dense immune complex deposits along glomerular basement membranes. However, early reports described deposits with a unique microspherular substructure. There was variability in what was identified as microspherular, sometimes overlapping with other entities such as podocyte infolding glomerulopathy. Currently, the nature, composition, and clinical significance of these microspherular deposits (MSDs) remain unknown.
    Method: We report the clinicopathologic features of a series of MN cases with MSD, with detailed ultrastructural characterization as well as PLA2R and THSD7A immunohistochemical and IgG subclass-staining characteristics. The proportion of MSD to overall deposits is segregated into two groups: global MSD with >50% MSD (
    Results: The size and appearance of the microspherules were nearly identical in global and segmental MSD groups (mean diameter of 77.9 nm and 77.2 nm, respectively), with subepithelial (
    Conclusion: The findings suggest that MSDs are more commonly associated with secondary MN. This case series is the largest to date, and the findings may yield etiologic and prognostic information on this rare but unique subset of MN and provide a well-characterized cohort of cases for future studies.
    Language English
    Publishing date 2023-08-20
    Publishing country Switzerland
    Document type Journal Article
    ISSN 2673-3633
    ISSN (online) 2673-3633
    DOI 10.1159/000529700
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

    Inter-library loan at ZB MED

    Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

  4. Article ; Online: C3 Glomerulopathy: A Review with Emphasis on Ultrastructural Features.

    Hou, Jean / Ren, Kevin Yi Mi / Haas, Mark

    Glomerular diseases

    2022  Volume 2, Issue 3, Page(s) 107–120

    Abstract: C3 glomerulopathy (C3G) is a rare disease resulting from dysregulation of the alternative complement pathway, resulting in the deposition of complement component 3 (C3) in the kidney. It encompasses two major subgroups: dense deposit disease and C3 ... ...

    Abstract C3 glomerulopathy (C3G) is a rare disease resulting from dysregulation of the alternative complement pathway, resulting in the deposition of complement component 3 (C3) in the kidney. It encompasses two major subgroups: dense deposit disease and C3 glomerulonephritis (C3GN). Although the alternative complement pathway is typically a very tightly controlled system, dysregulation can be a result of genetic mutations in the fluid phase or membrane-bound inhibitors or accelerators. In addition, de novo/acquired autoantibodies against any of the regulatory proteins can alter complement activation either by negating an inhibitor or activating an accelerator. Triggering events can be complex; however, the final pathway is characterized by the uncontrolled deposition of C3 in glomeruli and the formation of the membrane attack complex. Light microscopic findings can be quite heterogeneous with a membranoproliferative pattern most commonly encountered. Diagnostic confirmation of C3G is based on a characteristic pattern of glomerular immunofluorescence staining, with C3-dominant deposits that are at least 2 orders of intensity greater than staining for any immunoglobulin (Ig) or C1q. Electron microscopy is necessary for diagnosing DDD in particular, but can also help to distinguish C3GN from other glomerular disease mimickers.
    Language English
    Publishing date 2022-04-12
    Publishing country Switzerland
    Document type Journal Article ; Review
    ISSN 2673-3633
    ISSN (online) 2673-3633
    DOI 10.1159/000524552
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

    Inter-library loan at ZB MED

    Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

  5. Article ; Online: Artificial Intelligence: The Next Frontier in Kidney Biopsy Evaluation.

    Hou, Jean / Nast, Cynthia C

    Clinical journal of the American Society of Nephrology : CJASN

    2020  Volume 15, Issue 10, Page(s) 1389–1391

    MeSH term(s) Artificial Intelligence ; Biopsy ; Fluorescent Antibody Technique, Direct ; Humans ; Kidney ; Nephrology ; Neural Networks, Computer
    Keywords covid19
    Language English
    Publishing date 2020-09-16
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 2226665-3
    ISSN 1555-905X ; 1555-9041
    ISSN (online) 1555-905X
    ISSN 1555-9041
    DOI 10.2215/CJN.13450820
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 6584: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  6. Article ; Online: Changing concepts of HIV infection and renal disease.

    Hou, Jean / Nast, Cynthia C

    Current opinion in nephrology and hypertension

    2018  Volume 27, Issue 3, Page(s) 144–152

    Abstract: Purpose of review: Human immunodeficiency virus (HIV)-associated nephropathy (HIVAN) was identified as the major renal manifestation of HIV infection early in the HIV epidemic. However, HIV infection now is associated with a different spectrum of renal ... ...

    Abstract Purpose of review: Human immunodeficiency virus (HIV)-associated nephropathy (HIVAN) was identified as the major renal manifestation of HIV infection early in the HIV epidemic. However, HIV infection now is associated with a different spectrum of renal lesions leading to chronic kidney disease. This review examines the changes in kidney injury occurring in the current HIV era and the factors involved in this transformation of disease expression.
    Recent findings: The incidence of HIVAN and opportunistic infections in HIV-infected individuals has declined in concert with the use of effective combination antiretroviral agents. Chronic kidney disease has become more prevalent as patients infected with HIV are living longer and developing non-HIV-associated diseases such as hypertension and diabetes. Additionally, noncollapsing focal and segmental glomerulosclerosis, co-infection with hepatitis C, HIV-associated immune complex kidney disease, HIV-related accelerated aging, and antiretroviral therapies contribute to progressive loss of renal function.
    Summary: HIV infection is now associated with a variety of renal lesions causing chronic kidney disease, not all of which are virally induced. It is important to determine the cause of renal functional decline in an HIV-infected patient, as this will impact patient management and prognosis.
    MeSH term(s) AIDS-Associated Nephropathy/complications ; AIDS-Associated Nephropathy/drug therapy ; AIDS-Associated Nephropathy/epidemiology ; Anti-HIV Agents/adverse effects ; Anti-HIV Agents/therapeutic use ; Comorbidity ; Diabetes Mellitus/epidemiology ; Hepatitis C/epidemiology ; Humans ; Hypertension/epidemiology ; Incidence ; Prevalence ; Renal Insufficiency, Chronic/epidemiology ; Renal Insufficiency, Chronic/etiology ; Risk Factors
    Chemical Substances Anti-HIV Agents
    Language English
    Publishing date 2018-02-02
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1151092-4
    ISSN 1473-6543 ; 1535-3842 ; 1062-4813 ; 1062-4821
    ISSN (online) 1473-6543 ; 1535-3842
    ISSN 1062-4813 ; 1062-4821
    DOI 10.1097/MNH.0000000000000400
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 3686: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  8. Article ; Online: Alpha-mannosidosis: a case with novel ultrastructural and light microscopy findings.

    Leong, Matthew / Sathi, Bindu / Davis, Amy / Hamid, Syed / Wu, Sandy / Woods, Jeremy / Kharbanda, Sandhya / Li, Xiaomo / Hou, Jean

    Journal of pediatric endocrinology & metabolism : JPEM

    2023  Volume 36, Issue 12, Page(s) 1186–1190

    Abstract: Objectives: Alpha-mannosidosis is a rare genetic lysosomal storage condition leading to the systemic buildup of oligomannoside. Clinical presentation and associated conditions, as well as the full extent of histopathologic changes associated with this ... ...

    Abstract Objectives: Alpha-mannosidosis is a rare genetic lysosomal storage condition leading to the systemic buildup of oligomannoside. Clinical presentation and associated conditions, as well as the full extent of histopathologic changes associated with this disease process, are not fully understood.
    Case presentation: We present the case of an 8-year-1-month old patient with persistent anemia and who was initially diagnosed with Celiac disease before ultimately being diagnosed with alpha-mannosidosis. As part of his diagnostic work-up, duodenal and bone marrow biopsies were examined by pathology. Duodenal biopsies showed foamy plasma cells expanding the lamina propria which triggered a workup for a genetic storage disease; features suggestive of Celiac disease which resolved on gluten-free diet were also noted by pathology. Bone marrow analysis via electron microscopy showed cytoplasmic granules and inclusions in multiple immune cell lines.
    Conclusions: Alpha-mannosidosis can occur with Celiac disease and milder forms may only be suspected from incidental pathology findings. The ultrastructural bone marrow findings from this case, the first to be reported from human, show numerous disease-associated changes in multiple immune cell lines whose contribution to disease-associated immunodeficiency is unclear.
    MeSH term(s) Humans ; Infant ; alpha-Mannosidosis/diagnosis ; alpha-Mannosidosis/complications ; alpha-Mannosidosis/genetics ; Microscopy ; Celiac Disease/complications ; Lysosomal Storage Diseases/diagnosis
    Language English
    Publishing date 2023-11-20
    Publishing country Germany
    Document type Case Reports
    ZDB-ID 1231070-0
    ISSN 2191-0251 ; 0334-018X
    ISSN (online) 2191-0251
    ISSN 0334-018X
    DOI 10.1515/jpem-2023-0357
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 2258: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  9. Article ; Online: Temporal Trends in the Epidemiology of Biopsy-Proven Glomerular Diseases: An Alarming Increase in Diabetic Glomerulosclerosis.

    Hou, Jean / Haas, Mark

    Clinical journal of the American Society of Nephrology : CJASN

    2017  Volume 12, Issue 4, Page(s) 556–558

    MeSH term(s) Biopsy ; Diabetic Nephropathies ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney Glomerulus
    Language English
    Publishing date 2017-03-21
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 2226665-3
    ISSN 1555-905X ; 1555-9041
    ISSN (online) 1555-905X
    ISSN 1555-9041
    DOI 10.2215/CJN.02190217
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 6584: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  10. Article ; Online: Seronegative Atypical Anti-Glomerular Basement Membrane Glomerulonephritis Associated With Thrombotic Microangiopathy: Case Report and Literature Analysis.

    Roy, Sasmit / Hou, Jean / Chourasia, Prabal / Yalamanchili, Anish / Basuli, Debargha / Errabelli, Praveen Kumar / Sai Yarram, Samanvitha / Ayala, Raul / Adapa, Sreedhar

    Journal of investigative medicine high impact case reports

    2023  Volume 11, Page(s) 23247096231184760

    Abstract: Anti-glomerular basement membrane (GBM) antibody nephritis is defined by linear immunofluorescence staining of GBM by immunoglobulin G (IgG), typically associated with GBM rupture, fibrinoid necrosis, and crescent formation. Clinically, the patients ... ...

    Abstract Anti-glomerular basement membrane (GBM) antibody nephritis is defined by linear immunofluorescence staining of GBM by immunoglobulin G (IgG), typically associated with GBM rupture, fibrinoid necrosis, and crescent formation. Clinically, the patients present with rapidly worsening renal function, often with hematuria. Typical renal pathologic findings include necrotizing and crescentic glomerulonephritis. In contrast, thrombotic microangiopathy (TMA) is characterized by microvascular thrombosis, which can also lead to acute kidney injury. Thrombotic microangiopathy is associated with some systemic diseases and has characteristic clinical features of microangiopathic hemolytic anemia, platelet consumption, and multiple organ failure. Anti-GBM nephritis associated with TMA has rarely been reported. We describe an unusual case of atypical anti-GBM disease without crescent formation or necrosis but with light microscopic and ultrastructural features consistent with endothelial cell injury and glomerular-limited TMA.
    MeSH term(s) Humans ; Glomerulonephritis/complications ; Glomerulonephritis/pathology ; Nephritis ; Purpura, Thrombotic Thrombocytopenic ; Necrosis ; Basement Membrane/pathology
    Language English
    Publishing date 2023-07-07
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2710326-2
    ISSN 2324-7096 ; 2324-7096
    ISSN (online) 2324-7096
    ISSN 2324-7096
    DOI 10.1177/23247096231184760
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

To top