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  1. Article ; Online: Autoimmunity in Infection-Related Glomerulonephritis.

    Choung, Hae Yoon Grace / Grewal, Rickinder

    Glomerular diseases

    2022  Volume 3, Issue 1, Page(s) 32–41

    Abstract: Introduction: Autoimmune (AI) reactivity in the setting of infection-related GN (IRGN) is often viewed as an epiphenomenon and is not well described.: Methods: We report a cohort of 17 patients with IRGN during a 7-year period that highlights cases ... ...

    Abstract Introduction: Autoimmune (AI) reactivity in the setting of infection-related GN (IRGN) is often viewed as an epiphenomenon and is not well described.
    Methods: We report a cohort of 17 patients with IRGN during a 7-year period that highlights cases with AI reactivity and describes the clinical and pathologic characteristics of IRGN cases associated with AI reactivity.
    Results: Of the IRGN cases, 76% had clinical evidence of an autoimmune disease (AD) and/or positive AI serologies. Within the IRGN group with AI reactivity, 12 had positive AI serologies (92%) and 10 had AD (77%). 30% had a prior diagnosis of AD, while the remaining 70% did not have a history of AD and were either diagnosed or suspected of having an AD at the time of biopsy. The most common autoantibody detected was anti-nuclear antibody followed by anti-neutrophil cytoplasmic antibodies and autoantibodies associated with antiphospholipid syndrome.
    Conclusion: The study is not sufficiently powered to determine any significance but demonstrates the frequency with which AI features occur in IRGN and should prompt further future investigation. In summary, our findings suggest AI manifestations are common in IRGN.
    Language English
    Publishing date 2022-12-21
    Publishing country Switzerland
    Document type Journal Article
    ISSN 2673-3633
    ISSN (online) 2673-3633
    DOI 10.1159/000528712
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  2. Article ; Online: Segmental membranous nephropathy.

    Choung, Hae Yoon Grace / Goldman, Bruce

    Clinical and experimental nephrology

    2021  Volume 25, Issue 7, Page(s) 700–707

    Abstract: Most cases of membranous nephropathy (MGN) present with global and diffuse distribution of subepithelial deposits. However, segmental MGN, in which there is focal or diffuse segmental subepithelial deposits, are occasionally encountered. The clinical and ...

    Abstract Most cases of membranous nephropathy (MGN) present with global and diffuse distribution of subepithelial deposits. However, segmental MGN, in which there is focal or diffuse segmental subepithelial deposits, are occasionally encountered. The clinical and pathologic significance of segmental MGN is not well understood and thought to be more likely due to either early or resolving phase of the global form of MGN. Several case reports and literature available suggest that it may be a manifestation of secondary causes based on pathologic features such as presence of C1q and mesangial deposits, extra-glomerular deposits involving tubular basement membranes, absence of PLA2R and THSD7A, IgG1 and IgG3 subclass dominance, and the presence of other co-existing renal disease. Other reports, however, suggest that some of these cases may be a variant of the primary form of MGN. In this review, we integrate what is known about segmental MGN in order to better direct interpretation of renal biopsies in which it is identified.
    MeSH term(s) Glomerulonephritis, Membranous/immunology ; Glomerulonephritis, Membranous/pathology ; Humans ; Immunoglobulin G ; Kidney/pathology
    Chemical Substances Immunoglobulin G
    Language English
    Publishing date 2021-03-23
    Publishing country Japan
    Document type Journal Article ; Review
    ZDB-ID 1338768-6
    ISSN 1437-7799 ; 1342-1751
    ISSN (online) 1437-7799
    ISSN 1342-1751
    DOI 10.1007/s10157-021-02056-1
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  3. Article ; Online: Subepithelial deposits with microspherular structures in membranous glomerulonephritis.

    Choung, Hae Yoon Grace / Jean-Gilles, Jerome / Goldman, Bruce

    Ultrastructural pathology

    2022  Volume 46, Issue 4, Page(s) 377–387

    Abstract: Rare cases of membranous glomerulopathy (MGN) with subepithelial deposits consisting of microspherular structures identified by electron microscopy have been described in the literature as either MGN with spherules or podocyte infolding glomerulopathy ( ... ...

    Abstract Rare cases of membranous glomerulopathy (MGN) with subepithelial deposits consisting of microspherular structures identified by electron microscopy have been described in the literature as either MGN with spherules or podocyte infolding glomerulopathy (PIG). The paucity of available studies shows a strong association with underlying autoimmune disease. To further understand the significance of subepithelial microspherular deposits, we retrospectively identified native kidney biopsies from 10 patients diagnosed as MGN with subepithelial microspherular structures identified by ultrastructural examination at the University of Rochester Medical Center (URMC) during an 11-year period. The majority were Caucasian (80%) with a mean age of 51.3 (±12.9) years. 50% had an autoimmune disorder, of which 80% were SLE. Two SLE cases had concomitant rheumatoid arthritis and Sjogren's syndrome. One additional case had antiphospholipid syndrome and showed lupus-like features on biopsy. 40% were idiopathic and negative for PLA2R, NELL1, and THSD7A. MGN with subepithelial microspherular structures is frequently associated with an underlying autoimmune disease. The majority are negative for markers of primary MGN (PLA2R, THSD7A, and NELL1) and show features suggestive of secondary MGN.
    MeSH term(s) Biopsy ; Glomerulonephritis, Membranous/diagnosis ; Glomerulonephritis, Membranous/pathology ; Humans ; Lupus Erythematosus, Systemic ; Microscopy, Electron ; Retrospective Studies
    Language English
    Publishing date 2022-06-16
    Publishing country England
    Document type Journal Article
    ZDB-ID 603269-2
    ISSN 1521-0758 ; 0191-3123
    ISSN (online) 1521-0758
    ISSN 0191-3123
    DOI 10.1080/01913123.2022.2090646
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  4. Article ; Online: Myeloid bodies is not an uncommon ultrastructural finding.

    Choung, Hae Yoon Grace / Jean-Gilles, Jerome / Goldman, Bruce

    Ultrastructural pathology

    2022  Volume 46, Issue 1, Page(s) 130–138

    Abstract: The presence of myeloid bodies (MBs) is classically associated with Fabry disease (FD). However, MBs are also identified in patients without clinical evidence of FD. We attempt to further understand the clinicopathologic significance of incidental MBs in ...

    Abstract The presence of myeloid bodies (MBs) is classically associated with Fabry disease (FD). However, MBs are also identified in patients without clinical evidence of FD. We attempt to further understand the clinicopathologic significance of incidental MBs in those without FD. Among the 4400 renal biopsies accessioned at the University of Rochester Medical Center from 2010 to 2021, we identified 32 cases showing MBs, 6 of which had FD. Medications were compared between a non-FG and a control-group of randomly selected cases without MBs (non-MBs). Both Fabry-group (FG) and non-Fabry-group (non-FG) were predominantly middle-aged (mean 48 years vs 56, respectively). Non-FG had slight female predominance (1:4), while all in FG were female. The majority of both non-FG and non-MBs cohort were on the same medications reported to cause phospholipidosis except sertraline and hydralazine (
    MeSH term(s) Diagnosis, Differential ; Fabry Disease/complications ; Fabry Disease/diagnosis ; Fabry Disease/drug therapy ; Fabry Disease/pathology ; Female ; Humans ; Kidney Diseases/drug therapy ; Kidney Diseases/etiology ; Kidney Diseases/pathology ; Male ; Middle Aged ; Podocytes/pathology ; Podocytes/ultrastructure
    Language English
    Publishing date 2022-02-01
    Publishing country England
    Document type Journal Article
    ZDB-ID 603269-2
    ISSN 1521-0758 ; 0191-3123
    ISSN (online) 1521-0758
    ISSN 0191-3123
    DOI 10.1080/01913123.2021.2022054
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  5. Article: A rare case of diffuse crescents in fibrillary glomerulonephritis
.

    Choung, Hae Yoon Grace / Grewal, Rickinder / Goldman, Bruce

    Clinical nephrology

    2021  Volume 97, Issue 1, Page(s) 53–56

    Abstract: Diffuse crescentic involvement in fibrillary glomerulonephritis (FGN) is very rare. We describe a case of FGN with diffuse crescents in a patient who presented with clinical findings concerning for rapidly progressive kidney failure and pathologic ... ...

    Abstract Diffuse crescentic involvement in fibrillary glomerulonephritis (FGN) is very rare. We describe a case of FGN with diffuse crescents in a patient who presented with clinical findings concerning for rapidly progressive kidney failure and pathologic findings suggestive of anti-glomerular basement membrane (GBM) disease. Serologies for anti-neutrophil cytoplasmic antibody (ANCA) and anti-GBM were negative. IgG subtyping showed IgG1 dominance, which has not been described in FGN. We present this unique case to emphasize the importance of considering FGN in biopsies showing diffuse crescentic glomerulonephritis with linear IgG staining of glomerular capillary walls, especially in the absence of other significant proliferative changes.
    MeSH term(s) Anti-Glomerular Basement Membrane Disease ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Glomerulonephritis/diagnosis ; Humans ; Kidney Glomerulus
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies
    Language English
    Publishing date 2021-10-07
    Publishing country Germany
    Document type Case Reports ; Journal Article
    ZDB-ID 185101-9
    ISSN 0301-0430
    ISSN 0301-0430
    DOI 10.5414/CN110653
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    Zs.A 873: Show issues Location:
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  6. Article ; Online: Light chain deposition disease masquerading as smoking-associated nodular glomerulosclerosis with deposits identified by electron microscopy only.

    Alom, Md Saiful / Grewal, Rickinder / Passero, Frank C / Goldman, Bruce / Choung, Hae Yoon Grace

    CEN case reports

    2022  Volume 11, Issue 4, Page(s) 448–452

    Abstract: Light chain deposition disease (LCDD) is a form of monoclonal gammopathy of renal significance. The diagnosis is based on the immunofluorescence (IF) findings of linear monoclonal light chain staining of basement membranes throughout the kidney, which ... ...

    Abstract Light chain deposition disease (LCDD) is a form of monoclonal gammopathy of renal significance. The diagnosis is based on the immunofluorescence (IF) findings of linear monoclonal light chain staining of basement membranes throughout the kidney, which appear as non-organized, granular punctate to powdery electron dense deposits by electron microscopy (EM). Although "LCDD by IF only" without EM deposits has been well-described, LCDD identified by EM with negative IF is very rare and hardly mentioned in the literature. Herein we describe a case of lambda-type LCDD that appeared negative by IF and showed light microscopic findings of nodular glomerulosclerosis, which was initially attributed to the patient's history of significant tobacco use and uncontrolled hypertension. However, EM later showed powdery electron dense material in focal glomerular and tubular basement membranes and mesangium. Subsequent bone marrow analysis revealed greater than 60% lambda-restricted plasma cells. We report this case to illustrate that within the differential diagnosis of nodular sclerosis, monoclonal immunoglobulin deposition disease (MIDD) should remain in the differential even if immunofluorescence appears negative as EM can prove to be crucial in identifying cases of MIDD.
    MeSH term(s) Humans ; Diabetic Nephropathies/complications ; Immunoglobulin Light Chains ; Paraproteinemias/diagnosis ; Multiple Myeloma/complications ; Microscopy, Electron ; Smoking
    Chemical Substances Immunoglobulin Light Chains
    Language English
    Publishing date 2022-03-22
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 2660492-9
    ISSN 2192-4449 ; 2192-4449
    ISSN (online) 2192-4449
    ISSN 2192-4449
    DOI 10.1007/s13730-022-00698-y
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  7. Article ; Online: The Clinicopathologic Spectrum of Membranous Nephropathy with Lupus-Like Features.

    Choung, Hae Yoon Grace / Moore, Catherine / Le, Thu H / Guirguis, Paul / McKeown, Jack M / Jean-Gilles, Jerome / Goldman, Bruce

    Nephron

    2023  Volume 147, Issue 7, Page(s) 424–433

    Abstract: Introduction: The pathologic features of membranous lupus nephritis (MLN) are occasionally encountered in secondary membranous nephropathy (sMN) without overt clinical evidence of systemic lupus erythematosus. Moreover, some sMN with lupus-like features ...

    Abstract Introduction: The pathologic features of membranous lupus nephritis (MLN) are occasionally encountered in secondary membranous nephropathy (sMN) without overt clinical evidence of systemic lupus erythematosus. Moreover, some sMN with lupus-like features (lupus-like membranous nephropathy [LL-MN]) have a clinical presentation more typical of primary membranous nephropathy (pMN). Based on the confounding clinical and pathologic presentation, it is unclear how to categorize and treat these patients.
    Methods: We performed immunohistochemical staining for recently discovered target antigens associated with MN -NELL-1, THSD7A, and EXT1/2 and compared the clinicopathologic presentation of patients with LL-MN to those with pMN and MLN.
    Results: From 2015 to 2020, there were 21 patients with MLN and 99 with MN, of which 59% were diagnosed pMN and 41% sMN. 44% of sMN patients showed lupus-like features (LL-fx). All LL-MN patients were negative for PLA2R and NELL1, but 12% were positive for EXT1/2. 50% of LL-MN patients had an identifiable systemic disease, of which 56% were autoimmune disease (AD) and 44% infection. Compared to pMN, LL-MN had a higher incidence of underlying AD (p = 0.02). Within pMN, 24% also had LL-fx (LL-pMN), and all but 1 were PLA2R- (78%) or NELL1-positive (15%). Only 5% of pMN patients had an AD, 66% of which showed LL-fx. Most idiopathic LL-MN were treated and behaved clinically similarly to pMN. There were no differences in outcome in terms of progression toward end-stage renal disease or mortality between LL-MN versus pMN and MLN.
    Conclusion: LL-MN appears to have a significant association with underlying AD and has a subset showing EXT1/2 positivity, whereas most LL-pMN and idiopathic LL-MN likely represent an atypical pathologic presentation of pMN.
    MeSH term(s) Humans ; Glomerulonephritis, Membranous/pathology ; Lupus Nephritis/complications
    Language English
    Publishing date 2023-02-06
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 207121-6
    ISSN 2235-3186 ; 1423-0186 ; 1660-8151 ; 0028-2766
    ISSN (online) 2235-3186 ; 1423-0186
    ISSN 1660-8151 ; 0028-2766
    DOI 10.1159/000529437
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    Zs.A 169: Show issues Location:
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  8. Article ; Online: The spectrum of renal diseases with lupus-like features: a single-center study.

    Ahmed, Maliha / Love, Tanzy / Moore, Catherine / Le, Thu H / Jean-Gilles, Jerome / Goldman, Bruce / Choung, Hae Yoon Grace

    Renal failure

    2022  Volume 44, Issue 1, Page(s) 581–593

    Abstract: Background: A subset of patients without overt systemic lupus erythematosus (SLE) present with biopsy findings typically seen in lupus nephritis (LN). Although a minority eventually develops SLE, many do not. It remains unclear how to classify or treat ... ...

    Abstract Background: A subset of patients without overt systemic lupus erythematosus (SLE) present with biopsy findings typically seen in lupus nephritis (LN). Although a minority eventually develops SLE, many do not. It remains unclear how to classify or treat these patients. Our study attempted to further understand the clinical and pathological characteristics of cases with lupus-like nephritis (LLN).
    Methods: Among 2700 native kidney biopsies interpreted at University of Rochester Medical Center (URMC) from 2010 to 2019, we identified 27 patients with biopsies showing lupus-like features (LL-fx) and 96 with LN. Of those with LL-fx, 17 were idiopathic LLN and 10 were associated with a secondary etiology (e.g., infection/drugs).
    Results: At the time of biopsy, the LLN-group tended to be slightly older (44
    Conclusions: Lupus-like pathologic features are seen in a wide array of disease processes. The findings suggest that LLN may be a manifestation of an autoimmune process that overlaps with SLE.
    MeSH term(s) Biopsy ; Humans ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/epidemiology ; Lupus Nephritis/complications ; Male ; Proteinuria/complications ; Renal Insufficiency, Chronic/complications
    Language English
    Publishing date 2022-03-31
    Publishing country England
    Document type Journal Article
    ZDB-ID 632949-4
    ISSN 1525-6049 ; 0886-022X
    ISSN (online) 1525-6049
    ISSN 0886-022X
    DOI 10.1080/0886022X.2022.2057862
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    Zs.A 1331: Show issues Location:
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  9. Article ; Online: Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) in a young female athlete at 36 weeks gestation: a case report.

    Choung, Hae Yoon Grace / Vyas, Monika / Jacoby, Daniel / West, Brian

    Pathology, research and practice

    2017  Volume 213, Issue 10, Page(s) 1302–1305

    Abstract: A 26year old east African professional athlete presented to the obstetric clinic for a routine visit at 36 weeks gestation. She had a history of Right Ventricular Outflow Tract - Ventricular Tachycardia (RVOT-VT) with an episode of cardiac arrest in the ... ...

    Abstract A 26year old east African professional athlete presented to the obstetric clinic for a routine visit at 36 weeks gestation. She had a history of Right Ventricular Outflow Tract - Ventricular Tachycardia (RVOT-VT) with an episode of cardiac arrest in the past, and had been treated with ablation 4 years earlier. Her current visit was uneventful, her pregnancy progressing normally. Following the visit she went to a local restaurant where she suffered a cardiac arrest that was unresponsive to therapy. Chest compressions were continued from the time of her collapse until an emergency caesarian section was performed, delivering a healthy female infant. At autopsy a focal area of subtle pallor and myocardial thinning was present at the apex of the right ventricle. Histology showed myocyte degeneration and loss with focal full thickness replacement of myocardium by adipose tissue, consistent with the fatty form of arrhythmogenic right ventricular cardiomyopathy (ARVC). Molecular studies revealed a variant of unknown significance in the MYBPC3 gene, but no variant known to be associated with ARVC. In view of the subtlety of the lesion on gross examination this diagnosis could have been easily missed, emphasizing the importance of performing histologic examination of subtle gross cardiac lesions.
    MeSH term(s) Adult ; Arrhythmogenic Right Ventricular Dysplasia/complications ; Arrhythmogenic Right Ventricular Dysplasia/genetics ; Arrhythmogenic Right Ventricular Dysplasia/pathology ; Athletes ; Autopsy ; Biopsy ; Carrier Proteins/genetics ; DNA Mutational Analysis ; Death, Sudden, Cardiac/etiology ; Death, Sudden, Cardiac/pathology ; Fatal Outcome ; Female ; Genetic Predisposition to Disease ; Gestational Age ; Humans ; Mutation ; Myocardium/pathology ; Phenotype ; Pregnancy
    Chemical Substances Carrier Proteins ; myosin-binding protein C
    Language English
    Publishing date 2017-07-25
    Publishing country Germany
    Document type Case Reports ; Journal Article
    ZDB-ID 391889-0
    ISSN 1618-0631 ; 0344-0338
    ISSN (online) 1618-0631
    ISSN 0344-0338
    DOI 10.1016/j.prp.2017.07.015
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  10. Article ; Online: Glomerulopathy in adult recipients of pediatric kidneys.

    Choung, Hae Yoon Grace / Meleg-Smith, Suzanne

    Ultrastructural pathology

    2014  Volume 38, Issue 2, Page(s) 141–149

    Abstract: To compare "for cause" renal biopsies (bx) from adult recipients of pediatric-donor kidneys (PDK) versus adult-donor kidneys (ADKs), we reviewed 103 graft bx from 50 PDK recipients and 85 bx from 49 ADK recipients. PDK bx displayed more frequent ... ...

    Abstract To compare "for cause" renal biopsies (bx) from adult recipients of pediatric-donor kidneys (PDK) versus adult-donor kidneys (ADKs), we reviewed 103 graft bx from 50 PDK recipients and 85 bx from 49 ADK recipients. PDK bx displayed more frequent glomerular pathology with immune complex-mediated glomerulonephritis present in 11/103 PDK versus 1/85 ADK (p < 0.05). In 15/103 PDK bx and 1/85 ADK (p = 0.001), the association of glomerular sclerosis, expanded mesangium, and halo of prominent podocytes by light microscopy, and ultrastructural glomerular basement membrane lamellation, configured a characteristic glomerulopathy.
    MeSH term(s) Adult ; Age Factors ; Allografts ; Child ; Humans ; Kidney Diseases/epidemiology ; Kidney Diseases/therapy ; Kidney Transplantation ; Retrospective Studies ; Tissue Donors
    Language English
    Publishing date 2014-04
    Publishing country England
    Document type Journal Article
    ZDB-ID 603269-2
    ISSN 1521-0758 ; 0191-3123
    ISSN (online) 1521-0758
    ISSN 0191-3123
    DOI 10.3109/01913123.2014.888112
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