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  1. Article ; Online: Paraneoplastic pemphigus caused by pre-existing stroma-rich variant of Castleman disease: from a pathologist's point of view.

    Gwiti, Paida / Melegh, Zsombor / Johnston, Sarah / Sutak, Judit

    BMJ case reports

    2021  Volume 14, Issue 5

    Abstract: A young woman presented with mucocutaneous blisters and ulcerating lesions, and was diagnosed with erythrodermic pemphigus complicated by bronchiolitis obliterans. Her clinical condition did not improve on immunosuppressive therapy. She had a history of ... ...

    Abstract A young woman presented with mucocutaneous blisters and ulcerating lesions, and was diagnosed with erythrodermic pemphigus complicated by bronchiolitis obliterans. Her clinical condition did not improve on immunosuppressive therapy. She had a history of an asymptomatic retroperitoneal mass, presumed to be a dermoid cyst, followed up clinically. Due to the pre-existing nature of the retroperitoneal mass, the paraneoplastic nature of the pemphigus was initially not recognised, but after a multidisciplinary team meeting a biopsy was performed. Histology revealed a rare stroma-rich variant of Castleman disease with a prominent stroma demonstrating a myoid phenotype. Resection of the retroperitoneal tumour resulted in resolution of the cutaneous blisters. This emphasises the importance to consider paraneoplastic disease in treatment-resistant pemphigus as surgical removal of the tumour forms the mainstay of therapy. The differential diagnosis should include Castleman disease and careful evaluation of histology is essential with the awareness of this rare stroma-rich variant.
    MeSH term(s) Bronchiolitis Obliterans ; Castleman Disease/complications ; Castleman Disease/diagnosis ; Female ; Humans ; Paraneoplastic Syndromes/diagnosis ; Paraneoplastic Syndromes/etiology ; Pathologists ; Pemphigus
    Language English
    Publishing date 2021-05-11
    Publishing country England
    Document type Journal Article ; Case Reports
    ISSN 1757-790X
    ISSN (online) 1757-790X
    DOI 10.1136/bcr-2020-241374
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Two cases of rare HHV8-driven intravascular lymphoma with synchronous Kaposi sarcoma, both diagnosed at autopsy in renal transplant recipients.

    Gwiti, Paida / Jenkins, Megan / Sutak, Judit / Melegh, Zsombor

    Autopsy & case reports

    2020  Volume 10, Issue 4, Page(s) e2020206

    Abstract: We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 ... ...

    Abstract We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.
    Language English
    Publishing date 2020-11-20
    Publishing country Brazil
    Document type Case Reports
    ZDB-ID 2815488-5
    ISSN 2236-1960
    ISSN 2236-1960
    DOI 10.4322/acr.2020.206
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: BK virus nephropathy without haemorrhagic cystitis following bone marrow transplantation.

    Ghinai, Rosanna / Sutak, Judit / Saleem, Moin / Moppett, John

    British journal of haematology

    2019  Volume 188, Issue 2, Page(s) 200

    MeSH term(s) BK Virus/pathogenicity ; Bone Marrow Transplantation/adverse effects ; Bone Marrow Transplantation/methods ; Child, Preschool ; Glomerulonephritis, IGA/etiology ; Glomerulonephritis, IGA/virology ; Humans ; Male ; Transplantation Conditioning/adverse effects ; Transplantation Conditioning/methods
    Language English
    Publishing date 2019-10-16
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 80077-6
    ISSN 1365-2141 ; 0007-1048
    ISSN (online) 1365-2141
    ISSN 0007-1048
    DOI 10.1111/bjh.16234
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Mineralocorticoid receptor antagonism in diabetes reduces albuminuria by preserving the glomerular endothelial glycocalyx.

    Crompton, Michael / Ferguson, Joanne K / Ramnath, Raina D / Onions, Karen L / Ogier, Anna S / Gamez, Monica / Down, Colin J / Skinner, Laura / Wong, Kitty H / Dixon, Lauren K / Sutak, Judit / Harper, Steven J / Pontrelli, Paola / Gesualdo, Loreto / Heerspink, Hiddo L / Toto, Robert D / Welsh, Gavin I / Foster, Rebecca R / Satchell, Simon C /
    Butler, Matthew J

    JCI insight

    2023  Volume 8, Issue 5

    Abstract: The glomerular endothelial glycocalyx (GEnGlx) forms the first part of the glomerular filtration barrier. Previously, we showed that mineralocorticoid receptor (MR) activation caused GEnGlx damage and albuminuria. In this study, we investigated whether ... ...

    Abstract The glomerular endothelial glycocalyx (GEnGlx) forms the first part of the glomerular filtration barrier. Previously, we showed that mineralocorticoid receptor (MR) activation caused GEnGlx damage and albuminuria. In this study, we investigated whether MR antagonism could limit albuminuria in diabetes and studied the site of action. Streptozotocin-induced diabetic Wistar rats developed albuminuria, increased glomerular albumin permeability (Ps'alb), and increased glomerular matrix metalloproteinase (MMP) activity with corresponding GEnGlx loss. MR antagonism prevented albuminuria progression, restored Ps'alb, preserved GEnGlx, and reduced MMP activity. Enzymatic degradation of the GEnGlx negated the benefits of MR antagonism, confirming their dependence on GEnGlx integrity. Exposing human glomerular endothelial cells (GEnC) to diabetic conditions in vitro increased MMPs and caused glycocalyx damage. Amelioration of these effects confirmed a direct effect of MR antagonism on GEnC. To confirm relevance to human disease, we used a potentially novel confocal imaging method to show loss of GEnGlx in renal biopsy specimens from patients with diabetic nephropathy (DN). In addition, patients with DN randomized to receive an MR antagonist had reduced urinary MMP2 activity and albuminuria compared with placebo and baseline levels. Taken together, our work suggests that MR antagonists reduce MMP activity and thereby preserve GEnGlx, resulting in reduced glomerular permeability and albuminuria in diabetes.
    MeSH term(s) Rats ; Animals ; Humans ; Mineralocorticoid Receptor Antagonists/pharmacology ; Mineralocorticoid Receptor Antagonists/therapeutic use ; Mineralocorticoid Receptor Antagonists/metabolism ; Albuminuria/drug therapy ; Endothelial Cells/metabolism ; Receptors, Mineralocorticoid/metabolism ; Receptors, Mineralocorticoid/therapeutic use ; Glycocalyx/metabolism ; Rats, Wistar ; Diabetic Nephropathies/metabolism ; Diabetes Mellitus/metabolism
    Chemical Substances Mineralocorticoid Receptor Antagonists ; Receptors, Mineralocorticoid
    Language English
    Publishing date 2023-03-08
    Publishing country United States
    Document type Randomized Controlled Trial ; Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 2379-3708
    ISSN (online) 2379-3708
    DOI 10.1172/jci.insight.154164
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Paraneoplastic tumefactive demyelination with underlying combined germ cell cancer.

    Broadfoot, Jack R / Archer, Hilary A / Coulthard, Elizabeth / Appelman, Auke P A / Sutak, Judit / Braybrooke, Jeremy P / Love, Seth

    Practical neurology

    2015  Volume 15, Issue 6, Page(s) 451–455

    Abstract: Paraneoplastic demyelination is a rare disorder of the central nervous system. We describe a 60-year-old man with tumefactive demyelination who had an underlying retroperitoneal germ cell cancer. He presented with visuospatial problems and memory loss ... ...

    Abstract Paraneoplastic demyelination is a rare disorder of the central nervous system. We describe a 60-year-old man with tumefactive demyelination who had an underlying retroperitoneal germ cell cancer. He presented with visuospatial problems and memory loss and had a visual field defect. His MRI was interpreted as a glioma but stereotactic biopsy showed active demyelination. Investigation for multiple sclerosis was negative but CT imaging showed retroperitoneal lymphadenopathy, and nodal biopsy confirmed a combined germ cell cancer. He responded poorly to corticosteroid treatment, and his visual field defect progressed. However, 6 months after plasma exchange and successful chemotherapy, he has partially improved clinically and radiographically. Tumefactive demyelination is typically associated with multiple sclerosis but may be paraneoplastic. It is important to recognise paraneoplastic tumefactive demyelination early, as the neurological outcome relies on treating the associated malignancy.
    MeSH term(s) Antigens, CD/metabolism ; Brain/metabolism ; Brain/pathology ; Brain Neoplasms/complications ; Brain Neoplasms/diagnosis ; Demyelinating Diseases/complications ; Demyelinating Diseases/diagnosis ; Glial Fibrillary Acidic Protein/metabolism ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/complications ; Neoplasms, Germ Cell and Embryonal/diagnosis ; Paraneoplastic Syndromes/complications ; Paraneoplastic Syndromes/diagnosis ; Visual Fields/physiology
    Chemical Substances Antigens, CD ; Glial Fibrillary Acidic Protein
    Language English
    Publishing date 2015-12
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 2170881-2
    ISSN 1474-7766 ; 1474-7758
    ISSN (online) 1474-7766
    ISSN 1474-7758
    DOI 10.1136/practneurol-2015-001146
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 6001: Show issues Location:
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  6. Article ; Online: Lymphomatoid granulomatosis of the uterine cervix.

    Melegh, Zsombor / Sutak, Judit / Whiteway, Alastair / Rooney, Nicholas / Pawade, Joya

    Pathology, research and practice

    2009  Volume 205, Issue 5, Page(s) 371–374

    Abstract: Lymphomatoid granulomatosis is an Epstein-Barr virus-driven lymphoproliferative disorder, usually with a prominent pulmonary involvement and occasional extrapulmonary manifestations. Here, we present a case of lymphomatoid granulomatosis confined to the ... ...

    Abstract Lymphomatoid granulomatosis is an Epstein-Barr virus-driven lymphoproliferative disorder, usually with a prominent pulmonary involvement and occasional extrapulmonary manifestations. Here, we present a case of lymphomatoid granulomatosis confined to the uterine cervix at the initial diagnosis. The disease was preceded by an immunosuppressive condition, namely low-grade lymphoplasmacytic lymphoma treated with chemotherapy. This is the first report of lymphomatoid granulomatosis at this site and emphasizes that it can present at unusual sites, such as the female genital tract in immunosuppressed patients.
    MeSH term(s) Aged ; Antineoplastic Agents/therapeutic use ; Epstein-Barr Virus Infections/complications ; Female ; Humans ; Immunocompromised Host ; Immunohistochemistry ; In Situ Hybridization ; Lymphomatoid Granulomatosis/immunology ; Lymphomatoid Granulomatosis/metabolism ; Lymphomatoid Granulomatosis/pathology ; Neoplasms, Second Primary/immunology ; Neoplasms, Second Primary/pathology ; Palatine Tonsil/metabolism ; Palatine Tonsil/pathology ; RNA, Viral/analysis ; Uterine Cervical Neoplasms/immunology ; Uterine Cervical Neoplasms/metabolism ; Uterine Cervical Neoplasms/pathology ; Waldenstrom Macroglobulinemia/drug therapy ; Waldenstrom Macroglobulinemia/pathology
    Chemical Substances Antineoplastic Agents ; Epstein-Barr virus encoded RNA 2 ; RNA, Viral
    Language English
    Publishing date 2009
    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.2008.11.004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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