Publications by authors named "Kemeny J"

Article Synopsis
  • Atypical anti-glomerular basement membrane (GBM) nephritis is a rare kidney condition that presents differently than typical cases, showing bright linear immunoglobulin staining without the usual serum anti-GBM antibodies.
  • In a study involving patients diagnosed from 2003 to 2022, 25 out of 38 potential cases were confirmed, with a majority exhibiting symptoms like hematuria and varying forms of glomerulonephritis.
  • The findings suggest that atypical anti-GBM nephritis often progresses slower than typical cases, but further research is necessary to understand its complete nature and implications.
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Background And Aims: Aging is characterized, at the systemic level, by the development of low-grade inflammation, which has been identified as determining sarcopenia by blunting postprandial muscle anabolism. The causes of this "inflammageing" is still not clearly defined. An increased intestinal permeability, a microbiota dysbiosis and subsequent generation of intestinal then generalized inflammation have been hypothesized.

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Background: Acro-osteolysis (AO) refers to resorption of the distal finger and toe phalanges. It displays two patterns: (i) diffuse AO and (ii) transverse or bandlike AO. AO can be a sign of local distress (e.

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Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms.

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We report a case of a needlefish jaws retained near the C5-C6 joint that was associated with chronic pain and inflammation and seen confirmed by FDG-PET scan. Two unsuccessful surgeries using an anterior approach were complicated by vascular and nerve injuries. We used image-guided surgery with a posterior approach.

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Background: Thrombotic microangiopathy (TMA) syndromes are characterized by the association of hemolytic anemia, thrombocytopenia and organ injury due to arteriolar and capillary thrombosis.

Case Presentation: We report the first case of adult onset cobalamin C (Cbl C) disease associated with anti-factor H antibody-associated hemolytic uremic syndrome (HUS). A 19-year-old woman was admitted to the nephrology department owing to acute kidney failure, proteinuria, and hemolytic anemia with schizocytes.

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In cancer cells, aberrant DNA methylation is commonly associated with transcriptional alterations, including silencing of tumor suppressor genes. However, multiple epigenetic mechanisms, including polycomb repressive marks, contribute to gene deregulation in cancer. To dissect the relative contribution of DNA methylation-dependent and -independent mechanisms to transcriptional alterations at CpG island/promoter-associated genes in cancer, we studied 70 samples of adult glioma, a widespread type of brain tumor, classified according to their isocitrate dehydrogenase () mutation status.

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Background: Deregulation of cholesterol metabolism represents a hallmark of prostate cancer (PCa) and promotes its development.

Objective: To compare cholesterol metabolism on individual paired normal and tumour prostate tissues obtained from patients with PCa.

Design, Setting, And Participants: Between 2008 and 2012, normal and tumour paired tissue samples were collected from radical prostatectomy specimens from a cohort of 69 patients treated for localised PCa.

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Rationale: Kidney transplantation is considered the best treatment for patients with end stage renal disease. Ischemia- reperfusion injury (IRI) is an evitable event after deceased donor transplantation and influences short term and long term graft outcome. Few data on IRI's histology in the setting of kidney transplantation are available in the literature despite its frequency and its severity.

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Background: Open lung biopsy (OLB) is a rare procedure in intensive care units (ICUs) for therapeutic management of acute respiratory failure (ARF). The purpose of this study was to analyze the diagnostic yield, therapeutic contribution and complications of OLB in ICU patients with ARF of unclear etiology, including acute respiratory distress syndrome (ARDS) and ARDS mimics.

Methods: Retrospective study conducted in a 10-bed ICU over a 13-year period.

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Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome in child and adult. The collapsing forms are of poor renal prognosis and are usually secondary to viral infections with, first and foremost, the human immunodeficiency virus. Among other viral etiologies, cytomegalovirus (CMV) is an uncommon cause.

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Article Synopsis
  • Cutaneous manifestations (CM) are common in ANCA-associated vasculitides (AAV), with varying frequencies and types seen in different forms like EGPA, MPA, and GPA.
  • A study of 1553 AAV patients found specific lesions associated with each type; for instance, oral ulcers were more common in GPA, while urticarial lesions were frequent in EGPA.
  • Overall, CM indicated a worse prognosis in GPA, and while pathological analyses showed inflammation, distinct features were not identified for each AAV type, highlighting how lesion types affect granuloma organization.
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Kidney disease in the setting of a hematologic malignancy is common, with the frequency and type of kidney disease varying depending on the specific malignancy. Various glomerular diseases and tumor infiltration of the kidneys have been reported in patients with lymphoproliferative disorders. Descriptions of kidney involvement in myeloproliferative disorders have been much rarer.

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Article Synopsis
  • Human malignant gliomas utilize two main mechanisms for telomere maintenance: reactivation of telomerase or activation of alternative lengthening of telomeres (ALT).
  • The study analyzed 63 glioma samples, finding correlations between ALT-specific C-circles, IDH1/2 mutations, increased telomeric DNA content, and other histomolecular markers.
  • Elevated levels of TERRA were associated with improved patient survival, suggesting that telomeric markers could enhance diagnosis and treatment strategies in the future.
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The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC.

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Background: H3K27me3 histone marks shape the inhibition of gene transcription. In prostate cancer, the deregulation of H3K27me3 marks might play a role in prostate tumor progression.

Methods: We investigated genome-wide H3K27me3 histone methylation profile using chromatin immunoprecipitation (ChIP) and 2X400K promoter microarrays to identify differentially-enriched regions in biopsy samples from prostate cancer patients.

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Adrenocortical carcinoma (ACC) is a rare cancer with poor prognosis. Pan-genomic analyses identified p53/Rb and WNT/β-catenin signaling pathways as main contributors to the disease. However, isolated β-catenin constitutive activation failed to induce malignant progression in mouse adrenocortical tumors.

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Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy.

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Acute kidney injury (AKI) is a major complication in patients with liver disease. Although hepatorenal syndrome is frequently involved, bile cast nephropathy, characterized by tubular bile cast formation, has been scarcely described in the setting of severe liver failure. Few renal histology studies are available in these patients.

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Signal transducer and activator of transcription 3 (STAT3) is aberrantly activated in glioblastoma and has been identified as a relevant therapeutic target in this disease and many other human cancers. After two decades of intensive research, there is not yet any approved STAT3-based glioma therapy. In addition to the canonical activation by tyrosine 705 phosphorylation, concordant reports described a potential therapeutic relevance of other post-translational modifications including mainly serine 727 phosphorylation.

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