195 results on '"Kemeny JL"'
Search Results
2. Early dental loss in Sjogren's syndrome
- Author
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Baudet Pommel, M, Albuisson, F, Kemeny, Jl, Falvard, F, Ristori, Jm, Fraysse, Mp, Sauvezie, B, and DE VITA, Salvatore
- Published
- 1994
3. Diffuse interstitial lung disease due to AA amyloidosis
- Author
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C Planes, Michel Brauner, Kemeny Jl, J P Battesti, Dominique Valeyre, and D Kleinknecht
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Male ,Pulmonary and Respiratory Medicine ,Serum Amyloid A Protein ,Pathology ,medicine.medical_specialty ,business.industry ,Pulmonary Fibrosis ,Amyloidosis ,Respiratory disease ,Interstitial lung disease ,respiratory system ,medicine.disease ,AA amyloidosis ,Pulmonary fibrosis ,medicine ,Humans ,business ,Complication ,Nephrotic syndrome ,Aged ,Research Article - Abstract
A man developed interstitial lung disease and nephrotic syndrome due to AA amyloidosis. There was no evidence of an underlying disease predisposing to amyloidosis.
- Published
- 1992
4. Pleuropéricardite révélant un myélome multiple
- Author
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Vandel, B, primary, André, M, additional, Lipiecki, J, additional, Filaire, M, additional, Travade, P, additional, Schmidt, J, additional, Kemeny, JL, additional, Ponsonnaille, J, additional, and Aumaître, O, additional
- Published
- 1998
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5. Le vascular endothelial growth factor n’est pas exprimé par les cellules plasmocytaires au cours du syndrome POEMS
- Author
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Gherardi, RK, primary, Christov, C, additional, Kemeny, JL, additional, Authier, FJ, additional, and Soubrier, M, additional
- Published
- 1997
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6. Abcès aseptiques rétropharyngés: une localisation inaugurale inhabituelle des abcès aseptiques disséminés
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André, M, primary, Aumaître, O, additional, Bielsa, C, additional, Frances, C, additional, Beytout, J, additional, Grobost, O, additional, Kemeny, JL, additional, and Piette, JC, additional
- Published
- 1997
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7. Une fièvre prolongée avec lombalgies et tuméfactions costales
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Milesi, AM, primary, Schmidt, J, additional, Klisnick, A, additional, Kemeny, JL, additional, and Aumaitre, O, additional
- Published
- 1996
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8. Fièvre, malaises posturaux et douleurs thoraciques atypiques
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Klisnick, A, primary, Schmidt, J, additional, Milesi, AM, additional, Kemeny, JL, additional, Riberolles, C de, additional, and Aumaitre, O, additional
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- 1996
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9. Syndrome néphrotique avec glomérulopathie extramembraneuse et follicules épithélioïdes interstitiels chez un transplanté rénal. Diagnostic de sarcoïdose et rémission complète après corticothérapie
- Author
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Schmidt, J, primary, Deteix, P, additional, Dubost, JJ, additional, Chevenet, C, additional, Kemeny, JL, additional, Fonck, Y, additional, Rance, N, additional, Baguet, JC, additional, and Sauvezie, B, additional
- Published
- 1994
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10. Purpura rhumatoïde de l'adulte de plus de 50 ans: 8 observations
- Author
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Cauhape, P, primary, Milesi-Lecat, AM, additional, Minard, P, additional, Oualid, T, additional, Chadeyras, S, additional, Kemeny, JL, additional, Souteyrand, P, additional, Marcheix, JC, additional, and Aumaitre, O, additional
- Published
- 1993
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11. Mécanismes de l'atteinte cardiaque dans la sclérodermie systémique (à propos de trois observations)
- Author
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Milesi-Lecat, AM, primary, Cauhape, P, additional, Schmidt, J, additional, Marcaggi, X, additional, Peycelon, P, additional, Kemeny, JL, additional, Lusson, JR, additional, Frances, C, additional, Marcheix, JC, additional, and Aumaitre, O, additional
- Published
- 1993
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12. Syndrome primaire des antiphospholipides d'évolution fatale et cytostéatonécrose ostéoarticulaire
- Author
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Dubost Jj, Martin Soubrier, Kemeny Jl, Bommelaer G, D. Guelon, Amouroux J, and B. Sauvezie
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Autoimmune disease ,medicine.medical_specialty ,Pathology ,biology ,Shoulders ,Pulse (signal processing) ,business.industry ,Gastroenterology ,Weber–Christian disease ,medicine.disease ,Primary antiphospholipid syndrome ,Surgery ,Cytosteatonecrosis ,Immune system ,Steroid therapy ,immune system diseases ,Antiphospholipid syndrome ,Immunopathology ,Internal Medicine ,biology.protein ,Medicine ,Fat necrosis ,Antibody ,business ,Organ system - Abstract
The antiphospholipid syndrome produces acute occlusions of arteries and veins. This syndrome can cause a multiple organ systems failure whose outcome is often fatal. The authors report a case of the primary, antiphospholipid syndrome characterized by this fatal outcome, a so-called "devastating" syndrome following pulse steroids. In this patient, the antiphospholipid antibodies had been found after presenting bone-marrow fat necrosis, which led to extensive lesions of knees, hips and shoulders. Damage to the cell membranes in necrotic lesions might have promoted the immune response against phospholipids. The potential risks of pulse doses of steroids in the antiphospholipid syndrome are documented by the present observation, which also suggests that antiphospholipid antibodies should be determined in cases of fat necrosis of all origins.
- Published
- 1993
13. Réticulo-histiocytose multicentrique
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D. Daupleix, Garnier J, R.-N. Sachs, J. Lanfranchi, Amouroux J, and Kemeny Jl
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Gynecology ,medicine.medical_specialty ,business.industry ,Gastroenterology ,Internal Medicine ,medicine ,Multicentric reticulohistiocytosis ,medicine.disease ,business - Abstract
Resume La reticulo-histiocytose multicentrique ou dermo-arthrite lipoidique est une affection rare d'etiologie indeterminee. Un nouveau cas en est rapporte chez une femme de 75 ans, caracteristique par ses manifestations dermo-articulaires et sa lesion histologique. Il se distingue par un aspect caricatural des lesions articulaires et la fistulisation inhabituelle d'un nodule a la peau.
- Published
- 1983
14. Myocardiopathie hypertrophique et maladie de von recklinghausen
- Author
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Amouroux J, Kemeny Jl, J. Lanfranchi, Ch. Buschauer-Bonnet, and R.-N. Sachs
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Gynecology ,medicine.medical_specialty ,business.industry ,Gastroenterology ,Internal Medicine ,Medicine ,business - Published
- 1984
15. Hemolysis in a patient with alkaptonuria and chronic kidney failure.
- Author
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Heng AE, Courbebaisse M, Kemeny JL, Matesan R, Bonniol C, Deteix P, and Souweine B
- Abstract
In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogentisic acid (HGA) in the body. Fatal disease cases are infrequent, and death often results from kidney or cardiac complications. We report a 24-year-old alkaptonuric man with severe decreased kidney function who developed fatal metabolic acidosis and intravascular hemolysis. Hemolysis may have been caused by rapid and extensive accumulation of HGA and subsequent accumulation of plasma soluble melanins. Toxic effects of plasma soluble melanins, their intermediates, and reactive oxygen side products are increased when antioxidant mechanisms are overwhelmed. A decrease in serum antioxidative activity has been reported in patients with chronic decreased kidney function. However, despite administration of large doses of an antioxidant agent and ascorbic acid and intensive kidney support, hemolysis and acidosis could not be brought under control and hemolysis led to the death of the patient. [ABSTRACT FROM AUTHOR]
- Published
- 2010
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16. Le vascular endothelial growthfactor n’est pas exprimé par les cellules plasmocytaires au cours du syndrome POEMS
- Author
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Gherardi, RK, Christov, C, Kemeny, JL, Authier, FJ, and Soubrier, M
- Published
- 1997
- Full Text
- View/download PDF
17. Insuffisance rénale aiguë dans la granulomatose de Wegener. Aspects évolutifs et thérapeutiques : deux observations
- Author
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Klisnick, A, Schmidt, J, Milési, AM, Aumaître, O, André, M, Baguet, JC, Kémény, JL, Ristori, JM, and Marcheix, JC
- Published
- 1995
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18. Tamponnade révélatrice d'une périartérite noueuse
- Author
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Petaut, M, Schmidt, J, Klisnick, A, Amonchot, A, Malka, CE, Kémény, JL, Citron, B, and Ponsonnaille, J
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- 1995
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19. Sarcome de Kaposi compliquant une périartérite noueuse: rémission complète des deux affections sous immunoglobulines intraveineuses
- Author
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Schmidt, J, Mouillet, ML, Papo, T, Frances, C, Kémény, JL, Dechelotte, P, Ristori, JM, and Aumaître, O
- Published
- 1995
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20. Atypical Anti-Glomerular Basement Membrane Nephritis: A Case Series From the French Nephropathology Group.
- Author
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Chauveau B, Gibier JB, Olagne J, Morel A, Aydin S, McAdoo SP, Viallet N, Perrochia H, Pambrun E, Royal V, Demoulin N, Kemeny JL, Philipponnet C, Hertig A, Boffa JJ, Plaisier E, Domenger C, Brochériou I, Deltombe C, Duong Van Huyen JP, Buob D, Roufosse C, Hellmark T, Audard V, Mihout F, Nasr SH, Renaudin K, Moktefi A, and Rabant M
- Subjects
- Humans, Female, Male, Adult, Middle Aged, France epidemiology, Retrospective Studies, Aged, Glomerular Basement Membrane pathology, Glomerular Basement Membrane immunology, Glomerular Basement Membrane ultrastructure, Autoantibodies, Anti-Glomerular Basement Membrane Disease diagnosis, Anti-Glomerular Basement Membrane Disease pathology, Anti-Glomerular Basement Membrane Disease immunology
- Abstract
Rationale & Objective: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized by a bright linear immunoglobulin staining along the GBM by immunofluorescence without a diffuse crescentic glomerulonephritis nor serum anti-GBM antibodies by conventional enzyme-linked immunosorbent assay (ELISA). We characterized a series of patients with atypical anti-GBM disease., Study Design: Case series., Setting & Participants: Patients identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022., Findings: Among 38 potential cases, 25 were included, of whom 14 (56%) were female and 23 (92%) had hematuria. The median serum creatinine at diagnosis was 150 (IQR, 102-203) μmol/L and median urine protein-creatinine ratio (UPCR) was 2.4 (IQR, 1.3-5.2) g/g. Nine patients (36%) had endocapillary proliferative glomerulonephritis (GN), 4 (16%) had mesangial proliferative GN, 4 (16%) had membranoproliferative GN, 2 (8%) had pure and focal crescentic GN, 1 (4%) had focal segmental glomerulosclerosis, and 5 had glomeruli that were unremarkable on histopathology. Nine patients (36%) had crescents, involving a median of 9% of glomeruli. Bright linear staining for IgG was seen in 22 cases (88%) and for IgA in 3 cases (12%). The 9 patients (38%) who had a monotypic staining pattern tended to be older with less proteinuria and rarely had crescents. Kidney survival rate at 1 year was 83% and did not appear to be associated with the light chain restriction., Limitations: Retrospective case series with a limited number of biopsies including electron microscopy., Conclusions: Compared with typical anti-GBM disease, atypical anti-GBM nephritis frequently presents with an endocapillary or mesangial proliferative glomerulonephritis pattern and appears to have a slower disease progression. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes., Plain-Language Summary: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized histologically by bright linear immunoglobulin staining along the GBM without diffuse crescentic glomerulonephritis or circulating anti-GBM antibodies. We report a case series of 25 atypical cases of anti-GBM nephritis in collaboration with the French Nephropathology Group. Compared with typical anti-GBM disease, we observed a slower disease progression. Patients frequently presented with heavy proteinuria and commonly had evidence of endocapillary or mesangial proliferative glomerulonephritis. About half of the patients displayed a monotypic immune staining pattern; they tended to be older, with less proteinuria, and commonly without glomerular crescents in biopsy specimens. No concomitant circulating monoclonal gammopathy was detected. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes., (Copyright © 2023 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)
- Published
- 2024
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21. Challenging foreign body surgery: residual needlefish jaws.
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El Ouadih Y, Pham Dang N, Groza D, Thiombiano A, Khalil T, Kemeny JL, and Lemaire JJ
- Subjects
- Animals, Humans, Tomography, X-Ray Computed, Jaw, Beloniformes, Foreign Bodies diagnostic imaging, Foreign Bodies surgery
- Abstract
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.
- Published
- 2023
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22. Anti-inflammatory Streptococcus thermophilus CNRZ160 limits sarcopenia induced by low-grade inflammation in older adult rats.
- Author
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Savary-Auzeloux I, Jarzaguet M, Migné C, Kemeny JL, Novais-Gameiro L, de Azevedo M, Mathé V, Mariotti F, Langella P, Chatel JM, and Dardevet D
- Abstract
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. The objective of this study was to test in vivo during aging if (1) a chronic low-grade intestinal inflammation can lead to anabolic resistance and muscle loss and (2) if a bacterial strain presenting anti-inflammatory properties could prevent these adverse effects., Methods: Young adult (6 m) and elderly rats (18 m) received Dextran Sodium Sulfate (DSS) for 28 days to generate low-grade intestinal inflammation, and received (PB1 or PB2 groups) or not (DSS group) one of the two S. Thermophilus strains (5 × 10
9 CFU/day) previously shown to present an anti-inflammatory potential in vitro . They were compared to pair fed control (PF). Muscle and colon weights and protein synthesis (using13 C Valine) were measured at slaughter. Muscle proteolysis, gut permeability and inflammatory markers were assessed only in old animals by RT-PCR or proteins quantifications (ELISA)., Results: In both adult and old rats, DSS reduced absolute protein synthesis (ASR) in gastrocnemius muscle [-12.4% (PB1) and -9.5% (PB2) vs. PF, P < 0.05] and increased ASR in colon (+86% and +30.5%, respectively vs. PF, P < 0.05). PB1 (CNRZ160 strain) but not PB2 resulted in a higher muscle ASR as compared to DSS in adults (+18%, P < 0.05), a trend also observed for PB1 in old animals (+12%, P = 0.10). This was associated with a blunted increase in colon ASR. In old rats, PB1 also significantly decreased expression of markers of autophagy and ubiquitin-proteasome pathways vs. DSS groups and improved gut permeability (assessed by Occludin, Zonula Occludens 1 and Claudin 1 expression, P < 0.05) and alleviated systemic inflammation (A2M: -48% vs. DSS, P < 0.05)., Conclusion: The loss of muscle anabolism associated with low-grade intestinal inflammation can be prevented by supplementation with anti-inflammatory CNRZ160 strain. We propose that the moderated gut inflammation by CNRZ160 may result in curtailed amino acids (AA) utilization by the gut, and subsequent restored AA systemic availability to support muscle protein accretion. Therefore, CNRZ160 could be considered as an efficient probiotic to modulate muscle mass loss and limit sarcopenia during aging., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Savary-Auzeloux, Jarzaguet, Migné, Kemeny, Novais-Gameiro, de Azevedo, Mathé, Mariotti, Langella, Chatel and Dardevet.)- Published
- 2022
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23. Is an association of acro-osteolysis, bone fragility, and enchondromatosis a newfound disease caused by an amplification of PTHLH? A case report.
- Author
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Echaubard S, Pebrel-Richard C, Chausset A, Kemeny JL, Merlin E, and Laffargue F
- Subjects
- Adolescent, Child, Cross-Sectional Studies, Female, Humans, Parathyroid Hormone-Related Protein, Radiography, Acro-Osteolysis diagnosis, Acro-Osteolysis diagnostic imaging, Enchondromatosis complications
- Abstract
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.g. of toxic origin), but is very often a sign of a constitutional or systemic acquired disorder., Case Presentation: A 15-year-old girl was referred to a paediatric rheumatologist for recurrent pain in her fingertips. She presented a particular cross-sectional AO associated with the presence of intraosseous cysts and bone fragility with atypical fractures. Initial laboratory tests and radiological examination did not allow an etiological diagnosis. Genetic studies revealed a 12p11.22-p11.23 microduplication of 900 kb including the PTHLH (parathyroid hormone-like hormone) gene, which encodes for a hormone involved in the regulation of endochondral ossification and differentiation of chondrocytes, via its PTHLH receptor., Conclusions: To date, 12p11.22-p11.23 duplications have been reported in five families with skeletal abnormalities, and in particular AO and enchondromatosis associated with bone fragility. This new observation, added to the other reported cases, suggests a close relationship between the presence of this microduplication and the skeletal abnormalities found in the patient. We suggest the descriptive name ABES (acro-osteolysis, bone fragility and enchondromatosis syndrome) to designate this disorder., (© 2022. The Author(s).)
- Published
- 2022
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24. Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.
- Author
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Boucher S, Tai FWJ, Delmaghani S, Lelli A, Singh-Estivalet A, Dupont T, Niasme-Grare M, Michel V, Wolff N, Bahloul A, Bouyacoub Y, Bouccara D, Fraysse B, Deguine O, Collet L, Thai-Van H, Ionescu E, Kemeny JL, Giraudet F, Lavieille JP, Devèze A, Roudevitch-Pujol AL, Vincent C, Renard C, Franco-Vidal V, Thibult-Apt C, Darrouzet V, Bizaguet E, Coez A, Aschard H, Michalski N, Lefevre GM, Aubois A, Avan P, Bonnet C, and Petit C
- Subjects
- Age Factors, Age of Onset, Animals, Case-Control Studies, Cohort Studies, Heterozygote, Humans, Membrane Proteins genetics, Mice, MicroRNAs genetics, Mitochondria genetics, Exome Sequencing, Deafness genetics, Genes, Dominant, Mutation genetics, Presbycusis genetics
- Abstract
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. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls ( P = 0.001); half were previously unknown (AF < 0.000002). MYO6 , MYO7A , PTPRQ , and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1
N321I/+ mice, carrying the TMC1 :p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy., Competing Interests: The authors declare no competing interest.- Published
- 2020
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25. Kidney Transplantation With a Sickle Cell Disease Donor.
- Author
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Philipponnet C, Aniort J, Garrouste C, Kemeny JL, Hadj-Abdelkader M, and Heng AE
- Published
- 2020
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26. Cobalamin c deficiency associated with antifactor h antibody-associated hemolytic uremic syndrome in a young adult.
- Author
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Philipponnet C, Desenclos J, Brailova M, Aniort J, Kemeny JL, Deville C, Fremeaux-Bacchi V, Souweine B, and Heng AE
- Subjects
- Adult, Biopsy, Female, Hemolytic-Uremic Syndrome pathology, Hemolytic-Uremic Syndrome therapy, Humans, Autoantibodies blood, Complement Factor H immunology, Hemolytic-Uremic Syndrome complications, Hemolytic-Uremic Syndrome immunology, Vitamin B 12 Deficiency complications
- Abstract
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. TMA was diagnosed and plasma exchanges were started in emergency. Exhaustive analyses showed 1) circulating anti factor H antibody and 2) hyperhomocysteinemia, hypomethioninemia and high levels of methylmalonic aciduria pointing towards Clb C disease. Cbl C disease has been confirmed by methylmalonic aciduria and homocystinuria type C protein gene sequencing revealing two heterozygous pathogenic variants. The kidney biopsy showed 1) intraglomerular and intravascular thrombi 2) noticeable thickening of the capillary wall with a duplication aspect of the glomerular basement membrane and a glomerular capillary wall IgM associated with Cbl C disease related TMA. We initiated treatment including hydroxycobalamin, folinic acid, betaine and levocarnitine and Eculizumab. Rituximab infusions were performed allowing a high decrease in anti-factor H antibody rate. Six month after the disease onset, Eculizumab was weaning and vitaminotherapy continued. Outcome was favorable with a dramatic improvement in kidney function., Conclusion: TMA with renal involvement can have a complex combination of risk factors including anti-FH autoantibody in the presence of cblC deficiency.
- Published
- 2020
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27. Transcriptional alterations in glioma result primarily from DNA methylation-independent mechanisms.
- Author
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Court F, Le Boiteux E, Fogli A, Müller-Barthélémy M, Vaurs-Barrière C, Chautard E, Pereira B, Biau J, Kemeny JL, Khalil T, Karayan-Tapon L, Verrelle P, and Arnaud P
- Subjects
- Adult, Cell Line, Tumor, Chromatin genetics, CpG Islands genetics, Female, Gene Expression Regulation, Neoplastic genetics, Glioma pathology, Histones genetics, Humans, Isocitrate Dehydrogenase genetics, Jumonji Domain-Containing Histone Demethylases genetics, Male, Promoter Regions, Genetic, DNA Methylation genetics, Epigenesis, Genetic genetics, Glioma genetics, Transcription, Genetic
- Abstract
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 ( IDH1 ) mutation status. We found that most transcriptional alterations in tumor samples were DNA methylation-independent. Instead, altered histone H3 trimethylation at lysine 27 (H3K27me3) was the predominant molecular defect at deregulated genes. Our results also suggest that the presence of a bivalent chromatin signature at CpG island promoters in stem cells predisposes not only to hypermethylation, as widely documented, but more generally to all types of transcriptional alterations in transformed cells. In addition, the gene expression strength in healthy brain cells influences the choice between DNA methylation- and H3K27me3-associated silencing in glioma. Highly expressed genes were more likely to be repressed by H3K27me3 than by DNA methylation. Our findings support a model in which altered H3K27me3 dynamics, more specifically defects in the interplay between polycomb protein complexes and the brain-specific transcriptional machinery, is the main cause of transcriptional alteration in glioma cells. Our study provides the first comprehensive description of epigenetic changes in glioma and their relative contribution to transcriptional changes. It may be useful for the design of drugs targeting cancer-related epigenetic defects., (© 2019 Court et al.; Published by Cold Spring Harbor Laboratory Press.)
- Published
- 2019
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28. Individual Comparison of Cholesterol Metabolism in Normal and Tumour Areas in Radical Prostatectomy Specimens from Patients with Prostate Cancer: Results of the CHOMECAP Study.
- Author
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Celhay O, Bousset L, Guy L, Kemeny JL, Leoni V, Caccia C, Trousson A, Damon-Soubeyrant C, De Haze A, Sabourin L, Godfraind C, de Joussineau C, Pereira B, Morel L, Lobaccaro JM, and Baron S
- Subjects
- Acetyl-CoA C-Acetyltransferase genetics, Acetyl-CoA C-Acetyltransferase metabolism, Aged, Apolipoproteins E genetics, Apolipoproteins E metabolism, Gene Expression Profiling, Gene Expression Regulation, Neoplastic, Humans, Male, Middle Aged, Principal Component Analysis, Prostatectomy, Prostatic Neoplasms genetics, Prostatic Neoplasms metabolism, Scavenger Receptors, Class B genetics, Scavenger Receptors, Class B metabolism, Sterol Regulatory Element Binding Protein 2 genetics, Sterol Regulatory Element Binding Protein 2 metabolism, Cholesterol metabolism, Gene Regulatory Networks, Prostatic Neoplasms surgery
- Abstract
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., Outcome Measurements and Statistical Analysis: Tumour and normal tissues were subjected to gene analysis, sterol measurement, and immunohistochemistry. The Wilcoxon paired test and Spearman test were applied for comparison and correlation analyses, respectively. Principal component analysis was also carried out to investigate relationships between quantitative variables., Results and Limitations: Overall, cholesterol concentrations were not significantly different between tissue pairs. However, tumour samples were significantly associated with downregulated de novo cholesterol synthesis, but exhibited 54.7% overexpression of SCARB1 that could increase high-density lipoprotein uptake in PCa. Tumour tissues showed different trafficking of available cholesterol, with significantly lower ACAT1, and an altered efflux via APOE. Furthermore, cholesterol metabolism in tumour tissues was characterised by higher accumulation of 7α-hydroxycholesterol (OHC), 7βOHC, and 7-ketosterol, and a lower level of 27OHC., Conclusions: Focusing on individually paired prostate tissues, our results highlighted several differences between normal and tumour samples linked to a metabolic shift in cholesterol flux. PCa samples exhibited a specific tissue signature characterised by higher SCARB1 expression, higher accumulation of OHC species, and clear downregulation of de novo cholesterol synthesis., Patient Summary: Comparing normal and tumour tissues from the same prostates, our study identified a set of alterations in prostate cancer samples in terms of their use of cholesterol. These included higher cholesterol uptake, accumulation of oxidised cholesterol derivatives, and autonomous cellular production of cholesterol. Together, these data provide promising clinical targets to fight prostate cancer., (Copyright © 2018 European Association of Urology. Published by Elsevier B.V. All rights reserved.)
- Published
- 2019
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29. Ischemia reperfusion injury in kidney transplantation: A case report.
- Author
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Philipponnet C, Aniort J, Garrouste C, Kemeny JL, and Heng AE
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- Humans, Kidney blood supply, Male, Middle Aged, Transplants blood supply, Delayed Graft Function etiology, Kidney Transplantation adverse effects, Postoperative Complications etiology, Reperfusion Injury etiology
- Abstract
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., Patient Concerns: A 64-year-old patient was admitted for his 1st kidney transplantation. There were no pre-existing immunization. The surgery proceeded without complications; with cold ischemia estimated at 37 h 50 min and warm ischemia at 44 min. The immunosuppression protocol was as follows: induction by thymoglobulins, mycophelonate mofetil, corticosteroids. Few hours after transplantation, the patient remained anuric and the biological assessment highlighted in addition to renal failure, hyperlactatemia at 5 mmol/L and a high increase in lactate deshydrogenase (LDH) at 5239 U/L. An abdominopelvic angio-scanner was performed urgently to eliminate the hypothesis of thrombosis of the artery or vein of the graft. A kidney biopsy was performed the day after the transplant and revealed massive lesions of acute tubular necrosis including apoptosis, autophagy-associated cell death, and necrosis. Microvascular dysfunction with increased vascular permeability and endothelial cell inflammation were also present. Activation of coagulation is represented by thrombi in the lumens of the glomerular capillaries., Diagnosis: The diagnosis was ischemia reperfusion injury responsible for delayed graft function (DGF)., Interventions: Immunosuppressive regimen was delayed use of calcineurin inhibitors, mycophenolate mofetil, and corticosteroids., Outcomes: At 1 year post transplant, the patient has a renal autonomy with a graft function stable and physiological proteinuria., Lessons: The main clinical consequences of IRI in kidney transplant are DGF, acute and chronic graft rejection, and chronic graft dysfunction. Reducing IRI is one of the most relevant challenge in kidney transplantation.
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- 2018
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30. Diagnostic yield and therapeutic impact of open lung biopsy in the critically ill patient.
- Author
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Philipponnet C, Cassagnes L, Pereira B, Kemeny JL, Devouassoux-Shisheboran M, Lautrette A, Guerin C, and Souweine B
- Subjects
- Aged, Aged, 80 and over, Berlin, Biopsy methods, Critical Illness, Female, Humans, Intensive Care Units, Male, Middle Aged, Pulmonary Fibrosis diagnosis, Respiration, Artificial methods, Retrospective Studies, Simplified Acute Physiology Score, Lung pathology, Respiratory Distress Syndrome diagnosis, Respiratory Distress Syndrome pathology
- Abstract
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. Patients undergoing OLB for ARF with undiagnosed infiltrates on CT scan were included. ARDS was defined according to Berlin criteria, and ARDS mimics as a condition looking like ARDS except for the presence of a known cause. OLB was contributive when the OLB findings yielded a specific diagnosis resulting in a change in the patients' treatment or management., Results: Forty six patients were included (sex ratio = 2.5, median and [interquartile range] age = 69 [59-77] years, and admission SAPS II = 42 [33-50]. ARF corresponded to ARDS in 22 patients and to ARDS mimics in 16. OLB yielded 61 diagnoses in 45 patients including diffuse alveolar damage (N = 21), lung fibrosis (N = 18), and organizing pneumonia (N = 11). OLB was contributive in 37 patients (80%), including 13/16 ARDS mimickers. The main contributions of OLB were the introduction or maintenance of steroids (N = 32) and discontinuation of antibiotics (N = 9). In 4 patients OLB resulted directly in the decision to forgo life-sustaining treatment. OLB complications occurred in 16 patients (35%), in one case associated with fatal outcome., Conclusion: OLB can play a useful role in the management of ICU patients with ARF of undetermined origin, including ARDS mimickers. Further studies should be done to identify the groups of ICU patients likely to benefit from the procedure with minimum risk., Competing Interests: The authors have declared that no competing interests exist.
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- 2018
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31. Immunoallergic interstitial nephritis secondary to denosumab.
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Philipponnet C, Kemeny JL, Aniort J, Garrouste C, and Heng AE
- Subjects
- Aged, Antibodies, Monoclonal therapeutic use, Biopsy, Needle, Denosumab therapeutic use, Drug Hypersensitivity diagnosis, Female, Follow-Up Studies, Humans, Immunohistochemistry, Nephritis, Interstitial immunology, Nephritis, Interstitial pathology, Osteoporosis, Postmenopausal diagnostic imaging, Antibodies, Monoclonal adverse effects, Denosumab adverse effects, Drug Hypersensitivity immunology, Nephritis, Interstitial chemically induced, Osteoporosis, Postmenopausal drug therapy
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- 2018
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32. [Collapsing focal segmental glomerulosclerosis induced by cytomegalovirus: A case report].
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Grèze C, Garrouste C, Kemeny JL, Philipponnet C, Aniort J, and Heng AÉ
- Subjects
- Adult, Cytomegalovirus Infections drug therapy, Female, Ganciclovir analogs & derivatives, Ganciclovir therapeutic use, Glomerulosclerosis, Focal Segmental drug therapy, Glucocorticoids therapeutic use, Humans, Kidney pathology, Kidney physiopathology, Valganciclovir, Antiviral Agents therapeutic use, Cytomegalovirus, Cytomegalovirus Infections complications, Glomerulosclerosis, Focal Segmental virology
- Abstract
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. We report a case of a 32years-old patient with collapsing focal segmental glomerulosclerosis induced by cytomegalovirus with initial acute renal failure and proteinuria at 12.4g/24h. The treatment associated ganciclovir during 7days followed by valganciclovir during 14days and steroids at 1mg/kg/day. Renal function improved and proteinuria decreased with this treatment. Proteinuria increase again 3weeks after valganciclovir discontinuation while CMV Polymerase chain reaction (PCR) was positive. Therefore, valganciclovir has been resumed allowing renal function normalization and decrease in proteinuria to 4g/24h after negative CMVPCR assay after 15weeks. Anti-CMV therapy combined with steroids seems to provide a renal response in case of FSGS induced by CMV even if long-term prognosis stays uncertain., (Copyright © 2017 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.)
- Published
- 2018
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33. Membranous Nephropathy and Intrarenal Extramedullary Hematopoiesis in a Patient With Myelofibrosis.
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Philipponnet C, Ronco P, Aniort J, Kemeny JL, and Heng AE
- Subjects
- Acute Kidney Injury etiology, Acute Kidney Injury metabolism, Antineoplastic Agents therapeutic use, Edema etiology, Glomerulonephritis, Membranous complications, Glomerulonephritis, Membranous metabolism, Humans, Hydroxyurea therapeutic use, Male, Middle Aged, Nephrotic Syndrome etiology, Nephrotic Syndrome metabolism, Nitriles, Primary Myelofibrosis drug therapy, Pyrazoles, Pyrimidines, Acute Kidney Injury pathology, Glomerulonephritis, Membranous pathology, Hematopoiesis, Extramedullary, Kidney pathology, Nephrotic Syndrome pathology, Primary Myelofibrosis complications
- Abstract
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. We report a case of membranous nephropathy accompanied by kidney injury in a patient with primary myelofibrosis with additional features considered related to the patient's myeloproliferative disorder. A 63-year-old patient with primary myelofibrosis underwent kidney biopsy to investigate nephrotic-range proteinuria and reduced kidney function. Histologic analysis revealed mesangial sclerosis and hypercellularity, changes indicative of membranous nephropathy, and infiltration of hematopoietic cells into the renal interstitium, peritubular capillaries, and perirenal tissue consistent with extramedullary hematopoiesis. He was treated with renin-angiotensin blockade and a Janus kinase inhibitor, resulting in improvement in kidney function and proteinuria., (Copyright © 2017 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)
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- 2017
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34. Clinical and pathological significance of cutaneous manifestations in ANCA-associated vasculitides.
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Frumholtz L, Laurent-Roussel S, Aumaître O, Maurier F, Le Guenno G, Carlotti A, Dallot A, Kemeny JL, Antunes L, Froment N, Fraitag S, London J, Berezne A, Terris B, Le Jeunne C, Mouthon L, Aractingi S, Guillevin L, Dupin N, and Terrier B
- Subjects
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis pathology, Humans, Phenotype, Prognosis, Recurrence, Skin Diseases etiology, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis complications, Skin Diseases pathology
- Abstract
Objectives: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking., Methods: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner., Results: CM were more frequent in EGPA (53.0%) and MPA (51.9%) than in GPA (36.7%). Lesions more frequently associated with GPA were oral ulcers (4.6% vs. 2.5% in EGPA and 0.3% in MPA), while pyoderma gangrenosum and palpebral xanthoma were specific to GPA. Lesions associated with MPA were segmentary edema (19.5% vs. 12.7% in EGPA and 4.3% in GPA) and livedo (12.4% vs. 0.5% and 2.6%, respectively), whereas those associated with EGPA were urticarial lesions (11.5% vs. 1.9% in GPA and 3.5% in MPA) and nodules (12,2% vs. 8.9% in GPA and 4.7% in MPA). In GPA, CM patients had more frequent vasculitis than granulomatous phenotype, and poorer relapse-free and overall survival. Pathological analysis showed vasculitis and/or granulomatous infiltrates in 87.5% of GPA, in 61.1% of EGPA and in all MPA. Vasculitis was more frequently observed in purpura and nodules, while granulomas were differently located and organized within vessels or interstitium according to the type of lesions., Conclusion: Each AAV seemed to be associated with a peculiar pattern of cutaneous lesions. CM are associated with poorer prognosis in GPA. Clinical-pathological correlations showed no specific feature of each AAV, whereas granulomatous infiltrates differ according to the type of lesions., (Copyright © 2017 Elsevier B.V. All rights reserved.)
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- 2017
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35. Role of STAT3 in Genesis and Progression of Human Malignant Gliomas.
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Ouédraogo ZG, Biau J, Kemeny JL, Morel L, Verrelle P, and Chautard E
- Subjects
- Animals, Brain Neoplasms genetics, Disease Progression, Glioma genetics, Humans, Brain Neoplasms metabolism, Gene Expression Regulation, Neoplastic genetics, Glioblastoma genetics, Glioma metabolism, STAT3 Transcription Factor metabolism
- Abstract
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. Such reports reinforce the need to refine the strategy of targeting STAT3 in each concerned disease. This review focuses on the role of serine 727 and tyrosine 705 phosphorylation of STAT3 in glioma. It explores their contribution to glial cell transformation and to the mechanisms that make glioma escape to both immune control and standard treatment.
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- 2017
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36. A French retrospective study on clinical outcome in 102 choroid plexus tumors in children.
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Siegfried A, Morin S, Munzer C, Delisle MB, Gambart M, Puget S, Maurage CA, Miquel C, Dufour C, Leblond P, André N, Branger DF, Kanold J, Kemeny JL, Icher C, Vital A, Coste EU, and Bertozzi AI
- Subjects
- Adolescent, Carcinoma genetics, Carcinoma pathology, Child, Child, Preschool, Choroid Plexus Neoplasms genetics, Choroid Plexus Neoplasms pathology, Female, Follow-Up Studies, France, Humans, Infant, Male, Neoplasm Grading, Papilloma, Choroid Plexus genetics, Papilloma, Choroid Plexus pathology, Polymorphism, Single Nucleotide, Retrospective Studies, Rhabdoid Tumor genetics, Rhabdoid Tumor pathology, Survival Analysis, Teratoma genetics, Teratoma pathology, Treatment Outcome, Carcinoma therapy, Choroid Plexus Neoplasms therapy, Papilloma, Choroid Plexus therapy, Rhabdoid Tumor therapy, Teratoma therapy
- Abstract
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. The 5 year overall survival was 100% in CPP, 96.2% in aCPP and 64.7% in CPC. In patients with localized disease, complete surgical resection was achieved in 48/52 CPP, 20/26 aCPP and 7/14 CPC. In this group, patients with complete surgical resection had better event free survival than patients with partial resection (88.9 vs. 41.6%). 28 patients (1 CPP, 6 aCPP and 22 CPC) had adjuvant chemotherapy. 2 aCPP and 9 CPC had radiotherapy. We underlined the need for a central histological review to accurately analyze clinical data; we reported a much higher overall survival for CPC than in most previous CPT series probably including atypical teratoid rhabdoid tumors. In our series, the 5 years overall survival in CPC (64.7%) was higher than event free survival (25.2%) and could be interpreted as a clue for the efficiency of adjuvant/salvage therapy even if the heterogeneity of applied treatments in this retrospective series does not allow for meaningful statistical comparisons.
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- 2017
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37. P53/Rb inhibition induces metastatic adrenocortical carcinomas in a preclinical transgenic model.
- Author
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Batisse-Lignier M, Sahut-Barnola I, Tissier F, Dumontet T, Mathieu M, Drelon C, Pointud JC, Damon-Soubeyrand C, Marceau G, Kemeny JL, Bertherat J, Tauveron I, Val P, Martinez A, and Lefrançois-Martinez AM
- Subjects
- Animals, Humans, Mechanistic Target of Rapamycin Complex 1, Mice, Mice, Transgenic, Multiprotein Complexes physiology, Neoplasm Metastasis, Retinoblastoma Protein antagonists & inhibitors, Sirolimus pharmacology, TOR Serine-Threonine Kinases physiology, Tumor Suppressor Protein p53 antagonists & inhibitors, Wnt Signaling Pathway physiology, beta Catenin physiology, Adrenocortical Carcinoma pathology, Antigens, Polyomavirus Transforming genetics, Retinoblastoma Protein physiology, Tumor Suppressor Protein p53 physiology
- Abstract
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. Therefore, there still was a need for a relevant animal model to study ACC pathogenesis and to test new therapeutic approaches. Here, we have developed a transgenic mice model with adrenocortical specific expression of SV40 large T-antigen (AdTAg mice), to test the oncogenic potential of p53/Rb inhibition in the adrenal gland. All AdTAg mice develop large adrenal carcinomas that eventually metastasize to the liver and lungs, resulting in decreased overall survival. Consistent with ACC in patients, adrenal tumors in AdTAg mice autonomously produce large amounts of glucocorticoids and spontaneously activate WNT/β-catenin signaling pathway during malignant progression. We show that this activation is associated with downregulation of secreted frizzled related proteins (Sfrp) and Znrf3 that act as inhibitors of the WNT signaling. We also show that mTORC1 pathway activation is an early event during neoplasia expansion and further demonstrate that mTORC1 pathway is activated in ACC patients. Preclinical inhibition of mTORC1 activity induces a marked reduction in tumor size, associated with induction of apoptosis and inhibition of proliferation that results in normalization of corticosterone plasma levels in AdTAg mice. Altogether, these data establish AdTAg mice as the first preclinical model for metastatic ACC.
- Published
- 2017
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38. Immunotactoid glomerulopathy leading to the discovery of POEMS syndrome .
- Author
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Philipponnet C, Kemeny JL, Garrouste C, Soubrier M, and Heng AE
- Subjects
- Adult, Female, Humans, Glomerulonephritis, Membranoproliferative, POEMS Syndrome, Paraproteinemias
- Abstract
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. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits. Due to an unfavorable progression, a second renal biopsy was performed. Electron microscopy analysis revealed an immunotactoid glomerulopathy. At the same time, a POEMS syndrome diagnosis (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities) was confirmed in light of the following: 1) IgG κ monoclonal gammopathy, 2) axonal neuropathy, 3) osteosclerosis, 4) melanoderma, 5) hepatosplenomegaly and adenopathies, 6) Castleman disease, and 7) edema. Our observation is the first case of immunotactoid glomerulopathy leading to the discovery of a POEMS syndrome. Renal involvement in POEMS syndrome typically exhibits a thrombotic microangiopathy-like membranoproliferative glomerulonephritis appearance associated with endothelial lesions stigmata. However, monoclonal immunoglobulin deposition disorder should be considered in the event of an atypical case. In this indication, electron microscopy is the examination of choice for assessing immunoglobulin deposition nephropathy. .
- Published
- 2017
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39. Active plantar fibromatosis occurring under anti-TNFα therapy for spondyloarthritis.
- Author
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Couderc M, Kemeny JL, Lhoste A, Soubrier M, and Dubost JJ
- Subjects
- Adalimumab therapeutic use, Adult, Dose-Response Relationship, Drug, Drug Administration Schedule, Etanercept therapeutic use, Female, Follow-Up Studies, Humans, Male, Risk Assessment, Severity of Illness Index, Spondylarthritis diagnosis, Tumor Necrosis Factor-alpha administration & dosage, Tumor Necrosis Factor-alpha antagonists & inhibitors, Adalimumab adverse effects, Etanercept adverse effects, Fibromatosis, Plantar chemically induced, Fibromatosis, Plantar diagnostic imaging, Magnetic Resonance Imaging methods, Spondylarthritis drug therapy
- Published
- 2017
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40. Global analysis of H3K27me3 as an epigenetic marker in prostate cancer progression.
- Author
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Ngollo M, Lebert A, Daures M, Judes G, Rifai K, Dubois L, Kemeny JL, Penault-Llorca F, Bignon YJ, Guy L, and Bernard-Gallon D
- Subjects
- Adult, Aged, Aged, 80 and over, Chromobox Protein Homolog 5, Discriminant Analysis, Disease Progression, Epigenesis, Genetic, Gene Expression Regulation, Neoplastic, Humans, Male, Middle Aged, Prognosis, Promoter Regions, Genetic, Prostatic Neoplasms genetics, DNA Methylation, Gene Regulatory Networks, Histones metabolism, Oligonucleotide Array Sequence Analysis methods, Prostatic Neoplasms pathology
- Abstract
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. H3K27me3 marks were assessed in 34 prostate tumors: 11 with Gleason score > 7 (GS > 7), 10 with Gleason score ≤ 7 (GS ≤ 7), and 13 morphologically normal prostate samples., Results: Here, H3K27me3 profiling identified an average of 386 enriched-genes on promoter regions in healthy control group versus 545 genes in GS ≤ 7 and 748 genes in GS > 7 group. We then ran a factorial discriminant analysis (FDA) and compared the enriched genes in prostate-tumor biopsies and normal biopsies using ANOVA to identify significantly differentially-enriched genes. The analysis identified ALG5, EXOSC8, CBX1, GRID2, GRIN3B, ING3, MYO1D, NPHP3-AS1, MSH6, FBXO11, SND1, SPATS2, TENM4 and TRA2A genes. These genes are possibly associated with prostate cancer. Notably, the H3K27me3 histone mark emerged as a novel regulatory mechanism in poor-prognosis prostate cancer., Conclusions: Our findings point to epigenetic mark H3K27me3 as an important event in prostate carcinogenesis and progression. The results reported here provide new molecular insights into the pathogenesis of prostate cancer.
- Published
- 2017
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41. Bile Cast Nephropathy Caused by Obstructive Cholestasis.
- Author
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Aniort J, Poyet A, Kemeny JL, Philipponnet C, and Heng AE
- Subjects
- Bile, Humans, Male, Middle Aged, Acute Kidney Injury etiology, Cholestasis complications
- Abstract
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. We describe a case of bile cast nephropathy in a patient with obstructive cholestasis caused by stones in the common bile duct. The kidney biopsy confirmed this diagnosis, with several green casts in tubular lumens, tubular injury, and bilirubin composition of the tubular casts with Hall stain. The patient had no confounding cause of kidney failure, and complete kidney recovery followed removal of the bile duct obstruction. This case shows that severe cholestasis is sufficient to cause AKI, and that AKI can be reversible after treatment of the biliary obstruction., (Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)
- Published
- 2017
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42. Mitotic index, microvascular proliferation, and necrosis define 3 pathological subgroups of prognostic relevance among 1p/19q co-deleted anaplastic oligodendrogliomas.
- Author
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Figarella-Branger D, Mokhtari K, Dehais C, Carpentier C, Colin C, Jouvet A, Uro-Coste E, Forest F, Maurage CA, Vignaud JM, Polivka M, Lechapt-Zalcman E, Eimer S, Viennet G, Quintin-Roué I, Aubriot-Lorton MH, Diebold MD, Loussouarn D, Lacroix C, Rigau V, Laquerrière A, Vandenbos F, Michalak S, Sevestre H, Peoch M, Labrousse F, Christov C, Kemeny JL, Chenard MP, Chiforeanu D, Ducray F, Idbaih A, and Delattre JY
- Subjects
- Brain Neoplasms blood supply, Brain Neoplasms genetics, Female, Humans, Isocitrate Dehydrogenase genetics, Male, Middle Aged, Necrosis, Oligodendroglioma blood supply, Oligodendroglioma genetics, Prognosis, Survival Rate, Brain Neoplasms pathology, Chromosome Deletion, Chromosomes, Human, Pair 1 genetics, Chromosomes, Human, Pair 19 genetics, Mitotic Index, Neovascularization, Pathologic, Oligodendroglioma pathology
- Published
- 2016
- Full Text
- View/download PDF
43. The JMJD3 Histone Demethylase and the EZH2 Histone Methyltransferase in Prostate Cancer.
- Author
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Daures M, Ngollo M, Judes G, Rifaï K, Kemeny JL, Penault-Llorca F, Bignon YJ, Guy L, and Bernard-Gallon D
- Subjects
- Adenocarcinoma pathology, Biomarkers, Tumor genetics, Cell Line, Tumor, Enhancer of Zeste Homolog 2 Protein, Gene Expression, Humans, Jumonji Domain-Containing Histone Demethylases genetics, Male, Neoplasm Grading, Polycomb Repressive Complex 2 genetics, Promoter Regions, Genetic, Prostatic Neoplasms pathology, Receptors, Retinoic Acid genetics, Adenocarcinoma enzymology, Biomarkers, Tumor metabolism, Jumonji Domain-Containing Histone Demethylases metabolism, Polycomb Repressive Complex 2 metabolism, Prostatic Neoplasms enzymology
- Published
- 2016
- Full Text
- View/download PDF
44. STAT3 Serine 727 Phosphorylation: A Relevant Target to Radiosensitize Human Glioblastoma.
- Author
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Ouédraogo ZG, Müller-Barthélémy M, Kemeny JL, Dedieu V, Biau J, Khalil T, Raoelfils LI, Granzotto A, Pereira B, Beaudoin C, Guissou IP, Berger M, Morel L, Chautard E, and Verrelle P
- Subjects
- Brain Neoplasms drug therapy, Carbazoles pharmacology, Cell Line, Tumor, Cell Proliferation drug effects, Cell Proliferation radiation effects, Colony-Forming Units Assay, Dose-Response Relationship, Drug, Enzyme Inhibitors pharmacology, Gene Expression Regulation, Neoplastic drug effects, Gene Expression Regulation, Neoplastic radiation effects, Glioblastoma drug therapy, Glioblastoma radiotherapy, Humans, Phosphorylation drug effects, Phosphorylation radiation effects, Radiation, Radiation Tolerance drug effects, Radiation Tolerance radiation effects, Signal Transduction drug effects, Signal Transduction radiation effects, Spectrophotometry, Statistics, Nonparametric, Time Factors, X-Rays, Brain Neoplasms metabolism, Brain Neoplasms radiotherapy, Glioblastoma metabolism, Glioblastoma pathology, STAT3 Transcription Factor metabolism, Serine metabolism
- Abstract
Radiotherapy is an essential component of glioma standard treatment. Glioblastomas (GBM), however, display an important radioresistance leading to tumor recurrence. To improve patient prognosis, there is a need to radiosensitize GBM cells and to circumvent the mechanisms of resistance caused by interactions between tumor cells and their microenvironment. STAT3 has been identified as a therapeutic target in glioma because of its involvement in mechanisms sustaining tumor escape to both standard treatment and immune control. Here, we studied the role of STAT3 activation on tyrosine 705 (Y705) and serine 727 (S727) in glioma radioresistance. This study explored STAT3 phosphorylation on Y705 (pSTAT3-Y705) and S727 (pSTAT3-S727) in glioma cell lines and in clinical samples. Radiosensitizing effect of STAT3 activation down-modulation by Gö6976 was explored. In a panel of 15 human glioma cell lines, we found that the level of pSTAT3-S727 was correlated to intrinsic radioresistance. Moreover, treating GBM cells with Gö6976 resulted in a highly significant radiosensitization associated to a concomitant pSTAT3-S727 down-modulation only in GBM cell lines that exhibited no or weak pSTAT3-Y705. We report the constitutive activation of STAT3-S727 in all GBM clinical samples. Targeting pSTAT3-S727 mainly in pSTAT3-Y705-negative GBM could be a relevant approach to improve radiation therapy., (© 2015 International Society of Neuropathology.)
- Published
- 2016
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45. Imaging features of systemic cystic angiomatosis.
- Author
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Marraoui W, Michel JL, Kemeny JL, and Soubrier M
- Subjects
- Diagnostic Imaging, Humans, Male, Middle Aged, Angiomatosis diagnosis
- Published
- 2015
- Full Text
- View/download PDF
46. [Uveitis associated with nephrotic syndrome: Initial manifestations of secondary syphilis].
- Author
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Bons O, Bonnin N, Bacin F, Albaret J, Tiple A, Heng AE, Kemeny JL, and Chiambaretta F
- Subjects
- Adult, Humans, Male, Syphilis complications, Nephrotic Syndrome etiology, Syphilis diagnosis, Uveitis etiology
- Published
- 2015
- Full Text
- View/download PDF
47. [Case report of cholesterolosis bulbi in a Coats disease].
- Author
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Huard M, Bonnin N, Bacin F, Kemeny JL, Desjardins L, and Chiambaretta F
- Subjects
- Anterior Chamber diagnostic imaging, Anterior Chamber metabolism, Corneal Edema etiology, Diagnosis, Differential, Diagnostic Techniques, Ophthalmological, Eye Enucleation, Eye Neoplasms diagnosis, Humans, Infant, Male, Retinal Telangiectasis complications, Retinal Telangiectasis metabolism, Retinal Telangiectasis pathology, Retinal Telangiectasis surgery, Retinoblastoma diagnosis, Ultrasonography, Anterior Chamber pathology, Cholesterol metabolism, Retinal Detachment etiology, Retinal Telangiectasis diagnosis
- Published
- 2015
- Full Text
- View/download PDF
48. Prognostic Relevance of Histomolecular Classification of Diffuse Adult High-Grade Gliomas with Necrosis.
- Author
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Figarella-Branger D, Mokhtari K, Colin C, Uro-Coste E, Jouvet A, Dehais C, Carpentier C, Villa C, Maurage CA, Eimer S, Polivka M, Vignaud JM, Laquerriere A, Sevestre H, Lechapt-Zalcman E, Quintin-Roué I, Aubriot-Lorton MH, Diebold MD, Viennet G, Adam C, Loussouarn D, Michalak S, Rigau V, Heitzmann A, Vandenbos F, Forest F, Chiforeanu D, Tortel MC, Labrousse F, Chenard MP, Nguyen AT, Varlet P, Kemeny JL, Levillain PM, Cazals-Hatem D, Richard P, and Delattre JY
- Subjects
- Adult, Brain Neoplasms classification, Chromosome Aberrations, Chromosome Deletion, Chromosomes, Human, Pair 1 genetics, ErbB Receptors genetics, Female, Follow-Up Studies, Gene Expression Profiling, Glioma classification, Humans, Isocitrate Dehydrogenase genetics, Ki-67 Antigen metabolism, Male, Middle Aged, Mutation genetics, Necrosis, Oligonucleotide Array Sequence Analysis, Prognosis, Survival Analysis, Brain Neoplasms diagnosis, Brain Neoplasms genetics, Brain Neoplasms metabolism, Glioma diagnosis, Glioma genetics, Glioma metabolism
- Abstract
Diffuse adult high-grade gliomas (HGGs) with necrosis encompass anaplastic oligodendrogliomas (AOs) with necrosis (grade III), glioblastomas (GBM, grade IV) and glioblastomas with an oligodendroglial component (GBMO, grade IV). Here, we aimed to search for prognostic relevance of histological classification and molecular alterations of these tumors. About 210 patients were included (63 AO, 56 GBM and 91 GBMO). GBMO group was split into "anaplastic oligoastrocytoma (AOA) with necrosis grade IV/GBMO," restricted to tumors showing intermingled astrocytic and oligodendroglial component, and "GBM/GBMO" based on tumors presenting oligodendroglial foci and features of GBM. Genomic arrays, IDH1 R132H expression analyses and IDH direct sequencing were performed. 1p/19q co-deletion characterized AO, whereas no IDH1 R132H expression and intact 1p/19q characterized both GBM and GBM/GBMO. AOA with necrosis/GBMO mainly demonstrated IDH1 R132H expression and intact 1p/19q. Other IDH1 or IDH2 mutations were extremely rare. Both histological and molecular classifications were predictive of progression free survival (PFS) and overall survival (OS) (P < 10(-4) ). Diffuse adult HGGs with necrosis can be split into three histomolecular groups of prognostic relevance: 1p/19q co-deleted AO, IDH1 R132H-GBM and 1p/19q intact IDH1 R132H+ gliomas that might be classified as IDH1 R132H+ GBM. Because of histomolecular heterogeneity, we suggest to remove the name GBMO., (© 2014 International Society of Neuropathology.)
- Published
- 2015
- Full Text
- View/download PDF
49. [Intraocular lymphoma associated with primary malignant lymphoma of the central nervous system: Seven-year experience of a tertiary center].
- Author
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Joubert R, Bonnin N, Kemeny JL, Moluçon-Chabrot C, Tournilhac O, Bacin F, and Chiambaretta F
- Subjects
- Aged, Aged, 80 and over, Brain Neoplasms mortality, Brain Neoplasms pathology, Combined Modality Therapy, Eye Neoplasms mortality, Eye Neoplasms pathology, Female, Humans, Lymphoma, Large B-Cell, Diffuse mortality, Lymphoma, Large B-Cell, Diffuse pathology, Lymphoma, T-Cell mortality, Lymphoma, T-Cell pathology, Male, Middle Aged, Neoplasms, Multiple Primary mortality, Neoplasms, Multiple Primary pathology, Survival Rate, Brain Neoplasms diagnosis, Brain Neoplasms therapy, Eye Neoplasms diagnosis, Eye Neoplasms therapy, Lymphoma, Large B-Cell, Diffuse diagnosis, Lymphoma, Large B-Cell, Diffuse therapy, Lymphoma, T-Cell diagnosis, Lymphoma, T-Cell therapy, Neoplasms, Multiple Primary diagnosis, Neoplasms, Multiple Primary therapy, Tertiary Care Centers
- Abstract
Introduction: Primary intraocular lymphoma (PIOL), associated with primary central nervous system lymphoma (PCNSL), is a rare malignancy disease. By way of a seven-year experience of a tertiary center, we discuss the presentation and we review the diagnostic and therapeutic modalities., Observations: We report six cases of PIOL associated with PCNSL. For all patients, the clinical presentation was a vitreoretinal syndrome. The diagnosis was histologically confirmed by vitreal sample or brain biopsy. Five patients developed a diffuse large B-cell lymphoma. Only one patient developed a T-cell lymphoma. The treatment consisted of conformational radiation therapy, systemic chemotherapy and intravitreal injections of methotrexate. The median survival after the diagnosis was 24 months., Discussion: PIOL, associated with PCNSL, is the most common type of ocular lymphoma. In most cases, ocular manifestations inaugurate the disease. PIOL is often fatal because of ultimate central nervous system presentation. The role of the ophthalmologist consists in early diagnosis. Typical clinical findings include vitroretinal tumor syndrome but can mascarade other eye pathologies. Diagnosis requires histology. The majority of PIOL is diffused large B-cell lymphoma. Decisions are made through multidisciplinary consultation. PIOL exhibits high responsiveness to methotrexate., Conclusion: Through a literature review and many illustrations, we discuss epidemiological, clinical, histological, radiological and treatment characteristics of PIOL associated with PCNSL., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)
- Published
- 2015
- Full Text
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50. Infantile orbital myofibroma.
- Author
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Mbekeani JN, Kemeny JL, and Nezzar H
- Published
- 2015
- Full Text
- View/download PDF
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