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101. Transplantation in patients with SCID: mismatched related stem cells or unrelated cord blood?

Author

Fernandes, Jf, Rocha, V, Labopin, M, Neven, B, Moshous, D, Gennery, Ar, Friedrich, W, Porta, F, Diaz de Heredia, C, Wall, D, Bertrand, Y, Veys, P, Slatter, M, Schulz, A, Chan, Kw, Grimley, M, Ayas, M, Gungor, T, Ebell, W, Bonfim, C, Kalwak, K, Taupin, P, Blanche, S, Gaspar, Hb, Landais, P, Fischer, A, Gluckman, E, Cavazzana Calvo, M, Eurocord, Inborn Errors Working Party of European Group for Blood, Marrow Transplantation: Ahmed, A, Auiti, A, Biffi, A, Cant, A, Fasth, A, Gennery, A, Hassan, A, Lankester, A, O'Mera, A, Plabani, A, Rovelli, A, Salmon, A, Scarselli, A, Thrasher, A, Van Royen, A, Villa, A, Wawer, A, Wahadneh, A, Worth, A, Belohradsky, B, Wolska, B, Gaspar, B, Bonfirm, C, Booth, C, Klein, C, Messina, C, Peters, C, Steward, C, Lindemans, C, Schuetz, C, de Heredia Rubio CD, Bensoussan, D, Gleadow, D, Lilic, D, Gambineri, Eleonora, Smith, E, Aerts, F, Caracseghi, F, Roberts, G, Davies, G, Al Mousa, H, Jossanc, H, Ozsahim, H, Hirsch, I, Meyts, I, Tezcan, I, Mueller, I, Andresc, I, Boelens, J, Fernandes, J, Folloni, J, Keuhl, J, Reichenbach, J, Stary, J, Wachowiak, J, Xu Bayford, J, Cunha, Jm, Ehlert, J, Rao, K, Sykora, K, Andais, L, Brown, L, Dal Cortivo, L, Griffith, L, Notarangelo, L, Abinun, M, Albert, M, Bierings, M, Bouchet, M, Cavazzana, M, Hirschfield, M, Cowan, M, Hoenig, M, Loubser, M, Roncarolo, M, Sauer, M, Schneider, M, Verstegen, M, Schroeder, M, Essink, M, Yesilipek, M, Entz Werle, N, Mahlaoui, N, Schlautmann, N, Taylor, N, Vanroyen, N, Walffraat, N, Sanal, O, Amrolia, P, Bordigoni, P, De Coppi, P, Frange, P, Orchard, P, Sedlacek, P, Shaw, P, Stephensky, P, Bacchetta, R, Bredius, R, Formankova, R, Gale, R, Seger, R, Wynn, R, Corbacioglu, S, Ehl, S, Hacein Bey, S, Hambleton, S, Mohsen, S, Mueller, S, Pai, Sy, Espanol, T, Flood, T, Guengoer, T, Bordon, V, Ormoor, V, Pashano, V, Courteille, V, Czogala, W, Qasim, W, Camci, Y, and Nademi, Z.

Subjects
Male, medicine.medical_specialty, Transplantation Conditioning, medicine.medical_treatment, Immunology, Graft vs Host Disease, Kaplan-Meier Estimate, Cord Blood Stem Cell Transplantation, Hematopoietic stem cell transplantation, Biochemistry, Gastroenterology, SCID HSCT, Internal medicine, medicine, Humans, Proportional Hazards Models, Retrospective Studies, Preparative Regimen, Severe combined immunodeficiency, business.industry, Umbilical Cord Blood Transplantation, Incidence, Hematopoietic Stem Cell Transplantation, Infant, Newborn, Infant, Cell Biology, Hematology, medicine.disease, Surgery, Transplantation, Treatment Outcome, Child, Preschool, Histocompatibility, Cord blood, Female, Severe Combined Immunodeficiency, business
Abstract

Pediatric patients with SCID constitute medical emergencies. In the absence of an HLA-identical hematopoietic stem cell (HSC) donor, mismatched related-donor transplantation (MMRDT) or unrelated-donor umbilical cord blood transplantation (UCBT) are valuable treatment options. To help transplantation centers choose the best treatment option, we retrospectively compared outcomes after 175 MMRDTs and 74 UCBTs in patients with SCID or Omenn syndrome. Median follow-up time was 83 months and 58 months for UCBT and MMRDT, respectively. Most UCB recipients received a myeloablative conditioning regimen; most MMRDT recipients did not. UCB recipients presented a higher frequency of complete donor chimerism (P = .04) and faster total lymphocyte count recovery (P = .04) without any statistically significance with the preparative regimen they received. The MMRDT and UCBT groups did not differ in terms of T-cell engraftment, CD4+ and CD3+ cell recoveries, while Ig replacement therapy was discontinued sooner after UCBT (adjusted P = .02). There was a trend toward a greater incidence of grades II-IV acute GVHD (P = .06) and more chronic GVHD (P = .03) after UCBT. The estimated 5-year overall survival rates were 62% ± 4% after MMRDT and 57% ± 6% after UCBT. For children with SCID and no HLA-identical sibling donor, both UCBT and MMRDT represent available HSC sources for transplantation with quite similar outcomes.

Published
2012

102. An international registry on autoinflammatory diseases: the Eurofever experience

Author

Toplak, N., Frenkel, J., Ozen, S., Lachmann, H.J., Woo, P., Kone-Paut, I., Benedetti, F. De, Neven, B., Hofer, M., Dolezalova, P., Kummerle-Deschner, J., Touitou, I., Hentgen, V., Simon, A., Girschick, H., Rose, C., Wouters, C., Vesely, R., Arostegui, J., Stojanov, S., Ozgodan, H., Martini, A., Ruperto, N., Gattorno, M., Paediatric Rheumatology International Trials Organisation, E., and Eurofever, P.

Subjects
Male, Pediatrics, International Cooperation, Familial Mediterranean fever, Disease, Global Health, 0302 clinical medicine, Global health, Immunology and Allergy, Registries, Young adult, Child, Aged, 80 and over, 0303 health sciences, Behcet Syndrome, Middle Aged, Familial Mediterranean Fever, 3. Good health, Pathogenesis and modulation of inflammation [N4i 1], Child, Preschool, Female, Adult, Heterozygote, medicine.medical_specialty, Adolescent, Immunology, MEDLINE, Genes, Recessive, Context (language use), General Biochemistry, Genetics and Molecular Biology, Young Adult, 03 medical and health sciences, Rheumatology, Internal medicine, medicine, Humans, Genetic Predisposition to Disease, Aged, Demography, 030304 developmental biology, 030203 arthritis & rheumatology, Internet, business.industry, Hereditary Autoinflammatory Diseases, Infant, medicine.disease, Cryopyrin-Associated Periodic Syndromes, Clinical diagnosis, business
Abstract

Item does not contain fulltext OBJECTIVE: To report on the demographic data from the first 18 months of enrollment to an international registry on autoinflammatory diseases in the context of the Eurofever project. METHODS: A web-based registry collecting baseline and clinical information on autoinflammatory diseases and related conditions is available in the member area of the PRINTO web-site. Anonymised data were collected with standardised forms. RESULTS: 1880 (M:F=916:964) individuals from 67 centers in 31 countries have been entered in the Eurofever registry. Most of the patients (1388; 74%), reside in western Europe, 294 (16%) in the eastern and southern Mediterranean region (Turkey, Israel, North Africa), 106 (6%) in eastern Europe, 54 in Asia, 27 in South America and 11 in Australia. In total 1049 patients with a clinical diagnosis of a monogenic autoinflammatory diseases have been enrolled; genetic analysis was performed in 993 patients (95%): 703 patients have genetically confirmed disease and 197 patients are heterozygous carriers of mutations in genes that are mutated in patients with recessively inherited autoinflammatory diseases. The median diagnosis delay was 7.3 years (range 0.3-76), with a clear reduction in patients born after the identification of the first gene associated with autoinflammatory diseases in 1997. CONCLUSIONS: A shared online registry for patients with autoinflammatory diseases is available and enrollment is ongoing. Currently, there are data available for analysis on clinical presentation, disease course, and response to treatment, and to perform large scale comparative studies between different conditions.

Published
2012

103. Occurrence of myelodysplastic syndrome in 2 patients with reticular dysgenesis

Author

Lagresle-Peyrou, C., Neven, B., Six, E., Picard, C., Chappedelaine C., Demerens-De, Bertrand, Yves, Jabado, N., Chomienne, C., Radford-Weiss, I., Brouzes, C., Asnafi, V., Macintyre, E., Donadieu, J., Beaupain, B., Fenaux, P., Eclache, V., Fischer, A., Cavazzana-Calvo, M., Cytokines, hématopoïèse et réponse immune (CHRI), Université Paris Descartes - Paris 5 (UPD5) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Centre National de la Recherche Scientifique (CNRS), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and Slama, Catherine

Subjects
ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2011

107. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients

Author

Hofer, M, Pillet, P, Cochard, Mm, Berg, S, Krol, P, Kone Paut, I, Rigante, Donato, Hengten, V, Anton, J, Brik, R, Neven, B, Touitou, I, Kaiser, D, Duquesne, A, Wouters, C, Gattorno, M., Rigante, Donato (ORCID:0000-0001-7032-7779), Hofer, M, Pillet, P, Cochard, Mm, Berg, S, Krol, P, Kone Paut, I, Rigante, Donato, Hengten, V, Anton, J, Brik, R, Neven, B, Touitou, I, Kaiser, D, Duquesne, A, Wouters, C, Gattorno, M., and Rigante, Donato (ORCID:0000-0001-7032-7779)

Abstract

OBJECTIVES: The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of patients from different countries. METHODS: We established a web-based multicentre cohort through an international collaboration within the periodic fevers working party of the Pediatric Rheumatology European Society (PReS). The inclusion criterion was a diagnosis of PFAPA given by an experienced paediatric rheumatologist participating in an international working group on periodic fever syndromes. RESULTS: Of the 301 patients included from the 15 centres, 271 had pharyngitis, 236 cervical adenitis, 171 oral aphthosis and 132 with all three clinical features. A total of 228 patients presented with additional symptoms (131 gastrointestinal symptoms, 86 arthralgias and/or myalgias, 36 skin rashes, 8 neurological symptoms). Thirty-one patients had disease onset after 5 years and they reported more additional symptoms. A positive family history for recurrent fever or recurrent tonsillitis was found in 81 patients (26.9%). Genetic testing for monogenic periodic fever syndromes was performed on 111 patients, who reported fewer occurrences of oral aphthosis or additional symptoms. Twenty-four patients reported symptoms (oral aphthosis and malaise) outside the flares. The CRP was >50 mg/l in the majority (131/190) of the patients tested during the fever. CONCLUSION: We describe the largest cohort of PFAPA patients presented so far. We confirm that PFAPA may present with varied clinical manifestations and we show the limitations of the commonly used diagnostic criteria. Based on detailed analysis of this cohort, a consensus definition of PFAPA with better-defined criteria should be proposed.

Published
2014

113. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients

Author

Hofer, M., primary, Pillet, P., additional, Cochard, M.-M., additional, Berg, S., additional, Krol, P., additional, Kone-Paut, I., additional, Rigante, D., additional, Hentgen, V., additional, Anton, J., additional, Brik, R., additional, Neven, B., additional, Touitou, I., additional, Kaiser, D., additional, Duquesne, A., additional, Wouters, C., additional, and Gattorno, M., additional

Published
2014
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114. Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review

Author

Haar, N. Ter, Lachmann, H., Ozen, S., Woo, P., Uziel, Y., Modesto, C., Kone-Paut, I., Cantarini, L., Insalaco, A., Neven, B., Hofer, M., Rigante, D., Al-Mayouf, S., Touitou, I., Gallizzi, R., Papadopoulou-Alataki, E., Martino, S., Kuemmerle-Deschner, J., Obici, L., Iagaru, N., Simon, A., Nielsen, S., Martini, A., Ruperto, N., Gattorno, M., Frenkel, J., et al., Haar, N. Ter, Lachmann, H., Ozen, S., Woo, P., Uziel, Y., Modesto, C., Kone-Paut, I., Cantarini, L., Insalaco, A., Neven, B., Hofer, M., Rigante, D., Al-Mayouf, S., Touitou, I., Gallizzi, R., Papadopoulou-Alataki, E., Martino, S., Kuemmerle-Deschner, J., Obici, L., Iagaru, N., Simon, A., Nielsen, S., Martini, A., Ruperto, N., Gattorno, M., Frenkel, J., and et al.

Abstract

Item does not contain fulltext, OBJECTIVE: To evaluate the response to treatment of autoinflammatory diseases from an international registry and an up-to-date literature review. METHODS: The response to treatment was studied in a web-based registry in which clinical information on anonymised patients with autoinflammatory diseases was collected retrospectively as part of the Eurofever initiative. Participating hospitals included paediatric rheumatology centres of the Paediatric Rheumatology International Trial Organisation network and adult centres with a specific interest in autoinflammatory diseases. The following diseases were included: familial Mediterranean fever (FMF), cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor (TNF)-receptor associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), pyogenic arthritis pustulosis acne (PAPA) syndrome, deficiency of interleukin-1 receptor antagonist (DIRA), NLRP12-related periodic fever and periodic fever aphthosis pharyngitis adenitis (PFAPA) syndrome. Cases were independently validated by experts for each disease. A literature search regarding treatment of the abovementioned diseases was also performed using Medline and Embase. RESULTS: 22 months from the beginning of the enrolment, complete information on 496 validated patients was available. Data from the registry in combination with evidence from the literature confirmed that colchicine is the treatment of choice for FMF and IL-1 blockade for DIRA and CAPS. Corticosteroids on demand probably represent a valid therapeutic strategy for PFAPA, but also for MKD and TRAPS. Patients with poorly controlled MKD, TRAPS, PAPA or FMF may benefit from IL-1 blockade; anti-TNF treatment may represent a possible valuable alternative. CONCLUSIONS: In the absence of high-grade evidence, these results could serve as a basis for therapeutic guidelines and to identify candidate drugs for future therapeutic trials.

Published
2013

115. Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review

Author

Ter Haar, N, Lachmann, H, Özen, S, Woo, P, Uziel, Y, Modesto, C, Koné Paut, I, Cantarini, L, Insalaco, A, Neven, B, Hofer, M, Rigante, D, Al Mayouf, S, Touitou, I, Gallizzi, R, Papadopoulou Alataki, E, Martino, S, Kuemmerle Deschner, J, Obici, L, Iagaru, N, Simon, A, Martini, A, Manna, Raffaele, Ruperto, N, Gattorno, M, Frenkel, J., Manna, Raffaele (ORCID:0000-0003-1560-3907), Ter Haar, N, Lachmann, H, Özen, S, Woo, P, Uziel, Y, Modesto, C, Koné Paut, I, Cantarini, L, Insalaco, A, Neven, B, Hofer, M, Rigante, D, Al Mayouf, S, Touitou, I, Gallizzi, R, Papadopoulou Alataki, E, Martino, S, Kuemmerle Deschner, J, Obici, L, Iagaru, N, Simon, A, Martini, A, Manna, Raffaele, Ruperto, N, Gattorno, M, Frenkel, J., and Manna, Raffaele (ORCID:0000-0003-1560-3907)

Abstract

To evaluate the response to treatment of autoinflammatory diseases from an international registry and an up-to-date literature review.

Published
2013

116. PReS-FINAL-2194: Evidence-based clinical classification criteria for periodic fevers

Author

Federici, S, primary, Ozen, S, additional, Koné-Paut, I, additional, Lachmann, H, additional, Woo, P, additional, Cantarini, L, additional, Amaryan, G, additional, Insalaco, A, additional, Kuemmerle-Deschner, J, additional, Neven, B, additional, Dewarrat, N, additional, Uziel, Y, additional, Rigante, D, additional, Herlin, T, additional, Martino, S, additional, Simon, A, additional, Stojanov, S, additional, Ozdogan, H, additional, Frenkel, J, additional, Ruperto, N, additional, Martini, A, additional, Sormani, MP, additional, Hofer, M, additional, and Gattorno, M, additional

Published
2013
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117. PW02-037 - The Eurofever cohort of 136 patients with CAPS

Author

Levy, R, primary, Gerard, L, additional, Kuemmerle-Deschner, J, additional, Lachmann, H, additional, Koné-Paut, I, additional, Cantarini, L, additional, Woo, P, additional, Naselli, A, additional, Bader-Meunier, B, additional, De Benedetti, F, additional, Al-Mayouf, SM, additional, Ozen, S, additional, Hofer, M, additional, Frenkel, J, additional, Modesto, C, additional, Nikishina, I, additional, Martini, A, additional, Ruperto, N, additional, Neven, B, additional, and Gattorno, M, additional

Published
2013
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119. OR13-004 – Evidence-based clinical classification criteria for periodic fevers

Author

Federici, S, primary, Ozen, S, additional, Koné-Paut, I, additional, Lachmann, H, additional, Woo, P, additional, Cantarini, L, additional, Amaryan, G, additional, Insalaco, A, additional, Kuemmerle-Deschner, J, additional, Neven, B, additional, Dewarrat, N, additional, Uziel, Y, additional, Rigante, D, additional, Herlin, T, additional, Martino, S, additional, Simon, A, additional, Stojanov, S, additional, Ozdogan, H, additional, Frenkel, J, additional, Ruperto, N, additional, Martini, A, additional, Sormani, M, additional, Hofer, M, additional, and Gattorno, M, additional

Published
2013
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120. PW02-007 - The Eurofever registry: 3 years of enrollment

Author

Federici, S, primary, Frenkel, J, additional, Ozen, S, additional, Jordi, A, additional, Arostegui, JI, additional, De Benedetti, F, additional, Dolezalova, P, additional, Girschick, H, additional, Hentgen, V, additional, Hofer, M, additional, Lachmann, H, additional, Koné-Paut, I, additional, Kuemmerle-Deschner, J, additional, Neven, B, additional, Ozdogan, H, additional, Rose, C, additional, Simon, A, additional, Stojanov, S, additional, Toplak, N, additional, Touitou, I, additional, Vesely, R, additional, Woo, P, additional, Wouters, C, additional, Ruperto, N, additional, Martini, A, additional, and Gattorno, M, additional

Published
2013
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122. A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference.

Author

Piram, M., Frenkel, J., Gattorno, M., Ozen, S., Lachmann, H.J., Goldbach-Mansky, R., Hentgen, V., Neven, B., Stojanovic, K.S., Simon, A., Kuemmerle-Deschner, J., Hoffman, H., Stojanov, S., Duquesne, A., Pillet, P., Martini, A., Pouchot, J., Kone-Paut, I., Piram, M., Frenkel, J., Gattorno, M., Ozen, S., Lachmann, H.J., Goldbach-Mansky, R., Hentgen, V., Neven, B., Stojanovic, K.S., Simon, A., Kuemmerle-Deschner, J., Hoffman, H., Stojanov, S., Duquesne, A., Pillet, P., Martini, A., Pouchot, J., and Kone-Paut, I.

Abstract

1 februari 2011, Contains fulltext : 95590.pdf (publisher's version ) (Closed access), BACKGROUND: The systemic autoinflammatory disorders (SAID) share many clinical manifestations, albeit with variable patterns, intensity and frequency. A common definition of disease activity would be rational and useful in the management of these lifelong diseases. Moreover, standardised disease activity scores are required for the assessment of new therapies in constant development. The aim of this study was to develop preliminary activity scores for familial Mediterranean fever, mevalonate kinase deficiency, tumour necrosis factor receptor-1-associated periodic syndrome and cryopyrin-associated periodic syndromes (CAPS). METHODS: The study was conducted using two well-recognised consensus formation methods: the Delphi technique and the nominal group technique. The results from a two-step survey and data from parent/patient interviews were used as preliminary data to develop the agenda for a consensus conference to build a provisional scoring system. RESULTS: 24 of 65 experts in SAID from 20 countries answered the web questionnaire and 16 attended the consensus conference. There was consensus agreement to develop separate activity scores for each disease but with a common format based on patient diaries. Fever and disease-specific clinical variables were scored according to their severity. A final score was generated by summing the score of all the variables divided by the number of days over which the diary was completed. Scores varied from 0 to 16 (0-13 in CAPS). These scores were developed for the purpose of clinical studies but could be used in clinical practice. CONCLUSION: Using widely recognised consensus formation techniques, preliminary scores were obtained to measure disease activity in four main SAID. Further prospective validation study of this instrument will follow.

Published
2011

123. Clinical and immunologic outcome of patients with cartilage hair hypoplasia after hematopoietic stem cell transplantation

Author

Bordon, V, Gennery, A R, Slatter, M A, Vandecruys, E, Laureys, G, Veys, P, Friedrich, W, Qasim, W, Wulfraat, N M, Scherer, F, Cant, A J, Fischer, A, Cavazanna-Calvo, M, Bredius, R G M, Notarangelo, L D, Mazzolari, E, Neven, B, Güngör, Tayfun; https://orcid.org/0000-0002-3261-1186, Bordon, V, Gennery, A R, Slatter, M A, Vandecruys, E, Laureys, G, Veys, P, Friedrich, W, Qasim, W, Wulfraat, N M, Scherer, F, Cant, A J, Fischer, A, Cavazanna-Calvo, M, Bredius, R G M, Notarangelo, L D, Mazzolari, E, Neven, B, and Güngör, Tayfun; https://orcid.org/0000-0002-3261-1186

Abstract

Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disease caused by mutations in the RMRP gene. Beside dwarfism, CHH has a wide spectrum of clinical manifestations including variable grades of combined immunodeficiency, autoimmune complications, and malignancies. Previous reports in single CHH patients with significant immunodeficiencies have demonstrated that allogeneic hematopoietic stem cell transplantation (HSCT) is an effective treatment for the severe immunodeficiency, while growth failure remains unaffected. Because long-term experience in larger cohorts of CHH patients after HSCT is currently unreported, we performed a European collaborative survey reporting on 16 patients with CHH and immunodeficiency who underwent HSCT. Immune dysregulation, lymphoid malignancy, and autoimmunity were important features in this cohort. Thirteen patients were transplanted in early childhood ( approximately 2.5 years). The other 3 patients were transplanted at adolescent age. Of 16 patients, 10 (62.5%) were long-term survivors, with a median follow-up of 7 years. T-lymphocyte numbers and function have normalized, and autoimmunity has resolved in all survivors. HSCT should be considered in CHH patients with severe immunodeficiency/autoimmunity, before the development of severe infections, major organ damage, or malignancy might jeopardize the outcome of HSCT and the quality of life in these patients.

Published
2010

124. PFAPA (periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis) syndrome registry: analysis of a cohort of 214 patients

Author

Hofer, M, Pillet, P, Berg, S, Brik, R, Dolezalova, P, Kone Paut, I, Anton, J, Touitou, I, Neven, B, Kaiser, D, Rigante, Donato, Duquesne, A, Wouters, C, Gattorno, M., Rigante, Donato (ORCID:0000-0001-7032-7779), Hofer, M, Pillet, P, Berg, S, Brik, R, Dolezalova, P, Kone Paut, I, Anton, J, Touitou, I, Neven, B, Kaiser, D, Rigante, Donato, Duquesne, A, Wouters, C, Gattorno, M., and Rigante, Donato (ORCID:0000-0001-7032-7779)

Abstract

A multi-centre cohort of children with PFAPA syndrome is herein presented. The challenges associated with the discovery of pathogenetic pathways in PFAPA syndrome are immense, reflected by the small number of studies currently in progress. As new mechanisms underlying the regulation of innate immunity are discovered and novel therapeutic approaches are adopted, the next decade promises many exciting advances to manage and treat patients with this disorder.

Published
2008

127. Safety of hematopoietic stem cell transplantation from hepatitis B core antibodies-positive donors with low/undetectable viremia in HBV-naïve children

Author

Frange, P., primary, Leruez-Ville, M., additional, Neven, B., additional, Mascard, L., additional, Moshous, D., additional, Touzot, F., additional, Heritier, S., additional, Chaix, M.-L., additional, Cavazzana, M., additional, Casanova, J.-L., additional, Fischer, A., additional, and Blanche, S., additional

Published
2013
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130. OP0175 The eurofever registry for autoinflammatory disease: Update on enrollment after 2 years

Author

Naselli, A., primary, Frenkel, J., additional, Ozen, S., additional, Konè-Paut, I., additional, Lachmann, H., additional, Woo, P., additional, De Benedetti, F., additional, Hofer, M., additional, Neven, B., additional, Dolezalova, P., additional, Kümmerle-Deschner, J., additional, Hentgen, V., additional, Touitou, I., additional, Simon, A., additional, Wouters, C., additional, Toplak, N., additional, Anton, J., additional, Stojanov, S., additional, Girschick, H., additional, Vesely, R., additional, Arostegui, J.I., additional, Rose, C., additional, Ozdogan, H., additional, Ruperto, N., additional, Martini, A., additional, and Gattorno, M., additional

Published
2013
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135. Efficacy, safety and effect on gene expression profiling of anakinra in systemic-onset juvenile idiopathic arthritis: final results of a randomised, double-blind, placebo-controlled trial (ANAJIS)

Author

Quartier, P, primary, Allantaz, F, additional, Cimaz, R, additional, Pillet, P, additional, Richer, O, additional, Desjonqueres, M, additional, Duquesne, A, additional, Messiaen, C, additional, Bardin, C, additional, Bossuyit, X, additional, Boutten, A, additional, Bienvenu, J, additional, Menoni, V, additional, Sotou-Bere, S, additional, Neven, B, additional, Mahloui, N, additional, Mogenet, A, additional, Kassai, B, additional, Chaussabel, D, additional, Treluyer, JM, additional, Bresson, JL, additional, Landais, P, additional, and Pascual, V, additional

Published
2008
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136. SFP-48 – Pathologie osseuse et rhumatologie – Anakinra dans la forme systémique d’arthrite juvénile idiopathique

Author

Quartier, P., primary, Allantaz, F., additional, Cimaz, R., additional, Pillet, P., additional, Desjonqueres, M., additional, Richer, O., additional, Duquesne, A., additional, Lemelle, I., additional, Koné-Paut, I., additional, Neven, B., additional, Bardin, C., additional, Dimet, I., additional, Bienvenu, J., additional, Boutten, A., additional, Bossuyt, X., additional, Messiaen, C., additional, Menoni, V., additional, Mogenet, A., additional, Treluyer, J.-M., additional, Bresson, J.-L., additional, Landais, P., additional, and Pascual, V., additional

Published
2008
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137. Prospective validation of a novel IV busulfan fixed dosing for paediatric patients to improve therapeutic AUC targeting without drug monitoring

Author

Vassal, G., primary, Michel, G., additional, Espérou, H., additional, Gentet, J. C., additional, Valteau-Couanet, D., additional, Doz, F., additional, Mechinaud, F., additional, Galambrun, C., additional, Neven, B., additional, Zouabi, H., additional, Nguyen, L., additional, and Puozzo, C., additional

Published
2007
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138. Adoptive Immunotherapy with Donor Allodepleted T Cells.

Author

Dal Cortivo, L., primary, Mahlaoui, N., primary, Picard, C., primary, Neven, B., primary, Andre-Schmutz, I., primary, Luby, J.M., primary, Martinache, C., primary, Paquet, T., primary, Ledeist, F., primary, Fischer, A., primary, and Cavazzana-Calvo, M., primary

Published
2005
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144. Morphometry and Epidermal Fas Expression of Unexposed Aged Versus Young Skin.

Author

Abd El-Aal, Nagwa H., Abd El-Wadood, Faten A., Moftah, Nayera H., El-Hakeem, Mohamed S., El-Shaal, Abdalla Y., and Hassan, Neven B.

Subjects
AGE distribution, AGING, CHI-squared test, COLLAGEN, CONNECTIVE tissues, STATISTICAL correlation, ECOLOGY, GENETICS, IMMUNOHISTOCHEMISTRY, SKIN, T-test (Statistics), DATA analysis software
Abstract

Background: Identifying the molecular mechanisms of intrinsic aging is critical in developing modalities for reversal of cutaneous aging. Objective: The objective was to evaluate the expression of epidermal Fas, epidermal thickness, collagen, and elastic fibers degeneration in unexposed skin of aged individuals compared with young ones. Materials and Methods: Skin biopsies were taken from normal skin of the back of 22 old subjects (age range: 48-75 years) and 15 young subjects (age range: 18-28 years). Skin sections were stained with hematoxylin and eosin, Masson trichrome, orcein. Epidermal thickness was measured with image analyzer and scoring was done for collagen and elastic fiber degeneration. Fas immunostaining was done. Quantitative and qualitative data were compared statistically between the old and young subjects. Results: A statistically significant decreased epidermal thickness was found in old compared with young skin (P<0.05). A statistically significant number of patients showed decreased epidermal thickness, density, and fragmentation of both collagen and elastic fibers in old compared with young skin (P<0.001). Epidermal Fas expression was detected in 19 of 22 old subjects (86.4%) compared with 2 of 15 young subjects (13.3%) (P<0.001). There was no statistically significant correlation between age of old subjects and each of epidermal thickness, collagen, and elastic fiber degeneration. Conclusion: The decreased epidermal thickness and morphological alteration of collagen and elastic fibers are not correlated with aging and Fas-mediated apoptosis could be involved in thinning of the epidermis in unexposed aged skin. [ABSTRACT FROM AUTHOR]

Published
2012
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146. Toxicity of Dimethoate to Daphnia magna and freshwater fish.

Author

Beusen, J. and Neven, B.

Subjects
FRESHWATER fishes, DAPHNIA magna, TOXICITY testing, DIMETHOATE, ZEBRA danio, GUPPIES
Abstract

The article presents a study which investigates the toxicity of dimethoate to two freshwater fishes such as guppy Poecilia reticulate and zebrafish Brachydanio rerio and Daphnia magna Straus. The recommended guidelines from the Commission of the European Communities was used to examine the acute toxicity tests of freshwater fishes and daphnids. The results reveal that the 10% emulsible concentration of insecticide dimethoate is more toxic to the two freshwater fishes and D. magna.

Published
1989
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147. Could we prevent unilateral cleft lip/palate in the future?

Author

Mladina, Ranko, Skitarelić, Neven B., and Skitarelić, Nataša P.

Subjects
CLEFT lip, CLEFT palate, NASAL septum, HYPOTHESIS, HUMAN abnormalities, GENE therapy, CLEFT palate children, PREVENTION
Abstract

Summary: Surveillance studies have shown that cleft lip and palate is one of the commonest craniofacial anomalies, occurring in approximately 1 in 500 live births. Previous studies on craniofacial form in unilateral cleft lip/palate subjects have been carried out, but most attention has been focused on the deformity of the bony septum whereas the deformities of the nasal spine and cartilaginous component of the septum had received little attention. Our recent study was based on monitoring a very specific type of nasal septal deformity, type 6, and its relation to the unilateral cleft lip/palate disease. This type is very anteriorly located and refers to the cartilaginous part of the nasal septum and the inter-maxillary bone itself. Rhinoscopic view shows a typical, almost horizontal, unilateral groove at the nasal septum located very anteriorly. At the opposite septal side, but corresponding location, there is so called basal crest. The results of our study showed that the incidence of type 6 septal deformity was very high not only in unilateral cleft lip/palate children (80.6%) but also in their parents (58% in at least one of them). In contrast, in our previous study this type of septal deformity was seen in only 3.7% of non-unilateral cleft lip/palate children before puberty, rising to 7.4% in students and 9.4% in adults. In other words, perhaps we can expect the onset of unilateral cleft lip/palate in the offspring of parents who both have a type 6 septal deformity. Perhaps there is a gene responsible for the onset of both type 6 septal deformity and the cleft. If these clinical entities belong to the same gene, the cleft per se could perhaps disappear from the Earth in a near future owing to the gene therapy which will be able to eliminate it before the baby is born or even conceived. [Copyright &y& Elsevier]

Published
2009
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150. THE EUROFEVER REGISTRY FOR AUTOINFLAMMATORY DISEASE: UPDATE ON ENROLLMENT AFTER 2 YEARS

Author

Naselli, A., Frenkel, J., Ozen, S., Kone-Paut, I., Lachmann, H., Woo, P., Benedetti, F., Hofer, M., Neven, B., Dolezalova, P., Kuemmerle-Deschner, J., Hentgen, V., Touitou, I., Anna Simon, Wouters, C., Toplak, N., Anton, J., Stojanov, S., Girschick, H., Vesely, R., Arostegui, J. I., Rose, C., Ozdogan, H., Ruperto, N., Martini, A., and Gattorno, M.

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