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3. Incidence and Prognostic Significance of Immunophenotypic Subgroups in Childhood Acute Lymphoblastic Leukemia: The Experience of the AIEOP Cooperative Study

Author

Basso, G., Rondelli, R., Putti, M. C., Cantù Rajnoldi, A., Granchi, D., Cocito, M. G., Saitta, M., Santostasi, T., Guglielmi, C., Lippi, A., Santoro, N., Russo, M. G., Pession, A., Aricò, M., Biondi, A., Herfarth, Ch., editor, Senn, H.-J., editor, Baum, M., editor, Diehl, V., editor, Gutzwiller, F., editor, Rajewsky, M. F., editor, Wannenmacher, M., editor, Ludwig, Wolf-Dieter, editor, and Thiel, Eckhard, editor

Published
1993
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4. Body Composition and Bone Mineral Quality in Phenylketonuria: Influence of Pubertal Development

Author

Paterno G, Faienza Mf, Pesce S, Giovanni Dd, Arico M, Tummolo A, Melpignano L, and Fantasia Ml

Subjects
Bone mineral, media_common.quotation_subject, Quality (business), Composition (visual arts), Food science, Biology, media_common
Abstract

Background/Objectives: Early diagnosis and a low-Phe diet significantly improved prognosis of PKU patients whose management is now-a-day mostly focused on preventing nutritional imbalances and resulting comorbidities. Puberty is a turning point for the risk to develop overweight and bone quality impairment. The present study aims to evaluate body composition and bone quality in prepubertal and pubertal PKU patients. Subjects/Methods: This is an observational, prospective study on an historical cohort of patients with PKU aged between 4.1 and 18 years, with early diagnosis and continuous protein-restrictive diet treatment. Bioimpedance, weight measurements, height, body mass index calculation, and quantitative ultrasound were collected. Sexual maturity was evaluated by using the Tanner staging. Results: Thirty PKU patients (14 prepubertal, 16 pubertal) were included in the study. Mean BMI was within the normal range in both groups, although fat content was higher in prepubertal, whereas lean mass was higher in the pubertal group who had also a higher total protein content. Among QUS parameters, bone quality index and broadband ultrasound were lower in prepubertal children than in adolescents, with a Z-score BQI within the osteopenia range in both of them. Conclusions: Pubertal patients with PKU develop a higher lean mass and protein content. If on one hand the high non-Phe protein intake in the pubertal period may promote increase in bone mineral quality, on the other hand the higher protein retention, typical of this period, may increase the risk for hyperinsulinism and glucose intolerance in later life. Adjusting dietary management by modulating total protein intake in the peripubertal period could result in better management of PKU patients.

Published
2021

5. Pruritus characteristics in a large Italian cohort of psoriatic patients

Author

Damiani, G., Cazzaniga, S., Conic, R. R. Z., Naldi, L., Griseta, V., Miracapillo, A., Azzini, M., Mocci, L., Michelini, M., Offidani, A., Bernardini, L., Campanati, A., Ricotti, G., Giacchetti, A., Norat, M., Gualco, F., Castelli, A., Cuccia, A., Diana, A., Roncarolo, G., Belli, M. A., Baldassarre, M. A., Santoro, G., Vena, G. A., Lo Console, F., Filotico, R., Mastrandrea, V., Brunetti, B., Musumeci, F., Carrabba, E., Dal Mas, P., Annicchiarico, F., Benvegnu, B., Spaziani, G., Cusano, F., Saletta Iannazzone, S., Galluccio, A., Pezza, M., Marchesi, L., Imberti, G., Reseghetti, A., Barbera, C., Reggiani, M., Lanzoni, A., Patrizi, A., Bardazzi, F., Antonucci, A., De Tommaso, S., Wallnofer, W., Ingannamorte, F., Calzavara-Pinton, P., Iannazzi, S., Zane, C., Capezzera, R., Bassisi, S., Rossi, M. T., Altamura, V., Vigl, W., Nobile, C., Aste, N., Murgia, S., Mugheddu, C., Scuderi, G., Baglieri, F., Di Dio, C., Cilioni Grilli, E., Mastronardi, C., Agnusdei, C. P., Antrilli, A., Aulisa, L., Raimondo, U., Scotto di Luzio, G., Battarra, V. C., Farro, P., Plaitano, R., Micali, G., Musumeci, M. L., Massimino, D., Li Calzi, M., La Greca, S., Pettinato, M., Sapienza, G., Valenti, G., De Giacomo, P. F., Amico, Arcangeli, F., Brunelli, D., Ghetti, E., Tulli, A., Assi, G., Amerio, P., Laria, G., Prestinari, F., Spadafora, S., Coppola, M., Caresana, G., Pezzarossa, E., Felisi, C., Donato, L., Bertero, M., Musso, L., Pa lazzini, S., Bruscino, P., Agozzino, U. C., Ottaviani, M., Simoncini, C., Virgili, A., Osti, F., Fabbri, P., Volpi, W., Caproni, M., Lotti, T., Prignano, F., Buggiani, G., Troiano, M., Fenizi, G., Altobella, A., Amoruso, A., Condello, M., Goffredo, A., Righini, M. G., Alessandrini, F., Satolli, F., Zampetti, M., Bertani, E., Fossati, S., Parodi, A., Burlando, M., Fiorucci, C., Nigro, A., Ghigliotti, G., Massone, L., Moise, G. M., Serrai, M., Cannata, G., Campagnoli, A. M., Daly, M., Leporati, C., Peila, R., Filosa, G., Bugatti, L., Nicolini, M., Nazzari, G., Cestari, R., Anastasio, F., Larussa, F. M., Pollice, N., De Francesco, F., Mazzocchetti, G., Peris, K., Fargnoli, M. C., Di Cesare, A., De Angelis, L., Flati, G., Biamonte, A. S., Quarta, G., Congedo, M., Carcaterra, A., Strippoli, D., Fideli, D., Marsili, F., Celli, M., Ceccarini, M., Bachini, L., D'Oria, M., Schirripa, V., De Filippi, C., Martini, P., Lapucci, E., Mazzatenta, C., Ghilardi, A., Simonacci, M., Bettacchi, A., Gasco, R., Zanca, A., Battistini, S., Dattola, S., Vernaci, R., Postorino, F., Zampieri, P. F., Padovan, C., Gonzalez Intchaurraga, M. A., Ladurner, J., Guarneri, B., Cannavo, S., Manfre, C., Borgia, F., Puglisi Guerra, A., Cattaneo, A., Carrera, C., Fracchiolla, C., Mozzanica, N., Prezzemolo, L., Menni, S., Lodi, A., Martino, P., Monti, M., Mancini, L., Sacrini, F., Altomare, G. F., Taglioni, M., Lovati, C., Mercuri, S. R., Schiesari, G., Giannetti, A., Conti, A., Lasagni, C., Greco, M., Ronsini, G., Schianchi, S., Fiorentini, C., Niglietta, S., Maglietta, R., Padalino, C., Crippa, D., Pini, M., Rossi, E., Tosi, D., Armas, M., Ruocco, V., Ayala, F., Balato, N., Gaudiello, F., Cimmino, G. F., Monfrecola, G., Gallo, L., Argenziano, G., Fulgione, E., Berruti, G., Ceparano, S., De Michele, I., Giorgiano, D., Leigheb, G., Deledda, S., Peserico, A., Alaibac, M., Piaserico, S., Schiesari, L., Dan, G., Mattei, I., Oro, E., Arico, M., Bongiorno, M. R., Angileri, R., Amato, S., Todaro, F., Milioto, M., Bellastro, R., Di Nuzzo, S., De Panfilis, G., Zanni, M., Borroni, G., Cananzi, R., Brazzelli, V., Lisi, P., Stingeni, L., Hansel, K., Pierfelice, V., Donelli, S., Rastelli, D., Gasperini, M., Barachini, P., Cecchi, R., Bartoli, L., Pavesi, M., De Paola, S., Corradin, M. T., Ricciuti, F., Piccirillo, A., Viola, L., Tataranni, M., Mautone, M. G., Lo Scocco, G., Niccoli, M. C., Brunasso Vernetti, A. M. G., Gaddoni, G., Resta, F., Casadio, M. C., Arcidiaco, M. C., Luvara, M. C., Albertini, G., Di Lernia, V., Guareschi, E., Catrani, S., Morri, M., De Simone, C., D'Agostino, M., Agostino, I., Calvieri, S., Cantoresi, F., Richetta, A., Sorgi, P., Carnevale, C., Nicolucci, F., Berardesca, E., Ardigo, M., De Felice, C., Gubinelli, E., Talamonti, M., Camplone, G., Cruciani, G., Riccardi, F., Barbati, R., Zumiani, G., Pagani, W., Malagoli, P. G., Pellicano, R., Donadio, D., Di Vito, C., Cottoni, F., Montesu, M. A., Pirodda, C., Addis, G., Marongiu, P., Farris, A., Cacciapuoti, M., Verrini, A., Desirello, G., Gnone, M., Fimiani, M., Pellegrino, M., Castelli, G., Zappala, L., Sesana, G., Ingordo, V., Vozza, E., Di Giuseppe, D., Fasciocco, D., Nespoli, P., Papini, M., Cicoletti, M., Bernengo, M. G., Ortoncelli, M., Bonvicino, A., Capella, G., Doveil, G. C., Forte, M., Peroni, A., Salomone, B., Savoia, P., Pippione, M., Zichichi, L., Frazzitta, M., De Luca, G., Tasin, L., Simonetto, D., Ros, S., Trevisan, G., Patamia, M., Miertusova, S., Patrone, P., Frattasio, A., Piccirillo, F., La Spina, S., Di Gaetano, L., Marzocchi, V., Motolese, A., Venturi, C., Gai, F., Pasquinucci, S., Bellazzi, R. M., Silvestri, T., Girolomoni, G., Gisondi, P., Veller Fornasa, C., Trevisan, G. P., Damiani G., Cazzaniga S., Conic R.R.Z., Naldi L., Griseta V., Miracapillo A., Azzini M., Mocci L., Michelini M., Offidani A., Bernardini L., Campanati A., Ricotti G., Giacchetti A., Norat M., Gualco F., Castelli A., Cuccia A., Diana A., Roncarolo G., Belli M.A., Baldassarre M.A., Santoro G., Vena G.A., Lo Console F., Filotico R., Mastrandrea V., Brunetti B., Musumeci F., Carrabba E., Dal Mas P., Annicchiarico F., Benvegnu B., Spaziani G., Cusano F., Saletta Iannazzone S., Galluccio A., Pezza M., Marchesi L., Imberti G., Reseghetti A., Barbera C., Reggiani M., Lanzoni A., Patrizi A., Bardazzi F., Antonucci A., De Tommaso S., Wallnofer W., Ingannamorte F., Calzavara-Pinton P., Iannazzi S., Zane C., Capezzera R., Bassisi S., Rossi M.T., Altamura V., Vigl W., Nobile C., Aste N., Murgia S., Mugheddu C., Scuderi G., Baglieri F., Di Dio C., Cilioni Grilli E., Mastronardi C., Agnusdei C.P., Antrilli A., Aulisa L., Raimondo U., Scotto di Luzio G., Battarra V.C., Farro P., Plaitano R., Micali G., Musumeci M.L., Massimino D., Li Calzi M., La Greca S., Pettinato M., Sapienza G., Valenti G., De Giacomo P.F., Amico, Arcangeli F., Brunelli D., Ghetti E., Tulli A., Assi G., Amerio P., Laria G., Prestinari F., Spadafora S., Coppola M., Caresana G., Pezzarossa E., Felisi C., Donato L., Bertero M., Musso L., Pa lazzini S., Bruscino P., Agozzino U.C., Ottaviani M., Simoncini C., Virgili A., Osti F., Fabbri P., Volpi W., Caproni M., Lotti T., Prignano F., Buggiani G., Troiano M., Fenizi G., Altobella A., Amoruso A., Condello M., Goffredo A., Righini M.G., Alessandrini F., Satolli F., Zampetti M., Bertani E., Fossati S., Parodi A., Burlando M., Fiorucci C., Nigro A., Ghigliotti G., Massone L., Moise G.M., Serrai M., Cannata G., Campagnoli A.M., Daly M., Leporati C., Peila R., Filosa G., Bugatti L., Nicolini M., Nazzari G., Cestari R., Anastasio F., Larussa F.M., Pollice N., De Francesco F., Mazzocchetti G., Peris K., Fargnoli M.C., Di Cesare A., De Angelis L., Flati G., Biamonte A.S., Quarta G., Congedo M., Carcaterra A., Strippoli D., Fideli D., Marsili F., Celli M., Ceccarini M., Bachini L., D'Oria M., Schirripa V., De Filippi C., Martini P., Lapucci E., Mazzatenta C., Ghilardi A., Simonacci M., Bettacchi A., Gasco R., Zanca A., Battistini S., Dattola S., Vernaci R., Postorino F., Zampieri P.F., Padovan C., Gonzalez Intchaurraga M.A., Ladurner J., Guarneri B., Cannavo S., Manfre C., Borgia F., Puglisi Guerra A., Cattaneo A., Carrera C., Fracchiolla C., Mozzanica N., Prezzemolo L., Menni S., Lodi A., Martino P., Monti M., Mancini L., Sacrini F., Altomare G.F., Taglioni M., Lovati C., Mercuri S.R., Schiesari G., Giannetti A., Conti A., Lasagni C., Greco M., Ronsini G., Schianchi S., Fiorentini C., Niglietta S., Maglietta R., Padalino C., Crippa D., Pini M., Rossi E., Tosi D., Armas M., Ruocco V., Ayala F., Balato N., Gaudiello F., Cimmino G.F., Monfrecola G., Gallo L., Argenziano G., Fulgione E., Berruti G., Ceparano S., De Michele I., Giorgiano D., Leigheb G., Deledda S., Peserico A., Alaibac M., Piaserico S., Schiesari L., Dan G., Mattei I., Oro E., Arico M., Bongiorno M.R., Angileri R., Amato S., Todaro F., Milioto M., Bellastro R., Di Nuzzo S., De Panfilis G., Zanni M., Borroni G., Cananzi R., Brazzelli V., Lisi P., Stingeni L., Hansel K., Pierfelice V., Donelli S., Rastelli D., Gasperini M., Barachini P., Cecchi R., Bartoli L., Pavesi M., De Paola S., Corradin M.T., Ricciuti F., Piccirillo A., Viola L., Tataranni M., Mautone M.G., Lo Scocco G., Niccoli M.C., Brunasso Vernetti A.M.G., Gaddoni G., Resta F., Casadio M.C., Arcidiaco M.C., Luvara M.C., Albertini G., Di Lernia V., Guareschi E., Catrani S., Morri M., De Simone C., D'Agostino M., Agostino I., Calvieri S., Cantoresi F., Richetta A., Sorgi P., Carnevale C., Nicolucci F., Berardesca E., Ardigo M., De Felice C., Gubinelli E., Talamonti M., Camplone G., Cruciani G., Riccardi F., Barbati R., Zumiani G., Pagani W., Malagoli P.G., Pellicano R., Donadio D., Di Vito C., Cottoni F., Montesu M.A., Pirodda C., Addis G., Marongiu P., Farris A., Cacciapuoti M., Verrini A., Desirello G., Gnone M., Fimiani M., Pellegrino M., Castelli G., Zappala L., Sesana G., Ingordo V., Vozza E., Di Giuseppe D., Fasciocco D., Nespoli P., Papini M., Cicoletti M., Bernengo M.G., Ortoncelli M., Bonvicino A., Capella G., Doveil G.C., Forte M., Peroni A., Salomone B., Savoia P., Pippione M., Zichichi L., Frazzitta M., De Luca G., Tasin L., Simonetto D., Ros S., Trevisan G., Patamia M., Miertusova S., Patrone P., Frattasio A., Piccirillo F., La Spina S., Di Gaetano L., Marzocchi V., Motolese A., Venturi C., Gai F., Pasquinucci S., Bellazzi R.M., Silvestri T., Girolomoni G., Gisondi P., Veller Fornasa C., and Trevisan G.P.

Subjects
Male, Cross-sectional study, Severity of Illness Index, Cohort Studies, 030207 dermatology & venereal diseases, 0302 clinical medicine, Risk Factors, education, itch, pruritus, psoriasis, pustular psoriasis, treatment, Adolescent, Adult, Cross-Sectional Studies, Educational Status, Facial Dermatoses, Female, Foot Dermatoses, Genitalia, Hand Dermatoses, Humans, Italy, Middle Aged, Pruritus, Psoriasis, Registries, Sex Factors, Young Adult, Epidemiology, Young adult, skin and connective tissue diseases, Settore MED/33 - MALATTIE APPARATO LOCOMOTORE, Infectious Diseases, 030220 oncology & carcinogenesis, Cohort, PRURITIS EPIDEMIOLOGY, Settore MED/35 - MALATTIE CUTANEE E VENEREE, Cohort study, medicine.medical_specialty, PSORIAS, Dermatology, Article, 03 medical and health sciences, Pharmacotherapy, Settore MED/35, Severity of illness, medicine, business.industry, medicine.disease, Pruritus,Itch sensation, business
Abstract

Background: Psoriasis (Ps) is a chronic systemic autoimmune disease associated with pruritus in 64–98% of patients. However, few modestly sized studies assess factors associated with psoriatic pruritus. Objective: To investigate factors associated with Ps pruritus intensity. Methods: Psoriasis patients 18years or older seen in one of 155 centres in Italy between September 2005 and 2009 were identified from the Italian PsoCare registry. Patients without cutaneous psoriasis and those with missed information on pruritus were excluded. Results: We identified 10802 patients, with a mean age 48.8±14.3years. Mild itch was present in 33.2% of patients, moderate in 34.4%, severe in 18.7% and very severe in 13.7%. Higher itch intensity was associated with female gender, lower educational attainment compared to university degree, pustular psoriasis, psoriasis on the head, face, palmoplantar areas, folds and genitalia, more severe disease, disease duration

Published
2019

7. Genotype-phenotype study of familial haemophagocytic lymphohistiocytosis due to perforin mutations

Author

Trizzino, A., Zur Stadt, U., Ueda, I., Risma, K., Janka, G., Ishii, E., Beutel, K., Sumegi, J., Cannella, S., Pende, D., Mian, A., Henter, J.-I., Griffiths, G., Santoro, A., Filipovich, A., and Arico, M.

Subjects
Langerhans-cell histiocytosis -- Development and progression, Langerhans-cell histiocytosis -- Genetic aspects, Gene mutations -- Research, Genotype -- Research, Phenotype -- Research, Killer cells -- Genetic aspects, T cells -- Genetic aspects, Health
Published
2008

15. Road Traffic Pollution and Childhood Leukemia: A Nationwide Case-control Study in Italy

Author

Magnani, C, Ranucci, A, Badaloni, C, Cesaroni, G, Ferrante, D, Miligi, L, Mattioli, S, Rondelli, R, Bisanti, L, Zambon, P, Cannizzaro, S, Michelozzi, P, Cocco, P, Celentano, E, Assennato, G, Merlo, D, Mosciatti, P, Minelli, L, Cuttini, M, Torregrossa, M, Lagorio, S, Haupt, R, Forastiere, F, Farioli, A, Salvan, A, Masera, G, Rizzari, C, Greco Veneto, A, Gafa, L, Luzzatto, L, Benvenuti, A, Kirchmayer, U, Galassi, C, Guarino, E, de Nichilo, G, Bocchini, V, Chiavarini, M, Casotto, V, Valenti, R, Risica, S, Polichetti, A, Bochicchio, F, Nuccetelli, C, Biddau, P, Arico, M, Desalvo, G, Locatelli, F, Pession, A, Varotto, S, Poggi, V, Massaglia, P, Monetti, D, Targhetta, R, Bernini, G, Pannelli, F, Sampietro, G, Schiliro, G, Pulsoni, A, Parodi, S, Magnani C., Ranucci A., Badaloni C., Cesaroni G., Ferrante D., Miligi L., Mattioli S., Rondelli R., Bisanti L., Zambon P., Cannizzaro S., Michelozzi P., Cocco P., Celentano E., Assennato G., Merlo D. F., Mosciatti P., Minelli L., Cuttini M., Torregrossa M. V., Lagorio S., Haupt R., Forastiere F., Farioli A., Salvan A., Masera G., Rizzari C., Greco Veneto A., Gafa L., Luzzatto L. L., Benvenuti A., Kirchmayer U., Galassi C., Guarino E., de Nichilo G., Bocchini V., Chiavarini M., Casotto V., Valenti R. M., Risica S., Polichetti A., Bochicchio F., Nuccetelli C., Biddau P., Arico M., DeSalvo G. L., Locatelli F., Pession A., Varotto S., Poggi V., Massaglia P., Monetti D., Targhetta R., Bernini G., Pannelli F., Sampietro G., Schiliro G., Pulsoni A., Parodi S., Magnani, C, Ranucci, A, Badaloni, C, Cesaroni, G, Ferrante, D, Miligi, L, Mattioli, S, Rondelli, R, Bisanti, L, Zambon, P, Cannizzaro, S, Michelozzi, P, Cocco, P, Celentano, E, Assennato, G, Merlo, D, Mosciatti, P, Minelli, L, Cuttini, M, Torregrossa, M, Lagorio, S, Haupt, R, Forastiere, F, Farioli, A, Salvan, A, Masera, G, Rizzari, C, Greco Veneto, A, Gafa, L, Luzzatto, L, Benvenuti, A, Kirchmayer, U, Galassi, C, Guarino, E, de Nichilo, G, Bocchini, V, Chiavarini, M, Casotto, V, Valenti, R, Risica, S, Polichetti, A, Bochicchio, F, Nuccetelli, C, Biddau, P, Arico, M, Desalvo, G, Locatelli, F, Pession, A, Varotto, S, Poggi, V, Massaglia, P, Monetti, D, Targhetta, R, Bernini, G, Pannelli, F, Sampietro, G, Schiliro, G, Pulsoni, A, Parodi, S, Magnani C., Ranucci A., Badaloni C., Cesaroni G., Ferrante D., Miligi L., Mattioli S., Rondelli R., Bisanti L., Zambon P., Cannizzaro S., Michelozzi P., Cocco P., Celentano E., Assennato G., Merlo D. F., Mosciatti P., Minelli L., Cuttini M., Torregrossa M. V., Lagorio S., Haupt R., Forastiere F., Farioli A., Salvan A., Masera G., Rizzari C., Greco Veneto A., Gafa L., Luzzatto L. L., Benvenuti A., Kirchmayer U., Galassi C., Guarino E., de Nichilo G., Bocchini V., Chiavarini M., Casotto V., Valenti R. M., Risica S., Polichetti A., Bochicchio F., Nuccetelli C., Biddau P., Arico M., DeSalvo G. L., Locatelli F., Pession A., Varotto S., Poggi V., Massaglia P., Monetti D., Targhetta R., Bernini G., Pannelli F., Sampietro G., Schiliro G., Pulsoni A., and Parodi S.

Abstract

Background The association of childhood leukemia with traffic pollution was considered in a number of studies from 1989 onwards, with results not entirely consistent and little information regarding subtypes. Aim of the study We used the data of the Italian SETIL case-control on childhood leukemia to explore the risk by leukemia subtypes associated to exposure to vehicular traffic. Methods We included in the analyses 648 cases of childhood leukemia (565 Acute lymphoblastic–ALL and 80 Acute non lymphoblastic-AnLL) and 980 controls. Information on traffic exposure was collected from questionnaire interviews and from the geocoding of house addresses, for all periods of life of the children. Results We observed an increase in risk for AnLL, and at a lower extent for ALL, with indicators of exposure to traffic pollutants. In particular, the risk was associated to the report of closeness of the house to traffic lights and to the passage of trucks (OR: 1.76; 95% CI 1.03–3.01 for ALL and 6.35; 95% CI 2.59–15.6 for AnLL). The association was shown also in the analyses limited to AML and in the stratified analyses and in respect to the house in different period of life. Conclusions Results from the SETIL study provide some support to the association of traffic related exposure and risk for AnLL, but at a lesser extent for ALL. Our conclusion highlights the need for leukemia type specific analyses in future studies. Results support the need of controlling exposure from traffic pollution, even if knowledge is not complete.

Published
2016

16. Long-term results of the AIEOP MH’96 childhood Hodgkin’s lymphoma trial and focus on significance of response to chemotherapy and its implication in low risk patients to avoid radiotherapy

Author

Burnelli, R., Rinieri, S., Rondelli, R., Todesco, A., Bianchi, M., Garaventa, A., Zecca, M., Indolfi, P., Conter, V., Santoro, N., Arico, M., Cesaro, S., D'Amico, S., Farruggia, P., De Santis, R., Locatelli, Franco, Pileri, S. A., Scarzello, G., Mascarin, M., Vecchi, V., Locatelli F. (ORCID:0000-0002-7976-3654), Burnelli, R., Rinieri, S., Rondelli, R., Todesco, A., Bianchi, M., Garaventa, A., Zecca, M., Indolfi, P., Conter, V., Santoro, N., Arico, M., Cesaro, S., D'Amico, S., Farruggia, P., De Santis, R., Locatelli, Franco, Pileri, S. A., Scarzello, G., Mascarin, M., Vecchi, V., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Identify a subset of early-stage HL children (GR1) curable with limited chemotherapy+/-radiotherapy; improve outcome of intermediate (GR2) and high-risk (GR3) patients; establish impact of response to chemotherapy evaluated with conventional imaging (CI). One hundred and sixty GR1-patients received 3ABVD + involved-field (IF) low-dose (LD) (20 Gy) irradiation if mediastinal mass or partial response (PR) after chemotherapy. Eighty-five GR2- and 315 GR3-patients received 4 and 6 COPP/ABV + IFRT, respectively. The 63 GR1 patients spared from radiotherapy had 15-year survival and EFS of 100 and 84.5%, respectively. The GR2 and GR3 15-year FFP were 84.7 and 78.6%, respectively. No different prognosis for patients in CR or PR evaluated during and after chemotherapy was observed. In conclusion, low-risk patients in CR may be successfully treated with radiation-free, low-intensity chemotherapy. Good, but less satisfactory, results were registered in GR2 and GR3. Response evaluated with CI is not a prognostic factor, but permits identification of low-risk patients who can avoid radiotherapy.

Published
2018

17. Outcomes of Children with Hemophagocytic Lymphohistiocytosis Given Allogeneic Hematopoietic Stem Cell Transplantation in Italy

Author

Messina, C., Zecca, M., Fagioli, F., Rovelli, A., Giardino, S., Merli, P., Porta, F., Arico, M., Sieni, E., Basso, G., Ripaldi, M., Favre, C., Pillon, M., Marzollo, A., Rabusin, M., Cesaro, S., Algeri, M., Caniglia, M., Di Bartolomeo, P., Ziino, O., Saglio, F., Prete, A., Locatelli, Franco, Locatelli F. (ORCID:0000-0002-7976-3654), Messina, C., Zecca, M., Fagioli, F., Rovelli, A., Giardino, S., Merli, P., Porta, F., Arico, M., Sieni, E., Basso, G., Ripaldi, M., Favre, C., Pillon, M., Marzollo, A., Rabusin, M., Cesaro, S., Algeri, M., Caniglia, M., Di Bartolomeo, P., Ziino, O., Saglio, F., Prete, A., Locatelli, Franco, and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

We report on 109 patients with hemophagocytic lymphohistiocytosis (HLH) undergoing 126 procedures of allogeneic hematopoietic stem cell transplantation (HSCT) between 2000 and 2014 in centers associated with the Italian Pediatric Hematology Oncology Association. Genetic diagnosis was FHL2 (32%), FHL3 (33%), or other defined disorders known to cause HLH (15%); in the remaining patients no genetic abnormality was found. Donor for first transplant was an HLA-matched sibling for 25 patients (23%), an unrelated donor for 73 (67%), and an HLA-partially matched family donor for 11 children (10%). Conditioning regimen was busulfan-based for 61 patients (56%), treosulfan-based for 21 (20%), and fludarabine-based for 26 children (24%). The 5-year probabilities of overall survival (OS) and event-free survival (EFS) were 71% and 60%, respectively. Twenty-six patients (24%) died due to transplant-related causes, whereas 14 (13%) and 10 (9%) patients experienced graft rejection and/or relapse, respectively. Twelve of 14 children given a second HSCT after graft failure/relapse are alive and disease-free. Use of HLA-partially matched family donors was associated with higher risk of graft failure and thus with lower EFS (but not with lower OS) in multivariable analysis. Active disease at transplantation did not significantly affect prognosis. These data confirm that HSCT can cure most HLH patients, active disease not precluding successful transplantation. Because in HLH patients HLA-haploidentical HSCT performed through CD34 + cell positive selection was found to be associated with poor sustained engraftment of donor cells, innovative approaches able to guarantee a more robust engraftment are warranted in patients given this type of allograft.

Published
2018

19. Air pollution and childhood leukaemia: a nationwide case-control study in Italy

Author

Badaloni, C., Ranucci, A., Cesaroni, G., Zanini, G., Vienneau, D., Al Aidrous, F., De Hoogh, K., Magnani, C., Forastiere, F., Mattioli, Stefano, Miligi, L., Rondelli, R., Salvan, A., Masera, G., Rizzari, C., Bisanti, L., Zambon, P., Greco, A., Cannizzaro, S., Gafa, L., Luzzatto, L. L., Benvenuti, A., Michelozzi, P., Kirchmayer, U., Cocco, P., Galassi, C., Celentano, E., Guarino, E., Assennato, G., de Nichilo, G., Merlo, D. F., Bocchini, V., Mosciatti, P., Minelli, L., Chiavarini, M., Cuttini, M., Casotto, V., Torregrossa, M. V., Valenti, R. M., Haupt, R., Lagorio, S., Risica, S., Polichetti, A., Bochicchio, F., Nuccetelli, C., Biddau, P., Arico, M., De Salvo, G. L., Locatelli, F., Pession, Andrea, Varotto, S., Poggi, V., Massaglia, P., Monetti, D., Targhetta, R., Bernini, G., Pannelli, F., Sampietro, G., Schiliro, G., Pulsoni, A., Badaloni, C., Ranucci, A., Cesaroni, G., Zanini, G., Vienneau, D., Al-Aidrous, F., De Hoogh, K., Magnani, C., Forastiere, F., C. Badaloni, A. Ranucci, G. Cesaroni, G. Zanini, D. Vienneau, F. Al-Aidrou, K. De Hoogh, C. Magnani, F. Forastiere, S. Mattioli, L. Miligi, R. Rondelli, A. Salvan, G. Masera, C. Rizzari, L. Bisanti, P. Zambon, A. Greco, S. Cannizzaro, L. Gafa, L. L. Luzzatto, A. Benvenuti, P. Michelozzi, U. Kirchmayer, P. Cocco, C. Galassi, E. Celentano, E. Guarino, G. Assennato, G. de Nichilo, D. F. Merlo, V. Bocchini, P. Mosciatti, L. Minelli, M. Chiavarini, M. Cuttini, V. Casotto, M. V. Torregrossa, R. M. Valenti, R. Haupt, S. Lagorio, S. Risica, A. Polichetti, F. Bochicchio, C. Nuccetelli, P. Biddau, M. Arico, G. L. De Salvo, F. Locatelli, A. Pession, S. Varotto, V. Poggi, P. Massaglia, D. Monetti, R. Targhetta, G. Bernini, F. Pannelli, G. Sampietro, G. Schiliro, and A. Pulsoni

Subjects
Male, Pediatrics, Air pollution, NO2, Land use Regression Model, Logistic regression, medicine.disease_cause, Economica, Residence Characteristics, USE REGRESSION-MODELS, Medicine, Child, Children, Vehicle Emissions, General Environmental Science, USE REGRESSION-MODELS, RESIDENTIAL TRAFFIC DENSITY, MAGNETIC-FIELDS, POOLED ANALYSIS, RISK-FACTOR, CANCER, EXPOSURE, CHILDREN, NO2, ASSOCIATION, Leukemia, Incidence, Incidence (epidemiology), ASSOCIATION, CANCER, Childhood leukaemia, Italy, Child, Preschool, Female, Case-Control Studie, Human, medicine.medical_specialty, Socio-culturale, MAGNETIC-FIELDS, POOLED ANALYSIS, RISK-FACTOR, Air Pollution, Occupational Exposure, Environmental health, Traffic Indicator, Humans, EXPOSURE, RESIDENTIAL TRAFFIC DENSITY, Exposure assessment, Vehicle Emission, business.industry, Public Health, Environmental and Occupational Health, Case-control study, Ambientale, Infant, Carcinogens, Environmental, Automobile, Case-Control Studies, Residence Characteristic, Dispersion Model, Etiology, General Earth and Planetary Sciences, Particulate Matter, Residence, business, Automobiles
Abstract

Objectives Leukaemia is the most common cancer in children, but its aetiology is still poorly understood. We tested the hypothesis that traffic-related air pollution is associated with paediatric leukaemia because of chronic exposure to several potential carcinogens. Methods The Italian SETIL study (Study on the aetiology of lymphohematopoietic malignancies in children) was conducted in 14 Italian regions. All incident cases of leukaemia in children aged ≤10 years from these regions (period 1998–2001) were eligible for enrolment. Two controls per case, matched on birth date, gender and region of residence were randomly selected from the local population registries. Exposure assessment at birth residence included traffic indicators (distance to main roads and length of main roads within 100 m) and estimates of pollutants concentrations (particulate matter -PM 2.5 and PM 10 - and gases -NO 2 and O 3 -) from national dispersion model and land use regression models. The association between the exposure variables and leukaemia was assessed by logistic regression analyses. Results Participation rates were 91.4% among cases and 69.2% in controls; 620 cases (544 acute lymphocytic and 76 acute non-lymphocytic leukaemia) and 957 controls were included. Overall, when considering the residence at birth, 35.6% of cases and 42.4% of controls lived along busy roads, and the mean annual PM 10 levels were 33.3 (SD=6.3) and 33.4 µg/m 3 (SD=6.5), respectively. No association was found, and all ORs, independent of the method of assessment and the exposure windows, were close to the null value. Conclusions Using various exposure assessment strategies, air pollution appears not to affect the incidence of childhood leukaemia.

Published
2013

20. Inhibitory 2B4 contributes to NK cell education and immunological derangements in XLP1 patients

Author

Meazza, R., Falco, M., Marcenaro, S., Loiacono, F., Canevali, P., Bellora, F., Tuberosa, C., Locatelli, Franco, Micalizzi, C., Moretta, A., Mingari, M. C., Moretta, L., Arico, M., Bottino, C., Pende, D., Locatelli F. (ORCID:0000-0002-7976-3654), Meazza, R., Falco, M., Marcenaro, S., Loiacono, F., Canevali, P., Bellora, F., Tuberosa, C., Locatelli, Franco, Micalizzi, C., Moretta, A., Mingari, M. C., Moretta, L., Arico, M., Bottino, C., Pende, D., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

X-linked lymphoproliferative disease 1 (XLP1) is an inherited immunodeficiency, caused by mutations in SH2D1A encoding Signaling Lymphocyte Activation Molecule (SLAM)-associated protein (SAP). In XLP1, 2B4, upon engagement with CD48, has inhibitory instead of activating function. This causes a selective inability of cytotoxic effectors to kill EBV-infected cells, with dramatic clinical sequelae. Here, we investigated the NK cell education in XLP1, upon characterization of killer Ig-like receptor (KIR)/KIR-L genotype and phenotypic repertoire of self-HLA class I specific inhibitory NK receptors (self-iNKRs). We also analyzed NK-cell cytotoxicity against CD48+ or CD48− KIR-ligand matched or autologous hematopoietic cells in XLP1 patients and healthy controls. XLP1 NK cells may show a defective phenotypic repertoire with substantial proportion of cells lacking self-iNKR. These NK cells are cytotoxic and the inhibitory 2B4/CD48 pathway plays a major role to prevent killing of CD48+ EBV-transformed B cells and M1 macrophages. Importantly, self-iNKR defective NK cells kill CD48− targets, such as mature DCs. Self-iNKR− NK cells in XLP1 patients are functional even in resting conditions, suggesting a role of the inhibitory 2B4/CD48 pathway in the education process during NK-cell maturation. Killing of autologous mature DC by self-iNKR defective XLP1 NK cells may impair adaptive responses, further exacerbating the patients’ immune defect.

Published
2017

22. On the drivers of extended enterprise and the problems of integration and governance

Author

Gottardi, Giorgio, Arico', M. Rita, and Piovesana, Andrea

Subjects
Automobile drivers, Business, international
Abstract

Byline: Giorgio Gottardi, M. Rita Arico', Andrea Piovesana The notion of extended enterprise (EE) has been principally discussed in the supply chain management perspective, dealing with problems of business processes integration and time-cost reduction. Even if the literature is rich and different definitions and perspectives are proposed, perhaps, important questions, such as the drivers of EE and the problems of integration and governance, still have not been faced in an exhaustive way. This paper assumes that, in the real world, the diffusion of the EE model is not the effect of a simple reorganisation of the large enterprise, aiming at improving static efficiency. The thesis is that EE leadership does not originate from cost control capabilities or monopolistic arrangements, but directly from the market. Dealing with the problem of recognising the drivers and nature of EE, aspects such as new marketing paradigms, impact on competitive strategy and structure, and the problems of integration and governance are analysed. This new perspective is verified on some relevant cases.

Published
2009

23. Deregulation of Ion Channel and Transporter Encoding Genes in Pediatric Gliomas

Author

Masselli, M, Laise, P, Tonini, G, Fanelli, D, Pillozzi, S, Cetica, V, DA ROS, M, Sardi, I, Buccoliero, A, Arico', M, Genitori, L, Becchetti, A, Arcangeli, A, MASSELLI, M, LAISE, P, TONINI, G, FANELLI, D, PILLOZZI, S, CETICA, V, SARDI, I, BUCCOLIERO, AM, ARICO', M, GENITORI, L, ARCANGELI, A., BECCHETTI, ANDREA, Masselli, M, Laise, P, Tonini, G, Fanelli, D, Pillozzi, S, Cetica, V, DA ROS, M, Sardi, I, Buccoliero, A, Arico', M, Genitori, L, Becchetti, A, Arcangeli, A, MASSELLI, M, LAISE, P, TONINI, G, FANELLI, D, PILLOZZI, S, CETICA, V, SARDI, I, BUCCOLIERO, AM, ARICO', M, GENITORI, L, ARCANGELI, A., and BECCHETTI, ANDREA

Abstract

Brain tumors, including the majority gliomas, are the leading cause of cancer-related death in children. World Health Organization has divided pediatric brain tumors into different grades and, based upon cDNA microarray data identifying gene expression profiles (GEPs), it has become evident in the last decade that the various grades involve different types of genetic alterations. However, it is not known whether ion channel and transporter genes, intimately involved in brain functioning, are associated with such GEPs. We determined the GEPs in an available cohort of 10 pediatric brain tumors initially by comparing the data obtained from four primary tumor samples and corresponding short-term cultures. The correspondence between the two types of samples was statistically significant. We then performed bioinformatic analyses on those samples (a total of nine) which corresponded to tumors of glial origin, either tissues or cell cultures, depending on the best "RNA integrity number." We used R software to evaluate the genes which were differentially expressed (DE) in gliomas compared with normal brain. Applying a p-value below 0.01 and fold change ≥4, led to identification of 2284 DE genes. Through a Functional Annotation Analysis (FAA) using the NIH-DAVID software, the DE genes turned out to be associated mainly with: immune/inflammatory response, cell proliferation and survival, cell adhesion and motility, neuronal phenotype, and ion transport. We have shown that GEPs of pediatric brain tumors can be studied using either primary tumor samples or short-term cultures with similar results. From FAA, we concluded that, among DE genes, pediatric gliomas show a strong deregulation of genes related to ion channels and transporters. © 2012 Masselli, Laise, Tonini, Fanelli, Pillozzi, Cetica, Da Ros, Sardi, Buccoliero, Aricò, Genitori, Becchetti and Arcangeli.

Published
2012

24. 'Identification of a Deletion in Stxbp2 Causative of Familial Hemophagocytic Lymphohistiocytosis Type 5'

Author

M L Coniglio, Arico M, Giglio S, Cetica, E Sieni, Benedetta Ciambotti, Rizzari C, Pantaleo M, Grieve S, Griffiths Gm, and Favre C

Subjects
Genetics, Hemophagocytic lymphohistiocytosis, Perforin, STX11, RNA splicing, biology.protein, medicine, Missense mutation, Familial Hemophagocytic Lymphohistiocytosis, Biology, medicine.disease, Genetic analysis, Gene
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening immune deficiency, characterized by a hyper-inflammatory syndrome. The familial form of HLH (FHL) is caused by mutations in genes associated with lymphocyte granule-mediated cytotoxicity. Mutations in STXBP2 (Sintaxin binding protein 2) gene result in defect of Munc18-2 protein, the causative defect of the subtype defined as FHL5. Functional tests as intracytoplasmic expression of perforin and surface expression of CD107a, help to direct genetic analysis. Different mutations have been described in the FHL-related genes known so far (PRF1, UNC13-D, STX11, STXBP2): missense, nonsense, splicing, regulatory, small deletions/insertions. Recently a pathogenic inversion of 253 KB upstream of the 3’ UNC13D gene has been reported. Here we describe a new deletion causative of FHL5. We confirmed the deletion by Real-Time PCR and by CGHarray. We finally documented by western blot the absence of expression of Munc18-2 protein in our patient. These data shows the need to introduce new diagnostic strategies in order to screen mutations not detected by the methods classically used.

Published
2015

26. The contribution of the Italian Association of paediatric haematology and oncology (AIEOP)

Author

Rondelli, R., Jankovic, M., Soresina, A., Valsecchi, M. G., De Rosa, M., Cuttini, M., Haupt, R., Arico, M., Bisogno, G., Locatelli, Franco, Magnani, C., Merletti, F., Zecca, M., Pession, A., Locatelli F. (ORCID:0000-0002-7976-3654), Rondelli, R., Jankovic, M., Soresina, A., Valsecchi, M. G., De Rosa, M., Cuttini, M., Haupt, R., Arico, M., Bisogno, G., Locatelli, Franco, Magnani, C., Merletti, F., Zecca, M., Pession, A., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Since 1972, children affected by cancer took advantage from multi-centric diagnostic and therapeutic protocols produced by the Italian Association of paediatric haematology and oncology (AIEOP). At the beginning, these protocols were used in few well-trained centres, later in almost all Italian haemato-oncological centres. The need of a careful monitoring of his own activity induced AIEOP to achieve, in 1989, an Italian hospital- based registry (database Mod.1.01) of malignant tumours diagnosed and treated in the participating centres, with the aim to quantify the number of cases diagnosed and treated in the different centres, the agreement (or not) to official diagnostic- therapeutic protocols, and the extraregional migration. The database Mod.1.01, which is available via web to the current 55 AIEOP centres since 2000, recruits annually about 1,400 children (0-14 years) and 200 adolescents (15-19 years). While the first accounts for over 90% of expected subjects, the latter are only 25%. Leukaemias (30% of cases) are the most frequent childhood cancers, followed by central nervous system (CNS) tumours and lymphomas, 18% of cases both. In children, leukaemias (34%) are prevalent, mostly acute lymphoblastic leukaemia (26%), followed by CNS tumours (18%); in adolescents, lymphomas (30%) are prevalent, mostly Hodgkin lymphomas (22%), followed by bone sarcomas (16%). The recruitment of registered cases in AIEOP protocols is overall good both for children (70%) and for adolescents (60%), achieving outstanding results in leukaemia protocols. Extraregional migration of patients for diagnosis and therapy is decreasing over time, being currently about 20%, higher in adolescents, in solid tumours, and in residents in South Italy and in the islands. On the contrary, an increase of subjects born and resident abroad who are hospitalised in AIEOP centres for diagnosis and treatment, accounting at present for 5% of all cases, was observed. The results confirm that the database Mod

Published
2016

27. Road Traffic Pollution and Childhood Leukemia: A Nationwide Case-control Study in Italy

Author

Magnani, C., Ranucci, A., Badaloni, C., Cesaroni, G., Ferrante, D., Miligi, L., Mattioli, S., Rondelli, R., Bisanti, L., Zambon, P., Cannizzaro, S., Michelozzi, P., Cocco, P., Celentano, E., Assennato, G., Merlo, D. F., Mosciatti, P., Minelli, L., Cuttini, M., Torregrossa, M. V., Lagorio, S., Haupt, R., Forastiere, F., Farioli, A., Salvan, A., Masera, G., Rizzari, C., Greco Veneto, A., Gafa, L., Luzzatto, L. L., Benvenuti, A., Kirchmayer, U., Galassi, C., Guarino, E., de Nichilo, G., Bocchini, V., Chiavarini, M., Casotto, V., Valenti, R. M., Risica, S., Polichetti, A., Bochicchio, F., Nuccetelli, C., Biddau, P., Arico, M., Desalvo, G. L., Locatelli, Franco, Pession, A., Varotto, S., Poggi, V., Massaglia, P., Monetti, D., Targhetta, R., Bernini, G., Pannelli, F., Sampietro, G., Schiliro, G., Pulsoni, A., Parodi, S., Locatelli F. (ORCID:0000-0002-7976-3654), Magnani, C., Ranucci, A., Badaloni, C., Cesaroni, G., Ferrante, D., Miligi, L., Mattioli, S., Rondelli, R., Bisanti, L., Zambon, P., Cannizzaro, S., Michelozzi, P., Cocco, P., Celentano, E., Assennato, G., Merlo, D. F., Mosciatti, P., Minelli, L., Cuttini, M., Torregrossa, M. V., Lagorio, S., Haupt, R., Forastiere, F., Farioli, A., Salvan, A., Masera, G., Rizzari, C., Greco Veneto, A., Gafa, L., Luzzatto, L. L., Benvenuti, A., Kirchmayer, U., Galassi, C., Guarino, E., de Nichilo, G., Bocchini, V., Chiavarini, M., Casotto, V., Valenti, R. M., Risica, S., Polichetti, A., Bochicchio, F., Nuccetelli, C., Biddau, P., Arico, M., Desalvo, G. L., Locatelli, Franco, Pession, A., Varotto, S., Poggi, V., Massaglia, P., Monetti, D., Targhetta, R., Bernini, G., Pannelli, F., Sampietro, G., Schiliro, G., Pulsoni, A., Parodi, S., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Background The association of childhood leukemia with traffic pollution was considered in a number of studies from 1989 onwards, with results not entirely consistent and little information regarding subtypes. Aim of the study We used the data of the Italian SETIL case-control on childhood leukemia to explore the risk by leukemia subtypes associated to exposure to vehicular traffic. Methods We included in the analyses 648 cases of childhood leukemia (565 Acute lymphoblastic–ALL and 80 Acute non lymphoblastic-AnLL) and 980 controls. Information on traffic exposure was collected from questionnaire interviews and from the geocoding of house addresses, for all periods of life of the children. Results We observed an increase in risk for AnLL, and at a lower extent for ALL, with indicators of exposure to traffic pollutants. In particular, the risk was associated to the report of closeness of the house to traffic lights and to the passage of trucks (OR: 1.76; 95% CI 1.03–3.01 for ALL and 6.35; 95% CI 2.59–15.6 for AnLL). The association was shown also in the analyses limited to AML and in the stratified analyses and in respect to the house in different period of life. Conclusions Results from the SETIL study provide some support to the association of traffic related exposure and risk for AnLL, but at a lesser extent for ALL. Our conclusion highlights the need for leukemia type specific analyses in future studies. Results support the need of controlling exposure from traffic pollution, even if knowledge is not complete.

Published
2016

28. Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry

Author

Cetica, V., Sieni, E., Pende, D., Danesino, C., De Fusco, C., Locatelli, Franco, Micalizzi, C., Putti, M. C., Biondi, A., Fagioli, F., Moretta, L., Griffiths, G. M., Luzzatto, L., Arico, M., Locatelli F. (ORCID:0000-0002-7976-3654), Cetica, V., Sieni, E., Pende, D., Danesino, C., De Fusco, C., Locatelli, Franco, Micalizzi, C., Putti, M. C., Biondi, A., Fagioli, F., Moretta, L., Griffiths, G. M., Luzzatto, L., Arico, M., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Background Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease affecting mostly children but also adults and characterized by hyperinflammatory features. A subset of patients, referred to as having familial hemophagocytic lymphohistiocytosis (FHL), have various underlying genetic abnormalities, the frequencies of which have not been systematically determined previously. Objective This work aims to further our understanding of the pathogenic bases of this rare condition based on an analysis of our 25 years of experience. Methods From our registry, we have analyzed a total of 500 unselected patients with HLH. Results Biallelic pathogenic mutations defining FHL were found in 171 (34%) patients; the proportion of FHL was much higher (64%) in patients given a diagnosis during the first year of life. Taken together, mutations of the genes PRF1 (FHL2) and UNC13D (FHL3) accounted for 70% of cases of FHL. Overall, a genetic diagnosis was possible in more than 90% of our patients with FHL. Perforin expression and the extent of degranulation have been more useful for diagnosing FHL than hemophagocytosis and the cytotoxicity assay. Of 281 (56%) patients classified as having "sporadic" HLH, 43 had monoallelic mutations in one of the FHL-defining genes. Given this gene dosage effect, FHL is not strictly recessive. Conclusion We suggest that the clinical syndrome HLH generally results from the combined effects of an exogenous trigger and genetic predisposition. Within this combination, different weights of exogenous and genetic factors account for the wide disease spectrum that ranges from HLH secondary to severe infection to FHL.

Published
2016

29. Dexamethasone vs prednisone in induction treatment of pediatric ALL: results of the randomized trial AIEOP-BFM ALL 2000

Author

Moricke, A., Zimmermann, M., Valsecchi, M. G., Stanulla, M., Biondi, A., Mann, G., Locatelli, Franco, Cazzaniga, G., Niggli, F., Arico, M., Bartram, C. R., Attarbaschi, A., Silvestri, D., Beier, R., Basso, G., Ratei, R., Kulozik, A. E., Lo Nigro, L., Kremens, B., Greiner, J., Parasole, R., Harbott, J., Caruso, R., von Stackelberg, A., Barisone, E., Rossig, C., Conter, V., Schrappe, M., Locatelli F. (ORCID:0000-0002-7976-3654), Moricke, A., Zimmermann, M., Valsecchi, M. G., Stanulla, M., Biondi, A., Mann, G., Locatelli, Franco, Cazzaniga, G., Niggli, F., Arico, M., Bartram, C. R., Attarbaschi, A., Silvestri, D., Beier, R., Basso, G., Ratei, R., Kulozik, A. E., Lo Nigro, L., Kremens, B., Greiner, J., Parasole, R., Harbott, J., Caruso, R., von Stackelberg, A., Barisone, E., Rossig, C., Conter, V., Schrappe, M., and Locatelli F. (ORCID:0000-0002-7976-3654)

Abstract

Induction therapy for childhood acute lymphoblastic leukemia (ALL) traditionally includes prednisone; yet, dexamethasone may have higher antileukemic potency, leading to fewer relapses and improved survival. After a 7-day prednisone prephase, 3720 patients enrolled on trial Associazione Italiana di Ematologia e Oncologia Pediatrica and Berlin-Frankfurt-Münster (AIEOP-BFM) ALL 2000 were randomly selected to receive either dexamethasone (10 mg/m(2) per day) or prednisone (60 mg/m(2) per day) for 3 weeks plus tapering in induction. The 5-year cumulative incidence of relapse (± standard error) was 10.8 ± 0.7% in the dexamethasone and 15.6 ± 0.8% in the prednisone group (P < .0001), showing the largest effect on extramedullary relapses. The benefit of dexamethasone was partially counterbalanced by a significantly higher induction-related death rate (2.5% vs 0.9%, P = .00013), resulting in 5-year event-free survival rates of 83.9 ± 0.9% for dexamethasone and 80.8 ± 0.9% for prednisone (P = .024). No difference was seen in 5-year overall survival (OS) in the total cohort (dexamethasone, 90.3 ± 0.7%; prednisone, 90.5 ± 0.7%). Retrospective analyses of predefined subgroups revealed a significant survival benefit from dexamethasone only for patients with T-cell ALL and good response to the prednisone prephase (prednisone good-response [PGR]) (dexamethasone, 91.4 ± 2.4%; prednisone, 82.6 ± 3.2%; P = .036). In patients with precursor B-cell ALL and PGR, survival after relapse was found to be significantly worse if patients were previously assigned to the dexamethasone arm. We conclude that, for patients with PGR in the large subgroup of precursor B-cell ALL, dexamethasone especially reduced the incidence of better salvageable relapses, resulting in inferior survival after relapse. This explains the lack of benefit from dexamethasone in overall survival that we observed in the total cohort except in the subset of T-cell ALL patients with PGR. This trial was registered at www

Published
2016

30. Marriage and parenthood among subjects cured of childhood cancer: a report from the Italian AIEOP Off-Therapy Registry

Author

Pivetta, EMANUELE EMILIO, Maule, MILENA MARIA, Pisani, Paola, Zugna, Daniela, Haupt, R, Jankovic, M, Arico', M, Casale, F, Clerico, A, CORDERO DI MONTEZEMOLO, Luca, Kiren, V, Locatelli, F, Palumbo, G, Pession, A, Pillon, M, Santoro, N, Terenziani, M, Valsecchi, Mg, Dama, Elisa, Magnani, C, Merletti, Franco, Pastore, G., Pivetta E., Maule MM., Pisani P., Zugna D., Haupt R., Jankovic M., Arico' M., Casale F., Clerico A., Cordero di Montezemolo L., Kiren V., Locatelli F., Palumbo G., Pession A., Pillon M., Santoro N., Terenziani M., Valsecchi MG., Dama E., Magnani C., Merletti F., Pastore G., Pivetta, E, Maule, Mm, Pisani, P, Zugna, D, Haupt, R, Jankovic, M, Aricò, M, Casale, Fiorina, Clerico, A, CORDERO DI MONTEZEMOLO, L, Kiren, V, Locatelli, F, Palumbo, G, Pession, A, Pillon, M, Santoro, N, Terenziani, M, Valsecchi, Mg, Dama, E, Magnani, C, Merletti, F, and Pastore, G.

Subjects
Quality of life, Fertility, childhood cancer, Marriage, Long-term survivors, childhood cancer, marriage, fertility, long-term survivors, quality of life
Abstract

The aim of this study was to describe the patterns of marriage and parenthood in a cohort of childhood cancer survivors included in the Off-Therapy Registry maintained by the Italian Association of Pediatric Hematology and Oncology. Design and Methods We analyzed a cohort of 6,044 patients diagnosed with cancer between 1960 and 1998, while aged 0 to 14 years and who were 18 years old or older by December 2003. They were followed up through the regional vital statistics registers until death or the end of follow up (October 30, 2006), whichever occurred first, and their marital status and date of birth of their children were recorded. The cumulative probabilities of being married and having a first child were computed by gender and compared by tumor type within the cohort. Marriage and fertility rates (the latter defined as the number of live births per woman-year) were compared with those of the Italian population of the same age, gender, area of residence and calendar period by means of the observed to expected (O/E) ratios. Results During the follow-up period, 4,633 (77%) subjects had not married. The marriage O/E ratios were 0.56 (95% CI: 0.51-0.61) and 0.70 (95% CI: 0.65-0.76) among men and women, respectively. Overall, 263 men had 367 liveborn children, and 473 women had 697 liveborn children. The female fertility O/E ratio was 0.57 (95% CI: 0.53-0.62) overall, and 1.08 (95% CI: 0.99-1.17) when analyses were restricted to married/cohabiting women Conclusions Childhood cancer survivors are less likely to marry and to have children than the general population, confirming the life-long impact of their previous disease on their social behavior and choices. The inclusion of counseling in the strategies of management and long-term surveillance of childhood cancer patients could be beneficial to survivors as they approach adulthood.

Published
2011

31. Role of DNA index in childhood acute lymphoblastic leukemia treated with BFM based therapy: Long term results of the AIEOP-ALL 95

Author

Rizzari, C, Conter, V, Valsecchi, M, Barisone, E, Casale, F, De Rossi, G, Lo Nigro, L, Locatelli, F, Messina, C, Micalizzi, C, Parasole, R, Pession, A, Santoro, N, Testi, A, Biondi, A, Rondelli, R, Silvestri, D, Basso, G, Masera, G, Arico, M, Testi, AM, Arico, M., VALSECCHI, MARIA GRAZIA, BIONDI, ANDREA, MASERA, GIUSEPPE, Rizzari, C, Conter, V, Valsecchi, M, Barisone, E, Casale, F, De Rossi, G, Lo Nigro, L, Locatelli, F, Messina, C, Micalizzi, C, Parasole, R, Pession, A, Santoro, N, Testi, A, Biondi, A, Rondelli, R, Silvestri, D, Basso, G, Masera, G, Arico, M, Testi, AM, Arico, M., VALSECCHI, MARIA GRAZIA, BIONDI, ANDREA, and MASERA, GIUSEPPE

Published
2007

33. Bifocal manifestation of eosinophilic granuloma in a paediatric patient-A case report

Author

GUIGLIA, Rosario, PIZZO, Giuseppe, MARESI, Emiliano, COMPILATO, Domenico, CAMPISI, Giuseppina, ARICO', M, GUIGLIA, R, PIZZO, G, ARICO', M, MARESI, E, COMPILATO, D, and CAMPISI, G

Subjects
Settore MED/28 - Malattie Odontostomatologiche, Langerhans cell histiocytosi, Langerhans cell histiocytosis
Abstract

Background: Eosinophilic granuloma (EG) is a clinical variant of the Langerhans cell histiocytosis (LCH) characterized by unifocal or multifocal bone lesions which predominantly affects children, adolescents, and young adults. Case Report: A case is reported of a 13-year-old Caucasian boy who presented unifocal EG in the mandible as the first clinic manifestation. Radiographic examination and skeletal scintigraphy revealed a further localization with an osteolytic lesion in the right femur. The therapeutic protocol used for the mandibular lesion included causal periodontal therapy, extraction of the compromised teeth, alveolar curettage, and intralesional injections of corticosteroids, in correspondence with femoral and mandibular bone lesions. Conclusions: Early diagnosis of LCH is considered an important factor which can improve the patient's prognosis and quality of life and also the cost-effectiveness of therapy. Dentists could play a fundamental role in the diagnosis and management of EG. The aim of the treatment is to eradicate EG lesions and provide adequate oral rehabilitation after the tooth loss. A careful multidisciplinary follow-up program is mandatory to identify any signs of local recurrence or dissemination.

Published
2009

34. Pediatric in situ split liver transplantation for Langerhans' cell histiocytosis - Acute rejection and disease recurrence

Author

Spada, M, Arico, M, Cintorino, D, Clarizia, S, Guizzetti, M, Lucianetti, A, Melzi, M, Minervini, M, Riva, S, Sonzogni, A, Stroppa, P, Torre, G, Colledan, M, Gridelli, B, Spada M, Arico M, Cintorino D, Clarizia S, Guizzetti M, Lucianetti A, Melzi ML, Minervini M, Riva S, Sonzogni A, Stroppa P, Torre G, Colledan M, Gridelli B, Spada, M, Arico, M, Cintorino, D, Clarizia, S, Guizzetti, M, Lucianetti, A, Melzi, M, Minervini, M, Riva, S, Sonzogni, A, Stroppa, P, Torre, G, Colledan, M, Gridelli, B, Spada M, Arico M, Cintorino D, Clarizia S, Guizzetti M, Lucianetti A, Melzi ML, Minervini M, Riva S, Sonzogni A, Stroppa P, Torre G, Colledan M, and Gridelli B

Published
2005

36. A single amino acid change A19V in perforin: a novel, frequent predisposing factor to childhood acute lymphoblastic leukemia?

Author

Santoro, A., TRIZZINO A, C., LO NIGRO, L., Corsello, G., Arico, M., SANTORO A, CANNELLA S TRIZZINO A, LO NIGRO L, CORSELLO G, and ARICO M

Subjects
A91V, Childhood acute lymphoblastic leukemia, Perrforin
Abstract

We screened 100 children with acute lymphoblastic leukemia (ALL) to assess the incidence of single amino acid change A91V in perform. Heterozygous A91V was found in 12/100 patients and 5/127 controls (OR, 3.4; 95%CI: 1.15-9.95; p=0.014). A91V is a novel and frequent predisposing factor for childhood ALL.

Published
2005

40. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

Author

Girschikofsky, M, Arico, M, Castillo, D, Chu, A, Doberauer, C, Fichter, J, Haroche, J, Kaltsas, GA, Makras, P, Marzano, AV, de Menthon, M, Micke, O, Passoni, E, Seegenschmiedt, HM, Tazi, A, and McClain, KL

Subjects
Adult, Langerhans, Histiocytosis
Abstract

Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus.

Published
2013

41. Management of adult patients with Langerhans cell histiocytosis: Recommendations from an expert panel on behalf of Euro-Histio-Net

Author

Girschikofsky, M. Arico, M. Castillo, D. Chu, A. Doberauer, C. Fichter, J. Haroche, J. Kaltsas, G.A. Makras, P. Marzano, A.V. De Menthon, M. Micke, O. Passoni, E. Seegenschmiedt, H.M. Tazi, A. McClain, K.L.

Abstract

Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus. © 2013 Girschikofsky et al.; licensee BioMed Central Ltd.

Published
2013

42. A single high dose of idarubicin combined with high-dose ARA-C for treatment of first relapse in childhood 'high-risk' acute lymphoblastic leukaemia: a study of the AIEOP group

Author

TESTI AM, DEL GIUDICE I, ARCESE W, MOLETI ML, GIONA F, BASSO G, BIONDI A, CONTER V, MESSINA C, RONDELLI R, MICOZZI A, MICALIZZI C, BARISONE E, LOCATELLI F, DINI G, ARICO M, COMIS M, LADOGANA S, LIPPI A, MURA R, PINTA MF, SANTORO N, VALSECCHI MG, MASERA G, MANDELLI F, AIEOP COOPERATIVE GROUP, CASALE, Fiorina, Testi, Am, DEL GIUDICE, I, Arcese, W, Moleti, Ml, Giona, F, Basso, G, Biondi, A, Conter, V, Messina, C, Rondelli, R, Micozzi, A, Micalizzi, C, Barisone, E, Locatelli, F, Dini, G, Arico, M, Casale, Fiorina, Comis, M, Ladogana, S, Lippi, A, Mura, R, Pinta, Mf, Santoro, N, Valsecchi, Mg, Masera, G, Mandelli, F, AIEOP COOPERATIVE, Group, Testi, A, Del Giudice, I, Moleti, M, Aricò, M, Casale, F, Pinta, M, and Valsecchi, M

Subjects
Male, Antineoplastic Combined Chemotherapy Protocol, Adolescent, Cytarabine, Hematopoietic Stem Cell Transplantation, Infant, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Disease-Free Survival, Follow-Up Studie, Survival Rate, Recurrence, Child, Preschool, Antineoplastic Combined Chemotherapy Protocols, Humans, Female, Preschool, Child, Idarubicin, Follow-Up Studies, Settore MED/15 - Malattie del Sangue, Human
Abstract

The outcome of children with acute lymphoblastic leukaemia (ALL) and early relapse remains unsatisfactory. In January 1995, the AIEOP (Associazione Italiana di Oncologia ed Ematologia Pediatrica) group opened a trial for children with ALL in first isolated or combined bone marrow relapse defined at high risk according to the length of first remission and the immunophenotype. The treatment plan included the combination of a single high-dose idarubicin and high-dose cytarabine as induction therapy followed by an intensive consolidation and stem cell transplant (SCT). In total, 100 children from 16 Italian centres were enrolled; 80 out of the 99 evaluable patients (81%) achieved second complete remission; eight (8%) died during induction and 11 (11%) failed to respond. A total of 42 out of the 80 responders (52.5%) received a SCT: 19 from an identical sibling, 11 from a matched unrelated donor and 12 from umbilical cord blood cells. The estimated 4-year overall survival and event-free survival were 25% and 21% respectively. Disease-free survival at 4 years was 25.8% for the 80 responders. At 4 years, 39 out of 100 children remain alive, with 27 of them free of leukaemia. This induction therapy has shown antileukaemic efficacy with acceptable toxicity; moreover, all responders proved eligible for intensive consolidation.

Published
2002

43. Familial hemophagocytic lymphohistiocytosis may present during adulthood: clinical and genetic features of a small series

Author

Palau, F, Sieni, E, Cetica, V, Piccin, A, Gherlinzoni, F, Sasso, Fc, Rabusin, M, Attard, L, Bos, A, Pende, D, Moretta, Lorenzo, and Arico', M.

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, Science, Immune Cells, Population, Immunology, T cells, Genetic Counseling, Disease, NK cells, Clinical immunology, Lymphohistiocytosis, Hemophagocytic, Molecular Genetics, Autosomal Recessive, Genetic Mutation, Molecular Cell Biology, medicine, Genetics, Genetics of the Immune System, Humans, Genetic Testing, Family history, Age of Onset, education, Biology, Female, Agricultural and Biological Sciences (all), Biochemistry, Genetics and Molecular Biology (all), Medicine (all), Clinical Genetics, Cytopenia, education.field_of_study, Multidisciplinary, business.industry, Human Genetics, Familial Hemophagocytic Lymphohistiocytosis, Hematology, medicine.disease, Transplantation, Genetics of Disease, Medicine, Age of onset, Differential diagnosis, business, Cytometry, Research Article
Abstract

Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare immune deficiency with defective cytotoxic function. The age at onset is usually young and the natural course is rapidly fatal if untreated. A later onset of the disease has been sporadically reported even in adolescents and adults. We report the results of our retrospective data collection of all cases diagnosed with FHL at an age of 18 years or older and enrolled in the Italian Registry of HLH. All cases were diagnosed with FHL based on evidence of genetic defect in one FHL-related gene. A total of 11 patients were diagnosed with FHL. They were 9 males and 2 females, from 10 unrelated families; their age ranged between 18 and 43 years (median, 23 years). Family history was unremarkable in eight families at the time of the diagnosis. Their genetic diagnoses are: FHL2 (n = 6), FHL3 (n = 2), FHL5 (n = 1), XLP1 (n = 2). Clinical, molecular and functional data are described. These data confirm that FHL may present beyond the pediatric age and up to the fifth decade. FHL2 due to perforin defect is the most frequently reported subtype. Adult specialists should consider FHL in the differential diagnosis of patients with cytopenia and liver or central nervous system disorders, especially when a lymphoproliferative disease is suspected but eventually not confirmed. FHL may turn to be fatal within a short time course even in adults. This risk, together with the continuous improvement in the transplant technique, especially in the area of transplant from matched unrelated donor, resulting in reduced treatment related mortality, might suggest a wider use of SCT in this population. Current diagnostic approach allows prompt identification of patients by flow-cytometry screening, then confirmed by the genetic study, and treatment with chemo-immunotherapy followed by stem cell transplantation.

Published
2012

45. DNA libero circolante nei linfomi pediatrici

Author

Mussolin, Lara, Fuser, G., Pillon, M., Franceschetto, G., Carraro, E., Todesco, A., Lombardi, A., Garaventa, A., Arico', M., Buffardi, S., Burnelli, R., and Rosolen, Angelo

Published
2012

46. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol

Author

Trottestam, H., Horne, A., Arico, M., Egeler, R.M., Filipovich, A.H., Gadner, H., Imashuku, S., Ladisch, S., Webb, D., Janka, G., Henter, J.I., and Histiocyte Soc

Subjects
Male, Pediatrics, medicine.medical_specialty, Time Factors, Adolescent, Clinical Trials and Observations, medicine.medical_treatment, Immunology, Hematopoietic stem cell transplantation, Disease, Biochemistry, Lymphohistiocytosis, Hemophagocytic, Maintenance Chemotherapy, Clinical Protocols, Chemoimmunotherapy, hemic and lymphatic diseases, medicine, Humans, Combined Modality Therapy, Child, Survival analysis, Hemophagocytic lymphohistiocytosis, Cytotoxins, business.industry, Hematopoietic Stem Cell Transplantation, Infant, Newborn, Infant, Cell Biology, Hematology, Jaundice, medicine.disease, Survival Analysis, Surgery, Transplantation, Drug Combinations, Child, Preschool, Female, Immunotherapy, medicine.symptom, business, Immunosuppressive Agents, Follow-Up Studies
Abstract

Hemophagocytic lymphohistiocytosis (HLH) used to have a dismal prognosis. We report the final results of HLH-94, the largest prospective diagnostic/therapeutic HLH study so far. The treatment includes immunosuppressive and cytotoxic therapy aiming at clinical remission, followed by HSCT in patients with familial, persistent, or recurrent disease. Altogether, 249 patients fulfilled inclusion criteria and started HLH-94 therapy (July 1994-December 2003); 227 (91%) were followed-up for ≥ 5 years. At 6.2 years median follow-up, estimated 5-year probability of survival was 54% ± 6%. Seventy-two patients (29%) died before HSCT, 64 within 1 year, 97% of whom had active disease. In 124 patients who underwent HSCT, 5-year survival was 66 ± 8%; tendency to increased survival (P = .064) in patients with nonactive disease at HSCT. Patients with familial disease had a 5-year survival of 50% ± 13%; none survived without HSCT. Patients deceased during the first 2 months more often had jaundice, edema, and elevated creatinine. Forty-nine patients (20%) were alive without signs of HLH activity and off-therapy > 1-year without HSCT; they presented at older age (P < .001), were more often female (P = .011), and less often had CNS disease (P < .001) or hepatomegaly (P = .007). To conclude, HLH-94 chemoimmunotherapy has considerably improved outcome in HLH. Collaborative efforts are needed to further reduce early mortality, HSCT-related mortality, and neurologic late effects.

Published
2011

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