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The Quality of Life of Children and Adolescents with X-Linked Agammaglobulinemia

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Abstract

Introduction

The health-related quality of life in X-linked agammaglobulinemia was investigated in 25 children and adolescents patients through the Italian version of Pediatric Quality of Life Inventory 4.0 Generic Core Scale for patients aged less then 18 years, comparing child perception to that of the parents and the physician’s evaluation. The data were compared with the ones of 80 healthy controls and the literature data of a group of patients with rheumatic diseases.

Discussion

The agammaglobulinemia subjects perceived a lower global quality of life than the healthy subjects, but significantly higher than the rheumatic diseases controls. The clinical relevance of health-related quality of life assessment in X-linked agammaglobulinemia pediatric patients is discussed.

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References

  1. Wilkenstein JA, Marino MC, Lederman HM, Jones SM, Sullivan K, Burks AW, Conley ME, Cunningham-Rundles C, Ochs HD. X-linked agammaglobulinemia. Report on a United States Registry of 201 patients. Medicine 2006. 85:193–202. doi:10.1097/01.md.0000229482.27398.ad.

    Article  Google Scholar 

  2. Plebani A, Soresina A, Rondelli R, Amato GM, Azzari C, Cardinale F, Cazzola G, Consolini R, De Mattia D, Dell’Erba G, Duse M, Fiorini M, Martino S, Martire B, Masi M, Monafo V, Moschese V, Notarangelo LD, Orlandi P, Panei P, Pession A, Pietrogrande MC, Pignata C, Quinti I, Ragno V, Rossi P, Sciotto A, Stabile A. Italian Group for XLA-AIEOP: clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study. Clin Immunol 2002. 104:221–30. doi:10.1006/clim.2002.5241.

    Article  PubMed  CAS  Google Scholar 

  3. Zashikhina A, Hagglof B. Mental health in adolescents with chronic physical illness versus controls in Northern Russia. Acta Paediatr 2007. 96:890–6. doi:10.1111/j.1651-2227.2007.00277.x.

    Article  PubMed  CAS  Google Scholar 

  4. Fayers PM, Machin D. Quality of life: assessment, analysis and interpretation. New York: Wiley; 2000.

    Google Scholar 

  5. Varni JW, Seid M, Kurtin PS. Pediatric health-related quality of life measurement technology: a guide for health care decision makers. J Clin Outcomes Manag 1999;6:33–40.

    Google Scholar 

  6. Howard V, Greene JM, Pahwa S, Winkelstein JA, Boyle JM, Kocak M, Conley ME. The health status and quality of life of adults with X-linked agammaglobulinemia. Clin Immunol 2006. 118:201–8. doi:10.1016/j.clim.2005.11.002.

    Article  PubMed  CAS  Google Scholar 

  7. Zebracki K, Palermo TM, Hostoffer R, Duff K, Drotar D. Health-related quality of life of children with primary immunodeficiency disease: a comparison study. Ann Allergy Asthma Immunol 2004;93:557–61.

    Article  PubMed  Google Scholar 

  8. Mozaffari H, Pourpak Z, Pourseyed S, Moin M, Farhoodi A, Aghamohammadi A, Movahedi M, Gharagozlou M, Entezari N. Health-related quality of life in primary immune deficient patients. Iran J Allergy Asthma Immunol 2006;5:23–7.

    PubMed  Google Scholar 

  9. Varni JW, Seid M, Smith-Knight T, Burwinkle T, Brown J, Szer IS. The PedsQL in pediatric rheumatology. Reliability, validity and responsiveness of the Pediatric Quality of Life Inventory Generic Core Scale and Rheumatology Module. Arthritis Rheum 2002. 46:714–25. doi:10.1002/art.10095.

    Article  PubMed  Google Scholar 

  10. Hollingshead AB. Four factor index of social status. New Haven, CT: Yale University; 1975.

    Google Scholar 

  11. Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 Generic Core Scales in healthy and patient populations. Med Care 2001. 39:800–12. doi:10.1097/00005650-200108000-00006.

    Article  PubMed  CAS  Google Scholar 

  12. Huygen AC, Kuis W, Sinnema G. Psychological, behavioural, and social adjustment in children and adolescents with juvenile chronic arthritis. Ann Rheum Dis 2000. 59(4):276–82. doi:10.1136/ard.59.4.276.

    Article  PubMed  CAS  Google Scholar 

  13. Clarke SA, Eiser C. The measurement of health-related quality of life (QOL) in paediatric clinical trials: a systematic review. Health Qual Life Outcomes 2004;22:2–66.

    Google Scholar 

  14. Cremeens J, Eiser C, Blades M. Factors influencing agreement between child self-report and parent proxy-reports on the Pediatric Quality of Life Inventory 4.0 (PedsQL) Generic Core Scales. Health Qual Life Outcomes 2006;30:4–58.

    Google Scholar 

Download references

Acknowledgments

We are grateful to the patients and families for their generous cooperation and the following centers participating in Italian Primary Immunodeficiencies Network (IPINet): Ancona (P. Pierani), Bari (D. DeMattia, B. Martire), Bari (L. Armenio, F. Cardinale), Bari (F. Dammacco, G. Ranieri), Bologna (M. Masi, A. Miniaci), Bologna Centro Operativo AIEOP (A. Pession, R. Rondelli), Brescia (A. Plebani, L.D. Notarangelo, A. Soresina), Cagliari (Cao, F. Cossu), Cagliari (S. Del Giacco, P. Manconi), Campobasso (I. Evangelista), Catanzaro (S. Magro, S. Morgione), Catanzaro (P. Strisciuglio, E. Anastasio), Catania (G. Schillirò, A. Sciotto), Chieti (R. Paganelli), Como (M. Sticca), Cosenza (D. Sperlì, L. Carpino), Firenze (G. Bernini, C. Azzari), Firenze (E. Maggi, S. Romagnani, A. Matucci, A. Vultaggio), Genova (E. Castagnola, M. Gattorno), Lecce (G. Presta, A. Civino), Mantova (G. Gambaretto, S. Fasoli), Messina (C. Salpietro), Milano (M.C. Pietrogrande, R.M. DellePiane, C. Panisi), Milano (G. Cambiaghi), Milano (M. Pietrogrande), Milano (M.G. Roncarolo, A. Aiuti), Monza (G. Masera, A. Biondi, A. Sala), Napoli (C. Pignata), Napoli (V. Poggi, G. Menna), Napoli (R. Di Nardo), Napoli (A. D’Apuzzo), Napoli (A. Pelliccia), Napoli (A. Correra), Napoli (G. Marone, G. Spadaro), Padova (M. Carli, L. Zanesco, G. Basso, M.C. Putti), Padova (G. Semenzato, C. Agostini), Palermo (G.M. Amato), Palermo (M. Aricò, A. Trizzino), Parma (G. Izzi, P. Bertolini), Pavia (F. Locatelli, M. Zecca), Pavia (G. Rondini, G.L. Marseglia, R. Maccario, G. Bossi), Pesaro (L. Felici), Pisa (C. Favre, R. Consolini), Rimini (V. Vecchi, P. Sacchini, G. Rinaldi), Roma (A.G. Ugazio, P. Rossi, S. Livadiotti, C. Cancrini, A. Finocchi), Roma (A. Stabile), Roma (M. Duse, M. Iacobini), Roma (I. Quinti), Roma (V. Moschese), Salerno (F. Cecere), Siena (G. Morgese, A. Acquaviva), Treviso (G. De Zan, S. Strafella), Trieste (P. Tamaro, M. Rabusin), Torino (P.A. Tovo, S. Martino), Varese (L. Nespoli, M. Marinoni), Venezia (A. Porcellini), and Verona (GA. Cazzola).

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Authors and Affiliations

  1. Dipartimento di Pediatría, Università degli Studi di Brescia, P.le Spedali Civili 1, 25123, Brescia, Italy

    Annarosa Soresina & Alessandro Plebani

  2. Cattedra di Neuropsichiatria dell’Infanzia e dell’Adolescenza, Università di Brescia, Brescia, Italy

    Renata Nacinovich, Monica Bomba, Morena Cassani & Anna Molinaro

  3. Dipartimento di Pediatria, Università di Catania, Catania, Italy

    Antonella Sciotto

  4. Dipartimento di Pediatria, Università di Torino, Turin, Italy

    Silvana Martino

  5. Clinica Pediatrica “S. Maggiore”, Università di Bari, Bari, Italy

    Fabio Cardinale

  6. Dipartimento di Biomedicina dell’Età Evolutiva, Università di Bari, Bari, Italy

    Domenico De Mattia

  7. Dipartimento di Pediatria, Università di Padova, Padova, Italy

    Caterina Putti

  8. Dipartimento di Pediatria, IRCCS Fondazione Policlinico, Università di Milano, Milan, Italy

    Rosa Maria Dellepiane

  9. Divisione di Pediatria Pesaro, Università di Brescia, Brescia, Italy

    Leonardo Felici

  10. Sezione di Statistica Medica, Università di Brescia, Brescia, Italy

    Giovanni Parrinello

  11. Cattedra di Neuropsichiatria dell’Infanzia e dell’Adolescenza, Università di Milano (Bicocca), Milan, Italy

    Francesca Neri

Authors
  1. Annarosa Soresina
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  2. Renata Nacinovich
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  3. Monica Bomba
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  4. Morena Cassani
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  5. Anna Molinaro
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  7. Silvana Martino
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  8. Fabio Cardinale
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  9. Domenico De Mattia
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  10. Caterina Putti
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  11. Rosa Maria Dellepiane
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  12. Leonardo Felici
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  13. Giovanni Parrinello
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  14. Francesca Neri
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  15. Alessandro Plebani
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Consortia

IPINet (Italian Network for Primary Immunodeficiencies)

Corresponding author

Correspondence to Annarosa Soresina.

Additional information

Annarosa Soresina and Renata Nacinovich contributed equally to this article.

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Soresina, A., Nacinovich, R., Bomba, M. et al. The Quality of Life of Children and Adolescents with X-Linked Agammaglobulinemia. J Clin Immunol 29, 501–507 (2009). https://doi.org/10.1007/s10875-008-9270-8

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  • DOI: https://doi.org/10.1007/s10875-008-9270-8

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