Quality of life and psychological well-being in GH-treated, adult PWS patients: A longitudinal study

Journal of Intellectual Disability Research

Volumn 51, Issue 4, 2007, Pages 302-311

  Bertella, L ^a,f   Mori, I ^a,c   Grugni, G ^a   Pignatti, Ricardo ^a,d   Ceriani, F ^a   Molinari, E ^a,b   Ceccarelli, A ^a,b   Sartorio, A ^a   Vettor, R ^e   Semenza, C ^a,d  

^a ISTITUTO AUXOLOGICO ITALIANO   (Italy)

^b UNIVERSITÀ CATTOLICA DEL SACRO CUORE   (Italy)

^c UNIVERSITY OF BERGAMO   (Italy)

^d UNIVERSITY OF TRIESTE   (Italy)

^e UNIVERSITY OF PADOVA   (Italy)

^f Hildebrand Clinic   (Switzerland)

Author keywords

Growth hormone therapy; Prader Willisyndrome; Psychologicalwell being; Qualityof Life

Indexed keywords

GROWTH HORMONE; SOMATOMEDIN C;

ADULT; ARTICLE; BEHAVIOR DISORDER; CHROMOSOME 15; CHROMOSOME ABERRATION; CLINICAL ARTICLE; CONTROLLED STUDY; DRUG DOSE TITRATION; EDEMA; EMOTIONAL DISORDER; FEMALE; GROWTH HORMONE DEFICIENCY; HUMAN; HYPERPHAGIA; LONGITUDINAL STUDY; MALE; MUSCLE HYPOTONIA; OBESITY; OPTIMISM; PARENTAL BEHAVIOR; PATIENT ATTITUDE; PRADER WILLI SYNDROME; PSYCHOLOGIC ASSESSMENT; PSYCHOLOGICAL WELL BEING; QUALITY OF LIFE; QUESTIONNAIRE; SHORT FORM 36; SHORT STATURE; TREATMENT OUTCOME;

ADAPTATION, PSYCHOLOGICAL; ADULT; FEMALE; HUMAN GROWTH HORMONE; HUMANS; LONGITUDINAL STUDIES; MALE; PRADER-WILLI SYNDROME; PSYCHIATRIC STATUS RATING SCALES; QUALITY OF LIFE; QUESTIONNAIRES; TIME FACTORS; TREATMENT OUTCOME;

EID: 33847624541     PISSN: 09642633     EISSN: 13652788     Source Type: Journal    
DOI: 10.1111/j.1365-2788.2006.00878.x     Document Type: Article

References (39)

1. Aimaretti G., Corneli G., Razzore P., Bellone S., Baffoni C., Arvat E., Camanni F. & Ghigo E. (1998) Comparison between insulin-induced hypoglycemia and growth hormone (GH)-releasing hormone+arginine as provocative tests for the diagnosis of GH deficiency in adults. Journal of Clinical and Endocrinology Metabolism 83, 1615-18.
2. Allen D. B. & Carrel A. L. (2004) Growth hormone therapy for Prader-Willi syndrome: A critical appraisal. Journal of Pediatric Endocrinology and Metabolism 17, 1297-306.
3. Apolone G., Mosconi P. & Ware J. (1997) Questionario sullo stato di salute SF-36. Manuale d'uso e guida all'interpretazione dei risultati. Guerini e associati, Milano.
4. Barman P., Broman J. E., Hetta J., Wiklund I., Erfurth E. M., Hagg E. & Karlsson F. A. (1995) Quality of life in adults with growth hormone (GH) deficiency: Response to treatment with recombinant human GH in a placebo-controlled 21-month trial. Journal of Clinical and Endocrinology Metabolism 80, 3585-90.
5. Biller B., Samuels M., Zagar A., Cook D., Arafah B., Bonert V., Stavrou S., Kleinberg D., Chipman J. & Hartman M. (2002) Sensitivity and specificity of six tests for the diagnosis of adult GH deficiency. Journal of Clinical and Endocrinology Metabolism 87, 2067-79.
6. Burman P., Ritzen E. M. & Lindgren A. C. (2001) Endocrine dysfunction in Prader-Willi syndrome: A review with special reference to GH. Endocrine Reviews 22, 787-99.
7. Butler M. & Thompson T. (2000) Prader-Willi syndrome: Clinical and genetical findings. Endocrinologist 10, 3-16.
8. Carrel A. L., Myers S. E., Whitman B. Y. & Allen D. B. (2002) Benefits of long-term GH therapy in Prader-Willi syndrome: A 4-year study. Journal of Clinical and Endocrinology Metabolism 87, 1581-5.
9. Carroll P. V., Littlewood R., Weissberger A. J., Bogalho P., McGauley G., Sonksen P. H. & Russel-Jones D. L. (1997) The effects of two doses of replacement growth hormone on the biochemical, body composition and psychological profiles of growth hormone-deficient adults. European Journal of Endocrinology 137, 146-53.
10. Cassidy S. B. (1997) Prader-Willi syndrome. Journal of Medical Genetics 34, 917-23.
11. Davies P. S. (2001) Growth hormone therapy in Prader-Willi syndrome. International Journal of Obesity 25, 2-7.
12. De Boer H., Blok G.-J. & Van der Veen E. A. (1995) Clinical aspects of growth hormone deficiency in adults. Endocrine Reviews 16, 63-86.
13. Descheemaeker M. J., Vogels A., Govers V., Borghgraef M., Willekens D., Swillen A., Verhoeven W. & Fryns J. P. (2002) Prader-Willi syndrome: new insights in the behavioural and psychiatric spectrum. Journal of Intellectual Disability Research 46, 41-50.
14. Doga M., Bonadonna S., Gola M., Solerte S. B., Amato G., Carella C. & Giustina A. (2005) Current guidelines for adult GH replacement. Reviews in Endocrine and Metabolic Disorders 6, 63-70.
15. Donaldson M. D. C., Chu C. E., Cooke A., Wilson A., Greene S. A. & Stephenson J. B. P. (1994) The Prader-Willi syndrome. Archives of Disease in Childhood 70, 58-63.
16. Dupuy H. J. (1984) The Psychological General Well-being Index (PGWB). In: Assessment of Quality of Life in Clinical Trials of Cardiovascular Therapies (eds N. K. Wenger, M. E. Mattson, C. D. Furburg & J. Elinson), pp. 170-83. Le Jacq Publishing Inc, New York.
17. Eiholzer U. & Whitman B. Y. (2004) A comprehensive team approach to the management of patients with Prader-Willi syndrome. Journal of Pediatric Endocrinology and Metabolism 17, 1153-75.
18. Eker L. & Tuzun E. H. (2004) An evaluation of quality of life of mothers of children with cerebral palsy. Disability and Rehabilitation 26, 1354-9.
19. Folstein M. F., Folstein S. E. & McHugh P. R. (1975) 'Mini-Mental State': A practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research 12, 189-98.
20. Gilchrist F. J., Murray R. D. & Shalet S. M. (2002) The effect of long-term untreated growth hormone deficiency (GHD) and 9 years of GH replacement on the quality of life (QoL) of Gh-deficient adults. Clinical Endocrinology (Oxf) 57, 363-70.
21. Goldbeck L. & Melches J. (2005) Quality of life in families of children with congenital heart disease. Quality Life Research 14, 1915-24.
22. Grossi E., Mosconi P., Groth N., Niero M. & Apolone G. (2002) Questionario 'Psychological General Well-Being Index'. Versione Italiana. Istituto di Ricerche Farmacologiche Mario Negri, Milano.
23. Grugni G., Guzzaloni G., Moro D., Bettio D., De Medici C. & Morabito F. (1998) Reduced growth hormone (GH) responsiveness to combined GH-releasing hormone and pyridostigmine administration in the Prader-Willi syndrome. Clinical Endocrinology (Oxf) 48, 769-75.
24. Holm V. A., Cassidy S. B., Butler M. G., Hanchett J. M., Greenswag L. R., Whitman B. Y. & Greenberg F. (1993) Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics 91, 398-401.
25. Hoybye C. (2005) Endocrine and metabolic aspects of adult Prader-Willi syndrome with special emphasis on the effect of growth hormone treatment. Growth Hormone and IGF Research 14, 1-15.
26. Hoybye C., Hilding A., Jacobsson H. & Thoren M. (2003) Growth hormone treatment improves body composition in adults with Prader-Willi syndrome. Clinical Endocrinology 58, 653-61.
27. Hoybye C., Thorén M. & Böhm B. (2005) Cognitive, emotional, physical and social effects of growth treatment in adults with Prader-Willi syndrome. Journal of Intellectual Disability Research 49, 245-52.
28. Jokovic A., Locker D. & Guyatt G. (2004) How well do parents know their children? Implications for proxy reporting of child health-related quality of life. Quality Life Research 13, 1297-307.
29. Lee P. D. K., Allen D. B., Angulo M. A., Cappa M., Carrel A. L., Castro-Magana M., Chiumello G., Davies P. S. W., Eiholzer U., Grugni G., Hauffa B. P., Hintz R. L., Lammer C., Mogul H. R., Myers S. E., Partsch C.-J., Pescovitz O. H., Ritzen E. M., Rosenfeld R. G., Sipila I. & Wilson D. M. (2000) Consensus Statement -Prader-Willi syndrome: Growth Hormone (GH)/Insulin-like Growth Factor axis deficiency and GH treatment. The Endocrinologist 10 (Suppl. 1 to no. 4), 71S-74S.
30. Molinari E. (2002) Clinica psicologica in sindromi rare. Aspetti genetici e riabilitativi. Bollati Boringhieri, Torino.
31. Murray R. D., Skillcorn C. J., Howell S. J., Lissett C. A., Rahim A., Smethurst L. E. & Shalet S. M. (1999) Influences on quality of life in GH deficient adults and their effects on response to treatment. Clinical Endocrinology (Oxf) 51, 565-73.
32. Partsch C.-J., Lammer C., Gillessen-Kaesbach G. & Pankau R. (2000) Adult patients with Prader-Willi syndrome: Clinical characteristics, life circumstances and growth hormone secretion. Growth Hormone & IGF Research 10 (Suppl. B), S81-S85.
33. Paterson W. F. & Donaldson M. D. C. (2003) Growth hormone therapy in the Prader-Willi syndrome. Archives of Disease in Childhood 88, 283-5.
34. Santos M. E., Castro-Caldas A. & De Sousa L. (1998) Spontaneous complaints of long-term traumatic brain injured subjects and their close relatives. Brain Injury 12, 759-67.
35. Steinhausen H. C., Eiholzer U., Hauffa B. P. & Malin Z. (2004) Behavioural and emotional disturbances in people with Prader-Willi Syndrome. Journal of Intellectual Disability Research 48, 47-52.
36. Ware J. E. & Kosinski M. (2001) Interpreting SF-36 summary health measures: A response. Quality Life Research 10, 405-13.
37. Ware J. E., Sherbourne C. D. & Davies A. R. (1992) Developing and testing the MOS 20-item Short Form Health Survey: A general population application. In: Measuring Functioning and Well-Being: The Medical Outcome Study Approach (eds A. L. Stewart & J. E. Ware), pp. 227-90. Duke University Press, Durham.
38. Wechsler D. (1981) Wechsler Adult Intelligence Scale Revised (WAIS-R) Traduzione e adattamento italiano a cura di C. Laicardi & A. Orsini. Organizzazioni Speciali, Firenze.
39. Wiren L., Bengtsson B. A. & Johannsson G. (1998) Beneficial effects of long-term GH replacement therapy on quality of life in adults with GH deficiency. Clinical Endocrinology (Oxf) 48, 613-20.