Case Management Guidelines on Neurodevelopmental and Psychosocial Problems of Turner Syndrome

Abstract

Patients with Turner syndrome are referred to doctors because of neurodevelopmental and behavioural problems such as attention deficiency, hyperactivity, special learning disorders, immaturity, peer problems and social adaptation difficulties during their high school period. However the number of references about these issues is limited. Giving priority to the patients’ other problems like short stature, pubertal problems and absence of follow-up procedures providing multidisciplinary team comprehension can lead to the undermining of such neurodevelopmental and behavioural problems. Such negative effects on the lives of patients with Turner syndrome, as well as on those of family members, may be prevented by providing multidisciplinary yearly diagnosis-prevention-intervention programmes

Keywords

• Neurodevelopment, Turner, Case management

Turner Sendromunun Nörogelişimsel ve Psikososyal Sorunlarında Olgu Yönetim Rehberi

Abstract

Turner sendromu olguları ortaöğretim ve lise döneminde dikkat eksikliği, hiperaktivite, alana özel öğrenme bozukluğu, yaşıtlarına göre çocuksu olma, akran problemleri ve sosyal uyum güçlüğü gibi nörogelişimsel ve davranışsal sorunlarla doktora getirilmektedir. Buna rağmen, bu konularda kısıtlı sayıda literatür vardır. Hastanın boy kısalığı, pubertal sorunlar gibi diğer problemlerine öncelik verilmesi, ekip anlayışı içeren izlem protokollerinin olmaması nörogelişimsel-davranışsal sorunların ihmal edilmesine yol açmaktadır. Multidisipliner erken tanı-önleme-müdahale programları geliştirilerek bu sorunların Turner sendromu olgularının ve ailelerinin hayatlarını olumsuz etkilemesi önlenmiş olur.

Keywords

• Nörogelişim, Turner, Olgu yönetimi

References

1. Hong DS, Hoeft F, Marzelli MJ, Lepage JF, Roeltgen D, Ross J, et al. Influence of the X-chromosome on neuroanatomy: Evidence from Turner and Klinefelter syndromes. J Neurosci 2014;34:3509- 16.
2. Pinsker JE. Clinical review: Turner syndrome: Updating the paradigm of clinical care. J Clin Endocrinol Metab 2012;97:E994- 1003.
3. Dołęga Z, Jeż W, Irzyniec T. The cohort effect in studies related to differences in psychosocial functioning of women with Turner syndrome. Endokrynologia Polska 2014;65:287-94.
4. McCauley E, Sybert V. Social and behavioral development of girls and women with Turner syndrome. International Congress Series; Elsevier; 2006:93-9.
5. Orbañanos IR, Desojo AV, Martinez-Indart L, Bolado GG, Estevez AR, Echevarria IR. Turner syndrome: From birth to adulthood. Endocrinol Nutr 2015;62:499-506.
6. Fjermestad KW, Naess EE, Bahr D, Gravholt CH. A 6-year Follow- up survey of health status in middle-aged women with Turner syndrome. Clin Endocrinol (Oxf) 2016;85:423-9.
7. Yesilkaya E, Bereket A, Darendeliler F, Baş F, Poyrazoğlu Ş, Küçükemre Aydın B, et al. Turner syndrome and associated problems in Turkish children: A multicenter study. J Clin Res Pediatr Endocrinol 2015;7:27-36.
8. Kilic BG, Ergur AT, Ocal G. Depression, levels of anxiety and self- concept in girls with Turner’s syndrome. J Pediatr Endocrinol Metab 2005;18:1111-7.

9. Christopoulos P, Deligeoroglou E, Laggari V, Christogiorgos S, Creatsas G. Psychological and behavioural aspects of patients with Turner syndrome from childhood to adulthood: A review of the clinical literature. J Psychosom Obstet Gynaecol 2008;29:45-51.
10. Messina M, Zirilli G, Civa R, Rulli I, Salzano G, Aversa T, et al. Neurocognitive profile in Turner’s syndrome is not affected by growth impairment. J Pediatr Endocrinol Metab 2007;20:677-84.
11. Hong DS, Reiss AL. Cognition and behavior in Turner syndrome: A brief review. Pediatr Endocrinol Rev 2012;(9 Suppl 2): 710-2.
12. Hong DS, Reiss AL. Cognitive and neurological aspects of sex chromosome aneuploidies. Lancet Neurol 2014;13:306-18.
13. Hong D, Scaletta Kent J, Kesler S. Cognitive profile of Turner syndrome. Dev Disabil Res Rev 2009;15:270-8.
14. Kesler SR. Turner syndrome. Child Adolesc Psychiatr Clin N Am 2007;16:709-22.