Literature DB >> 16759035

The psychosocial impact of Klinefelter syndrome--a 10 year review.

P J Simm1, M R Zacharin.   

Abstract

OBJECTIVE: To describe psychosocial morbidity in a cohort of young males with hypogonadism due to Klinefelter syndrome, to document the effect of androgen replacement on behaviour, to underline issues confronting clinicians involved in treatment of this condition and to demonstrate a need for a structured program for prospective intervention for this group. We also compare this group to young men with hypogonadotrophic hypogonadism.
DESIGN: A retrospective audit of patients with Klinefelter and Kallmann syndromes, presenting for medical assessment from 1994-2004. PATIENTS: Postpubertal males with Klinefelter syndrome (n = 32) and Kallmann syndrome (n = 19) were audited by chart review for psychosocial comorbidities, pubertal management, and the need for exogenous testosterone.
RESULTS: Seventeen of 32 postpubertal patients with Klinefelter syndrome required testosterone therapy while 11 were documented to have serum testosterone in the normal adult range. All patients with Kallmann syndrome required long term testosterone treatment. Significant psychosocial and behavioural problems were present in 22/32 of patients with Klinefelter syndrome, including seven who were testosterone replete, with an identifiable pattern of disorder, including marked lack of insight, poor judgement and impaired ability to learn from adverse experience. Use of long term replacement testosterone treatment reduced episodes of behavioural indiscretion. Of those patients with Kallmann syndrome, 5/19 reported mild depressive symptoms only, all resolving with testosterone replacement.
CONCLUSION: Inadequately treated hypogonadism in Klinefelter syndrome increases recognized psychosocial morbidity. There is a need for prospectively planned and timed support for young men with Klinefelter syndrome, to ameliorate current poor psychosocial outcomes.

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Year:  2006        PMID: 16759035

Source DB:  PubMed          Journal:  J Pediatr Endocrinol Metab        ISSN: 0334-018X            Impact factor:   1.634


  10 in total

Review 1.  Consensus statement on diagnosis and clinical management of Klinefelter syndrome.

Authors:  A F Radicioni; A Ferlin; G Balercia; D Pasquali; L Vignozzi; M Maggi; C Foresta; A Lenzi
Journal:  J Endocrinol Invest       Date:  2010-12       Impact factor: 4.256

2.  Neural systems for social cognition in Klinefelter syndrome (47,XXY): evidence from fMRI.

Authors:  Sophie van Rijn; Hanna Swaab; Daan Baas; Edward de Haan; René S Kahn; André Aleman
Journal:  Soc Cogn Affect Neurosci       Date:  2011-07-06       Impact factor: 3.436

3.  A case of Klinefelter syndrome, mosaicism (46,XY/47,XXY), associated with anorexia nervosa.

Authors:  A Gritti; F Salerno; S Pisano; F Formicola; D Melis; A Franzese
Journal:  Eat Weight Disord       Date:  2010-11-04       Impact factor: 4.652

Review 4.  Neuroanatomical and molecular correlates of cognitive and behavioural outcomes in hypogonadal males.

Authors:  O B Akinola; M O Gabriel
Journal:  Metab Brain Dis       Date:  2017-12-11       Impact factor: 3.584

Review 5.  Klinefelter syndrome: the commonest form of hypogonadism, but often overlooked or untreated.

Authors:  Eberhard Nieschlag
Journal:  Dtsch Arztebl Int       Date:  2013-05-17       Impact factor: 5.594

6.  Pubertal induction and transition to adult sex hormone replacement in patients with congenital pituitary or gonadal reproductive hormone deficiency: an Endo-ERN clinical practice guideline.

Authors:  A Nordenström; S F Ahmed; E van den Akker; J Blair; M Bonomi; C Brachet; L H A Broersen; H L Claahsen-van der Grinten; A B Dessens; A Gawlik; C H Gravholt; A Juul; C Krausz; T Raivio; A Smyth; P Touraine; D Vitali; O M Dekkers
Journal:  Eur J Endocrinol       Date:  2022-04-21       Impact factor: 6.558

7.  Social behavior and autism traits in a sex chromosomal disorder: Klinefelter (47XXY) syndrome.

Authors:  Sophie van Rijn; Hanna Swaab; André Aleman; René S Kahn
Journal:  J Autism Dev Disord       Date:  2008-03-07

Review 8.  Sex differences in psychiatric disorders: what we can learn from sex chromosome aneuploidies.

Authors:  Tamar Green; Shira Flash; Allan L Reiss
Journal:  Neuropsychopharmacology       Date:  2018-07-16       Impact factor: 7.853

Review 9.  Klinefelter syndrome: From pediatrics to geriatrics.

Authors:  Koji Shiraishi; Hideyasu Matsuyama
Journal:  Reprod Med Biol       Date:  2018-12-08

10.  Navigating Disrupted Puberty: Development and Evaluation of a Mobile-Health Transition Passport for Klinefelter Syndrome.

Authors:  Andrew A Dwyer; Vanessa Héritier; Sofia Llahana; Lauren Edelman; Georgios E Papadakis; Laurent Vaucher; Nelly Pitteloud; Michael Hauschild
Journal:  Front Endocrinol (Lausanne)       Date:  2022-06-24       Impact factor: 6.055

  10 in total

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