Disorders of the eye, ear, skin, and nervous system in women with Turner syndrome -a nationwide cohort study

The literature about eye, ear, nose, skin, and nervous system disorders in women with Turner syndrome is equivocal. Impaired vision and hearing in women with Turner syndrome have been described, and case reports of Turner syndrome girls suffering from epilepsy have been published, but no large popul...

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Bibliographic Details
Published in:European journal of human genetics : EJHG 2022-02, Vol.30 (2), p.229-236
Main Authors: Viuff, Mette Hansen, Stochholm, Kirstine, Juul, Svend, Gravholt, Claus Højbjerg
Format: Article
Language:English
Subjects:
Atopic dermatitis
Case reports
Cataracts
Cohort analysis
Cohort Studies
Cytogenetics
Ear
Epilepsy
Eye disorders
Female
Females
Genetic disorders
Glaucoma
Hearing loss
Hormone replacement therapy
Humans
Incidence
Nervous System
Nose
Otitis media
Population studies
Population-based studies
Psoriasis
Registries
Skin
Skin diseases
Statistics
Turner Syndrome - complications
Turner Syndrome - drug therapy
Turner Syndrome - epidemiology
Turner's syndrome
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Summary:The literature about eye, ear, nose, skin, and nervous system disorders in women with Turner syndrome is equivocal. Impaired vision and hearing in women with Turner syndrome have been described, and case reports of Turner syndrome girls suffering from epilepsy have been published, but no large population-based-studies have explored the occurrence of any of these disorders. We aimed to investigate the risk of admission with disorders related to the eye, ear, nose, skin, and nervous system, compared with background females, and the impact of hormone replacement therapy on these conditions. 1,156 females with TS diagnosed during 1960-2014 were identified using the Danish Cytogenetic Central Registry and linked with personal-level data from the National Patient Registry and the Medication Statistics Registry. Statistics Denmark randomly identified 115,577 age-matched background females. Negative binomial regression was used to analyze hospital discharge diagnoses, reporting incidence rate ratios (IRR). Women with Turner syndrome have an increased risk of developing eye disorders (IRR 4.3 (95% CI 3.5-5.4), including cataract, glaucoma, ocular movement, and accommodation. The risk of ear disorders (IRR 35.0 (27.9-43.9)) and nose (IRR 2.2 (1.4-3.6)) was increased in women with Turner syndrome, due to otitis media, cholesteatoma, and hearing loss. Disorders of the nervous system such as epilepsy were increased IRR 6.2 (2.4-15.9), along with skin conditions IRR 2.2 (95%CI 1.7-2.7) like psoriasis, atopic dermatitis, and ingrown nails.
ISSN:1018-4813
1476-5438
DOI:10.1038/s41431-021-00989-5