Otological problems in children with Turner's syndrome

Stenberg, Annika Elmqvist; Nylén, O; Windh, Marianne; Hultcrantz, Malou

doi:10.1016/s0378-5955(98)00113-0

Cited by 93 publications

(87 citation statements)

References 14 publications

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“…This increased frequency of middle ear infections has been attributed to the congenital craniofacial malformation with possible distortion of the Eustachian tube and impaired ventilation of the middle ear. In keeping with this, auricular anomalies have been noted, primarily in females with the 45,X karyotype, but in fewer patients than were affected by middle ear infections (3). In addition to middle ear infections, pneumonia (relative risk (RR): 12 (95% confidence interval (CI): 4 -26)) and diseases of the respiratory system (RR: 8 (95% CI: 4-15)) are frequent causes of death in TS in comparison with the background population (4), suggesting a defective immune system leading to increased susceptibility to infections.…”

Section: Introductionsupporting

confidence: 53%

The effects of GH and hormone replacement therapy on serum concentrations of mannan-binding lectin, surfactant protein D and vitamin D binding protein in Turner syndrome

Gravholt

Leth-Larsen

Lauridsen³

et al. 2004

European Journal of Endocrinology

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Objective: Studies in animals and humans indicate that growth hormone (GH) and insulin-like growth factor-I (IGF-I) modulate immune function. Recently, it was reported that GH therapy increased the level of mannan-binding lectin (MBL) in normal patients, and that treatment of acromegalics with pegvisomant decreased the levels of MBL. The effect on MBL was thought to be due to a specific action of GH, since IGF-I treatment did not affect MBL. Whether it is advantageous or not to have high or low levels of MBL is not known. Likewise, it is not clear how the modifications induced by GH affect immune function. In the present study we examined whether GH or hormone replacement therapy (HRT) in Turner syndrome (TS) influence the serum concentrations of MBL and two other proteins partaking in the innate immune defence, surfactant protein D (SP-D) and vitamin D binding protein (DBP). Design: Study 1: a double-blind crossover study of 12 healthy TS adolescents examined during treatment with either placebo or GH for 2 months, and compared with a control group. Study 2: tripleblind crossover study of 9 healthy TS adolescents randomized to treatment with placebo, GH or GH þ 17b-estradiol. Study 3: 60 adult TS patients (55 received HRT) compared with 59 age-matched controls. Study 4: 27 patients with TS were examined before and during sex hormone replacement with 17b-estradiol and norethisterone and compared with age-matched controls (n ¼ 24). Methods: Measurement of MBL, SP-D, DBP, and other inflammation markers. Results: Study 1: the levels of MBL (P ¼ 0.002) and SP-D (P ¼ 0.012) increased during GH treatment, whereas no changes were observed in comparison with controls. DBP was unchanged by GH, but was significantly higher in TS compared with controls (P ¼ 0.017). Study 2: treatment with GH increased MBL (P ¼ 0.045) and SP-D (P ¼ 0.05) concentrations in TS, while treatment with GH þ 17b-estradiol did not increase levels further. DBP was unchanged by treatment. Study 3: levels of MBL, SP-D, and DBP were similar in adult TS and control subjects. Study 4: DBP levels decreased in response to HRT, while MBL and SPD levels were unchanged. Levels of all three plasma proteins were similar to controls. Conclusion: We show that treatment with GH significantly increases MBL and SP-D concentrations in TS, while HRT marginally decreases DBP. Whether the present findings, suggesting a link between the endocrine and the immune system, have clinical consequences needs to be studied further. European Journal of Endocrinology 150 355-362

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Section: Introductionsupporting

confidence: 53%

The effects of GH and hormone replacement therapy on serum concentrations of mannan-binding lectin, surfactant protein D and vitamin D binding protein in Turner syndrome

Gravholt

Leth-Larsen

Lauridsen³

et al. 2004

European Journal of Endocrinology

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“…Recurrent otitis media occurs in most girls with Turner syndrome. In addition, a progressive midfrequency sensorineural hearing loss often occurs, which necessitates the use of hearing aids in some children 49 and more than 25% of women by their mid-forties. 50 …”

Section: Hearing Lossmentioning

confidence: 99%

Health Supervision for Children With Turner Syndrome

Frías¹,

Davenport²

2003

Pediatrics

108

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ABSTRACT. This report is designed to assist the pediatrician in caring for the child in whom the diagnosis of Turner syndrome has been confirmed by karyotyping. The report is meant to serve as a supplement to the American Academy of Pediatrics' "Recommendations for Preventive Pediatric Care" and emphasizes the importance of continuity of care and the need to avoid its fragmentation by ensuring a medical home for every girl with Turner syndrome. The pediatrician's first contact with a child with Turner syndrome may occur during infancy or childhood. This report also discusses interactions with expectant parents who have been given the prenatal diagnosis of Turner syndrome and have been referred for advice.

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“…Conductive deafness is attributed to congenital craniofacial abnormalities which cause distortion of the eustachian tubes and defective ventilation, as well as impaired mucociliary transport [100]. The resulting otitis media affects up to 68–78% of patients [6], and of these 43% have been reported to have conductive hearing loss [101]. …”

Section: Clinical Manifestations Of Turner Syndrome In Adultsmentioning

confidence: 99%

“…It is known to progress with age and may be due to a premature aging process. It has been reported in 58% of girls, down to the age of 6 years [101], and in 61% of women over 35 years of age [49]. Oestrogen receptors have been demonstrated in the inner ear which may be of significance [102].…”

Section: Clinical Manifestations Of Turner Syndrome In Adultsmentioning

confidence: 99%

Adulthood in Women with Turner Syndrome

Ostberg

Conway²

2003

Horm Res Paediatr

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Turner syndrome, resulting from a complete or partial absence of one X chromosome, is the most commonly occurring chromosomal abnormality in females. Patients have traditionally been carefully followed in paediatric practice during childhood, but were often discharged to primary care on reaching adulthood. Adults with Turner syndrome are thought to have a reduced life expectancy, mainly due to excess cardiovascular risk, but they may also have multiple comorbidities including hypothyroidism, deafness, osteoporosis and the attendant problems of oestrogen deficiency and infertility. A multidisciplinary approach to focused adult care is needed, with consideration of how to optimise surveillance strategies in these women.

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Otological problems in children with Turner's syndrome

Cited by 93 publications

References 14 publications

The effects of GH and hormone replacement therapy on serum concentrations of mannan-binding lectin, surfactant protein D and vitamin D binding protein in Turner syndrome

The effects of GH and hormone replacement therapy on serum concentrations of mannan-binding lectin, surfactant protein D and vitamin D binding protein in Turner syndrome

Health Supervision for Children With Turner Syndrome

Adulthood in Women with Turner Syndrome

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