BACKGROUND: Turner syndrome (TS) is a sex chromosome abnormality in females characterized by gonadal dysgenesis, short stature and skeletal malformations like kyphosis and scoliosis. AIM: To evaluate the prevalence of scoliosis and its incidence over 4 year follow-up. DESIGN: Consists in two parts: cross sectional study and longitudinal study. SETTING: Outpatient. POPULATION: Forty-nine TS assessed at the Pediatric Outpatients Clinic. METHODS: Clinical and radiological evaluation of spine. RESULTS: Cross sectional study: at baseline an high prevalence of minor scoliosis was observed (59%, 95% CI 44-73). The prevalence increased with age (trend test P=0.01). Patients with scoliosis were more frequently on GH therapy (69% vs. 35%, P=0.023). At multivariable analysis (including age, height and GH therapy), height was the only independent correlate of scoliosis. Longitudinal study: of the 20 cases without scoliosis at baseline, 9 were diagnosed with new scoliosis (classified as minor ) after 4 years (incidence of 45% , 95% CI 23-68). We didn't found any predictor of new scoliosis; patients who developed scoliosis 4 years later were older and taller at baseline. CONCLUSION: TS have a higher risk to develop scoliosis and the age at risk is protracted further with respect to normal subjects. This risk appears influenced by the height of the patient and, indirectly, by the GH therapy. Clinical rehabilitation impact. In TS is necessary a prolonged time of observation (until age twenty) for identifying scoliosis and beginning a rehabilitation program.
BACKGROUND:Turner syndrome (TS) is a sex chromosome abnormality in females characterized by gonadal dysgenesis, short stature and skeletal malformations like kyphosis and scoliosis. AIM: To evaluate the prevalence of scoliosis and its incidence over 4 year follow-up. DESIGN: Consists in two parts: cross sectional study and longitudinal study. SETTING:Outpatient. POPULATION: Forty-nine TS assessed at the Pediatric Outpatients Clinic. METHODS: Clinical and radiological evaluation of spine. RESULTS: Cross sectional study: at baseline an high prevalence of minor scoliosis was observed (59%, 95% CI 44-73). The prevalence increased with age (trend test P=0.01). Patients with scoliosis were more frequently on GH therapy (69% vs. 35%, P=0.023). At multivariable analysis (including age, height and GH therapy), height was the only independent correlate of scoliosis. Longitudinal study: of the 20 cases without scoliosis at baseline, 9 were diagnosed with new scoliosis (classified as minor ) after 4 years (incidence of 45% , 95% CI 23-68). We didn't found any predictor of new scoliosis; patients who developed scoliosis 4 years later were older and taller at baseline. CONCLUSION: TS have a higher risk to develop scoliosis and the age at risk is protracted further with respect to normal subjects. This risk appears influenced by the height of the patient and, indirectly, by the GH therapy. Clinical rehabilitation impact. In TS is necessary a prolonged time of observation (until age twenty) for identifying scoliosis and beginning a rehabilitation program.
Authors: Philippe Backeljauw; Shankar Kanumakala; Sandro Loche; Karl Otfried Schwab; Roland Werner Pfäffle; Charlotte Höybye; Elena Lundberg; Tadej Battelino; Berit Kriström; Tomasz Giemza; Hichem Zouater Journal: Horm Res Paediatr Date: 2021-07-07 Impact factor: 2.852