| Turner syndrome(TS) due to chromosomalabnormalities caused womendiseases and showed that patients with TS in the incidence rate of female live births was about1/2000~1/2500[1].Butperhaps because of the clinical phenotype of patients with mild failure to get a diagnosis, so it was difficult to determine the actual prevalence rate.TS usually had special signs of typical, but different karyotypesmay had different effects on the clinical manifestations of patients.It was reportedthat 76.4%~94.5% of TS patients [2-4] showed short stature, it had been confirmed that the effective way for the treatment ofrecombinant human growth hormone(recombinant human growth hormone, rh GH). Research showed that about 80% of patients with TS in children withgrowth hormonetherapy for at least 4 years after the heightcould reachthe range of normal children [5].The disease due to chromosome number or structure resulting fromabnormal most patient did not have self-conscious adolescent development, so application of estrogen replacement therapy(ERT) promoted patients with secondary syndrome development and the prevention of its complications.Thisstudy intended to analyze the clinical features of TS patients and the clinical outcome after treatment.All TS patients were selected from Nanism Clinic of Endocrinology Department of Peking Union Medical College Hospital from January2008 to August 2015,a retrospective analysis was performed a clearkaryotype of 192 cases of TS patients,to explore the clinical characteristics and clinical outcome after treatment of different karyotype.In order toprovide more evidence for clinical diagnosis and treatment.This retrospective study of clinical data of 192 cases of patients with TS were statistically analyzed.It was found that the proportion of 45 X was themost and there were differences in the special signs of different karyotype.TS patients with hypothyroidism or subclinical hypothyroidism incidence ratewas 44.3%.The incidence of TGAb and TPOAb abnormalitywere 30.7%,the incidenceof TGAb was42.1%,the incidence of TPOAb was 37.5%.The incidence of abnormal liver function in TS patients in patients with TS was29.6%.Rh GH therapy in patients with TS after3 months,6 months,12 months,growth velocity were(8.5±4.4)cm/y、(8.1±3.2)cm/y、(7.4±2.6) cm/yrespectively, significantly lower than annual growth velocity of GHDpatients(10.8±3.1)cm/y 、(10.3±2.5)cm/y 、( 9.2±2.6)cm/y.TS patients with regular rh GH combined with estrorogen therapy or singleestrogen replacement therapy after the average final height were 151.0 cm and 148.0 cm,were higherthan the final height of patients without any treatment.Estrogen treatment after one year, 1~3 years,>3 years,L2-4 BMD were(0.96±0.13) g/cm2、(0.92±0.13) g/cm2 and(0.93±0.14) g/cm2 respectively, significantly higher than basline(0.73±0.08) g/cm2、(0.70±0.13) g/cm2 、(0.75±0.07) g/cm2.Thepatients were divided into 2 groups according to the treatment age,the BMD L2-4 ofwhoseestrogen therapy was initiated before 18 years old(0.96±0.14)g/cm2 was significantly higher than the girls who startedestrogentherapy after18(0.90±0.10)g/cm2.To sum up, TS patients due to the differences in chromosome karyotype may had different clinical signs.The incidence of abnormal thyroidfunction in TS patients was significantly higherthanthat in the normal group.Although the effect of rh GH treatment on TS patients is worse than that of GHD patients, but after treatment can significantly improve the patient’s final height and rh GH combined with estrogen treatment can better improve the final height of TS patients.Rh GH treatment can increase the incidence of abnormal liver function, But there was no significant correlation with abnormal glucose metabolism.Early estrogen replacement therapy can effectively improve BMD in patients with TS. |
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