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Objective:To analyze clinical and imaging features of Sturge-Weber syndrome in children.Methods:Clinical data were collected from 27 children with Sturge-Weber syndrome in Xuzhou Children′s Hospital, Xuzhou Medical University from July 2013 to December 2019, and analyzed retrospectively.Results:Among the 27 children, 17 were males and 10 were females. Their age at the clinic visit ranged from 2 days to 10 years and 7 months, and averaged 2.54 years. All the 27 patients presented with facial port-wine stains of varied color from light red to purple red, which were all distributed across the facial midline, including 21 with predominantly unilateral port-wine stains and 6 with bilateral symmetrical port-wine stains. There were 17 patients with ocular choroidal vascular malformations, including 14 with congenital glaucoma, 5 with high intraocular pressure, and 1 with optic nerve atrophy accompanied by transient blindness. Neurological impairment occurred in 12 patients, and all manifested as epilepsy. All the 27 children underwent imaging examination, and abnormalities were found in 20. Among the 10 patients with abnormal computed tomography images, local calcification was observed in 8, and local thickening of the skull on the side affected by skin lesions in 8; 13 of 14 patients with abnormal magnetic resonance imaging scan results had signs of brain atrophy, 9 showed enhanced gyrus-like blood vessel formation by enhanced magnetic resonance imaging, and 5 showed decreased branches of the anterior and middle cerebral artery on the affected facial side by magnetic resonance angiography.Conclusions:Children with Sturge-Weber syndrome are clinically characterized by predominantly unilateral port wine stains on the face, some of whom are accompanied by epilepsy, glaucoma or mental retardation, and imaging examinations mainly show local calcification, brain atrophy, local thickening of the skull plate, enhanced gyrus-like blood vessel formation, etc. Early definite diagnosis and comprehensive systemic treatment are needed to reduce disability and mortality rates in patients with Sturge-Weber syndrome, and long-term follow-up should be considered.
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Objective This study aims to establish a reference range of thyroid function indicators for women in different periods of pregnancy in Hubei Province.Methods Using the prospective method,1 487 healthy pregnant women who did pregnancy check-up at Wuhan Maternal and Child Healthcare Hospital from March 2017 to May 2018 were asked to complete questionnaires and whose serum samples were examined,aged (29 ± 4) years within the range of 20-41,include 498 in early pregnancy,525 in middle pregnancy,and 464 in late pregnancy.Then their thyroid stimulating hormone (TSH),free triiodothyronine (FT3) and free thyroxine (FT4) levels were determined using chemiluminescence immunoassay method to establish reference ranges of the indexes of thyroid function,reference ranges were in median (M) and percentage (P2.5-P97.5).At the same time,the newly established reference range was compared with the reference range recommended by the American Thyroid Association (ATA) guidelines to evaluate the thyroid function of 10 232 pregnant women in counties (cities,districts) of Hubei Province.Results Reference ranges in early pregnancy,middle pregnancy and late pregnancy were shown as follows,TSH:1.41 (0.19-4.26),1.45 (0.25-4.39),2.04 (0.51-5.10) mU/L;FT3:4.76 (3.78-16.01),4.65 (3.72-5.91),4.22 (3.41-5.33) pmol/L;FT4:63.71(47.40-86.25),58.28 (41.18-82.36),48.95 (33.41-66.82) nmol/L.The thyroid function assessment of pregnant women with the newly established reference range shows that the detection rate of thyroid dysfunction in pregnant women is was 13.58％ (1 390/10 232),which was lower than 37.98％ (3 886/10 232,x2 =102.58,P ＜ 0.05) diagnosed by the ATA guidelines.Conclusions The reference range of women's specific thyroid function indicators in different stages of pregnancy in Hubei Province is established.Compared with the reference range recommended by ATA,it is more suitable for thyroid function monitoring and thyroid disease screening for pregnant women in Hubei Province.
ABSTRACT
Objective To establish the laboratory and geography-specific reference intervals for thyroid hormones during pregnancy and changes of serum thyroid hormone in Wuhan. Methods According to NACB guidelines,a total of 1 186 female including in or without pregnancy were recruited in our hospital randomly when they came to routine antenatal clinic. The 2.5 th and 97.5 th percentiles were calculated as the reference intervals for specialty of serum thyroid function including serum thyroid stimulating hormone(TSH),serum free three io-dine thyroid(FT3),serum free thyroxine(FT4)levels during each trimester by chemiluminescence.Results On comparing to non-pregnant women,the serum thyroid hormone in pregnant women had significant difference as the following:(1)FT3 concentrations showed decreased median centile throughout gestation step by step;(2)FT4 lev-els in second and third trimester had slightly decreased;(3)TSH levels had significant decreased in first and sec-ond trimeste and had no significant difference between the early pregnancy and middle pregnancy,and had recoved to the similar level of before pregnancy in middle pregnancy and late pregnancy.Compared with control group,the serum thyroid function levels of pregnant women had significant differences in trimesters. There were 5.84%, 19.09% and 28.91% of women in the first,second and third trimester,respectively,with serum TSH higher than the upper reference limit,or lower than the lower reference limit.Conclusions Serum thyroid function had signifi-cant correlations with pregnancy in Wuhan area. To establish pregnancy-specific thyroid function reference inter-vals of local area can reduce underdiagnosis effectively in thyroid gland related diseases.