Objective To investigate the changes of component and morphology in internal carotid vulnerable plaque,for helping to make clinical intervention strategy individually. Methods A total of 47 patients with internal carotid vulnerable plaques and primary hypertension underwent 2 high-resolution and multi-contrast MRI scans, from March 2008 to April 2014 were retrospectively reviewed. At baseline, the plaque was mainly located at the proximal internal carotid artery,and maximum plaque thickness ≥1.5 mm with intraplaque hemorrhage(IPH)and(or)thin or ruptured fibrous cap.Interscan interval was 0.5 years and above. Patients with carotid occlusion or surgery were excluded. Morphological measurements included maximum plaque thickness, maximum plaque area and cross-sectional vessel area (CSVA) on the level of plaque with maximum thickness. The paired-samples t test was performed to compare the difference of plaque morphology between baseline and follow-up carotid MRI.Results The interscan interval was 1.83 (1.59,1.99)years for 47 internal carotid vulnerable plaques.One case(interscan interval 2.16 years)showed IPH within those 11 plaques without IPH at baseline,and one case(interscan interval 1.42 years)had new incident IPH within those 36 plaques with IPH at baseline. Maximum plaque thickness increased significantly from(3.94±1.44)mm to(4.24±1.68)mm(t=2.30,P<0.05)by 5.14%(-3.83,11.34)% per year. Maximum plaque area increased significantly from(49.19±21.15)mm2to(56.03±24.91)mm2(t=3.87,P<0.01)by 6.67%(-2.26,19.60)% per year.CSVA increased significantly from(66.22±27.51)mm2to(73.68±31.47)mm2(t=4.08,P<0.01)by 5.18%(-1.63,12.34)% per year.Conclusion The progression of component,burden and outer remodeling in the internal carotid vulnerable plaque may be faster in hypertension, therefore reasonable intervention strategy and regular follow-up carotid MRI should be performed.

[Objective] We assessed in a retrospective unicenter study the state of metabolism and gonadal axis of early menarche girls and girls who treated with Gonadotropin-releasing hormone analogs (GnRHa).[Methods] Thirty-nine early menarche girls and 58 girls who had been treated with GnRHa were enrolled in our study and 19 normal menarche girls were enrolled as control group.Data were collected in height,weight,gonadal hormone,blood glucose,insulin,blood lipid,leptin,adiponectin and the size of uterus and ovary.[Results] Both BMI SDS for chronological age (CA) and for bone age (BA) of early menarche girls were significantly higher than normal menarche girls (P ＜ 0.05).The ratio of insulin resistance in early menarche girls (20.5％) was also significantly higher than normal girls (0％).No significant difference in lipid metabolism and gonadal axis between two groups.In girls treated with GnRHa,BMISDS,insulin,HOMA-IR and the ratio of insulin resistance (20.7％) were all significantly higher than normal group (P ＜ 0.05).Meanwhile,DHEAS,androstenedione and testosterone of GnRHa treated girls were significantly higher than early menache girls,and DHEAS was higher than normal girls.The size of uterus in treated group was larger than the other two groups.[Conclusion] Early menarche and GnRHa treatment may take negative effect to BMI and glucose metabolism.Androgen was higher in GnRHa treated group.Therefore,suggestion was that BMI,insulin,blood glucose and androgen should be monitored in early menarche girls and girls treated with GnRHa.

Objective To compare the different efficacies of recombinant human growth hormone (rhGH) alone and rhGH combined with low-does stanozolol on growth velocity (GV) of girls with Turner syndrome (TS). Methods 51 girls with TS were divided into two groups: Group 1 (n = 23) were treated with rhGH alone and group 2 (n = 28) with rhGH combined with low-does stanozolol both for more than six months. The two groups were compared in terms of GV, height standard deviation score for chronologic age (HtSDSCA), HtSDS for bone age (HtSDSBA), HtSDS (ΔHtSDS) and the ratio of ΔBA/ΔCA. Results In the first year, the GV was (6.29 ± 1.44) and (8.13 ± 1.87) cm/a in Group 1 and Group 2, respectively. HtSDSCA changed from (-3.51 ± 0.99) to (-3.19 ± 1.09) and (-4.21 ± 1.19) to (-3.43 ± 1.06), and ΔBA/ΔCA was (0.60 ± 0.39) and (0.77 ± 0.56) in Group 1 and Group 2, respectively. The GV and ΔHtSDS in Group 2 were significantly better than Group 1 (P < 0.05). The GV was negatively correlated with the age. Conclusion Compared with the therapy with rhGH alone, the one with rhGH combined with low-dose stanazolol is more effective in improving GV without accelerating bone maturation among the girls with Turner syndrome.

Objective To study the effect on body mass index ( BMI ) caused by treatment with Gonadotropin-releasing hormone analogs ( GnRHa) in girls with central precocious puberty ( CPP) or early and fast puberty ( EFP ) . Methods The BMI in 318 girls ( 227 CPP and 91 EFP ) treated with GnRHa alone was analyzed. Among them 89 were followed up to their final adult height. Results Before GnRHa treatment was started, thegirlswithCPPandEFPhadameanBMIstandarddeviationscore(SDS)forchronologicalage(BMISDSCA)of0.39 ±0.84andforboneage(BMISDSBA)of-0.11±0.69. Attheendoftreatment,themeanBMISDSCAwas0.59±1.01 and BMISDSBA was 0. 24 ± 0. 89, both were significantly higher than those at initiation. The increment in BMISDSBA (0.38±0.50)wasgreaterthanthatinBMISDSCA(0.21±0.56). Moreover,theratioofoverweight(BMI>85%)was significantly elevated. BMISDSCA of 89 girls who were visited at their final adult height was 0. 17 ± 1. 15, which was similar with BMISDS at initiation and in normal population. Conclusion The mean BMISDSCA of CPP and EFP was significantly higher than the general population, while BMISDSBA was significantly lower. During GnRHa treatment, BMISDS tended to be elevated. But it was reversible, for the mean BMISDS was back to normal at final adult height.

Objective To investigate the changes of pulsatile tinnitus on HRCT and MRI CE 3D-SPGR image.Methods CT and MRI images of 1 5 pulsatile tinnitus patients were retrospective analysis.All 1 5 patients underwent temporal bone HRCT and MRI CE 3D-SPGR scan.Abnormal changes on CT and MRI image,which caused the pulsatile tinnitus,were compared.Results In 1 5 patients,9 sides (8 cases)showed abnormal sigmoid sinuses including 4 sides of sigmoid sinuses diverticulum,5 sides sigmoid sinu-ses uncovering,8 sides dominant sigmoid sinuses.The high jugular bulb was showed in 10 sides (8 cases),the thick emissaria mas-toidea in 5 sides (4 cases),bilateral semicircular canal dehiscence in one case,glomus tympanicum tumor in one case and the cochle-ar nerve was constricted by small vessel in one case.Conclusion Cranial base vessel abnormity can be visualized more precisely on MRI CE 3D-SPGR image than that on HRCT.On the contrary,the detection of temporal bone abnormalities is superior on HRCT.

Next-generation sequencing (NGS) technologies have improved as well as the costs have gradually decreased in the detections of genetic diseases. This article describes the principle, platform, and data analysis of NGS and the application of NGS technologies to the molecular diagnosis of hereditary hearing loss (HL). The use of NGS technologies makes the discovery of HL genes more feasible than ever. And the data obtained by NGS used in genetic counseling for clinical practice may assist in defining genetic profiles of HL individuals and expedite the pace of personalized medical care.
Hearing Loss, Sensorineural ; diagnosis ; genetics ; High-Throughput Nucleotide Sequencing ; Humans

Forty girls with Turner's syndrome (TS),aged (12.6 ± 1.9) years,were treated with daily subcutaneous injection of recombinant human growth hormone (rhGH,1.0 ～ 1.1 IU · kg-1 · w-1) and oral stanozolol (0.02 ～ 0.04 mg · kg-1 · d-1) for 1 ～ 5 years.Growth velocity (GV),height standard deviation score (SDS) by reference of healthy Chinese girls (HtSDSNor) and height SDS by reference of untreated Chinese TS girls (HtSDSTs)were evaluated regularly.Of the forty girls studied,thirteen had discontinued the treatment after a mean duration of (2.9 ± 1.2)years when GV was less than 2 cm/year or when patients were satisfied with the achieved height.Final adult height (FAH) or near-final height,which was defined as the most recent available height after discontinuation of treatment,and the height gained in the thirteen girls were evaluated.Estrogen therapy was started at the age of(16.0 ± 1.1) years.HtSDSNor increased from-4.2 ± 1.0 to-3.4 ± 1.0 in the first year,and-2.8 ± 1.0,-2.4 ± 0.8,-2.5 ± 0.5,-2.3 ±0.3 respectively in the 2nd,3rd,4th,and 5th year.The change in HtSDSTs was similar to HtSDSNor.It was increased from 0.1 ± 0.9 to 1.0 ± 0.9 in the first year,and to 1.5 ± 0.8,1.9 ± 0.6,1.7 ± 0.4,1.7 ± 0.2 in the subsequent 4 years.The predicted adult height (PAH) in 13 girls was (142.8 ± 4.2) cm before treatment.FAH was (151.7 ± 4.1) cm,which was significantly higher than PAH (P＜0.01),and the mean height gain was (8.9 ± 2.8) cm (5.1 ～ 12 cm).FAHSDSNor was increased to-1.6 ± 0.8 from-3.8 ± 0.8.For girls with TS around 9 years of age,combined therapy with rhGH and low dosage of stanazolol may significantly increase growth velocity and improve final adult height.

Objective To explore the relationship between adrenarche and gonadarche.Methods Total 49 idiopathic central precocious puberty(ICPP)girls,whose serum dehydroepiandrosterone sulfate(DHEAS)Z scores for chronological age were higher than+2 s at diagnosis.were enrolled.Physical examinations during pubertal stage were repeated at 3-6 months intervals,and serum DHEAS levels were monitored yearly within an average period of 4.08 years.Of them,16 girls were followed up until more than one year after discontinuation of gonadotropin-releasing hormone analogue(GnRHa)treatment.Results Before GnRHa treatment,these49 girls presented a younger average age at attainment of pubic hair stage2(PH2)and pubic hair stage3(PH3)than normal(8.07 years vs 11.16 years,8.82 years vs 12.40 years respectively).During GnRHa treatment,the intervals between PH2 and PH3,PH3 and pubic hair stage4(PH4),breast stage 2(B2),and PH2 were longer than normal(1.69 years vs 0.83 years,1.64 years vs 0.60 years,and3.62 years vs 0.76 years respectively).The intervals between PH2 and PH3,as well as B2 and PH2 during GnRHa treatment were also longer than that before GnRHa treatment(1.69 years/35 0.88 years,3.62 years vs 1.13 years respectively).The serum DHEAS Z scores decreased during GnRHa treatment,and increased significantly after GnRHa cessation.Conclusion Gonadarche after age of 6-year-old may lead to earlier adrenarehe.GnRHa treatment might slow down the progression of adrenarche and suppress the hypothalamuspituitary-gonadal axis.

Objective To analyze the clinical characters of childhood adrenocortical tumors, and to enhance the knowledge of diagnosis of this disease. Methods A retrospective analysis of clinical characters,laboratory tests,and imaging findings in 31 cases of childhood adrenocortical tumors was carried out. Results 16 cases of adenoma and 15 cases of carcinoma were included. The average age was (4.49±3.51) years old, and 67.7% of the patients were younger than 5 years old. The ratio of male to female was 1.0: 1.4. 12 patients presented only precocious sexual development, 4 patients presented only Cushing syndrome, 10 patients showed sexual precocity combined with Cushing syndrome, and 5 patients did not have any endocrine abnormalities. Raised testosterone level in 92.3% of these patients was the most common finding in laboratory tests. Only 12.5% of ultrasound images and 20.8% of CT images were consistent with pathologic diagnosis. Conclusions The clinical manifestations of adrenocortical tumors in childhood are precocious sexual development, Cushing's syndrome, or nonfunctional. The common laboratory findings are elevation of sex hormone and disorder of cortisol circadian rhythm. Precocious sexual development and elevation of androgens are more common in childhood adrenocortical tumor than those in adults. Imaging usually cannot give proper diagnosis. Final diagnosis should be established by clinical features, laboratory tests, imaging, and pathologic results.

Objective:To study the relationship between genetic polymorphisms of GSTM1 GSTT1 and the susceptibility of laryngeal and hypopharyngeal carcinomas(LHC).Method:The GSTM1 an GSTT1 genotypes were determined by multiplex PCR analysis in 76 LHC patients and 76 population controls.The association be tween the genotypes and LHC risk was measured by odds ratios(ORs)and 95% confidence intervals(95%Cls).Resuit:The frequency of GSTM1 null genotype was 59.2% in the LHC patients and 42.1% in controls(OR=1.935,95%CI=1.069-3.510),the difference was significant(P＜0.01).The frequency of GSTT1 null genotype was 57.9% in the LHC patients and 51.3% in controls.The difference was not significant(P＞0.05).In smokers,the risk of the LHC increased in subjects of GSTM1 null genotype(OR=5.545,95%CI=2.158-13.528).Conclusion:GSTM1 polymorphisms are associated with susceptibility to the LHC.It has the synergistic effects with smoking in the development of the LHC.GSTT1 genotypes might have no association with risk of the LHC in urban Linyi.