Objective To explorethe influence of leptin on the onset of puberty, and to study the relationship between leptin and hormones relevant to gonadal axis and its effect on idiopathic central precocious puberty (ICPP) in obese girls. Methods Fifty-six girls with ICPP fulfilled the current diagnostic criteria and came from pediatric endocrinology clinic. The 56 cases were divided into 2 groups according to whether there was obesity or not: 38 non-obese girls with ICPP and 18 obese girls with ICPP. Forty-three age-matched prepubertal girls were divided into non-obese prepuberty group (25 girls) and obese prepuberty group (18 girls). Serum leptin wasassayedwithELISA,andserum FSH, LH and E 2 were determined with immunochemiluminescent. Results As compared with non-obese prepuberty group [(4.1?1.5)?g/L], the leptin concentrations in obese ICPP group [(14.7?7.5)?g/L], non-obese ICPP group [(8.8?5.1)?g/L] and obese prepuberty group [(8.0?5.3)?g/L] were significantly higher (P

Objective To analyze the clinical characteristic s of an infant with congenital lipoid adrenal hyperplasia (CLAH),and to sequence the acute regulatory protein (steroid acute regulatory,StAR) gene of the infant patient and her pedigree.Methods Physical examination,laboratory tests,and imaging examination of the 11-month-old patient with CLAH were collected.DNA was extracted from blood samples of the patient and her parents.The 7 exons of StAR gene were amplified by PCR and then sequenced.Results Dark skin,girl vulva,and one each 1.0 cm×1.0 cm palpable lump in inguinal area bilaterally were observed.The adrenocorticotropic hormone (ACTH) was 253 pg/ml,cortisol was 27.6 nmol/L at 8 am,17-hydroxyprogesterone was 3 nmol/L.Uhrasound showed that sign of testicular ultrasonography existed in bilateral inguinal regions.Karyotype analysis showed 46,XY.Sequencing of PCR amplified fragments showed that there were two heterozygous mutations c.229C ＞ T,p (Gln77X) and C.659A＞G,p (His220Arg) of StAR gene in this patient.By rectifying the disturbance of electrolyte,and treating with hydrocortisone and 9α fludrocortisone,etc,the patient has been stable so far.Conclusion The patient presents typical clinical manifestations.Two heterozygous mutations including c.229C ＞T,p (Gln77X) from maternal and C.659A＞G,p (His220Arg) from paternal of StAR gene were detected.Wherein the c.659A＞G,p.(His220Arg) as a novel point mutation of StAR gene,has not been reported so far.

Objective Turner syndrome (TS) is characterized with reduced adult height and gonadal dysgenesis and associated with a number of complications including thyroid disease.The purpose of this study was to investigate the prevalence of thyroid diseases and the association between thyroid autoantibodies (TAA) and thyroid dysfunction,age,and karyotype.Methods Fifty-five girls with TS were diagnosed by chromosome analyses and were divided into 2 groups according to whether there was TAA-positive or not:TAA-positive group and TAA-negative group.Thyroid autoantibodies (antithyroglobulin antibody,thyroperoxidase antibody),thyroid function (free T3,free T4,and TSH) were determined with immunochemiluminescent.Ultrasound was applied in TAA-positive group.Results Thyroid functions in 34 TAA-negative girls were normal.Of the 21 TAA-positive girls (21/55,38.2％),7 girls suffered from hypothyroidism and 3 girls had hyperthyroidism.All of 21 TAA-positive girls were diagnosed as cases of Hashimoto's thyroiditis.As compared with the girls in TAA-negative group,the age of girls in TAA-positive group was significantly higher [(12.16 ± 2.55 vs 9.95 ± 4.50) years,P＜0.05].6 cases under 5 years old were TAA-negative.31.3 ％ (5/16) of patients aged 5-10 years old,48.1％ (13/27) aged 10-15 years old and 50.0％ (3/6) above the age of 15 were TAA-positive.There were no significant differences in the numbers of TAApositive cases among different karyotypes (P ＞ 0.05).Thyroid ultrasound in girls with positive-TAA showed the abnormal echogenicity in bilateral lobus lateralis of thyroid.Conclusion Patients with TS are prone to suffer from Hashimoto's thyroiditis leading to thyroid dysfunction,when they are older than 5 years.Thyroid function should be evaluated yearly in girls with TS after 5 years old.There is no specific association between the incidence of autoimmune thyroid disease and certain karyotypes.

Objective To explore the efficacy of modified uvula palatopharyngeal plasty in the treatment of obstructive sleep ap-nea syndrome(OSAHS) .Methods 145 cases of OSAHS patients were treated from June 2006 to June 2010 in our hospital ,of which 54 were treated by traditional UPPP surgery ,and the remaining 91 cases were treated by h-UPPP surgery .Compared its effi-cacy by the preoperative and postoperative polysomnography map (PSG) analysis .Results In 145 cases of patients with OSAHS , the PSG monitoring indicators of 1 year after surgery were compared with preoperative ,and there were significant difference in the apnea total time(AI) ,the breathing hypopnea index(AHI) ,the lowest oxygen saturation(SaO2 )(P<0 .05) .The subjective symp-toms in postoperative recovery process of modified UPPP ,such as dry throat ,open nasal ,nasopharyngeal reflux symptoms were sig-nificantly reduced than that of traditional UPPP surgery .Conclusion h-UPPP surgery have a good effect .

Objectives To investigate the causes of short stature in Turner syndrome (TS). Methods 86 patients were di-agnosed with TS by karyotypes from 2004 to 2013. According to the deletion types of the X chromosome short arm, growth hor-mone (GH), insulin-like growth factor-1 (IGF-1) and thyroid function, the TS patients were divided into different groups and com-parison was made among groups. Results Ht SDS in three groups with different extent of the deletion of the X chromosome short arm were (-4.39±1.08), (-3.26±1.25) and (-2.84±0.15) (P<0.05). The proportion of growth hormone deifciency (GHD) in the three groups were 62.5%, 38.9%and 0%(P<0.05). Ht SDS in groups with different degree of IGF-1 level were (-4.37±1.10), (-3.82±1.07) and (-3.25±0.91) (P<0.05). There was no signiifcant difference of Ht SDS between hypothyroidism patients with and without GHD (P>0.05). Conclusions The deletion of X chromosome short arm may cause the short stature in TS. The GH-IGF-1 axis in TS is impaired, but GHD is not related to short stature in TS.

BACKGROUND: Miniscrew plant attachment to maxillary sinus is an important factor for implant safety and bone density can influence implant stability. Studies have explored implantation location of implant anchorage, such as bone thickness or bone density. However, the idea on implantation angle remains controversial. OBJECTIVE: To investigate the relationship between miniscrew implant in the interradicular space between the second premolars and the first molars in the maxilla and the maxillary sinus as well as bone density in the surroundings. DESIGN, TIME AND SETTING: Observation experiment was performed at dinan University from October 2007 to May 2008. PARTICIPANTS: A total of 38 patients with cone beam CT data were selected from Uni-Care Dental Center, Macao, including 11 males and 27 females, aged 18-35 (24.8±3.8) years. METHODS: Three dimensional images were reconstructed from cone beam CT images. For each subject, the buccolingual bone thickness was measured by postulating that the miniscrews would be inserted at 16 positions (10, 12, 14 and 16 mm above the median sagittal planes of the interradicular spaces between the first molars and the second premolars in the maxilla, and 30°, 45°, 60°, 90° from the cortical bone surface). The danger rate representing for implantation sites where buccolingual bone thickness was less than 6 mm was calculated. The density of the bone around the entire circumference of each miniscrew was also measured. MAIN OUTCOME MEASURES: Height of maxillary sinus, buccolingual bone thickness at implantation sites and bone density. The implantation sites could be divided into three groups. Group 2 exhibited the greatest danger rate and bone density and group 1 had the lowest danger rate and bone density.

Objective To evaluate the patterns of intracardiac flow of left ventricle (LV) in patients with premature ventricular complexes (PVCs) from the right ventricular outflow tract (RVOT) by analyzing the vortex during isovolumetric contraction phase and the distribution rules of flow-time curves in each layer of LV.Methods Twenty-seven patients with frequent isolated PVCs from RVOT were involved and 25 healthy subjects as control.The color Doppler image of LV at apical four-chamber view was acquired.Vector flow mapping (VFM) was performed to assess the parameters of vortex during isovolumic contraction phase, including diameter (transverse and vertical diameter), velocity (maximal positive and negative velocity) and the number of vortex rings.Positive flow during systole and negative flow during diastole of LV in each layer were measured by flow-time curve.All values of patients with PVCs were recorded during sinus beats (PVC-S) and PVC (PVC-V) respectively.Results Significant differences were demonstrated in all parameters of vortex between the PVC-V and control subjects.And the flow-time curves disarrayed in PVC-V.The velocity of vortex in PVC-S was lower than that in control subjects.And the distribution pattern of flow-time curves in LV of PVC-S differed from that of control subjects.Conclusions Alternation of intracardiac fluid pattern in LV was demonstrated in patients with PVCs from RVOT during both sinus beats and PVC.VFM can be used to analyze the intracardiac flow field in normal and pathological electrical activation.

Objective To analyze the relationship between the number as well as distributions of positive lymph nodes, and the clinical outcomes of postoperative radiotherapy for thoracic esophageal squamous cell carcinoma (TESCC) , And to evaluate the efficacy of postoperative radiotherapy . Methods From January 1993 to March 2007,945 patients with TESCC treated with three-field lymphadenectomy were involved in this study. All patients were with lymphoid metastasis but without distant metastasis. Among them, 590 patients received surgery alone and the other 355 received surgery plus postoperative radiotherapy. The radiotherapy were begun in the third or fourth week after operation and the median total radiation dose was 50 Gy in 25 fractions of 2 Gy, 5 fractions per week. Results The follow-up rate was 94.5%. 189 patients finished minimal follow-up of five years. The 5-year survival rates in the surgery alone group and in the postoperative radiotherapy group were 29.6% and 38.0%, respectively (χ~2 = 10.44 ,P = 0.001). In stratification analysis, compared with the surgery alone, postoperative radiotherapy could increase the 5-year survival rate of the patients with 3 to 5 (30.5% : 23.1%, χ~2 = 4.11, P = 0.043) or > 5 positive nodes (16.7% : 8.9%, χ~2= 6.87, P= 0.009) , or metastastatic node in the region of supraclavicular or upper mediastinum (45.5% : 34.9%, χ~2= 5.37, P = 0.020). In patients with positive nodes number less than 3, or with medium mediastinum or lower mediastinum lymph nodes metastasis, postoperative radiotherapy could not increase the 5-year survival rates (50.7% : 41.2%, χ~2 = 3.30, P = 0.069 ; 32.0% : 27.7% , χ~2= 2.22 , P = 0. 137) , Though could decrease lymph nodes metastasis in supraclavicular and medium-upper mediastinum (15 : 76, χ~2 = 18.10, P = 0.000; 18 : 97, χ~2= 26.81, P = 0.000). Conclusions In TESCC patients with positive lymph nodes number ≥3 or nodes in the region of supraclavicular and upper mediastinum, postoperative radiotherapy can improve the survival after three field lymphadenectomy. And the rate of metastastatic lymph nodes in the supraclavicular region or upper-medium mediastinum can also be decreased.

Objective:To investigate the diagnostic value and cutoff value of the basal luteinizing hormone (LH) level for central precocious puberty (CPP) in girls with different body mass index (BMI) values.Methods:A total of 1 087 girls aged 6 to 8 years old with breast development and visiting the Department of Endocrine Genetics and Metabolism, Fuzhou Children′s Hospital of Fujian Medical University from January 2014 to December 2018 were enrolled.The girls had the breast development of Tanner stage B2 or B3.They were divided into groups of normal weight (840 cases), overweight (133 cases) and obesity (114 cases) according to BMI percentiles corresponding to their age and sex.All individuals underwent gonadotropin-releasing hormone (GnRH) agonist test.Statistical analysis was performed using SPSS 18.0 software, and the receiver operating characteristic (ROC) curve was drawn.Results:In the normal weight group, 393 girls were positive and 447 girls were negative for GnRH test.In the overweight group, 72 girls were positive and 61 girls were negative for GnRH test.In the obesity group, 40 girls were positive and 74 girls were negative for GnRH test.The areas under the ROC curve (AUC) of the basal LH level for the diagnosis of CPP in the normal weight, overweight, and obese girls were 0.826, 0.774, and 0.801, respectively.When the Youden index was maximum, the cutoff values of the basal LH level were 0.245 IU/L, 0.225 IU/L and 0.145 IU/L, respectively; the sensitivities were 63.9%, 65.3%, and 80.0%, respectively; the specificities were 87.9%, 82.0%, and 71.6%, respectively.When the cutoff value of the basal LH level was 0.455 IU/L in the normal weight group, 0.480 IU/L in the overweight group, and 0.335 IU/L in the obesity group, the specificities of the 3 groups were all above 96.6%.Conclusions:It is reliable to use the basal LH level for CPP diagnosis for girls with different BMI values.The cutoff value of the basal LH level for CPP diagnosis in obese girls is the lowest.