Humans ; Neurilemmoma ; diagnosis ; Nose ; Nose Neoplasms ; diagnosis

Objective To investigate the molecular defects of CYPl7A1 gene in a pedigree with two 46,XY patients suffering from 17α-hydroxylase deficiency (17-OHD) and explore the steroid biosynthetic difference in carriers of 17-OHD before and after adrenocorticotrophic hormone (ACTH) test.Methods Clinical data and hormone profiles were collected from the members of the pedigree.CYPl7A1 genotyping was performed in the patients and family members with PCR-direct sequencing.A short ACTH test was evaluated in some cases.Results The CYP17 genes of the patients were proved to hold a homozygous mutation with a base deletion and a base transversion (TAC/AA) in exon 6,which produced a missense mutation of Tyr→ Lvs at codon 329 and changed the open reading frame following this codon.The hormone response of the carriers after ACTH stimulation was abnormal between the patients and normal controls.Conclusion 17-OHD in this family was caused by CYP17A1 mutation (TAC329AA):some hormonal response to ACTH stimulation Was abnormal in carriers.

Objective: To investigate the sleep characteristics of children aged 3-12 years old in Shanghai, and to provide a reference for improving pediatric sleep health. Methods: From April to June 2019, 16 542 children aged 3-12 years old were randomly selected from 31 kindergartens and 17 primary schods in Shanghai, and a cross sectional epidemiological survey was conducted using multi stage cluster sampling. The Children s Sleep Habits Questionnaire (CSHQ) was used to evaluate sleep characteristics. Results: The bedtime routine of children in Shanghai was (21:27±00:36) and (21:46±00:46) on weekdays and weekends, respectively. Corresponding wake up times were (6:52±00:28) and (7:47±00:48), total sleep duration was (9.05±1.07) and (9.52±1.17)h, and the prevalence of inadequate sleep was 81.0% and 55.4%. The average score of sleep problems was (51.56±8.04), and the prevalence of disturbed sleep was 93.5%. With advancing age, the total sleep time of children in Shanghai decreased, and the difference in weekend weekday sleep patterns increased. Furthermore, two major sleep problems were observed, namely, short sleep duration and daytime sleepiness, which became increasingly more prominent. Girls usually went to bed later on weekdays and woke up later on weekends, and a greater difference in weekend weekday sleep patterns was observed. Moreover, psychological problems such as sleep anxiety were more serious among girls. Conclusion At present, children in Shanghai have insufficient sleep time and late bedtimes. Large differences in weekend weekday sleep patterns were observed, and various sleep problems were identified. Sleep related anxiety and differences in weekend weekday sleep patterns were particularly high among girls.

OBJECTIVEThe purpose of this study was to investigate the influence of cryogenic treatment and age-hardening heat treatment on the micro-Vicker's hardness of palladium-silver dental alloys.

METHODSA low-gold content dental casting alloy composed of Ag-Pd-Cu-Au was prepared for this study. Experimental specimens according to standard requirements were prepared following a standard dental laboratory casting procedure, cast specimens were heated to 900 degrees C and quenched in ice water. The specimens were then divided into 4 groups. They were subsequently subjected to different treatments, including age-hardening heat treatment, cryogenic treatment, heat treatment combined with cryogenic treatment. The non-treated group was used as control. The micro-Vicker's hardness value was examined. The significance of correlation was analyzed.

RESULTSThe micro-Vicker's hardness of specimens after age-hardening heat treatment, cryogenic treatment, heat treatment combined with cryogenic treatment increased by 129%, 13% and 141%, respectively, compared with that of the non-treated control group. Conclusion Age-hardening heat treatment and cryogenic treatment were effective in elevating the hardness of Ag-Pd-Cu-Au alloy.

Growth factors play critical roles in the process of wound healing. Application of growth factor locally is a good way of promoting wound healing, while it is easy to be hydrolyzed in wounds and its efficacy has dose- and time-dependent manner. Therefore, appropriate growth factor delivery system is needed to assist it to function in wounds. In addition to delivering growth factor directly to wounds, viral and non-viral vectors can be used for gene transfection of growth factor in wounds. The gene can be transformed to growth factor to promote wound healing by transcription and translation. This article reviews the advances in the research of delivery system of growth factor and the gene for promoting wound healing.

Objective: To retrospectively analyze the prognostic value of platelet count recovery in the early stage post burn for patients with extremely severe burn, so as to evaluate their severity. Methods: A study involving 244 adult patients with extremely severe burn admitted to our hospital from January 2006 to December 2015, conforming to the inclusion criteria, was conducted. Data of their demography, injury, transmission, disease change in hospital, and platelet count from post injury day (PID) 1 to 10 were collected. (1) Patients were divided into survival group (n=212) and non-survival group (n=32) according to whether death or not. The dynamic change characteristic of platelet count in patients of two groups from PID 1 to 10 was analyzed and compared. (2) Patients were divided into return to normal group (RN, n=163) and non-return to normal group (NRN, n=81) according to whether platelet count returned to normal within one week post burn. The proportion of patients who received mechanical ventilation and mortality in groups RN and NRN, and length of stay in ICU between patients with platelet count that returned to normal and that did not return to normal in the early stage post burn in survival group were compared. Data were processed with independent samples t test , analysis of variance of repeated measurement, chi-square test, and binomial distribution test. Correlation was analyzed between data of sex, age, weight, total burn area, full-thickness burn area, inhalation injury, length of hospital stay, receiving mechanical ventilation, platelet count recovery condition within one week post burn and death of patients using the univariate and multivariate Cox regression analysis. Receiver operating characteristic (ROC) curve of platelet count on PID 8 was drawn to evaluate predicting value for death of 244 patients. Results: (1) The platelet count kept declining from PID 1 to 4, declined to below the normal low limit on PID 2, and reached its lowest level on PID 4 both in survival group and non-survival group. The platelet count both in survival group and non-survival group rose gradually from PID 4 to 10, and returned to normal on PID 8 and 10, respectively. There was no significant difference in platelet count of patients in two groups on PID 1 (t=1.01, P>0.05), while platelet count of patients in non-survival group was obviously lower than that in survival group from PID 2 to 10 (with t values from 2.64 to 7.17, P values below 0.01). The daily increment of platelet count in survival group from PID 4 to 10 was (26±13)×10⁹/L, obviously higher than that in non-survival group [(19±11)×10⁹/L, t=2.76, P<0.01]. (2) The proportion of patients who received mechanical ventilation was obviously lower than that of patients who did not receive mechanical ventilation in group RN (P<0.01), while the proportion of patients who received mechanical ventilation was obviously higher than that of patients who did not receive mechanical ventilation in group NRN (P<0.05). The proportion of patients who received mechanical ventilation in group NRN was obviously higher than that in group RN (χ²=32.93, P<0.01). The mortality of patients in group NRN was obviously higher than that in group RN (χ²=20.99, P<0.01). The length of stay in ICU of patients whose platelet count did not return to normal in the early stage was significantly longer than that of patients whose platelet count returned to normal in the early stage in survival group (t=4.20, P<0.01). (3) Total burn area, receiving mechanical ventilation, and platelet count did not return to normal within one week post burn were independent risk factors for death of patients with extremely severe burn (with hazard ratio respectively 1.073, 16.552, and 2.249, 95% confidence interval respectively 1.033-1.115, 2.147-127.580, and 0.993-5.096, P<0.05 or P<0.01). (4) The area under the ROC curve of platelet count on PID 8 to predict death of 244 patients with extremely severe burn was 0.745 (with 95% confidence interval 0.645-0.845, P<0.01), and 150×10⁹/L was chosen as the optimal threshold value, with sensitivity of 71.4% and specificity of 71.0%. Conclusions Platelet count recovery in the early stage post burn of patients with extremely severe burn was significantly associated with their prognosis and could be used as an important indicator to evaluate the severity of illness.

Objective: To analyze the influencing factors and clinical significance of severe hypocalcemia in patients with extremely severe burns in early stage. Methods: Clinical data of 142 patients with extremely severe burns admitted to our wards from January 2010 to July 2015, conforming to the study criteria, were retrospectively analyzed. (1) The incidence of hypocalcemia and severe hypocalcemia on admission were calculated. (2) Patients were divided into the male group (n=113) and the female group (n=29) according to gender. The levels of serum calcium of patients whose age more than 55 years old and less than or equal to 55 years old in the two groups were compared with t test. (3) Patients were divided into severe hypocalcemia group (n=52) and non-severe hypocalcemia group (n=90) according to the level of serum calcium on admission. The data including gender, age, flame burn, total burn area, inhalation injury, admission time, the pH value, and the albumin level of patients on admission between two groups were compared with chi-square test or t test. Indexes with P<0.1 between two groups were selected, and multivariate logistic regression analysis was conducted to screen the influencing factors of severe hypocalcemia in patients with extremely severe burns in early stage. (4) According to the prognosis, patients were divided into survival group (n=112) and non-survival group (n=30). The data including gender, age, flame burn, total burn area, inhalation injury, admission time, the level of serum calcium, the pH value, and the albumin level of patients on admission between two groups were compared with chi-square test or t test. Indexes with P<0.1 between two groups were selected, and multivariate Cox regression analysis was conducted to screen the influencing factors of prognosis of patients with extremely severe burns. Results: (1) The incidence of hypocalcemia on admission was 97.2% (138/142), and patients diagnosed as severe hypocalcemia accounted for 36.6% (52/142). (2) In the male group, the level of serum calcium of patients with age more than 55 years old was (1.84±0.19) mmol/L, which was close to (1.88±0.21) mmol/L of patients with age less than or equal to 55 years old within the same group and (1.96±0.13) mmol/L of patients with age more than 55 years old in the female group (t=0.833, 1.560, P>0.05). In the female group, the level of serum calcium of patients with age less than or equal to 55 years old was (1.78±0.19) mmol/L, which was significantly lower than that of patients with age less than or equal to 55 years old in the male group and that of patients with age more than 55 years old in the female group (t=-2.197, -2.472, P<0.05). (3) Compared with those of patients in severe hypocalcemia group, the total burn area and the proportion of inhalation injury of patients in non-severe hypocalcemia group were obviously smaller (t=2.379, χ² =13.410, P<0.05 or P<0.01), and the admission time was obviously earlier (t=2.675, P<0.01), while the albumin level was obviously higher (t=-6.163, P<0.01). There were no statistically significant differences between patients of the two groups in gender, flame burn, age, and the pH value on admission (χ²=1.869, 2.850, t=-0.578, 0.645, P>0.05). Multivariate logistic regression analysis showed that only the albumin level on admission was the independent influencing factor of severe hypocalcemia in patients with extremely severe burns (with odds ratio 1.179, 95% confidence interval 1.092-1.273, P<0.01). (4) Compared with those of patients in non-survival group, the total burn area and the proportion of inhalation injury in survival group were smaller (t=-5.515, χ²=27.573, P<0.05 or P<0.01), while the pH value and the albumin level on admission were higher (t=2.208, 3.321, P<0.05 or P<0.01). There were no statistically significant differences between patients of the two groups in gender, flame burn, age, admission time, and the level of serum calcium on admission (χ²=0.198, 2.545, t=-1.316, -1.397, 1.857, P>0.05). Multivariate Cox regression analysis showed that total burn area and inhalation injury were the independent risk factors to predict prognosis of patients with extremely severe burns (with relative risk 1.066 and 4.081, 95% confidence interval 1.023-1.110 and 1.144-14.559, P<0.05 or P<0.01), but the pH value and levels of albumin and serum calcium were not independent risk factors to predict prognosis of patients with extremely severe burns (with relative risk 0.003, 1.025, and 0.634, 95% confidence interval <0.001-1.183, 0.956-1.099, and 0.055-7.321, P>0.05). Conclusions The level of serum calcium of the majority of patients with extremely severe burns on admission is decreasing significantly, especially the female patients less than or equal to 55 years old. Compared with non-severe hypocalcemia patients, patients with severe hypocalcemia are with larger total burn area, higher proportion of inhalation injury, later admission time, and lower albumin level on admission. However, only the albumin level on admission is the independent influencing factor of severe hypocalcemia in patients with extremely severe burns. And the level of serum calcium on admission can not predict the prognosis of patients with extremely severe burns.

Objective: To explore the relationship of sleep with C reactive protein (CRP) abnormality and hyperinsulinemia in adolescents, and to provide reference for early prevention of metabolic disorders. Methods: Based on the Chinese Metabolic Syndrome Cohort Study in Anhui Province, a total of 653 adolescents aged 12 to 19 were selected to examine the relationship between wakeup time, bedtime, sleep disordered breathing (SDB) and CRP abnormality as well as hyperinsulinemia. Results: Later wakeup time (OR=1.68, 95%CI=1.03-2.75) was positively correlated with a higher risk of fasting hyperinsulinemia. Late bedtime (OR=1.96, 95%CI=1.29-2.99) was associated with 2 h postprandial hyperinsulinemia. Among those with high CRP concentration, late wakeup time and late bedtime were positely associated with hyperinsulinemia than those with normal CRP concentration; and the correlation between SDB and hyperinsulinemia was observed. Conclusion Later wakeup and late bedtime may be risk factors for hyperinsulinemia in adolescents. High concentrations of CRP may further increase the risk of hyperinsulinemia, a condition associated with sleep problems.Teenagers should get up and go to bed as early as possible.

At present, the most commonly used surgical measures for treatment of severe traumatic brain injury (sTBI) mainly include external ventricular puncture and drainage (EVD), decompressive craniectomy (DC), intracranial hematoma clearance and intracerebral decompression of contusion and laceration. All of these procedures can reduce intracranial pressure (ICP) and mortality, but they have the disadvantage of high postoperative adverse functional outcomes. With further study of glyymphatic system and intracranial microsurgery, some scholars have proposed a new neurosurgical technique: cisternostomy, which could achieve good results in the treatment of some refractory intracranial hypertension patients with sTBI. In this paper, we will summarize the recent advance in cisternostomy in the treatment of sTBI by reviewing the relevant literature at domestic and abroad in recent years, including the systematic evaluation of its pathophysiological basis, surgical techniques, surgical indications and contraindications, and surgical efficacy.

Objective: This study aimed to evaluate the zygomaticotemporal suture (ZTS) maturation, analyze the age distribution patterns of ZTS maturation stages, and investigate the relationship between ZTS and cervical vertebral maturation (CVM). Methods: A total of 261 patients who underwent cone-beam computed tomography (112 males, mean age, 13.1 ± 3.3 years; 149 females, mean age, 13.7 ± 3.1 years) were examined to evaluate the ZTS stages. The ZTS stages were defined based on a modified method from previous studies on zygomaticomaxillary sutures. Differences between groups and correlations between indicators were analyzed using the Spearman correlation test, intraclass coefficient of correlation (ICC), one-way analysis of variance and rank sum test.Statistical significance was set at p < 0.05. The diagnostic value of CVM stages in identifying ZTS maturation stages was evaluated using positive likelihood ratios (LRs). Results: A positive relationship was found between the ZTS and CVM stage (r = 0.747, ICC = 0.621, p < 0.01) and between the ZTS stage and chronological age (r = 0.727, ICC = 0.330, p < 0.01). Positive LRs > 10 were found for several cervical stages (CSs), including CS1 and CS2 for the diagnosis of stage B, CS1 to CS3 for the diagnosis of stages B and C, and CS6 for the diagnosis of stages D and E. Conclusions The ZTS maturation stage may be more relevant to the CVM stage than to the chronological age. The CVM stages can be good indicators for clinical decisions regarding maxillary protraction, except for CS4 and CS5.