Objective:To retrospectively analyze the clinical and genetic characteristics of six patients with Acromicric dysplasia due to variants of the FBN1 gene. Methods:Six patients who had visited the Affiliated Hospital of Qingdao University between February 2018 and October 2020 were selected as the study subjects. Clinical data of the patients were collected. High-throughput sequencing was carried out. And candidate variants were verified by Sanger sequencing.Results:All of the six patients had presented with severe short stature (< 3 s), brachydactyly, short and broad hands and feet. Other manifestations included joint stiffness, facial dysmorphism, delayed bone age, liver enlargement, coracoid femoral head, and lumbar lordosis. Genetic testing revealed that all had harbored heterozygous variants of the FBN1 gene. Patient 1 had harbored a c. 5183C>T (p.A1728V) missense variant in exon 42, which had derived from his father (patient 2). Patient 3 had harbored a c. 5284G>A (p.G1762S) missense variant in exon 43, which had derived from her mother (patient 4). Patient 5 had harbored a c. 5156G>T (p.C1719F) missense variant in exon 42, which was de novo in origin. Patient 6 had harbored a c. 5272G>T (p.D1758Y) missense variant in exon 43, which was also de novo in origin. The variants carried by patients 1, 3 and 6 were known to be pathogenic. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the FBN1: c. 5156G>T was rated as a pathogenic variant (PS2+ PM1+ PM2_Supporting + PM5+ PP3). Conclusion:All of the six patients had severe short stature and a variety of other clinical manifestations, which may be attributed to the variants of the FBN1 gene.

Objective:To construct a core index system of job responsibilities for specialist trauma nurses tailored to the current trauma care system in China and provide a reference for trauma centers to set up specialist trauma nurses.Methods:Literature review and group discussion were used to develop the first draft of the core index system of job responsibilities for specialist trauma nurses. The Delphi method was used to conduct two rounds of consultation among 15 experts from 7 provinces or cities in China to determine the final draft. The two rounds of expert active coefficient, expert authority coefficient ( Cr), Kendal coefficient (Kendall′s W), and average value of the importance and variation coefficient ( CV) of each indicator were calculated. Results:In both rounds of consultations, the expert active coefficient was 100%, and the Cr was 0.953. In the second round of consultation, the Kendall′s W was 0.188 ( χ2 =155.29, P<0.001), and the average value of the importance of each indicator ranged from 4.27-5.00 points with the CV of less than 0.25 points. The final index system consisted of 7 first-level indicators and 49 second-level indicators. Conclusion:The core index system of job responsibilities for specialist trauma nurses is reasonable and comprehensive, which can provide guidance for setting up positions of specialist trauma nurses in China.

ObjectiveTo explore the characteristics of oral sensory-motor function in children with functional articulation disorders (FAD). MethodsFrom June, 2021 to January, 2022, 61 children with FAD in the Department of Children's Health of the Affiliated Hospital of Qingdao University were as case group, 90 normal healthy children were as control group. They were assessed with Oral Sensory-Motor Assessment, and were compared among different genders and different ages. Hyperactivity problems were assessed using Conners Parent Symptoms Questionnaire, and their oral sensory-motor function were compared between patients with hyperactivity problem (n = 13) and without hyperactivity problem (n = 48). ResultsThe total score and the scores of oral sensation, mandibular motion, lip motion and tongue motion were significantly lower in the case group than in the control group (t > 4.471, P < 0.001). There were significant differences in the total score and motor function scores among different ages in the control group (H > 17.015, P < 0.001), and they increased with age. There were significant differences in the total score, oral sensory and motor function scores among different ages in the case group (H > 10.567, P < 0.01), and they increased with age. The total scores, and the scores of mandibular, lip and tongue movements were lower in the boys and girls of the case group than in the same gender of the control group (t > 2.49, P < 0.05). The total score and the scores of the lip and tongue movements were lower in boys than in girls in the case group (|Z| > 2.409, P < 0.05). There was no significant difference in the total score and other scores between the patients with hyperactivity problem and without hyperactivity problem (P > 0.05). ConclusionThe oral sensory-motor function is poor for children with FAD, and can develop with age in both normal and FAD children.

Objective:To explore the influence of early essential newborn care (EENC) on breastfeeding after vaginal delivery.Methods:Women with full-term vaginal delivery at Jiaxing Women and Children's Hospital Affiliated to Wenzhou Medical University from January to June 2019 were enrolled in this prospective randomized controlled trial. These participants and their neonates were assigned randomly to receive early skin-to-skin contact for 90 min after delayed umbilical clamping (EENC group) or routine nursing care after delivery and skin-to-skin contact for 30 min (control group). The main outcomes were successful initiation and duration of first-time breastfeeding, initiation time of second-stage lactation, self-efficacy score of breastfeeding, acceptance and satisfaction score of breastfeeding, and the rate of exclusive breastfeeding at 24, 48, 72 h, and 42 d postpartum. The secondary outcomes included the onset time of first deep sleep of the newborn, the baby's first breast-fed stool, and the neonatal weight within 4 days of birth. Two independent samples t-test and Chi-square test were used for statistical analysis. Results:A total of 200 women with full-term vaginal delivery were recruited and 117 (61 in EENC group and 56 in control group) completed the study. (1)Main outcomes: Compared with the control group, the EENC group had earlier initiation of the first breastfeeding [(25.5±6.9) vs (33.2±7.2) min, t=5.902] and the second stage of lactation [(72.1±3.3) vs (78.6±4.3) h, t=9.504], longer duration of the first breastfeeding [(40.6±5.2) vs (32.6±6.1) min, t=7.806], and higher self-efficacy score [(122.5±2.1) vs (98.2±3.1) scores, t=50.660], acceptance score [(21.8±3.1) vs (15.3±4.3) scores, t=9.444], satisfaction score of breastfeeding [(23.2±2.1) vs (18.8±3.1) scores, t=9.278], and higher rate of exclusive breastfeeding at 24, 48 and 72 h after delivery[43% (26/61) vs 21% (12/56), 59%(36/61) vs 36%(20/56), 89%(54/61) vs 64%(36/56); χ 2 were 5.980, 6.353, and 9.663, respectively], all P<0.05. (2)Secondary outcomes: In the EENC group, the onset time of first deep sleep [(90.1±10.6) vs (118.3±9.9) min, t=7.645] and the the baby's first breast-fed stool [(57.1±6.4) vs (66.4±5.5) h, t=8.435] were earlier; neonatal weight at 3 d [(3 720±329) vs (3 558±412) g, t=2.382] and 4 d after birth [(3 778±289) vs (3 562±315) g, t=3.857] were significantly heavier, comparing with the control group (all P<0.01). Conclusions:EENC can improve multiple short-term breastfeeding indicators and maternal acceptance and satisfaction of breastfeeding in women after full-term vaginal delivery.

Objective: To conduct an epidemiological investigation on a cluster of overseas imported cases of coronavirus disease 2019(COVID-19)in Qingtian County, Lishui,so as to provide reference for the prevention and control of COVID-19 imported from abroad. Methods: According to the COVID-19 Prevention and Control Program (FiFth Edition) issued by National Health Commission of China,a field investigation was employed and the close contacts of the case were tracked down ;the real-time reverse transcription polymerase chain reaction (RT-PCR) assay was performed to detect the nucleic acid of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) from the sputum specimens or throat swabs of cases;the epidemiological characteristics,source of infection,route of transmission and disposal of the cluster were analyzed. Results: From March 1 to March 6,2020,eight confirmed cases and one asymptomatic case of COVID-19 were reported. Their median age was 33 years old. The nine cases all had no fever,no decrease in leukocyte and lymphocyte levels,and the clinical symptoms of seven cases were not obvious. The asymptomatic case had been tested negative for SARS-CoV-2 for six times,but had been weakly positive for IgM antibody and strongly positive for IgG antibody. Nine cases were all the employees of the same restaurant in Begamo,Italy. They lived in three independent villas and usually had lunch and dinner in the restaurant where they worked. Begamo had COVID-19 epidemic,but the staff of the restaurant did not take any protective measures such as wearing masks and environmental disinfection. Eight cases reported to have cold symptoms in Italy during mid February. Through a closed-loop management of“all people,all sites,all chains ”,such as treatment of current cases,screening and isolation of close contacts and health education,totally 372 close contacts were traced back,yet no fever or respiratory symptoms have been found. Conclusions It was a cluster of COVID-19 cases imported from abroad. The clinical symptoms of the cases were not obvious. Qingtian County adopted the closed-loop management in time,and effectively controlled the spread of the epidemic. No second-generation cases have been found.

To analyze the difficulty, distinction and result of the first national public health practice skills competition among college students, it showed the general situation of this competition, and discussed the present situation, problems and countermeasures of skills training for students majoring in preventive medicine. Based on such competition, educators can promote teaching reform and post competency training.

Objective:To design a checklist for interhospital transfer of patients undergoing extracorporeal membrane oxygenation (ECMO) therapy and apply it in clinical practice to ensure the transfer safety of patients.Methods:Using convenience sampling, 27 cases of critically ill patients who were transported by the ECMO team of the Department of Critical Care Medicine of Henan Provincial People's Hospital from September 2017 to November 2018 were selected as the control group, and the ECMO interhospital transport was implemented from December 2018 to February 2020, 14 cases of critically ill patients were set as the experimental group.The checklist for interhospital transfer of patients undergoing ECMO therapy was compiled based on relevant literature and guidelines, and applied to critically ill patients who were transported between hospitals under the support of ECMO. The incidence of adverse events between two groups was compared.Results:The incidence of adverse events decreased after the application of the checklist, the difference was not statistically significant (χ 2=3.516, P=0.061) . Conclusions:The use of the ECMO interhospital transfer checklist can effectively standardize the transfer process, improve work efficiency and quality, may reduce transfer risks, and ensure patients' safety.

Objective: The purpose of this study was to explore the surgery-first approach in sequential combined orthodontic-orthognathic treatment to shorten total treatment duration and improve the clinical outcome. Methods: This study included 185 patients with Angle classⅢ malocclusion. The patients were divided into 3 different types according to cephalometry analyses and facial features. ①Type Ⅰ: mandibular prognathism or asymmetry mandibular prognathism; ②Type Ⅱ: mandibular prognathism and maxillary retrusion; ③ Type Ⅲ: mild Angle′s Class Ⅲ malocclusion, cross bite in anterior teeth, or normal overlap and overbite relation with midfacial hypoplasia. All of patients received surgery first approach therapy. The surgical procedures were chosen according to different malformation types. Type Ⅰ was treated with the sagittal split ramus osteotomy (SSRO). Type Ⅱ was treated by Le Fort Ⅰmaxillary osteotomy combined with SSRO. Type Ⅲ underwent anterior subapical osteotomy combined pyriform aperture augmentation with biomaterials as well as maxillary anterior orthodontics. All patients received postoperative rapid orthodontic treatment for 6-12 month after 2 weeks of operation. Using the straight arch wire techniques and the class Ⅲ intermaxillary traction, we removed the overcrowding upper and lower teeth, the compensatory axial tilt of teeth, and the deviation of the dental arch and maintained the neutral relationship of the molar. The mandible Hawley retaining devices were used during the maintaining stage. Results: The cases in study acquired satisfactory clinical outcome, which included the shortened overall treatment duration, the significantly improved facial features, the corrected occlusion relationship, and the restored function of mastication and temporomandibular joint. There were some complications as follows: intraoperative fracture (6 cases, 3.24%), the inferior alveolar nerve bundle injury (2 cases, 1.1%), and temporary open-bite that diminished by inter-maxillary elastic distraction one month after operation (19 cases, 10%). All cases in this study accepted postoperative orthodontic treatment. Follow-up time ranged from 6 months to 5 years. The cephalometric analysis results of 126 cases who had complete image data and over 6 months of follow-up showed that hard and soft tissue indexes were restored to normal range after combined orthognathic-orthodontic treatment. The stability of the maxillary and occlusive relationship of SFA(surgery-first approach) was similar to that of the COS(conventional orthodontics-first system) [relapse ratio=(T2-T1)/(T1-T0)×100%]. Over six months of follow-up , SNB and ANB showed that the average relapse ratio were 22% and 19.8%, whereas the relapse angle are less than 2°. Conclusions The Surgery-first approach could be used to treat most patients with Angle skeletal Class Ⅲ malocclusion, but the indications and the surgical procedures should be noticed and chosen.

Objective To explore the influence of endoplasmic reticulum stress on fatty liver in mice feeding with high-fat diet.Methods The 8-week-old male C57BL/6J mice were randomly divided into two groups:high-fat diet group (with 60％ calories by high saturated fatty acid) and control group (with chow diet),both groups had been fed for 16 weeks.H&E-staining and Sudan Ⅳ-staining reflected lipid deposition in liver.The levels of 78-kDa glucose-regulated protein (GRP78),protein kinase R-like endoplasmic reticulum kinase (PERK),phosphorylated α subunit of eukaryotic initiation factor 2 (p-eIF1 α),C/EBP homologous protein (CHOP),steroid regulated element binding proteins 1 (SREBP-1),and fatty acid synthetase (FAS) protein were determined by Western blot to reflect the endoplasmic reticulum stress and lipid synthesis.Results In liver of high fat diet (HFD) group,H&E staining showed that the cytoplasm of hepatocytes were filled with vacuoles,Sudan Ⅳ staining also displayed that many different sizes of red lipid drops exist in hepatocytes.Compared to the liver of control group,high-fat diet induced endoplasmic reticulum stress and elevated lipid synthesis,as evidenced by increases in the level of peroxisome proliferator-activated receptor alpha (PPARα) mRNA expression,and the protein levels of GRP78,PERK,phosphorylated eIF2α,CHOP were also significantly increased.In primary normal hepatocytes incubated with exogenous oleic acid intervention for 24-72 hours,the expression of GRP78,PERK,phosphorylated eIF2α,CHOP protein levels,and the expression of SREBP-1 and FAS protein were significantly increased in dose-dependent manner.Conclusions Feeding with high-fat diet led to accumulation of lipid deposition in liver and fatty liver,the underlying mechanisms might be related to induction of endoplasmic reticulum stress.

Objective: To invesigate the therapeutic effect of anterior subapical osteotomy combined with postoperative orthodontic treatment for bimaxillary protrusion based on surgery-first approach(SFA). Methods: From April 2015 to April 2016, 22 adult patients with bimaxillary protrusion were treated with bimaxillary anterior subapical osteotomy without peroperative orthodontic treatment. Digital computer-aided technology was used for preoperative design and dental model simulation surgery. Then the occlusal guide plate was made for intraoperative built-up of temporary occlusion. Two weeks after orthognathic surgery, a short-term orthodontic treatment was carried out to improve the occlusion. Cephalometric analysis was done to evaluate the treatment effects. 19 cephalometric parameters about hard and soft tissues were measured and compared in all cases before and after SFA combined treatments. All data were statistically analyzed by SPSS software package. Results: Primary healing was achieved in all the 22 cases without any infection or necrosis of bone and dental pulp. During the follow-up period of 6-12 months, all the patients were satisfied with the profile contour. The cephalometric parameters of hard and soft tissues changed remarkably. U1E-McN and L1E-McN set back (-10.5±4.9) mm and (-8.0± 6.9) mm respectively. Upper and lower lip set back obviously. SNA decreased from (82.5±2.8) to (77.9±2.3) degrees on average. SNB decreased from (79.1±5.4) to (74.6±3.8) degrees on average. Upper and lower lip protrusion decreased obviously. TUL-E line decreased from the mean value of (2.7±2.7) mm to (0.5±3.2) mm and TLL-E line from (5.2±4.0) mm to (1.4±2.7) mm. The aesthetic contours were remarkably improved. Conclusions Anterior subapical osteotomy combined with postoperative orthodontic treatment based on SFA could correct adult bimaxillary protrusion effectively with normal occlusion, satisfactory soft tissue aesthetic profile.