Osteonecrosis of the mandible is also called avascular necrosis of the jaw, and it is a rare complication of bisphosphonates. It is characterized with pain, swelling, exposure of bone, local infection and pathologic fractures of the jaw. With the widespread usage of bisphosphonates in bone metastasis of malignant tumors and osteoporosis, this rare complication has received more attention in recent years. Here, we reported a case of bisphosphonates-related osteonecrosis of the jaw (BRONJ) caused by intravenous zoledronic acid for osteoporosis. A 62-year-old female patient with 7-year history of Sjögren's syndrome and 3-year history of osteoporosis developed BRONJ after 3-year treatment of zoledronic acid. Two months before she went to the Peking University International Hospital, she visited the dentist for periodontal purulent secretion and extracted one tooth from the right mandible. However, the condition was not improved and she felt persistent pain and swelling in the right mandible. Hence, she received repeated root curettage, but there was no improvement. Finally, she was diagnosed with osteonecrosis of the mandible based on the digital volume tomography scan, which showed right mandibular osteonecrosis bone destruction. She underwent surgical debridement of the necrotic bone and administered intravenous antibio-tics at the Peking University International Hospital. Histopathological analysis of the bone biopsy further confirmed the diagnosis of BRONJ. Her condition was improved successfully during a 3-year follow-up. Osteonecrosis of the mandible become more common with the increased use of bisphosphonates. Recent study has reported that osteonecrosis of the mandible is more likely to occur in patients with Sjögren's syndrome. In addition, age, long-term and irregular administration of glucocorticoids, irregular oral examination and treatment also might be the risk factors in the pathogenesis of osteonecrosis of the mandible. For the elder osteoporosis patients who would receive or had received bisphosphonate-related drugs, oral health status and the disease states associated with necrosis of the mandible such as Sjögren's syndrome should be comprehensively measured and fully evaluated during the whole process. Furthermore, to better understand and prevent or reduce the occurrence of this complication, we reviewed the patho-genesis, diagnosis, treatment, and prevention of BRONJ.
Humans ; Female ; Middle Aged ; Bisphosphonate-Associated Osteonecrosis of the Jaw/etiology* ; Diphosphonates/administration & dosage* ; Zoledronic Acid ; Imidazoles/administration & dosage* ; Bone Density Conservation Agents/adverse effects* ; Osteoporosis/drug therapy*

All four patients were female, with an average age of 28.8 days and an average weight of 3.64 kg. Only case 4 was born prematurely at 34 W+5 and was treated conservatively until 71 days to complete operation. All the others completed primary corrective surgery in the neonatal period, and all survived after operation. Two different surgical techniques were used to repair the aortic-pulmonary window and the aortic origin of the right pulmonary artery, including 1 case using the aortic internal baffle technique and another 3 cases underwent replantation of the right pulmonary artery (1 case was reconstructed in situ, and the other 2 cases were reconstructed by moving the right pulmonary artery in the anterior of aorta). Case 2 who received aortic internal baffle technique underwent two reoperation because of right pulmonary artery stenosis. While, right pulmonary artery of cases 3 and 4 developed well after being reconstructed the right pulmonary artery anterior translocation. After the diagnosis of Berry syndrome through cardiac color ultrasound and CT angiography, a primary surgical radical treatment should be performed as soon as possible at an experienced pediatric cardiac center. Although the surgery is high-risk and complex, it is safe and effective.

Objective:To analyze the characteristics of eyes with congenital optic disc pits (ODPs) through multimodal imaging.Methods:A cross-sectional study was conducted.Thirty-eight patients (38 eyes) diagnosed with congenital ODPs in the Second Hospital of Hebei Medical University from January 2009 to January 2020 were enrolled.A comprehensive summary analysis of the imaging results including fundus photography, spectral domain-optical coherence tomography (SD-OCT), infrared imaging, fundus autofluorescence (FAF), fluorescein fundus angiography (FFA) and indocyanine green angiography (ICGA) was performed.This study protocol adhered to the Declaration of Helsinki and was approved by an Ethics Committee of The Second Hospital of Hebei Medical University (No.2021-P011). Written informed consent was obtained from each subject prior to any medical examination.Results:Among the 38 eyes, there were 32 eyes with ODPs located in or below the temporal side of optic disc, 4 eyes with ODPs located above the temporal side of optic disc, and 2 eyes with ODPs located at the center of optic disc, which were round or quasi-circular pale depression, and dark red eminences with clear or unclear boundaries between milk spots were found in 29 eyes with optical-disc macular degeneration (ODP-M) by fundus photography.SD-OCT examination showed that the structure of lamina cribrosa in the lesion area in all ODPs patients was incomplete, which presented a dark area with no tissue reflection, and the fissure led to the deep optic nerve.Fluid was found in the outer nuclear layer in all ODP-M patients, and there were 27 eyes with fluid in the inner nuclear layer, 13 eyes in the ganglion cell layer, and 4 eyes under the inner limiting membrane.Among the 29 eyes with ODP-M, there were 21 eyes with retinoschisis in outer layer, 27 eyes with neuroepithelial detachment.In the 27 eyes with neuroepithelial detachment, spot-like high reflection and reduced or disappeared ellipsoid band reflectance were seen above the neuroepithelium in 18 eyes.In infrared images, there were circular or quasi-circular low-reflection areas in the temporal side of the optic disc, and the lesion of ODP-M eyes presented low-reflection areas.FAF examination showed that in 27 eyes with ODP-M, there was a hypofluorescent region at the posterior pole consistent with the lesion range, among which, there was a granular or sheet-like hyperfluorescence at the center of the hypofluorescent region in 18 eyes.FFA showed that the optic disc depression in the arterial phase of patients was in a localized hypofluorescence state.During the venous phase, fluorescein dye extravasation along the temporal side of the optic disc could be found.A strong fluorescent arc with unclear boundaries at the temporal edge of the optic disc was formed in the late stage of angiography.Among the 29 eyes with ODP-M, the area of the macular lesion showed hyperfluorescence during the late stage of angiography in 27 eyes with neuroepithelial detachment, and no extension of dye toward the macula was found.ICGA showed that the optic disc depression of ODPs patients presented a localized hypofluorescence, and the lesion showed hyperfluorescence in 27 of the 29 ODP-M eyes with neuroepithelial detachment.Conclusions:Multimodal imaging can be helpful to realize the early diagnosis, etiology analysis of ODPs and make treatment plan.

Ossifying fibroma (OF) and fibrous dysplasia (FD) are two fibro-osseous lesions with overlapping clinicopathological features, making diagnosis challenging. In this study, we applied a whole-genome shallow sequencing approach to facilitate differential diagnosis via precise profiling of copy number alterations (CNAs) using minute amounts of DNA extracted from morphologically correlated microdissected tissue samples. Freshly frozen tissue specimens from OF (n = 29) and FD (n = 28) patients were obtained for analysis. Lesion fibrous tissues and surrounding normal tissues were obtained by laser capture microdissection (LCM), with ~30-50 cells (5 000-10 000 µm
DNA Copy Number Variations ; Diagnosis, Differential ; Fibroma, Ossifying/genetics* ; Fibrous Dysplasia of Bone/genetics* ; Galactosyltransferases ; Humans ; Jaw ; Neoplasm Recurrence, Local ; Nuclear Proteins

OBJECTIVE: To explore effects of different delivery and storage conditions on concentrations of amino acids and carnitines in neonatal dried blood spots (DBS), so as to provide evidence for improving accurate and reliable detection by tandem mass spectrometry. METHODS: A total of 1 254 616 newborn DBS samples in Newborn Screening Center of Zhejiang Province were delivered and stored at room temperature (group A, RESULTS: The concentrations of amino acids and carnitines in the three groups were skewed, and the differences in amino acid and carnitine concentrations among groups were statistically significant (all CONCLUSIONS Cold-chain logistics system and storage in low temperature and low humidity can effectively reduce degradation of some amino acids and carnitines in DBS, improve the accuracy and reliability of detection, and thus ensures the quality of screening for neonatal metabolic diseases.
Amino Acids/analysis* ; Carnitine/analysis* ; Dried Blood Spot Testing/standards* ; Humans ; Humidity ; Infant, Newborn ; Neonatal Screening ; Reproducibility of Results ; Specimen Handling/standards* ; Tandem Mass Spectrometry ; Temperature ; Time Factors

Objective:To compare positional and volumetric differences between the gross target volumes (GTV) delineated on three-dimensional CT (3D-CT) referencing 18F-FDG PET/CT and the GTV on the deformed image derived from 3D-CT and 18F-FDG PET/CT for primary thoracic esophageal cancer (EC). Methods:Seventy-two patients underwent chemoradiotherapy were enrolled. All the patients sequentially underwent 18F-FDG PET/CT scans for diagnosis and 3D-CT scans for simulation. The GTV 3D was delineated on 3D-CT without referencing 18F-FDG PET/CT. The GTV PET-ref was delineated on 3D-CT referencing 18F-FDG PET/CT. The GTV PET-regwas delineated on the deformed image derived from 3D-CT and 18F-FDG PET/CT by MIM deformable registration software. The differences in position, volume, length, conformity index (CI), and degree of inclusion (DI) of target volumes were compared, respectively. Results:The median volume of GTV 3D, GTV PET-ref, GTV PET-reg were 44.90, 40.36 and 41.15 cm 3, respectively. There was no statistical difference between the volumes of any two targets. The mean lengths of GTV 3D, GTV PET-ref, GTV PET-reg were 8.54, 9.29 and 8.38 cm, respectively. The length of GTV PET-ref was longer than that of GTV 3D ( t=2.134, P<0.05). The median DIs of GTV PET-ref, GTV PET-regin GTV 3D were 0.86, 0.82( Z=-2.741, P<0.05), and that of GTV 3D in GTV PET-ref, GTV PET-reg were 0.87, 0.84 ( Z=-1.429, P<0.05). The median CIs of GTV 3D in GTV PET-ref and GTV PET-reg were 0.72, 0.68 ( Z=2.756, P<0.05), and the difference was significant. The CIs of GTV 3D and GTV PET-ref, GTV 3D and GTV PET-reg, GTV PET-ref and GTV PET-reg had significant negative correlation with the distance of target centers. Conclusions:There was no significant difference between GTV contoured on three-dimensional CT (3D-CT) referencing 18F-FDG PET/CT and the GTV on the deformed image derived from 3D-CT and 18F-FDG PET/CT either in volume size or in spatial position. Therefore, it is recommended that radiation oncologists can refer to the recent diagnostic PET/CT when delineating the gross target volume for primary thoracic esophageal cancer.

Objective To explore the application value of the extra-glissonian pedicle transection approach guided by arantius' ligament in laparoscopic left hemihepatectomy.Methods The retrospective cohort study was conducted.The clinical data of 57 patients who underwent laparoscopic left hemihepatectomy in the First Affiliated Hospital of Fujian Medical University from January 2015 to January 2018 were collected.There were 18 males and 39 females,aged from 29 to 75 years,with an average age of 57 years.Of the 57 patients,22 undergoing the laparoscopic left hemihepatectomy with extra-glissonian pedicle transection approach guided by arantius' ligament and 35 undergoing laparoscopic left hemihepatectomy with regular intra-glissonian pedicle transection approach were allocated into the extra-glissonian transection group and intra-glissonian transection group,respectively.Observation indicators:(1) intraoperative situations and postoperative short-term outcomes;(2) postoperative complications;(3) follow-up.Patients were followed up by outpatient examination and telephone interview to investigate postoperative recurrence of diseases up to June 2018.Measurement data with normal distribution were represented as Mean±SD and comparison between groups was analyzed using the t test.Count data were described as absolute number or percentage and comparison between groups was analyzed using the chi-square test.Results (1) Intraoperative situations and postoperative short-term outcomes:the operation time,dissection time of left hepatic pedicle,volume of intraoperative blood loss were (123± 37) minutes,(14± 5) minutes,(337± 169) mL in the extra-glissonian transection group and (148± 27) minutes,(22± 3) minutes,(495±203) mL in the intra-glissonian transection group,respectively,showing statistically significant differences between the two groups (t =2.992,7.733,3.045,P＜0.05).Cases with intraoperative blood transfusion,time of gastrointestinal recovery,time for postoperative drainage-tube removal,duration of postoperative hospital stay were 1,(1.8±0.9)days,(3.2±0.9)days,(8.2± 1.7)days in the extra-glissonian transection group and 4,(2.0± 0.8)days,(3.6±0.8)days,(10.0±4.0)days in the intra-glissonian transection group,respectively,showing no statistically significant difference between the two groups (x2 =0.171,t=1.304,1.857,1.622,P＞0.05).There was no uncontrolled hemorrhage or air embolism in the two groups.(2) Postoperative complications:3 patients had complications of Clavien-Dindo classification Ⅰ in the extra-glissonian transection group including 1 of pulmonary infection,1 of abdominal infection,1 of incisional infection and 7 had complications in the intraglissonian transection group including 2 of pulmonary infection,2 of liver sectional effusion,1 of subphrenic abscess,1 of biliary leakage,1 of incisional infection,showing no statistically significant difference between the two groups (x2=0.066,P＞0.05).Patients with postoperative complications were cured and discharged after symptomatic treatment.There was no perioperative death in the two groups.(3) Follow-up:57 patients were followed up for 5-41 months,with a median time of 23 months.Two and 1 patients had tumor recurrence and hepatolithiasis recurrence in the intra-glissonian transection group,without recurrence of hepatic hemangioma or cholangiocarcinoma.Five patients had tumor recurrence in the intra-glissonian transection group,without recurrence of hepatic hemangioma,hepatolithiasis or cholangiocarcinoma.The disease recurrence rate was 13.6％ (3/22) and 14.3％(5/35) in the extra-glissonian transection group and intra-glissonian transection group,respectively,showing no statistically significant difference between the two groups (x2 =0.104,P＞0.05).Conclusions Extraglissonian pedicle transection approach guided by arantius' ligament in laparoscopic left hemihepatectomy is feasible and effective,which can control hepatic blood inflow of left liver,simplify the surgery procedure and shorten dissection time of left pedicle,in order to save operation time and reduce volume of intraoperative blood loss.

Objective: To investigate the clinical value of combined detection of serum angiopoietin 2 (Ang-2) and Clara cell protein 16 (CC16) in the early diagnosis of acute respiratory distress syndrome (ARDS). Methods: Two hundred critical patients, treated at the Department of Critical Care Medicine, Bao'an District People's Hospital, Shenzhen during March 2015 and September 2016,were included in the study. According to the Berlin standard, patients were divided into two groups (n=100 each group): the ARDS group and non-ARDS group.The serum levels of Ang-2 and CC16 were measured by enzyme-linked immunosorbent assay (ELISA) on the first and second day of admission for each patient, in addition to completing APACHEⅡ score, medical history, vital signs collection and other biochemical indicators detection. Finally, paired-samples t test was used to analyze the data. The multiple ROC curve was used to calculate the area under the curve of the reference index of the serum levels of Ang-2 and CC16 on the first and second day. Results: On the first and second day, the serum levels of Ang-2 and CC16 were significantly higher in ARDS patients than those in non-ARDS patients, and there was a correlation between the serum levels of Ang-2 and CC16 in ARDS patients. The ROC curve showed that the combined detection of Ang-2 and CC16 on the first day achieved a highest sensitivity of 75.9% and detection of CC16 on the first day achieved a highest specificity of 70.2%. Conclusion Single-detection of serum levels of Ang-2 and CC16 could be used for early diagnosis of ARDS, and the combined detection of both has a higher sensitivity than single detection.

Objective To measure quantitatively and analysis the differences in the anterior segment biological parameters between the normal subject and patients suffering primary angle closure glaucoma (PACG),as well as the distinction among different stages of PACG by using anterior segment optical coherence tomography (OCT).Methods A retrospective case series study was designed.Medical records of 217 cases (217 eyes) from The Second Hospital of Hebei Medical University from December 2013 to December 2014 were recruited,including 5 groups as follows:35 cases (35 eyes) with pre-clinical stage acute primary angle closure glaucoma (APACG),32 cases (32 eyes) with remission period of APACG,35 cases (35 eyes) with early stage of chronic primary angle closure glaucoma (CPACG),35 cases (35 eyes) with progress period of CPACG and 80 cases (80 eyes) coming for regular eye health examination in general clinic.The anterior segment biological parameters of each group was measured by Heidelberg Spectralis OCT,including the anterior chamber width (ACW),angle opening distance (AOD),trabecular iris area (TISA),iris thickness (IT) and crystalline lens rise (CLR).Results The IT and CLR of APACG and CPACG were significantly greater than normal control group,while other anterior segment parameters were significantly smaller,with significant differences between them (all at P＜0.01).The IT and CLR of APACG was bigger than those of CPACG,with significant differences between them (both at P＜0.05),the ACW,AOD,TISA of the two gruops showed no significant differences.The AOD and TISA of remission period of APACG were significantly decreased than those of pre-clinical stage (all at P＜O.01).The IT and CLR of remission period APACG was significantly greater than pre-clinical stage (both at P＜0.01).The difference in ACW of the two group was not statistically significant (P＞0.05).Compared with progress period of CPACG,the IT of the early stage of CPACG was thicker,while the CLR was smaller (both at P＜0.01).There was no significant difference in ACW,AOD and TISA between the two groups.The IT2000 and ITmax of pre-clinical stage of APACG were significantly smaller than those of early stage of CPACG (both at P＜0.01).There was no significant difference in other parameters between the two groups (P＞0.05).The IT750,IT2 000 and ITmax of the pre-clinical stage of APACG were significantly thicker than those of progress period of CPACG (all at P＜0.05).There was no significant difference in other parameters between the two groups (P＞0.05).Conclusions Compared with normal people,the PACG patients have a more crowding anterior segment structure,smaller AOD,smaller TISA,thicker IT and more anterior located lens.The APACG patient at remission period has a more crowding anterior segment structure,smaller AOD,smaller TISA,thicker IT and more anterior located lens than APACG patient at per-clinic stage.The CPACG patient at progress period has a higher CLR,but thinner IT than patient at early stage.The APACG patients at per-clinic stage has thicker IT and a more crowding anterior segment structure than the CPACG patient at early stage,and the APACG patient at remission period has thicker IT than CPACG patient at progress period.

Objective To explore the effectiveness of Team Strategies & Tools to Enhance Performance and Patient Safety (TeamSTEPPS) in reducing hyperphosphatemia of outpatients on maintenance hemodialysis.Methods A total of 56 outpatients on hemodialysis received traditional dietary management from April to June,2016 were as control group,and 60 patients from October to December,2016 with TeamSTEPPS implemented in hyperphosphatemic dietary management were as observation group.The difference of the incidence rate of hyperphosphatemia and hypoproteinemia,grasp of knowledge of hyperphosphatemic dietary management were compared between two groups and medical staffs.Results The incidence rate of hyperphosphatemia were dropped from 60.7％(34/56) in control group to 41.7％(25/60) in observation group,difference between two groups had statistical significance(x2=4.20,P=0.036).The hypoproteinemia had no statistical difference between two group(x2=2.72,P=0.102),but still dropped from 39.3％ (22/56) in control group to 25.0％ (15/60) in observation group.Grasp of knowledge of hyperphosphatemic dietary management were rised from (75.1±5.1) points and (69.7 ±6.4)points in control group and medical staffs to (88.4±2.6) points and (80.1±6.5) points in observation group and medical staffs respectively,and there were significant differences(t=15.82,-8.84,P=0.00).Conclusions The application of TeamSTEPPS for hyperphosphatemic dietary management in outpatients on maintenance hemodialysis can effectively decrease the incidence of the hyperphosphatemia.