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*

Objective:Coarctation of the aorta is a genetically related diffuse aortic disease. Even after successful repair and with normal blood pressure, patients have a significant increase of risk in long-term cardiovascular events. Coarctation of the aorta accounts for 6%-8% of congenital heart diseases. Although the results of surgery and interventional treatment are satisfactory, the long-term survival rate of patients is still lower than that of health people, and there is a high proportion of re-intervention. Factors affecting prognosis include concomitant diseases, genetic characteristics, restenosis, hypertension, heart failure, neurodevelopmental disorders, obesity, psychology, etc. Therefore, people with coarctation of the aorta need cardiovascular monitoring throughout their life cycle. Currently, there are relevant guidelines of follow-up management for adult patients, but there is still no clear cardiovascular monitoring strategy for child and adolescent patients in transition. Based on a large number of domestic and foreign literature and clinical experience, this article summarizes the follow-up management strategies for full-life cardiovascular monitoring immediately after the repair of coarctation of the aorta in infancy and childhood from the perspective of concomitant diseases, genetic characteristics and long-term complications.

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 evaluate the feasibility of multi spiral computed tomography(MSCT)for etiological analysis of patients with pelvic venous congestion syndrome(PVCS).Methods The clinical and imaging data of 134 patients diagnosed with PVCS were analyzed retrospectively.The anatomical etiology of PVCS was analyzed,and the clinical and imaging differences between the two groups with and without vascular variation were compared.Results Among the 134 patients,69 had vascular variation,which included 37 distal hypoplasia of left renal vein(LRV),22 nutcracker phenomenon,6 ovarian vein(OV)variation,2 LRV variation,2 portal hyper-tension,2 pelvic arteriovenous fistula,1 left iliac vein compression syndrome,1 left renal malrotation and 1 left renal arteriovenous fistula.The remaining 65 cases showed no vascular variation.There were statistically significant differences in the left ovarian vein(LOV)diameter and reflux degree between the two groups(t=2.544,P=0.012;χ2=21.797,P<0.001).Patients with vascular varia-tion had wider diameter of LOV and higher degree of reflux.There were no statistically significant differences between the two groups regarding age,symptom,times of pregnancy and delivery and body mass index(BMI)values(P>0.05).Conclusion MSCT can provide important reference value for etiological analysis of PVCS,which is helpful for precise treatment.

Objective To evaluate the feasibility of multi spiral computed tomography(MSCT)for etiological analysis of patients with pelvic venous congestion syndrome(PVCS).Methods The clinical and imaging data of 134 patients diagnosed with PVCS were analyzed retrospectively.The anatomical etiology of PVCS was analyzed,and the clinical and imaging differences between the two groups with and without vascular variation were compared.Results Among the 134 patients,69 had vascular variation,which included 37 distal hypoplasia of left renal vein(LRV),22 nutcracker phenomenon,6 ovarian vein(OV)variation,2 LRV variation,2 portal hyper-tension,2 pelvic arteriovenous fistula,1 left iliac vein compression syndrome,1 left renal malrotation and 1 left renal arteriovenous fistula.The remaining 65 cases showed no vascular variation.There were statistically significant differences in the left ovarian vein(LOV)diameter and reflux degree between the two groups(t=2.544,P=0.012;χ2=21.797,P<0.001).Patients with vascular varia-tion had wider diameter of LOV and higher degree of reflux.There were no statistically significant differences between the two groups regarding age,symptom,times of pregnancy and delivery and body mass index(BMI)values(P>0.05).Conclusion MSCT can provide important reference value for etiological analysis of PVCS,which is helpful for precise treatment.

Objective:Coarctation of the aorta is a genetically related diffuse aortic disease. Even after successful repair and with normal blood pressure, patients have a significant increase of risk in long-term cardiovascular events. Coarctation of the aorta accounts for 6%-8% of congenital heart diseases. Although the results of surgery and interventional treatment are satisfactory, the long-term survival rate of patients is still lower than that of health people, and there is a high proportion of re-intervention. Factors affecting prognosis include concomitant diseases, genetic characteristics, restenosis, hypertension, heart failure, neurodevelopmental disorders, obesity, psychology, etc. Therefore, people with coarctation of the aorta need cardiovascular monitoring throughout their life cycle. Currently, there are relevant guidelines of follow-up management for adult patients, but there is still no clear cardiovascular monitoring strategy for child and adolescent patients in transition. Based on a large number of domestic and foreign literature and clinical experience, this article summarizes the follow-up management strategies for full-life cardiovascular monitoring immediately after the repair of coarctation of the aorta in infancy and childhood from the perspective of concomitant diseases, genetic characteristics and long-term complications.

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 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 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