A case report on deficiency of adenosine deaminase 2 with relapse-remission course and analysis of genotype-phenotype correlation.

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic disease caused by biallelic mutations in adenosine deaminase 2 (ADA2). The varying phenotypes of the disease often lead to delayed diagnosis or misdiagnosis. We report an 11-year-old boy with DADA2 and provide a preliminary analysis of genotype-phenotype correlation. The age of onset of the disease was 8 years old. The disease successively involved the brainstem, muscles, joints, and cerebrum. After three relapse-remission episodes over 3 years, the patient was finally diagnosed with DADA2 by whole-exome sequencing. Compound heterozygous variants in the ADA2 gene (NM_001282225.2: c.1072G>A, p.Gly358Arg; c.419dupC, p.Arg141Lysfs*37) were found in the patient. He did not receive anti-TNF therapy and had no relapse after a 8-month follow-up. We identified a novel variant of the ADA2 gene, and the associated disease course may follow a relapse-remission pattern. Homozygous mutations of p.Gly358Arg can cause pure red cell aplasia, whereas compound heterozygous variations may lead to different phenotypes. Variants in the catalytic domain and frameshift mutations may also cause relatively benign phenotypes besides causing hematological disorders. Further studies are needed to clarify the genotypic-phenotypic relationship of this disease.

Novel GRIA2 variant in a patient with atypical autism spectrum disorder and psychiatric symptoms: a case report.

BACKGROUND: As sequencing technology has advanced in recent years, a series of synapse-related gene variants have been reported to be associated with autism spectrum disorders (ASDs). The α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor is a subtype of the ionotropic glutamate receptor, whose number or composition changes can regulate the strength and plasticity of synapses. CASE PRESENTATION: Here, we report a de novo GRIA2 variant (NM_001083619.3: c.2308G > A, p.Ala770Thr) in a patient with obvious behavior regression and psychiatric symptoms. It encodes GluA2, which is the crucial subunit of the AMPA receptor, and the missense variation is predicted to result in instability of the protein structure. CONCLUSIONS: The association between GRIA2 variants and onset of ASD symptoms is rare, and our study expands the spectrum of phenotypic variations. For patients with an unexplained etiology of ASD accompanied by psychiatric symptoms, genetic causes should be considered, and a complete genetic evaluation should be performed.

The predictive ability of occipital to C3 angle for dysphagia after occipitocervical fusion in patients with combined C2-3 Klippel-Feil syndrome.

BACKGROUND: Improper occipitocervical alignment after occipitocervical fusion (OCF) may lead to devastating complications, such as dysphagia and/or dyspnea. The occipital to C2 angle (O-C2a), occipital and external acoustic meatus to axis angle (O-EAa) have been used to evaluate occipitospinal alignment. However, it may be difficult to identify the inferior endplate of the C2 vertebra in patients with C2-3 Klippel-Feil syndrome (KFS). The purpose of this study aimed to compare four different parameters for predicting dysphagia after OCF in patients with C2-3 KFS. METHODS: There were 40 patients with C2-3 KFS undergoing OCF between 2010 and 2019. Radiographs of these patients were collected to measure the occipital to C3 angle (O-C3a), O-C2a, occipito-odontoid angle (O-Da), occipital to axial angle (Oc-Axa), and narrowest oropharyngeal airway space (nPAS). The presence of dysphagia was defined as the patient complaining of difficulty or excess endeavor to swallow. Patients were divided into two groups according to whether they had postoperative dysphagia. We evaluated the relationship between each of the angle parameters and nPAS and analyzed their influence to the postoperative dysphagia. RESULTS: The incidence of dysphagia after OCF was 25% in patients with C2-3 KFS. The Oc-Axa, and nPAS were smaller in the dysphagia group compared to non-dysphagia group at the final follow-up (p < 0.05). Receiver-operating characteristic (ROC) curves showed that dO-C3a had the highest accuracy as a predictor of the dysphagia with an area under the curve (AUC) of 0.868. The differences in O-C3a, O-C2a, O-Da, and Oc-Axa were all linearly correlated with nPAS scores preoperatively and at the final follow-up within C2-3 KFS patients, while there was a higher R2 value between the dO-C3a and dnPAS. Multiple linear regression analysis showed that the difference of O-C3a was the only significant predictor for dnPAS (ß = 0.670, p < 0.001). CONCLUSIONS: The change of O-C3a (dO-C3a) is the most reliable indicator for evaluating occipitocervical alignment and predicting postoperative dysphagia in C2-3 KFS patients. Moreover, dO-C3a should be more than - 2° during OCF to reduce the occurrence of postoperative dysphagia.

Isolation, Purification, and Antioxidant Activities of Polysaccharides from Choerospondias axillaris Leaves.

The extraction, characterization and antioxidant activity of polysaccharides from Choerospondias axillaris leaves were investigated in the present study. Two purified polysaccharide fractions, CALP-1 and CALP-2, were isolated from crude Choerospondias axillaris leaf polysaccharides (CALP) by DEAE-52 cellulose chromatography and Sephadex G-100 column chromatography. The characteristics of CAL-1 and CALP-2 were determined by using High-performance Gel Permeation Chromatography (HPGPC), High-Performance Anion-Exchange Chromatography, HPAEC (HPAEC-PAD) and Fourier transform infrared spectroscopy (FTIR). CALP-1 with molecular weight of 11.20 KDa was comprised of Rhamnose, Arabinose, Galactose, Glucose, Xylose, Mannose and galacturonic acid in a molar ratio of 5.16:2.31:5.50:27.18:1.00:0.76:1.07. CAL-2 with molecular weight of 8.03 KDa consisted of Rhamnose, Arabinose, Galactose, Glucose, and galacturonic acid at a ratio of 1.38:3.63:18.84:8.28:1.45. FTIR revealed that CALP-1 and CALP-2 were acidic polysaccharides. The antioxidant activity of crude CALP, CALP-1 and CALP-2 was evaluated in vitro. The fraction CALP-2 was demonstrated to be of polysaccharide nature containing a large percentage of Galactose but no Xylose and Mannose. The antioxidant activity assays showed that CALP-1 and CALP-2 exhibited antioxidant and scavenging activities on hydroxyl and DPPH radicals in vitro. Compared with pure polysaccharide, crude CALP exhibited stronger anti-oxidant activities. These results will provide a better understanding of Choerospondias axillaris leaf polysaccharide and promote the potential applications of Choerospondias axillaris leaf polysaccharide in the pharmacological field and as a natural antioxidant.

A novel posterior multiple screws distraction reducer system versus anterior release, posterior internal distraction, and subsequent spinal fusion for severe scoliosis.

PURPOSE: We previously reported anterior release, posterior internal distraction, and subsequent spinal fusion (ARPIDF) for the correction of severe scoliosis with a satisfactory correction rate. However, surgical procedures were completed in 2-3 stages. Here we compare Cobb angle of ≥90° in scoliosis correction between a novel posterior multiple screws distraction reducer (MSDR) system and ARPIDF. METHODS: Thirty-six patients with severe scoliosis treated by MSDR or ARPIDF (n = 18 in both groups). We retrospectively analyzed and compared outcome measures between the two groups over a minimum follow-up duration of 2 years. The following variables were compared between the two groups: age at surgery, sex, etiology, flexibility of the main thoracic curve, number of fused segments and screws, operation time, estimated blood loss, hospitalization time, follow-up duration, various radiological parameters, complication rate, and Scoliosis Research Society-30 score. RESULTS: There were no significant between-group differences with respect to age, sex, etiology, flexibility of the main thoracic curve, number of fused segments and screws, and follow-up duration. Further, there was no significant difference in terms of preoperative, postoperative, and final follow-up findings of the radiographic data. However, the ARPIDF group had longer operation and hospitalization times and greater blood loss. In the ARPIDF group, 4 patient developed complications (infection, intraoperative neuromonitoring changes, transient dyspnea); none of these events occurred in the MSDR group. CONCLUSION: The use of MSDR helped achieve greater scoliosis correction with a shorter operation time, lower blood loss, and lower complication rate than the use of ARPIDF. MSDR facilitates safer and easier correction of severe scoliosis without increasing surgical risk.

Proximal junctional kyphosis in Lenke 5 AIS patients: the important factor of pelvic incidence.

BACKGROUND: The purpose of the study was to investigate whether pelvic incidence (PI) will affect the occurrence of PJK in Lenke 5 AIS patients after correction surgery and try to explore a better surgical scheme based on PI. METHODS: Lenke 5C AIS patients that underwent correction surgery with a minimum of a 2-year follow-up were identified. Demographic and radiographic data were collected preoperatively, postoperatively, and at the final follow-up. The comparison between the PJK and the Non-PJK group was conducted and the subgroup analysis was performed based on the preoperative value of PI to investigate the potential mechanism of PJK. Clinical assessments were performed using the Scoliosis Research Society (SRS)-22 questionnaire. RESULTS: The mean preoperative Cobb angle of the TL/L curve was 53.4°±8.6. At the final follow-up, the mean TL/L Cobb angle was drastically decreased to 7.3°±6.8 (P < 0.001). The incidence of PJK in Lenke 5 AIS was 18.6 %, 21.9 % (7/32) in the low PI group (PI < 45°) and 15.8 % (6/38) in the high PI group (PI ≥ 45°), and there was no statistical difference between the two groups (χ2 = 0.425, P = 0.514). For low PI patients, there is no significant difference where the UIV is located with regards to the TK apex between the PJK and Non-PJK subgroups (χ2 = 1.103, P = 0.401). For high PI patients, PJK was more likely to occur when UIV was cephalad to than caudal to the TK apex (31.25 % vs. 4.7 %, P = 0.038). There was no significant difference in the selection of LIV between the two groups. CONCLUSIONS: There is no difference in the incidence of PJK between the Lenke 5 AIS patients with low PI (< 45°) and high PI (≥45°), but the main risk factor of PJK should be different. For patients with low PI, overcorrection of LL should be strictly avoided during surgery. For patients with high PI, the selection of UIV should not be at or cephalad to the apex of thoracic kyphosis to retain more mobile thoracic segments.

Coronal and sagittal balance in Lenke 5 AIS patients following posterior fusion: important role of the lowest instrument vertebrae selection.

BACKGROUND: Lenke 5 AIS is a kind of three-dimensional deformity and literature reported it usually accompany with coronal or/and sagittal imbalance. However, the postoperative coronal and sagittal balance in these patients has rarely be analyzed previously and the predict factors for postoperative trunk balance are still unclear. To synthetically analysis coronal and sagittal balance of Lenke 5 AIS patients simultaneously and found out predict factors for postoperative coronal or/and sagittal imbalance. METHODS: Fifty-six Lenke 5 AIS patients who underwent posterior surgery and be followed up more than 2 years were included in this study. Coronal parameters included main curve Cobb angle, lumbosacral hemi-curve Cobb angle, preoperative LEV/LIV tilt and translation and C7-CSVL distance; While sagittal parameters included pelvic incidence(PI), sacral slope(SS), pelvic tilt(PT), lumbar lordosis(LL), thoracic kyphosis(TK), and sagittal vertical axis(SVA). Coronal imbalance was defined as C7-CSVL> 20 mm, and sagittal imbalance defined as (1) SVA > 40 mm or (2) PT < 20% PI/2 or PT > 20° or (3) PI-LL > 10°. And relative parameters were compared between balance and imbalance group to find out predict factors. RESULTS: All seven final coronal imbalance patients occurred in LIV = L5 group. Preoperative LIV tilt(11.4°) and translation(5.2 mm) in coronal imbalance group were abnormally lower than balance group (21.7° and 15.7 mm respectively). Eighteen patients performed final sagittal imbalance. The PI in these patients (37.7°) was significantly lower than balance group (48.0°). And most of finial sagittal imbalance patients also occurred in LIV = L5 group. CONCLUSIONS: LIV = L5 as a threshold point, represents higher risk of postoperative coronal and/or sagittal imbalance. Besides, large LEV-S1 curve in reduce-bending film and small PI is directly related to final coronal imbalance and sagittal imbalance respectively.

Foxc2 enhances proliferation and inhibits apoptosis through activating Akt/mTORC1 signaling pathway in mouse preadipocytes.

Forkhead box C2 (Foxc2) protein is a transcription factor in regulation of development, metabolism, and immunology. However, the regulatory mechanisms of Foxc2 on proliferation and apoptosis of preadipocytes are unclear. In this study, we found that high-fat-diet-induced obesity elevated the expression of Foxc2 and cyclin E after 6 weeks. Additionally, Foxc2 suppressed preadipocyte differentiation, increased cell counts and augmented G1-S transition of preadipocytes, along with the elevation of cyclin E expression and the reduction levels of p27 and p53. Furthermore, Foxc2 knockdown reduced early apoptotic cells with accompanying reduction of mitochondrial membrane potential and increased fragmentation of genomic DNA. We show that Foxc2 reduces the expression of Bax, caspase-9, and caspase-3 in both serum-starved and palmitic acid-induced cell apoptotic models, which confirms the anti-apoptotic role of Foxc2. Moreover, the protein kinase B (Akt)/mammalian target of rapamycin (mTOR)C1 signaling pathway and the ERK/mTORC1 signaling pathway were activated along with preadipocyte proliferation in response to Foxc2 overexpression, whereas apoptosis marker genes were downregulated during this process. Those effects were blocked by the interference of Foxc2 or signal pathways specific inhibitors. These data collectively reveal that Foxc2 enhances proliferation of preadipocytes and inhibits apoptosis of preadipocytes by activating the Akt/mTORC1 and ERK/mTORC1 signaling pathways.

[Micro magnetic sensitive weighted imaging for intracerebral hemorrhage].

OBJECTIVE: To detect cerebral microbleeds by susceptibility weighted imaging and explore the relationship between intracerebral hemorrhage, cerebral microbleeds and risk factors of cerebral vascular disease. METHODS: A retrospective analysis was conducted for 70 patients from August 2012 to June 2013 undergoing susceptibility weighted imaging (SWI). The average age was (68 ± 14) years. Their demographic and clinical profiles were recorded. Conventional magnetic resonance imaging (MRI) and SWI images were performed and images analyzed by an experienced radiologist. The occurrence of infarction, hemorrhage and number of microbleeds were recorded. Then regression analysis with SPSS 13.0 was used to define the relationship between the existence of infarction, hemorrhage and the number of microbleeds. RESULTS: There were 0-65 microbleeds in brains on SWI. There were infarction (n = 44) and chronic intracranial hemorrhage (n = 7). The binary Logistic regression analysis showed that intracranial hemorrhage was associated with hyperlipidemia (P = 0.01).Linear regression analysis showed that the number of microbleeds was related with intracranial hemorrhage (P = 0.02). CONCLUSION: Evaluation of microbleeds in brain by SWI may help to assess the possibility of intracranial hemorrhage so as to provides rationales for clinical therapeutics.

Bilateral apical vertebral derotation technique by vertebral column manipulation compared with vertebral coplanar alignment technique in the correction of Lenke type 1 idiopathic scoliosis.

BACKGROUND: Widely used rod rotation and translation techniques for idiopathic scoliosis (IS) are effective in correcting spinal coronal deformity. Bilateral apical vertebral derotation technique by vertebral column manipulation (VCM) and vertebral coplanar alignment (VCA) technique are two strategies for three-dimensional (3D) correction for IS. The purpose of this study is to compare the post-surgical results and technical features of the bilateral apical vertebral derotation technique by VCM against the VCA technique in patients with Lenke type 1 IS. METHODS: Forty-eight patients with Lenke type 1 IS were enrolled in the present prospective clinical assay. They were divided into groups A (bilateral apical vertebral derotation technique by VCM, n=24) and B (VCA technique, n=24). Radiographic parameters measured before and after surgery included the Cobb angle, thoracic kyphosis, and apical vertebral rotation. Scoliosis Research Society (SRS)-22 scores were evaluated during the final follow-up. The differences in the demographics, surgical details, and radiographic measurements between the two groups were determined using a T test. The Mann-Whitney U test was used to evaluate the differences in the SRS-22 scores. A value of P<0.05 was considered statistically significant. RESULTS: In the coronal plane, a significant difference was found in the correction rate of the major curve (group A: 84.8%, group B: 78.4%; P=0.045) and in the Cincinnati Correction Index between two groups (group A: 2.21, group B: 1.98; P=0.047). In the sagittal plane, no difference was found in the postoperative thoracic kyphosis between the two groups (P=0.328). In the transverse plane, no difference was found between the two groups in the correction rates of the rotation angle sagittal (P=0.298), rib hump (P=0.934), apical vertebral body-to-rib ratio (P=0.988), or apical rib spread difference (P=0.184). Patients underwent follow up for an average of 21.9 and 22.2 months in groups A and B, respectively. Results obtained at the final follow-up indicated no significant loss of correction. No differences were found in the SRS-22 scores between the two groups. No aortic or neurological complications were observed. CONCLUSIONS: The 3D deformity of the spine was effectively corrected using the bilateral apical vertebral derotation technique by VCM and the VCA technique, and encouraging post-surgical results were obtained for patients with Lenke type 1 IS. The two techniques were effective in allowing 3D correctional force that was applied in different ways.

[Clinical application of multi-slice computed tomography angiography low-dose scan in pulmonary veins and left atrium].

OBJECTIVE: To investigate the clinical application of multi-slice computed tomography angiography (MSCTA) low-dose scan in pulmonary veins and left atrium and to reduce the radiation dose. METHOD: 100 middle-sized patients underwent pulmonary veins and left atrium MSCTA were collected and randomly divided into five groups averaged. The tube current were 250 mAs (conventional dose), 200, 150, 100, and 80 mAs (low dose), respectively. The CT scan length (L), volume CT dose index (CTDIvol) and dose length product (DLP) of five groups were recorded automatically, and the effective dose(ED) was measured. The signal to noise ratio (SNR) and the contrast to noise (CNR) of thoracic aorta at the middle level of left atrium were measured to evaluate the image quality objectively. The image quality of pulmonary veins and left atrium MSCTA was evaluated by two experienced radiologists using double-blind method. The results were analyzed by statistics. RESULT: (1) The CTDIvol(mGy), DLP(mGy*cm) and ED decreased evidently with the scan dose reduced, and the difference had statistical significance. (2) The image quality was mainly excellent and good in 100 mAs and the above, and it was mainly good in 80 mAs group. The image quality decreased with mAs reduced. The difference of SNR and CNR within five groups had statistical significance. The satisfaction degree of subjective evaluation on image quality of the five groups was up to 100%. CONCLUSION: On the premise that the image quality confirmed to the clinical diagnosis, the low dose scan in pulmonary veins and left atrium SCTA can obviously reduce radiation dose, and it had important clinical significance.

Rare improperly treated traumatic vertical atlantoaxial dislocation: A case report and literature review.

BACKGROUND: Because of the severity and fatal outcome of traumatic vertical atlantoaxial dislocation (AAD), most patients may die in the early post-traumatic period. The post-injury management of patients with vertical AAD has been rarely reported. Improper treatment may lead to disastrous outcome and further aggravate the neurologic symptoms. CASE PRESENTATION: This report describes the perioperative management and outcome of a rare improperly treated patient with traumatic vertical AAD. The severe pulmonary infection of this patient prevented further surgery for vertical AAD. After placement of a halo vest, combined with effective antibiotic drug treatment, the patient's pulmonary infection was brought under control. The patient underwent atlantoaxial fusion using C1 lateral mass screws and C2 pedicle screws with the assistance of the halo vest. A computed tomography scan at 1 year follow-up indicated that the bone graft was fused and the patient was able to walk independently. CONCLUSION: Skull traction is contraindicated in patients with traumatic vertical AAD. Application of a halo vest can be used for temporary fixation of the cervical spine and atlantoaxial fixation should be performed to maintain the stability of atlantoaxial articulation.

Comparison of Long-Term Clinical and Radiographical Outcomes between the Anterior and Combined Anterior and Posterior Approaches for Treating Lumbosacral Tuberculosis.

OBJECTIVE: Both anterior and combined anterior and posterior approaches have been used to treat lumbosacral tuberculosis. However, long-term follow-up studies of each approach have not been conducted. We aimed to compare the long-term clinical and radiographical outcomes between the two approaches. METHODS: In this retrospective cohort study, we included 49 patients with a minimum 6-year follow-up between January 2008 and March 2012. Twenty-four patients underwent the anterior approach (anterior group), and 25 underwent the combined anterior and posterior approach (anterior-posterior group). Student's t test, Mann-Whitney U test, and Pearson's chi-square test were used to compare the two groups regarding clinical data, such as visual analogue scale scores, Oswestry disability index scores and neurological status, and radiographical data, such as lumbosacral angle, lumbar lordosis, and L5-S1 height. Furthermore, operative time, length of stay, and intraoperative and postoperative blood loss (IBL, PBL) were recorded. RESULTS: Both groups had satisfactory clinical and radiographical outcomes until the final follow-up. All patients achieved bony fusion, and no group differences were found in any of the clinical indices. Both groups corrected and maintained the lumbosacral angle, lumbar lordosis, and L5-S1 height. However, the operative time, length of stay, maximum Hb drop, IBL, and PBL of the anterior group (140.63 ± 24.73 min, 12.58 ± 2.45 days, 28.33 ± 9.70 g/L, 257.08 ± 110.47 ml, and 430.60 ± 158.27 ml, respectively) were significantly lower than those of the anterior-posterior group (423.60 ± 82.81 min, P < 0.001; 21.32 ± 3.40 days, P < 0.001; 38.48 ± 8.03 g/L, P < 0.001; 571.60 ± 111.04 ml, P < 0.001; and 907.01 ± 231.99 ml, P < 0.001). CONCLUSION: This retrospective study demonstrated long-term efficacy of the anterior approach with a single screw fixation, which was as effective as that of the combined anterior and posterior approach, with the advantage of less trauma.

The Effect of Halo-Pelvic Traction on Bone Mineral Density of Vertebrae and Corresponding Risk Factors.

OBJECTIVES: Decreased bone mineral density (BMD) is associated with complications in implantation surgery for severe spinal deformity. In this quantitative study, we aimed to investigate the impact of halo-pelvic traction on vertebral bone mineral density (BMD) and identify the risk factors for a decrease in BMD. METHODS: Patients who underwent halo-pelvic traction at our hospital between 2019 and 2022 were included in the study. Patients' data, including height, weight, and BMD pre- and post-traction, were collected and analyzed. Quantitative computed tomography (QCT) was used to determine the BMD. The paired rank sum test was used to evaluate the changes in each measurement parameter. Linear regression was used to identify risk factors for a decrease in BMD. RESULTS: Fifteen patients were included in the study, nine women and six men, with an average age of 21.2 ± 7.3 years. Eleven patients had severe rigid scoliosis, while four had tuberculotic kyphosis. One expert measured the BMD values of 345 vertebrae using QCT. The average traction time was 143.3 ± 44.4 days. The average pre-traction BMD was 183.1 ± 73.8 mg/cm3 , and the average post-traction BMD was 140.5 ± 61.3 mg/cm3 (p < 0.01) Patients' height increased from an average of 151.3 ± 12.8 cm pre-traction to 165.5 ± 13.7 cm post-traction (p < 0.01), with traction length averaging 14.3 ± 6.2 cm (p < 0.01). The Cobb angle of the main curve declined from an average of 112.5° ± 24.4° pre-traction to 67.7° ± 19.8° post-traction (p < 0.01). Linear regression revealed a positive correlation between BMD loss and traction length and a negative correlation between BMD loss and correction rate. CONCLUSIONS: Halo-pelvic traction can lead to a decrease in the BMD of the spinal vertebrae, with traction length positively correlated with BMD loss and correction rate negatively correlated with BMD loss. To prevent osteoporosis, physicians should ensure a limited traction length while utilizing better management techniques.

The role of multi-modal intra-operative neurophysiological monitoring in corrective surgeries for thoracic tuberculosis with kyphosis.

OBJECTIVE: The aim of this study was to assess the performance and utility of motor evoked potentials (MEP) and somatosensory evoked potentials (SSEP) during corrective surgery for thoracic tuberculosis with kyphosis (TTK). METHODS: 68 patients (mean age 31.7 ± 20.3 years) who underwent corrective surgery for TTK from 2012 to 2019 were included in this retrospective study. Patients were neurologicaly evaluated before and after surgery with systematic neurologic examinations. Intraoperative neurophysiological monitoring (IONM) with SSEP and MEP was carried out. A receiver operating characteristic (ROC) curve and area under ROC curve (AUC) were used to identify the diagnostic accuracy of potential recovery. RESULTS: IONM alerting occurred in 12 surgeries (12/68, 17.6%), of which 6 were SSEP alerting, 2 MEP alerting, and 4 combinations of both SSEP and MEP. Among the 12 cases where there was IONM alerting, 3 (25%) had postoperative neurological deficits(PND), whereas one patient had PND without IONM alerting. IONM sensitivity and specificity were 0.75 (95% CI 0.22-0.99) and 0.86 (95% CI 0.74-0.93) respectively. Positive predictive value (PPV) and negative predictive value (NPV) were 0.25 and 0.98 respectively. The AUC of evoked potential recovery in diagnosing PND was 0.884. CONCLUSION: Our study showed that multi-modal IONM with SSEP and MEP can effectively indicate a potential neural injury and predict PND during TTK corrective surgery. LEVEL OF EVIDENCE: Level IV, Therapeutic Study.

Metastatic Ceruminous Adenoid Cystic Carcinoma of the Lumbar Spine Causing Neurological Compromise: A Case Report.

Background: Ceruminous glands are modified apocrine glands of the external auditory canal (EAC). Malignant tumours within the ceruminous glands are extremely rare, and the most common histological type is adenoid cystic carcinoma (ADCC), which has high recurrence and metastasis risks. Although a few cases of metastatic ADCC from other head and neck glands have been reported, metastatic ADCC originating from the ceruminous gland are extremely rare. Case presentation: We present an unusual case of spinal metastases of ADCC from ceruminous glands. A 61-year-old woman complaining of low back pain and both lower limbs pain was referred to our department. The primary ceruminous tumour was resected 26 years ago and recurred 6 years later, which was treated by radiotherapy. Three years ago, she presented with low back pain and was diagnosed as multiple lungs and bone metastases. The patient underwent tumour excision, decompression and fusion. The biopsy revealed metastatic ADCC. The symptoms were alleviated after surgery. Conclusions: ADCC of EAC is a pernicious malignant tumour that is characterized by slow-growing patterns and a high predisposition to recurrence and metastasis. Differential diagnoses of ADCC and benign tumours in the EAC are challenging, particularly at early stages. We report a rare case of ceruminous ADCC with a prolonged clinical history as well as spinal metastasis and highlight the significance of regular follow-ups for patients undergoing tumour excision in the EAC.

[Predictive abilities of O-C2 angle, O-EA angle, and Oc-Ax angle for the development of dysphagia in patients after occipitocervical fusion].

OBJECTIVE: To compare the predictive abilities of O-C2 angle (O-C2a), O-EA angle (O-EAa), and Oc-Ax angle (Oc-Axa) for development of dysphagia in patients after occipitocervical fusion (OCF). METHODS: Between April 2010 and May 2019, 114 patients who underwent OCF and met the selection criteria were selected as the research objects. Among them, 54 were males and 60 were females; they were 14-76 years old, with an average of 50.6 years old. The follow-up time was 13-122 months (median, 60.5 months). The O-C2a, O-EAa, Oc-Axa, and the narrowest oropharyngeal airway space (nPAS) were measured by the lateral X-ray films before operation and at last follow-up, and the differences before and after operation (dO-C2a, dO-EAa, dOc-Axa, and dnPAS) were calculated. Patients were divided into two groups according to whether they had developed postoperative dysphagia. The general data including age, gender, fixed segment, proportion of patients with rheumatoid arthritis (RA), atlantoaxial subluxation (AS), and combined with anterior release surgery (ARS), and imaging indicators were compared between the two groups. The correlations between dO-C2a, dO-EAa, and dOc-Axa and dnPAS in 114 patients were analyzed to further compare the predictive value of three imaging indicators for occurrence of dysphagia after OCF. RESULTS: Dysphagia occurred after OCF in 31 cases with the incidence of 27.2%. There was significant difference in gender between the dysphagia group and the non-dysphagia group ( χ 2=7.940, P=0.005). There was no significant difference between the two groups in age, fixed segment, the proportion of patients with RA, the proportion of patients with AS, and the proportion of patients combined with ARS ( P>0.05). There was no significant difference in O-C2a and Oc-Axa of 114 patients before operation and at last follow-up ( P>0.05). The differences in O-EAa and nPAS were significant ( P<0.05). There was no significant difference in preoperative O-EAa, Oc-Axa, and nPAS between the dysphagia group and the non-dysphagia group ( P>0.05); the difference in the O-C2a was significant ( t=2.470, P=0.016). At last follow-up, the differences in the above imaging indicators were significant ( P<0.05). There were significant differences in the dO-C2a, dO-EAa, dOc-Axa, and dnPAS between the two groups ( P<0.05). Correlation analysis showed that the dO-C2a, dO-EAa, dOc-Axa were all positively correlated with dnPAS ( P<0.05). The dO-C2a≤-5°, postoperative O-EAa≤100°, postoperative Oc-Axa≤65° were all related to postoperative dysphagia ( P<0.05), and the highest risk factor suffering postoperative dysphagia was dO-C2a ≤-5° with a significant OR of 14.4. CONCLUSION: The dO-C2a, postoperative O-EAa, and postoperative Oc-Axa can be used as the predictive indexes of dysphagia after OCF, among which dO-C2a has the highest predictive value.

The effect of multiple-dose oral versus intravenous tranexamic acid in reducing postoperative blood loss and transfusion rate after adolescent scoliosis surgery: a randomized controlled trial.

BACKGROUND CONTEXT: Tranexamic acid (TXA) is widely used in surgery for adolescent idiopathic scoliosis (AIS) and has been proved to be efficacious in reducing intraoperative blood loss (IBL) and the transfusion rate. However, the routine TXA regimen was intraoperative administration alone, in which the concentration of TXA could not cover the whole process of hyperfibrinolysis. And, its ability to control the massive postoperative blood loss (PBL) may be insufficient. Thus, we promoted a multiple-dose regimen of TXA for patients with AIS who underwent surgical correction. PURPOSE: The primary aims were (1) to determine whether the multiple-dose regimen of TXA could reduce PBL and the postoperative transfusion rate, and (2) to compare the efficacy of oral administration with intravenous administration. The secondary aims were (3) to evaluate whether this regimen could alleviate inflammatory response, and (4) to assess the occurrence of drug-related side effects. STUDY DESIGN: Prospective, double-blinded, randomized controlled trial. PATIENT SAMPLE: A total of 108 patients with AIS who underwent posterior scoliosis correction and spinal fusion (PSS) were enrolled in this study. OUTCOME MEASURES: The primary parameters were PBL and postoperative transfusion rate. Other parameters such as total blood loss (TBL), maximum hemoglobin (Hb) decrease, volume of drainage, inflammation markers (interleukin-6 [IL-6] and C-reactive protein [CRP]), and occurrence of complications were also collected and compared. Multiple regression analysis was used to examine the variables that affected PBL. METHODS: Patients were randomized into three groups. All patients received intravenous TXA 50 mg/kg loading dose and 10 mg/kg/h maintenance dose during surgery. Group A received 1 g oral TXA at 4 hours, 10 hours, and 16 hours postoperatively; group B received 0.5 g intravenous TXA at 6 hours, 12 hours, and 18 hours postoperatively; group C received placebo. RESULTS: The mean PBL and postoperative transfusion rate in group A (957.8±378.9 mL, 13.89%) and B (980.3±491.8 mL, 11.11%) were significantly lower than those in group C [1,495.9±449.6 mL, mean differences=538.1 mL, 95% confidence interval (CI), 290.1-786.1 mL, p<0.001; 515.6 mL, 95% CI, 267.6-763.6 mL, p<.001]; (36.11%, p=.029, p=.013). Meanwhile, the mean TBL, maximum Hb decrease, and volume of drainage were also significantly lower in group A and B than in group C. IL-6 and CRP in group A and B were significantly lower than in group C from postoperative days 1 to 3. All these differences were not significant between groups A and B. No drug-related complications were observed in any patient. Multiple regression showed that the application of postoperative TXA and number of screws were significant parameters affecting PBL. CONCLUSIONS: A multiple-dose regimen of TXA, either by oral or intravenous application, could be a safe and effective means of controlling PBL and decreasing the postoperative transfusion rate in patients with AIS who underwent scoliosis surgery. In addition, it could inhibit postoperative inflammatory response.

Primary psoas abscess extending to thigh adductors: case report.

BACKGROUND: Psoas abscess is a rare condition consisting of pyomyositis of the psoas. The worldwide incidence was 12 cases per 100,000 per year in 1992, but the current incidence is unknown. Psoas abscess can descend along the psoas sheath and reach the inner upper third of the thigh, but only infrequently does it penetrate the sheath and involve the thigh adductors. Because of insidious clinical presentation, the diagnosis of psoas abscess is a challenge. Delayed diagnosis can result in poor prognosis. CASE PRESENTATION: A 45-year-old male with no significant past medical history presented with pain in the left thigh, and limitation of movement at the left hip and knee joint for one month. Ultrasound, CT, and MRI revealed a liquid mass in the left psoas. Percutaneous drainage of this mass yielded 300 ml pus from the psoas. After surgery, the patient reported relief of pain; however, ten days after removal of the drainage tube, the patient complained of persistent pain in his left thigh. CT revealed that the psoas abscess had extended inferiorly, and involved the entire set of adductors of the left thigh. Open surgical drainage was performed at the flank and at the thigh, yielding 350 ml of pus from the thigh. After open drainage and adequate antibiotic therapy, the patient made a good recovery. Follow-up CT confirmed complete resolution of the abscess. CONCLUSIONS: Large psoas abscess can penetrate the psoas sheath, and descend to thigh adductors even after percutaneous drainage. Appropriate treatment includes open surgical drainage along with antibiotic therapy.

miR-384-5p promotes spinal cord injury recovery in rats through suppressing of autophagy and endoplasmic reticulum stress.

BACKGROUND: Spinal cord injury (SCI) is one of the most serious neurological disorders and is characterized by high morbidity and disability. Unfortunately, there is a lack of effective treatment. Recently, the micro RNA, miR-384-5p, was reported to play a significant role in cell survival in response to different insults. METHODS: In vitro model of traumatic neuronal injury was induced by application of a sharp sterile blade to generate cuts in PC12 cells, and in vivo SCI was produced by applying vascular clips (force of 15 g) to the dura via T9-T10 laminectomy, and then, the role of miR-384-5p in the development of SCI was investigated. RESULTS: Dual-luciferase reporter assays confirmed that miR-384-5p regulates the gene expression of Beclin-1, an important promoter of autophagy. Quantitative polymerase chain reaction and western blot analyses revealed that treatment with miR-384-5p decreased mRNA and protein expression of Beclin-1 in the mechanically injured PC12 cells. In rats with spinal cord compression injuries, miR-384-5p expression was significantly decreased. Treatment with miR-384-5p increased spinal cord neuron survival and promoted locomotor function recovery in rats. Further study revealed that miR-384-5p administration decreased immunofluorescent labeling of Beclin-1 in spinal cord tissues and reduced autophagosome formation in neurons, as shown by transmission electron microscopy. These results indicated that miR-384-5p promotes recovery of rats with SCI by suppressing autophagy via direct targeting of Beclin-1. Moreover, miR-384-5p also inhibited the activation of endoplasmic reticulum (ER) stress by decreasing GRP78 expression in both in vitro and in vivo models. CONCLUSIONS: This study for the first time demonstrates that the protective role of miR-384-5p in the process of SCI is associated with simultaneous suppression of autophagy and ER stress and miR-384-5p could be a promising candidate for SCI therapeutics.