Thoracic Morphology and Bronchial Narrowing Are Related to Pulmonary Function in Adolescent Idiopathic Scoliosis.

BACKGROUND: In adolescent idiopathic scoliosis (AIS), lung function impairment is not necessarily related to the coronal spinal deformity. Recently, right-sided bronchial narrowing has been reported in thoracic AIS. The aim of this study was to describe the relation of chest and spinal deformity parameters, bronchial narrowing, and lung volumes with pulmonary function in preoperative AIS. METHODS: Spinal radiographs, low-dose computed tomographic (CT) scans of the spine including the chest, and pulmonary function tests were retrospectively collected for 85 preoperative patients with thoracic AIS in 2 centers and were compared with 14 matched controls. Three-dimensional lung and airway reconstructions were acquired. Correlation analysis was performed in which radiographic spinal parameters, CT-based thoracic deformity parameters (rib-hump index [RHi], spinal penetration index, endothoracic hump ratio, hemithoracic-width ratio), lung volume asymmetry, and bronchial cross-sectional area were compared with percent-of-predicted spirometry results. RESULTS: Forty-one patients (48%) had a percent-of-predicted forced expiratory volume in 1 second (FEV1%) or percent-of-predicted forced vital capacity (FVC%) of <65%, and 17 patients (20%) had obstructive lung disease. All thoracic deformity parameters correlated significantly with FEV1% and FVC%; RHi was found to be the best correlate (rs = -0.52 for FEV1% and -0.54 for FVC%). Patients with AIS with impaired pulmonary function had hypokyphosis, a larger rib hump, increased spinal and thoracic rotation, a narrower right hemithorax, and increased intrusion of the spine into the chest. Spinal intrusion correlated with right-sided bronchial narrowing, relative right lung volume loss, and decreased FEV1% and FVC%. Multivariate regression including spinal and thoracic deformity parameters, lung volume asymmetry, and airway parameters could explain 57% of the variance in FEV1% and 54% of the variance in FVC%. CONCLUSIONS: Chest intrusion by the endothoracic hump is related to right-sided bronchial narrowing and lung function loss in preoperative AIS. The findings support the theory that ventilatory dysfunction in thoracic AIS is not only restrictive but frequently has an obstructive component, especially in patients with hypokyphosis. RHi is the most predictive chest parameter for lung function loss. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Long-term follow-up of untreated Scheuermann's kyphosis.

STUDY DESIGN: Long-term cross-sectional study. OBJECTIVES: To investigate the long-term effects of untreated Scheuermann's kyphosis on quality of life, and its relationship to radiographic parameters of spinal deformity. Previous studies reported reduced self-image, increased pain and impaired physical status. Little is known of the long-term impact of sagittal plane deformity in untreated SK. METHODS: One hundred and thirteen consecutive untreated patients with SK were identified from a national service database prior to 2000, when surgery was not offered at this unit. 81 of these patients were available for evaluation; 66 (81%) consented to questionnaire and clinical evaluation, and 47 (58%) consented to additional radiological evaluation. Health-related quality of life (HRQoL) was compared to normative population values. Mean age was 45.1 years (31-65), and mean follow-up was 27 years (16-36). 57 patients had thoracic kyphosis and 9 had thoracolumbar deformity. RESULTS: SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative population values. Kyphosis progressed from mean 66° at skeletal maturity to 78° (p < 0.001) after mean follow-up of 27 years. Long-term progression of untreated SK was 0.45°/year (n = 47). Multilinear regression showed good correlation between increasing SVA and worse ODI scores (r = 0.59; p = 0.001). Increasing SVA also correlated with worse function, pain and mental health scores reported by SRS-22, and with worse physical function and bodily pain scores reported by SF-36. Increasing CL correlated with worse SF-36 physical function scores. Increasing cSVA and increasing TK correlated with worse SRS-22 self-image scores. CONCLUSION: SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative data. Long-term progression of untreated SK was 0.45°/year (n = 47). Increasing SVA correlated with worse SF-36 physical function, SRS-22 function, SRS-22 pain and higher ODI scores. Total kyphosis (TK) and cSVA were independent predictors of low SRS self-image. LEVEL OF EVIDENCE: III.

Association between air pollution in Lima and the high incidence of COVID-19: findings from a post hoc analysis.

BACKGROUND: Coronavirus disease 2019 (COVID-19) originated in the People's Republic of China in December 2019. Thereafter, a global logarithmic expansion of cases occurred. Some countries have a higher rate of infections despite the early implementation of quarantine. Air pollution might be related to high susceptibility to the virus and associated case fatality rates (deaths/cases*100). Lima, Peru, has the second highest incidence of COVID-19 in Latin America and also has one the highest levels of air pollution in the region. METHODS: This study investigated the association of levels of PM2.5 exposure in previous years (2010-2016) in 24 districts of Lima with cases, deaths and case fatality rates for COVID-19. Multiple linear regression was used to evaluate this association controlled by age, sex, population density and number of food markets per district. The study period was from March 6 to June 12, 2020. RESULTS: There were 128,700 cases in Lima and 2382 deaths due to COVID-19. The case fatality rate was 1.93%. Previous exposure to PM2.5 (2010-2016) was associated with the number of COVID-19- cases (ß = 0.07; 95% CI: 0.034-0.107) and deaths (ß = 0.0014; 95% CI: 0.0006-0.0.0023) but not with the case fatality rate. CONCLUSIONS: After adjusting for age, sex and number of food markets, the higher rates of COVID-19 in Metropolitan Lima are attributable to the increased PM2.5 exposure in the previous years, among other reasons. Reduction in air pollution from a long-term perspective and social distancing are needed to prevent the spread of virus outbreaks.

Health-related quality of life and sagittal balance at two to 25 years after posterior transfixation for high-grade dysplastic spondylolisthesis.

AIMS: High-grade dysplastic spondylolisthesis is a disabling disorder for which many different operative techniques have been described. The aim of this study is to evaluate Scoliosis Research Society 22-item (SRS-22r) scores, global balance, and regional spino-pelvic alignment from two to 25 years after surgery for high-grade dysplastic spondylolisthesis using an all-posterior partial reduction, transfixation technique. METHODS: SRS-22r and full-spine lateral radiographs were collected for the 28 young patients (age 13.4 years (SD 2.6) who underwent surgery for high-grade dysplastic spondylolisthesis in our centre (Scottish National Spinal Deformity Service) between 1995 and 2018. The mean follow-up was nine years (2 to 25), and one patient was lost to follow-up. The standard surgical technique was an all-posterior, partial reduction, and S1 to L5 transfixation screw technique without direct decompression. Parameters for segmental (slip percentage, Dubousset's lumbosacral angle) and regional alignment (pelvic tilt, sacral slope, L5 incidence, lumbar lordosis, and thoracic kyphosis) and global balance (T1 spino-pelvic inclination) were measured. SRS-22r scores were compared between patients with a balanced and unbalanced pelvis at final follow-up. RESULTS: SRS-22r domain and total scores improved significantly from preoperative to final follow-up, except for the mental health domain that remained the same. Slip percentage improved from 75% (SD 15) to 48% (SD 19) and lumbosacral angle from 70° (SD 11) to 101° (SD 11). Preoperatively, 35% had global imbalance, and at follow-up all were balanced. Preoperatively, 63% had an unbalanced pelvis, and at final follow-up this was 32%. SRS-22r scores were not different in patients with a balanced or unbalanced pelvis. However, postoperative pelvic imbalance as measured by L5 incidence was associated with lower SRS-22r self-image and total scores (p = 0.029). CONCLUSION: In young patients with HGDS, partial reduction and transfixation improves local lumbosacral alignment, restores pelvic, and global balance and provides satisfactory long-term clinical outcomes. Higher SRS-22r self-image and total scores were observed in the patients that had a balanced pelvis (L5I < 60°) at two to 25 years follow-up. Cite this article: Bone Jt Open 2021;2(3):163-173.

Association between air pollution in Lima and the high incidence of COVID-19: findings from a post hoc analysis.

Background: Corona virus disease (COVID-19) originated in China in December 2019. Thereafter, a global logarithmic expansion of the cases has occurred. Some countries have a higher rate of infections despite of early implementation of quarantine. Air pollution could be related to the high susceptibility to SARS-CoV-2 and the associated case-fatality rates (deaths/cases*100). Lima, Peru has the second highest incidence of COVID-19 in Latin America, and it is also one of the cities with highest levels of air pollution in the Region. Methods: This study investigated the association of the levels of PM2.5 exposure in the previous years (2010-2016) in 24 districts of Lima with the positive-cases, deaths and case-fatality rates of COVID-19. Multiple Linear regression was used to evaluate this association controlled by age, sex, population density and number of food markets per district. The study period was from March 6 to June 12, 2020. Results: There were in Lima 128,700 SARS-CoV-2 positive cases, and 2,382 deaths due to COVID-19. The case-fatality rate was 1.93%. Previous exposure to PM2.5 (years 2010-2016) was associated with number of Covid-19 positive-cases (ß = 0.07; 95% CI: 0.034-0.107) and deaths (ß = 0.0014; 95% CI: 0.0006-0.0.0023), but not with case-fatality rate. Conclusions: the higher rates of COVID-19 in Metropolitan Lima is attributable, among others, to the increased PM2.5 exposure in the previous years after adjusting for age, sex and number of food markets. Reduction of air pollution since a long-term perspective, and social distancing are needed to prevent spreads of virus outbreak.

Predicting preoperative pulmonary function in patients with thoracic adolescent idiopathic scoliosis from spinal and thoracic radiographic parameters.

PURPOSE: The objective was to analyse the effect of thoracic morphology on pulmonary function in adolescent idiopathic scoliosis (AIS) to predict preoperative lung function. METHODS: A total of 170 consecutive preoperative patients (average age 15.1 years) with Lenke 1 and 2 AIS underwent pulmonary function testing. Thirteen deformity parameters, including rib hump size, rib asymmetry, spinal intrusion and thoracic/lung dimensions in the sagittal and coronal plane, were measured on whole-spine radiographs. Lung function parameters were expressed as z-scores. Correlation and regression analysis of these parameters with lung function were performed. RESULTS: Mean thoracic Cobb (MT) was 69.1°, and mean T5-T12 thoracic kyphosis (TK) was 21.8°. MT correlated significantly with FEV1 and FVC (rs = - 0.40 and - 0.38). TK correlated weakly with FEV1 and FEV1/FVC (rs = 0.23 and 0.25). FEV1 and FVC were best predicted by the inverse apical vertebra body-rib ratio (AVBRr-1, rs = 0.46 and 0.42), rib hump depth index (RHDi, rs = - 0.52 and - 0.50) and spinal intrusion ratio (SIr, rs = - 0.50 and - 0.45). The kyphosis-lordosis index (KLi) correlated with FEV1/FVC (rs = 0.29). Multivariate regression analysis of coronal and sagittal Cobb angles produced a model which explained 35% and 30% of the variance in FEV1 and FVC, whilst a regression model consisting of AVBRr-1 and SIr was able to predict 54% and 48%. CONCLUSION: AVBRr-1, RHDi and SIr measured on posterior-anterior and lateral radiographs provide better estimations of lung function in preoperative AIS patients than Cobb measurements. KLi was an indicator of airway obstruction as measured by FEV1/FVC.

Combination of Congenital and Deep Penetrating Nevus by Acquisition of ß-Catenin Activation.

Deep penetrating nevus (DPN) is an intradermal, sometimes compound benign melanocytic lesion, which involves the reticular dermis, occasionally reaching the subcutis, which can raise concern for melanoma both clinically and histologically. Recently, it has been genetically defined by the combination of MAPK activating and ß-catenin activating mutations. We sought to investigate genetic alterations in 2 cases of combined nevi of congenital melanocytic and DPN. Case 1 was a 16-year-old woman with a pigmented lesion on the trunk since birth, which was completely excised. Histopathological examination revealed a combined congenital nevus with a DPN. Comparative genomic hybridization showed no major genetic alterations, except for gain of 6q11.1 and point mutation of B-RAF V600E. Case 2 was a 62-year-old woman with a congenital pigmented lesion on the back. The lesion was diagnosed as a combined nevus of congenital and DPN. Comparative genomic hybridization showed no genetic alterations, and the NRAS Q61K was detected in both components. DPN is in most cases part of a combined nevus. Our cases showed strong and uniform nuclear expression of ß-catenin and cyclin D1 in the DPN component suggesting the evolution of the congenital nevus to the DPN clone by acquiring ß-catenin activating mutation.

Initial impact of COVID-19 on paediatric spinal services in Scotland.

COVID-19 has affected many National Health Service Scotland services. Our aim is to describe this impact on the Scottish National Spine Deformity Service (SNSDS). All referrals to the SNSDS from 1 January 2020 to 30 June 2020 were analysed and compared with the same period in 2019. There was a 64.3% decline in referrals during the pandemic to the SNSDS. The mean waiting time to be seen in first clinical appointment for a new referral was 6.5 weeks in 2020 compared with 10.9 in 2019. There were 60 patients still waiting to be seen at the end of the study period.

Effect of idiopathic thoracic scoliosis on the tracheobronchial tree.

INTRODUCTION: High prevalence of obstructive lung disease has been reported in patients undergoing surgical correction of thoracic scoliosis. Airway narrowing due to spine morphology is analysed as a contributing factor. METHODS: Preoperative surgical planning CTs of 34 patients with right-sided thoracic scoliosis (age: 17.6±9.0) were retrospectively analysed and compared with 15 non-scoliotic controls (age: 16.3±5.1). Three-dimensional models of spine and airway lumen were reconstructed. Based on thoracic sagittal profile, patients were divided into hypokyphosis (HypoS: <10°), normal kyphosis (NormS: ≥10° and <40°) and hyperkyphosis (HyperS: ≥40°) groups. Lumen area of bronchi, bifurcation angles and minimum spine-airway distance were measured. Pulmonary function tests were correlated to scoliosis, kyphosis and lumen area. RESULTS: Loss of kyphosis led to proximity between bronchus intermedius (BI) and spine. HypoS (NormS) had lumen area reductions in the right main bronchus of 29% (19%), BI of 45% (23%), right middle lobar bronchus of 46% (32%) and right lower lobe bronchus (RLL7) of 66% (37%), respectively (P<0.05). The lower right superior segmental bronchus was reduced across all scoliotic groups (P<0.05). Airways were displaced caudal by 0.65±0.45 vertebra in patients with scoliosis. Loss of kyphosis correlated negatively with forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC), FVC/(FVC predicted) and FEV1/(FEV1 predicted) (P<0.01). Lumen area of trachea, right upper lobar bronchus, BI and RLL7 correlated negatively with FEV1/FVC. BI and RLL7 narrowing were strong predictors of FVC and FEV1 loss (P<0.001). CONCLUSIONS: Right-sided main stem airways are narrowed in HypoS and NormS. Loss of kyphosis leads to narrowing of BI and its trifurcation. FEV1/FVC correlated negatively with airway narrowing, implying an obstructive element to lung function impairment in patients with scoliosis and hypokyphosis.

Transcranial magnetic stimulation in the semi-quantitative, pre-operative assessment of patients undergoing spinal deformity surgery.

PURPOSE: To compare measurements of motor evoked potential latency stimulated either magnetically (mMEP) or electrically (eMEP) and central motor conduction time (CMCT) made pre-operatively in conscious patients using transcranial and intra-operatively using electrical cortical stimulation before and after successful instrumentation for the treatment of adolescent idiopathic scoliosis. METHODS: A group initially of 51 patients with adolescent idiopathic scoliosis aged 12-19 years was evaluated pre-operatively in the outpatients' department with transcranial magnetic stimulation. The neurophysiological data were then compared statistically with intra-operative responses elicited by transcranial electrical stimulation both before and after successful surgical intervention. MEPs were measured as the cortically evoked compound action potentials of Abductor hallucis. Minimum F-waves were measured using conventional nerve conduction methods and the lower motor neuron conduction time was calculated and this was subtracted from MEP latency to give CMCT. RESULTS: Pre-operative testing was well tolerated in our paediatric/adolescent patients. No neurological injury occurred in any patient in this series. There was no significant difference in the values of mMEP and eMEP latencies seen pre-operatively in conscious patients and intra-operatively in patients under anaesthetic. The calculated quantities mCMCT and eCMCT showed the same statistical correlations as the quantities mMEP and eMEP latency. CONCLUSIONS: The congruency of mMEP and eMEP and of mCMCT and eCMCT suggests that these measurements may be used comparatively and semi-quantitatively for the comparison of pre-, intra-, and post-operative spinal cord function in spinal deformity surgery.

Paniculitis mesentérica / Mesenteric panniculitis

La paniculitis mesentérica es una entidad caracterizada por inflamación crónica e inespecífica del mesenterio. Existe poca información y a menudo confusa sobre sus características, así como sobre la actitud a seguir una vez que se evidencia en una prueba de imagen. Nos proponemos describir las características epidemiológicas, clínicas, analíticas, radiológicas y patológicas de los pacientes con paniculitis mesentérica publicados en la literatura, así como las entidades posiblemente causales o asociadas a la paniculitis mesentérica, según la opinión de los autores de cada estudio. Por último, revisaremos las diferentes opciones terapéuticas utilizadas y la respuesta a las mismas. Para ello se realizó una búsqueda bibliográfica en las principales bases de datos médicas, seleccionando aquellos artículos con información sobre estos aspectos, recogiendo dicha información en una base de datos que se almacenó en el paquete estadístico SPSS para su posterior análisis y resumen (AU)

Mesenteric panniculitis is a condition characterized by chronic nonspecific inflammation of the mesentery. There is little and often confusing information about its characteristics and the approach to take once it has been demonstrated by an imaging test. We propose to describe the epidemiological, clinical, laboratory, radiological and pathological features of the patients with mesenteric panniculitis reported in the literature, as well as possible disorders causal or associated with mesenteric panniculitis, in the opinion of the authors of each study. Finally, we will review the different therapeutic options used and the response to them. To that end a literature search was performed from the main medical databases selecting ítems with information on these aspects. This information was collected on a database stored in SPSS software for further analysis and summary (AU)

[Mesenteric panniculitis]. / Paniculitis mesentérica.

Mesenteric panniculitis is a condition characterized by chronic nonspecific inflammation of the mesentery. There is little and often confusing information about its characteristics and the approach to take once it has been demonstrated by an imaging test. We propose to describe the epidemiological, clinical, laboratory, radiological and pathological features of the patients with mesenteric panniculitis reported in the literature, as well as possible disorders causal or associated with mesenteric panniculitis, in the opinion of the authors of each study. Finally, we will review the different therapeutic options used and the response to them. To that end a literature search was performed from the main medical databases selecting ítems with information on these aspects. This information was collected on a database stored in SPSS software for further analysis and summary.

PXK locus in systemic lupus erythematosus: fine mapping and functional analysis reveals novel susceptibility gene ABHD6.

OBJECTIVES: To perform fine mapping of the PXK locus associated with systemic lupus erythematosus (SLE) and study functional effects that lead to susceptibility to the disease. METHODS: Linkage disequilibrium (LD) mapping was conducted by using 1251 SNPs (single nucleotide polymorphism) covering a 862 kb genomic region on 3p14.3 comprising the PXK locus in 1467 SLE patients and 2377 controls of European origin. Tag SNPs and genotypes imputed with IMPUTE2 were tested for association by using SNPTEST and PLINK. The expression QTLs data included three independent datasets for lymphoblastoid cells of European donors: HapMap3, MuTHER and the cross-platform eQTL catalogue. Correlation analysis of eQTLs was performed using Vassarstats. Alternative splicing for the PXK gene was analysed on mRNA from PBMCs. RESULTS: Fine mapping revealed long-range LD (>200 kb) extended over the ABHD6, RPP14, PXK, and PDHB genes on 3p14.3. The highly correlated variants tagged an SLE-associated haplotype that was less frequent in the patients compared with the controls (OR=0.89, p=0.00684). A robust correlation between the association with SLE and enhanced expression of ABHD6 gene was revealed, while neither expression, nor splicing alterations associated with SLE susceptibility were detected for PXK. The SNP allele frequencies as well as eQTL pattern analysed in the CEU and CHB HapMap3 populations indicate that the SLE association and the effect on ABHD6 expression are specific to Europeans. CONCLUSIONS: These results confirm the genetic association of the locus 3p14.3 with SLE in Europeans and point to the ABHD6 and not PXK, as the major susceptibility gene in the region. We suggest a pathogenic mechanism mediated by the upregulation of ABHD6 in individuals carrying the SLE-risk variants.

Influence of psychological stress on headache in patients with systemic lupus erythematosus.

OBJECTIVE: To compare the prevalence and disability of headache in patients with systemic lupus erythematosus (SLE) with the general population and to assess the role of chronic psychological stress (CPS) in headache development. METHODS: One hundred seventy patients with SLE and 102 control subjects matched for age, sex, and level of education were included in this multicenter, cross-sectional study. CPS, headache-related disability, and chronic analgesic intake (CAI) were evaluated in all participants. RESULTS: No statistical differences in the prevalence of headache between both groups were observed but headache disability was significantly higher in patients with SLE. In addition, a higher average score in the Cohen Perceived Stress Scale (CPSS) and a higher prevalence of patients with CAI were observed in patients with SLE. In multivariate analysis, CPSS score was positively (OR 1.09; 95% CI: 1.03-1.14; p = 0.001) and CAI negatively (OR 0.43; 95% CI: 0.19-0.99; p = 0.049) associated with headache in patients with SLE. CONCLUSION: Despite the prevalence of headache in patients with SLE and the general population being similar, headache-related disability may be higher in patients with SLE. Moreover, CPS might play a role in the pathogenesis of SLE headache, whereas CAI might have a protective effect against it.

Combined spinal arthrodesis with instrumentation for the management of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis.

PURPOSE: To evaluate the outcome and complications of a novel technique for the treatment of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis (MPS). METHODS: The medical records and spinal imaging of four consecutive paediatric patients who underwent a single stage anteroposterior spinal fusion with segmental pedicle screw instrumentation were reviewed. RESULTS: Patients underwent spinal deformity correction at the mean age of 3 years (2.4-3.7) with mean clinical follow-up of 3.2 years (2.1-4.5) and mean postoperative radiographic follow-up was 2.4 years (0.8-3). Preoperative kyphosis was corrected from a mean angle of 65º (63º-70º) to 6.5º (-12º-13º). Vertebral subluxation at the apex of the deformity was corrected from an average 64% (56-83%) to 12% (0-24%). Spinal cord monitoring with somatosensory evoked potentials (SSEP) was successfully obtained and stable throughout surgery. No instrumentation failure, loss of correction or junctional problems occurred at final follow-up. CONCLUSIONS: Anterior and posterior spinal arthrodesis with segmental pedicle screw instrumentation is a safety and reliable technique for the treatment of severe thoracolumbar kyphosis in children with MPS. This technique achieves excellent correction of the deformity with adequate decompression of the spinal canal. The fusion is limited to the thoracolumbar junction and interferes minimally with the longitudinal growth of the thorax. No neurological complications or intraoperative spinal cord monitoring events occurred. No loss of correction or junctional kyphosis was observed.

Health-related internet use by lupus patients in southern Spain.

Internet has become a widely used tool by patients seeking information on different diseases. The information regarding lupus patients' Internet use is scarce. This study aims to explore the attitudes and practices of lupus patients in southern Spain, regarding Internet use to find health-related information. A survey was carried out including 150 patients from six Andalusian Hospitals. To search for information, 67.3 % of the patients used Internet. The proportion of female Internet users was higher (69.3 vs 46.2 %), particularly those belonging to a patients' association (81.8 vs 32.7 %), and are regular users of Internet (80.2 vs 44.4 %); 37.5 % thought the information found in the Internet was of little use or not useful at all, and 58 % of the respondents stated that the information found caused them concern while for 27 %, it was a relief. Most patients preferred the information given by their physicians (63.6 %); 33.9 % considered that the information from both sources was complementary, and 2.5 % preferred the information obtained from the Internet. A percentage of 85.3 of the patients would like their physicians to provide them with information on high-quality sites regarding their illness. Lupus patients make frequent use of the Internet to look for information on their disease. Considering this, and because better-informed patients follow more precisely the indications given by the physician, medical staff should collaborate in the development of high-quality sites for the patient to have additional sources of information.

Rare true-positive isolated SSEP loss with preservation of MEPs response during scoliosis correction.

STUDY DESIGN: Case report. OBJECTIVE: To report a case of a true-positive isolated somatosensory evoked potential (SSEP) loss with preservation of motor evoked potential (MEP) response during scoliosis correction. SUMMARY OF BACKGROUND DATA: Combined intraoperative monitoring uses SSEPs and MEPs to decrease the probability of observing false-negative events. In combination, SSEPs and MEPs have become a standard of care for spinal deformity surgery. However, literature review reveals several cases of false-negative response with combined SSEPs and MEPs, raising the contention that intraoperative monitoring does not reliably identify all isolated selective spinal cord dysfunction. METHODS: A 15-year-old female patient with a 65° right thoracic adolescent idiopathic scoliosis underwent correction and posterior spinal fusion with segmental pedicle screw instrumentation. After capture and derotation of the left concave rod, left-sided irreversible SSEP loss occurred whereas MEPs remained unchanged. After excluding systemic factors, anesthetic causes, or technical fault, deformity correction was released and instrumentation removed. No cortical breach was reported during pedicle screw removal. RESULTS: Postoperatively, no clinical sensory or motor deficit was present; computed tomography demonstrated a burst left pedicle at T10 with the medial pedicle wall fragment in direct contact with the dorsal spinal cord. Magnetic resonance imaging excluded cord edema or other evidence of injury. Three days after surgery, intraoperative monitoring showed delayed latencies and amplitudes of the left SSEP. An uneventful reinsertion of instrumentation and correction excluding the left T10 pedicle screw was performed 7 days after the initial surgery. CONCLUSION: This case report provides evidence of selective posterior spinal cord dysfunction with sparing of the anterior columns immediately after a correction maneuver and emphasizes the importance of simultaneous SSEP and MEP monitoring. To the authors' knowledge, there is no previous report of a true-positive isolated SSEP loss with preservation of MEP response during scoliosis correction. LEVEL OF EVIDENCE: N/A.

IL2/IL21 region polymorphism influences response to rituximab in systemic lupus erythematosus patients.

To determine whether the IL2/IL21 region, a general autoimmunity locus, contributes to the observed variation in response to rituximab in patients with systemic lupus erythematosus as well as to analyze its influence in a cohort including other autoimmune diseases. rs6822844 G/T polymorphism at the IL2-IL21 region was analyzed by TaqMan assay in 84 systemic lupus erythematosus (SLE) and 60 different systemic autoimmune diseases Spanish patients receiving rituximab. Six months after the first infusion patients were classified, according to the EULAR criteria, as good responders, partial responders and non-responders. A statistically significant difference was observed in GG genotype frequency between responder (total and partial response) (83.56%) and non-responder (45.45%) SLE patients (p=0.010, odds ratio (OR)=6.10 [1.28-29.06]). No association with the response was evident in the group of patients with autoimmune diseases other than lupus. Furthermore, when both groups of patients were pooled in a meta-analysis, a reduced statistical significance of the association was observed (p=0.024, OR=3.53 [1.06-11.64]). Our results show for a first time that IL2-IL21 region seems to play a role in the response to rituximab in SLE patients but not in other autoimmune diseases.

Association of the FCGR3A-158F/V gene polymorphism with the response to rituximab treatment in Spanish systemic autoimmune disease patients.

Rituximab is being used as treatment for systemic autoimmune diseases. The objective of this study was to determine whether the genetic variant in the Fc gamma-receptor III a (FCGR3A) gene, 158F/V, contributes to the observed variation in response to rituximab in patients with systemic autoimmune diseases. DNA samples from 132 Spanish patients with different systemic autoimmune diseases receiving rituximab were genotyped for FCGR3A-158F/V (rs396991) gene polymorphism using the TaqMan(®) allelic discrimination technology. Six months after infusion with rituximab we evaluated the response to the drug: 61% of the patients showed a complete response, partial 27% and 12% did not respond to the treatment. A statistically significant difference was observed in V allele frequency between responder (38%) and nonresponder (16%) patients (p=0.01; odds ratio [OR]=3.24, 95% confidence interval [CI] 1.17-11.1). Rituximab was also more effective in V allele carriers (94%) than in homozygous FF patients (81%): p=0.02; OR=3.96, 95% CI 1.10-17.68. These results suggest that FCGR3A-158F/V (rs396991) gene polymorphism play a role in the response to rituximab in autoimmune diseases. Validation of these findings in independent cohorts is warranted.

Livedo racemosa como signo de presentación de un síndrome antifosfolípido secundario a lupus eritematoso sistémico / Livedo racemosa as a presenting sign of antiphospholipid syndrome secondary to systemic lupus erythematosus

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