Did you mean: scheuermann s disease
-
Journal of Neuroinflammation Jun 2024Pediatric acute transverse myelitis (ATM) accounts for 20-30% of children presenting with a first acquired demyelinating syndrome (ADS) and may be the first clinical...
BACKGROUND
Pediatric acute transverse myelitis (ATM) accounts for 20-30% of children presenting with a first acquired demyelinating syndrome (ADS) and may be the first clinical presentation of a relapsing ADS such as multiple sclerosis (MS). B cells have been strongly implicated in the pathogenesis of adult MS. However, little is known about B cells in pediatric MS, and even less so in pediatric ATM. Our lab previously showed that plasmablasts (PB), the earliest B cell subtype producing antibody, are expanded in adult ATM, and that these PBs produce self-reactive antibodies that target neurons. The goal of this study was to examine PB frequency and phenotype, immunoglobulin selection, and B cell receptor reactivity in pediatric patients presenting with ATM to gain insight to B cell involvement in disease.
METHODS
We compared the PB frequency and phenotype of 5 pediatric ATM patients and 10 pediatric healthy controls (HC) and compared them to previously reported adult ATM patients using cytometric data. We purified bulk IgG from the plasma samples and cloned 20 recombinant human antibodies (rhAbs) from individual PBs isolated from the blood. Plasma-derived IgG and rhAb autoreactivity was measured by mean fluorescence intensity (MFI) in neurons and astrocytes of murine brain or spinal cord and primary human astrocytes. We determined the potential impact of these rhAbs on astrocyte health by measuring stress and apoptotic response.
RESULTS
We found that pediatric ATM patients had a reduced frequency of peripheral blood PB. Serum IgG autoreactivity to neurons in EAE spinal cord was similar in the pediatric ATM patients and HC. However, serum IgG autoreactivity to astrocytes in EAE spinal cord was reduced in pediatric ATM patients compared to pediatric HC. Astrocyte-binding strength of rhAbs cloned from PBs was dependent on somatic hypermutation accumulation in the pediatric ATM cohort, but not HC. A similar observation in predilection for astrocyte binding over neuron binding of individual antibodies cloned from PBs was made in EAE brain tissue. Finally, exposure of human primary astrocytes to these astrocyte-binding antibodies increased astrocytic stress but did not lead to apoptosis.
CONCLUSIONS
Discordance in humoral immune responses to astrocytes may distinguish pediatric ATM from HC.
Topics: Humans; Myelitis, Transverse; Animals; Female; Astrocytes; Child; Mice; Male; Adolescent; Plasma Cells; Autoantibodies; Mice, Inbred C57BL; Cells, Cultured; Child, Preschool; Immunoglobulin G; Spinal Cord
PubMed: 38915059
DOI: 10.1186/s12974-024-03127-2 -
BMC Medical Genomics Jun 2024Respiratory Syncytial Virus (RSV) disease in young children ranges from mild cold symptoms to severe symptoms that require hospitalization and sometimes result in death....
BACKGROUND
Respiratory Syncytial Virus (RSV) disease in young children ranges from mild cold symptoms to severe symptoms that require hospitalization and sometimes result in death. Studies have shown a statistical association between RSV subtype or phylogenic lineage and RSV disease severity, although these results have been inconsistent. Associations between variation within RSV gene coding regions or residues and RSV disease severity has been largely unexplored.
METHODS
Nasal swabs from children (< 8 months-old) infected with RSV in Rochester, NY between 1977-1998 clinically presenting with either mild or severe disease during their first cold-season were used. Whole-genome RSV sequences were obtained using overlapping PCR and next-generation sequencing. Both whole-genome phylogenetic and non-phylogenetic statistical approaches were performed to associate RSV genotype with disease severity.
RESULTS
The RSVB subtype was statistically associated with disease severity. A significant association between phylogenetic clustering of mild/severe traits and disease severity was also found. GA1 clade sequences were associated with severe disease while GB1 was significantly associated with mild disease. Both G and M2-2 gene variation was significantly associated with disease severity. We identified 16 residues in the G gene and 3 in the M2-2 RSV gene associated with disease severity.
CONCLUSION
These results suggest that phylogenetic lineage and the genetic variability in G or M2-2 genes of RSV may contribute to disease severity in young children undergoing their first infection.
Topics: Humans; Respiratory Syncytial Virus Infections; Phylogeny; Genetic Variation; Severity of Illness Index; Infant; Respiratory Syncytial Virus, Human; Male; Genotype; Female; Genome, Viral
PubMed: 38898440
DOI: 10.1186/s12920-024-01930-7 -
BioRxiv : the Preprint Server For... Apr 2024The use of single-cell transcriptomic technologies that quantitively describe cell transcriptional phenotypes using single cell/nucleus RNA sequencing (scRNA-seq) is...
The use of single-cell transcriptomic technologies that quantitively describe cell transcriptional phenotypes using single cell/nucleus RNA sequencing (scRNA-seq) is revolutionizing our understanding of cell biology, leading to new insights in cell type identification, disease mechanisms, and drug development. The tremendous growth in scRNA-seq data has posed new challenges in efficiently characterizing data-driven cell types and identifying quantifiable marker genes for cell type classification. The use of machine learning and explainable artificial intelligence has emerged as an effective approach to study large-scale scRNA-seq data. NS-Forest is a random forest machine learning-based algorithm that aims to provide a scalable data-driven solution to identify minimum combinations of necessary and sufficient marker genes that capture cell type identity with maximum classification accuracy. Here, we describe the latest version, NS-Forest version 4.0 and its companion Python package (https://github.com/JCVenterInstitute/NSForest), with several enhancements, to select marker gene combinations that exhibit selective expression patterns among closely related cell types and more efficiently perform marker gene selection for large-scale scRNA-seq data atlases with millions of cells. By modularizing the final decision tree step, NS-Forest v4.0 can be used to compare the performance of user-defined marker genes with the NS-Forest computationally-derived marker genes based on the decision tree classifiers. To quantify how well the identified markers exhibit the desired pattern of being exclusively expressed at high levels within their target cell types, we introduce the On-Target Fraction metric that ranges from 0 to1, with a metric of 1 given to markers that are only expressed within their target cell types and not in cells of any other cell types. We have applied NS-Forest v4.0 on scRNA-seq datasets from three human organs, including the brain, kidney, and lung. We observe that NS-Forest v4.0 outperforms previous versions on its ability to identify markers with higher On-Target Fraction values for closely related cell types and outperforms other marker gene selection approaches on the classification performance with significantly higher F-beta scores.
PubMed: 38712147
DOI: 10.1101/2024.04.22.590194 -
Viruses Apr 2024This study investigates the roles of T, B, and Natural Killer (NK) cells in the pathogenesis of severe COVID-19, utilizing mouse-adapted SARS-CoV-2-MA30 (MA30). To...
This study investigates the roles of T, B, and Natural Killer (NK) cells in the pathogenesis of severe COVID-19, utilizing mouse-adapted SARS-CoV-2-MA30 (MA30). To evaluate this MA30 mouse model, we characterized MA30-infected C57BL/6 mice (B6) and compared them with SARS-CoV-2-WA1 (an original SARS-CoV-2 strain) infected K18-human ACE2 () mice. We found that the infected B6 mice developed severe peribronchial inflammation and rapid severe pulmonary edema, but less lung interstitial inflammation than the infected mice. These pathological findings recapitulate some pathological changes seen in severe COVID-19 patients. Using this MA30-infected mouse model, we further demonstrate that T and/or B cells are essential in mounting an effective immune response against SARS-CoV-2. This was evident as showed heightened vulnerability to infection and inhibited viral clearance. Conversely, the depletion of NK cells did not significantly alter the disease course in mice, underscoring the minimal role of NK cells in the acute phase of MA30-induced disease. Together, our results indicate that T and/or B cells, but not NK cells, mitigate MA30-induced disease in mice and the infected mouse model can be used for dissecting the pathogenesis and immunology of severe COVID-19.
Topics: Animals; Killer Cells, Natural; COVID-19; Mice; SARS-CoV-2; Disease Models, Animal; Mice, Inbred C57BL; DNA-Binding Proteins; Mice, Knockout; Humans; Lung; Angiotensin-Converting Enzyme 2; B-Lymphocytes; Female; T-Lymphocytes
PubMed: 38675952
DOI: 10.3390/v16040611 -
Turkish Neurosurgery 2024To evaluate the quality of life of patients with postural kyphosis (PK) compared with those adolescent idiopathic scoliosis (AIS) and Scheuermann Kyphosis (SK). (Comparative Study)
Comparative Study
AIM
To evaluate the quality of life of patients with postural kyphosis (PK) compared with those adolescent idiopathic scoliosis (AIS) and Scheuermann Kyphosis (SK).
MATERIAL AND METHODS
Health-related quality of life (HRQoL) of adolescents with PK who were admitted to our clinic between January 2020 and June 2022 was compared with that of patients with AIS and SK who were admitted during the same period by using the Scoliosis Research Society-22 (SRS-22) questionnaire. All patients were asked to complete the SRS-22 questionnaire before the radiological evaluation. In the radiological evaluation, the sagittal and coronal deformities of the patients were measured.
RESULTS
In total, 126 patients with PK were compared with age and sex-matched AIS patients and 42 SK patients. The mean SRS-22 function score of the PK group was 4.72 ± 0.3 while it was 4.38 ± 0.6 (p=0.015) in SK patients and it was 4.34 ± 0.6 (p < 0.001) in AIS patients. The mean SRS-22 pain scores of PK patients was 4.18 ± 0.7. The mean pain score was 3.68 ± 0.8 (p=0.033) in the SK group and 3.6 ± 0.8 (p=0.010) in the AIS group. Adolescents with PK perceived less pain than those with AIS or SK. The scores for the other domains of SRS-22 revealed no differences.
CONCLUSION
HRQoL of patients with PK is reduced, similar to that of patients with common structural spine deformities. Recognizing the effects of PK on the HRQoL in adolescents can help physicians to treat these patients.
Topics: Humans; Quality of Life; Adolescent; Female; Male; Kyphosis; Cross-Sectional Studies; Scoliosis; Surveys and Questionnaires; Scheuermann Disease; Child
PubMed: 38650564
DOI: 10.5137/1019-5149.JTN.43440-23.2 -
SICOT-J 2024Scheuermann's disease is a diagnosis of hyperkyphosis commonly encountered in pediatric patients. Studies in animal models suggest an association with vitamin D...
INTRODUCTION
Scheuermann's disease is a diagnosis of hyperkyphosis commonly encountered in pediatric patients. Studies in animal models suggest an association with vitamin D deficiency, however, extensive studies have not been performed in humans. This study analyzes the role of vitamin D deficiency on unfavorable results in patients with Scheuermann's disease.
METHODS
The TriNetX database was utilized to perform a retrospective analysis. Patients in the United States aged 0-18 years with Scheuermann's disease were identified using International Classification of Diseases, Tenth Revision (ICD-10) codes and categorized into those with and without a diagnosis of vitamin D deficiency. Comparison of patient groups depending on age, sex, ethnic origin, prior diagnosis of fibromyalgia, anxiety disorder, myositis, and major depressive disorder. Statistical analysis was conducted to identify the association between vitamin D levels and unfavorable results including pain, depression, suicide attempt, emergency department (ED) consult, hospitalization, and procedures on the spine or spinal cord.
RESULTS
In total, 11,277 patients were identified, 39% of whom had a concurrent diagnosis of scoliosis. A total of 1,024 (9.08%) were deficient in vitamin D. Patients with vitamin D deficiency had greater odds of pain (P < 0.0001), depression (P < 0.0001), suicide attempt (P = 0.0021), ED visits (P = 0.0246), and hospital admission (P < 0.0015). Conversely, patients with vitamin D deficiency had decreased odds of surgery on the spine or spinal cord (P = 0.0009).
CONCLUSION
Vitamin D deficiency is associated with an elevated risk of pain, depression, suicide attempts, ED visits, and hospitalization. Our analysis highlights the need for more research to study the effect of vitamin D on Scheuermann's disease.
LEVEL OF EVIDENCE
Level III, Prognostic.
PubMed: 38568088
DOI: 10.1051/sicotj/2024010 -
Turkish Neurosurgery 2024To evaluate the occurrence of proximal junctional kyphosis (PJK) as well as both the clinical and radiologic outcomes of patients who underwent surgery for Scheuermann?s...
AIM
To evaluate the occurrence of proximal junctional kyphosis (PJK) as well as both the clinical and radiologic outcomes of patients who underwent surgery for Scheuermann?s Kyphosis (SK) using either exclusively pedicle screws or a combination of proximal hooks and pedicle screws constructs.
MATERIAL AND METHODS
Surgically treated 37 patients with the diagnosis of SK were evaluated retrospectively. The patients were divided into two groups based on the type of instrumentation employed. The first group contained 22 patients with only pedicle screws (PP) while the second group consisted of 15 patients with mixed constructs that were proximal hooks and pedicle screws (HP) at the rest of the levels. The clinical and radiological data were compared in patients who were followed up for a minimum of 2 years.
RESULTS
The average duration of follow-up for the PP group was approximately 94.7 ± 53.1 months, whereas the HP group had an average follow-up period of around 103 ± 64.4 months. After conducting the analyses, no statistically significant findings were identified in the measurements taken for the SRS-22 scores in preoperative, postoperative, and the most recent follow-up radiographs (p > 0.05). It is worth noting that among patients who exclusively utilized pedicle screws, both the proximal (p=0.045) and distal (p=0.030) junctional kyphosis angles experienced more pronounced increases compared to hybrid structures.
CONCLUSION
While no notable distinction was observed between the two groups, patients with pedicle screws fixation had a higher PJK angle. Conversely, the use of hooks at the upper end seems to be a preventive measure against the development of PJK.
Topics: Humans; Male; Female; Retrospective Studies; Pedicle Screws; Treatment Outcome; Scheuermann Disease; Spinal Fusion; Adolescent; Kyphosis; Adult; Follow-Up Studies; Young Adult; Postoperative Complications; Child; Middle Aged
PubMed: 38497580
DOI: 10.5137/1019-5149.JTN.45194-23.2 -
Journal of the American Academy of... Mar 2024Scheuermann kyphosis can be treated surgically to restore proper sagittal alignment. Thoracic curves >70° are typically indicated for surgical intervention. However,...
Scheuermann kyphosis can be treated surgically to restore proper sagittal alignment. Thoracic curves >70° are typically indicated for surgical intervention. However, patients who have reached their natural limit of compensatory lumbar hyperlordosis are at risk of accelerated degeneration. This can be determined by comparing lumbar lordosis on standing neutral radiographs and supine extension radiographs. Minimal additional lordosis in extension compared with neutral, abutment of the spinous processes, or greater lumbar lordosis standing than with attempted extension suggest the patient is maximally compensated. We present a case of an adolescent boy with Scheuermann kyphosis who had reached the limit of his hyperlordosis compensation reserve. He subsequently underwent a T4 to L2 posterior spinal fusion with T7 to T11 Ponte Smith-Petersen grade two osteotomies. He tolerated the procedure well with no intraoperative complications or neuromonitoring changes. The patient has continued to do well and progressed to normal activity at 5-month follow-up.
Topics: Adolescent; Male; Animals; Humans; Scheuermann Disease; Lordosis; Intraoperative Complications; Osteotomy; Spinal Fusion
PubMed: 38441155
DOI: 10.5435/JAAOSGlobal-D-23-00187 -
Orthopaedic Surgery Apr 2024Degenerative thoracolumbar hyperkyphosis (DTH) is a disease that negatively affects individual health and requires surgical intervention, yet the ideal surgical approach...
OBJECTIVE
Degenerative thoracolumbar hyperkyphosis (DTH) is a disease that negatively affects individual health and requires surgical intervention, yet the ideal surgical approach and complications, especially distal junctional failures (DJF), remain poorly understood. This study aims to investigate DJF in DTH and to identify the risk factors for DJF so that we can improve surgical decision-making, and advance our knowledge in the field of spinal surgery to enhance patient outcomes.
METHODS
This study retrospectively reviewed 78 cases (late osteoporotic vertebral compression fracture [OVCF], 51; Scheuermann's kyphosis [SK], 17; and degenerative disc diseases [DDD], 10) who underwent corrective surgery in our institute from 2008 to 2019. Clinical outcomes were assessed using health-related quality of life (HRQOL) measures, including the visual analogue scale (VAS) scores for back and leg pain, the Oswestry disability index (ODI), and the Japanese Orthopaedic Association (JOA) scoring system. Multiple radiographic parameters, such as global kyphosis (GK) and thoracolumbar kyphosis (TLK), were assessed to determine radiographic outcomes. Multivariate logistic regression analysis was employed to identify the risk factors associated with DJF.
RESULTS
HRQOL improved, and GK, TLK decreased at the final follow-up, with a correction rate of 67.7% and 68.5%, respectively. DJF was found in 13 of 78 cases (16.7%), two cases had wedging in the disc (L3-4) below the instrumentation, one case had a fracture of the lowest instrumented vertebrae (LIV), one case had osteoporotic fracture below the fixation, nine cases had pull-out or loosening of the screws at the LIV and three cases (23.1%) required revision surgery. The DJF group had older age, lower computed tomography Hounsfield unit (CT HU), longer follow-up, more blood loss, greater preoperative sagittal vertical axis (SVA), and poorer postoperative JOA and VAS scores (back). The change in TLK level was larger in the non-DJF group. Post-sagittal stable vertebrae (SSV) moved cranially compared with pre-SSV.
CONCLUSION
Age, CT HU, length of follow-up, estimated blood loss, and preoperative SVA were independent risk factors for DJF. We recommend fixation of the two vertebrae below the apex vertebrae for DTH to minimize surgical trauma.
Topics: Humans; Quality of Life; Retrospective Studies; Fractures, Compression; Treatment Outcome; Thoracic Vertebrae; Spinal Fractures; Kyphosis; Osteoporotic Fractures; Lumbar Vertebrae; Spinal Fusion
PubMed: 38384146
DOI: 10.1111/os.13973 -
Asian Spine Journal Feb 2024Level 3 retrospective cohort case-control study.
STUDY DESIGN
Level 3 retrospective cohort case-control study.
PURPOSE
This study aimed to investigate the risk factors for distal junctional kyphosis (DJK) caused by osteoporotic vertebral fractures following spinal reconstruction surgery, with a focus on the sagittal stable vertebra.
OVERVIEW OF LITERATURE
Despite the rarity of reports on DJK in this setting, DJK was reported to reduce when the lower instrumented vertebra (LIV) was extended to the sagittal stable vertebra in the posterior corrective fixation for Scheuermann's disease.
METHODS
This study included 46 patients who underwent spinal reconstruction surgery for thoracolumbar osteoporotic vertebral fractures and kyphosis and were followed up for 1 year postoperatively. DJK was defined as an advanced kyphosis angle >10° between the LIV and one lower vertebra. The patients were divided into groups with and without DJK. The risk factors of the two groups, such as patient background, surgery-related factors, radiographic parameters, and clinical outcomes, were analyzed.
RESULTS
The DJK and non-DJK groups included 14 and 32 patients, respectively, without significant differences in patient background. Those with instability in the distal adjacent LIV disc had a significantly higher risk of DJK occurrence (28.6% vs. 3.2%, p=0.027). DJK occurrence significantly increased in those with the sagittal stable vertebra not included in the fixation range (57.1% vs. 18.8%, p=0.020). Other preoperative radiographic parameters were not significantly different. Instability in the distal adjacent LIV disc (adjusted odds ratio, 14.50; p=0.029) and the exclusion of the sagittal stable vertebra from the fixation range (adjusted odds ratio, 5.29; p=0.020) were significant risk factors for DJK occurrence.
CONCLUSIONS
Regarding spinal reconstruction surgery in patients with osteoporotic vertebral fractures, instability in the distal adjacent LIV disc and the exclusion of the sagittal stable vertebra from the fixation range were risk factors for DJK occurrence in the short term.
PubMed: 38379382
DOI: 10.31616/asj.2023.0174