Ivy sign: a diagnostic and prognostic biomarker for pediatric moyamoya

OBJECTIVE Ivy sign is a radiographic finding on FLAIR MRI sequences and is associated with slow cortical blood flow in moyamoya. Limited data exist on the utility of the ivy sign as a diagnostic and prognostic tool in pediatric patients, particularly outside of Asian populations. The authors aimed to investigate a modified grading scale with which to characterize the prevalence and extent of the ivy sign in children with moyamoya and evaluate its efficacy as a biomarker in predicting postoperative outcomes, including stroke risk. METHODS Pre- and postoperative clinical and radiographic data of all pediatric patients (21 years of age or younger) who underwent surgery for moyamoya disease or moyamoya syndrome at two major tertiary referral centers in the US and Israel, between July 2009 and August 2019, were retrospectively reviewed. Ivy sign scores were correlated to Suzuki stage, Matsushima grade, and postoperative stroke rate to quantify the diagnostic and prognostic utility of ivy sign. RESULTS A total of 171 hemispheres in 107 patients were included. The median age at the time of surgery was 9 years (range 3 months–21 years). The ivy sign was most frequently encountered in association with Suzuki stage III or IV disease in all vascular territories, including the anterior cerebral artery (53.7%), middle cerebral artery (56.3%), and posterior cerebral artery (47.5%) territories. Following surgical revascularization, 85% of hemispheres with Matsushima grade A demonstrated a concomitant, statistically significant reduction in ivy sign scores (OR 5.3, 95% CI 1.4–20.0; p = 0.013). Postoperatively, revascularized hemispheres that exhibited ivy sign score decreases had significantly lower rates of postoperative stroke (3.4%) compared with hemispheres that demonstrated no reversal of the ivy sign (16.1%) (OR 5.5, 95% CI 1.5–21.0; p = 0.008). CONCLUSIONS This is the largest study to date that focuses on the role of the ivy sign in pediatric moyamoya. These data demonstrate that the ivy sign was present in approximately half the pediatric patients with moyamoya with Suzuki stage III or IV disease, when blood flow was most unstable. The authors found that reversal of the ivy sign provided both radiographic and clinical utility as a prognostic biomarker postoperatively, given the statistically significant association with both better Matsushima grades and a fivefold reduction in postoperative stroke rates. These findings can help inform clinical decision-making, and they have particular value in the pediatric population, as the ability to minimize additional radiographic evaluations and tailor radiographic surveillance is requisite.

2014 ◽  
Vol 2 (2) ◽  
pp. 104-109 ◽  
Author(s):  
Ozgur Dede ◽  
Patrick Bosch ◽  
Austin J. Bowles ◽  
William Timothy Ward ◽  
James W. Roach

Neurosurgery ◽  
2008 ◽  
Vol 63 (suppl_3) ◽  
pp. A54-A68 ◽  
Author(s):  
Justin S. Smith ◽  
Christopher I. Shaffrey ◽  
Mark F. Abel ◽  
Christopher P. Ames

ABSTRACT OBJECTIVE To review the concepts involved in the decision-making process for management of pediatric patients with spinal deformity. METHODS The literature was reviewed in reference to pediatric deformity evaluation and management. RESULTS Pediatric spinal deformity includes a broad range of disorders with differing causes, natural histories, and treatments. Appropriate categorization of pediatric deformities is an important first step in the clinical decision-making process. An understanding of both nonoperative and operative treatment modalities and their indications is requisite to providing treatment for pediatric patients with spinal deformity. The primary nonoperative treatment modalities include bracing and casting, and the primary operative treatments include nonfusion instrumentation and fusion with or without instrumentation. In this article, we provide a review of pediatric spinal deformity classification and an overview of general treatment principles. CONCLUSION The decision-making process in pediatric deformity begins with appropriate diagnosis and classification of the deformity. Treatment decisions, both nonoperative and operative, are often predicated on the basis of the age of the patient and the natural history of the disorder.


2021 ◽  
Author(s):  
Bin Xie ◽  
Shiyong Tan ◽  
Chao Li ◽  
Junyang Liang

Abstract Purpose: Osteosarcoma is one of the most prevalent malignancies, and despite significant advances in its treatment, patient prognosis remains poor and survival rates are low. It is undoubtedly important to explore the possible reasons for the low survival rates of patients and to reveal the differences.Methods and Results: We obtained RNA-Seq (HT seq) and clinical characteristics of osteosarcoma patients from the TCGA database and divided them into survival group and death group. We defined the differentially expressed genes (DEGs) between the two groups as death-related genes (DRGs) and used them to construct a prognostic signature for overall survival of patients with osteosarcoma. The results of the validation demonstrated satisfactory accuracy and predictive prognostic value of the model. In addition, we performed a series of bioinformatic analyses that identified two key genes and the regulatory networks they constituted that may play a role in the progression of osteosarcoma.Conclusion: Our DRGs signature represents a novel and clinically useful prognostic biomarker for patients with osteosarcoma, helping to aid clinical decision-making.


2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S273-S274
Author(s):  
Lorne W Walker ◽  
Andrew J Nowalk ◽  
Shyam Visweswaran

Abstract Background Deciding whether to attempt salvage of an infected central venous catheter (CVC) can be challenging. While line removal is the definitive treatment for central-line associated bloodstream infection (CLABSI), salvage may be attempted with systemic antibiotics and antibiotic lock therapy (ALT). Weighing risk and benefit of CVC salvage is limited by uncertainty in the future viability of salvaged CVCs. If a CVC is likely to require subsequent removal (e.g., due to recurrent infection) salvage may not be beneficial, whereas discarding a viable CVC is also not desirable. Here we describe a machine learning approach to predicting outcomes in CVC salvage. Methods Episodes of pediatric CLABSI cleared with ALT were identified by retrospective record review between January 1, 2008 and December 31, 2018 and were defined by a single positive central blood culture of a known pathogen or two matching cultures of a possible contaminant. Clearance was defined as 48-hours of negative cultures and relapse was defined as a matching positive blood culture after clearance. Predictive models [logistic regression (LR), random forest (RF), support vector machine (SVM) and an ensemble combining the three] were used to predict recurrence-free CVC retention (RFCR) at various time points using a training and test set approach. Results Overall, 712 instances CLABSI cleared with ALT were identified. Demographic and microbiological data are summarized in Tables 1 and 2. Few (8%) instances recurred in the first 28 days. 58% recurred at any time within the study period. Rates of RFCR were 75%, 43%, 22% and 10% at 28, 91, 182 and 365 days. Machine learning (ML) models varied in their ability to predict RFCR (Table 3). RF models performed best overall, although no model performed well at 91 days. Conclusion ML models provide an opportunity to augment clinical decision making by learning patterns from data. In this case, estimating the likelihood of useful line retention in the future could help guide informed decisions on salvage vs. removal of infected CVCs. Limitations include the heterogeneity of clinical data and the use of an outcome capturing both clinical decision making (line removal) and infection recurrence. With further model development and prospective validation, practical machine learning models may prove useful to clinicians. Disclosures All authors: No reported disclosures.


2008 ◽  
Vol 4 (1) ◽  
pp. 9-14 ◽  
Author(s):  
Patti M. Johnstone

Abstract Audiologists are seeking evidence to guide clinical decision-making regarding the tests used to diagnose auditory processing disorder (APD) in children. Novel research paradigms using a population health framework hold promise in helping clinicians understand the prevalence, profile, and social underpinnings of APD in the general pediatric population. In addition, tests employing novel stimuli like speech-evoked auditory potentials or spatial and vocal cues may lead the way toward a new and more reliable APD test battery.


2010 ◽  
Vol 29 (1) ◽  
pp. E2 ◽  
Author(s):  
William R. Stetler ◽  
Paul Park ◽  
Stephen Sullivan

Object Tethering of the spinal cord has been a recognized cause of neurological symptoms in pediatric patients and is increasingly being recognized as a cause of symptoms in adults as well. The pathophysiology surrounding spinal cord tethering has begun to be understood in the pediatric population but is still unclear in adult patients. Methods Using a PubMed database literature search, the authors reviewed the pathology and pathophysiology surrounding the tethered spinal cord, focusing particularly on the pathophysiology of adult tethered cord syndrome (TCS). Results Experimental data obtained in pediatric patients at surgery and in animal models indicate that spinal cord tethering causes a reduction in spinal cord blood flow and dysfunction of neuronal mitochondrial terminal oxidase. Retrospective analyses of patients undergoing surgery for adult TCS show that many adults developed symptoms following an event that could stretch the spinal cord, while others did not. Many patients also were found to have structural lesions in addition to a tethered spinal cord at diagnosis. Conclusions Both adult and pediatric TCSs are likely the result of a relative lack of blood flow to the spinal cord, causing dysfunction in mitochondrial oxidative phosphorylation. The likely reason the syndrome present later and differently in adults is that a secondary threshold of tension or a cumulative effect of repetitive, transient tension is placed on the cord before symptoms are recognized.


2020 ◽  
Vol 58 (4) ◽  
pp. 605-617 ◽  
Author(s):  
Houman Tahmasebi ◽  
Shervin Asgari ◽  
Alexandra Hall ◽  
Victoria Higgins ◽  
Ashfia Chowdhury ◽  
...  

AbstractBackgroundAccurate pediatric reference intervals (RIs) for laboratory tests determined in a healthy pediatric population are essential for correct laboratory test interpretation and clinical decision-making. In pediatrics, RIs require partitioning by age and/or sex; however, the need for partitioning based on ethnicity is unclear. Here, we assessed the influence of ethnicity on biomarker concentrations in the Canadian Laboratory Initiative on Pediatric Reference Intervals (CALIPER) cohort of healthy children and adolescents and compared the results with the National Health and Nutrition Examination Survey (NHANES).MethodsA total of 52 biomarkers were measured in a multiethnic population of 846–1179 healthy children (aged 5 to <19 years) upon informed consent. Biomarker concentrations were retrospectively compared between four major ethnic groups (i.e. Black, Caucasian, East Asian, and South Asian, determined by parental ethnicity). Retrospective results were verified prospectively using an additional 500 healthy pediatric samples with equal sample size across ethnicities. Ethnic-specific differences were assessed based on statistical significance and biological and analytical variations. Appropriate age-, sex-, and ethnic-specific RIs were calculated.ResultsEthnic-specific differences were not observed for 34 biomarkers examined in the retrospective analysis, while 18 demonstrated statistically significant ethnic differences. Among these, seven analytes demonstrated ethnic-specific differences in the prospective analysis: vitamin D, amylase, ferritin, follicle-stimulating hormone (FSH), immunoglobulin A (IgA), immunoglobulin G (IgG), and immunoglobulin M (IgM). Analysis of select NHANES data confirmed CALIPER findings.ConclusionsThis is the first comprehensive Canadian pediatric study examining ethnic-specific differences in common biomarkers. While the majority of biomarkers did not require ethnic partitioning, ethnic-specific RIs were established for seven biomarkers showing marked differences. Further studies in other populations are needed to confirm our findings.


2016 ◽  
Vol 3 (suppl_1) ◽  
Author(s):  
David Jacobs ◽  
Maya Holsen ◽  
Shirley Chen ◽  
Nicholas Fusco ◽  
Amanda Hassinger

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