scholarly journals Risk Factors for Bullying Victimization in Children with Neurofibromatosis Type 1 (NF1)

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 145
Author(s):  
Peter L. Stavinoha ◽  
Cody Solesbee ◽  
Susan M. Swearer ◽  
Steven Svoboda ◽  
Laura J. Klesse ◽  
...  
Keyword(s):  
Risk Factors ◽  
Neurofibromatosis Type 1 ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Neurofibromatosis Type ◽  
Parent Report ◽  
Self Report ◽  
Bullying Victimization ◽  
Adhd Symptoms

Neurofibromatosis type 1 (NF1) is an autosomal disorder associated with numerous physical stigmata. Children with NF1 are at known risk for attention-deficit/hyperactivity disorder (ADHD), academic struggles, and significant social difficulties and adverse social outcomes, including bullying victimization. The primary aim of this study was to identify risk factors associated with bullying victimization in children with NF1 to better inform clinicians regarding targets for prevention and clinical intervention. Children and a parent completed questionnaires assessing the bully victim status, and parents completed a measure of ADHD symptoms. Analyses were completed separately for parent-reported victimization of the child and the child’s self-report of victimization. According to the parent report, results suggest ADHD symptoms are a significant risk factor for these children being a target of bullying. Findings for academic disability were not conclusive, nor were findings related to having a parent with NF1. Findings indicate the need for further research into possible risk factors for social victimization in children with NF1. Results provide preliminary evidence that may guide clinicians working with children with NF1 and their parents in identifying higher-risk profiles that may warrant earlier and more intensive intervention to mitigate later risk for bullying victimization.

scholarly journals Duodenal major papilla morphology can affect biliary cannulation and complications during ERCP, an observational study

2020 ◽  
Author(s):  
Po-Han Chen ◽  
Chun-Fang Tung ◽  
Yen-Chung Peng ◽  
Hong-Zen Yeh ◽  
Chi-Sen Chang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Risk Factor ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Biliary Cannulation ◽  
Small Papilla ◽  
Type 3

Abstract BackgroundWe investigated whether duodenal major papilla morphology could be a risk factor for failure of selective biliary cannulation (SBC) and post endoscopic retrograde cholangiography and pancreatography (ERCP) complications.MethodsA prospectively recorded database was reviewed retrospectively. Patients were included if they received therapeutic ERCP and had naïve major duodenal papilla. We used Haraldsson’s classification for papilla morphology, as follows: Regular (Type 1), Small (Type 2), Protruding or Pendulous (Type 3) and Creased or Ridged (Type 4). Risk factors for failing SBC and post-ERCP complications were analyzed by multivariate analysis.ResultsA total of 286 cases were included. Age, gender, indications and therapeutic procedures were not different among the four types of papillae. The failure rates of SBC with Type 3 papilla and Type 4 papilla were 11.11% and 6.25%, respectively. In the multivariate analysis, Type 2 papilla (odd ratio 7.18, p= 0.045) and Type 3 papilla (odd ratio 7.44, p= 0.016) were associated with greater SBC failure compared with Type 1 papilla. Malignant obstruction compared to stone (odds ratio 4.45, p=0.014) and age (odd ratio=1.06, p=0.010) were also risk factors for cannulation failure. Type 2 papilla was correlated with a higher rate of post-ERCP pancreatitis (20%, p=0.020) compared to the other types of papilla However, papilla morphology was not a significant risk factor for any complications in the multivariate analysis.ConclusionSmall papilla and protruding or pendulous papilla are more difficult to cannulate compared to regular papilla. Small papilla is associated with a higher rate of post-ERCP pancreatitis.

scholarly journals Effects of methylphenidate on cognition and behaviour in children with neurofibromatosis type 1: a study protocol for a randomised placebo-controlled crossover trial

BMJ Open ◽  
2018 ◽  
Vol 8 (8) ◽  
pp. e021800 ◽  
Author(s):  
Natalie A Pride ◽  
Belinda Barton ◽  
Paul Hutchins ◽  
David R Coghill ◽  
Mayuresh S Korgaonkar ◽  
...  
Keyword(s):  
Working Memory ◽  
Neurofibromatosis Type 1 ◽  
Performance Test ◽  
Spatial Working Memory ◽  
Memory Task ◽  
Neurofibromatosis Type ◽  
Fine Motor ◽  
Mouse Strains ◽  
Adhd Symptoms

IntroductionDopamine dysregulation has been identified as a key modulator of behavioural impairment in neurofibromatosis type 1 (NF1) and a potential therapeutic target. Preclinical research demonstrates reduced dopamine in the brains of genetically engineered NF1 mouse strains is associated with reduced spatial-learning and attentional dysfunction. Methylphenidate, a stimulant medication that increases dopaminergic and noradrenergic neurotransmission, rescued the behavioural and dopamine abnormalities. Although preliminary clinical trials have demonstrated that methylphenidate is effective in treating attention deficit hyperactivity disorder (ADHD) symptoms in children with NF1, its therapeutic effect on cognitive performance is unclear. The primary aim of this clinical trial is to assess the efficacy of methylphenidate for reducing attention deficits, spatial working memory impairments and ADHD symptoms in children with NF1.Methods and analysisA randomised, double-blind, placebo-controlled trial of methylphenidate with a two period crossover design. Thirty-six participants with NF1 aged 7–16 years will be randomised to one of two treatment sequences: 6 weeks of methylphenidate followed by 6 weeks of placebo or; 6 weeks of placebo followed by 6 weeks of methylphenidate. Neurocognitive and behavioural outcomes as well as neuroimaging measures will be completed at baseline and repeated at the end of each treatment condition (week 6, week 12). Primary outcome measures are omission errors on the Conners Continuous Performance Test-II (attention), between-search errors on the Spatial Working Memory task from the Cambridge Neuropsychological Test Automated Battery (spatial working memory) and the Inattentive and Hyperactivity/Impulsivity Symptom Scales on the Conners 3-Parent. Secondary outcomes will examine the effect of methylphenidate on executive functions, attention, visuospatial skills, behaviour, fine-motor skills, language, social skills and quality of life.Ethics and disseminationThis trial has hospital ethics approval and the results will be disseminated through peer-reviewed publications and international conferences.Trial registration numberACTRN12611000765921.

Rate and frequency of bullying victimization in school-age children with neurofibromatosis type 1 (NF1).

2019 ◽  
Vol 34 (6) ◽  
pp. 687-694
Author(s):  
Alice Ann Holland ◽  
Peter L. Stavinoha ◽  
Susan M. Swearer ◽  
Cody Solesbee ◽  
Sarita Patel ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
School Age Children ◽  
Bullying Victimization ◽  
School Age

INNV-04. A MULTI-INSTITUTIONAL CLINICAL AND MRI REPOSITORY OF NEUROFIBROMATOSIS TYPE 1-ASSOCIATED PERIPHERAL NERVE SHEATH TUMORS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi105-vi106
Author(s):  
Ina Ly ◽  
Angela Hirbe ◽  
Justin Jordan ◽  
Olivia Michaels ◽  
Daniel Kwon ◽  
...  
Keyword(s):  
Risk Factors ◽  
Peripheral Nerve ◽  
Clinical Data ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Histologic Diagnosis ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Abstract BACKGROUND Individuals with neurofibromatosis type 1 (NF1) frequently have peripheral nerve sheath tumors (PNST), including plexiform neurofibromas (PNF), atypical neurofibromas (ANF), and malignant peripheral nerve sheath tumors (MPNST). These tumors reflect a histologic spectrum from benign to malignant. Various clinical and MRI-based features are proposed as risk factors for MPNST development based on small single-institution studies. A major barrier to study these risk factors is collation and annotation of multi-center serial MRIs. To address this, we created a standardized database of clinical data and longitudinal MRIs from NF1-associated PNST from nine international NF1 referral centers. METHODS Clinical data from NF1 patients are collected in Research Electronic Data Capture databases housed at Massachusetts General Hospital and Washington University, including demographic information, genotype, disease course, treatment history, and survival. ANF and MPNST require histologic confirmation whereas a diagnosis of PNF can also be made based on clinical/radiographic stability. Longitudinal MRIs predating the histologic diagnosis are uploaded to a HIPAA-compliant cloud-based system. RESULTS Data from 200 patients (87 females, 113 males) with 217 tumors (75 PNF, 40 ANF, 102 MPNST) have been collected. 280 regional and 108 whole-body MRIs have been identified. Median age at the time of histologic diagnosis is 30 years (range 5-64). All tumors are histologically confirmed except for 6 PNF which remained stable over time. Median follow-up time is 32 months. Of 147 patients with available survival data, 32 (21.7%) have died from MPNST progression; estimated median overall survival is 20 months. CONCLUSIONS In this ongoing work, we have assembled one of the largest systematically annotated clinical and MRI repositories of NF1-associated PNST from pediatric and adult NF1 patients. The data will be accessible to outside researchers which will promote interdisciplinary and multi-center collaborations. Active efforts include the identification of radiomic MRI features to differentiate between PNF and MPNST.

scholarly journals Investigation of early signs of systolic and diastolic dysfunction among persons with type 1 diabetes

Open Heart ◽  
2019 ◽  
Vol 6 (2) ◽  
pp. e001020
Author(s):  
Daniel Vestberg ◽  
Magnus Carl Johansson ◽  
Anette Letho ◽  
Aldina Pivodic ◽  
Sara Hallström ◽  
...  
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Type 1 Diabetes ◽  
Diastolic Dysfunction ◽  
Systolic Dysfunction ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Early Signs ◽  
Study Population

BackgroundPersons with type 1 diabetes have a higher risk to develop heart failure than the general population, and the mechanism behind the increased risk is unclear. In epidemiological studies with hospitalisation for heart failure as endpoint HbA1c, body mass index and decreased kidney function are significant risk factors, but it is unclear how these risk factors influence the development of heart failure.MethodsIn this study, we investigated early signs of systolic and diastolic dysfunction with transthoracic echocardiography. Statistical analysis on correlation of risk factors and early signs of diastolic and systolic dysfunction was made.ResultsIn this study population of 287 persons with type 1 diabetes, 160 were men and 127 were women with a mean age of 53.8 (SD 11.6) years and a mean diabetes duration of 36.2 (SD 13.5) years. There were 23 (8.2%) persons who fulfilled the definition of systolic dysfunction (ejection fraction <50% or regional wall motion abnormalities) and 24 persons (9%) the definition for diastolic dysfunction. When comparing the groups with either systolic or diastolic dysfunction to the rest of the population, the only significant risk factor was age in both groups and previous myocardial infarction in the systolic group.ConclusionIn our study population with type 1 diabetes, we found signs of diastolic dysfunction in 9% and systolic dysfunction in 8.2%. Compared with published data from the general population, this rate is somewhat higher in a younger population. Only age was a significant risk factor in the study.

scholarly journals Duodenal major papilla morphology can affect biliary cannulation and complications during ERCP, an observational study

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Po-Han Chen ◽  
Chun-Fang Tung ◽  
Yen-Chung Peng ◽  
Hong-Zen Yeh ◽  
Chi-Sen Chang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Risk Factor ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Biliary Cannulation ◽  
Small Papilla ◽  
Type 3

Abstract Background We investigated whether duodenal major papilla morphology could be a risk factor for failure of selective biliary cannulation (SBC) and post endoscopic retrograde cholangiography and pancreatography (ERCP) complications. Methods A prospectively recorded database was reviewed retrospectively. Patients were included if they received therapeutic ERCP and had naïve major duodenal papilla. We used Haraldsson’s classification for papilla morphology, as follows: Regular (Type 1), Small (Type 2), Protruding or Pendulous (Type 3) and Creased or Ridged (Type 4). Risk factors for failing SBC and post-ERCP complications were analyzed by multivariate analysis. Results A total of 286 cases were included. Age, gender, indications and therapeutic procedures were not different among the four types of papillae. The failure rates of SBC with Type 3 papilla and Type 4 papilla were 11.11% and 6.25%, respectively. In the multivariate analysis, Type 2 papilla (odd ratio 7.18, p = 0.045) and Type 3 papilla (odd ratio 7.44, p = 0.016) were associated with greater SBC failure compared with Type 1 papilla. Malignant obstruction compared to stone (odds ratio 4.45, p = 0.014) and age (odd ratio = 1.06, p = 0.010) were also risk factors for cannulation failure. Type 2 papilla was correlated with a higher rate of post-ERCP pancreatitis (20%, p = 0.020) compared to the other types of papilla However, papilla morphology was not a significant risk factor for any complications in the multivariate analysis. Conclusion Small papilla and protruding or pendulous papilla are more difficult to cannulate compared to regular papilla. Small papilla is associated with a higher rate of post-ERCP pancreatitis.

scholarly journals Apparent Breed Predilection for Equid Herpesvirus-1-Associated Myeloencephalopathy (EHM) in a Multiple-Breed Herd

Pathogens ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 537
Author(s):  
Eva Klouth ◽  
Yury Zablotski ◽  
Lutz S. Goehring
Keyword(s):  
Risk Factors ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Herpesvirus Type ◽  
Factors Associated ◽  
Equid Herpesvirus ◽  
Herpesvirus 1 ◽  
Bayesian Logistic Regression ◽  
Age And Sex

Equid herpesvirus type 1 (EHV-1) causes several outbreaks of abortion and/or equid herpesvirus-associated myeloencephalopathy (EHM) worldwide each year. EHM is of great concern, as permanent neurological gait anomalies can leave a horse unfit for future use. The study assesses the risk factors associated with the occurrence of EHM. During an unmitigated outbreak, 141 adult horses/ponies of several distinct breeds were evaluated—using multiple Bayesian logistic regression calculating the odds ratios for breed, age, and sex. In total, 33 of the 141 horses showed signs of EHM. Fjord horses and warmblood horses were overrepresented among those developing EHM. The pony breeds, Welsh and Shetland ponies, were underrepresented. In addition, age and sex were not associated with the risk for EHM. The main limitation was that it was a retrospective analysis with some flaws of documentation. It can be concluded that breed was a significant risk factor for developing EHM during this outbreak.

scholarly journals Duodenal major papilla morphology can affect biliary cannulation and complications during ERCP, an observational study

2020 ◽  
Author(s):  
Po-Han Chen ◽  
Chun-Fang Tung ◽  
Yen-Chung Peng ◽  
Hong-Zen Yeh ◽  
Chi-Sen Chang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Risk Factor ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Biliary Cannulation ◽  
Small Papilla ◽  
Type 3

Abstract Introduction We investigated whether duodenal major papilla morphology could be a risk factor for failure of selective biliary cannulation (SBC) and post endoscopic retrograde cholangiography and pancreatography (ERCP) complications. Method A prospectively recorded database was reviewed retrospectively. Patients were included if they received therapeutic ERCP and had naïve major duodenal papilla. We used Haraldsson’s classification for papilla morphology, as follows: Regular (Type 1), Small (Type 2), Protruding or Pendulous (Type 3) and Creased or Ridged (Type 4). Risk factors for failing SBC and post-ERCP complications were analyzed by multivariate analysis. Results A total of 286 cases were included. Age, gender, indications and therapeutic procedures were not different among the four types of papillae. The failure rates of SBC with Type 3 papilla and Type 4 papilla were 11.11% and 6.25%, respectively. In the multivariate analysis, Type 2 papilla (odd ratio 7.18, p= 0.045) and Type 3 papilla (odd ratio 7.44, p= 0.016) were associated with greater SBC failure compared with Type 1 papilla. Malignant obstruction compared to stone (odds ratio 4.45, p=0.014) and age (odd ratio=1.06, p=0.010) were also risk factors for cannulation failure. Type 2 papilla was correlated with a higher rate of post-ERCP pancreatitis (20%, p=0.020) compared to the other types of papilla However, papilla morphology was not a significant risk factor for any complications in the multivariate analysis. Conclusion Small papilla and protruding or pendulous papilla are more difficult to cannulate compared to regular papilla. Small papilla is associated with a higher rate of post-ERCP pancreatitis.

Visual outcomes after chemotherapy for optic pathway glioma in children with and without neurofibromatosis type 1: results of the International Society of Paediatric Oncology (SIOP) Low-Grade Glioma 2004 trial UK cohort

2018 ◽  
Vol 102 (10) ◽  
pp. 1367-1371 ◽  
Author(s):  
Kevin Falzon ◽  
Evangelos Drimtzias ◽  
Susan Picton ◽  
Ian Simmons
Keyword(s):  
Risk Factors ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Paediatric Oncology ◽  
Optic Pathway Glioma ◽  
Final Visit ◽  
Low Grade ◽  
Optic Pathway ◽  
Associated Risk Factors

AimsTo report visual acuity (VA) outcomes following chemotherapy for optic pathway glioma (OPG) in children with or without neurofibromatosis type-1 (NF1) and to analyse associated risk factors.MethodsA prospective, multicentre, cohort study involving 155 children treated between September 2004 and December 2012. Initial and final VA was used for per-eye and per-subject analysis. Correlation tests were performed to determine whether initial VA predicted final VA. Logistic regression was used to determine whether age and tumour location were associated risk factors.Results90 children had complete ophthalmological data. At initiation of chemotherapy, 26% and 49% of eyes with NF1-OPG and sporadic OPG, respectively, had VA of ≥0.7 log of the minimum angle of resolution (logMAR). At final visit, per eye, 49% had ≤0.2, 23% had 0.30–0.60 and 28% had VA≥0.70 logMAR in the NF1-OPG group. In the sporadic OPG group, per eye, 32% had ≤0.2, 11% had VA 0.30–0.60 and 57% had ≥0.70 logMAR. Children with sporadic OPG, per eye, were significantly less likely to have VA outcomes ≤0.60 logMAR compared with children with NF1-OPG (OR=0.30; 95% CI 0.16 to 0.56; P<0.0001). Per subject, VA improved in 24%, remained stable in 35% and worsened in 41% of children with NF1-OPG and improved in 18%, remained stable in 43% and worsened in 39% of children with sporadic OPG.ConclusionsChildren with and without NF1 demonstrated the same rate of VA improvement, stabilisation or worsening; however, children with sporadic OPG had a poorer VA outcome. Better initial VA, older age, absence of postchiasm tumour and presence of NF1 were associated with improved or stable VA outcomes.

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