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Therapy-related myeloid neoplasm (t-MN) in the pediatric population is not well characterized. We studied 12 pediatric patients diagnosed with t-MN in our institution since 2006. The median age at the t-MN diagnoses was 14.8 years (range, 9 to 20 y). The primary malignancies included 9 solid tumors and 3 hematopoietic malignancies. Rhabdomyosarcoma (n=4) was the most common primary malignancy. Five of the 9 patients with solid tumors and all 3 patients with hematopoietic malignancies had primary neoplasms involving bone marrow. The median latency period was 5.2 years (range, 1.8 to 13.8 y). Thrombocytopenia was present in all patients at the t-MN diagnoses. Complete or partial monosomy of chromosome 5 or 7 were the 2 most common cytogenetic abnormalities. A quarter of patients demonstrated a genetic predisposition to t-MN: 1 with Li-Fraumeni syndrome with a germline TP53 R248Q mutation, 1 with Noonan syndrome with a somatic mutation (PTPN11 S502T), and 1 with a constitutive chromosomal translocation [t(X;9)(p22;q34)] and a germline TP53 L130V mutation. Outcomes remain poor. Two patients survived 3 and 5.1 years after hematopoietic stem cell transplantation.

Vascular anomalies comprise a spectrum of disorders characterized by the abnormal development or growth of blood and lymphatic vessels. These growths have unique features and diverse behaviors, mandating a multidisciplinary approach in their evaluation, diagnosis, and management. Here we describe the case of a male toddler presenting with an abdominal mass, originally treated as a metastatic retroperitoneal tumor, but subsequently felt to represent a vascular anomaly.

Objectives: Physical health conditions cause significant disability and mortality among people living with alcohol and other drug problems. There has been limited research on the prevalence of heal...

Facial expressions are one way in which infants and adults communicate emotion. Infants scan expressions similarly to adults, yet it remains unclear whether they are receptive to the affective information they convey. The current study investigates six-, nine- and twelve-month infants’ (N = 146) pupillary responses to the six ‘basic’ emotional expressions (happy, sad, surprise, fear, anger, and disgust). To do this we use dynamic stimuli and gaze-contingent eye-tracking to simulate brief interactive exchanges, alongside a static control condition. Infants’ arousal responses were stronger for dynamic compared to static stimuli. And for dynamic stimuli we found that, compared to neutral, infants showed dilatory responses for happy and angry expressions only. Although previous work has shown infants can discriminate perceptually between facial expressions, our data suggest that sensitivity to the affective content of all six basic emotional expressions may not fully emerge until later in ontogeny.

Abstract International air travel has been highlighted as a concern since the beginning of the COVID-19 pandemic with respect to importation of cases. We summarise the available evidence for in-flight transmission of wild type SARS-CoV-2 during 2020, and for imported COVID-19 clusters to cause outbreaks. This paper provides a data baseline prior to the emergence of new mutations causing SARS-CoV-2 variants of concern, whose characteristics may increase the potential risk of in-flight transmission and imported outbreaks. The evidence on in-flight transmission of wild-type SARS-CoV-2 is limited, and is described in a small number of published reports. Most of the available evidence pertains to the early phase of the COVID-19 pandemic, during a period without non-pharmaceutical interventions such as distancing and in-flight mask wearing. There is considerable potential for outbreaks of COVID-19 from imported cases or clusters when public health guidance around quarantine of travellers and self-isolation of cases is not adhered to. Risks can be mitigated by measures such as: avoiding non-essential travel, targeted testing and quarantine of travellers from high incidence regions or regions of concern, managed quarantine processes, and protocols for rapid investigation and control of transmission from a possible variant of concern. Measures should be dynamically assessed and proportionate to the level of risk. Graphical abstract

Purpose We previously identified three microRNAs (miRNAs) with significantly increased expression in the serum of patients with age-related macular degeneration (AMD) compared with healthy controls. Our objective was to identify potential functional roles of these upregulated miRNAs (miR-19a, miR-126, and miR-410) in AMD, using computational tools for miRNAs prediction and identification, and to demonstrate the miRNAs target genes and signaling pathways. We also aim to demonstrate the pathologic role of isolated sera-derived exosomes from patients with AMD and controls using in vitro models. Methods miR-19a, miR-126, and miR-410 were investigated using bioinformatic approaches, including DIANA-mirPath and miR TarBase. Data on the resulting target genes and signaling pathways were incorporated with the differentially expressed miRNAs in AMD. Apoptosis markers, human apoptosis miRNAs polymerase chain reaction arrays and angiogenesis/vasculogenesis assays were performed by adding serum-isolated AMD patient or control patient derived exosomes into an in vitro human angiogenesis model and ARPE-19 cell lines. Results A number of pathways known to be involved in AMD development and progression were predicted, including the vascular endothelial growth factor signaling, apoptosis, and neurodegenerative pathways. The study also provides supporting evidence for the involvement of serum-isolated AMD-derived exosomes in the pathology of AMD, via apoptosis and/or angiogenesis. Conclusions miR-19a, miR-126, miR-410 and their target genes had a significant correlation with AMD pathogenesis. As such, they could be potential new targets as predictive biomarkers or therapies for patients with AMD. Translational relevance The functional analysis and the pathologic role of altered miRNA expression in AMD may be applicable in developing new therapies for AMD through the disruption of individual or multiple pathophysiologic pathways.

Calcinosis cutis describes a condition of pathologic calcium deposition in the dermis. Several subtypes exist, including the subepidermal calcified nodule. The oral mucosal calcified nodule (OMCN) was posited in 1992 as a specific term for a subepidermal calcified nodule occurring in the oral cavity, and since that time only six such lesions have been described in the literature. This report explores a case of OMCN on the palate of a 3-month-old infant with the goal of supplementing extant literature, providing a consideration of the differentials of palatal lesions in the pediatric population, and describing a unique instance in which OMCN resulted in a full-thickness defect requiring palatoplasty for repair.

Primary pulmonary malignancies are rare in childhood. The most common, pleuropulmonary blastoma (PPB), has an incidence of 25–50 cases per year in the United States (Knight and et al., 2019) [1]. The majority of children are diagnosed with PPB before the age of four years. PPB is divided into subtypes I, Ir (type I- regressed), II, and III, which correlates to the age of diagnosis and patient prognosis [2,3]. Here we report an unusual presentation of PPB in a teen-aged female who presented with a one month history of a non-productive cough.

CIC-rearranged sarcomas rarely occur in visceral organs including the kidney. The most common fusion partner with CIC is the DUX4 gene, but variant fusion partners have also been reported. Herein, we describe the clinicopathologic features and comprehensive molecular profiling of 4 cases of primary renal CIC-rearranged sarcomas. All cases occurred in females, age range 13 to 82 years and included 3 resections and 1 needle biopsy specimen. There was a tendency for development of metastatic disease predominantly to the lungs and poor disease outcome despite different treatment strategies. Histologically, variable round cell (20% to 100%), spindle cell (0% to 80%), and rhabdoid morphologies (0% to 20%) were seen. By immunohistochemistry diffuse WT1 nuclear (2 to 3+, ∼90%) labeling was present in 1 case, with cytoplasmic staining in the others (3+, 40% to 75%). CD99 was focally positive in all 4 cases (≤10%); 1 case each was diffusely positive for c-myc (2 to 3+, ∼90%) and ETV4 (3+, ∼90%); 1 case was focally positive for c-myc (2+, ∼5%) and calretinin (2+, ∼5%); and all cases were negative for cytokeratin and NKX2.2. CIC rearrangement by fluorescence in situ hybridization was present in the 3 cases tested. Comprehensive genomic profiling (CGP) of 3 cases revealed a CIC-DUX4 fusion in 2 cases, and 1 CIC-NUTM1 fusion. All 4 CIC-rearranged renal sarcomas had low mutation burden, and except HLA-A and MLL mutations lacked genomic alterations in other oncogenic drivers. Material from the needle biopsy was insufficient for CGP but that case was positive with the DUX4 immunohistochemical stain as were the 2 CIC-DUX4 tumors. In conclusion, CIC-rearranged sarcomas rarely occur in the kidney with a tendency for poor outcome and in this series we illustrate an example with CIC-NUTM1 fusion, an emerging variant, at a visceral site. Testing by fluorescence in situ hybridization or CGP is optimal to avoid missing cases that harbor variant fusion partners.

The implementation of surveillance biopsies in pediatric kidney transplantation remains controversial. Surveillance biopsies detect subclinical injury prior to clinical dysfunction, which could allow for early interventions that prolong allograft survival. We conducted a single-center retrospective cohort study of 120 consecutive pediatric kidney recipients, of whom 103 had surveillance biopsies ≤6 months posttransplant. We tested the hypothesis that subclinical inflammation (borderline or T cell–mediated rejection without clinical dysfunction) is associated with a 5-year composite endpoint of acute rejection and allograft failure. Overall, 36% of subjects had subclinical inflammation, which was associated with increased hazard for the composite endpoint (adjusted hazard ratio 2.89 [1.27, 6.57]; P < .01). Subjects with treated vs untreated subclinical borderline rejection had a lower incidence of the composite endpoint (41% vs 67%; P < .001). Subclinical vascular injury (subclinical inflammation with Banff arteritis score > 0) had a 78% incidence of the composite endpoint vs 11% in subjects with no major surveillance abnormalities (P < .001). In summary, we showed that subclinical inflammation phenotypes were prevalent in pediatric kidney recipients without clinical dysfunction and were associated with increased acute rejection and allograft failure. Once prospectively validated, our data would support implementation of surveillance biopsies as standard of care in pediatric kidney transplantation.

Background Outcomes following major lower limb amputation (MLLA) between 2000 and 2002 from the Department of Vascular Surgery at Royal Perth Hospital have been published; mean postoperative length of stay 20 days, inpatient complication rate 54%, and 30-day mortality 10%. The last decade has seen increasing endovascular revascularization techniques, increased focus on MLLA patients, and general improvements in the model of care. The aim of this study is to compare outcomes between 2000–2002 and 2010–2012. Methods Data on all patients undergoing MLLA, transtibial or proximal, in the 2 time periods were extracted from the department of vascular surgery database. Medical records, government registries, and phone calls to primary care providers were used to clarify mortality. Results Limb ischemia remains the most common indication for MLLA with smoking, hypertension, and diabetes being the main comorbid diseases. The rates of wound infections have fallen from 26.4% to 12.4% ( P = 0.023), rate of admission to ICU has fallen from 48.3% to 17.5% ( P = 0.001), and revision amputation to a higher level has fallen from 11.5% to 7.2% ( P = 0.043). Acute hospital, postoperative length of stay has trended down from 15.74 to 20.29 days ( P = 0.075). Mortality overall has fallen from 60.92% to 46.39% ( P = 0.049). Thirty-day mortality fallen from 10.34% to 5.15% ( P = 0.185), 6-month 28.76% to 16.5% ( P = 0.046), and 1-year 40.22% to 21.65% ( P = 0.006). Conclusions Patients undergoing MLLA still carry a high burden of comorbid disease. With changes in revascularization technique, consultant supervision, and multidisciplinary model of care, we have seen the rate of complications fall, length of stay trend down, and overall mortality reduce. Despite improvements, outcomes remain sobering and more can be done.

BACKGROUND Mucociliary clearance is a main defense mechanism of the airway and is impaired in ciliary dyskinesia. The objective of this study was to evaluate the prevalence of chronic rhinosinusitis (CRS) and its characteristics in bronchiectasis patients suspected of harboring ciliary dyskinesia. METHODS Bronchiectasis patients referred to a rhinology clinic for nasal brush biopsy (NBB) were included in this study. NBB was performed using a curettage technique whereby ciliated epithelial cells were obtained from the surface of the inferior nasal turbinate. Results of transmission electron microscopy findings, primary ciliary dyskinesia (PCD) gene (35 genes) analyses (Invitae), and sinus computed tomography (CT) scans were reviewed. RESULTS Twenty-three patients (age, 54 ± 2.9 years) were referred for NBB between 2015 and 2018. Thirteen patients (56.5%) met the criteria for diagnosis of CRS. Nineteen patients had ciliary ultrastructural defects. The most common finding was compound cilia (n = 11, 47.8%). Five patients (21.7%) had central microtubule defects (CMD) with higher forced expiratory volume in 1 second (FEV1 ) at the time of referral than those without CMD (CMD+ , 91 ± 3.7%; CMD- , 73.5 ± 5.7%; p = 0.023). Of 15 subjects with a PCD gene panel, 67% (9 of 15) carried at least 1 gene associated with PCD. Only 1 patient reached diagnosis of PCD. Approximately 50% of non-PCD carriers had a smoking history (p < 0.05). Lund-Mackay scores did not significantly differ between PCD and non-PCD carriers (p = 0.72). CONCLUSION Nearly half of bronchiectasis patients referred for NBB had concurrent CRS. The presence of ciliary abnormalities was not amplified in bronchiectasis patients with CRS compared to those without CRS. Extrinsic factors may be related to ciliary structural abnormalities in non-PCD gene carriers.

In this study, we assessed the acute kinematic effects of different sled load conditions (unloaded and at 10%, 20%, 30% decrement from maximum velocity (Vdec)) in different sporting populations. It is well-known that an athlete’s kinematics change with increasing sled load. However, to our knowledge, the relationship between the different loads in resisted sled sprinting (RSS) and kinematic characteristics is unknown. Thirty-three athletes (sprinters n = 10, team sport athletes n = 23) performed a familiarization session (day 1), and 12 sprints at different loads (day 2) over a distance of 40 m. Sprint time and average velocity were measured. Sagittal-plane high-speed video data was recorded for early acceleration and maximum velocity phase and joint angles computed. Loading introduced significant changes to hip, knee, ankle, and trunk angle for touch-down and toe-off for the acceleration and maximum velocity phase (p &lt, 0.05). Knee, hip, and ankle angles became more flexed with increasing load for all groups and trunk lean increased linearly with increasing loading conditions. The results of this study provide coaches with important information that may influence how RSS is employed as a training tool to improve sprint performance for acceleration and maximal velocity running and that prescription may not change based on sporting population, as there were only minimal differences observed between groups. The trunk lean increase was related to the heavy loads and appeared to prevent athletes to reach mechanics that were truly reflective of maximum velocity sprinting. Lighter loads seem to be more adequate to not provoke changes in maxV kinematics. However, heavy loading extended the distance over which it is possible to train acceleration.

Pulmonary complications post hematopoietic stem cell transplant in dyskeratosis congenita: analysis of oxidative stress in lung fibroblasts

Objectives To investigate influenza vaccination uptake rates, attitudes and motivations towards influenza vaccination among student health care workers (HCWs). Material and methods Self-reported influenza vaccination uptake among student HCWs at The University of Notre Dame Australia, Fremantle, Western Australia (UNDAF) was surveyed before and after implementation of a peer-led, student-centered campaign to raise awareness of, and improve access to, influenza vaccination. Data were weighted and analysed using logistic regression. Results Pre-campaign influenza vaccination uptake was 36.3% (95% CI = 31.8%–40.8%), with students identifying lack of awareness of both the Australian Government's recommendations and university policy, cost, and inconvenience of vaccine access as key barriers. Post-campaign vaccination coverage increased significantly to 55.9% (95% CI = 52.2%–59.6%). Multivariate logistic regression, controlled for statistically insignificant confounders of age and gender, showed that being a student HCW in 2014 (campaign year) was significantly and independently associated with influenza vaccination (OR 2.2, 95% CI = 1.7–2.9, P Conclusions Student HCWs' influenza vaccination uptake improved significantly following a low-cost, peer-led promotional campaign. This approach can be adapted to other settings.

Background:Tibial tubercle transfer (TTT) and medial patellofemoral ligament (MPFL) reconstruction have both shown, either in isolation or in combination, to provide improved patellofemoral joint (PFJ) stability. There are few studies that provide evidence that this remains true in the long term.Purpose:To compare the long-term results of patellar instability after TTT with and without MPFL reconstruction in 2 randomized groups.Study Design:Randomized controlled trial; Level of evidence, 1.Methods:A total of 34 patients (36 knees) were randomized to 2 groups. The first group underwent lateral release (LR) and TTT for confirmed maltracking of the patella (control group). The second group underwent MPFL reconstruction in addition to TTT and LR (reconstruction group). Patients were followed up with validated questionnaires (Kujala score, Tegner activity score), a visual analog scale (VAS) assessing their insecurity, and a clinical assessment at a minimum of 5 years postoperatively. Participants also underwent quantitative computed tomography (CT) at 1 year for comparison. Two patients in the control group and 1 patient in the reconstruction group were lost to follow-up at 5 years.Results:There were no significant differences in the Kujala ( P = .75), Tegner ( P = .36), or VAS ( P = .75) scores at any time period. One patient in the control group sustained a patellar redislocation at 3 years. Five patients in the control group and 2 in the reconstruction group had functional failures and required reoperations; however, this was not statistically significant ( P = .30). There were no significant differences between groups in the time to return to school or work ( P = .65) or sports ( P = .38) after surgery. Overall patient satisfaction was higher in the reconstruction group compared with the control group ( P = .04), and quantitative CT scans showed that the reconstruction group had a statistically significant improvement in the mean patellar tilt (6° vs −8°, respectively; P = .03) and mean congruence angle (13° vs −11°, respectively; P = .03) in the quadriceps-contracted state compared with the control group.Conclusion:Reconstruction of the MPFL in addition to TTT and LR resulted in improved alignment parameters (congruence angle, patellar tilt angle) as well as patient satisfaction. The Kujala and Tegner scores were no different between the 2 groups at any time period. There was insufficient evidence to conclude that the addition of MPFL reconstruction to TTT results in fewer redislocations or reoperations. This study concludes that MPFL reconstruction improves PFJ alignment and patient satisfaction; however, further studies with larger patient numbers are required to satisfy its significance with respect to redislocation rates and functional scores in the long term.

We describe the epidemiological trends and report and review the public health restrictions implemented during the first wave of the COVID-19 pandemic in Ireland.The study reviewed confirmed cases of COVID-19 notified from 1 March to 18 July 2020. Data were obtained from the national COVID-19 Data Hub, the National Health Protection Surveillance Centre, the National Contact Management Programme and the European Centre for Disease Prevention and Control.A total of 25 617 cases were notified during the study period. Weekly cases and deaths peaked in mid-April 2020 at 5701 and 316, respectively. Mean number of close contacts per case was lowest at 0.7 in April, rising to 6.6 by July. Outbreak settings shifted from travel and workplace in March, to healthcare in April. Restrictions implemented on 12 March extended to full lockdown on 27 March. Phased relaxation of restrictions commenced 18 May. Effective suppression of community transmission of COVID-19 was achieved by June 2020.Lockdown is a crude population-level restriction effective in controlling COVID-19. Phased relaxation of restrictions in Ireland, however, led to an immediate increase in mean number of contacts per case, which facilitates viral transmission unless individual-level restrictions are adhered to. This demonstrates a limitation of lockdown as a long-term mechanism of pandemic control.

The measurement of relative physiological stress during training is important because this is the stimulus for the long-term adaptive response. Measurements of perceived exertion (RPE) have been reported to correlate with the heart rate during field-based training sessions. Nevertheless, there are few studies on how well RPE tracks with the heart rate over repeated training sessions in elite soccer players. Therefore, we aimed to quantify the within-participant correlations between variability in session-RPE (sRPE) and the heart rate in elite male soccer players, and to determine whether the playing position moderated these correlations. The field-based training of four central defenders, four wide defenders, six central midfielders, two wide midfielders and three attackers from an elite English Premier League squad were monitored over an entire in-season competitive period, giving a total of 1010 individual training sessions for study. Correlations between session-RPE and heart rates were quantified using a within-participant model. The correlation between changes in sRPE and heart rates was r = 0.75 (95% CI: 0.71-0.78). This correlation remained high across the various player positions (wide-defender, r = 0.81; central-defender, r = 0.74; wide midfielder, r = 0.70; central midfielder, r = 0.70; attacker, r = 0.84; P

Using a retrospective approach, this study explored psychological maltreatment by teachers of students from kindergarten through the 12th grade. Participants were 453 undergraduates who completed a survey about their negative experiences with teachers. Overall, 44 % of those surveyed reported at least one experience that they labeled as emotional abuse, and 52 % reported that a teacher had bullied them. The most commonly reported negative teacher behaviors included being shouted at and being given a lower grade than deserved. Although adaptive responses were reported, serious negative responses were reported, too. Many perceived these experiences as having an adverse effect on their life. Despite the frequency of negative teacher behaviors that were reported, relatively few participants (

Malignant rhabdoid tumors (MRT; atypical teratoid/rhabdoid tumor [ATRT] in the central nervous system) are aggressive tumors in infants and children which can overlap with other sarcomas, such as synovial sarcoma (SS). The gold standard for SS diagnosis is characterization of the t(X;18) chromosomal translocation. However, stratification of cases for molecular analysis is not always straightforward or feasible. Recent literature suggests transducer-like enhancer of split 1 (TLE1) protein expression may distinguish SS from certain histologic mimics; however, this has not been investigated in MRT and ATRT. We stained whole-tissue sections of 18 archived cases of MRT and ATRT with TLE1. Nuclear expression was scored using a 4-tiered (0, 1+, 2+, and 3+) scale describing staining intensity, extent, or combination of both. The majority of MRT and ATRT cases showed some TLE1 immunoreactivity (n = 16; 89% for ≥1 + staining); 14 (78%) of total cases showed ≥2 + positivity using any of the 3 scoring systems. Over half (n = 10; 56%) of cases showed ≥2 + staining; 4 (22%) cases showed 3 + strong and diffuse TLE1 staining measured by all scoring systems in agreement. Although still of potential use, we urge caution in the interpretation of TLE1 when the differential diagnosis includes both SS and MRT or ATRT.

Kelly, DT, Tobin, C, Egan, B, Carren, AM, O'Connor, PL, McCaffrey, N, and Moyna, NM. Comparison of sprint interval and endurance training in team sport athletes. J Strength Cond Res 32(11): 3051-3058, 2018-High-volume endurance training (ET) has traditionally been used to improve aerobic capacity but is extremely time-consuming in contrast to low-volume short-duration sprint interval training (SIT) that improves maximal oxygen uptake (V[Combining Dot Above]O2max) to a similar extent. Few studies have compared the effects of SIT vs. ET using running-based protocols, or in team sport athletes. Club level male Gaelic football players were randomly assigned to SIT (n = 7; 21.6 ± 2.1 years) or ET (n = 8; 21.9 ± 3.5 years) for 6 sessions over 2 weeks. V[Combining Dot Above]O2max, muscle mitochondrial enzyme activity, running economy (RE), and high-intensity endurance capacity (HEC) were measured before and after training. An increase in V[Combining Dot Above]O2max (p ≤ 0.05) after 2 weeks of both SIT and ET was observed. Performance in HEC increased by 31.0 and 17.2% after SIT and ET, respectively (p ≤ 0.05). Running economy assessed at 8, 9, 10, and 11 km·h, lactate threshold and vV[Combining Dot Above]O2max were unchanged after both SIT and ET. Maximal activity of 3-β-hydroxylacyl coenzyme A dehydrogenase (β-HAD) was increased in response to both SIT and ET (p ≤ 0.05), whereas the maximal activity of citrate synthase remained unchanged after training (p = 0.07). A running-based protocol of SIT is a time-efficient training method for improving aerobic capacity and HEC, and maintaining indices of RE and lactate threshold in team sport athletes.

Meckel diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract and the most common cause of gastrointestinal bleeding in children. Although it usually follows the rule of 2's, exceptions to this rule are reported in the literature. Often asymptomatic, MD is commonly an incidental finding during surgical interventions. When symptomatic, the most common presentation of this condition is painless rectal bleeding. A myriad of other nonspecific symptoms are however possible, especially in adults, thus making this diagnosis difficult. Meckel diverticulum has been reported to mimic other abdominal pathologies like appendicitis, inflammatory bowel disease, and pancreatitis to name a few.We report a patient with acute abdomen in whom the more common causes of acute abdomen were ruled out and a diagnosis of MD was established on exploratory laparoscopy, only after he developed perforation. This report emphasizes the need for maintaining a high index of suspicion towards a possibility of a complicated MD in patients presenting with an acute abdomen, once other causes of acute abdomen are ruled out.

Key Clinical Message The uses of amniocentesis are numerous, including determination of chromosomal abnormalities, lung maturity, and infections. A common complication of amniocentesis is loss of the pregnancy, but rare complications should be considered. The role of patient history and clinical observation of uncommon presentations are critical in the management of the patient.

Soft tissue perineuriomas are rare but benign tumours that are composed of peripheral nerve sheath and are most commonly located in the extremity. Diagnosis is confirmed by the immunohistochemical and ultrastructural features of the tumour. Paratesticular location is extremely rare; so far, only one case is reported. Herein, we report a second case of perineurioma in a 16-year-old male in the paratesticular region.

There have been few previous attempts to assess the development of early markers of executive\ud function in infants born preterm despite well-established deficits reported for older preterm\ud children that have been closely linked to poorer academic functioning. The present study investigates\ud early attention control development in healthy 12-month-old age-corrected pre-term\ud infants who were born less than 30 weeks and compares their performance to full-term infants.\ud Eye-tracking methodology was used to measure attention control. Preterm Infants spent less time\ud focused on the target and were slower to fixate attention, with lower gestational age associated\ud with poorer target fixation and slower processing speed. There were no significant group differences\ud observed for inhibition of return or interference control. These findings suggest that\ud specific emerging deficits in attention control may be observed using eye tracking methodology\ud in very preterm infants at this early stage of development, despite scores within the average\ud range on the Bayley Scales of Infant Development.

Purpose To identify circulating microRNAs (miRNA) associated with age-related macular degeneration (AMD). Thus differentially expressed serum miRNA could be used as AMD biomarkers. Methods This study involved total RNA isolation from sera from patients with atrophic AMD (n = 10), neovascular AMD (n = 10), and age- and sex-matched controls (n = 10). A total of 377 miRNAs were coanalyzed using array technologies, and differentially regulated miRNAs were determined. Extensive validation studies (n = 90) of serum from AMD patients and controls confirmed initial results. Total RNA isolation was carried out from sera from patients with atrophic AMD (n = 30), neovascular AMD (n = 30), and controls (n = 30). Fourteen miRNAs from the discovery dataset were coanalyzed using quantitative real-time polymerase chain reaction (qRT-PCR) to validate their presence. Results Unsupervised hierarchical clustering indicated that AMD serum specimens have a different miRNA profile to healthy controls. We successfully identified and validated the differentially regulated miRNAs in serum from AMD patients versus controls. The biomarker potential of three miRNAs (miR-126, miR-19a, and miR-410) was confirmed by qRT-PCR, with significantly increased quantities in serum of AMD patients compared with healthy controls. Conclusions Increased quantities of miR-126, miR-410, and miR-19a in serum from AMD patients indicate that these miRNAs could potentially serve as diagnostic AMD biomarkers. All three miRNAs significantly correlated with AMD pathogenesis. Translational relevance The discovery of new AMD miRNA may act as biomarkers in evaluating AMD diagnosis and prognosis.

OBJECTIVE Chronic blood transfusion therapy reduces clinical events in children with sickle cell anemia but increases risk for an iron-related liver injury. Liver biopsy is the gold standard technique for quantifying liver iron content (LIC) and evaluating liver pathology. Ferritin, liver enzymes, and R2* magnetic resonance imaging of the liver are obtained as surrogate markers. In this study we compared surrogate markers with the gold standard, liver biopsy, in assessing liver histology. METHODS We conducted a retrospective review of 259 liver biopsies in 109 children with sickle cell anemia on chronic transfusion therapy and chelation therapy during a 9-year period at a single center. Liver pathology was compared with LIC, ferritin, and alanine aminotransferase. RESULTS Ferritin correlates with LIC (r = 0.74, P

Background Neonates and young infants exposed to extracorporeal circulation during extracorporeal membrane oxygenation (ECMO) and cardiopulmonary bypass (CPB) are at risk of developing a systemic inflammatory response syndrome (SIRS) with multi-organ dysfunction. We used a piglet model of ECMO to investigate the hypothesis that epithelial apoptosis is an early event that precedes villous damage during ECMO-related bowel injury. Methods Healthy 3-week-old piglets were subjected to ECMO for up to 8h. Epithelial apoptosis was measured in histopathological analysis, nuclear imaging, and terminal deoxynucleotidyl transferase dUTP nick end labeling. Plasma intestinal-fatty acid-binding protein (I-FABP) levels were measured by enzyme immunoassay. Intestinal mast cells were isolated by fluorescence-assisted cell sorting. Cleaved caspase-8, caspase-9, phospho-p38 MAPK, and fas ligand expression was investigated by immunohistochemistry, Western blots, and reverse transcriptase-quantitative polymerase chain reaction. Results Piglet ECMO was associated with increased gut epithelial apoptosis. Extensive apoptotic changes were noted on villus tips and in scattered crypt cells after 2h of ECMO. After 8h, the villi were denuded and apoptotic changes were evident in a majority of crypt cells. Increased circulating I-FABP levels, a marker of gut epithelial injury, showed that epithelial injury occurred during ECMO. We detected increased cleaved caspase-8, but not cleaved caspase-9, in epithelial cells indicating that the extrinsic apoptotic pathway was active. ECMO was associated with increased fas ligand expression in intestinal mast cells, which was induced through activation of the p38 mitogen-activated protein kinase. Conclusions Epithelial apoptosis is an early event that initiates gut mucosal injury in a piglet model of ECMO.

There is little data on why glioblastomas (GBM) hemorrhage and how it may affect patient outcomes. The aim of this study was to investigate the mechanisms of hemorrhage in glioblastoma by examining molecular and genetic features by immunohistochemistry (IHC) and mRNA expression profiles in association with imaging and clinical outcomes. An observational retrospective cohort analysis was performed on 43 FFPE GBM tissue samples. MR images were assessed for the presence of hemorrhage and extent of resection. Specimens were examined for CD34 and CD105 expression using IHC. Tumor mRNA expression profiles were analyzed for 92 genes related to angiogenesis and vascularity. Forty-three specimens were analyzed, and 20 showed signs of hemorrhage, 23 did not. The average OS for patients with GBM with hemorrhage was 19.12 months (95% CI 10.39–27.84), versus 13.85 months (95% CI 8.85–18.85) in those without hemorrhage (p > 0.05). Tumors that hemorrhaged had higher IHC staining for CD34 and CD105. mRNA expression analysis revealed tumor hemorrhage was associated with increased expression of HIF1α and MDK, and decreased expression of F3. Hemorrhage in GBM was not associated with worsened OS. Increased expression of angiogenic factors and increased CD34 and CD105 IHC staining in tumors with hemorrhage suggests that increased hypoxia-induced angiogenesis and vessel density may play a role in glioblastoma hemorrhage. Characterizing tumors that are prone to hemorrhage and mechanisms behind the development of these hemorrhages may provide insights that can lead to the development of targeted, individualized therapies for glioblastoma.

Objective: To review a case of quintuplets with all babies developing necrotizing enterocolitis. Methods: A retrospective study of preterm quintuplets all developing necrotizing enterocolitis. Clinical outcomes were reviewed. Results: Quintuplets were born at 24 weeks gestation. Each baby developed NEC and was treated. One baby died. Currently the remaining 4 infants are on full enteral nutrition. Conclusion: Further studies are needed to better understand this emerging population of multiple birth pregnancy and the frequency of NEC development.

The patient was born in a town near Bogota, Colombia and was adopted in the United States at 2 years of age. At the time of adoption, there was a suspicion of renal disease with failure to thrive. Within a year of arriving in Alabama, she was diagnosed with hypoplastic-dysplastic syndrome. Her renal disease resulted in dialysis at age 16, and she received a living unrelated renal transplant from her adoptive mother at age 18. She was treated with an immunosuppressive regimen that included tacrolimus, mycophenolate, and prednisone. Her immediate posttransplant course was notable only for complaints of mild diarrhea that worsened when her regimen changed from tacrolimus to sirolimus in the 8th month after transplant for a research protocol. At 9 months posttransplant, she developed severe, constant, right lower quadrant abdominal pain. Her diarrhea worsened and she developed headaches, but she denied having fever, cough, difficulty breathing, vomiting, or bloody stools. A computerized tomography scan showed findings associated with appendicitis, which prompted appendectomy. Pathologic examination of the specimen identified intense transmural eosinophilic infiltration of the appendiceal wall consistent with eosinophilic appendicitis (Figure ​(Figure1).1). Biopsies of the terminal ileum, cecum, and colon at the hepatic flexure also revealed eosinophilic infiltrates. No parasitic forms were found in any of the tissue samples by direct visualization. A peripheral eosinophilia of up to 9.5%was documented, and a stool test was negative for ova and parasites (O&P). Figure 1. Pathology of appendix. Numerous eosinophils fill the lamina propria (left panel) and infiltrate the submucosa (right panel) of the appendix (hematoxylin and eosin stain, ×132). After appendectomy, the patient's abdominal pain resolved and her diarrhea improved. However, her eosinophilia persisted, rising as high as 18.3% 3 months later, even after empiric treatment with 2 doses of mebendazole for “intestinal parasite” 1 month after appendectomy. Subsequent evaluation by the gastrointestinal and allergy/immunology services included negative immunoassays for food and upper respiratory allergens, negative screens for celiac disease, negative serology for Toxocara, and a Strongyloides immunoglobulin G (IgG) antibody by enzyme-linked immunosorbent assay (ELISA) that was positive (4.28, reference range 10%) that subsided with transplant but reappeared with the onset of her abdominal symptoms (Figure ​(Figure22). Figure 2. Eosinophil percentage from peripheral blood samples over a 10-year span. Time of transplant (a: 5/21/09), appendectomy (b: 2/18/10), and completion of first (c: 6/5/10) and second (d: 6/27/10) treatments with ivermectin are marked with arrows. On physical examination the patient was well-appearing and of short stature (143.5 cm

Angiodysplasia (vascular malformations) of the colon is extremely rare in children, and, as in adults, present with lower gastrointestinal hemorrhage. Here we report an unusual pediatric case of angiodysplasia of the terminal ileum and cecum presenting as an acute abdomen with radiological features suggestive of lymphoma.

The acetylcholinesterase (AChE) stain has been a commonly accepted adjunctive study for rectal biopsy specimens in patients being evaluated for Hirschsprung disease (HD). However, the requirement for immediately frozen rectal biopsy specimens (to preserve the enzyme activity) has proved to be an obstacle to its use in some centers. In this study, we developed a microwave procedure for the AChE stain, which we then used to evaluate a special tissue transport medium, Michel’s medium, for its utility in preserving AChE enzyme activity without immediate freezing in fresh rectal specimens collected from known HD patients. When compared with similar rectal biopsy specimens that were immediately snap-frozen, the specimens stored in Michel’s medium at room temperature or with refrigeration (for up to 108 hours) followed by sucrose cryoprotection showed preservation of AChE enzyme activity, with comparable nerve fiber staining and no significant background staining. This technique provides a convenient alt...

Context: Type 1 hyperlipoproteinemia (T1HLP) in childhood is most often due to genetic deficiency of lipoprotein lipase (LPL) or other related proteins. Objective: The aim was to report a case of marked hypertriglyceridemia and recurrent acute pancreatitis due to the presence of LPL autoantibody in a young girl who was subsequently diagnosed with Sjögren's syndrome. Subject and Methods: A 9-yr-old African-American girl presented with acute pancreatitis and serum triglycerides of 4784 mg/dl. Strict restriction of dietary fat reduced serum triglycerides, but she continued to experience recurrent pancreatitis. Approximately 18 months thereafter, she developed transient pauciarticular arthritis with elevated serum antinuclear antibody (>1:1280). Minor salivary gland biopsy revealed chronic sialadenitis with a dense periductal lymphocytic aggregate suggestive of Sjögren's syndrome. Genomic DNA was analyzed for LPL, GPIHBP1, APOA5, APOC2, and LMF1. Immunoblotting was performed to detect serum LPL autoantibody. Results: The patient had no disease-causing variants in LPL, GPIHBP1, APOA5, APOC2, or LMF1. Immunoblotting revealed serum LPL antibody. The patient responded to immunosuppressive therapy for Sjögren's syndrome with resolution of hypertriglyceridemia. Conclusions: Unexplained T1HLP in childhood could be secondary to LPL deficiency induced by autoantibodies. Therefore, diagnosis of autoimmune T1HLP should be entertained if clinical features are suggestive of an autoimmune process.

Lymphatic malformations that involve the nervous system are uncommon. The authors review their experience with involvement of the brachial plexus and its branches by cystic hygromas. A retrospective review of the authors' experience with pathology of the pediatric brachial plexus revealed 4 cases involving patients with compression of this structure and its branches due to cystic hygroma. Although such cases are apparently rare, the neurosurgeon should consider malformations of the lymphatic system in the differential diagnosis of masses involving the brachial plexus and its branches.

We report two cases of primary CNS lymphoma (PCNSL) treated with high-dose methotrexate. Though standard adult treatment of PCNSL incorporates whole-brain radiotherapy, the literature suggests it may be possible to delay or avoid radiotherapy and the associated increased risk of neurologic sequelae in pediatric patients. Studies in adults indicate methotrexate therapy can be effective against PCNSL and has advantages over the current standard of treatment. Both patients have no evidence of disease 9 and 7 years after treatment, suggesting high-dose methotrexate may lead to disease control in pediatric patients with PCNSL while avoiding the effects of radiotherapy. Pediatr Blood Cancer. 2010;55:1227–1230. © 2010 Wiley-Liss, Inc.

Since fibrous particles such as asbestos and some man-made fibers (MMF) have been known to produce carcinogenic or fibrogenic effects, disk-shaped potassium octatitanate (POT) particles (trade name: Terracess TF) were manufactured as nonfibrous particles. A 90-day inhalation toxicity study of Terracess TF was performed to evaluate comparative inhalation toxicity of the disk shape with a fibrous shape that was previously evaluated. Four groups of 20 male and 15 female rats each were exposed to Terracess TF aerosols at concentrations of 0, 2, 10, or 50 mg/m(3) for 90 days. Ten male and 10 female rats per group were sacrificed at 90 days of exposure. After 90 days of exposure, 5 male rats per group were sacrificed at 3 wk of recovery period and 4-5 male rats per group or 5 female rats per group were sacrificed at 15 wk of recovery for lung clearance and histopathology. The mass median aerodynamic equivalent diameter (MMAED) of the aerosols of test materials ranged from 2.5 to 2.9 microm. There were no test-substance-related adverse effects on clinical observations. At the end of the 90-day exposure, a slight increase in lung-to-body weight ratios was observed at 50 mg/m(3) in male but not in female rats. However, lung weights were within normal limits after 3- or 15-wk recovery periods. Microscopically, inhaled Terracess TF particles were mostly phagocytized by free alveolar macrophages (AMs) in the alveolar airspaces and alveolar walls maintained normal structure at 2 and 10 mg/m(3). At 50 mg/m(3), some alveoli were distended and filled with aggregates of particle-laden AMs. The alveolar walls showed slight type II pneumocyte hyperplasia, but neither proliferative inflammation nor alveolar fibrosis was present at 50 mg/m(3). The clearance half-times for Terracess TF were estimated to be in the order of 6 to 9 mo for the 50-mg/m(3) group and 2 to 3 mo for the 10- and 2-mg/m(3) groups. The lung responses and lung clearance rate were comparable to those of "nuisance" type dusts at these concentrations. Based on interpretation that aggregated particle-laden AMs in alveoli was considered to be an early histopathological sign of lung overloading, an effect level was considered to be 50 mg/m(3) and no-observedadverse- effect level (NOAEL) was 10 mg/m(3). This experiment clearly demonstrated that particle morphology was considered to be an important factor to determine inhaled particle toxicity.

Background Extracorporeal membrane oxygenation (ECMO) is a life-saving support system used in neonates and young children with severe cardiorespiratory failure. Although ECMO has reduced mortality in these critically-ill patients, almost all patients treated with ECMO develop a systemic inflammatory response syndrome (SIRS) characterized by a ‘cytokine storm’, leukocyte activation, and multisystem organ dysfunction. We used a neonatal porcine model of ECMO to investigate whether rising plasma concentrations of inflammatory cytokines during ECMO reflect de novo synthesis of these mediators in inflamed tissues, and therefore, can be used to assess the severity of ECMO-related SIRS. Methods Three-week-old previously-healthy piglets were subjected to venoarterial ECMO for up to 8 hours. SIRS was assessed by histopathological analysis, measurement of neutrophil activation (flow cytometry), plasma cytokine concentrations (enzyme immunoassays), and tissue expression of inflammatory genes (polymerase chain reaction/western blots). Mast cell degranulation was investigated by measurement of plasma tryptase activity. Results Porcine neonatal ECMO was associated with systemic inflammatory changes similar to those seen in human neonates. TNF-α and interleukin-8 (IL-8) concentrations rose rapidly during the first 2 hours of ECMO, faster than the tissue expression of these cytokines. ECMO was associated with increased plasma mast cell tryptase activity, indicating that increased plasma concentrations of inflammatory cytokines during ECMO may result from mast cell degranulation and associated release of preformed cytokines stored in mast cells. Conclusions TNF-α and IL-8 concentrations rose faster in plasma than in the peripheral tissues during ECMO, indicating that rising plasma levels of these cytokines immediately following the initiation of ECMO may not reflect increasing tissue synthesis of these cytokines. Mobilization of preformed cellular stores of inflammatory cytokines such as in mucosal mast cells may play an important pathophysiological role in ECMO-related SIRS.

This study of 59 undergraduate men and 85 undergraduate women explored how defining emotional and physical sibling abuse affected the frequency of reported sibling abuse. In addition, the current study examined how the emotional context of the sibling relationship (i.e., rivalry and conflict) moderated the relationship between sibling abuse and later psychological adjustment (i.e., depression and anxiety). Respondents completed self-report questionnaires of sibling abuse (CTS2-SP), self-labeling of sibling abuse, quality of sibling relationships (SRQ), depression (CES-D), and anxiety (ZAS). Results indicated differences in frequency of reported abuse depending on how sibling abuse was defined. Also, there were no statistically significant correlations between the CTS2-SP and measures of psychological adjustment. Although self-labeling as emotionally abused correlated with later anxiety, the emotional context of the sibling relationship did not moderate this relationship.

Epithelial ovarian carcinoma (EOC) is a leading cause of death from gynecologic malignancies, due mainly to the prevalence of undetected metastatic disease. The process of cell invasion during intraperitoneal anchoring of metastatic lesions requires concerted regulation of many processes, including modulation of adhesion to the extracellular matrix and localized invasion. Exploratory cDNA microarray analysis of early response genes (altered after 4 hr of 3D collagen culture) coupled with confirmatory real-time reverse-transcriptase polymerase chain reaction, multiple 3D cell culture matrices, Western blot, immunostaining, adhesion, migration and invasion assays were used to identify modulators of adhesion pertinent to EOC progression and metastasis. cDNA microarray analysis indicated a dramatic downregulation of connective tissue growth factor (CTGF) in EOC cells placed in invasion- mimicking conditions (3D Type I collagen). Examination of human EOC specimens revealed that CTGF expression was absent in 46% of the tested samples (n = 41), but was present in 100% of normal ovarian epithelium samples (n = 7). Reduced CTGF expression occurs in many types of cells and may be a general phenomenon displayed by cells encountering a 3D environment. CTGF levels were inversely correlated with invasion such that downregulation of CTGF increased, while its upregulation reduced collagen invasion. Cells adhered preferentially to a surface comprised of both collagen I and CTGF relative to either component alone using alpha6beta1 and alpha3beta1 integrins. Together these data suggest that downregulation of CTGF in EOC cells may be important for cell invasion through modulation of cell-matrix adhesion.

The effects of intravenous Verapamil (V) on exercise haemodynamics and ventricular function were compared in 13 patients with stable effort angina. Intravascular pressures, thermodilution cardiac output and radionuclide ejection fraction were measured simultaneously at rest and during exercise. At rest Verapamil produced systemic vasodilation. During exercise at identical workloads compared to control, Verapamil increased cardiac index (CI) and decreased AVO2 difference. Pulmonary artery wedge pressure was lower. Ejection fraction was higher (control-55 +/- 11% vs V-64 +/- 11%) as the disproportionate increase in end systolic volume relative to the end diastolic volume was prevented. Verapamil is effective in exercise induced angina and alters haemodynamics primarily through its vasodilating properties associated with an increased CI.

Separating ischiopagus conjoined twins presents the challenge of closing complex pelvic and abdominal wall defects. Traditionally, synthetic mesh has been used to reconstruct large body wall defects. Recent studies demonstrate increased complications while using synthetic mesh for complex musculofasciocutaneous defects. Acellular dermis is a bioprosthetic material that offers advantages over synthetic mesh. These include the ability to become revascularized and remodeled with host tissue, thereby increasing resistance to infection, providing tolerance to overlying skin breakdown, and augmenting strength. The authors describe the use of acellular dermis to reconstruct the pelvic and abdominal walls in the separation of 4-year-old female ischiopagus tripus conjoined twins. Acellular dermis has proven to be effective, as 18 months postoperatively there is no evidence of ventral herniation, wall weakness, or infection. Acellular dermis is a valuable and effective alternative to synthetic mesh for reconstructing large pelvic and abdominal wall defects.

The rare cases of subgaleal hematoma in childhood reported previously have all been related to head trauma. A case of apparently spontaneous subgaleal hematoma is reported which was associated with a qualitative platelet defect and not with trauma. Subgaleal hematoma must be differentiated from subgaleal infection and air from frontal sinusitis with bony erosion, and from an encephalocele or tumor erosion through the skull. Computed cranial tomography is useful in that differentiation. Most cases have been managed conservatively, but subgaleal tap may be indicated if there is severe headache or potential scalp necrosis.

According to most authors, dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) represent the adult and juvenile forms, respectively, of the same disease entity, as evidenced by similar morphology, an identical chromosomal translocation, and CD34 positivity. It has been shown that DFSP and nuchal-type fibroma (NTF) (which is also CD34-positive) are related lesions, and that there might possibly be a continuum between the two. In addition, NTF exhibits CD99 positivity. It was therefore, hypothesized that both DFSP and GCF would show similar immunopositivity for CD99. Archives of pathology at several institutions were searched for DFSP and GCF tissue blocks. A total of 29 DFSP and 5 GCF were analyzed by immunohistochemistry for expression of CD99. Twenty-three of 29 DFSP (79%) and 2 of 5 GCP (40%) expressed CD99. Comparison of CD99 and CD34 showed that the non-tumoral periphery of DFSP was less probable to be CD99 positive, but this finding was not statistically significant.

Advanced and metastatic ovarian cancer is a leading cause of death from gynecologic malignancies. A more detailed understanding of the factors controlling invasion and metastasis may lead to novel anti-metastatic therapies. To model cellular interactions that occur during intraperitoneal metastasis, comparative cDNA microarray analysis and confirmatory real-time reverse transcription PCR (RT-PCR) were employed to uncover changes in gene expression that may occur in late stage ovarian cancer in response to microenvironmental cues, particularly native three-dimensional collagen I. Gene expression in human ovarian carcinoma tissues was evaluated on the RNA and protein level using real-time RT-PCR and immunohistochemistry. Cell invasion and migration were evaluated in a collagen invasion assay and a scratch wound assay. Three-dimensional collagen I culture led to differential expression of several genes. The role of actinin alpha-4 (ACTN4), a cytoskeleton-associated protein implicated in the regulation of cell motility, was examined in detail. ACTN4 RNA and protein expression were associated with advanced and metastatic human ovarian carcinoma. This report demonstrates that a cytoskeletal-associated protein ACTN4 is upregulated by three-dimensional collagen culture conditions, leading to increased invasion and motility of ovarian cancer cells. Expression of ACTN4 in human ovarian tumors was found to be associated with advanced-stage disease and peritoneal metastases.

Adult T-cell leukemia/lymphoma is a T-cell malignancy caused by the human T-cell lymphotropic virus-I. Adult T-cell leukemia/lymphoma is primarily a disease of adults due to the long latency period between initial infection and development of leukemia. We present a case of acute adult T-cell leukemia/lymphoma in an adolescent. Skin lesions had appeared 3 years earlier and were the initial sign of human T-cell lymphotropic virus-I infection and T-cell malignancy. Her disease failed to respond to both intensive chemotherapy and antiviral therapy. Cutaneous lesions are sometimes the initial sign of adult T-cell leukemia/lymphoma and early recognition is imperative.

Object Meningioma research has been hindered by the inability to sequentially measure intracranial tumor growth in a cost-effective, efficient manner. Recently, the luciferase gene has been transfected into cancer lines to obtain cells that express the luciferase enzyme, which oxidizes luciferin in a reaction that releases photon energy that can be measured noninvasively by bioluminescence imaging (BLI) systems. The authors describe a mouse model of intracranial meningioma that uses this novel BLI system. Methods The immortal meningioma cell lines CH-157-MN and IOMM-Lee were transfected with luciferase and neomycin phosphotransferase (LucNeo) and selected with G418. These cells were stereotactically implanted at skull base and cerebral convexity locations in nude mice. Animals were imaged for bioluminescence biweekly, and 5 mice underwent magnetic resonance (MR) imaging. Tumors were harvested for immunohistochemical and ultrastructural analysis. Results The CH-157-MN-LucNeo and IOMM-Lee-LucNeo cell lines were successfully implanted intracranially in mice. The tumor induction rate for CH-157-MN-LucNeo skull base tumors was 90% (36 of 40 procedures). Statistical analysis of CH-157-MN-LucNeo skull base tumor volume measured on MR imaging correlated with the results of BLI showed an R value of 0.900. The tumors exhibited characteristics of aggressive meningiomas by insinuating along arachnoid planes and invading brain. Conclusions Noninvasive BLI was successfully used to image intracranial meningiomas in mice. The tumors grew in a fashion similar to that of aggressive meningiomas in humans, and exhibited the microscopic, immunohistochemical, and ultrastructural features characteristic of meningiomas. This animal model overcomes the main obstacle in studying intracranial meningiomas by enabling sequential noninvasive tumor measurement in a cost-effective manner.