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Background Our aim was to examine the association between preterm delivery and incident maternal mental disorders using a population-based cohort of mothers in Canada. Methods Retrospective matched cohort study using Manitoba Centre for Health Policy (MCHP) administrative data in Manitoba. Mothers who delivered preterm babies (Results Mothers of preterm children ( N = 5,361) had similar incidence rates of any mental disorder (17.4% vs. 16.6%, IRR = 0.99, 95% CI, 0.91 to 1.07) compared to mothers of term children ( N = 24,932). Mothers of term children had a higher rate of any mental disorder in the first year while mothers of preterm children had higher rates from 2 to 5 years. Being the mother of a child born Interpretation Mothers of preterm and term children had similar rates of incident mental disorders within 5-years post-delivery. Extreme prematurity was a risk factor for any mental disorder. Targeted screening and support of this latter group may be beneficial.

Purpose This study aimed to evaluate the impact of the COVID-19 pandemic on adolescent and young adult (AYA) cancer survivors. Methods We conducted a cross-sectional survey of AYAs aged 18–49 with cancer in Canada between January and February 2021. Data from survivors, defined as AYAs more than one year off cancer treatment, were analysed. Multiple logistic regression was used to identify factors associated with psychological distress, loneliness and insomnia. Results The analysis included 384 survivors. Moderate-to-severe psychological distress was reported by 257 (68.9%) survivors and was associated with an income ≥ $60,000 (adjusted odds ratio [AOR] 2.15, 95% CI 1.11–4.17) and the presence of a pre-existing chronic physical health condition (AOR 2.05, 95% CI 1.18–3.56). Loneliness was reported by 204 (54.0%) survivors and was associated with being unemployed (AOR 2.26 95%CI 1.18–4.31), pandemic causing finances to be worse (AOR 1.82, 95%CI 1.08–3.06) and the presence of a pre-pandemic mental health condition (AOR 1.88, 95% CI 1.03–3.42). Clinical insomnia was reported by 74 (19.5%) survivors and was associated with employment status as a student (AOR 3.00, 95% CI 1.08–8.29) or unemployed (AOR 3.97, 95% CI 1.46–10.83), earning $60,000 or more in the year 2020 (AOR 4.36, 95% CI 1.43–13.32), having haematologic cancer (AOR 2.21, 95% CI 1.05–4.70) and being single (AOR 2.52, 95% CI 1.08–5.91). Pandemic negatively affected employment, finances, physical activity, cancer care and substance use for 73.9%, 66.5%, 32.5%, 21.8% and 19.2% of survivors, respectively. Worries about finances, contracting COVID-19, cancer treatment increasing the risk of COVID-19 infection, and having poor health outcomes from contracting COVID-19 were reported by 46.0%, 45.6%, 55.0% and 47.3% of survivors, respectively. Conclusions The COVID-19 pandemic has had a significant impact on AYA cancer survivors, and these individuals report high levels of psychological distress, insomnia and loneliness. Implications for Cancer Survivors. Cancer survivors are at risk for worsening mental and physical health outcomes during the COVID-19 pandemic. Targeted interventions and support programs are urgently needed to support the mental health of AYA cancer survivors and optimize their health outcomes. Supplementary Information The online version contains supplementary material available at 10.1007/s11764-021-01158-9.

Background Adolescents and young adults (AYAs) diagnosed with cancer are at an increased risk of experiencing social isolation and loneliness secondary to their cancer and its treatment. The physical distancing measures implemented during the COVID-19 pandemic may have further increased loneliness among this group. This study examined the prevalence of loneliness and factors associated with loneliness among AYAs with cancer during this pandemic. Methods We conducted a self-administered, online, cross-sectional survey of Canadian AYAs diagnosed with cancer between 15 and 39 between January and February 2021. Loneliness was measured using the 3-item UCLA Loneliness Scale. Factors associated with higher levels of loneliness were identified using multiple logistic regression. Results The analysis included 805 AYAs. The prevalence of loneliness was 52.2% [N = 419, 95% CI (confidence interval) 48.7 to 55.6%]. Individuals who were 18–25 years old [AOR (adjusted odds ratio)1.60, CI 1.03–2.47, p = 0.035], currently undergoing cancer therapy (AOR 1.46, 95% CI 1.03–2.07, p = 0.035), who self-disclosed the presence of a pre-pandemic mental health condition (AOR 2.09, 95% CI = 1.22–3.58, p = 0.007), or were not in a relationship (AOR 2.22, 95% CI 1.57–3.14, p

We aimed to describe the negative and positive impacts of changes in cancer care delivery due to COVID-19 pandemic for adolescents and young adults (AYAs) in Canada, as well as the correlates of negative impact and their perspectives on optimization of cancer care. We conducted an online, self-administered survey of AYAs with cancer living in Canada between January and February 2021. Multiple logistic regression was used to identify factors associated with a negative impact on cancer care. Of the 805 participants, 173 (21.5%) experienced a negative impact on their cancer care including delays in diagnostic tests (11.9%), cancer treatment (11.4%), and appointments (11.1%). A prior diagnosis of mental or chronic physical health condition, an annual income of &lt, 20,000 CAD, ongoing cancer treatment, and province of residence were independently associated with a negative cancer care impact (p-value &lt, 0.05). The majority (n = 767, 95.2%) stated a positive impact of the changes to cancer care delivery, including the implementation of virtual healthcare visits (n = 601, 74.6%). Pandemic-related changes in cancer care delivery have unfavorably and favorably influenced AYAs with cancer. Interventions to support AYAs who are more vulnerable to the adverse effects of the pandemic, and the thoughtful integration of virtual care into cancer care delivery models is essential.

There are minimal data regarding the safety and efficacy of cannabis used as an anti-cancer agent or for symptom management in pediatric oncology. We aimed to characterize the prevalence and factors associated with the use of cannabis for the treatment of cancer and management of cancer-related symptoms in children during or after cancer treatment. An anonymous 40 question paper survey was offered to patients/caregivers of children with cancer attending a pediatric oncology clinic in a provincially mandated cancer agency between October 2019 and March 2020. There were 64 respondents included in the analysis. Fourteen participants (N=14/64; 22%) reported use of cannabis, of which half used cannabis for either cancer treatment or symptom management, or both. Leukemia (n=9/14; 64%) was the most frequent diagnosis in children whose caregivers reported using cannabis and the majority of them were still receiving active cancer treatment (N= 5/9; 56%). All of the respondents using cannabis (14/14, 100%) experienced symptom improvement. Most of the caregivers procured cannabis from their friends (N=5/14; 36%), and oil was the most commonly used formulation (N=12/14; 86%). Cannabis-related information was received from another parent (N=4/14; 29%) or from a doctor (N=4/14; 29%). The reported monthly expenditure on cannabis varied widely from less than $50 CAD (N=4/14; 29%) to more than $500 CAD (N=3/14; 21%). Our survey shows that cannabis, mostly oil products, was used by one-fifth of children with cancer during or after the completion of cancer treatment. These findings require validation in a larger nationwide survey.

ObjectiveTo study the association between prematurity and grade 3 school performance in a contemporary cohort of children.MethodsPopulation-based retrospective cohort study in Manitoba, Canada. Children born between 1999 and 2011 who had their grade 3 school performance data available were eligible. Preterm birth (ResultsOf the 186 956 eligible children, 101 436 children (7187 preterm (gestational age, median (IQR) 35 weeks (34, 36)) and 94 249 term (40 weeks (39,40)) were included. Overall, 19% of preterm and 14% of term children had the numeracy outcome (adjusted OR (aOR) 1.38; 95% CI 1.29 to 1.47, pConclusions and relevanceChildren born preterm had poorer performance in grade 3 numeracy and reading proficiencies than children born full term. All children born preterm, not just those born extremely preterm, should be screened for reading and numeracy performance in school and strategies implemented to address any deficits.

Combined rearrangements of MYC and BCL2 are rare in precursor B-cell acute lymphoblastic leukemia (B-ALL). A 14-year-old boy presented with swelling of the knee and face. Imaging revealed diffuse infiltration of lacrimal glands, parotid glands along with the extensive epidural disease. Morphology and immunophenotype of knee joint aspirate were consistent with precursor B-ALL. Fluorescent in situ hybridization identified rearrangements of MYC and BCL2 genes. The disease was refractory to intensive treatment. The patient died of progressive disease. Precursor B-ALL with combined MYC and BCL2 rearrangements is rare, characterized by an aggressive clinical course, and has an inadequate response to standard therapeutic approaches.

RMS most commonly presents in children and adolescents, however a subset of tumors are diagnosed in infants under one year of age. Due to the rarity of infant RMS, utilization of different treatment approaches and goals, and small sample sizes, the published studies of infants with RMS have yielded heterogeneous results. In this review, we discuss the outcomes of infants with RMS treated in various clinical trials and the strategies that various international cooperative groups have employed to reduce the morbidity and mortality related to treatment without compromising the overall survival of this population. This review discusses the unique scenarios of diagnosing and managing congenitals or neonatal RMS, spindle cell RMS and relapsed RMS. This review concludes by exploring novel approaches to diagnosis and management of infants with RMS that are currently being studied by various international cooperative groups.

The use of cannabis for medical purposes by pediatric patients is expanding across Canada; however, supporting evidence, federal regulations and treatment guidelines are lacking. To understand factors affecting treatment decisions in this landscape, we sought to delineate clinician perspectives, ethics priorities and values for cannabis authorization.We sampled participants purposefully through Canadian Childhood Cannabinoid Clinical Trials listservs, which include the majority of pediatric oncologists and palliative care physicians practising in Canada, among many other pediatric physicians and clinicians. Inclusion criteria were being a practising clinician in Canada, involvement in the care of children and willingness to be interviewed regardless of stance on medical cannabis. In November and December 2020, we conducted semistructured interviews focusing on principles, values and priorities, including medical, professional, regulatory, evidentiary and social considerations, for authorizing medical cannabis to children. Interviews were recorded, transcribed and analyzed by means of deductive and inductive thematic methods.We conducted 18 interviews with a diverse group of clinicians representing a range of specialties within pediatric care, including neurology, palliative care, oncology, family medicine and pharmacology. The interviews yielded 4 themes and 12 subthemes related to a priori (medical, professional, regulatory, evidentiary and social themes) and emergent themes. The 4 themes of access, relationships and relational autonomy (autonomy within relationships), medically appropriate use and research priorities were grounded in principles of harm reduction. Participants described problematic authorization procedures that negatively affect patient use. Principles associated with relational autonomy were highlighted as a feature of open clinical communication. Benefits of appropriate medical uses weighed positively over risks, even in the context of potential effects on neurodevelopment. Participants expressed that more research is essential to align medical cannabis with biomedical standards.Clinicians reported pursuing ethical use of medical cannabis for pediatric patients and prioritizing their safety under principles of harm reduction. There is a need for evidence about neurodevelopmental risks, support for research, treatment guidelines and greater knowledge about stakeholder perspectives to alleviate burdens related to use of medical cannabis for pediatric patients in Canada.

Background The COVID-19 pandemic has greatly altered the behavior of adolescents and young adults with cancer. No data exists as to the impact of gender on adherence of individuals with cancer to COVID-19 related public health guidelines and their preferred methods of receiving COVID-19 related information. Methods We conducted a cross-sectional survey of adolescents and young adults with cancer. Data were summarized using descriptive statistics. Multiple logistic regression was used to assess differences in adherence to COVID-19 preventative behaviors, and differences in preferred information sources of COVID-19 related information between men and women. Results Among 633 participants, adherence to key preventative measures was 44.9-58.8% for males and 53.4-68.1% for females. After adjusting for key confounding variables in multivariable analysis, males were less likely to adhere to frequent hand washing (AOR 1.45, CI 1.03-2.03), not touching face (AOR 1.82, CI 1.29-2.56) and social distancing (AOR 1.93, CI 1.37-2.71) than females. Both genders preferred to receive information from their cancer institutes and social media. Discussion: Gender-specific interventions are needed to improve adherence amongst males to COVID-19 precautionary measures. Information should be disseminated via cancer institutes and social media as these are the preferred sources of COVID-19 related information., Graphical Abstract

The COVID-19 pandemic has been particularly distressing for adolescents and young adults (AYAs) diagnosed with cancer. High levels of distress are associated with sleep disturbances. This study examined the prevalence of insomnia symptoms in AYAs during the COVID-19 pandemic and identified factors related to greater insomnia severity.An online survey was administered to Canadian AYAs (N = 805) diagnosed with cancer between 15 and 39 years from January to February 2021.The primary measure was the Insomnia Severity Index (ISI). Univariable and multivariable binomial logistic regression examined demographic, clinical, and psychological factors associated with significant insomnia symptoms (ISI score ≥12).Three hundred twelve (38.8%) participants reported insomnia symptoms. In the multivariable model, severe psychological distress (ie, depression and anxiety; adjusted odds ratio (AOR) = 28.75, p ≤.001), having a pre-existing mental health condition (AOR = 1.92, p = .03), worsened mental health during the pandemic (AOR = 1.73, p = .02), finished cancer treatment ≥1 year ago (AOR = 1.72, p = .03), and experiencing no changes to schooling during the COVID-19 pandemic (AOR = 2.18, p = .004) were associated with significant insomnia symptoms. Standardized coefficients also indicated that worrying about possible disruptions to cancer care and being a homemaker/caretaker contributed to insomnia symptoms.Nearly 40% of AYAs with cancer reported insomnia symptoms during the COVID-19 pandemic. Insomnia was associated with potentially modifiable factors such as psychological distress, highlighting possible targets for intervention.

This study investigated prevalence of psychological distress, factors associated with distress, and experiences of Adolescents and Young Adults (AYAs) with cancer during the COVID-19 pandemic. It also compared distress in this group to previously surveyed Canadian AYAs with cancer in 2018 by the Young Adults with Cancer in their Prime (YACPRIME) study.A cross-sectional, online, self-administered survey of AYAs diagnosed with cancer between 15 and 39 years of age was conducted. Psychological distress was measured by the Kessler Psychological Distress Scale (K10). Associations between variables and high psychological distress (K10 ≥ 25), and comparison of prevalence of psychological distress with the YACPRIME study were done using multivariable logistic regression. Summative qualitative content analysis analyzed participant experiences during this pandemic.We included 805 participants. High psychological distress was present in over two-thirds of the group (68.0%; 95% CI, 64.7%-71.2%). Employment impact during pandemic (AOR (adjusted odds ratio), 2.16; 95% CI, 1.41-3.31) and hematologic malignancy (AOR, 1.76; 95% CI 1.08-2.97) were associated with higher psychological distress, while older age [AOR, 0.95; 95% CI, 0.92-0.99] and personal income$40,000 (AOR, 0.38; 95% CI, 0.24-0.58) were associated with lower distress. Adjusted odds of experiencing psychological distress among AYAs with cancer during pandemic compared to pre-pandemic years was 1.85 (95% CI: 1.36-2.53). Overarching themes of pandemic experiences included: inferior quality of life, impairment of cancer care, COVID-19 related concerns and extreme social isolation.AYAs diagnosed with cancer are experiencing high psychological distress during this pandemic. Distress screening and evidence-based interventions to alleviate distress are essential.

ImportanceChildren born preterm may experience learning challenges at school. However, there is a paucity of data on the school readiness of these children as they prepare to begin grade 1.ObjectiveTo examine the association between prematurity and school readiness in a population-based cohort of children.Design, Setting, and ParticipantsThis cohort study was conducted in the province of Manitoba, Canada, and involved 2 cohorts of children in kindergarten at the time of data collection. The population-based cohort included children born between January 1, 2000, and December 31, 2011, whose school readiness was assessed in kindergarten using the Early Development Instrument (EDI) data. The sibling cohort comprised children born preterm and their closest-in-age siblings born full term. Data were analyzed between March 12 and September 28, 2021.ExposuresPreterm birth, defined as gestational age (GA) less than 37 weeks.Main Outcomes and MeasuresThe primary outcome was vulnerability in the EDI, defined as a score below the tenth percentile of the Canadian population norms for any 1 or more of the 5 EDI domains (physical health and well-being, social competence, emotional maturity, language and cognitive development, and communication skills and general knowledge). Logistic regression models were used to identify the factors associated with vulnerability in the EDI. P values were adjusted for multiplicity using the Simes false discovery method.ResultsOf 86 829 eligible children, 63 277 were included, of whom 4352 were preterm (mean [SD] GA, 34 [2] weeks; 2315 boys [53%]) and 58 925 were full term (mean [SD] GA, 39 (1) weeks; 29 885 boys [51%]). Overall, 35% of children (1536 of 4352) born preterm were vulnerable in the EDI compared with 28% of children (16 449 of 58 925) born full term (adjusted odds ratio [AOR], 1.32; 95% CI, 1.23-1.41; P Conclusions and RelevanceResults of this study suggest that, in a population-based cohort, children born preterm had a lower school-readiness rate than children born full term, but this difference was not observed in the sibling cohort. Child and maternal factors were associated with lack of school readiness among this population-based cohort.

Background Delay in diagnosis and treatment initiation can be associated with adverse outcomes in children with cancer. Diagnostic interval (DI) is defined as the time between the date of first health care contact for symptoms related to cancer to the date of cancer diagnosis, and treatment interval (TI) is defined as interval between the definitive cancer diagnosis and cancer treatment initiation. We aimed to determine the predictors of DI and TI in children with rhabdomyosarcoma (RMS) and their association with event-free survival (EFS) and overall survival (OS). Methods Using the Cancer in Young People in Canada (CYP-C) national population-based database, we conducted a retrospective cohort study of children (0-14.99 years) newly diagnosed with RMS between 2001 and 2015 in Canada. Quantile regression was used to assess the predictors of DI and TI, and Cox regression was used to determine if these intervals were associated with EFS and OS. Results Median DI and TI were 16.5 days (interquartile range [IQR] 6.0-38.0) and 5 days (IQR 0-12), respectively. DI and TI were not significantly associated with age at diagnosis, sex, race, tumor site, stage or histology, treatment region, distance from treatment center, income quintile or diagnosis year (all p > .05). DI and TI were not associated with EFS (DI: hazard ratio [HR] 1.00, 95% CI 0.96-1.05, p = .871; TI: HR 1.03, 95% CI 1.00-1.05, p = .053) or OS (DI: HR 0.99, 95% CI 0.94-1.05, p = .797; TI: HR 1.02, 95% CI 0.99-1.05, p = .155). Conclusions In the publicly funded Canadian health care system, DI and TI did not affect the survival of children with RMS.

There are minimal data regarding the safety and efficacy of cannabis used as an anti-cancer agent or for symptom management in pediatric oncology. We aimed to characterize the prevalence and factors associated with the use of cannabis for the treatment of cancer and management of cancer-related symptoms in children during or after cancer treatment.An anonymous 40 question paper survey was offered to patients/caregivers of children with cancer attending a pediatric oncology clinic in a provincially mandated cancer agency between October 2019 and March 2020.There were 64 respondents included in the analysis. Fourteen participants (N=14/64; 22%) reported use of cannabis, of which half used cannabis for either cancer treatment or symptom management, or both. Leukemia (n=9/14; 64%) was the most frequent diagnosis in children whose caregivers reported using cannabis and the majority of them were still receiving active cancer treatment (N= 5/9; 56%). All of the respondents using cannabis (14/14, 100%) experienced symptom improvement. Most of the caregivers procured cannabis from their friends (N=5/14; 36%), and oil was the most commonly used formulation (N=12/14; 86%). Cannabis-related information was received from another parent (N=4/14; 29%) or from a doctor (N=4/14; 29%). The reported monthly expenditure on cannabis varied widely from less than $50 CAD (N=4/14; 29%) to more than $500 CAD (N=3/14; 21%).Our survey shows that cannabis, mostly oil products, was used by one-fifth of children with cancer during or after the completion of cancer treatment. These findings require validation in a larger nationwide survey.

Febrile neutropenia (FN) is an important cause of morbidity and mortality in children with acute lymphoblastic leukemia (ALL). We aimed to look at complications in febrile neutropenia and to derive a risk model for developing complications from the variables predicting complications. Children on treatment for ALL, presenting with FN, were prospectively enrolled over a period of 1 year. Their clinical presentation, course during hospital stay, and outcomes were recorded. Complications recorded included septic shock, pneumonia requiring invasive or non-invasive ventilation, renal failure, neutropenic enterocolitis, encephalopathy, congestive heart failure, and bleeding manifestations. There were 320 episodes of FN among 176 patients. Complications occurred during 73 (22.8%) episodes. Time since last chemotherapy ≤7 days [OR 2.2 (1–4.5)], clinical focus of infection [OR 2.7 (1.3–5.5)], undernutrition [OR 2.5 (1.1–5.5)], absolute neutrophil count (ANC) ≤ 100/μL [OR 2.8 (1.3–5.9)], and C-reactive protein (CRP) > 60 mg/L at admission [OR 13.3 (5.2–33.8)] were independent predictors of complications. A risk model (total score = 13) was developed based on these predictors. Children with score of ≥7 had 17.2 (7.7–38.6) odds of developing complications as compared to those with score

Importance Mindfulness-based interventions (MBIs), grounded in mindfulness, focus on purposely paying attention to experiences occurring at the present moment without judgment. MBIs are increasingly used by patients with cancer for the reduction of anxiety, but it remains unclear if MBIs reduce anxiety in patients with cancer. Objective To evaluate the association of MBIs with reductions in the severity of anxiety in patients with cancer. Data Sources Systematic searches of MEDLINE, Embase, Cochrane Central Register of Controlled Trials, CINAHL, PsycINFO, and SCOPUS were conducted from database inception to May 2019 to identify relevant citations. Study Selection Randomized clinical trials (RCTs) that compared MBI with usual care, waitlist controls, or no intervention for the management of anxiety in cancer patients were included. Two reviewers conducted a blinded screening. Of 101 initially identified studies, 28 met the inclusion criteria. Data Extraction and Synthesis Two reviewers independently extracted the data. The Cochrane Collaboration risk-of-bias tool was used to assess the quality of RCTs, and the Preferred Reporting Items for Systematic Reviews and Meta-analyses reporting guideline was followed. Summary effect measures were reported as standardized mean differences (SMDs) and calculated using a random-effects model. Main Outcomes and Measures Our primary outcome was the measure of severity of short-term anxiety (up to 1-month postintervention); secondary outcomes were the severity of medium-term (1 to ≤6 months postintervention) and long-term (>6 to 12 months postintervention) anxiety, depression, and health-related quality of life of patients and caregivers. Results This meta-analysis included 28 RCTs enrolling 3053 adults with cancer. None of the trials were conducted in children. Mindfulness was associated with significant reductions in the severity of short-term anxiety (23 trials; 2339 participants; SMD, −0.51; 95% CI, −0.70 to −0.33;I2 = 76%). The association of mindfulness with short-term anxiety did not vary by evaluated patient, intervention, or study characteristics. Mindfulness was also associated with the reduction of medium-term anxiety (9 trials; 965 participants; SMD, −0.43; 95% CI, −0.68 to −0.18;I2 = 66%). No reduction in long-term anxiety was observed (2 trials; 403 participants; SMD, −0.02; 95% CI, −0.38 to 0.34;I2 = 68%). MBIs were associated with a reduction in the severity of depression in the short term (19 trials; 1874 participants; SMD, −0.73; 95% CI; −1.00 to −0.46;I2 = 86%) and the medium term (8 trials; 891 participants; SMD, −0.85; 95% CI, −1.35 to −0.35;I2 = 91%) and improved health-related quality of life in patients in the short term (9 trials; 1108 participants; SMD, 0.51; 95% CI, 0.20 to 0.82;I2 = 82%) and the medium term (5 trials; 771 participants; SMD, 0.29; 95% CI, 0.06 to 0.52;I2 = 57%). Conclusions and Relevance In this study, MBIs were associated with reductions in anxiety and depression up to 6 months postintervention in adults with cancer. Future trials should explore the long-term association of mindfulness with anxiety and depression in adults with cancer and determine its efficacy in more diverse cancer populations using active controls.

Meningioma is a rare intracranial tumor of children and adolescents. Meningioma occurring outside the central nervous system is even rarer. We describe an 11- year old child who presented with extracranial paravertebral skull base meningioma and left vestibular schwannoma with an underlying neurofibromatosis type 2 (NF2). The patient presented with hearing loss, painless left neck mass and right sided horner syndrome. A CT scan and MRI revealed a skull base tumor in the region of the left carotid body encasing the vessels, opacifying the left middle ear cavity, extending intracranially through the left jugular foramen along with regional lymphadenopathy and left vestibular schwannoma. MRI spine identified Arnold-Chiari I malformation and two extramedullary enhancing lesions (1-2cms) at the level of thoracic spine. With a differential diagnosis of sarcoma and peripheral never sheath tumor with metastasis to spinal cord, partial excision of the skull-base tumor was performed along with suprahyoid neck dissection. Bone marrow examination to rule out metastasis was normal. Pathology confirmed the diagnosis of meningioma (meningothelial variant, WHO grade-1). Lymph nodes showed reactive hyperplasia. With this clinical presentation of hearing loss, extracranial meningioma, vestibular schwannoma and spinal tumour likely to be spinal meningioma or schwannoma, a diagnosis of NF2 was suspected. Next generation sequencing showed deletion of exon 4 in NF2 gene. On further screening, early changes of cataract were seen in the right eye. Surgery couldn’t be done for treatment of meningioma due to the encasement of the vital structures. Therapy with bevacizumab and everolimus was started to improve hearing loss secondary to schwannoma and extracranial meningioma. This report highlights the uncommon presentation of NF-2 and extracranial meningioma. Extracranial meningioma must be considered in the differential diagnosis in a child presenting with tumor of the skull base, middle ear and neck.

Our objective was to determine whether, compared with control interventions, pharmacologic interventions reduce the severity of fatigue in patients with cancer or recipients of hematopoietic stem-cell transplantation (hsct). For a systematic review, we searched medline, embase, the Cochrane Central Register of Controlled Trials, cinahl, and Psychinfo for randomized trials of systemic pharmacologic interventions for the management of fatigue in patients with cancer or recipients of hsct. Two authors independently identified studies and abstracted data. Methodologic quality was assessed using the Cochrane Risk of Bias tool. The primary outcome was fatigue severity measured using various fatigue scales. Data were synthesized using random-effects models. In the 117 included trials (19,819 patients), the pharmacologic agents used were erythropoietins (n = 31), stimulants (n = 19), l-carnitine (n = 6), corticosteroids (n = 5), antidepressants (n = 5), appetite stimulants (n = 3), and other agents (n = 48). Fatigue was significantly reduced with erythropoietin [standardized mean difference (smd): &minus, 0.52, 95% confidence interval (ci): &minus, 0.89 to &minus, 0.14] and with methylphenidate (smd: &minus, 0.36, 95% ci: &minus, 0.56 to &minus, 0.15), modafinil (or armodafinil) and corticosteroids were not effective. Erythropoietin and methylphenidate significantly reduced fatigue severity in patients with cancer and in recipients of hsct. Concerns about the safety of those agents might limit their usefulness. Future research should identify effective interventions for fatigue that have minimal adverse effects.

Summary Fatigue is a prevalent and distressing symptom in children and adolescents with cancer and in those who have undergone haemopoietic stem-cell transplantation. A multidisciplinary and multinational group of experts in paediatric oncology and fatigue, together with patient advocates, developed a clinical practice guideline for management of fatigue on the basis of systematic reviews that included six paediatric and 456 adult randomised studies. We used the Grading of Recommendations Assessment, Development and Evaluation approach to generate recommendations, and made strong recommendations, supported by evidence of moderate quality, for use of physical activity, relaxation, and mindfulness to reduce fatigue. When these approaches are unsuccessful or not feasible, cognitive or cognitive behavioural therapies may be offered (weak recommendation supported by evidence of moderate quality). Maturity and cognitive ability of individual patients will affect the feasibility of interventions. Systemic pharmacological approaches should not be routinely used to manage fatigue in children. Apart from identification of optimal approaches to implement recommended interventions in clinical practice, future research should also address knowledge gaps, including establishment of minimum age thresholds for interventions and inclusion of paediatric patients in randomised trials of fatigue management.

Purpose Objective was to determine whether physical activity reduces the severity of fatigue in patients with cancer or hematopoietic stem cell transplant (HSCT) recipients. Methods We conducted a meta-analysis of randomized trials comparing physical activity with control interventions for the management of fatigue in patients with cancer or HSCT recipients. Results There were 170 trials included. Physical activity reduced the severity of fatigue when compared to all control groups (standardized mean difference −0.49, 95% confidence interval −0.60 to −0.37; P

Background Medication errors occur universally. Inappropriate administration of chemotherapy drugs can have adverse effects in cancer patients. Our objective was to assess the rate and type of medication errors in children with acute lymphoblastic leukemia (ALL) receiving oral chemotherapy in outpatient setting. Procedure Prescription and administration of oral chemotherapy drugs in children with ALL were evaluated prospectively to determine rate and type of medication errors. Errors were defined as prescription (physician) level or administration (patient) level errors. Results Two hundred eighty-nine drugs were prescribed to 121 patients. Medication errors occurred in 36 (12.5%) prescriptions; 21(7.3%) were administration errors, 13 (4.5%) were prescribing errors, and two errors occurred at both levels. Mercaptopurine (6-MP) was significantly associated with higher rates of errors (Odds ratio [OR] = 2.1, 95% CI [confidence interval] 1–4.1) whereas lapses were less with dexamethasone (OR = 0.25, 95% CI 0.09–0.67). As a result of medication errors 28 (23.1%) patients received inappropriate doses. Twenty five (21%) patients received sub-optimal doses whereas three got higher doses of chemotherapy. On univariate analysis, socioeconomic status, education status of the caregiver, 6-MP and methotrexate were significantly associated with errors (P ≤ 0.05). On multivariate analysis, ≤ primary school education of the caregiver and prescription of methotrexate were independent predictors of errors. Conclusions Medication errors affected nearly one fourth of the children receiving oral chemotherapy. Future studies are needed to look at effective interventions to avoid chemotherapy associated errors especially amongst the lower strata of society. Pediatr Blood Cancer 2014;61:2218–2222. © 2014 Wiley Periodicals, Inc.

Compound heterozygous HbSD-Punjab is an uncommon hemoglobinopathy encountered in Indians. Limited literature is available about its clinical course. The aim of this study was to describe the clinical and hematological profile of HbSD-Punjab patients from North India.HbSD-Punjab patients diagnosed in the hematology clinics between year 2000 and 2010 were reviewed retrospectively. The diagnosis was established using high-performance liquid chromatography, molecular analysis, and family screening. Clinical details, laboratory parameters, and therapy details were recorded from case records.Ten patients were identified. Median age at onset of symptoms was 3.5 years (interquartile range [IQR], 1.9 to 7.2). Clinical presentation included: anemia in 3, painful vaso-occlusive crisis in 2, acute chest syndrome in 2, and 3 were diagnosed incidentally. All had moderate to severe anemia (mean hemoglobin [Hb]: 6.8 ± 1.2 g/dL). Eight required red cell transfusions (median: 3 [IQR, 2 to 8]). On high-performance liquid chromatography, median HbF, HbD, and HbS were 12.1% (IQR, 9 to 18.3), 39.7% (IQR, 35 to 42), and 38.5% (IQR, 29 to 43). Five patients received hydroxyurea (HDU), median dose: 20 mg/kg/d (IQR, 18 to 23) with median duration of 7 months (IQR; 6, 45). Increment in Hb and reduction in painful crisis was observed in response to HDU.HbSD-Punjab has a heterogeneous clinical presentation. Anemia and sickle crises are quite common. HDU may be considered for those presenting with severe phenotype.

Purpose The study aims to validate a score predicting risk of complications in pediatric patients with chemotherapy-related febrile neutropenia (FN) and evaluate the performance of previously published models for risk stratification. Patients and Methods Children diagnosed with cancer and presenting with FN were evaluated in a prospective single-center study. A score predicting the risk of complications, previously derived in the unit, was validated on a prospective cohort. Performance of six predictive models published from geographically distinct settings was assessed on the same cohort. Results Complications were observed in 109 (26.3%) of 414 episodes of FN over 15 months. A risk score based on undernutrition (two points), time from last chemotherapy ( 60 mg/l = five points), and absolute neutrophil count (

Febrile neutropenia (FN) is an oncological emergency to be treated within an hour. In a developing country, patients are often unable to reach hospital speedily. Our aim was to determine the symptom to door interval (SDI) in febrile neutropenic children with acute lymphoblastic leukaemia [ALL] and to identify factors resulting in delay.All consecutive children of ALL (14 years) presenting with FN over a period of 1 year were evaluated. Data for demographics, clinical details, phase of therapy, profile of caregivers, travelling time, SDI, reasons for delay, modes of transport, complications, invasive bacterial infections (IBI), length of hospital stay and outcome were recorded.Among 320 FN episodes, median SDI (in hours) was 24 (IQR 8, 36). SDI during intensive phases was significantly less as compared to nonintensive phases 12 (IQR 6, 24) and 24 (IQR 24, 48) (p 0.001). Children on induction phase reported to hospital at earliest [median 8 (IQR 4, 12)], while those on maintenance phase came late [median 36 (24, 48)]. Median travelling time was 15 min (IQR 15, 25) for patients on intensive phase and was 180 min (IQR 60, 285) for those on nonintensive phase (p 0.001). Ingestion of acetaminophen at home (30 %), inability to realise the gravity of the situation (27 %), unawareness of parents (9 %) and nonavailability of transport (12 %) were the most common reasons for delay. No significant association of SDI was seen with complications, IBI, duration of hospital stay and mortality (p 0.05).Considerable time lag was seen between onset of symptoms and reaching hospital. Health education and establishment of shared care are urgent needs in countries where tertiary care facilities are limited.

Febrile neutropenia is a common emergency encountered in children receiving chemotherapy for a malignancy. Left untreated, it can lead to serious morbidity and mortality. Febrile neutropenia is suspected in any patient on chemotherapy who presents with fever. Prompt evaluation and management by the primary contact pediatrician is essential for a successful outcome. A detailed history and physical examination is warranted to identify source of infection, although two thirds of them may not have localizing symptoms or signs. Risk stratification is valuable in categorizing the severity and guiding therapy. Initial stabilization, prompt initiation of appropriate antibiotics and adequate supportive care are the cornerstone of treatment. Knowledge of the locally prevailing bacteriological profile and antimicrobial susceptibility data is crucial for each hospital/unit to frame and periodically modify guidelines for the choice of antimicrobials. Delay in initiating antimicrobials significantly worsens the outcome. Education of the family as well as the members of the treating unit is important in this regard. Pro-active steps must be taken to reduce incidence of hospital acquired sepsis. Diagnosis and management in relevance to the emergency room is reviewed and institutional practice is shared.

Haematological disorders, both benign and malignant, may present with osteoarticular complaints. Overlapping rheumatological symptoms can easily mislead the treating physician away from the underlying haematological illness. Suspecting and diagnosing the primary blood disorder is crucial in these patients. In this article, we review the spectrum of haematological disorders commonly associated with arthropathy, their clinical presentation and management. We will further highlight the common diagnostic pitfalls that will help the treating clinician in managing such children.

Background To determine the prevalence of Xmn1-Gγ polymorphism in North Indian children and adolescents with β thalassemia intermedia (TI) and to correlate it with disease severity. Methods All patients of thalassemia intermedia presenting to the pediatric hematology clinic of a tertiary care hospital in North India were enrolled. Clinical severity of their disease was assessed by a phenotypic score proposed by Phadke and Agarwal. They were classified according to status of their Xmn1-Gγ polymorphism as Xmn1-Gγ +/+, Xmn1-Gγ +/−, and Xmn1-Gγ −/− by molecular analysis. Results A total of 104 patients were enrolled. Severe TI was seen in 56.7% (59) patients, while 43.3% (45) had non-severe TI. Jaundice was more frequent in severe TI than in non-severe TI. Xmn1-Gγ +/+ was present in 25.9% (25) patients. The frequency of the Xmn1-Gγ +/− and Xmn1-Gγ −/− was 22% and 37.3% in severe TI children. The corresponding frequencies were 31.1% and 42.2% in non-severe TI group respectively. No significant correlation was observed between the Xmn1-Gγ polymorphism and severity of thalassemia, age at onset of symptoms, age at diagnosis, age at first transfusion, transfusion frequency or average hemoglobin levels. HbF level was significantly higher in Xmn1-Gγ +/+ and Xmn1-Gγ +/− patients. Conclusions This study showed that although the prevalence of Xmn1-Gγ polymorphism is high in β thalassemia intermedia patients, it alone could not predict clinical severity in TI patients. Further refinement and validation of clinical scoring system is necessary for guiding appropriate management. Pediatr Blood Cancer 2011; 57: 1025–1028. © 2011 Wiley-Liss, Inc.

Purpose To determine whether non-physical activity mind and body practices reduce the severity of fatigue in patients with cancer or hematopoietic stem cell transplant (HSCT) recipients compared to control interventions. Methods We included randomized trials which compared non-physical activity mind and body practices compared with control interventions for the management of fatigue in cancer and HSCT patients. Results Among 55 trials (4975 patients), interventions were acupuncture or acupressure (n = 12), mindfulness (n = 11), relaxation techniques (n = 10), massage (n = 6), energy therapy (n = 5), energizing yogic breathing (n = 3) and others (n = 8). When combined, all interventions significantly reduced fatigue severity compared to all controls (standardized mean difference −0.51, 95% confidence interval −0.73 to −0.29). More specifically, mindfulness and relaxation significantly reduced fatigue severity. Conclusions Mindfulness and relaxation were effective at reducing fatigue severity in patients with cancer and HSCT recipients. Future studies should evaluate how to translate these findings into clinical practice across different patient groups.

Long-term damage to the residual kidney is of concern in the survivors of Wilms tumor. Our objective was to evaluate the long-term glomerular function and size of the residual kidney in these patients. Twenty-nine survivors of Wilms tumor diagnosed between July 1999 and June 2004 were enrolled. The glomerular function was assessed by creatinine clearance, 99mTc DTPA radionuclide scintigraphy and 24-hour urinary protein. Renal size was evaluated by ultrasonography. Median age at diagnosis and at enrollment were 2.87 ± 1.8 (range: 0.5-7.5) and 7.9 ± 3.8 years (range: 2.5-18). Median duration of follow-up was 4.78 ± 2.6 years (range: 1-8.8). Evidence of renal dysfunction in the form of either function or size was identified in eight (27.6%) children. Six children had subnormal glomerular filtration rate and one had proteinuria. Subnormal size of the residual kidney was observed in one child. Age at diagnosis, stage, and duration elapsed after nephrectomy had no association with renal dysfunction (P.05). Long-term follow up is crucial to identify clinical nephrotoxicity among survivors of Wilms tumor.

Medication errors occur universally. Inappropriate administration of chemotherapy drugs can have adverse effects in cancer patients. Our objective was to assess the rate and type of medication errors in children with acute lymphoblastic leukemia (ALL) receiving oral chemotherapy in outpatient setting.Prescription and administration of oral chemotherapy drugs in children with ALL were evaluated prospectively to determine rate and type of medication errors. Errors were defined as prescription (physician) level or administration (patient) level errors.Two hundred eighty-nine drugs were prescribed to 121 patients. Medication errors occurred in 36 (12.5%) prescriptions; 21(7.3%) were administration errors, 13 (4.5%) were prescribing errors, and two errors occurred at both levels. Mercaptopurine (6-MP) was significantly associated with higher rates of errors (Odds ratio [OR] = 2.1, 95% CI [confidence interval] 1-4.1) whereas lapses were less with dexamethasone (OR = 0.25, 95% CI 0.09-0.67). As a result of medication errors 28 (23.1%) patients received inappropriate doses. Twenty five (21%) patients received sub-optimal doses whereas three got higher doses of chemotherapy. On univariate analysis, socioeconomic status, education status of the caregiver, 6-MP and methotrexate were significantly associated with errors (P ≤ 0.05). On multivariate analysis, ≤ primary school education of the caregiver and prescription of methotrexate were independent predictors of errors.Medication errors affected nearly one fourth of the children receiving oral chemotherapy. Future studies are needed to look at effective interventions to avoid chemotherapy associated errors especially amongst the lower strata of society.

SUMMARY Rh isoimmunization manifesting as isolated early onset neonatal anemia has not been reported. We describe the presentation of 3 infants who manifested with isolated early severe anemia. All the infants presented early (3 to 7 d of age) with severe pallor. None had clinically significant jaundice. Evidence for hemolysis was present in all and their direct antiglobulin test was positive. To reduce the hemolysis, immunoglobulin was administered after which their hemoglobin improved. This report highlights the possibility of early onset anemia without significant jaundice as the sole manifestation of Rh isoimmunization and the possible beneficial role of immunoglobulin in them.

Background Intravenous immunoglobulin (IVIg) is used in neonates with isoimmune haemolytic disease to prevent exchange transfusion (ET). However, studies supporting IVIg had methodological issues. Objective To update the systematic review of efficacy and safety of IVIg in neonates with isoimmune haemolytic disease. Methods MEDLINE, Embase databases and Cochrane Central Register of Controlled Trials (Cochrane Library) were searched (from inception to May 2013) for randomised or quasi-randomised controlled trials comparing IVIg with placebo/controls in neonates with isoimmune haemolytic disease without any language restriction. Three investigators assessed methodological quality of included trials. Meta-analyses were performed using random effect model and risk ratio (RR)/risk difference (RD) and mean difference with 95% CI calculated. Main results Twelve studies were included, ten trials (n=463) of Rh isoimmunisation and five trials (n=350) of ABO isoimmunisation (three studies had both population). Significant variations in risk of bias precluded an overall meta-analysis of Rh isoimmunisation. Studies with high risk of bias showed that IVIg reduced the rate of ET in Rh isoimmunisation (RR 0.23, 95% CI 0.13 to 0.40), whereas studies with low risk of bias that also used prophylactic phototherapy did not show statistically significant difference (RR 0.82, 95% CI 0.53 to 1.26). For ABO isoimmunisation, only studies with high risk of bias were available and meta-analysis revealed efficacy of IVIg in reducing ET (RR 0.31, 95% CI 0.18 to 0.55). Conclusions Efficacy of IVIg is not conclusive in Rh haemolytic disease of newborn with studies with low risk of bias indicating no benefit and studies with high risk of bias suggesting benefit. Role of IVIg in ABO disease is not clear as studies that showed a benefit had high risk of bias.

The role of leukapheresis and low-dose chemotherapy is unclear in decreasing early mortality in acute myeloid leukemia (AML) patients with hyperleukocytosis. This systematic review was conducted to describe early mortality (deaths during first induction) in patients with AML with an initial white blood count≥100×10(9)L(-1) stratified by the approach to leukapheresis and hydroxyurea/low-dose chemotherapy. Twenty-one studies were included. Weighted mean early deaths rate (20 studies, 1354 patients) was 20.1% (95% confidence interval 15.0-25.1). Neither leukapheresis strategy (p=0.67) nor hydroxyurea/low-dose chemotherapy (p=0.23) influenced the early death rate. Early mortality related to hyperleukocytosis in AML is not influenced by universal or selected use of leukapheresis or hydroxyurea/low-dose chemotherapy.