Prognostic utility and characteristics of MIB-1 labeling index as a proliferative activity marker in childhood low-grade glioma: a retrospective observational study.

PURPOSE: The prognostic utility of MIB-1 labeling index (LI) in pediatric low-grade glioma (PLGG) has not yet conclusively been described. We assess the correlation of MIB-1 LI and tumor growth velocity (TGV), aiming to contribute to the understanding of clinical implications and the predictive value of MIB-1 LI as an indicator of proliferative activity and progression-free survival (PFS) in PLGG. METHODS: MIB-1 LI of a cohort of 172 nonependymal PLGGs were comprehensively characterized. Correlation to TGV, assessed by sequential MRI-based three-dimensional volumetry, and PFS was analyzed. RESULTS: Mean MIB-1 LI accounted for 2.7% (range: < 1-10) and showed a significant decrease to 1.5% at secondary surgery (p = .0013). A significant difference of MIB-1 LI in different histopathological types and a correlation to tumor volume at diagnosis could be shown. Linear regression analysis showed a correlation between MIB-1 LI and preoperative TGV (R2 = .55, p < .0001), while correlation to TGV remarkably decreased after incomplete resection (R2 = .08, p = .013). Log-rank test showed no association of MIB-1 LI and 5-year PFS after incomplete (MIB-1 LI > 1 vs ≤ 1%: 48 vs 46%, p = .73) and gross-total resection (MIB-1 LI > 1 vs ≤ 1%: 89 vs 95%, p = .75). CONCLUSION: These data confirm a correlation of MIB-1 LI and radiologically detectable TGV in PLGG for the first time. Compared with preoperative TGV, a crucially decreasing correlation of MIB-1 LI and TGV after surgery may result in limited prognostic capability of MIB-1 LI in PLGG.

CNS tumors with PLAGL1-fusion: beyond ZFTA and YAP1 in the genetic spectrum of supratentorial ependymomas.

A novel methylation class, "neuroepithelial tumor, with PLAGL1 fusion" (NET-PLAGL1), has recently been described, based on epigenetic features, as a supratentorial pediatric brain tumor with recurrent histopathological features suggesting an ependymal differentiation. Because of the recent identification of this neoplastic entity, few histopathological, radiological and clinical data are available. Herein, we present a detailed series of nine cases of PLAGL1-fused supratentorial tumors, reclassified from a series of supratentorial ependymomas, non-ZFTA/non-YAP1 fusion-positive and subependymomas of the young. This study included extensive clinical, radiological, histopathological, ultrastructural, immunohistochemical, genetic and epigenetic (DNA methylation profiling) data for characterization. An important aim of this work was to evaluate the sensitivity and specificity of a novel fluorescent in situ hybridization (FISH) targeting the PLAGL1 gene. Using histopathology, immunohistochemistry and electron microscopy, we confirmed the ependymal differentiation of this new neoplastic entity. Indeed, the cases histopathologically presented as "mixed subependymomas-ependymomas" with well-circumscribed tumors exhibiting a diffuse immunoreactivity for GFAP, without expression of Olig2 or SOX10. Ultrastructurally, they also harbored features reminiscent of ependymal differentiation, such as cilia. Different gene partners were fused with PLAGL1: FOXO1, EWSR1 and for the first time MAML2. The PLAGL1 FISH presented a 100% sensitivity and specificity according to RNA sequencing and DNA methylation profiling results. This cohort of supratentorial PLAGL1-fused tumors highlights: 1/ the ependymal cell origin of this new neoplastic entity; 2/ benefit of looking for a PLAGL1 fusion in supratentorial cases of non-ZFTA/non-YAP1 ependymomas; and 3/ the usefulness of PLAGL1 FISH.

Maternal diabetes and risk of offspring congenital heart diseases: the Japan Environment and Children's Study.

BACKGROUND: Few prospective cohort studies have examined the association between maternal diabetes, including pre-pregnancy and gestational diabetes, and the risk of congenital heart disease (CHD) in Asian offspring. METHODS: We examined the association between maternal diabetes and offspring CHD among 97,094 mother-singleton infant pairs in the Japan Environment and Children's Study (JECS) between January 2011 and March 2014. Odds ratios (OR) and 95% confidence intervals (CI) of offspring CHD based on maternal diabetes (pre-pregnancy diabetes and gestational diabetes) were estimated using logistic regression after adjusting for maternal age at delivery, pre-pregnancy body mass index (BMI), maternal smoking habits, alcohol consumption, annual household income, and maternal education. The diagnosis of CHD in the offspring was ascertained from the transcript of medical records. RESULTS: The incidence of CHD in the offspring was 1,132. Maternal diabetes, including both pre-pregnancy diabetes and gestational diabetes, was associated with a higher risk of offspring CHD: multivariable OR (95%CI) = 1.81 (1.40-2.33) for maternal diabetes, 2.39 (1.05-5.42) for pre-pregnancy diabetes and 1.77 (1.36-2.30) for gestational diabetes. A higher risk of offspring CHD was observed in pre-pregnancy BMI ≥25.0 kg/m2 (OR = 2.55, 95% CI: 1.74-3.75) than in pre-pregnancy BMI <25.0 kg/m2 (OR = 1.49, 95% CI: 1.05-2.10, p for interaction = 0.04). CONCLUSIONS: Maternal diabetes, including both pre-pregnancy and gestational, was associated with an increased risk of CHD in offspring.

Meta-analysis of machine learning models for the diagnosis of central precocious puberty based on clinical, hormonal (laboratory) and imaging data.

Background: Central precocious puberty (CPP) is a common endocrine disorder in children, and its diagnosis primarily relies on the gonadotropin-releasing hormone (GnRH) stimulation test, which is expensive and time-consuming. With the widespread application of artificial intelligence in medicine, some studies have utilized clinical, hormonal (laboratory) and imaging data-based machine learning (ML) models to identify CPP. However, the results of these studies varied widely and were challenging to directly compare, mainly due to diverse ML methods. Therefore, the diagnostic value of clinical, hormonal (laboratory) and imaging data-based ML models for CPP remains elusive. The aim of this study was to investigate the diagnostic value of ML models based on clinical, hormonal (laboratory) and imaging data for CPP through a meta-analysis of existing studies. Methods: We conducted a comprehensive search for relevant English articles on clinical, hormonal (laboratory) and imaging data-based ML models for diagnosing CPP, covering the period from the database creation date to December 2023. Pooled sensitivity, specificity, positive likelihood ratio (LR+), negative likelihood ratio (LR-), summary receiver operating characteristic (SROC) curve, and area under the curve (AUC) were calculated to assess the diagnostic value of clinical, hormonal (laboratory) and imaging data-based ML models for diagnosing CPP. The I2 test was employed to evaluate heterogeneity, and the source of heterogeneity was investigated through meta-regression analysis. Publication bias was assessed using the Deeks funnel plot asymmetry test. Results: Six studies met the eligibility criteria. The pooled sensitivity and specificity were 0.82 (95% confidence interval (CI) 0.62-0.93) and 0.85 (95% CI 0.80-0.90), respectively. The LR+ was 6.00, and the LR- was 0.21, indicating that clinical, hormonal (laboratory) and imaging data-based ML models exhibited an excellent ability to confirm or exclude CPP. Additionally, the SROC curve showed that the AUC of the clinical, hormonal (laboratory) and imaging data-based ML models in the diagnosis of CPP was 0.90 (95% CI 0.87-0.92), demonstrating good diagnostic value for CPP. Conclusion: Based on the outcomes of our meta-analysis, clinical and imaging data-based ML models are excellent diagnostic tools with high sensitivity, specificity, and AUC in the diagnosis of CPP. Despite the geographical limitations of the study findings, future research endeavors will strive to address these issues to enhance their applicability and reliability, providing more precise guidance for the differentiation and treatment of CPP.

Understanding lived experiences and perceptions of resilience in black and South Asian Muslim children living in East London: a qualitative study protocol.

INTRODUCTION: It is important to promote resilience in preadolescence; however, there is limited research on children's understandings and experiences of resilience. Quantitative approaches may not capture dynamic and context-specific aspects of resilience. Resilience research has historically focused on white, middle-class Western adults and adolescents, creating an evidence gap regarding diverse experiences of resilience in middle childhood which could inform interventions. East London's Muslim community represents a diverse, growing population. Despite being disproportionately affected by deprivation and racial and cultural discrimination, this population is under-represented in resilience research. Using participatory and arts-based methods, this study aims to explore lived experiences and perceptions of resilience in black and South Asian Muslim children living in East London. METHODS AND ANALYSIS: We propose a qualitative study, grounded in embodied inquiry, consisting of a participatory workshop with 6-12 children and their parents/carers to explore lived experiences and perceptions of resilience. Participants will be identified and recruited from community settings in East London. Eligible participants will be English-speaking Muslims who identify as being black or South Asian, have a child aged 8-12 years and live in East London. The workshop (approx. 3.5 hours) will take place at an Islamic community centre and will include body mapping with children and a focus group discussion with parents/carers to explore resilience perspectives and meanings. Participants will also complete a demographic survey. Workshop audio recordings will be transcribed verbatim and body maps and other paper-based activities will be photographed. Data will be analysed using systematic visuo-textual analysis which affords equal importance to visual and textual data. ETHICS AND DISSEMINATION: The Queen Mary Ethics of Research Committee at Queen Mary University of London has approved this study (approval date: 9 October 2023; ref: QME23.0042). The researchers plan to publish the results in peer-reviewed journals and present findings at academic conferences.

'Joining the Dots: Linking Prenatal Drug Exposure to Childhood and Adolescence' - research protocol of a population cohort study.

INTRODUCTION: Prenatal drug exposure (PDE) is one of the most important causes of child harm, but comprehensive information about the long-term outcomes of the families is difficult to ascertain. The Joining the Dots cohort study uses linked population data to understand the relationship between services, therapeutic interventions and outcomes of children with PDE. METHODS AND ANALYSIS: Information from routinely collected administrative databases was linked for all births registered in New South Wales (NSW), Australia between 1 July 2001 and 31 December 2020 (n=1 834 550). Outcomes for seven mutually exclusive groups of children with varying prenatal exposure to maternal substances of addiction, including smoking, alcohol, prescription/illicit drugs and neonatal abstinence syndrome will be assessed. Key exposure measures include maternal drug use type, maternal social demographics or social determinants of health, and maternal physical and mental health comorbidities. Key outcome measures will include child mortality, academic standardised testing results, rehospitalisation and maternal survival. Data analysis will be conducted using Stata V.18.0. ETHICS AND DISSEMINATION: Approvals were obtained from the NSW Population and Health Services Research Ethics Committee (29 June 2020; 2019/ETH12716) and the Australian Capital Territory Health Human Research Ethics Committee (11 October 2021; 2021-1231, 2021-1232, 2021-1233); and the Aboriginal Health and Medical Research Council (5 July 2022; 1824/21), and all Australian educational sectors: Board of Studies (government schools), Australian Independent Schools and Catholic Education Commission (D2014/120797). Data were released to researchers in September 2022. Results will be presented in peer-reviewed academic journals and at international conferences. Collaborative efforts from similar datasets in other countries are welcome.

[Blood cadmium level and the change of cadmium exposure risk in Chinese urban school-age children in 2002 and 2012].

OBJECTIVE: To investigate the blood cadmium concentrations and the related change in Chinese urban children derived from the China Nutrition and Health Survey 2002 and 2012(CNHS 2002 and CNHS 2012). METHODS: The Chinese urban children aged 6-11 years were selected according to gender, age and regional distribution using the multi-stage stratified cluster random sampling method, as well as the corresponding whole blood samples. The blood cadmium concentration was carefully determined by the quadrupole inductively coupled plasma mass spectrometry(ICP-MS) and the percentage of blood cadmium over 2 µg/L was subsequently estimated. In addition, the upper limit values of the 95%CI of the 95th percentiles of available blood cadmium data was assessed as the threshold of cadmium exposure. RESULTS: Totally, 2182 Chinese urban children were included, and of these, 1036 children were from the CNHS 2002 and 1146 children were from the CNHS 2012. From the CNHS 2002 to the CNHS 2012, the median blood cadmium concentration was increased from 0.28 µg/L to 0.95 µg/L, and the percentage of blood cadmium with over 2 µg/L was elevated from 1.45% to 10.47%. In addition, the new estimated threshold of blood cadmium was ascended from 1.24 µg/L up to 2.89 µg/L. CONCLUSION: The risk of cadmium exposure in Chinese urban children aged 6-11 years was increasingly aggravated from the CHNS 2002 to the CNHS 2012.

Is more always better? Effectiveness of constraint-induced movement therapy in children with high-risk or unilateral cerebral palsy (0-6 years): Systematic review and meta-analysis.

BACKGROUND: While constraint-induced movement therapy is strongly recommended as an intervention for infants with unilateral cerebral palsy, the optimal dosage remains undefined. This systematic review aims to identify the most effective level of intensity of constraint-induced movement therapy to enhance manual function in infants at high risk of asymmetric brain lesions or unilateral cerebral palsy diagnosis. METHODS: This systematic review with meta-analysis encompassed a comprehensive search across four electronic databases to identify articles that met the following criteria: randomised controlled trials, children aged 0-6 with at high risk or with unilateral cerebral palsy, and treatment involving constraint-induced movement therapy for upper limb function. Studies with similar outcomes were pooled by calculating the standardised mean difference score for each subgroup, and subgroups were stratified every 30 h of total intervention dosage (30-60, 61-90, >90 h). Risk of bias was assessed with Cochrane Collaboration's tool. RESULTS: Seventeen studies were included. Meta-analyses revealed significant differences among subgroups. The 30-60 h subgroup showed a weak effect for spontaneous use of the affected upper limb during bimanual performance, grasp function, and parents' perception of how often children use their affected upper limb. Additionally, this subgroup demonstrated a moderate effect for the parents' perception of how effectively children use their affected upper limb. CONCLUSIONS: Using a dosage ranging from 30 to 60 h when applying a constraint-induced movement therapy protocol holds promise as the most age-appropriate and cost-effectiveness approach for improving upper limb functional outcomes and parent's perception.

Systematic review on efficacy and safety of empirical versus pre-emptive antifungal therapy among children with febrile neutropenia reveals paucity of data.

BACKGROUND: Two approaches are used to manage invasive fungal disease (IFD) in febrile neutropenic patients viz. empirical therapy (without attempting to confirm the diagnosis), or pre-emptive therapy (after screening tests for IFD). OBJECTIVE: This systematic review was undertaken to compare these approaches in children. METHODS: We searched PubMed, EMBASE, Cochrane Library, Scopus, Web of Science, CINAHL, Clinical Trial Registries and grey literature, for randomized controlled trials (RCT) comparing empirical versus pre-emptive antifungal therapy in children with FN suspected to have IFD. We used the Cochrane Risk of bias 2 tool for quality assessment, and evaluated the certainty of evidence using the GRADE approach. RESULTS: We identified 7989 citations. Stepwise screening identified only one relevant RCT that administered empirical (n = 73) or pre-emptive (n = 76) antifungal therapy. There were no significant differences in all-cause mortality (RR 1.56, 95% CI: 0.46, 5.31), IFD mortality (RR 1.04, 95% CI:0.15, 7.20) and other clinically important outcomes such as duration of fever, duration of hospitalization and proportion requiring ICU admission. There were no safety data reported. The number of days of antifungal therapy was significantly lower in the pre-emptive therapy arm. The certainty of evidence for all outcomes was 'moderate'. CONCLUSIONS: This systematic review highlighted the paucity of data, comparing empirical versus pre-emptive antifungal therapy in children with febrile neutropenia having suspected invasive fungal disease. Data from a single included trial suggests that both approaches may be comparable in research settings. Robust trials are warranted to address the gap in existing knowledge about the optimal approach in clinical practice.

Pediatric donor heart utilization variability among organ procurement organizations.

BACKGROUND: Organ procurement organizations (OPOs) are responsible for the medical management of organ donors. Given the variability in pediatric donor heart utilization among OPOs, we examined factors that may explain this variability, including differences in donor medical management, organ quality, and candidate factors. METHODS: The Organ Procurement and Transplant Network database was queried for pediatric (<18 years) heart donors and candidates receiving pediatric donor heart offers from 2010 to 2019. OPOs were stratified by pediatric donor heart utilization rate, and the top and bottom quintiles were compared based on donor management strategies and outcomes. A machine learning algorithm, combining 11 OPO, donor, candidate, and offer variables, was used to determine factors most predictive of whether a heart offer is accepted. RESULTS: There was no clinically significant difference between the top and bottom quintile OPOs in baseline donor characteristics, distance between donor and listing center, management strategies, or organ quality. Machine learning modeling suggested neither OPO donor management nor cardiac function is the primary driver of whether an organ is accepted. Instead, number of prior donor offer refusals and individual listing center receiving the offer were two of the most predictive variables of organ acceptance. CONCLUSIONS: OPO clinical practice variation does not seem to account for the discrepancy in pediatric donor heart utilization rates among OPOs. Listing center acceptance practice and prior number of donor refusals seem to be the important drivers of heart utilization and may at least partially account for the variation in OPO heart utilization rates given the regional association between OPOs and listing centers.

Fertility and self-rated health of migrant women of childbearing age-an analysis of moderating effects based on socioeconomic status.

BACKGROUND: As fertility rates continue to decline and negative population growth emerges, China has sequentially introduced encouraging fertility policies to raise fertility levels. The impact of fertility on women's health remains inconclusive. It is essential to explore further the correlation between fertility and the health status of 113 million migrant women of childbearing age in China. OBJECTIVE: To investigate how fertility affects the health status of migrant women of childbearing age and determine if migrant women's socioeconomic status plays a moderating role in this process. METHODS: Using a nationally representative dataset from the 2018 China Migrants Dynamic Survey (CMDS), we examined the effects of fertility on the self-rated health of migrant women of childbearing age. An ordinary least squares regression model with moderating effects was used for the empirical study, and robustness tests were conducted based on the ordered probit model and propensity score matching to address endogeneity. RESULTS: The empirical results indicated that a rise in the number of children born significantly reduces the self-rated health of migrant women of childbearing age. An increase in years of schooling and household income can significantly mitigate the negative impact of childbearing on the health of migrant women. The robustness of the above results was validated through alternative models and propensity score matching (PSM) methods. The heterogeneity analysis revealed that fertility exerts a negative impact on the health status of migrant women with rural household registration and on the health status of inter-provincial and inter-city migrant women. Further investigation found that the occurrence of childbirth during migration and an increase in the number of girls significantly negatively impacted the health status of migrant women. In contrast, the increase in the number of boys did not show a significant effect. Improving the health of migrant women of childbearing age significantly positively impacted their future childbearing intentions. CONCLUSIONS: Migrant women of childbearing age bear the dual burden of migration and childbirth. Our findings showed the rise in the number of children born and the occurrence of childbirth during migration posed greater challenges to the health status of female migrants, particularly among those with lower socioeconomic status. Government and community efforts for enhancing health among migrant women of childbearing age are recommended.

An anthropological history of Nepal's Female Community Health Volunteer program: gender, policy, and social change.

BACKGROUND: Community health workers (CHWs) are central to Primary Health Care globally. Amidst the current flourishing of work on CHWs, there often is a lack of reference to history-even in studies of programs that have been around for decades. This study examines the 35-year trajectory of Nepal's Female Community Health Volunteers (FCHVs). METHODS: We conducted a content analysis of an archive of primary and secondary research materials, grey literature and government reports collected during 1977-2019 across several regions in Nepal. Documents were coded in MAXQDA using principles of inductive coding. As questions arose from the materials, data were triangulated with published sources. RESULTS: Looking across four decades of the program's history illuminates that issues of gender, workload, and pay-hotly debated in the CHW literature now-have been topics of discussion for observers and FCHVs alike since the inception of the program. Following experiments with predominantly male community volunteers during the 1970s, Nepal scaled up the all-female FCHV program in the late 1980s and early 1990s, in part because of programmatic goals focused on maternal and child health. FCHVs gained legitimacy as health workers in part through participation in donor-funded vertical campaigns. FCHVs received a stable yet modest regular stipend during the early years, but since it was stopped in the 1990s, incentives have been a mix of activity-based payments and in-kind support. With increasing outmigration of men from villages and growing work responsibilities for women, the opportunity cost of health volunteering increased. FCHVs started voicing their dissatisfaction with remuneration, which gave rise to labor movements starting in the 2010s. Government officials have not comprehensively responded to demands by FCHVs for decent work, instead questioning the relevance of FCHVs in a modern, medicalized Nepali health system. CONCLUSIONS: Across public health, an awareness of history is useful in understanding the present and avoiding past mistakes. These histories are often not well-archived, and risk getting lost. Lessons from the history of Nepal's FCHV program have much to offer present-day debates around CHW policies, particularly around gender, workload and payment.

Anesthesia management for a child with the Koolen-de Vries syndrome: a case report.

BACKGROUND: The Koolen-de Vries syndrome (KdVS) is a relatively new rare disease caused by micro-deletion of 17q21.31 which was first reported by Koolen in 2006. Typical phenotypes for KdVS include hypotonia, developmental delay, moderate intellectual disability, and characteristic facial dysmorphism. Up to now, there was only one case report about anesthesia management of patient diagnosed KdVS. It was a 2-year-old girl who experienced an MRI exam under anesthesia. CASE PRESENTATION: We described a 21-month-old boy who planned to undergo an orchidopexy under general anesthesia diagnosed with KdVS. He had an intellectual disability, characteristic facial dysmorphism, tracheo/laryngomalacia, patent foramen ovale, and cryptorchidism related to KdVS. Due to the complex condition especially the presence of tracheo/laryngomalacia, we took some special measures, including reducing the amount of long-acting opioid, keeping the spontaneous breath, performing a caudal block, and applying the laryngeal mask. But the laryngeal mask was changed to an endotracheal tube because it failed to provide adequate ventilation. The boy experienced mild laryngeal spasm and hypoxia after extubation, but lateral position and etomidate eased his breathing problem and re-intubation was avoided. It is indicated that anesthesia management for patients with orphan disease is a real challenge for all anesthesia providers. CONCLUSIONS: The Koolen-de Vries syndrome is a relatively new orphan disease involving multiple systems. Keeping spontaneous breath, evaluating airway potency to anesthetics, applying endotracheal tube, and post-extubation lateral or prone position may be helpful for airway management for patient with hypotonia and tracheo/laryngomalacia. KdVS patient needs prolonged post-anesthesia monitoring and/or medication for airway complications.

Single-port robotic-assisted laparoscopic synchronous surgery in pediatric patent processus vaginalis.

PURPOSE: Patent processus vaginalis (PPV) is usually observed in pediatric abdominal surgery; however, robotic single-port surgery in repairing processus vaginalis has not been reported in children. Herein, we present our clinical experiences in single-port robotic surgeries for PPV repair to evaluate both efficacy and safety. METHODS: Retrospective analysis of patients underwent single-port robotic-assisted laparoscopic surgery for genitourinary diseases from May 2020 and May 2023 in our center. Among these patients, 21 children had PPV repaired at the same time. The case characteristics and follow-up data were recorded. RESULTS: Twenty-one of the 53 children were found to have PPV during genitourinary surgery. The simultaneous treatment of the primary disease and PPV with a single-port robotic-assisted platform was both convenient and safe. There was no significant increase in total operation time, and no excessive intraoperative hemorrhage was observed in any of the operations. There were no complications observed on follow-up. CONCLUSION: With a high incidence of PPV in children, a single-port robotic-assisted procedure is feasible and effective if simultaneously performed when addressing a primary abdominal disease.

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death.

Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (Interquartile Range 21-35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC/year in 17.9%, and stroke in 13.8%. At the terminal admission, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest X-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test). All patients received antibiotics, whereas, 95.9% and 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.

Influenza antibody breadth and effector functions are immune correlates from acquisition of pandemic infection of children.

Cross-reactive antibodies with Fc receptor (FcR) effector functions may mitigate pandemic virus impact in the absence of neutralizing antibodies. In this exploratory study, we use serum from a randomized placebo-controlled trial of seasonal trivalent influenza vaccination in children (NCT00792051) conducted at the onset of the 2009 H1N1 pandemic (pH1N1) and monitored for infection. We found that seasonal vaccination increases pH1N1 specific antibodies and FcR effector functions. Furthermore, prospective baseline antibody profiles after seasonal vaccination, prior to pH1N1 infection, show that unvaccinated uninfected children have elevated ADCC effector function, FcγR3a and FcγR2a binding antibodies to multiple pH1N1 proteins, past seasonal and avian (H5, H7 and H9) strains. Whereas, children that became pH1N1 infected after seasonal vaccination have antibodies focussed to seasonal strains without FcR functions, and greater aggregated HA-specific profiles for IgM and IgG3. Modeling to predict infection susceptibility, ranked baseline hemagglutination antibody inhibition as the highest contributor to lack of pH1N1 infection, in combination with features that include pH1-IgG1, H1-stem responses and FcR binding to seasonal vaccine and pH1 proteins. Thus, seasonal vaccination can have benefits against pandemic influenza viruses, and some children already have broadly reactive antibodies with Fc potential without vaccination and may be considered 'elite influenza controllers'.

Lip Print Pattern among Children Visiting Dental Out patient department.

BACKGROUND: Lip print patterns are unique in every individual. The uniqueness of lip print pattern assists in the personal identification. The objectives of the present study were to study the different lip print patterns among the children visiting Kanti Children's Hospital, and to compare the distribution of lip print patterns based on gender and race. METHODS: A cross-sectional study was conducted among 300 children visiting the Dental Outpatient Department of Kanti Children's Hospital, Kathmandu, Nepal. Convenience sampling method was used to choose the study subjects. Digital photography method was used to record the image of lips. RESULTS: The study showed 'Long vertical' (Type I) as the most frequent (41%) type of lip print pattern, whereas 'Unspecified' (Type V) was the least frequent (2.7%) type. Prevalence of 'Type I' pattern was significantly higher in males as compared to females (p=0.007) whereas prevalence of 'Type IV' pattern was significantly higher in females as compared to males (p=0.006). 'Type I' pattern was the commonest lip print pattern among both Caucasians and Mongolian, whereas 'Type V' (2%) and 'Type IV' (3.7%) were the least common lip print patterns in Caucasians and Mongolian children respectively. CONCLUSIONS: 'Long vertical' (Type I) was the most common lip print pattern among the children visiting the Dental Outpatient Department of Kanti Children's Hospital, Kathmandu, Nepal. Nevertheless, in higher or lesser numbers, all kinds of lip print patterns as described by Suzuki and Tsuchihashi were seen in the study population.

Clinical profile of functional constipation in Saudi children.

BACKGROUND: Functional constipation (FC) is a common condition in children, and information on the clinical characteristics of FC in Saudi children is scarce. OBJECTIVE: Describe the clinical profile of FC in Saudi children. DESIGN: Retrospective. SETTING: Hospital that provides primary, intermediate and tertiary care. PATIENTS AND METHODS: All children diagnosed with FC according to the Rome IV criteria were included and had at least one follow-up clinic visit. Demographic and clinical data collected from medical records included the age at onset, duration of constipation, clinical features, treatment modalities, and factors associated with clinical response. Descriptive statistics and Pearson's chi-squared test were used in the statistical analysis to see how categorical study variables were linked to clinical response. A P value of ≤.05 was used to report statistical significance. MAIN OUTCOME MEASURE: Compliance and clinical response to polyethylene glycol (PEG) compared with lactulose. SAMPLE SIZE: 370 children from 0.1 to 13 years of age. RESULTS: The median (IQR) age of onset was 4 (5) years and less than one year in 14%. The median (IQR) duration of constipation was 4 months (11) and less than two months in 93/370 (25%). Abdominal pain was the most commonly associated feature (44%). Screening for celiac disease and hypothyroidism was negative. A Fleet enema was the most common disimpaction method (54%) and PEG was the most common maintenance medication (63.4%). PEG was significantly better tolerated (P=.0008) and more effective than lactulose (P<.0001). Compliance was the only variable significantly associated with clinical response. CONCLUSIONS: PEG was better tolerated and more effective than lactulose in our study, a finding in agreement with the literature. Therefore, PEG should be the drug of choice in the initial management of FC in Saudi children. Prospective studies on the causes of noncompliance are needed to improve the response to treatment. LIMITATIONS: The limitations of retrospective design are missing data, recall bias, and hospital-based limitation, such as missing milder cases treated at the outpatient level. However, the sample size of 370 may have minimized these limitations.

Barodontalgia during hyperbaric oxygen therapy of an 8-year-old male: A case report.

Barodontalgia, barometric pressure-induced dental pain, may occur during hyperbaric oxygen(HBO2) therapy due to pressure changes. This case report presents an 8-year-old male patient with barodontalgia. The patient declared a severe toothache during HBO2 therapy. The diving medicine specialist referred the patient to the dental clinician immediately. On clinical examination, the pain was thought to be caused by caries lesions of the deciduous teeth in the left maxillary molar region. Tooth extraction was suggested. After extraction, the patient continued hyperbaric oxygen therapy sessions without any pain. The patient was recommended for an intraoral and radiographic examination session one week after the extraction. In conclusion, caries lesions and faulty restorations should be examined before hyperbaric oxygen therapy sessions. Even though barodontalgia is a rare phenomenon, dental examination is essential to avoid these kinds of pain-related complications. All carious lesions and defective restorations must be treated, if necessary. Removal of faulty restorations and management of inflammation as part of the treatment is suggested before exposure to pressure changes.

Metabolomics of cerebrospinal fluid reveals candidate diagnostic biomarkers to distinguish between spinal muscular atrophy type II and type III.

AIMS: Classification of spinal muscular atrophy (SMA) is associated with the clinical prognosis; however, objective classification markers are scarce. This study aimed to identify metabolic markers in the cerebrospinal fluid (CSF) of children with SMA types II and III. METHODS: CSF samples were collected from 40 patients with SMA (27 with type II and 13 with type III) and analyzed for metabolites. RESULTS: We identified 135 metabolites associated with SMA types II and III. These were associated with lysine degradation and arginine, proline, and tyrosine metabolism. We identified seven metabolites associated with the Hammersmith Functional Motor Scale: 4-chlorophenylacetic acid, adb-chminaca,(+/-)-, dodecyl benzenesulfonic acid, norethindrone acetate, 4-(undecan-5-yl) benzene-1-sulfonic acid, dihydromaleimide beta-d-glucoside, and cinobufagin. Potential typing biomarkers, N-cyclohexylformamide, cinobufagin, cotinine glucuronide, N-myristoyl arginine, 4-chlorophenylacetic acid, geranic acid, 4-(undecan-5-yl) benzene, and 7,8-diamino pelargonate, showed good predictive performance. Among these, N-myristoyl arginine was unaffected by the gene phenotype. CONCLUSION: This study identified metabolic markers are promising candidate prognostic factors for SMA. We also identified the metabolic pathways associated with the severity of SMA. These assessments can help predict the outcomes of screening SMA classification biomarkers.