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Purpose The role of chemotherapy (CT) and radiotherapy (RT) for management of extraskeletal osteosarcoma (ESOS) remains controversial. We examined disease outcomes for ESOS patients and investigated the association between CT/RT with recurrence and survival. Patients and methods Retrospective review at 25 international sarcoma centers identified patients ≥18 years old treated for ESOS from 1971 to 2016. Patient/tumour characteristics, treatment, local/systemic recurrence, and survival data were collected. Kaplan–Meier survival and Cox proportional-hazards regression and cumulative incidence competing risks analysis were performed. Results 370 patients with localized ESOS treated definitively with surgery presented with mainly deep tumours (n = 294, 80%). 122 patients underwent surgical resection alone, 96 (26%) also received CT, 70 (19%) RT and 82 (22%) both adjuvants. Five-year survival for patients with localized ESOS was 56% (95% CI 51%–62%). Almost half of patients (n = 173, 47%) developed recurrence: local 9% (35/370), distant 28% (102/370) or both 10% (36/370). Considering death as a competing event, there was no significant difference in cumulative incidence of local or systemic recurrence between patients who received CT, RT, both or neither (local p = 0.50, systemic p = 0.69). Multiple regression Cox analysis showed a significant association between RT and decreased local recurrence (HR 0.46 [95% CI 0.26–0.80], p = 0.01). Conclusion Although the use of RT significantly decreased local recurrences, CT did not decrease the risk of systemic recurrence, and neither CT, nor RT nor both were associated with improved survival in patients with localized ESOS. Our results do not support the use of CT; however, adjuvant RT demonstrates benefit in patients with locally resectable ESOS.

To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normalweight and overweight schoolchildren. Cross-sectional study. Thirteen private schools in urban Faridabad, Haryana. 961 school children aged 5-10 years. Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing. Prevalence of fatty liver on ultrasound, and NAFLD and its association with biochemical abnormalities and demographic risk factors. On ultrasound, 215 (22.4%) children had fatty liver; 18.9% in normal-weight and 45.6% in overweight category. Presence and severity of fatty liver disease increased with body mass index (BMI) and age. Among the children with NAFLD, elevated SGOT and SGPT was observed in 21.5% and 10.4% children, respectively. Liver enzyme derangement was significantly higher in overweight children (27% vs 19.4% in normal-weight) and severity of fatty liver (28% vs 20% in mild fatty liver cases). Eleven (8.1%) children with NAFLD had metabolic syndrome. Higher BMI (OR 35.9), severe fatty liver disease (OR 1.7) and female sex (OR 1.9) had strong association with metabolic syndrome. 22.4% of normal-weight and overweight children aged 5-10 years had fatty liver. A high proportion (18.9%) of normal-weight children with fatty liver on ultrasound indicates the silent burden in the population.

Background Fear of autism has led to a decline in childhood-immunization uptake and to a resurgence of preventable infectious diseases. Identifying characteristics of parents who believe in a causal role of vaccines for autism spectrum disorder (ASD) in their child may help targeting educational activities and improve adherence to the immunization schedule. Objectives To compare caregivers of children with ASD who agree or disagree that vaccines play an etiological role in autism for 1) socio-demographics characteristics and 2) developmental and clinical profiles of their children. Methods Data from 16,525 participants with ASD under age 18 were obtained from SPARK, a national research cohort started in 2016. Caregivers completed questionnaires at registration that included questions on beliefs about the etiologic role of childhood immunizations and other factors in ASD. Data were available about family socio-demographic characteristics, first symptoms of autism, developmental regression, co-occurring psychiatric disorders, seizures, and current levels of functioning. Results Participants with ASD were 80.4% male with a mean age of 8.1 years (SD = 4.1). Overall, 16.5% of caregivers endorsed immunizations as perceived causes of autism. Compared to caregivers who disagreed with vaccines as a cause for ASD, those who believed in vaccine causation came disproportionately from ethnic minority, less educated, and less wealthy backgrounds. More often their children had experienced developmental regression involving language and other skills, were diagnosed earlier, had lost skills during the second year of life, and had worse language, adaptive, and cognitive outcomes. Conclusion One in six caregivers who participate in a national research cohort believe that child immunizations could be a cause of autism in their child. Parent social background (non-White, less educated) and child developmental features (regression in second year, poorer language skills, and worse adaptive outcomes) index caregivers who are more likely to harbor these beliefs and could benefit from targeted educational activities.

Objective To evaluate first-trimester screening protocols for detecting trisomy 21 in an Indian population. Methods The present prospective study collected data from women with singleton pregnancies and a crown-to-rump length of 45–84 mm who presented at the fetal medicine unit of a tertiary care center in North India between June 1, 2006, and December 31, 2015, for combined first-trimester screening. Maternal age, nuchal translucency, nasal bone, and maternal serum levels of free beta human chorionic gonadotropin and pregnancy-associated plasma protein A were assessed for calculating the risk of trisomy 21. Tricuspid regurgitation and qualitative analysis of ductus venosus data were available from June 2010, and were included where available. Trisomy-21 detection rates were calculated for various screening protocols and were compared. Results There were 4523 women screened and 24 records of trisomy 21. Combined screening with maternal age, nuchal translucency, nasal bone, tricuspid regurgitation, and ductus venosus demonstrated optimal detection and false-positive rates of 93.8% and 1.9%, respectively. Screening using only maternal age yielded a detection rate of 37.5%; using fixed nuchal translucency cut-off values of 2.5 mm and 3 mm resulted in detection rates of 66.7% and 37.5%, respectively. Conclusion Combined first-trimester screening performed well in an Indian population; combining maternal age, nuchal translucency, nasal bone, ductus venosus, and tricuspid regurgitation yielded the most accurate screening. This article is protected by copyright. All rights reserved.

Background and Objective: To determine posterior capsule opacification (PCO) rate and identify contributory factors in a series of patients undergoing combined 23-gauge transconjunctival phacovitrectomy. Patients and Methods: Retrospective data on 221 consecutive patients operated on by a single senior surgeon using a standard technique were collected and subjected to univariate analysis with chi-square tests and multivariate analysis with logistic regression. Results: PCO developed in 46 of 221 cases (20.8%). Major risk factors were: rhegmatogenous retinal detachment (relative risk = 3.3, P = .002), axial length greater than 24.5 mm (relative risk = 2.0, P = .093), intraoperative/postoperative complications (relative risk = 3.3, P = .04), C 2 F 6 tamponade (versus SF 6 /air) (relative risk = 2.8, P = .01), and postoperative posturing (relative risk = 4.1, P < .001). Multiple logistic regression analysis showed the latter two to be most important. Conclusion: PCO rate following 23-gauge phacovitrectomy is lower than most rates reported for 20-gauge phacovitrectomy. Using shorter-acting gas tamponade and avoiding postoperative posturing may help lower PCO rates.

We describe two cases of chorioretinal dysplasia and microcephaly and review the current literature regarding this topic.

Aims: Eye injuries are the leading cause of monocular blindness in children and are challenging to manage. However, limited follow-up studies currently exist. We describe the clinical characteristics and outcomes of open globe injuries presenting to a major UK centre and discuss factors affecting long-term prognosis.PurposeIdentification of (1) demographic features, (2) causes, types and location of injuries, (3) initial clinical features and correlation with visual outcome, and (4) predictors of poor visual outcome.MethodRetrospective study by reviewing and analysing records of 20 patients, aged 16 years and below, who had undergone repair of an open globe injuries at the Manchester Royal Eye Hospital, UK. Data recorded included demographic data, mechanisms and location of injury, visual acuity, clinical signs on presentation, surgical procedures, postoperative complications, and final visual acuity.ResultsThe study group comprised of 85% male subjects, 15% female subjects. The average age was 9.8 years (range: 1-15). Average follow-up was 16.3 months (range: 3-48 months). Sharp objects (mainly glass or knife) accounted for the majority (65%) of injuries. Initial clinical signs associated with poor visual outcomes included poor initial visual acuity, cataract, RAPD, and no initial red reflex. Younger patients and blunt injuries (especially BB gun injuries) had worse visual outcomes.ConclusionFactors suggesting unfavourable final visual acuity after open globe injuries in children are (1) young age at presentation, (2) poor initial visual acuity, (3) presence of a relative afferent papillary defect, (4) absence of red reflex, (5) cataract, and (6) types and number of surgeries performed.Awareness and education with regard to prevention of paediatric injuries need to be actively implemented. Language: en

With a view to evaluating the putative involvement of cytokine gene variants in human essential hypertension, we carried out an association (case-control) study on 174 unrelated nationals (81 hypertensives and 93 normotensives) from the Abu Dhabi Emirate (UAE), a genetically homogeneous population also characterised by the absence of traditional confounding factors such as alcohol consumption and smoking. To that end, we targeted our investigation to five candidate gene loci-transforming growth factor beta1 (TGF-beta1), interferon gamma (IFN-gamma), epidermal growth factor (EGF), interleukin-1 beta (IL-1beta) and tumour-necrosis factor (TNF-alpha) genes. We investigated the distribution of genotypes and alleles of the six following dimorphic variants: TGF-beta1(*)10(T>C) and TGF-beta1(*)25(G>C), located at codons 10 and 25, respectively, of TGF-beta1; T874A in intron 1 of IFN-gamma; G61A in exon 1 of EGF; TaqI dimorphism at +3962 (exon 5) of IL-1beta; and -308A>G in the promoter of TNF-alpha. These six bi-allelic markers were visualised by methods based on the techniques of amplification refractory mutation system-polymerase chain reaction (for TGF-beta1, IFN-gamma, EGF and TNF-alpha) and by polymerase chain reaction-TaqI restriction endonuclease analysis in the case of IL-1beta. In each of the two groups (normotensives and hypertensives), genotype frequencies of all six markers occurred in Hardy-Weinberg proportions. There were, however, no statistical differences in the allele and genotype frequencies of any of the six markers between the two groups of subjects: TGF-beta1(*)10C frequencies were 0.46 and 0.49 (chi(2)=0.61; 2 d.f.; P=0.74) and TGF-beta1(*)25C were 0.07 and 0.08 (chi(2)=0.61; 2 d.f.; P=0.74) amongst normotensives and hypertensives, respectively; p(IFN-gamma(*)A874) were 0.41 in normotensives versus 0.46 in hypertensives (chi(2)=3.07; 2 d.f.; P=0.22); p(EGF (*)G61) were 0.51 versus 0.58 (chi(2)=1.76; 2 d.f.; P=0.41); p[IL-1beta (*)TaqI(+)] were 0.43 versus 0.36 (chi(2)=2.08; 2 d.f.; P=0.35); and p(TNF-alpha(*)-308G) were 0.80 versus 0.85 (chi(2)=1.29; 2 d.f.; P=0.53). There was also no difference in distribution and frequencies of haplotypes constructed with combinations of TGF-beta1(*)10(T>C) and TGF-beta1(*)25(G>C) sites. However, although they do not reach statistical significance (which may be due to the relatively restricted number of subjects included in this study), the distribution differences (in normotensives and hypertensives) observed in the cases of EGF and TNF-alpha reflect trends that could be expected from a mechanistic explanation of the pathways that underlie the patho-physiology of hypertension.

Cervical cancer screening by Papanicolaou (Pap) smear lowers the incidence and provides early detection of cervical cancer and is a preventative health care measure that should be available on a regular basis to all women at risk. As the population of ethnic women increases, it is important to assess whether these women are aware of and are utilizing this health service. A self-administered questionnaire was completed by 124 South Asian women, aged 18 to 60 years. Sixty-two South Asian students were selected from a university setting and 62 Tamil women were selected from a community center for South Asian women. This study examined the knowledge and use of Pap smears in South Asian women in Canada, and whether their level of acculturation or formal education influenced whether they know about and receive Pap smears. Low level of knowledge about the Pap test and a low prevalence of Pap testing behaviour was significantly correlated (p < 0.001) with a low level of formal education, education taking place outside of Canada, and a low index of acculturation. South Asian students were significantly more acculturated than Tamil women (p < 0.001). In conclusion, there was a low level of knowledge and prevalence of cervical cancer screening among South Asian women. This study highlights the need for educational interventions in Canada directed to ethnic women especially from South Asia.

Advancements in childhood cancer treatment have led to increasing survivorship, creating a greater emphasis on long-term management of patients, including quality of life and side effects from therapy; foremost of which is preserving fertility. The American Society of Clinical Oncology (ASCO) recently revised their guidelines and recommend fertility preservation options be discussed at the earliest possible opportunity for newly diagnosed patients, including methods available for children that remain investigational. Herein, we discuss the current barriers to and the impact of these guidelines for pediatric oncologists caring for young female patients, and provide some suggestions on how to approach this complicated topic.

The objective of this study was to explore the usability of a bilingual (English and French) Internet-based self-management program for adolescents with cancer and their parents and refine the Internet program. A qualitative study design with semistructured, audio-taped interviews and observation was undertaken with 4 iterative cycles. A purposive sample of English-speaking and French-speaking adolescents with cancer and one of their parents/caregivers was recruited. Adolescents and parents provided similar feedback on how to improve the usability of the Internet program. Most changes to the website were completed after the initial cycles of English and French testing. Both groups also found information presented on the website to be appropriate, credible, and relevant to their experiences of going through cancer. Participants reported the program would have been extremely helpful when they were first diagnosed with cancer. Usability testing uncovered some issues that affected the usability of the website that led to refinements in the online program.

Optic disc asymmetry is a common finding. In the majority of cases automated visual fields are normal. However, in a minority, a visual field defect may lead to a diagnosis of normal tension glaucoma. Rarely, the optic discs and visual field deteriorate irrespective of the normal or reduced intraocular pressure.

The presence of coronary artery calcium is closely associated with the presence of atherosclerotic lesions in the coronary vasculature. Detection of coronary calcium by imaging techniques has evolved over the last few decades and has become especially more sophisticated with advanced imaging technology. Whereas the status of coronary artery calcium as a marker of increased cardiovascular risk is well established, the indication for testing continues to be a topic of debate.

Thyroid cancer (TC) is an uncommon childhood malignancy, but the incidence may be increasing. Recent American Thyroid Association guidelines focus primarily on adult data. Natural history studies of TC in childhood are important to determine outcomes. The objectives of this study were to describe the demographics and outcomes in children with TC treated at The Hospital for Sick Children, Toronto, from 1983 to 2006. We hypothesized that childhood TC was increasing at our institution.Cases of papillary TC (PTC) (including follicular variant PTC) and follicular TC (FTC) were identified from pathology databases. Chart review was performed, and data were extracted on clinical, treatment, and outcome variables.Sixty-one cases were identified, and complete data were available in 54, including 36 girls and 18 boys. There was no statistical change in numbers of cases diagnosed yearly during the study period. Younger children were more likely to have metastases at presentation or during follow-up. Pathological TC diagnosis included 40 PTC, 1 diffuse-sclerosing papillary, 7 follicular variant PTC, and 6 FTC. There was no difference in pathology findings between children less than or greater than 10 years old. Five patients had a history of previous malignancy, and five had a history of previous thyroid conditions. Three patients were born in areas of high TC endemnicity. Twenty-three patients had thyroiditis on pathology examination. All patients underwent total thyroidectomy, and 53/54 patients received therapeutic radioactive iodine ablation. Twenty-seven patients had metastases at presentation (19 lymph nodes only, 2 lung only, and 6 lymph node and distant) and 6 developed distant metastases during follow-up (3 lung, 2 thymus, and 1 paraspinal). Male sex was associated with development of metastases during follow-up. On multiple regression, tumor size was predicted positively by PTC but not by age, sex, or metastases at presentation or during follow-up.We did not find evidence of increasing numbers of cases of TC diagnosed yearly during the study period, or difference in tumor aggressiveness, or between outcomes in children aged less than or greater than 10 years. Children with metastases at presentation or during follow-up were likely to be younger than children without metastases. There is a need for prospective, collaborative multicenter studies of TC.

We describe the use of intravitreal triamcinolone in the treatment of macular edema associated with systematic lupus erythematosus providing short-term resolution of cystoid macular edema and increase in visual acuity. No side effects from the injection have been seen as yet. We suggest this treatment to be considered in patients for whom laser treatment may be inappropriate or not feasible, or even as an alternative/safer option for all patients.

Previously we have shown decreased Fas-mediated apoptosis in cord blood lymphocyte subsets. In this study, we compared tumor necrosis factor (TNF)-alpha-induced apoptosis in T lymphocytes and their subsets between cord blood and peripheral blood from healthy young controls. The expression of TNF receptor I (TNFR-I) was assessed by flow cytometry and quantitative RT-PCR. The expression of adapter molecules TNF receptor-associated death domain (TRADD), Fas-associated death domain (FADD) and TNF-associated factor-2 (TRAF-2) and caspase 3 was analyzed by Western blotting. The activity of caspase 3 and caspase 8 was measured by colorimetric assay. The susceptibility of CD4+ and CD8+ T cells to TNF-alpha-induced apoptosis was measured by terminal deoxytidyl transferase-mediated dUTP nick end labelling (TUNEL) assay. Both CD4+ and CD8+ T cell subsets from cord blood demonstrated decreased susceptibility to TNF-alpha-induced apoptosis that was associated with decreased activation of both caspase 8 and caspase 3 as compared to T cell subsets in peripheral blood. Furthermore, expression of TNFR-I, TRADD and caspase 3 was decreased in cord blood lymphocytes as compared to peripheral blood lymphocytes. The significance of these observations is discussed.

Zinc copper magnesium and calcium content of preterm and term milk from Indian mothers was analyzed at different periods of lactation to examine its adequacy as a source of nutrients for preterm infants. Breast milk samples were obtained from 14 mothers who gave birth to preterm infants (mean gestation--35 weeks) and from 50 mothers who gave birth to full term infants (mean gestation--39 weeks) after a normal pregnancy. The levels of zinc copper magnesium and calcium are given for colostrum transitional and mature milk. Although the mean levels of all these elements were higher in breast milk of term mothers the difference is not statistically significant. A small preterm infant weighing 1000 grams would receive about 180 ml of milk a day. Highest and lowest daily concentrations of minerals which would be obtained daily by a 1000 gram baby receiving 180 ml milk are shown. Also shown is recommended daily intake of minerals for a 1000 gram baby. Expressed breast milk is likely to be short of the requirement of these minerals in a premature infant and it seems reasonable to consider supplementation of minerals for Indian infants.

The stool pH and sugar were examined in 76 breast fed and 38 formula fed premature infants. The stool pH decreased gradually till 5th day of life and then increased again. Stool sugar was not more than 0.75 percent in breast fed and 0.5 percent in formula fed during first seven days of life. A premature infant with stool pH 0.5 percent after seventh day of life needs to be investigated for pathological sugar malabsorption.

Disseminated intravascular coagulation, thrombocytopenia, consumption of factors VIII and II, and antithrombin deficiency have been previously demonstrated in pre-eclampsia. However, the precise mechanism responsible for initiation of disseminated intravascular coagulation has not been elucidated. The present study documents activation of the intrinsic coagulation pathway in a patient with severe pre-eclampsia. The studies revealed marked reductions of plasma coagulant activities of all intrinsic pathway factors, i.e., XII, XI, IX, and VIII. In addition, the ratio of plasma factor XII activity to antigen concentration was markedly abnormal, and plasma high-molecular-weight kininogen concentration was diminished. It is suggested that activation of the intrinsic coagulation pathway may be operative in the genesis of disseminated intravascular coagulation in pre-eclampsia.