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There is limited information on the potential effects of repeated intrathecal antisense oligonucleotide drug delivery on cerebrospinal fluid (CSF) biochemical and blood cell profiles. This study aimed to examine longitudinal changes in the biochemical components (glucose, protein) and blood cell counts in the CSF of spinal muscular atrophy (SMA) patients treated with intrathecal nusinersen.We collected and analyzed clinical and CSF parameters (cell count, protein, glucose, culture) of 50 individuals with SMA during nusinersen treatment (22 type 1, 17 type 2, and 11 type 3).The median protein concentration at baseline and during treatment was within the normal range but rose during treatment and was significantly above baseline at the time of the ninth intrathecal injection (p = 0.02, two-tailed Wilcoxon matched-pairs test, and p = 0.0015, Friedman test for repeated measures). Further analysis showed that the increase in CSF protein concentration was evident for SMA types 2 and 3 patients, but not for type 1. This observation was also demonstrated by a significant correlation between the SMN2 gene copy number and the increase in CSF protein concentration (Spearman rank correlation test).Our results demonstrate that a delayed increase in CSF protein concentration is expected during nusinersen treatment for SMA types 2 and 3. This might reflect the medication's effect and a possible therapeutic biochemical marker.

Treatment options for inherited metabolic epilepsies are rapidly expanding with advances in molecular biology and the genomic revolution. Traditional dietary and nutrient modification, and inhibitors or enhancers of protein and enzyme function, the mainstays of therapy, are undergoing continuous revisions to increase biological activity and reduce toxicity. Enzyme replacement, and gene replacement and editing hold promise for genetically targeted treatment and cures. Molecular, imaging and neurophysiologic biomarkers are emerging as key indicators of disease pathophysiology, severity, and response to therapy.

The objective of this study was to assess the prevalence of disordered eating behaviors (DEBs) in young individuals with idiopathic intracranial hypertension (IIH) and to identify predictors of DEBs in this population.Individuals with IIH aged eight to 25 years and their matched controls responded to a self-rating survey comprised of the Eating Attitude Test-26 for assessing the presence of DEBs and the Depression, Anxiety and Stress Scale.Fifty-three subjects with IIH and 106 healthy controls were included. DEBs were significantly more prevalent in individuals with IIH (P 0.001). Individuals with IIH and DEBs were more likely to have longer periods of treatment [odds ratio: 1.07, 95% CI: 1.02-1.41), P = 0.008] and to have lost a significant amount of weight during the course of treatment [odds ratio: 9.06 (95% CI: 1.30-62.9), P = 0.026]. Depression, anxiety, and stress were more prevalent in the IIH group than in the controls (P = 0.004) and were associated with DEBs in these individuals (P = 0.01).There is an increased prevalence of DEBs among young individuals with IIH, which persists even after disease resolution, and is associated with higher reported rates of depression, anxiety, and stress. Medical caregivers should have heightened awareness to DEBs in individuals with IIH with the aim of early identification and intervention.

OBJECTIVE The Klotho protein family plays important roles in several metabolic pathways. Soluble Klotho has been recently put forward as an antiaging protein, demonstrating renal and cardiovascular protective traits. Cardiopulmonary bypass (CPB) support during cardiac surgery has been implicated in several adverse outcomes in pediatric and adult patients. Our goal was to assess whether serum Klotho levels can be used to predict outcomes in children undergoing cardiac surgery with CPB due to congenital heart defects (CHDs). METHODS This prospective study was conducted on pediatric patients admitted to two Pediatric Cardiac Intensive Care Units, between 2012 and 2018. All patients were born with CHD and underwent corrective surgery with CPB. Sequential blood samples were analyzed by enzyme-linked immunosorbent assay for soluble Klotho levels at baseline, 2, 6, and 24 h after surgery. The association between Klotho levels and several demographic, intraoperative, and postoperative clinical and laboratory parameters was studied. RESULTS Twenty-nine children undergoing cardiac surgery with CPB support were included. Serum Klotho levels were shown to significantly decrease 2 h after surgery and increase to baseline levels after 6 h (p

Pompe's disease occurs due to an autosomal recessive trait resulting from numerous distinctive mutations in the GAA gene. It manifests as a broad spectrum of clinical phenotypes with progressive weakness that impairs motor and respiratory functions being common for all its forms. Cardiac hypertrophy is a prominent feature of its classic infantile form. To date, the pathogenic variant c.2015G > A (p.Arg672Gln) in exon 14 of the GAA gene has been described in 10 children of different ethnic groups, with variable phenotypic presentations. This work describes three children from two unrelated families of Arab ethnicity who presented with infantile-onset Pompe's disease as a result of a c.2015G > A (p.Arg672Gln) mutation. The clinical course of the children we report was more severe than previous reports. This further emphasizes the lack of a strict genotype–phenotype correlation in regard to the unique c.2015G > A (p.R672Q) mutation that causes Pompe's disease. This information contributes to the knowledge of the phenotypic expression of the specific mutation c.2015G > A (p.Arg672Gln) that causes Pompe's disease.

The existing data on the neurodevelopmental outcome of children born with an isolated atretic cephalocele (IAC) are scant. We aimed to expand upon these data by describing our experience with affected children, as well as assist parents and clinicians in deciding how to proceed when an IAC is diagnosed prenatally. A follow-up study was conducted on nine children who were born with an IAC. Evaluations were performed by pediatric neurologists and child development specialists. Developmental outcomes were based on a global development evaluation that assessed gross and fine motor skills, receptive and expressive language levels, activities of daily living, communication skills, and social domains. Adaptive skills were estimated by the Adaptive Behavior Assessment System, Second Edition. None of the nine children (median age 4 years and 6 months) had abnormal findings on neurological examination. Six children had age-appropriate developmental milestones, two had a mild motor delay, and one had mild expressive language delay (catchup was achieved by all of the latter three by ~ 3.5 years of age). The mean general adaptive composite score was 105 ± 11.7 (normal = 100). None of the children had behavioral, social, or communication problems. Children diagnosed with an IAC with/without a falcine sinus and devoid of coexisting intracranial abnormalities seem to have a normal neurodevelopmental outcome. Continuation of pregnancy may be recommended when an IAC is detected prenatally, and reassurance if detected postnatally.

The clinical applicability and yield of brain magnetic resonance imaging (MRI) in the setting of an inpatient pediatric department has not been investigated. The authors performed a retrospective chart review of nontraumatic/nonneurosurgical children who underwent brain MRI during their hospitalization in a general pediatric department over a 5-year period. Of the 331 children who underwent brain MRI, 148 (45%) had abnormal findings. High-risk headaches and focal seizures were significantly correlated with findings on brain MRI. Diagnostic and therapeutic yields were most significant in acute demyelinating events, acute cerebrovascular disorders, high-risk headaches when supported by neurologic and ophthalmologic findings, focal seizures with evidence of multifocal epileptic activity on an electroencephalogram and ophthalmic complaints when accompanied by cranial nerve palsy and optic nerve impairment. Since the contributions of a brain MRI in hospitalized children is pivotal in specific clinical situations, a judicious decision-making process should be done before its scheduling, in order to optimize clinical care.

The antisense oligonucleotide nusinersen (Spinraza) regulates splicing of the survival motor neuron 2 (SMN2) messenger RNA to increase SMN protein expression. Nusinersen has improved ventilator-free survival and motor function outcomes in infantile onset forms of spinal muscular atrophy (SMA), treated early in the course of the disease. However, the response in later onset forms of SMA is highly variable and dependent on symptom severity and disease duration at treatment initiation. Therefore, we aimed to identify novel noninvasive biomarkers that could predict the response to nusinersen in type II and III SMA patients.Thirty-four SMA patients were included. We applied next generation sequencing to identify microRNAs in the cerebrospinal fluid (CSF) as candidate biomarkers predicting response to nusinersen. Hammersmith Functional Motor Scale Expanded (HFMSE) was conducted at baseline and 6 months after initiation of nusinersen therapy to assess motor function. Patients changing by ≥3 or ≤0 points in the HFMSE total score were considered to be responders or nonresponders, respectively.Lower baseline levels of two muscle microRNAs (miR-206 and miR-133a-3p), alone or in combination, predicted the clinical response to nusinersen after 6 months of therapy. Moreover, miR-206 levels were inversely correlated with the HFMSE score.Lower miR-206 and miR-133a-3p in the CSF predict more robust clinical response to nusinersen treatment in later onset SMA patients. These novel findings have high clinical relevance for identifying early treatment response to nusinersen in later onset SMA patients and call for testing the ability of miRNAs to predict more sustained long-term benefit.

Night eating syndrome (NES) is an eating disorder (ED) characterized by nocturnal ingestion (NI), evening hyperphagia, morning anorexia, as well as mood and sleep disturbances. This study compared subjective and objective sleep quality and ED-related psychopathologies in patients seeking treatment for ED. Method: The sample was composed of 170 women, aged 18–68, who were referred for an ED assessment from 2011 to 2020. The participants were divided into three subgroups: NES-NI only (n = 30), NES+ binge eating (BE) (including binge eating disorders or bulimia nervosa (n = 52), and BE-only (n = 88). The measures consisted of a psychiatric evaluation, objective sleep monitoring measured by an actigraph for 1 week, a subjective sleep self-report, and ED-related psychopathology questionnaires. Results: Objective sleep monitoring revealed significant group differences, with higher sleep efficiency in participants with BE-only and longer sleep durations for the NES-NI only group. Subjectively, the BE-only group described a significantly lower sleep quality than either the NES-NI only or the NES+BE groups. ED-related psychopathology was lower in the NES-NI-only group. A stepwise linear regression revealed that general psychopathology (the brief symptom inventory total score) was a significant predictor of subjective sleep quality. Conclusion: NES-NI-only was correlated with less psychopathology, but with more subjective and objective sleep disturbances. These results lend weight to the supposition that NES lies on a continuum of ED psychopathologies, and that NES-NI-only appears to be a separate entity from NES+BE and BE-only in terms of its psychopathology.

Epilepsy is estimated to exist in approximately 40% of individuals with cerebral palsy; however, the specific features that make it drug resistant are not well defined. The main aim of this study was to determine the clinical risk factors that could predict drug-resistant epilepsy, in children with cerebral palsy. The study was performed via a retrospective chart review, analyzing clinical parameters of 118 children with cerebral palsy with either drug-resistant epilepsy or controlled epilepsy, between the years 2013 and 2018. We established a predictive model for drug-resistant epilepsy in children with cerebral palsy that is simple to apply in clinical settings and composed of the additive effect of a low Apgar score at 5 minutes, neonatal seizures, focal-onset epilepsy, and focal slowing on electroencephalogram (EEG; area under the receiver operating characteristic of 0.840). Early prediction of drug-resistant epilepsy may benefit to achieve better seizure control in children with cerebral palsy.

INTRODUCTION AND OBJECTIVES Diaphragmatic paralysis (DP) in children can result from various etiologies. Guidelines for patient selection for diaphragmatic plication (DPL) are lacking. Our objectives were to describe the etiologies of DP and to determine the risk factors and predictors for DPL in the pediatric population. METHODS Retrospective data were retrieved from departmental databases on patients with DP from the pediatric, cardiac, and neonatal intensive care departments of Safra Children's Hospital from 2010 to 2017. RESULTS DP was diagnosed in 88 patients, 29 with noncardiac surgery-related etiologies, for example, congenital, surgery, trauma, and shock and 59 with cardiac surgery-related etiologies. In total, 27 (31%) patients underwent DPL, and they had significant comorbidities involving respiratory, central nervous, and cardiovascular systems, higher lung injury scores, and lower weight compared with the patients who did not undergo DPL (P = .002, P = .002, P

OBJECTIVES Stenotrophomonas maltophilia is a gram-negative opportunistic bacterium that may cause a myriad of clinical diseases in immunocompromised individuals. We aimed to describe the clinical characteristics, risk factors, mortality, and treatment of S. maltophilia bacteremia in critically ill children, a topic on which data are sparse. DESIGN A multicenter observational retrospective study in which medical charts of critically ill children with S. maltophilia bacteremia were reviewed between 2012 and 2017. SETTING Data were collected from each of the four largest PICUs nationwide, allocated in tertiary medical centers to which children with complex conditions are referred regularly. PATIENTS A total of 68 suitable cases of S. maltophilia bacteremia were retrieved and reviewed. MEASUREMENTS AND MAIN RESULTS The total occurrence rate of S. maltophilia isolation had increased significantly during the study period (r = 0.65; p = 0.02). The crude mortality was 42%, and the attributed mortality was 18%. Significant risk factors for mortality were a longer length of hospital stay prior to infection (33 d in nonsurvivors vs 28 in survivors; p = 0.03), a nosocomial source of infection (p = 0.02), presentation with septic shock (p < 0.001), and treatment with chemotherapy (p = 0.007) or carbapenem antibiotics (p = 0.05) prior to culture retrieval. On multivariate analysis, septic shock (odds ratio, 14.6; 95% CI, 1.45-147.05; p = 0.023) and being treated with chemotherapy prior to infection (odds ratio, 5.2; 95% CI, 1.59-17.19; p = 0.006)] were associated with mortality. The combination of ciprofloxacin, trimethoprim-sulfamethoxazole, and minocycline resulted in the longest survival time (p < 0.01). CONCLUSIONS The significant attributed mortality associated with S. maltophilia bacteremia in critically ill children calls for an aggressive therapeutic approach. The findings of this investigation favor a combination of trimethoprim-sulfamethoxazole, ciprofloxacin, and minocycline.

Multiple sclerosis (MS) is a chronic disease marked by progressive disability and decreased mobility over time. We studied whether individuals with MS of higher disability levels will be more overweight/obese as a result of their immobility and/or recurrent steroid treatments. In a prospective study, 130 individuals with MS and significant disability were classified according to the Expanded Disability Status Scale (EDSS) score as belonging to four groups: EDSS 3.0–4.0 (n = 31, 24%), EDSS 4.5–5.5 (n = 24, 18%), EDSS = 6.0 (n = 44, 34%) and EDSS ≥ 6.5 (n = 31, 24%). Medical history, body mass index (BMI), waist circumference and the level of engagement in physical activity were obtained. The mean ± standard error age was 55.8 ± 0.5 years, disease duration 18.2 ± 1.0 years and EDSS score 5.5 ± 0.1. Disease duration, the number of steroid courses per disease duration, weight, BMI and physical activity did not differ according to the four disability groups. The mean waist circumference increased significantly with increased severity of EDSS, p = 0.03. Increased disability in individuals with MS was not correlated with disease duration, lifestyle habits or overweight/obesity. However, increased disability was associated with central obesity.

The lockdown that was imposed by governments as part of the attempt to contain the COVID-19 pandemic included extreme measures, such as home confinement and the shutting down of special education systems. Our aim was to learn about the core experiences of parents of children with autism during this significant life disturbance. Thirty-one parents of 25 children with autism participated in semi-structured telephone interviews which were transcribed verbatim and underwent a qualitative, immersion/crystallization analysis. The analysis was conducted in an iterative consensus-building process to identify parents’ experiences, concerns, challenges, coping strategies, and perceived needs during the lockdown. The main themes that emerged related to the various parental concerns; the major difficulties encountered during this unique time, the functional, social, and behavioral implications the lockdown had on these children; and the manner in which the parents coped as an indication of their resourcefulness and outlook. Our findings broaden the insight into the underlying elements of the hardships and gains experienced by children with autism and their parents in times of significant life adversity. Programs in such times should be directed at supporting and guiding parents on how to better accommodate to the situation, thereby optimizing their coping strategies and resilience. Lay abstract The lockdown and home isolation due to the COVID-19 pandemic led to significant transformation in lifestyles. Being a parent in this situation was not easy for anyone, much less for parents of children with special needs. The shutting down of special education systems meant that parents lost a vital support network and had to be the sole full-time caregivers despite often lacking the skills to cope with this new and daunting situation. We interviewed parents and learned that the main difficulties faced by homebound autistic children stemmed from the change in routine, lack of special education services, limited physical space, and food- and sleep-related issues. Some children experienced worsening in behavioral, social, and developmental domains, yet others seemed to not only overcome the challenges of changing conditions but even benefit from them. The children’s success or failure was directly related to how their parents coped. The key factors that enabled successful coping were the parents’ ability to accommodate to the child’s needs, their own creativeness and resourcefulness, and a generally positive outlook. The results of this analysis revealed that the best way to benefit autistic children caught up in drastic changes in their routine lifestyle is to invest in a strong support system for their parents.

The existing data on the neurodevelopmental outcome of children born with an isolated atretic cephalocele (IAC) are scant. We aimed to expand upon these data by describing our experience with affected children, as well as assist parents and clinicians in deciding how to proceed when an IAC is diagnosed prenatally.A follow-up study was conducted on nine children who were born with an IAC. Evaluations were performed by pediatric neurologists and child development specialists. Developmental outcomes were based on a global development evaluation that assessed gross and fine motor skills, receptive and expressive language levels, activities of daily living, communication skills, and social domains. Adaptive skills were estimated by the Adaptive Behavior Assessment System, Second Edition.None of the nine children (median age 4 years and 6 months) had abnormal findings on neurological examination. Six children had age-appropriate developmental milestones, two had a mild motor delay, and one had mild expressive language delay (catchup was achieved by all of the latter three by ~ 3.5 years of age). The mean general adaptive composite score was 105 ± 11.7 (normal = 100). None of the children had behavioral, social, or communication problems.Children diagnosed with an IAC with/without a falcine sinus and devoid of coexisting intracranial abnormalities seem to have a normal neurodevelopmental outcome. Continuation of pregnancy may be recommended when an IAC is detected prenatally, and reassurance if detected postnatally.

ObjectiveThe number of transgender and gender non-conforming children is on the rise. For these children, the timing of medical intervention is crucial, yet transgender children report poorer overall physical and mental health outcomes compared with their cisgender peers. We aim to describe how paediatricians perceive transgender people.SettingThe ‘Transgender Attitudes and Beliefs Scale’, which consists of 29 items in three domains—human value, interpersonal comfort and sex/gender beliefs—was administered to 391 senior and resident paediatricians in Israel. The responses on a 7-point Likert scale were collapsed into two categories: a mean score of ≥6 for each domain was a ‘Favourable’ perception and ResultsOf 355 respondents (91% response rate), 221 (62%) were females, 132 (37%) were males and 2 identified as ‘other’; 290 (82%) were born in ‘trans-respect countries’, 274 (77%) identified as secular, 223 (63%) were senior physicians and 132 (27%) were residents. Overall, 90% of the cohort scored favourably on the ‘Human value’ domain, 68% on ‘Interpersonal comfort’ and 40% on ‘Sex/gender beliefs’. In the ‘Interpersonal comfort’ domain, being a man, birthplace in a transphobic country, identification as religious and being a senior physician were all associated with increased ORs for an unfavourable score: 2.1 (95% CI 1.3 to 3.4), 3.4 (95% CI 1.9 to 6.3), 2.4 (95% CI 1.4 to 4.2) and 1.8 (95% CI 1.1 to 3.0), respectively. In the ‘Sex/gender beliefs’ domain, being a man and identifying as religious had significantly increased ORs for unfavourable scores: 2.2 (95% CI 1.3 to 3.5) and 10.6 (95% CI 4.7 to 24.1), respectively.ConclusionsNegative attitudes towards transgender people are still widespread among paediatricians. Interventions are warranted to positively impact these attitudes.

Cancer Stem Cells (CSCs) have been suggested as the culprit responsible for tumor resistance to treatment and disease recurrence. Wilms' tumor (WT) is a paradigm for studying the relation between development and tumorigenesis, showing three main histological elements: undifferentiated blastema, epithelia and stroma, mimicking human kidney development. NCAM + ALDH1+ cells were previously found to contain the cancer stem like-cell population in WT. Thus far, the correlation between histologic characterization of this cell population, clinicopathologic parameters and prognostic outcome has yet been investigated in WT.Paraffin-imbedded primary WT specimens from twenty-four patients were immunostained for NCAM and ALDH1. Positivity and histologic compartment localization were determined by two independent observers, blinded to the clinical outcome. Clinicopathologic parameters and prognostic outcomes were determined based on the patients' medical records. The association of NCAM and ALDH1 co-localization with clinicopathologic characteristics was analyzed byχBlastemal co-localization of NCAM and ALDH1 was observed in 33% of WTs. Metastases, ICE chemotherapy protocol, blastemal predominance following preoperative chemotherapy, recurrence and patient demise were found to significantly correlate with blastemal NCAM + ALDH1+ cell staining (p 0.05). A significant inverse correlation between blastemal double positive cells, disease-free survival and overall survival was also observed.WT blastemal NCAM + ALDH1+ CSCs significantly correlate with adverse clinicopathologic parameters and poorer prognosis. These results underscore the role of CSCs in disease progression. Additionally, this pilot study supports the addition of these markers for risk stratification of WTs.

AIM To examine the prevalence of attention-deficit/hyperactivity disorder (ADHD) and related post-surgical weight loss in adolescents with severe obesity seeking bariatric surgery. METHODS The study population included 84 adolescents (age 13-19 years; 44 males, 40 females) with severe obesity who sought bariatric surgery at the Sheba Medical Centre in Israel during the years 2011-2017. Anthropometric and clinical data were collected from medical records. A subgroup of 20 participants filled questionnaires that evaluated ADHD-like symptoms, eating behaviours and quality of life. Data on adolescents that ultimately underwent surgery (n = 45) were also obtained. RESULTS The prevalence of ADHD was 28.6% in adolescents seeking bariatric surgery, much higher than that of the general adolescent population of Israel (17.1%, P

Background Anorexia nervosa is a perplexing psycho-biological disorder with a systemic nature, which can present in almost every organ and system of the body. Among the different presentations of starvation, several immunological and dermatological manifestations have been documented. To the best of our knowledge the occurrence of urticaria and angioedema in patients with binge or purge behaviors has yet to be documented. Case presentation We present a 16-year-old female patient diagnosed with anorexia nervosa binge/purge type, who presented with urticaria and angioedema shortly after binge/purge episodes that subsided when these behaviors ceased. Other possible causes for the urticaria were ruled out. Discussion This finding may represent a form of inducible urticaria, exacerbated in low-weight patients by the occurrence of binge/purge behaviors. We wish to report this observation in an attempt to widen the scope of the physical signs that may accompany eating disorders and bring this specific phenomenon into awareness. © 2016 Wiley Periodicals, Inc. (Int J Eat Disord 2016)

Background The purpose of this study was to assess the benefit of a short workshop addressing knowledge and confidence of inpatient caregivers (physicians and nurses) treating pediatric type 1 diabetes mellitus (T1DM) patients. Methods Questionnaires to determine familiarity with T1DM management and confidence in care were distributed at three university-affiliated medical centers. A 5-h practical-skills workshop was conducted at one center. Same questionnaires were filled out immediately after the workshop and 3 months later. Evaluation of knowledge was based on 16 multiple-choice questions, and confidence based on 10 categorical questions. Results Nurses and physicians (total 135, 106 women, 85 nurses) completed the questionnaires. The median knowledge score was 53.33 (40–66) and 46.7 (26.7–66.7) for nurses and physicians, respectively (p = 0.76). The confidence scores were 1.44 (1.1–1.9) and 1.56 (1.2–1.8), respectively (p = 0.7). More experience among nurses (>10 years) was associated with higher confidence scores (p = 0.04). Twenty-one physicians and 52 nurses, 66 women, attended the workshop. The median knowledge grade improved from 60 (47–67) to 81 (69–81), p Conclusions Knowledge and confidence in care of medical staff treating inpatient T1DM pediatric patients are lacking. Both improved significantly after the workshop, independent of seniority or profession.

Objective To identify the threshold of total body fat percentage (TBF%) required for the resumption of menses (ROM) in hospitalized female adolescents with anorexia nervosa (AN) using bioimpedance analysis (BIA). Methods All female adolescents hospitalized with AN in our medical center were evaluated in a longitudinal prospective study during the years of 2012–2017. Anthropometric data, body fat measured by BIA, and hormonal determinants were collected periodically, in addition to routine medical and gynecological assessments. Results Sixty-two participants presented with secondary amenorrhea, of which 20 remained with amenorrhea and 42 had ROM during hospitalization. At discharge, participants with ROM regained significantly more weight, and had higher mean body mass index (BMI), BMI standard deviation scores, and TBF% than those who remained with amenorrhea. Receiver operating characteristic analysis identified that a TBF% of 21.2% had the highest discriminative ability for ROM (sensitivity = 88%, specificity = 85%, positive predictive value = 93%). Compared with the anthropometric parameters, TBF% had the highest area under curve (AUC = .895), which significantly differed from that of BMI standard deviation scores (AUC = .643, p = .007) and body weight (AUC = .678, p = .03). Conclusions BIA is a safe and relatively simple method to assess the TBF% required for the return of balanced menstrual cycles in female adolescents with AN. The TBF% with the highest discriminative ability for menstrual resumption as assessed by BIA is 21.2%.

Celiac disease (CD) is a chronic immune-mediated systemic disease characterized by inflammation and villous atrophy of the small intestine. A strict, lifelong gluten-free diet (GFD) is the only treatment for CD. Disordered eating behaviors (DEBs) prevail in adolescence and young adulthood, and confer a risk of developing into full-blown eating disorders. The aims of the current study were to assess the incidence and risk factors for DEBs among individuals with CD, and to examine an association between adherence to GFD and DEBs. A cohort of 136 individuals with CD responded to a web-mediated survey that assessed DEBs and adherence to a GFD. The survey included demographic data (gender, age, weight, disease duration) and two self-rating questionnaires: the Eating Attitudes Test-26 and the gluten-free diet questionnaire. DEBs were found in 19% of female and 7% of male responders. These individuals were characterized by being overweight (p = 0.02), of an older age (p = 0.04) and female sex (p = 0.06). Strict adherence to a GFD was reported by 32% of the responders and was not correlated with age, disease duration, age at diagnosis of CD and with being overweight. Caregivers should be aware of the increased occurrence of DEBs in adolescents with CD, especially those who are overweight, older and of a female gender. Level V, cross-sectional descriptive study.

Stenotrophomonas maltophilia is a life-threatening nosocomial pathogen with profound multidrug-resistant attributes. It is associated with high mortality, particularly in immunocompromised patients. Data on therapy for S. maltophilia infections are scarce, especially in children hospitalized in intensive care settings (pediatric intensive care unit).A retrospective chart review of pediatric patients with isolates of S. maltophilia hospitalized over a 5-year period in 2 pediatric intensive care units.Thirty-one patients and 91 isolates from blood, respiratory secretions and soft tissues were identified and reviewed. The overall incidence of S. maltophilia infections increased during the study period (P = 0.003). The all-cause crude mortality was 61%, and the attributed mortality was approximately 16%. Risk factors associated with mortality included longer hospitalization before infection (P = 0.002), septic shock (P = 0.003), mechanical ventilation (P = 0.004), an indwelling central vein catheter (P = 0.03) and prior use of steroids (P = 0.04) and carbapenems (P = 0.004). On multivariate analysis, mortality was associated with mechanical ventilation (P = 0.02) and preinfection hospitalization days (P = 0.01). Combination treatment of trimethoprim and sulfamethoxazole, ciprofloxacin and/or minocycline significantly extended survival time (P0.001). The method of treatment did not significantly affect the interval between S. maltophilia isolation to resolution of infection (P = 0.200).Combinations of trimethoprim and sulfamethoxazole, ciprofloxacin and minocycline are proposed for pediatric intensive care unit patients harboring S. maltophilia. Meticulous evaluation of central vascular access and prior treatment with carbapenems are indicated, especially for mechanically ventilated and septic children.

Background Disordered eating behaviors (DEBs) may lead to full blown eating disorders. Both type 1 diabetes mellitus (T1DM) and celiac disease (CD) have been linked to DEBs. Objective To compare the presence of DEBs between adolescents and young adults with a dual diagnosis of T1DM and CD, and individuals with only one of the diagnoses. Methods Individuals with a dual diagnosis of T1DM and CD ("T1DM + CD group" n = 39), with a diagnosis of T1DM only ("T1DM group" n = 97) and with a diagnosis of CD only ("CD group" n = 267) filled the Eating Attitude Test-26 (EAT-26) questionnaire. Those with T1DM completed in addition to the Diabetes Eating Problem Survey-Revised (DEPS-R). Results The study population comprised of 403 individuals, of whom 65% were females. There were no statistically significant differences among the groups in distribution of sex, age, hemoglobin A1c (HbA1c) levels, age of disease diagnosis and duration. The prevalence of DEBs in the T1DM + CD group was 3-fold higher (26.0%) than in the T1DM (8.2%) and CD (8.2%) groups (P = .003). This trend was observed for both females and males. Multivariate analysis demonstrated that the T1DM + CD group had an increased risk for DEBs (odds ratio, OR: 4.7, 95% confidence interval, CI: 1.9-11.2, P = .001) after adjustment for age, sex, and body mass index. Additionally, being female, older and overweight increased the risk for DEBs. HbA1c values were not associated with an increased DEBs rate. Conclusions Individuals with the dual diagnoses of T1DM and CD have an increased likelihood to develop DEBs compared to those with only one of these diagnoses.

Background Cancer Stem Cells (CSCs) have been suggested as the culprit responsible for tumor resistance to treatment and disease recurrence. Wilms' tumor (WT) is a paradigm for studying the relation between development and tumorigenesis, showing three main histological elements: undifferentiated blastema, epithelia and stroma, mimicking human kidney development. NCAM + ALDH1+ cells were previously found to contain the cancer stem like-cell population in WT. Thus far, the correlation between histologic characterization of this cell population, clinicopathologic parameters and prognostic outcome has yet been investigated in WT. Procedures Paraffin-imbedded primary WT specimens from twenty-four patients were immunostained for NCAM and ALDH1. Positivity and histologic compartment localization were determined by two independent observers, blinded to the clinical outcome. Clinicopathologic parameters and prognostic outcomes were determined based on the patients' medical records. The association of NCAM and ALDH1 co-localization with clinicopathologic characteristics was analyzed byχ2-test. Survival analysis was carried out by the log-rank test using Kaplan-Meier method. Results Blastemal co-localization of NCAM and ALDH1 was observed in 33% of WTs. Metastases, ICE chemotherapy protocol, blastemal predominance following preoperative chemotherapy, recurrence and patient demise were found to significantly correlate with blastemal NCAM + ALDH1+ cell staining (p Conclusions WT blastemal NCAM + ALDH1+ CSCs significantly correlate with adverse clinicopathologic parameters and poorer prognosis. These results underscore the role of CSCs in disease progression. Additionally, this pilot study supports the addition of these markers for risk stratification of WTs.