Cystic Fibrosis–Related Diabetes

Cystic fibrosis–related diabetes (CFRD) results in significant morbidity and mortality for patients with cystic fibrosis (CF). It is the endpoint of a spectrum of progressive insulin deficiency with resulting abnormalities of glucose tolerance. The consequence of glycaemic abnormalities in CF is poorer nutritional status, an increase in respiratory exacerbations with decline in lung function and ultimately greater morbidity and mortality. CFRD can be diagnosed by the standard oral glucose tolerance test (OGTT) usually performed from 10 years of age. However, this may miss early glycaemic abnormalities which appear to be clinically important. Early recognition of CFRD and treatment have been shown to improve outcomes in CF. Novel diagnostic methods such as 30-min sampled OGTT and continuous glucose monitoring (CGM) may prove to be useful in screening for this disorder and in the early identification of glycaemic abnormalities

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been fully characterized

What can the gut microbiome teach us about the connections between child physical and mental health? A systematic review

A deeper understanding of the gut-brain axis is of significance in pediatrics, given the influential role of early childhood experiences and exposures in shaping the microbiome, and health, across the life course. This systematic review synthesized evidence on the connection between the gut microbiome and mental health in children with physical illness. Six electronic databases were systematically searched and data extracted according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Of 1,476 identified articles, 11 articles reporting on nine unique studies (all randomized controlled trials) were included. Most studies examined the gut microbiome in infants with colic, while the remaining studies investigated outcomes in children aged 1 day to 18 years at risk for atopic dermatitis or irritable bowel syndrome. Baseline and postintervention gut microbiome differences varied across studies. Findings on psychological functioning also varied, with only half of the captured studies showing a positive effect of intervention on psychological well-being. Only two studies analyzed the association between the gut microbiome and psychological outcomes, each with a different pattern of results. As the field moves forward, it will be critical to gain a better understanding of the microbiome characteristics that influence mental health outcomes in pediatric populations

Brief Overview of Bioinformatics Activities in Singapore

10.1371/journal.pcbi.1000508PLoS Computational Biology5

Seroepidemiology of coxsackievirus A6, coxsackievirus A16, and Enterovirus 71 infections among children and adolescents in Singapore, 2008-2010

10.1371/journal.pone.0127999PLoS ONE105e012799

The changing seroepidemiology of enterovirus 71 infection among children and adolescents in Singapore

<p>Abstract</p> <p>Background</p> <p>Enterovirus 71 (EV71) has caused recurrent epidemics of hand, foot and mouth disease among children in Singapore. Between August 2008 and July 2010, we conducted a survey to estimate the seroprevalence of EV71 infection among children and adolescents aged 1-17 years. We compared our EV71 seroepidemiologic findings with a previous study conducted in 1996-1997.</p> <p>Methods</p> <p>The survey involved the prospective collection of 1,200 residual sera from Singapore residents aged 1-17 years in two hospitals. Neutralizing antibodies to EV71 were detected by the microneutralization test. The geometric mean titer (GMT) of EV71 antibodies and 95% confidence intervals (CI) were calculated and compared by age groups. Statistical significance was taken as <it>P </it>< 0.05.</p> <p>Results</p> <p>The overall EV71 antibody prevalence was 26.9% (95% CI: 24.5-29.5%). It increased significantly from 14.3% in children aged 1-6 years to 27.8% in those aged 7-12 years, and reached 38.8% in adolescents aged 13-17 years. The seroconversion rate differed by about 12% between the consecutive age groups. The GMT of EV71 antibodies was higher among primary school children aged 7-12 years in our study than that among the 6-12 year age group in the 1996-1997 study.</p> <p>Conclusions</p> <p>Higher antibody titers were observed in children aged 1-6 years than those in the other two age groups, indicating that most of the infections had been acquired during early childhood. EV71 infection is common among children and adolescents in Singapore, with 39% infected by the time they are in secondary school (13-17 years of age).</p

Impact of Obesity on Pediatric Acute Recurrent and Chronic Pancreatitis

OBJECTIVE: The aim of this study was to assess the impact of obesity on pediatric acute recurrent pancreatitis or chronic pancreatitis (CP). METHODS: We determined body mass index (BMI) status at enrollment in INSPPIRE (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) cohort using CDC criteria for pediatric-specific BMI percentiles. We used the Cochran-Armitage test to assess trends and the Jonckheere-Terpstra test to determine associations. RESULTS: Of 446 subjects (acute recurrent pancreatitis, n = 241; CP, n = 205), 22 were underweight, 258 normal weight, 75 overweight, and 91 were obese. The BMI groups were similar in sex, race, and age at presentation. Hypertriglyceridemia was more common in overweight or obese. Obese children were less likely to have CP and more likely to have acute inflammation on imaging. Compared with children with normal weight, obese or overweight children were older at first acute pancreatitis episode and diagnosed with CP at an older age. Obese or overweight children were less likely to undergo medical or endoscopic treatment, develop exocrine pancreatic insufficiency, and require total pancreatectomy with islet autotransplantation. Diabetes was similar among all groups. CONCLUSIONS: Obesity or overweight seems to delay the initial acute pancreatitis episode and diagnosis of CP compared with normal weight or underweight. The impact of obesity on pediatric CP progression and severity deserves further study

Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)

Over the past two decades there has been considerable progress with the evaluation and management of infants with an inconclusive diagnosis following Newborn Screening (NBS) for cystic Fibrosis (CF). In addition, we have an increasing amount of evidence on which to base guidance on the management of these infants and, importantly, we have a consistent designation being used across the globe of CRMS/CFSPID. There is still work to be undertaken and research questions to answer, but these infants now receive more consistent and appropriate care pathways than previously. It is clear that the majority of these infants remain healthy, do not convert to a diagnosis of CF in childhood, and advice on management should reflect this. However, it is also clear that some will convert to a CF diagnosis and monitoring of these infants should facilitate their early recognition. Those infants that do not convert to a CF diagnosis have some potential of developing a CFTR-RD later in life. At present, it is not possible to quantify this risk, but families need to be provided with clear information of what to look out for. This paper contains a number of changes from previous guidance in light of developing evidence, but the major change is the recommendation of a detailed assessment of the child with CRMS/CFSPID in the sixth year of age, including respiratory function assessment and imaging. With these data, the CF team can discuss future care arrangements with the family and come to a shared decision on the best way forward, which may include discharge to primary care with appropriate information. Information is key for these families, and we recommend consideration of a further appointment when the individual is a young adult to directly communicate the implications of the CRMS/CFSPID designation