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Background and Aim High rates of inflammatory bowel disease (IBD) have been documented in New Zealand (NZ) children. The objectives of this study were to describe the outcomes and disease course of childhood IBD in the first 3 years following diagnosis. Methods All children diagnosed with IBD in 2015 in NZ were included. Clinical data obtained during routine care for 3 years following diagnosis were analyzed. Growth parameters, disease activity scores, and blood parameters were compared at diagnosis and follow up. Results Three‐year outcome data were available for 48 of 51 children. At follow up, median age was 15.1 years, and 34 had Crohn's disease (CD), 11 had ulcerative colitis (UC), and three had IBD‐unclassified (IBDU). Although disease progression including development of perianal disease occurred in 13 (38%) of 34 children with CD, the majority (n = 30) had inflammatory disease at follow up. Disease extension occurred in 25% (2/8) of children initially diagnosed with UC. Of all IBD patients, the mean body mass index z‐score increased from −0.40 to +0.10 (P = 0.01). Disease activity scores reduced from diagnosis to follow up in both CD (mean pediatric Crohn's disease activity index 35–6, P, This manuscript aimed, for the first time, to assess and describe the outcomes of a cohort of New Zealand children diagnosed with inflammatory bowel disease. Three years after diagnosis, this group of children was predominantly in remission with satisfactory improvements in growth parameters.

A chronic and gradual increase in pulse pressure (PP) is associated with cognitive decline and dementia in older individuals, but the mechanisms remain ill-defined. We hypothesized that a chronic elevation of PP would cause brain microvascular endothelial mechanical stress, damage the neurovascular unit, and ultimately induce cognitive impairment in mice, potentially contributing to the progression of vascular dementia and Alzheimer disease. To test our hypothesis, male control wild-type mice and Alzheimer disease model APP/PS1 (amyloid precursor protein/presenilin 1) mice were exposed to a transverse aortic constriction for 6 weeks, creating a PP overload in the right carotid (ipsilateral). We show that the transverse aortic constriction procedure associated with high PP induces a cascade of vascular damages in the ipsilateral parenchymal microcirculation: in wild-type mice, it impairs endothelial dilatory and blood brain barrier functions and causes microbleeds, a reduction in microvascular density, microvascular cell death by apoptosis, leading to severe hypoperfusion and parenchymal cell senescence. These damages were associated with brain inflammation and a significant reduction in learning and spatial memories. In APP/PS1 mice, that endogenously display severe cerebral vascular dysfunctions, microbleeds, parenchymal inflammation and cognitive dysfunction, transverse aortic constriction–induced high PP further aggravates cerebrovascular damage, Aβ (beta-amyloid) accumulation, and prevents learning. Our study, therefore, demonstrates that brain microvessels are vulnerable to a high PP and mechanical stress associated with transverse aortic constriction, promoting severe vascular dysfunction, disruption of the neurovascular unit, and cognitive decline. Hence, chronic elevated amplitude of the PP could contribute to the development and progression of vascular dementia including Alzheimer disease.

Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner. The aim of this review is to provide evidence-based information and clinical algorithms to guide the nutritional and gastrointestinal management of patients with CF.

The global incidence of paediatric inflammatory bowel disease (IBD) is increasing. Much of the evidence attesting to this has arisen from North America and Europe. There is a relative paucity of information on the epidemiology of paediatric IBD in the Southern Hemisphere. The present study aimed to document the prospectively collected incidence of paediatric IBD in New Zealand in 2015.All patients younger than 16 years of age and diagnosed with IBD in New Zealand between 1 January 2015 and 31 December 2015 were identified. Demographic and disease phenotypic details were collected and entered into a secure database. Age-specific population data for New Zealand were obtained and national incidence rates for IBD and its subtypes were calculated.The prospectively calculated incidence of paediatric IBD, Crohn disease, ulcerative colitis (UC), and IBD unclassified in New Zealand in 2015 were 5.2 (95% confidence interval 3.9-6.8), 3.5 (2.4-4.8), 1.0 (0.5-1.8), and 0.7 (0.3-1.4) per 100,000 children, respectively.Incidence rates of paediatric IBD in New Zealand are comparable to the highest rates published in the literature from Western Europe and North America. Ongoing prospective ascertainment of the incidence of paediatric IBD is required to better understand the environmental factors, which are accounting for this increase in disease burden.

Background The incidence of pediatric inflammatory bowel disease (IBD) around the world is increasing. However, there is a scarcity of data on the epidemiology of pediatric IBD in the Southern Hemisphere. This study aimed to document the point prevalence of pediatric IBD in New Zealand on June 30, 2015. Methods All patients in New Zealand, under 16 years of age, with a diagnosis of IBD on June 30, 2015 were identified. Demographic and disease phenotypic details were collected and entered into a secure database. Age-specific population data for New Zealand were obtained and national and regional prevalence rates were calculated. Results The point prevalence of pediatric IBD, Crohn's disease, ulcerative colitis, and inflammatory bowel disease unclassified in New Zealand on June 30, 2015 was (95% confidence intervals) 21.7 (18.9-24.8), 16.5 (14.0-19.2), 3.3 (2.2-4.6), and 1.9 (1.2-3.0) per 100,000 children, respectively. There was a striking disparity between the prevalence rates in the North and South Islands. Conclusions The point prevalence of pediatric IBD in New Zealand represents the first-ever national, population-based prevalence rates of pediatric IBD published. Results from the Paediatric IBD in New Zealand (PINZ) study are also the first to show markedly higher prevalence rates of IBD in the southern part of a country compared with its northern counterpart. Ongoing prospective ascertainment of the incidence of pediatric IBD is required.

Proximal femoral fractures are a major public health concern with estimated annual direct and social costs amounting to £2 billion and average 30-day mortality risk of 7.5%. In response to the recent debate over out-of-hours hospital provision we investigated the ‘weekend effect’ at a major trauma centre, caring for acute injuries. A single centre, multi-surgeon review of 2060 patients performed. The distribution of patient and treatment variables compared in patients admitted on a weekday or the weekend. Fewer patients met performance indicators during weekend admission, time to surgery (63 vs. 71%) and time to geriatric review (86 vs. 91%). Weekend admission 30-day mortality was marginally lower than weekday (9.7% vs. 10.2%, OR 0.94, 95% CI 0.67 to 1.32, p = 0.7383). Increasing age, female gender, co-morbidities and confusion increased mortality risk. Binary regression analysis including these variables found no significant ‘weekend effect’. Despite the unit observing an increasing workload in the last five years, with meticulous workforce planning, senior doctor provisions and careful use of resources, it is possible to provide a seven-day fracture neck of femur service with no variation in thirty-day mortality by the day of admission.

BackgroundIn children, paracetamol overdose due to deliberate self-poisoning, accidental exposure or medication errors can lead to paediatric acute liver failure and death. In Australia and New Zealand, the nature of ingestion and outcomes of paracetamol-associated paediatric acute liver failure have not been described.ObjectiveTo describe the nature and outcomes of paracetamol-associated paediatric acute liver failure.DesignRetrospective analysis of paracetamol-associated paediatric acute liver failure cases presenting 2002–2012.SettingNew Zealand and Queensland Paediatric Liver Transplant Services.Results14 of 54 cases of paediatric acute liver failure were attributed to paracetamol, the majority were secondary to medication errors. 12 of the 14 children were under the age of 5 years. Seven children received doses in excess of 120 mg/kg/day. Many of the other children received either a double dose, too frequent administration, coadministration of other medicines containing paracetamol or regular paracetamol for up to 24 days. Three children underwent transplant. One of these and one other child died.ConclusionsIn Australia and New Zealand, paracetamol overdose secondary to medication errors is the leading cause of paediatric acute liver failure. A review of regional safety practices surrounding paracetamol use in children is indicated.

A disability is a physical or mental impairment that has an adverse long-term impact on someone's ability to carry out normal day-to-day activities. This is often thought of in terms of medical conditions with clearly defined symptoms. This chapter, however, argues that it is these symptoms that can be considered to be the impairments and that in the right environment can in fact be advantageous. Someone may be have a medical diagnosis but not be symptomatic due to medication, for instance. In this chapter, a set of symptoms is made up from a number of different scales, called Social-Behavioural Traits (SBTs), which are affected by a person's Serotonergic-Dopamagenic Asynchronicity (SDA). This chapter discusses the role of affective computing in achieving harmony between people with various personality differences, including a model for explaining how technology can influence SDA and SBTs for the better.

It is unclear whether recent therapeutic advances have improved the growth of children with Crohn's disease (CD).To assess the frequency of short stature and poor growth and their relationship to disease course and therapy in children with CD. WHAT IS ALREADY KNOWN ON THIS TOPIC: Growth retardation may occur in children with Crohn's disease (CD). Current therapy for CD in the UK is less likely than previously to involve the use of long-term glucocorticoids.Despite advances in therapy, short stature and slow growth continue to be encountered in children with CD. There is a need for simple and consistent definitions of growth that can identify poor growth in children with chronic disease.The anthropometric and treatment details of 116 children (68 male) with a mean (range) age at diagnosis of 10.8 years (4.9-15.5) and a mean age at maximum follow-up (MF) of 15.4 years (9.4-19.3) were studied retrospectively at diagnosis (T0), at 1 (T1), 2 (T2) and 3 years (T3) after diagnosis and at MF.At T0, mean height SD score (HtSDS) was -0.5 (-3.3 to 2.6) compared to a mid-parental HtSDS of 0.2 (-2.0 to 01.4) (p=0.002). At T1, T2, T3 and MF, mean HtSDS was -0.6 (-4.8 to 7.8), -0.6 (-2.9 to 2.2), -0.7 (-3.6 to 2.5) and -0.5 (-3.5 to 2.9), respectively. Mean Ht velocity (HV) SDS at T1, T2, T3 and MF was -1.4 (-7.4 to 7.4), -0.6 (-7.5 to 6.1), -0.1 (-6.6 to 7.6) and 0.6 (-4.8 to 7.8), respectively (p0.05). In final models, HtSDS was associated negatively with the use of prednisolone (p=0.0001), azathioprine (p=0.0001), methotrexate (p=0.0001) and weight SDS (WtSDS) (p=0.0001). HVSDS was associated positively with age (p=0.0001) and WtSDS (p=0.01). ΔHtSDS was associated negatively with use of prednisolone (p0.02).Although current therapy for CD is associated with improved rate of growth for the first few years, a substantial proportion of children remain short. This study also highlights the need for consistency in describing growth in children with chronic diseases.

Lower jaw development is a complex process in which multiple signaling cascades establish a proximal-distal organization. These cascades are regulated both spatially and temporally and are constantly refined through both induction of normal signals and inhibition of inappropriate signals. The connective tissue of the tongue arises from cranial neural crest cell-derived ectomesenchyme within the mandibular portion of the first pharyngeal arch and is likely to be impacted by this signaling. Although the developmental mechanisms behind later aspects of tongue development, including innervation and taste acquisition, have been elucidated, the early patterning signals driving ectomesenchyme into a tongue lineage are largely unknown. We show here that the basic helix-loop-helix transcription factor Hand2 plays key roles in establishing the proximal-distal patterning of the mouse lower jaw, in part through establishing a negative-feedback loop in which Hand2 represses Dlx5 and Dlx6 expression in the distal arch ectomesenchyme following Dlx5- and Dlx6-mediated induction of Hand2 expression in the same region. Failure to repress distal Dlx5 and Dlx6 expression results in upregulation of Runx2 expression in the mandibular arch and the subsequent formation of aberrant bone in the lower jaw along with proximal-distal duplications. In addition, there is an absence of lateral lingual swelling expansion, from which the tongue arises, resulting in aglossia. Hand2 thus appears to establish a distal mandibular arch domain that is conducive for lower jaw development, including the initiation of tongue mesenchyme morphogenesis.

We report the first two pediatric patients with CF who underwent successful combined liver-pancreas transplantation in Australia and New Zealand for CF liver disease and CF-related diabetes mellitus.

Hyperammonemia is a rare and important complication post-liver transplantation. We review a case of a 5-month-old boy with biliary atresia who received a split liver transplant following a variceal bleed. The transplant was complicated by recurrent portal vein thrombosis. Colonized with Serratia marcescens pretransplant, he developed disseminated infection associated with very high levels of ammonia that led to his death. It is important to be aware of serum ammonia levels in patients with portal vein thrombosis, particularly in the setting of gastrointestinal bleeding and sepsis.

Gastroesophageal reflux occurs in the majority of infants, with severity ranging from asymptomatic to severe esophagitis and failure to thrive. Omeprazole is recognized as a safe and effective treatment of gastroesophageal reflux in older children, at an initial dosage of 0.7 mg x kg(-1) x day(-1). To our knowledge, no dose-finding studies have been carried out in children under 2 years of age. The aim of the present study was to prospectively determine the dosage of omeprazole required to treat symptomatic gastroesophageal reflux in children younger than 2 years.Children under 2 years with clinical suspicion of gastroesophageal reflux underwent 24-hour dual-channel intraesophageal/gastric pH monitoring. A reflux index above 10% in children under 1 year and above 6% in children older than 1 year was deemed significant. Treatment with omeprazole at an initial dosage of 0.7 mg x kg(-1) x day(-1) (in 2 divided doses) was followed by dual-channel pH study after 14 days. The dosage was increased in increments of 0.7 mg x kg(-1) x day(-1), and pH studies were repeated until the gastroesophageal reflux was controlled.Ten children (5 male, 5 female), mean age 7.75 months (range, 1.25-20 months), were investigated. The initial median reflux index was 18.5% (range, 6.5%-56.3%). Follow-up median reflux index was improved at 1.6% (0.1%-8.1%) (P0.05). The median dosage required was 1.05 mg x kg(-1) x day(-1). Four children required 1.4 mg x kg(-1) x day(-1), and 1 required 2.8 mg x kg(-1) x day(-1). Corrected reflux index improved from 34.8% (16.8%-90.8%) to 20.1% (0.4%-100%) but did not achieve statistical significance. There were no serious complications or side effects.Omeprazole is an effective treatment for gastroesophageal reflux in children younger than 2 years. The majority respond to a dosage of 0.7 mg x kg(-1) x day(-1), but increased dosages up to 2.8 mg x kg(-1) x day(-1) may be required.

The small bowel is anatomically difficult to examine and was investigated by invasive, indirect modalities, such as push enteroscopy and small bowel follow-through. The aim of this study was to assess the efficacy and clinical impact of wireless capsule endoscopy (WCE) in children.Over the last 3 years (2002-2005), 37 patients with suspected small-bowel disease were investigated with WCE at a median age of 11 years (range, 16 months-16 years). The indications for WCE was suspected Crohn's disease (CD) (18), obscure or occult gastrointestinal bleeding (7), polyposis syndromes (5), protein losing enteropathy (4), recurrent abdominal pain (2), and malabsorption syndrome (1). All patients had preceding upper gastrointestinal endoscopy (OGD), ileocolonoscopy, and 26 cases had a small bowel follow-through (SBFT). These results were compared with the findings on WCE.Thirty-three (33) cases successfully completed the WCE through the small bowel. Four (4) patients were unable to swallow the capsule, 3 of which had to be placed in the duodenum endoscopically. In 3 patients, the capsule remained in the stomach and no small bowel images were obtained. The overall diagnostic yield was 85% (28/33 patients). The diagnostic findings included CD (13), source of gastrointestinal bleeding (7), polyposis syndromes (3), erosive enteropathy and patchy lymphangiectasia (4), and intussusception (1). WCE was found to be more sensitive for small bowel pathology than SBFT (20 vs. 6 [30% sensitivity, compared to WCE]) and endoscopic investigations (28 vs. 12 [43% sensitivity compared to WCE]). As a result of WCE findings, there was a positive alteration in the management in 28 of 33 (85%) cases.WCE is a novel, noninvasive, and useful tool for the investigation of the small intestine in children. It is superior and more sensitive than other conventional endoscopic and radiologic investigations in the assessment of the small bowel. It can help in guiding surgical decisions and should be routinely integrated as a part of the diagnostic work-up of small bowel pathology.

Tbx5 del/+ mice provide a model of human Holt-Oram syndrome. In this study, the cardiac functional phenotypes of this mouse model were investigated with 30-MHz ultrasound by comparing 12 Tbx5 del/+ mice with 12 wild-type littermates at 1, 2, 4, and 8 wk of age. Cardiac dimensions were measured with two-dimensional and M-mode imaging. The flow patterns in the left and right ventricular inflow channels were evaluated with Doppler flow sampling. Compared with wild-type littermates, Tbx5 del/+ mice showed significant changes in the mitral flow pattern, including decreased peak velocity of the left ventricular (LV) early filling wave (E wave), increased peak velocity of the late filling wave (A wave), and decreased or even reversed peak E-to-A ratio. The prolongation of LV isovolumic relaxation time was detected in Tbx5 del/+ neonates as early as 1 wk of age. In Tbx5 del/+ mice, LV wall thickness appeared normal but LV chamber dimension was significantly reduced. LV systolic function did not differ from that in wild-type littermates. In contrast, the Doppler flow spectrum in the enlarged tricuspid orifice of Tbx5 del/+ mice demonstrated increased peak velocities of both E and A waves and increased total time-velocity integral but unchanged peak E/A. In another 13 mice (7 Tbx5 del/+, 6 wild-type) at 2 wk of age, significant correlation was found between Tbx5 gene expression level in ventricular myocardium and LV filling parameters. In conclusion, the LV diastolic function of Tbx5 del/+ mice is significantly deteriorated, whereas the systolic function remains normal.

Social impairments materialise in a number of forms, from developmental disabilities such as autistic spectrum disorder, to psychiatric conditions such as social phobia. The individuals diagnosed with these problems find it difficult to deal with social situations through either the inability to perform in these situations or the fear of not being able to do so. The study investigated the social and practical implications of using Mobile Internet technology to deliver information relating to a social situation in real-time to participants with Autistic Spectrum Disorders (n = 10) and General Social Phobia (n = 3) diagnosed using DSM-IV. The participants used the agent on their mobile phone to convert phrases they found offensive or confusing into more concise and understandable definitions. Analysing their attitudes revealed that the technology enables socially impaired individuals to learn the meaning of emotions and understand more about how they communicate with their peers. However, the study concludes that governmental organisations, education providers and society as a whole need to adopt a cohesive approach to communication to ensure socially impaired individuals are fully included in society

A quasistatic magnetic resonance elastography (MRE) method for the evaluation of breast cancer is proposed. Using a phase contrast, stimulated echo MRI approach, strain imaging in phantoms and volunteers is presented. First-order assessment of tissue biomechanical properties based on inverse strain mapping is outlined and demonstrated. The accuracy of inverse strain imaging is studied through simulations in a two-dimensional model and in an anthropomorphic, three-dimensional finite-element model of the breast. To improve the accuracy of modulus assessment by elastography, inverse methods are discussed as an extension to strain imaging, and simulations quantify MRE in terms of displacement signal/noise required for robust inversion. A direct inversion strategy providing information on tissue modulus and pressure distribution is described along with a novel iterative method utilizing a priori knowledge of tissue geometry. It is shown that through the judicious choice of information from previous contrast-enhanced MRI breast images, MRE data acquisition requirements can be significantly reduced while maintaining robust modulus reconstruction in the presence of strain noise. An experimental apparatus for clinical breast MRE and preliminary images of a normal volunteer are presented.

Increasing numbers of adolescents are being diagnosed with Crohn's disease or ulcerative colitis, the two main subtypes of inflammatory bowel disease. These young people face many short- and long-term challenges; one or more medical therapies may be required indefinitely; their disease may have great impact, in terms of their schooling and social activities. However, the management of adolescents with one of these incurable conditions needs to encompass more than just medical therapies. Growth, pubertal development, schooling, transition, adherence, and psychological well-being are all important aspects. A multidisciplinary team setting, catering to these components of care, is required to ensure optimal outcomes in adolescents with inflammatory bowel disease.

Background Genetic polymorphisms, gender and age all influence the risk of developing chronic neuropathic pain following peripheral nerve injury (PNI). It is known that there are significant inter-strain differences in pain hypersensitivity in strains of mice after PNI. In response to PNI, one of the earliest events is thought to be the disruption of the blood-spinal cord barrier (BSCB). The study of BSCB integrity after PNI may lead to a better understanding of the mechanisms that contribute to chronic pain. Results Here we used in vivo dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) to establish a timecourse for BSCB permeability following PNI, produced by performing a spared nerve injury (SNI). From this longitudinal study, we found that the SNI group had a significant increase in BSCB permeability over time throughout the entire spinal cord. The BSCB opening had a delayed onset and the increase in permeability was transient, returning to control levels just over one day after the surgery. We also examined inter-strain differences in BSCB permeability using five mouse strains (B10, C57BL/6J, CD-1, A/J and BALB/c) that spanned the range of pain hypersensitivity. We found a significant increase in BSCB permeability in the SNI group that was dependent on strain but that did not correlate with the reported strain differences in PNI-induced tactile hypersensitivity. These results were consistent with a previous experiment using Evans Blue dye to independently assess the status of the BSCB permeability. Conclusions DCE-MRI provides a sensitive and non-invasive method to follow BSCB permeability in the same group of mice over time. Examining differences between mouse strains, we demonstrated that there is an important genetically-based control of the PNI-induced increase in BSCB permeability and that the critical genetic determinants of BSCB opening after PNI are distinct from those that determine genetic variability in PNI-induced pain hypersensitivity. Electronic supplementary material The online version of this article (doi:10.1186/1744-8069-10-60) contains supplementary material, which is available to authorized users.