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Nowak-Wegrzyn A.,Mount Sinai School of Medicine | Katz Y.,Tel Aviv University | Mehr S.S.,Childrens Hospital at Westmead | Koletzko S.,Ludwig Maximilians University of Munich
Journal of Allergy and Clinical Immunology | Year: 2015

Non-IgE-mediated gastrointestinal food-induced allergic disorders (non-IgE-GI-FAs) account for an unknown proportion of food allergies and include food protein-induced enterocolitis syndrome (FPIES), food protein-induced allergic proctocolitis (FPIAP), and food protein-induced enteropathy (FPE). Non-IgE-GI-FAs are separate clinical entities but have many overlapping clinical and histologic features among themselves and with eosinophilic gastroenteropathies. Over the past decade, FPIES has emerged as the most actively studied non-IgE-GI-FA, potentially because of acute and distinct clinical features. FPIAP remains among the common causes of rectal bleeding in infants, while classic infantile FPE is rarely diagnosed. The overall most common allergens are cow's milk and soy; in patients with FPIES, rice and oat are also common. The most prominent clinical features of FPIES are repetitive emesis, pallor, and lethargy; chronic FPIES can lead to failure to thrive. FPIAP manifests with bloody stools in well-appearing young breast-fed or formula-fed infants. Features of FPE are nonbloody diarrhea, malabsorption, protein-losing enteropathy, hypoalbuminemia, and failure to thrive. Non-IgE-GI-FAs have a favorable prognosis; the majority resolve by 1 year in patients with FPIAP, 1 to 3 years in patients with FPE, and 1 to 5 years in patients with FPIES, with significant differences regarding specific foods. There is an urgent need to better define the natural history of FPIES and the pathophysiology of non-IgE-GI-FAs to develop biomarkers and novel therapies. © 2015 American Academy of Allergy, Asthma & Immunology.

Mehr S.,Childrens Hospital at Westmead
Paediatric Respiratory Reviews | Year: 2012

Immunological investigations need to be considered in any child presenting with chronic wet cough. Not infrequently, such children are subjected to a detailed, expensive battery of immune function tests, without consideration as to whether such extensive testing is necessary or indeed helpful. The main aim of this review is to discuss which immune function tests are and are not particularly helpful when investigating a child with persistent wet cough. © 2012 .

Gaskin K.J.,Childrens Hospital at Westmead
European Journal of Clinical Nutrition | Year: 2013

Over the last 30 years, major advances have occurred in our understanding of the disorder cystic fibrosis (CF) with the discoveries of the underlying chloride transport defect and the 'CF gene', the CF transmembrane conductance regulator gene. Equally important from a clinical and patient perspective are the improvements in median survival from less than 10 to 20 years prior to 1980, approaching 30 years during the 1980s and over 45 years more recently. Improved antibiotic regimens and lung clearance therapy contributed to the enhanced survival, but a key factor accredited as adding a further 10 years to the median survival was improving and then maintaining normal growth and nutrition. In the main, the latter were achieved by adherence to a 'high-fat high-energy' diet rather than the advocated virtually universal policy of the 'low fat', which was associated with wasting and linear growth failure. The high-fat diet in conjunction with better control of malabsorption due to microspheric pancreatic enzyme replacement therapy, attention to adequate fat-soluble vitamin supplementation and newborn screening has ensured that at least 80-90% of children with CF will achieve better health and survival through their adult years. © 2013 Macmillan Publishers Limited.

Robinson P.D.,Childrens Hospital at Westmead
European Respiratory Journal | Year: 2013

Inert gas washout tests, performed using the single- or multiple-breath washout technique, were first described over 60 years ago. As measures of ventilation distribution inhomogeneity, they offer complementary information to standard lung function tests, such as spirometry, as well as improved feasibility across wider age ranges and improved sensitivity in the detection of early lung damage. These benefits have led to a resurgence of interest in these techniques from manufacturers, clinicians and researchers, yet detailed guidelines for washout equipment specifications, test performance and analysis are lacking. This manuscript provides recommendations about these aspects, applicable to both the paediatric and adult testing environment, whilst outlining the important principles that are essential for the reader to understand. These recommendations are evidence based, where possible, but in many places represent expert opinion from a working group with a large collective experience in the techniques discussed. Finally, the important issues that remain unanswered are highlighted. By addressing these important issues and directing future research, the hope is to facilitate the incorporation of these promising tests into routine clinical practice.

Deshpande A.V.,Childrens Hospital at Westmead
BJU international | Year: 2012

• To report the early observations of using ambulatory urodynamic studies (UDS) using a Bluetooth-enabled device in children • To evaluate the incremental value of ambulatory over conventional UDS. • Ambulatory UDS were performed in selected children with voiding dysfunction between August 2009 and October 2010. • Conventional UDS were concurrently performed wherever possible. • The test results and treatment consequences of the two tests were compared. • In all, 12 ambulatory and seven conventional UDS were performed on 10 children (five boys, median [range] age 7 [4-16] years). • Six of the seven children had a normal conventional UDS. Ambulatory UDS detected phasic detrusor overactivity (DO) in five children and generalised DO in one. • Direct correlation of symptoms to DO was possible in two children during ambulatory UDS. Pressure rise during filling, seen in two children on conventional UDS, was not seen during ambulatory UDS. • Five children showed clinical improvement when therapy was guided by ambulatory UDS results. • Ambulatory UDS was generally well tolerated in eight children, with two complaining of discomfort. Inadequate information was obtained in two children who underwent ambulatory UDS due to technical problems in one and distress induced by the UDS in the other. • Ambulatory UDS provides useful additional information over conventional UDS and can be used to guide further therapy in selected children with voiding dysfunction. • It is safe and well tolerated in children. • There is a need for explicit guidance for the technical delivery and interpretation of ambulatory UDS in children. © 2012 THE AUTHORS. BJU INTERNATIONAL © 2012 BJU INTERNATIONAL.

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