Categorizing diffuse parenchymal lung disease in children

Background Aim of this study was to verify a systematic and practical categorization system that allows dynamic classification of pediatric DPLD irrespective of completeness of patient data. Methods The study was based on 2322 children submitted to the kids-lung-register between 1997 and 2012. Of these children 791 were assigned to 12 DPLD categories, more than 2/3 belonged to categories manifesting primarily in infancy. The work-flow of the pediatric DPLD categorization system included (i) the generation of a final working diagnosis, decision on the presence or absence of (ii) DPLD and (iii) a systemic or lung only condition, and (iv) the allocation to a category and subcategory. The validity and inter-observer dependency of this workflow was re-tested using a systematic sample of 100 cases. Results Two blinded raters allocated more than 80 % of the re-categorized cases identically. Non-identical allocation was due to lack of appreciation of all available details, insufficient knowledge of the classification rules by the raters, incomplete patient data, and shortcomings of the classification system itself. Conclusions This study provides a suitable workflow and hand-on rules for the categorization of pediatric DPLD. Potential pitfalls were identified and a foundation was laid for the development of consensus-based, international categorization guidelines

ElectrochemicalN-demethylation of tropane alkaloids

A practical, efficient, and selective electrochemicalN-demethylation method of tropane alkaloids to their nortropane derivatives is described. Nortropanes, such as noratropine and norscopolamine, are important intermediates for the semi-synthesis of the medicines ipratropium or oxitropium bromide, respectively. Synthesis was performed in a simple home-made electrochemical batch cell using a porous glassy carbon electrode. The reaction proceeds at room temperature in one step in a mixture of ethanol or methanol and water. The method avoids hazardous oxidizing agents such as H(2)O(2)orm-chloroperbenzoic acid (m-CPBA), toxic solvents such as chloroform, as well as metal-based catalysts. Various key parameters were investigated in electrochemical batch or flow cells, and the optimized conditions were used in batch and flow-cells at gram scale to synthesize noratropine in high yield and purity using a convenient liquid-liquid extraction method without any need for chromatographic purification. Mechanistic studies showed that the electrochemicalN-demethylation proceeds by the formation of an iminium intermediate which is converted by water as the nucleophile. The optimized method was further applied to scopolamine, cocaine, benzatropine, homatropine and tropacocaine, showing that this is a generic way ofN-demethylating tropane alkaloids to synthesize valuable precursors for pharmaceutical products

Long-term follow-up and treatment of congenital alveolar proteinosis

<p>Abstract</p> <p>Background</p> <p>Clinical presentation, diagnosis, management and outcome of molecularly defined congenital pulmonary alveolar proteinosis (PAP) due to mutations in the GM-CSF receptor are not well known.</p> <p>Case presentation</p> <p>A 2 1/2 years old girl was diagnosed as having alveolar proteinosis. Whole lung lavages were performed with a new catheter balloon technique, feasible in small sized airways. Because of some interstitial inflammation in the lung biopsy and to further improve the condition, empirical therapy with systemic steroids and azathioprin, and inhaled and subcutaneous GMCSF, were used. Based on clinical measures, total protein and lipid recovered by whole lung lavages, all these treatments were without benefit. Conversely, severe respiratory viral infections and an invasive aspergillosis with aspergilloma formation occurred. Recently the novel homozygous stop mutation p.Ser25X of the GMCSF receptor alpha chain was identified in the patient. This mutation leads to a lack of functional GMCSF receptor and a reduced response to GMCSF stimulation of CD11b expression of mononuclear cells of the patient. Subsequently a very intense treatment with monthly lavages was initiated, resulting for the first time in complete resolution of partial respiratory insufficiency and a significant improvement of the overall somato-psychosocial condition of the child.</p> <p>Conclusions</p> <p>The long term management from early childhood into young adolescence of severe alveolar proteinosis due to GMCSF receptor deficiency requires a dedicated specialized team to perform technically demanding whole lung lavages and cope with complications.</p

Crop Updates 2003 - Cereals

This session covers twenty one papers from different authors: PLENARY 1. Recognising and responding to new market opportunities in the grains industry, Graham Crosbie, Manager, Grain Products Research, Crop Breeding, Plant Industries, Department of Agriculture 2. Stripe rust – where to now for the WA wheat industry? Robert Loughman1, Colin Wellings2 and Greg Shea11Department of Agriculture, 2University of Sydney Plant Breeding Institute, Cobbitty (on secondment from NSW Agriculture) 3. Benefits of a Grains Biosecurity Plan, Dr Simon McKirdy, Plant Health Australia, Mr Greg Shea, Department of Agriculture 4. Can we improve the drought tolerance of our crops? Neil C. Turner, CSIRO Plant Industry, Wembley 5. The silence of the lambing, Ross Kingwell, Department of Agriculture AGRONOMY AND VARIETIES 6. Maximising performance of wheat varieties, Brenda Shackley, Wal Anderson, Darshan Sharma, Mohammad Amjad, Steve Penny Jr, Melanie Kupsch, Anne Smith, Veronika Reck, Pam Burgess, Glenda Smith and Elizabeth Tierney, Department of Agriculture 7. Wheat variety performance in wet and dry, Peter Burgess 8. e-VarietyGuide for stripe rust – an updated version (1.02 – 2003), Moin Salam, Megan Collins, Art Diggle and Robert Loughman, Department of Agriculture 9. Baudin and Hamelin – new generation of malting barley developed in Western Australia, Blakely Paynter, Roslyn Jettner and Kevin Young, Department of Agriculture 10. Oaten hay production, Jocelyn Ball, Natasha Littlewood and Lucy Anderton, Department of Agriculture 11. Improving waterlogging tolerance in wheat and barley, Irene Waters and Tim Setter, Department of Agriculture 12. Broadscale variety comparisons featuring new wheat varieties, Jeff Russell, Department of Agriculture, Centre for Cropping Systems BIOTECHNOLOGY 13. Barley improvement in the Western Region – the intergration of biotechnologies, Reg Lance, Chengdao Li and Sue Broughton, Department of Agriculture 14. The Western Australian State Agricultural Biotechnology Centre – what we are and what we do, Michael Jones, WA State Agricultural Biotechnology Centre, Murdoch University 15. Protein and DNA methods for variety identification, Dr Grace Zawko, Saturn Biotech Limited 16. The Centre for High-throughput Agricultural Genetic Analysis (CHAGA), Keith Gregg, CHAGA, Murdoch University NUTRITION 17. Potassium – topdressed, drilled or banded? Stephen Loss, Patrick Gethin, Ryan Guthrie, Daniel Bell, Wesfarmers CSBP 18. Liquid phosphorus fertilisers in WA, Stephen Loss, Frank Ripper, Ryan Guthrie, Daniel Bell and Patrick Gethin, Wesfarmers CSBP 19. Wheat nutrition in the high rainfall cropping zone, Narelle Hill1and Laurence Carslake2, 1Department of Agriculture, 2Wesfarmers Landmark PESTS AND DISEASES 20. Managenent options for root lesion nematode in West Australian cropping systems, Vivien Vanstone, Sean Kelly and Helen Hunter, Department of Agriculture STORAGE 21. Aeration can profit your grain enterprise, Christopher R. Newman, Department of Agricultur

Categorizing diffuse parenchymal lung disease in children

Background Aim of this study was to verify a systematic and practical categorization system that allows dynamic classification of pediatric DPLD irrespective of completeness of patient data. Methods The study was based on 2322 children submitted to the kids-lung-register between 1997 and 2012. Of these children 791 were assigned to 12 DPLD categories, more than 2/3 belonged to categories manifesting primarily in infancy. The work-flow of the pediatric DPLD categorization system included (i) the generation of a final working diagnosis, decision on the presence or absence of (ii) DPLD and (iii) a systemic or lung only condition, and (iv) the allocation to a category and subcategory. The validity and inter-observer dependency of this workflow was re-tested using a systematic sample of 100 cases. Results Two blinded raters allocated more than 80 % of the re-categorized cases identically. Non-identical allocation was due to lack of appreciation of all available details, insufficient knowledge of the classification rules by the raters, incomplete patient data, and shortcomings of the classification system itself. Conclusions This study provides a suitable workflow and hand-on rules for the categorization of pediatric DPLD. Potential pitfalls were identified and a foundation was laid for the development of consensus-based, international categorization guidelines

Additional file 4: Table S3. of Categorizing diffuse parenchymal lung disease in children

Summary of reasons for incorrect categorization. (DOCX 15 kb

Additional file 6: Table S5. of Categorizing diffuse parenchymal lung disease in children

Centers and physicians contributing cases to the kids-lung-register. (DOCX 34 kb

Additional file 2: Figure S1. of Categorizing diffuse parenchymal lung disease in children

Routine work-flow used in the Kids lung register (KLR) to obtain a final working diagnosis and to categorize and subcategorize cases with suspected DPLD (black and red). The work-flow used for re-rating is depicted in red. *reference pathologist was Frank Brasch, **genetical diagnosis was made by Peter Lohse and ***lavage report on surfactant protein analysis, as well as the establishment of the final working diagnosis during routine KLR workflow were done by Matthias Griese. (PDF 176 kb