Oral presentations
Authors: R Brüggemann, E Mavridou, D Burger, W Melchers, P Verweij, J Mouton, W Heinz, C Kloeser, A Kuhn, K Kahle, D Keller, H Einsele, H Klinker, M Peiffer, J Bulitta, H Haeberle, M Kinzig-Schippers, M Rodamer, V Jakob, B Nohé, F Sörgel, W Krueger
Journal: Clinical Microbiology and Infection (2009)

Approach to primary immunodeficiency in children and adults S1 Classification of primary immunodeficiencies C. Picard °(Paris, FR) Human primary immunodeficiencies (PIDs) comprise a broad group of inherited disorders characterised by developmental or functional defects of myeloid or lymphoid haemapoietic-derived cells, as well as non-haemapoietic cells involved in protective immunity.More than 150 different forms of PIDs affecting distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins, natural killer cells, and T and B lymphocytes have been described [1].Clinically, PIDs can be associated with any combination of infectious disease, autoimmunity, auto-inflammatory, allergy and malignancy.The best known PIDs show Mendelian inheritance and first become symptomatic during childhood.However, the field of PIDs is rapidly expanding and PIDs showing non-Mendelian inheritance and/or affecting primarily adult patients is being increasingly recognized.Conventional PIDs are typically seen as rare monogenic conditions associated with detectable immunologic abnormalities, resulting in a broad susceptibility to multiple and recurrent infections caused by weakly pathogenic and more virulent microorganisms.By opposition to these conventional PIDs, nonconventional primary immunodeficiencies as Mendelian conditions manifesting in otherwise healthy patients as a narrow susceptibility to infections, recurrent or otherwise, caused by weakly pathogenic or more virulent microbes are now reported [2].By now, up to 120 diseasecausing genes have been identified.This molecular characterisation of PIDs has helped to increase our understanding of their physiopathology.The study of these diseases has provided essential insights into the functioning of the immune system with the ultimate goal of facilitating diagnosis and treatment. War, famine and beyond . . . infections in disaster settings S14 EarthquakesV. Korten °(Istanbul, TR) Infectious consequences of an earthquake mainly involve several types of communicable diseases and crush related infections.Water-borne and food-borne illnesses often result from the disruption of the public water and sewage systems and contamination of water supply.Overcrowding, poor hygiene and sanitation in temporary shelters also may be factors.The type of infectious diseases are associated with the epidemiology of communicable diseases in the area where the earthquake occurred.The most common outbreaks associated with earthquakes are gastroenteritis, infectious hepatitis and pulmonary infections.In unvaccinated populations, there are reports of increased measles.Tetanus can be seen in populations where vaccination coverage levels are low.The risk for diarrhoeal disease outbreaks following earthquakes is higher in developing countries than in industrialised countries.An outbreak of acute watery diarrhoea involved >750 cases occurred in a camp after the 2005 earthquake in Pakistan.Acute respiratory infections, hepatitis E clusters and measles (>400 clinical cases in the 6 months) also occurred among the displaced victims after the same earthquake.Contamination of drinking water led to an outbreak of rotavirus after the 2005 earthquake in Kashmir, India.An unusual outbreak of coccidiomycosis associated with exposure to increased levels of airborne dust occurred after the 1994 Southern California earthquake.Persons who have been trapped by rubble for several hours or days may develop compartment syndromes requiring fasciotomy or amputation.Infectious complications were common in renal victims of the1999 Marmara Earthquake in Turkey and were associated with increased mortality when complicated by sepsis.Of 639 renal victims, 223 (34.9%) had infectious complications, mainly sepsis and wound infections.Most of the infections were nosocomial in origin and caused by Gram-negative aerobic bacteria and Staphylococcus spp.Multivariate analysis of the risk-factors for nosocomial infections revealed a significant association with fasciotomy and length of hospital stay in a back up university hospital.The most frequent pathogens isolated from pus and/or wounds culture in 2008 Wenchuan earthquake survivors were S. aureus, E. coli, A. baumannii, E. cloacae, and P. aeruginosa.Disaster-preparedness plans, focused on trauma and mass casualty management and also on health needs of the surviving affected populations may decrease the health impact of earthquakes. S16 Infections in the disaster setting: famine. Experience fromDarfour, SudanClinic malnutrition is a known risk factor for ID worldwide.Subsaharan Africa and India is at higher risk due to vegetarian habits on absolute absence of animal meat proteins, resulting to depletion of micronutritients (Zinc, Iron, Selenium), responsible for recovery of postmalarial anaemia.In addition, depletion of proteins results to immunoglobulinaemia and to delayed response to many bacterial pathogens causing ID in topics (pneumococci, Salmonella, etc.).Third problem is absence of vitamins dissolved in oil and fat, resulting to delayed phagocytic activity.Therefore proteinocaloric malnutrition results to significant adverse outcome in HIV, TB (diarrhoea, pneumonia), the major killers of children under five.St. Elizabeth University Tropical programme runs 4 antimalnutrition centres: 1 in Sudan, Darfour and 2 in Kenya Amaong upcountry refugees from major conflict areas (Sudan -Turrana Border) and 1 in Uganda trying to rehabilitate malnourished children under 5 and helping them to combat disease, responsible for 12.5 million deaths in children mean 5 a year -malaria (1.2 mil), TB (1.1 mil), HIV (2.0 mil), pneumonia (7.5 mil) and diarrhoea (0.5 mil.children deaths approximately a year).