ObjectivesShiga-toxin producing O157:H7 Entero Haemorrhagic E. coli (STEC/EHEC) is one of the most common causes of Haemolytic Uraemic Syndrome (HUS) related to infectious haemorrhagic colitis. Nearly all recommendations on clinical management of EHEC infections refer to this strain. The 2011 outbreak in Northern Europe was the first to be caused by the serotype O104:H4. This EHEC strain was found to carry genetic features of Entero Aggregative E. coli (EAEC) and extended spectrum β lactamase (ESBL). We report symptoms and complications in patients at one of the most affected centres of the 2011 EHEC O104 outbreak in Northern Germany.MethodsThe courses of patients admitted to our hospital due to bloody diarrhoea with suspected EHEC O104 infection were recorded prospectively. These data include the patients’ histories, clinical findings, and complications.ResultsEHEC O104 infection was confirmed in 61 patients (female = 37; mean age: 44±2 years). The frequency of HUS was 59% (36/61) in our cohort. An enteric colonisation with co-pathogens was found in 57%. Thirty-one (51%) patients were treated with plasma-separation/plasmapheresis, 16 (26%) with haemodialysis, and 7 (11%) with Eculizumab. Patients receiving antibiotic treatment (n = 37; 61%) experienced no apparent change in their clinical course. Twenty-six (43%) patients suffered from neurological symptoms. One 83-year-old patient died due to comorbidities after HUS was successfully treated.ConclusionsEHEC O104:H4 infections differ markedly from earlier reports on O157:H7 induced enterocolitis in regard to epidemiology, symptomatology, and frequency of complications. We recommend a standard of practice for clinical monitoring and support the renaming of EHEC O104:H4 syndrome as “EAHEC disease”.

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Background Nasal involvement seems to be common in Churg-Strauss Syndrome (CSS, (1,2)). Detailed investigations of nasal function are not available. Objectives To analyze nasal function in CSS regarding especially olfactory and respiratory capacities using validated modern test systems. Methods From 6/1990 until 12/2009 96 CSS-patients (297 contacts) were included following standardized examination protocols (mean age 54, 47 female, 49 male). All patients had clinical and/or histological evidence for vasculitis and fulfilled the ACR criteria for classification. They were examined using rigid endoscopes (n=96), ENT activity score (ENTAS (3), n=37), anterior rhinomanometry (n=96), sniffin’sticks and acetic acid (n=63). Results Of 48 contacts investigated using ENTAS 38 showed no and 10 mild to high endonasal activity. By endoscopy in 86 contacts polyps, 65 septal deviations and 24 hyperplasias of the turbinates were detected. In 35 contacts (30 patients, 31%) profoundly nasal obstruction was detected and showed no correlation to ENTAS (p=0.5), polyps (p=0.7), septal deviation (p=0.5) or hyperplasia of the turbinates (p=0.08). Fourteen patients (22%) showed dysosmia. CSS-patients with compared to CSS-patients without olfactory dysfunction showed significantly less polyps (p=0.027), less often hyperplasia of the turbinates (p=0.017), less often subjective obstruction of the nose (0.002), no endonasal activity (ENTAS, p=0.04) and higher frequency of sinusitis (anamnestic data, p=0.035). No significant correlation could be detected ragarding nasal obstruction or trigeminal dysfunction and olfactory function (p=0.8, p=0.48). Compared to the general german population the risk for olfactory dysfunction is significantly increased in CSS patients Odds ratio 5.4 (95% CI 1.8 to 16, p=0.0018). Conclusions Nasal function in CSS is severely disturbed. 31% of CSS-patients suffer from profoundly nasal obstruction being one of the major complaints in chronic rhinosinusitis leading to reduced quality of life and tremendous medical costs. Olfactory dysfunction in CSS-patients compared to healthy population seems to be an effect of disease rather than obstruction and no general affection of cranial nerves was detectable. Further investigation to analyze olfactory function in detecting odors, differentiating odors and threshold as well as electrophysiological studies to differentiate peripheral and central origin of olfactory dysfunction are currently undertaken. References Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V, Mercante G, Grasselli C, Bacciu S. Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 118: 325-9, 2008. Bacciu A, Bacciu S, Mercante G, Ingegnoli F, Grasselli C, Vaglio A, Pasanisi E, Vincenti V, Garini G, Ronda N, Ferri T, Corradi D, Buzio C. Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 126: 503-9, 2006. Garske U, Haack A, Berltran O, Flores-Su’rez LF, Bremer JP, Lamprecht P, Hedderich J, Quetz J, Gross WL, Ambrosch P, Laudien M. Intra- and Interrater reliability of ...

Background Results from descriptive studies on eosinophilic granulomatosis with polyangiitis (EGPA) hint at distinct clinical subclasses, which might notably be determined by ANCA status. Objectives This study explored the possibility of subclassifying EGPA using hierarchical cluster analysis. Methods A standardized retrospective dataset from a cohort with clinical diagnoses of EGPA followed in 4 tertiary referral centers was used. Hierarchical cluster analysis using Ward’s method was performed based on the following 12 input variables assessed at diagnosis: constitutional symptoms, mucucutaneous, ophthalmological, ENT, cardiovascular, gastrointestinal, renal, and central nervous involvement, peripheral neuropathy, non-fixed lung infiltrates and ANCA positivity. The resulting clusters were described by their most prominent summary characteristics. In addition, the distribution of clinical variables according to ANCA status was compared with chi-square tests. Results The analyzed dataset included 262 EGPA cases diagnosed 1984–2012. ANCA were detected in 30.9%. The cluster analysis produced 3 clusters of respectively 39 (cluster 1), 92 (cluster 2) and 131 subjects (cluster 3). They were characterized as follows: cluster 1 by renal involvement (84.6%) and high ANCA prevalence (92.3%); cluster 2 by virtually absent renal involvement (3.3%) and ANCA (4.3%); and cluster 3 by an intermediate phenotype with renal involvement (13%), presence of ANCA (31.3%) and more frequent cardiovascular (59.5% vs. 17.9% and 35.9% for clusters 1 and 2, respectively) and gastrointestinal involvement (42% vs. 15.4% and 12%). Stratification of the 11 clinical input variables by ANCA status showed that ANCA positivity was associated with renal disease (P < 0.0001), peripheral neuropathy (P = 0.005) and constitutional symptoms (P = 0.02). Conclusions Although reinforcing the link between ANCA positivity and renal involvement, the cluster analysis does not suggest that EGPA is composed of clearly separated and mutually exclusive subclasses. Disclosure of Interest None Declared