[Jejunal ulcerations - a diagnostic challenge in a patient with coeliac disease]. / Jejunale Ulzerationen - eine diagnostische Herausforderung bei einem Patienten mit Zöliakie.

A subset of patients with coeliac disease (CD) suffers persistent or recurrent complaints despite a strict adherence to a gluten-free diet (GFD) that can be caused by refractory coeliac disease (RCD). We present a patient with weight loss and signs of malassimilation secondary to villous atrophy and jejunal ulcerations complicating known CD. We demonstrate a stepwise approach to the diagnosis and subtyping of RCD and to rule out important alternative causes of jejunal ulcerations. RCD can be classified as type I based on the absence or as type II based on the presence of an aberrant intestinal mucosal lymphocyte population. RCD type I shows a more benign course as these patients usually improve on a treatment consisting of nutritional support and immunosuppressive therapies such as budesonide or azathioprine. In contrast, clinical response to standard therapies in RCD type II is less certain and the prognosis is poor. Several groups suggest that RCD type II should be regarded as low-grade intraepithelial lymphoma which frequently transforms into an aggressive enteropathy associated T-cell lymphoma with a high mortality rate. Therefore, a rapid differentiation of RCD type I and RCD type II is a major clinical challenge to early initiate appropriate treatment modalities.

[Morbus Behçet or inflammatory bowel disease--a diagnostic and therapeutic dilemma]. / Morbus Behçet oder chronisch-entzündliche Darmerkrankung--ein diagnostisches und therapeutisches Dilemma.

We present the case of a 43-year old caucasian male suffering from a condition initially diagnosed as colitis ulcerosa. For 2 years Azathioprine and anti-TNF-alpha antibodies were used for treatment without convincing benefit but with serious adverse events. After the first occurrence of complex accompanying symptoms like oral and scrotal ulcerations, arthritis and scratch-induced skin lesions the differential diagnosis of a Morbus Adamantiades-Behçet with intestinal evolvement was considered. After introduction of a parenteral Ciclosporin medication, which was later switched to Tacrolimus and Azathioprin, a remission could be achieved that lasted for several months. When a drug-induced acute kidney injury occurred, the regime was changed to Golimumab and a delayed but significant improvement was achieved. To separate Morbus Adamantiades-Behçet from inflammatory bowel disease is of some difficulty, demands interdisciplinary cooperation and is the basis for a successful therapy.

Hybrid repair of the aortic arch in patients with extensive aortic disease.

OBJECTIVE: To evaluate the outcome of hybrid treatment of the aortic arch with supra-aortic debranching and endovascular stent-graft repair in a selected group of patients with complex disease. DESIGN: Case series study with retrospective analysis of prospectively collected non-randomised data. METHODS: Patients with hybrid repair of complex arch disease at a single centre over a 6-year period were enrolled in the study. Only patients with extensive arch pathologies requiring debranching of at least the left carotid artery were considered. Patients were divided into those who underwent complete and partial supra-aortic revascularisation. The χ2 test was used to evaluate differences in outcomes. Logistic regression analyses were applied to identify predictors of poor outcome. RESULTS: A total of 33 patients were included in the study. Complete and partial arch repair was performed in nine and 24 patients, respectively. The aortic disease extended to the thoracic and abdominal aorta in 39% and 52% of the patients, respectively. One-third of the patients (30%) were treated on an urgent/emergency basis. Elective 30-day mortality and morbidity rates were 13% and 35%, respectively. Early mortality was significantly higher in the complete arch repair group (p=0.046). Pre-existing renal impairment was identified as a poor prognostic factor. All extra-anatomic bypasses remained patent and no aortic disease-related deaths occurred during a mean follow-up period of 23 months (range, 1.5-58 months). Complete arch repair was associated with an increased incidence of late endoleak (p=0.018). CONCLUSIONS: Hybrid treatment of the aortic arch provides a feasible alternative treatment in patients who are high risk for conventional open surgical repair. Careful selection of patients is required to achieve satisfactory results.

Visceral ischemia and neutrophil activation in sepsis and organ dysfunction.

BACKGROUND: It has previously been shown that a rise in intraoperative neutrophil CD11b expression during supracoeliac cross-clamping is a marker for subsequent development of postoperative organ dysfunction. Prolonged visceral ischemia and increased aneurysm extent are associated with higher risks of morbidity and mortality after TAAA repair. This study investigates the relationship between visceral ischemia and neutrophil activation in sepsis and organ dysfunction following visceral reperfusion. METHOD: Fifty-one patients undergoing supracoeliac cross-clamping, 5 patients undergoing suprarenal clamping, and 8 patients undergoing infrarenal clamping for repair of aortic aneurysms were studied. Perioperative neutrophil CD11b expression was measured by flow cytometry. RESULTS: There was significant correlation between visceral clamp time and intraoperative CD11b expression. More extensive aneurysms resulted in increased visceral clamp times and CD11b expression. There were no differences between bypass and non-bypass-assisted surgery with regard to neutrophil expression. There were increased clamp time in patients who developed severe sepsis and postoperative organ dysfunction. Differences in preoperative levels of CD11b expression were observed between groups and high levels of preoperative CD11b expression were observed in patients who died intraoperatively, in type II patients who went on to develop severe sepsis and organ failure, and in patients who developed multiple organ failure rather than single organ failure. CONCLUSION: Longer periods of visceral ischemia are associated with higher levels of intraoperative CD11b expression, severe sepsis, and organ failure. High preoperative levels of CD11b may identify an "at-risk" subset of patients.