Schnitzler-Syndrom mit Urtikaria-Vaskulitis

Zusammenfassung: Das Schnitzler-Syndrom ist eine seltene Erkrankung, welche mit Urtikaria, periodischem Fieber, Knochenschmerzen bei Hyperostosis, Arthritiden und einer monoklonalen IgM-Gammopathie einhergeht. Es wurde 1974 durch die französische Dermatologin Liliane Schnitzler beschrieben. Aufgrund der sehr unterschiedlichen Symptome werden die Betroffenen oft von verschiedenen Fachärzten gesehen und sind insbesondere für Internisten, Rheumatologen, Hämatologen und Dermatologen von Interesse. Bislang war die Therapie oft schwierig und enttäuschend. Ein neuer therapeutischer Ansatz ist die Gabe eines Interleukin-1-Rezeptorantagoniste

Diffuse Bauchschmerzen und Eosinophilie

Zusammenfassung: Die eosinophile Gastroenteritis ist eine relativ seltene Erkrankung unklarer Ätiologie mit einem heterogenen Krankheitsbild. Wichtige Differenzialdiagnosen stellen intestinale parasitäre Infektionen, das hypereosinophile Syndrom, Lymphome und andere maligne und allergische Erkrankungen dar. Die Diagnose kann meist mittels Anamnese und Standardlabor, zusammen mit den Ergebnissen von Biopsien oder Parazentese gestellt werden. Milde Formen mit lediglich Diarrhö als klinischer Manifestation können symptombezogen behandelt werden. Die Therapie bei schwereren Verläufen erfolgt initial mit Kortikosteroide

Supplementary Material for: Acutely Ill Patients in Internal Medicine Departments Want Treatment for Undiagnosed, Symptomatic Skin Conditions

<b><i>Objective:</i></b> Concomitant skin conditions may be neglected in internal medicine patients due to lack of knowledge or resources. Thus, we investigated the prevalence of undiagnosed skin conditions in this population. <b><i>Methods:</i></b> 200 patients in a university medical center’s internal medicine division were examined clinically for dermatoses and quality of life in a prospective, 2-month, single-center study. <b><i>Results:</i></b> All patients had several dermatological problems (mean per patient: 13; range: 3–25). There was no relationship between the patient’s main medical problem and the number or nature of dermatological conditions. Most patients (84%) requested treatment for their skin condition during hospitalization, especially for xerosis (76%), warts (69%), seborrheic eczema (67%) and onychorrhexis (53%) but not for asymptomatic dermatoses. The impairment in skin-related quality of life was mild but significant, with a mean ± SD Dermatology Life Quality Index of 3 ± 4 (p < 0.001), and global quality of life impairment was severe (p < 0.001). <b><i>Conclusions:</i></b> Inpatients suffered from many different, mostly age-related, skin conditions that remained undiagnosed. When prompted, however, patients requested treatment, particularly for symptomatic dermatological conditions such as xerosis, revealing an unmet need that needs to be addressed by qualified evaluation and care

Diffuse Bauchschmerzen und Eosinophilie

Die eosinophile Gastroenteritis ist eine relativ seltene Erkrankung unklarer Ätiologie mit einem heterogenen Krankheitsbild. Wichtige Differenzialdiagnosen stellen intestinale parasitäre Infektionen, das hypereosinophile Syndrom, Lymphome und andere maligne und allergische Erkrankungen dar. Die Diagnose kann meist mittels Anamnese und Standardlabor, zusammen mit den Ergebnissen von Biopsien oder Parazentese gestellt werden. Milde Formen mit lediglich Diarrhö als klinischer Manifestation können symptombezogen behandelt werden. Die Therapie bei schwereren Verläufen erfolgt initial mit Kortikosteroiden. = Eosinophilic gastroenteritis is a rare clinical condition of unknown aetiology and heterogenic etiopathology. Important differential diagnoses are intestinal parasitic infections, hypereosinophilic syndrome, malignancies such as lymphoma and allergic diseases. The diagnosis can be made in most cases by patient history, routine laboratory testing and endoscopic biopsies or paracentesis. Patients with only mild diarrhea can be treated with antidiarrheal medications. More symptomatic patients are usually treated with corticosteroids

Urinary proteomic biomarkers on coronary artery disease

Urinary proteomics is emerging as a powerful non-invasive tool for diagnosis and monitoring of variety of human diseases. We tested whether signatures of urinary polypeptides can contribute to the existing biomarkers for coronary artery disease (CAD). We examined a total of 359 urine samples from 88 patients with severe CAD and 282 controls. Spot urine was analyzed using capillary electrophoresis on-line coupled to ESI-TOF-MS enabling characterization of more than 1000 polypeptides per sample. In a first step a "training set" for biomarker definition was created. Multiple biomarker patterns clearly distinguished healthy controls from CAD patients, and we extracted 15 peptides that define a characteristic CAD signature panel. In a second step, the ability of the CAD-specific panel to predict the presence of CAD was evaluated in a blinded study using a "test set." The signature panel showed sensitivity of 98% (95% confidence interval, 88.7-99.6) and 83% specificity (95% confidence interval, 51.6-97.4). Furthermore the peptide pattern significantly changed toward the healthy signature correlating with the level of physical activity after therapeutic intervention. Our results show that urinary proteomics can identify CAD patients with high confidence and might also play a role in monitoring the effects of therapeutic interventions. The workflow is amenable to clinical routine testing suggesting that non-invasive proteomics analysis can become a valuable addition to other biomarkers used in cardiovascular risk assessmen

Urinary proteomic diagnosis of coronary artery disease: identification and clinical validation in 623 individuals

Objectives We studied the urinary proteome in a total of 623 individuals with and without coronary artery disease (CAD) in order to characterize multiple biomarkers that enable prediction of the presence of CAD. Methods Urine samples were analyzed by capillary electrophoresis coupled online to micro time-of-flight mass spectrometry. Results We defined a pattern of 238 CAD-specific polypeptides from comparison of 586 spot urine samples from 408 individuals. This pattern identified patients with CAD in a blinded cohort of 138 urine samples (71 patients with CAD and 67 healthy individuals) with high sensitivity and specificity (area under the receiver operator characteristic curve 87%, 95% confidence interval 81-92) and was superior to previously developed 15-marker (area under the receiver operator characteristic curve 68%, P &lt; 0.0001) and 17-marker panels (area under the receiver operator characteristic curve 77%, P &lt; 0.0001). The sequences of the discriminatory polypeptides include fragments of alpha-1-antitrypsin, collagen types 1 and 3, granin-like neuroendocrine peptide precursor, membrane-associated progesterone receptor component 1, sodium/potassium-transporting ATPase gamma chain and fibrinogen-alpha chain. Several biomarkers changed significantly toward the healthy signature following 2-year treatment with irbesartan, whereas short-term treatment with irbesartan did not significantly affect the polypeptide pattern. Conclusion Urinary proteomics identifies CAD with high confidence and might also be useful for monitoring the effects of therapeutic interventions

Vascular function in patients with end-stage renal disease and/or coronary artery disease: A cardiac magnetic resonance imaging study

Decreased arterial compliance in end-stage renal disease (ESRD) is associated with increased cardiovascular risk. Our aim was to examine aortic compliance in patients with ESRD using cardiac magnetic resonance imaging (MRI) and to compare these with patients with advanced atherosclerotic disease who are known to be at high cardiovascular risk. We examined a total of 83 subjects matched for age: 24 had ESRD and were on dialysis therapy for 3plusminus6 years, 24 had severe coronary artery disease (CAD), 11 had both ESRD and CAD (4plusminus5 years on dialysis therapy), and 24 healthy subjects with no evidence of CAD. Vascular and cardiac function was assessed using cardiac MRI. Aortic compliance was significantly reduced in patients with CAD compared to control subjects (11.3plusminus6.3 mldot10-3/mm Hg vs 15.6plusminus6.0 mldot10-3/mm Hg, P=0.009). Patients with ESRD also exhibited significantly reduced aortic compliance compared to healthy controls (12.4plusminus5.8 mldot10-3/mm Hg vs 15.6plusminus6.0 ml 10-3/mm Hg, P=0.012), whereas there was no significant difference in aortic compliance between patients with CAD and ESRD. Even in the absence of symptomatic CAD, patients with ESRD have significantly reduced aortic compliance compared to normal subjects. Patients with ESRD have equivalent aortic compliance to patients with advanced CAD. These findings suggest that a significantly reduced aortic compliance is one of many mechanisms promoting premature cardiovascular events in patients with ESRD compared to age-matched controls from the general population