Refine
Year of publication
Document Type
- Article (38)
- Review (23)
- Conference Proceeding (7)
Has Fulltext
- yes (68)
Is part of the Bibliography
- no (68)
Keywords
- Immunturbidimetrie (2)
- guidelines (2)
- "alternative" technology (1)
- "conventional" analysis technics (1)
- 18-Hydroxycorticosteron (1)
- 18-OH corticosterone (1)
- Access® immunoassay system (1)
- Access®-Immunoassaysystem (1)
- Akute (1)
- Anämie (1)
- Atomic and Molecular Physics (1)
- Behandlungskontrolle (1)
- Bence Jones (BJ) proteinuria (1)
- Bence Jones-Proteinurie (1)
- Bilirubinbestimmung (1)
- C-reaktive protein (1)
- C-reäktives Protein (1)
- CRP (1)
- CVID (1)
- Cell signalling (1)
- Cytokine (1)
- Differenzierung (1)
- Ektachem® (1)
- Entzündung (1)
- Erprobung (1)
- Erwachsene (1)
- European Society for Immunodeficiencies (ESID) (1)
- German PID-NET registry (1)
- Gesamteiweißbestimmung im Harn (1)
- Haematopoietic stem cells (1)
- Hitachi 705 (1)
- Hormone (1)
- IgG substitution therapy (1)
- Immunfixations-Elektrophorese (1)
- Immunnephelometrie (1)
- Immunonephelometry (1)
- Immunoturbidimetry (1)
- Impräzision (1)
- Inflammation (1)
- Kallestad QM 300 (1)
- Kallestad QM 30 (1)
- Nachteile (1)
- Nebennierenrinden-Adenom (1)
- Nebennierenrinden-Hyperplasie (1)
- Neugeborene (1)
- Non-clear cell renal cell cancer (1)
- PID prevalence (1)
- Pharmaka (1)
- Phase II trial (1)
- Phase-Proteine (1)
- Phospholipasen A2 (1)
- Plasma proteins (1)
- Plasmaprotein analyzer (1)
- Plasmaproteinanalysensysteme (1)
- Plasmaproteinbestimmung im Harn (1)
- Plasmaproteine (1)
- Prostaglandine (1)
- Proteinbestimmung (1)
- Proteinuria (1)
- Proteinurie (1)
- Präsenzdiagnostik (1)
- Prävalenz (1)
- Qualitätssicherung (1)
- RA-1000 (1)
- Randomized trial (1)
- Reflotron® (1)
- Retikulozytenbestimmung (1)
- Richtigkeit (1)
- Richtlinien (1)
- Schilddrüsenfunktion (1)
- Screening (1)
- Self-renewal (1)
- Sensitivität (1)
- Seralyzer-System (1)
- Seralyzer® (1)
- Spezifität (1)
- Sunitinib (1)
- T4-Bestimmung (1)
- T4-analysis (1)
- Temsirolimus (1)
- Teststreifenanalytik (1)
- Tetrahydro-Aldosteron (1)
- Tris-Teepol-Puffer (1)
- Tris-Teepol-buffer (1)
- Tumormarker (1)
- Turbidimetric determination (1)
- Vision® (1)
- Vorteile (1)
- accuracy (1)
- acute phase reactants (1)
- adrenal adenomas (1)
- adults (1)
- advantages and shortcomings (1)
- alternative Analysentechniken (1)
- anemia (1)
- bilirubin determination (1)
- classification (1)
- cytokines (1)
- drugs (1)
- ectosomes (1)
- evaluation (1)
- exosomes (1)
- extracellular vesicles (1)
- hormones (1)
- immunchemische Bestimmungsmethoden (1)
- immunoassay (1)
- immunofixation electrophoresis (1)
- immunoturbidimetric inhibition technique (1)
- immunturbidimetrische Inhibitionstechnik (1)
- imprecision (1)
- konventionelle Analysentechniken (1)
- miRNAs (1)
- microparticles (1)
- microvesicles (1)
- minimal information requirements (1)
- monoclonal antibody (1)
- monoklonale Antikörper (1)
- newborn (1)
- phospholipase A2 (1)
- plasma protein determination in urine (1)
- polyclonal antibody (1)
- polyklonale Antikörper (1)
- predictive value (1)
- prevalence (1)
- primary aldosteronism (1)
- primary immunodeficiency (PID) (1)
- primärer Hyperaldosteronismus (1)
- prostanoids (1)
- prädiktive Werte (1)
- quality assurance (1)
- real time analysis (1)
- registry for primary immunodeficiency (1)
- reproducibility (1)
- response to therapy (1)
- reticulocyte count (1)
- rigor (1)
- screening (1)
- sensitivity (1)
- solid phase reagent chemistry (1)
- specificity (1)
- standardization (1)
- tetrahydroaldosterone (1)
- thyroid functioning (1)
- total protein determination in urine (1)
- total urinary protein (1)
- tumor marker (1)
Institute
- Medizin (55)
- Physik (12)
- Georg-Speyer-Haus (2)
- Biowissenschaften (1)
- Sportwissenschaften (1)
Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs.
Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel.
Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy.
Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment.
We present measurements of exclusive ensuremathπ+,0 and η production in pp reactions at 1.25GeV and 2.2GeV beam kinetic energy in hadron and dielectron channels. In the case of π+ and π0 , high-statistics invariant-mass and angular distributions are obtained within the HADES acceptance as well as acceptance-corrected distributions, which are compared to a resonance model. The sensitivity of the data to the yield and production angular distribution of Δ (1232) and higher-lying baryon resonances is shown, and an improved parameterization is proposed. The extracted cross-sections are of special interest in the case of pp → pp η , since controversial data exist at 2.0GeV; we find \ensuremathσ=0.142±0.022 mb. Using the dielectron channels, the π0 and η Dalitz decay signals are reconstructed with yields fully consistent with the hadronic channels. The electron invariant masses and acceptance-corrected helicity angle distributions are found in good agreement with model predictions.
Background: Non-clear cell renal cell cancers (nccRCC) are rare entities, and the optimal therapy in metastatic disease has still to be defined. Methods: In this small prospectively randomized phase IIa multicenter trial, we investigated temsirolimus (TEM) versus sunitinib (SUN) as first-line therapy in patients with metastatic nccRCC. The patients were randomized 1:1 to either TEM in a dose of 25 mg i.v. once a week or SUN with 50 mg p.o. daily for 4 weeks on and 2 weeks off. Primary endpoint was progression-free survival (PFS). In total, 22 patients were included with predominantly papillary RCC (16/22) followed by chromophobe RCC and others. Results: The male to female ratio was 16:6. The tumor control rate (CR + PR + SD) was 58% for TEM and 90% for SUN-treated patients. There was also a trend for improved PFS with 9.3 versus 13.2 months (HR 1.64; 95% CI 0.65–4.18) in favor of SUN. There was no trend for overall survival. Conclusions: Despite this trial had to be terminated earlier due to low recruitment, the results match the other studies published so far with the mTOR inhibitor everolimus and SUN, which show a trend in favor of SUN for ORR and PFS.
Haematopoietic stem cells (HSCs) require the right composition of microRNAs (miR) for proper life-long balanced blood regeneration. Here we show a regulatory circuit that prevents excessive HSC self-renewal by upregulation of miR-193b upon self-renewal promoting thrombopoietin (TPO)-MPL-STAT5 signalling. In turn, miR-193b restricts cytokine signalling, by targeting the receptor tyrosine kinase c-KIT. We generated a miR-193b knockout mouse model to unravel the physiological function of miR-193b in haematopoiesis. MiR-193b−/− mice show a selective gradual enrichment of functional HSCs, which are fully competent in multilineage blood reconstitution upon transplantation. The absence of miR-193b causes an accelerated expansion of HSCs, without altering cell cycle or survival, but by decelerating differentiation. Conversely, ectopic miR-193b expression restricts long-term repopulating HSC expansion and blood reconstitution. MiR-193b-deficient haematopoietic stem and progenitor cells exhibit increased basal and cytokine-induced STAT5 and AKT signalling. This STAT5-induced microRNA provides a negative feedback for excessive signalling to restrict uncontrolled HSC expansion.
Zur quantitativen Plasmaproteinbestimmung sind die Immunnephelometrie und die Immunturbidimetrie häufig eingesetzte Bestimmungstechniken. Aufgrund der kürzeren Analysenzeit bei vergleichbarer oder besserer Präzision und Empfindlichkeit haben diese Techniken die radiale Immundiffusion in vielen Laboratorien ersetzt.
Die mechanisierte Plasmaproteinbestimmung erfolgt als Antigen-Antikörper-Reaktion entweder mit Proteinanalyzern oder mit klinisch-chemischen Analysensystemen, an denen normalerweise Enzyme und Substrate bestimmt werden.
Zwischen den verschiedenen Plasmaproteinen im Serum besteht ein bis zu 10.OOOfacher Konzentrationsunterschied und die biologische Varianz des einzelnen Plasmaproteins ist breit Damit die Plasmaproteine mit hoher analytischer Sensitivität über einen weiten Konzentrationsbereich, mit guter Präzision und der erforderlichen Richtigkeit mechanisiert bestimmt werden können, ist eine gute Adaption des Immunreagenzes auf das mechanisierte Analysensystem erforderlich. Diese Übersicht soll dazu Grundlagen vermitteln.
Die Entzündung ist eine Folge von Reaktionen mit der Zielsetzung, die Ausbreitung einer Gewebeschädigung, oder eines Infektionserregers einzudämmen. Zelluläre und humorale Mechanismen interagieren dabei in einem komplexen Netzwerk. In diesem Übersichtsbeitrag zeigen wir die wichtigsten Wege des inflammatorischen Reaktionsgeschehens auf und diskutieren die Bedeutung von Laboratoriumsuntersuchungen für die Diagnostik und das Monitoring von Entzündungen.
Wesentliche Schritte im Ablauf der Entzündungsreaktion sind
- die Synthese von Prostaglandinen aus Arachidonsäure, die durch Phospholipasen A2 (PLA2)-katalysierte Hydrolyse aus Membranphospholipiden gebildet wird;
- Interaktionen zwischen Gefäßendothel und Leukozyten, die Leukozytenextravasation und die Freisetzung freier Sauerstoffradikale und von Elastase im Gewebe;
- die Bildung inflammatorischer Cytokine, ihr Effekt auf Entzündungszellen und ihre systemische Wirkung auf Organe;
- die Synthese von Akute-Phase-Proteinen, deren Plasmakonzentration bei Entzündung als Antwort auf eine Vielfalt von Schädigungen ansteigt.
Zur Diagnostik und Verlaufsbeurteilung entzündlicher Krankheiten hat die Bestimmung des C-reaktiven Proteins den höchsten Stellenwert. Die Elastase hat nur eine begrenzte Bedeutung. Die Bestimmung von PLA2, der 'inflammatorischen Cytokine TNFa, IL-1, IL-6 und des s!L-2R als generelle Entzündungsmarker kann in der Routinediagnostik noch nicht empfohlen werden. Eingehende klinische Untersuchungen zur diagnostischen Bedeutung müssen noch abgewartet werden.