Reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation.

QUESTIONS UNDER STUDY: starting treatment of reactive macrophage activation syndromes as early as possible (rMAS, haemophagocytic lymphohistiocytosis), e.g., with intravenous immunoglobulins (IVIG), seems to be essential for optimal outcome. However, there is no diagnostic gold standard which reliably indicates need for early treatment. We used a simple screening strategy consisting of serum ferritin measurements and/or morphological assessment of haemophagocytosis and compared the studied patient population with published series. METHODS: Retrospective analysis of clinical and laboratory data of 57 patients experiencing 60 episodes of rMAS. RESULTS: Screening by serum ferritin measurements and/or morphological assessment of haemophagocytosis of patients presenting with a systemic inflammatory response syndrome (SIRS) indicates that rMAS might be considerably more frequent than stated in the literature. Serum ferritin exceeded >10,000 microg/L in 91% rMAS episodes. Although the patient population studied was otherwise similar in most aspects to the published rMAS series, the fact that 40% of patients fulfilled the criteria for Still's disease (SD) as the disorder underlying rMAS is remarkable and questions the distinct nature of the two diseases. IVIG responders and non-responders did not differ regarding their initial characteristics with exception to the timepoint of IVIG administration, confirming the importance of early treatment initiation. Malignancy-associated rMAS however, has a poor prognosis and seems to be refractory to manipulation with IVIG in most instances, even when responding initially. CONCLUSIONS: rMAS has to be considered in patients with a SIRS- or SD-like clinical presentation. Hyperferritinaemia >or=10,000 microg/l seems to be a good marker for defining patients with or at risk for developing rMAS and should be completed with a morphological assessment of haemophagocytosis. The perception of acute SD and rMAS as two distinct entities has to be questioned at least in a subgroup of patients.

Improvement of nitrogen removal at WWTP Zürich Werdhöelzli after connection of WWTP Zürich-Glatt.

Optimisation of nitrifying activated sludge plants towards nutrient removal (denitrification and enhanced P-removal) leads to a substantial reduction of operating costs and improves effluent and operating conditions. At WWTP Zürich-Werdhöelzli, initially designed for nitrification only, an anoxic zone of 28% of total activated sludge volume was installed and allowed 60% nitrogen elimination besides several other optimisations. In 2001 the operation of WWTP Zürich-Glatt was stopped and the wastewater was connected to WWTP Werdhöelzli. To improve nitrogen removal, WWTP Werdhöelzli co-financed two research projects; one for separate digester supernatant treatment with the anammox process operating two SBRs in series and the other applying NH4 sensors for aeration control in order to decrease energy consumption and raise effluent quality. The results of both projects and the consequences for WWTP Werdhöelzli are discussed in this paper.

Life threatening bleeding under adequate oral anticoagulation. Cases 4a, b.

We report on two men with severe and recurrent bleeding episodes under well performed phenprocoumon therapy. Both patients showed an INR value within the therapeutic/subtherapeutic range with an inappropriately prolonged activated partial thromboplastin time (aPTT). The vitamin K-dependent coagulation factors were in the expected range, except for factor IX, which was as low as in moderate haemophilia B. After substitution of vitamin K, factor IX and the aPTT recovered completely in one case. In the other case factor IX was not measured but aPTT normalized. Diagnosis of an increased sensitivity of factor IX to phenprocoumon was assumed and proven in both cases by the demonstration of a missense mutation at ALA-10 in the factor IX propeptide.