EH 1_2016

• therapy driven interface • quickly ready for use • scalable trend • redundant controls • high - resolution display NEW: The Upgrade to make it easy for you Visit us at the ISICEM Brussels, Hall 2, booth 2.61 - 2.64 and our Symposium: „EVOLUTION OF INTERVENTIONAL LUNG ASSIST“ Thursday, March 17th 12:30 - 13:30 in 100 Hall console www.novalung.com www.healthcare-in-europe.com 3 NEWS & MANAGEMENT ‘that a high incidence of milder forms of microcephaly has occurred well before the current outbreak, but that only those extreme cases, with classical phenotypes, were being notified. As the number of extreme cases increased over these past three or four months so did awareness of health professionals who started to notify milder forms.’ (Ref: Report published Feb. 4 in the Bulletin of the World Health Organization. Mattos’s team did find an increase in the most severe forms of micro- cephaly in the last months of 2015, and doctors have reported a recent rise in the number of severe micro- cephaly cases in Brazil. Critical to understanding the degree of micro- cephaly is the need to standardise diagnostic criteria. Researchers at the Federal University of Pelotas, Brazil reported that ‘…the num- ber of suspected cases relied on a screening test that had very low specificity and therefore over-esti- mated the actual number of cases by including mostly normal children with small heads.’ Currently, health coordinator Dr Adriana Scavuzzi and team at IMIP, a maternal and infant care hospital in Recife, is leading a case-controlled study in which 200 women are being interviewed regarding their lives and health history – includ- ing vaccinations received. Half the mothers have microcephaly babies. The rest are normal. Whilst she her- self is ‘almost convinced” of a micro- cephaly-Zika connection, this could blow away at least some demand- ing questions. Meanwhile, a cloud of pesticide ‘fog’ looms over Brazil – prompting questions about the health of people liberally sprayed from moving vehicles, without prior warning, in the battle to kill Aedes – scapegoat or villain. ment commence. ‘Even very simple measures can reduce the impact of the disease and the high early mortality rates drastically,’ point out Dr Stephan Lobitz of the Clinic for Paediatrics, Division of Oncology and Haematology at the Charité Hospital in Berlin, one of the three centres participating in the study. ‘Educating parents to recognise acute anaemia and to seek medical help quickly if a child develops a fever, along with prophylactic treat- ment with penicillin and vaccina- tions, can prevent almost all related deaths in childhood and adoles- cence.’ However, a prerequisite for this is that the blood disorder has been diagnosed and that the par- ents have been informed about it. Therefore, Lobitz believes, standard newborn screening is essential. Sickle cell anaemia results from inherited changes to the haemoglo- bin in red blood cells (erythrocytes). The most important function of hae- moglobin is to carry oxygen from the lungs to organs and tissues. Erythrocytes are filled with fluid and haemoglobin usually remains dissolved in this fluid. Erythrocytes can be deformed However, the haemoglobin of patients with sickle cell anaemia clumps together into solid structures inside the erythrocytes once it has released the oxygen and loses its functionality. These solid structures impact on the usually very high flexibility of the erythrocytes and damage the cells, shortening their lifespan. As a result, the erythro- cytes become deformed, remain in small blood vessels and block them. This leads to small infarctions and very small damage to the organs, which, over time, can add up and in turn lead to a significant impairment to the function of certain organs, and even their total destruction. Simultaneously, the premature death of blood cells causes an increased accumulation of metabolic products, which in turn can damage larger blood vessels. Along with these slow, but stead- ily progressive changes there are also acute problems caused by the sudden accumulation of large num- bers of sickle cells. Screening with tandem mass spectrometry These so-called crises, the triggers of which are not yet entirely clearly defined, often go hand in hand with severe pain, frequently can only be treated in hospital and should be treated with the strongest painkill- ers, severely impacting on qual- ity of life. There are other acute and serious complications, such as splenic sequestration, where blood only flows into the spleen but not out again. Every ninth patient is destined to suffer a stroke before his or her 18th birthday. Viral infections can lead to acute, life-threatening anaemia. The project is now examining whether testing for sickle cell dis- ease in Germany is possible with standard laboratory procedures. ‘German laboratories are usually not equipped with the devices that are used internationally,’ Lobitz reports. However, he believes that tandem mass spectrometry, which is usually part of standard screening in this country, could also be used to diag- nose sickle cell disease. ‘We would have to ensure that reliable results are available fast and that the affect- ed newborns can be transferred quickly to wards that specialise in the treatment of blood disorders,’ he emphasised. So far, 90,000 children have been examined in the context of screen- ing at the three locations (Hamburg, Heidelberg, Berlin); 24 were con- firmed with sickle cell disease. It is not yet clear how the findings will be translated into actions. The rel- evant political negotiations are cur- rently taking place, Lobitz reports. Researchers are also receiving sup- port from the German Society of Internal Medicine (DGIM). ‘The most important concern for DGIM members is also to find solutions for the best possible diagnosis and treatment for diseases that only affect a relatively small number of people,’ says Professor Ulrich Fölsch, General Secretary of the DGIM from Kiel. Therefore the DGIM welcomes advances made by this research group. Cases are rare but rising due to human migration Should we screen for sickle cell disease? Report: Sylvia Schulz Patients who do not have sickle cell disease diagnosed in time often die in childhood or adolescence. In countries such as the USA, The Netherlands and France tests for this disease are already part of standard newborn screening. The families of those affected originate from areas where Malaria has been, or still is, endemic. The prevalence is highest in West Africa. In this region, every fifth death amongst children under the age of five is caused by sickle cell disease. Testing for sickle cell may become routine for babies In countries such as Germany early detection is currently not assured. However, three model projects in Berlin, Hamburg and Heidelberg have now been set up to find out whether testing for sickle cell dis- ease can and should become part of regular newborn screening. Only once the disease has been diagnosed can the appropriate treat- Every year around a quarter of a million children worldwide die from sickle cell disease. In Germany around 3,000 people suffer from this rare inherited blood disorder. However, the trend is rising: the influx of refugees is most probably linked to this increase, since the disease exclusively affects those from a migrant background. Dr Stephan Lobitz works in the Clinic for Paediatrics, Division of Oncology/ Haematology at the Charité Hospital Berlin. After completing his medical degree at the Heinrich-Heine University in Dusseldorf he worked as a research associate at the Charité Paediatrics Clinic. He has specialised in Paediatric Medicine since 2012 and is also a Speaker for the management of the GPOH (Society for Paediatric Oncology and Haematology) Sickle Cell Disease Registry Study. Red blood cells under the microscope: In the case of sickle cell diseases they may take the form of sickles

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