Der Anaesthesist
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Hemophagocytic lymphohistiocytosis (HLH) has well been studied as a genetic disorder in children (primary HLH). Mutations in the regulatory complex of the cellular immune synapse lead to a loss of function of cytotoxic T‑cells and natural killer cells with excessive inflammation based on a cytokine storm. During the last decade, an increasing number of adult HLH patients without a family history of HLH (secondary or acquired HLH) have been reported. Various triggers - infections, malignancies or autoimmune diseases - result in an acquired loss of function of these cells and a sepsis-like disease. Missed or late diagnosis is believed to be a major cause of the high mortality. ⋯ Because of the similar clinical presentation to that of sepsis, HLH is often not recognized, resulting in a fatal outcome. In "sepsis" patients on the ICU with deterioration despite a standard of care, HLH needs to be considered by testing for ferritin when considering differential diagnoses. The complexity of the illness requires interdisciplinary patient care with specific integration of the hematologist in the diagnostic workup and therapeutic management, because of the frequent use of chemotherapy-based immunosuppression.
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Tracheal agenesis (TA) is a very rare congenital malformation of unknown aetiology. It is often associated with polymalformative syndromes; the neonates commonly present a critical condition during post-natal treatment. ⋯ In most cases, prognosis is very poor. The case report at hand portrays the treatment of a neonate suffering from tracheal agenesis.
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The external post-mortem examination in Germany is a non-delegable medical duty for determination of death, identity of the deceased, cause of death, manner of death, time of death and notifiable infectious diseases. Within the framework of rescue service missions the physician is limited to ascertaining that death has occurred. The determination of death must be reliable and is automatically followed by a complete external post-mortem examination of the body, if necessary by another physician. ⋯ Reliable features for the occurrence of death are injuries which are not compatible with life and brain death. The external post-mortem examination is the basis for the decision on whether further criminal investigations are necessary. The external post-mortem examination and the accompanying death certification must always be meticulously carried out.
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Here, we present a case of a tracheal fistula due to an anastomotic insufficiency following abdominothoracic esophageal resection. Despite immediate discontinuity resection, the tracheal fistula could not be surgically closed, resulting in incomplete control of the source of infection and an alternative treatment concept in the form of interventional fistula closure using a Y-tracheal stent. However, owing to existing severe acute respiratory distress syndrome (ARDS), which is associated with a considerable risk of peri-interventional hypoxia, a temporary bridging concept using venovenous extracorporeal membrane oxygenation (ECMO) was implemented successfully.
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Obesity leads to better survival in critically ill patients. Although there are several studies confirming this thesis, the "obesity paradox" is still surprising from the clinician's perspective. One explanation for the "obesity paradox" is the fact that the body mass index (BMI), which is used in almost all clinical evaluations to determine weight categories, is not an appropriate measure of fat and skeletal muscle mass and its distribution in critically ill patients. ⋯ For future clinical observation or interventional studies, single cross-sectional CT is a more sophisticated tool for measuring patients' anthropometry than a measuring tape and callipers. Patients with sarcopenic obesity, for example, who may be at a particular risk, can only be identified using imaging procedures such as single cross-sectional CT. Thus, BMI should take a back seat as an anthropometric tool, both in the clinic and in research.