Eine akute Nierenschädigung (AKI) tritt heute bei 50 % aller kritisch kranken Patienten auf und etwa 15 % müssen mit einer Nierenersatztherapie (NET) behandelt werden. Obwohl eine NET ein häufiges ...und essenzielles Organersatzverfahren in der deutschen Intensivmedizin darstellt, ist es ist nicht bekannt, in welchem Umfang Nierenersatzverfahren zur Verfügung stehen, wer mit welcher Qualifikation eine NET durchführt, welche Formen der Antikoagulation verwendet werden und wie die Dosis der NET verschrieben wird. Die Deutsche Interdisziplinäre Vereinigung für Intensiv- und Notfallmedizin (DIVI) hat deshalb Ende 2019 unter ihren Mitgliedern eine Umfrage zu den strukturellen Gegebenheiten der NET in ihrem Arbeitsumfeld durchgeführt. Es konnten 897 Datensätze erfasst werden (31,1 % der Befragten), anhand derer die aktuellen strukturellen und prozeduralen Gegebenheiten bei der Durchführung der NET auf deutschen Intensivstationen beschrieben werden können. Es waren Krankenhäuser aller Versorgungsstufen vertreten, allerdings waren Krankenhäuser mit einer Bettenzahl von > 400 Betten (69,1 %) und Krankenhäuser der Schwerpunkt- und Maximalversorgung und Unikliniken (74,5 %) verstärkt vertreten. Kontinuierliche Nierenersatzverfahren stehen auf 93,3 % und intermittierende Verfahren auf 75,8 % der Intensivstationen in Deutschland zur Verfügung. Die Indikation zur NET wird in 91,9 % durch eine/n Facharzt/Fachärztin oder einen Facharzt/Fachärztin mit Zusatzweiterbildung Intensivmedizin und/oder Nephrologie gestellt. In Fragen der Therapiedurchführung sind jedoch Aspekte der Dialysedosis besser zu implementieren und dokumentieren.
To describe three coronavirus disease 2019 patients suffering from acute respiratory distress syndrome under venovenous extracorporeal membrane oxygenation therapy and tight anticoagulation ...monitoring presenting a novel pattern of multifocal brain hemorrhage in various degrees in all cerebral and cerebellar lobes.
Clinical observation of three patients. Post mortem examinations.
Two ICUs at the University Hospital Erlangen.
Three patients (medium age 56.6 yr, two male with hypertension and diabetes, one female with no medical history) developed severe acute respiratory distress syndrome on the basis of a severe acute respiratory syndrome coronavirus 2 infection. All required mechanical ventilation and venovenous extracorporeal membrane oxygenation support.
Clinical observation, CT, data extraction from electronic medical records, and post mortem examinations.
We report on an unusual multifocal bleeding pattern in the white matter in three cases with severe acute respiratory distress syndrome due to coronavirus disease 2019 undergoing venovenous extracorporeal membrane oxygenation therapy. Bleeding pattern with consecutive herniation was found in CT scans as well as in neuropathologic post mortem examinations. Frequency for this unusual brain hemorrhage in coronavirus disease 2019 patients with extracorporeal membrane oxygenation therapy at our hospital is currently 50%, whereas bleeding events in extracorporeal membrane oxygenation patients generally occur at 10-15%.
Multifocality and high frequency of the unusual white matter hemorrhage pattern suggest a coherence to coronavirus disease 2019. Neuropathological analyses showed circumscribed thrombotic cerebrovascular occlusions, which eventually led to microvascular and later on macrovascular disseminated bleeding events. However, signs of cerebrovascular inflammation could not be detected. Polymerase chain reaction analyses of brain tissue or cerebrospinal fluid remained negative. Increased susceptibility for fatal bleeding events should be taken into consideration in terms of systemic anticoagulation strategies in coronavirus disease 2019.
A new restricted access molecularly imprinted polymer coated with bovine serum albumin (RAMIP-BSA) was developed, characterized, and used for direct analysis of chlorpromazine in human plasma ...samples. The RAMIP-BSA was synthesized using chlorpromazine, methacrylic acid, and ethylene glycol dimethacrylate as template, functional monomer, and cross-linker, respectively. Glycerol dimethacrylate and hydroxy methyl methacrylate were used to promote a hydrophilic surface (high density of hydroxyl groups). Afterward, the polymer was coated with BSA using glutaraldehyde as cross-linker, resulting in a protein chemical shield around it. The material was able to eliminate ca. 99 % of protein when a 44-mg mL
−1
BSA aqueous solution was passed through it. The RAMIP-BSA was packed in a column and used for direct analysis of chlorpromazine in human plasma samples in an online column switching high-performance liquid chromatography system. The analytical calibration curve was prepared in a pool of human plasma samples with chlorpromazine concentrations ranging from 30 to 350 μg L
−1
. The correlation coefficient obtained was 0.995 and the limit of quantification was 30 μg L
−1
. Intra-day and inter-day precision and accuracy presented variation coefficients and relative errors lower than 15 % and within −15 and 15 %, respectively. The sample throughput was 3 h
−1
(sample preparation and chromatographic analysis steps) and the same RAMIP-BSA column was efficiently used for about 90 cycles.
Aromatic l-amino acid decarboxylase (AADC, EC 4.1.1.28) deficiency is a rare genetic disorder characterized by developmental delay, oculogyric crises, autonomic dysfunction and other problems, caused ...by biallelic mutations in the DDC gene leading to deficient activity of aromatic l-amino acid decarboxylase, an enzyme involved in the formation of important neurotransmitters, such as dopamine and serotonin. A clinical development program of gene therapy for AADC deficiency is ongoing. An important step for the success of this therapy is the early and precise identification of the affected individuals, but it has been estimated that around 90% of the cases remain undiagnosed. The availability measurement of the AADC activity is mandatory for an accurate biochemical diagnosis. Based on these statements, our objectives were to develop a liquid chromatography tandem mass spectrometry (LC-MS/MS) method suitable for the determination of the AADC activity, and to evaluate its capacity to confirm the deficiency of AADC in potential patients in Brazil. The AADC activities were measured in plasma samples of seven AADC deficient patients and 35 healthy controls, after enzymatic reaction and LC-MS/MS analysis of dopamine, the main reaction product. The results obtained showed clear discrimination between confirmed AADC deficient patients and healthy controls. The method presented here could be incorporated in the IEM laboratories for confirmation of the diagnosis of when a suspicion of AADC deficiency is present due to clinical signs and/or abnormal biomarkers, including when an increased level of 3-O-methyldopa (3-OMD) is found in dried blood spots (DBS) samples from high-risk patients or from newborn screening programs.
•A new LC-MS/MS method for the assay of aromatic amino acid decarboxylase (AADC) in plasma samples is presented.•The assay is focused in the measurement of dopamine after the addition of L-DOPA in the reaction mix.•A clear discrimination between confirmed AADC deficient patients and healthy controls was achieved.•Incorporation of the new method by reference laboratories could be considered.
Niemann-Pick disease type C (NPC) is a lysosomal disorder caused by impaired cholesterol metabolism. Levels of lysosphingomyelin 509 (LysoSM509) have been shown elevated in dried blood spots (DBS) of ...NPC and acid sphingomyelinase deficiency patients. In this study, we report our experience using a two-tier approach (1st tier is the quantification of lysoSM509 by ultra-performance liquid chromatography tandem mass spectrometry followed by the 2nd tier with next-generation sequencing of the NPC1 and NPC2 genes). DBS samples from 450 suspected patients were received by the NPC Brazil network. Of these, 33 samples had elevated levels of lysoSM509, and in 25 of them, variants classified as pathogenic, likely pathogenic, or of unknown significance were identified in the NPC1 or NPC2 genes by next-generation sequencing. The quantification of lysoSM509 in DBS as a first-tier test for the diagnosis of NPC followed by molecular analysis of the NPC1 and NPC2 genes almost doubled the detection rate when compared to the performance of chitotriosidase activity as a first-tier biomarker, and it could likely be increased with the addition of a third tier with MLPA of the two genes involved. This strategy seems suitable for the neonatal screening (NBS) of NPC if this disease is eventually adopted by NBS programs.
This study evaluated the use of waste materials to produce Technosol, a "tailor-made" soil consisting of construction and demolition waste (CDW), solid waste from stone mining (WSM), natural soil ...(NS), and compost from plant pruning (CPP), for improving soil quality in forest recovery projects. Samples of CDW were characterized using X-ray fluorescence and X-ray diffraction. The potential of the Technosol as a substrate was assessed using a mixture of "Class A" CDW, WSM, NS, and CPP in the following proportions: (a) 10 % CDW + 42.5 % WSM + 42.5 % NS + 5 % CPP, (b) 20 % CDW + 37.5 % WSM + 37.5 % NS + 5 % CPP, and (c) natural soil as a control. A randomized block design was used with nine treatments and three replications using 5.5 L pots. The experimental treatments consisted of two proportions of mixtures, natural soil, and three tree species: pioneer, Cecropia pachystachya, secondary, Handroanthus impetiginosus and climax, Copaifera langsdorffii. The activity of the enzymes β-glucosidase and arylsulfatase was determined in the Technosol and natural soil at the end of the experiment. During 180 days, the height of the plants and the diameter of the stem were determined at intervals of 30 days and, at the end of the experiment, the aerial part dry mass (leaves and trunk) and roots were evaluated. The Technosol constructed from the mixture (a) proved to be viable for improving soil quality, as indicated by a greater enzymatic activity compared to other soils, and for the growth of H. impetiginosus seedlings, showing the capacity to gain plant height over 180 days after planting.
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•Technosol made with urban and industrial waste improved enzymatic activity.•H. impetiginosus grown up in Technosol with 52.5 % industrial and 47.5 natural wastes.•The Technosol proved to be viable for improving biological soil quality.