The Danish National Acute Leukemia Registry Østgård, Lene Sofie Granfeldt; Nørgaard, Jan Maxwell; Raaschou-Jensen, Klas Kræsten ...
Clinical epidemiology,
01/2016, Letnik:
8
Journal Article
Recenzirano
Odprti dostop
The main aim of the Danish National Acute Leukemia Registry (DNLR) was to obtain information about the epidemiology of the hematologic cancers acute myeloid leukemia (AML), acute lymphoblastic ...leukemia (ALL), and myelodysplastic syndrome (MDS).
The registry was established in January 2000 by the Danish Acute Leukemia Group and has been expanded over the years. It includes adult AML patients diagnosed in Denmark since 2000, ALL patients diagnosed since 2005, and MDS patients diagnosed since 2010. The coverage of leukemia patients exceeds 99%, and the coverage of MDS patients is currently 90%.
Approximately, 250 AML patients, 25 ALL patients, and 230 MDS patients are registered in the DNLR every year. In January 2015, the registry included detailed patient characteristics, disease characteristics, treatment characteristics, and outcome data on more than 3,500 AML, 300 ALL, and 1,100 MDS patients. Many of the included prognostic variables have been found to be of high quality including positive predictive values and completeness exceeding 90%. These variables have been used in prognostic observational studies in the last few years. To ensure this high coverage, completeness, and quality of data, linkage to the Danish Civil Registration System and the Danish National Registry of Patients, and several programmed data entry checks are used.
The completeness and positive predictive values of the leukemia data have been found to be high. In recent years, the DNLR has shown to be an important high-quality resource for clinical prognostic research.
Here, we present emission data on VOCs and particles emitted during simulated use of four commercial nanofilm spray products (NFPs) used for making easy-to-clean or self-cleaning surfaces on floors, ...ceramic tiles, and windows. The aim was to characterize the emitted VOCs and to provide specific source strength data for VOCs and particles released to the air during use of the products. Containers with NFP were mounted on a spray-stand inside a closed stainless steel chamber with no air exchange. NFPs were sprayed in amounts corresponding to 1 m2 surface toward a target plate at a distance of 35 cm. Released VOCs were measured by a combination of air sampling on Tenax TA adsorbent followed by thermal desorption GC/MS and GC/FID analysis and real time measurements using a miniature membrane inlet mass spectrometer. Particles were measured using a fast mobility particle sizer and an aerosol particle sizer. A number of VOCs were identified, including small alcohols, ketones and ethers, chlorinated acetones, a perfluorinated silane, limonene, and cyclic siloxanes. The number of generated particles was on the order of 3 × 108 to 2 × 1010 particles/m3 per g sprayed NFP and were dominated by nanosize particles.
Background
Portal vein occlusion (PVO) is an established method to increase the volume of the future liver remnant (FLR). The main reasons for not proceeding to radical hepatectomy are lack of volume ...increase and tumor progression due to a wait-time interval of up to 8 weeks. The hypothesis was that the increase in FLR volume is not linear and is largest during the first weeks.
Methods
Patients with colorectal liver metastases (CRLM) and standardized future liver remnant (sFLR) < 30% treated with PVO were prospectively included. All patients had at least one CT evaluation before radical hepatectomy.
Results
Forty-eight patients were included. During the first week after PVO, the kinetic growth rate (KGR) was 5.4 (± 4), compared to 1.5 (± 2) between the first and second CT (
p
< 0.05). For patients reaching adequate FLR and therefore treated with radical hepatectomy, the KGR was 7 (± 4) the first week, compared to 4.3 (± 2) for patients who failed to reach a sufficient volume (
p
= 0.4). During the interval between the first and second CT, the KGR was 2.2 (± 2), respectively (± 0.1) (
p
= 0.017).
Discussion
The increase in liver volume after PVO is largest during the first week. As KGR decreases over time, it is important to shorten the interval between PVO and the first volume evaluation; this may aid in decision-making and reduce unnecessary waiting time.
The particle density (ρs) is a fundamental physical property needed for calculating the soil porosity and phase distributions. While ρs is often estimated using soil organic matter (SOM) content and ...particle size distribution, the specific densities of each soil component remain unclear in a subarctic agricultural setting. This study aimed to evaluate the ρs of soils from Southwest Greenland using a three‐compartment model (3CM) based on the mixing ratio of SOM derived from loss‐on‐ignition, mineral particles <20 μm (FC), and mineral particles ≥20 μm (CC). We further evaluated the accuracy of the 3CM against pedotransfer functions (PTFs) and visible near‐infrared (vis–NIR) spectroscopic models. A total of 324 soil samples from 16 Greenlandic agricultural fields were investigated, covering a wide range in SOM content (0.021–0.602 kg kg–1) and clay content (0.020–0.185 kg kg–1). Despite their high SOM content, the Greenlandic soils exhibited relatively high ρs (1.936–3.044 Mg m–3), which together with a large SOM/organic carbon ratio of 2.16 indicated a high SOM density of 1.493 Mg m–3. The 3CM fit on all soils indicated FC and CC densities of 3.047 and 2.713 Mg m–3, respectively, while a subset of soils (n = 203) from the same geological setting resulted in FC and CC densities of 2.738 and 2.731 Mg m–3. Prediction accuracy of the 3CM (RMSE = 0.067 Mg m–3) was similar to PTFs (RMSE = 0.068–0.070 Mg m–3) and better than vis–NIR spectroscopic models (RMSE = 0.091 Mg m–3).
Core Ideas
The particle density of South Greenlandic soils ranged from 1.94 to 3.04 Mg m−3.
Soil organic matter and soil fines content (<20 μm) were primary drivers of particle density.
A three‐compartment model was developed for particle density from soil organic matter, fines content, and soils particles >20 μm.
The three‐compartment model accuracy was similar (RMSE = 0.068 Mg m−3) to pedotransfer functions and better than spectroscopic models.
Organic C content‐based compartment models require careful consideration of the soil organic matter/organic C content ratio.
The secondary ozonide (SOZ) of limonene is a potential indoor pollutant from the gas-phase limonene/ozone-reaction. A screening in the liquid phase was performed to investigate the yield and ...stability of SOZs from ten cyclic monoterpenes. They were cryo-ozonolyzed in pentane, and the reaction mixtures were analyzed with GC–MS with negative and positive chemical ionization and electron ionization. The investigated terpenes were: limonene, 4-carene, 3-carene, 2-carene, terpinolene, (+)-α-pinene, (−)-β-pinene, isolimonene, sabinene and camphene. The only identified endo-SOZs were from: limonene, 3-carene, 4-carene and possibly isolimonene. Collision induced dissociation (CID) of the quasi-molecular-ions as a proxy measure of the stability of the pristine SOZs was investigated. LimoneneSOZ and 3-careneSOZ were found to be more stable than 4-careneSOZ and isolimoneneSOZ, which corresponded well to their relative yields. 3-careneSOZ was found to be a major product from the gas-phase ozonolysis.
In recent years, psychotropic drug use among children and adolescents in Europe and USA has increased. However, the majority of psychotropic drugs are not formally approved for use in children and ...adolescents, and consequently, use is often off‐label. The objectives were to describe time trends in off‐label prescribing rates and the most commonly used types of psychotropic drugs by age and gender in Danish children and adolescents. Using the Register of Medicinal Product Statistics, we identified all prescriptions for sedatives, hypnotics and antidepressants filled for children and adolescents in 2006–2012. Information on diagnoses was obtained from the Danish National Registry of Patients and allowed classification of prescriptions as either on‐ or off‐label. We identified 186,831 prescriptions filled for 29,851 children and adolescents: 88.0% of these were classified as off‐label. During 2006–2012, off‐label rates for sedatives and hypnotics increased significantly, except for prescriptions for girls aged 15–17 years range 24.1–98.2% (girls), 31.9% to 99.0% (boys). In the same period, the number of registered melatonin prescriptions (all off‐label) increased expansively. For antidepressants, we found decreasing trends in off‐label rates over time range 94.5–65.6% (girls), 93.8–71.2% (boys). Off‐label prescribing of psychotropic drugs to Danish children and adolescents is common. Off‐label rates for sedatives and hypnotics increased in the period of 2006–2012, whereas off‐label rates for antidepressants declined. Off‐label rates might be underestimated and should be considered a conservative estimate.
Corporatism may be seen as variety of capitalism in which specific structural prerequisites such as unionization, centralization, and strong states combined with bargaining and concertation produce ...certain economic outputs. Corporatism may also be seen as a variety of democracy in which interest groups are integrated in the preparation and/or implementation of public policies. Departing in the last position, we measure the strength of Scandinavian corporatism by the involvement of interest groups in public committees, councils, and commissions. Corporatism in relation to the preparation of policy has gone down in all three Scandinavian countries whereas corporatism in implementation processes are more varied among the three countries. Le corporatisme peut être envisagé comme une forme de capitalisme, dans lequel des conditions préalables structurelles spécifiques, comme le syndicalisme, la centralisation, et des Etats forts, associés à la négociation et à la concertation produisent certains résultats économiques. Le corporatisme peut également être vu comme une forme de démocratie, dans laquelle des groupes d'intérêts forment un ensemble cohérent pour la préparation et la mise en œuvre des politiques publiques. Partant de cette dernière considération, nous mesurons la force du corporatisme existant en Scandinavie, par l'engagement des groupes d'intérêt dans les comités publiques, les conseils et les commissions. Le corporatisme dans le cadre de la préparation des politiques a été retenu dans les trois pays scandinaves, tandis que les procédés de mise du corporatisme recouvrent une variété plus étendue dans ces trois pays. Korporatismus könnte als eine Form des Kapitalismus angesehen werden, in der bestimmte strukturelle Vorraussetzungen wie gewerkschaftliche Organisierung, Zentralisierung und ein starker Staat kombiniert mit Tarifverhandlungen and Konzertierung bestimmte ökonomische Ergebnisse bewirken. Korporatismus könnte auch als eine Form von Demokratie angesehen werden, in der Interessengruppen in die Vorbereitung und/oder Implementierung von politischen Maßnahmen integriert sind. Ausgehend von der letzteren Position haben wir die Stärke des skandinavischen Korporatismus anhand der Involvierung von Interessengruppen in öffentlichen Ausschüssen, Gremien und Kommissionen bewertet. Korporatismus in Verbindung mit der Vorbereitung von Maßnahmen hat in allen drei skandinavischen Ländern abgenommen, während Korporatismus in Implementierungsprozessen in den drei Ländern vielfältiger geworden ist. El corporativismo puede verse como una variedad del capitalismo en la que determinados requisitos previos estructurales, como la unionización, la centralización y los estados fuertes combinados con la negociación y la concertación, producen determinados resultados económicos. El corporativismo también puede verse como una variedad de democracia, en la cual se integran grupos de interés para la preparación o la aplicación de políticas públicas. Partiendo de la última postura, evaluamos la fuerza del corporativismo escandinavo mediante la participación de grupos de interés en comités públicos, consejos y comisiones. El corporativismo relacionado con la preparación política ha descendido en los tres países escandinavos, mientras que el corporativismo en los procesos de aplicación presenta más variedad entre los tres.
Two patients were treated with immunotherapy for metastatic malignant melanoma (MM) despite suffering from systemic autoimmune disease, i.e., ulcerative colitis (UC) and Behcets disease (BD), ...respectively. Both patients benefitted from the treatment. The patient with UC achieved partial remission of all measurable parameters after treatment with Ipilimumab, while the patient with BD achieved a complete remission of MM after treatment with Interleukin-2 (IL-2) and Interferon-α (IFN-α). Moreover, no aggravation of symptoms related to the autoimmune diseases was seen during treatment, in contrast, clinical indications of improvement were observed. These two cases illustrate that the presence of autoimmune disease does not necessarily predict increased autoimmune toxicity in connection with immunotherapy. They also raise the question of whether autoimmune disease should continue to be an absolute exclusion criterion for treatment of MM with immunotherapy. Consequently, given the poor prognosis of refractory MM, immunotherapies need to be taken into consideration even in cases of autoimmune comorbidity due to the potential long-term benefit that these therapies offer to MM patients.
Background
Autosomal recessive polycystic kidney disease is a cystic kidney disease with early onset and clinically characterized by enlarged echogenic kidneys, hypertension, varying degrees of ...kidney dysfunction, and liver fibrosis. It is most frequently caused by sequence variants in the
PKHD1
gene, encoding fibrocystin. In more rare cases, sequence variants in
DZIP1L
are seen, encoding the basal body protein DAZ interacting protein 1-like protein (DZIP1L). So far, only four different
DZIP1L
variants have been reported.
Methods
Four children from three consanguineous families presenting with polycystic kidney disease were selected for targeted or untargeted exome sequencing.
Results
We identified two different, previously not reported homozygous
DZIP1L
sequence variants: c.193 T > C; p.(Cys65Arg), and c.216C > G; p.(Cys72Trp). Functional analyses of the c.216C > G; p.(Cys72Trp) variant indicated mislocalization of mutant DZIP1L.
Conclusions
In line with published data, our results suggest a critical role of the N-terminal domain for proper protein function. Although patients with
PKHD1
-associated autosomal recessive polycystic kidney disease often have liver abnormalities, none of the present four patients showed any clinically relevant liver involvement. Our data demonstrate the power and efficiency of next-generation sequencing-based approaches. While
DZIP1L-
related polycystic kidney disease certainly represents a rare form of the disease, our results emphasize the importance of including
DZIP1L
in multigene panels and in the data analysis of whole-exome sequencing for cystic kidney diseases.
Graphical abstract
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