Urological anomalies in anorectal malformations in the Netherlands: Effects of screening all patients on long-term outcome

Introduction: Urological anomalies are frequently seen in patients with anorectal malformations (ARM) and can result in upper urinary tract deterioration. Whether the current method of screening is valid, adequate and needed for all patients is not clear. We, therefore, evaluated the urological screening methods in our ARM patients for changes in urological treatment, outcome and follow-up. Methods: The medical records of 331 children born with an ARM in the period 1983-2003 were retrospectively studied. Documentation of diagnosis, screening method, urological anomalies, treatment, complications, follow-up and outcome were measured. Results: The overall incidence of urological anomalies was 52%. The incidence of urological anomalies and urological follow-up time decreased with diminishing complexity of the ARM. Hydronephrosis, vesico-urethral reflux, lower urinary tract dysfunction and urinary incontinence were encountered most. Treatment invasiveness increased with the increase of complexity of an ARM. Lower urinary tract dysfunction needing urological care occurred in 43% in combination with lumbosacral or spinal cord anomalies and in 8% with no abnormalities in the lumbosacral-/spinal region. Conclusions: Urological anomalies in patients with complex ARM are more severe than in patients with less complex ARM. Ultrasonography of the urinary tract should be performed in all patients. Voiding cysto-urethrography can be reserved for patients with dilated upper urinary tracts, urinary tract infections or lumbosacral and spinal abnormalities. All patients with complex ARM need urodynamic investigations. When using these indications, the screening for urological anomalies in ARM patients can be optimized with long-term follow-up in selected patients

Получение полуизолирующего кремния для высоковольтных приборов

Исследовано влияние условий осаждения на структуру и электрофизические свойства пленок поликристаллического кремния, легированного кислородом. Результаты использованы при изготовлении изделий силовой электроники

Vascular white matter lesions negatively correlate with brain metastases in malignant melanoma - results from a retrospective comparative analysis

Brain metastasis (BM) is a major complication of different cancers. There is increasing evidence for influence of vascular factors on BM in patients with non-small cell lung cancer (NSCLC). It is not known if the same is true for other tumors that might rely on different forms of vasculogenesis. The objective of this retrospective study was to evaluate a possible negative association of vascular white matter lesions and vascular risk factors (vasRF) with brain metastases in patients with melanoma

Determination of food synthetic dyes in acid-dairy products by the method of solid-phase spectrophotometry

Solid-phase extraction was studied for food synthetic dyes Tartrazine E102, Yellow "Sunset" E110, Ponso 4R E124 and the Patented Blue E131 from yoghurts and biokefirs in a transparent polymethylmethacrylate matrix as a solid extractant. The effectiveness of the proposed approach is shown to identify and determine the content of 2 food-grade synthetic dyes by visual and spectrophotometric methods. The optimal conditions for the analysis correspond to pH <3, the duration of dye extraction into the polymer matrix is 20 min, the range of detected concentrations is 0.2-40.0 mg / kg with a detection limit of 0.05 mg/kg, and an excess of sweeteners and preservatives does not significantly affect the results of the analysis

Outcome after modern proton beam therapy in childhood craniopharyngioma: results of the prospective registry study KiProReg

Background Craniopharyngiomas (CPs) are rare tumours of the sellar region often leading to significant comorbidities due to their close proximity to critical structures. Aim of this study was to analyse survival outcome and late toxicities after surgery and proton beam therapy (PBT) in childhood CPs. Patients and methods Within the prospective XXXX study (XXXX), data of 74 childhood patients with CP, receiving PBT between 08/2013-06/2022 were eligible. Late toxicities were analysed according to the grading system of CTCAE 4.0. Results : Median follow-up (FU) since first diagnosis was 4.3 years (range, 0.8-14.7). 75.7% of patients received PBT at time of disease progression or recurrence, whereas 24.3% as part of their primary therapy (definitive or adjuvant). Predominantly (85.1%), pencil beam scanning technique was used. Median total dose and initial tumour volume were 5400 cGyRBE (relative biological effectiveness) and 17.64 cm³ (range, 3.07-300.59), respectively. The estimated (±SE) 3-year overall survival, progression-free and cystic failure-free survival rate after PBT were 98.2% (±1.7), 94.7% (±3.0), and 76.8% (±5.4), respectively. All local failures (n=3) were in-field relapses necessitating intervention and occurred exclusively in patients receiving PBT at progression or recurrence. Early cystic enlargements after PBT were typically asymptomatic and self-limiting. Fatigue, headaches, vision disorders, obesity and endocrinopathies were the predominant late toxicities. No high grade (≥3) new-onset visual impairment or cognitive deterioration occurred compared to baseline. The presence of cognitive impairments at the end of FU correlated with size of the planning target volume (p=0.034), Dmean dose to the temporal lobes (p=0.032,p=0.045) and the number of surgical interventions prior to PBT (p=0.029). Conclusions : Our findings demonstrate favourable local control rates using modern PBT with acceptable late toxicities. Cyst growth within 12 month after radiotherapy is typically not associated with tumour progression. Longer FU has to be awaited to confirm results

The extreme HBL behaviour of Markarian 501 during 2012

A multiwavelength campaign was organized to take place between March and July of 2012. Excellent temporal coverage was obtained with more than 25 instruments, including the MAGIC, FACT and VERITAS Cherenkov telescopes, the instruments on board the Swift and Fermi spacecraft, and the telescopes operated by the GASP-WEBT collaboration. Mrk 501 showed a very high energy (VHE) gamma-ray flux above 0.2 TeV of $\sim$0.5 times the Crab Nebula flux (CU) for most of the campaign. The highest activity occurred on 2012 June 9, when the VHE flux was $\sim$3 CU, and the peak of the high-energy spectral component was found to be at $\sim$2 TeV. This study reports very hard X-ray spectra, and the hardest VHE spectra measured to date for Mrk 501. The fractional variability was found to increase with energy, with the highest variability occurring at VHE, and a significant correlation between the X-ray and VHE bands. The unprecedentedly hard X-ray and VHE spectra measured imply that their low- and high-energy components peaked above 5 keV and 0.5 TeV, respectively, during a large fraction of the observing campaign, and hence that Mrk 501 behaved like an extreme high-frequency- peaked blazar (EHBL) throughout the 2012 observing season. This suggests that being an EHBL may not be a permanent characteristic of a blazar, but rather a state which may change over time. The one-zone synchrotron self-Compton (SSC) scenario can successfully describe the segments of the SED where most energy is emitted, with a significant correlation between the electron energy density and the VHE gamma-ray activity, suggesting that most of the variability may be explained by the injection of high-energy electrons. The one-zone SSC scenario used reproduces the behaviour seen between the measured X-ray and VHE gamma-ray fluxes, and predicts that the correlation becomes stronger with increasing energy of the X-rays

Paediatric radiation therapy across Europe: A European questionnaire survey supported by the SIOPe, ESTRO, PROS and several national paediatric hematology-oncology societies (NAPHOS)

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c-MYC expression sensitizes medulloblastoma cells to radio- and chemotherapy and has no impact on response in medulloblastoma patients

BACKGROUND: To study whether and how c-MYC expression determines response to radio- and chemotherapy in childhood medulloblastoma (MB). METHODS: We used DAOY and UW228 human MB cells engineered to stably express different levels of c-MYC, and tested whether c-MYC expression has an effect on radio- and chemosensitivity using the colorimetric 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium inner salt (MTS) assay, clonogenic survival, apoptosis assays, cell cycle analysis, and western blot assessment. In an effort to validate our results, we analyzed c-MYC mRNA expression in formalin-fixed paraffin-embedded tumor samples from well-documented patients with postoperative residual tumor and compared c-MYC mRNA expression with response to radio- and chemotherapy as examined by neuroradiological imaging. RESULTS: In DAOY - and to a lesser extent in UW228 - cells expressing high levels of c-MYC, the cytotoxicity of cisplatin, and etoposide was significantly higher when compared with DAOY/UW228 cells expressing low levels of c-MYC. Irradiation- and chemotherapy-induced apoptotic cell death was enhanced in DAOY cells expressing high levels of c-MYC. The response of 62 of 66 residual tumors was evaluable and response to postoperative radio- (14 responders (CR, PR) vs. 5 non-responders (SD, PD)) or chemotherapy (23 CR/PR vs. 20 SD/PD) was assessed. c-MYC mRNA expression was similar in primary MB samples of responders and non-responders (Mann-Whitney U test, p = 0.50, ratio 0.49, 95% CI 0.008-30.0 and p = 0.67, ratio 1.8, 95% CI 0.14-23.5, respectively). CONCLUSIONS: c-MYC sensitizes MB cells to some anti-cancer treatments in vitro. As we failed to show evidence for such an effect on postoperative residual tumors when analyzed by imaging, additional investigations in xenografts and larger MB cohorts may help to define the exact function of c-MYC in modulating response to treatment

Clinical and molecular characterization of isolated M1 disease in pediatric medulloblastoma: experience from the German HIT-MED studies

PURPOSE: To evaluate the clinical impact of isolated spread of medulloblastoma cells into cerebrospinal fluid without additional macroscopic metastases (M1-only). METHODS: The HIT-MED database was searched for pediatric patients with M1-only medulloblastoma diagnosed from 2000 to 2019. Corresponding clinical and molecular data was evaluated. Treatment was stratified by age and changed over time for older patients. RESULTS: 70 patients with centrally reviewed M1-only disease were identified. Clinical data was available for all and molecular data for 45/70 cases. 91% were non-WNT/non-SHH medulloblastoma (Grp3/4). 5-year PFS for 52 patients ≥ 4 years was 59.4 (± 7.1) %, receiving either upfront craniospinal irradiation (CSI) or SKK-sandwich chemotherapy (CT). Outcomes did not differ between these strategies (5-year PFS: CSI 61.7 ± 9.9%, SKK-CT 56.7 ± 6.1%). For patients < 4 years (n = 18), 5-year PFS was 50.0 (± 13.2) %. M1-persistence occurred exclusively using postoperative CT and was a strong negative predictive factor (p(PFS/OS) < 0.01). Patients with additional clinical or molecular high-risk (HR) characteristics had worse outcomes (5-year PFS 42.7 ± 10.6% vs. 64.0 ± 7.0%, p = 0.03). In n = 22 patients ≥ 4 years with full molecular information and without additional HR characteristics, risk classification by molecular subtyping had an effect on 5-year PFS (HR 16.7 ± 15.2%, SR 77.8 ± 13.9%; p = 0.01). CONCLUSIONS: Our results confirm that M1-only is a high-risk condition, and further underline the importance of CSF staging. Specific risk stratification of affected patients needs attention in future discussions for trials and treatment recommendations. Future patients without contraindications may benefit from upfront CSI by sparing risks related to higher cumulative CT applied in sandwich regimen. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s11060-021-03913-5