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Syndrom týraneho a zanedbavaneho ditěte (child abuse and neglect) je velmi zavažný celospolecenský problem, který velkou měrou nepřiznivě ovlivňuje zdravi a dlouhodobý vývoj jedince. Mezi fyzicke formy syndromu týraneho a zanedbavaneho ditěte s nejvyssi mortalitou patři takzvaný syndrom třeseneho ditěte (shaken baby syndrom, SBS). Jedna se o urazove poraněni hlavy maleho ditěte v důsledku nasilneho třeseni, tupeho uderu nebo kombinace těchto mechanismů. Nespecificke přiznaky v kombinaci s casto neuplnou ci nejasnou anamnezou a spolecenska kontroverze jsou přicinou nedostatecne evidence připadů. Udavana incidence je proto velmi pravděpodobně podhodnocena a můžeme mluvit o pouhem vrcholu pomyslneho ledovce vsech připadů syndromu třeseneho ditěte. V nasledujicim sděleni prezentujeme připad 4. měsicniho kojence, u ktereho se pomoci kombinace různých vysetřovacich metod podařilo odhalit řadu poraněni typických pro SBS. V diskuzi autoři podrobně rozebiraji diferencialni diagnostiku a diagnostický postup při podezřeni na SBS.

This work presents a case series of four children diagnosed with severe cerebrovascular disease in association with recent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, yet no patient from the group met typical diagnostic criteria for multisystem inflammatory syndrome in children. Our aim was to highlight the possible vascular involvement and coagulopathies associated with SARS-CoV-2 infection in the pediatric population. Further data are needed to better understand the pathophysiological basis of this condition in children and to ensure its optimal management.

Background Various different injection parameters influence enhancement of the coronary arteries. There is no consensus in the literature regarding the optimal contrast media (CM) injection protocol. The aim of this study is to provide an update on the effect of different CM injection parameters on the coronary attenuation in coronary computed tomographic angiography (CCTA). Method Studies published between January 2001 and May 2014 identified by Pubmed, Embase and MEDLINE were evaluated. Using predefined inclusion criteria and a data extraction form, the content of each eligible study was assessed. Initially, 2551 potential studies were identified. After applying our criteria, 36 studies were found to be eligible. Studies were systematically assessed for quality based on the validated Quality Assessment of Diagnostic Accuracy Studies (QUADAS)-II checklist. Results Extracted data proved to be heterogeneous and often incomplete. The injection protocol and outcome of the included publications were very diverse and results are difficult to compare. Based on the extracted data, it remains unclear which of the injection parameters is the most important determinant for adequate attenuation. It is likely that one parameter which combines multiple parameters (e. g. IDR) will be the most suitable determinant of coronary attenuation in CCTA protocols. Conclusion Research should be directed towards determining the influence of different injection parameters and defining individualized optimal IDRs tailored to patient-related factors (ideally in large randomized trials). Key points Citation Format

OBJECTIVE. The purpose of this study was to systematically investigate radiation dose reduction using automated tube voltage selection during CT angiography (CTA) and to evaluate the impact of contrast medium (CM) injection protocols on dose reduction. MATERIALS AND METHODS. A circulation phantom containing the thoracic and abdominal vasculature was used. Four different concentrations of CM (iopromide 300 and 370 mg I/mL and iomeprol 350 and 400 mg I/mL) were administered while maintaining an identical iodine delivery rate (1.8 g I/s) and total iodine load (20.0 g). Three different scanning protocols for CTA of the thoracoabdominal aorta were used: protocol A, no dose modulation; protocol B, automated tube current modulation (CARE Dose4D); and protocol C, automated tube voltage selection (CARE kV). The dose-length product was recorded to calculate the effective dose. Attenuation values (in Hounsfield units), image noise levels, and signal-to-noise ratios (SNRs) in six predefined intravascular sites (three thoracic and three abdominal) were measured by two readers. All values were analyzed using the Kruskal-Wallis test and two-way ANOVA. RESULTS. There was a significant reduction in the effective dose (in millisieverts) for protocols B (mean +/- SD, 2.03 +/- 0.1 mSv) and C (1.00 +/- 0.0 mSv) compared with protocol A (4.34 +/- 0.0 mSv). The dose was reduced by 53% for protocol B and by 77% for protocol C. No significant differences were found in the effective dose among the different CM injection protocols within the scanning protocols; all p values were > 0.05. The attenuation values and SNRs were comparable among all the different CM injection protocols; all p values were > 0.05. CONCLUSION. A large radiation dose reduction (77%) can be achieved using automated tube voltage selection independent of the CM injection protocol.

Purpose Iodinated contrast media (CM) in computed tomographic angiography is characterized by its concentration and, consecutively, by its viscosity. Viscosity itself is directly influenced by temperature, which will furthermore affect injection pressure. Therefore, the purposes of this study were to systematically evaluate the viscosity of different CM at different temperatures and to assess their impact on injection pressure in a circulation phantom. Materials and Methods Initially, viscosity of different contrast media concentrations (240, 300, 370, and 400 mgI/mL) was measured at different temperatures (20 degrees C-40 degrees C) with a commercially available viscosimeter. In the next step, a circulation phantom with physical conditions was used. Contrast media were prepared at different temperatures (20 degrees C, 30 degrees C, 37 degrees C) and injected through a standard 18-gauge needle. All other relevant parameters were kept constant (iodine delivery rate, 1.9 g I/s; total amount of iodine, 15 g I). Peak flow rate (in milliliter per second) and injection pressure (psi) were monitored. Differences in significance were tested using the Kruskal-Wallis test (Statistical Package for the Social Sciences). Results Viscosities for iodinated CM of 240, 300, 370, and 400 mg I/mL at 20 degrees C were 5.1, 9.1, 21.2, and 28.8 mPa.s, respectively, whereas, at 40 degrees C, these were substantially lower (2.8, 4.4, 8.7, and 11.2 mPa.s). In the circulation phantom, mean (SD) peak pressures for CM of 240 mg I/mL at 20 degrees C, 30 degrees C, and 37 degrees C were 107 (1.5), 95 (0.6), and 92 (2.1) psi; for CM of 300 mg I/mL, 119 (1.5), 104 (0.6), and 100 (3.6) psi; for CM of 370 mg I/mL, 150 (0.6), 133 (4.4), and 120 (3.5) psi; and for CM of 400 mg I/mL, 169 (1.0), 140 (2.1), and 135 (2.9) psi, respectively, with all P values less than 0.05. Conclusions Low concentration, low viscosity, and high temperatures of CM are beneficial in terms of injection pressure. This should also be considered for individually tailored contrast protocols in daily routine scanning.

Background Infantile myofibromatosis belongs to a family of soft tissue tumors. The majority of these tumors have benign behavior but resistant and malignant courses are known, namely in tumors with visceral involvement. The standard of care is surgical resection. Observations suggest that low dose chemotherapy is beneficial. The treatment of resistant or relapsed patients with multifocal disease remains challenging. Patients that harbor an actionable mutation in the kinase domain are potential subjects for targeted tyrosine kinase inhibitor therapy. Case presentation An infant boy with inborn generalized infantile myofibromatosis that included bone, intracranial, soft tissue and visceral involvement was treated according to recent recommendations with low dose chemotherapy. The presence of a partial but temporary response led to a second line of treatment with six cycles of chemotherapy, which achieved a partial response again but was followed by severe toxicity. The generalized progression of the disease was observed later. Genetic analyses were performed and revealed a PDGFRB gene c.1681C>A missense heterozygous germline mutation, high PDGFRβ phosphokinase activity within the tumor and the heterozygous germline Slavic Nijmegen breakage syndrome 657del5 mutation in the NBN gene. Targeted treatment with sunitinib, the PDGFRβ inhibitor, plus low dose vinblastine led to an unexpected and durable response without toxicities or limitations to daily life activities. The presence of the Slavic NBN gene mutation limited standard chemotherapy dosing due to severe toxicities. Sister of the patient suffred from skull base tumor with same genotype and histology. The same targeted therapy led to similar quick and durable response. Conclusion Progressive and resistant incurable infantile myofibromatosis can be successfully treated with the new approach described herein. Detailed insights into the biology of the patient’s tumor and genome are necessary to understand the mechanisms of activity of less toxic and effective drugs except for up to date population-based chemotherapy regimens. Electronic supplementary material The online version of this article (doi:10.1186/s12885-017-3115-x) contains supplementary material, which is available to authorized users.