Symptoms and healthcare contact preceding sudden cardiac death in persons aged 1–49 years

Sudden Cardiac Death (SCD) is a leading cause of death among persons in their youth and early middle-age. To prevent SCD it is crucial to identify persons at high-risk of SCD. Knowledge of symptoms and medical contact prior to SCD could potentially aid in the identification of high-risk persons in the general population who would benefit from further investigation. This review aims to summarize the current knowledge of symptoms and healthcare contact preceding SCD in persons aged 1–49 years, and to explore how the symptoms differ according to SCD cause and age of the deceased.There was a high frequency of both cardiac and non-specific symptoms prior to SCD. Additionally, many SCD victims contacted the healthcare system prior to death on the basis of their symptoms and only a few were diagnosed with cardiovascular disease. This information underlines that young persons reporting potential cardiac symptoms should also be thoroughly examined. Furthermore, such symptoms could be used in combination with other easily accessible information in non-invasive prediction models aiming at identifying persons at high risk of SCD that would benefit from further investigation and possibly treatment.     

Compressed sensing with signal averaging for improved sensitivity and motion artifact reduction in fluorine‐19 MRI

Fluorine‐19 (¹⁹F) MRI of injected perfluorocarbon emulsions (PFCs) allows for the non‐invasive quantification of inflammation and cell tracking, but suffers from a low signal‐to‐noise ratio and extended scan time. To address this limitation, we tested the hypotheses that a ¹⁹F MRI pulse sequence that combines a specific undersampling regime with signal averaging has both increased sensitivity and robustness against motion artifacts compared with a non‐averaged fully sampled pulse sequence, when both datasets are reconstructed with compressed sensing. As a proof of principle, numerical simulations and phantom experiments were performed on selected variable ranges to characterize the point spread function of undersampling patterns, as well as the vulnerability to noise of undersampling and reconstruction parameters with paired numbers of x signal averages and acceleration factor x (NAx ‐AFx ). The numerical simulations demonstrated that a probability density function that uses 25% of the samples to fully sample the k‐space central area allowed for an optimal balance between limited blurring and artifact incoherence. At all investigated noise levels, the Dice similarity coefficient (DSC) strongly depended on the regularization parameters and acceleration factor. In phantoms, the motion robustness of an NA8‐AF8 undersampling pattern versus NA1‐AF1 was evaluated with simulated and real motion patterns. Differences were assessed with the DSC, which was consistently higher for the NA8‐AF8 compared with the NA1‐AF1 strategy, for both simulated and real cyclic motion patterns (P < 0.001). Both strategies were validated in vivo in mice (n = 2) injected with perfluoropolyether. Here, the images displayed a sharper delineation of the liver with the NA8‐AF8 strategy than with the NA1‐AF1 strategy. In conclusion, we validated the hypotheses that in ¹⁹F MRI the combination of undersampling and averaging improves both the sensitivity and the robustness against motion artifacts.     

Atezolizumab and bevacizumab-induced encephalitis in advanced hepatocellular carcinoma: Case report and literature review

Introduction:On the basis of the results of the IMBRAVE-150 trial, the combination of atezolizumab, a programmed cell death ligand 1 (PD-L1) antibody, as well as bevacizumab, a vascular endothelial growth factor (VEGF) antibody, represents a promising novel first-line therapy in patients with advanced hepatocellular carcinoma (HCC). Despite favorable safety data, serious adverse events have been described. However, central nervous system complications such as encephalitis have rarely been reported. We present the case of a 70-year-old woman with hepatitis C virus (HCV)-related liver cirrhosis and advanced HCC who developed severe encephalitis after only one cycle of atezolizumab/bevacizumab.Patient concerns:Ten days after administration, the patient presented with confusion, somnolence, and emesis. Within a few days, the patient''s condition deteriorated, and mechanical ventilation became necessary.Diagnosis:Cerebrospinal fluid (CSF) analysis showed increased cell count and elevated protein values. Further work-up revealed no signs of an infectious, paraneoplastic, or other autoimmune cause.Intervention:Suspecting an atezolizumab/bevacizumab-related encephalitis, we initiated a high-dose steroid pulse therapy as well as repeated plasmapheresis, which resulted in clinical improvement and remission of CSF abnormalities.Outcome:Despite successful weaning and transfer to a rehabilitation ward, the patient died of progressive liver cancer 76 days after initial treatment with atezolizumab/bevacizumab, showing no response.Conclusion:This case illustrates that rapid immunosuppressive treatment with prednisolone can result in remission even of severe encephalitis. We discuss this case in the context of available literature and previously reported cases of atezolizumab-induced encephalitis in different tumor entities, highlighting the diagnostic challenges in oncologic patients treated with immune checkpoint-inhibitors.     

Real-Time Sonoelastography of Salivary Glands for Diagnosis and Functional Assessment of Primary Sjögren’s Syndrome

The purpose of this study was to investigate the value of real-time sonoelastography (RTS) of salivary glands for the diagnosis and assessment of glandular damage in primary Sjögren’s syndrome (pSS). After institutional review board approval, 45 pSS patients, 24 sicca patients and 11 healthy controls were investigated prospectively. Questionnaires were completed and Saxon and Schirmer tests and routine blood tests carried out in all patients. All patients underwent B-mode ultrasonography and RTS of parotid and submandibular glands. Abnormal findings were graded from 0 to 48 and from 0 to 16, respectively. Sialoscintigraphy was done according to a routine protocol; scoring ranged from 0 to 12. Statistical analysis comprised receiver operating characteristic curve and multivariate regression analysis. Patients with pSS had higher B-mode (median score = 25 [range: 2–44] vs. 9 [1–20], p < 0.001) and RTS (6.5 [2–13] versus 4 [1–9], p < 0.001) scores than controls with sicca syndrome, yielding areas under the curve of 0.83 and 0.85 (p < 0.05 each), respectively for pSS diagnosis. In cases with an inconclusive B-mode ultrasonography result, RTS (cutoff score: ≥6) led to a sensitive (66.7%) and specific (85.7%) classification of patients and sicca controls. In multivariate regression analysis, RTS (regression coefficient = –0.48, p = 0.005), but not B-mode ultrasonography, reflected impaired salivary gland function according to the Saxon test, whereas none of the subjective measures of dryness or discomfort were related to ultrasonography results. B-mode and RTS results were both associated with sialoscintigraphy scores (regression coefficient = 0.66, p < 0.001, and regression coefficient = 0.55, p = 0.001, respectively). Reproducibility of B-mode ultrasonography and RTS was good, with intra-class correlation coefficients of 0.93 (95% confidence interval: 0.57–0.98) and 0.93 (95% confidence interval: 0.79–0.98), respectively. In summary, RTS might be a useful adjunct to B-mode ultrasonography for diagnosis and assessment of salivary gland impairment in primary Sjögren’s syndrome.     

Concomitant MDS with isolated 5q deletion and MGUS: case report and review of molecular aspects

Patients with monoclonal gammopathy of undetermined significance (MGUS) have a higher risk for the development of concomitant primary cancers such as multiple myeloma (MM) and myelodysplastic syndrome (MDS). We report the case of patient initially suffering from MGUS of the IgG lambda subtype for more than 10 yr, which evolved to MM and MDS with deletion (5q) with severe pancytopenia. Due to pancytopenia, he received dose‐reduced treatment with lenalidomide and dexamethasone. He achieved an ongoing transfusion independency after about 1 month of treatment. Bone marrow taken 14 months after start of treatment showed a complete cytogenetic response of the del(5q) clone and a plasma cell infiltration below 5%. In contrast to the development of MM in MGUS patients, the subsequent occurrence of MDS after diagnosis of MGUS is infrequent. Moreover, the biological association of MDS with MGUS is not sufficiently understood, but the non‐treatment‐related occurrence supports the pathogenetic role of pre‐existing alterations of stem cells. Here, we summarize data on concomitant MDS and MGUS/MM with particular emphasis on molecular aspects.     

Diagnosis and Treatment of Allergic Angiitis (Anaphylactoid Purpura)

Diagnosis of allergic angiitis depends on clinical recognition of the entity and histopathologic examination of the skin. The cause is rarely identified.

Acute forms of allergic angiitis require vigorous treatment, including rest in bed; transfusions; removal of all questionable chemicals or drugs from the patient's environment and elimination of foods known to produce or aggravate the condition; antihistamine therapy, and usually steroid therapy.

In its chronic form, the disease may be managed conservatively.     

Detection of acute pulmonary embolism: feasibility of diagnostic accuracy of MRI using a stepwise protocol

Multidetector computed tomography (MDCT) has become the imaging method of choice in patients suspected of having pulmonary embolism (PE) but has the inherent limitation of radiation dose and the side effects of contrast agents. The purpose of the study was to assess the feasibility of a stepwise MRI protocol in the clinical setting of acute PE. The stepwise approach should make it possible to diagnose acute PE in critically ill patients using fast MR sequences and included the option to add comprehensive sequences when patients were stable. Forty-five patients with acute PE (26 men, 19 women; 41?±?16 years) were included in this prospective study. The diagnosis was initially confirmed by MDCT as gold standard. MRI at 1.5 T was subsequently performed without any delay in medical treatment. The MRI protocol proceeded stepwise from robust to detailed imaging techniques (i.e., from TrueFISP and single shot HASTE sequence to MR perfusion and 3D-MR angiography) if the patient was able to tolerate additional imaging time. Diagnostic accuracy was evaluated on the central (lobar) and peripheral (segmental) levels. The complete MR protocol was applied in 40 of the 45 patients (88 %). In the remaining five patients with severe dyspnea the diagnosis of acute PE was established by using fast TrueFISP sequences that were insensitive to respiratory movement. All five patients suffered from a major central PE. Highest sensitivity was achieved by MR perfusion (lobar, 98 %; segmental, 95 %). Real-time TrueFISP and MR angiography showed the highest specificity (lobar, 90–100 %; segmental, 95–97 %). The combination of all MR sequences matched closely the results of MDCT (lobar: sensitivity 98 %, specificity 100 %; segmental: sensitivity 95 %, specificity 97 %). MRI using a stepwise protocol is a promising approach for diagnosing acute PE. The protocol can be tailored for dyspneic patients with central PE using real-time MRI sequences. The diagnostic accuracy for peripheral PE can be improved by using combined MR techniques, achieving comparable results to MDCT.