Case report: VEXAS as an example of autoinflammatory syndrome in pulmonology clinical practice.

Lung involvement is not widely recognized as a complication of auto-inflammatory diseases. We present a broad approach to diagnose a severe form of autoinflammatory syndrome in an adult male patient. A 63-year-old Caucasian male presented with recurrent episodes of high fever, interstitial lung infiltration, and pleural effusion. Laboratory tests performed during the flares revealed lymphopenia and increased levels of C-reactive protein and ferritin. Broad diagnostic research on infections, connective tissue diseases, and malignancies yielded negative results. The patient's symptoms promptly resolved upon the administration of glucocorticoids; however, they reappeared when the prednisone dose was reduced. All attempts to administer immunomodulatory and immunosuppressive medications were ineffective. During follow-up, autoinflammatory syndrome was suspected; however, no pathological variants of monogenic autoinflammatory diseases were identified by genome-exome sequencing. The patient did not respond to interleukin 1 blockade with anakinra. He died due to multi-organ failure, and his condition remained unresolved until the first reported description of vacuole, E1 enzyme, X-linked, autoinflammatory, and somatic syndrome (VEXAS). We describe the diagnostic traps and reasoning process involved in establishing that the patient's symptoms were autoinflammatory in nature based on clinical symptoms, in addition to the proof of concept gained from genetic reevaluation and identification of pathogenic variants in the UBA1 gene. The aim of this review is to increase the awareness of VEXAS among pulmonologists. Genetic screening for UBA1 should be considered in patients with recurrent pneumonitis of unknown origin with elevated inflammatory markers and signs of cytopenia, especially if they require chronic steroids to control the disease. Respiratory manifestations are part of VEXAS; these may be dominant in the course of the disease and severe at presentation.

Utility of non-contrast Dual Energy Computed Tomography in diagnosis of differentiated thyroid cancer - two case study.

BACKGROUND: Dual Energy Computed Tomography (DECT) is a technology that allows for viewing computed tomography spectral images. This method, due to ability of presenting specific elements and substances (like water, calcium and iodine), can be used to locate selected type of tissues. Thyroid tissue due to being rich in endogenous iodine, can be located even without administration of contrast agent. CASE PRESENTATION: In presented cases authors used a feature of accumulating endogenous iodine in thyroid derivative tissue for diagnosis of differentiated thyroid cancer metastases. In Patient One DECT was a decisive parameter qualifying for the surgery. Due to use of DECT in Patient Two it was possible to directly localize thyroid cancer metastases, which was unfeasible using standard techniques (scintigraphy and [18 F]FDG PET/CT). It helped to perform targeted biopsy and confirm diagnosis of thyroid cancer metastases, allowing to introduce treatment with sorafenibe. CONCLUSION: DECT confirmed its utility in locating thyroid tissues, including differentiated thyroid cancer (DTC) metastases. The method could be used in the future, especially in borderline or ambiguous cases with no localization of DTC in ultrasonography, RAI scintigraphy, or [18 F]FDG PET/CT, and among patients having contraindications for contrast-CT.

Ultrasound Doppler Flow Parameters Are Independently Associated with Renal Cortex Contrast-Enhanced Multidetector Computed Tomography Perfusion and Kidney Function.

BACKGROUND: The assessment of kidney perfusion has an emerging significance in many diagnostic applications. However, whether and which of the ultrasound Doppler parameters better express renal cortical perfusion (RCP) was not shown. The study aimed to prove the usefulness of Doppler ultrasound parameters in the assessment of RCP regarding low-dose contrast-enhanced multidetector computer tomography (CE-MDCT) blood flow. METHODS: Thirty non-stenotic kidneys in twenty-five hypertensive patients (age 58.9 ± 19.0) with mild-to-severe renal dysfunction were included in the study. Resistive index (RI) and end-diastolic velocity (EDV) in segmental arteries, color Doppler dynamic RCP intensity (dRCP), RI (dRI), pulsatility index (dPI), and CE-MDCT blood flow (CBF) in the renal cortex were estimated. RESULTS: CBF correlated significantly with age, estimated glomerular filtration rate (eGFR), RI, EDV, dRI, dPI, and dRCP. In separate multivariable backward regression analyses, RI (R2 = 0.290, p = 0.003) and dRCP (R2 = 0.320, p = 0.001) were independently associated with CBF. However, in the common ultrasound model, only dRCP was independently related to CBF (R2 = 0.317, p = 0.001). Only CBF and EDV were independently associated with eGFR (R2 = 0.510, p < 0.001). CONCLUSIONS: Renal cortical perfusion intensity is the best ultrasound marker expressing renal cortical perfusion. In patients with hypertension and kidney dysfunction, renal resistive index and end-diastolic velocity express renal cortical perfusion and kidney function, respectively.

Dual-energy computed tomography as a lower radiation dose alternative to perfusion computed tomography in tumor viability assessment.

To present the utility of dual-energy computed tomography (DECT) in the assessment of angiogenesis of focal lesions as an example of a solitary pulmonary nodule (SPN). This prospective study comprised 28 patients with SPN who underwent DECT and perfusion computed tomography (CTP), according to a proprietary protocol. Two radiologists independently analyzed four perfusion parameters, namely blood flow (BF), blood volume (BV), the time to maximum of the tissue residue function (Tmax), permeability surface area product (PS) from CTP, in addition to the iodine concentration (IC) and normalized iodine concentration (NIC) of the SPN from DECT. We used the Pearson R correlation and interclass correlation coefficients (ICCs). Statistical significance was assumed at p < 0.05. The mean tumor size was 23.5 ± 6.5 mm. We observed good correlations between IC and BF (r = 0.78, p < 0.000) and NIC and BF (r = 0.71, p < 0.000) as well as between IC and BV (r = 0.73, p < 0.000) and NIC and BV (r = 0.73, p < 0.000) and poor correlation between IC and PS (r = 0.38, p = 0.044).There was no correlation between NIC and PS (r = 0.35, p = 0.064), IC content and Tmax (r = - 0.28, p = 0.147) and NIC and Tmax (r = - 0.21, p = 0.266). Inter-reader agreement on quantitative parameters at CTP (ICCPS = 0.97, ICCTmax = 0.96, ICCBV = 0.98, and ICCBF = 0.99) and DECT (ICCIC = 0.98) were excellent. The radiation dose was significantly lower in DECT than that in CTP (4.84 mSv vs. 9.07 mSv, respectively). DECT is useful for the functional assessment of oncological lesions with less exposure to radiation compared to perfusion computed tomography.

Vascular and Vasogenic Manifestations of Systemic ANCA-Associated Vasculitis with Renal Involvement in Non-Contrast Brain MRI in Patients with Acute Disease Onset.

Background. Data concerning central nervous system (CNS) alterations in ANCA-associated vasculitis with renal involvement (AAVR) are sparse. The study aimed to assess vascular and vasogenic brain alterations in patients with acute onset of AAVR and the applicability of non-contrast magnetic resonance imaging (MRI) techniques in this diagnosis. Methods. Thirty-eight patients with acute onset of AAVR were included in the study. BVAS/WG, c-ANCA, p-ANCA, renal function and perfusion, neurological assessment, and brain MRI were performed. Results. Cerebral vascular alternating narrowing and dilatation (VAND) was detected in 42.1% of patients, and the black-blood was significantly more diagnostic than the TOF technique (p < 0.001). VAND occurrence was independently associated with the concentration of p-ANCA. The vasogenic white matter lesions (VWML) were found in 94.4% of patients, and in their detection, SWAN was significantly better than the FLAIR technique (p = 0.002). The number of VWML correlated with age and cranial nerve damage. Hemosiderin deposits were found in 21.6% of patients and were associated with a gait impairment and paresthesia. Conclusions. Vascular and vasogenic alterations in the CNS are frequent in patients with acute onset of systemic ANCA-associated vasculitis with renal involvement. Non-contrast MRI is useful in the diagnosis of brain vasculitis.

Spheno-Orbital Meningioma and Vision Impairment-Case Report and Review of the Literature.

(1) Background: Spheno-orbital meningioma (SOM) is a very rare subtype of meningioma which arises from the sphenoid ridge with an orbital extension. It exhibits intraosseous tumor growth with hyperostosis and a widespread soft-tissue growth at the dura. The intra-orbital invasion results in painless proptosis and slowly progressing visual impairment. (2) Methods: We present a case of a 46-year-old woman with SOM and compressive optic nerve neuropathy related to it. Her corrected distance visual acuity (CDVA) was decreased to 20/100, she had extensive visual field (VF) scotoma, dyschromatopsia, impaired pattern-reversal visual-evoked potential (PVEP), and decreased thicknesses of the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC), measured with the swept-source optical coherence tomography (SS-OCT), and a pale optic nerve disc in her left eye. Brain CT and MRI showed a lesion at the base of the anterior cranial fossa, involving the sphenoid wing and orbit. Pterional craniotomy and a partial removal of the tumor at the base of the skull and in the left orbit with the resection of the lesional dura mater and bony defect reconstruction were performed. (3) Results: The histological examination revealed meningothelial meningioma (WHO G1). Decreased CDVA and VF defects completely recovered, and the color vision score and PVEP improved following the surgery, but RNFL and GCC remained impaired. No tumor recurrence was observed at a follow-up of 78 months. (4) Conclusions: Optic nerve dysfunction has the capacity to improve once the compression has been relieved despite the presence of the structural features of optic nerve atrophy.

Colour Doppler imaging of retrobulbar circulation in different severity of glaucoma optic neuropathy.

AIMS: To compare retrobulbar hemodynamic measured by colour Doppler imaging (CDI) in patients with a different severity of glaucoma and to correlate variables CDI with the structural parameters of the retina and optic disc. MATERIAL AND METHODS: Of 89 eyes studied, 31 had preperimetric, 29 early, 12 moderate and 17 advanced glaucoma. Peak systolic velocity (PSV), end-diastolic velocity (EDV), resistance index (RI) in ophthalmic artery (OA), central retinal artery (CRA) and short posterior ciliary arteries (SPCAs) were evaluated by CDI and compared between study groups. Retinal nerve fibre layer thickness (RNFL) and optic disc parameters measured with optical coherent tomography (OCT) were correlated with the Doppler variables. RESULTS: In eyes with advanced, moderate and early glaucoma EDV SPCAs were significantly lower as well as RI SPCAs were significantly higher compared to the eyes with preperimetric glaucoma. In eyes with advanced and moderate glaucoma RI CRA (0.74 and 0.71) were significantly higher than in eyes with preperimetric glaucoma (0.68) (p=0.014 and 0.026). In eyes with advanced glaucoma PSV OA and PSV CRA were significantly lower than in eyes with preperimetric glaucoma (32.5 vs. 40.7 cm/s p=0.034 and 8.25 vs. 9.7 cm/s p=0.022, respectively). Positive, variable correlations were found between RNFL and EDV of all measured arteries (0.21

A New Outlook on the Ability to Accumulate an Iodine Contrast Agent in Solid Lung Tumors Based on Virtual Monochromatic Images in Dual Energy Computed Tomography (DECT): Analysis in Two Phases of Contrast Enhancement.

For some time, dual energy computed tomography (DECT) has been an established method used in a vast array of clinical applications, including lung nodule assessment. The aim of this study was to analyze (using monochromatic DECT images) how the X-ray absorption of solitary pulmonary nodules (SPNs) depends on the iodine contrast agent and when X-ray absorption is no longer dependent on the accumulated contrast agent. Sixty-six patients with diagnosed solid lung tumors underwent DECT scans in the late arterial phase (AP) and venous phase (VP) between January 2017 and June 2018. Statistically significant correlations (p ≤ 0.001) of the iodine contrast concentration were found in the energy range of 40-90 keV in the AP phase and in the range of 40-80 keV in the VP phase. The strongest correlation was found between the concentrations of the contrast agent and the scanning energy of 40 keV. At the higher scanning energy, no significant correlations were found. We concluded that it is most useful to evaluate lung lesions in DECT virtual monochromatic images (VMIs) in the energy range of 40-80 keV. We recommend assessing SPNs in only one phase of contrast enhancement to reduce the absorbed radiation dose.

Assessment of Solitary Pulmonary Nodules Based on Virtual Monochrome Images and Iodine-Dependent Images Using a Single-Source Dual-Energy CT with Fast kVp Switching.

With lung cancer being the most common malignancy diagnosed worldwide, lung nodule assessment has proved to be one of big challenges of modern medicine. The aim of this study was to examine the usefulness of Dual Energy Computed Tomography (DECT) in solitary pulmonary nodule (SPN) assessment. Between January 2017 and June 2018; 65 patients (42 males and 23 females) underwent DECT scans in the late arterial phase (AP) and venous phase (VP). We concluded that imaging at an energy level of 65 keV was the most accurate in detecting malignancy in solitary pulmonary nodules (SPNs) measuring ≤30 mm in diameter on virtual monochromatic maps. Both virtual monochromatic images and iodine concentration maps prove to be highly useful in differentiating benign and malignant pulmonary nodules. As for iodine concentration maps, the analysis of venous phase images resulted in the highest clinical usefulness. To summarize, DECT may be a useful tool in the differentiation of benign and malignant SPNs. A single-phase DECT examination with scans acquired 90 s after contrast media injection is recommended.

The role of modern diagnostic imaging in diagnosing and differentiating kidney diseases in children.

Urinary tract diseases are in the group of the most commonly diagnosed medical conditions in pediatric patients. Many diseases with different etiologies are accompanied by pain, fever, hematuria, or urinary tract dysfunction. Those most common ones in children are urinary tract infections and congenital malformation. They can also represent tumors or changes caused by systemic diseases. Clinical tests and even more often additional imaging studies are required to make a proper diagnosis of urinary tract diseases. Just a few decades ago urography, cystography or voiding cystourethrography were the main methods in diagnostic imaging of the urinary tract. Today's imaging methods supported by digital radiographic and fluoroscopy systems, high sensitivity detectors with quantum detection, advanced algorithms eliminating motion artifacts, modern medical imaging monitors with a resolution of three or even eight megapixels significantly differ from conventional radiographic methods. The methods that are currently usually performed are: computed tomography, magnetic resonance imaging, isotopic methods and ultrasonography using elastography and new solutions in Doppler imaging. Modern techniques are currently focused on reducing radiation exposure with better imaging capabilities. The development of these techniques became an essential diagnostic aid in nephrological and urological practice. The aim of this paper is to present the latest solutions that are currently used in the diagnostic imaging of urinary tract diseases.

Early operative comparison of two epicardial left atrial appendage occluding systems applied during off-pump coronary revascularisation in patients with persistent atrial fibrillation.

INTRODUCTION: Atrial fibrillation (AF) increases long-term mortality and stroke rate in patients having coronary artery bypass grafting (CABG). Because oral anticoagulation (OAC) is associated with both a significant incidence of discontinuation and well known complication rates, left atrial appendage occlusion might be beneficial for stroke prevention. This study presents the first clinical and practical comparison of two epicardial left appendage occluders (LAAO) accruing experience in application during off-pump coronary revascularisation in patients with persistent AF. MATERIAL AND METHODS: Fifteen consecutive patients with persistent AF were assigned to intraoperative LAA occlusion with either TigerPaw System II (n = 8) or AtriClip (n = 7) device during off-pump CABG and concomitant left atrial epicardial ablation. Both systems were analysed in terms of ease and safety of application along with intraoperative LAA occlusion success. RESULTS: Surgical risk was increased in the study population (mean EuroScore II: 3.2 ± 0.3%). In all patients in the AtriClip group successful off-pump LAA occlusion confirmed by intraoperative transoesophageal echocardiography was achieved. The TigerPaw application was quicker and easier, but in 2 patients it was unsuccessful. During the hospital stay there were no bleeding or thromboembolic events recorded. CONCLUSIONS: In a pilot cohort epicardial LAAO during off-pump CABG in patients with persistent AF was performed safely and successfully with an AtriClip device. The TigerPaw System requires technological improvement. It might be useful to adapt the use of the type of occluding device to the LAA morphologic type and target revascularisation vessels to avoid the additional use of a heart positioner or obviate coronary compression.

Comparative Analysis of Clinical Outcomes of Tibial Plateau Fractures and Computed Tomography Examinations.

BACKGROUND: The aim of the study was to evaluate the outcomes of treatment of tibial plateau fractures. Based on CT examination, we set out to determine what factors were associated with a poor functional outcome. MATERIAL AND METHODS: The study involved 13 patients after surgery for tibial plateau fracture by locking plate osteosynthesis. CT studies using a MARS application were performed. The presence of subchondral defects was evaluated, including their area and depth. The reduction and widening of the tibial plateau were measured. The study data were correlated with the clinical outcome: pain according to a VAS scale, knee joint range of motion and the subjective IKDC score. RESULTS: Bone defects were observed in the majority of patients. On the basis of surface reconstruction, it was possible to accurately evaluate the articular step-off of the tibial plateau, which closely correlated with the range of motion and the subjective IKDC score (p <0.05). A correlation was also observed between widening of the lateral condyle and the IKDC score and pain level in the VAS scale (p<0.05). CONCLUSIONS: 1. Articular step-off and widening of the lateral condyle strongly correlates with the clinical outcome of tibial plateau fracture treatment. 2. The presence and extent of subchondral bone defects in the tibial plateau are related to knee pain in patients but have no effect on the range of motion and the IKDC score. 3. Bone graft remodeling cannot be fully assessed despite the use of MARS in CT imaging.

The additive effect of dorzolamide hydrochloride (Trusopt) and a morning dose of bimatoprost (Lumigan) on intraocular pressure and retrobulbar blood flow in patients with primary open-angle glaucoma.

AIMS: To assess the additive effect of dorzolamide hydrochloride 2% on the diurnal intraocular pressure (IOP) curve and retrobulbar haemodynamics in patients with primary open-angle glaucoma (POAG) treated with morning-dosed bimatoprost 0.03%. METHODS: Twenty-five patients with POAG were evaluated in a prospective, single-masked study. After a 1 week run-in period with bimatoprost all patients were treated with bimatoprost dosed once in the morning for 1 month, after which dorzolamide was added twice daily for 2 months. Goldmann applanation IOP, arterial blood pressure (ABP) and heart rate were measured every 2 h for 24 h and diurnal ocular perfusion pressure (OPP) was calculated. Colour Doppler imaging (CDI) of the ophthalmic artery (OA) and the central retinal artery (CRA) was recorded five times daily. All measurements were taken after the two phases of treatment and were compared. RESULTS: The mean baseline IOP was 14.8 ± 3.5 mm Hg. Mean IOP following bimatoprost monotherapy (12.8 ± 2.9 mm Hg) and after 2 months of dorzolamide adjunctive therapy (12.2 ± 2.6 mm Hg) were not statistically significantly different (p=0.544). Only at the 4:00 h time point was IOP significantly reduced using the bimatoprost/dorzolamide combined treatment (p=0.013). The 24 h IOP fluctuations were lower when dorzolamide was added (6.0 ± 2.3 mm Hg vs 4.6 ± 1.5 Hg, p=0.0016). Repeated analysis of variance detected a significant decrease of vascular resistance in the OA (p=0.0167) with adjunctive dorzolamide treatment. CONCLUSIONS: The addition of dorzolamide to morning-dosed bimatoprost had an additive hypotensive effect only on the night-time IOP curve at 4:00 h and resulted in a lower IOP fluctuation. Dorzolamide added to bimatoprost may reduce vascular resistance in the OA.

[Microwave ablation of liver tumors as a new instrument for minimally invasive liver surgery]. / Mikrofalowa ablacja guzów watroby jako nowe narzedzie w malo inwazyjnej chirurgii watroby.

Incidence of primary and secondary liver tumors is increasing. Hepatic resection remains the treatment of choice for hepatic tumors. For various reasons the vast majority of patients with liver tumors are not suitable for resection. These patients are candidates for several image-guided focal thermal ablative therapies as alternatives to resection. Currently available ablative techniques include cryotherapy, radiofrequency ablation (RFA), microwave ablation (MWA), laser ablation, high-intensity focused ultrasound ablation (HIFU), and ethanol injection. Presently RFA is most widely heat-based technology used for treatment of liver malignancies due to its availability, efficacy and low complication rates. However, RFA can be time-consuming and associated with higher recurrence rates in larger lesions. MWA is a new thermal ablative technique that uses electromagnetic energy to produce coagulation necrosis. MWA has several advantages over RFA such as an improved convection profile, consistently higher intratumoral temperatures, larger ablation volumes, faster ablation times, and the option of using multiple antennae simultaneously. We report our first experience using MWA and a Coviden Valleyab 915 MHz generator for ablation of liver tumor with respect to our previous experience with RFA. Further this study reviews current literature on the RFA and MWA for the treatment of the liver malignances. In our opinion although experience is limited MWA appears to be a safe and effective technology for hepatic tumor ablation and in some cases may be superior alternative to RFA.

[Mediastinal cyst in a patient on continuous ambulatory peritoneal dialysis successfully treated with video-assisted thoracic surgery (VATS)]. / Torbiel sródpiersia leczona skutecznie torakowideoskopia u chorej leczonej ciaglq ambulatoryjna dializq otrzewnowa.

Renal transplantation is the most effective form of renal replacement therapy of irreversible renal insufficiency. Due to posttransplant long term immunosuppressive treatment careful evaluation of the recipient is essential, especially detection and elimination of all potential sources of malignancy. In this paper, we describe successful video assisted thoracoscopic surgery of mediastinal cyst in peritoneal dialysis patient prepared for renal transplantation.