Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ’ and clinicians’ unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union’s Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ’ preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
The term ‘undifferentiated connective tissue disease’ (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.
Introduction: The Femoral angle of anteversion (FAA) is responsible for the medial and anterior direction of the femoral neck and therefore the femoral head towards the acetabulum. The aim of this study was to determine the difference in FAA between male and female samples, the correlation between the FAA and biomechanically relevant parameters and to provide a review of relevant clinical features related to FAA. Methods: We included 100 human dry femora and analyzed FAA, Angle of Inclination (AI), Femoral Head Diameter (FHD), Femoral Biomechanical Length (FBL) and Linear Condylar Parameters (Epicondylar Breadth Width (EBW), Lateral Condyle Depth and Medial Condyle Depth). The measurements were made using a goniometer, sliding calipers and pieces of colored string. Results: Mean FAA values were 9.84±7.97° and 8.72±8.23° for the male and female samples, respectively (p<0.05). FAA and AI in both male and female correlated negatively (-0.076), while there was a positive correlation between FAA and FHD (0.069), FAA and FBL (0.072), FAA and EBW (0.029), while the correlation was negative between FAA and LCD (-0.072), FAA and MCD (-0.063). Conclusion: The difference in FAA between male and female femora was found to be significant. This finding may help better understanding such as hip impingement, total hip arthroplasty failure, and design of femoral endoprosthesis parts.
Aim To identify rates of most common pre-analytical errors and to document possible (different) error rates between inpatients and outpatients. Methods This retrospective study was conducted at the Department of Medical Biochemistry and Immunology Diagnostics, Cantonal Hospital Zenica, from December 2016 until March 2017. Data on rejected blood samples in the laboratory information system were analysed. Results During the 3-month period 35,343 patient blood samples (25,545 inpatients and 9,798 outpatients) were received in the laboratory. The study identified 602 (1.70%) rejected samples because of pre-analytical errors, mostly due to haemolysis, 292 (48.50%), and clotted samples, 240 (39.87%). The remaining 70 (11.63%) samples were rejected because of inappropriate sample volume, inappropriate container and identification errors (7.81%, 2.16% and 1.66%, respectively). The proportion of inpatient rejected samples was 8.7-fold higher than in the outpatient samples. The proportion of inpatient rejected samples because of haemolysis, clotted samples, inappropriate sample volume and inappropriate containers were higher than in the outpatient samples (20.5-, 12.1-, 2.3- and 1.3-fold higher, respectively); proportion of rejected samples because of identification errors was 8.0-fold higher in the outpatient (collection sites outside the hospital) than in the inpatient samples. Conclusion Higher pre-analytical sample error rates were connected with inpatient samples, while higher identification error rates were connected with outpatient samples. Establishment of periodic stuff training and introduction of information technology could reduce pre-analytical errors.
Aim To assess hand function and explore the relationship between hand function and neuroimaging findings in children with unilateral spastic cerebral palsy (US CP). Methods Hand function was assessed using Manual Ability Classification System (MACS, I-V). Brain lesions were divided into five groups: brain maldevelopment (MAL), periventricular white matter lesions (PV WM), cortical/subcortical gray matter lesions (C/SC GM), nonspecific and normal findings. Results Of 114 children with US CP (77 boys and 37 girls), 56 were with right-sided and 58 with left-sided involvement. MACS I was found in 49 (42.9%), MACS II in 19 (16.7%), MACS III in 19 (16.7 %), MACS IV in 9 (7.9%) and MACS V in 18 (15.8%) children (p=0.002). Computed tomography (CT) as the only neuroimaging has been done in 18 (15.8%), magnetic resonance imaging (MRI) at 94 (82.5%) children, whereas 2 (1.7%) children had neither CT nor MRI. The CT showed PV WM in eight (44.4%), C/SC GM lesions in 6 (33.3%), and normal findings in 4 (22.2%) children (p=0.709). The MRI showed MAL in 8 ( 8.5%), PV WM in 46 (48.9%), C/SC GM in 28 (29.8%), miscellaneous in 2 (2.1%), and normal finding in 10 (10.7%) children (p=0.0001). Mild hand dysfunction (MACS I and II) was assessed in 68 (59.7%) children, of which 33 had PV WM lesions (p=0.001). Conclusion Mild hand dysfunction in children with US CP has been significantly associated with PV WM lesions. The type of brain lesion may help to identify its timing and predict the level of hand dysfunction.
Aim To develop an online biofilm calculation tool (Biofilm Classifier), which calculates the optical density cut off value and accordingly determines the biofilm forming categories for the tested isolates by standardized formulas, as well as to compare the results obtained by Biofilm Classifier to manual calculations and the use of predefined values. Methods The biofilm forming capacity of tested strains was evaluated using tissue culture plate method in 96 well plates, and optical density (OD) value of the formed biofilm was measured on an ELISA Microplate reader at 595 nm on a total of 551 bacterial isolates from clinical specimen. Results Comparative analysis indicated that the manual calculation was 100% in accordance with results obtained by the designed software as opposed to the results obtained by use of predefined values for biofilm categorization. When using predefined values compared to manual biofilm categorization for the determination of biofilm positive and biofilm negative strains the specificity was 100%, sensitivity 97.81%, positive predictive value 100%, negative predictive value 96.04% and accuracy 98.57%. Conclusion Considering obtained results, the use of the designed online calculator would simplify the interpretation of biofilm forming capacity of bacteria using tissue culture plate method.
This paper aims to analyze the beta convergence of Western Balkan countries towards the EU-15 Member States in the period 2004-2016, and two sub-periods: 2004-2008 and 2009-2013. Beta convergence is based on the neoclassical growth theory and tests the hypothesis that poor countries tend to grow faster than rich countries, in per capita terms. The empirical findings support the economic convergence hypothesis, with convergence rates ranging from 1.1% to 2.3%. The results show that the recent financial crisis negatively affected the absolute and conditional convergence process, when economic variables are included. The main limitation of the research is the availability of data.
Aim To present our experience with a diagnostic ability of endoscopic ultrasound (EUS) and magnetic retrograde cholangiopancreatography (MRCP) in cases of choledocholithiasis verified by endoscopic retrograde cholangiopancreatography (ERCP). Methods This retrospective study was conducted after a collection of data involving 58 suspected choledocholithiasis patients who underwent ERCP from January 2013 to December 2015. Patients who were diagnosed with choledocholithiasis on the basis of clinical symptoms and radiological findings and who underwent ERCP were included in this study. The first group (29 patients) underwent EUS, and the second group (29 patients) underwent MRCP. The ERCP was performed in both groups. Sensitivity, specificity and diagnostic accuracy of EUS and MRCP were determined by comparing them with ERCP, which was considered to be a gold standard. Results Gender representation was in favour of males, 58:42%. The mean age was 55.5 years. In the group 1 (EUS) 22 patients were found to have choledocholithiasis using ERCP. The EUS stone detection rate was 88%. Endoscopic ultrasound showed sensitivity (97%), specificity (67%) and accuracy (88%), positive predictive value (PPV) of 88%, negative predictive value (NPV) of 80%. In the group 2 (MRCP) 16 patients were found to have choledocholithiasis by ERCP. MRCP sensitivity was 81%, specificity 40%, PPV of 74%, NPV of 50%. Conclusion The EUS was a superior non-invasive tool in comparison with MRCP for detecting choledocholithiasis, which was confirmed using ERCP.
Abstract In this video, we demonstrate epidermoid tumor microsurgical resection of the cerebellopontine angle (CPA) performed by the senior author (K.I.A.). Epidermoid tumors arise from ectoderm trapped within/displaced into the central nervous system. They show predilection for CPA Angle (up to 40%), 4th ventricle, suprasellar region, and spinal cord.1 They are the 3rd most common CPA tumor, comprising approximately 7% of CPA pathology. CPA lesions can produce 5th and 7–12th cranial nerve neuropathies.2 3 4 Recurrent episodes of aseptic meningitis caused by cyst content rupture may occur. Symptoms include fever, meningeal irritation, and hydrocephalus. A 26-year-old female presented with headaches. Head magnetic resonance imaging (MRI) revealed right CPA tumor with brain stem compression (Fig. 1, A–C). There was evidence of restricted diffusion in diffusion-weighted imaging, typical of epidermoid tumor. Surgery was performed in prone position with head turned 25 degrees to the ipsilateral side using retrosigmoid craniotomy.5 Tumor was ventral to the 7th and 8th cranial nerve complexes, between the 5th nerve as well as toward the brainstem. The surgical plan was gross total resection with tumor capsule resection to prevent recurrence.6 (Small residuals can be left behind when capsule is adherent to critical structures.) Tumor was adherent to brain stem perforators which were preserved using meticulous dissection. Cranial nerves and vascular structures were also left intact. We irrigated with antibiotic saline and used perioperative treatment to prevent aseptic meningitis. The pathohistological diagnosis revealed epidermoid tumor cyst. Postoperative MRI revealed complete resection (Fig. 1, D–F). The patient recovered fully and was neurologically intact. The link to the video can be found at: https://youtu.be/LyWl-KZUSGY.
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