Nagy adatbázisok neurológiai kórképekben: nemzetközi áttekintés a sclerosis multiplex példáján = Databases in neurological diseases: overview of international examples in multiple sclerosis

Absztrakt: A rutin-betegellátás során hatalmas adattömeg keletkezik, amelyet jelenleg elszórva, többféle informatikai és papíralapú rendszerben tárolnak az egészségügyi intézményekben. A nagy adatbázisok elemzése egy adott betegség vonatkozásában segít egy populáció egészségügyi szükségleteinek meghatározásában és egészségügyi ellátásuk megszervezésében. Modellként a sclerosis multiplexet (SM) választottuk, melynek jól meghatározott diagnosztikus kritériumai vannak; a kezdeti diagnózis általában fekvőbeteg-ellátó intézményben történik, és a betegek gondozása és követése a járóbeteg-ellátásban zajlik. A sclerosis multiplex példáján célunk a nemzetközi és hazai adatbázisok feltérképezése és elemzése. A Semmelweis Egyetemen induló adattófejlesztés elemeként célnak tűztük ki a betegségspecifikus adatbázisok szükséges jellemzőinek meghatározását. A lényeges adatbeviteli kritériumok meghatározása céljából áttekintjük a legfontosabb nemzetközi SM-adatbázisokat. Vizsgáljuk a rögzített adatokat, az adatbázisok struktúráját, kitérünk az adatvédelmi szempontokra, az egyes adatbázisok hozzáférhetőségére és alkalmazási lehetőségeire. A SM helyi adatbázisok kezdetben elsősorban a betegek hatékonyabb gondozását célozták. Következő lépésként helyi és nemzetközi tudományos kutatásokhoz is szükség volt a betegek adataira. A betegségspecifikus adatbázisok létrehozása nagy jelentőségű volt a finanszírozók számára is, és a nagy betegpopulációról szerzett hosszú távú információk (úgynevezett „real-world” adatok) rendkívül fontosak a készítmények hatékonyságának és mellékhatásainak pontosabb megítélésére a populáció szintjén. Elemzésünk hozzájárul egy egészségügyi intézményi „adattó” létrehozásának szempontjaira fókuszáló projekthez, melyben a Semmelweis Egyetem egyes klinikái és diagnosztikus egységei mellett a Digitális Egészségtudományi Intézet is részt vesz. Orv Hetil. 2019; 160(4): 123–130. | Abstract: Vast amounts of data are created during routine patient care which are stored in unstructured digital and hardcopy formats in healthcare institutions. Analysis of large databases help to define the healthcare needs of the population and to organize healthcare services for specific diseases. As a model, we selected multiple sclerosis (MS), a disease with well-defined diagnostic criteria, a usually inpatient initial diagnosis, and a need for regular outpatient check-up. Using multiple sclerosis as an example, we set forth to screen and analyze international and Hungarian databases. In the framework of the initiation of the data lake system of Semmelweis University, we aim to define features of the data system needed for disease-specific databases for future applications. To determine essential data-entry criteria for such a database, we review the most important multiple sclerosis registries. We evaluate the type of registered data, structure of database, privacy issues, the availability and ways of application of the databases. Initially, the MS databases were created locally, aiming for better care of patients. As a further step, data were collected for scientific research by national and international co-operations. Disease-specific databases have become of high priority for national healthcare providers, and long-term information on a population (“real-world” data) is extremely important to assess the effectivity and safety of a treatment at the population level. Our analysis contributes to a project which focuses on the aspects of developing a data lake at a service provider level including clinical, diagnostic and digital healthcare departments of Semmelweis University, Budapest, Hungary. Orv Hetil. 2019; 160(4): 123–130

Sclerosis multiplex a közép-magyarországi régióban: a helyi adatbázisfejlesztés tapasztalatai és jövőbeli lehetőségei = Multiple sclerosis in central Hungary: experiences and future possibilities of developing a local database

Absztrakt: Bevezetés: A rutin-betegellátás során keletkező adatok többféle digitális és papíralapú rendszerben jönnek létre, egy adott beteg gondozása során előkeresésük nehézkes. A sclerosis multiplex (SM) a fiatalkorban jelentkező leggyakoribb neurológiai betegség, jelentős egészségügyi és gazdasági következményekkel. Célkitűzés: Célunk egy jól használható, kellően részletes adatbázis létrehozása, mely gyorsan hozzáférhetővé teszi a SM-beteg adatainak áttekintését, rögzítését és elemzését. Helyi regiszterünk létrehozásának tapasztalatai alapján dolgozzuk ki egy korszerű adatbázis modelljét. Módszer: Az adatbázis alapját Excel-programban hoztuk létre, majd a nemzetközileg is használt iMed-rendszerben dolgoztunk. A regiszterben az alapadatok mellett rögzítettük a megjelenéseket, a relapsusokat, a kísérő betegségeket, az MRI, liquor-, kiváltottválasz- és egyéb laboratóriumi vizsgálatok leletét, a gyógyszeres és nem gyógyszeres kezeléseket. Eredmények: Az adatbázis jelenleg 316 beteg adatát tartalmazza. MRI a betegek 96%-ánál, liquorvizsgálat 45%-nál történt. A SM-altípusok között a betegség kezdetekor a primer progresszív kórforma aránya 9%. A kórlefolyás során immunmoduláns kezelésben a betegek 82%-a részesült. Következtetés: Az adatbevitel jelenlegi „hagyományos” módszere időigényes, a manuális adatbevitel és a PDF formátumban történő adatexport (leletek) a mai informatikai lehetőségek mellett elavultak. A korszerű és mind klinikai, mind kutatási célokra alkalmazható, helyi betegségspecifikus adatbázis folyamatos és nagyrészt automatikus adatbevitelt igényel. Ehhez elvárás az egységes dokumentációs nyelv és struktúra kialakítása és a különböző digitális rendszerekben rendelkezésre álló információk automatizált átemelése. A mindezt teljesítő leendő regiszter működési modelljét mutatjuk be, amely egy egészségügyi adattóra épül. Orv Hetil. 2019; 160(4): 131–137. | Abstract: Introduction: Data during routine patient care are created in multiple digital and paper-based hardcopy systems, therefore their retrieval is cumbersome in the follow-up of patients. Multiple sclerosis is the most prevalent neurological disorder in the young age, with major consequences on health and socio-economic status. Aim: We set forth to create a user-friendly, detailed local database where it is easy to access, register and analyze data. Based on our experiences during building this registry, we develop the model of a modern type of database. Method: First we established a local registry in Excel, then data were transferred to the worldwide used iMed system. Separate pages were used to register basic data, follow-up visits, relapses, accompanying diseases, results of neuroimaging, cerebrospinal fluid, evoked response and other tests, pharmacological and non-pharmacological treatments. Results: The database currently contains data of 316 patients. MRI was performed in 96%, cerebrospinal fluid examination in 45% of the patients. The rate of primary progressive disease at disease onset is 9%. Disease modifying treatments were applied in 82% of the patients. Conclusion: The traditional manual data entry and data export in PDF format is obsolete and time-consuming. The development of local disease-specific databases appropriate for clinical and research purposes requires continuous and mostly automatic data entry. In future local registries the establishment of uniform documentational language and structure, and automatic transfer of information among different digital systems are required. We present the model of such a registry, which is based on a healthcare data lake. Orv Hetil. 2019; 160(4): 131–137

Ultrasonography of MADSAM neuropathy: Focal nerve enlargements at sites of existing and resolved conduction blocks

Using the emerging technique of peripheral nerve ultrasonography, multiple focal nerve swellings corresponding to sites of existing conduction blocks have been described in demyelinating polyneuropathies. We report two cases of multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). In the first, multiple focal nerve enlargements were detected by ultrasound at sites of previous conduction blocks, well after complete clinical and electrophysiological resolution. In the second case, existing proximal conduction blocks could be localized by ultrasound. Our cases highlight the importance of nerve ultrasound in identifying conduction blocks and demonstrate that ultrasonographic morphological changes may outlast functional recovery in demyelinating neuropathies. © 2012 Elsevier B.V

Szérumbiomarkerek akut lumbalis-lumbosacralis fájdalomban

Inflammation contributes to the pathogenesis of low back pain and sciatica. Growing evidence suggests that elevated levels of some inflammatory biomarkers are associated with these conditions. Much of the research evaluating the association between pro- and anti-inflammatory cytokines, chemokines, other regulatory molecules, and low back pain and sciatica, focused on patients with chronic low back pain, while fewer studies addressed the issue of detectable biomarkers in the acute phase. Previous studies suggest that pro-inflammatory cytokines such as TNF-alpha, IL-6, and IL-8 and anti-inflammatory IL-4 and IL-10 play an important role in the inflammatory response following intervertebral disc herniation. According to the approach of personalized medicine it is important to identify subsets of patients within the acute patient group regarding etiology, prognosis and treatment. In addition, if we can identify subgroups based on levels of pro-inflammatory biomarkers, where inflammation may be the leading cause of pain, we assume that this subgroup would likely be effectively treated with anti-inflammatory medication. The efficacy of TNF-alpha inhibitors and IL-6 inhibitors in treating low back pain and sciatica has already been tested in clinical trials, but further studies are required. Overall, identification of circulating biomarkers of acute low back pain and sciatica may assist in refining personalized diagnosis and treatment. Further research is needed to evaluate the role of inflammation in acute low back pain and sciatica, to identify what methods are appropriate for evaluation in clinical practice, and whether there are biomarkers of prognostic value in these patients

Incidence and prevalence of multiple sclerosis in Hungary based on record linkage of nationwide multiple healthcare administrative data

ObjectivesAs there were only regional studies in Hungary about the prevalence of multiple sclerosis (MS), we aimed to estimate its epidemiological features using data of Hungary's single-payer health insurance system.MethodsPseudonymized database of claims reported by hospitals and outpatient services between 2004-2016 was analyzed and linked with an independent database of outpatient pharmacy refills between 2010-2016. We established an administrative case definition of MS and validated it on medical records of 309 consecutive patients. A subject was defined as MS-patient if received MS diagnosis (International Classification of Diseases, 10th edition, code G35) on three or more occasions at least in 2 calendar years and at least once documented by a neurologist. Patients were counted as incident cases in the year of the first submitted claim for MS. We allowed a 6-year-long run-in period, so only data between 2010-2015 are discussed.ResultsSensitivity of the administrative case definition turned out to be 99%, while specificity was >99%. Crude prevalence of MS has increased from 109.3/100,000 in 2010 to 130.8/100,000 in 2015 (p-value = 0.000003). Crude incidence declined from 7.1/100,000 (2010) to 5.4/100,000 (2015) (p-value = 0.018). Direct standardization - based on European standard population and results of nationwide Hungarian census of 2011 - revealed that age standardized prevalence was 105.2/100,000 (2010), which has grown to 127.2/100,000 (2015) (p-value = 0.000001). Age standardized incidence rate declined from 6.7/100,000 (2010) to 5.1/100,000 (2015) (p-value = 0.016). The ratio of MS-patients receiving ≥1 prescription for disease modifying treatment increased from 0.19 (2010) to 0.29 (2015) (p-value = 0.0051). The female/male ratio of prevalent cases remained 2.6.DiscussionThe prevalence of MS in Hungary is higher than previously reported, the incidence rate is moderate. The prevalence is rising, the incidence rate shows decline. The proportion of patients receiving disease modifying treatment grows but was still around 30% in 2015

Treatment of relapsing multiple sclerosis in Hungary – consensus recommendation from the Hungarian neuroimmunology society

Multiple sclerosis (MS) may impact quality of life, careers and family plans of the affected individuals. The current treatments with disease modifying therapies aim to prevent people with MS (pwMS) from disability accumulation and progression. Different countries have different reimbursement policies resulting in inequalities in patient care among geographical regions. Access to anti-CD20 therapies for relapsing MS is restricted in Hungary because therapy of individual cases only is reimbursed. In the light of the latest research and national guidelines, 17 Hungarian MS experts agreed on 8 recommendations regarding relapsing pwMS using the Delphi round method. Strong agreement (> 80%) was achieved in all except one recommendation after three rounds, which generated a fourth Delphi round. The experts agreed on treatment initiation, switch, follow-up and discontinuation, as well as on special issues such as pregnancy, lactation, elderly population, and vaccination. Well-defined national consensus protocols may facilitate dialogue between policymakers and healthcare professionals and thus contribute to better patient care in the long run

The Urine Proteome Profile Is Different in Neuromyelitis Optica Compared to Multiple Sclerosis: a Clinical Proteome Study

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In Vivo Evaluation of Retinal Neurodegeneration in Patients with Multiple Sclerosis

To evaluate macular morphology in the eyes of patients with multiple sclerosis (MS) with or without optic neuritis (ON) in previous history.Optical coherence tomography (OCT) examination was performed in thirty-nine patients with MS and in thirty-three healthy subjects. The raw macular OCT data were processed using OCTRIMA software. The circumpapillary retinal nerve fiber layer (RNFL) thickness and the weighted mean thickness of the total retina and 6 intraretinal layers were obtained for each eye. The eyes of MS patients were divided into a group of 39 ON-affected eyes, and into a group of 34 eyes with no history of ON for the statistical analyses. Receiver operating characteristic (ROC) curves were constructed to determine which parameter can discriminate best between the non-affected group and controls.The circumpapillary RNFL thickness was significantly decreased in the non-affected eyes compared to controls group only in the temporal quadrant (p = 0.001) while it was decreased in the affected eyes of the MS patients in all quadrants compared to the non-affected eyes (p<0.05 in each comparison). The thickness of the total retina, RNFL, ganglion cell layer and inner plexiform layer complex (GCL+IPL) and ganglion cell complex (GCC, comprising the RNFL and GCL+IPL) in the macula was significantly decreased in the non-affected eyes compared to controls (p<0.05 for each comparison) and in the ON-affected eyes compared to the non-affected eyes (p<0.001 for each comparison). The largest area under the ROC curve (0.892) was obtained for the weighted mean thickness of the GCC. The EDSS score showed the strongest correlation with the GCL+IPL and GCC thickness (p = 0.007, r = 0.43 for both variables).Thinning of the inner retinal layers is present in eyes of MS patients regardless of previous ON. Macular OCT image segmentation might provide a better insight into the pathology of neuronal loss and could therefore play an important role in the diagnosis and follow-up of patients with MS