240 év dobozokban : A Szegedi Zsidó Hitközség Archívuma

A szegedi zsidóság írott történetének kezdeti időpontja 1785. Az egykori hitközségi tagoknak és rabbiknak, iparosoknak, tanítóknak, tudósoknak és művészeknek köszönhetően vált Szeged a 20. század eleji vidéki Magyarország egyik legfontosabb gazdasági és kulturális központjává. A hitközség archívuma csaknem 200 éves időszak hivatalos és magándokumentumait őrzi, továbbá textilek és más tárgyak szintén részei a gyűjteménynek. Mivel sorsuk szorosan összefügg a közösségével, ezért a gyűjtemény magán viseli mindazon történelmi események nyomait és következményeit, amit a helyi zsidóság is átélt. Az archívum gyűjtőkörét illetően a fondjegyzék nyújt eligazítást, amely az intézmény honlapján olvasható (szegedjewisharchives.org). 2017-től 2020-ig a gyűjtemény anyagát 1970-ig bezárólag a levéltári előírásoknak megfelelően rendeztük, jelenleg is tart a kulcsszavazásuk. Archívumunk munkatársai folyamatosan publikálnak, előadásokat tartanak, rendezvényeket szerveznek a felejtés megállításának szándékával. Az iratok nélkülözhetetlen forrásul szolgálnak a tudományos szakemberek, a felsőfokú oktatásban résztvevők és a családkutatók számára. Jelen tanulmány célja, hogy átfogóan bemutassa a hitközség archívumát

The value of PLA2R antigen and IgG subclass staining relative to anti-PLA2R seropositivity in the differential diagnosis of membranous nephropathy

The diagnostic performance of PLA2R and IgG subclass staining of kidney biopsies relative to anti-PLA2R seropositivity in the differentiation of primary and secondary membranous nephropathy (pMN, sMN) was examined. Besides PLA2R staining - which has a lower specificity than anti-PLA2R antibody serology - there is insufficient knowledge to decide which IgG1-4 subtype immunohistological patterns (IgG4-dominance, IgG4-dominance/IgG1-IgG4-codominance or IgG4-dominance/IgG4-codominance with any IgG subtype) could be used to distinguish between pMN and sMN.87 consecutive Hungarian patients (84 Caucasians, 3 Romas) with the biopsy diagnosis of MN were classified clinically as pMN (n = 63) or sMN (n = 24). The PLA2R and IgG subclass staining was part of the diagnostic protocol. Anti-PLA2R antibodies were determined by an indirect immunofluorescence test in 74 patients with disease activity.For pMN, the sensitivity of anti-PLA2R seropositivity was 61.1%, and the specificity was 90.0%; and similar values for PLA2R staining were 81.0%, and 66.7%, respectively. In all stages of pMN, IgG4-dominance was the dominant subclass pattern, while the second most frequent was IgG3/IgG4-codominance. The sensitivity and specificity scores were: IgG4-dominance 52.2% and 91.7%, IgG4-dominance/IgG3-IgG4-codominance 76.2% and 87.5%, IgG4-dominance/IgG1-IgG4-codominance 64.2% and 75%, and IgG4-dominance/codominance with any IgG subclass 92.1% and 70.8%, respectively. Anti-PLA2R seropositivity, glomerular PLA2R, and IgG4-dominance/codominance significantly correlated with each other. The IgG4 subclass was rarely encountered in sMN.In our series, IgG4-dominance had the highest specificity in the differentiation of MN, just as high as that for anti-PLA2R seropositivity. The specificity values of PLA2R staining and IgG4-dominance/codominance with any IgG subclass or IgG4-dominance/IgG1-IgG4 codominance were ≤ 75%. Apart from IgG4 dominance, IgG4-dominance/IgG3-IgG4-codominance also had good statistical value in differentiating pMN from sMN. As IgG subclass switching during the progression of pMN was not the feature of our cohort, pMN in Hungarian patients is presumed to be an IgG4-related disorder right from the start. Although anti-PLA2R seropositivity has become the cornerstone for diagnosing pMN, if a kidney biopsy evaluation is conducted, besides the staining of PLA2R antigen, the evaluation of IgG subclasses provides relevant information for a differential diagnosis. Even in cases with IgG4-dominance, however, malignancy should be thoroughly checked

Enyhe szövettani eltérések ellenére gyors progressziójú proliferativ glomerulonephritis monoklonális immunglobulin-G-depozitumokkal = Rapidly progressive proliferative glomerulonephritis with monoclonal immunoglobulin G deposits despite the mild histological changes. Case report

Absztrakt: A proliferativ glomerulonephritis monoklonális immunglobulin-G (IgG)-depozitumokkal entitást immunfluoreszcens vizsgálattal szemcsés mintázatú monoklonális IgG (többnyire IgG3-kappa), elektronmikroszkóppal elektrondenz depozitumok, fénymikroszkóppal jobbára membranoproliferativ vagy endocapillaris proliferativ laesio jellemzi; előfordulhatnak félholdak. A glomerulonephritist plazmasejt/B-sejt klón által szekretált immunglobulinmolekula lerakódása okozza; a biopszia időpontjában csupán a betegek harmadánál mutatható ki paraproteinaemia. A proteinuriához gyakran társul haematuria és valamilyen szintű veseelégtelenség, a betegek negyedénél alakul ki végstádiumú veseelégtelenség. Egy 62 éves nőbeteg gyors vesefunkció-romlásának hátterében a klinikai kép, a laboratóriumi és a képalkotó vizsgálatok nem támogattak praerenalis és postrenalis okot, illetve intrinsic vascularis vagy tubulointerstitialis eredetet. A proteinuria és a glomerularis microhaematuria alapján gyorsan progrediáló glomerulonephritist valószínűsítettünk. Az ez irányú kivizsgálás antineutrofil citoplazma-antitest, antiglomerularis bazálmembrán, sejtmagellenes ellenanyagok, illetve cryoglobulin tekintetében negatívnak bizonyult, a szérum-C3- és -C4-szint a normális tartományban volt. Vesebiopsziát végeztünk. Immunfluoreszcens vizsgálattal a mesangiumban IgG3-kappa-, C3- és C1q-pozitív szemcsés depozitumok látszottak, melyek ultrastrukturálisan elektrondenz depozitumoknak bizonyultak. Fénymikroszkóppal 2 heges és 29 nyitott glomerulust vizsgáltunk, az utóbbiakban csupán enyhe mesangialis sejtproliferatio mutatkozott. A glomerularis elváltozásokat enyhe arteriola hyalinosis, interstitialis fibrosis és tubulus atrophia kísérte. Proliferativ glomerulonephritis monoklonális IgG-depozitumokkal betegséget kórisméztünk (gyakorisága felnőtt natív vesebiopsziás anyagunkban 0,18%). A beteg hematológiai kivizsgálása paraproteint, myeloma multiplexet nem igazolt. Az enyhe morfológiai eltérések ellenére a veseelégtelenség előrehaladt, és a diagnózis felállítása után két héttel hemodialíziskezelést kellett kezdenünk. Szteroid, ciklofoszfamid, majd rituximab adása a vesefunkciót nem befolyásolta; a beteg krónikus hemodialízisprogramba került. Az esetismertetés tudomásunk szerint az első hazai közlés; nefrológiai érdekessége a gyorsan progrediáló glomerulonephritis szindróma és a szövettanilag látott enyhe elváltozások közötti szembetűnő eltérés. Orv Hetil. 2018; 159(38): 1567–1572. | Abstract: Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits is characterized by granular deposits of monoclonal IgG; histologically it has typically a membranoproliferative or endocapillary pattern, and seen electronmicroscopically there are dense deposits without substructure. Here, we present the case of a 62-year-old Caucasian woman who was admitted with rapidly progressive kidney failure. The patient’s status, the laboratory and imaging examinations did not support prerenal, postrenal and – among the intrinsic causes – vascular and tubulointerstitial origin. The proteinuria and dysmorphic microhematuria suggested rapidly progressive glomerulonephritis. Tests for anti-neutrophil cytoplasmic antibodies, anti-glomerular basement membrane, antinuclear antibodies and cryoglobulins were negative, the C3 and C4 levels were normal. The biopsy evaluation diagnosed proliferative glomerulonephritis with monoclonal IgG deposits because of mesangial granular deposits of IgG3-kappa, C3, and C1q, and ultrastructurally electron-dense deposits (incidence in our adult native kidney biopsy series: 0.18%). 31 glomeruli were assessed histologically. 29 glomeruli displayed mild mesangial hypercellularity, 2 glomeruli were globally sclerotic. Crescents were not observed. Mild arteriolar hyalinosis, interstitial fibrosis and tubular atrophy accompanied the glomerular alterations. In the postbiopsy evaluation, paraprotein or multiple myeloma was not detected. Despite the mild histological findings, the kidney failure progressed, and hemodialysis had to be started two weeks after the biopsy. Steroids, cyclophosphamide and rituximab did not affect her kidney function, and she remained on hemodialysis during the follow-up of 39 months. This report presents for the first time proliferative glomerulonephritis with monoclonal IgG deposits as the possible cause of rapidly progressive nephritic syndrome in the absence of pronounced glomerular proliferative, sclerotic or tubulointerstitial lesions. Orv Hetil. 2018; 159(38): 1567–1572

Multidisciplinary management of acute cholecystitis during the COVID-19 pandemic

The coronavirus disease 2019 pandemic had a major impact on most medical services. Our aim was to assess the outcome of acute cholecystitis during the nationwide lockdown period. All patients admitted to our emergency department for AC were analysed. Patient characteristics, performance status, AC severity, treatment modality and outcome of AC were assessed during the lockdown period (Period II: 1 April 2020–30 November 2021) and compared to a historical control period (Period I: 1 May 2017–31 December 2018). AC admissions increased by 72.8% in Period II. Patients were younger (70 vs. 74 years, p = 0.017) and greater in number in the CCI 1 group (20.4% vs. 11.2%, p = 0.043) in Period II. The unplanned readmission rate (6.3 vs. 0%, p = 0.004) and the gallbladder perforation (GP) rate was higher (18.0 vs. 7.3%, p = 0.006) in Period II. Percutaneous transhepatic gallbladder drainage (PTGBD) was more frequent (24.1 vs. 12.8%, p = 0.012) in Period II. In addition to a drop in patient age and CCI, a significant rise in the prevalence of acute cholecystitis, GP and unplanned readmissions was observed during the nationwide lockdown due to the COVID-19 pandemic. PTGBD was more frequent during this period, whereas successful conservative treatment was less frequent

Dipper-like variability of the Gaia alerted young star V555 Ori

V555 Ori is a T Tauri star, whose 1.5 mag brightening was published as a Gaia science alert in 2017. We carried out optical and near-infrared photometric, and optical spectroscopic observations to understand the light variations. The light curves show that V555 Ori was faint before 2017, entered a high state for about a year, and returned to the faint state by mid-2018. In addition to the long-term flux evolution, quasi-periodic brightness oscillations were also evident, with a period of about 5 days. At optical wavelengths both the long-term and short-term variations exhibited colourless changes, while in the near-infrared they were consistent with changing extinction. We explain the brightness variations as the consequence of changing extinction. The object has a low accretion rate whose variation in itself would not be enough to reproduce the optical flux changes. This behaviour makes V555 Ori similar to the pre-main sequence star AA Tau, where the light changes are interpreted as periodic eclipses of the star by a rotating inner disc warp. The brightness maximum of V555 Ori was a moderately obscured ($A_V$=2.3 mag) state, while the extinction in the low state was $A_V$=6.4 mag. We found that while the Gaia alert hinted at an accretion burst, V555 Ori is a standard dipper, similar to the prototype AA Tau. However, unlike in AA Tau, the periodic behaviour was also detectable in the faint phase, implying that the inner disc warp remained stable in both the high and low states of the system.Comment: Accepted to MNRA