Hydroxychloroquine (HCQ) is an important medication for treating systemic lupus erythematosus (SLE). Its blood concentration ([HCQ]) varies widely between patients and is a marker and predictor of SLE flares. This prospective randomised, double-blind, placebo-controlled, multicentre study sought to compare standard and adjusted HCQ dosing schedules that target [HCQ] ≥1000 ng/ml to reduce SLE flares. [HCQ] was measured in 573 patients with SLE (stable disease and SELENA-SLEDAI≤12) treated with HCQ for at least 6 months. Patients with [HCQ] from 100 to 750 ng/ml were randomised to one of two treatment groups: no daily dose change (group 1) or increased HCQ dose to achieve the target [HCQ] (group 2). The primary end point was the number of patients with flares during 7 months of follow-up. Overall, mean [HCQ] was 918±451 ng/ml. Active SLE was less prevalent in patients with higher [HCQ]. A total of 171 patients were randomised and followed for 7 months. SLE flare rates were similar in the two groups (25% in group 1 vs 27.6% in group 2; p=0.7), but a significant spontaneous increase in [HCQ] in both groups between inclusion and randomisation strongly suggested improved treatment adherence. Patients at the therapeutic target throughout follow-up tended to have fewer flares than those with low [HCQ] (20.5% vs 35.1%, p=0.12). Although low [HCQ] is associated with higher SLE activity, adapting the HCQ dose did not reduce SLE flares over a 7-month follow-up.
130 Autoren, vier Bände auf Polnisch und fünf Bände auf Deutsch: Das größte gemeinsame deutsch-polnische Projekt im Bereich der Geisteswissenschaften wirft einen neuen Blick auf die nachbarschaftliche Beziehungsgeschichte beider Länder. Was verbindet Napoleon, Rosa Luxemburg, Flucht und Vertreibung, das Jahr 1989, Solidarnos´c´ und Kreisau? Oder Preußen, die Oder-Neiße-Grenze und Russland? Es handelt sich um (einige) deutsch-polnische Erinnerungsorte. Erinnerungsorte sind keine topographischen Orte, sondern historische Bezugspunkte der kulturellen Identität einer Gesellschaft. Dies können Personen, Ereignisse oder historische Phänomene sein. Der hier erstmals angewandte bilaterale Ansatz der Erinnerungsforschung hinter-fragt nationale Vorstellungen und entwickelt neue vergleichende Perspektiven. Deutsche und Polen teilen viele Erinnerungen, die jedoch unterschiedlichen Identitätsbedürfnissen in beiden Gesellschaften entsprechen. Die hier vorliegenden Essays über gemeinsame und geteilte deutsch-polnische Erinnerungsorte erlauben analytische Einblicke in die Erinnerungskulturen beider Länder, in ihre Gemeinsamkeiten und Unterschiede. Die deutsch-polnische Nachbarschaft bringt es mit sich, dass sich ohne die Kenntnis der polnischen Geschichte die deutsche und umgekehrt ohne die Kenntnis der deutschen die polnische Geschichte nur unvollkommen verstehen lassen.
Abstract Objectives The objective of this study is to better characterize the features and outcomes of a large population of patients with mixed connective tissue disease (MCTD). Methods We performed an observational retrospective multicenter cohort study in France. Patients who fulfilled at least one diagnostic criterion set for MCTD and none of the criteria for other differentiated CTD (dCTD) were included. Results Three hundred and thirty patients (88% females, median [interquartile range] age of 35 years [26–45]) were included. The diagnostic criteria of Sharp or Kasukawa were met by 97.3% and 93.3% of patients, respectively. None met other classification criteria without fulfilling Sharp or Kasukawa criteria. After a median follow‐up of 8 (3–14) years, 149 (45.2%) patients achieved remission, 92 (27.9%) had interstitial lung disease, 25 (7.6%) had pulmonary hypertension, and 18 (5.6%) died. Eighty‐five (25.8%) patients progressed to a dCTD, mainly systemic sclerosis (15.8%) or systemic lupus erythematosus (10.6%). Median duration between diagnosis and progression to a dCTD was 5 (2–11) years. The presence at MCTD diagnosis of an abnormal pattern on nailfold capillaroscopy (odds ratio [OR] = 2.44, 95% confidence interval [95%CI] [1.11–5.58]) and parotid swelling (OR = 3.86, 95%CI [1.31–11.4]) were statistically associated with progression to a dCTD. Patients who did not progress to a dCTD were more likely to achieve remission at the last follow‐up (51.8% vs. 25.9%). Conclusions This study shows that MCTD is a distinct entity that can be classified using either Kasukawa or Sharp criteria, and that only 25.8% of patients progress to a dCTD during follow‐up.
Gartner's duct cysts are usually small and asymptomatic and occur in about 1% of all women. Sometimes they present as perivaginal masses and usually appear in infants. These cysts can be large enough to protrude from the vaginal introitus and can be associated with symptoms such as vaginal discharge or recurrent infections. Gartner's duct cysts are embryological remnants of the Wollfian duct and can therefore be associated with other urogenital malformations like ectopic ureters, unilateral renal agenesis or multicystic dysplastic kidneys.
Abstract Eosinophils have widespread procoagulant effects. Eosinophilic cardiovascular toxicity mostly consists of endomyocardial damage or eosinophilic vasculitis, while reported cases of venous thrombosis (VT) are scarce. We aimed to report on the clinical features and treatment outcomes of patients with unexplained VT and eosinophilia, and to identify predictors of relapse. This retrospective, multicenter, observational study included patients aged over 15 years with VT, concomitant blood eosinophilia ≥ 1G/L and without any other moderate-to-strong contributing factors for VT. Fifty-four patients were included. VT was the initial manifestation of eosinophil-related disease in 29 (54%) patients and included pulmonary embolism (52%), deep venous thrombosis (37%), hepatic (11%) and portal vein (9%) thromboses. The median [IQR] absolute eosinophil count at VT onset was 3.3G/L [1.6–7.4]. Underlying eosinophil-related diseases included FIP1L1-PDGFRA-associated chronic myeloid neoplasm (n = 4), Eosinophilic Granulomatosis with Polyangiitis (n = 9), lymphocytic (n = 1) and idiopathic (n = 29) variants of hypereosinophilic syndrome. After a median [IQR] follow-up of 24 [10–62] months, 7 (13%) patients had a recurrence of VT. In multivariate analysis, persistent eosinophilia was the sole variable associated with a shorter time to VT relapse (HR 7.48; CI95% [1.94–29.47]; p = 0.015). Long-term normalization of eosinophil count could prevent the recurrence of VT in a subset of patients with unexplained VT and eosinophilia ≥ 1G/L.
Zusammenfassung Anhand der Polnischen Republik nach dem deutschen Überfall am 1. September 1939 zeigt der Text, dass die Evakuierungsplanungen zum Transport der wichtigsten Gefangenen noch auf die Erfahrungen des Zusammenbruchs staatlicher Herrschaft des Russischen Reichs am Beginn und Ende des Ersten Weltkriegs zurückgingen. Ein Teil der Aufseher der im Süden Polens unweit der deutsch-polnischen Grenze gelegenen Strafvollzugsanstalt Święty Krzyż eskortierte die ihnen unterstellten Gefangenen in Fußmärschen nach Osten. Währendessen ließ der Direktor der Anstalt mehrere Dutzend wegen Hochverrats zu lebenslänglichen Strafen verurteilter polnischer Staatsbürger noch auf dem Gelände des Gefängnisses hinrichten. Die meisten Opfer waren wahrscheinlich Teil eines von der Gestapo bereits vor dem September 1939 aufgebauten Spitzelnetzes in Polen. Das erklärt, warum dieser Gefangenenmord – anders als die als Todesmärsche beschriebene Evakuierung polnischer Staatsbürger deutscher Herkunft sowie die Gefangenenmorde des NKVD im Osten Polens – nicht von der deutschen Propaganda aufgegriffen wurde und damit bis heute weitgehend unbekannt ist. Statt Św. Krzyż in eine direkte Kontinuität mit den im Juni 1941 durchgeführten sowjetischen Massenmorden in polnischen Gefängnissen zu stellen, werden im Beitrag die Kontexte herausgearbeitet, in denen das Handeln des Direktors von Św. Krzyż zu erklären ist. Der Beitrag argumentiert, dass dafür seine früheren und vielfältigen Evakuierungserfahrungen während des Ersten Weltkriegs sowie das hohe Maß an Gewalt innerhalb des Gefängnisses wichtig waren.
PRINCIPLES: As a result of the relatively low sensitivity of coronary risk charts, such as the Swiss coronary risk calculator (Arbeitsgruppe Lipide und Atherosklerose, AGLA), for detecting subjects with future myocardial infarction, the performance of arterial age (aa) as a surrogate marker for chronological age (ca) was tested. METHODS: In a practice based sample, burden of carotid plaque was obtained with ultrasound, using total plaque area (TPA). In this derivation cohort, sex-specific 5-year groups of mean TPA were calculated in subjects aged between 35 and 79 years. The arterial age formula was found by fitting an exponential function on these data. AGLAca and AGLAaa were tested externally for their ability to detect 13 myocardial infarctions in 684 subjects (validation cohort). RESULTS: The derivation cohort included 1,500 subjects (mean age 59 ± 9 years, mean TPA 54 ± 52 mm2, 5% diabetics, 43% women). Arterial age was found to be y = 5.4175e0.0426x in men and y = 4.1942e0.0392x in women. Mean 10-year AGLAca coronary risk was comparable to AGLAaa (8% ± 9% vs 9% ± 15%). Receiver operating characteristic (ROC) analysis of AGLAca and AGLAaa results showed areas under the curve of 0.65 (p = 0.041) and 0.78 (p <0.0001), respectively, (p = 0.041 for the difference = 0.13). This finding was also confirmed by a Cox proportional hazards regression model on patients' event-free survival (p = not significant for AGLAca, p = 0.0003 for AGLAaa). CONCLUSIONS: Arterial age derived from TPA could be used instead of chronological age in the AGLA coronary risk function. Further studies on the external validity and cost effectiveness of the additional ultrasound imaging study are necessary.
Felix Ackermann, Palimpsest Grodno. Nationalisierung, Nivellierung und Sowjetisierung einer mitteleuropaischen Stadt 1919-1991(Quellen und Studien, 23), Wiesbaden (Harrassowitz) 2010, 370 S., ISBN: 978-3-447-06425-5
