Dataset Information

Changing pattern of clinical manifestations of Behcet's disease in Tunisia: comparison between two decades.

ABSTRACT: Objectives:To investigate the changes over time in extraocular and ocular manifestations of Behçet's disease (BD) in Tunisian patients. Material and methods:Retrospective study of 246 patients divided into two groups: group 1 (147 patients examined from 1995 to 2005) and group 2 (99 patients examined from 2006 to 2017). Results:Active or scarred genital ulcers observed by physician at presentation were significantly less frequent in group 2 (47.2% vs. 29.6%; p = 0.007), as were articular involvement (50.3% vs. 34.7%; p = 0.016) and erythema nodosum (18.4% vs. 8.1%; p = 0.024). One hundred-seven patients (43.5%) developed ocular manifestations during the 23-year study period. Intermediate uveitis was significantly more frequent in group 2 than in group 1 (11.7% vs. 28.4%; p = 0.003), and posterior uveitis less frequent in group 2 than in group 1 (34.2% vs. 19.7%; p = 0.016). Patients from group 2 were more likely to have macular edema (19.8% vs. 45.6%; p = 0.001). However, better visual prognosis, with a lower rate of legal blindness, was noted in group 2. Conclusions:Changes over time included a decrease in the rate of articular involvement and cutaneous involvement. There was an increase in the rate of intermediate uveitis and a decrease in the rate of posterior uveitis over time. Despite an increase in the rate of macular edema, there was an improvement in visual prognosis, with less legal blindness over time.

SUBMITTER: Ksiaa I

PROVIDER: S-EPMC7249524 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Changing pattern of clinical manifestations of Behçet's disease in Tunisia: comparison between two decades.

Ksiaa Imen I Kechida Melek M Abroug Nesrine N Bchir Sabri S Attia Sonia S Khochtali Sana S Khairallah Moncef M

Reumatologia 20200430 2

<h4>Objectives</h4>To investigate the changes over time in extraocular and ocular manifestations of Behçet's disease (BD) in Tunisian patients.<h4>Material and methods</h4>Retrospective study of 246 patients divided into two groups: group 1 (147 patients examined from 1995 to 2005) and group 2 (99 patients examined from 2006 to 2017).<h4>Results</h4>Active or scarred genital ulcers observed by physician at presentation were significantly less frequent in group 2 (47.2% vs. 29.6%; <i>p</i> = 0.00 ...[more]

PMID: 32476681

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Project description:BackgroundGaucher disease (GD) is a lysosomal storage disorder characterized by deficient glucocerebrosidase activity that results from biallelic mutations in the GBA1 gene. Its phenotypic variability allows GD to be classified into 3 subtypes based on the presence and extent of neurological manifestations. Enzyme replacement therapy (ERT) has been available for all patients with GD in Taiwan since 1998. Newborn screening (NBS) for GD has been available since 2015. This study attempted to unveil the clinical features of patients diagnosed with GD during different eras in Taiwan.Materials and methodsData from the health records of two tertiary hospitals responsible for two-thirds of the patients with GD in Taiwan were used. The study population included all patients identified as having GD between 1998, and April 2022, in these two hospitals for review. A total of 42 individuals were included, six of whom were diagnosed by NBS.ResultsOur cohort presented a higher proportion of GD3 individuals, both by clinical suspicion and by NBS diagnosis, than that reported worldwide. The major subtypes that were recognized following NBS diagnosis were GD2 and GD3. The majority of GD patients carry at least one p.Leu483Pro variant. The 5-year survival rates were 0% for GD2 patients and 100% for patients with other subtypes. Patients diagnosed during the post-NBS era were free of symptoms on initial presentation, except for those with the GD2 subtype. For those diagnosed earlier, ERT was shown to be effective in terms of improved hemograms and prevented bone crises. However, the neurological symptoms in GD3 patients progressed despite ERT intervention.ConclusionERT is essential in reversing the hematological presentations and preventing the skeletal complications of GD. Timely diagnosis of GD with NBS allows for early intervention with ERT to prevent disease progression and complications. However, the need for effective intervention for neurological dysfunction remains unmet.

Project description:ObjectivesUsing the cardiac surgery database is of high importance in referral centers and can lead to a better quality of care for patients. Tehran Heart Center (THC) is a cardiovascular referral center that was inaugurated in 2001. In this report, we aimed to present the third report of trends in patients' cardiovascular risk factors and surgical procedures from 2002 to 2021 that have been gathered for all THC patients.MethodsThis serial cross-sectional study was conducted at Tehran Heart Center from 2002 to 2021. All patients undergoing cardiac surgeries were eligible to enter the study (N = 63,974). Those with miscellaneous types of surgeries were excluded (N = 9556). The distribution of cardiac surgeries (including isolated coronary artery bypass graft (CABG), isolated valve, and CABG + valve surgeries) and their respective in-hospital mortality were recorded. Furthermore, 20-year trends in the prevalence of various cardiovascular risk factors (CVRFs) among the following groups were evaluated: a) isolated CABG, b) aortic valve replacement/repair for aortic stenosis (AS/AVR/r), and c) isolated other valve surgeries (IVS).ResultsA total of 54,418 patients (male: 70.7%, age: 62.7 ± 10.8 years) comprised the final study population, with 84.5% prevalence of isolated CABG. Overall, the AS/AVR/r group was in between the CABG and IVS groups concerning CVRFs distribution. Excluding some exceptions for the AS/AVR/r group (in which the small sample size (N = 909) precluded observing a clear trend), all studied CVRFs demonstrated an overall rising trend from 2002 to 2021 in all three groups. Regarding in-hospital mortality, the highest rate was recorded as 4.0% in 2020, while the lowest rate was 2.0% in 2001.ConclusionsIsolated CABG remained the most frequent procedure in THC. Notable, increasing trends in CVRFs were observed during this 20-year period and across various types of cardiac surgeries, which highlights the clinical and policy-making implications of our findings.

Dataset Information

Changing pattern of clinical manifestations of Behcet's disease in Tunisia: comparison between two decades.

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Changing pattern of clinical manifestations of Behçet's disease in Tunisia: comparison between two decades.

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