Pediatric Behçet’s Disease and Thromboses

2010 ◽  
Vol 38 (2) ◽  
pp. 387-390 ◽  
Author(s):  
BRICE KRUPA ◽  
ROLANDO CIMAZ ◽  
SEZA OZEN ◽  
MICHEL FISCHBACH ◽  
PIERRE COCHAT ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
International Study ◽  
Retrospective Chart Review ◽  
Lower Limbs ◽  
Disease Course ◽  
Protein C Deficiency ◽  
Behcet's Disease ◽  
Increased Risk

Objective.To describe the characteristics of a group of pediatric patients with Behçet’s disease (BD) who presented at least 1 episode of thrombosis during their disease course.Methods.We made a retrospective chart review of the clinical, biological, and radiological data of children with BD who presented at least 1 episode of either arterial or venous thrombosis. Data were extracted from both an international pediatric Behçet cohort and files referred from 7 French centers.Results.Twenty-one patients were included. Diagnosis of BD was based on the criteria of the International Study Group for BD. Main locations for thrombosis were the cerebral sinuses, in 11 patients (52.4%); and lower limbs, in 9 patients (40.9%). Recurrent episodes were observed in 4 patients (21%). Thrombophilia measurements were normal in 14 patients out of 21, while anticardiolipin antibodies were positive in 4 patients, and 2 out of 21 had protein C deficiency. One patient had lupus anticoagulant. All patients were treated with colchicine. Corticosteroids were also added for variable periods in 13 patients. Five patients out of 21 were treated with anticoagulants (heparin, then anti-vitamin K) and 3 with antiplatelets (acetylsalicylic acid).Conclusion.Thromboses are a serious complication of BD and may occur early in the disease course. The presence of thrombophilic markers could increase the risk of thrombosis in BD, but the size of our population does not allow any conclusion. An international cohort (PED-BD) is currently in place and will allow study of such cases longitudinally, as well as assessment of the elements that correlate with an increased risk of thrombosis in children with BD.

P060 Childhood uveitis: epidemiological, clinical and etiological profile, about 52 cases

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
A Sakhi ◽  
R Chahir ◽  
A Elkettani ◽  
K Bouayed
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Care Center ◽  
Tertiary Care ◽  
Diagnostic Delay ◽  
Posterior Uveitis ◽  
Disease Course ◽  
Behcet's Disease ◽  
Pediatric Uveitis

Abstract Background Childhood uveitis is a rare, often serious condition, and accounts for 2% to13% of all uveitis. It is characterized by an asymptomatic onset delaying the diagnosis and exposing a high risk of complications. The etiological profile of childhood uveitis seems to be different from that of adults. The objective of our work is to analyze the epidemiological, clinical, etiological, therapeutic and disease course characteristics of pediatric uveitis. Method This retrospective study, carried out in a tertiary care center, involved a number of 87 eyes in 52 children under the age of 16, collected over a period extending between January 2015 and September 2019 with a minimum follow-up of 6 months. Results The patients mean age was 8.15 years with a sex ratio of 1.47 and a mean consultation delay of 6.7 months. Uveitis was bilateral in 67.3% of cases. Pan-uveitis predominated, followed by intermediate, anterior and posterior uveitis in 50%, 21%, 17% and 12% of cases, respectively. Uveitis was idiopathic in 67.3% of cases. Inflammatory uveitis was the most frequent aetiology with 5 cases of Behçet's disease, 2 cases of juvenile idiopathic arthritis (JIA) and 2 cases of TINU syndrome. Ophthalmologic complications were dominated by iridocrystalline synechiae in 13.5%, retinal detachment in 10.4%, cataract in 5.4% and macular oedema in 2.7%. Corticosteroid therapy was administered to all children, combined with immunosuppressive therapy in 48% of cases (azathioprine, methotrexate). The use of anti-TNF alpha was justified in 11 patients. The disease course was marked by complete remission in 11 children and partial remission in 15 patients, 26 children presented relapses of their uveitis with satisfactory final visual acuity in 73% of cases. Conclusion Our results show the predominance of panuveitis, probably due to diagnostic delay. Idiopathic uveitis, as described in the literature, and inflammatory causes of Behçet's disease are at the top of the list of etiologies. Pediatric uveitis is a particular entity, which requires regular monitoring by a team of experts in order to avoid eye complications and amblyopia in the youngest.

Behcet’s disease and pregnancy: obstetrical and neonatal outcomes in a population-based cohort of 12 million births

2019 ◽  
Vol 47 (4) ◽  
pp. 381-387 ◽  
Author(s):  
Seohyuk Lee ◽  
Nicholas Czuzoj-Shulman ◽  
Haim Arie Abenhaim
Keyword(s):  
Venous Thromboembolism ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
The United States ◽  
Population Based ◽  
Fetal Outcomes ◽  
Behcet's Disease ◽  
Increased Risk ◽  
In Pregnancy

AbstractBackgroundBehcet’s disease (BD) is a rare, multi-systemic inflammatory disorder for which only limited and contradictory data exists in the context of pregnancy. Our objective was to estimate the prevalence of BD in pregnancy and to evaluate maternal and fetal outcomes associated with pregnant women living with BD.MethodsUsing the 1999–2013 Healthcare Cost and Utilization Project-Nationwide Inpatient Sample from the United States, we performed a population-based retrospective cohort study consisting of pregnancies that occurred during this time period. ICD-9 codes were used to identify delivery admissions to women with or without BD. Multivariate logistic regression was used to estimate the adjusted effects of BD on maternal and fetal outcomes.ResultsAmong the 12,592,676 pregnancies in our cohort, 144 were to women with BD, for an overall prevalence of 1.14 cases/100,000 births between 1999 and 2013. Over the study period, the prevalence of BD rose from 0.5 to 2.4/100,000 births. Women with BD demonstrated a two-fold greater frequency of non-delivery hospital admissions during pregnancy, and were more likely to be Caucasian, have private medical insurance, be of the upper income quartiles, and deliver at an urban teaching hospital. Women with BD were at greater risk for preterm labor and postpartum venous thromboembolism, while their newborns were more likely to be born premature.ConclusionBD-associated pregnancies are increasing in prevalence and are associated with a greater risk for adverse maternal and fetal outcomes in pregnancy. Appropriate thromboprophylaxis during pregnancy should be considered given the increased risk for venous thromboembolism.

Assessment of nasal mucociliary activity in patients with Behçet's disease

2016 ◽  
Vol 130 (4) ◽  
pp. 348-351 ◽  
Author(s):  
I Ozbay ◽  
C Kucur ◽  
F Temİzturk ◽  
Y Ozkan ◽  
C Kahraman ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Disease Duration ◽  
Mucociliary Clearance ◽  
Behçet's Disease ◽  
International Study ◽  
Clearance Time ◽  
Behcet's Disease ◽  
Control Subjects ◽  
Nasal Mucociliary Clearance

AbstractObjective:To investigate nasal mucociliary clearance in patients with Behçet's disease.Methods:Thirty patients with Behçet's disease, diagnosed on the basis of criteria defined by the International Study Group for Behçet's Disease, and 30 healthy individuals were recruited. In both groups, individuals with conditions that could affect nasal mucociliary clearance were excluded. Nasal mucociliary clearance time was evaluated using saccharin tests. The time between placement of a saccharin tablet and the tasting of the saccharin was measured and recorded as the saccharin clearance time.Results:The mean nasal mucociliary clearance time was significantly longer in the Behçet's disease patients than in the control subjects (p < 0.001). Furthermore, there was a positive correlation between Behçet's disease duration and nasal mucociliary clearance time (p < 0.001, r = 0.882).Conclusion:The results suggest that nasal mucociliary clearance time is longer in Behçet's disease patients than in control subjects, and is positively correlated with disease duration.

Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis

2007 ◽  
Vol 36 (1) ◽  
pp. 48-52 ◽  
Author(s):  
E. Rabinovich ◽  
Y. Shinar ◽  
M. Leiba ◽  
M. Ehrenfeld ◽  
P. Langevitz ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease ◽  
Increased Risk

Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

2019 ◽  
Vol 18 (04) ◽  
pp. 175-181
Author(s):  
Yaşar B. Turgut ◽  
Ayşe F. Tosun ◽  
Beste K. Yüzbaşı ◽  
Hasan Tunca
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
International Study ◽  
Skin Lesions ◽  
Disease Classification ◽  
Behcet's Disease ◽  
Male Predominance ◽  
Pediatric Age Group ◽  
Pathergy Test

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

VALIDATION OF THE INTERNATIONAL STUDY GROUP CRITERIA FOR BEHÇET'S DISEASE

Rheumatology ◽  
1994 ◽  
Vol 33 (2) ◽  
pp. 115-117 ◽  
Author(s):  
T. W. O'NEILL ◽  
A. S. RIGBY ◽  
A. J. SILMAN ◽  
C. BARNES
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
International Study ◽  
Study Group ◽  
Behcet's Disease ◽  
International Study Group
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