Ab0958 pediatric Behcet’s Disease With Sinus Venous Thrombosis: Three Center Experience From Turkey

Açarı, Ceyhun; Başaran, Özge; Sag, Erdal; Oğuz, Kader K.; Bilginer, Yelda; Ünsal, Erbil; Özen, Seza

doi:10.1136/annrheumdis-2019-eular.4574

Cited by 5 publications

(14 citation statements)

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“…The use of anti-coagulant agents is controversial, because patients with BD may also have pulmonary or other aneurysms, associated with an increased risk of bleeding (18). However, in recent studies, no increased risk of bleeding was reported in patients who received anticoagulation (16,19). Although our study presents a small series, six patients were diagnosed with BD; three of them had diagnosis after presentation with CSVT.…”

Section: Discussionmentioning

confidence: 64%

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Clinical and Radiological Findings in Children with Cerebral Sinovenous Thrombosis

Genç¹,

Kalın²,

Kutlubay³

et al. 2021

Turkish Journal of Pediatric Disease

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Objective: Cerebral sinovenous thrombosis (CSVT) is increasingly recognized in children with advanced use of neuroimaging techniques. In this study, we aimed to present clinical and radiologic features of CSVT in children. Material and Methods:Children aged 1 month to 18 years old diagnosed with CSVT were retrospectively analyzed. Demographic and clinical data of patients including age at diagnosis, sex, presenting symptoms and signs, risk factors for thrombosis, treatment and prognosis were collected.Results: Twelve children were included in the study, 8 of them were male. Headache (n=7), seizures (n=5) and visual impairment (n=4) were the most common presenting symptoms. The superficial venous system was involved in all patients, in five patients deep venous system was also involved. Parenchymal involvement was present in three patients. Two children had isolated cortical vein thrombosis. All patients had underlying risk factors. Five children had recent infections, six children were diagnosed with Behçet's disease, three with mastoiditis. High lipoprotein (a) level was found

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Section: Discussionmentioning

confidence: 64%

“…In a retrospective study by Ünver et al (15); 3 out of 11 CSVT patients were diagnosed with BD. Demir et al (16), evaluated 12 children with CSVT and BD; half of them presented with CSVT and diagnosed with BD after neurologic involvement. Subacute presentation is common in BD, with severe headache progressing in weeks (11).…”

Section: Discussionmentioning

confidence: 99%

Clinical and Radiological Findings in Children with Cerebral Sinovenous Thrombosis

Genç¹,

Kalın²,

Kutlubay³

et al. 2021

Turkish Journal of Pediatric Disease

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“…Arterial complications with thrombosis and aneurysms occurred in six patients, and pulmonary arteries were the most frequently affected, resulting in haemoptysis [ 10 ]. In another study involving 12 paediatric cases with central venous sinus thrombosis (CVST), headache was universal at presentation (100%), followed by vomiting (25%) and blurred vision (16.7%) [ 58 ]. The transverse venous sinus was affected in the majority (75%), followed by the superior sagittal sinus [ 58 ].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…In another study involving 12 paediatric cases with central venous sinus thrombosis (CVST), headache was universal at presentation (100%), followed by vomiting (25%) and blurred vision (16.7%) [ 58 ]. The transverse venous sinus was affected in the majority (75%), followed by the superior sagittal sinus [ 58 ]. Of 12 patients, 4 had another venous thrombosis in addition to CVST [ 58 ].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…The transverse venous sinus was affected in the majority (75%), followed by the superior sagittal sinus [ 58 ]. Of 12 patients, 4 had another venous thrombosis in addition to CVST [ 58 ]. Likewise, in a cohort of 26 paediatric neuro-BD cases, CVST was confirmed in 23/26, the majority of whom presented with headaches [ 59 ].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

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Paediatric Behçet’s Disease: A Comprehensive Review with an Emphasis on Monogenic Mimics

Cınar

Romano

Güzel³

et al. 2022

JCM

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Behçet’s disease (BD) is a polygenic condition with a complex immunopathogenetic background and challenging diagnostic and therapeutic concepts. Advances in genomic medicine have provided intriguing insights into disease pathogenesis over the last decade, especially into monogenic mimics of BD. Although a rare condition, paediatric BD should be considered an important differential diagnosis, especially in cases with similar phenotypes. Emerging reports of monogenic mimics have indicated the importance of genetic testing, particularly for those with early-onset, atypical features and familial aggregation. Treatment options ought to be evaluated in a multidisciplinary setting, given the complexity and diverse organ involvement. Owing to the rarity of the condition, there is a paucity of paediatric trials; thus, international collaboration is warranted to provide consensus recommendations for the management of children and young people. Herein, we summarise the current knowledge of the clinical presentation, immunopathogenetic associations and disease mechanisms in patients with paediatric BD and BD-related phenotypes, with particular emphasis on recently identified monogenic mimics.

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Factors Associated with the Development of Thrombosis in Pediatric Behçet Disease

Güngörer¹,

Polat²,

Çelikel³

et al. 2022

J Clin Rheumatol

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ObjectiveThe present study aimed to compare the demographic, clinical, and laboratory characteristics of patients with pediatric Behçet disease (BD) with and without thrombosis to elucidate the factors that may contribute to the development of thrombosis.MethodsThis observational, descriptive, medical records review study included patients with BD (n = 85) who were diagnosed at age younger than 16 years at our clinic between 2010 and 2022. The demographic, clinical, and available laboratory data of patients with and without thrombosis were compared. The potential risk factors for the development of thrombosis were evaluated with multivariable logistic regression analysis.ResultsCentral venous sinus thrombosis was the most common type of thrombosis. Thrombosis was significantly more common in male patients (p = 0.002), and regression analysis revealed that being male was a risk factor for developing thrombosis. Genital ulcers were less common in patients with thromboses. Patients with thrombosis had higher erythrocyte sedimentation rates, C-reactive protein, leukocyte, and neutrophil counts, as well as antinuclear antibody positivity. In contrast, mean platelet volume and lymphocyte counts were significantly lower in patients with thrombosis. According to the logistic regression analysis, erythrocyte sedimentation rate value >17 mm/h was a risk factor for developing thrombosis (odds ratio, 1; confidence interval, 1.1–1.8; p = 0.012).ConclusionsMale sex has been associated with an increased risk of thrombosis in children with BD. Inflammatory parameters may serve as predictive factors for thrombosis in pediatric BD.

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Ab0958 pediatric Behcet’s Disease With Sinus Venous Thrombosis: Three Center Experience From Turkey

Cited by 5 publications

References 0 publications

Clinical and Radiological Findings in Children with Cerebral Sinovenous Thrombosis

Clinical and Radiological Findings in Children with Cerebral Sinovenous Thrombosis

Paediatric Behçet’s Disease: A Comprehensive Review with an Emphasis on Monogenic Mimics

Factors Associated with the Development of Thrombosis in Pediatric Behçet Disease

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