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  • Title: Juvenile Behçet's disease: a tertiary center experience.
    Author: Ekici Tekin Z, Çelikel E, Aydin F, Kurt T, Sezer M, Tekgöz N, Karagöl C, Coşkun S, Kaplan MM, Çelikel Acar B.
    Journal: Clin Rheumatol; 2022 Jan; 41(1):187-194. PubMed ID: 34476647.
    Abstract:
    OBJECTIVES: Juvenile Behçet's disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behçet's disease in a tertiary center. METHODS: The retrospective study included patients who were diagnosed Behçet's disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded. RESULTS: Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone. CONCLUSION: In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death. Key Points • In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality. • It is important noticing and managing jBD in early phase in order to prevent the devastating results. • The awareness of jBD provides timely treatment of patients. • The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.
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