Childhood Behçet's disease: clinical features and comparison with adult-onset disease.

This study was done to evaluate the frequency, intensity, and specificity of a positive pathergy reaction (PR) in Behcet's disease (BD) patients, to clarify an association between the PR and the clinical features or disease severity, and to assess whether patients with pustule formation at the venous puncture site (PFVPS) without positive PR could be regarded as a positive reaction. The PR was tested in 64 BD patients, 74 disease controls, and 20 healthy controls. Venous PR was performed in 8 BD patients with PFVPS. Follow-up PR was done in 14 patients with positive reaction during inactive phase. The PR was positive in 35.9% of BD patients, in 1 patient among disease controls, and in none of healthy controls. The pustule formation was observed in one BD patient. There was no statistical significance between positive PR and the clinical variables. The mean clinical activity score of BD patients with positive PR was similar to patients with negative reaction. Venous PR was positive in 7 patients. The follow-up PR was positive in 2 patients during inactive phase. Conclusively the positive PR appeared to be specific for BD, and was not associated with the clinical variables or disease severity, but was usually found during active phase in cases with positive reaction. The PFVPS in patients with negative PR might be considered to be positive.

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“…This score was established with reference to the study of Yazici et al (3) and in view of the morbidity and the disease severity of the BD (12).…”

Section: The Clinical Significance Of a Pathergy Reaction In Patientsmentioning

confidence: 99%

The Clinical Significance of a Pathergy Reaction in Patients with Behcet's Disease

Chang

Cheon

2002

J Korean Med Sci

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“…All the patients analyzed in their study (6) fulfilled the conventional criteria ( 1), and patients with childhood-onset BDin the general population may not be immediately diagnosed due to incompleteness of criteria. By evaluating the severity scales in childhood-and adult-onset disease, it was concluded that the children with BDhad a significantly lower activity index of disease compared with adults (6).…”

Section: Discussionmentioning

confidence: 99%

Fatal Thoracic Aortic Aneurysm in a Patient with Childhood-onset Vasculo-Behcet's Disease: An Autopsy Report.

2001

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A 33-year-old mandied following sudden unexpected rupture of a saccular thoracic aortic aneurysm.Thepatient had been treated as suspicious Beh^et's disease (BD) for 9 years, however, the medication was discontinued at the age of 24. Autopsy disclosed multiple aneurysms at the descending thoracic and abdominal aorta. Histopathologically, infiltration of inflammatory cells and destruction with loss of elastic and muscle fibers of the aortic medial wall were seen. Taking the clinical course into account, all the postmortem findings were compatible with those of vasculo-BD. It is important that medical follow-up should be implemented even for childhood-onset patients who do not satisfy criteria of BD.

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“…The clinical features of juvenile BD in diferent series are seen in Table 3 [40][41][42][44][45][46][47][48].…”

Section: Age and Gendermentioning

confidence: 99%

The Epidemiology of Behçet’s Disease

Oğuz¹,

Hizli²,

Gönül³

2017

Behcet's Disease

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Behçet's disease (BD), a chronic vasculitis afecting any type of the blood vessels, was irst described by a Turkish dermatologist Hulusi Behçet. Although it has a worldwide distribution, it is commonly seen in the Silk Road countries around the Mediterranean Sea. The country in which the disease is most commonly seen is Turkey with a prevalence of 20-602/100,000. The disease most appears between the second and fourth decades of the life. BD afects both genders equally. However, the gender distribution may difer among diferent regions.

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Childhood Behçet's disease: clinical features and comparison with adult-onset disease.

Abstract: Our results point to a similar systemic expression of BD in children and adults; however, the disease seems to run a less severe course in children.

Cited by 144 publications

References 4 publications

The Clinical Significance of a Pathergy Reaction in Patients with Behcet's Disease

The Clinical Significance of a Pathergy Reaction in Patients with Behcet's Disease

Fatal Thoracic Aortic Aneurysm in a Patient with Childhood-onset Vasculo-Behcet's Disease: An Autopsy Report.

The Epidemiology of Behçet’s Disease

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