کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4167756 1607545 2007 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Long-Term Follow-Up in 12 Children with Pulmonary Arteriovenous Malformations: Confirmation of Hereditary Hemorrhagic Telangiectasia in all Cases
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی پریناتولوژی (پزشکی مادر و جنین)، طب اطفال و بهداشت کودک
پیش نمایش صفحه اول مقاله
Long-Term Follow-Up in 12 Children with Pulmonary Arteriovenous Malformations: Confirmation of Hereditary Hemorrhagic Telangiectasia in all Cases
چکیده انگلیسی

ObjectiveTo assess whether pulmonary arteriovenous malformation (PAVM) is associated with hereditary hemorrhagic telangiectasia (HHT).Study designThis study was a review of 12 children (sex ratio = 1) including family history, mutation analysis, and long-term follow-up.ResultsFive children were under age 3 years when PAVM was diagnosed. Presentations included pulmonary symptoms (n = 8), cerebral abscess (n = 2), and transient ischemic attack (TIA) (n = 1); 1 patient was asymptomatic. Nine of the 12 children (75%) had a family history of PAVM. The diagnosis of HHT was confirmed in all cases. A mutation in ENG was found in 9 of the 10 children available for testing. No mutation in ACVRL1 was found. During long-term follow-up (mean, 16 years), the following complications occurred: TIA (n = 2), hemoptysis (n = 2), and cerebral abscess (n = 2). Nine children experienced recurrence of PAVM. The children with no recurrence were those without a family history of PAVM.ConclusionsThe diagnosis of HHT should be considered in a child with an apparently isolated PAVM. Because serious complications may occur at any age, we recommend screening for PAVM and long-term follow-up in children from families with HHT, especially those with an ENG mutation.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: The Journal of Pediatrics - Volume 151, Issue 3, September 2007, Pages 299–306
نویسندگان
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