دورية أكاديمية
Screening children for pulmonary arteriovenous malformations: Evaluation of 18 years of experience.
| العنوان: | Screening children for pulmonary arteriovenous malformations: Evaluation of 18 years of experience. |
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| المؤلفون: | Hosman AE; Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands., de Gussem EM; Department of Internal Medicine, University of Manitoba, Winnipeg, Canada., Balemans WAF; Department of Pediatrics, St Antonius Hospital, Nieuwegein, The Netherlands., Gauthier A; Department of Pulmonology, Centre Hospitalier de l'Université de Montréal, Montréal, Canada., Westermann CJJ; Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands., Snijder RJ; Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands., Post MC; Department of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands., Mager JJ; Department of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands. |
| المصدر: | Pediatric pulmonology [Pediatr Pulmonol] 2017 Sep; Vol. 52 (9), pp. 1206-1211. Date of Electronic Publication: 2017 Apr 13. |
| نوع المنشور: | Journal Article; Observational Study |
| اللغة: | English |
| بيانات الدورية: | Publisher: Wiley-Liss Country of Publication: United States NLM ID: 8510590 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1099-0496 (Electronic) Linking ISSN: 10990496 NLM ISO Abbreviation: Pediatr Pulmonol Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: <2005-> : Hoboken, NJ : Wiley-Liss Original Publication: [Philadelphia, PA] : W.B. Saunders, [c1985- |
| مواضيع طبية MeSH: | Arteriovenous Fistula/*diagnosis , Arteriovenous Malformations/*diagnosis , Pulmonary Artery/*abnormalities , Pulmonary Veins/*abnormalities , Telangiectasia, Hereditary Hemorrhagic/*diagnosis, Adolescent ; Arteriovenous Fistula/diagnostic imaging ; Arteriovenous Fistula/therapy ; Arteriovenous Malformations/diagnostic imaging ; Arteriovenous Malformations/therapy ; Child ; Child, Preschool ; Echocardiography ; Embolization, Therapeutic ; Humans ; Infant ; Mass Screening ; Oximetry ; Pulmonary Artery/diagnostic imaging ; Pulmonary Veins/diagnostic imaging ; Radiography ; Telangiectasia, Hereditary Hemorrhagic/diagnostic imaging ; Telangiectasia, Hereditary Hemorrhagic/therapy |
| مستخلص: | Background: Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disease with multi-systemic vascular dysplasia. Early diagnosis through screening is important to prevent serious complications. How best to screen children of affected parents for pulmonary arteriovenous malformations (PAVMs) is often subject to debate. Transthoracic contrast echocardiogram (TTCE) is considered optimal in screening for PAVMs in adults. Guidelines for the screening of children are not specific, reflecting the lack of scientific evidence on the best method to use. Objective: Aims of this study are (i) to evaluate our current screening method, consisting of history, physical examination, pulse oximetry, and chest radiography and (ii) to assess whether postponing more invasive screening for PAVMs until adulthood is safe. Methods: This is a prospective observational cohort study using a patient database. Results: Over a period of 18 years (mean follow-up 9.21 years, SD 4.72 years), 436 children from HHT families were screened consecutively. A total of 175/436 (40%) children had a diagnosis of HHT. PAVMs were detected in 39/175 (22%) children, 33/39 requiring treatment by embolotherapy. None of the screened children suffered any PAVM-associated complications with this screening method. Conclusion: This study shows that a conservative screening method during childhood is sufficient to detect large PAVMs and protect children with HHT for PAVM-related complications. Postponing TTCE and subsequent chest CT scanning until adulthood to detect any smaller PAVMs does not appear to be associated with major risk. (© 2017 Wiley Periodicals, Inc.) |
| فهرسة مساهمة: | Keywords: ACVRL1; endoglin; hereditary hemorrhagic telangiectasia; pulmonary arteriovenous malformation; vascular disease |
| SCR Disease Name: | Pulmonary Arteriovenous Fistulas |
| تواريخ الأحداث: | Date Created: 20170414 Date Completed: 20180221 Latest Revision: 20181202 |
| رمز التحديث: | 20221213 |
| DOI: | 10.1002/ppul.23704 |
| PMID: | 28407366 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 1099-0496 |
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| DOI: | 10.1002/ppul.23704 |