Loeys-Dietz syndrome: Intermediate-term outcomes of medically and surgically managed patients
نویسندگان
چکیده
منابع مشابه
Neuroradiologic Manifestations of Loeys - Dietz Syndrome Type
V.J. Rodrigues S. Elsayed B.L. Loeys H.C. Dietz D.M. Yousem BACKGROUND AND PURPOSE: Loeys-Dietz syndrome (LDS) is a recently described entity that has the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate. Its neuroradiologic manifestations have not been well delineated. We sought to describe the neuroradiologic features of LDS and to assess the manifest...
متن کاملPrenatal diagnosis of Loeys-Dietz syndrome.
Introduction ▼ Loeys–Dietz is an autosomal dominant aortic aneurysm syndrome with widespread systemic involvement. The disease is characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate and is caused by heterozygous mutations in the genes encoding transforming growth factor β receptors 1 and 2 (TGFBR1 and TGFBR2, respectively) (B.L. Loeys ...
متن کاملMultiple facial milia in patients with Loeys-Dietz syndrome.
BACKGROUND Loeys-Dietz syndrome (LDS) results from mutations in the TGFBR1 or TGFBR2 genes and is known to cause aggressive cardiovascular disease, including aortic aneurysms and dissections at an early age. Currently, craniofacial, skeletal, and cardiovascular findings play an important role in early recognition of the disease. While many patients do have recognizable cutaneous features of LDS...
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Loeys-Dietz syndrome (LDS) is a rare autosomal dominant disease related to genetic mutations in receptors for the cytokine transforming growth factor-receptor type 1 (TGFB-R1) or 2 gene (TGFB-R2) on the cell surface. LDS results in abnormal protein synthesis and dysfunctional connective tissue, which can result in unique cardiovascular anesthesia challenges related to perioperative management. ...
متن کاملNeurovascular Manifestation of Loeys-Dietz Syndrome: A Case Report
A 5-year-old boy was referred to our hospital for evaluation of headache, tortuous intracranial arteries and vertebral arteries found up on brain magnetic resonance angiography. The patient’s family history was unremarkable for any genetic disease. He was born at 39 weeks of gestation by spontaneous vaginal delivery without complications. His birth weight was 3,580 gm. At 3 years Neurovascular ...
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ژورنال
عنوان ژورنال: The Journal of Thoracic and Cardiovascular Surgery
سال: 2019
ISSN: 0022-5223
DOI: 10.1016/j.jtcvs.2018.03.172