Clinical outcome in the Marfan syndrome with ascending aortic dilatation followed annually by echocardiography

Kornbluth, Murray; Schnittger, Ingela; Eyngorina, Irina; Gasner, Cheryll; Liang, David

doi:10.1016/s0002-9149(99)00430-0

Cited by 31 publications

(17 citation statements)

References 9 publications

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“…The multiple factors predisposing to aortic dilatation, such as medial degeneration, atherosclerosis, hypertension, aortic valve disease, could differently combine in the determination of each clinico-anatomical picture. For instance, the typical anatomical presentation of aortic aneurysms in Marfan patients is root enlargement [17,18] and this relates to the pattern of normal distribution of fibrillin-rich elastic fibers, which is maximal at the aortic sinuses and then decreases gradually into the distal segments [19]. In the present study, the exclusion of patients with inherited elastic tissue disorders could account for the low prevalence of root type aneurysms-about one-fifth of the study population.…”

Section: Discussionmentioning

confidence: 62%

Echocardiographic anatomy of ascending aorta dilatation: Correlations with aortic valve morphology and function

Corte

Romano

Tizzano

et al. 2006

International Journal of Cardiology

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Section: Discussionmentioning

confidence: 62%

Echocardiographic anatomy of ascending aorta dilatation: Correlations with aortic valve morphology and function

Corte

Romano

Tizzano

et al. 2006

International Journal of Cardiology

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“…Dilation of the aortic root is the leading cause of AV insufficiency in MaSy. In annuloaortic ectasia, severe AR occurs and may progress to aortic root dissection or rupture 12. On mid‐systolic images, the cusps of the AV appear tethered instead of demonstrating their normal arching appearance; the tethered appearance results from the dilation of the Valsalva sinus.…”

Section: Connective Tissue Diseasementioning

confidence: 99%

Peptidergic clock neurons in Drosophila: Ion transport peptide and short neuropeptide F in subsets of dorsal and ventral lateral neurons

Johard

Yoishii

Dircksen

et al. 2009

J of Comparative Neurology

197

209

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About 150 clock neurons are clustered in different groups in the brain of Drosophila. Among these clock neurons, some pigment-dispersing factor (PDF)-positive and PDF-negative lateral neurons (LNs) are principal oscillators responsible for bouts of activity in the morning and evening, respectively. The full complement of neurotransmitters in these morning and evening oscillators is not known. By using a screen for candidate neuromediators in clock neurons, we discovered ion transport peptide (ITP) and short neuropeptide F (sNPF) as novel neuropeptides in subpopulations of dorsal (LN(d)s) and ventral (s-LN(v)s) LNs. Among the six LN(d)s, ITP was found in one that coexpresses long neuropeptide F (NPF) and cryptochrome. We detected sNPF in two LN(d)s that also express cryptochrome; these cells are distinct from three LN(d)s expressing NPF. Thus, we have identified neuropeptides in five of the six LN(d)s. The three LN(d)s expressing cryptochrome, with either ITP or sNPF, are the only ones with additional projections to the accessory medulla. Among the five s-LN(v)s in the adult brain, ITP was detected in the fifth neuron that is devoid of PDF and sNPF in the four neurons that also express PDF. By using a choline acetyltransferase (Cha) Gal4, we detected Cha expression in the two sNPF producing LN(d)s and in the fifth s-LN(v). In the larval brain, two of the four PDF-producing s-LN(v)s coexpress sNPF. Our findings emphasize that the LN(d)s are heterogeneous both anatomically and with respect to content of neuropeptides, cryptochrome, and other markers and suggest diverse functions of these neurons.

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“…The risk of type A dissection is proportional to the overall size of the proximal aorta, in Marfan patients as well as in other causes of noninflammatory proximal aortic aneurysms. 39,41,43,78 Mortality and event rates (aortic dissection, need for surgical intervention) are significantly higher in adults with Marfan syndrome when the absolute aortic root dimension is Ͼ5.0 cm, 78 has an aortic ratio Ͼ3, 43 or has an aortic size index of Ն4.25 cm/m 2 . 41 Rate of aortic dilatation is an additional risk factor for dissection or death; change in aortic ratio of Ͼ5% per year portends a poor outcome.…”

Section: Timing Of Aortic Surgical Intervention In Marfan Syndromementioning

confidence: 99%

Medical Management of Marfan Syndrome

2008

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Clinical outcome in the Marfan syndrome with ascending aortic dilatation followed annually by echocardiography

Cited by 31 publications

References 9 publications

Echocardiographic anatomy of ascending aorta dilatation: Correlations with aortic valve morphology and function

Echocardiographic anatomy of ascending aorta dilatation: Correlations with aortic valve morphology and function

Peptidergic clock neurons in Drosophila: Ion transport peptide and short neuropeptide F in subsets of dorsal and ventral lateral neurons

Medical Management of Marfan Syndrome

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