Acute Aortic Valvular Regurgitation Secondary to Avulsion of Aortic Valve Commissure in a Patient with Pseudoxanthoma Elasticum.

Hirata, Kazuhito; Kakazu, Masanori; Wake, Minoru; Kyushima, Masahiro; Asato, Hiroaki; Tengan, Toshiho; Mototake, Hidemitsu; Henzan, Eisei

doi:10.2169/internalmedicine.39.940

Cited by 10 publications

(16 citation statements)

References 10 publications

(5 reference statements)

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“…It should be noted that PXE associated with aortic regurgitation has been described previously in only one case report 6 and it was thus difficult to determine whether the aortic regurgitation of case 4 was due to PXE. However, even when we excluded this case from the CVD-positive group, the presence of CVD was still significantly associated with a high distribution score (P = 0.013) and the presence of mucosal lesions (P = 0.003).…”

Section: Case Reportsmentioning

confidence: 89%

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Relationship between the distribution of pseudoxanthoma elasticum skin and mucous membrane lesions and cardiovascular involvement

Utani¹,

Tanioka²,

Yamamoto³

et al. 2010

The Journal of Dermatology

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Pseudoxanthoma elasticum (PXE) primarily affects organs that are abundant in elastic fibers, such as the skin, eye and blood vessels, and may eventually cause loss of vision or cardiovascular disease (CVD). Because CVD is a potentially life-threatening complication, its early detection is important for improving the quality of life of PXE patients. To determine the relationship between the distribution of skin and mucous membrane lesions and the prevalence of CVD in patients with PXE, we examined 14 PXE cases who presented between 2004 and 2007. All patients had angioid streaks (AS) and positive pathological findings. The skin lesions in PXE patients are distributed discontinuously and thus the degrees of skin involvement were assessed by determining the presence or absence of PXE skin and mucous membrane lesions in six sites (oral mucosa, neck, periumbilical region, cubital fossa, axillae and inguinal regions). Each site was given a binary score (i.e. present = 1, absent = 0) irrespective of severity and the scores were summed to yield a total distribution score (potential range of 0-6). Four cases had PXE-associated CVD. Their mean distribution score was 5.7, which was significantly higher than the score of the cases without CVD (1.8) (P = 0.0049). There was also significant correlation between the high distribution score (P = 0.0053) as well as CVD (P = 0.029) with the maximum width of AS. A higher distribution score and the presence of oral mucosal lesions were associated with CVD. This scoring method may be useful for predicting the presence of CVD in PXE patients.

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Section: Case Reportsmentioning

confidence: 89%

“…In 2006, Ringpfeil proposed simple criteria that excluded the autosomal dominant type of PXE, in which a patient is diagnosed as having minimally affected PXE when he or she exhibits pathological changes and angioid streaks. 6 According to these criteria, all of the PXE patients in this study can be diagnosed as having minimally affected PXE.…”

Section: Diagnosismentioning

confidence: 92%

Relationship between the distribution of pseudoxanthoma elasticum skin and mucous membrane lesions and cardiovascular involvement

Utani¹,

Tanioka²,

Yamamoto³

et al. 2010

The Journal of Dermatology

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“…In some cases, echocardiography demonstrated marked MVP of the both leaflets and vegetation‐like thickening of the anterior leaflet of the MV. Only 1 case was reported with right‐sided cardiac involvement in osteogenesis imperfecta 15–17…”

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

191

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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“…Rupture of fenestrated aortic cusps has also been implicated to cause acute, severe AI . Connective tissue abnormalities that weaken aortic cusp tissue have also been implicated in AV spontaneous rupture, such as was reported by Hirata et al in a patient with confirmed pseudoxanthoma elasticum …”

Section: Patient Profilementioning

confidence: 73%

“…Aortic valve (AV) rupture is a rare, but well described event . It occurs most commonly in the setting of blunt chest trauma but can also occur spontaneously in the setting of structurally or molecularly abnormal valves.…”

Section: Patient Profilementioning

confidence: 99%

Aortic Valve Rupture During Isometric Muscle Contraction

2015

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Acute Aortic Valvular Regurgitation Secondary to Avulsion of Aortic Valve Commissure in a Patient with Pseudoxanthoma Elasticum.

Cited by 10 publications

References 10 publications

Relationship between the distribution of pseudoxanthoma elasticum skin and mucous membrane lesions and cardiovascular involvement

Relationship between the distribution of pseudoxanthoma elasticum skin and mucous membrane lesions and cardiovascular involvement

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

Aortic Valve Rupture During Isometric Muscle Contraction

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