1/12. coronary artery disease in an 18 year old with pseudoxanthoma elasticum: successful surgical therapy.An 18 year old girl with pseudoxanthoma elasticum, a 7 year history of angina pectoris and evidence of an old anteroseptal myocardial infarction was found on coronary angiography to have three vessel coronary artery disease. A triple coronary artery-saphenous vein bypass graft was performed, and she has been asymptomatic for 1 year. Histologic examination of a segment of the right coronary artery revealed changes consistent with the vascular lesion of pseudoxanthoma elasticum.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/12. Sudden cardiac death owing to pseudoxanthoma elasticum: a case report.A 26-year-old woman collapsed and died suddenly while dancing. autopsy findings included the cutaneous lesions of pseudoxanthoma elasticum (PXE), a rare genetic disease with autosomal dominant and recessive inheritance patterns. Pathologic findings of PXE (degenerated elastic fibers) were seen in the stenotic epicardial coronary arteries, the intramyocardial arterioles, the subendocardium, the mitral valve, and the blood vessels of other viscera. The mitral valve was slightly myxoid. Intramyocardial arteriolar involvement has not been previously described in PXE. The other cardiac findings have only been described in a few cases. Although mitral valve prolapse in PXE has been shown echocardiographically, it is unclear whether or not the mitral valve findings in this case represent the substrate for this condition. It is important that autopsy pathologists search carefully for the pathognomonic skin lesions of PXE in cases of sudden death associated with coronary disease, mitral valve prolapse, or endocardial lesions. Recognition of this disease is essential for proper genetic counseling of surviving family members.- - - - - - - - - - ranking = 10.044593808933keywords = blood vessel, vessel (Clic here for more details about this article) |
3/12. pseudoxanthoma elasticum diagnosed 25 years after the onset of cardiovascular disease.A 63-year-old man who had experienced cerebral infarction and myocardial infarction at an early age, and repeatedly underwent coronary angioplasty was admitted to our hospital for cardiac evaluation. A coronary angiography showed complex multi-vessel disease with significant stenosis in all major vessels and coronary-artery bypass grafts. A funduscopic examination to evaluate hypertensive and diabetic changes revealed angioid streaks. Therefore, a skin biopsy was performed despite the absence of characteristic skin lesions. Histopathologic examinations revealed calcification and fragmentation of elastic fibers. Therefore, he was finally diagnosed as having pseudoxanthoma elasticum 25 years after the onset of cardiovascular disease.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/12. pseudoxanthoma elasticum with abnormal nailfold microcirculatory findings.pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue. Here we report a 34-year-old male patient who developed multiple symptomless yellowish papules over his neck for several months. He visited our dermatologic out-patient-clinic because his sister had similar skin lesions and mild visual impairment. The pathologic features of the skin biopsy showed fragmented calcified elastic fibers in the mid-to-lower dermis under the H&E, Verhoeff-van Gieson and Von Kossa stains. Under electron microscopy, calcified degenerated elastic fibers were noted. No other internal organ involvement was found except angioid streaks on the fundus. In addition, morphological changes of the nailfold capillaries, including increased tortuosity, dilated venous limbs of capillary loops, and decreased red-blood-cell velocity, were observed under the capillaroscopy. Though former reports have indicated that cardiovascular manifestations are caused by degeneration of elastic fibers of blood vessels, this study is the first to emphasize the microcirculatory disturbance of nailfold capillary, including morphology and blood-cell velocity, in PXE.- - - - - - - - - - ranking = 10.044593808933keywords = blood vessel, vessel (Clic here for more details about this article) |
5/12. Intravascular ultrasound findings of coronary wall morphology in a patient with pseudoxanthoma elasticum.pseudoxanthoma elasticum (PXE) is an inherited disorder characterised by progressive calcification of the elastic fibres in the skin, eye, and cardiovascular system. Recently, mutations in the ATP binding cassette transporter gene (ABCC6) were identified as cause of this disease. Although patients with PXE often have coronary artery disease, little is known about the process and the mechanism of coronary artery disease in PXE. In this report, intravascular ultrasound (IVUS) imaging was performed in a female patient with PXE seven years after the onset of skin lesion to assess the coronary wall morphology in detail. IVUS showed a unique five layer appearance without acoustic shadowing along the vessel wall observed in the angiographically normal portion. These findings may reflect the earlier stage of coronary artery disease caused by PXE before calcification of the internal elastic laminae.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/12. Cerebral small vessel disease in pseudoxanthoma elasticum: three cases.BACKGROUND: Cerebral small vessel disease is rarely described in association with pseudoxanthoma elasticum (PXE), a hereditary connective tissue disorder with skin, eye and vascular manifestations. This autosomally inherited elastic tissue disease has been attributed to mutations in the ABCC6 gene located on chromosome 16p13.1. Different stroke mechanisms are suggested in PXE patients, arterial hypertension and accelerated atherosclerosis being the leading ones. CASE DESCRIPTIONS: Case 1: A 49-year-old man with history of mild hypertension presented with recurrent transient ischemic attacks. At the age of 42, evaluation for progressive visual loss and skin changes led to diagnosis of PXE. brain magnetic resonance imaging (MRI) disclosed multiple lacunar infarctions and confluent periventricular white matter lesions (WML). Case 2: A 71-year-old woman with history of mild hypertension suffered right-sided stroke. diagnosis of PXE was made at the age of 48 due to severe visual loss and skin changes. brain MRI revealed multiple lacunar infarctions and subcortical ischemic leukoencephalopathy. Case 3: A 47-year-old woman with prominent skin changes and bilateral amblyopia developed right-sided weakness. skin biopsy confirmed PXE. Several lacunar infarcts in deep white matter and pons were revealed on MRI. DISCUSSION: We present three patients with clinical and histopathological features of PXE who presented with multiple lacunar strokes, two with extensive confluent WML. These cases illustrate that PXE is a rare but significant risk factor for small vessel disease and stroke in patients of all age groups. Occlusive small vessel disease and subsequent lacunar infarcts and WML represent important PXE manifestations.- - - - - - - - - - ranking = 7keywords = vessel (Clic here for more details about this article) |
7/12. Sonographic detection of renal changes in pseudoxanthoma elasticum.pseudoxanthoma elasticum (PXE) is a rare hereditary disease whose basic abnormality is degeneration of elastic tissue, with calcification of the tonaca media of the arteries, and typical yellowish papules of the skin. We report the sonographic findings observed in a young patient with PXE at the level of the kidneys: fine hyperechogenic spots suggesting small calcifications were diffuse at the corticomedullary junction; however, normal waveforms were obtained at the level of intraparenchymal renal vessels. The presence of this structural pattern in a young patient with dermatologic abnormalities should lead to the consideration of PXE in the differential diagnosis list.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/12. pregnancy in women with pseudoxanthoma elasticum.pseudoxanthoma elasticum (PXE) is characterized by degeneration of elastic tissue. Clinically, its effects are most readily noted in the skin, retina, blood vessels, and myocardium. Several hundred patients with this condition, signs and symptoms of which usually appear during the early portion of the reproductive years, have been reported. fertility is unaffected. Despite this, only five articles describing the outcome of pregnancies in seven women with PXE have been recorded, none in the obstetric literature. This is particularly odd considering that five of the seven women suffered serious gastrointestinal hemorrhage during one or more of their pregnancies. One woman suffered congestive heart failure and cardiac arrhythmia during pregnancy, and a fatal attack of cardiac arrhythmia 2 months postpartum. The five articles are reviewed, basic information regarding PXE is given, a case history is recorded, and the possibility that pregnancy favors the development of these complications in patients with PXE is discussed.- - - - - - - - - - ranking = 10.044593808933keywords = blood vessel, vessel (Clic here for more details about this article) |
9/12. Neurological complications of Gronblad-Strandberg syndrome.Neurological complications resulting from pronounced cerebrovascular changes were observed in a 59-year-old female suffering from Gronblad-Strandberg syndrome. In addition to typical skin changes of the pseudoxanthoma elasticum and ocular alterations (exudative macular degeneration and angioid streaks) there were an unusual number of disturbed endocrinological functions. The ophthalmological findings revealed characteristic breaks in Bruch's membrane, proliferation of chorioidal vessels into the subretinal space, hyperplasia of retinal pigment epithelium and disciform macular degeneration. Postmortem examination of the brain showed multiple cerebral infarcts due to recurrent disturbances in cerebral blood supply and particularly pronounced changes in the elastica interna of many cerebral arteries. Although the observed neurological signs in our patient were not characteristic of Gronblad-Strandberg syndrome, the histopathological findings led to the assumption that the same basic mechanisms result in alterations of elastic fibres and collagen, mainly in the ocular-cerebral system.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/12. pseudoxanthoma elasticum: a review of neurological complications.A case of pseudoxanthoma elasticum with multisystem involvement is described. Neurological complications, as reported in the literature, are reviewed. These include cerebrovascular insufficiency, multiple lacunar infarcts, aneurysms, subarachnoid and intracerebral hemorrhages, progressive intellectual deterioration, and psychic and mental disturbance which may be due to cortical atrophy. seizures occur more frequently than in the general population. hypertension and alteration of cerebral vessels are the two basic pathophysiological mechanisms responsible for the neurological complications of this disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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