Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 17-year-old male suffered recurrent subarachnoid hemorrhages due to multiple aneurysms in the distal branches of the cerebral arteries. Autopsy revealed arteriopathy as well as the aneurysms. The arteriopathy was widespread, affecting the distal branches of small and medium-sized muscular cerebral arteries, as well as the anterior and posterior spinal arteries, and vasocorona. The arteriopathy was characterized by prominent intimal thickening, discontinuity or absence of the internal elastic lamina, and thinning and/or disappearance of smooth muscle in the tunica media with fibroplasia. Slight intimal thickening was also observed in the arteries of the circle of Willis and its major branches, as well as in the basilar artery. However, the arterioles, venules and veins showed no remarkable features. The arterial lesions were found only in the central nervous system. The multiple aneurysms in the distal branches of the cerebral arteries, which had produced the main symptoms and clinical signs, were due to the arteriopathy.
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PMID:Isolated central nervous system arteriopathy with multiple aneurysms in an adolescent. A case report. 167 41

The matrix metalloproteinases (MMPs) and endothelin-1, a potent vasoconstrictor and mitogen for smooth muscle cells, have been shown to be involved in the pathogenesis of various vascular disorders. However, the expression of endothelin-1 and the activation of MMPs have not been fully evaluated in plexogenic pulmonary arteriopathy (PPA). Immunohistochemical and confocal microscopic studies were conducted to evaluate the reactivity of lung tissue from six patients with pulmonary hypertension for alpha-smooth muscle actin (alpha-SMA), desmin, vimentin, factor VIII, endothelin-1, various types of MMPs (MMP-1, MMP-2, MMP-3, MMP-7 and MMP-9), membrane type-MMPs (MT-MMPs), tissue inhibitors of MMPs (TIMPs), and type IV collagen. Four major arterial morphological abnormalities were recognized in PPA: muscularization of pulmonary arterioles, onion-skin lesions, cellular and mature plexiform lesions, and atheromas in elastic pulmonary arteries. Reactivity for MMP-2 and MT-1-MMP was found in endothelial cells and, to a lesser extent, in myofibroblasts proliferating in various lesions of PPA. Increased expression of endothelin-1 was observed in the latter cells and in endothelial cells. Some myofibroblasts were positive for MMP-3 and MMP-7 in the vascular lesions except for mature plexiform lesions. MMP-1, MMP-9 and TIMP-2 tended to be positive only in the atheromatous lesions. Staining for type IV collagen showed focal thinning and discontinuities of the endothelial basement membrane in plexiform lesions. This study demonstrates colocalization of MMP-2 with MT-1-MMP and increased expression of endothelin-1 in various arterial lesions of PPA. These changes may play important roles in the remodeling of arterial structures, particularly of basement membranes, in this disorder.
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PMID:Immunohistochemical study of endothelin-1 and matrix metalloproteinases in plexogenic pulmonary arteriopathy. 1216 97

Dolichoectasia (dilatative arteriopathy) describes marked elongation, widening, and tortuosity of arteries. The intracranial vertebral and basilar arteries are preferentially involved. Dolichoectatic arteries usually have an abnormally large external diameter and a thin arterial wall, with degeneration of the internal elastic lamina, multiple gaps in the internal elastica, thinning of the media secondary to reticular fiber deficiency, and smooth muscle atrophy. The most important clinical presentations of dilatative arteriopathy include acute brain ischemia; a progressive course related to compression of cranial nerves, the brain stem, or the third ventricle; and catastrophic outcome caused by vascular rupture. Flow in dilated arteries can become bidirectional, resulting in reduced antegrade flow and thrombus formation. Elongation and angulation of arteries can stretch and distort the orifices of arterial branches, leading to decreased blood flow, especially in penetrating branches.
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PMID:Vertebrobasilar dilatative arteriopathy (dolichoectasia). 2014 94