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Query: UMLS:C0162871 (abdominal aortic aneurysm)
 8,664 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Collagens provide the tensile strength of the aortic wall. Variations in collagen structure are recognised in Ehlers Danlos syndrome type IV and could also be associated with a predisposition to aortic aneurysm. The frequency of some minor genetic variants of type III collagen, present in the normal population, can be detected by restriction enzyme digestion of genomic DNA from peripheral leucocytes. The frequency of a minor collagen type III allele demonstrated with the restriction enzyme Ava II was compared in patients with abdominal aortic aneurysm (n = 70) and aortoiliac stenosis (n = 47). The frequency of the minor allele was significantly higher in aneurysm patients (0.30) compared with patients with aortoiliac stenosis (0.17), p less than 0.05. The presence of the minor allele was associated with a less elastic aneurysm wall (median pressure strain elastic modulus 4575, n = 7), compared with patients homozygous for the common allele (median pressure strain elastic modulus 1990, n = 6), p less than 0.05. These results indicate that genetic variants of type III collagen may influence the extensile properties of the aortic wall and that mutations in the type III collagen gene may be associated with aortic aneurysms.
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PMID:Genetic variants of collagen III and abdominal aortic aneurysm. 167 99

Aortic aneurysm occurs rarely in childhood. Most aneurysms in children are associated with conditions such as Marfan's syndrome, coarctation of the aorta, Ehlers-Danlos syndrome and neurofibromatosis. We report a case of descending thoracic-abdominal aortic aneurysm in an eighteen month old boy with a mask-like face, ocular hypertelorism, blepharoptosis, a high arched palate and low set ears. He was scheduled for a bypass graft of the descending thoracic and abdominal aorta under partial cardiopulmonary bypass. High dose morphine anesthesia (2mg/kg) was employed and halothane was used to control the blood pressure. To our knowledge, anesthetic management of such an infant had not been reported. Therefore, we anesthetized this child according to anesthetic managements for adult cases considering carefully the boy's multiple anomalies. Anesthetic managements for the surgery of aortic aneurysms in childhood are discussed.
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PMID:[Anesthetic management in surgery of a descending aortic aneurysm in an eighteen-month-old boy]. 270 3

We report and analyze two cases of Ehlers-Danlos syndrome (EDS) type 4. The first manifestation of the disease was a spontaneous perforation of the colon in a 47-year-old man; he was successfully reoperated on five years later for the rupture of an abdominal aortic aneurysm. Abdominal pain demonstrated the syndrome in a 33-year-old woman in whom multiple abdominal aneurysms were found. A ligation of the anterior tibial artery for spontaneous rupture was performed five years later. Light and electron microscopic studies of the skin disclosed similar alterations in both cases. The diameter of the collagen fiber bundles was reduced and the diameter of collagen fibrils was increased. It appears that EDS type 4 might be less characteristic than has been previously described. Classification of the different types of EDS according to electron microscopy is not possible.
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PMID:Changing patterns in the vascular form of Ehlers-Danlos syndrome. 374 Nov 1

We have experienced graft replacement of a thoracic aortic aneurysm in a 42-year-old man with Ehlers-Danlos syndrome. The patient received graft replacement of the abdominal aortic aneurysm 1 year before this thoracic operation but had no abnormality in his outside appearance. Thoracic CT scan revealed a thoracic aortic aneurysm of 80 mm in maximal diameter. We performed a graft replacement of the thoracic aorta from the ascending aorta to the proximal descending thoracic aorta using deep hypothermia and retrograde cerebral perfusion. The aortic wall was so thin that we used Teflon felt for reinforcement of graft anastomosis at the outside wall of the aortic stump. Type III collagen stain of the resected aortic wall showed deficiency of type III collage, which was consistent with Ehlers-Danlos syndrome (type IV). Postoperative course was uneventful, and the patient returned to his ordinary life.
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PMID:[Successful graft replacement of a thoracic aortic aneurysm in a patient with Ehlers-Danlos syndrome]. 852 72

A 38-yr-old man with an unusual type of Ehlers-Danlos syndrome presented for elective abdominal aortic aneurysm repair. During surgery he developed acute myocardial ischaemia, resulting in abandonment of the procedure. He was shown subsequently to have severe triple vessel coronary artery disease. Silent ischaemia associated with severe coronary artery disease, although rare, may be associated with the syndrome and is difficult to recognize as other cardiac abnormalities are frequently present.
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PMID:Myocardial ischaemia associated with Ehlers-Danlos syndrome. 878 54

Aortocaval fistula (ACF) is a rare complication of spontaneous abdominal aortic aneurysm (AAA) rupture, with an incidence of 2-4%. A unique case of ruptured AAA complicated by multiple aortovenous fistulas involving the inferior vena cava and left internal iliac vein is presented, and is the first published report of a patient with Ehlers-Danlos syndrome undergoing surgical treatment for an ACF.
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PMID:Multiple aortocaval fistulas associated with a ruptured abdominal aneurysm in a patient with Ehlers-Danlos syndrome. 1046 25

Ehlers-Danlos syndrome is a connective tissue disorder caused by abnormal collagen synthesis. Vascular complications, including aneurysm formation and spontaneous arterial perforations, are difficult to manage surgically and result in significant operative mortality due to blood vessel fragility. We describe the first reported successful endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. We discuss the advantages endovascular surgery offers over open surgery in these patients. We believe that endovascular repair of abdominal aortic aneurysms preferentially over open repair merits consideration in patients with Ehlers-Danlos syndrome.
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PMID:Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. 1766 11

Tenascin-X (TNX), which has a molecular mass of roughly 450 kDa, is the largest member of the tenascin family. Complete deficiency of TNX in humans leads to a recessive form of Ehlers-Danlos syndrome (EDS). TNX is expressed abundantly in a variety of tissues, especially in cardiac muscle and in perivascular stroma. Human TNX is also present in serum with an apparent molecular size of 140 kDa. In the present study, we investigated the expression levels of TNX protein in thoracic and abdominal aortic aneurysm tissues. The level of TNX was significantly increased in both aortic aneurysm tissues compared with that in adjacent normal tissues. Next, to compare TNX levels in serum from both patients with thoracic aortic aneurysm and patients with abdominal aortic aneurysm with levels in serum from healthy individuals, we developed a sandwich enzyme-linked immunosorbent assay (ELISA) using TNX-specific antibodies. Measurement of TNX serum concentrations in both aortic aneurysm patients and controls showed that the levels were almost the same. These results indicate that TNX expression is significantly elevated in both thoracic and abdominal aortic aneurysm tissues but that the increase in TNX levels in both tissues does not result in an increase in TNX serum concentration in patients with TAA or AAA.
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PMID:Increased expression of tenascin-x in thoracic and abdominal aortic aneurysm tissues. 2104 18

An abdominal aortic aneurysm is a rare disease in the paediatric population and is mainly caused by intrauterine infection, connective tissue diseases, such as Ehlers-Danlos syndrome and Marfan's syndrome, and iatrogenic trauma due to umbilical artery catheterization. Although several cases have been reported in the English literature, they were rarely diagnosed prenatally. Vascular obstruction in utero is also believed to be the major cause of porencephaly. Recently, gene mutations have been reported as the cause of both the above-mentioned diseases. We present a prenatally diagnosed case of congenital abdominal aortic aneurysm with porencephaly.
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PMID:Congenital abdominal aortic aneurysm with porencephaly: a case report. 2116 Jan 69

Abdominal aortic aneurysms (AAAs) are a major cause of illness and death in the United States and abroad. Along with progress in the surgical management of this condition, numerous advances have been made in understanding the pathogenesis of AAAs. Since the time of Scarpa (1804), AAA disease has been associated with, and attributed to, atherosclerotic vessel changes. Excluding patients with Marfan's Syndrome and Ehlers-Danlos type IV diseases, virtually all human AAA specimens contain some degree of atherosclerosis. However, atherosclerotic changes are associated with diverse arteriopathies (ie, aorto-occlusive disease versus AAAs). Over the last 25 years, considerable research has been performed comparing aneurysmal, occlusive, and normal aortae. AAA disease is a unique process with pathogenic mechanisms that may operate independently of atherosclerosis. In this chapter we discuss the incidence of AAA as well as its pathogenesis concerning genetics, molecular biology, biochemistry, and immunology.
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PMID:Incidence and pathogenesis of aneurysmal disease of the abdominal aorla. 2131 6


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