Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0162871 (abdominal aortic aneurysm)
 8,664 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A unique case of a Chinese boy with Wiskott-Aldrich syndrome (WAS) associated with Takayasu's arteritis is reported. He had eczema, epistaxis and recurrent infections since early infancy and was found to have thrombocytopenia, negative delayed-type skin hypersensitivity, low T cell number and impaired lymphocyte proliferation to phytohaemagglutinin and concanavalin A. He had high normal serum immunoglobulin (Ig)G and IgA with low IgM and isohaemagglutinin. He presented with hypertensive encephalopathy at 5.5 years of age and an aortogram demonstrated abdominal aortic aneurysm with bilateral stenosis of renal arteries resulting in renovascular hypertension. His hypertension was difficult to control medically and autotransplant of his kidneys to the iliac arteries was performed, but he died in the immediate postoperative period. The relationship between immunodeficiency and collagen-vascular disease was discussed.
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PMID:Takayasu's arteritis associated with Wiskott-Aldrich syndrome. 135 86

The reported familial clustering of abdominal aortic aneurysm (AAA) indicates the possible rewards of family-based screening programmes with respect both to the number of asymptomatic aneurysms detected and to identifying associated genes. Ultrasonographic screening of 28 families (25 brothers and 28 sisters) was carried out together with collecting a history and a blood sample for analysis of the cholesterol level and genetic markers. Among the screened siblings six (11 per cent), all > 60 years old, had an AAA > or = 3.0 cm in diameter. A further 11 siblings (21 per cent), six of whom were < 60 years old, had a wide (2.5-2.9 cm) aorta. The presence of an aneurysmal or wide aorta was significantly associated with smoking (P = 0.027), male sex (P = 0.008) and a proband age of < 60 years (P = 0.031). Polymorphic genetic markers for type III collagen and haptoglobin were not informative in these families. These results indicate that the efficiency of screening siblings of patients with AAA could be improved by limiting it to brothers with a smoking history and/or siblings of younger patients. The familial component appears to be greatest in these younger patients.
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PMID:Selection for screening for familial aortic aneurysms. 142 49

We examined the synthesis of collagenous proteins by cultured skin fibroblasts taken from 14 patients with an abdominal aortic aneurysm and either an aneurysm at a second site (8 patients) or a first order relative with an abdominal aortic aneurysm (6 patients). Fibroblasts were labeled with [3H] proline and, following pepsin digestion of media proteins, the ratio of type I/III collagen was examined by denaturing polyacrylamide gel electrophoresis (SDS-PAGE). With the exception of two patients, the ratio of type I/III collagen in the media of fibroblasts from aneurysm patients was similar to control values (6 controls). In two of the patients, the type I/III collagen ratio was greater than 3 standard deviations from the mean of both control ratios and those of other aneurysm patients. mRNA levels coding for type III procollagen, however, were normal in both patients. Patient #1 (ME) showed reduced type III procollagen on SDS-PAGE analysis of intracellular proteins. Intracellular and media type III procollagen levels were normal in patient #2 (HR), but media type III collagen was reduced by over 50% after digestion with a combination of trypsin and alpha-chymotrypsin for 5 minutes at 36 degrees C. Control type III collagen was only reduced after digestion at 39 degrees C. These data suggest an altered thermal stability of the type III collagen trimer synthesized by this patient, probably due to a mutation in the amino acid sequence. The data presented in this paper suggest that some forms of common abdominal aortic aneurysms may be caused by mutations in the gene coding for type III procollagen.
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PMID:Abnormalities in the biosynthesis of type III procollagen in cultured skin fibroblasts from two patients with multiple aneurysms. 160 41

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

The successful resection of an abdominal aortic aneurysm is presented in a patient who had undergone kidney transplantation 4 years previously. Because the transplanted kidney is more sensitive to ischemia than a normal one, a femoro-femoral bypass with a pump oxygenator was used for perfusion of the transplanted kidney during crossclamping. During the clamping time of 40 minutes kidney perfusion was maintained with a perfusion pressure of 60 to 80 mmHg and the flow was 600 to 1000 ml/min. A collagen-seeded Dacron graft (diameter: 18 mm, length: 12 mm) was interposed. The postoperative course was uncomplicated. We believe that performing the femoro-femoral bypass with a pump oxygenator is an effective and simple method for kidney protection in such operations.
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PMID:Abdominal aortic aneurysm repair after renal transplantation with extracorporeal bypass. 178 46

Atherosclerosis of the infrarenal aorta results in distinct clinical entities--aortoiliac occlusive disease (AOD) and abdominal aortic aneurysm (AAA). Although loss of collagen has been implicated in AAA, collagen accumulation plays a role in AOD. In vivo collagen-gene expression can be assessed using complementary DNA for collagen types I and III alpha-chains. The purpose of this study is to compare total collagen (type I + III) and collagen types I and III messenger RNA in AAA, AOD and normal aorta. Specimens were collected from the infrarenal aorta during operation for AOD (n = 7), AAA (n = 7), autopsy, or organ procurement (normal; n = 7). Northern transfer analysis of total RNA was used to compare mRNA levels for type I and III collagen. After preliminary extraction, specimens were hydrolyzed for hydroxyproline analysis used to calculate total collagen (type I + III). Relative levels of type I (pro-a1[1]) mRNA were greater in both AOD (0.77 +/- 0.35) and AAA tissue (0.94 +/- 0.24; p = 0.6) than in normal aorta (0.02 +/- 0.03). Type III (pro-a1[III]) mRNA levels were also greater in AOD (2.52 +/- 0.19; p = 0.09) and AAA tissue (3.15 +/- 1.3) than in normals (0.97 +/- 0.47). Total collagen concentration was increased in AOD (45.6% +/- 3.1% dry weight; p less than 0.05) but not AAA tissue (27.8% +/- 4%) when compared to normal aorta (34.7% +/- 2.3%). Collagen type I and III gene expression is greater in older, diseased aorta, yet collagen accumulated only in AOD. This implies a similar synthetic response in both AOD and AAA. Thus, proteolytic degradation in AAA appears to determine collagen content and possibly the clinical course of the atherosclerotic process.
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PMID:Aneurysm or occlusive disease--factors determining the clinical course of atherosclerosis of the infrarenal aorta. 185 45

Eight cases of inflammatory abdominal aortic aneurysm (IAAA) (group I) and a control group of ten cases of atherosclerotic abdominal aortic aneurysm (AAA) with little or no parietal inflammatory infiltrate (group II) were studied; using light microscopy, transmission electron microscopy (TEM), and immunohistochemistry. These were used to define cell composition in the inflammatory process, the degree of cell activation and alteration of connective tissue. Large numbers of B lymphocytes were present in IAAA with preservation of the T4/T8 ratio. In addition, HLA-DR and the IL2-R antigen (specific for activated cells) were widely expressed in the cell population. The interstitial matrix contained deposits of IgG, IgM and C3c together with an increase in type III collagen and a reduction in elastin which appeared fragmented and swollen. This study, therefore, characterised the cellular component of the parietal inflammatory infiltrate in IAAA. The degree of activation shown by these cell elements and the activation of complement suggest that the relevant antigen may have been localised in the aneurysm wall at the time of observation.
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PMID:The cellular component in the parietal infiltrate of inflammatory abdominal aortic aneurysms (IAAA). 200 88

This report describes our first clinical experience with collagen-impregnated Dacron grafts in the aortic position. Fifty-four consecutive patients (43 men and 11 women), average age 67.5 years (44-84), received 11 tube grafts and 23 bifurcated grafts for abdominal aortic aneurysm replacement, 17 bifurcated grafts for aortic occlusive disease, two grafts for aortic pseudoaneurysm, and one graft for thoracoabdominal aneurysm repair. No preclotting maneuvers were used and there was no blood leak from the fabric upon restoration of blood flow, in spite of the use of systemic heparin. The hospital transfusion rate averaged 1.2 units in patients with occlusive disease and 2.7 units in patients undergoing aneurysm resection, which were similar to the transfusion rate in patients receiving standard knitted grafts prior to this study. There were two postoperative deaths from a myocardial infarction and a cerebral hemorrhage and no perioperative thromboembolic events. During a follow-up averaging 41.5 months (26-59), there were two late graft limb thromboses secondary to outflow disease progression. One patient died of aspiration two months postoperatively. Four patients died secondary to cardiac disease in at 13 and 21 months with functioning grafts. The rest of the patients are alive with functioning grafts. We conclude that the collagen-impregnated Dacron graft obviates the need for preclotting maneuvers because of a zero functional porosity.
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PMID:Clinical experience with a collagen-impregnated knitted Dacron vascular graft. 214 59

Aneurysm of abdominal aorta was found in 58 cases (0.87 per cent) of a group of 6646 postmortems. The group comprised persons over 15, 45 males and 13 females. The highest frequency of aneurysms (36) was at the age 71 to 80. Rupture of the aneurysm occurred in 15 cases and vascular diseases represented a half part of other causes of death. All the aneurysms were atherosclerotic, in one case a combination with mucold dystrophy was found. Evidence of arterial hypertension was in 43 per cent of the analyzed cases. Histology was performed in 24 cases and proved a destruction of elastic structures in tunica media, fibrosis, loss of smooth muscle cells followed by substantial thinning of the vessel wall. Widening of adventicia was caused by increase of collagen fibres. Discussion concerned formal pathogenesis of the lesion.
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PMID:[Atherosclerotic aneurysms of the abdominal aorta]. 235 Aug 15

The clinical presentation of an abdominal aortic aneurysm can be misleading. The typical triad of abdominal pain, pulsate mass, and hypotension may be absent. Delay in diagnosis is associated with a high mortality. Described in the article is a case of a ruptured abdominal aneurysm presenting as a large bowel obstruction. When found at surgery, the aneurysm had ruptured and was sealed by overlying colonic mesentery, along with retroperitoneal fascia. Elderly male patients who are smokers are believed to be at higher risk for atypical presentations from ruptured abdominal aortic aneurysms. This is thought to result from a generalized defect in collagen combined with diffuse atherosclerotic vascular disease. The diagnosis requires maintenance of a high clinical suspicion, in particular for those patients with higher risk factors.
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PMID:Ruptured abdominal aortic aneurysm presenting as an obstruction of the left colon. 235 97


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