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

We describe a Jehovah's Witness patient who survived emergency repair of a leaking abdominal aortic aneurysm. In accordance with his beliefs, the patient expressed a wish not to be given blood and this was respected. At completion of surgery, his haemoglobin was 2.8 g dl-1 and his albumin was 8 g l-1. He was kept heavily sedated in the intensive care unit and treated with i.v. iron, folinic acid and s.c. epoetin alfa. He was discharged to the high dependency unit 18 days after surgery with a haemoglobin of 6.4 g dl-1 and an albumin of 27 g l-1. After rehabilitation, he was discharged home approximately 14 weeks after surgery.
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PMID:Perioperative care of a Jehovah's Witness with a leaking abdominal aortic aneurysm. 981 36

We investigated the characteristics of ethylene biosynthesis associated with ripening in banana (Musa sp. [AAA group, Cavendish subgroup] cv Grand Nain) fruit. MA-ACS1 encoding 1-aminocyclopropane-1-carboxylic acid (ACC) synthase in banana fruit was the gene related to the ripening process and was inducible by exogenous ethylene. At the onset of the climacteric period in naturally ripened fruit, ethylene production increased greatly, with a sharp peak concomitant with an increase in the accumulation of MA-ACS1 mRNA, and then decreased rapidly. At the onset of ripening, the in vivo ACC oxidase activity was enhanced greatly, followed by an immediate and rapid decrease. Expression of the MA-ACO1 gene encoding banana ACC oxidase was detectable at the preclimacteric stage, increased when ripening commenced, and then remained high throughout the later ripening stage despite of a rapid reduction in the ACC oxidase activity. This discrepancy between enzyme activity and gene expression of ACC oxidase could be, at least in part, due to reduced contents of ascorbate and iron, cofactors for the enzyme, during ripening. Addition of these cofactors to the incubation medium greatly stimulated the in vivo ACC oxidase activity during late ripening stages. The results suggest that ethylene production in banana fruit is regulated by transcription of MA-ACS1 until climacteric rise and by reduction of ACC oxidase activity possibly through limited in situ availability of its cofactors once ripening has commenced, which in turn characterizes the sharp peak of ethylene production.
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PMID:Characterization of ethylene biosynthesis associated with ripening in banana fruit. 1059 12

In mammalian cells, mitochondria provide energy from aerobic metabolism. They play an important regulatory role in apoptosis, produce and detoxify free radicals, and serve as a cellular calcium buffer. Neurodegenerative disorders involving mitochondria can be divided into those caused by oxidative phosphorylation (OXPHOS) abnormalities either due to mitochondrial DNA (mtDNA) abnormalities, e.g., chronic external ophthalmoplegia, or due to nuclear mutations of OXPHOS proteins, e.g., complex I and II associated with Leigh syndrome. There are diseases caused by nuclear genes encoding non-OXPHOS mitochondrial proteins, such as frataxin in Friedreich ataxia (which is likely to play an important role in mitochondrial-cytosolic iron cycling), paraplegin (possibly a mitochondrial ATP-dependent zinc metalloprotease of the AAA-ATPases in hereditary spastic paraparesis), and possibly Wilson disease protein (an abnormal copper transporting ATP-dependent P-type ATPase associated with Wilson disease). Huntingon disease is an example of diseases with OXPHOS defects associated with mutations of nuclear genes encoding non-mitochondrial proteins such as huntingtin. There are also disorders with evidence of mitochondrial involvement that cannot as yet be assigned. These include Parkinson disease (where a complex I defect is described and free radicals are generated from dopamine metabolism), amyotrophic lateral sclerosis, and Alzheimer disease, where there is evidence to suggest mitochondrial involvement perhaps secondary to other abnormalities.
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PMID:Mitochondria and degenerative disorders. 1157 22

We report herein the case of a 47-year-old woman of the Jehovah's Witness faith in whom Y-grafting for a ruptured abdominal aortic aneurysm was successfully performed without a homologous blood transfusion. We used a Cell Saver (Haemonetics, Braintree, MA. USA) red cell salvaging device and an aortic occlusion balloon catheter, and performed gentle and minimal dissection during the operation. Postoperatively. the patient was kept heavily sedated and required hypothermic therapy for only 14h. We treated her severe anemia using conventional drugs, including iron and folic acid, and her hemoglobin increased smoothly. Although her hemoglobin level decreased to 2.8g/dl during the operation, her postoperative course was uneventful.
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PMID:Successful surgical treatment of a ruptured abdominal aortic aneurysm without homologous blood transfusion in a Jehovah's Witness: report of a case. 1175 89

We previously reported a patient with hereditary hypotransferrinemia who suffered from severe anemia and growth retardation and was diagnosed on the basis of an extremely low level of serum transferrin (TF). By an isoelectric focusing analysis, we found that the patient and his father shared a variant TF protein with an abnormal isoelectric point. The study suggested that the patient was a compound heterozygote with a variant allele, encoding the mutant TF, of paternal origin and a null allele of maternal origin. In the present study, we investigated the TF gene of the patient and his family. We showed that the patient and his father shared a variant TF gene bearing a GAA to AAA transition at codon 394. This nucleotide substitution causes a nonconservative amino acid change from glutamate to lysine in amino acid residue 375 of the TF protein. This single amino acid mutation is predicted to cause a conformational change in the coiled region of the carboxyl-terminal iron-binding lobe. As for the maternal null allele, no mutation was found in either the coding region or the exon-intron boundaries, suggesting an abnormality in the transcription or stability of mRNA of maternal allele origin.
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PMID:Molecular analysis of the transferrin gene in a patient with hereditary hypotransferrinemia. 1211 69

We previously identified a gene, slr0374, in the unicellular cyanobacterium, Synechocystis sp. strain PCC 6803, that was highly expressed under iron-deficient conditions [J. Bacteriol. 182 (2000) 3536]. The gene product contains an AAA domain, a putative leucine zipper and a phosphorylation site and is part of an operon (with slr0373 and slr0376) that is responsive to various environmental stresses. Primer extension mapping and transcript analysis in insertion mutants showed that all transcripts from this operon originated upstream of slr0373 at four contiguous transcription start sites before being processed into individual transcripts. Both primary and processed transcripts were quite stable. The start sites were sensitive to changes in sulfur, light and redox agent, as well as iron. The structural and regulatory elements of this operon were highly conserved in phycobilisome-containing cyanobacteria that have been sequenced to date. Slr0374 and Slr0376 show homology with Ycf46 and Ycf35, respectively.
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PMID:Characterization of a stress-responsive operon in the cyanobacterium Synechocystis sp. strain PCC 6803. 1238 81

Reactive oxygen species (ROS) have been implicated in the cellular membrane damage and postoperative morbidity associated with obligatory ischemia-reperfusion (I-R) during vascular surgery. Thus, a clinical study was undertaken to evaluate the effects of ascorbate prophylaxis on ROS exchange kinetics in 22 patients scheduled for elective abdominal aortic aneurysm (AAA) or infra-inguinal bypass (IIB) repair. Patients were assigned double-blind to receive intravenous sodium ascorbate (2 g vitamin C, n=10) or placebo (0.9% saline, n=12) administered 2 h prior to surgery. Blood samples were obtained from the arterial and venous circulation proximal to the respective sites of surgical repair (local) and from an antecubital vein (peripheral) during cross-clamping (ischemia) and within 60 s of clamp release (reperfusion). Ascorbate supplementation increased the venoarterial concentration difference (v-adiff) of lipid hydroperoxides (LH), interleukin (IL)-6 and vascular endothelial growth factor (VEGF) protein during ischemia. This increased the peripheral concentration of LH, total creatine phosphokinase (CPK), and VEGF protein during reperfusion (P<0.05 vs placebo). Electron paramagnetic resonance (EPR) spectroscopy confirmed that free iron was available for oxidative catalysis in the local ischemic venous blood of supplemented patients. An increased concentration of the ascorbate radical (A.-) and alpha-phenyl-tert-butylnitrone (PBN) adducts assigned as lipid-derived alkoxyl (LO.) and alkyl (LC.) species were also detected in the peripheral blood of supplemented patients during reperfusion (P<0.05 vs ischemia). In conclusion, these findings suggest that ascorbate prophylaxis may have promoted iron-induced oxidative lipid damage via a Fenton-type reaction initiated during the ischemic phase of surgery. The subsequent release of LH into the systemic circulation may have catalyzed formation of second-generation radicals implicated in the regulation of vascular permeability and angiogenesis.
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PMID:Vitamin C prophylaxis promotes oxidative lipid damage during surgical ischemia-reperfusion. 1645 89

Calcified deposits exist in almost all abdominal aortic aneurysms (AAAs). The significant difference in stiffness between these hard deposits and the compliant arterial wall may result in local stress concentrations and increase the risk of aneurysm rupture. Calcium deposits may also complicate AAA repair by hindering the attachment of a graft or stent-graft to the arterial wall or cause vessel wall injury at the site of balloon dilation or vascular clamp placement. Knowledge of the composition and properties of calcified deposits helps in understanding the risks associated with their presence. This work presents results of elemental composition, microscopic morphology, and mechanical property measurements of human calcified deposits obtained from within AAAs. The elemental analyses indicate the deposits are composed primarily of calcium phosphate with other assorted constituents. Microscopy investigations show a variety of microstructures within the deposits. The mechanical property measurements indicate an average elastic modulus in the range of cortical bone and an average hardness similar to nickel and iron.
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PMID:Elemental composition, morphology and mechanical properties of calcified deposits obtained from abdominal aortic aneurysms. 1683 27

We investigated two unrelated children with an isolated defect of mitochondrial complex III activity. The clinical picture was characterized by a progressive encephalopathy featuring early-onset developmental delay, spasticity, seizures, lactic acidosis, brain atrophy and MRI signal changes in the basal ganglia. Both children were compound heterozygotes for novel mutations in the human bc1 synthesis like (BCS1L) gene, which encodes an AAA mitochondrial protein putatively involved in both iron homeostasis and complex III assembly. The pathogenic role of the mutations was confirmed by complementation assays, using a DeltaBcs1 strain of Saccharomyces cerevisiae. By investigating complex III assembly and the structural features of the BCS1L gene product in skeletal muscle, cultured fibroblasts and lymphoblastoid cell lines from our patients, we have demonstrated, for the first time in a mammalian system, that a major function of BCS1L is to promote the maturation of complex III and, more specifically, the incorporation of the Rieske iron-sulfur protein into the nascent complex. Defective BCS1L leads to the formation of a catalytically inactive, structurally unstable complex III. We have also shown that BCS1L is contained within a high-molecular-weight supramolecular complex which is clearly distinct from complex III intermediates.
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PMID:Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy. 1740 14

OPA1, an intra-mitochondrial dynamin GTPase, is a key actor of outer and inner mitochondrial membrane dynamic. OPA1 amino-terminal cleavage by PARL and m-AAA proteases was recently proposed to participate to the mitochondrial network dynamic in a DeltaPsi(m)-dependent way, and to apoptosis. Here, by an in vitro approach combining the use of purified mitochondrial fractions and mitochondrial targeting drugs, we intended to identify the central stimulus responsible for OPA1 cleavage. We confirm that apoptosis induction and PTPore opening, as well as DeltaPsi(m) dissipation induce OPA1 cleavage. Nevertheless, our experiments evidenced that decreased mitochondrial ATP levels, either generated by apoptosis induction, DeltaPsi(m) dissipation or inhibition of ATP synthase, is the common and crucial stimulus that controls OPA1 processing. In addition, we report that ectopic iron addition activates OPA1 cleavage, whereas zinc inhibits this process. These results suggest that the ATP-dependent OPA1 processing plays a central role in correlating the energetic metabolism to mitochondrial dynamic and might be involved in the pathophysiology of diseases associated to excess of iron or depletion of zinc and ATP.
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PMID:OPA1 cleavage depends on decreased mitochondrial ATP level and bivalent metals. 1782 66


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