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)

We studied the plasma amino acid profiles in the postabsorptive period in three groups of cirrhotic patients: stable, unstable, and with acute portal systemic encephalopathy (PSE), and compared them with a healthy control group in order to investigate the differences among the different groups and reevaluate the use of the branched-chain amino acid/aromatic amino acid (BCAA/AAA) ratio. Although plasma amino acid levels were similar to the control group, stable cirrhotics had a significantly decreased BCAA/AAA ratio (2.9 +/- 0.2) compared to the control group (3.9 +/- 0.3) (p < 0.05). Unstable cirrhotics had differences in plasma amino acid levels and the BCAA/AAA ratio was even lower (1.7 +/- 0.3) compared with stable cirrhotics and controls, respectively (p < 0.05 and p < 0.01). Patients with PSE had extreme elevations of most amino acids and showed the lowest BCAA/AAA ratio of all four groups (0.8 +/- 0.07) (p < 0.001 compared with controls). We conclude that it is possible to detect differences in plasma amino acid concentrations in different groups of cirrhotics, and that the BCAA/AAA ratio is a good index for the assessment of liver impairment. The latter could be used in the follow-up of a selected group of patients such as those undergoing major surgery or liver transplantation in whom the BCAA/AAA ratio could be used to help determine the best time for the transplant.
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PMID:The BCAA/AAA ratio of plasma amino acids in three different groups of cirrhotics. 148 30

From October 1986 to March 1989, a total of 7 patients who had preoperative profound shock underwent surgical treatment at the National Taiwan University Hospital for a ruptured aorta or vena cava. All 7 patients were men. Their age ranged from 21 to 70 years with a mean of 45 years. Emergency operations were performed due to a ruptured abdominal aortic aneurysm in 2, a ruptured dissecting thoracic aneurysm in 1, a penetrating injury which transected the intrahepatic vena cava in 2, and blunt chest injury which resulted in acute traumatic aortic transection in 2. One of these 7 patients died of acute tubular necrosis, anoxic encephalopathy and secondary sepsis, in spite of successful restoration of circulation. Two patients had postoperative complications. One had a transient paraparesis after an aortic cross-clamp, and the other had a transient impairment of the hepatic function due to the penetrating hepatic injury and the hypoxic hepatic damage. All 6 survivors were restored to an excellent state of health and had minimal post-resuscitation sequelae. We emphasize the importance of aggressive surgical treatment for those patients with the threat of impending death due to massive hemorrhage from a ruptured great vessel.
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PMID:Emergency surgery of patients with ruptured great vessels and profound shock. 197 58

Serum amino acid determinations were made in 40 patients with chronic cor pulmonale in the period of 1985-1987 in our hospital. There were slight type 14 cases. severe type 26 cases (including pulmonary encephalopathy 10 cases). In severe type the ratio of branched chain amino acid to aromatic amino acid (BACC/AAA) was significantly lower than normal (P less than 0.01) and there was significant negative correlation between PaCO2 and BCAA/AAA (r = -0.49 P less than 0.05). The plasma amino acid pattern in severe type cor pulmonale is similar to that found in hepatic encephalopathy. The mechanism of coma in cor pulmonale is at least in some degree, similar with that of hepatic encephalopathy.
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PMID:[Plasma amino acid changes in 40 cases of chronic cor pulmonale]. 263 32

In a controlled cross-over trial, we have compared a conventional 40-g protein diet (30 g animal and 10 g vegetable, diet A) with an 80-g vegetable-protein-supplemented diet (30 g animal and 50 g vegetable, diet B) in the treatment of six patients with chronic stable portal systemic encephalopathy, requiring dietary and lactulose therapy. Each diet was given, in random order, for 5 days in hospital. EEG, clinical indices of encephalopathy, and the plasma amino acid profile were assessed at the end of each treatment period. The increase in vegetable protein intake was associated with minor improvement in EEG and clinical performance in two patients, and no change in the others. Fasting plasma phenylalanine and tyrosine were higher on diet B [phenylalanine 108.6 +/- 9.3 (SEM) mumol/L versus 99.6 +/- 8.37, p less than 0.05 (paired t test); tyrosine 153 +/- 15.2 mumol/L versus 140 +/- 14, p less than 0.05). The plasma branched-chain amino acid levels did not change, and the branched chain/aromatic amino acid ratio (BCAA/AAA) was lower on diet B (p less than 0.02). Fecal weights were not significantly altered. These results indicate that patients with chronic portal systemic encephalopathy are tolerant of protein supplementation from vegetable sources. A minor improvement in parameters of encephalopathy was seen in some individuals, despite a lowering of BCAA/AAA which some investigators have thought important in the pathogenesis of encephalopathy.
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PMID:Dietary protein supplementation from vegetable sources in the management of chronic portal systemic encephalopathy. 639 Nov 54

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

Albeit uncommon, delayed renal dysfunction after endovascular abdominal aortic aneurysm repair (EVAR) can be attributed to proximal stent graft migration or unrecognized partial renal artery coverage. We report two patients who were found to have renal artery occlusion 1 week after EVAR with Zenith (Cook, Bloomington, Ind) infrarenal devices despite patent bilateral renal arteries shown on completion angiograms. Both patients presented with prolonged symptoms of acute renal failure, and uremic encephalopathy developed in one. Both patients were successfully treated with renal artery stenting, which led to symptom resolution and recovery of renal function. Our cases highlight that although postoperative renal occlusion after EVAR is rare, a high index of suspicion and urgent intervention are warranted because renal salvage can be achieved after prolonged ischemic insult.
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PMID:Prolonged renal artery occlusion after endovascular aneurysm repair: endovascular rescue and renal function salvage. 1795 May 60

Hyperammonemia and severe amino acid imbalances play central role in hepatic encephalopathy (HE). In the article is demonstrated that the main source of ammonia in cirrhotic subjects is activated breakdown of glutamine (GLN) in enterocytes and the kidneys and the main source of GLN is ammonia detoxification to GLN in the brain and skeletal muscle. Branched-chain amino acids (BCAA; valine, leucine, and isoleucine) decrease due to activated GLN synthesis in muscle. Aromatic amino acids (AAA; phenylalanine, tyrosine, and tryptophan) and methionine increase due to portosystemic shunts and reduced ability of diseased liver. The effects on aminoacidemia of the following variables that may affect the course of liver disease are discussed: nutritional status, starvation, protein intake, inflammation, acute hepatocellular damage, bleeding from varices, portosystemic shunts, hepatic cancer, and renal failure. It is concluded that (1) neither ammonia nor amino acid concentrations correlate closely with the severity of liver disease; (2) BCAA/AAA ratio could be used as a good index of liver impairment and for early detection of derangements in amino acid metabolism; (3) variables potentially leading to overt encephalopathy exert substantial but uneven effects; and (4) careful monitoring of ammonia and aminoacidemia may discover important break points in the course of liver disease and indicate appropriate therapeutic approach. Of special importance might be isoleucine deficiency in bleeding from varices, arginine deficiency in sepsis, and a marked rise of GLN and ammonia levels that may appear in all events leading to HE.
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PMID:Ammonia and amino acid profiles in liver cirrhosis: effects of variables leading to hepatic encephalopathy. 2522 Aug 75

Central Nervous System (CNS) degeneration appearing in patients with cirrhosis is responsible for cognitive and persistent motor impairments that lead to an important impact on life quality. Brain injury affects certain areas of the CNS that might affect two types of cells: neurons and astrocytes. The process leading to brain injury could be induced by portosystemic shunting accompanied by hyperammonemia and by the activation of peripheral inflammation, manifested as episodic encephalopathy. Hyperammonemia combined with a decrease on the BCA/AAA ratio induces alterations of energetic metabolism and the formation of free radicals in the CNS. This process would be stimulated by the activation of peripheral inflammatory mediators that could act on receptors of the blood brain barrier such as TLR4, activating inflammatory responses in the CNS. As a result, a persistent activation of microglia and an irreversible neuronal and astrocytic injury would be induced. A new knowledge of the mechanisms leading to brain injury in cirrhosis would develop protective strategies to correct changes of nitrogen metabolism and inflammation.
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PMID:What we know: the inflammatory basis of hepatic encephalopathy. 2649 51