Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.3.1.21 (CPT)
 4,580 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Whether infection with influenza B virus alters hepatic function was examined in the ferret. Also, the possibility that viral-specific antibodies (Ab) could be produced well before their detection in serum was explored. During the febrile period of influenza, reductions in the serum potassium, anion gap, ammonia, albumin and CPK and elevations of the BUN, creatinine and the GGTP levels occurred. With convalescence, the electrolytes, BUN and creatinine normalized, FFA, SGPT and CPK levels rose and the serum GGTP rose even further. Hepatic fatty acid (FA) oxidation, ornithine transcarbamylase (OTC) and carnitine palmitoyltransferase (CPT) activities were minimally altered and liver ATP and total lipid content remained normal. Following experimental secondary viremia, serum FFA continued to rise, TG decreased and CPK remained elevated while SGPT and GGTP levels normalized. In the liver, FA oxidation and OTC rates remained unchanged but CPT activity was inhibited and the liver content of ATP was significantly reduced. Immune complex (IC) protein recovered from postmicrosomal supernatant fractions by polyethylene glycol precipitation was progressively increased in livers from convalescent and viremic animals. While the amount of IC protein recovered in the spleen also increases during convalescence, this is not the case after viremia when the IC formed seem to be processed largely by the liver. By SDS/PAGE, the major proteins identified in the IC were IgM and other viral proteins. However, the viral proteins could not be validated by immunoblot with Ab produced against purified influenza B hemagglutinin (HA) and neuraminidase (NA) most probably due to phagocytic alterations of glycoprotein immunodeterminants. These findings indicate that during influenza, convalescence and post viremia changes in the concentrations of several serum and liver components occur that reflect hepatic involvement. Also, antiviral Ab, largely IgM, appears to be produced early, complexes with Ag and can be found sequestered in both the liver and spleen at a time when Ab is not detectable in the serum.
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PMID:Potential for hepatic and renal dysfunction during influenza B infection, convalescence, and after induction of secondary viremia. 135 41

A large number of intellectually engaging and potentially serious neuromuscular diseases have been presented. The emergency medicine physician must be able to recognize those entities that have the potential to clinically deterioration. The evaluation of weakness requires a comprehensive, broad-based differential that is driven by the history and physical. Diagnostic testing is determined by the clinical suspicion as is the urgency for further work-up. The following are the final diagnoses of the eight illustrative cases that were presented at the beginning of this article. Case 1. This unfortunate woman had a metabolic myopathy that was only diagnosed after enzymatic analysis of a muscle biopsy. Her genetic defect, carnitine palmitoyltransferase deficiency, is unusual as it does not present until late in adolescence or slightly later in life. It is a defect in lipid metabolism in which long-chain fatty acids are unable to gain entrance into the mitochondrion for oxidative degradation. The defect is apparent only after prolonged exercise or fasting. In this patient, rhabddomyolysis led to acute renal failure that resolved without requiring temporary dialysis. Case 2. This patient had an elevated CPK-MM. Her EMG showed myopathic changes and her nerve conduction studies were normal. She had a positive test for antinuclear antibodies. A biopsy of her quadriceps muscle revealed lymphocytic infiltration of the muscle fibers that showed some focal myocyte degeneration. The diagnosis of dermatomyositis was made based on the findings noted previously and the heliotrope hue of her periorbital skin. A search for an occult neoplasm was negative. She responded moderately to a course of high-dose prednisone. Case 3. The laboratory test that confirmed this diagnosis was the potassium of 2.4 mEq/L. The remainder of the electrolytes were normal. Infusion of 20 mEq of potassium over 2 hours led to a prompt return of normal muscle strength. The final diagnosis was hypokalemic periodic paralysis. In this disease there is an inherited defect in the ability of the myocyte to maintain a normal transmembrane potential. The defect is latent until there is a precipitating factor, such as an high carbohydrate meal or prolonged immobility. There is also a form seen with thyrotoxicosis and is essentially cured when the patient becomes euthyroid. The disease is seen most frequently in Asian males, although it is reported in most ethnic groups. Prophylaxis in these patients is with acetazolamide which raises the serum potassium indirectly by causing a metabolic acidosis. Triamterene and spironolactone have also been successfully used on occasion. This patient turned out to have thyrotoxicosis as well. Case 4. This man had both cranial motor and peripheral muscular dysfunction. There was no evidence of nonmotor cranial nerve dysfunction, nor was there evidence of any peripheral sensory deficits. The diagnosis of myasthenia gravis was established by the rapid and transient response of this patient to 2 mg of edrophonium. He was found to have antiacetylcholine receptor antibodies and was also thyrotoxic. He had a stormy course requiring intubation and prolonged ventilation. Eventually, he underwent thymectomy and is stable on pyridostigmine. Case 5. Initially suspected to be hysteria, this patient and his relatives had botulism from home-canned peppers. The index case required prolonged intubation and ventilation. The patients were treated with polyvalent antiserum and gastric lavage to remove the residual contaminated food which was still in their stomachs due to the gastric atony seen with this disease. The botulinus toxin prevents the release of acetylcholine molecules from their storage vesicles in nerve terminals. Thus, this disease is the opposite of the cholinergic syndrome seen with organophosphate insecticide poisonings except that cognitive functioning is not impaired in botulism. Case 6. This is a celebrated case that took a great deal of sleuthing
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PMID:Approach to generalized weakness and peripheral neuromuscular disease. 925 35