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sodium phenylbutyrate

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sodium

 [so´de-um]
a chemical element, atomic number 11, atomic weight 22.990, symbol Na. (See Appendix 6.) Sodium is the major cation of the extracellular fluid, constituting 90 to 95 per cent of all cations in the blood plasma and interstitial fluid; it thus determines the osmolality of the extracellular fluid. The serum sodium concentration is normally about 140 mEq/L. If the sodium level and osmolality fall, osmoreceptors in the hypothalamus are stimulated and cause the release of antidiuretic hormone from the posterior lobe of the pituitary gland. This hormone increases the absorption of water in the collecting ducts of the kidneys so that water is conserved while sodium and other electrolytes are excreted in the urine. If the sodium level and osmolality rise, neurons in the thirst center of the hypothalamus are stimulated. The thirsty person then drinks enough water to restore the osmolality of the extracellular fluid to the normal level.



A decrease in the serum sodium concentration (hyponatremia) can occur in a variety of conditions. It is often associated with deficient fluid volume due to diarrhea or vomiting when water is replaced faster than sodium. It can also occur in syndrome of inappropriate antidiuretic hormone, in the late stages of congestive heart failure or cirrhosis of the liver, in acute or chronic renal failure, and in diuretic therapy. An increase in the serum sodium concentration (hypernatremia) occurs when insensible water loss is not replaced by drinking, as in a comatose patient with diabetes insipidus.
sodium acetate a source of sodium ions for hemodialysis and peritoneal dialysis, as well as a systemic and urinary alkalizer.
sodium ascorbate an antiscorbutic vitamin and nutritional supplement for parenteral administration. It is also used as an aid to deferoxamine therapy in the treatment of chronic iron toxicity.
sodium benzoate an antifungal agent also used in a test of liver function.
sodium bicarbonate NaHCO3, a white powder commonly found in households. It has a wide variety of uses in chemistry, in pharmaceuticals, and in consumer products. It is sometimes taken in water as a remedy for acid indigestion but should not be used regularly since when taken in excess it tends to cause alkalosis. It can be mixed with water and applied as a paste for relief of pain in treatment of minor burns and insect bites and stings. A cupful in the bath water may help relieve itching caused by an allergic reaction. Called also baking soda and bicarbonate of soda.
sodium biphosphate monobasic sodium phosphate.
sodium carbonate a compound now used primarily as an alkalizing agent in pharmaceuticals; it has been used as a lotion or bath in the treatment of scaly skin, and as a detergent.
sodium chloride common table salt, a necessary constituent of the body and therefore of the diet, involved in maintaining osmotic tension of blood and tissues; uses include replenishment of electrolytes in the body, irrigation of wounds and body cavities, enema, inhaled mucolytic, topical osmotic ophthalmic agent, and preparation of pharmaceuticals. Called also salt.
sodium citrate a sodium salt of citric acid, used as an anticoagulant for blood or plasma that is to be fractionated or for blood that is to be stored. It is also administered orally as a urinary alkalizer.
dibasic sodium phosphate a salt of phosphoric acid; used alone or in combination with other phosphate compounds, it is given intravenously as an electrolyte replenisher, orally or rectally as a laxative, and orally as a urinary acidifier and for prevention of kidney stones.
sodium ferric gluconate a hematinic used especially in treatment of hemodialysis patients with iron deficiency anemia who are also receiving erythropoietin therapy. Administered by intravenous injection.
sodium fluoride a dental caries preventative used in fluoridation of drinking water or applied topically to teeth. Topical preparations include gels (sodium fluoride and phosphoric acid gel, also called APF gel) and solutions (sodium fluoride and acidulated phosphate topical solution, also called APF solution).
sodium glutamate monosodium glutamate.
sodium hydroxide NaOH, a strongly alkaline and caustic compound; used as an alkalizing agent in pharmaceuticals.
sodium hypochlorite a compound having germicidal, deodorizing, and bleaching properties; used in solution to disinfect utensils, and in diluted form (Dakin's solution) as a local antibacterial.
sodium iodide a compound used as a source of iodine.
sodium lactate a compound used in solution to replenish body fluids and electrolytes.
monobasic sodium phosphate
1. a monosodium salt of phosphoric acid; used in buffer solutions, as a urinary acidifier, as a laxative, and as a source of phosphorus in hypophosphatemia, often in combination with potassium phosphate.
2. a monosodium salt of phosphoric acid; used in buffer solutions. Used alone or in combination with other phosphate compounds, it is given intravenously as an electrolyte replenisher, orally or rectally as a laxative, and orally as a urinary acidifier and for prevention of kidney stones.
sodium monofluorophosphate a dental caries preventative applied topically to the teeth.
sodium nitrite an antidote for cyanide poisoning; also used as a preservative in cured meats and other foods.
sodium nitroprusside an antihypertensive agent used in the treatment of acute congestive heart failure and of hypertensive crisis and to produce controlled hypotension during surgery; also used as a reagent.
sodium phenylbutyrate an agent used as adjunctive treatment to control the hyperammonemia of pediatric urea cycle enzyme disorders.
sodium phosphate any of various compounds of sodium and phosphoric acid; usually specifically dibasic sodium phosphate.
sodium polystyrene sulfonate an ion-exchange resin used for removal of potassium ions in hyperkalemia, administered orally or rectally.
sodium propionate a salt used as an antifungal preservative in foods and pharmaceuticals and as a topical antifungal agent.
sodium salicylate see salicylate.
sodium sulfate a cathartic and laxative.
sodium thiosulfate a compound used intravenously as an antidote for cyanide poisoning, in foot baths for prophylaxis of ringworm, and as a topical antifungal agent for tinea versicolor. Also used in measuring the volume of extracellular body fluid and the renal glomerular filtration rate.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

buphenyl

An orphan drug for managing urea cycle disorders—e.g., deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase—and indicated for infants with neonatal-onset deficiencies presenting within the first 28 days of life, in whom episodes of acute hyperammonemic encephalopathy are often fatal, and when not, often result in profound mental retardation.

Adverse effects
Amenorrhoea, anorexia, body odour due to phenylacetate excess, abdominal pain, nausea, arrhythmias, renal tubular acidosis, rash, headaches, syncope, weight gain, metabolic acidosis, increased alkaline phosphatase, anaemia.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
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References in periodicals archive
It is a known fact that sodium phenylbutyrate usage increases enzyme activity of alpha-ketoacid dehydrogenase, effectively decreasing blood branched-chain amino acid levels.
He was discharged home on Sildenafil, Citrulline, and sodium phenylbutyrate and given a protein-restricted diet.
Currently, Sigmapharm's product line consists of Adefovir Dipivoxil Tablets, for hepatitis B, which is the first and only generic product equivalent to Hepsera tablets; Sodium Phenylbutyrate Powder, for urea cycle disorders, which is equivalent to Buphenyl powder; Acitretin Capsules, which is equivalent to Soriatane capsules, with a primary indication for the treatment of severe psoriasis; and Liothyronine Sodium Tablets, for hypothyroidism, which is the most stable Liothyronine product on the market, with a recently Food and Drug Administration-approved shelf life of five years.
Acer said it also intends to use the financing to support manufacturing and clinical development of ACER-001, a proprietary taste-masked, immediate-release formulation of sodium phenylbutyrate (NaPBA) for Maple Syrup Urine Disease (MSUD) and Urea Cycle Disorder (UCD), serious genetic diseases which impact amino acid and ammonia metabolism, respectively.
* Botanically Enhanced Medicinal Mushrooms, a multivitamin nutrient supplement, coenzyme QIOfCoQIO), omega 3, vitamin D3, melatonin, Prolysin C, artemesinin, sodium phenylbutyrate, curcumin, green tea extract, quercetin, feverfew, and oral homeopathics
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