| 영문 | iron deficiency anemia | 한글 | 철결핍빈혈 |
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| 설명 | 적혈구의 기능은 산소를 운반하는데 있다. 적혈구 속에 산소와 결합을 하여 산소를 운반하는 혈색소라는 물질이 있다. 철은 이 혈색소의 중요한 부분을 이루는 것으로 철이 없으면 혈색소가 만들어질 수가 없다. 혈색소가 없으면 역시 적혈구도 만들어지지 않으므로 체내에 철이 부족하면 빈혈이 생긴다. 이 철결핍성 빈혈은 빈혈의 원인 중에서 가장 흔한 것이다(약 25%를 차지한다). 철저장량의 저하-결핍, 혈청철농도의 저하, 트란스페린량 상승, 트란스페린포화도의 저하, 혈색소농도 또는 헤마토크리트의 저하, 저색소성대적혈구를 특징으로 하는 빈혈로서, 생체 내에서 철이 장기에 걸쳐 결핍되며 그 때문에 혈색소 생산 감소에 의해 일어난다. 창자에서의 철흡수량 부족, 철의 수요 증대(유아기, 사춘기, 임신), 철소실과잉(출혈)에 의해 일어나며, 특히 사춘기에서 폐경기까지의 여성에게 많다. 증상으로서는 얼굴창백, 피로감, 피부창백, 손톱 변화(스푼 모양) 등을 나타낸다. 구강 영역에서는 혀의 접촉통, 발적, 건조감, 삼킴곤란을 수반하면 플러머-빈슨(Plummer-Vinson)증후군이라고 한다. 혈액 소견은 혈청철은 저하하며, 철결합능력의 상승, 저색소성 작은적혈구성을 나타낸다. |
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| MD | Doctor of Medicine [Lat. Medicinae Doctor]; magnesium deficiency; main duct; maintenance dose; major... |
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| GSD | genetically significant dose; Gerstmann-Straussler disease; glutathione synthetase deficiency; glyco... |
| CPS | Carbamyl Phosphate Synthetase |
| IGD | idiopathic growth hormone deficiency; interglobal distance; isolated gonadotropin deficiency |
| MCD | magnetic circular dichroism; mast-cell degranulation; mean cell diameter; mean of consecutive differ... |
| CPS | Carbamoyl phosphate synthetase |
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| CPSase | Carbamoyl phosphate synthetase |
| CP | Carbamoyl phosphate |
| 2-5A synthetase | 2', 5'-oligoadenylate synthetase |
| OCTase | Ornithine carbamoyl transferase |
| carbamoyl phosphate synthetase | A phosphotransferase catalyzing the formation of carbamoyl phosphate. There are two significant isozymes. Carbomoyl phosphate synthetase I is a mitochondrial enzyme that catalyses the reaction of 2ATP, NH3, CO2, and H2O to carbamoyl phosphate, 2ADP, and Pi. It is activated by N-acetylglutamate and participates in urea biosynthesis. A deficiency of carbamoyl phosphate synthetase I can result in hyperammonaemia. Carbamoyl phosphate synthetase II is a cytosolic enzyme that, under physiological conditions, uses l-glutamine as the nitrogen source (producing l-glutamate) instead of NH3, is not activated by N-acetylglutamate, and is found in pyrimidine biosynthesis. (05 Mar 2000) |
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| carbamoyl phosphate | H2NCO-OPO32-;a reactive intermediate capable of transferring its carbamoyl group (H2NCO-) to an acceptor molecule, forming citrulline from ornithine in the urea cycle, and ureidosuccinic acid from aspartic acid in pyrimidine ring formation. (05 Mar 2000) |
| carbamoyl-phosphate synthase (ammonia) | <enzyme> An enzyme that catalyses the formation of carbamoyl phosphate from ATP, carbon dioxide, and ammonia. This enzyme is specific for arginine biosynthesis or the urea cycle. Chemical name: Carbon dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating) Registry number: EC 6.3.4.16 (12 Dec 1998) |
| carbamoyl-phosphate synthase (glutamine-hydrolyzing) | <enzyme> An enzyme that catalyses the formation of carbamoyl phosphate from ATP, carbon dioxide, and glutamine. This enzyme is important in the de novo biosynthesis of pyrimidines. Chemical name: Carbon dioxide:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating) Registry number: EC 6.3.5.5 (12 Dec 1998) |
| carbamoylphosphate synthetase deficiency | <biochemistry> Carbamoylphosphate synthetase is the initial enzyme of the urea cycle, catalysing the synthesis of carbamoylphosphate from ammonia, bicarbonate and ATP as the first step of ammonia detoxification. The enzyme is an intramitochondrial form called CPS I. A different isozyme found in the cytoplasm, called CPS II, is much less active and apparently not involved in the urea cycle. The deficiency state is autosomal recessive and presents in infancy with massive hyperammonaemia and neurologic deficits in survivors. Diagnosis is suggested by the blood biochemistry and confirmed by specific enzyme assay on liver or rectal biopsy. Prenatal diagnosis by molecular methods has been used successfully in informative families. Inheritance: autosomal recessive. (07 Apr 1998) |
| glutathione synthetase deficiency | An inborn error of metabolism associated with massive urinary excretion of 5-oxyproline, elevated levels of 5-oxyproline in the blood and cerebrospinal fluid, severe metabolic acidosis, tendency toward haemolysis, and defective central nervous systems function. Glutathione synthetase deficiency has been reported as a generalised condition or with a deficiency restricted to erythrocytes. (05 Mar 2000) |
| carbamoyl | The acyl radical, NH2-CO-, the transfer of which plays an important role in certain biochemical reactions; e.g., in the urea cycle, via carbamoyl phosphate. (05 Mar 2000) |
| carboxyl and carbamoyl transferases | <enzyme> A group of enzymes that catalyze the transfer of carboxyl- or carbamoyl- groups. Registry number: EC 2.1.3 (12 Dec 1998) |
| N-carbamoyl-D-amino acid amidohydrolase | <enzyme> From comamonas sp.e222c; mw 120 kD; hydrolyzes d-enantiomers of various n-carbamoyl-d-amino acids to d-amino acids, ammonia and co2; sensitive to thiol reagents; does not require metal ions Registry number: EC 3.5.1.- Synonym: caa-amidohydrolase, carbamoyl d-aa amidohyrolase (26 Jun 1999) |
| 1-carbamoyl-L-amino acid amidohydrolase | <enzyme> From bacillus stearothermophilus; amino acid sequence given in first source Registry number: EC 3.5.1.- Synonym: carbamoyl l-aa amidohydrolase, n-carbamyl-l-amino acid amidohydrolase (26 Jun 1999) |
| glucose-6-phosphate dehydrogenase deficiency | A deficiency of glucose-6-phosphate dehydrogenase, an enzyme important for maintaining cellular concentrations of reduced nucleotides. Deficiency of this enzyme is the commonest disease-causing enzyme defect in humans affecting an estimated 400 million people. The gene for this enzyme is on the X chromosome and there are various polymorphic forms. Males with the enzyme deficiency develop haemolytic anaemia when red blood cells are exposed to oxidant drugs such as the antimalarial primaquine, the sulfonamide antibiotics or sulfones, naphthalene moth balls, or fava beans. It can also cause anaemia of the newborn, and chronic nonspherocytic haemolytic anaemia. Inheritance: X-linked. (12 Sep 2002) |
| deficiency, glucose-6-phosphate dehydrogenase | Deficiency of G6PD is the commonest disease-causing enzyme defect in humans affecting an estimated 400 million people. The G6PD gene is on the X chromosome. Males with the enzyme deficiency develop anaemia due to breakup of their red blood cells when they are exposed to oxidant drugs such as the antimalarial primaquine, the sulfonamide antibiotics or sulfones, naphthalene moth balls, or fava beans. (12 Dec 1998) |
| bis(monoacylglycero)phosphate synthetase | <enzyme> Converts phosphatidylglycerol, 1-acyl- or 2-acyllysophosphatidylglycerol to bis(monoacylglycero)phosphate Registry number: EC 2.3.1.- (26 Jun 1999) |
| hexose phosphate synthetase | <enzyme> Ribulosephosphate formaldehyde gives d-erythro-l-glycero-3-hexulose Registry number: EC 4.1.2.- Synonym: 3-hexulose phosphate synthase, d-arabino-3-hexulose 6-phosphate formaldehyde-lyase, 3-hexulosephosphate synthase, d-arabino-3-hexulose-6-phosphate-lysase, hexulose-6-phosphate synthase, hump synthase (26 Jun 1999) |
| N-(5-amino-1-ribosyl-4-imidazolylcarbonyl)-L-aspartic acid 5'-phosphate synthetase | <enzyme> 5-amino-1-ribosyl-4-imidazole carboxylic acid 5'-phosphate (carboxy-air), ATP and aspartate yield n(5-amino-4-imidazolylcarbonyl)-l-aspartic acid 5'-phosphate(succino-aicar), ADP and p Registry number: EC 6.3.4.- (26 Jun 1999) |
| carbamoyl phosphate synthetase deficiency |
a genetic aminoacidopathy due to a deficiency of carbamoyl phosphate synthase (ammonia); characteristic symptoms include pronounced hyperammonemia without oroticaciduria, protein intolerance, and neurologic disorders. Symptoms may begin in the neonatal period or appear later in infancy, with varying degrees of severity. Written also carbamoyl phosphate synthetase I (CPSI) deficiency.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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