| 영문 | porphyria | 한글 | 포르피린증 |
|---|---|---|---|
| 설명 | 포르피린 또는 그 전구물질의 형성이나 배설의 현저한 증가를 특징으로 하는 포르피린 대사장애에 대한 총칭. 네 개의 피롤핵이 메틸기로 연결된 포피린유도체의 총칭이다. 측쇄에 메틸기, 에틸기, 비닐기, 프로피온산기 등이 들어간 유로포피린, 코프로포피린, 프로토포피린, 헤마토포피린 등이 알려져 있다. 포르피린환에 Fe2+가 들어간 GPA은 글로빈과 결합하여 혈색소를 구성한다. 철포르피린으로서는 헤모글로빈, 시토크롬, 카타라아제 등이 있고, Mg2+를 갖는 것으로서는 엽록소가 있다. 생체조직 중, 주로 골수의 어린 적혈구 그리고 간에서 이루어지는 헴합성의 중간대사물질로, 혈색소나 각종 헴단백의 소재가 된다. 헴합성 이상에 의해 적혈구, 혈장, 소변, 대변의 포르피린체가 증가한다. 헴합성의 이상은 각 형의 포피리아, 빈혈, 납중독 등에서 볼 수 있다. |
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| PCT | 1) Post-Coital Test = Sims-Hubner Test 2) Porp... |
|---|---|
| PTC | 1) Percutaneous Transhepatic Cholangiography = PTHC ... |
| PCT | peripheral carcinoid tumor; plasma clotting time; plasmacrit test; plasmacytoma; polychlorinated tri... |
| AIP | Acute Intermittent Porphyria; 급성 간혈증 Porphyria |
| CEP | Congenital Erythropoetic Porphyria(= Gnther Disease; 선천성 조혈기성 Porphyria |
| PCT | Porphyria Cutanea Tarda |
|---|---|
| AIP | Acute Intermittent Porphyria |
| CEP | Congenital erythropoietic porphyria |
| HEP | Hepatoerythropoietic porphyria |
| AHO | Albright hereditary osteodystrophy |
| porphyria cutanea tarda | A form of hepatic porphyria (porphyria, hepatic) characterised by photosensitivity resulting in bullae that rupture easily to form shallow ulcers. This condition occurs in two forms: a sporadic, nonfamilial form that begins in middle age and has normal amounts of uroporphyrinogen decarboxylase with diminished activity in the liver; and a familial form in which there is an autosomal dominant inherited deficiency of uroporphyrinogen decarboxylase in the liver and red blood cells. (12 Dec 1998) |
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| porphyria cutanea tarda hereditaria | A form of hepatic porphyria (porphyria, hepatic) characterised by photosensitivity resulting in bullae that rupture easily to form shallow ulcers. This condition occurs in two forms: a sporadic, nonfamilial form that begins in middle age and has normal amounts of uroporphyrinogen decarboxylase with diminished activity in the liver; and a familial form in which there is an autosomal dominant inherited deficiency of uroporphyrinogen decarboxylase in the liver and red blood cells. (12 Dec 1998) |
| porphyria cutanea tarda symptomatica | A form of hepatic porphyria (porphyria, hepatic) characterised by photosensitivity resulting in bullae that rupture easily to form shallow ulcers. This condition occurs in two forms: a sporadic, nonfamilial form that begins in middle age and has normal amounts of uroporphyrinogen decarboxylase with diminished activity in the liver; and a familial form in which there is an autosomal dominant inherited deficiency of uroporphyrinogen decarboxylase in the liver and red blood cells. (12 Dec 1998) |
| vena cutanea | <anatomy, vein> One of a number of veins that course in the subcutaneous tissue and empty into deep veins; they form prominent systems of vessels in the limbs and are usually not accompanied by arteries. Synonym: vena cutanea, cutaneous vein. (05 Mar 2000) |
| sclerosis cutanea | Synonym: scleroderma. (05 Mar 2000) |
| rachitis tarda | <pathology> A condition marked by softening of the bones (due to impaired mineralisation, with excess accumulation of osteoid), with pain, tenderness, muscular weakness, anorexia and loss of weight, resulting from deficiency of vitamin D and calcium. Origin: Gr. Malakia = softness (18 Nov 1997) |
| neurosis tarda | Neurotic patterns developing in older people, related to organic cerebral lesions. (05 Mar 2000) |
| dentia tarda | Delayed tooth eruption. Origin: L. Delayed (05 Mar 2000) |
| syphilis hereditaria tarda | Syphilis, believed to be congenital, but not manifesting itself until several years after birth. (05 Mar 2000) |
| acute intermittent porphyria | <gastroenterology, haematology> A group of rare inherited metabolic disorders that result from a disturbance in porphyrin metabolism, causing increased formation and excretion of porphyrin or its precursors. It is caused by hepatic overproduction of d-aminolevulinic acid, which has greatly increased urinary excretion and of porphobilinogen, and some increase of uroporphyrin, due to a deficiency of porphobilinogen deaminase. Clinical features: intermittent acute attacks of hypertension, abdominal colic, psychosis, and polyneuropathy, but with no photosensitivity. It is exacerbated by the ingestion of certain drugs such as; barbiturates). Inheritance: autosomal dominant. (20 Sep 2002) |
| acute porphyria | <gastroenterology, haematology> A group of metabolic disorders that result from a disturbance in porphyrin metabolism, causing increased formation and excretion of porphyrin or its precursors. Acute intermittent porphyria is a rare inherited (autosomal dominant) form that can result in abdominal pain, photosensitivity and neurological disturbances. The various forms can be differntiated measuring various blood prophyrins. (27 Sep 1997) |
| bovine porphyria | Porphyria as a mendelian recessive trait in certain breeds of cattle. (05 Mar 2000) |
| variegate porphyria | Porphyria characterised by abdominal pain and neuropsychiatric abnormalities, by dermal sensitivity to light and mechanical trauma, by increased faecal excretion of proto-and coproporphyrin, and by increased urinary excretion of d-aminolevulinic acid, porphobilinogen, and porphyrins; due to a deficiency of protoporphyrinogen oxidase; autosomal dominant inheritance. Synonym: protocoproporphyria hereditaria, South African type porphyria. (05 Mar 2000) |
| congenital erythropoietic porphyria | A group of metabolic disorders that result from a disturbance in porphyrin metabolism, causing increased formation and excretion of porphyrin or its precursors. Acute intermittent porphyria is a rare inherited (autosomal dominant) form that can result in abdominal pain, photosensitivity and neurological disturbances. The various forms can be differntiated measuring various blood prophyrins. Inheritance: autosomal dominant. (27 Sep 1997) |
| porphobilinogen synthase porphyria | An inherited disorder in which there is a deficiency of porphobilinogen synthase; d-aminolevulinate levels are elevated, leading to neurological disturbances. Synonym: porphobilinogen synthase porphyria. (05 Mar 2000) |
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|