| 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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| 영문 | congenital syphilis | 한글 | 선천매독 |
|---|---|---|---|
| 설명 | 임부가 매독에 감염되어 있으면 임신 후기에 매독균이 태반을 통해 혈행성으로 태아에 감염(수직감염)된 것을 말하다. 대부분은 유산, 사산이 되지만 출생하면 제2기 이후의 발진을 보인다. 발현시기에 따라서 ① 태아매독, ② 유아매독, ③ 만발성 선천매독으로 분류된다. ①에서는 뼈연골염, 간-지라 비대와 매독성 천포창, ②에서는 파로가성마비와 매독성 코염, ③에서는 허친슨 세징후(허친슨 치아, 속귀성 난청, 실질성 각막염)에 따라 특징이 있다. 기타 수두증, 지능발육 불량 등을 자주 볼 수 있다. 매독 혈청반응은 대부분의 경우 양성으로 나온다. 매우 드물게 간세포내에서 매독균을 무수히 볼 수 있다. 간세포 주변의 섬유화와 함께 불규칙한 흉터(hepar lobatum)를 만들 수 있다. |
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| 영문 | congenital rubella syndrome | 한글 | 선천풍진증후군 |
|---|---|---|---|
| 설명 | 임신기간 중에 산모가 풍진에 걸리면 이 풍진 바이러스는 태반을 통해서 태아에게 전달되어서 태아의 풍진감염을 일으킨다. 임신 첫 3개월 동안, 특히 임신 첫달에 태아가 풍진의 감염을 받으면, 신생아에서 선천기형, 즉 눈에서 촛점을 정확히 맞추어주는 렌즈의 역할을 하는 수정체의 혼탁(백내장), 심장기형, 귀머거리 및 심한 지능박약을 동반하는 소두증 등이 발생하는 수가 많다. |
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| 영문 | congenital heart disease | 한글 | 선천심장병 |
|---|---|---|---|
| 설명 | 선천적으로 심장의 구조에 이상이 있는 병. |
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| CEP | Congenital Erythropoetic Porphyria(= Gnther Disease; 선천성 조혈기성 Porphyria |
|---|---|
| CEP | chronic eosinophilic pneumonia; chronic erythropoietic porphyria; congenital erythropoietic porphyri... |
| AIP | Acute Intermittent Porphyria; 급성 간혈증 Porphyria |
| PCT | 1) Post-Coital Test = Sims-Hubner Test 2) Porp... |
| VP | 1) Variegate Porphyria; 발문상 Porphyria 2) Viral Protein |
| CEP | Congenital erythropoietic porphyria |
|---|---|
| AIP | Acute Intermittent Porphyria |
| HEP | Hepatoerythropoietic porphyria |
| PCT | Porphyria Cutanea Tarda |
| CCHB | Complete congenital heart block |
| 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) |
|---|---|
| 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) |
| 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) |
| porphyria | A pathological state in man and some lower animals that is often due to genetic factors, is characterised by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. (18 Nov 1997) |
| porphyria, acute intermittent | A form of hepatic porphyria (porphyria, hepatic) characterised by periodic attacks of gastrointestinal disturbances, abdominal colic, paralyses, and psychiatric disorders. The onset of this condition is usually in the third or fourth decade of life. (12 Dec 1998) |
| 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) |
| 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) |
| porphyria, erythrohepatic | A form of porphyria characterised by a wide range of photocutaneous changes, liver disease, and an excess of protoporphyrin. (12 Dec 1998) |
| porphyria, erythropoietic | Autosomal recessive porphyria characterised by splenomegaly, photosensitivity, haemolytic anaemia, and the appearance of red urine in early infancy. This condition results from increased synthesis of uroporphyrinogen I relative to uroporphyrinogen III in bone marrow normoblasts. (12 Dec 1998) |
| porphyria, hepatic | Porphyria in which the liver is the site where excess formation of porphyrin or its precursors is found. Porphyria, acute intermittent and porphyria cutanea tarda are types of hepatic porphyria. (12 Dec 1998) |
| porphyria hepatica | A category of porphyria that includes porphyria cutanea tarda, variegate porphyria, and coproporphyria. Synonym: porphyria hepatica. (05 Mar 2000) |
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|