| 영문 | Klinefelter syndrome | 한글 | 클라인펠터증후군 |
|---|---|---|---|
| 설명 | 1942년 H.F. 클라인펠터가 기재한 성염색체이상증후군. 정상인의 성염색체형은 남성 XY, 여성 XX를 나타내지만, 이 증후군에서는 성염색체형이 XXY. XXYY, XXXXY 등의 여러 가지 이상한 형태를 나타낸다. 외성기-체격-성징 등의 특징적인 증세로 볼 때에 완전한 남성이 결혼하여 성생활까지 하였으나, 자식이 없자 부부가 함께 병원을 찾아가서 염색체를 검사해 보고 남자에게 이 증후군이 있음을 알게 되는 경우가 많다. 이 밖에 성인이 되어 나타나는 주요 증세를 들면, 작은고환, 여성형 유방증, 무정자증, 불임, 요중 고나도트로핀의 상승, 지능 저하 등이다. 치료는 2차 성징의 촉진을 위하여 호르몬요법에 의한 남성화를 시도한다. |
||
| 영문 | fetal alcohol syndrome | 한글 | 태아알코올증후군 |
|---|---|---|---|
| 설명 | 임신기간 중 만성적으로 알코올을 섭취한 여자에게서 태어난 영아에게 나타나는 형태발생의 이상을 나타내는 증후군으로서 위턱뼈발육부전, 앞머리와 아래턱의 돌출, 짧은검열, 작은안구증, 눈구석주름, 심한 성장지연, 정신지체 등을 나타낸다. |
||
| 영문 | Horner syndrome | 한글 | 호르너증후군 |
|---|---|---|---|
| 설명 | 교감신경경로의 장애로 생기는 병이다. 교감신경은 자율신경의 하나로 온몸에 분포를 한다. 특히 얼굴쪽에는 눈꺼플을 올리는 근육과 땀샘에 분포하고 있다. 이와 같은 교감신경의 작용으로 눈꺼풀을 정상적으로 올리고 얼굴에 땀이 나오게 된다. 그 외에도 눈의 빛의 양을 조절하는 홍채를 수축시키는 근육에 분포해서 그 작용으로 눈의 홍채가 수축하여 동공이 커지게 된다. 교감신경은 그 기원이 대뇌속에 존재하는 시상하부라는 곳이고 이곳에서 시작한 교감신경은 척수를 타고 내려와서 목부위에서 척수를 빠져나와서 교감신경을 줄기를 형성하여 다시 뇌로 가는 혈관을 따라서 얼굴쪽으로 가게된다. 만약 이 교감신경의 주행부위에 병변이 생기면 그쪽의 얼굴에 교감신경이 차단되므로 병터쪽의 눈의 눈꺼풀쳐짐, 축동 그리고 병변측 얼굴부의 땀이 나지 않는 것 등의 증상을 나타나게 된다. 이런 현상을 호르너증후군이라고 한다. 이것은 여러 가지 병에서 나타날 수가 있는데 뇌나 척수의 질환중에서 이 교감신경로를 압박하거나 침범하는 병에서 생길 수도 있으며, 또는 폐암이 척수에서 빠져나와 목부분에서 이룬 교감신경의 줄기를 누를 경우에도 생길 수도 있다. |
||
| 영문 | respiratory distress syndrome(RDS) | 한글 | 호흡곤란증후군 |
|---|---|---|---|
| 설명 | 폐포와 폐모세혈관 사이에 부종으로 인한 확산능 감소로 호흡곤란과 청색증을 보이는 상태로 감염, 수술, 외상 등 모든 종류의 스트레스상황에서 발생할 수 있다. 치료는 선행 요인의 교정과 적절한 혈액내 산소농도 유지이다. |
||
| ABBQ | Acquired Immunodeficiency Syndrome Beliefs and Behavior Questionnaire |
|---|---|
| AIDSLINE | on-line information on acquired immunodeficiency syndrome [MEDLARS data base] |
| AIDSTRIALS | clinical trials of acquired immunodeficiency syndrome drugs [MEDLARS data base] |
| ARV | acquired immunodeficiency syndrome-related virus; anterior right ventricle; avian reovirus |
| ASHAC | acquired immunodeficiency syndrome self-help and care |
| congenital dysplasia of the hip | A malformation of the hip joint that is present at birth. Genetic factors likely play a role in this disorder. Features include hip dislocation, asymmetry of leg positions, asymmetric fat folds and diminished movement on the affected side. Some children will exhibit little or no features and must be diagnosed by physical examination of the hip joints. (27 Sep 1997) |
|---|---|
| congenital dysplastic angiectasia | <syndrome> A congenital malformation syndrome characterised by the triad of asymmetric limb hypertrophy, haemangiomata, and nevi. Asymmetric limb hypertrophy is enlargement of one limb and not the corresponding limb on the other side, the enlarged limb being 3 times more likely to be a leg than an arm in ktw; and the limb enlargement is of bone as well as soft tissue. The haemangiomas, abnormal nests of blood vessels that proliferate inappropriately and excessively, cover a remarkable range from small innocuous capillary haemangiomas ( strawberry marks ) to huge cavernous haemangiomas. The nevi are pigmented moles on the skin; in ktw there are often also dark linear streaks on the skin, streaks due to too much pigment. There can be other abnormalities but the triad is the consistent clinical centrepiece of the disease. most persons with ktw have an enlarged leg and do relatively well without treatment or, for example, with only compression from an elastic stocking. Skin ulcers and other skin problems can occur over the swollen leg. Usually, the treatment is conservative. Surgery is almost never needed. The only possible exceptions are the very rare situations in which the leg reaches gigantic proportions or secondary clotting difficulties arise (due to trapping and destruction of blood platelets in a huge haemangioma). Then, amputation may become necessary. The cause of ktw syndrome is unknown. (12 Dec 1998) |
| congenital dysplastic angiomatosis | Autosomal dominant angiomatosis in which there is dysplasia of the underlying tissues, sometimes with overgrowth of bone (Klippel-Trenaunay-Weber syndrome), or encephalotrigeminal angiomatosis (Sturge-Weber syndrome) in which there is an angioma in the distribution of one or more branches of the trigeminal nerve, with vascular anomalies and calcification of the cerebral cortex. (05 Mar 2000) |
| congenital ectodermal defect | Incomplete development of the epidermis and skin appendages; the skin is smooth and hairless, the facies abnormal, and the teeth and nails may be affected; sweating may be deficient. Synonym: congenital ectodermal dysplasia. (05 Mar 2000) |
| congenital ectodermal dysplasia | Incomplete development of the epidermis and skin appendages; the skin is smooth and hairless, the facies abnormal, and the teeth and nails may be affected; sweating may be deficient. Synonym: congenital ectodermal dysplasia. (05 Mar 2000) |
| congenital elephantiasis | Congenital enlargement of one or more of the limbs or other parts, due to dilation of the lymphatics. (05 Mar 2000) |
| congenital epulis of newborn | A congenital benign nodular tumour of the alveolar ridge, of unknown histogenesis; histologically, it is composed of large cells with a granular cytoplasm similar to that of a granular cell tumour (myoblastoma). (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) |
| congenital facial diplegia | <syndrome> A developmental bilateral facial paralysis usually associated with oculomotor or other neurological disorders. Synonym: congenital facial diplegia. (05 Mar 2000) |
| congenital fibrosis of the extraocular muscles | An autosomal dominant disorder associated with blepharoptosis and absence of eye movements. (05 Mar 2000) |
| congenital generalised fibromatosis | Multiple subcutaneous and visceral fibrous tumours present at birth; a rare disorder often fatal in the first week of life, although sometimes undergoing spontaneous remission; probable autosomal recessive inheritance. (05 Mar 2000) |
| congenital giant pigmented nevus | These large pigmented (often hairy) congenital nevi are important because of their increased risk (10 to 15%) of conversion into malignant melanoma. A biopsy can confirm if cells have turned malignant. Any change in a pre-existing nevus should prompt a physician evaluation. (27 Sep 1997) |
| congenital glaucoma | An affection of infancy, marked by an increase of intraocular pressure with enlargement of the eyeball. Synonym: congenital glaucoma, hydrophthalmia, hydrophthalmos, hydrophthalmus. Origin: G. Bous, ox, + ophthalmos, eye (05 Mar 2000) |
| congenital haemolytic anaemia | Accelerated destruction of red blood cells due to an inherited defect, such as in the membrane in hereditary spherocytosis. (05 Mar 2000) |
| congenital haemolytic icterus | <haematology> A hereditary disorder that leads to a chronic haemolytic anaemia due to an abnormality in the red blood cell membrane. This disorder is caused by a defective gene. Red cells are resistant to stress and rupture easily. Infants may appear jaundiced and pale. Fatigue, weakness and shortness of breath are other symptoms that may be seen in older patients. The spleen may also be enlarged. Treatment includes splenectomy (removal of the spleen). After this is accomplished the life-span of the red blood cells returns to normal. (27 Sep 1997) |