黑朦

A study has identified a gene responsible for Leber Congenital Amaurosis, the most common cause of congenital blindness in infants and small children.

最新的一项研究确定了一个与先天性黑朦，一种婴儿和低龄儿童最常见先天性失明的原因，相关的基因。

AIM: To explore the cause of peribulbar anaesthesia causing amaurosis fugax.

目的：探讨球周麻醉致黑朦的原因。

Gene therapy restores vision in dogs with Leber congenital amaurosis.

恢复视力的基因治疗对犬莱伯先天性黑朦。

The test subjects suffer from Leber congenital amaurosis, a form of hereditary retinal degeneration that occurs in infants and young children and is relatively rare.

接受试验的患者患有Leber先天性黑朦，这是一种遗传性视网膜病变，该病相对少见，发生在婴儿和少儿身上。

Most faint has dazed, bosom first before happen frowsty, at the moment the symptom such as amaurosis, what not be like little show effect is abrupt happen, the refreshment of consciousness and physical strength is slower also.

大多数的晕厥在发生前先有头昏、胸闷、眼前黑朦等症状，不似小发作的突然发生，意识和体力的恢复也比较缓慢。

RESULTS: Of the 74 patients, 23 (31%) had ocular manifestations accompanied by other symptoms, including 3 (4.1%) with ocular syndrome accompanied with headache as the first symptoms of Moyamoya disease. Digital subtract angiography examination revealed 24 patients (32.4%) had unilateral or double stenosis of internal carotid artery, among which 3 patients had ocular syndrome. All positive eye findings included amaurosis fugax (13%), hemianopia (17%), decline or loss of the visual ability and lesions of visual fields (44%), papillary size dysfunction (13%), pupil direct/indirect light reflex (13%), ptosis (9%), nystagmus (4%), ocular dyscinesia (13%), and fossa orbitalis tenderness (9%).

结果：74例Moyamoya病患者中眼部症状与其它症状同时发生者23例（31%），其中仅以眼部症状伴头痛表现为首发症状就诊者3例（4%）；DSA检查显示单侧或双侧颈内动脉狭窄者24例（32%），其中3例患者双侧颈内动脉完全闭塞，而无任何眼部症状；23例患者所伴眼症表现主要为一过性黑朦13%，偏盲17%，视力下降甚至丧失44%，瞳孔大小异常13%，瞳孔直接/间接对光反射消失13%，上睑下垂9%，眼球震颤4%，眼球运动障碍13%，眼眶压痛9%。

Dizziness, headache, tinnitus, vision loss, behavioral abnormalities, unresponsiveness, memory decline, involuntary drooling mouth, side of limb numbness, inability or activity problems, palpitation, chest tightness, chest pain, amaurosis eyes, cough, dyspnea activity, paroxysmal nocturnal dyspnea, lower extremity edema and so on.

头晕，头痛，耳鸣，视力减退，行为异常，反应迟钝，记忆力下降，嘴角不自主流口水，一侧肢体麻木、无力或活动不灵，心慌，胸闷，胸痛，眼前黑朦，咳嗽、活动时气急，夜间阵发性呼吸困难，下肢浮肿等。

Familiar inducement were prolonged standing(43.64%),change of position(20.00%),and so on.Common prodromes were dizziness(78.18%),impotence(29.09%),pallor(27.27%),sweating(27.27%),palpitation(25.45%),amaurosis(23.64%),dyspnea(21.82%),headache(21.82%)and so on.

结果74.55%和96.36%的VVS儿童存在晕厥诱因和先兆症状，常见诱因有长久站立(43.64%)、体位改变(20.00%)等；常见先兆症状为头晕(78.18%)、乏力(29.09%)、面色苍白(27.27%)、大汗(27.27%)、心慌(25.45%)、黑朦(23.64%)、胸闷(21.82%)、头痛(21.82%)等。

Do you know, i need you to come back

你知道吗，我需要你回来

Yang yinshu、Wang xiangsheng、Li decang,The first discovery of haemaphysalis conicinna.

1〕 杨银书，王祥生，李德昌。安徽省首次发现嗜群血蜱。