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主要症状为胸痛，可牵涉到腹部、肩部和背部。 | [
{
"indices": [
5,
6
],
"label": "sym",
"score": 1,
"text": "胸痛"
},
{
"indices": [
5
],
"label": "bod",
"score": 1,
"text": "胸"
},
{
"indices": [
12,
13
],
"label": "bod",
"score": 1,
"text": "腹部"
},
{
"indices": [
15,
16
],
"label": "bod",
"score": 1,
"text": "肩部"
},
{
"indices": [
18,
19
],
"label": "bod",
"score": 1,
"text": "背部"
}
] |
患儿喜患侧卧位，患侧呼吸运动受限制、听诊呼吸音减弱。 | [
{
"indices": [
2,
3,
4,
5,
6
],
"label": "sym",
"score": 1,
"text": "喜患侧卧位"
},
{
"indices": [
3,
4
],
"label": "bod",
"score": 1,
"text": "患侧"
},
{
"indices": [
8,
9,
10,
11,
12,
13,
14,
15,
16
],
"label": "sym",
"score": 1,
"text": "患侧呼吸运动受限制"
},
{
"indices": [
8,
9
],
"label": "bod",
"score": 1,
"text": "患侧"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24
],
"label": "sym",
"score": 1,
"text": "听诊呼吸音减弱"
},
{
"indices": [
18,
19
],
"label": "pro",
"score": 1,
"text": "听诊"
}
] |
腹痛明显者，尚须排除急性肠系膜淋巴结炎、阑尾炎。 | [
{
"indices": [
0,
1,
2,
3
],
"label": "sym",
"score": 1,
"text": "腹痛明显"
},
{
"indices": [
0
],
"label": "bod",
"score": 1,
"text": "腹"
},
{
"indices": [
10,
11,
12,
13,
14,
15,
16,
17,
18
],
"label": "dis",
"score": 1,
"text": "急性肠系膜淋巴结炎"
},
{
"indices": [
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "阑尾炎"
}
] |
可适当给镇痛剂止痛。 | [
{
"indices": [
4,
5,
6
],
"label": "dru",
"score": 1,
"text": "镇痛剂"
},
{
"indices": [
8
],
"label": "sym",
"score": 1,
"text": "痛"
}
] |
环境因素对尿道发育形成影响也主要是通过母亲和胎儿的内分泌系统。 | [
{
"indices": [
5,
6
],
"label": "bod",
"score": 1,
"text": "尿道"
},
{
"indices": [
25,
26,
27,
28,
29
],
"label": "bod",
"score": 1,
"text": "内分泌系统"
}
] |
本病的狭窄程度决定临床严重性；大多患儿无症状，胸骨左缘上部有一喷射性收缩期杂音，并向腋下及背面传导；如伴有收缩早期喀喇音，提示有肺动脉瓣狭窄同时存在，但肺动脉瓣关闭音可增强，提示瓣膜狭窄后还有梗阻存在。 | [
{
"indices": [
23,
24,
25,
26,
27,
28
],
"label": "bod",
"score": 1,
"text": "胸骨左缘上部"
},
{
"indices": [
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38
],
"label": "sym",
"score": 1,
"text": "胸骨左缘上部有一喷射性收缩期杂音"
},
{
"indices": [
42,
43,
44,
45,
46
],
"label": "bod",
"score": 1,
"text": "腋下及背面"
},
{
"indices": [
53,
54,
55,
56,
57,
58,
59
],
"label": "sym",
"score": 1,
"text": "收缩早期喀喇音"
},
{
"indices": [
64,
65,
66,
67,
68,
69
],
"label": "dis",
"score": 1,
"text": "肺动脉瓣狭窄"
},
{
"indices": [
76,
77,
78,
79
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
},
{
"indices": [
76,
77,
78,
79,
80,
81,
82,
83,
84,
85
],
"label": "sym",
"score": 1,
"text": "肺动脉瓣关闭音可增强"
},
{
"indices": [
89,
90,
91,
92
],
"label": "dis",
"score": 1,
"text": "瓣膜狭窄"
},
{
"indices": [
96,
97
],
"label": "dis",
"score": 1,
"text": "梗阻"
}
] |
胸部X线上多属正常，但有时可见某侧或某段肺野血管影特少；心电图上右心室肥厚的程度可反映狭窄的严重性；二维超声可显示肺动脉主干及其近支的解剖。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "pro",
"score": 1,
"text": "胸部X线上"
},
{
"indices": [
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "某侧或某段肺野血管影特少"
},
{
"indices": [
20,
21,
22,
23
],
"label": "bod",
"score": 1,
"text": "肺野血管"
},
{
"indices": [
28,
29,
30,
31
],
"label": "pro",
"score": 1,
"text": "心电图上"
},
{
"indices": [
32,
33,
34
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
43,
44
],
"label": "sym",
"score": 1,
"text": "狭窄"
},
{
"indices": [
50,
51,
52,
53
],
"label": "pro",
"score": 1,
"text": "二维超声"
},
{
"indices": [
57,
58,
59,
60,
61,
62,
63,
64,
65
],
"label": "bod",
"score": 1,
"text": "肺动脉主干及其近支"
}
] |
治疗可用球囊导管予以扩张，支架的应用可防止扩张后再狭窄，局部的严重狭窄可以进行手术治疗。 | [
{
"indices": [
4,
5,
6,
7
],
"label": "equ",
"score": 1,
"text": "球囊导管"
},
{
"indices": [
13,
14
],
"label": "equ",
"score": 1,
"text": "支架"
},
{
"indices": [
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "扩张后再狭窄"
},
{
"indices": [
28,
29,
30,
31,
32,
33,
34
],
"label": "sym",
"score": 1,
"text": "局部的严重狭窄"
},
{
"indices": [
39,
40,
41,
42
],
"label": "pro",
"score": 1,
"text": "手术治疗"
}
] |
主要症状为咳嗽伴气急。 | [
{
"indices": [
5,
6
],
"label": "sym",
"score": 1,
"text": "咳嗽"
},
{
"indices": [
8,
9
],
"label": "sym",
"score": 1,
"text": "气急"
}
] |
当血清胆红素超过生理性黄疸的水平，临床诊断为高胆红素血症（高胆）。 | [
{
"indices": [
1,
2,
3,
4,
5
],
"label": "ite",
"score": 1,
"text": "血清胆红素"
},
{
"indices": [
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "生理性黄疸"
},
{
"indices": [
22,
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "高胆红素血症"
},
{
"indices": [
29,
30
],
"label": "dis",
"score": 1,
"text": "高胆"
}
] |
分为早发型（母乳喂养性黄疸）和晚发型（母乳性黄疸）。 | [
{
"indices": [
6,
7,
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "母乳喂养性黄疸"
},
{
"indices": [
19,
20,
21,
22,
23
],
"label": "dis",
"score": 1,
"text": "母乳性黄疸"
}
] |
喂奶最好在每天10次以上，血清胆红素达到光疗指征时可光疗。 | [
{
"indices": [
13,
14,
15,
16,
17
],
"label": "bod",
"score": 1,
"text": "血清胆红素"
},
{
"indices": [
20,
21
],
"label": "pro",
"score": 1,
"text": "光疗"
},
{
"indices": [
26,
27
],
"label": "pro",
"score": 1,
"text": "光疗"
}
] |
主要有新生儿肝炎和胆道闭锁。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "新生儿肝炎"
},
{
"indices": [
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "胆道闭锁"
}
] |
3个月后可逐渐发展至肝硬化。 | [
{
"indices": [
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "肝硬化"
}
] |
如感染伴有溶血，则可出现贫血。 | [
{
"indices": [
1,
2
],
"label": "dis",
"score": 1,
"text": "感染"
},
{
"indices": [
5,
6
],
"label": "sym",
"score": 1,
"text": "溶血"
},
{
"indices": [
6
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
12,
13
],
"label": "dis",
"score": 1,
"text": "贫血"
}
] |
治疗主要是积极控制感染，加强支持疗法。 | [
{
"indices": [
9,
10
],
"label": "dis",
"score": 1,
"text": "感染"
},
{
"indices": [
14,
15,
16,
17
],
"label": "pro",
"score": 1,
"text": "支持疗法"
}
] |
其临床表现多种多样，以血尿最为常见。 | [
{
"indices": [
11,
12
],
"label": "sym",
"score": 1,
"text": "血尿"
},
{
"indices": [
11
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
12
],
"label": "bod",
"score": 1,
"text": "尿"
}
] |
IgA肾病可分为原发性和继发性两种类型，后者常继发于肝硬化、肠道疾病、关节炎以及疱疹性皮炎等疾病，也以肾小球系膜区显著的IgA沉积为特点。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
26,
27,
28
],
"label": "dis",
"score": 1,
"text": "肝硬化"
},
{
"indices": [
30,
31,
32,
33
],
"label": "dis",
"score": 1,
"text": "肠道疾病"
},
{
"indices": [
35,
36,
37
],
"label": "dis",
"score": 1,
"text": "关节炎"
},
{
"indices": [
40,
41,
42,
43,
44
],
"label": "dis",
"score": 1,
"text": "疱疹性皮炎"
},
{
"indices": [
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64
],
"label": "sym",
"score": 1,
"text": "肾小球系膜区显著的IgA沉积"
},
{
"indices": [
51,
52,
53,
54,
55
],
"label": "bod",
"score": 1,
"text": "肾小球系膜"
},
{
"indices": [
60,
61,
62
],
"label": "bod",
"score": 1,
"text": "IgA"
}
] |
原发性IgA肾病在世界许多地方被认为是一种最常见的肾小球肾炎，而且是导致终末期肾衰的常见原因之一。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "原发性IgA肾病"
},
{
"indices": [
25,
26,
27,
28,
29
],
"label": "dis",
"score": 1,
"text": "肾小球肾炎"
},
{
"indices": [
36,
37,
38,
39,
40
],
"label": "dis",
"score": 1,
"text": "终末期肾衰"
}
] |
另一种形式的IgA称为分泌型IgA（SIgA），存在于人的外分泌物中，如唾液、眼泪、肠内分泌物以及初乳中。 | [
{
"indices": [
6,
7,
8
],
"label": "bod",
"score": 1,
"text": "IgA"
},
{
"indices": [
11,
12,
13,
14,
15,
16
],
"label": "bod",
"score": 1,
"text": "分泌型IgA"
},
{
"indices": [
18,
19,
20,
21
],
"label": "bod",
"score": 1,
"text": "SIgA"
},
{
"indices": [
29,
30,
31,
32
],
"label": "bod",
"score": 1,
"text": "外分泌物"
},
{
"indices": [
36,
37
],
"label": "bod",
"score": 1,
"text": "唾液"
},
{
"indices": [
39,
40
],
"label": "bod",
"score": 1,
"text": "眼泪"
},
{
"indices": [
42,
43,
44,
45,
46
],
"label": "bod",
"score": 1,
"text": "肠内分泌物"
},
{
"indices": [
49,
50,
51
],
"label": "bod",
"score": 1,
"text": "初乳中"
}
] |
IgA分子结构的这些特性在IgA肾病的发生上有重要意义。 | [
{
"indices": [
0,
1,
2
],
"label": "bod",
"score": 1,
"text": "IgA"
},
{
"indices": [
13,
14,
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
}
] |
近年报道，肾小球基底膜超微结构也有变化，10%左右的IgA肾病有基底膜变薄，究竟是合并薄基底膜病还是属于IgA肾病的继发改变尚不清楚。 | [
{
"indices": [
5,
6,
7,
8,
9,
10
],
"label": "bod",
"score": 1,
"text": "肾小球基底膜"
},
{
"indices": [
26,
27,
28,
29,
30
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
32,
33,
34,
35,
36
],
"label": "sym",
"score": 1,
"text": "基底膜变薄"
},
{
"indices": [
32,
33,
34
],
"label": "bod",
"score": 1,
"text": "基底膜"
},
{
"indices": [
41,
42,
43,
44,
45,
46,
47
],
"label": "dis",
"score": 1,
"text": "合并薄基底膜病"
},
{
"indices": [
52,
53,
54,
55,
56
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
}
] |
临床表现多样化，从仅有镜下血尿到肾病综合征，均可为起病时的表现，各临床表现型间也可在病程中相互转变，但在病程中其临床表现可相互转变。 | [
{
"indices": [
11,
12,
13,
14
],
"label": "sym",
"score": 1,
"text": "镜下血尿"
},
{
"indices": [
11,
12
],
"label": "equ",
"score": 1,
"text": "镜下"
},
{
"indices": [
13
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
14
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "肾病综合征"
}
] |
80%的儿童IgA肾病以肉眼血尿为首发症状，北美及欧洲的发生率高于亚洲，常和上呼吸道感染有关（Berger病）；与上呼吸道感染间隔很短时间（24～72小时），偶可数小时后即出现血尿。 | [
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
12,
13,
14,
15
],
"label": "sym",
"score": 1,
"text": "肉眼血尿"
},
{
"indices": [
14
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
15
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
38,
39,
40,
41,
42,
43
],
"label": "dis",
"score": 1,
"text": "上呼吸道感染"
},
{
"indices": [
47,
48,
49,
50,
51,
52,
53
],
"label": "dis",
"score": 1,
"text": "Berger病"
},
{
"indices": [
57,
58,
59,
60,
61,
62
],
"label": "dis",
"score": 1,
"text": "上呼吸道感染"
},
{
"indices": [
88,
89
],
"label": "sym",
"score": 1,
"text": "血尿"
},
{
"indices": [
88
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
89
],
"label": "bod",
"score": 1,
"text": "尿"
}
] |
且多存在扁桃体肿大，扁桃体切除后多数患者肉眼血尿停止发作。 | [
{
"indices": [
4,
5,
6,
7,
8
],
"label": "sym",
"score": 1,
"text": "扁桃体肿大"
},
{
"indices": [
4,
5,
6
],
"label": "bod",
"score": 1,
"text": "扁桃体"
},
{
"indices": [
10,
11,
12,
13,
14
],
"label": "pro",
"score": 1,
"text": "扁桃体切除"
},
{
"indices": [
20,
21,
22,
23
],
"label": "sym",
"score": 1,
"text": "肉眼血尿"
},
{
"indices": [
22
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
23
],
"label": "bod",
"score": 1,
"text": "尿"
}
] |
以肾病综合征为表现的IgA肾病约占15%～30%，三高一低表现突出，起病前也往往很少合并呼吸道感染。 | [
{
"indices": [
1,
2,
3,
4,
5
],
"label": "dis",
"score": 1,
"text": "肾病综合征"
},
{
"indices": [
10,
11,
12,
13,
14
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
25,
26,
27,
28,
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "三高一低表现突出"
},
{
"indices": [
44,
45,
46,
47,
48
],
"label": "dis",
"score": 1,
"text": "呼吸道感染"
}
] |
亦有部分病例表现为肾炎综合征，除血尿外，还有高血压及肾功能不全。 | [
{
"indices": [
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "肾炎综合征"
},
{
"indices": [
16,
17
],
"label": "sym",
"score": 1,
"text": "血尿"
},
{
"indices": [
16
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
17
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
22,
23,
24
],
"label": "dis",
"score": 1,
"text": "高血压"
},
{
"indices": [
26,
27,
28,
29,
30
],
"label": "dis",
"score": 1,
"text": "肾功能不全"
}
] |
高血压好发于年龄偏大者，成人占20%，儿童仅5%。 | [
{
"indices": [
0,
1,
2
],
"label": "dis",
"score": 1,
"text": "高血压"
}
] |
不足5%的IgA肾病患者表现为急进性肾炎。 | [
{
"indices": [
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
15,
16,
17,
18,
19
],
"label": "dis",
"score": 1,
"text": "急进性肾炎"
}
] |
Ⅴ级：与Ⅳ级相似但更严重，节段和/或球性硬化、玻璃样变以及球囊粘连，50%以上的肾小球有新月体，称之为弥漫硬化性肾小球肾炎。 | [
{
"indices": [
13,
14,
15,
16,
17,
18,
19,
20,
21
],
"label": "sym",
"score": 1,
"text": "节段和/或球性硬化"
},
{
"indices": [
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "玻璃样变"
},
{
"indices": [
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "球囊粘连"
},
{
"indices": [
29,
30
],
"label": "bod",
"score": 1,
"text": "球囊"
},
{
"indices": [
34,
35,
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46
],
"label": "sym",
"score": 1,
"text": "50%以上的肾小球有新月体"
},
{
"indices": [
40,
41,
42
],
"label": "bod",
"score": 1,
"text": "肾小球"
},
{
"indices": [
51,
52,
53,
54,
55,
56,
57,
58,
59,
60
],
"label": "dis",
"score": 1,
"text": "弥漫硬化性肾小球肾炎"
}
] |
IgA肾病从病理变化到临床表现都有很大差异，预后也有很大区别，因此，治疗措施必须做到个体化。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
}
] |
短期抗生素治疗对于控制急性期症状也有一定作用。 | [
{
"indices": [
2,
3,
4,
5,
6
],
"label": "pro",
"score": 1,
"text": "抗生素治疗"
}
] |
对于合并水肿、高血压的患儿，应相应给予利尿消肿，降压药物治疗，并采用低盐、低蛋白饮食。 | [
{
"indices": [
4,
5
],
"label": "sym",
"score": 1,
"text": "水肿"
},
{
"indices": [
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "高血压"
},
{
"indices": [
19,
20,
21,
22
],
"label": "pro",
"score": 1,
"text": "利尿消肿"
},
{
"indices": [
24,
25,
26,
27,
28,
29
],
"label": "pro",
"score": 1,
"text": "降压药物治疗"
},
{
"indices": [
32,
33,
34,
35,
36,
37,
38,
39,
40,
41
],
"label": "pro",
"score": 1,
"text": "采用低盐、低蛋白饮食"
}
] |
日本曾作全国范围多中心对照研究，采用泼尼松及免疫抑制治疗IgA肾病的患儿，其远期肾功能不全的比例要明显低于使用一般性治疗的患儿。 | [
{
"indices": [
18,
19,
20
],
"label": "dru",
"score": 1,
"text": "泼尼松"
},
{
"indices": [
22,
23,
24,
25,
26,
27
],
"label": "pro",
"score": 1,
"text": "免疫抑制治疗"
},
{
"indices": [
28,
29,
30,
31,
32
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
40,
41,
42,
43,
44
],
"label": "dis",
"score": 1,
"text": "肾功能不全"
}
] |
最近，Nagaoka等报道一种新型免疫抑制剂——咪唑立宾（mizoribine），用于儿童IgA肾病治疗，该药安全、易耐受，可长期服用，并能显著减少蛋白尿和血尿程度，重复肾活体组织检查证实肾组织病变程度减轻。 | [
{
"indices": [
15,
16,
17,
18,
19,
20,
21
],
"label": "dru",
"score": 1,
"text": "新型免疫抑制剂"
},
{
"indices": [
24,
25,
26,
27
],
"label": "dru",
"score": 1,
"text": "咪唑立宾"
},
{
"indices": [
29,
30,
31,
32,
33,
34,
35,
36,
37,
38
],
"label": "dru",
"score": 1,
"text": "mizoribine"
},
{
"indices": [
43,
44,
45,
46,
47,
48,
49
],
"label": "dis",
"score": 1,
"text": "儿童IgA肾病"
},
{
"indices": [
74,
75,
76
],
"label": "sym",
"score": 1,
"text": "蛋白尿"
},
{
"indices": [
74,
75
],
"label": "bod",
"score": 1,
"text": "蛋白"
},
{
"indices": [
76
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
78,
79
],
"label": "sym",
"score": 1,
"text": "血尿"
},
{
"indices": [
78
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
79
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
85,
86,
87,
88,
89,
90,
91
],
"label": "pro",
"score": 1,
"text": "肾活体组织检查"
},
{
"indices": [
94,
95,
96,
97,
98
],
"label": "dis",
"score": 1,
"text": "肾组织病变"
}
] |
中医中药治疗IgA肾病也有一定疗效，对于中等程度的蛋白尿，使用雷公藤多甙片1mg/（kg•d）治疗3个月，可获明显疗效。 | [
{
"indices": [
0,
1,
2,
3,
4,
5
],
"label": "pro",
"score": 1,
"text": "中医中药治疗"
},
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "IgA肾病"
},
{
"indices": [
25,
26,
27
],
"label": "sym",
"score": 1,
"text": "蛋白尿"
},
{
"indices": [
25,
26
],
"label": "bod",
"score": 1,
"text": "蛋白"
},
{
"indices": [
27
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
31,
32,
33,
34,
35,
36
],
"label": "dru",
"score": 1,
"text": "雷公藤多甙片"
}
] |
临床主要特征是丑陋面容、骨骼异常及运动受限、肝脾肿大和智能低下。 | [
{
"indices": [
7,
8,
9,
10
],
"label": "sym",
"score": 1,
"text": "丑陋面容"
},
{
"indices": [
9
],
"label": "bod",
"score": 1,
"text": "面"
},
{
"indices": [
12,
13,
14,
15
],
"label": "sym",
"score": 1,
"text": "骨骼异常"
},
{
"indices": [
12,
13
],
"label": "bod",
"score": 1,
"text": "骨骼"
},
{
"indices": [
17,
18,
19,
20
],
"label": "sym",
"score": 1,
"text": "运动受限"
},
{
"indices": [
22,
23,
24,
25
],
"label": "sym",
"score": 1,
"text": "肝脾肿大"
},
{
"indices": [
22
],
"label": "bod",
"score": 1,
"text": "肝"
},
{
"indices": [
23
],
"label": "bod",
"score": 1,
"text": "脾"
},
{
"indices": [
27,
28,
29,
30
],
"label": "sym",
"score": 1,
"text": "智能低下"
}
] |
目前各型黏多糖病均发现有基因突变，包括无义突变、错义突变、剪接位点突变和缺失或插入。 | [
{
"indices": [
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "黏多糖病"
},
{
"indices": [
12,
13,
14,
15
],
"label": "sym",
"score": 1,
"text": "基因突变"
},
{
"indices": [
12,
13
],
"label": "bod",
"score": 1,
"text": "基因"
},
{
"indices": [
19,
20,
21,
22
],
"label": "sym",
"score": 1,
"text": "无义突变"
},
{
"indices": [
24,
25,
26,
27
],
"label": "sym",
"score": 1,
"text": "错义突变"
},
{
"indices": [
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40
],
"label": "sym",
"score": 1,
"text": "剪接位点突变和缺失或插入"
}
] |
由于各型病情轻重不一，又有各自的临床特征，在诊断中需鉴别，主要表现为身材矮小和特殊面容，表情淡漠，头大、面部丑陋，眼裂小，眼距宽，鼻梁低平，鼻孔大，唇厚，前额和双颧突出，毛发多而发际低，颈短，大部分有角膜混浊。 | [
{
"indices": [
34,
35,
36,
37
],
"label": "sym",
"score": 1,
"text": "身材矮小"
},
{
"indices": [
39,
40,
41,
42
],
"label": "sym",
"score": 1,
"text": "特殊面容"
},
{
"indices": [
44,
45,
46,
47
],
"label": "sym",
"score": 1,
"text": "表情淡漠"
},
{
"indices": [
49
],
"label": "bod",
"score": 1,
"text": "头"
},
{
"indices": [
49,
50
],
"label": "sym",
"score": 1,
"text": "头大"
},
{
"indices": [
52,
53
],
"label": "bod",
"score": 1,
"text": "面部"
},
{
"indices": [
52,
53,
54,
55
],
"label": "sym",
"score": 1,
"text": "面部丑陋"
},
{
"indices": [
57
],
"label": "bod",
"score": 1,
"text": "眼"
},
{
"indices": [
57,
58,
59
],
"label": "sym",
"score": 1,
"text": "眼裂小"
},
{
"indices": [
61
],
"label": "bod",
"score": 1,
"text": "眼"
},
{
"indices": [
61,
62,
63
],
"label": "sym",
"score": 1,
"text": "眼距宽"
},
{
"indices": [
65,
66
],
"label": "bod",
"score": 1,
"text": "鼻梁"
},
{
"indices": [
65,
66,
67,
68
],
"label": "sym",
"score": 1,
"text": "鼻梁低平"
},
{
"indices": [
70,
71
],
"label": "bod",
"score": 1,
"text": "鼻孔"
},
{
"indices": [
70,
71,
72
],
"label": "sym",
"score": 1,
"text": "鼻孔大"
},
{
"indices": [
74
],
"label": "bod",
"score": 1,
"text": "唇"
},
{
"indices": [
74,
75
],
"label": "sym",
"score": 1,
"text": "唇厚"
},
{
"indices": [
77,
78
],
"label": "bod",
"score": 1,
"text": "前额"
},
{
"indices": [
77,
78,
79,
80,
81,
82,
83
],
"label": "sym",
"score": 1,
"text": "前额和双颧突出"
},
{
"indices": [
80,
81
],
"label": "bod",
"score": 1,
"text": "双颧"
},
{
"indices": [
85,
86
],
"label": "bod",
"score": 1,
"text": "毛发"
},
{
"indices": [
85,
86,
87,
88,
89,
90,
91
],
"label": "sym",
"score": 1,
"text": "毛发多而发际低"
},
{
"indices": [
89
],
"label": "bod",
"score": 1,
"text": "发"
},
{
"indices": [
93
],
"label": "bod",
"score": 1,
"text": "颈"
},
{
"indices": [
93,
94
],
"label": "sym",
"score": 1,
"text": "颈短"
},
{
"indices": [
100,
101
],
"label": "bod",
"score": 1,
"text": "角膜"
},
{
"indices": [
100,
101,
102,
103
],
"label": "sym",
"score": 1,
"text": "角膜混浊"
}
] |
临床有智能低下，面容丑陋，肝脾肿大，骨骼病变，心血管病变，角膜混浊和耳聋。 | [
{
"indices": [
3,
4,
5,
6
],
"label": "sym",
"score": 1,
"text": "智能低下"
},
{
"indices": [
8
],
"label": "bod",
"score": 1,
"text": "面"
},
{
"indices": [
8,
9,
10,
11
],
"label": "sym",
"score": 1,
"text": "面容丑陋"
},
{
"indices": [
13
],
"label": "bod",
"score": 1,
"text": "肝"
},
{
"indices": [
13,
14,
15,
16
],
"label": "sym",
"score": 1,
"text": "肝脾肿大"
},
{
"indices": [
14
],
"label": "bod",
"score": 1,
"text": "脾"
},
{
"indices": [
18,
19,
20,
21
],
"label": "dis",
"score": 1,
"text": "骨骼病变"
},
{
"indices": [
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "心血管病变"
},
{
"indices": [
29,
30
],
"label": "bod",
"score": 1,
"text": "角膜"
},
{
"indices": [
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "角膜混浊"
},
{
"indices": [
34
],
"label": "bod",
"score": 1,
"text": "耳"
},
{
"indices": [
34,
35
],
"label": "sym",
"score": 1,
"text": "耳聋"
}
] |
ⅢA型为乙酰肝素-N-硫酸酯酶缺乏，ⅢB型为α-N-乙酰氨基葡萄糖苷酶缺乏，ⅢC型为α-氨基葡萄糖乙酰转移酶缺乏，ⅢD型为N-乙酰氨基葡萄糖硫酸酯酶缺乏。 | [
{
"indices": [
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16
],
"label": "dis",
"score": 1,
"text": "乙酰肝素-N-硫酸酯酶缺乏"
},
{
"indices": [
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36
],
"label": "dis",
"score": 1,
"text": "α-N-乙酰氨基葡萄糖苷酶缺乏"
},
{
"indices": [
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55
],
"label": "dis",
"score": 1,
"text": "α-氨基葡萄糖乙酰转移酶缺乏"
},
{
"indices": [
61,
62,
63,
64,
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75
],
"label": "dis",
"score": 1,
"text": "N-乙酰氨基葡萄糖硫酸酯酶缺乏"
}
] |
临床上患儿在1岁内发育尚正常，以后逐渐出现语言、行为障碍，生长发育落后，在儿童期神经系统退行性病变较明显，有肝脾肿大，疝气，面容粗陋，关节强直等。 | [
{
"indices": [
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27
],
"label": "sym",
"score": 1,
"text": "逐渐出现语言、行为障碍"
},
{
"indices": [
29,
30,
31,
32,
33,
34
],
"label": "sym",
"score": 1,
"text": "生长发育落后"
},
{
"indices": [
40,
41,
42,
43
],
"label": "bod",
"score": 1,
"text": "神经系统"
},
{
"indices": [
40,
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51
],
"label": "sym",
"score": 1,
"text": "神经系统退行性病变较明显"
},
{
"indices": [
54
],
"label": "bod",
"score": 1,
"text": "肝"
},
{
"indices": [
54,
55,
56,
57
],
"label": "sym",
"score": 1,
"text": "肝脾肿大"
},
{
"indices": [
55
],
"label": "bod",
"score": 1,
"text": "脾"
},
{
"indices": [
59,
60
],
"label": "sym",
"score": 1,
"text": "疝气"
},
{
"indices": [
62
],
"label": "bod",
"score": 1,
"text": "面"
},
{
"indices": [
62,
63,
64,
65
],
"label": "sym",
"score": 1,
"text": "面容粗陋"
},
{
"indices": [
67,
68
],
"label": "bod",
"score": 1,
"text": "关节"
},
{
"indices": [
67,
68,
69,
70
],
"label": "sym",
"score": 1,
"text": "关节强直"
}
] |
有明显的生长障碍，骨骼畸形，X线呈典型的黏多糖病表现，脊椎有鸟嘴样突出改变，椎骨扁平，飘带肋骨，鸡胸等，面容丑陋，鼻矮、口大、牙齿发育不良，角膜混浊。 | [
{
"indices": [
1,
2,
3,
4,
5,
6,
7
],
"label": "sym",
"score": 1,
"text": "明显的生长障碍"
},
{
"indices": [
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "骨骼畸形"
},
{
"indices": [
14,
15
],
"label": "pro",
"score": 1,
"text": "X线"
},
{
"indices": [
20,
21,
22,
23
],
"label": "dis",
"score": 1,
"text": "黏多糖病"
},
{
"indices": [
27,
28,
29,
30,
31,
32,
33,
34,
35,
36
],
"label": "sym",
"score": 1,
"text": "脊椎有鸟嘴样突出改变"
},
{
"indices": [
27,
28
],
"label": "bod",
"score": 1,
"text": "脊椎"
},
{
"indices": [
38,
39,
40,
41
],
"label": "sym",
"score": 1,
"text": "椎骨扁平"
},
{
"indices": [
38,
39
],
"label": "bod",
"score": 1,
"text": "椎骨"
},
{
"indices": [
43,
44,
45,
46
],
"label": "sym",
"score": 1,
"text": "飘带肋骨"
},
{
"indices": [
45,
46
],
"label": "bod",
"score": 1,
"text": "肋骨"
},
{
"indices": [
48,
49
],
"label": "sym",
"score": 1,
"text": "鸡胸"
},
{
"indices": [
49
],
"label": "bod",
"score": 1,
"text": "胸"
},
{
"indices": [
52,
53,
54,
55
],
"label": "sym",
"score": 1,
"text": "面容丑陋"
},
{
"indices": [
57,
58
],
"label": "sym",
"score": 1,
"text": "鼻矮"
},
{
"indices": [
57
],
"label": "bod",
"score": 1,
"text": "鼻"
},
{
"indices": [
60,
61
],
"label": "sym",
"score": 1,
"text": "口大"
},
{
"indices": [
60
],
"label": "bod",
"score": 1,
"text": "口"
},
{
"indices": [
63,
64,
65,
66,
67,
68
],
"label": "dis",
"score": 1,
"text": "牙齿发育不良"
},
{
"indices": [
70,
71,
72,
73
],
"label": "sym",
"score": 1,
"text": "角膜混浊"
},
{
"indices": [
70,
71
],
"label": "bod",
"score": 1,
"text": "角膜"
}
] |
青春发育可正常，随年龄增长出现脊髓压迫症状，晚期出现压迫性截瘫和呼吸麻痹。 | [
{
"indices": [
15,
16,
17,
18,
19,
20
],
"label": "sym",
"score": 1,
"text": "脊髓压迫症状"
},
{
"indices": [
15,
16
],
"label": "bod",
"score": 1,
"text": "脊髓"
},
{
"indices": [
26,
27,
28,
29,
30
],
"label": "sym",
"score": 1,
"text": "压迫性截瘫"
},
{
"indices": [
32,
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "呼吸麻痹"
}
] |
黏多糖病Ⅳ型有两种亚型，黏多糖病Ⅳ型A为半乳糖胺-6-硫酸酯酶缺乏，黏多糖病Ⅳ型B为β-半乳糖苷酶缺乏，导致硫酸角质素和硫酸软骨素降解障碍，导致这些物质在细胞与组织中积聚。 | [
{
"indices": [
0,
1,
2,
3
],
"label": "dis",
"score": 1,
"text": "黏多糖病"
},
{
"indices": [
12,
13,
14,
15
],
"label": "dis",
"score": 1,
"text": "黏多糖病"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32
],
"label": "dis",
"score": 1,
"text": "半乳糖胺-6-硫酸酯酶缺乏"
},
{
"indices": [
34,
35,
36,
37
],
"label": "dis",
"score": 1,
"text": "黏多糖病"
},
{
"indices": [
42,
43,
44,
45,
46,
47,
48,
49,
50
],
"label": "dis",
"score": 1,
"text": "β-半乳糖苷酶缺乏"
},
{
"indices": [
54,
55,
56,
57,
58
],
"label": "bod",
"score": 1,
"text": "硫酸角质素"
},
{
"indices": [
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67,
68
],
"label": "sym",
"score": 1,
"text": "硫酸角质素和硫酸软骨素降解障碍"
},
{
"indices": [
60,
61,
62,
63,
64
],
"label": "bod",
"score": 1,
"text": "硫酸软骨素"
},
{
"indices": [
72,
73,
74,
75,
76,
77,
78,
79,
80,
81,
82,
83,
84
],
"label": "sym",
"score": 1,
"text": "这些物质在细胞与组织中积聚"
},
{
"indices": [
77,
78
],
"label": "bod",
"score": 1,
"text": "细胞"
},
{
"indices": [
80,
81
],
"label": "bod",
"score": 1,
"text": "组织"
}
] |
最近基因工程生产的重组特异性酶的问世为黏多糖病的治疗带来了希望，MPSⅠ型、MPSⅡ型以及MPSⅥ型已有药物上市，酶的替代治疗取得了较好的效果。 | [
{
"indices": [
9,
10,
11,
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "重组特异性酶"
},
{
"indices": [
19,
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "黏多糖病"
},
{
"indices": [
32,
33,
34,
35,
36
],
"label": "dis",
"score": 1,
"text": "MPSⅠ型"
},
{
"indices": [
38,
39,
40,
41,
42
],
"label": "dis",
"score": 1,
"text": "MPSⅡ型"
},
{
"indices": [
45,
46,
47,
48,
49
],
"label": "dis",
"score": 1,
"text": "MPSⅥ型"
},
{
"indices": [
57
],
"label": "bod",
"score": 1,
"text": "酶"
}
] |
①上消化道梗阻：食管气管瘘、食管闭锁、食管裂孔疝、胃扭转、幽门肥厚性狭窄、环状胰腺、先天性膈疝等；②下消化道梗阻：如肠旋转不良、小肠重复畸形、肠狭窄、肠闭锁、先天性巨结肠、肛门闭锁等。 | [
{
"indices": [
1,
2,
3,
4,
5,
6
],
"label": "dis",
"score": 1,
"text": "上消化道梗阻"
},
{
"indices": [
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "食管气管瘘"
},
{
"indices": [
14,
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "食管闭锁"
},
{
"indices": [
19,
20,
21,
22,
23
],
"label": "dis",
"score": 1,
"text": "食管裂孔疝"
},
{
"indices": [
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "胃扭转"
},
{
"indices": [
29,
30,
31,
32,
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "幽门肥厚性狭窄"
},
{
"indices": [
37,
38,
39,
40
],
"label": "dis",
"score": 1,
"text": "环状胰腺"
},
{
"indices": [
42,
43,
44,
45,
46
],
"label": "dis",
"score": 1,
"text": "先天性膈疝"
},
{
"indices": [
50,
51,
52,
53,
54,
55
],
"label": "dis",
"score": 1,
"text": "下消化道梗阻"
},
{
"indices": [
58,
59,
60,
61,
62
],
"label": "dis",
"score": 1,
"text": "肠旋转不良"
},
{
"indices": [
64,
65,
66,
67,
68,
69
],
"label": "dis",
"score": 1,
"text": "小肠重复畸形"
},
{
"indices": [
71,
72,
73
],
"label": "dis",
"score": 1,
"text": "肠狭窄"
},
{
"indices": [
75,
76,
77
],
"label": "dis",
"score": 1,
"text": "肠闭锁"
},
{
"indices": [
79,
80,
81,
82,
83,
84
],
"label": "dis",
"score": 1,
"text": "先天性巨结肠"
},
{
"indices": [
86,
87,
88,
89
],
"label": "dis",
"score": 1,
"text": "肛门闭锁"
}
] |
喂养不当呕吐时，新生儿一般情况较好，改进喂养方法后呕吐可停止。 | [
{
"indices": [
4,
5
],
"label": "sym",
"score": 1,
"text": "呕吐"
},
{
"indices": [
25,
26
],
"label": "sym",
"score": 1,
"text": "呕吐"
}
] |
许多患儿无临床呕吐表现，而发生呼吸暂停、心动过缓、反复吸入甚至猝死。 | [
{
"indices": [
7,
8
],
"label": "sym",
"score": 1,
"text": "呕吐"
},
{
"indices": [
15,
16,
17,
18
],
"label": "sym",
"score": 1,
"text": "呼吸暂停"
},
{
"indices": [
20,
21,
22,
23
],
"label": "sym",
"score": 1,
"text": "心动过缓"
},
{
"indices": [
25,
26,
27,
28
],
"label": "sym",
"score": 1,
"text": "反复吸入"
},
{
"indices": [
31,
32
],
"label": "dis",
"score": 1,
"text": "猝死"
}
] |
如生后数天排便很少，或胎粪排空时间延迟，患儿可出现呕吐，呕吐物为黄绿色，常伴有腹胀，腹壁可见肠型，用生理盐水灌肠排出胎粪后，呕吐即可缓解。 | [
{
"indices": [
5,
6,
7,
8
],
"label": "sym",
"score": 1,
"text": "排便很少"
},
{
"indices": [
6
],
"label": "bod",
"score": 1,
"text": "便"
},
{
"indices": [
11,
12,
13,
14,
15,
16,
17,
18
],
"label": "sym",
"score": 1,
"text": "胎粪排空时间延迟"
},
{
"indices": [
11,
12
],
"label": "bod",
"score": 1,
"text": "胎粪"
},
{
"indices": [
25,
26
],
"label": "sym",
"score": 1,
"text": "呕吐"
},
{
"indices": [
28,
29,
30,
31,
32,
33,
34
],
"label": "sym",
"score": 1,
"text": "呕吐物为黄绿色"
},
{
"indices": [
28,
29,
30
],
"label": "bod",
"score": 1,
"text": "呕吐物"
},
{
"indices": [
39,
40
],
"label": "sym",
"score": 1,
"text": "腹胀"
},
{
"indices": [
39
],
"label": "bod",
"score": 1,
"text": "腹"
},
{
"indices": [
42,
43,
44,
45,
46,
47
],
"label": "sym",
"score": 1,
"text": "腹壁可见肠型"
},
{
"indices": [
42,
43
],
"label": "bod",
"score": 1,
"text": "腹壁"
},
{
"indices": [
49,
50,
51,
52,
53,
54,
55
],
"label": "pro",
"score": 1,
"text": "用生理盐水灌肠"
},
{
"indices": [
56,
57,
58,
59
],
"label": "sym",
"score": 1,
"text": "排出胎粪"
},
{
"indices": [
58,
59
],
"label": "bod",
"score": 1,
"text": "胎粪"
},
{
"indices": [
62,
63
],
"label": "sym",
"score": 1,
"text": "呕吐"
}
] |
有些患儿出现类似螃蟹吐泡沫，插胃管时胃管受阻折返。 | [
{
"indices": [
14,
15,
16,
17,
18,
19,
20,
21,
22,
23
],
"label": "sym",
"score": 1,
"text": "插胃管时胃管受阻折返"
},
{
"indices": [
14,
15,
16
],
"label": "pro",
"score": 1,
"text": "插胃管"
},
{
"indices": [
18,
19
],
"label": "equ",
"score": 1,
"text": "胃管"
}
] |
对吞咽功能不全、食管气管瘘可行碘油造影。 | [
{
"indices": [
1,
2,
3,
4,
5,
6
],
"label": "dis",
"score": 1,
"text": "吞咽功能不全"
},
{
"indices": [
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "食管气管瘘"
},
{
"indices": [
15,
16,
17,
18
],
"label": "pro",
"score": 1,
"text": "碘油造影"
}
] |
对幽门肥厚性狭窄，可做腹部超声检查。 | [
{
"indices": [
1,
2,
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "幽门肥厚性狭窄"
},
{
"indices": [
11,
12,
13,
14,
15,
16
],
"label": "pro",
"score": 1,
"text": "腹部超声检查"
}
] |
对肠道炎症、感染、低位肠梗阻，可摄腹部X线平片。 | [
{
"indices": [
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "肠道炎症"
},
{
"indices": [
6,
7
],
"label": "dis",
"score": 1,
"text": "感染"
},
{
"indices": [
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "低位肠梗阻"
},
{
"indices": [
16,
17,
18,
19,
20,
21,
22
],
"label": "pro",
"score": 1,
"text": "摄腹部X线平片"
}
] |
对结肠疾病如先天性巨结肠，可做钡剂灌肠造影检查。 | [
{
"indices": [
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "结肠疾病"
},
{
"indices": [
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "先天性巨结肠"
},
{
"indices": [
15,
16,
17,
18,
19,
20,
21,
22
],
"label": "pro",
"score": 1,
"text": "钡剂灌肠造影检查"
}
] |
外周血白细胞计数大多正常。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7
],
"label": "ite",
"score": 1,
"text": "外周血白细胞计数"
}
] |
治疗应采用兼治原型和L型菌的抗生素，如氨苄西林或头孢霉素类加大环内酯类。 | [
{
"indices": [
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16
],
"label": "dru",
"score": 1,
"text": "兼治原型和L型菌的抗生素"
},
{
"indices": [
19,
20,
21,
22
],
"label": "dru",
"score": 1,
"text": "氨苄西林"
},
{
"indices": [
24,
25,
26,
27,
28
],
"label": "dru",
"score": 1,
"text": "头孢霉素类"
},
{
"indices": [
30,
31,
32,
33,
34
],
"label": "dru",
"score": 1,
"text": "大环内酯类"
}
] |
在以免疫复合物介导的疾病中，补体下降往往提示疾病活动，免疫复合物介导的肾炎尤其如此，如狼疮肾炎、血清病、链球菌感染后肾炎、冷球蛋白血症及部分与风湿性疾病相关的肾炎（如多动脉炎肾炎）。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "免疫复合物介导的疾病"
},
{
"indices": [
14,
15,
16,
17
],
"label": "sym",
"score": 1,
"text": "补体下降"
},
{
"indices": [
14,
15
],
"label": "ite",
"score": 1,
"text": "补体"
},
{
"indices": [
27,
28,
29,
30,
31,
32,
33,
34,
35,
36
],
"label": "dis",
"score": 1,
"text": "免疫复合物介导的肾炎"
},
{
"indices": [
43,
44,
45,
46
],
"label": "dis",
"score": 1,
"text": "狼疮肾炎"
},
{
"indices": [
48,
49,
50
],
"label": "dis",
"score": 1,
"text": "血清病"
},
{
"indices": [
52,
53,
54,
55,
56,
57,
58,
59
],
"label": "dis",
"score": 1,
"text": "链球菌感染后肾炎"
},
{
"indices": [
61,
62,
63,
64,
65,
66
],
"label": "dis",
"score": 1,
"text": "冷球蛋白血症"
},
{
"indices": [
70,
71,
72,
73,
74,
75,
76,
77,
78,
79,
80
],
"label": "dis",
"score": 1,
"text": "与风湿性疾病相关的肾炎"
},
{
"indices": [
83,
84,
85,
86,
87,
88
],
"label": "dis",
"score": 1,
"text": "多动脉炎肾炎"
}
] |
成人类风湿关节炎可见80%RF阳性，而幼年特发性关节炎（JIA）患儿阳性率仅约5%～15%，因此不能依靠它诊断JIA。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "成人类风湿关节炎"
},
{
"indices": [
13,
14
],
"label": "ite",
"score": 1,
"text": "RF"
},
{
"indices": [
13,
14,
15,
16
],
"label": "sym",
"score": 1,
"text": "RF阳性"
},
{
"indices": [
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "dis",
"score": 1,
"text": "幼年特发性关节炎"
},
{
"indices": [
28,
29,
30
],
"label": "dis",
"score": 1,
"text": "JIA"
},
{
"indices": [
55,
56,
57
],
"label": "dis",
"score": 1,
"text": "JIA"
}
] |
因此建议将APF作为JIA诊断指标。 | [
{
"indices": [
5,
6,
7
],
"label": "ite",
"score": 1,
"text": "APF"
},
{
"indices": [
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "JIA"
}
] |
最近的报道显示成人DCM患者中47%为特发性，12%与心肌炎有关，11%与冠状动脉病变有关，另有30%为其他原因。 | [
{
"indices": [
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "成人DCM"
},
{
"indices": [
27,
28,
29
],
"label": "dis",
"score": 1,
"text": "心肌炎"
},
{
"indices": [
37,
38,
39,
40,
41,
42
],
"label": "dis",
"score": 1,
"text": "冠状动脉病变"
}
] |
在另外两个不同年龄儿童DCM的研究表明其中2%～15%有活体组织检查证实的心肌炎，其余85%～90%的患儿原因不明。 | [
{
"indices": [
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "儿童DCM"
},
{
"indices": [
28,
29,
30,
31,
32,
33
],
"label": "pro",
"score": 1,
"text": "活体组织检查"
},
{
"indices": [
37,
38,
39
],
"label": "dis",
"score": 1,
"text": "心肌炎"
}
] |
其他可应用的利尿剂包括依他尼酸、布美他尼。 | [
{
"indices": [
6,
7,
8
],
"label": "dru",
"score": 1,
"text": "利尿剂"
},
{
"indices": [
11,
12,
13,
14
],
"label": "dru",
"score": 1,
"text": "依他尼酸"
},
{
"indices": [
16,
17,
18,
19
],
"label": "dru",
"score": 1,
"text": "布美他尼"
}
] |
有效的口服降低后负荷制剂包括ACE抑制剂。 | [
{
"indices": [
3,
4
],
"label": "pro",
"score": 1,
"text": "口服"
},
{
"indices": [
7,
8,
9,
10,
11
],
"label": "dru",
"score": 1,
"text": "后负荷制剂"
},
{
"indices": [
14,
15,
16,
17,
18,
19
],
"label": "dru",
"score": 1,
"text": "ACE抑制剂"
}
] |
在儿科，最常用的为卡托普利及依那普利。 | [
{
"indices": [
1,
2
],
"label": "dep",
"score": 1,
"text": "儿科"
},
{
"indices": [
9,
10,
11,
12
],
"label": "dru",
"score": 1,
"text": "卡托普利"
},
{
"indices": [
14,
15,
16,
17
],
"label": "dru",
"score": 1,
"text": "依那普利"
}
] |
因而这些患儿应考虑应用华法林等类抗凝剂。 | [
{
"indices": [
11,
12,
13
],
"label": "dru",
"score": 1,
"text": "华法林"
},
{
"indices": [
15,
16,
17,
18
],
"label": "dru",
"score": 1,
"text": "类抗凝剂"
}
] |
如已明确有心腔内血栓，应积极以肝素治疗，最终过渡到长期华法林治疗。 | [
{
"indices": [
5,
6,
7,
8,
9
],
"label": "sym",
"score": 1,
"text": "心腔内血栓"
},
{
"indices": [
5,
6,
7,
8
],
"label": "bod",
"score": 1,
"text": "心腔内血"
},
{
"indices": [
15,
16,
17,
18
],
"label": "pro",
"score": 1,
"text": "肝素治疗"
},
{
"indices": [
25,
26,
27,
28,
29,
30,
31
],
"label": "pro",
"score": 1,
"text": "长期华法林治疗"
}
] |
如确定系心动过速诱导的心肌病，应予以抗心律失常药物治疗。 | [
{
"indices": [
4,
5,
6,
7,
8,
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "心动过速诱导的心肌病"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "pro",
"score": 1,
"text": "抗心律失常药物治疗"
}
] |
普鲁卡因胺及β受体阻滞剂是有效的抗心律失常药物，但因其有负性肌力作用，在这组患儿应慎用。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dru",
"score": 1,
"text": "普鲁卡因胺"
},
{
"indices": [
6,
7,
8,
9,
10,
11
],
"label": "dru",
"score": 1,
"text": "β受体阻滞剂"
},
{
"indices": [
16,
17,
18,
19,
20,
21,
22
],
"label": "dru",
"score": 1,
"text": "抗心律失常药物"
}
] |
咳嗽多较严重，初为干咳，很快转为顽固性剧咳，有时表现为百日咳样咳嗽，咳少量黏痰，偶见痰中带血丝或血块。 | [
{
"indices": [
0,
1,
2,
3,
4,
5
],
"label": "sym",
"score": 1,
"text": "咳嗽多较严重"
},
{
"indices": [
9,
10
],
"label": "sym",
"score": 1,
"text": "干咳"
},
{
"indices": [
16,
17,
18,
19,
20
],
"label": "sym",
"score": 1,
"text": "顽固性剧咳"
},
{
"indices": [
27,
28,
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "百日咳样咳嗽"
},
{
"indices": [
27,
28,
29
],
"label": "dis",
"score": 1,
"text": "百日咳"
},
{
"indices": [
34,
35,
36,
37,
38
],
"label": "sym",
"score": 1,
"text": "咳少量黏痰"
},
{
"indices": [
38
],
"label": "bod",
"score": 1,
"text": "痰"
},
{
"indices": [
40,
41,
42,
43,
44,
45,
46,
47,
48,
49
],
"label": "sym",
"score": 1,
"text": "偶见痰中带血丝或血块"
},
{
"indices": [
42
],
"label": "bod",
"score": 1,
"text": "痰"
},
{
"indices": [
45,
46
],
"label": "bod",
"score": 1,
"text": "血丝"
},
{
"indices": [
48,
49
],
"label": "bod",
"score": 1,
"text": "血块"
}
] |
早期患儿可用PCR法检测患儿痰等分泌物中MP-DNA，亦可从痰、鼻分泌物、咽拭子中分离培养出MP。 | [
{
"indices": [
6,
7,
8,
9
],
"label": "pro",
"score": 1,
"text": "PCR法"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "bod",
"score": 1,
"text": "痰等分泌物"
},
{
"indices": [
20,
21,
22,
23,
24,
25
],
"label": "ite",
"score": 1,
"text": "MP-DNA"
},
{
"indices": [
30
],
"label": "bod",
"score": 1,
"text": "痰"
},
{
"indices": [
32,
33,
34,
35
],
"label": "bod",
"score": 1,
"text": "鼻分泌物"
},
{
"indices": [
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47
],
"label": "sym",
"score": 1,
"text": "咽拭子中分离培养出MP"
},
{
"indices": [
37,
38,
39
],
"label": "pro",
"score": 1,
"text": "咽拭子"
},
{
"indices": [
41,
42,
43,
44
],
"label": "pro",
"score": 1,
"text": "分离培养"
},
{
"indices": [
46,
47
],
"label": "bod",
"score": 1,
"text": "MP"
}
] |
对难治性患儿应关注并发症如胸腔积液、阻塞性甚至坏死性肺炎的可能，及时进行胸腔穿刺或胸腔闭锁引流，必要时进行纤维支气管镜下支气管灌洗治疗。 | [
{
"indices": [
13,
14,
15,
16
],
"label": "dis",
"score": 1,
"text": "胸腔积液"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "阻塞性甚至坏死性肺炎"
},
{
"indices": [
36,
37,
38,
39
],
"label": "pro",
"score": 1,
"text": "胸腔穿刺"
},
{
"indices": [
41,
42,
43,
44,
45,
46
],
"label": "pro",
"score": 1,
"text": "胸腔闭锁引流"
},
{
"indices": [
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66
],
"label": "pro",
"score": 1,
"text": "纤维支气管镜下支气管灌洗治疗"
}
] |
在我国乙型脑炎主要发生于夏秋季节（7～9月份），与其主要传媒——库蚊的繁殖季节相关。 | [
{
"indices": [
3,
4,
5,
6
],
"label": "dis",
"score": 1,
"text": "乙型脑炎"
}
] |
国外报道约70%的病毒性脑炎和脑膜炎发生于6～11月份，儿童发病者约占50%，男孩发病稍多。 | [
{
"indices": [
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "病毒性脑炎"
},
{
"indices": [
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "脑膜炎"
}
] |
1960年以前，腮腺炎和脊髓灰质炎病毒感染约占中枢神经系统病毒感染的35%，而近年来在实施了此两种病毒计划免疫的国家，发病者明显减少。 | [
{
"indices": [
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "腮腺炎"
},
{
"indices": [
12,
13,
14,
15,
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "脊髓灰质炎病毒感染"
},
{
"indices": [
23,
24,
25,
26,
27,
28,
29,
30,
31,
32
],
"label": "dis",
"score": 1,
"text": "中枢神经系统病毒感染"
},
{
"indices": [
49,
50,
51,
52,
53,
54
],
"label": "pro",
"score": 1,
"text": "病毒计划免疫"
}
] |
但由于在病理解剖研究中比较容易准确判断骑跨的大动脉瓣与下方室间隔的相互连接关系，因此其诊断标准为当两根大动脉根部一半以上的周边均连接至同一心室时即诊断为右室双出口。 | [
{
"indices": [
19,
20,
21,
22,
23,
24,
25
],
"label": "bod",
"score": 1,
"text": "骑跨的大动脉瓣"
},
{
"indices": [
27,
28,
29,
30,
31
],
"label": "bod",
"score": 1,
"text": "下方室间隔"
},
{
"indices": [
49,
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67,
68,
69,
70
],
"label": "sym",
"score": 1,
"text": "两根大动脉根部一半以上的周边均连接至同一心室"
},
{
"indices": [
49,
50,
51,
52,
53,
54,
55
],
"label": "bod",
"score": 1,
"text": "两根大动脉根部"
},
{
"indices": [
69,
70
],
"label": "bod",
"score": 1,
"text": "心室"
},
{
"indices": [
76,
77,
78,
79,
80
],
"label": "dis",
"score": 1,
"text": "右室双出口"
}
] |
绝大多数的右室双出口伴有室间隔缺损，室间隔缺损可以是膜周型、肌部、远离大动脉或双动脉下。 | [
{
"indices": [
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
12,
13,
14,
15,
16
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
18,
19,
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
30,
31
],
"label": "bod",
"score": 1,
"text": "肌部"
},
{
"indices": [
35,
36,
37
],
"label": "bod",
"score": 1,
"text": "大动脉"
},
{
"indices": [
39,
40,
41,
42
],
"label": "bod",
"score": 1,
"text": "双动脉下"
}
] |
几乎3/4的右室双出口均有不同程度的肺动脉狭窄甚至闭锁，多为圆锥隔组织导致的肺动脉瓣下狭窄。 | [
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "不同程度的肺动脉狭窄甚至闭锁"
},
{
"indices": [
18,
19,
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
30,
31,
32,
33,
34
],
"label": "bod",
"score": 1,
"text": "圆锥隔组织"
},
{
"indices": [
38,
39,
40,
41,
42,
43,
44
],
"label": "dis",
"score": 1,
"text": "肺动脉瓣下狭窄"
}
] |
由于法洛四联症通常由其特殊的流出道形态作出诊断，但该特殊形态在法洛四联症及右室双出口均可存在，因此，右室双出口与法洛四联症较难明确区分。 | [
{
"indices": [
2,
3,
4,
5,
6
],
"label": "dis",
"score": 1,
"text": "法洛四联症"
},
{
"indices": [
14,
15,
16
],
"label": "bod",
"score": 1,
"text": "流出道"
},
{
"indices": [
31,
32,
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "法洛四联症"
},
{
"indices": [
37,
38,
39,
40,
41
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
50,
51,
52,
53,
54
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
56,
57,
58,
59,
60
],
"label": "dis",
"score": 1,
"text": "法洛四联症"
}
] |
右室双出口可能合并的心脏节段连接异常包括：房室连接不一致、心室双入口或房室瓣闭锁，在以上情况下，外科的纠治方案完全不同。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
10,
11
],
"label": "bod",
"score": 1,
"text": "心脏"
},
{
"indices": [
10,
11,
12,
13,
14,
15,
16,
17
],
"label": "sym",
"score": 1,
"text": "心脏节段连接异常"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27
],
"label": "sym",
"score": 1,
"text": "房室连接不一致"
},
{
"indices": [
21,
22
],
"label": "bod",
"score": 1,
"text": "房室"
},
{
"indices": [
29,
30,
31,
32,
33
],
"label": "sym",
"score": 1,
"text": "心室双入口"
},
{
"indices": [
29,
30
],
"label": "bod",
"score": 1,
"text": "心室"
},
{
"indices": [
35,
36,
37,
38,
39
],
"label": "dis",
"score": 1,
"text": "房室瓣闭锁"
},
{
"indices": [
48,
49
],
"label": "dep",
"score": 1,
"text": "外科"
}
] |
右室双出口常见的合并畸形包括房室瓣异常、左右流出道梗阻、主动脉弓中断、水肿及房室间隔缺损。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
10,
11
],
"label": "dis",
"score": 1,
"text": "畸形"
},
{
"indices": [
14,
15,
16
],
"label": "bod",
"score": 1,
"text": "房室瓣"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "sym",
"score": 1,
"text": "房室瓣异常"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "左右流出道梗阻"
},
{
"indices": [
20,
21,
22,
23,
24
],
"label": "bod",
"score": 1,
"text": "左右流出道"
},
{
"indices": [
28,
29,
30,
31,
32,
33
],
"label": "sym",
"score": 1,
"text": "主动脉弓中断"
},
{
"indices": [
28,
29,
30,
31
],
"label": "bod",
"score": 1,
"text": "主动脉弓"
},
{
"indices": [
35,
36
],
"label": "sym",
"score": 1,
"text": "水肿"
},
{
"indices": [
38,
39,
40,
41,
42,
43
],
"label": "dis",
"score": 1,
"text": "房室间隔缺损"
}
] |
在法四型右室双出口，如果存在严重的肺血供不足，可在新生儿期即有青紫表现。 | [
{
"indices": [
1,
2,
3,
4,
5,
6,
7,
8
],
"label": "dis",
"score": 1,
"text": "法四型右室双出口"
},
{
"indices": [
14,
15,
16,
17,
18,
19,
20,
21
],
"label": "sym",
"score": 1,
"text": "严重的肺血供不足"
},
{
"indices": [
17,
18
],
"label": "bod",
"score": 1,
"text": "肺血"
},
{
"indices": [
31,
32
],
"label": "sym",
"score": 1,
"text": "青紫"
}
] |
伴主动脉下室间隔缺损的右室双出口的典型临床表现是在出生近1月时充血性心力衰竭而无青紫表现，与单纯大型室间隔缺损临床表现相似，如果生后早期出现心力衰竭则应考虑是否同时伴有水肿。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15
],
"label": "dis",
"score": 1,
"text": "伴主动脉下室间隔缺损的右室双出口"
},
{
"indices": [
31,
32,
33,
34,
35,
36,
37
],
"label": "dis",
"score": 1,
"text": "充血性心力衰竭"
},
{
"indices": [
40,
41
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
46,
47,
48,
49,
50,
51,
52,
53,
54
],
"label": "dis",
"score": 1,
"text": "单纯大型室间隔缺损"
},
{
"indices": [
70,
71,
72,
73
],
"label": "dis",
"score": 1,
"text": "心力衰竭"
},
{
"indices": [
84,
85
],
"label": "sym",
"score": 1,
"text": "水肿"
}
] |
不同程度的肺动脉狭窄可伴有不同程度的青紫，可在生后1岁以内即出现。 | [
{
"indices": [
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
18,
19
],
"label": "sym",
"score": 1,
"text": "青紫"
}
] |
当狭窄严重时，早期即可出现青紫、乏力、活动后气促、蹲踞及高血红蛋白血症。 | [
{
"indices": [
1,
2,
3,
4
],
"label": "sym",
"score": 1,
"text": "狭窄严重"
},
{
"indices": [
13,
14
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
16,
17
],
"label": "sym",
"score": 1,
"text": "乏力"
},
{
"indices": [
19,
20,
21,
22,
23
],
"label": "sym",
"score": 1,
"text": "活动后气促"
},
{
"indices": [
25,
26
],
"label": "sym",
"score": 1,
"text": "蹲踞"
},
{
"indices": [
28,
29,
30,
31,
32,
33,
34
],
"label": "dis",
"score": 1,
"text": "高血红蛋白血症"
}
] |
体格检查可发现有青紫和杵状指（趾），心前区搏动弥散，左侧胸骨旁2～4肋间可及4/6～5/6级的喷射性收缩期杂音，高位左侧胸骨旁可及震颤。 | [
{
"indices": [
0,
1,
2,
3
],
"label": "pro",
"score": 1,
"text": "体格检查"
},
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16
],
"label": "sym",
"score": 1,
"text": "体格检查可发现有青紫和杵状指（趾）"
},
{
"indices": [
13
],
"label": "bod",
"score": 1,
"text": "指"
},
{
"indices": [
15
],
"label": "bod",
"score": 1,
"text": "趾"
},
{
"indices": [
18,
19,
20
],
"label": "bod",
"score": 1,
"text": "心前区"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24
],
"label": "sym",
"score": 1,
"text": "心前区搏动弥散"
},
{
"indices": [
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54
],
"label": "sym",
"score": 1,
"text": "左侧胸骨旁2～4肋间可及4/6～5/6级的喷射性收缩期杂音"
},
{
"indices": [
26,
27,
28,
29,
30,
31,
32,
33,
34,
35
],
"label": "bod",
"score": 1,
"text": "左侧胸骨旁2～4肋间"
},
{
"indices": [
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66
],
"label": "sym",
"score": 1,
"text": "高位左侧胸骨旁可及震颤"
},
{
"indices": [
56,
57,
58,
59,
60,
61,
62
],
"label": "bod",
"score": 1,
"text": "高位左侧胸骨旁"
}
] |
在伴有水肿时，婴儿早期即可有心功能衰竭、青紫和股动脉搏动减弱或消失。 | [
{
"indices": [
3,
4
],
"label": "sym",
"score": 1,
"text": "水肿"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "sym",
"score": 1,
"text": "心功能衰竭"
},
{
"indices": [
14
],
"label": "bod",
"score": 1,
"text": "心"
},
{
"indices": [
20,
21
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
23,
24,
25,
26,
27,
28,
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "股动脉搏动减弱或消失"
},
{
"indices": [
23,
24,
25
],
"label": "bod",
"score": 1,
"text": "股动脉"
}
] |
体格检查可发现典型的青紫和杵状指（趾），身高、体重明显落后于同龄人。 | [
{
"indices": [
0,
1,
2,
3
],
"label": "pro",
"score": 1,
"text": "体格检查"
},
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18
],
"label": "sym",
"score": 1,
"text": "体格检查可发现典型的青紫和杵状指（趾）"
},
{
"indices": [
15
],
"label": "bod",
"score": 1,
"text": "指"
},
{
"indices": [
17
],
"label": "bod",
"score": 1,
"text": "趾"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "身高、体重明显落后于同龄人"
},
{
"indices": [
20,
21
],
"label": "ite",
"score": 1,
"text": "身高"
},
{
"indices": [
23,
24
],
"label": "ite",
"score": 1,
"text": "体重"
}
] |
心前区隆起且心尖搏动弥散，在左侧胸骨旁可及高调的2～3级收缩期杂音，当伴有肺动脉狭窄时则可及3～4级响亮的收缩期杂音，第二心音通常响亮而单一。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "sym",
"score": 1,
"text": "心前区隆起"
},
{
"indices": [
0,
1,
2
],
"label": "bod",
"score": 1,
"text": "心前区"
},
{
"indices": [
6,
7
],
"label": "bod",
"score": 1,
"text": "心尖"
},
{
"indices": [
6,
7,
8,
9,
10,
11
],
"label": "sym",
"score": 1,
"text": "心尖搏动弥散"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32
],
"label": "sym",
"score": 1,
"text": "在左侧胸骨旁可及高调的2～3级收缩期杂音"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "bod",
"score": 1,
"text": "左侧胸骨旁"
},
{
"indices": [
37,
38,
39,
40,
41
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56,
57
],
"label": "sym",
"score": 1,
"text": "可及3～4级响亮的收缩期杂音"
},
{
"indices": [
59,
60,
61,
62,
63,
64,
65,
66,
67,
68,
69
],
"label": "sym",
"score": 1,
"text": "第二心音通常响亮而单一"
},
{
"indices": [
61
],
"label": "bod",
"score": 1,
"text": "心"
}
] |
左侧胸骨旁可及3/6～4/6级的全收缩期杂音伴震颤，心尖区可及舒张期杂音及第三心音。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24
],
"label": "sym",
"score": 1,
"text": "左侧胸骨旁可及3/6～4/6级的全收缩期杂音伴震颤"
},
{
"indices": [
0,
1,
2,
3,
4
],
"label": "bod",
"score": 1,
"text": "左侧胸骨旁"
},
{
"indices": [
26,
27,
28
],
"label": "bod",
"score": 1,
"text": "心尖区"
},
{
"indices": [
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40
],
"label": "sym",
"score": 1,
"text": "心尖区可及舒张期杂音及第三心音"
},
{
"indices": [
39
],
"label": "bod",
"score": 1,
"text": "心"
}
] |
一般手术方式的选择：①伴主动脉下室缺不伴有右室流出道狭窄，可以根据室间隔缺损的大小，临床症状及一般情况选择姑息术（肺动脉Banding术）或根治术；②伴主动脉下室间隔缺损伴有右室流出道狭窄可行体-肺分流术；③伴肺动脉下室间隔缺损常合并有主动脉弓缩窄，早期易发生肺动脉梗阻，可行姑息术（房间隔造口术）+肺动脉Banding术；④伴远离大动脉室间隔缺损的手术方式基本与伴主动脉下室间隔缺损合并或不合并右室流出道狭窄的手术方式相同。 | [
{
"indices": [
12,
13,
14,
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "主动脉下室缺"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "右室流出道狭窄"
},
{
"indices": [
33,
34,
35,
36,
37
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
53,
54,
55
],
"label": "pro",
"score": 1,
"text": "姑息术"
},
{
"indices": [
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67
],
"label": "pro",
"score": 1,
"text": "肺动脉Banding术"
},
{
"indices": [
70,
71,
72
],
"label": "pro",
"score": 1,
"text": "根治术"
},
{
"indices": [
76,
77,
78,
79,
80,
81,
82,
83,
84
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
87,
88,
89,
90,
91,
92,
93
],
"label": "dis",
"score": 1,
"text": "右室流出道狭窄"
},
{
"indices": [
96,
97,
98,
99,
100,
101
],
"label": "pro",
"score": 1,
"text": "体-肺分流术"
},
{
"indices": [
105,
106,
107,
108,
109,
110,
111,
112,
113
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损"
},
{
"indices": [
118,
119,
120,
121,
122,
123
],
"label": "dis",
"score": 1,
"text": "主动脉弓缩窄"
},
{
"indices": [
130,
131,
132,
133,
134
],
"label": "dis",
"score": 1,
"text": "肺动脉梗阻"
},
{
"indices": [
138,
139,
140
],
"label": "pro",
"score": 1,
"text": "姑息术"
},
{
"indices": [
142,
143,
144,
145,
146,
147
],
"label": "pro",
"score": 1,
"text": "房间隔造口术"
},
{
"indices": [
150,
151,
152,
153,
154,
155,
156,
157,
158,
159,
160
],
"label": "pro",
"score": 1,
"text": "肺动脉Banding术"
},
{
"indices": [
164,
165,
166,
167,
168,
169,
170,
171,
172,
173
],
"label": "dis",
"score": 1,
"text": "远离大动脉室间隔缺损"
},
{
"indices": [
183,
184,
185,
186,
187,
188,
189,
190,
191
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
198,
199,
200,
201,
202,
203,
204
],
"label": "dis",
"score": 1,
"text": "右室流出道狭窄"
}
] |
近年来根据尿红细胞形态改变等改变，常将血尿分为肾小球性血尿及非肾小球性血尿，有利于临床诊断。 | [
{
"indices": [
5,
6,
7,
8,
9,
10
],
"label": "ite",
"score": 1,
"text": "尿红细胞形态"
},
{
"indices": [
5,
6,
7,
8,
9,
10,
11,
12
],
"label": "sym",
"score": 1,
"text": "尿红细胞形态改变"
},
{
"indices": [
19,
20
],
"label": "sym",
"score": 1,
"text": "血尿"
},
{
"indices": [
19
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
20
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
23,
24,
25,
26,
27,
28
],
"label": "sym",
"score": 1,
"text": "肾小球性血尿"
},
{
"indices": [
23,
24,
25
],
"label": "bod",
"score": 1,
"text": "肾小球"
},
{
"indices": [
27
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
28
],
"label": "bod",
"score": 1,
"text": "尿"
},
{
"indices": [
30,
31,
32,
33,
34,
35,
36
],
"label": "sym",
"score": 1,
"text": "非肾小球性血尿"
},
{
"indices": [
31,
32,
33
],
"label": "bod",
"score": 1,
"text": "肾小球"
},
{
"indices": [
35
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
36
],
"label": "bod",
"score": 1,
"text": "尿"
}
] |
5.先天性肾及血管畸形如多囊肾、膀胱憩室，动静脉瘘，血管瘤等。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "先天性肾及血管畸形"
},
{
"indices": [
12,
13,
14
],
"label": "dis",
"score": 1,
"text": "多囊肾"
},
{
"indices": [
16,
17,
18,
19
],
"label": "dis",
"score": 1,
"text": "膀胱憩室"
},
{
"indices": [
21,
22,
23,
24
],
"label": "dis",
"score": 1,
"text": "动静脉瘘"
},
{
"indices": [
26,
27,
28
],
"label": "dis",
"score": 1,
"text": "血管瘤"
}
] |
多见于接近足月儿和足月儿，有剖宫产、羊水吸入、母亲产前应用大量镇静剂等病史。 | [
{
"indices": [
14,
15,
16
],
"label": "pro",
"score": 1,
"text": "剖宫产"
},
{
"indices": [
18,
19,
20,
21
],
"label": "sym",
"score": 1,
"text": "羊水吸入"
},
{
"indices": [
18,
19
],
"label": "bod",
"score": 1,
"text": "羊水"
},
{
"indices": [
31,
32,
33
],
"label": "dru",
"score": 1,
"text": "镇静剂"
}
] |
临床症状类似早产儿RDS，一般主要表现为气促，60～100次/分，可以出现吸气性凹陷征，肺内有湿啰音。 | [
{
"indices": [
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "早产儿RDS"
},
{
"indices": [
20,
21
],
"label": "sym",
"score": 1,
"text": "气促"
},
{
"indices": [
37,
38,
39,
40,
41,
42
],
"label": "sym",
"score": 1,
"text": "吸气性凹陷征"
},
{
"indices": [
44,
45
],
"label": "bod",
"score": 1,
"text": "肺内"
},
{
"indices": [
44,
45,
46,
47,
48,
49
],
"label": "sym",
"score": 1,
"text": "肺内有湿啰音"
}
] |
研究亦显示产前给予糖皮质激素和出生后给予肺表面活性物质可以产生增强效果，更有利于预防RDS。 | [
{
"indices": [
9,
10,
11,
12,
13
],
"label": "dru",
"score": 1,
"text": "糖皮质激素"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26
],
"label": "dru",
"score": 1,
"text": "肺表面活性物质"
},
{
"indices": [
42,
43,
44
],
"label": "dis",
"score": 1,
"text": "RDS"
}
] |
在RDS恢复阶段，可以考虑补充氨基酸、脂肪乳剂等。 | [
{
"indices": [
1,
2,
3
],
"label": "dis",
"score": 1,
"text": "RDS"
},
{
"indices": [
15,
16,
17
],
"label": "dru",
"score": 1,
"text": "氨基酸"
},
{
"indices": [
19,
20,
21,
22
],
"label": "dru",
"score": 1,
"text": "脂肪乳剂"
}
] |
应用肺表面活性物质治疗RDS可以减少BPD的发生，主要在于肺表面活性物质可以显著减少患儿对机械通气和氧疗的依赖时间，并降低机械通气压力和吸入氧浓度。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10
],
"label": "pro",
"score": 1,
"text": "应用肺表面活性物质治疗"
},
{
"indices": [
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "RDS"
},
{
"indices": [
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "BPD"
},
{
"indices": [
29,
30,
31,
32,
33,
34,
35
],
"label": "dru",
"score": 1,
"text": "肺表面活性物质"
},
{
"indices": [
45,
46,
47,
48
],
"label": "pro",
"score": 1,
"text": "机械通气"
},
{
"indices": [
50,
51
],
"label": "pro",
"score": 1,
"text": "氧疗"
},
{
"indices": [
61,
62,
63,
64
],
"label": "pro",
"score": 1,
"text": "机械通气"
},
{
"indices": [
68,
69,
70,
71,
72
],
"label": "ite",
"score": 1,
"text": "吸入氧浓度"
}
] |
早产儿贫血的病因仍未明确，可能与下列因素有关：①早产儿红细胞寿命40～60天，比足月儿（70～90天）更短；②促红细胞生成素（EPO）水平低下，由于早产儿发育未成熟，生后前几周产生EPO的部位主要在肝脏，而肝脏对缺氧的敏感性不如肾脏，因此产生EPO较少；③早产儿生长迅速，血液稀释；④疾病因素：早产儿易患许多疾病，加重贫血；⑤医源性失血：早产儿病情重，需取血做各种检查；⑥营养因素。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "早产儿贫血"
},
{
"indices": [
24,
25,
26
],
"label": "dis",
"score": 1,
"text": "早产儿"
},
{
"indices": [
27,
28,
29
],
"label": "bod",
"score": 1,
"text": "红细胞"
},
{
"indices": [
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37
],
"label": "sym",
"score": 1,
"text": "红细胞寿命40～60天"
},
{
"indices": [
55,
56,
57,
58,
59,
60,
61
],
"label": "bod",
"score": 1,
"text": "促红细胞生成素"
},
{
"indices": [
55,
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67,
68,
69,
70
],
"label": "sym",
"score": 1,
"text": "促红细胞生成素（EPO）水平低下"
},
{
"indices": [
63,
64,
65
],
"label": "bod",
"score": 1,
"text": "EPO"
},
{
"indices": [
74,
75,
76,
77,
78,
79,
80,
81
],
"label": "sym",
"score": 1,
"text": "早产儿发育未成熟"
},
{
"indices": [
74,
75,
76
],
"label": "dis",
"score": 1,
"text": "早产儿"
},
{
"indices": [
90,
91,
92
],
"label": "bod",
"score": 1,
"text": "EPO"
},
{
"indices": [
99,
100
],
"label": "bod",
"score": 1,
"text": "肝脏"
},
{
"indices": [
103,
104
],
"label": "bod",
"score": 1,
"text": "肝脏"
},
{
"indices": [
106,
107
],
"label": "sym",
"score": 1,
"text": "缺氧"
},
{
"indices": [
114,
115
],
"label": "bod",
"score": 1,
"text": "肾脏"
},
{
"indices": [
121,
122,
123
],
"label": "bod",
"score": 1,
"text": "EPO"
},
{
"indices": [
128,
129,
130
],
"label": "dis",
"score": 1,
"text": "早产儿"
},
{
"indices": [
136,
137
],
"label": "bod",
"score": 1,
"text": "血液"
},
{
"indices": [
147,
148,
149
],
"label": "dis",
"score": 1,
"text": "早产儿"
},
{
"indices": [
159,
160
],
"label": "dis",
"score": 1,
"text": "贫血"
},
{
"indices": [
163,
164,
165,
166,
167
],
"label": "dis",
"score": 1,
"text": "医源性失血"
},
{
"indices": [
169,
170,
171
],
"label": "dis",
"score": 1,
"text": "早产儿"
},
{
"indices": [
177,
178,
179,
180,
181,
182,
183
],
"label": "pro",
"score": 1,
"text": "取血做各种检查"
}
] |
前者见于非裔美国人，酶活性在15%以下；后者见于地中海地区国家如意大利、希腊，和中东、非洲及亚洲诸国的人群，酶活性在5%～40%之间，也称G-6-PD地中海型。 | [
{
"indices": [
10,
11,
12
],
"label": "ite",
"score": 1,
"text": "酶活性"
},
{
"indices": [
54,
55,
56
],
"label": "ite",
"score": 1,
"text": "酶活性"
},
{
"indices": [
69,
70,
71,
72,
73,
74,
75,
76,
77
],
"label": "dis",
"score": 1,
"text": "G-6-PD地中海"
}
] |