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其他的表现尚包括生长发育迟缓、喂养困难、反复尿路感染和血尿等UPJO所致尿路梗阻由于是不完全的，病情进展缓慢，也无明确症状，有时在尿量较多时，因引流不畅致肾盂收缩而出现腹痛，但多难指出具体部位，可以伴恶心呕吐。
|
[
{
"id": 0,
"entity": "生长发育迟缓",
"start_offset": 8,
"end_offset": 14,
"label": "sym"
},
{
"id": 1,
"entity": "喂养困难",
"start_offset": 15,
"end_offset": 19,
"label": "sym"
},
{
"id": 2,
"entity": "血尿",
"start_offset": 27,
"end_offset": 29,
"label": "dis"
},
{
"id": 3,
"entity": "反复尿路感染和血尿等",
"start_offset": 20,
"end_offset": 30,
"label": "sym"
},
{
"id": 4,
"entity": "UPJO",
"start_offset": 30,
"end_offset": 34,
"label": "dis"
},
{
"id": 5,
"entity": "尿路梗阻",
"start_offset": 36,
"end_offset": 40,
"label": "dis"
},
{
"id": 6,
"entity": "在尿量较多时",
"start_offset": 64,
"end_offset": 70,
"label": "sym"
},
{
"id": 7,
"entity": "因引流不畅致肾盂收缩而出现腹痛",
"start_offset": 71,
"end_offset": 86,
"label": "sym"
},
{
"id": 8,
"entity": "但多难指出具体部位，可以伴恶心呕吐",
"start_offset": 87,
"end_offset": 104,
"label": "sym"
}
] |
在迷走血管导致的UPJO中，由于间隙性的梗阻，患儿有阵发性腹痛，有时可伴呕吐。
|
[
{
"id": 0,
"entity": "迷走血管导致",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "UPJO",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "间隙性的梗阻",
"start_offset": 16,
"end_offset": 22,
"label": "dis"
},
{
"id": 3,
"entity": "患儿有阵发性腹痛",
"start_offset": 23,
"end_offset": 31,
"label": "sym"
},
{
"id": 4,
"entity": "有时可伴呕吐",
"start_offset": 32,
"end_offset": 38,
"label": "sym"
}
] |
血尿常由积水的肾盂黏膜血管破裂引起。
|
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"id": 0,
"entity": "血尿",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
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{
"id": 1,
"entity": "肾盂黏膜血管",
"start_offset": 7,
"end_offset": 13,
"label": "bod"
}
] |
年长的患儿，如青春期的常在大量饮水后出现腹痛。
|
[
{
"id": 0,
"entity": "腹痛",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
}
] |
大龄患者可出现高血压，原因是由于集合系统扩张，肾血流下降，肾脏功能性缺血，由肾素、血管紧张素介导产生。
|
[
{
"id": 0,
"entity": "高血压",
"start_offset": 7,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "集合系统扩张",
"start_offset": 16,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "肾血流下降",
"start_offset": 23,
"end_offset": 28,
"label": "sym"
},
{
"id": 3,
"entity": "肾脏功能性缺血",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "肾素",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 5,
"entity": "血管紧张素",
"start_offset": 41,
"end_offset": 46,
"label": "bod"
}
] |
【诊断】对胎儿期诊断有肾积水的患儿，出生后必须进行复查，时间最好是出生后和出生数周后（一般为4周）。
|
[
{
"id": 0,
"entity": "肾积水",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
}
] |
在生后大约3个月以内，肾脏尚在发育中，肾锥体和髓质都是透声的，可以产生肾积水的假象，故出生后B超肾积水期检查非常必要。
|
[
{
"id": 0,
"entity": "肾脏",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 1,
"entity": "肾锥体和髓质都是透声",
"start_offset": 19,
"end_offset": 29,
"label": "sym"
},
{
"id": 2,
"entity": "B超",
"start_offset": 46,
"end_offset": 48,
"label": "pro"
},
{
"id": 3,
"entity": "肾积水",
"start_offset": 48,
"end_offset": 51,
"label": "dis"
}
] |
目前在胎儿及新生儿的肾积水的程度估计上仍有困难。
|
[
{
"id": 0,
"entity": "肾积水",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
}
] |
从已有报告来看，轻中度的肾积水［肾盂扩张（2cm）的大部分患儿］，在2年内可以自行缓解。
|
[
{
"id": 0,
"entity": "轻中度的肾积水",
"start_offset": 8,
"end_offset": 15,
"label": "dis"
}
] |
这部分患儿的同位素肾图曲线上表现为对放射性核素的摄取正常，但排泄明显推迟。
|
[
{
"id": 0,
"entity": "部分患儿的同位素肾图曲线上表现为对放射性核素的摄取正常",
"start_offset": 1,
"end_offset": 28,
"label": "sym"
},
{
"id": 1,
"entity": "但排泄明显推迟",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
}
] |
UPJO导致肾积水的实验室检查绝大部分患儿的尿常规都是正常的尿常规都是正常的，当出现尿路感染时，可以有白细胞出现；也有部分患儿表现为血尿。
|
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{
"id": 0,
"entity": "UPJO",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
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{
"id": 1,
"entity": "肾积水",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "绝大部分患儿的尿常规都是正常的",
"start_offset": 15,
"end_offset": 30,
"label": "sym"
},
{
"id": 3,
"entity": "当出现尿路感染时，可以有白细胞出现",
"start_offset": 39,
"end_offset": 56,
"label": "sym"
},
{
"id": 4,
"entity": "也有部分患儿表现为血尿",
"start_offset": 57,
"end_offset": 68,
"label": "sym"
}
] |
除非是非常严重的双肾积水，一般肾积水的患儿其肾功能都在正常范围。
|
[
{
"id": 0,
"entity": "除非是非常严重的双肾积水，一般肾积水的患儿其肾功能都在正常范围",
"start_offset": 0,
"end_offset": 31,
"label": "sym"
}
] |
（一）超声是最常用和最有效的无创检查手段，可以发现扩张而分离的肾盂及肾盏，并可测量出肾皮质的厚薄。
|
[
{
"id": 0,
"entity": "超声",
"start_offset": 3,
"end_offset": 5,
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{
"id": 1,
"entity": "肾盂",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
},
{
"id": 2,
"entity": "肾盏",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 3,
"entity": "肾皮质",
"start_offset": 42,
"end_offset": 45,
"label": "bod"
}
] |
一般来说，UPJO致肾积水其输尿管口径基本正常。
|
[
{
"id": 0,
"entity": "UPJO",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
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{
"id": 1,
"entity": "肾积水",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "输尿管口径基本正常",
"start_offset": 14,
"end_offset": 23,
"label": "sym"
}
] |
超声对肾积水程度的判断除非是非常严重的巨大积水，其数据并不能反映肾积水的严重程度和肾功能的实际情况，但仍是首选的方法。
|
[
{
"id": 0,
"entity": "肾积水",
"start_offset": 32,
"end_offset": 35,
"label": "dis"
}
] |
（二）排泄性静脉尿路造影（IVU）显示患肾肾盂扩张，肾盏扩张，肾盂输尿管连接部中断，输尿管不显示。
|
[
{
"id": 0,
"entity": "排泄性静脉尿路造影",
"start_offset": 3,
"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "IVU",
"start_offset": 13,
"end_offset": 16,
"label": "pro"
},
{
"id": 2,
"entity": "肾肾盂扩张",
"start_offset": 20,
"end_offset": 25,
"label": "dis"
},
{
"id": 3,
"entity": "肾盏扩张",
"start_offset": 26,
"end_offset": 30,
"label": "dis"
},
{
"id": 4,
"entity": "肾盂输尿管连接部中断",
"start_offset": 31,
"end_offset": 41,
"label": "sym"
},
{
"id": 5,
"entity": "输尿管不显示",
"start_offset": 42,
"end_offset": 48,
"label": "sym"
}
] |
我们常用76%泛影葡胺2.2ml/kg快速经静脉推入，肾脏开始显影时间的长短以及造影剂显示的程度可以反映患肾功能的变化。
|
[
{
"id": 0,
"entity": "泛影葡胺2",
"start_offset": 7,
"end_offset": 12,
"label": "dru"
},
{
"id": 1,
"entity": "肾脏",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 2,
"entity": "造影剂",
"start_offset": 40,
"end_offset": 43,
"label": "dru"
}
] |
从肾脏的大小、肾盂和肾盏扩张的程度以及造影剂排泄的时间可以反映积水的严重程度。
|
[
{
"id": 0,
"entity": "肾脏",
"start_offset": 1,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "肾盂",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "肾盏",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 3,
"entity": "造影剂",
"start_offset": 19,
"end_offset": 22,
"label": "dru"
}
] |
依积水严重的程度，肾盏的表现可以从较轻的杯口变钝、变平，发展至严重的肾盏向外膨出、隆起及饱胀的肾盏向外膨出、隆起及饱胀。
|
[
{
"id": 0,
"entity": "肾盏的表现可以从较轻的杯口变钝、变平",
"start_offset": 9,
"end_offset": 27,
"label": "sym"
},
{
"id": 1,
"entity": "发展至严重的肾盏向外膨出、隆起及饱胀",
"start_offset": 28,
"end_offset": 46,
"label": "sym"
}
] |
有时造影剂滞留于扩张的肾盏中而未进入肾盂，状如作画用的调色板。
|
[
{
"id": 0,
"entity": "造影剂",
"start_offset": 2,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "肾盂",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
}
] |
但目前肾脏因积水严重而不显影时，多用MRU替代。
|
[
{
"id": 0,
"entity": "肾脏",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
}
] |
UPJO一般情况下输尿管不显示，但即使是肾盂输尿管连接部远端的输尿管能够显示，仍可能在肾盂输尿管连接部处存在严重的梗阻。
|
[
{
"id": 0,
"entity": "肾盂输尿管连接部远端",
"start_offset": 20,
"end_offset": 30,
"label": "bod"
},
{
"id": 1,
"entity": "输尿管",
"start_offset": 31,
"end_offset": 34,
"label": "bod"
},
{
"id": 2,
"entity": "肾盂输尿管连接部",
"start_offset": 43,
"end_offset": 51,
"label": "bod"
}
] |
经膀胱镜输尿管逆行插管造影可以明确输尿管全程的情况，一般主张其在手术当日进行。
|
[
{
"id": 0,
"entity": "膀胱镜输尿管逆行插管造影",
"start_offset": 1,
"end_offset": 13,
"label": "pro"
},
{
"id": 1,
"entity": "输尿管",
"start_offset": 17,
"end_offset": 20,
"label": "bod"
}
] |
（三）利尿性肾图肾盂积水的另一重要诊断方法是利尿性肾图，采用同位素技术，对双肾的血流灌注、分肾功能及示踪剂排泄进行测定、分析。
|
[
{
"id": 0,
"entity": "利尿性肾图",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "肾盂积水",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "同位素技术",
"start_offset": 30,
"end_offset": 35,
"label": "pro"
}
] |
一般来说肾图曲线的同位素摄取相评价肾功能，注射呋塞米后的排泄相可以确定梗阻的严重程度。
|
[
{
"id": 0,
"entity": "肾图曲线的同位素摄取",
"start_offset": 4,
"end_offset": 14,
"label": "pro"
},
{
"id": 1,
"entity": "呋塞米",
"start_offset": 23,
"end_offset": 26,
"label": "dru"
}
] |
对间隙性肾盂输尿管连接部梗阻的患儿来说，利尿性肾图尤其有价值。
|
[
{
"id": 0,
"entity": "间隙性肾盂输尿管连接部梗阻",
"start_offset": 1,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "利尿性肾图",
"start_offset": 20,
"end_offset": 25,
"label": "pro"
}
] |
这部分患儿仅在腹痛时才能发现PUJO的存在，而无症状时其排泄性尿路造影可以完全正常，上述情况多见于迷走血管压迫引起的肾积水。
|
[
{
"id": 0,
"entity": "排泄性尿路造影",
"start_offset": 28,
"end_offset": 35,
"label": "pro"
},
{
"id": 1,
"entity": "迷走血管压迫引起的肾积水",
"start_offset": 49,
"end_offset": 61,
"label": "dis"
}
] |
注射呋塞米，尿量增加，肾盂排空无法及时完成，肾积水的症状得以出现。
|
[
{
"id": 0,
"entity": "呋塞米",
"start_offset": 2,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "尿量",
"start_offset": 6,
"end_offset": 8,
"label": "ite"
},
{
"id": 2,
"entity": "肾盂",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "肾积水",
"start_offset": 22,
"end_offset": 25,
"label": "dis"
}
] |
当然有些患儿在做利尿性肾图时可以出现腹痛。
|
[
{
"id": 0,
"entity": "利尿性肾图",
"start_offset": 8,
"end_offset": 13,
"label": "dru"
},
{
"id": 1,
"entity": "出现腹痛",
"start_offset": 16,
"end_offset": 20,
"label": "sym"
}
] |
利尿性肾图避免了常规造影方法的放射性危害，探测敏感，即使功能很差的肾脏，如果有同位素示踪剂进入即可显示，可以提供分肾功能的指标，从而对术前、术后的情况进行比较。
|
[
{
"id": 0,
"entity": "同位素示踪剂",
"start_offset": 39,
"end_offset": 45,
"label": "dru"
}
] |
从我们的经验来看，术后的肾图复查以术后6个月为宜，如在3个月以前进行利尿性肾图检查，可能因吻合口水肿影响尿液引流，仍呈机械性梗阻征象。
|
[
{
"id": 0,
"entity": "利尿性肾图",
"start_offset": 34,
"end_offset": 39,
"label": "ite"
}
] |
（四）磁共振尿路显像（MRU）由于尿路梗阻导致积水、扩张，在磁共振的T2</sub>相上可以显示，尤其在肾功能不良，IVU和同位素肾图均不能显示的情况下，通过MRU可以显示尿路的解剖形态，提示梗阻的部位。
|
[
{
"id": 0,
"entity": "磁共振尿路显像",
"start_offset": 3,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "MRU",
"start_offset": 11,
"end_offset": 14,
"label": "pro"
},
{
"id": 2,
"entity": "尿路梗阻",
"start_offset": 17,
"end_offset": 21,
"label": "dis"
},
{
"id": 3,
"entity": "积水、扩张",
"start_offset": 23,
"end_offset": 28,
"label": "sym"
},
{
"id": 4,
"entity": "IVU",
"start_offset": 58,
"end_offset": 61,
"label": "pro"
},
{
"id": 5,
"entity": "同位素肾图",
"start_offset": 62,
"end_offset": 67,
"label": "pro"
}
] |
（五）排尿性膀胱尿道造影肾积水的患儿都必须做排尿性膀胱尿道造影以排除膀胱输尿管反流导致的肾积水。
|
[
{
"id": 0,
"entity": "排尿性膀胱尿道造影",
"start_offset": 3,
"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "排尿性膀胱尿道造影",
"start_offset": 22,
"end_offset": 31,
"label": "pro"
},
{
"id": 2,
"entity": "肾积水",
"start_offset": 44,
"end_offset": 47,
"label": "dis"
}
] |
【治疗】积水量少、肾功能正常，如果无严重的腹痛及血尿等症状，生长发育良好的患儿可以随访观察，依据其积水发展的情况再作决定。
|
[
{
"id": 0,
"entity": "腹痛",
"start_offset": 21,
"end_offset": 23,
"label": "sym"
},
{
"id": 1,
"entity": "血尿",
"start_offset": 24,
"end_offset": 26,
"label": "sym"
}
] |
对诊断明确，肾功能已有损害或肾功能尽管尚属正常，但有反复的腹痛、血尿及尿路感染等症状，均宜手术治疗。
|
[
{
"id": 0,
"entity": "反复的腹痛",
"start_offset": 26,
"end_offset": 31,
"label": "sym"
},
{
"id": 1,
"entity": "血尿及尿路感染",
"start_offset": 32,
"end_offset": 39,
"label": "sym"
},
{
"id": 2,
"entity": "手术治疗",
"start_offset": 45,
"end_offset": 49,
"label": "pro"
}
] |
手术方式是成熟的，进行离断型肾盂输尿管成形术。
|
[
{
"id": 0,
"entity": "离断型肾盂输尿管成形术",
"start_offset": 11,
"end_offset": 22,
"label": "pro"
}
] |
关于吻合口是否需支撑管的问题，日前尚有争论，美国的报告多不用支撑管，仅留置创面引流；国内有留置双“J”管的，问题是3～4个月后需再在麻醉下通过膀胱镜将其取出。
|
[
{
"id": 0,
"entity": "支撑管",
"start_offset": 8,
"end_offset": 11,
"label": "equ"
},
{
"id": 1,
"entity": "支撑管",
"start_offset": 30,
"end_offset": 33,
"label": "equ"
},
{
"id": 2,
"entity": "创面引流",
"start_offset": 37,
"end_offset": 41,
"label": "pro"
},
{
"id": 3,
"entity": "麻醉",
"start_offset": 66,
"end_offset": 68,
"label": "pro"
},
{
"id": 4,
"entity": "膀胱镜",
"start_offset": 71,
"end_offset": 74,
"label": "equ"
}
] |
如系小婴儿，膀胱镜恐不能进入，不得已要切开膀胱，总之都需要麻醉。
|
[
{
"id": 0,
"entity": "膀胱镜",
"start_offset": 6,
"end_offset": 9,
"label": "equ"
},
{
"id": 1,
"entity": "膀胱",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
}
] |
我们常规采用8Fr的胃管，从肾盂置入输尿管，另一端从肾皮质穿过，固定于切口皮肤。
|
[
{
"id": 0,
"entity": "胃管",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 1,
"entity": "肾盂",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "输尿管",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "肾皮质",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
}
] |
近十年来，内腔镜技术得到了比较快的发展，采用腹腔镜经腹腔或后腹膜途径进行肾盂成形术也有了不少报道。
|
[
{
"id": 0,
"entity": "内腔镜",
"start_offset": 5,
"end_offset": 8,
"label": "equ"
},
{
"id": 1,
"entity": "腹腔镜",
"start_offset": 22,
"end_offset": 25,
"label": "equ"
},
{
"id": 2,
"entity": "腹腔",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 3,
"entity": "腹膜",
"start_offset": 30,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "肾盂成形术",
"start_offset": 36,
"end_offset": 41,
"label": "pro"
}
] |
由于难度较大的小切口肾盂成形术也达到了超过99%的手术成功率，且手术时间一般为1小时左右，比较多的意见认为腹腔镜肾盂成形术广泛开展尚待时机，尤其依赖于机器人腹腔镜技术的进一步发展。
|
[
{
"id": 0,
"entity": "肾盂成形术",
"start_offset": 10,
"end_offset": 15,
"label": "pro"
},
{
"id": 1,
"entity": "腹腔镜肾盂成形术",
"start_offset": 53,
"end_offset": 61,
"label": "pro"
},
{
"id": 2,
"entity": "机器人腹腔镜",
"start_offset": 75,
"end_offset": 81,
"label": "pro"
}
] |
2002年美国儿科年会上波士顿儿童医院报告采用机器人腹腔镜进行肾盂成形术，受到了极大的关注，目前的主要问题是仪器及其使用的费用太高，难以普及。
|
[
{
"id": 0,
"entity": "机器人腹腔镜",
"start_offset": 23,
"end_offset": 29,
"label": "equ"
},
{
"id": 1,
"entity": "肾盂成形术",
"start_offset": 31,
"end_offset": 36,
"label": "pro"
}
] |
严重巨大肾积水这类病例现在已少见，由于小儿的肾脏多有相当的代偿功能，故对积水巨大的肾脏要谨慎处理，不宜草率作出切除肾脏的决定。
|
[
{
"id": 0,
"entity": "严重巨大肾积水",
"start_offset": 0,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肾脏",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
},
{
"id": 2,
"entity": "肾脏",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 3,
"entity": "肾脏",
"start_offset": 57,
"end_offset": 59,
"label": "bod"
}
] |
我们对这类患儿先做肾造瘘，观察其肾功能恢复情况，从我们总结来看，36例造瘘肾经6～9个月的观察，其中32例肾功能有恢复，IVU上肾由不显影至显影，同位素肾图分肾功能增加，均做肾盂成形术，随访下来肾功能均能维持比较好的状态。
|
[
{
"id": 0,
"entity": "肾造瘘",
"start_offset": 9,
"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "IVU",
"start_offset": 60,
"end_offset": 63,
"label": "pro"
},
{
"id": 2,
"entity": "同位素肾图",
"start_offset": 73,
"end_offset": 78,
"label": "pro"
},
{
"id": 3,
"entity": "肾盂成形术",
"start_offset": 87,
"end_offset": 92,
"label": "pro"
}
] |
UPJO致肾积水尤其是胎儿及新生儿肾积水的手术时机一直存在争议。
|
[
{
"id": 0,
"entity": "UPJO",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "肾积水",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
}
] |
20世纪80年代初，常见有胎儿肾脏减压以保护肾功能的报道，现在来看，仅在胎儿双侧肾积水伴羊水进行性减少时才要施用。
|
[
{
"id": 0,
"entity": "双侧肾积水伴羊水",
"start_offset": 38,
"end_offset": 46,
"label": "dis"
}
] |
但胎儿肾积水减压后肾功能及肺功能恢复的程度目前都未证实。
|
[
{
"id": 0,
"entity": "胎儿肾积水",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "肾",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "肺",
"start_offset": 13,
"end_offset": 14,
"label": "bod"
}
] |
对于肾积水患儿出生后的手术时机，争论也比较大。
|
[
{
"id": 0,
"entity": "肾积水",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
}
] |
另一方面，也有许多医师，尤其是美国的小儿泌尿外科医师对肾积水的患儿进行长期随访，发现即使是肾盂扩张程度比较重的，肾功能已有损伤的新生儿，其积水也并未恶化。
|
[
{
"id": 0,
"entity": "肾积水",
"start_offset": 27,
"end_offset": 30,
"label": "dis"
}
] |
参考文献1.AmoryJK，AnawaltBD，PaulsenCA，etal.Klinefelter’ssyndrome.Lancet.2000，356：333-3332.BeesleyCE，MeaneyCA，GreenlandG，etal.Mutationalanalysisof85mucopolysaccharidosistypeIfamilies：frequencyofknownmutations，identificationof17novelmutationsandinvitroexpressionofmissensemutations.HumGenet.2001，109：503-5033.BlairJ，TolmieJ，HollmanAS，etal.Phenotype，ovarianfunction，andgrowthinpatientswith45，X/47，XXXTurnermosaicism：implicationsforprenatalcounselingandestrogentherapyatpuberty.JPediatr.2001，139：724-7244.BramswigJH.Long-termresultsofgrowthhormonetherapyinTurnersyndrome.Endocrine.2001，15：5-55.ChenRG.TheinitiationandprogressofPhenylketonuriaprogrammesinChina.HongKongMedicalJournal，1996，2：282-2846.ClimentC，RubioV.Identificationofsevennovelmissensemutations，twosplice-sitemutations，twomicrodeletionsandapolymorphicaminoacidsubstitutioninthegeneforornithinetranscarbamylase（OTC）inpatientswithOTCdeficiency.HumMutat.2002，19：185-1857.CravchikA，SubramanianG，BroderS，etal.Sequenceanalysisofthehumangenome：implicationsfortheunderstandingofnervoussystemfunctionanddisease.ArchNeurol.2001，58：1772-17728.DaviesW，IslesAR，WilkinsonLS.Imprintedgenesandmentaldysfunction.AnnMed.2001，33：428-4369.EgozcueJ，SantaloJ，GimenezC，etal.Preimplantationgeneticdiagnosis.MolCellEndocrinol.2000，15：21-2110.GuXuefan，ZhiguoWang，JunYe，etal.NewbornScreeninginChina：Phenylketonuria，CongenitalHypothyroidismandExpandedNeonatalScreening.AnnalsoftheAcademyofMedicine，Singapore，2008，37（suppl）12：107-10711.KoestingerA，GilletM，ChioleroR.EffectoflivertransplantationonhepaticglucosemetabolisminapatientwithtypeIglycogenstoragedisease.Transplantation.2000，27：2205-220512.LevyHL.Newbornscreeningbytandemmassspectrometry：anewera.ClinChem，1988，44：2401-240113.LoeberG，WebsterD，AznarezA.Qualityevaluationofnewbornscreeningprograms.Actapediatr，1999，Suppl432：3-614.BehrmanRE，KliegmanRM，JensonHB，etal.NelsonTextbookofPediatrics17thed.SciencePress，Harconrt2.AsiaandSaunder，200415.KyriakieSarafoglou.PediatricEndocrinologyandInbornErrorsofMetabolism，McGrawHillMedical，200916.陈瑞冠，陈蕙英，石树中.新生儿三种代谢病筛查.上海医学.1983，6：344-34417.顾学范，韩连书，高晓岚，等.串联质谱技术在遗传代谢病高危儿童筛查中的初步应用.中华儿科杂志，2004，42：401-40418.顾学范，王治国.中国580万苯丙酮尿症和先天性甲状腺功能减低症的新生儿疾病筛查调查.中华预防医学杂志，2004，38：99-9919.顾学范.新生儿疾病筛查.上海科学技术文献出版社：上海，2003：1-120.卢大儒，邱信芳，薛京伦.医学分子遗传学.第1版，上海：复旦大学出版社.1998：624-63421.沈明，喻唯民，徐力，等.苯丙酮尿症患儿发育商与早期干预初步探讨.中日友好医院学报，1994，8：151-15122.施惠平，黄尚志.遗传代谢病的产前诊断.中国实用儿科杂志，2000，15：80-8223.王怀立，刘松茂，高铁铮，等.130例智力低下儿童脆性X检测报告.遗传，1995，17：11-1124.叶军，邱文娟，韩连书，等.398例新生儿各型高苯丙氨酸血症的研究及26年诊治经验.中华围产医学杂志，2008，11（6）：382-38225.张惠文，王瑜，叶军，等.黏多糖贮积症47例的常见酶学分型.中华儿科杂志，2009，47（4）：276-27626.胡亚美.诸福堂实用儿科学.第7版.北京：人民卫生出版社，2002
|
[
{
"id": 0,
"entity": "代谢病",
"start_offset": 2036,
"end_offset": 2039,
"label": "dis"
},
{
"id": 1,
"entity": "遗传代谢病",
"start_offset": 2085,
"end_offset": 2090,
"label": "dis"
},
{
"id": 2,
"entity": "苯丙酮尿症",
"start_offset": 2142,
"end_offset": 2147,
"label": "dis"
},
{
"id": 3,
"entity": "先天性甲状腺功能减低症",
"start_offset": 2148,
"end_offset": 2159,
"label": "dis"
},
{
"id": 4,
"entity": "新生儿疾病",
"start_offset": 2160,
"end_offset": 2165,
"label": "dis"
},
{
"id": 5,
"entity": "苯丙酮尿症",
"start_offset": 2295,
"end_offset": 2300,
"label": "dis"
},
{
"id": 6,
"entity": "遗传代谢病",
"start_offset": 2349,
"end_offset": 2354,
"label": "dis"
},
{
"id": 7,
"entity": "儿科",
"start_offset": 2364,
"end_offset": 2366,
"label": "dep"
},
{
"id": 8,
"entity": "脆性X检测",
"start_offset": 2409,
"end_offset": 2414,
"label": "pro"
},
{
"id": 9,
"entity": "高苯丙氨酸血症",
"start_offset": 2458,
"end_offset": 2465,
"label": "dis"
},
{
"id": 10,
"entity": "黏多糖贮积症",
"start_offset": 2519,
"end_offset": 2525,
"label": "dis"
},
{
"id": 11,
"entity": "儿科",
"start_offset": 2538,
"end_offset": 2540,
"label": "dep"
},
{
"id": 12,
"entity": "儿科学",
"start_offset": 2573,
"end_offset": 2576,
"label": "dep"
}
] |
参考文献1.陈清棠.临床神经病学.北京科学技术出版社，2000：12.李庆有，等.肝豆状核变性的脑MRI与临床表现的相关分析.中华神经科杂志，1997，30（6）：367-3673.吴斌.小儿肝豆状核变性的诊治新进展.国外医学儿科学分册，1997，24（4）：202-2024.杨任民.肝豆状核变性的治疗.中国实用儿科杂志，2000，15（2）：79-805.刘晓青，张雅芳，刘孜孜等.肝豆状核变性基因类型与临床表型关系研究.中华儿科杂志，2003，41（1）：35-356.LoudianosG，GitlinJD.Wilson’sdisease.SeminLiverDis，2000，20（3）：353-3537.RichardsRJ，HammittJK.Findingtheoptimalmultiple-teststrategyusingamethodanalogoustologisticregression：thediagnosisofhepatolenticulardegeneration（Wilson’sdisease）.MedDecisMaking，1996，16（4）：367-3678.RobertsEA，CoxDW.Wilsondisease.BaillieresClinGastroenterol，1998，12（2）：237-2379.SmoloarekC，StremmelW.TherapyofWilsondisease.ZGastroenterol，1999，37（4）：293-29310.中华医学会神经病学分会帕金森病及运动障碍学组.中华医学会神经病学分会神经遗传病学组.肝豆状核变性的诊断与治疗指南《中华神经科杂志》2008，41（8）：566-566
|
[
{
"id": 0,
"entity": "神经病",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "肝豆状核变性",
"start_offset": 41,
"end_offset": 47,
"label": "dis"
},
{
"id": 2,
"entity": "脑MRI",
"start_offset": 48,
"end_offset": 52,
"label": "ite"
},
{
"id": 3,
"entity": "肝豆状核变性",
"start_offset": 96,
"end_offset": 102,
"label": "dis"
},
{
"id": 4,
"entity": "肝豆状核变性",
"start_offset": 143,
"end_offset": 149,
"label": "dis"
},
{
"id": 5,
"entity": "肝豆状核变性",
"start_offset": 193,
"end_offset": 199,
"label": "dis"
},
{
"id": 6,
"entity": "神经病",
"start_offset": 667,
"end_offset": 670,
"label": "dis"
},
{
"id": 7,
"entity": "帕金森病",
"start_offset": 673,
"end_offset": 677,
"label": "dis"
},
{
"id": 8,
"entity": "运动障碍",
"start_offset": 678,
"end_offset": 682,
"label": "dis"
},
{
"id": 9,
"entity": "神经病",
"start_offset": 690,
"end_offset": 693,
"label": "dis"
},
{
"id": 10,
"entity": "肝豆状核变性",
"start_offset": 704,
"end_offset": 710,
"label": "dis"
}
] |
四、弥漫性淋巴管瘤弥漫性淋巴管瘤又称淋巴管瘤性巨肢症。
|
[
{
"id": 0,
"entity": "弥漫性淋巴管瘤",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "弥漫性淋巴管瘤",
"start_offset": 9,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "淋巴管瘤性巨肢症",
"start_offset": 18,
"end_offset": 26,
"label": "dis"
}
] |
系由胚胎早期的胚芽所生长，因弥漫性淋巴管瘤占领整个肢体，从指端到肩部，或从脚趾到腹股沟。
|
[
{
"id": 0,
"entity": "胚胎",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "胚芽",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "弥漫性淋巴管瘤",
"start_offset": 14,
"end_offset": 21,
"label": "dis"
},
{
"id": 3,
"entity": "肢体",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
},
{
"id": 4,
"entity": "指端",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 5,
"entity": "肩部",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
}
] |
皮肤上有时有毛细血管斑痣肿瘤病变广泛，分布在肢体的所有组织，浸润肌肉和肌间隙，甚至骨膜。
|
[
{
"id": 0,
"entity": "毛细血管",
"start_offset": 6,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "皮肤上有时有毛细血管斑痣",
"start_offset": 0,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "肿瘤",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "肢体",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
},
{
"id": 4,
"entity": "组织",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 5,
"entity": "肌肉",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 6,
"entity": "肌间隙",
"start_offset": 35,
"end_offset": 38,
"label": "bod"
},
{
"id": 7,
"entity": "骨膜",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
}
] |
二、肺大疱肺大疱（pneumatoceles）又称为泡性肺气肿（bullousemphysematousblebsorcysts）。
|
[
{
"id": 0,
"entity": "肺大疱",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "肺大疱",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
},
{
"id": 2,
"entity": "pneumatoceles",
"start_offset": 9,
"end_offset": 22,
"label": "dis"
},
{
"id": 3,
"entity": "泡性肺气肿",
"start_offset": 26,
"end_offset": 31,
"label": "dis"
},
{
"id": 4,
"entity": "bullousemphysematousblebsorcysts",
"start_offset": 32,
"end_offset": 64,
"label": "dis"
}
] |
多数见于婴幼儿，最常见的病因为葡萄球菌性肺炎，由于支气管黏膜广泛充血水肿渗出，导致小气道狭窄，形成活瓣，肺泡内空气不断积聚，过度膨胀发生破裂，许多肺泡融合在一起，形成一个或多个肺大疱。
|
[
{
"id": 0,
"entity": "葡萄球菌性肺炎",
"start_offset": 15,
"end_offset": 22,
"label": "dis"
},
{
"id": 1,
"entity": "支气管黏膜",
"start_offset": 25,
"end_offset": 30,
"label": "bod"
},
{
"id": 2,
"entity": "充血水肿渗出",
"start_offset": 32,
"end_offset": 38,
"label": "sym"
},
{
"id": 3,
"entity": "小气道狭窄",
"start_offset": 41,
"end_offset": 46,
"label": "sym"
},
{
"id": 4,
"entity": "形成活瓣",
"start_offset": 47,
"end_offset": 51,
"label": "sym"
},
{
"id": 5,
"entity": "肺泡",
"start_offset": 52,
"end_offset": 54,
"label": "bod"
},
{
"id": 6,
"entity": "空气不断积聚",
"start_offset": 55,
"end_offset": 61,
"label": "sym"
},
{
"id": 7,
"entity": "过度膨胀发生破裂",
"start_offset": 62,
"end_offset": 70,
"label": "sym"
},
{
"id": 8,
"entity": "肺泡",
"start_offset": 73,
"end_offset": 75,
"label": "bod"
},
{
"id": 9,
"entity": "肺大疱",
"start_offset": 88,
"end_offset": 91,
"label": "bod"
}
] |
肺大疱体积小者可无任何症状，体积大而压力高者可致急性呼吸困难。
|
[
{
"id": 0,
"entity": "肺大疱",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "急性呼吸困难",
"start_offset": 24,
"end_offset": 30,
"label": "sym"
}
] |
诊断有赖于X线肺部摄片，胸片可见四周有薄壁构成的环状透亮阴影，含空气或含空气和液体，后者可见气液平面，且随体位而改变，其位置、透明区可迅速出现、迅速消失、忽大忽小，此为本病的特点。
|
[
{
"id": 0,
"entity": "X线肺部摄片",
"start_offset": 5,
"end_offset": 11,
"label": "ite"
},
{
"id": 1,
"entity": "胸片",
"start_offset": 12,
"end_offset": 14,
"label": "ite"
},
{
"id": 2,
"entity": "四周有薄壁构成的环状透亮阴影",
"start_offset": 16,
"end_offset": 30,
"label": "sym"
},
{
"id": 3,
"entity": "含空气或含空气和液体",
"start_offset": 31,
"end_offset": 41,
"label": "sym"
},
{
"id": 4,
"entity": "气液平面",
"start_offset": 46,
"end_offset": 50,
"label": "sym"
},
{
"id": 5,
"entity": "随体位而改变",
"start_offset": 52,
"end_offset": 58,
"label": "sym"
},
{
"id": 6,
"entity": "位置、透明区可迅速出现",
"start_offset": 60,
"end_offset": 71,
"label": "sym"
},
{
"id": 7,
"entity": "迅速消失",
"start_offset": 72,
"end_offset": 76,
"label": "sym"
},
{
"id": 8,
"entity": "忽大忽小",
"start_offset": 77,
"end_offset": 81,
"label": "sym"
}
] |
本病预后大多良好，症状随呼吸道感染的痊愈及支气管梗阻的消除而消退。
|
[
{
"id": 0,
"entity": "呼吸道感染",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "支气管梗阻",
"start_offset": 21,
"end_offset": 26,
"label": "dis"
}
] |
第四节新生儿颅内出血颅内出血（intracraninalhemorrhage）是新生儿期常见的临床问题，出血部位包括硬膜下出血、蛛网膜下腔出血、脑室周围-脑室内出血、小脑出血和脑实质出血。
|
[
{
"id": 0,
"entity": "新生儿颅内出血",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "颅内出血",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "intracraninalhemorrhage",
"start_offset": 15,
"end_offset": 38,
"label": "dis"
},
{
"id": 3,
"entity": "硬膜下出血",
"start_offset": 58,
"end_offset": 63,
"label": "dis"
},
{
"id": 4,
"entity": "蛛网膜下腔出血",
"start_offset": 64,
"end_offset": 71,
"label": "dis"
},
{
"id": 5,
"entity": "脑室周围-脑室内出血",
"start_offset": 72,
"end_offset": 82,
"label": "dis"
},
{
"id": 6,
"entity": "小脑出血",
"start_offset": 83,
"end_offset": 87,
"label": "dis"
},
{
"id": 7,
"entity": "脑实质出血",
"start_offset": 88,
"end_offset": 93,
"label": "dis"
}
] |
近年，由于产科技术的进步，产伤所致的硬膜下出血明显减少，而早产儿缺氧所致的脑室周围-脑室内出血已成为新生儿颅内出血最常见的类型。
|
[
{
"id": 0,
"entity": "产科",
"start_offset": 5,
"end_offset": 7,
"label": "dep"
},
{
"id": 1,
"entity": "硬膜下出血",
"start_offset": 18,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "缺氧",
"start_offset": 32,
"end_offset": 34,
"label": "sym"
},
{
"id": 3,
"entity": "脑室周围-脑室内出血",
"start_offset": 37,
"end_offset": 47,
"label": "dis"
},
{
"id": 4,
"entity": "新生儿颅内出血",
"start_offset": 50,
"end_offset": 57,
"label": "dis"
}
] |
一、脑室周围-脑室内出血脑室周围-脑室内出血（intraventricularhemorrhage，IVH）是早产儿最常见的颅内出血类型。
|
[
{
"id": 0,
"entity": "脑室周围-脑室内出血",
"start_offset": 2,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "脑室周围-脑室内出血",
"start_offset": 12,
"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "intraventricularhemorrhage",
"start_offset": 23,
"end_offset": 49,
"label": "dis"
},
{
"id": 3,
"entity": "IVH",
"start_offset": 50,
"end_offset": 53,
"label": "dis"
},
{
"id": 4,
"entity": "颅内出血",
"start_offset": 62,
"end_offset": 66,
"label": "dis"
}
] |
近年，随着新生儿医疗护理水平的改善，极低出生体重儿成活率显著提高，IVH已成为NICU早产儿的重要问题。
|
[
{
"id": 0,
"entity": "IVH",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
}
] |
【病因与发病机制】早产儿脑室周围室管膜下生发基质富含血管，这些血管在解剖学上是一种不成熟的毛细血管网，仅由一层内皮细胞组成，缺乏肌层和结缔组织支持，该区域对缺氧和高碳酸血症极为敏感，当缺氧致脑血流自我调节功能受损时，惊厥、气管吸引、快速扩容、静脉输注高渗溶液等可致血压波动而促发管破裂出血。
|
[
{
"id": 0,
"entity": "脑室",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "室管膜",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "生发基质",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "血管",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "血管",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "毛细血管网",
"start_offset": 45,
"end_offset": 50,
"label": "bod"
},
{
"id": 6,
"entity": "内皮细胞",
"start_offset": 55,
"end_offset": 59,
"label": "bod"
},
{
"id": 7,
"entity": "肌层",
"start_offset": 64,
"end_offset": 66,
"label": "bod"
},
{
"id": 8,
"entity": "结缔组织",
"start_offset": 67,
"end_offset": 71,
"label": "bod"
},
{
"id": 9,
"entity": "缺氧",
"start_offset": 78,
"end_offset": 80,
"label": "sym"
},
{
"id": 10,
"entity": "高碳酸血症",
"start_offset": 81,
"end_offset": 86,
"label": "dis"
},
{
"id": 11,
"entity": "缺氧",
"start_offset": 92,
"end_offset": 94,
"label": "sym"
},
{
"id": 12,
"entity": "惊厥",
"start_offset": 108,
"end_offset": 110,
"label": "sym"
},
{
"id": 13,
"entity": "血压",
"start_offset": 132,
"end_offset": 134,
"label": "ite"
}
] |
此外，生发基质的毛细血管网在引流入静脉系统时的血流方向呈独特的U形，这在生发基质出血中起重要作用，当胎头娩出困难、颅骨过度受压时可使该处血流停滞而发生出血。
|
[
{
"id": 0,
"entity": "生发基质",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "毛细血管",
"start_offset": 8,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "生发基质出血",
"start_offset": 36,
"end_offset": 42,
"label": "dis"
},
{
"id": 3,
"entity": "颅骨",
"start_offset": 57,
"end_offset": 59,
"label": "bod"
},
{
"id": 4,
"entity": "血流停滞",
"start_offset": 68,
"end_offset": 72,
"label": "sym"
},
{
"id": 5,
"entity": "出血",
"start_offset": 75,
"end_offset": 77,
"label": "dis"
}
] |
生发基质的宽度在胎龄23～25周时为2.5mm，32周为1.4mm，36周时几乎完全退化，因此IVH主要发生在胎龄小于33周的早产儿。
|
[
{
"id": 0,
"entity": "生发基质",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 47,
"end_offset": 50,
"label": "dis"
}
] |
在生发基质出血的病例中，80%的患儿血液可进入侧脑室，血液通过马氏孔和路氏孔进入后颅凹的基底池，引起闭塞致使脑脊液循环障碍，血凝块也可阻塞大脑导水管和蛛网膜绒毛而引起出血后脑积水和脑室周围出血性梗死。
|
[
{
"id": 0,
"entity": "生发基质出血",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "血液",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "侧脑室",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "血液",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 4,
"entity": "后颅凹",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
},
{
"id": 5,
"entity": "脑脊液",
"start_offset": 54,
"end_offset": 57,
"label": "bod"
},
{
"id": 6,
"entity": "血凝块",
"start_offset": 62,
"end_offset": 65,
"label": "bod"
},
{
"id": 7,
"entity": "大脑",
"start_offset": 69,
"end_offset": 71,
"label": "bod"
},
{
"id": 8,
"entity": "出血",
"start_offset": 83,
"end_offset": 85,
"label": "dis"
},
{
"id": 9,
"entity": "脑积水",
"start_offset": 86,
"end_offset": 89,
"label": "dis"
},
{
"id": 10,
"entity": "脑室",
"start_offset": 90,
"end_offset": 92,
"label": "bod"
},
{
"id": 11,
"entity": "出血性梗死",
"start_offset": 94,
"end_offset": 99,
"label": "dis"
}
] |
虽然IVH是早产儿的常见病，但足月儿也可发生，足月儿IVH的起源主要为脉络膜丛和室管膜下残存的生发基质。
|
[
{
"id": 0,
"entity": "IVH",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "足月儿IVH",
"start_offset": 23,
"end_offset": 29,
"label": "dis"
},
{
"id": 2,
"entity": "脉络膜丛",
"start_offset": 35,
"end_offset": 39,
"label": "bod"
},
{
"id": 3,
"entity": "室管膜",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
},
{
"id": 4,
"entity": "生发基质",
"start_offset": 47,
"end_offset": 51,
"label": "bod"
}
] |
在足月儿IVH的发病机制中，产伤的作用比缺氧更为重要，其中30%的患儿有产钳分娩或臀位牵引史，还有25%的患儿可无明显诱因，既无产伤也无缺氧。
|
[
{
"id": 0,
"entity": "足月儿IVH",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "缺氧",
"start_offset": 20,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "缺氧",
"start_offset": 68,
"end_offset": 70,
"label": "sym"
}
] |
近年研究发现早产儿IVH与机体凝血状况有关，某些凝血因子表达减少可能会加重IVH。
|
[
{
"id": 0,
"entity": "早产儿IVH",
"start_offset": 6,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 37,
"end_offset": 40,
"label": "dis"
}
] |
【临床表现】IVH主要见于围生期窒息和早产儿，出血50%开始于生后第1天，30%发生在第2天，到生后72小时头颅超声可发现90%的IVH。
|
[
{
"id": 0,
"entity": "IVH",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "头颅超声",
"start_offset": 54,
"end_offset": 58,
"label": "pro"
},
{
"id": 2,
"entity": "IVH",
"start_offset": 65,
"end_offset": 68,
"label": "dis"
}
] |
临床表现可有三种类型：急剧恶化型、断续进展型和临床寂静型。
|
[
{
"id": 0,
"entity": "急剧恶化型",
"start_offset": 11,
"end_offset": 16,
"label": "sym"
},
{
"id": 1,
"entity": "断续进展型",
"start_offset": 17,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "临床寂静型",
"start_offset": 23,
"end_offset": 28,
"label": "sym"
}
] |
以寂静型最为常见，占IVH病例的50%，无临床症状或体征，仅在超声或CT检查时发现。
|
[
{
"id": 0,
"entity": "寂静型",
"start_offset": 1,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "超声",
"start_offset": 31,
"end_offset": 33,
"label": "pro"
},
{
"id": 3,
"entity": "CT检查",
"start_offset": 34,
"end_offset": 38,
"label": "pro"
}
] |
【诊断】早产儿IVH的临床症状和体征较少，单凭临床表现很难诊断。
|
[
{
"id": 0,
"entity": "早产儿IVH",
"start_offset": 4,
"end_offset": 10,
"label": "dis"
}
] |
影像学检查是IVH的主要诊断手段，要根据具体情况选择头颅B超或CT检查。
|
[
{
"id": 0,
"entity": "影像学检查",
"start_offset": 0,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "头颅B超",
"start_offset": 26,
"end_offset": 30,
"label": "pro"
},
{
"id": 3,
"entity": "CT检查",
"start_offset": 31,
"end_offset": 35,
"label": "pro"
}
] |
（一）头颅超声是诊断IVH的首选方法。
|
[
{
"id": 0,
"entity": "头颅超声",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
}
] |
床旁头颅超声对早产儿IVH的开始时间、出血部位及严重程度提供可靠的信息，而且价廉方便，又无放射线损伤。
|
[
{
"id": 0,
"entity": "头颅超声",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "早产儿IVH",
"start_offset": 7,
"end_offset": 13,
"label": "dis"
}
] |
极低出生体重儿是易发生IVH的高危人群，应常规进行头颅超声的筛查。
|
[
{
"id": 0,
"entity": "IVH",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "头颅超声",
"start_offset": 25,
"end_offset": 29,
"label": "pro"
}
] |
头颅超声检查可将IVH分为4级：①Ⅰ级：出血限于室管膜下，不伴脑室内出血；②Ⅱ级：不伴脑室扩张的IVH；③Ⅲ级：IVH（＞50%脑室区域）伴脑室扩大；④Ⅳ级：脑室内出血合并脑实质出血或脑室周围出血性梗死。
|
[
{
"id": 0,
"entity": "头颅超声检查",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "Ⅰ级",
"start_offset": 17,
"end_offset": 19,
"label": "dis"
},
{
"id": 3,
"entity": "室管膜",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
},
{
"id": 4,
"entity": "脑室",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "脑室内出血",
"start_offset": 31,
"end_offset": 36,
"label": "sym"
},
{
"id": 6,
"entity": "Ⅱ级",
"start_offset": 38,
"end_offset": 40,
"label": "dis"
},
{
"id": 7,
"entity": "脑室",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
},
{
"id": 8,
"entity": "脑室扩张",
"start_offset": 43,
"end_offset": 47,
"label": "sym"
},
{
"id": 9,
"entity": "IVH",
"start_offset": 48,
"end_offset": 51,
"label": "dis"
},
{
"id": 10,
"entity": "Ⅲ级",
"start_offset": 53,
"end_offset": 55,
"label": "dis"
},
{
"id": 11,
"entity": "IVH",
"start_offset": 56,
"end_offset": 59,
"label": "dis"
},
{
"id": 12,
"entity": "脑室",
"start_offset": 64,
"end_offset": 66,
"label": "bod"
},
{
"id": 13,
"entity": "脑室",
"start_offset": 70,
"end_offset": 72,
"label": "bod"
},
{
"id": 14,
"entity": "Ⅳ级",
"start_offset": 76,
"end_offset": 78,
"label": "dis"
},
{
"id": 15,
"entity": "脑室内出血",
"start_offset": 79,
"end_offset": 84,
"label": "dis"
},
{
"id": 16,
"entity": "脑实质出血",
"start_offset": 86,
"end_offset": 91,
"label": "dis"
},
{
"id": 17,
"entity": "脑室周围出血性梗死",
"start_offset": 92,
"end_offset": 101,
"label": "dis"
}
] |
（二）CT检查CT是证实IVH部位和程度的有效手段，对硬膜下出血、后颅凹出血、蛛网膜下腔出血和某些脑实质的损害，CT的诊断价值优于超声。
|
[
{
"id": 0,
"entity": "CT检查",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "CT",
"start_offset": 7,
"end_offset": 9,
"label": "pro"
},
{
"id": 2,
"entity": "IVH",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 3,
"entity": "硬膜下出血",
"start_offset": 27,
"end_offset": 32,
"label": "dis"
},
{
"id": 4,
"entity": "后颅凹出血",
"start_offset": 33,
"end_offset": 38,
"label": "dis"
},
{
"id": 5,
"entity": "蛛网膜下腔出血",
"start_offset": 39,
"end_offset": 46,
"label": "dis"
},
{
"id": 6,
"entity": "CT",
"start_offset": 56,
"end_offset": 58,
"label": "pro"
},
{
"id": 7,
"entity": "超声",
"start_offset": 65,
"end_offset": 67,
"label": "pro"
}
] |
但CT不能床旁进行，还有使患儿暴露于放射线的缺点。
|
[
{
"id": 0,
"entity": "CT",
"start_offset": 1,
"end_offset": 3,
"label": "pro"
}
] |
（三）脑脊液检查IVH的脑脊液表现为出血早期脑脊液红细胞数量和蛋白含量增高，部分病例白细胞增高，然后脑脊液变为黄色，葡萄糖含量降低。
|
[
{
"id": 0,
"entity": "脑脊液检查",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "脑脊液",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "脑脊液红细胞",
"start_offset": 22,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "出血早期脑脊液红细胞数量和蛋白含量增高，部分病例白细胞增高",
"start_offset": 18,
"end_offset": 47,
"label": "sym"
},
{
"id": 5,
"entity": "脑脊液",
"start_offset": 50,
"end_offset": 53,
"label": "bod"
},
{
"id": 6,
"entity": "葡萄糖",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
}
] |
但是，有些病例脑脊液不呈血性，因此不能将腰椎穿刺作为IVH的确诊手段。
|
[
{
"id": 0,
"entity": "脑脊液",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "腰椎穿刺",
"start_offset": 20,
"end_offset": 24,
"label": "pro"
},
{
"id": 2,
"entity": "IVH",
"start_offset": 26,
"end_offset": 29,
"label": "dis"
}
] |
【预后】与出血的严重程度及部位有关，如出血仅限于生发基质或伴少量IVH者预后较好，很少发生脑室扩张。
|
[
{
"id": 0,
"entity": "出血",
"start_offset": 5,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "出血",
"start_offset": 19,
"end_offset": 21,
"label": "sym"
},
{
"id": 2,
"entity": "生发基质",
"start_offset": 24,
"end_offset": 28,
"label": "bod"
},
{
"id": 3,
"entity": "IVH",
"start_offset": 32,
"end_offset": 35,
"label": "dis"
},
{
"id": 4,
"entity": "脑室",
"start_offset": 45,
"end_offset": 47,
"label": "bod"
},
{
"id": 5,
"entity": "脑室扩张",
"start_offset": 45,
"end_offset": 49,
"label": "sym"
}
] |
中度出血者，病死率略为增高，存活者中20%～30%发生脑积水。
|
[
{
"id": 0,
"entity": "出血",
"start_offset": 2,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "脑积水",
"start_offset": 27,
"end_offset": 30,
"label": "dis"
}
] |
严重出血病例病死率20%～30%左右，存活者常发生脑积水。
|
[
{
"id": 0,
"entity": "脑积水",
"start_offset": 25,
"end_offset": 28,
"label": "dis"
}
] |
重度IVH伴脑室周围出血性梗死者，病死率和脑积水发生率均较高，分别为40%和70%。
|
[
{
"id": 0,
"entity": "IVH",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "脑室周围出血性梗死",
"start_offset": 6,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "脑积水",
"start_offset": 21,
"end_offset": 24,
"label": "dis"
}
] |
IVH的远期预后取决于伴随的脑实质损害的程度，如伴有脑室周围白质软化，可发生四肢对称性痉挛性瘫痪，下肢重于上肢。
|
[
{
"id": 0,
"entity": "IVH",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "脑实质损害",
"start_offset": 14,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "脑室",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 3,
"entity": "四肢",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "下肢",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
},
{
"id": 5,
"entity": "上肢",
"start_offset": 53,
"end_offset": 55,
"label": "bod"
},
{
"id": 6,
"entity": "脑室周围白质软化，可发生四肢对称性痉挛性瘫痪，下肢重于上肢",
"start_offset": 26,
"end_offset": 55,
"label": "sym"
}
] |
如伴有脑室周围出血性梗死，常造成早产儿痉挛性偏瘫。
|
[
{
"id": 0,
"entity": "脑室周围出血性梗死",
"start_offset": 3,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "痉挛性偏瘫",
"start_offset": 19,
"end_offset": 24,
"label": "sym"
}
] |
4.纠正凝血异常，可应用维生素K1</sub>等药物。
|
[
{
"id": 0,
"entity": "维生素K1",
"start_offset": 12,
"end_offset": 17,
"label": "dru"
}
] |
5.曾有人提出对极低出生体重儿出生后常规使用苯巴比妥预防IVH，但经过多中心对照试验未被证实能降低IVH的发生率或严重性，目前尚未在早产儿推荐应用。
|
[
{
"id": 0,
"entity": "苯巴比妥",
"start_offset": 22,
"end_offset": 26,
"label": "dru"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 28,
"end_offset": 31,
"label": "dis"
},
{
"id": 2,
"entity": "IVH",
"start_offset": 49,
"end_offset": 52,
"label": "dis"
}
] |
【治疗】（一）维持正常脑灌注大量IVH时，由于动脉压降低和颅内压增高，脑灌流减少，因此必须维持血压在足够的水平，同时避免血压的过度波动和脑血流速度的突然升高，没有必要的过分积极治疗反而会加重已经存在的脑损伤。
|
[
{
"id": 0,
"entity": "维持正常脑灌注",
"start_offset": 7,
"end_offset": 14,
"label": "pro"
},
{
"id": 1,
"entity": "IVH",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "动脉压",
"start_offset": 23,
"end_offset": 26,
"label": "ite"
},
{
"id": 3,
"entity": "颅内压",
"start_offset": 29,
"end_offset": 32,
"label": "ite"
},
{
"id": 4,
"entity": "血压",
"start_offset": 47,
"end_offset": 49,
"label": "ite"
},
{
"id": 5,
"entity": "血压",
"start_offset": 60,
"end_offset": 62,
"label": "ite"
},
{
"id": 6,
"entity": "脑损伤",
"start_offset": 100,
"end_offset": 103,
"label": "dis"
}
] |
（二）支持疗法维持正常通气，维持水、电解质和酸碱平衡，维持体温和代谢正常等。
|
[
{
"id": 0,
"entity": "支持疗法",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "维持正常通气",
"start_offset": 7,
"end_offset": 13,
"label": "pro"
},
{
"id": 2,
"entity": "维持水、电解质和酸碱平衡",
"start_offset": 14,
"end_offset": 26,
"label": "pro"
},
{
"id": 3,
"entity": "维持体温和代谢正常",
"start_offset": 27,
"end_offset": 36,
"label": "pro"
}
] |
（三）预防出血后脑积水脑脊液中的血液和蛋白质可引起蛛网膜炎及粘连，导致出血后脑积水，可连续腰椎穿刺放出血性脑脊液，在病情稳定后，每天或隔天1次，每次放2～3ml/kg，但连续腰椎穿刺对预防出血后脑积水的价值还有争议。
|
[
{
"id": 0,
"entity": "出血",
"start_offset": 5,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "脑积水",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "脑脊液",
"start_offset": 11,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "血液",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
},
{
"id": 4,
"entity": "蛋白质",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
},
{
"id": 5,
"entity": "蛛网膜炎",
"start_offset": 25,
"end_offset": 29,
"label": "dis"
},
{
"id": 6,
"entity": "出血",
"start_offset": 35,
"end_offset": 37,
"label": "sym"
},
{
"id": 7,
"entity": "脑积水",
"start_offset": 38,
"end_offset": 41,
"label": "dis"
},
{
"id": 8,
"entity": "腰椎穿刺",
"start_offset": 45,
"end_offset": 49,
"label": "pro"
},
{
"id": 9,
"entity": "腰椎穿刺",
"start_offset": 87,
"end_offset": 91,
"label": "pro"
},
{
"id": 10,
"entity": "脑积水",
"start_offset": 97,
"end_offset": 100,
"label": "dis"
}
] |
用纤溶药物已被尝试预防出血后脑积水的发生，但需要进一步证实。
|
[
{
"id": 0,
"entity": "纤溶药物",
"start_offset": 1,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "出血",
"start_offset": 11,
"end_offset": 13,
"label": "sym"
},
{
"id": 2,
"entity": "脑积水",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
}
] |
（四）出血后脑室扩张的处理急性期过后，应随访颅脑超声，评估脑室大小，随访间隔时间根据病程而定，病情越重，间隔时间越短，一般5～10天随访1次。
|
[
{
"id": 0,
"entity": "脑室",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "出血后脑室扩张",
"start_offset": 3,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "颅脑超声",
"start_offset": 22,
"end_offset": 26,
"label": "pro"
}
] |
根据超声测定脑室扩张的进展速率和严重程度，进行相应处理。
|
[
{
"id": 0,
"entity": "超声",
"start_offset": 2,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "脑室",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "脑室扩张",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
}
] |
对快速进展的脑室扩张（每周头围增长速率＞1.5～2cm），由于脑室扩张迅速，可在短期内发生明显的颅内压增高，应当积极治疗。
|
[
{
"id": 0,
"entity": "脑室",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "脑室扩张",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "脑室",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "脑室扩张",
"start_offset": 31,
"end_offset": 35,
"label": "sym"
},
{
"id": 4,
"entity": "颅内压",
"start_offset": 48,
"end_offset": 51,
"label": "ite"
},
{
"id": 5,
"entity": "颅内压增高",
"start_offset": 48,
"end_offset": 53,
"label": "sym"
}
] |
对缓慢进展的脑室扩张（＜4周），主要是严密观察，改变体位（床头抬高30°）有助于颅内压的降低。
|
[
{
"id": 0,
"entity": "脑室",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "脑室扩张",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "颅内压",
"start_offset": 40,
"end_offset": 43,
"label": "ite"
}
] |
因为有相当部分的患儿脑室扩张可自发停止，过早的干预不能改善其神经系统的远期预后。
|
[
{
"id": 0,
"entity": "脑室",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 1,
"entity": "脑室扩张",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "神经系统",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
}
] |
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