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其基本缺陷是血小板的致密颗粒内含物ATP、ADP、钙离子、焦磷酸盐、5-羟色胺等减少，其中ADP的减少较ATP更为显著。 | [
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δ-SPD的血小板对ADP或肾上腺素的诱导缺乏二相聚集波，胶原诱导的聚集反应降低或缺失，花生四烯酸引起聚集反应亦降低，电镜下可见致密颗粒减少。 | [
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"entity": "致密颗粒减少",
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α及δ颗粒同时发生缺陷者称为α、δ储存池病（αδ-SPD）其中δ颗粒内容物的减少往往更严重，α颗粒内容物的减少为轻到中度，临床及实验室检查类似δ-SPD。 | [
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致密体缺陷又可是其他遗传性疾病的一部分，如Hermansky-Pudlak综合征、Chediak-Higashi综合征、Wiskott-Aldrich综合征、成骨不全及血小板减少伴桡骨缺如综合征（TAR综合征）等。 | [
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1.Hermansky-Pudlak综合征本病属常染色体隐性遗传，特点是眼与皮肤的白化症、单核-巨噬系统内脂质样物质积聚和血小板功能缺陷性出血倾向。 | [
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"entity": "血小板功能缺陷性出血倾向",
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血小板中致密体减少或实际缺如，由于5-羟色胺、钙离子和腺嘌呤核苷酸水平很低，因此血小板对ADP、肾上腺素和凝血酶的诱导缺乏二相聚集波。 | [
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2.Chediak-Higashi综合征本病属常染色体隐性遗传，特点是中性粒细胞、单核细胞、成纤维细胞和黑色素细胞中常有异常颗粒形成，对细菌和真菌的易感性增高。 | [
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临床常有眼和皮肤的白化病，出血症较轻，检查粒细胞中异常颗粒常可帮助确诊。 | [
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3.Wiskott-Aldrich综合征本病属性联隐性遗传疾病，特点是细胞免疫缺陷引起的反复感染、湿疹和血小板减少性出血。 | [
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4.TAR综合征本病属常染色体隐性遗传，患儿有多发性畸形如骨骼、心、肾等，多见双侧桡骨缺如。 | [
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血小板常减少伴骨髓巨核细胞数量减少或缺如。 | [
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（二）α储存池病α储存池（α-SPD）又名灰色血小板综合征，这是因为瑞氏染色涂片上的血小板呈灰色的缘故。 | [
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电镜观察发现患者的巨核细胞及血小板特异性缺乏α颗粒。 | [
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生化研究发现病人血小板α颗粒蛋白质如血小板第四因子（PF4）、血小板球蛋白（β-TG）vWF、血小板凝血酶原敏感蛋白、纤维连接蛋白、因子Ⅴ、高分子量的激肽原、血小板生长因子等降低或缺乏。 | [
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进一步研究揭示α颗粒蛋白合成正常，但储存障碍；本病同时还兼有传导缺陷。 | [
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病人有轻度的血小板数降低，出血时间延长和出血倾向，血小板对凝血酶介导的聚集和释放反应异常，但对胶原和ADP诱导的聚集反应不变。 | [
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瑞氏染色血片上的灰色血小板常可提示诊断，确诊多依赖电镜观察结果。 | [
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第三节肠旋转不良小肠旋转及固定异常是宫内小肠发育过程受扰而致的解剖学异常，可引发肠梗阻或肠扭转，并可威胁生命，大多在婴儿及儿童期出现症状。 | [
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了解小肠胚胎发育对认识及手术治疗该病具有重要意义。 | [
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确定中肠旋转及固定异常的具体发病率相当困难，有报道称占存活婴儿的1/6000。 | [
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尸解发现有0.5%存在完全性肠旋转不良，即所有小肠位于腹腔右侧，结肠位于左侧。 | [
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胃肠道钡剂造影检查偶尔发现的无症状发病率为0.2%，见于任何年龄。 | [
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【胚胎学】孕早期时，胚胎中肠发育快于体腔发育，因此，胎儿发育至第4周时，中肠通常凸出腹腔，形成脐疝；孕10周左右，肠段回纳入腹腔，逐步完成旋转和固定，直至最终形成足月儿形态的小肠与结肠。 | [
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若中肠在旋转过程中受扰，生后即可能出现相应的临床症状。 | [
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中肠发育分为3个阶段。 | [
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（一）第一阶段脐疝形成孕4周，胎儿中肠开始向腹侧弯曲突起，进入体蒂腔，形成疝。 | [
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"entity": "体蒂腔",
"start_offset": 31,
"end_offset": 34,
"label": "bod"
},
{
"id": 3,
"entity": "疝",
"start_offset": 37,
"end_offset": 38,
"label": "dis"
}
] |
疝中轴为肠系膜上动脉（SMA）（图13-13A），并以此为标志，将中肠分为头侧段（动脉前，十二指肠空肠段）与尾侧段（动脉后，盲结肠段）。 | [
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"id": 0,
"entity": "疝中轴",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "肠系膜上动脉",
"start_offset": 4,
"end_offset": 10,
"label": "bod"
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{
"id": 2,
"entity": "SMA",
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{
"id": 3,
"entity": "中肠",
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"label": "bod"
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{
"id": 4,
"entity": "动脉",
"start_offset": 41,
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"label": "bod"
},
{
"id": 5,
"entity": "十二指肠空肠段",
"start_offset": 45,
"end_offset": 52,
"label": "bod"
},
{
"id": 6,
"entity": "动脉",
"start_offset": 58,
"end_offset": 60,
"label": "bod"
},
{
"id": 7,
"entity": "盲结肠段",
"start_offset": 62,
"end_offset": 66,
"label": "bod"
}
] |
第一阶段中肠头侧段发育快于尾侧段。 | [
{
"id": 0,
"entity": "中肠",
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"label": "bod"
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] |
1.头侧段的发育中肠疝形成并在体腔外继续生长过程中，头侧段被发育中的肝脏与左脐静脉推向下方，逆时针旋转90°至SMA右侧（图13-13B）；随着肠腔进一步发育，于第二阶段开始前，头侧段再旋转90°至SMA后方，共计旋转180°（图13-13C）。 | [
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"id": 0,
"entity": "中肠疝",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "体腔",
"start_offset": 15,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "肝脏",
"start_offset": 34,
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{
"id": 3,
"entity": "左脐静脉",
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{
"id": 4,
"entity": "SMA",
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"entity": "肠腔",
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"label": "bod"
},
{
"id": 6,
"entity": "SMA",
"start_offset": 99,
"end_offset": 102,
"label": "bod"
}
] |
2.尾侧段的发育第一阶段，中肠尾侧段与头侧段平行旋转，回盲部最初位于SMA下方（图13-13A），与头侧段首次90°旋转到达SMA右侧同步，回盲部逆时针旋转90°至SMA左侧（图13-13B）。 | [
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"entity": "中肠",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "头侧段",
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"label": "bod"
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{
"id": 2,
"entity": "回盲部",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
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{
"id": 3,
"entity": "SMA",
"start_offset": 34,
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"label": "bod"
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{
"id": 4,
"entity": "头侧段",
"start_offset": 50,
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"label": "bod"
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{
"id": 5,
"entity": "SMA",
"start_offset": 62,
"end_offset": 65,
"label": "bod"
},
{
"id": 6,
"entity": "回盲部",
"start_offset": 70,
"end_offset": 73,
"label": "bod"
},
{
"id": 7,
"entity": "SMA",
"start_offset": 82,
"end_offset": 85,
"label": "bod"
}
] |
在第10周回纳入腹腔前，回盲部继续旋转90°，此时恰位于SMA腹侧（图13-13C）。 | [
{
"id": 0,
"entity": "腹腔",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
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{
"id": 1,
"entity": "回盲部",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "SMA",
"start_offset": 28,
"end_offset": 31,
"label": "bod"
}
] |
（二）第二阶段中肠回纳入腹1.头侧段的发育肠段继续发育，于孕10周（此时胚胎长约40mm）开始返回腹腔，至孕11周完成。 | [
{
"id": 0,
"entity": "中肠",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "肠段",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "腹腔",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
}
] |
头侧段首先回纳，期间绕SMA继续旋转90°，共计逆时针旋转270°。 | [
{
"id": 0,
"entity": "头侧段",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "SMA",
"start_offset": 11,
"end_offset": 14,
"label": "bod"
}
] |
最终，十二指肠空肠连接部经屈氏韧带固定于SMA左侧的后腹壁（图13-4A）。 | [
{
"id": 0,
"entity": "十二指肠",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "空肠",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "屈氏韧带",
"start_offset": 13,
"end_offset": 17,
"label": "bod"
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{
"id": 3,
"entity": "SMA",
"start_offset": 20,
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"label": "bod"
},
{
"id": 4,
"entity": "后腹壁",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
}
] |
2.尾侧段的发育在盲结肠回纳入腹腔过程中，也继续旋转90°至SMA右侧，共计绕SMA逆时针旋转270°（13-4B）。 | [
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"id": 0,
"entity": "盲结肠",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
},
{
"id": 1,
"entity": "腹腔",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "SMA",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "SMA",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
}
] |
（三）第三阶段中肠固定从孕12周至出生，如中肠正常旋转，则结肠逐渐完成固定，腹膜形成束带样组织即升、降结肠系膜附着于后腹壁。 | [
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"id": 0,
"entity": "中肠",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "中肠",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "结肠",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
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{
"id": 3,
"entity": "腹膜",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "束带样组织",
"start_offset": 42,
"end_offset": 47,
"label": "bod"
},
{
"id": 5,
"entity": "升、降结肠系膜",
"start_offset": 48,
"end_offset": 55,
"label": "bod"
},
{
"id": 6,
"entity": "后腹壁",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
}
] |
如盲肠和升结肠旋转不彻底，未达到右侧腹，腹膜束带仍会把升结肠连至右结肠沟，此时束带横跨于十二指肠腹侧，压迫肠段造成梗阻。 | [
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"id": 0,
"entity": "盲肠",
"start_offset": 1,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "升结肠",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "腹膜束带",
"start_offset": 20,
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"label": "bod"
},
{
"id": 3,
"entity": "升结肠",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "右结肠沟",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
},
{
"id": 5,
"entity": "束带",
"start_offset": 39,
"end_offset": 41,
"label": "bod"
},
{
"id": 6,
"entity": "十二指肠",
"start_offset": 44,
"end_offset": 48,
"label": "bod"
},
{
"id": 7,
"entity": "肠段",
"start_offset": 53,
"end_offset": 55,
"label": "bod"
}
] |
【旋转不良分型】旋转不良的解剖异常主要还是根据头侧段和尾侧段来分型，固定异常通常只发生于盲结肠部。 | [
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"entity": "旋转不良",
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{
"id": 1,
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{
"id": 2,
"entity": "盲结肠部",
"start_offset": 44,
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"label": "bod"
}
] |
（一）完全不旋转临床上最为常见的是中肠完全不旋转，即头侧段和尾侧段均不旋转（图13-13）。 | [
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{
"id": 1,
"entity": "中肠完全不旋转",
"start_offset": 17,
"end_offset": 24,
"label": "dis"
}
] |
正常情况下，十二指肠旋转至SMA后方，屈氏韧带恰位于中线左侧胃窦水平。 | [
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"id": 0,
"entity": "十二指肠",
"start_offset": 6,
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{
"id": 1,
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{
"id": 2,
"entity": "屈氏韧带",
"start_offset": 19,
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},
{
"id": 3,
"entity": "胃窦",
"start_offset": 30,
"end_offset": 32,
"label": "bod"
}
] |
如果中肠不旋转，十二指肠长度变短，外观呈螺旋状，完全位于中线右侧，导致十二指肠不全梗阻；而肠段不固定，使中肠易发生扭转。 | [
{
"id": 0,
"entity": "中肠不旋转",
"start_offset": 2,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "十二指肠长度变短",
"start_offset": 8,
"end_offset": 16,
"label": "sym"
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{
"id": 2,
"entity": "外观呈螺旋状",
"start_offset": 17,
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"label": "sym"
},
{
"id": 3,
"entity": "完全位于中线右侧",
"start_offset": 24,
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"label": "sym"
},
{
"id": 4,
"entity": "十二指肠不全梗阻",
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"label": "dis"
},
{
"id": 5,
"entity": "肠段不固定",
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"label": "sym"
},
{
"id": 6,
"entity": "中肠易发生扭转",
"start_offset": 52,
"end_offset": 59,
"label": "sym"
}
] |
十二指肠和结肠的肠系膜包绕SMA彼此融合成一系膜蒂，中肠即以此为轴发生扭转。 | [
{
"id": 0,
"entity": "十二指肠和结肠的肠系膜包绕SMA彼此融合成一系膜蒂",
"start_offset": 0,
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"label": "sym"
},
{
"id": 1,
"entity": "中肠",
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"label": "bod"
}
] |
正常情况下，小肠系膜附着的基底部很宽，从左上腹屈氏韧带直至右下腹回盲部，小肠及系膜一般不可能发生轴性扭转；若近端空肠和远端回肠均位于中腹部，系膜附着部相对较窄，则扭转的可能性大大增加。 | [
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"entity": "小肠系膜",
"start_offset": 6,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "左上腹屈氏韧带",
"start_offset": 20,
"end_offset": 27,
"label": "bod"
},
{
"id": 2,
"entity": "右下腹回盲部",
"start_offset": 29,
"end_offset": 35,
"label": "bod"
},
{
"id": 3,
"entity": "小肠",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 4,
"entity": "系膜",
"start_offset": 39,
"end_offset": 41,
"label": "bod"
},
{
"id": 5,
"entity": "近端空肠",
"start_offset": 54,
"end_offset": 58,
"label": "bod"
},
{
"id": 6,
"entity": "远端回肠",
"start_offset": 59,
"end_offset": 63,
"label": "bod"
},
{
"id": 7,
"entity": "中腹部",
"start_offset": 66,
"end_offset": 69,
"label": "bod"
},
{
"id": 8,
"entity": "系膜",
"start_offset": 70,
"end_offset": 72,
"label": "bod"
}
] |
图13-2中肠头侧段旋转肠系膜上动脉（SMA）从主动脉右侧角发出，圆盘代表中肠头侧段绕SMA旋转的角度（A）十二指肠空肠段发生时（胚胎5mm大）位于SMA下面或0°；（B）在胚胎10mm时，十二指肠空肠段绕SMA逆时针旋转90°；（C）到胚胎40mm大时，十二指肠空肠段继续旋转，总共旋转180°图13-3中肠尾侧段旋转肠系膜上动脉（SMA）从主动脉右侧角发出，圆盘代表中肠尾侧段绕SMA旋转的角度（A）盲肠发生时（胚胎5mm大）位于SMA下面或0°；（B）在胚胎10mm时，盲肠绕SMA逆时针旋转90°；（C）到胚胎40mm大时，盲肠继续旋转，总共旋转180°，位于SMA腹侧图13-4胚胎时的中肠旋转完成（A）十二指肠空肠连接部的最终位置。 | [
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{
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{
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{
"id": 5,
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{
"id": 6,
"entity": "肠段",
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{
"id": 7,
"entity": "SMA",
"start_offset": 74,
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"label": "bod"
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{
"id": 8,
"entity": "十二指肠空肠段",
"start_offset": 95,
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"label": "bod"
},
{
"id": 9,
"entity": "SMA",
"start_offset": 103,
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"label": "bod"
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{
"id": 10,
"entity": "十二指肠空肠段",
"start_offset": 128,
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"label": "bod"
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{
"id": 11,
"entity": "中肠尾侧段旋转",
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{
"id": 12,
"entity": "肠系膜上动脉",
"start_offset": 160,
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"label": "bod"
},
{
"id": 13,
"entity": "SMA",
"start_offset": 167,
"end_offset": 170,
"label": "bod"
},
{
"id": 14,
"entity": "主动脉",
"start_offset": 172,
"end_offset": 175,
"label": "bod"
},
{
"id": 15,
"entity": "中肠尾侧段",
"start_offset": 185,
"end_offset": 190,
"label": "bod"
},
{
"id": 16,
"entity": "SMA",
"start_offset": 191,
"end_offset": 194,
"label": "bod"
},
{
"id": 17,
"entity": "盲肠",
"start_offset": 202,
"end_offset": 204,
"label": "bod"
},
{
"id": 18,
"entity": "SMA",
"start_offset": 217,
"end_offset": 220,
"label": "bod"
},
{
"id": 19,
"entity": "盲肠",
"start_offset": 238,
"end_offset": 240,
"label": "bod"
},
{
"id": 20,
"entity": "SMA",
"start_offset": 241,
"end_offset": 244,
"label": "bod"
},
{
"id": 21,
"entity": "盲肠",
"start_offset": 266,
"end_offset": 268,
"label": "bod"
},
{
"id": 22,
"entity": "SMA",
"start_offset": 284,
"end_offset": 287,
"label": "bod"
},
{
"id": 23,
"entity": "中肠",
"start_offset": 298,
"end_offset": 300,
"label": "bod"
},
{
"id": 24,
"entity": "十二指肠空肠",
"start_offset": 307,
"end_offset": 313,
"label": "bod"
}
] |
它发生时位于SMA腹侧，在逆时针旋转270°后，位于SMA左侧；（B）盲肠的最终位置。 | [
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"id": 0,
"entity": "SMA",
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{
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{
"id": 2,
"entity": "盲肠",
"start_offset": 35,
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}
] |
它发生时位于SMA的背侧，在绕SMA逆时针旋转270°后位于SMA右侧图13-5中肠完全不旋转十二指肠空肠连接部和盲肠都不旋转。 | [
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"id": 0,
"entity": "SMA",
"start_offset": 6,
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{
"id": 1,
"entity": "SMA",
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{
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"label": "sym"
}
] |
所有的小肠都位于SMA的右侧，而结肠位于SMA的左侧。 | [
{
"id": 0,
"entity": "所有的小肠都位于SMA的右侧",
"start_offset": 0,
"end_offset": 14,
"label": "sym"
},
{
"id": 1,
"entity": "结肠位于SMA的左侧",
"start_offset": 16,
"end_offset": 26,
"label": "sym"
}
] |
这种类型在肠旋转不良中最为常见，常可并发肠扭转（二）头侧段旋转异常如果只是头侧段不旋转，而尾侧段旋转和固定正常，亦可因结肠系膜束带的压迫，导致十二指肠梗阻（图13-6）。 | [
{
"id": 0,
"entity": "肠旋转不良",
"start_offset": 5,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "肠扭转",
"start_offset": 20,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "头侧段旋转异常",
"start_offset": 26,
"end_offset": 33,
"label": "dis"
},
{
"id": 3,
"entity": "结肠系膜束带",
"start_offset": 59,
"end_offset": 65,
"label": "bod"
},
{
"id": 4,
"entity": "十二指肠梗阻",
"start_offset": 71,
"end_offset": 77,
"label": "dis"
}
] |
但由于十二指肠空肠连接部至回盲部之间的系膜附着部仍相对较宽广，中肠扭转的可能性较小。 | [
{
"id": 0,
"entity": "十二指肠空肠连接部",
"start_offset": 3,
"end_offset": 12,
"label": "bod"
},
{
"id": 1,
"entity": "回盲部",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "系膜附着部",
"start_offset": 19,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "中肠",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
}
] |
（三）头侧段反方向旋转导致十二指肠位于SMA前面（正常应位于后方）；而尾侧段的逆向旋转则导致横结肠位于SAM后方（图13-13），出现结肠梗阻。 | [
{
"id": 0,
"entity": "头侧段反方向旋转",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "十二指肠位于SMA前面",
"start_offset": 13,
"end_offset": 24,
"label": "sym"
},
{
"id": 2,
"entity": "横结肠",
"start_offset": 46,
"end_offset": 49,
"label": "bod"
},
{
"id": 3,
"entity": "结肠梗阻",
"start_offset": 67,
"end_offset": 71,
"label": "dis"
}
] |
若尾侧段正常旋转，随着升结肠从左上腹旋转至右下腹，其系膜跨越SMA前方，覆盖头侧段发育而来的小肠，形成疝囊，称十二指肠旁疝（图13-8）。 | [
{
"id": 0,
"entity": "升结肠",
"start_offset": 11,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "左上腹",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "右下腹",
"start_offset": 21,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "系膜",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "SMA",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "小肠",
"start_offset": 46,
"end_offset": 48,
"label": "bod"
},
{
"id": 6,
"entity": "疝囊",
"start_offset": 51,
"end_offset": 53,
"label": "dis"
},
{
"id": 7,
"entity": "十二指肠旁疝",
"start_offset": 55,
"end_offset": 61,
"label": "dis"
}
] |
（四）头侧段不完全旋转导致十二指肠空肠连接部（屈氏韧带）位置低于其左上腹的正常位置；而尾侧段异常旋转可致中肠扭转。 | [
{
"id": 0,
"entity": "头侧段不完全旋转",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "十二指肠空肠连接部（屈氏韧带）位置低于其左上腹的正常位置",
"start_offset": 13,
"end_offset": 41,
"label": "sym"
},
{
"id": 2,
"entity": "中肠扭转",
"start_offset": 52,
"end_offset": 56,
"label": "dis"
}
] |
此外，区分头侧段不旋转或不完全旋转，并无客观标准，一般认为，只要屈氏韧带位于腹部中线右侧，即为不旋转。 | [
{
"id": 0,
"entity": "头侧段不旋转或不完全旋转",
"start_offset": 5,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "屈氏韧带位于腹部中线右侧",
"start_offset": 32,
"end_offset": 44,
"label": "sym"
}
] |
（五）尾侧段的旋转异常如果头侧段旋转正常，但尾侧段旋转异常，亦可引起中肠扭转。 | [
{
"id": 0,
"entity": "尾侧段的旋转异常",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "中肠扭转",
"start_offset": 34,
"end_offset": 38,
"label": "dis"
}
] |
与完全不旋转相同，此时屈氏韧带与回盲部之间的肠系膜根部附着处很窄。 | [
{
"id": 0,
"entity": "屈氏韧带与回盲部之间的肠系膜根部附着处很窄",
"start_offset": 11,
"end_offset": 32,
"label": "sym"
}
] |
（六）尾侧段不完全旋转可能导致结肠固定异常，回盲部的不完全固定可致盲肠扭转；而结肠肝曲固定不全时，则有结肠延伸至右上后腹壁的腹膜束带形成，导致十二指肠部分梗阻。 | [
{
"id": 0,
"entity": "尾侧段不完全旋转",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "结肠固定异常",
"start_offset": 15,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "回盲部的不完全固定",
"start_offset": 22,
"end_offset": 31,
"label": "dis"
},
{
"id": 3,
"entity": "盲肠扭转",
"start_offset": 33,
"end_offset": 37,
"label": "dis"
},
{
"id": 4,
"entity": "结肠肝曲固定不全",
"start_offset": 39,
"end_offset": 47,
"label": "dis"
},
{
"id": 5,
"entity": "结肠延伸至右上后腹壁的腹膜束带形成",
"start_offset": 51,
"end_offset": 68,
"label": "sym"
},
{
"id": 6,
"entity": "十二指肠部分梗阻",
"start_offset": 71,
"end_offset": 79,
"label": "dis"
}
] |
图13-6十二指肠空肠连接部未旋转而盲肠正常旋转。 | [
{
"id": 0,
"entity": "十二指肠",
"start_offset": 5,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "空肠",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "盲肠",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
}
] |
在临床上常因结肠系膜束带压迫引起十二指肠梗阻图13-7十二指肠空肠连接部反向旋转，位于SMA腹侧，同时结肠反向旋转，位于SMA背侧。 | [
{
"id": 0,
"entity": "结肠",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "十二指肠梗阻",
"start_offset": 16,
"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "十二指肠空肠连接部",
"start_offset": 27,
"end_offset": 36,
"label": "bod"
},
{
"id": 3,
"entity": "SMA",
"start_offset": 43,
"end_offset": 46,
"label": "bod"
},
{
"id": 4,
"entity": "结肠",
"start_offset": 51,
"end_offset": 53,
"label": "bod"
},
{
"id": 5,
"entity": "SMA",
"start_offset": 60,
"end_offset": 63,
"label": "bod"
}
] |
临床上常引起横结肠梗阻图13-8（A）十二指肠空肠连接部的反向旋转（横过SMA腹侧），但结肠旋转正常；（B）十二指肠旁疝【伴发畸形】中肠不旋转或旋转不全往往是先天性膈疝和腹壁缺损——脐膨出及腹裂的重要并发症。 | [
{
"id": 0,
"entity": "横结肠梗阻",
"start_offset": 6,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "十二指肠空肠连接部",
"start_offset": 19,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "SMA",
"start_offset": 36,
"end_offset": 39,
"label": "bod"
},
{
"id": 3,
"entity": "结肠",
"start_offset": 44,
"end_offset": 46,
"label": "bod"
},
{
"id": 4,
"entity": "十二指肠旁疝",
"start_offset": 54,
"end_offset": 60,
"label": "dis"
},
{
"id": 5,
"entity": "畸形",
"start_offset": 63,
"end_offset": 65,
"label": "dis"
},
{
"id": 6,
"entity": "中肠不旋转或旋转不全",
"start_offset": 66,
"end_offset": 76,
"label": "dis"
},
{
"id": 7,
"entity": "先天性膈疝",
"start_offset": 79,
"end_offset": 84,
"label": "dis"
},
{
"id": 8,
"entity": "腹壁缺损",
"start_offset": 85,
"end_offset": 89,
"label": "dis"
},
{
"id": 9,
"entity": "脐膨出",
"start_offset": 91,
"end_offset": 94,
"label": "dis"
},
{
"id": 10,
"entity": "腹裂",
"start_offset": 95,
"end_offset": 97,
"label": "dis"
}
] |
有报道称：30%～62%旋转不良患儿有伴发畸形，大多为消化道畸形。 | [
{
"id": 0,
"entity": "旋转不良",
"start_offset": 12,
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"label": "dis"
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{
"id": 1,
"entity": "畸形",
"start_offset": 21,
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"label": "dis"
},
{
"id": 2,
"entity": "消化道畸形",
"start_offset": 27,
"end_offset": 32,
"label": "dis"
}
] |
50%十二指肠闭锁患儿、1/3空回肠闭锁患者伴有肠旋转不良，原因之一是宫内肠扭转使肠系膜血供受阻，导致肠闭锁。 | [
{
"id": 0,
"entity": "十二指肠闭锁",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "空回肠闭锁",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "肠旋转不良",
"start_offset": 24,
"end_offset": 29,
"label": "dis"
},
{
"id": 3,
"entity": "宫内肠",
"start_offset": 35,
"end_offset": 38,
"label": "bod"
},
{
"id": 4,
"entity": "肠系膜",
"start_offset": 41,
"end_offset": 44,
"label": "bod"
},
{
"id": 5,
"entity": "肠闭锁",
"start_offset": 51,
"end_offset": 54,
"label": "dis"
}
] |
其他畸形包括：梅克尔憩室、十二指肠瓣膜或狭窄、巨结肠、肛门闭锁、食管闭锁合并食管气管瘘、先天性短肠、胆道闭锁、先天性心脏病、内脏反位、肠系膜囊肿和梨状腹综合征。 | [
{
"id": 0,
"entity": "畸形",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "梅克尔憩室",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "十二指肠瓣膜",
"start_offset": 13,
"end_offset": 19,
"label": "dis"
},
{
"id": 3,
"entity": "狭窄",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
},
{
"id": 4,
"entity": "肛门闭锁",
"start_offset": 27,
"end_offset": 31,
"label": "dis"
},
{
"id": 5,
"entity": "食管闭锁",
"start_offset": 32,
"end_offset": 36,
"label": "dis"
},
{
"id": 6,
"entity": "食管气管瘘",
"start_offset": 38,
"end_offset": 43,
"label": "dis"
},
{
"id": 7,
"entity": "先天性短肠",
"start_offset": 44,
"end_offset": 49,
"label": "dis"
},
{
"id": 8,
"entity": "胆道闭锁",
"start_offset": 50,
"end_offset": 54,
"label": "dis"
},
{
"id": 9,
"entity": "先天性心脏病",
"start_offset": 55,
"end_offset": 61,
"label": "dis"
},
{
"id": 10,
"entity": "内脏反位",
"start_offset": 62,
"end_offset": 66,
"label": "dis"
},
{
"id": 11,
"entity": "肠系膜囊肿",
"start_offset": 67,
"end_offset": 72,
"label": "dis"
},
{
"id": 12,
"entity": "梨状腹综合征",
"start_offset": 73,
"end_offset": 79,
"label": "dis"
}
] |
有报道发现家族性肠旋转不良、伴发颜面或肢体异常，提示该病可能与遗传有关。 | [
{
"id": 0,
"entity": "肠旋转不良",
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"label": "dis"
},
{
"id": 1,
"entity": "颜面或肢体异常",
"start_offset": 16,
"end_offset": 23,
"label": "dis"
}
] |
【临床表现】旋转不良可表现为：①中肠扭转；②十二指肠不全或完全梗阻，胆汁性呕吐；③间歇性或长期腹痛，间歇性腹泻或便血；④偶然发现的无症状者。 | [
{
"id": 0,
"entity": "旋转不良",
"start_offset": 6,
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"label": "dis"
},
{
"id": 1,
"entity": "中肠扭转",
"start_offset": 16,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "十二指肠不全或完全梗阻",
"start_offset": 22,
"end_offset": 33,
"label": "sym"
},
{
"id": 3,
"entity": "胆汁性呕吐",
"start_offset": 34,
"end_offset": 39,
"label": "sym"
},
{
"id": 4,
"entity": "间歇性或长期腹痛",
"start_offset": 41,
"end_offset": 49,
"label": "sym"
},
{
"id": 5,
"entity": "间歇性腹泻或便血",
"start_offset": 50,
"end_offset": 58,
"label": "sym"
}
] |
（一）中肠扭转中肠扭转（midgutvolvulus）多见于婴儿及儿童，但也可发生在其他年龄段，甚至成年后。 | [
{
"id": 0,
"entity": "中肠扭转",
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"label": "dis"
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{
"id": 1,
"entity": "中肠扭转",
"start_offset": 7,
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"label": "dis"
},
{
"id": 2,
"entity": "midgutvolvulus",
"start_offset": 12,
"end_offset": 26,
"label": "dis"
}
] |
系外科急诊，如果未能及时手术纠治，可因小肠坏死导致短肠与死亡。 | [
{
"id": 0,
"entity": "小肠坏死",
"start_offset": 19,
"end_offset": 23,
"label": "dis"
},
{
"id": 1,
"entity": "短肠",
"start_offset": 25,
"end_offset": 27,
"label": "dis"
}
] |
典型症状为新生儿突发胆汁性呕吐，呕吐尚与十二指肠折叠成角及腹膜束带压迫导致十二指肠梗阻有关。 | [
{
"id": 0,
"entity": "呕吐",
"start_offset": 13,
"end_offset": 15,
"label": "sym"
},
{
"id": 1,
"entity": "呕吐",
"start_offset": 16,
"end_offset": 18,
"label": "sym"
},
{
"id": 2,
"entity": "十二指肠",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "腹膜",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 4,
"entity": "十二指肠",
"start_offset": 37,
"end_offset": 41,
"label": "bod"
}
] |
肠梗阻、败血症及出血性颅高压也可引起胆汁性呕吐，需快速做出鉴别诊断。 | [
{
"id": 0,
"entity": "肠梗阻",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
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{
"id": 1,
"entity": "败血症",
"start_offset": 4,
"end_offset": 7,
"label": "dis"
},
{
"id": 2,
"entity": "出血性颅高压",
"start_offset": 8,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "胆汁性呕吐",
"start_offset": 18,
"end_offset": 23,
"label": "sym"
}
] |
一旦出现肠坏死，生存机会大大减少，保留足够长度小肠亦极度困难。 | [
{
"id": 0,
"entity": "肠坏死",
"start_offset": 4,
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"label": "dis"
},
{
"id": 1,
"entity": "小肠",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
}
] |
所以一旦发现存在旋转不良，即应手术纠治。 | [
{
"id": 0,
"entity": "旋转不良",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "手术",
"start_offset": 15,
"end_offset": 17,
"label": "pro"
}
] |
中肠扭转也可出现间歇性的症状，主要见于年长患儿。 | [
{
"id": 0,
"entity": "中肠扭转",
"start_offset": 0,
"end_offset": 4,
"label": "sym"
}
] |
包括慢性腹痛、间歇性呕吐（有时为非胆汁性）、厌食、体重下降、生长发育不良、肠道吸收障碍及腹泻等。 | [
{
"id": 0,
"entity": "慢性腹痛",
"start_offset": 2,
"end_offset": 6,
"label": "sym"
},
{
"id": 1,
"entity": "厌食",
"start_offset": 22,
"end_offset": 24,
"label": "sym"
},
{
"id": 2,
"entity": "体重下降",
"start_offset": 25,
"end_offset": 29,
"label": "sym"
},
{
"id": 3,
"entity": "生长发育不良",
"start_offset": 30,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "肠道吸收障碍",
"start_offset": 37,
"end_offset": 43,
"label": "sym"
},
{
"id": 5,
"entity": "腹泻",
"start_offset": 44,
"end_offset": 46,
"label": "sym"
}
] |
肠部分扭转者肠系膜静脉和淋巴回流受阻可致营养素吸收障碍、肠腔内蛋白质丢失；动脉供血不足致黏液缺血，出现黑便。 | [
{
"id": 0,
"entity": "肠部分扭转",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "肠系膜静脉和淋巴回流受阻可致营养素吸收障碍",
"start_offset": 6,
"end_offset": 27,
"label": "sym"
},
{
"id": 2,
"entity": "肠腔内蛋白质丢失",
"start_offset": 28,
"end_offset": 36,
"label": "sym"
},
{
"id": 3,
"entity": "动脉供血不足致黏液缺血",
"start_offset": 37,
"end_offset": 48,
"label": "sym"
},
{
"id": 4,
"entity": "黑便",
"start_offset": 51,
"end_offset": 53,
"label": "sym"
}
] |
（二）十二指肠梗阻十二指肠梗阻系头侧段的十二指肠空肠未绕SMA旋转或旋转不彻底，引起十二指肠折叠、扭曲，产生间歇性梗阻。 | [
{
"id": 0,
"entity": "十二指肠梗阻",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "十二指肠梗阻",
"start_offset": 9,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "十二指肠",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "SMA",
"start_offset": 28,
"end_offset": 31,
"label": "bod"
},
{
"id": 4,
"entity": "十二指肠折叠",
"start_offset": 42,
"end_offset": 48,
"label": "sym"
},
{
"id": 5,
"entity": "扭曲",
"start_offset": 49,
"end_offset": 51,
"label": "sym"
},
{
"id": 6,
"entity": "产生间歇性梗阻",
"start_offset": 52,
"end_offset": 59,
"label": "sym"
}
] |
此外，从升结肠行经十二指肠腹侧至右上后腹壁的先天性束带可压迫十二指肠，引起梗阻。 | [
{
"id": 0,
"entity": "升结肠",
"start_offset": 4,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "十二指肠",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "后腹壁",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "十二指肠",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
}
] |
典型症状为胆汁性呕吐（偶见非胆汁性）和腹痛，或二者兼有。 | [
{
"id": 0,
"entity": "胆汁性呕吐",
"start_offset": 5,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "腹痛",
"start_offset": 19,
"end_offset": 21,
"label": "sym"
}
] |
（三）间歇性慢性腹痛旋转不良者可出现间歇性或慢性腹痛，原因很多，且往往并存。 | [
{
"id": 0,
"entity": "间歇性慢性腹痛",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "旋转不良",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "慢性腹痛",
"start_offset": 22,
"end_offset": 26,
"label": "sym"
}
] |
间歇性肠扭转或其他原因所致肠梗阻均可引发肠腔扩张，产生痉挛性疼痛及呕吐；肠系膜静脉或淋巴系统部分或间断闭塞，可致肠壁、肠系膜及系膜淋巴结水肿，以上原因也可引起腹痛。 | [
{
"id": 0,
"entity": "间歇性肠扭转",
"start_offset": 0,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "肠梗阻",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "肠腔扩张",
"start_offset": 20,
"end_offset": 24,
"label": "sym"
},
{
"id": 3,
"entity": "痉挛性疼痛",
"start_offset": 27,
"end_offset": 32,
"label": "sym"
},
{
"id": 4,
"entity": "呕吐",
"start_offset": 33,
"end_offset": 35,
"label": "sym"
},
{
"id": 5,
"entity": "肠系膜静脉或淋巴系统部分或间断闭塞",
"start_offset": 36,
"end_offset": 53,
"label": "sym"
},
{
"id": 6,
"entity": "肠壁、肠系膜及系膜淋巴结水肿",
"start_offset": 56,
"end_offset": 70,
"label": "sym"
},
{
"id": 7,
"entity": "腹痛",
"start_offset": 79,
"end_offset": 81,
"label": "sym"
}
] |
部分扭转亦可引发慢性动脉供血不足，导致腹泻、慢性腹痛及餐后加重的肠绞痛，或因黏液缺血出现黑便。 | [
{
"id": 0,
"entity": "慢性动脉供血不足",
"start_offset": 8,
"end_offset": 16,
"label": "sym"
},
{
"id": 1,
"entity": "腹泻",
"start_offset": 19,
"end_offset": 21,
"label": "sym"
},
{
"id": 2,
"entity": "慢性腹痛",
"start_offset": 22,
"end_offset": 26,
"label": "sym"
},
{
"id": 3,
"entity": "餐后加重的肠绞痛",
"start_offset": 27,
"end_offset": 35,
"label": "sym"
},
{
"id": 4,
"entity": "黑便",
"start_offset": 44,
"end_offset": 46,
"label": "sym"
}
] |
（四）无症状患者因其他疾病行腹部手术或上下消化道造影检查时偶可发现无症状肠旋转不良。 | [
{
"id": 0,
"entity": "腹部手术",
"start_offset": 14,
"end_offset": 18,
"label": "pro"
},
{
"id": 1,
"entity": "上下消化道造影检查",
"start_offset": 19,
"end_offset": 28,
"label": "pro"
},
{
"id": 2,
"entity": "肠旋转不良",
"start_offset": 36,
"end_offset": 41,
"label": "dis"
}
] |
【诊断】凡怀疑旋转不良或中肠扭转，术前需行影像学检查，包括腹部平片、上下消化道造影，或两者兼之。 | [
{
"id": 0,
"entity": "旋转不良",
"start_offset": 7,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "中肠扭转",
"start_offset": 12,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "影像学检查",
"start_offset": 21,
"end_offset": 26,
"label": "pro"
},
{
"id": 3,
"entity": "腹部平片",
"start_offset": 29,
"end_offset": 33,
"label": "pro"
},
{
"id": 4,
"entity": "上下消化道造影",
"start_offset": 34,
"end_offset": 41,
"label": "pro"
}
] |
（一）腹部平片每个有胆汁性呕吐的新生儿都应立即接受影像学检查，通常为前后直立位及侧卧位腹部平片。 | [
{
"id": 0,
"entity": "腹部平片",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "胆汁性呕吐",
"start_offset": 10,
"end_offset": 15,
"label": "sym"
},
{
"id": 2,
"entity": "影像学检查",
"start_offset": 25,
"end_offset": 30,
"label": "pro"
},
{
"id": 3,
"entity": "腹部平片",
"start_offset": 43,
"end_offset": 47,
"label": "pro"
}
] |
婴儿呕吐胆汁样物，即使腹部平片表现正常，也不能排除肠旋转不良，需进一步检查明确诊断。 | [
{
"id": 0,
"entity": "呕吐胆汁样物",
"start_offset": 2,
"end_offset": 8,
"label": "sym"
},
{
"id": 1,
"entity": "腹部平片",
"start_offset": 11,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "肠旋转不良",
"start_offset": 25,
"end_offset": 30,
"label": "dis"
}
] |
中肠扭转患者腹部平片肠腔充气可完全正常，也可表现为完全性小肠梗阻，包括多个充气扩张肠袢及液平，此时可能已有肠坏死。 | [
{
"id": 0,
"entity": "中肠扭转",
"start_offset": 0,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "腹部平片",
"start_offset": 6,
"end_offset": 10,
"label": "pro"
},
{
"id": 2,
"entity": "完全性小肠梗阻",
"start_offset": 25,
"end_offset": 32,
"label": "sym"
},
{
"id": 3,
"entity": "多个充气扩张肠袢及液平",
"start_offset": 35,
"end_offset": 46,
"label": "sym"
},
{
"id": 4,
"entity": "肠坏死",
"start_offset": 53,
"end_offset": 56,
"label": "sym"
}
] |
若腹部平片提示肠梗阻，则无需进一步检查，应立即手术。 | [
{
"id": 0,
"entity": "腹部平片",
"start_offset": 1,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "肠梗阻",
"start_offset": 7,
"end_offset": 10,
"label": "dis"
}
] |
（二）上消化道造影当怀疑有肠旋转不良时，常规应行消化道造影检查，首选钡剂造影，也可选用水溶性造影剂。 | [
{
"id": 0,
"entity": "上消化道造影",
"start_offset": 3,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "肠旋转不良",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "消化道造影检查",
"start_offset": 24,
"end_offset": 31,
"label": "pro"
},
{
"id": 3,
"entity": "钡剂造影",
"start_offset": 34,
"end_offset": 38,
"label": "pro"
},
{
"id": 4,
"entity": "水溶性造影剂",
"start_offset": 43,
"end_offset": 49,
"label": "pro"
}
] |
经鼻胃管注入造影剂后，即可在透视下行动态观察，以获得更多有用信息。 | [
{
"id": 0,
"entity": "鼻胃管",
"start_offset": 1,
"end_offset": 4,
"label": "equ"
},
{
"id": 1,
"entity": "造影剂",
"start_offset": 6,
"end_offset": 9,
"label": "dru"
}
] |
需要指出，怀疑急性肠扭转时不宜行此检查。 | [
{
"id": 0,
"entity": "急性肠扭转",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
}
] |
如果肠旋转不良患儿未发生肠扭转，上消化道造影对确定十二指肠空肠连接部（屈氏韧带）位置具有十分重要的作用。 | [
{
"id": 0,
"entity": "肠旋转不良",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肠扭转",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "上消化道造影",
"start_offset": 16,
"end_offset": 22,
"label": "pro"
},
{
"id": 3,
"entity": "十二指肠空肠连接部",
"start_offset": 25,
"end_offset": 34,
"label": "bod"
},
{
"id": 4,
"entity": "韧带",
"start_offset": 37,
"end_offset": 39,
"label": "bod"
}
] |
正常情况下，该韧带应位于脊柱左侧胃窦水平，紧贴后腹壁。 | [
{
"id": 0,
"entity": "韧带",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "脊柱左侧胃窦水平",
"start_offset": 12,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "后腹壁",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
}
] |
如果有肠旋转不良，十二指肠未绕SMA发生正常旋转，则位于脊柱右侧十二指肠球部下方，且较正常部位更靠近腹侧。 | [
{
"id": 0,
"entity": "肠旋转不良",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "十二指肠",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "SMA",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "脊柱",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "十二指肠",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
}
] |
腹部平片中未能显示的充满液体的扩张肠段也可使十二指肠空肠连接部下移，造成旋转不良假象，此时可经肛门注入造影剂，以确定回盲部位置。 | [
{
"id": 0,
"entity": "腹部平片",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "肠段",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "十二指肠空肠连接部",
"start_offset": 22,
"end_offset": 31,
"label": "bod"
},
{
"id": 3,
"entity": "旋转不良",
"start_offset": 36,
"end_offset": 40,
"label": "dis"
},
{
"id": 4,
"entity": "肛门",
"start_offset": 47,
"end_offset": 49,
"label": "bod"
},
{
"id": 5,
"entity": "造影剂",
"start_offset": 51,
"end_offset": 54,
"label": "dru"
},
{
"id": 6,
"entity": "回盲部",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
}
] |
（三）钡剂灌肠尽管钡灌肠是诊断肠旋转不良的方法之一，但同上消化道造影相比，有较大局限性。 | [
{
"id": 0,
"entity": "肠旋转不良",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 1,
"entity": "上消化道造影",
"start_offset": 28,
"end_offset": 34,
"label": "pro"
}
] |
主要因为15%的正常婴儿回盲部游离或位置较高；而婴儿的结肠相对较长，给回盲部识别和摄片带来困难；更重要的是，肠旋转不良患者回盲部位置可能正常。 | [
{
"id": 0,
"entity": "回盲部",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "结肠",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 2,
"entity": "回盲部",
"start_offset": 35,
"end_offset": 38,
"label": "bod"
},
{
"id": 3,
"entity": "肠旋转不良",
"start_offset": 54,
"end_offset": 59,
"label": "dis"
},
{
"id": 4,
"entity": "回盲部",
"start_offset": 61,
"end_offset": 64,
"label": "bod"
}
] |
虽然存在以上局限，但如果钡灌肠检查发现全部结肠位于左腹部（见于完全不旋转），或（和）升结肠异常缩短且回盲部位于髂骨翼上方，亦可诊断肠旋转不良。 | [
{
"id": 0,
"entity": "钡灌肠",
"start_offset": 12,
"end_offset": 15,
"label": "pro"
},
{
"id": 1,
"entity": "结肠",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "升结肠",
"start_offset": 42,
"end_offset": 45,
"label": "bod"
},
{
"id": 3,
"entity": "回盲部",
"start_offset": 50,
"end_offset": 53,
"label": "bod"
},
{
"id": 4,
"entity": "肠旋转不良",
"start_offset": 65,
"end_offset": 70,
"label": "dis"
}
] |
（四）超声作为一种非创伤性检查，通过探查肠系膜上血管位置和走向，超声有助于确诊肠旋转不良。 | [
{
"id": 0,
"entity": "超声",
"start_offset": 3,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "非创伤性检查",
"start_offset": 9,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "肠系膜",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
},
{
"id": 3,
"entity": "超声",
"start_offset": 32,
"end_offset": 34,
"label": "pro"
},
{
"id": 4,
"entity": "肠旋转不良",
"start_offset": 39,
"end_offset": 44,
"label": "dis"
}
] |
但是，超声并不能精确诊断肠旋转不良。 | [
{
"id": 0,
"entity": "超声",
"start_offset": 3,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "肠旋转不良",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
}
] |
有作者报道，9例手术证实为肠旋转不良的患者，仅有6例B超发现肠系膜血管异常；另一组249例为排除幽门狭窄而接受超声检查患者中，仅9名发现肠系膜血管异常，其中SMV位于SMA左侧的5例均患有肠旋转不良，其余4例SMV位于SMA腹侧，但仅1例证实患有肠旋转不良。 | [
{
"id": 0,
"entity": "肠旋转不良",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
},
{
"id": 1,
"entity": "B超",
"start_offset": 26,
"end_offset": 28,
"label": "pro"
},
{
"id": 2,
"entity": "肠系膜",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "狭窄",
"start_offset": 50,
"end_offset": 52,
"label": "dis"
},
{
"id": 4,
"entity": "超声",
"start_offset": 55,
"end_offset": 57,
"label": "pro"
},
{
"id": 5,
"entity": "肠系膜",
"start_offset": 68,
"end_offset": 71,
"label": "bod"
},
{
"id": 6,
"entity": "SMV",
"start_offset": 78,
"end_offset": 81,
"label": "bod"
},
{
"id": 7,
"entity": "SMA",
"start_offset": 83,
"end_offset": 86,
"label": "bod"
},
{
"id": 8,
"entity": "肠旋转不良",
"start_offset": 94,
"end_offset": 99,
"label": "dis"
},
{
"id": 9,
"entity": "SMV",
"start_offset": 104,
"end_offset": 107,
"label": "bod"
},
{
"id": 10,
"entity": "SMA",
"start_offset": 109,
"end_offset": 112,
"label": "bod"
},
{
"id": 11,
"entity": "肠旋转不良",
"start_offset": 123,
"end_offset": 128,
"label": "dis"
}
] |
因肠旋转不良患者的肠系膜血管位置可能完全正常，而血管位置异常亦不能确定肠旋转不良，故而超声并非诊断肠旋转不良的首选方法，其作用仅限于排除呕吐患儿的幽门狭窄。 | [
{
"id": 0,
"entity": "肠旋转不良",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "肠系膜",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "肠旋转不良",
"start_offset": 35,
"end_offset": 40,
"label": "dis"
},
{
"id": 3,
"entity": "超声",
"start_offset": 43,
"end_offset": 45,
"label": "pro"
},
{
"id": 4,
"entity": "肠旋转不良",
"start_offset": 49,
"end_offset": 54,
"label": "dis"
},
{
"id": 5,
"entity": "呕吐",
"start_offset": 68,
"end_offset": 70,
"label": "sym"
},
{
"id": 6,
"entity": "幽门狭窄",
"start_offset": 73,
"end_offset": 77,
"label": "dis"
}
] |
【治疗】肠扭转肠梗阻患儿需急诊手术，首先应立即予静脉补液，同时置胃肠减压管、Foley导尿管，血液交叉配型，应用广谱抗生素。 | [
{
"id": 0,
"entity": "肠扭转",
"start_offset": 4,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肠梗阻",
"start_offset": 7,
"end_offset": 10,
"label": "dis"
},
{
"id": 2,
"entity": "静脉补液",
"start_offset": 24,
"end_offset": 28,
"label": "pro"
},
{
"id": 3,
"entity": "胃肠减压管",
"start_offset": 32,
"end_offset": 37,
"label": "pro"
},
{
"id": 4,
"entity": "Foley导尿管",
"start_offset": 38,
"end_offset": 46,
"label": "pro"
},
{
"id": 5,
"entity": "血液交叉配型",
"start_offset": 47,
"end_offset": 53,
"label": "pro"
},
{
"id": 6,
"entity": "广谱抗生素",
"start_offset": 56,
"end_offset": 61,
"label": "dru"
}
] |
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