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胰腺外分泌功能不足会导致营养不良、贪食及生长障碍、营养缺乏,尤其是脂溶性维生素、维生素B<sub>12</sub>以及必需脂肪酸的缺乏。
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(一)慢性钙化性胰腺炎1.遗传性胰腺炎是一种常染色体隐性遗传性疾病。
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遗传性胰腺炎的基因定位于7号染色体长臂。
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胰蛋白酶原的第117位的精氨酸被组氨酸替代,导致胰腺的自身消化并诱发胰腺炎。
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病理发现包括胰腺萎缩、纤维化及钙化,腺泡细胞几乎均萎缩,导管堵塞以及广泛的纤维化。
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体格检查和临床过程与其他原因所致的急性胰腺炎相似,急性期症状在4~8天后缓解。
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每次发作后都会加重胰腺组织损害。
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当发展为慢性胰腺炎时,淀粉酶和脂肪酶在急性发作时可以正常。
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2.青少年热带性(营养性)胰腺炎热带性(营养性)胰腺炎是儿童慢性胰腺炎较常见的原因。
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临床过程与其他类型的慢性胰腺炎类似。
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(二)慢性阻塞性胰腺炎1.胰腺分裂症胰腺分裂症在总人群中的发病率为5%~15%,是胰腺最常见的畸形。
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由于背侧和腹侧胰腺始基不能融合,导致胰尾、胰体和部分胰头通过相对狭窄的副胰管引流,而不是通过主胰管引流。
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许多学者认为胰腺分裂症和复发性胰腺炎有关。
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ERCP可以诊断胰腺分裂症。
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2.腹部外伤腹部受外伤后,胰腺导管的隐性损伤可以导致狭窄、假性囊肿形成以及慢性阻塞。
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(三)特发性纤维化胰腺炎特发性纤维化胰腺炎很罕见,可以有腹痛或阻塞性黄疸。
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(四)其他高脂血症Ⅰ、Ⅳ、Ⅴ型患者可以发生胰腺炎。
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胰腺炎时可以出现一过性高脂血症,所以急性胰腺炎时的血脂升高必须在好转后重测。
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【诊断】根据患者有典型的胰腺炎病史,以及影像学上有慢性征象,慢性胰腺炎很容易诊断。
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更典型的是患者有反复发作的腹痛、呕吐及血清淀粉酶升高。
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3.胰腺功能测试如核素脂肪试验、CCK-促胰液素、BT-PABA试验等。
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无任何胰功能试验有足够敏感性可诊断出轻度早期甚至中度胰腺炎。
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4.腹部平片腹部平片见到胰腺钙化,则慢性胰腺炎的诊断成立。
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5.ERCP诊断慢性胰腺炎的敏感性最高。
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【治疗】1.疼痛对于儿童慢性胰腺炎,控制疼痛很重要,但有时却很困难。
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可以用甾体类或非甾体类抗炎药;补充胰酶以抑制胆囊收缩素的分泌;药物治疗失败时,可行内镜下或外科手术解除胰管梗阻。
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2.吸收不良慢性胰腺炎时吸收不良常见,但儿童中的发生率尚不清楚。
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第十二节膀胱外翻与尿道上裂膀胱外翻和尿道上裂是由泄殖腔发育异常所导致的一组相互关联的泌尿生殖系畸形,尤其是膀胱外翻,是最为复杂的小儿先天性畸形之一。
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一、膀胱外翻膀胱外翻是1597年由VonGrafenberg首先描述,1780年Chaussier始用膀胱外翻(bladderexstrophy)一词。
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1885年,Nyman在一位5天的膀胱外翻新生儿中进行了膀胱关闭。
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其后的实践包括试用骨盆截骨使耻骨靠近、回肠扩大膀胱提高膀胱容量等。
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1942年Young和1948年Micbon分别报道首例女性及男性膀胱外翻关闭术后能控制排尿。
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膀胱外翻并不常见,发病率为1/10000~1/50000,男性为女性1.7~2.3倍,有一定的遗传倾向,如报告膀胱外翻和尿道上裂患者子女的发病率为1/70,是正常人群发病率的500倍。
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【胚胎发生】胚胎3周时后肠末端和尿囊基部的扩大部分成为泄殖腔,其尾端有一层由内、外胚层组成的薄膜与羊膜腔分隔,称为泄殖腔膜。
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胚胎第4~7周泄殖腔被尿直肠膈分为背侧的直肠与腹侧的尿生殖窦,尿直肠膈与泄殖腔膜会合处形成会阴体。
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胚胎第4~10周时泄殖腔膜内、外胚层之间有间充质向内生长,发育成下腹部的肌肉和耻骨,构成脐以下的腹壁。
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泄殖腔膜发育不正常将阻碍间充质组织的移行,影响下腹壁发育。
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如泄殖腔膜有破溃,则其破溃的位置和时间的不同决定了膀胱外翻、尿道上裂系列畸形的各种类型。
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其中典型膀胱外翻占50%~60%,尿道上裂约占30%,最严重的泄殖腔发育异常是泄殖腔外翻(cloacalexstrophy),占l0%,其他畸形包括尿道上裂合并重复膀胱等。
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【临床表现】膀胱外翻涉及整个下腹部及盆腔脏器的发育异常,包括腹壁肌肉、骨盆骨骼、泌尿生殖系统及直肠肛门。
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骨骼肌肉异常表现为耻骨联合分离、骨盆外旋、耻骨支外旋及外转。
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分离的耻骨之间三角形筋膜缺损由外翻膀胱占据,膀胱上端是脐,位置低于两侧髂嵴连线,脐与肛门之间距离缩短。
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泌尿系统异常表现为下腹壁缺如,膀胱外翻,可见喷尿的两侧输尿管口。
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出生时外翻的膀胱黏膜正常,异位肠黏膜或岛状肠襻可位于外翻膀胱表面。
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长期暴露的黏膜可有鳞状上皮化生、炎性水肿及炎性息肉。
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膀胱容量差别很大。
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如膀胱过小,严重纤维化,难于做功能性修复。
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由于阴茎海绵体附着于耻骨下支,耻骨联合分离使两侧阴茎海绵体分离很宽,阴茎变短,阴茎严重向背侧弯曲。
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阴茎头靠近精阜,尿道板短。
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女性尿道短,阴道口前移并常有狭窄,阴蒂对裂,阴唇阴阜分开。
[ { "id": 0, "entity": "尿道", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "阴道口前移并常有狭窄", "start_offset": 6, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "阴蒂对裂", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "阴唇阴阜分开", "start_offset": 22, "end_offset": 28, "label": "sym" } ]
子宫、输卵管及卵巢一般正常,有时副中肾管结构重复。
[ { "id": 0, "entity": "子宫", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "输卵管", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 2, "entity": "卵巢", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 3, "entity": "副中肾管结构重复", "start_offset": 16, "end_offset": 24, "label": "sym" } ]
如有肛提肌、坐骨直肠肌以及外括约肌异常,可引起不同程度肛门失禁以及脱肛。
[ { "id": 0, "entity": "肛提肌", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "坐骨直肠肌", "start_offset": 6, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "外括约肌", "start_offset": 13, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "肛门失禁", "start_offset": 27, "end_offset": 31, "label": "dis" }, { "id": 4, "entity": "脱肛", "start_offset": 33, "end_offset": 35, "label": "dis" } ]
【治疗】手术重建是唯一的治疗方法,包括修复腹壁和外翻膀胱,在男性重建外观接近正常并且有性功能的阴茎,使能控制排尿,保护肾功能。
[ { "id": 0, "entity": "手术重建", "start_offset": 4, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "修复腹壁和外翻膀胱", "start_offset": 19, "end_offset": 28, "label": "pro" }, { "id": 2, "entity": "阴茎", "start_offset": 47, "end_offset": 49, "label": "bod" } ]
手术多主张分期完成,新生儿期修复腹壁和外翻膀胱,至2岁时修复尿道上裂,3~4岁时做双侧抗反流输尿管移植、后尿道延长及膀胱颈紧缩成形术。
[ { "id": 0, "entity": "新生儿期修复腹壁和外翻膀胱", "start_offset": 10, "end_offset": 23, "label": "pro" }, { "id": 1, "entity": "2岁时修复尿道上裂", "start_offset": 25, "end_offset": 34, "label": "pro" }, { "id": 2, "entity": "3~4岁时做双侧抗反流输尿管移植、后尿道延长及膀胱颈紧缩成形术", "start_offset": 35, "end_offset": 66, "label": "pro" } ]
如膀胱容量小,同时进行膀胱扩大。
[ { "id": 0, "entity": "膀胱容量小", "start_offset": 1, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "进行膀胱扩大", "start_offset": 9, "end_offset": 15, "label": "pro" } ]
在修复腹壁和外翻膀胱时必须做髂骨截骨。
[ { "id": 0, "entity": "修复腹壁和外翻膀胱时必须做髂骨截骨", "start_offset": 1, "end_offset": 18, "label": "pro" } ]
多数作者主张在生后72小时内做膀胱内翻缝合。
[ { "id": 0, "entity": "膀胱内翻缝合", "start_offset": 15, "end_offset": 21, "label": "pro" } ]
其优点为:膀胱壁柔软易于复位;尽早使膀胱黏膜不受外界刺激,避免一系列继发改变和废用性膀胱萎缩;有助于排尿控制。
[ { "id": 0, "entity": "膀胱壁", "start_offset": 5, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "膀胱黏膜", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "废用性膀胱萎缩", "start_offset": 39, "end_offset": 46, "label": "dis" } ]
1998年,美国西雅图儿童医院的Grady和Mitchell报告在新生儿期一期完成腹壁修复、骨盆成形、膀胱成形和尿道成形,16例外观均非常满意,其中11例已接受排尿训练,可以尿不失禁。
[ { "id": 0, "entity": "腹壁修复", "start_offset": 41, "end_offset": 45, "label": "pro" }, { "id": 1, "entity": "骨盆成形", "start_offset": 46, "end_offset": 50, "label": "pro" }, { "id": 2, "entity": "膀胱成形和尿道成形", "start_offset": 51, "end_offset": 60, "label": "pro" } ]
膀胱外翻的一系列修复手术的效果应该说是在逐渐进步中,但还难令人满意,如患儿控制排尿率约在60%左右。
[ { "id": 0, "entity": "膀胱外翻", "start_offset": 0, "end_offset": 4, "label": "dis" } ]
一部分患儿要做尿流改道。
[ { "id": 0, "entity": "尿流改道", "start_offset": 7, "end_offset": 11, "label": "pro" } ]
第五章儿童淋巴瘤第一节儿童霍奇金病1832年,霍奇金(Hodgkin)首先对本病在解剖学水平进行描述,因此而命名为霍奇金病(Hodgkindisease,HD),当时认为它是一种脾脏和淋巴结异常性疾病。
[ { "id": 0, "entity": "儿童淋巴瘤", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "儿童霍奇金病", "start_offset": 11, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "霍奇金病", "start_offset": 57, "end_offset": 61, "label": "dis" }, { "id": 3, "entity": "Hodgkindisease", "start_offset": 62, "end_offset": 76, "label": "dis" }, { "id": 4, "entity": "HD", "start_offset": 77, "end_offset": 79, "label": "dis" }, { "id": 5, "entity": "脾脏", "start_offset": 89, "end_offset": 91, "label": "bod" }, { "id": 6, "entity": "淋巴结", "start_offset": 92, "end_offset": 95, "label": "bod" } ]
直至19世纪50年代以后,由于显微镜技术的发展才对本病有了更进一步的了解,镜下观察到巨大畸形的细胞作为霍奇金病的诊断依据。
[ { "id": 0, "entity": "显微镜", "start_offset": 15, "end_offset": 18, "label": "equ" }, { "id": 1, "entity": "巨大畸形的细胞", "start_offset": 42, "end_offset": 49, "label": "sym" }, { "id": 2, "entity": "霍奇金病", "start_offset": 51, "end_offset": 55, "label": "dis" } ]
Sternberg和Reed分别在1898年和1902年对霍奇金病的组织病理学变化作了全面的定义和说明。
[ { "id": 0, "entity": "霍奇金病", "start_offset": 29, "end_offset": 33, "label": "dis" }, { "id": 1, "entity": "组织", "start_offset": 34, "end_offset": 36, "label": "bod" } ]
霍奇金病的浸润细胞有多样性,多数为形态正常的反应性细胞,其中的R-S细胞由相对成熟的淋巴细胞恶性转化而来。
[ { "id": 0, "entity": "霍奇金病", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "浸润细胞", "start_offset": 5, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "R-S细胞", "start_offset": 31, "end_offset": 36, "label": "bod" }, { "id": 3, "entity": "淋巴细胞", "start_offset": 42, "end_offset": 46, "label": "bod" } ]
【流行病学】根据我国上海市肿瘤登记系统,1986~1992年间,0~14岁组儿童HD的年发病率为2.39/百万,男女比为2.3∶1。
[ { "id": 0, "entity": "儿童HD", "start_offset": 38, "end_offset": 42, "label": "dis" } ]
【组织病理学】病变组织中常有正常淋巴细胞、浆细胞、嗜酸粒细胞、组织细胞反应性浸润,伴有细胞形态异常的R-S细胞。
[ { "id": 0, "entity": "淋巴细胞", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 1, "entity": "浆细胞", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "嗜酸粒细胞", "start_offset": 25, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "组织细胞", "start_offset": 31, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "R-S细胞", "start_offset": 50, "end_offset": 55, "label": "bod" } ]
R-S细胞大而畸形,直径≥15~45μm,有丰富的胞质,多核或多叶核,核膜染色深,有细致的染色质网,在核仁周围形成淡染的圈影、核仁大而明显。
[ { "id": 0, "entity": "R-S细胞", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "胞质", "start_offset": 25, "end_offset": 27, "label": "bod" }, { "id": 2, "entity": "核膜", "start_offset": 35, "end_offset": 37, "label": "bod" }, { "id": 3, "entity": "染色质网", "start_offset": 45, "end_offset": 49, "label": "bod" }, { "id": 4, "entity": "核仁", "start_offset": 51, "end_offset": 53, "label": "bod" }, { "id": 5, "entity": "核仁", "start_offset": 63, "end_offset": 65, "label": "bod" }, { "id": 6, "entity": "R-S细胞大而畸形,直径≥15~45μm,有丰富的胞质,多核或多叶核,核膜染色深,有细致的染色质网,在核仁周围形成淡染的圈影、核仁大而明显", "start_offset": 0, "end_offset": 69, "label": "sym" } ]
未见到R-S细胞时很难诊断本病,但在其他一些疾病中如传染性单核细胞增多症、非霍奇金淋巴瘤及其他非淋巴系恶性肿瘤中也可见到类似细胞。
[ { "id": 0, "entity": "R-S细胞", "start_offset": 3, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "单核细胞增多症", "start_offset": 29, "end_offset": 36, "label": "dis" }, { "id": 2, "entity": "非霍奇金淋巴瘤", "start_offset": 37, "end_offset": 44, "label": "dis" }, { "id": 3, "entity": "非淋巴系恶性肿瘤", "start_offset": 47, "end_offset": 55, "label": "dis" } ]
1966年提出RYE分类系统,将HD分为4个组织学亚型。
[ { "id": 0, "entity": "HD", "start_offset": 16, "end_offset": 18, "label": "dis" } ]
表11-12HD分型标准变迁及WHO-2008分型标准【临床表现】(一)全身症状非特异性症状包括发热、乏力、厌食、轻度消瘦、瘙痒。
[ { "id": 0, "entity": "HD", "start_offset": 6, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "发热", "start_offset": 48, "end_offset": 50, "label": "sym" }, { "id": 2, "entity": "乏力", "start_offset": 51, "end_offset": 53, "label": "sym" }, { "id": 3, "entity": "厌食", "start_offset": 54, "end_offset": 56, "label": "sym" }, { "id": 4, "entity": "轻度消瘦", "start_offset": 57, "end_offset": 61, "label": "sym" }, { "id": 5, "entity": "瘙痒", "start_offset": 62, "end_offset": 64, "label": "sym" } ]
(二)淋巴结肿大无痛性锁骨上、颈部或其他部位淋巴结肿大淋巴结质硬有橡皮样感觉。
[ { "id": 0, "entity": "淋巴结肿大", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "锁骨", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "颈部", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "淋巴结", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "无痛性锁骨上、颈部或其他部位淋巴结肿大", "start_offset": 8, "end_offset": 27, "label": "sym" }, { "id": 5, "entity": "淋巴结质硬有橡皮样感觉", "start_offset": 27, "end_offset": 38, "label": "sym" } ]
肿瘤原发于锁骨上、颈部较多见,腋下、腹股沟、腹腔淋巴结为原发者相对少见。
[ { "id": 0, "entity": "肿瘤", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "锁骨", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "颈部", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 3, "entity": "腋下", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 4, "entity": "腹股沟", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 5, "entity": "腹腔淋巴结", "start_offset": 22, "end_offset": 27, "label": "bod" } ]
(三)可合并免疫功能紊乱如合并免疫性溶血性贫血,有贫血、黄疸、网织红细胞升高、Coomb试验阳性。
[ { "id": 0, "entity": "免疫功能紊乱", "start_offset": 6, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "免疫性溶血性贫血", "start_offset": 15, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "贫血", "start_offset": 25, "end_offset": 27, "label": "sym" }, { "id": 3, "entity": "黄疸", "start_offset": 28, "end_offset": 30, "label": "sym" }, { "id": 4, "entity": "网织红细胞升高", "start_offset": 31, "end_offset": 38, "label": "sym" } ]
合并免疫性血小板减少症时,有血小板减少、出血倾向、血小板相关抗体增高髓巨核细胞成熟障碍。
[ { "id": 0, "entity": "免疫性血小板减少症", "start_offset": 2, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "血小板减少", "start_offset": 14, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "出血倾向", "start_offset": 20, "end_offset": 24, "label": "sym" }, { "id": 3, "entity": "抗体", "start_offset": 30, "end_offset": 32, "label": "bod" }, { "id": 4, "entity": "血小板相关抗体增高", "start_offset": 25, "end_offset": 34, "label": "sym" }, { "id": 5, "entity": "髓巨核细胞成熟障碍", "start_offset": 34, "end_offset": 43, "label": "sym" } ]
【实验室检查】(一)血液学检查血常规检查常无特异性异常,偶可见到嗜酸粒细胞或单核细胞增多。
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血沉可增快。
[ { "id": 0, "entity": "血沉可增快", "start_offset": 0, "end_offset": 5, "label": "sym" } ]
(二)淋巴结活检病理组织形态检查是确诊的必需手段。
[ { "id": 0, "entity": "淋巴结活检", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "病理组织形态检查", "start_offset": 8, "end_offset": 16, "label": "pro" } ]
(三)影像学检查可选择性作胸部X线平片、腹部B超、胸部CT、腹部CT,以确定病变的范围。
[ { "id": 0, "entity": "影像学检查", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "胸部X线平片", "start_offset": 13, "end_offset": 19, "label": "pro" }, { "id": 2, "entity": "腹部B超", "start_offset": 20, "end_offset": 24, "label": "pro" }, { "id": 3, "entity": "胸部CT", "start_offset": 25, "end_offset": 29, "label": "pro" }, { "id": 4, "entity": "腹部CT", "start_offset": 30, "end_offset": 34, "label": "pro" } ]
由于67</sup>镓对淋巴组织亲和力高,可作67</sup>镓扫描作为补充检查确定肿瘤浸润范围。
[ { "id": 0, "entity": "淋巴组织", "start_offset": 12, "end_offset": 16, "label": "bod" } ]
(四)骨髓活检HD可发生灶性骨髓转移,因此骨髓活检比骨髓涂片容易发现肿瘤细胞,在治疗前应常规作骨髓活检。
[ { "id": 0, "entity": "骨髓活检", "start_offset": 3, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "HD", "start_offset": 7, "end_offset": 9, "label": "dis" }, { "id": 2, "entity": "骨髓活检", "start_offset": 21, "end_offset": 25, "label": "pro" }, { "id": 3, "entity": "骨髓涂片", "start_offset": 26, "end_offset": 30, "label": "pro" }, { "id": 4, "entity": "肿瘤细胞", "start_offset": 34, "end_offset": 38, "label": "bod" }, { "id": 5, "entity": "骨髓活检", "start_offset": 47, "end_offset": 51, "label": "pro" } ]
【诊断】完整的诊断必须包括疾病分期,以指导临床治疗与随访,根据体格检查及相关实验室检查可作出分期诊断,较常用的HD分期系统为AnnArbor分期(表11-11)。
[ { "id": 0, "entity": "HD", "start_offset": 55, "end_offset": 57, "label": "dis" } ]
表11-13HD的分期【治疗】主要的治疗手段为化疗和放疗。
[ { "id": 0, "entity": "HD", "start_offset": 6, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "化疗", "start_offset": 23, "end_offset": 25, "label": "pro" }, { "id": 2, "entity": "放疗", "start_offset": 26, "end_offset": 28, "label": "pro" } ]
(一)放疗HD对放疗敏感,20世纪70年代以前,无论年龄、分型和分期的差别均采用放疗。
[ { "id": 0, "entity": "放疗", "start_offset": 3, "end_offset": 5, "label": "pro" }, { "id": 1, "entity": "放疗", "start_offset": 8, "end_offset": 10, "label": "pro" }, { "id": 2, "entity": "放疗", "start_offset": 40, "end_offset": 42, "label": "pro" } ]
目前对生长期儿童主要采用联合化疗加肿瘤浸润野低剂量放疗,有试图进一步减少或删除放疗的倾向。
[ { "id": 0, "entity": "联合化疗", "start_offset": 12, "end_offset": 16, "label": "pro" }, { "id": 1, "entity": "浸润野低剂量放疗", "start_offset": 19, "end_offset": 27, "label": "pro" }, { "id": 2, "entity": "放疗", "start_offset": 39, "end_offset": 41, "label": "pro" } ]
对已完全发育的青少年局限性病变采用肿瘤扩大野高剂量放疗。
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常用的放疗野有以下几个:1.Waldeyer野用于Waldeyer或耳前淋巴结病变。
[ { "id": 0, "entity": "放疗", "start_offset": 3, "end_offset": 5, "label": "pro" }, { "id": 1, "entity": "Waldeyer", "start_offset": 25, "end_offset": 33, "label": "dis" }, { "id": 2, "entity": "耳前淋巴结病变", "start_offset": 34, "end_offset": 41, "label": "dis" } ]
上颈部病变并以放疗为单一治疗手段时应同时作Waldeyer野预防性放疗。
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2.横膈上斗篷样野包括颌下、颏下、颈部、锁骨上下、腋下、纵隔和肺门淋巴结。
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3.横膈下野包括脾和主动脉旁淋巴结。
[ { "id": 0, "entity": "脾", "start_offset": 8, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "主动脉旁淋巴结", "start_offset": 10, "end_offset": 17, "label": "bod" } ]
4.倒“Y”野包括髂总、髂外、腹股沟淋巴结。
[ { "id": 0, "entity": "髂总", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "髂外", "start_offset": 12, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "腹股沟淋巴结", "start_offset": 15, "end_offset": 21, "label": "bod" } ]
(二)化疗经典联合化疗方案MOPP对成人与儿童的晚期HD有50%的治愈率。
[ { "id": 0, "entity": "化疗", "start_offset": 3, "end_offset": 5, "label": "pro" }, { "id": 1, "entity": "联合化疗", "start_offset": 7, "end_offset": 11, "label": "pro" }, { "id": 2, "entity": "HD", "start_offset": 26, "end_offset": 28, "label": "dis" } ]
化疗剂量宜大,但过长的维持治疗并不延长缓解期,根据不同分期以4~6个疗程为宜。
[ { "id": 0, "entity": "化疗", "start_offset": 0, "end_offset": 2, "label": "pro" } ]
表11-14HD化疗方案*</sup>MOPP中M为氮芥**</sup>COPP中C为环磷酰胺【预后】HD在合理的治疗下预后良好,治愈率可达80%~90%,但反复复发的晚期广泛病变预后仍不良,HD可见远期复发。
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常见的与放疗、化疗相关并影响远期生活质量的并发症有放疗部位的软组织、骨骼发育不良及畸形,放疗野内脏器功能障碍,心、肺功能障碍,不育和第二肿瘤等。
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第三章小儿急性肺损伤和急性呼吸窘迫综合征【概述】小儿呼吸系统危重症中急性呼吸窘迫综合征(acuterespiratorydistresssyndrome,ARDS)是由于多种原发病和诱因作用下发生的急性呼吸衰竭,以非心源性肺水肿和顽固性低氧血症为特征,表现为肺顺应性降低、严重呼吸困难、呼吸窘迫;其病理特征为肺泡毛细血管损伤、膜通透性增高、肺表面活性物质失去功能、弥漫性肺损伤和蛋白性肺泡水肿液聚集,发病机制本质上是“全身炎症反应综合征在肺部的表现”。
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与新生儿RDS不同的是,ARDS起病多有一段时间,可以由肺内、外多种原因造成,多见于感染、严重外伤等。
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小儿ARDS可被作为重症肺炎、呼吸衰竭进行诊治。
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ARDS早期肺病变为急性肺损伤(acutelunginjury,ALI),由于病情发展迅速,ALI发展为ARDS往往是不典型的;因此,在儿科危重呼吸疾病中发现临床病例特点为急性发病、肺部感染和高渗出性病变时,要考虑作为ARDS慎重处理。
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【流行病学】有关ARDS在我国儿童人群中的发病率尚不清,分析ARDS在儿科ICU病房年收治病例的比例,发达国家20世纪80~90年代小儿ARDS发病率约占ICU收治数的1%;国内孙波教授主持的中国儿童呼吸衰竭协作组2009年12月结束的26个PICU急性低氧性呼吸衰竭和ARDS的研究表明,PICU中2.7%的危重病例发生了ARDS,肺炎和脓毒症为其主要原因。
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