Journal of Clinical Pediatrics ›› 2022, Vol. 40 ›› Issue (2): 155-159.doi: 10.12372/jcp.2022.21e1093
• Continuing Medical Education • Previous Articles
ZHANG Hongwen
Received:
2021-07-26
Published:
2022-02-15
Online:
2022-02-11
ZHANG Hongwen. Diagnostic approach on childhood renal dysfunction[J].Journal of Clinical Pediatrics, 2022, 40(2): 155-159.
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肾小球疾病 |
---|
原发性:IgA肾病、链球菌感染后肾小球肾炎、非链球菌感染后肾小球肾炎、肾病综合征等 |
继发性:狼疮性肾炎、紫癜性肾炎、ANCA相关性血管炎、溶血尿毒综合征、遗传代谢性疾病继发肾小球损害等 |
遗传性:Alport综合征、各种遗传性肾病综合征、遗传代谢性疾病累及肾小球等 |
肾小管疾病 |
原发性:特发性间质性肾炎等 |
继发性:感染、药物、毒物、糖尿病等 |
遗传性:Dent病、Lowe综合征、Gitelman综合征、Bartter综合征、Fanconi综合征等 |
肾血管疾病 |
原发性:大动脉炎、肾动脉狭窄等 |
继发性:血栓、血管瘘、血管瘤等 |
遗传性:纤维肌性发育不良等 |
囊性肾脏病 |
原发性:多囊性肾发育不良等 |
继发性:长期低钾血症、酸中毒等 |
遗传性:常染色体显性遗传性多囊肾、常染色体隐性遗传性多囊肾、肾单位肾痨等 |
肾脏和泌尿道先天畸形(CAKUT) |
环境因素:主要包括母亲饮食、疾病和药物/毒品/酒精应用等 |
遗传因素:单基因遗传约占10%,拷贝数变异约占16% |
其他 |
反流性肾病、肾结核、肾脏肿瘤等 |
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畸形类型 | 病 因 | 定 义 |
---|---|---|
肾脏数目 | 肾缺如(renal agenesis) | 单侧或双侧,肾脏和流出道未形成 |
肾脏大小和形态 | 肾发育不全(renal hypoplasia) | 单侧或双侧,肾脏外形正常,但体积小、肾单位数目减少 |
肾发育不良(renal dysplasia) | 单侧或双侧,肾脏外形和组织分化异常,肾单位数目减少 | |
多囊性肾发育不良(MCDK) | 肾发育不良伴多囊性病变,肾脏外形异常 | |
肾脏位置 | 马蹄肾(horseshoe kidney) | 双侧肾脏后部融合形成马蹄样外形 |
异位/盆腔肾(ectopic/pelvic kidney) | 肾脏位置异常,通常位于盆腔 | |
流出道畸形 | 肾盂输尿管连接部梗阻(UPJO) | 单侧或双侧,肾脏和输尿管连接部梗阻,阻碍了肾盂的尿液引流入输尿管 |
膀胱输尿管连接部梗阻(UVJO) | 单侧或双侧,输尿管和膀胱连接部梗阻,阻碍了输尿管的尿液引流入膀胱 | |
膀胱输尿管反流(VUR) | 单侧或双侧,膀胱输尿管连接部缺陷,导致尿液从膀胱反流进入输尿管 | |
双集合系统(DCS) | 单侧或双侧,输尿管和肾盂重复,可伴随重复肾;流出道系统反流或梗阻 | |
巨输尿管(megaureter) | 单侧或双侧,输尿管扩张导致尿液流出障碍 | |
后尿道瓣膜(PUV) | 后尿道瓣膜形成,阻碍了膀胱尿液排出,仅限于男性 |
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