溶血性尿毒症综合症HUS-武汉大学人民医院肾内科 内容预览： 

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《溶血性尿毒症综合症HUS-武汉大学人民医院肾内科》内容简介：

溶血性尿毒症综合症
（Hemolytic Uremic Syndrome，HUS)
武汉大学人民医院肾内科
刘红燕

概   念
微血管病 性溶血性贫血（microangiopathic haemolytic anaemia,MAHA)
血小板减少(thrombocytopeania)

急性肾衰竭(acute renal failure)
HUS和TTP
1925年Moschcowitz首先提出TTP
1955年Gasser首先提出HUS，1970年用于成人
两者同属血栓性微血管病（TMA），具相似的临床症状和相同的病理表现

HUS分类的进展
腹泻型（D＋）与非腹泻型（D－）
志贺样毒素（Shiga-like toxin,Stx）HUS与非志贺样毒素HUS

HUS的病因
Part 1 etiology advanced

1  Infection induced
(a)Shiga and verocytotoxin(shiga-like toxin)-producing    bacteria;
enterohemorrhagic Escherichia coli, Shigella dysenteriaen type    1,Citrobater
(b)Streptococcus pneumoniae,neuraminidase,and T-antigen exposure

2  Disorders of complement regulation
(a)Genetic disorders of complement regulation
(b)Acquired disorders of complement regulation, for example anti-FH antibody
N Besbas, D Karpman et al.Kidney Int 2006vol70:423-431

3  Von Willebrand proteinase, ADAMTS13 deficiency
(a) Genetic disorders of ADAMTS13
(b)Acquired von Willebrand proteinase deficiency;autoimmune, drug induced

4   Defective cobalamine metabolism

5   Quinine induced

Part 2 etiology unknown
1 HIV
2 Malignancy,cancer chemotheray and ionizing radiation
3 Calcineurin inhibitors and transplantation
4 Pregnancy, HELLP syndrome and oral contraceptive pill
5 Systemic lupus erythematosis and antiphospholipid antibody syndrome
6 Glomerulopathy
7 Familial, not included in part 1
8 Unclassified

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