溶血性尿毒癥綜合癥HUS-武漢大學(xué)人民醫院腎內科 內容預覽： 

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《溶血性尿毒癥綜合癥HUS-武漢大學(xué)人民醫院腎內科》內容簡(jiǎn)介：

溶血性尿毒癥綜合癥
（Hemolytic Uremic Syndrome，HUS)
武漢大學(xué)人民醫院腎內科
劉紅燕

概   念
微血管病 性溶血性貧血（microangiopathic haemolytic anaemia,MAHA)
血小板減少(thrombocytopeania)

急性腎衰竭(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
兩者同屬血栓性微血管病（TMA），具相似的臨床癥狀和相同的病理表現

HUS分類(lèi)的進(jìn)展
腹瀉型（D＋）與非腹瀉型（D－）
志賀樣毒素（Shiga-like toxin,Stx）HUS與非志賀樣毒素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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