13卷1期疑难病案讨论选登

１３卷１期疑难病案讨论选登（刘桂芬０５４２００河北省内邱县人民医院）诊断：产后溶血性尿毒症综合征分析：产后溶血性尿毒症综合征，是一种起病急，病情严重，病死率高的产后罕见并发症。临床表现为急性肾功能衰竭及微血管病溶血性贫血两大特征。此患者为过期妊娠可有...     

产后溶血性尿毒症性综合征21例分析

产后溶血性尿毒症性综合征２１例分析曹书华，李瑾产后溶血性尿毒症性综合征（ｐｏｓｔｐａｒｔｕｍｈｅｍｏｌｙｔｉｃｕｒｅｍｉｕｓｙｎｄｒｏｍｅ，简称产后ＨＵＳ）是一组妊娠合并急性肾功能衰竭、微血管病性溶血性贫血、血小板减少的综合征，发病率低。至今对其发病...     

产后溶血性尿毒症性综合征的病因临床表现及诊治

产后溶血性尿毒症性综合征的病因临床表现及诊治浙江医科大学附属妇产科医院（３１０００６）郑宝琴产后溶血性尿毒症性综合征（ｐｏｓｔｐａｒｔｕｍｈｅｍｏｌｙｔｉｃｕｒｅｍｉｃｓｙｎｄｒｏｍ，ＰＨＵＳ）是一种由溶血引起肾损害导致尿毒症性综合征的一组疾病，由...     

产后溶血性尿毒症综合征诊治进展

产后溶血性尿毒症综合征(PHUS)属于血栓性微血管疾病,是非典型溶血性尿毒症综合征中的一种,主要临床表现为产后10周内发生不可逆急性肾功能衰竭伴血小板减少、微血管病性贫血,临床罕见,预后较差,少数病例发生于妊娠晚期。目前研究认为其发病的病理生理机制为原发性或继发性补体系统调节异常。早期诊断和及时治疗可以明显改善患者预后,但其诊断与鉴别诊断较为困难,目前仍以临床诊断为主,并应尽快启动诊断性治疗,特异性基因和分子检测有助于明确诊断。血液透析、血浆置换仍然是PHUS首选的治疗方法,而针对病因的特异性补体抑制剂可用于该病的长期治疗。     

产后溶血性尿毒症综合征九例临床分析

产后溶血性尿毒症综合征(postpartum hemolytic uremic syndrome,PHUS)是一种少见的综合征,是指主要发生在产后,以微血管性病理性溶血性贫血、急性肾功能衰竭和血小板减少为特征的一组综合征,约25 000例分娩中发生1例~([1]).     

产后溶血性尿毒症综合征研究进展

产后溶血性尿毒症综合征是指在产后以急性微血管病性溶血性贫血、血小板减少及急性肾功能衰竭三大特征为主的危重性综合征。病因与妊娠期凝血功能异常、机械性因素、遗传、免疫及感染等因素有关。其发病机制是肾脏局部的微血管病性溶血及血管内凝血。该综合征虽然发生率极低,但由于发病急,进展快,病死率高,应引起临床高度重视,不断提高对本病的认识,力求做到早诊断、早治疗、争取改善预后。     

产后溶血性尿毒症综合征的发病机制及其诊治

产后溶血性尿毒症综合征(postpartum hemolytic uremic syndrome，PHUS)是指在产后(个别可发生在孕末期临产前)以急性微血管病性溶血性贫血、血小板减少及急性肾功能衰竭三大特征为主的综合征。本病特点是妊娠过程顺利，但产后发生肾功能衰竭，临床少见，据报道约25000次分娩中发生1例，其中23．0％在妊娠中期即有严重的肾功     

妊高征与产后溶血性尿毒症性综合征

近年来由于对高危孕产妇加强管理,孕产妇的病死率已显著下降。唯有产后溶血性尿毒症性综合征(postpartum hemolytic uremic syndrome)因病因不明,死亡率仍很高。据国外不全统计1966年为83％,1976年为64％,1979年为58%,近年来可能又稍有下降。国内仅1980年报道3例死亡病例,可能对此综合征还未引起足够的认识和重视,死亡率还很高。作者自1976年至今共会诊院内、外产后溶血性尿毒症性综合征16例,其中院内10例(活4人),院外6例(活2人),合计死亡率为62.5％。     

产后出血性尿毒症的血浆置换治疗

产后出血性尿毒症也称为溶血性尿毒综合征（ｈｅｍｏｌｙｔｉｃｕｒｅｍｉｃｓｙｎｄｒｏｍｅ，ＨＵＳ），其临床特点是微血管性溶血性贫血、急性肾功能衰竭和血小板减少。如果伴有神经系统症状和发热时，则称为血栓性血小板减少性紫癜（ｔｈｒｏｍｂｏｃｙｔｉｃｔｈｒｏ...     

HELLP综合征和HUS-TTP的诊断和鉴别诊断

<正>HELLP综合征与溶血性尿毒症综合征(hemolytic uremia syndrome,HUS)-血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)均是妊娠严重并发症,其发病急骤,严重威胁孕产妇及胎儿生命。临床上二者因其相似的发病机制及临床症状,易引起临床医师混淆,给临床诊断、治疗带来困难。因此,充分认识HELLP综合征和HUS-TTP的诊断和鉴别诊断,对疾病的临床处理,降低患者死亡率和改善母儿预后具有重大意义。     

产后溶血性尿毒症性综合征的临床表现及处理——3例病例分析及文献复习

目的 探讨产后溶血尿毒症性综合征(postpartum hemolytic uremic syndrome,PHUS)的临床特征、诊断、治疗及预后. 方法 结合3例PHUS病例,复习有关文献,讨论PHUS的临床特征、诊断治疗及预后. 结果 3例患者均于剖宫产分娩后3 d内出现微血管病性溶血性贫血、血小板减少、肾功能急剧恶化.经血浆置换及血液透析等治疗病情稳定.发病后随访至今,1例患者肾功能恢复正常,2例仍需血液透析维持. 结论 早期诊断和及时正确治疗对于降低PHUS患者的病死率至关重要.     

血浆置换联合血液透析滤过治疗产后溶血尿毒综合征三例疗效分析

目的:探讨血浆置换(PE)联合血液透析滤过(HDF)对产后溶血尿毒综合征(PHUS)的治疗效果.方法:回顾性分析2017年9月—2019年9月天津市第一中心医院收治的3例采用PE联合HDF治疗的PHUS患者的临床资料,分析患者治疗前后临床症状及实验室检查结果的变化情况,并进行随访.结果:经过PE联合HDF治疗,患者临床...     

Hemolytic uremic syndrome in twin pregnancy at 32 weeks gestation with HELLP syndrome. Case report]

Renal failure occurring in pregnancy or post partum is an unusual but well-described complication. Acute renal failure seems to be associated more often with HELLP syndrome rather than with pre-eclampsia or chronic hypertension. Probable overlapping of HELLP and hemolytic uremic syndrome in pregnancy or postpartum should be taken into consideration when treating pregnant women who show signs of proteinuria, hypertension, hematuria, increase of reticulocytes, decrease of haptoglobin with thrombocytopenia and microangiopathic hemolytic anemia. Our case refers to a 32 year old woman at 32 weeks gestation in twin pregnancy who presented with HELLP syndrome and renal failure. Immediately postpartum oliguria was noted and the laboratory analyses suggested the coexistence of HELLP and hemolytic uremic syndrome. In patients with gestosis and/or HELLP syndrome presenting oliguria combined with a decrease of hemoglobin level not due to intraoperative hematic leaks it is always necessary to ask for haptoglobin dosage. In treating hemolytic uremic syndrome it is very important to use a high dosage of plasma and sometimes plasmapheresis. HELLP syndrome contributes to various complications which are sometimes responsible for kidney or maternal mortality. In treating these patients early diagnosis combined with a specific treatment can considerably reduce kidney and maternal mortality.     

Postpartum hemorrhagic shock resulting in thrombotic thrombocytopenic purpura–hemolytic uremic syndrome

Thrombotic microangiopathies may be initiated by a number of antecedent events. When presented with postpartum hemorrhage and unexplained thrombocytopenia, it is prudent to consider microangiopathic hemolytic anemia in the differential diagnosis. A 25-year-old woman, gravida 2, para 1, had an uncomplicated repeat Cesarean delivery at 38 weeks' gestation. She subsequently had an exploratory laparotomy for hemoperitoneum resulting from a left uterine artery laceration. On postoperative day 3, she developed thrombotic thrombocytopenic purpura–hemolytic uremic syndrome and was treated with plasma exchange therapy and dialysis. It is critical that clinicians consider this potentially fatal disease in the differential diagnosis when hemorrhagic shock is associated with unexplained thrombocytopenia, so that appropriate and early treatment may lead to a favorable outcome.     

Postpartum hemolytic-uremic syndrome associated with lupus anticoagulant. A case report.

BACKGROUND: Hemolytic uremic syndrome is a rare thrombotic microangiopathy characterized by acute renal failure, thrombocytopenia and hemolysis. The underlying abnormality is currently thought to involve enothelial injury within the microcirculation. CASE: A 30-year-old woman, gravida 2, para 1, underwent emergency cesarean delivery at 36 +/- 2 weeks' estimated gestational age for repetitive late decelerations and presumed severe preeclampsia. Postoperatively, the blood pressure remained persistently elevated despite multigent hypertensive therapy. By postpartum day 4 the patient continued to display acute oliguric renal failure, persistent severe thrombocytopenia and worsening hemolysis. Percutaneous renal biopsy was consistent with the clinical diagnosis of hemolytic uremic syndrome. Lupus anticoagulant was present, corroborated by markedly abnormal tissue thromboplastin inhibition and platelet neutralization procedures. With supportive therapy and daily plasmapheresis, the patient was discharged 22 days after delivery, with full recovery of renal function and resolution of the hemolytic process. CONCLUSION: Hemolytic uremic syndrome can be associated with lupus anticoagulant. This autoantibody may promote localized platelet aggregation, causing endothelial damage.     

Possible role for exchange plasmapheresis with fresh frozen plasma for maternal indications in selected cases of preeclampsia and eclampsia

A new case of a preeclamptic who developed the diagnostic criteria for the hemolytic uremic syndrome and who was treated with plasmapheresis is presented. Fourteen case reports of gravidas with preeclampsia treated by exchange plasmapheresis with fresh frozen plasma for maternal indications are reviewed. Only four cases had been reported as eclampsia or preeclampsia. The other cases had been reported as thrombotic thrombocytopenic purpura, the postpartum hemolytic uremic syndrome, or fatty liver of pregnancy. The possible role of plasmapheresis in treating very carefully selected cases of preeclampsia is discussed.     

产后溶血性尿毒症综合征六例临床分析

目的探讨产后溶血性尿毒症综合征(postpartum hemolytic uremic syndrome, PPHUS)的临床表现、实验室检查、诊断、治疗及预后。 方法采用回顾性研究方法对南京医科大学附属苏州医院2008年1月至2011年12月期间收治的6例PPHUS患者的临床资料进行分析。 结果6例PPHUS患者均于剖宫产术后1 d左右出现无尿和急性肾功能衰竭,实验室检查呈微血管病性溶血性贫血、进行性血小板减少改变,经连续性静脉-静脉血液滤过、血浆置换、血液透析、输注冰冻血浆、肾上腺皮质激素、抗感染以及降压等综合治疗,1例治愈出院;5例好转出院,其中4例出院后随访3～6个月,肾功能均恢复正常,另一例治疗中自动出院,自行转院治疗,随访6个月,留有肾功能损害。 结论PPHUS较罕见,属临床危急重症,早期诊断,及时正确治疗,有助于改善PPHUS患者预后。     

Imitators of severe preeclampsia

There are several obstetric, medical, and surgical disorders that share many of the clinical and laboratory findings of patients with severe preeclampsia-hemolysis, elevated liver enzymes, and low platelets syndrome. Imitators of severe preeclampsia-hemolysis, elevated liver enzymes, and low platelets syndrome are life-threatening emergencies that can develop during pregnancy or in the postpartum period. These conditions are associated with high maternal mortality, and survivors may face long-term sequelae. Perinatal mortality and morbidity also remain high in many of these conditions. The pathophysiologic abnormalities in many of these disorders include thrombotic microangiopathy, thrombocytopenia, and hemolytic anemia. Some of these disorders include acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and acute exacerbation of systemic lupus erythematosus. Because of the rarity of these conditions during pregnancy and postpartum, the available literature includes only case reports and case series describing these syndromes. Consequently, there are no systematic reviews or randomized trials on these subjects. Differential diagnosis may be difficult due to the overlap of several clinical and laboratory findings of these syndromes. It is important that the clinician make the accurate diagnosis when possible because the management and complications from these syndromes may be different. For example, severe preeclampsia and acute fatty liver of pregnancy are treated by delivery, whereas it is possible to continue pregnancy in those with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and exacerbation of systemic lupus erythematosus. This review focuses on diagnosis, management, and counseling of women who develop these syndromes based on results of recent studies.     

A case of postpartum hemolytic uremic syndrome with severe elevations of liver enzymes

An unusual case of multifocused postpartum thrombotic microangiopathy was encountered in a woman with pregnancy-induced hypertension, which presented clinical features different from those generally seen in postpartum hemolytic uremic syndrome or thrombotic thrombocytopenic purpura. These changes included acute disseminated intravascular coagulation, severe elevations of liver enzymes, severe renal dysfunction, and hemolytic anemia. The patient was treated successfully with heparin, gabexate mesilate, furosemide, fresh whole blood, and large doses of corticosteroid with antibiotics, and she recovered completely without sequelae. The hypothetical pathogenesis of the complex symptoms of this catastrophic clinical condition is proposed.     

Postpartum hemolytic uremic syndrome following abruptio placenta: report of a case.

Postpartum hemolytic uremic syndrome (HUS) is an unusual complication that presents with microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure after delivery. In this report, we describe a 32-year-old patient (gravida 3, para 1, artificial abortion 1) who developed postpartum HUS following abruptio placenta. After cesarean delivery due to abruptio placenta, the patient developed acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia followed by hypertension. Plasma exchange led to recovery from thrombocytopenia and improvement in renal function. This case highlights the importance of observation of peripheral blood smears in patients with abruptio placenta who develop thrombocytopenia after delivery.