HELLP综合征九例临床分析

目的探讨溶血、肝酶升高、血小板减少（HELLP）综合征的临床特点及发病率、诊断、治疗和预后。方法对我院5年来HELLP综合征9例患者的临床资料进行回顾性分析。结果HELLP综合征占同期妊娠期高血压疾病的6.61%,主要并发症为肝被膜下出血、DIC、胎盘早剥,急性肾衰、肺水肿、胎儿生长受限、胎儿宫内窘迫等。常规应用镇静、降压、解痉、输注血液制品外,早期应用肾上腺皮质激素治疗,及时终止妊娠是治疗HELLP综合征的有效措施。结论HELLP综合征是一种严重威胁母婴生命安全的并发症,积极治疗妊娠期高血压疾病同时早期应用肾上腺皮质激素,尽快终止妊娠,加强围生期的处理,多学科合作,警惕发生多器官系统衰竭综合征。     

探讨血浆置换在产后合并血栓性血小板减少性紫癜中的应用

目的探讨血浆置换(PE)在产后合并血栓性血小板减少性紫癜(TTP)中的应用时机及方法。方法回顾分析我院利用血浆置换成功救治1例产后合并TTP患者的临床资料,查阅相关文献,总结分析血浆置换在产后合并TTP中的有效治疗时机及方法。结果患者剖宫产术后经早期确诊产后合并TTP,立即给予血浆置换并辅助其他抢救治疗,于术后第7天复查血小板(PLT)升至110×109/L,痊愈出院,随访无复发。结论产后合并TTP,病情危重,死亡率高,易误诊或漏诊,应早期诊断并行血浆置换,降低死亡率,提高生存质量。     

婴儿急性肠扭转并脓毒性休克一例报告暨文献复习

目的了解婴儿急性肠扭转并脓毒性休克的临床表现、实验室检查、诊断及治疗方法;方法回顾性分析宁德市闽东医院1例婴儿急性肠扭转并脓毒性休克的临床资料;结果男性患儿,因突发面色苍白、精神差2h入院,入院后给予抗休克治疗的同时行床边腹部平片、床边B超及腹腔积液穿刺检查,及时行剖腹探查术,术中发现急性小肠扭转,并予手法复位成功,术后恢复好,痊愈出院;结论婴儿急性肠扭转起病急、进展快,易并发脓毒性休克,病死率高,须及早手术治疗。     

重度子痫前期合并HELLP综合征临床分析

目的探讨重度子痫前期并发HELLP综合征的诊断与治疗方法，降低孕产妇及围生儿死亡率。方法对11例HELLP的临床资料进行回顾性分析。结果HELLP综合征占重度子痫前期的10．38％。11例患者，平均孕龄31“周，1例于产前囟动出院，8例痊愈出院，1例转入肾内科，确诊为慢性肾小球肾炎，1例疑骨髓增生异常综合征（MDS），拒行骨穿于术后10天自动出院。孕产妇死亡数为0，围生儿死亡率为20％。结论HELLP综合征病情凶险，进展迅速，是重度子痫前期严重威胁母婴安全的并发症。早期诊断，早期正确治疗，及时终止妊娠，是降低母婴死亡率的关键。     

妊娠高血压疾病并发脑水肿3例报告

1病例报告 例1、患者28岁，因妊娠4l周，发现血压偏高1天，于2004年4月26日入院。血压166／120mmHg，水肿（＋＋＋＋），尿蛋白（＋＋＋＋）。24小时尿蛋白定量5．08g／1690ml尿量。诊断为妊娠4l周孕2产1妊娠高血压疾病，重度子痫前期。予硫酸镁解痉，心痛定降压，安定镇静等治疗，血压136-144／86-96mmHg，入院第2天因超声提示羊水少，行剖宫产术，娩出1活婴，阿氏评分10分，取胎儿时患者诉恶心，血压由166／120mmHg下降至124／78mmHg，随后神志不清，无应答反应，5分钟后血压回升至154／90mmHg。术毕患者仍昏迷不醒，颜面浮肿。眼底检查：双眼底视网膜小动脉痉挛，视网膜水肿伴絮状渗出。予甘露醇及速尿脱水、利尿，硝酸甘油降压，半小时后患者可唤醒，自述有明显头痛，1小时后才能应答、睁眼，视物清。此后一般情况稳定继续降压利尿对症治疗。术后第7天出院。     

血栓性血小板减少性紫癜治疗体会

目的探究血栓性血小板减少性紫癜（TTP）诊断、临床特征及治疗。方法通过观察1例病例诊治过程、随访情况,进行分析并复习相关文献。结果患者行血浆输注（PI）联合皮质激素和长春新碱治疗,病情不能控制,行血浆置换（PE）治疗很快达到缓解,但停止PE则出现病情反复。在缓解期内,患者外周血象表现为单一持久性的血小板减少,经皮质激素联合长春新碱治疗达到痊愈。随访18个月疾病无复发。结论 PE为TTP首选治疗措施,联合皮质激素和长春新碱治疗,有助于减少和防止复发,提高缓解率和治愈率。     

血栓性血小板减少性紫癜治疗体会

目的 探究血栓性血小板减少性紫癜(TTP)诊断、临床特征及治疗.方法 通过观察1例病例诊治过程、随访情况,进行分析并复习相关文献.结果 患者行血浆输注(PI)联合皮质激素和长春新碱治疗,病情不能控制,行血浆置换(PE)治疗很快达到缓解,但停止PE则出现病情反复.在缓解期内,患者外周血象表现为单一持久性的血小板减少,经皮质激素联合长春新碱治疗达到痊愈.随访18个月疾病无复发.结论 PE为TTP首选治疗措施,联合皮质激素和长春新碱治疗,有助于减少和防止复发,提高缓解率和治愈率.     

妊娠期高血压并发HELLP综合症6例分析

目的：探究孕妇妊娠期高血压严重并发HELLP综合症终止妊娠的时机以及对胎儿和孕妇的影响,它是以溶血(hemolysis,H)、肝酶升高(elevated liver enzymes,EL)和血小板减少(low platelets,LP)为特点。方法通过对6例HELLP综合征患者进行跟踪调查研究,进行临床数据记录和比对。结果在抽取的6例妊娠期高血压并发HELLP综合症患者中均未出现严重的并发症,其中有4例是采取剖宫产术终止了妊娠,有1例是进行了阴道分娩,其中子痫1例,死胎1例。结论妊娠期高血压并发HELLP综合症对产妇和围生有着极大的影响,严重者可能会导致婴儿死亡,在治疗方法上最为有效的治疗手段就是在技术条件允许的情况下适时终止妊娠。     

HELLP综合征56例临床分析

HELLP综合征是妊娠期高血压疾病的严重并发症之一，除有妊高征症侯群外，尚有溶血、肝酶升高、血小板减少等综合征，并有不同程度的消化道症状。HELLP综合征病情凶险，诊断处理不及时，对母儿的危害极大属于产科危重症之一。我院于2000年1月至2006年1月间共收治HELLP综合征患者56例，现对其临床资料分析报道如下。     

探讨临床综合治疗方法对费县老年性肺炎的治疗效果

目的：探讨临床综合治疗方法对费县老年性肺炎的治疗效果。方法随机选择山东省费县新庄中心卫生院收治的老年肺炎患者作为研究对象，入院后积极明确诊断并予以抗感染、解痉、平喘、预防并发症等措施综合治疗，并观察该疗法的治疗效果。结果老年性肺炎患者中有62例患者临床症状完全消失(有效率62%)，彻底治愈康复出院，38例患者临床症状明显改善出院。结论临床上采用综合疗法对老年肺炎有明确的治疗效果，为今后治疗与预防老年肺炎的发生和发展提供有力的临床依据。     

Plasmapheresis. A therapeutic option in the management of heparin-associated thrombocytopenia with thrombosis

Heparin-associated thrombocytopenia with thrombosis (HATT) is an uncommon syndrome that is estimated to occur in 1-5% of patients with heparin-induced thrombocytopenia. Early diagnosis requires careful clinical surveillance, and the management of these patients can be complex. Cessation of heparin therapy and substitution or addition of oral anticoagulants, antiplatelet agents, dextrans, and prostacyclin analogues have been advocated. The authors are aware of only two case reports in the literature that examine the use of plasmapheresis as a therapeutic alternative. The authors report a case of a 53-year-old white man who developed HATT after a single protamine-reversed exposure to heparin. Controlled platelet aggregation studies performed before and after apheresis sessions documented a dramatic response and rapid normalization of platelet number and function in the patient. The authors conclude that plasmapheresis could be a valuable adjunct in the successful management of patients with HATT. When done in conjunction with platelet aggregation studies, an objective measurement of therapeutic efficacy can be achieved.     

COVID-19 and hemolysis,elevated liver enzymes and thrombocytopenia syndrome in pregnant women - association or causation?

Pregnant women are among the high-risk population for severe coronavirus disease 2019 (COVID-19) with unfavorable peripartum outcomes and increased incidence of preterm births. Hemolysis, the elevation of liver enzymes, and low platelet count (HELLP) syndrome and severe preeclampsia are among the leading causes of maternal mortality. Evidence supports a higher odd of pre-eclampsia in women with COVID-19, given overlapping pathophysiology. Involvement of angiotensin-converting enzyme 2 receptors by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) for the entry to the host cells and its downregulation cause dysregulation of the renin-angiotensin-aldosterone system. The overexpression of Angiotensin II mediated via p38 Mitogen-Activated Protein Kinase pathways can cause vasoconstriction and uninhibited platelet aggregation, which may be another common link between COVID-19 and HELLP syndrome. On PubMed search from January 1, 2020, to July 30, 2022, we found 18 studies on of SARS-COV-2 infection with HELLP Syndrome. Most of these studies are case reports or series, did not perform histopathology analysis of the placenta, or measured biomarkers linked to pre-eclampsia/HELLP syndrome. Hence, the relationship between SARS-CoV-2 infection and HELLP syndrome is inconclusive in these studies. We intend to perform a mini-review of the published literature on HELLP syndrome and COVID-19 to test the hypothesis on association vs causation, and gaps in the current evidence and propose an area of future research.     

HELLP综合征的母胎结局

 目的探讨HELLP综合征的母胎并发症及相关因素。方法回顾性分析2005～2009年在本院分娩的32例HELLP综合征的临床资料,用χ2检验进行统计学分析。结果;该综合征病人占同期重度子痫前期的2.16% (32/1484),诊断时的平均孕周为32周4天（24+～40周）,多发于27～37周。终止妊娠前发生的27例,发生在产后的均在48 h之内发病。该综合征病人中完全HELLP18例,部分HELLP即ELLP14例,母体的并发症如子痫、胎盘早剥、产后出血、急性肾功不全等HELLP明显多于ELLP,当血小板≤50x109/L时HELLP明显多于ELLP。孕周≤30周,死胎或死产,新生儿重度窒息明显增多,而且均表现为生长受限。初诊或急诊的病人HELLP多于定期产检的。结论;HELLP较ELLP母体的并发症多,其严重程度与血小板计数有关,胎儿的病死率与发病时的孕周有关,定期产检可预防HELLP综合征的发病。     

儿童Andersen-Tawil综合征1例并文献复习

目的分析1例Andersen-Tawil综合征（ATS）患儿的诊断和治疗经过,提高对ATS的认识。方法报道1例基因确诊ATS患儿的临床特征及诊疗过程,并进行文献复习。结果患儿,女,12岁10个月,因＂意识丧失3 h伴抽搐＂入院。患儿既往有发作性双下肢无力及不明原因胸闷史。入院前3 h患儿在参加智力竞赛时突然晕倒、意识丧失、口唇发绀伴肢体抽动。查体神志不清,眼窝凹陷、眼距略宽,心律不齐,四肢呈迟缓性麻痹,脊柱、四肢无畸形。实验室检查示血清钾一过性降低。ECG检查示室性颤动、室性心动过速和频发多形性室性期前收缩。入院后予机械通气,胺碘酮和美托洛尔抗心律失常治疗,疗效欠佳,心律失常反复发作。基因检测示KCNJ2杂合错义突变：c.899 G〉T hetero,GGC〉GTC,p.G300V,确诊为ATS。出院后1个月余在外院植入心律转复除颤器（ICD）,并加用氟卡尼100 mg.d-1口服治疗。治疗后患儿心律失常减少,迄今随访8个月余,未见抽搐及晕厥发作,目前继续随访中。结论 ATS以室性心律失常,周期性麻痹,轻度面部和（或）骨骼发育异常为特征。心源性晕厥及心跳骤停不常见,但可危及生命,需植入ICD治疗。KCNJ2基因突变有助确诊。     

Kartagener综合征一例并文献复习

目的探讨Kartagener综合征的临床特点、诊断标准,以提高对Kartagener综合征认识。方法 Kartagener综合征1例。患儿,女,10岁余。因反复咳嗽1年,咯血4次入院。回顾性分析该患儿临床资料,检索国内外文献,进行总结分析。结果患儿隐匿起病,主要表现为反复咳嗽,咳脓痰,伴咯血。CT示双肺支扩伴感染,全内脏反位,鼻窦炎。经抗感染对症治疗1周后,临床症状消失出院。检索国内外文献发现,Kartagener综合征以全内脏反位,支气管扩张,副鼻窦炎三联征为诊断标准。治疗以对症治疗为主。结论 Kartagener综合征是一种罕见的常染色体隐性遗传病,加强对该病的认识,有利于提高对该病的诊断率,减少误诊率。对该病的致病基因、机制的进一步研究,可能提供更好的诊断和治疗方法。     

S-nitrosoglutathione preserves platelet function during in vitro ventricular assist device circulation

Complications (severe bleeding/thromboembolism) may occur during ventricular assist device (VAD) circulation, caused mainly by platelet dysfunction from platelet activation. We hypothesized that S-nitrosoglutathione (GSNO), having platelet activity preservation properties like nitric oxide (NO), may be a titratable agent to diminish platelet activation and thus preserve platelet function. Dose-response measurement of platelet aggregation by GSNO was performed using an aggregometer. GSNO (1,000 microM) caused inhibition of collagen and ristocetin induced aggregation by approximately 50%. Next, in vitro ventricular assist device (VAD) circulation was performed (over 48 hours using human whole blood), both without (control) and with GSNO (1,000 microM), and the aggregability of perfusate was measured at 0, 0.5, 1, 3, 6, 12, 24, and 48 hours. In control VAD circuits, collagen induced platelet aggregability gradually decreased and became significantly lower after 3 hours of circulation. With GSNO, platelet function did not significantly decrease until after 12 hours. Similar results were seen for ristocetin induced aggregation; control aggregation dropped significantly after 6 hours, but not until after 24 hours with GSNO. Liquid phase measurement of total nitrogen oxides (NO(T)) confirmed added GSNO maintained high perfusate NO(T) compared with control. GSNO is effective in preserving platelet aggregation during the first 12 to 24 hours in vitro and may be effective in preserving platelet function by inhibiting platelet activation during in vivo VAD circulation.     

Ventricular Tachycardia in Acute Myocardial Infarction: Clinical Features of 113 Patients

This study was undertaken to evaluate the clinical significance of ventricular tachycardia as a complication of acute myocardial infarction in a large public hospital. An analysis of the clinical, electrocardiographic, therapeutic, and prognostic implications of this large patient group is presented and several unique features emphasized.     

Pregnancy in Bloom's Syndrome

This report describes the clinical and cytogenetic findings in a young woman with Bloom's Syndrome, who was successfully delivered of a healthy child 6 years after the diagnosis of the condition. This is the first reported case of pregnancy in a patient with Bloom's Syndrome.     

Platelet function in hairy-cell leukaemia

A quantitative study of various aspects of platelet function was carried out in eight patients with typical hairy-cell leukaemia (HCL). In at least two patients platelet aggregation was convincingly reduced to more than one aggregating agent (ADP, adrenaline, collagen, thrombin, and ristocetin). Granular storage capacity for {(14)C} 5-HT was reduced in five of the six patients tested. The two patients with definitely abnormal aggregation had the greatest reduction in granular storage pool and the longest bleeding times of those tested but, like the other patients, they did not have a clinical haemostatic defect. It was concluded that a granular storage pool defect (SPD) was at least partly responsible for aggregation abnormalities in HCL since the platelet release reaction in response to thrombin appeared to be normal. All our patients ran a chronic course uncomplicated by any of the factors known to predispose to a platelet SPD acquired in the circulation. Although in the one patient tested before and after splenectomy there was some improvement in platelet aggregation after operation, there was no clear general relationship between defective platelet function and either previous splenectomy or platelet count. Since a direct involvement of the megakaryocytic series in the underlying cell proliferation of HCL seems unlikely, it is concluded that the platelet defect can most reasonably be attributed to the production of abnormal platelets as a result of marrow fibrosis and/or infiltration by hairy cells.     

Cerebral infarction in a patient with macrothrombocytopenia with leukocyte inclusions (MTCP, May-Hegglin anomaly/Sebastian syndrome)

We report on a 78-year-old woman patient with macrothrombocytopenia with leukocyte inclusions (MTCP, May-Hegglin anomaly/Sebastian syndrome), who had no history of hemorrhagic symptoms and had a platelet count of 10,000 or less, but had a cerebral infarction. The patient was found to have idiopathic thrombocytopenic purpura, hypertension, and atrial fibrillation 16 years ago, yet received no medication. She was found to have had a cerebral infarction with aphasia as the chief complaint and was admitted to our hospital. Thrombocytopenia was found in three family members. Blood examinations revealed normal bleeding time and platelet aggregation ability. The patient was found to have the triad of giant platelets, thrombocytopenia, and inclusion bodies in leukocytes. Genetic analysis showed a mutation of the MYH-9 gene in the patients second daughter. Consequently, this patient received a diagnosis of MTCP. There have only been a few reports of the onset of thrombosis in patients with MTCP and no reports of the onset of cerebral infarction. Our report is the first case of MTCP in a patient with cerebral infarction.