横纹肌溶解症合并急性肾衰竭供者的维护及其供肾移植1例并文献复习

目的 探讨横纹肌溶解症(RM)合并急性肾衰竭(ARF)的供者维护及其供肾移植的临床效果.方法 回顾性分析2019年4月本院收治的1例RM伴ARF供者的维护及其供肾移植的临床资料,并复习相关文献.结果 供者因RM导致ARF,经积极维护治疗,获取供肾前血肌酐为52 μmol/L,血浆肌酸激酶(CK)256 U/L.移植前快...     

成人免疫性血小板减少性紫癜合并肝血管瘤1例报告并文献复习

背景与目的 成人免疫性血小板减少性紫癜（ITP）合并肝血管瘤临床罕见,容易误诊误治。笔者报告的1例成人ITP合并肝血管瘤患者的诊治经过,以期为临床提供参考和借鉴。方法 回顾性分析桂林医学院第二附属医院收治的1例成人ITP合并肝血管瘤患者的临床资料,并结合文献总结该病的临床诊治特点。结果 患者为46岁女性,因“全身皮肤黏膜出血1个月余,血小板减少1 d”入住血液内科。血细胞分析血小板计数4.0×10⁹/L,凝血功能正常,骨髓穿刺活检提示巨核细胞相对增多,血小板少见,高度疑似ITP。经激素等治疗后皮肤黏膜出血好转,血小板升高至59×10⁹/L。上腹部增强CT提示右肝巨大血管瘤,转至肝胆胰外科行肝血管瘤切除,术后术区渗血明显,阴道流血,血小板逐步降低至3.0×10⁹/L,右侧胸腔大量积液,但凝血功能正常。经止血、输血、调节免疫、抗感染、胸腔穿刺引流处理后逐步恢复。术后第13天患者血小板升高至220×10⁹/L。随访32个月,患者皮肤黏膜不再出血,血小板计数维持在正常范围。结论 成人ITP,需警惕合并肝血管瘤,处理血管瘤可使患者获得良好的远期效果,但需做好围术期管理以使患者顺利恢复。     

重组人红细胞生成素治疗慢性肾衰竭非透析病人贫血30例疗效观察

贫血是慢性肾衰竭的常见并发症 ,近来重组人红细胞生成素 (ｒ -ＨｕＥＰＯ)应用于临床使慢性肾衰贫血治疗取得良好效果 ,本文收集我院 30例肾性贫血患者 ,应用国产重组人红细胞生成素注射液 (益比奥 ,沈阳三生制药公司产品 )治疗 ,取得较好疗效 ,现报告如下。材料与方法1　一般资料　 30例患者均符合慢性肾衰竭诊断标准 ,男 13例 ,女 17例 ;其原发病为慢性肾炎 2 4例 ,痛风肾 2例 ,多囊肾 2例 ,慢性梗阻性肾病 2例。本组病例氮质血症期 16例 ,尿毒症期 14例 ,均有不同程度贫血 ,其中红细胞(ＲＢＣ)最高 2 .90× 10 12 /Ｌ ,最低 1.6 3× 10 …     

ITP合并下消化道出血腹腔镜探查1例报告

患者男,16岁,因“腹痛,解血水样便”入院于血液科。患者2个月前发现患有特发性血小板减少性紫癜,一直服用激素治疗。入院前4h开始出现脐周阵发性腹痛,不向他处放射,解血水样便,每天2～3次,每次约150ml。查体：BP110／60mmHg,P96次／min,满月脸,水牛背,全腹平坦,未见胃肠型及蠕动波,左上腹压痛（＋）,无反跳痛,未扪及包块,     

脾切除术治疗特发性血小板减少性紫癜29例报告

目的探讨脾切除术治疗特发性血小板减少性紫癜（idiopathic thromboeytpertic purpura,ITP）手术以及围手术期处理需要注意的问题和疗效。方法回顾性分析29例ITP患者临床资料,包括年龄、性别、围手术期治疗等,对临床疗效进行观察。结果手术前10例患者行腹部CT检查,8例发现副脾,1例发现右侧肾肿瘤,1例发现腹膜后血肿,25例行腹部B超检查,5例发现副脾。29例均行脾切除术,手术中切除副脾8例。手术后1例颅内出血死亡,其余存活,死亡率3．45％。手术后胰尾小动脉出血1例,二次手术予以止血,1例并发左侧膈下脓肿。29例患者脾切除术后第一天有效率100％,手术后1周有效率93％（27／29）。随访15例,随访2～87个月,其中有效73．7％（11／15）,部分有效13．3％（2／15）,无效6．67％（1／15）。结论完善检查、围手术期处理措施得当是ITP外科手术治疗的关键因素。     

腹腔镜脾切除术32例报告

目的探讨腹腔镜脾切除术（laparoscopic splenectomy，LS）的可行性。方法我院1999年6月-2005年12月行LS32例，超声刀游离脾周韧带，血管切割缝合器离断脾蒂，脾脏装入塑料袋剪碎取出。结果LS成功29例，手术时间60-270min，平均100min；术中出血量30-1000ml，平均230ml。术后住院3—7d，平均5d，无术后并发症。3例中转开腹，1例为脾蒂出血，1例为胃短血管出血，1例为脾周围炎粘连紧密出血。22例特发性血小板减少性紫癜中18例血小板恢复正常，4例术后无升高。2例溶血性贫血术后血红蛋白升高。4例肝炎后肝硬化合并脾功能亢进者术后血小板恢复正常。结论腹腔镜脾切除术安全可行．尤其适合于血液系统疾病中须行脾切除者及脾脏本身病变者。     

肾移植术后移植肾癌肉瘤1例报告并文献复习

目的：提高对肾移植术后移植肾癌肉瘤的认识及诊疗水平。方法：回顾性分析1例肾移植术后移植肾癌肉瘤患者的临床资料：患者女,73岁,因尿毒症于1996年10月在外院行肾移植术,移植肾置入右髂窝;因移植肾失功能于2001年3月行第二次肾移植术．移植肾置于左侧髂窝。2006年11月出现右侧移植肾区疼痛,伴镜下血尿。B超、CT检查发现右侧移植肾占位性病变而入院。在全麻下行移植。肾探查术．将右侧移植肾完整切除。结果：手术顺利,手术时间3h．出血量150ml,病理检查报告为移植肾癌肉瘤。术后2个月,患者出现排斥反应与肠梗阻,病情急剧恶化,家属放弃治疗。结论：移植肾癌肉瘤恶性程度高,预后极差．早期诊断有助于提高生存率。     

150例妊娠合并特发性血小板减少性紫癜的临床护理

目的:探讨、掌握并总结妊娠合并血小板减少性紫痰的并发症及围生期的护理.方法:对本科2007～2010年收治的150例妊娠合并特发性血小板减少性紫赢患者病例的临床资料、治疗方法、及护理措施进行系统性回顾,将其对妊娠结局的影响及护理进行总结.结果:149例患者均治愈,无并发症发生,其中1例患者因并发心力衰竭,抢救无效死亡.结论;特发性血小板减少性紫癜是妊娠的较常见并发症,是由各种生理和病理情况引起的,是孕产妇较常见的内科血液系统合并症,约占妊娠总数的3.7%,在妊娠末期其发生率达6%～15%,量度血小板减少症的血小板计数<50×10<'9>/L,其中血小板计数和功能的降低,可引起严重出血、贫血,胎死宫内及新生儿颅内出血等.为降低孕产妇及围生儿的病死率,减低妊娠合并血小板减少症对母婴的危害,提高护士对妊娠合并血小板减少症的认识,因此产首要做好充分准备,严密观察病情变化,积极有效的治疗,现将对本科收治的妊娠合并特发性血小板减少紫癜病例进行了回顾,能根据不同情况采取相应处理,减少患者的出血风险,对妊娠结局的影响及其护理进行了总结,现报告如下.     

肾移植治疗终末期肾衰竭伴纯红再障临床研究--附2例远期疗效随访报告及文献复习

目的:通过总结2例终末期肾衰竭(ESRD)患者伴纯红再障(pure red cell aplasia,PRCA)的临床资料,介绍该病的诊断与治疗.方法:回顾性总结2例ESRD合并PRCA的诊治并复习相关文献.结果:2例ESRD患者均确诊为肾性贫血常规给予α-促红素(α-rhuEPO)治疗后出现PRCA.2例经肾移植术,随访3年以上,目前病情稳定.结论:使用促红素治疗ESRD所致肾性贫血出现的PRCA,其首选的治疗方法是肾移植.     

高致敏受者再次肾移植3例报告并文献复习

目的探讨对高致敏受者再次进行肾移植的方法。方法 3例高致敏受者再次肾移植术前选择合适的供者,包括亲属活体供肾,供受者间HLA配型良好,淋巴细胞毒交叉配型试验结果均为1%,供者特异性抗体检测阴性。免疫抑制方案采用他克莫司＋吗替麦考酚酯＋泼尼松三联方案并分别联合利妥昔单抗、抗胸腺细胞球蛋白、巴利昔单抗治疗。脱敏方案采用血浆置换或免疫吸附或血浆置换＋低剂量静脉用免疫球蛋白。结果 3例手术均成功,受者无排斥反应发生,术后1～3 d血清肌酐降至正常。3例受者随访29～42个月人肾均存活,术后未发生病毒感染及肺炎,无肝功能损害发生。结论良好的HLA配型和避免供者特异性抗体是高致敏患者再次肾移植成功的关键。采用巴利昔单抗、抗胸腺细胞球蛋白、利妥昔单抗及脱敏治疗可以减少排斥反应的发生。     

特发性血小板减少性紫癜患者接受肾移植的安全性观察

目的  探讨特发性血小板减少性紫癜(ITP)患者接受肾移植的安全性。 方法  回顾性分析两例ITP肾移植患者的临床资料并结合相关文献进行复习。 结果  两例患者肾移植术前血小板计数分别为41×10⁹/L及34×10⁹/L, 凝血功能正常, 临床无活动性出血表现, 均成功进行肾移植手术, 术中、术后无明显出血。1例患者术后接受氢化可的松冲击治疗3 d并服用以环孢素为基础免疫抑制剂, 术后7 d血小板计数恢复至正常范围并维持稳定。另1例患者术后接受甲泼尼龙冲击治疗3 d并服用以他克莫司为基础的免疫抑制剂, 术后应用多种提升血小板药物并输注血小板治疗, 血小板波动在10×10⁹/L~30×10⁹/L。两例患者移植肾功能均恢复良好。 结论  ITP患者的肾移植手术出血的风险性主要与患者在术前有否活动性出血有关, 术前无活动性出血的尿毒症患者行肾移植是相对安全的。     

Renal failure after anti-D globulin treatment of idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is a disorder of rapid destruction of antibody-coated platelets. Anti-D immune globulin has been used for treatment of ITP in the United States since 1995. Initial studies identified no significant side effects of treatment. However, a recent report highlighted occasional episodes of intravascular hemolysis after anti-D immune globulin. We describe two children with ITP who developed acute renal failure (ARF) after treatment with anti-D immune globulin and also analyze ten additional cases of ARF reported to the manufacturer, Cangene Corporation, through postmarketing surveillance. All episodes of ARF were associated with intravascular hemolysis. Four patients required dialysis. Patient age ranged from 1 to 82 years, but those requiring dialysis were all under age 15 years. Several patients with ARF had preexisting creatinine elevation. Three of the patients with ARF had serologic evidence of acute Epstein-Barr virus (EBV) infection. Renal biopsy in one patient showed acute tubular necrosis, with findings consistent with pigment nephropathy. Anti-D immune globulin, used to treat ITP, may be associated with intravascular hemolysis and resultant ARF. Renal function should be monitored in patients with evidence of intravascular hemolysis. Children and adolescents may have increased risk of ARF requiring dialysis. Received: 30 March 2001 / Revised: 17 September 2001 / Accepted: 18 September 2001     

IgA nephropathy and idiopathic thrombocytopenic purpura with splenectomy: a case report

A 14-year-old boy who had had a splenectomy at the age of 2 years for idiopathic thrombocytopenic purpura, suffered from IgA nephropathy. Serum IgA and IgE levels were elevated and low levels of circulating immune complexes were detected. Splenectomy may play a role in the pathogenesis or susceptibility to IgA nephropathy by means of decreased clearance of circulating immune complexes or impaired immune regulation, such as increased IgA synthesis.     

Hematological long-term results of laparoscopic splenectomy for patients with idiopathic thrombocytopenic purpura: a case control study

Background Laparoscopic splenectomy (LS) for idiopathic thrombocytopenic purpura (ITP) appears, when compared to open splenectomy (OS), associated with immediate important advantages. However, in a number of patients splenectomy does not lead to an adequate response, or after initial adequate response a relapse occurs after some time. A relapse may be associated to the presence of accessory spleens and splenosis. The purpose of this study was to compare the operative outcome and the hematological results on the long term of a series of LS with a historic series of OS for the treatment of ITP.Methods A retrospective review was done of 50 consecutive patients who underwent LS for ITP. Patient characteristics, outcome of surgery, and hematological results were compared to a historical group of patients who underwent conventional splenectomy for ITP (n = 31). Response to splenectomy was defined in three groups: complete remission, partial remission, and no response. Grouping was based on hematological data.Results Concerning operative outcome and postoperative complications, there was a significant difference in favor of LS. Moreover, the hematological outcome of both groups showed no differences after a median period of 66 months (OS) and 35 months (LS), respectively.Conclusions Hematological results after laparoscopic splenectomy for ITP are comparable to those after open splenectomy in both the short and the long term.     

腹腔镜下脾切除术治疗特发性血小板减少性紫癜的临床应用研究(附15例报告)

目的:探讨腹腔镜下脾切除术的手术方法和治疗特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的临床效果。方法:选择15例ITP患者在腹腔镜下完成脾切除术,比较手术前后的血小板计数。结果:15例均在腹腔镜下完成脾切除术,平均手术时间160min,术中平均失血50ml,术后平均住院8d,总有效率为94%。术后1周内血小板的反应率为100%。无死亡、出血等严重并发症发生。结论:ITP患者行腹腔镜脾切除术是安全可行的,手术效果满意。     

Idiopathic thrombocytopenic purpura complicated with biliary atresia: a rare occurrence and literature review

The association of biliary atresia (BA) and idiopathic thrombocytopenic purpura (ITP) is extremely rare, with only 2 cases being reported in the literature. This report describes the very rare case of a 1-year-old boy with BA complicated with ITP after cholangitis and the successful steroid treatment of ITP. We review the literature on this type of relationship between BA and ITP and discuss the clinical features of this complication. Furthermore, the possible cause of the onset of ITP complicated with BA was explored in this report.     

An 18-year review of open and laparoscopic splenectomy for idiopathic thrombocytopenic purpura

BACKGROUND: Laparoscopic splenectomy has become the preferred surgical procedure for the management of idiopathic thrombocytopenic purpura (ITP). However, there studies have directly compared the incidence of recurrent ITP secondary to missed accessory spleens in open versus laparoscopic splenectomy. METHODS: Open and laparoscopic splenectomies performed for ITP at 4 sites over 18 years were analyzed. The incidence of recurrent disease secondary to missed accessory spleens was compared between the open and laparoscopic splenectomy groups. RESULTS: A total of 105 splenectomies (54 open/51 laparoscopic) were performed. Accessory spleens were identified in 6 laparoscopic and 6 open cases (P = .57). Recurrent disease occurred in 27.6% of open and 14.6% of laparoscopic cases (P = .222). There were no cases of recurrent ITP secondary to a missed accessory spleen in either group. CONCLUSIONS: The incidence of missed accessory spleens causing recurrent disease is similar when splenectomy is performed either open or laparoscopically.     

Resection of bulky chromophobe renal cell carcinoma resolved severe idiopathic thrombocytopenic purpura: A case report

Idiopathic thrombocytopenic purpura associated with renal cell carcinoma is relatively rare. We report the case of a 48‐year‐old woman with massive renal cell carcinoma, measuring approximately 20 × 14 × 14 cm, who presented with severe thrombocytopenia: platelet count, 2000 cells/μL. After confirming normal bone marrow, she received high‐dose dexamethasone and intravenous gamma globulin, which raised the platelet count to normal levels. She then underwent left radical nephrectomy. The pathological examination showed chromophobe renal cell carcinoma. After the resection, the platelet count was maintained within the normal range without any treatment. The current case is the first report of chromophobe renal cell carcinoma causative of severe idiopathic thrombocytopenic purpura.     

Postoperative thrombotic thrombocytopenic purpura in an infant: case report and literature review

Thrombotic thrombocytopenic purpura is caused by an imbalance of von Willebrand factor and its cleaving protease, which leads to the formation of microthrombi in end-organs. It rarely occurs in the pediatric population. Plasma exchange can significantly reduce mortality and morbidity. We present a 14-month-old infant in whom clinical and laboratory abnormalities compatible with thrombotic thrombocytopenic purpura were noted several days after resection of a large pelvic tumor. Treatment with double volume plasma exchange on postoperative day 5 led to complete resolution of the renal failure, thrombocytopenia, anemia, and neurological manifestations. ADAMTS13 inhibitors were negative and no mutations were found in factor H, factor I, membrane cofactor protein, and thrombomodulin to account for genetic predisposition to thrombotic thrombocytopenic purpura or atypical hemolytic uremic syndrome. Postoperative anemia, thrombocytopenia, fever, and neurological deficits in children should raise the suspicion of thrombotic thrombocytopenic purpura. Early diagnosis is important because the disorder is readily and efficiently treated with plasma exchange.     

Reversible diabetes mellitus during growth hormone therapy in chronic renal failure

A 12-year-old girl with short stature due to idiopathic Fanconi syndrome and chronic renal failure was treated with recombinant human growth hormone (rhGH). There was no family history of diabetes mellitus and the glucose tolerance before treatment was normal. Intravenous glucose tolerance tests were performed before, during and after treatment. Two months after starting rhGH the early phase of insulin secretion (1-+3-min values) was diminished, and the patient developed manifest diabetes mellitus with hyperglycemia and an elevated hemoglobin A_1c. Following discontinuation of rhGH, glucose tolerance slowly returned to normal. Received May 29, 1997; received in revised form November 5, 1997; accepted November 12, 1997