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1.
Renal function tests (cystatin C, serum and urine creatinine, creatinine clearance, serum and urine 2-microglobulin, microalbuminuria, osmolality) were performed in 21 children at the diagnosis and during the treatment for acute lymphoblastic leukemia (ALL) (group I) and in 37 children (group II) treated for ALL 3.9±3.7 years before the study. The results were compared to 20 healthy children. Mean values of renal tests were in normal range at all points of analysis in groups I and II compared to the control group. Transitory higher cystatin C values (but in normal range) were observed after methotrexate administration and after the end of treatment. Deteriorated renal function was observed in one child during the treatment (after each protocol) and in five children treated previously for ALL. In conclusion, combined treatment for ALL is not associated with severe or long-term impairment of renal function.  相似文献   

2.
A 5-year-old African-American male was diagnosed with nephrotic syndrome (NS). Because of concomitant leukopenia, bone marrow aspiration was performed, which did not demonstrate a hematological malignancy. The patient received standard daily steroid therapy for treatment of NS. Steroid resistance at 5 weeks of therapy led to a renal biopsy, which documented focal segmental glomerulosclerosis (FSGS). He was begun on cyclosporin A (CsA) and later switched to tacrolimus because of side-effects of CsA. Seven months after the initial diagnosis of NS, the patient was diagnosed with acute lymphoblastic leukemia (ALL). The patient is in complete remission of ALL and partial remission of NS with continued nephrotic-range proteinuria. Review of the literature shows four other cases of pediatric ALL after NS. No particular immunosuppressive agent seemed to be causative in the evolution of ALL. Although the exact mechanism for development of ALL after NS is unknown, the incidence of leukemia may be increased after immunosuppressive therapy when used in this context.  相似文献   

3.
A 3.5-year-old girl with fever had a pancytopenic blood smear that also showed schistocytes and blast cells. Bone marrow examination resulted in a diagnosis of acute lymphoblastic leukemia (ALL). Although creatinine on admission was normal, she had mild hematuria and moderate proteinuria. Chemotherapy was started, but she was initially given only steroids (dexamethasone) due to high liver enzymes. Her renal parameters worsened, and her creatinine doubled. She also developed nephrotic-range proteinuria and hypertension. Renal biopsy showed thrombotic microangiopathy that was clinically consistent with hemolytic uremic syndrome (HUS). Some reports of HUS preceding ALL do exist. However, to the best of our knowledge, this is the first case that describes ALL and HUS presenting simultaneously.  相似文献   

4.
Osteonecrosis (ON) is a disabling complication of acute lymphoblastic leukemia (ALL) treatment in children and young adults. Isolated talus involvement is thought to be uncommon. A unique case of a 11-year-old female patient with ON in her left talus which developed six months after the completion of chemotherapy that she received for ALL is reported. A conservative treatment protocol was followed including activity modification, analgesia and prevention of weight-bearing. However, the disease significantly progressed during follow-up period. The present study makes an important contribution to the literature with unusual involvement pattern and location of ON after ALL treatment and with a long follow-up duration.Level of evidenceIV.  相似文献   

5.
The Authors describe the case of a 14-year-old girl who developed a multifocal osteonecrosis (ON), after treatment with chemotherapy and corticosteroids for acute lymphoblastic leukemia (ALL). She came to our attention about 6 months after the beginning of treatment complaining of pain in her left knee and later in both hips and shoulders. Radiography and MRI confirmed the presence of avascular ON at all these sites. The patient underwent bilateral total hip arthroplasty, which was still functioning well at the last follow-up visits at 5 years, and at 5 years and 4 months. ON is increasingly recognized as a complication of the treatment of cancer in children and adolescents, and as patients now frequently survive ALL into adulthood, orthopedists will be increasingly called on to manage this complication affecting multiple joints in children and young adults.  相似文献   

6.
Neutropenic enterocolitis (NE) is a life-threatening complication most commonly seen in patients receiving intensive chemotherapy for acute leukemia. The condition usually affects the terminal ileum, cecum, or ascending colon. In rare instances, NE may occur before the initiation of chemotherapy or involve more distal bowel. The authors report the case of a 2-year-old girl who had NE affecting the descending colon as a presenting complication of acute lymphoblastic leukemia. Despite aggressive medical interventions, including granulocyte infusions, she had a delayed bowel perforation that was managed successfully with surgery. This case highlights the challenges of treating patients who have NE as an initial manifestation of acute leukemia.  相似文献   

7.
目的 观察造血干细胞移植(HSCT)联合伊马替尼治疗费城染色体阳性(Ph+)急性淋巴细胞白血病(ALL)患者的疗效,分析影响预后的因素.方法 Ph+ALL患者32例,全部患者移植前均达血液学完全缓解,其中第1次缓解(CR1)期27例,CR2期5例.19例患者移植前达到分子生物学缓解(MR).32例中,自体移植4例,异基因移植28例.采用全身照射(TBI)+环磷酰胺(Cy)+氟达拉滨(Flu)+阿糖胞苷(Ara-C)的预处理方案.患者输注单个核细胞的中位数为5.6×108/kg,输注CD34+细胞中位数为2.94×106/kg.31例患者于HSCT前应用伊马替尼,口服,400~600 mg/d;16例患者于HSCT后应用伊马替尼,包括预防性治疗7例,剂量为300~400mg/d,治疗9例,剂量为400~600 mg/d.结果 32例患者移植后均获得造血重建.全部患者预期3年总体存活率为(62.1±8.6)%,无白血病存活率为(59.2±8.7)%,复发率为(17.7±7.2)%,移植相关死亡率为(26.2±8.0)%.4例自体移植患者均存活,其中3例已持续缓解14、18和67个月.对异基因移植进行单因素分析显示,行HLA匹配亲缘HSCT者的总体存活率为76.5%,高于行无关供者或HLA半相合亲缘HSCT者(27.3%,P<0.05),前者无白血病存活率也高于后者(分别为70.6%和27.3%,P<0.05).移植前获得MR者的复发率为5.6%,低于未缓解者(40.0%,P<0.05),移植前处于CR1期者的复发率为12.5%,低于CR2期者(50.0%,P<0.05).结论 伊马替尼联合HSCT可用于治疗Ph+ALL患者,移植前获得MR或于CR1期进行移植者的预后更佳.
Abstract:
Objective To analyze the outcomes and the prognostic factors of hematopoietic stem cell transplantation (HSCT) in combination with imatinib for Philadelphia chromosome positive acute lymphoblastic leukemia (Ph+ ALL). Methods All 32 patients with Ph+ ALL achieved hematologic complete remission (CR) at time of transplantation, including 27 cases in the first CR (CR1) and 5 in CR2. Nineteen patients achieved molecular remission (MR). Among 32 patients, 4 received autologous HSCT (AHSCT), and 28 allogeneic HSCT (allo-HSCT). The conditioning regimens comprised of total body irradiation (TBI), cyclophosphamide, fludarabine and cytarabine. The median number of transfused mononuclear cells was 5. 6 × 108/kg, and that of CD34+ cells was 2. 94 × 106 /kg. Thirty-one patients were administrated imatinib orally before transplantion, at a dose of 400~600 mg/day, and 16 patients after transplantation, including 7 for prevention at a dose of 300~400 mg/day and 9 for salvage treatment at a dose of 400 ~ 600 mg/day. Results Hematopoietic reconstitution was achieved in all 32 patients. Three-year estimate of overall survival (OS) was (62. 1±8. 6)%, leukemia-free survival (LFS) (59. 2 ± 8. 7)%, relapse rate (RR) (17. 7 ± 7. 2)% and transplant-related mortality (26. 2 ± 8. 0) %. All 4 undergoing AHSCT were alive, and 3 out of them were in continuous CR with durations of 14, 18 and 67 months respectively. The univariate analysis for prognosis in allo-HSCT showed that the OS of HLA-matched sibling donors group was 76. 5 %,higher than that of unrelated or haploidentical donors group (27. 3 %, P<0. 05), and so was LFS (70. 6 % vs 27. 3 %, P<0. 05). RR in patients achieving MR at time of transplantation was 5. 6 %,lower than that in those not achieving MR (40. 0 %, P<0. 05). RR in patients in CR1 at time of transplantation was 12. 5 %, lower than that in those in CR2 (50 %, P <0. 05). Conclusion Imatinib improved the outcomes of HSCT for Ph+ ALL, especially to patients achieving MR at time of transplantation and transplantation in early stage (CR1).  相似文献   

8.
目的 探讨急性淋巴细胞白血病患儿父母养育倦怠现状及影响因素,为临床针对性护理干预提供参考。方法 采用一般资料问卷、养育倦怠评估量表、疾病不确定感量表和领悟社会支持量表对广州市4所三级甲等医院收治的259例急性淋巴细胞白血病患儿的父母进行调查。结果 患儿父母养育倦怠得分为(90.19±14.25)分;患儿疾病严重程度、与患儿关系、家庭人均月收入、疾病不确定感总分、领悟社会支持总分是养育倦怠的主要影响因素(均P<0.05),可解释总变异的50.9%。结论 急性淋巴细胞白血病患儿父母存在养育倦怠,给予患儿疾病严重程度高、患儿母亲、家庭人均月收入低的患儿父母更多关注,降低其疾病不确定感并提高其领悟社会支持水平,从而降低其养育倦怠水平。  相似文献   

9.
目的  探讨含去甲氧柔红霉素(IDA)的预处理方案在异基因造血干细胞移植(allo-HSCT)治疗高危难治性白血病中的临床疗效。方法  对116例接受了allo-HSCT的高危难治性白血病患者,采用7种含IDA的预处理方案。总结116例受者的植入情况。采用Kaplan-Meier曲线对2年总生存率(OS)、2年无病生存率(DFS)、累积复发率、复发病死率、移植相关病死率(TRM)、急性移植物抗宿主疾病(aGVHD)及慢性移植物抗宿主疾病(cGVHD)的累积发生率进行统计学分析。结果  116例受者均成功植入。中位随访时间为28(7~70)个月,64例受者存活,2年OS为55.2%,2年DFS为51.7%,2年复发病死率23.3%,2年TRM为18.1%。116例受者中有72例发生aGVHD,aGVHD 2年累积发生率为62.1%,其中Ⅲ~Ⅳ度aGVHD 20例,2年累积发生率为17.2%。59例发生cGVHD,2年累积发生率为55.4%,其中广泛型cGVHD 2年累积发生率为14.7%。116例受者中有30例复发,2年累积复发率为25.9%。结论  含IDA的预处理方案安全性和有效性均较高,可以作为高危难治性白血病患者移植预处理的有效方案。  相似文献   

10.
张競文  陆英  李旭东 《器官移植》2015,6(6):415-419
目的  分析儿童高危急性淋巴细胞白血病脐血移植失败后白血病长期缓解的原因。方法  收集2013年9月在中山大学附属第三医院血液内科接受脐血干细胞移植的1例高危急性淋巴细胞白血病患儿的临床资料。分析患儿术后治疗过程及预后, 并结合文献资料分析患者移植失败但白血病治疗获得良好效果的可能原因。结果  患儿首次脐血干细胞移植后造血恢复不良, 2013年11月行脐血输注, 后因为经济原因未针对原发病进一步治疗, 然而该患者脐血移植术后15个月造血功能恢复, 至2015年9月, 脐血移植后2年无病生存。结论  脐血移植或输注可能具有修复造血及免疫系统的功能, 且无需通过成功植入发挥作用。儿童高危白血病进行脐血移植植入失败并不意味白血病治疗失败, 患儿有可能获得长期无病生存。  相似文献   

11.
异基因造血干细胞移植是恶性血液病患者获得治愈的最有效手段,在临床上已广泛开展。急性白血病合并骨髓坏死(bone marrow necrosis,BMN)的病例临床有不少报道,但异基因造血干细胞移植治疗此类病例报道鲜见。我们于2011年收治了2例急性淋巴细胞白血病(acute lymphoblastic leukemia。ALL)合并BMN患者,经化疗诱导BMN逆转后为其实施了亲缘间单倍体相合外周血造血干细胞移植,均成功重建造血功能,现报道如下。  相似文献   

12.
13.

Background

The aim of this study was to describe the spectrum of disease in children with acute pancreatitis and assess predictors of severity.

Methods

Children (≤18 years) admitted to a single institution with acute pancreatitis from 2000 to 2009 were included. The accuracy of the Ranson, modified Glasgow, and pediatric acute pancreatitis severity (PAPS) scoring systems for predicting major complications was assessed.

Results

The etiology of pancreatitis in these 211 children was idiopathic (31.3%), medication-induced (19.9%), gallstones (11.8%), trauma (7.6%), transplantation (7.6%), structural (5.2%), and hemolytic-uremic syndrome (3.3%). Fifty-six patients (26.5%) developed severe complications. Using the cutoff thresholds in the PAPS scoring system, only admission white blood cell count more than 18,500/μL (odds ratio [OR], 3.1; P = .010), trough calcium less than 8.3 mg/dL (OR, 3.0; P = .019), and blood urea nitrogen rise greater than 5 mg/dL (OR, 4.1; P = .004) were independent predictors of severe outcome in a logistic regression model. The sensitivity (51.8%, 51.8%, 48.2%) and negative predictive value (83.2%, 83.5%, 80.5%) of the Ranson, modified Glasgow, and PAPS scores were, respectively, insufficient to guide clinical decision making.

Conclusion

Commonly used scoring systems have limited ability to predict disease severity in children and adolescents with acute pancreatitis. Careful and repeated evaluations are essential in managing these patients who may develop major complications without early signs.  相似文献   

14.
目的  探讨肝移植受者术后发生急性排斥反应时淋巴细胞亚群的变化及意义。方法  选取接受肝移植且发生急性排斥反应的受者作为排斥组(17例),利用倾向评分匹配方法按1∶1比例选取肝功能稳定的肝移植受者作为对照组(17例)。分析肝移植术后急性排斥反应的发生情况,对比两组受者他克莫司浓度。比较两组受者外周血淋巴细胞亚群的绝对值和比例,采用受试者工作特征(ROC)曲线分析淋巴细胞亚群对肝移植术后急性排斥反应发生的诊断价值。比较排斥组治疗前后淋巴细胞亚群绝对值和比例的变化。结果  排斥组17例受者中,4例在术后28 d内发生急性排斥反应,13例在术后29~180 d发生急性排斥反应。两组他克莫司谷浓度差异无统计学意义(P=0.295)。与对照组比较,排斥组受者外周血T细胞、CD4+T细胞、B细胞和自然杀伤(NK)T细胞的比例均上升(均为P < 0.05)。肝移植术后早期NKT细胞比例升高是肝移植术后发生急性排斥反应的独立危险因素[比值比(OR)1.774,95%可信区间(CI)1.059~2.971,P=0.029]。ROC曲线分析结果提示,CD4+T细胞、B细胞和NKT细胞比例的曲线下面积(AUC)分别为0.76、0.73和0.77。联用CD4+T细胞、B细胞和NKT细胞比例的AUC为0.89,当临界值为0.69时,灵敏度为0.706,特异度为0.941。排斥组所有受者治疗后均逐渐恢复,最终肝功能正常,治疗后T细胞、CD4+T细胞、CD8+T细胞和NK细胞比例均降低(均为P < 0.05)。结论  NKT细胞比例升高提示肝移植术后急性排斥反应发生风险增加,联用CD4+T细胞、B细胞和NKT细胞比例可早期发现和诊断肝移植术后急性排斥反应。  相似文献   

15.
目的提高对特殊临床表现猩红热的认识。 方法通过1例罕见的肱骨闭合性骨折后发生全身剥脱性皮损、病原学阴性的猩红热患者的临床诊疗情况,强调与葡萄球菌烫伤样皮肤综合征(SSSS)、川崎病、麻疹鉴别诊断的必要性。 结果通过临床症状、体征、辅检,患者得到准确诊断。经科学隔离、规范治疗而达到临床治愈。 结论鉴于猩红热的主要病原为A组β型溶血性链球菌(GABHS),具有传染性,因而快速诊断、消毒隔离极为重要。  相似文献   

16.
重症急性胆管炎时核因子-kB活性的变化及其临床意义   总被引:1,自引:0,他引:1  
目的 观察重症急性胆管炎 (ACST)患者术后外周血单核细胞 (PBMC)中核因子 kB(NF kB)活性的变化及其与胆道感染严重程度和预后的关系。方法  2 0例ACST患者根据其预后情况分为生存组 ( 14例 )和死亡组 ( 6例 ) ,同时选择胃溃疡行大部切除术或疝修补术等非炎症疾病患者 10例作为对照组 ,分别于术后 2 4h取外周血 ,用密度梯度离心法分离PBMC ,提取核蛋白 ,并用凝胶电迁移改变分析法 (EMSA)测定PBMC中NF kB活性的变化 ,所得结果用图像分析仪分析并以相对光密度 (ROD)表示。用酶联免疫法 (ELISA)测定血浆中TNF α、IL 6和IL 10含量的变化。结果 ACST死亡组NF kB活性最高 ( 5.0 2± 1.0 3 ) ,生存组次之 ( 2 .98± 0 .51) ,对照组最低 ( 1.0 2± 0 .3 4 ) ,各组间比较差异有显著性意义 (P<0 .0 5)。ACST死亡组血浆TNF α和IL 6含量明显增高 ,分别为 ( 4 96.2 8± 52 .3 5)ng/L和 ( 578.13±67.72 )ng/L ,生存组分别为 ( 2 84.47± 3 9.41)ng/L和 ( 3 18.67± 3 4 .92 )ng/L ,对照组分别为 ( 89.43± 10 .3 9)ng/L和( 10 1.2 7± 13 .47)ng/L ,各组间比较差异有显著性意义 (P<0 .0 5)。ACST死亡组和生存组IL 10分别为 ( 3 78.42± 41.0 2 )ng/L和 ( 3 84.75± 3 9.2 4)ng/L ,均明显高于对照组的 ( 69.3 6±  相似文献   

17.
目的 观察CT鉴别诊断儿童TFE3重排肾细胞癌(TFE3 RCC)与Wilms瘤(WT)的价值。方法 回顾性分析经手术病理证实的10例单发TFE3 RCC(TFE3 RCC组)及20例单发WT(WT组)患儿的术前腹盆腔CT资料。对比组间病灶CT表现差异;针对差异有统计学意义的参数绘制受试者工作特征(ROC)曲线,计算曲线下面积(AUC),评估其鉴别儿童TFE3 RCC与WT的效能。结果 组间CT所示病灶最大径、平扫CT净值、边界清晰与否、有无病灶内钙化、动脉期和静脉期强化CT值、强化程度差异均有统计学意义(P均<0.05),上述各项指标鉴别诊断TFE3 RCC与WT的AUC分别为0.82、0.97、0.80、0.75、0.91、0.83及0.93。结论 CT可有效鉴别诊断儿童TFE3 RCC与WT。  相似文献   

18.

Purpose

Acute compartment syndrome (ACS) of the upper extremity is a rare but serious condition. The purpose of this study was to determine the etiology, diagnosis, treatment, and outcome of ACS of the upper extremity in a pediatric population.

Methods

We performed a retrospective chart review of all patients who underwent a decompressive fasciotomy for ACS of the upper extremity. Data collected included demographics, injury details, presenting symptoms, compartment measurements, time to diagnosis, time to treatment, and outcomes at the latest follow-up.

Results

Twenty-three children underwent fasciotomies for ACS of the forearm (15) and hand (8), at an average age of 9.3 years (range 0–17.8 years). The most common etiologies were fracture (13) and intravenous (IV) infiltration (3). The most common presenting symptoms were pain (83 %) and swelling (65 %). Compartment pressures were measured in 17/23 patients, and all but two patients had at least one compartment with a pressure >30 mmHg. The final two patients were diagnosed and treated for ACS based on clinical signs and symptoms. The average time from injury to fasciotomy was 32.8 h (3.7–158.0 h). Long-term outcome was excellent for 17 patients (74 %) and fair for 5 (22 %), based on the presence of loss of motor function, stiffness, or decreased sensation. One patient with brachial plexus injury and poor baseline function was excluded from functional outcome scoring. There was no association between the time from diagnosis to fasciotomy and functional outcome at the final follow-up (p = 1.000).

Conclusions

Although ACS of the upper extremity in children is often associated with a long delay between injury and fasciotomy, most children still achieve excellent outcomes. The majority of patients presented with pain and at least one additional symptom, but treatment was often delayed, implying that ACS of the upper extremity in children is a difficult diagnosis to establish and may be associated with a prolonged clinical time course.  相似文献   

19.
目的  探讨肾移植术后稳定状态受者外周血淋巴细胞亚群的动态变化及其与肾功能的相关性。方法  筛选行首次肾移植且术后半年内移植肾功能稳定的受者45例, 采用流式细胞术(FCM)检测受者术后15 d及1、3、6个月共计180份外周血样本淋巴细胞亚群比例和绝对值。分析淋巴细胞亚群随术后时间延长的动态变化及其与血清肌酐(Scr)和血尿素氮(BUN)的相关性。结果  受者术后4个时间点Scr值比较, 差异均无统计学意义(均为P > 0.05)。术后15 d与术后1个月、术后1个月与术后3个月BUN比较, 差异均有统计学意义(P=0.002、P=0.001);术后15 d与术后1个月比较, CD3+CD8+T细胞、CD3+CD4+T细胞、自然杀伤(NK)细胞比例及CD4/CD8比值, 差异均有统计学意义(P=0.009、P=0.004、P < 0.001、P=0.004)。B细胞比例术后15 d与术后1个月比较、术后1个月与术后3个月比较, 差异均有统计学意义(均为P < 0.001)。CD3+T细胞、CD3+CD8+T细胞、CD3+CD4+T细胞和NK细胞绝对值术后15 d与术后1个月比较, 差异均有统计学意义(P=0.001、P=0.002、P=0.003、P < 0.001)。CD3+CD8+T细胞绝对值术后3个月和术后6个月比较, 差异有统计学意义(P=0.015)。B细胞绝对值术后1个月与术后3个月比较, 差异有统计学意义(P=0.001)。淋巴细胞亚群比例和绝对值与Scr均不相关(均为P > 0.05), CD3+CD8+T细胞、NK细胞比例和绝对值与BUN均呈负相关(P < 0.001~0.05), CD3+CD4+T细胞、B细胞比例与BUN均呈正相关(P < 0.001~0.05), CD3+T细胞绝对值与BUN呈负相关(P < 0.05)。结论  肾移植术后稳定状态受者的淋巴细胞亚群中T细胞和NK细胞术后1个月内升高至稳定状态, B细胞术后3个月内降低至稳定状态, 且淋巴细胞亚群的动态变化与BUN相关。  相似文献   

20.
目的 研究环孢素A(CsA)联合IL-2融合蛋白对小鼠移植术后乙肝疫苗的体液免疫反应影响,寻求肝移植术后预防乙型肝炎的有效方法.方法 将C57BL/6小鼠40只,分为实验组、对照组和空白组,未免疫组,每组10只.实验组、对照组行皮肤移植后(DBA小鼠为供体),实验组给予CsA(30 mg/kg,腹腔内注射)+IL-2融合蛋白,对照组只给予CsA,空白组只行皮肤移植,3组均给予乙肝疫苗安在时,未免疫组不作任何处理.14 d后测定各组乙肝抗体滴度水平、IL-4、IL-10、IL-2、IFN-γ水平,流式细胞测定各组淋巴组织中滤泡辅助T细胞(follicular helperT cell,Tfh)的比例及应用RT-PCR测定各组淋巴组织中IL-21和叉状头转录因子FoxP3的表达.结果 实验组移植术后14 d乙肝抗体滴度明显高于对照组(F=29.886,P=0.010),皮肤移植物的存活时间也明显长于对照组(F=29.772,P=0.011),ELISA测定血清Th2类因子IL-4,IL-10的水平实验组明显低于对照组(F=42.102,P=0.009;F=23.734,P-0.015),Th1类因子IL-2和IFN-γ水平明显高于对照组(F=18.156,P=0.0020;F =90.042,P =0.003).实验组混合淋巴细胞RT-PCR测定IL-21、FoxP3的表达水平明显高于对照组(F=9.784,P =0.048;F=27.883,P=0.012).结论 CsA联合IL-2融合蛋白能够增强乙肝疫苗的抗体滴度,同时能够延长小鼠皮肤移植物的存活时间,可能与促进IL-21及FoxP3基因的表达有关.  相似文献   

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