Acute lymphoblastic leukemia in children: correlation of musculoskeletal manifestations and immunophenotypes

Purpose Studies on musculoskeletal manifestations (MSM) of childhood acute lymphoblastic leukemia (ALL) have yielded variable findings with regard to their clinical impact. We investigated the significance for differential diagnosis, treatment and outcome of musculoskeletal complaints as presenting symptoms of ALL, and their correlation with leukemia immunophenotypes, for which data is lacking. Methods Data on 783 children in the national study for childhood ALL between 1984 and 2003 were reviewed retrospectively. Statistical analysis examined possible relationships between MSM at the time of diagnosis and demographic and clinical data, biological features of leukemia (peripheral blood counts, immunophenotype and main cytogenetic aberration), response to initial prednisone treatment, and outcome. Results Of 765 children with data on orthopaedic complaints, 240 presented with MSM (31.4%). Among these children, B cell precursor (BCP) was much more common (209/576, 36.3%) than T cell ALL (25/176, 14.2%). Patients with MSM had lower white blood cell counts (WBC) (median of 9 vs. 20 × 10⁹/L, P < 0.001) and percentage of blast cells in the peripheral blood at diagnosis compared to those without (median of 27 vs. 53%, P < 0.001). Hepatomegaly and splenomegaly were less common in MSM group (67 vs. 53% <3 cm, P < 0.001, and 63 vs. 50% <3 cm, P < 0.001, respectively). Poor response to initial treatment with prednisone was recorded in 7.1% of patients with MSM versus 11.5% of those without (P = 0.086). The analysis revealed no independent effect of MSM on event-free survival (EFS), after correcting for differences in EFS related to immunophenotype or initial WBC. Conclusions MSM occur mostly in children with BCP ALL who present with less involvement of extramedullary organs, low peripheral blood blasts and white blood cells counts. These findings highlight the importance of including ALL in the differential diagnosis of MSM even in the presence of an apparently normal peripheral blood count. Our study also suggests that MSM are caused by leukemic cells with enhanced biological propensity to remain relatively confined within the intramedullary bone-marrow space.     

The Hemodynamic Mechanisms of Lung Injury and Systemic Inflammatory Response Following Brain Death in the Transplant Donor

Brain-dead donors are the major source of lungs for transplantation. Brain death is characterized by two hemodynamic phases. Initially, massive sympathetic discharge results in a hypertensive crisis. This is followed by neurogenic hypotension. Up-regulation of pro-inflammatory mediators occurs in all organs and lung injury develops; this can adversely affect graft function post-transplantation. The mechanisms of the systemic and lung inflammation are unknown. We hypothesized that the hemodynamic changes are responsible for these inflammatory phenomena. Brain death was induced by intra-cranial balloon inflation in rats. This resulted in hypertensive crisis, followed by hypotension. There was a significant increase in blood neutrophil CD11b/CD18 expression and pro-inflammatory cytokine levels in serum and bronchoalveolar lavage, compared with control animals. Rupture of the capillary-alveolar membrane was demonstrated by electron microscopy. Elimination of the hypertensive response by α-adrenergic antagonist pre-treatment prevented inflammatory lung injury, reduced the systemic inflammatory markers and preserved capillary-alveolar membrane integrity. Correction of the neurogenic hypotension with noradrenaline ameliorated the systemic inflammatory response and improved oxygenation. We conclude that the sympathetic discharge triggers systemic and lung inflammation, which can be further enhanced by neurogenic hypotension. Management of the brain-dead donor with early anti-inflammatory treatment and vasoconstrictors is warranted.     

Characteristics of Acute Asthma Patients Attended Frequently Compared with Those Attended Only Occasionally in an Emergency Department

Asthma patients that depend on emergency department (ED) services are generally considered to have extremely poor disease control and prognosis. It is important to identify characteristics related to poor disease control and frequent visits to the ED to apply appropriate clinical management. This study comprised a cross-sectional survey of consecutive patients with asthma exacerbation (age ≥12 years) presenting at the adult ED of a large, tertiary care, university-affiliated hospital over a 2-month period. The frequent visitors (FV) were defined by ≥3 visits to the ED in the preceding year, and the occasional visitors (OV) by ≤2 visits. Eighty-six patients (61 females and 25 males) were included in the study (mean age 38 ± 18 years). Of these patients, 51.2% were FV and 48.8% were OV. Sixty-nine percent had annual income lower than A$3000 and 66.3% had ≤8 years of the formal education. Only 18.6% had used inhaled corticosteroids, 79.1% identified the asthma attack severity, 70.9% increased or initiated inhaled β-agonist, 20.9% increased or initiated steroid therapy, and 55.8% had an asthma action plan for attack. The number of hospital admissions in past year (OR 4.3, P = .02), use of home nebulizer (OR 3.6, P = .05) and the lack of a written asthma action plan (OR 3.3, P = .03) were independently associated with frequent visits to the ED. We conclude that a substantial proportion of the patients that visit the ED are FV. These patients are more likely to have hospital admission in the past year, to use a home nebulizer, and to lack a written asthma action plan. They should be considered the most important target for asthma education.     

Fas和mdr-1在急性白血病的表达及其相关性研究

本研究应用流式细胞仪 (FCM)直接免疫荧光法和半定量RT PCR方法分别测定 5 9例初发急性白血病(AL)患者治疗前及完全缓解 (completeremission ,CR)后骨髓Fas和mdr 1mRNA表达情况 ,旨在探讨Fas和mdr 1在AL的表达及二者在多药耐药 (multidrugresistance ,MDR)中的相关性。结果显示 :Fas在初发AML阳性表达率比ALL高 ,两表达率间有差异 (P <0 .0 5 ) ,mdr 1在AML和ALL阳性表达率间无差异 (P >0 .0 5 ) ,Fas 与mdr 1 有负相关性 (r =- 0 .2 82 ,P <0 .0 5 ) ;经单因素及多因素COX分析 ,Fas和mdr 1独立于其他参数 ,更具判断预后价值 ;在Fas 与Fas-的AML和AL ,CR率均有显著性差异 (P <0 .0 1) ,在mdr 1 、mdr 1-的AML和AL中的CR率有显著性差异 (P <0 .0 1) ;经Logrank检验 ,Fas 与Fas-组CR率及中数缓解时间有显著性差异 (χ2 =7.35 ,P =0 .0 0 6 7) ,mdr 1 与mdr 1-组CR率及中数缓解时间有显著性差异 (χ2 =10 .71,P =0 .0 0 11)。结论 :Fas、mdr 1与疗效高度相关 ;Fas阳性表达可能是AL预后良好因素 ;mdr 1为疗效差、预后不良的重要因素。     

早期肠内营养及其输注方式对大鼠重症急性胰腺炎病理和肠道功能的影响

目的:观察早期肠内营养及其不同输注方式对重症急性胰腺炎的影响。方法:采用胰管逆行注射建立大鼠重症急性胰腺炎(SAP)模型,假手术组用生理盐水,SAP组随机分为持续、间隙和未行肠内营养3个亚组(每组12只),假手术组同样分为3个亚组(每组8只)。各组于48 h后启动肠内营养,72 h后再次麻醉大鼠,收集组织及血液标本;检测指标包括肠道转运系数、血淀粉酶、器官细菌移位率、血清细胞因子TNF-α、胰腺病理评分及血浆D-乳酸等。结果:①、SAP后48h进行肠内营养并未加重胰腺炎;肠内营养组与未行肠内营养组在血淀粉酶、胰腺病理评分方面,差异无统计学意义(P>0.05);然而早期肠内营养可以促进肠蠕动,减少肠道细菌移位率,两组间在肠道转运系数、脏器细菌移位率及血清TNF-α水平方面,差异有统计学意义(P<0.05)。②、SAP行肠内营养的两种方式间,各项指标比较无显著差异。结论:SAP大鼠在早期行肠内营养是可行的,其可保持肠道生理的完整性、减少了脏器细菌移位率,但未改善预后;持续和间隙二种肠内营养输注方式间无显著差异。     

综合医院医务人员感染SARS情况对比分析

目的：分析在防治SARS工作初期、后期医务人员感染SARS的差异和采取的对策，为减少综合医院内医务人员SARS的感染提供参考。方甚：对本院自2003年3月24日至6月2日SARS诊断治疗任务中发生的医务人员的感染原因和采取的对策进行回顾性分析。结果：防治SARS工作初期，急诊科交叉感染和在SARS隔离病房等与SARS患者密切接触的工作岗位，共有17名医务人员感染，及时隔离治疗，均已痊愈，未造成医护人员的进一步交叉感染：经加强防护培训，改善工作条件，在防治SARS工作后期，仅发生2名护工感染，亦已痊愈，派出非典医疗队实现零感染。结论：综合医院内医务人员感染SARS的问题严峻，加强医院内的全员防护培训，按岗分区管理，改善医务人员的工作条件和病人的隔离观察环境，做好监督检查，积极应对，可以减少医务人员的SARS感染。     

Diagnostic ultrasound of acute colonic diverticulitis by surgical residents

Background: Recent studies have documented the feasibility of ultrasonography (US) to diagnose acute colonic diverticulitis (ACD). This prospective observational trial determined the sonomorphology of ACD and evaluated the diagnostic accuracy of routine US performed on admission by surgeons in training. Methods: Fifty-seven consecutive patients with a confirmed episode of ACD were entered into this study, and the sonomorphology of the involved colon was assessed. US findings were compared to the results of the clinical evaluation and correlated to the clinicopathological outcome. Results: The sonomorphology of ACD was characterized by segmental inflammatory transformation of the colon averaging 9.9 ± 3.2 cm (range, 6–20) in length and visualized as target phenomena of a mean 3.5 ± 0.8 cm (range, 2.4–4.8) width. Targets were caused by hypoechogenic thickening of the colonic wall of an average 7.7 ± 2.6 mm (range, 4–18). In 40% of cases, a hyperechogenic halo representing peridiverticulitis (average width, 2.3 ± 0.6; range, 1.2–3 cm) was noted. Diverticula were seen in almost half of the cases. Of the 57 cases with confirmed ACD, the diagnosis was made by US in 48, for a global accuracy of 84.2%. US was false negative in nine patients, suggesting perforated appendicitis in five cases and acute appendicitis in one (the final diagnoses were perforated sigmoid diverticulitis in five cases and cecal diverticulitis in one case). In three patients, US was nondiagnostic. Conclusion: In the hands of sonographically trained surgeons, ultrasound is a useful modality to image acute colonic diverticulitis. US reveals diagnostic sonomorphology in most cases of ACD and therefore facilitates early confirmation of the diagnosis and assessment of severity. Received: 3 October 1996/Accepted: 9 May 1997     

TPA诱导的血小板聚集的研究

应用不同浓度ＴＰＡ诱发血小板聚集，观察２０名正常人血小板的聚集率。结果显示：ＴＰＡ从５ｎｇ／ｍｌ到１０ｎｇ／ｍｌ之间，随着浓度的增加，血小板聚集率逐渐增加，最佳浓度为１０ｎｇ／ｍｌ。当ＴＰＡ浓度〉１０ｍｇ／ｍｌ时，继续增加ＴＰＡ浓度，血小板聚集率将不再升高，且于２０ｍｇ／ｍｌ时出现明显的解聚现象。     

他汀类药物早期干预对急性冠脉综合征患者血脂、高敏C反应蛋白和纤维蛋白原的影响

目的 比较他汀类药物和阿司匹林联合用药与单用阿司匹林对急性冠脉综合征患者血脂、高敏C反应蛋白及纤维蛋白原的影响。方法 所有患者均在急性冠脉综合征发病后72h内开始接受药物治疗，他汀组(40例)应用他汀类药物加阿司匹林治疗8周，对照组(16例)单用阿司匹林治疗，观察两组总胆固醇、低密度脂蛋白胆固醇、高敏C反应蛋白和纤维蛋白原水平的变化。结果 治疗8周后，他汀组各指标显著降低，面对照组仅高敏C反应蛋白水平显著下降。他汀类药物降低高敏C反应蛋白、纤维蛋白原的程度与其降脂作用无关。结论 他汀类药物与阿司匹林联合用药降低高敏C反应蛋白和纤维蛋白原的作用可能优于单用阿司匹林，并与其抗炎作用有关。