Management of Nelarabine induced neurotoxicity in a child with T-cell acute lymphoblastic lymphoma.

INTRODUCTION: Nelarabine is now increasingly being used for the treatment of relapsed T-cell acute lymphoblastic leukemia/lymphoma, and about 18% of patients experience ≥ grade 3 toxicity. Despite the increasing use of this drug, there are no guidelines for managing its neurotoxicity. We would like to share our experience with one such case. CASE REPORT: A sixteen-year-old girl with T-lymphoblastic lymphoma received Nelarabine as part of her relapse treatment. Three weeks post-treatment, patient presented with worsening encephalopathy, bulbar palsy, and seizures. MANAGEMENT AND OUTCOME: After a detailed evaluation, Nelarabine neurotoxicity was strongly considered and was managed with a combination of steroids, intravenous immunoglobulin, and aminophylline, with almost complete recovery starting at 72 hours of treatment initiation. DISCUSSION: Despite the increasing use of this drug, guidelines for the management of the neurological adverse effects of Nelarabine are lacking. The above-mentioned combination of drugs worked for our patient, but larger numbers are needed to validate this as an approved treatment regimen.

Cartilage hair hypoplasia with cutaneous lymphomatoid granulomatosis.

Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity. We describe a young girl who was diagnosed with CHH based on the findings of recurrent infections, short stature with metaphyseal chondrodysplasia, and a confirmed bi-allelic RMRP gene mutation. At 13 years, the patient developed an Epstein-Barr virus (EBV)-driven lymphoproliferative disorder involving the lung, which responded partially to chemotherapy. Simultaneously, she developed multiple indurated plaques involving her face, which had histological findings of granulomatous inflammation and EBV-associated low-grade lymphomatoid granulomatosis. The patient received a matched unrelated peripheral blood stem cell transplant at 15 years of age, and her immunological parameters and skin lesions improved. Lymphomatoid forms of granulomatosis and cutaneous EBV-associated malignancies have not been described previously in CHH. This case highlights the possibility of EBV-associated cutaneous malignancy in CHH.

Troponin-I positivity in patients referred to Rapid Access Chest Pain Clinic.

BACKGROUND: Rapid Access Chest Pain Clinics (RACPCs) are set up to access patients with new onset chest pain (within the preceding three weeks), of possible cardiac origin. These patients are seen in the clinic within two weeks of referral and the attending physician takes a history, performs a routine clinical examination, and if clinically justified, a treadmill exercise test is performed according to Bruce Protocol. Within the group of patients referred to the RACPC with new onset but otherwise stable angina, there is a potential overlap with patients who in fact may have an evolving acute coronary syndrome, i.e., unstable angina. The aim of this study was to assess the prevalence of Troponin-I positivity as an indicator of acute coronary syndrome. METHODS: This cross-sectional descriptive study included 60 consecutive patients referred to the RACPC with history of recent onset chest pain (within the last three weeks) of possible cardiac origin and positive ETT or confirmed abnormal ischemic ECG at baseline. Troponin-L was measured in these patients. RESULTS: Out of the total 60 patients, 8.33% of the patients referred to RACPC with new onset angina had positive cTnI. CONCLUSION: Point of care test (POCT) for cTnI can help to identify the high risk patient referred to RACPC.

Disease progression and adverse events in patients listed for elective percutaneous coronary intervention.

OBJECTIVE: To record disease progression and the timing of adverse events in patients on a waiting list for elective percutaneous coronary intervention (PCI). DESIGN: Observational prospective study. SETTINGS: A UK tertiary cardiothoracic centre, at a time when waiting lists for PCI were up to 18 months. PATIENTS: 145 patients (116 men, median age 59.5 years) placed on an elective waiting list for PCI between October 1998 and September 1999. MAIN OUTCOME MEASURES: Adverse events recorded were death, myocardial infarction, need for urgent hospital admission because of unstable angina, and need for emergency revascularisation while waiting for PCI. RESULTS: During a median follow up of 10 months (range 1-18 months), nine (6.2%) patients experienced an adverse event. Eight (5.52%) patients were admitted with unstable angina as emergencies. One was admitted with a myocardial infarction. Twenty nine (20.0%) patients had significant disease progression at the time of the repeat angiogram before PCI. In 10 (7%), disease had progressed so that PCI was no longer feasible and patients were referred for coronary artery bypass graft. Sixteen (11%) were removed from the PCI waiting list because of almost complete resolution of their anginal symptoms. CONCLUSION: Adverse coronary events and clinically significant disease progression occur commonly in patients waiting for PCI. Despite the presence of severe coronary lesions, myocardial infarction was rare and no patients died while on the waiting list. Resolution of anginal symptoms was also comparatively common. The pathophysiology of disease progression frequently necessitates a change in the treatment of patients waiting for PCI.

Successful HSCT using nonradiotherapy-based conditioning regimens and alternative donors in patients with Fanconi anaemia--experience in a single UK centre.

Seven children with Fanconi anaemia (FA) (five female, two male), who had not undergone transformation, received nine haemopoietic stem cell transplantation (HSCT) between 2000 and 2004. Conditioning regimen was: fludarabine 25-30 mg/m2/day for 5 days, antilymphocyte globulin 12.5 mg/kg/day for 3 days and cyclophosphamide 5-7.5 mg/kg/day for 4 days. Radiation was not used. One male patient who had multiple HSCT and one female who was retransplanted, received slightly different conditioning regimens. Four patients received fully matched unrelated umbilical cord blood (UCB), two matched unrelated peripheral blood stem cell (PBSC) grafts, and three haploidentical T-cell-depleted (TCD) PBSC grafts. None of the patients had any significant conditioning-related toxicity or severe infections. All engrafted within 2-3 weeks. One patient rejected her first HSCT after 10 weeks and had a second successful transplant from the same donor. One male patient rejected his TCD haploidentical HSCT from his mother, and subsequently had a successful fully matched unrelated UCB transplant. Rejection rate was 22%. Acute and chronic graft-versus-host disease (GVHD) was seen in 77 and 22% patients. In all, 57% patients developed autoimmune complications, all of which have resolved. All patients are well with stable or full donor chimerism after a median follow-up of 37 months (range 13-54).

Mitral valve endocarditis in hypertrophic cardiomyopathy: case report and literature review.

Mitral endocarditis complicating hypertrophic cardiomyopathy occurs predominantly on the left ventricular aspect of the anterior mitral valve leaflet in the presence of outflow tract obstruction. It is a rare condition and the estimated cumulative 10 year probability of developing endocarditis in patients with obstruction is < 5%. Combined mitral valve replacement and septal myectomy has been reported in this setting. A case of community acquired Staphylococcus aureus mitral valve endocarditis is reported in a previously asymptomatic young man with hypertrophic obstructive cardiomyopathy. The potential treatment options are discussed.

Effectiveness of rotational atherectomy of right coronary artery ostial stenosis.

Balloon angioplasty and stenting of right coronary ostial stenosis may frequently be impeded by lesion calcification, whereas rotational atherectomy, which ablates calcified plaque, should treat these lesions effectively. Accordingly, we evaluated procedural success and longterm clinical outcome of rotational atherectomy of right coronary ostial stenosis. Procedural data were obtained from a comprehensive interventional registry and follow-up information was obtained by chart review and patient enquiry. All patients who developed recurrent angina underwent angiographic restudy. During a 5-year interval, 119 patients underwent rotational atherectomy of right coronary ostial stenosis. Multilesion interventions were performed in 55% of patients. Ostial lesions were 3.73+/-3.69 mm in length (mean +/- SD), and 57.1% were significantly calcified. Reference vessel diameter was 3.42+/-0.56 mm. Maximum burr:artery ratio was 0.64+/-0.1 with adjunct balloon angioplasty in 89.1% and adjunct stenting in 9.2%. Procedural success (<50% residual stenosis without major complication) was 97.5%, with 1.7% uncomplicated failure and 0.8% Q-wave infarction. Maximum residual stenosis was 15+/-17%. During 6-month follow-up, available in 94% of patients, 82.7% remained angina-free, 10.9% developed recurrent angina due to right coronary ostial restenosis, and 6.4% developed recurrent angina due to another lesion. Two years after intervention, target lesion revascularization rate was 16%. Predictors of symptomatic angiographic restenosis were dissection >10 mm, final minimal luminal diameter <2.5 mm, lesion length >10 mm, restenotic lesion, and diabetes. We conclude that rotational atherectomy of right coronary ostial stenosis results in excellent acute procedural success and in low incidence of clinical recurrence, with a high proportion of patients remaining angina-free 2 years after intervention.

Striving toward continuous quality improvement: a case study of Saint Mary's Hospital.

This case analysis is the result of a year-long study designed to identify and assess the ingredients that led to the successful implementation of a continuous quality improvement (CQI) program at Saint Mary's Hospital in Grand Rapids, Michigan. The key ingredients of success included: (1) an organizational structure and leadership commitment for identifying and improving processes, (2) use of data-based statistical and analytical tools to study processes, (3) empowerment of teams of employees to take charge of the operations of their own work tasks in a manner that encourages continuous learning, (4) involvement of internal and external customers through the improvement process, and (5) development of effective measures for monitoring improvement. The benefits of the CQI efforts at Saint Mary's have been remarkable and hospital-wide.

Continuous quality improvement, total quality management, and reengineering: one hospital's continuous quality improvement journey.

In recent years, there has been significantly increasing interest in the application of continuous quality improvement (CQI) and total quality management (TQM) in the health care arena. This case analysis is designed to identify and assess the strategies and processes that led to the successful implementation of CQI in the Emergency Care Center at St. Mary's Hospital in Grand Rapids, MI.

Aortocoronary saphenous vein graft disease: pathogenesis, predisposition, and prevention.

Aortocoronary saphenous vein graft disease, with its increasing clinical sequelae, presents an important and unresolved dilemma in cardiological practice. During the 1st month after bypass surgery, vein graft attrition results from thrombotic occlusion, while later the dominant process is atherosclerotic obstruction occurring on a foundation of neointimal hyperplasia. Although the risk factors predisposing to vein graft atherosclerosis are broadly similar to those recognized for native coronary disease, the pathogenic effects of these risk factors are amplified by inherent deficiencies of the vein as a conduit when transposed into the coronary arterial circulation. A multifaceted strategy aimed at prevention of vein graft disease is emerging, elements of which include: continued improvements in surgical technique; more effective antiplatelet drugs; increasingly intensive risk factor modification, in particular early and aggressive lipid-lowering drug therapy; and a number of evolving therapies, such as gene transfer and nitric oxide donor administration, which target vein graft disease at an early and fundamental level. At present, a key measure is to circumvent the problem of vein graft disease by preferential selection of arterial conduits, in particular the internal mammary arteries, for coronary bypass surgery whenever possible.

Validity of background data as a predictor of employee tenure among nursing aides in long-term care facilities.

Employee turnover and its associated costs continue to be an important concern for American industry. The purpose of this study was to determine whether differences exist in personal history data (biodata) of nurses aides who remained with the target nursing home corporation and those who either voluntarily or involuntarily separated employment. Results indicate that significant differences exist between the groups with respect to several important dimensions of personal history data.

Reducing nurse aide turnover through the use of weighted applications blank procedure.

High turnover rates among hospital nurses demand rigorous and valid processes of research to determine the reasons motivating such attrition. This study was designed to determine whether use of a weighted application blank has the potential to improve the selection of employees in the nursing home industry. The use of weighted application blanks is, in essence, a proactive approach to reducing turnover. The initial results exhibit great potential to reduce high levels of turnover by reducing the number of poor candidates selected for employment.

Implementing TQM in the health care sector.

This article examines the issue of implementing TQM/CQI programs in the health care industry by grouping the prescriptive literature into four research streams. Based on the literature, a strategic programming model for implementing TQM/CQI in the health care industry is suggested. Finally, issues relating to TQM in the health care sector, which need to be addressed within each research stream in the future, are provided.

Implementation of ISO 9000 in the healthcare sector: a case study.

In order to compete at the international level, companies are beginning to recognize ISO 9000 registration as a virtual necessity. As a result, the number of ISO certifications issued in the U.S. and Canada tripled between 1992 and 1994. By means of a case study, this paper describes how a large healthcare manufacturing organization recently achieved its ISO 9001 certification. Specifically, the strategies and the process used by the organization in obtaining the registration is explained.

Natriuretic response to neutral endopeptidase inhibition is blunted by enalapril in healthy men.

We studied six healthy male subjects in a randomized, placebo-controlled, single-blind fashion to determine the comparative effects on renal hemodynamics and natriuresis of the angiotensin-converting enzyme inhibitor enalapril (5 mg on each of 5 days preceding the study), the neutral endopeptidase inhibitor candoxatrilat (200 mg IV), and the combination of enalapril and candoxatrilat. Enalapril pretreatment alone, compared with placebo, produced slight nonsignificant increments in absolute and fractional sodium excretions and a marked increase in effective renal plasma flow but no change in glomerular filtration rate. Candoxatrilat alone produced marked augmentation of both absolute and fractional sodium excretions. The candoxatrilat-mediated increment in absolute sodium excretion was significantly correlated with increases in urinary cGMP and plasma atrial natriuretic peptide in response to this drug, but neither effective renal plasma flow nor glomerular filtration rate was altered compared with placebo. Combining enalapril pretreatment with candoxatrilat significantly attenuated the increments in absolute and fractional sodium excretions in response to the neutral endopeptidase inhibitor. Blood pressure was reduced by enalapril alone compared with placebo, whereas candoxatrilat treatment alone led to a marginal but significant enhancement of blood pressure. The combination of enalapril and candoxatrilat abolished any significant blood pressure change compared with placebo. Thus, candoxatrilat-mediated natriuresis occurs via a renal tubular rather than glomerular mechanism and is blunted by enalapril. This attenuation by enalapril may occur by interference with angiotensin II-dependent effects on the renal tubule or on systemic blood pressure.

Managing diversity in the health care industry: a conceptual model and an empirical investigation.

Between 1985 and 2000, 8 out of every 10 new entrants to the work force will be minorities, immigrants, or women. If the U.S. health care industry has to retain a competitive edge, it will have to effectively attract and manage the diverse talent that will characterize its new work force. This article analyzes the authors' cultural awareness survey results and suggests a strategic model for managing diversity in the health care industry.

Ethical issues in purchasing: a field study of Midwest hospitals.

A large sum of money is spent annually by salespeople on gifts and favors for purchasing executives. The provision of gifts and favors to buyers remains a common practice despite the fact that it often leads to ethical conflicts for purchasing executives, sales managers, and salespeople. This paper investigates the perceptions of 51 purchasing executives of midwest hospitals regarding their behavior towards certain buying practices, the favors offered by vendors, favors actually accepted, as well as purchasers' discomfort and repayment levels regarding indebtedness. Based on the data analysis, this paper provides conclusions and directions for future research.

The need for implementing TQM in the health care industry: an empirical investigation.

The U.S. health care industry is facing an era of increased competition, declining profits, decreed hospital utilization, and consolidation. By reviewing existing literature on the application of total quality management in the health care industry, and by surveying a hospital in which this technique has been recently applied, this paper shows that service providing organization can indeed use the same methods used by manufacturing organizations to increase quality.