Prognostic factors for patients with chronic myeloid leukaemia in chronic phase treated with imatinib mesylate after failure of interferon alfa.

We assessed clinical results in 145 patients with chronic myeloid leukaemia in chronic phase who satisfied criteria for interferon-alpha failure and were thus eligible for treatment with imatinib at the Hammersmith Hospital. We used univariate and multivariate analyses to develop a risk score based on features defined after treatment for 3 months. We identified a low neutrophil count and poor cytogenetic response (<35% Ph-negative marrow metaphases) at 3 months as principal independent predictive factors and incorporated them into a three-tier prognostic scoring system for individual patients. For patients in the low-, intermediate- and high-risk groups, the probabilities of survival at 24 months were 100, 82 and 40% (P<0.0001) and progression-free survival 100, 66 and 15% (P<0.0001), respectively. This Hammersmith prognostic scoring system was validated with an independent cohort of patients treated at another UK centre.     

Non-compliance with lens care and maintenance in diabetic contact lens wearers

We hypothesize that diabetic contact lens wearers may represent a special group displaying higher levels of compliance with their lens care regimens as a result of learned behaviour relating to maintenance of their diabetic condition. To test this hypothesis, a prospective, single centre, controlled, masked study was performed whereby 29 diabetic contact lens patients and 29 non-diabetic control subjects were issued with disposable hydrogel contact lenses and a multipurpose lens care regimen. All participants were given identical instruction on lens care and maintenance. Compliance levels were assessed at a 12-month aftercare appointment by demonstration and questionnaire. Twenty-four different aspects of compliance were scored, 12 by observation and 12 by questionnaire report, of which only two showed a significant difference between the diabetic and control groups. Although the combined population of contact lens wearers was generally compliant, there were examples of non-compliance in both groups. Neither the duration of diabetes nor the degree of metabolic control appeared to have a significant effect on compliance. The results suggest that eye care practitioners cannot assume that diabetic patients will be more compliant with contact lens care and maintenance than non-diabetic patients.     

Quality of Care for Acute Myocardial Infarction in Rural and Urban US Hospitals

CONTEXT: Acute myocardial infarction (AMI) is a common and important cause of admission to US rural hospitals, as transport of patients with AMI to urban settings can result in unacceptable delays in care. PURPOSE: To examine the quality of care for patients with AMI in rural hospitals with differing degrees of remoteness from urban centers. METHODS: This cohort study used data from the Cooperative Cardiovascular Project (CCP), including 4,085 acute care hospitals (408 remote small rural, 893 small rural, 619 large rural, and 2,165 urban) with 135,759 direct admissions of Medicare beneficiaries ages 65 and older for a confirmed AMI between February 1994 and July 1995. Outcomes included use of aspirin, reperfusion, heparin, and intravenous nitroglycerin during hospitalization; use of beta-blockers, aspirin, and angiotensin-converting enzyme (ACE) inhibitors at discharge; avoidance of calcium channel blockers at discharge; and 30-day mortality. FINDINGS: Substantial proportions of Medicare beneficiaries in both urban and rural hospitals did not receive the recommended treatments for AMI. Medicare patients in rural hospitals were less likely than urban hospitals' patients to receive aspirin, intravenous nitroglycerin, heparin, and either thrombolytics or percutaneous transluminal coronary angioplasty. Only ACE inhibitors at discharge was used more for patients in rural hospitals than urban hospitals. Medicare patients in rural hospitals had higher adjusted 30-day post-AMI death rates from all causes than those in urban hospitals (odds ratio for large rural 1.14 [1.10 to 1.18], small rural 1.24 [1.20 to 1.29], remote small rural 1.32 [1.23 to 1.41]). CONCLUSIONS: Efforts are needed to help hospital medical staffs in both rural and urban areas develop systems to ensure that patients receive recommended treatments for AMI.     

Controversies in the management of children with bronchiolitis

In this review, we present and summarize data from recently conducted research regarding controversial aspects of the management of children with bronchiolitis. These data suggest that chronic medical history, gestational age at birth, postnatal age, and physiological variables can identify those children at higher risk for a more severe course of bronchiolitis. Large prospective studies also suggest that the likelihood of significant bacterial illness in febrile infants with bronchiolitis may be lower than in children without bronchiolitis. Nevertheless, urinary tract infections remain relatively common in young febrile children with bronchiolitis. Lastly and unfortunately, the data note a relative lack of effective therapies for children with bronchiolitis, although certain therapies such as systemic corticosteroids show potential efficacy and are in need of further study. The remaining uncertainty surrounding many issues pertaining to bronchiolitis highlight the need for more research aimed to: (1) develop prognostic models to identify patients at risk for a more severe clinical course, (2) develop generalizable diagnostic models to identify febrile infants with bronchiolitis at high and very low risk of significant bacterial illness, and (3) evaluate the effectiveness of promising therapies.     

A benign congenital myopathy in an inbred Samaritan family.

We describe a novel form of myopathy in a mother and her two daughters from an inbred Samaritan family. The patients displayed severe neonatal hypotonia, lethargy and dysmorphic features. Motor milestones were delayed; however, the hypotonia and muscle weakness gradually improved during the first 2 years of life and independent walking was achieved by 18 months. The mother at the age of 23 years shows myopathic facies and minimal proximal weakness. Her intelligence is normal. Her muscle biopsy revealed central nuclei and disruption of the intermyofibrillary network with moth eaten and spiral fibers. Mutations in SMN, MTM1 and the myotonic dystrophy genes were excluded. We suggest this is a new benign form of congenital myopathy. Inheritance is probably autosomal recessive.