Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase-deficient children receiving dapsone.

Drug-induced acute hemolytic anemia led to the discovery of G6PD deficiency. However, most clinical data are from isolated case reports. In 2 clinical trials of antimalarial preparations containing dapsone (4,4'-diaminodiphenylsulfone; 2.5 mg/kg once daily for 3 days), 95 G6PD-deficient hemizygous boys, 24 G6PD-deficient homozygous girls, and 200 girls heterozygous for G6PD deficiency received this agent. In the first 2 groups, there was a maximum decrease in hemoglobin averaging -2.64 g/dL (range -6.70 to +0.30 g/dL), which was significantly greater than for the comparator group receiving artemether-lumefantrine (adjusted difference -1.46 g/dL; 95% confidence interval -1.76, -1.15). Hemoglobin concentrations were decreased by ≥ 40% versus pretreatment in 24/119 (20.2%) of the G6PD-deficient children; 13/119 (10.9%) required blood transfusion. In the heterozygous girls, the mean maximum decrease in hemoglobin was -1.83 g/dL (range +0.90 to -5.20 g/dL); 1 in 200 (0.5%) required blood transfusion. All children eventually recovered. All the G6PD-deficient children had the G6PD A- variant, ie, mutations V68M and N126D. Drug-induced acute hemolytic anemia in G6PD A- subjects can be life-threatening, depending on the nature and dosage of the drug trigger. Therefore, contrary to current perception, in clinical terms the A- type of G6PD deficiency cannot be regarded as mild. This study is registered at http://www.clinicaltrials.gov as NCT00344006 and NCT00371735.

Side effects with inhaled corticosteroids: the physician's perception.

The National Asthma Education and Prevention Program 1997 guidelines and 2002 update provide an overview of potential local and systemic side effects associated with inhaled corticosteroids (ICS) and suggest ways of minimizing the risk of these side effects occurring. Despite the guidelines and extensive clinical experience of the safe use of ICS, a significant number of physicians retain concerns regarding side effects. Local side effects may lead to patients discontinuing therapy, with or without the knowledge of their physicians. In particular, concerns regarding systemic side effects, such as growth retardation in children and osteoporosis, remain relatively widespread. Pharmacokinetic studies reveal that different ICS compounds and formulations result in different degrees of systemic bioavailability, indicating possible differences in their potential to cause systemic side effects. However, clinical studies that can be used to differentiate between ICS formulations are generally lacking. Consequently, there is a need to continue to further our understanding of side effects with ICS, with the aim of identifying formulations, devices, and doses with an optimal risk/benefit ratio. The introduction of new agents with potentially improved safety profiles may reassure physicians and patients as to the relative benefits of ICS therapy in asthma.

Patient-focused care: using the right tools.

Patient-focused or patient-centered care is not a new concept, but its value has been overlooked in preference to the technology-based, disease-centered model that has prevailed in medicine for the last 50 years. Patient-focused care includes four broad areas of intervention: communication with patients, partnerships, health promotion, and physical care (medications and treatments). We can conceptualize patient-focused care as being the care we would like our loved ones to receive. There is considerable evidence that patients prefer a patient-focused approach. Unfortunately, there are also many studies detailing physicians' disconnection with patients' needs, particularly the need for information, and misunderstandings and assumptions based on poor communication. However, it is possible to develop physicians' skills in patient-focused care and provide physicians with the tools to overcome the barriers to this approach. The patient-focused approach has been shown to improve physicians' performance, patient satisfaction, and health outcomes without requiring additional investment in time or resources. Patient-focused care has also been shown to improve adherence to medication/advice, a well-known problem in asthma. There are also benefits to the physician in terms of improved outcomes for their patients, higher patient retention, and potentially a reduced risk of litigation. Patient-focused care may be a particularly valuable approach for the management of "difficult-to-treat" patients. In summary, the "three Cs" of patient-focused care-communication, continuity of care, and concordance (finding common ground)-are highly relevant to the effective treatment of pulmonary disease and should be a key component of asthma management.