Comparison of American and European practices in the management of patients with primary immunodeficiencies.

Primary immunodeficiency diseases (PIDs) comprise a heterogeneous group of rare disorders. This study was devised in order to compare management of these diseases in the northern hemisphere, given the variability of practice among clinicians in North America. The members of two international societies for clinical immunologists were asked about their management protocols in relation to their PID practice. An anonymous internet questionnaire, used previously for a survey of the American Academy of Allergy, Asthma and Immunology (AAAAI), was offered to all full members of the European Society for Immunodeficiency (ESID). The replies were analysed in three groups, according to the proportion of PID patients in the practice of each respondent; this resulted in two groups from North America and one from Europe. The 123 responses from ESID members (23·7%) were, in the majority, very similar to those of AAAAI respondents, with > 10% of their practice devoted to primary immunodeficiency. There were major differences between the responses of these two groups and those of the general AAAAI respondents whose clinical practice was composed of < 10% of PID patients. These differences included the routine use of intravenous immunoglobulin therapy (IVIg) for particular types of PIDs, initial levels of IVIg doses, dosing intervals, routine use of prophylactic antibiotics, perceptions of the usefulness of subcutaneous immunoglobulin therapy (SCIg) and of the risk to patients' health of policies adopted by health-care funders. Differences in practice were identified and are discussed in terms of methods of health-care provision, which suggest future studies for ensuring continuation of appropriate levels of immunoglobulin replacement therapies.

Enhanced integumental and ocular amelanosis following the termination of cyclosporine administration.

The Smyth delayed amelanotic line of chickens display symptoms commonly associated with human vitiligo. Administration of the immunosuppressive compound, cyclosporine, significantly delayed the mean age of onset and incidence of integumental pigment losses in this mutant line of vitiliginous chickens. Associated ocular pathology was also less severe in treated chicks. Termination of cyclosporine administration resulted in enhanced integumental and choroidal amelanosis, choroidal inflammation, and chorioretinal damage beyond that observed in nontreated controls. These results suggest that withdrawal of cyclosporine in treatment of this spontaneous autoimmune disease may exacerbate associated symptoms.

Effect of corticosterone on the incidence of amelanosis in Smyth delayed amelanotic line chickens.

The Smyth Delayed Amelanotic (SDA) line chicken exhibits a postnatal loss of pigment in feathers and choroid presumably due to an autoimmune reaction triggered by a basic pigment cell defect. The present study was designed to determine if the incidence and severity of amelanosis would be affected by administration of corticosterone (CS) in feed. The SDA line chickens were given 0, 20, 30, 40, and 50 ppm CS from 4 to 12 weeks of age. Body weight and incidence of amelanosis were determined weekly and immune response to sheep red blood cells was determined at the end of the trials. Body weight and immune response levels were significantly lower in treated groups than in controls (P less than .001), but no significant differences were found between treated groups on measures of amelanosis or immune response. Incidence of amelanosis in controls (60%) was significantly greater than in treated groups: 20 ppm, 14.2%; 30 ppm, 17.9%; 40 ppm, 12.9%; and 50 ppm, 16.7%. Six weeks posttreatment, treated groups were no longer significantly different from controls in terms of the incidence of amelanosis. It is proposed that the reduction in amelanosis during CS treatment is attributable to suppression of antibody response to the pigment cell antigen by the CS.

Effect of eumelanic phenotypes on the expression of amelanosis in the Smyth chicken.

The Smyth line is characterized by an autoimmune loss of melanin in the feather and eye in association with a hypermelanizing melanocyte, which presumably triggers immune system intervention. Inheritance appears to be multigenic. The present study was designed to determine if eumelanin-enhancing modifiers influence the incidence and severity of the line-associated amelanosis. Smyth chicks with dark brown (eb) down had a higher incidence of amelanosis (P less than .01) than did their hatchmates with light brown down. Furthermore, parents with dark down at hatch produced a higher incidence of amelanotic progeny than parents with light down. Reciprocal crosses of the Smyth line to a highly eumelanized (eb/eb) Recessive Black (RB) line produced F1 amelanosis. However, sires from the Smyth line produced significantly more amelanotics than did RB males (P less than .01). The influence of dark down on amelanotic development was also apparent in the Smyth-RBF1. The use of amelanotic F1 parents produced a significantly higher incidence of affected F2 offspring than did the use of unaffected parents. A backcross to the Smyth line produced an incidence of 67.6% amelanosis, whereas only one chick (2.04%) developed amelanosis from an F1 x RB mating. The finding that dark-downed Smyth chicks exhibit, and subsequently produce, a significantly higher incidence of amelanosis supports ultrastructural observations that associate the Smyth line amelanosis with a hyperactive melanocyte. The unusually high expression of amelanosis (22.7%) in the Smyth-RB F1 suggests that the two lines share one or more common eumelanogenic genetic factors.

The Cool Kids Coalition.

The Cool Kids Coalition was initiated as a community response to more than 214 hospitalizations of children under the age of five for burns over a 6-year period in one township in Long Island, NY. The coalition was started by public health nurses in partnership with the local chapter of the National Safe Kids Campaign. Goals included: 1. parent education regarding scald burn prevention; 2. development of innovative interventions for those at risk; and 3, development of innovative community approaches to scald prevention. Coalition members had diverse backgrounds and the coalition integrated non-traditional partners in injury control. The coalition doubled in size due to overwhelming community interest, growing within a few months from an initial group of 15 to a well-represented group of 30. Innovative programs were implemented that reached more than 3,000 parents, both in the community and home. Teaching was conducted with parents in the target population in Head Start centers, homeless shelters, the home, libraries, child care centers, a shelter for teen parents, etc. Member agencies incorporated the booklet and materials into their individual programs. The development of the Cool Kids Coalition illustrates the power of nursing in community health.