Macrophage activation syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. This review summarizes the characteristics of patients with J-SLE-associated MAS reported in the literature or seen by the authors and analyses the distinctive clinical, diagnostic and therapeutic issues that the occurrence of MAS may raise in patients with J-SLE.

Functional ability and physical and psychosocial well-being of hypermobile schoolchildren.

OBJECTIVE: To compare the functional ability and the physical and psychosocial well-being of children with joint hypermobility to those of age- and sex-matched non-hypermobile subjects. METHODS: 311 healthy Italian schoolchildren aged 6.3 to 19.3 years were examined for hypermobility of the joints. Functional ability was assessed through the Childhood Health Assessment Questionnaire (CHAQ) and the physical and psychosocial well-being through the Childhood Health Questionnaire (CHQ). The parent's assessment of the child's overall well-being and of the child's pain was measured on a visual analogue scale. RESULTS: The overall prevalence of articular hypermobility was 34% (106/311), with the median hypermobility score being 3 (interquartile range 1, 5). Although the hypermobility score of girls (median 3: interquartile range 2, 5) exceeded that of boys (median 2.5; interquartile range 0, 5), this difference was not statistically significant (p = 0.16). The level of hypermobile children's pain in the preceding weeks, as assessed by the parents, was comparable to that recorded in the non-hypermobile peers. There was a weak negative correlation between the hypermobility score and the age of the child (r = -0.14, p=0.01). All instrument scores were comparable between hypermobile and non-hypermobile subjects, with the sole exception of a borderline significant greater impairment of the Role/social limitations-physical subscale of the CHQ in the hypermobile group. The hypermobility score was not correlated with any instrument score. CONCLUSIONS: The presence of joint hypermobility does not affect the functional ability and the physical and psychosocial well being of otherwise healthy children. These results suggest that the physical functioning in everyday life and the general health status of hypermobile children are not impaired.

Comparison of survivorship between standard and long-stem souter-strathclyde total elbow arthroplasty.

This study compared 107 cases of rheumatoid arthritis that had the long-stem humeral component of the Souter-Strathclyde total elbow arthroplasty introduced as a primary procedure with a similar group of 202 cases that had the standard component. Both groups were comparable with regard to age range and sex ratio. Follow-up of the longer-stem component was somewhat shorter, being a mean of 4.4 years as opposed to 9.3 years. Kaplan-Meier survival analyses were undertaken for both revision and radiologic loosening of components as terminal events. There was no significant difference between the cumulative survival of the standard and long-stem humeral implants when revision was the endpoint. Although survival of both implants was similar, the reasons for this were different. More specifically, for the standard humeral component, 25 out of 32 revisions were for humeral loosening. For the long stem, however, 5 out of a total of 7 revisions were due to instability; no long-stem humeral component had been revised for loosening. In addition, linking the components by way of a snap-fit component was associated with a higher rate of loosening; more specifically, 5 out of 16 cases were revised. As a result of this study, we recommend the use of a long-stem Souter-Strathclyde humeral component as a primary implant. Other crucial components for long-stem survival, however, are surgical technique and alignment of the implant.

Reproductive health beliefs and behaviors in teens with diabetes: application of the Expanded Health Belief Model.

OBJECTIVE: To identify significant correlates among constructs of the Expanded Health Belief Model (EHBM) with reproductive health behaviors [preventing an unplanned pregnancy and seeking preconception counseling (PC)] and metabolic control in teenaged women with type 1 diabetes. RESEARCH DESIGN AND METHODS: Eighty adolescent women with type 1 diabetes participated in a multisite, exploratory, case-control study. Subjects (only cases, and not controls, were used for the analyses of this paper) had a single, 1-h structured phone interview with a trained, same gender research assistant. Variables of interest were awareness, knowledge, health beliefs, attitudes, intention and behaviors regarding diabetes and reproductive health. RESULTS: Several major constructs of the EHBM were significantly correlated in the expected direction with reproductive health behaviors and metabolic control of adolescents with type 1 diabetes. In particular, perceived susceptibility, barriers, threat, intention, and self-efficacy with birth control (BC) use, and motivational cue (initial awareness of PC) with seeking PC. CONCLUSIONS: Being told by a health care professional to seek out PC is a motivational cue that triggers action. The timing of this message would seem to play an important role in preventing an unplanned pregnancy. Therefore, interventions focusing on changing health beliefs and on increasing awareness may be effective in promoting positive reproductive health behaviors in adolescent females with diabetes.

Guidelines for developing a nurse practitioner practice.

Nurse practitioners must take an active role in defining and establishing their practices. In many cases, medical and administrative staffs develop the NP job description. As a result, many NPs are finding themselves in unsatisfactory, often task-oriented roles. This problem is largely due to NPs' inexperience in establishing practices and a lack of documentation of current practices. When developing the NP practice within the National Cancer Institute-Medicine Branch, it was apparent that the nursing literature offered no information on how to establish an NP practice. This article presents guidelines for developing an NP practice and is based on information obtained during the developmental stages of the nurse practitioner practice at the National Cancer Institute-Medicine Branch.