Pregnancy among patients with systemic lupus erythematosus receiving immunosuppressive therapy

We evaluated retrospectively the effect of superimposed pregnancy upon the course of systemic lupus erythematosus (SLE) in patients receiving glucocorticoids or azathioprine at conception. About equal numbers of flares (10 vs 12) occurred among 22 pregnant patients with lupus and 22 nonpregnant control patients with lupus matched by disease duration and prior organ system involvement. Pregnancy per se did not appear to accelerate SLE in patients receiving immunosuppressive therapy.     

Calciphylaxis – a topical overview

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.     

Reliability analysis of the Motivation Assessment Scale: a failure to replicate.

The Motivation Assessment Scale (MAS) has been proposed as an efficient questionnaire for identifying the source of reinforcement for an individual's self-injurious behavior (SIB). A previous reliability analysis of the MAS (Durand & Crimmins, 1988) reported interrater correlation coefficients ranging from .66 to .92, based on a comparison of responses provided by classroom teachers. In this study, the reliability of the MAS was reexamined with two independent groups of developmentally disabled individuals who exhibited SIB (N = 55). For the institutional sample (n = 39), the MAS was given to two staff members (a supervisor and therapy aide) who work with the individual daily. For the school sample (n = 16), the MAS was given to the teacher and teacher's aide who taught the student. The correlational analyses completed by Durand and Crimmins (1988) were repeated; in addition, a more precise analysis of interrater reliability was calculated based on the actual number of scoring agreements between the two raters. Results showed that only 16 of the 55 raters agreed on the category of reinforcement maintaining their client's or student's SIB, that only 15% of the correlation coefficients obtained were above .80, and that none of the reliability scores based on percent agreement between raters was above 80%.     

Determinants of enrollment among applicants to PACE.

During the 1970s and 1980s, a new approach to the integration of acute and long-term care (LTC) services was conceived and refined at On Lok, an organization in the Chinese community of San Francisco. Since then, On Lok and 10 Federal demonstration sites have tested this model which is today called the Program of All-Inclusive Care for the Elderly (PACE). This program has gained considerable political support and as a result, the 1997 Balanced Budget Agreement establishes PACE as a permanent provider under Medicare. The Federal demonstration of PACE was designed as a voluntary program. By exploiting its voluntary enrollment design, this study analyzes the determinants of program participation within a group of screened applicants. Findings of this study support the theory that the capitated payment structure of PACE creates incentives for program staff to avoid costly individuals. However, home ownership and provider attachment also act as important and significant barriers to enrollment.     

Mutations in the retinal guanylate cyclase (RETGC-1) gene in dominant cone-rod dystrophy

The dominant cone-rod dystrophy gene CORD6 has previously been mapped to within an 8 cM interval on chromosome 17p12-p13. The retinal- specific guanylate cyclase gene (RETGC-1), which maps to within this genetic interval and previously was implicated in Leber's congenital amaurosis, was screened for mutations within this family and in a panel of small families and individuals with various cone and cone- rod dystrophy phenotypes. A missense mutation (E837D) was identified in affected members of the CORD6 family, as well as a second missense mutation (R838C) in three other families with dominant cone-rod dystrophy. RETGC-1 is only the fourth gene to be implicated in cone-rod dystrophy and this is the first report of dominant mutations in this gene.      

Comparison between testosterone oenanthate-induced azoospermia and oligozoospermia in a male contraceptive study. IV. Suppression of endogenous testicular and adrenal androgens

Administration of supraphysiological doses of testosterone to normal men causes inhibition of spermatogenesis, but while most become azoospermic, 30-55% maintain a low rate of spermatogenesis. We have investigated whether there are differences in endogenous androgen production, of testicular and adrenal origin, which may be related to the degree of suppression of spermatogenesis. Thirty-three healthy Caucasian men were given weekly i.m. injections of 200 mg testosterone oenanthate (TE), 18 became azoospermic, while 15 remained oligozoospermic. Urinary excretion of epitestosterone, a specific testicular product, was reduced to <10% of pretreatment values, with no differences between the groups. Similar results were obtained for other markers of testicular steroidogenesis. Urinary and plasma adrenal androgens were also reduced during TE treatment: a statistically significant decrease in both (P < 0.001 and P < 0.05 respectively) was seen in the azoospermic but not oligozoospermic responders. These results suggest that testicular steroidogenesis is decreased to <10% by the administration of supraphysiological doses of exogenous testosterone. Differences in the degree of ongoing steroidogenesis in the testis do not appear to account for incomplete suppression of spermatogenesis, thus differences in androgen metabolism may underlie this heterogeneous response. A small but significant reduction in secretion of adrenal androgens was also detectable, the relevance of which is unclear.      

A locus for autosomal dominant anterior polar cataract on chromosome 17p

Inherited cataract is a clinically and genetically heterogeneous disease. Here we report the identification of a new locus for an autosomal dominant anterior polar cataract on the short arm of chromosome 17. To map this new locus we performed genetic linkage analysis with microsatellite markers in a four-generation pedigree. After exclusion of seven candidate loci for cataract, we obtained significant positive LOD scores for markers D17S849 (Z = 4.01 / theta = 0.05) and D17S796 (Z = 4.17 / theta = 0.05). Multipoint analysis gave a maximum LOD score of 5.2 (theta max = 0.06) between these two markers. From haplotype analysis, the cataract locus lies in the 13 cM interval between markers D17S849 and D17S796. This study provides the first genetic mapping of an autosomal dominant anterior polar cataract.      

Application of Evidence-Based Treatment in Community Mental Health Settings: Examining EBT Delivery Duration and Client Discharge

The Journal of Behavioral Health Services & Research - Characterizing community mental health (CMH) treatment duration and discharge is an important step toward understanding how to better meet...