Perthes-like changes in geleophysic dysplasia

Geleophysic dysplasia is a rare skeletal dysplasia characterised by 'happy natured' facies, short stature with short limbs, brachydactyly, and joint contractures. This report describes a case of a patient with unilateral Legg-Calvé-Perthes-like changes associated with dysplastic proximal capital femoral epiphysis, typical to geleophysic dysplasia.     

Tetrahydrobiopterin protects against guanabenz-mediated inhibition of neuronal nitric-oxide synthase in vitro and in vivo.

It is established that guanabenz inhibits neuronal nitric-oxide (NO) synthase (nNOS) and causes the enhanced proteasomal degradation of nNOS in vivo. Although the time- and NADPH-dependent inhibition of nNOS has been reported in studies where guanabenz was incubated with crude cytosolic preparations of nNOS, the exact mechanism for inhibition is not known. Moreover, even less is known about how the inhibition of nNOS triggers its proteasomal degradation. In the current study, we show, with the use of purified nNOS, that guanabenz treatment leads to the oxidation of tetrahydrobiopterin and formation of a pterin-depleted nNOS, which is not able to form NO. With the use of 14C-labeled guanabenz, we were unable to detect any guanabenz metabolites or guanabenz-nNOS adducts, indicating that reactive intermediates of guanabenz probably do not play a role in the inhibition. Superoxide dismutase, however, prevents the guanabenz-mediated oxidation of tetrahydrobiopterin and inhibition of nNOS, suggesting the role of superoxide as an intermediate. Studies in rats show that administration of tetrahydrobiopterin prevents the inhibition and loss of penile nNOS due to guanabenz, indicating that the loss of tetrahydrobiopterin plays a major role in the effects of guanabenz in vivo. Our findings are consistent with the destabilization and enhanced degradation of nNOS found after tetrahydrobiopterin depletion. These studies suggest that drug-mediated destabilization and subsequent enhanced degradation of protein targets will likely be an important toxicological consideration.     

Survival enhancing indications for coronary artery bypass graft surgery in California

Background  

Coronary artery bypass graft (CABG) surgery is performed because of anticipated survival benefit, improvement in quality of life, or both. We performed this study to explore variations in clinical indications for CABG surgery among California hospitals and surgeons.     

Visual field loss following vitrectomy for stage 2 and 3 macular holes.

AIM--To describe the phenomenon of peripheral field loss following routine pars plana vitrectomy for stage 2 and 3 full thickness macular hole and to investigate the underlying mechanism. METHODS--Five patients, who reported peripheral field defects after apparently uncomplicated vitrectomy, posterior cortical vitreous peeling, and perfluoropropane (C3F8) gas tamponade, were studied retrospectively with slit-lamp biomicroscopy, automated and kinetic perimetry, fundal fluorescein angiography, focal electroretinography (ERG), and colour contrast sensitivity (CCS) testing. RESULTS--All five patients, who were between 50 and 73 years of age, reported an inferotemporal field defect following resolution of the intraocular gas bubble. In all eyes, the scotomata encroached to within 20 degrees to 30 degrees of fixation and to within 5 degrees to 15 degrees of the blind spot. In one eye, a partial altitudinal component was evident. All scotomata subsequently remained stable and three eyes developed subtle segmental nasal disc pallor and nerve fibre loss corresponding to the field defect. CCS testing revealed absent colour contrast in the scotomatous area, in the presence of a preserved focal quadrantic flash ERG, compared with normal CCS protan thresholds and focal ERGs in unaffected quadrants, indicating preserved outer retinal function in the area of the scotoma. CONCLUSIONS--These observations support the hypothesis that field defects occur as a result of retinal nerve fibre layer damage. It is proposed, on the basis of intraoperative observations and other evidence, that the most likely site of nerve fibre damage is at the nasal portion of the optic nerve rim or peripapillary retina, probably due to traction during cortical vitreous peeling.     

Constancy of relative body weight in children

The height and weight records of 404 schoolchildren at age 6 to 7 and at age 13 to 14 were analysed. Relative weight (i.e.% of ideal weight for height) of a child tended to change little over this period of 7 to 8 years of rapid growth. The correlation coefficient of relative weight for the group at the two ages was 0·81. In only 6% of the children did relative weight change more than 20% in either direction.This stability of relative body weight, despite the variations in environmental factors during school years, emphasizes the dominant role of genetic factors in the monitoring of body weight.     

Health care resource allocation and individuals' health care needs: examining the degree of fit

Previous studies examine associations between health system resources and an individual's use of health services, yet the importance of these resources in meeting an individual's health care needs is unclear. This paper examines the relationship between health care system characteristics, other social and economic characteristics of counties in a mid-western U.S. state and an individual's ability to meet health care needs over a 12-month period. We conclude that policy interventions are needed that look beyond health resource allocation to address problems in health care access that contribute to inequities in health.     

Personal narrative and an African-American perspective on medical ethics

In recent years, there has been increasing interest in how medical professionals develop and articulate a moral foundation on which to base a way of leading their professional lives. In this essay, however, the author focuses more narrowly on how black physicians do it. The author explains that black physicians confront a unique set of circumstances and experiences that define reality for black doctors and other professionals from non-dominant groups in the United States. From this particular background, black physicians go on to develop a unique perspective on medical ethics. The author uses his own narrative to demonstrate his argument and to show its application to a current debate on the ethics of forensic psychiatry practice.     

Behçet's disease and pregnancy

BACKGROUND: Beh?et's disease (BD) is a multisystem inflammatory chronic disorder, which is characterized by relapsing oral and genital ulceration and iridocyclitis. While being of unknown etiology, vasculitic changes of possible autoimmune origin are common to all involved organs, and thrombotic complications, which may adversely affect gestation, are frequently seen. Very little is known to date about the reciprocal influence of BD and pregnancy. We have undertaken to explore the mutual effect of BD and pregnancy with emphasis on maternal and fetal complications. METHODS: In this case-control study, we have evaluated pregnancies that occurred in women suffering from BD, who were treated in our institution during the last 25 years. All records were reviewed, and data were confirmed by a telephone interview and compared with a matched control group. To review the current knowledge, a Medline search together with a manual search of selected articles was performed. RESULTS: Thirty-one Beh?et's patients who had 135 pregnancies were studied. Remissions were significantly more frequent during both pregnancy and postpartum periods, while exacerbations were observed only in one-sixth of the patients (P < 0.001). Pregnancy complications (P < 0.001), cesarean section (P < 0.001), and miscarriage (P < 0.02) rates, however, were significantly higher in the study group. CONCLUSIONS: Our study suggests that pregnancy does not have a deleterious effect on the course of BD and may possibly ameliorate its course. However, it seems that BD may adversely affect pregnancy. The miscarriage rate was higher, and the pregnancy complications and cesarean section rates were significantly elevated.