Ruvalcaba-Myhre-Smith Syndrome

In 1980 a syndrome was first described in two adult males, consisting of macrocephaly, pigmented macules on the glans and shaft of the penis, and hamartomatous intestinal polyps. Since then, 10 additional cases have been identified. Herein, we present two new cases and review the cutaneous manifestations as well as additional features in patients with the Ruvalcaba-Myhre-Smith syndrome.     

A survey of household food purchases and dietary habits in relation to affluence among Singapore Chinese

A survey of 40 Singapore Chinese households was performed, obtaining information on major perishable foods purchased for home consumption in the previous week, extent of home consumption (eating in), household dietary habits and housing type, the last as a measure of affluence. Eating out was very common: nine (23 per cent) households ate less than 50 per cent of meals at home. Excluding these nine households, consumption and habits were compared between more affluent and less affluent households. The more affluent households purchased more red meat/offal (P = 0.001), poultry (P = 0.002), vegetables other than green leafy (P = 0.04), fruit (P = 0.007) and eggs (P = 0.04); they also differed significantly in various dietary habits. Significant differences were also observed between English-speaking and Chinese-speaking households. The results suggest that increasing affluence may have brought about substantial dietary change in Singapore, thus meriting further study of the role of diet in changing disease patterns in Singapore.     

An External Aortic Root Device for Decreasing Aortic Regurgitation: In Vitro and In Vivo Animal Studies

Objectives: The purpose of this study was to determine if a device placed externally around the aortic root decreases regurgitant flow in acute aortic regurgitation. Background: Aortic regurgitant flow is dependent on central aortic pressure and the aortic root and leaflet geometry. It may be possible to decrease aortic regurgitant severity by reducing aortic root size or dimension changes. Methods: Aortic regurgitation was created in eight calf heart specimens suspended in a continuous flow system. Retrograde and antegrade aortic flow and distending aortic pressure were measured at baseline and after placement of an external aortic device at the level of the aortic annulus. In two additional specimens, the incompetent aortic valve was visualized fiberoptically before and after placement of the external device. Acute aortic regurgitation was created surgically in four live calves by excising a portion of the aortic leaflets. Antegrade and retrograde flow, left ventricular pressure, and central aortic pressure were measured at baseline, after creation of aortic regurgitation, and after placement of the external device. Results: In the in vitro calf specimens, regurgitant flow decreased from 46.9 cc/sec to 15.1 cc/sec (66.0%± 21.8% decrease) after placement of the external device (p < 0.001). The regurgitant orifice area decreased from 0.13 ± 0.04 cm² to 0.04 ± 0.02 cm² after device placement (p < 0.001). Antegrade flow was reduced to a smaller extent (20.0%± 19.2% decrease) by the device (p < 0.05). Placement of the device around the aorta resulted in improved coaptation of the leaflets with a marked reduction in defect size by endoscopic visualization. Use of the external aortic device was associated with improvement in aortic regurgitant severity in three of four calves with surgically created aortic regurgitation. Concluslons: In these preliminary studies, acute experimental aortic regurgitant severity is decreased by the use of an external aortic device, probably due to reduction in aortic annular dimension changes and improved aortic leaflet apposition. Further studies are needed to determine the effectiveness of this device in chronic aortic regurgitation. (J Card Surg 1994;9:304–313)     

Evaluation of loose bodies in the elbow with MR imaging

The evaluation of loose bodies in the elbow is usually done by means of clinical examination, radiography, and postarthrographic computed tomography (CT). The authors review their experience with magnetic resonance (MR) imaging in place of postarthrographic CT for the evaluation of loose bodies in the elbow. The prospective interpretation of MR studies of the elbow in 20 patients was compared with arthroscopic findings. All elbows were imaged in multiple planes with thin sections, surface coils, and combinations of T1, T2, and proton-density weighting. The sensitivity for showing loose bodies with MR imaging was 100%, and the specificity was 67%. Because this was a nonblinded study, the results are biased and caution must be used when extrapolating these results to the general population. In this limited experience, MR imaging has reliably shown loose bodies in the elbow, and in the authors' institutions has replaced postarthrographic CT for that purpose.     

Comparison of Simulated and in-Vivo Plasma Levels of Cilastatin Following Intravenous in-Line Drug Administration

The primary objective of this work was to establish a method to simulate the plasma levels of cilastatin, a model drug, following an intravenous in-line delivery scheme. In-vivo data in dogs obtained from this work were used to demonstrate the validity of the proposed approach. The in-line drug delivery system consists of a drug containing device which is placed between a large volume parenteral and a patient. Numerous advantages have been identified for this automatic in-line reconstitution delivery system. The numerical convolution integral algorithm was used in this work to perform plasma profile simulation. The results indicated that the simulated cilastatin plasma profile following in-line delivery closely agreed with the in-vivo data.     

A prospective study of tobacco use and multiple myeloma: evidence against an association

The relationship between the use of cigarettes and other tobacco products and the risk of multiple myeloma was examined in a cohort of nearly 250,000 American veterans followed prospectively for 26 years. Compared with men who had never used tobacco, the risk of death from myeloma was not increased among current (relative risk [RR]=0.9, 95 percent confidence interval [CI]=0.8–1.2) or former (RR=1.0, CI=0.8–1.3) cigarette smokers, nor among users of chewing tobacco or snuff (RR=1.0, CI=0.4–2.3). Risk was only slightly and nonsignificantly increased among pipe or cigar smokers (RR=1.2, CI=0.9–1.5). There was no indication of increasing risk with amount of tobacco used or earlier age at first use. With over 90 percent power to detect a 30 percent increased risk of this tumor occuring among current cigarette smokers, this study provides the strongest evidence to date against an association of cigarette smoking with multiple myeloma.Epidemiology and Biometry Program, Division of Cancer Etiology, National Cancer Institute. Westat, Inc. Rockville, MD. National Cancer Institute, 6130 Executive Blvd, Room 418, Rockville, MD 20892, USA.     

Effect of the 5-lipoxygenase inhibitor ZD2138 on allergen-induced early and late asthmatic responses.

BACKGROUND--Leukotrienes are lipid mediators generated from arachidonic acid by the 5-lipoxygenase pathway which may play an important part in the pathophysiology of asthma. Previous studies have demonstrated attenuation of the allergen-induced early and late asthmatic responses by leukotriene receptor antagonists. The effect of the 5-lipoxygenase inhibitor ZD2138, a non-redox lipoxygenase inhibitor which inhibits leukotriene synthesis for 24 hours after single doses of 350 mg, on allergen-induced early and late asthmatic responses has been assessed. METHODS--Eight asthmatic subjects with baseline FEV1 > 70% were studied. On screening, all subjects developed an allergen-induced biphasic asthmatic response to grass pollen, cat dander, or house dust mite. ZD2138 (350 mg) or placebo was given on two occasions separated by two weeks in a randomised double blind fashion. Allergen inhalation challenge was performed four hours after dosing and FEV1 was measured for eight hours. The inhibitory activity of ZD2138 on the 5-lipoxygenase pathway was assessed by measurements of calcium ionophore-stimulated generation of LTB4 in whole blood ex vivo and by analysis of urinary LTE4 levels before administration of drug or placebo and at regular intervals after oral drug dosing and allergen challenge. RESULTS--ZD2138 produced no significant bronchodilatation or attenuation of the early or late asthmatic response, although there was 82% inhibition of whole blood generation of LTB4 in response to calcium ionophore stimulation and 52% reduction in urinary excretion of LTE4. CONCLUSIONS--In asthmatic subjects the 5-lipoxygenase inhibitor ZD2138 did not protect against allergen-induced asthmatic responses, despite substantial inhibition of 5-lipoxygenase.