Chronic diarrhoea as a dominating symptom in two children with cerebrotendinous xanthomatosis

The objective of this study was to describe diarrhoea as a dominating symptom of cerebrotendinous xanthomatosis (CTX), a lipid storage disease, and investigate its cause. Two children with chronic diarrhoea as the dominating symptom of CTX are presented. Before and after therapy with orally administered chenodeoxycholic acid (15 mg kg⁻¹ 24 h, in three divided doses) bile alcohol excretion in urine, serum cholestanol level, serum bile acid patterns and faecal bile acids were measured. All routine gastro-intestinal investigations before therapy were normal. Diarrhoea ceased immediately after starting treatment with chenodeoxycholic acid. Abnormal bile alcohol excretion in urine decreased rapidly during the first days and elevated serum cholestanol level normalized in 2 years. We postulate the presence of bile alcohols in the lumen of the gut as most likely cause for diarrhoea in CTX, since the rapid decrease of bile alcohol excretion is associated with prompt cessation of diarrhoea after starting treatment with chenodeoxycholic acid.     

Ascorbate-2-phosphate in red cell preservation. Clinical trials and active components

A red cell additive solution (AS-005) containing ascorbate-2-phosphate (AsP) to maintain 2,3-diphosphoglycerate, plus adenine, phosphate, and mannitol to retain viability and reduce hemolysis, was evaluated by human clinical trials. A crossover design was used with another additive solution (Nutricel AS-3, Cutter Laboratories) serving as the control for each donor. Each additive solution was evaluated at 35 and 42 days of storage. There was no significant difference between the red cell viability of the two storage solutions at either time period. Split-bag, AS-005 in vitro studies at two temperatures (2.5 and 5.5 degrees C), both within the range of 1 to 6 degrees C approved by the American Association of Blood Banks and the Food and Drug Administration, resulted in dramatically different in vitro parameters, including a threefold difference in 2,3-diphosphoglycerate (2,3-DPG), a fivefold difference in glucose, and significant differences in pH and adenosine triphosphate. High-pressure liquid chromatography data confirmed the preliminary report that 1 to 2 percent (wt/wt) oxalate was present in preparations of AsP. In vitro storage data confirmed that oxalate is the active component of AsP that preserves 2,3-DPG during storage.     

Magnetic resonance imaging of choroidal melanoma with and without gadolinium contrast enhancement

Choroidal melanoma is the most common intraocular tumor and is uniquely suited for evaluation by magnetic resonance imaging (MRI) because of the paramagnetic effect of the melanin molecule. The authors performed T1-, T2-, and proton-density-weighted MRI on 34 patients with choroidal melanoma. Nineteen patients received gadolinium contrast, T1-weighted images were superior in both detecting and delineating tumors, showing increased contrast-to-noise ratios over other images. Gadolinium contrast further increased this ratio. These images are presented as evidence that gadolinium-enhanced MRI is valuable in the evaluation of choroidal melanoma.     

Going Dutch in nocturnal enuresis

Based on several intervention programmes, a strategy for the treatment of nocturnal enuresis has recently been developed by an expert committee in the Netherlands. It consists of three parts. First, two structured interviews are given: one to differentiate between enuresis and incontinence and one to detect associated problems such as diurnal enuresis, constipation or behavioural problems. Secondly, a medical examination is made, confined to the inspection of the external genitalia and lower back, palpation of the abdomen and urine examination. Thirdly, the following guidelines for treatment at different age levels are applied: up to the age of 6 years no intervention is needed; between the ages of 6 and 8 years, lifting out of bed and/or the calendar method; between the ages of 8 and 12 years, enuresis alarm (if not successful, medication with desmopressin is prescribed for a restricted period of time), and ambulatory dry-bed training in a group setting may follow; over 13 years of age, clinical dry-bed training according to the Messer/Azrin method is advised. According to the expert committee, these guidelines offer sufficient possibilities to deal with the problem of nocturnal enuresis.     

Measurement systems in principle and in practice: the example of nursing workload

There has been a rapid development of measurement systems in the health services in the United Kingdom (UK) over recent years, not always matched by a thorough understanding of the phenomenon being measured and rarely based on any assessment of reliability or validity A particularly flagrant example of this process is the development of nursing workload measurement systems (NWMS) The estimates from four NWMS were examined They were substantially different from each other for no obvious reason, and the difference between any of the estimates and the actual nursing hours worked could not be explained in terms of any other aspect of the nursing process There is no evidence that the NWMS deployed in the UK are anything more than an expensive numbers game, without this kind of investigation of how they actually work in practice , it would be prudent to be wary about any of the measurement systems which have been proposed Yet many of the measurement systems used in other sectors of the health service are equally untested     

Morphological differences in cutaneous and skeletal muscle vasculature during compensatory and decompensatory hemorrhagic hypotension

Purpose: to determine if morphological differences exist between vasculature shown to decompensate, ie, skeletal muscle (SM) and vasculature that maintains the compensatory effort, ie, cutaneous (C). Methods: dogs were anesthetized and subjected to a Wiggers hemorrhagic shock protocol. SM and C flows were monitored using electromagnetic flowmeters. Gracilis (SM) and saphenous (C) arterial segments were removed during: prehemorrhage control, compensation, and decompensation. Segments were fixed at in vivo intraluminal pressures. Both light and EM sections were made from each sample. Results: During the compensatory phase, both SM and C showed signs of strong vasoconstriction. Even though the amount of endothelial folding was comparable, the C did and the SM did not sustain the vasoconstriction. Vascular diameters of the SM but not C increased during decompensation. Ultrastructural changes consisted of extreme folding of the internal elastic lamina (IEL) during compensation and subsequent flattening out of the IEL during decompensation in the SM but not the C tissues. Cell deformation, damage, and exfoliation were seen in both SM and C tissues. Conclusion: The data are consistent with the theory that SM vasculature, specifically endothelium, synthesizes an inhibitor of NE release, probably prostaglandins.     

The use of a radiolabelled saccharin solution to monitor the effect of the preservatives thiomersal, benzalkonium chloride and EDTA on human nasal clearance.

The effect of thiomersal, benzalkonium chloride and ethylenediaminetetraacetic acid (EDTA) on the nasal mucociliary clearance of healthy volunteers has been investigated using a modified saccharin test and gamma scintigraphy concomitantly. A significant correlation was found between the two techniques. Using each subject as his/her own control, none of the preservatives significantly altered the rate of clearance or proportion cleared from the nasal cavity after the administration of a single dose. This result is at variance with some in-vitro findings.