Cost of management of patients with haemophilia

The cost of managing 114 adult haemophiliacs in the west of Scotland was assessed for the period 1 March 1971 to 28 February 1974. Altogether 23 of them (20%) accounted for 80% of the resources used. The cost of hospital treatment of these patients during the period was compared with the predicted cost of home treatment, given the availability of freeze-dried factor VIII concentrate in sufficient amounts. We calculate that adequate on-demand home treatment would cost only 16% more than the present treatment, which is substantially less efficient.     

Nuclear distribution in Alternaria tenuis

Renal functional abnormalities constituting the syndrome of postobstructive diuresis imply both altered tubular and glomerular membrane properties. To determine the morphologic and ultrastructural correlates of this disorder a rat model was developed and 32 postobstructed kidneys were studied by light and electron microscopy at the midpoint of diuresis and compared to 22 controls. The abnormal morphology was: dilated distal tubules and collecting ducts, isolated proximal and distal tubule cells that allowed free access of luminal contents to the basement membrane, widened terminal bars and intercellular spaces, thickening of the glomerular basement membrane and, depending upon the portion of nephron, normal or reduced adenosine triphosphatase and acid phosphatase content. In order to confirm the functional nature of the nephrons studied as well as to assess glomerular and tubular permeability, horseradish peroxidase and cytochrome c were infused. These tracers, normally permeable to the glomerular basement membrane, were found in the intercellular spaces and to a lesser extent within cell organelles in the postobstructed diuretic animals whereas controls demonstrated a retarded filtration of horseradish peroxidase, no tracer in the intercellular spaces and large amounts of tracer contained within cell organelles. Absence of enzyme activity in the medulla and reduced dark to light cell ratios in the cortical collecting ducts correlated with prior observations made by others of diminished concentration and acidification processes, respectively. An increase in adenosine triphosphatase activity and renin granules within the juxtaglomerular cells indicated increased renin activity. These observations suggest that the renal functional abnormalities of postobstructive diuresis are attributable to altered glomerular and tubular permeabilities as well as with changes in metabolic activity.     

Overselectivity, developmental level, and overtraining in autistic and normal children

Thirteen autistic children were compared to 13 normal children matched to them in mental age, on performance of a visual discrimination task. Form, color, and size were relevant and redundant cues. The groups did not differ significantly in mean trials to reach criterion or in breadth of learning, and both groups increased their breadth of learning after 50 trials of overtraining. Form was preferred to color and size by both autistic and normal children. Within each group, rank on mental age was highly correlated with rank in breadth of learning. Verbal and nonverbal autistic children did not differ in breadth of learning or in dimensional preference. Even nonverbal autistics equaled the performance of their normal controls. Our results suggest that overselective attention is better understood as part of a general developmental lag in cognition in autistic children than as a specific deficit underlying psychotic behavior.     

The urinary ratio of 5-hydroxytryptophol to 5-hydroxyindole-3-acetic acid in surgical patients with chronic alcohol misuse

Interactions among growth factors are important in a variety of physiological and pathological processes. The regulation of IGF-I mRNA expression by bFGF was investigated in cultured rat Müller cells and the mechanism of regulation studied. Müller cells from 1- to 3-day-old Sprague-Dawley rats were isolated and cultured with Eagle MEM+10% FCS. Cultured cells were identified by immunocytochemistry using antibodies against vimentin, carbonic anhydrase C, and glutamine synthetase. Cells of passage 1-4 were treated with bFGF, the PKC inhibitor H-7, calphostin C, the PKC activator PMA or the PKA inhibitor H-89, as well as the adenylate cyclase activator forskolin, or adenylate cyclase inhibitor SQ22536. IGF-I and bFGF expression levels were assessed by Northern blot analysis. The addition of bFGF to culture medium down-regulated IGF-I expression in a dose- and time-dependent manner. Decrease of IGF-I expression started at a bFGF concentration of 1 ng ml-1. IGF-I mRNA level declined to 44% of baseline level at 10 ng ml-1 of bFGF, and reached a trough of 40% at 50 ng ml-1. At 10 ng ml-1 of bFGF, down-regulation of IGF-I expression was observed as early as 4 hr (60%) after treatment, and reached a trough of 42% by 8 hr. The temporal and concentration dependence of IGF-I expression by addition of the PKC activator PMA, to culture medium was similar to that due to the addition of bFGF. The down-regulation of IGF-I expression by bFGF (10 ng ml-1) and PMA (0.1 microM) was blocked by the PKC inhibitors H-7 (30 microM) and calphostin C (1 microM). Forskolin (5 microM), an adenylate cyclase activator, had activator, had no effect on IGF-I expression. SQ22536 (100 microM), an adenylate cyclase inhibitor, and H-89, a PKA inhibitor, had no inhibitory effect on bFGF-induced down-regulation of IGF-I expression. These results indicate that bFGF down-regulates IGF-I expression in cultured rat M uller cells through PKC activation.     

Isolation, structure elucidation, and biological activity of the steroid oligoglycosides and polyhydroxysteroids from the Antarctic starfish Acodontaster conspicuus

A total of 19 steroids, of which 13 steroidal oligoglycosides (nine new and four known) and six polyhydroxylated steroids (four new and two known), has been isolated from the Antarctic starfish Acodontaster conspicuus. The mixture is dominated by glycosides composed of steroidal aglycons having the hydroxyl groups typically disposed on one side of the tetracyclic nucleus, i.e., 3 beta,4 beta,6 alpha,8,15 beta-, with some having a sulfate at C-6, and differing in the side chains and/or in the disaccharide moieties that are usually attached at C-26, with some at C-28 and C-29. Those compounds are accompanied by minute amounts of glycosides with a delta 8(14)-double bond in the steroid, which is a structural feature not previously found among polyhydroxysteroids derived from starfish. Small amounts of six related unglycosidated polyhydroxysteroids and three higher-molecular-weight asterosaponins complete the composition of the mixture. The structures of the new compounds were determined by interpretation of their spectral data and by comparison with spectral data of known compounds. Eighteen of these compounds were evaluated for their ability to inhibit growth in Antarctic marine bacteria isolated from either the water column or the surfaces of benthic marine invertebrates. Of these compounds, 50% were active against at least one Antarctic marine bacterium. This suggests that these compounds may play an important role in deterring microbial fouling.