Neuropsychological assessment, neuroimaging, and neuropsychiatric evaluation in pediatric and adult patients with sickle cell disease (SCD)

Traditionally, neuropsychological deficits due to Sickle Cell Disease (SCD) have been understudied in adults. We have begun to suspect, however, that symptomatic and asymptomatic Cerebrovascular Events (CVE) may account for an alarming number of deficits in this population. In the current brief review, we critically evaluated the pediatric and adult literatures on the neurocognitive effects of SCD. We highlighted the studies that have been published on this topic and posit that early detection of CVE via neurocognitive testing, neuropsychiatric evaluations, and neuroimaging may significantly reduce adult cognitive and functional morbidities.     

A light-hearted look at a lion-hearted organ (or, a perspective from three standard deviations beyond the norm) Part 2 (of 2 parts)

Throughout history, the heart has been associated not only with its life-sustaining function but also with its close ties to the human emotions. In this literature and internet review, we attempt to gather and organize information from both of these perspectives as they relate to the heart in the following 11 categories: (1) fun facts, (2) medical photography, (3) history, (4) languages (etymology), (5) nonmedical English expressions, (6) death, (7) the arts, (8) movie titles, (9) song titles, (10) Shakespeare, and (11) the Bible. Part 1 (previously published) covered the first five topics, and Part 2 will cover the last six topics. These data may be useful to those who are engaged in teaching about the cardiovascular system.     

The attachments of the anteromedial and posterolateral fibre bundles of the anterior cruciate ligament

The tibial attachments of the individual anteromedial (AM) and posterolateral (PL) fibre bundles and the entire attachment of the anterior cruciate ligament are described, relating them to consistent bony landmarks; 55 fresh-frozen specimens were measured. The fibre bundles were separated and excised at their attachments and their peripheries marked with a pen. High-resolution scaled digital photographs were taken of each dissected specimen and transferred onto a computer for analysis. A wide variation was found when using the posterior tibial axis, the anterior tibial surface and the medial tibial spine as reference points. The most consistent measurements used the tibial interspinous “over-the-back” ridge as a datum. The attachments of the PL and AM bundles were centred 10 ± 1 mm (mean ± SD) and 17 ± 2 mm anterior to the over-the-back ridge. They were 4 ± 1 and 5 ± 1 mm, respectively, lateral to the medial tibial spine border. The positions of 6 mm circles in the posterior-medial limits of the fibre bundles (representing tunnels in a double-tunnel reconstruction) were measured. The overall dimensions of the tibial plateaux correlated significantly with many measurements. The results from this study could be used to guide ACL reconstruction techniques.     

Apoptosis in perinatal hypoxic-ischaemic cerebral damage

Perinatal hypoxia-ischaemia induces a biphasic cerebral injury: the depletion in high energy phosphates during the insult returns to normal soon after resuscitation. However, some 8–15 h later a second phase of impaired energy metabolism begins, which is related to the severity of later neurodevelopmental impairment. Delayed injury differs from acute hypoxia-ischaemia because intracellular acidosis does not occur. Apoptosis may be a mechanism of delayed cellular injury. Apoptotic cells and typical DNA fragmentation have been found after perinatal hypoxia-ischaemia. In newborn piglets, fraction of apoptotic cells was directly related to the degree of high energy phosphate depletion during hypoxia-ischaemia. Apoptosis may be interrupted: in piglets, brain cooling for 12 h following resuscitation reduced the fraction of apoptotic but not necrotic cells. These results have implications for both the understanding of cerebral injury and the use of hypothermia as a neural rescue strategy in the developing brain.     

Do viscous polysaccharides slow absorption by inhibiting diffusion or convection?

Viscous polysaccharides such as guar gum delay absorption probably by impairing the access of luminal contents to the absorptive epithelium. Measurements of the electrical resistivity of saline solutions were carried out to determine if guar gum (0.5, 0.75 and 1 per cent w/v), in concentrations which impaired absorption in vitro, delayed diffusion of solutes or inhibited convection of luminal contents. Our results indicated that guar had no effect on the mobility of ions in a completely unstirred solution but delayed the time taken for saline solutions of different resistivity to achieve complete mixing, when they were brought into contact inside a vessel, which was rotated at a constant speed. The time taken to achieve complete mixing was directly related to the viscosity of the guar solutions. The effect of intestinal contraction on the absorption of glucose was simulated in an in vitro model, consisting of a length of dialysis tubing, alternately contracted at each end. Movement of glucose out of the dialysis tubing was increased by increasing the rate of contraction. The incorporation of guar gum into the solution prevented the rise in glucose uptake produced by increasing the rate of contraction. These effects suggest that guar probably reduces absorption by resisting the convective effects of intestinal contractions.     

Evolution of white matter lesions in neurofibromatosis type 1: MR findings.

To characterize further the evolution of white matter lesions in neurofibromatosis type 1, we reviewed 68 MR images in 43 patients (age, 1-31 years), including 25 follow-up studies (mean interval, 27 months). Lesion number, location, morphology, signal characteristics, and contrast enhancement were assessed. Lesion characteristics and changes thereof were correlated with the patients' ages. Thirty-four patients (79%) had white matter lesions. These lesions were hyperintense on T2-weighted images, were isointense on T1-weighted images, and showed no mass effect or contrast enhancement in 31 patients; in three patients, T1-prolongation was observed (one with significant mass effect). None of the lesions evolved into a glioma. The most common locations were the cerebellum (49%), brainstem (22%), and internal capsule (19%). Nineteen patients had white matter lesions and follow-up studies. Lesions decreased in size or number in seven patients (average age, 13 years), showed no change in three (average age, 12 years), increased in size or number in four (average age, 5 years), and showed a mixed pattern (increased/decreased size/number) in four (average age, 7 years). White matter lesions in neurofibromatosis type 1 frequently increase in size or number early in childhood; this did not indicate neoplasia in our study. The lesions tend to resolve with increasing age. Lesion progression in a child more than 10 years old warrants close follow-up to rule out a neoplasm.     

In vitro stability of a novel compliant poly(carbonate-urea)urethane to oxidative and hydrolytic stress.

Poly(ester)urethane and poly(ether)urethane vascular grafts fail in vivo because of hydrolytic and oxidative degradative mechanisms. Studies have shown that poly(carbonate)urethanes have enhanced resistance. There is still a need for a viable, nonrigid, small-diameter, synthetic vascular graft. In this study, we sought to confirm this by exposing a novel formulation of compliant poly(carbonate-urea)urethane (CPU) manufactured by an innovative process, resulting in a stress-free. Small-diameter prosthesis, and a conventional poly(ether)urethane Pulse-Tec graft known to readily undergo oxidation in a variety of degradative solutions, and we assessed them for the development of oxidative and hydrolytic degradation, changes in elastic properties, and chemical stability. To simulate the in vivo environment, we used buffered solutions of phospholipase A(2) and cholesterol esterase; solutions of H(2)O(2)/CoCl(2), t-butyl peroxide/CoCl(2) (t-but/CoCl(2)), and glutathione/t-butyl peroxide/CoCl(2) (Glut/t-but/CoCl(2)); and plasma fractions I-IV, which were derived from fresh human plasma centrifuged in poly(ethylene glycol). To act as a negative control, both graft types were incubated in distilled water. Samples of both graft types (100 mm with a 5.0-mm inner diameter) were incubated in these solutions at 37 degrees C for 70 days before environmental scanning electron microscopy, radial tensile strength and quality control, gel permeation chromatography, and in vitro compliance assessments were performed. Oxidative degradation was ascertained from significant changes in molecular weight with respect to a control on all Pulse-Tec grafts treated with t-but/CoCl(2), Glut/t-but/CoCl(2), and plasma fractions I-III. Pulse-Tec grafts exposed to the H(2)O(2)/CoCl(2) mixture had significantly greater compliance than controls incubated in distilled water (p < 0.001 at 50 mmHg). No changes in molecular weight with respect to the control were observed for the CPU samples; only those immersed in t-but/CoCl(2) and Glut/t-but/CoCl(2) showed an 11% increase in molecular weight to 108,000. Only CPU grafts treated with the Glut/t-but/CoCl(2) mixture exhibited significantly greater compliance (p < 0.05 at 50 mmHg). Overall, results from this study indicate that CPU presents a far greater chemical stability than poly(ether)-urethane grafts do.