Deficiency of alpha-L-fucosidase.

A new form of alpha-L-fucosidase deficiency has been found in a 20-year-old severely retarded male. Additional signs include angiokeratoma corporis diffusum and anhydrosis. The skin lesion is due to an accumulation of residual bodies, presumably containing oligosaccharides and glycoproteins, in endothelial cells and fibrocytes. The enzyme activity in blood relatives indicates that the disease is inherited as a simple autosomal recessive trait that segregates according to Mendelian principles. Because the enzyme activity in the heterozygotes was consistently below that of normal controls, the carriers of the trait in this family could be ascertained.

Subacute sclerosing panencephalitis: propagation of measles virus from brain biopsy in tissue culture.

Measles virus was propagated in monolayer cultures established from brain tissue of a patient with subacute sclerosing panencephalitis. Syncytial cells were rendered fluorescent with measles specific antiserums only, by means of an indirect technique. The ultrastructural appearance of the microtubular aggregates was identical in brain tissue and in the cultured cells. Fusion experiments produced a cytopathic effect in humnan embryonic kidney and VERO cell cultures. The virus was identified by hemiagglutination-inhibition, but only in the supernatant of disrupted cultured cells.

Hallervorden-Spatz syndrome.

Two siblings with Hallervorden-Spatz syndrome showed striking homotypism and homochronism. Neuropathologic examination and electron microscopic studies were done; neutron activation analysis showed an increase in the uptake of iron in the basal ganglia. Of particular relevance is the application of radioactive iron studies in the clinical course of this syndrome. These studies disclosed an increase in the uptake of iron in the area of the basal ganglia in one sibling and in another isolated patient. This procedure will be helpful toward the clinical diagnosis.

On the ultrastructural diversity and essence of residual bodies in neuronal ceroid-lipofuscinosis.

In 4 patients with neuronal ceroid-lipofuscinoses (NCL) (3 patients with the junvenile type, 1 patient with the late infantile type), the ultrastructural spectrum of residual bodies in the central and peripheral nervous system presented curvilinear profiles in all cases and regions investigated and many more ultrastructural patterns within and beyond regions commonly accessible to biopsy, probably due to age dependence, local tissue and cellular biochemical factors. Sampling from basal ganglia especially yielded combined curvilinear-fingerpint bodies, from peripheral ganglia additional membranous bodies. Residual bodies in NCL were present in almost every cell type, similar to the distribution of regular lipofuscin. Although the classical subgroups of NCL contain electronmicroscopically well defined residual bodies, permitting distinction of the late infantile type from the juvenile type, the ultrastructural differences are more of a quantitative than of a qualitative nature. However, they are not pathognomonic. N.m.r. spectra of ceroid and lipofuscin support the concept of their biochemical similarity, and argue against the proposition that they contain a single major component.

Development of a minimally invasive technique for coronary revascularization in a porcine model.

BACKGROUND: This porcine model was designed to develop a minimally invasive method for internal mammary artery (IMA) grafting using an anterior mediastinal approach and without routine use of cardiopulmonary bypass. METHODS: Assessment was made of IMA mobilization through a small parasternal incision, the feasibility of coronary artery grafting with cardiopulmonary bypass using this approach, and conditions for off-pump bypass grafting. RESULTS: In group 1, 6 pigs underwent IMA mobilization through a 5-cm horizontal midparasternal incision. Of the 2 group 2 pigs, 1 underwent IMA grafting to the left anterior descending coronary artery and the other, bilateral IMA grafting to the left anterior descending and right coronary arteries using femoral-vessel cardiopulmonary bypass. In group 3, 4 of 10 pigs had successful off-pump grafting during retrograde regional coronary venous perfusion of arterial blood. Retrograde coronary venous perfusion could not be established in the other 6 pigs, and attempts at off-pump grafting failed. CONCLUSIONS: The study demonstrates that coronary artery grafting with the IMA by this minimally invasive off-pump method is feasible, although it draws attention to areas of concern and potential methods of correction. The model provides a realistic and important learning platform for the surgical issues involved with this minimally invasive technique.

Editorial: Post-therapeutic radiation injuries of the nervous system. Reflections on their prevention.

The introduction of the concept of Nominal Standard Dose and of Time, Dose Fractionation Factors ostensibly permits definition of tolerance doses for normal tissues in unequivocal terms. However, even with these refinements, tolerance doses remain, at best, guidelines, because radiobiologic effectiveness is governed not only by the effective dose, but also by individual factors, which will modify the response. Attention must be accorded to these biologic parameters, in order to prevent injury to healthy tissues. Of particular significance are the relative size or volume of the irradiated tissue, the possible presence of co-existing pathology in the exposed organ and the development of disease after tge termination of the treatment. Even if these factors are properly respected, the risk of radiation injury cannot entirely be eliminated. The radiotherapist is therefore obligated to use an approach which minimizes the exposure of the healthy nervous tissue, a goal which has become attainable with the advent of modern accelerators as radiation sources.

Ultrastructural investigations of peripheral nerves in neuronal ceroid-lipofuscinoses (NCL).

Specimens of brachial plexus, sural nerve and two cranial nerves of one patient with Jansky-Bielschowsky type and 3 patients with the Spielmeyer-Sjögren type of NCL were studied by electron microscopy. Significant light microscopic changes were absent in all specimens. Ultrastructurally, curvilinear and/or fingerprint inclusions were present in each case, located chiefly in Schwann cells. These diagnostic findings were, however, overshadowed by masses of lamellar pi-granule-like cytosomes, usually not mixed with curvilinear or finger-print profiles in the juvenile cases and only rarely associated with curvilinear profiles in the late infantile case. Since secondary changes of axons and myelin sheaths were mild, these lamellar cytosomes might indicate chronic damage to Schwann cells, perhaps by "wear and tear" as seen in aging as well as NCL. On account of the abundance of pi-granules in NCL, peripheral nerve biopsy appears less suitable for confirming this diagnosis than biopsy of skin, striated muscle and rectal tissue.

A multicenter study of lomefloxacin versus ciprofloxacin in the treatment of complicated urinary tract infections.

Lomefloxacin is a new difluorinated quinolone antimicrobial agent with broad antibacterial activity and a long half-life which allows once-daily dosing. A multicenter, randomized trial was conducted to compare the safety and efficacy of once-daily oral lomefloxacin with twice-daily oral ciprofloxacin in the treatment of complicated urinary tract infections (UTIs). All 203 patients enrolled in the study had significant bacteriuria of - 10(5) colony-forming units/ml (CFU/ml) and clinical signs and symptoms of UTI such as dysuria, frequency, urgency, pain, or hematuria. Patients were randomized to receive either 400 mg lomefloxacin once daily (n = 101) or 250 mg ciprofloxacin twice daily (n = 102). The predominant baseline pathogen isolated from the patients in both groups was Escherichia coli. At the third visit (5-9 days post-treatment), 97.8% of patients in the lomefloxacin group and 96.8% in the ciprofloxacin group showed satisfactory bacteriologic results. Clinical success was achieved in 98.9% of patients in both treatment groups and there were no statistically significant differences between the two groups. Both drug regimens were well tolerated and no patient discontinued treatment due to adverse events. It was concluded that once-daily lemofloxacin was as effective as twice-daily ciprofloxacin in patients with complicated UTIs.

An ultrastructural study of the retina in the Jansky-Bielschowsky type of neuronal ceroid-lipofuscinosis.

A 7 1/2-year-old boy died of the Jansky-Bielschowsky type of neuronal ceroidlipofuscinosis. Although an electroretinogram showed reduced activity, pigmentary retinal atrophy was not demonstrable by ophthalmoscopy two years before death. At autopsy, the retina had severe loss of photoreceptor cells, pigment displacement, gliosis, and uniformly structured lipopigment bodies with an internal structure of curvilinear profiles in ganglionic and Müller cells, in pigment epithelia, in the remaining photoreceptors, and in the elements of the inner nuclear layer. These findings conformed to those in the juvenile type of neuronal ceroid-lipofuscinosis, differing only as to the severity.

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: III. Dosimeter design, construction, and implantation.

To detect the passage of cosmic ray particles through the heads of the pocket mice during the Apollo XVII flight, a "monitor" (dosimeter) composed of plastics was prepared and implanted under the scalp. The monitor was mounted on a platform, the undersurface of which fitted the contour of the skull. Numerous tests were run to assure that the presence of the monitor assembly beneath the scalp would be compatible with the well-being of the mice and that the capacity of the monitor to detect the traversal of cosmic ray particles would be preserved over the several weeks during which it would remain under the scalp.

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: XII. Results of examination of the calvarium, brain, and meninges.

Tissue reactions were found around the monitor (dosimeter) assemblies that had been implanted beneath the scalp of the five pocket mice that flew on Apollo XVII. Mitosis in the dentate gyrus of the hippocampal formation was considerably reduced in comparison with that in control animals. Otherwise the brain tissue as well as the menings in the flight animals appeared unaltered. Since the animals were exposed primarily to high Z-high energy (HZE) cosmic ray particles at the lower end of the high LET spectrum, the lack of changes in the brain cannot be taken as evidence that the brain will suffer no damage from the heavier HZE particles on prolonged manned missions.

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: VI. launch, flight, and recovery.

The final phase to fly five pocket mice in the Apollo XVII command module was carried out at the NASA Kennedy Space Center. Upon completion of the 13-d space flight, the package was removed from the spacecraft and, after having been purged with an oxygen-helium gas mixture, was flown to American Samo. Four of the five mice were recovered alive from the package. Analysis of the mouse that died during the flight revealed several factors that could have contributed to its death, the chief of which was massive hemorrhage in its middle ear cavities.

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: X. Results of ear examination.

In the five pocket mice flown on Apollo XVII, no evidence was found that the inner ear had been damaged, though poor fixation precluded detailed study. On the other hand, the middle ear cavity was involved in all the mice, hemorrhage having occurrred in response to excursions in pressure within the canister that housed the mice during their flight. The same occurred in flight control mice which had been subjected to pressure excursions of much the same magnitude. A greater degree of exudation into air cells and greater leukotaxis were noted in the flight animals than in the control animals. There was no increase in leukocyte population along the paths of the 23 cosmic ray particles registered in the subscalp dosimeters that traversed the middle ear cavities of the flight mice. The increased exudation and the greater response by leukocytes in the flight mice may have been causally related to the lesions found in their olfactory mucosa but there were no data in support of this possibility.

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: IX Results of examination of the nasal mucosa.

The olfactory epithelium, but not the nasal respiratory epithelium, of the four pocket mice (Perognathus longimembris) that survived their flight on Apollo XVII showed both diffuse alterations and numerous disseminated focal lesions. The olfactory mucosa of the mouse that died during flight was also affected, but to a minor degree insofar as could be determined. All this was in contrast to the normal appearance of the olfactory mucosa of the numerous control animals. A number of possible causes were considered: systemic or regional infection; inhaled particulate material (seed dust); by-products from the KO2 bed in aerosol or particulate form; gas contaminants originating in the flight package; volatile substances from the dead mouse; weightlessness; and cosmic ray particle radiation. Where feasible, studies were conducted in an effort to rule in or rule out some of these potentially causative factors. No definitive conclusions were reached as to the cause of the lesions in the flight mice.