Crystal deposition following keratoplasty in nephropathic cystinosis.
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Adult↗
Biomedical subjects
B Katz
Publications and source records attributed to B Katz.
Abnormal pattern electroretinogram in Alzheimer's disease: evidence for retinal ganglion cell degeneration?
We recorded pattern-reversal electroretinograms, flash electroretinograms, pattern-reversal visual evoked potentials, and flash visual evoked potentials in 6 patients with clinically diagnosed Alzheimer's disease and 6 age- and sex-matched control subjects. The mean amplitude of the pattern-reversal electroretinogram in the Alzheimer patients was significantly less than that of the control group (p = 0.004). This anomaly of the pattern-reversal electroretinogram may be a reflection of documented axonal depletion within the optic nerve and the degeneration of retinal ganglion cells seen in Alzheimer's disease. We found Alzheimer patients to have normal pattern-reversal visual evoked potentials and flash electroretinograms, but a delayed second positive component of the flash visual evoked potential. This combination of findings may be of diagnostic import in Alzheimer's disease.
Action Potentials↗
[The Teller Acuity Card Test: possibilities and limits of clinical use].
The Teller Acuity Card test was used to examine 49 normal children, 77 with strabismus, 9 with anisometropia and 19 with various organic ocular diseases. The vision of some of these children was also tested with the Landolt C and fixation preference tests. A comparison of the three tests showed that strabismic amblyopia is not reliably detected with the Teller Acuity Cards. On the other hand, this test appears to be a good one for detecting loss of acuity due to ocular diseases.
Amblyopia↗
Corneal thickness in nephropathic cystinosis.
Cystinosis is a rare autosomal recessive metabolic disorder in which non-protein cystine accumulates within cellular lysosomes owing to a defect in lysosomal cystine transport. The pathognomonic ocular manifestation of cystinosis is the deposition of distinctive iridescent crystals in the cornea, not associated with any inflammatory response or recognised change in corneal function. We measured corneal thickness in nine patients with infantile nephropathic cystinosis. We also studied a corneal button from one of these patients who underwent corneal transplantation. All nine patients had increased corneal thickness in comparison with an age matched control population. Electron microscopy analysis of the cystinotic button revealed structural changes of both epithelium and endothelium layers. Increased corneal thickness in patients with nephropathic cystinosis may reflect subclinical corneal oedema.
Adolescent↗
The ferret as an animal model in cerebrovascular research.
Clinical and pathologic observations have suggested analogies between the developing nervous system of ferrets (Mustela putorius furo) and those of more traditional animal models employed in stroke research. Experimental work has demonstrated advantages of the ferret as a model of visual development. We performed in vivo cerebral angiography and postmortem neurovascular dissection of latex-injected specimens of adult ferrets. The great vessels include a cervical arterial trunk that gives rise to both carotid arteries. The anatomy of the cranial arteries is similar to that of rabbits. No carotid rete mirabile is present. There are no intracranial anastomoses between the external and internal carotid systems. We present in vivo cerebral angiograms with pathologic correlation that demonstrate that ferrets may provide the same anatomic advantages as a rabbit model for the experimental study of cerebrovascular disease, with the additional advantage of a long extracranial cervical segment of the carotid artery, affording easier access to the intracranial vasculature.
Animals↗
Effect of tube-feeding osmolality on serum sodium levels.
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Adult↗
Amyloid ophthalmoplegia. Ophthalmoparesis secondary to primary systemic amyloidosis.
We report a patient with primary systemic amyloidosis and orbital involvement associated with ophthalmoparesis. We support this with the first demonstration of eye movement recordings and magnetic resonance imaging (MRI) of such a patient. The patient presented with a restrictive ophthalmoparesis. Eye movement recordings documented slowed horizontal saccades. MRI of orbital structures suggests that all extraocular muscles were enlarged, with possible involvement of their tendinous insertions.
Amyloidosis↗
Ophthalmoplegic migraine with superior ramus oculomotor paresis.
We report a 13-year-old girl with recurrent paroxysmal superior division oculomotor palsy as a manifestation of ophthalmoplegic migraine. Neurodiagnostic tests (including head computed tomography scan, cerebral angiography, and Tensilon tests) were normal. This case demonstrates that ophthalmoplegic migraine may selectively affect superior division fibers of the oculomotor nerve.
Adolescent↗
Can psychometric tools be used to analyse pain in a geriatric population?
Pain is both a sensory and an emotional experience. In a multidisciplinary pain management clinic for a geriatric population, pain and mood have been assessed prior to the initiation of management in 49 of 100 referred patients. Patients have been assessed clinically, for psychological disability using the profile of mood states (POMS) and a visual analogue scale for mood, for pain using the McGill pain questionnaire (short form), word descriptor scale and visual analogue scale and for activities of daily living (ADL) using the Disability and Impairment Interview Schedule and the Rapid Disability Rating Scale -2. All testing has followed initial screening to exclude dementia. For the group assessed by psychometric measures, median age was 75 years (range 56 to 91); 41 were female. Major pathologies were degenerative musculoskeletal disease (15), post-herpetic neuralgia (9), and psychiatric conditions (7). Intra-test item correlations were found to be significant for the McGill questionnaire and the POMS. Inter-test correlations were observed for pain measures (McGill present pain intensity with VAS pain, r = 0.67; with word descriptor, 0.64; p less than 0.001), for ADL measures (r = 0.53, p less than 0.001) and for measures of mood (VAS mood with POMS (depression), r = 0.45; p less than 0.001; with POMS (anxiety), r = 0.35; p less than 0.01. There was no significant correlation between measures of mood and pain on the McGill scale. The data to this time support the view that standard psychometric tools can be used reliably to evaluate pain, mood and activity in this population. Furthermore, it appears that mood and pain may be measured separately.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
The pattern electroretinogram: technical aspects and clinical significance.
The pattern electroretinogram (PERG), thought to be generated in retinal ganglion cells, is a recent addition to the neurophysiologic assessment of retinal function. Factors that affect the PERG include type of pattern-reversal stimulus, spatial frequency of the pattern, luminance and contrast of the pattern, method of waveform processing, type of electrode employed, electrode montage, background illumination, position of stimulus on the retina, temporal frequency of pattern reversal, refractive error, and patient age. Clinical entities reported to affect the PERG include glaucoma and ocular hypertension, optic neuritis, other optic neuropathies, maculopathies, and amblyopia. Technical aspects and clinical significance of the PERG are reviewed.
Electroretinography↗
Retinocortical time exhibits spatial selectivity.
We recorded simultaneous electroretinograms and visual evoked potentials to pattern-reversal stimulus in ten normal subjects employing check sizes subtending 7.5, 15, 30, 60, 120, 240 and 480 min of visual arc. The mean peak latency of the pattern electroretinogram (PERG) b wave decreased logarithmically with increasing check size. The mean peak latency of the pattern visual evoked potential (PVEP) P100 component was shortest for 30 min check size and was longer at both smaller and larger check sizes, a phenomenon termed spatial selectivity. Retinocortical time (RCT) was calculated as the latency difference between the PVEP P100 peak and the PERG b wave. The RCT also exhibited spatial selectivity similar to that of the PVEP P100 component; mean RCT was shortest for 30 min check size and was longer at both smaller and larger check sizes. Implications are discussed in light of our current knowledge of the origins of the PERG and PVEP and visual processing of pattern-reversal stimuli. Simultaneous recording of PERGs and PVEPs in patients with disease of the afferent visual pathways employing a large range of check sizes may increase the diagnostic sensitivity of visual electrophysiologic testing.
Adult↗
The three-dimensional structure of Bacillus amyloliquefaciens subtilisin at 1.8 A and an analysis of the structural consequences of peroxide inactivation.
The three-dimensional structure of the subtilisin from Bacillus amyloliquefaciens (BAS) has been refined to 1.8 A using the amino acid sequence deduced from the DNA coding sequence. The structure is essentially the same as the previously reported structures of subtilisin BPN' (Wright, C.S., Alden, R.A., and Kraut, J. (1969) Nature 221, 235-242) and Novo (Drenth, J., Hol, W. G. J., Jansonius, J. N., and Koekoek, R. (1972) Eur. J. Biochem. 26, 177-181) determined in different crystal forms, at 2.5 and 2.8 A resolution, respectively. The largest differences in the three crystallographic models are seen in regions where the amino acid sequence used in the fit to the electron density maps of BPN' and Novo differs from the gene sequence of BAS (Wells, J. A., Ferrari, E., Henner, D. J., Estell, D. A., and Chen, E. Y. (1983) Nucleic Acids Res. 11, 7911-7925). The refined BAS model shows new features of cation binding, hydrogen bonding, and internal solvent structure. The refined BAS model has served as a basis for the analysis of stereochemical factors involved in the peroxide inactivation of the enzyme. Methionine 222, which is adjacent to the catalytic Ser221, is quantitatively oxidized to the sulfoxide by hydrogen peroxide as had been previously shown for the related Bacillus licheniformis enzyme (Stauffer, C. E., and Etson, D. (1969) J. Biol. Chem. 244, 5333-5338). In addition to this site of modification, we observe partial to full oxidation of two of the four remaining methionines. The oxidation of the methionines does not correlate well with their solvent accessibility calculated from the x-ray structure coordinates; in addition, only one of the two possible stereoisomers of methionine sulfoxide is formed. We also detect hydrogen peroxide-induced modification of the hydroxyl groups of two tyrosines. Modeling suggests that most of the observed effect of oxidation on the enzyme's catalytic efficiency can be attributed to unfavorable interactions at the oxyanion binding site between the sulfoxide group at 222 and the carbonyl oxygen of the scissile peptide bond of the bound substrate.
Bacillus↗
Optic nerve head drusen and pseudotumor cerebri.
A 42-year-old woman presented with a history of headache. Results of funduscopic examination revealed elevated disc margins and bilateral optic nerve head drusen. Lumbar puncture, head computed tomography, and fluorescein fundus angiography results were consistent with the diagnosis of pseudotumor cerebri and coexistent disc drusen. Visual loss was demonstrated by formal perimetry. Headaches were unresponsive to a medical regimen that included prednisone, glycerol, acetazolamide, furosemide, and repeated lumbar punctures. A lumbar peritoneal shunt was performed, with immediate resolution of headache. Optic disc drusen can be associated with pseudotumor cerebri and can lead to diagnostic confusion.
Adult↗
Infantile Gaucher's disease: a case with neuronal storage.
The brain of a 17-month-old boy with infantile Gaucher's disease and oculomotor apraxia was studied by light and electron microscopy. Light microscopic examination showed large perivascular accumulations of Gaucher's cells in frontal lobe white matter, severe neuronal loss in the calcarine cortex and dentate nucleus of the cerebellum, and neuronophagia and microglial nodules in the brainstem. Electron microscopy demonstrated intraneuronal cytoplasmic inclusions containing twisted tubules characteristic of Gaucher's disease in both the oculomotor nucleus and a random sample of cortex.
Brain↗
Increased expression of Fc gamma receptor in cancer patients and tumor bearing mice.
In this study we report on some lines of ongoing research performed in our laboratory, in relation to the increased expression of FcR on tumor cells, as well as on cells present in the tumor-bearing host, and its possible role in tumor progression. In a previous study we have shown that a Polyoma virus (PyV)-induced anaplastic carcinoma (SEYF-a tumor) contained an FcR-expressing subpopulation of tumorigenic cells. We tested the effect of in vivo passaging of FcR-expressing and of non-FcR-expressing sub-populations of SEYF-a tumor cells on the expression of FcR, as revealed by the ability of these cells to bind the 2.4G2 monoclonal antibody, which is directed against mouse Fc gamma 2b/gamma 1R. It was found that upon in vivo passaging these two sub-populations became practically identical in their ability to bind anti-Fc gamma R antibody. On the other hand, in vitro passaging of FcR-expressing SEYF-a cells resulted in a gradual decrease in the expression of Fc gamma R. These results, indicating that the expression of Fc gamma R on tumor cells, per se, is dependent on a factor present in the in vivo environment were confirmed using 3T3 cells transformed in vitro by PyV (C) and forming tumors at first injection to mice (CTC). C cultures of various clones did not express Fc gamma R, while CTC cultures (cultures from tumors) became positive. We also detected an increase in the level of a soluble form of Fc gamma 2b/gamma 1R in the circulation of mice bearing PyV induced tumors. This increase paralleled the appearance of palpable tumors. A similar pattern of increase was observed in mice inoculated with the c-H-ras transformed tumorigenic clone 8/F/5, but not in mice inoculated with non-tumorigenic 3T3 cells. Data published by us show that metastatic breast cancer patients had significantly elevated Fc gamma R levels on their peripheral blood mononuclear cells (PBMC). Experiments presented here indicate a direct correlation between increased Fc gamma R levels on PBMC and tumor mass in colon, ovary and lung metastatic carcinoma patients. The possibility that malignantly transformed cells have the potential to cause proliferation of Fc gamma R expressing T cells was tested. It was found that extract derived from r-H-ras transformed 3T3 cells triggers the proliferation of a T cell hybridoma expressing Fc gamma R.
Animals↗
Night terrors and sudden unexplained nocturnal death.
A high incidence of sudden unexplained nocturnal deaths has been reported among young Asian males. These deaths are known as Pokkuri in Japan, Bangungut in the Philippines and Sudden Unexplained Nocturnal Death in the United States. Post mortem analysis has demonstrated cardiac conduction defects in many of the victims. Careful review of the terminal events surrounding these deaths suggests that the victims suffered from night terrors. Night terrors are a sleep disorder characterized by vocalization, motor activity, a nonarousable state, and severe autonomic discharge. The proposed recognition of both night terrors and cardiac anomalies in these patients offers a pathophysiologic mechanism for their sudden death.
Asia↗
Neurologic and cognitive deficits in children with cystinosis.
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Adolescent↗
Surgical treatment of burns in elderly patients.
This study evaluates our experience with surgical treatment of burns in the elderly. Forty-two patients more than 59 years old were treated from 1982 to 1986. The mean age was 73, and the mean TBSA burned was 29%. The patients were divided into three groups. Group I had 22 patients with less than 20% TBSA burn (mean of 11%, with a mean of 6% full-thickness burns). Their survival rate was 91%. Group II had 11 patients with 21-40% TBSA burns (mean of 32%, with a mean of 17% full-thickness burns). Their survival rate was 82%. Group III had nine patients with burns greater than 40% TBSA (mean, 71%). None of these patients survived. Twenty of 29 (68%) survivors required a total of 36 operations. The mean area grafted per procedure was 8%. Each procedure required a mean of 2U packed red blood cells, and a mean of 2 1/4 hours. The complication rate was 33%, with partial graft loss (14%) being the most frequent. The average hospital stay was 27 days in Group I and 45 days in Group II. Only 25% of the patients required nursing home assistance at discharge.
Aged↗