Nosological significance of retinopathies in neurodegenerative disorders with emphasis on Batten disease.
Explore the source record for details and available documents.
3',5'-Cyclic-GMP Phosphodiesterases↗
Biomedical subjects
J Marshall
Publications and source records attributed to J Marshall.
Survival of cone responses in postmortem human retina.
A cone-mediated electroretinogram was recordable from human retinas 17-18 hours postmortem, after regeneration of visual pigments by application of 11-cis and 9-cis retinal. The cone was separated from the rod component by stimulation with flickering red light. In addition to these neuronal responses, a P2 and a slow P3 were present.
Adolescent↗
Histamine does not mediate mucosal permeability changes after subclinical intestinal ischemia-reperfusion injury.
UNLABELLED: Subclinical intestinal ischemia-reperfusion injury (IRI) increases mucosal permeability, and may be an important mechanism in the etiology of necrotizing enterocolitis. The current study was designed to assess the role of histamine in mediating this phenomenon. Six-week-old rats underwent 10-minute superior mesenteric artery occlusion (SMAO) or sham operation, and ileal mucosal permeability to 51Cr EDTA was measured 30 minutes after reperfusion. Rats were pretreated with intravenous saline, mepyramine (6 mg/kg), or ranitidine (5 mg/kg). SMAO resulted in a significant increase in permeability compared to sham, which was not attenuated by either of the histamine antagonists. In a second experiment, mucosal permeability to 51Cr EDTA was measured in 6-week-old rats during aortic infusion of saline or histamine (0.5 mg/kg/min). There was no significant increase in permeability as a result of histamine infusion. In a third experiment, 6-week-old and 10-day-old rats underwent sham or 10-minute SMAO, and both portal vein and ileal tissue histamine levels were measured 30 minutes after reperfusion. There was no significant difference between sham and SMAO with respect to portal vein histamine or tissue histamine at either age. IN CONCLUSION: (1) increased permeability was not blocked by either H1 or H2 blockers; (2) histamine infusion did not increase permeability; and (3) SMAO did not increase portal vein or tissue histamine levels. These data suggest that histamine does not play a role in mediating the increase in permeability after subclinical IRI in this model.
Animals↗
Coffee, cigarette smoking, and bladder cancer in western New York.
The association between consumption of coffee and bladder cancer and the effect modification of cigarette smoking was investigated as part of a comprehensive case-control study. The study population consisted of 351 case patients with histologically confirmed transitional cell carcinomas of the bladder among white males and 855 white male control subjects selected from Erie, Niagara, and Monroe counties in New York from 1979 to 1985. Usual diet, coffee consumption, and cigarette use were estimated by comprehensive interviews using a detailed food frequency questionnaire. After adjustment for age, education, and dietary risk factors by multiple logistic regression, risk was found to increase with increasing pack-years of cigarette use with an odds ratio in the highest quartile of 2.7 (95% confidence interval, 1.8 to 4.0) when compared to the lowest quartile. Coffee consumption was associated with an increased risk for bladder cancer among the heaviest coffee drinkers after adjustment for cigarette smoking and other dietary risk factors (odds ratio, 2.1; 95% confidence interval, 1.3 to 3.2). The effect was more pronounced among nonsmokers, especially among those 65 years and older. These findings support the contention that coffee is a weak carcinogen.
Adult↗
Correlation between lipids extracted from Bruch's membrane and age.
BACKGROUND: There is increasing circumstantial evidence that the chemical composition of the deposits in Bruch's membrane influences the clinical outcome in age-related macular disease. In particular, it has been postulated that deposition of neutral lipids in Bruch's membrane may cause hydrophobicity and predispose to detachment of the retinal pigment epithelium and cause functional loss. METHODS: Analysis of the lipid extracted from Bruch's membrane of eye bank eyes from donors of different ages has been undertaken using thin layer and gas chromatography. No clinical information was available concerning any previous eye disease. RESULTS: It is shown that the lipid extracted increases with the age of the donor, and that the total quantity and ratio of neutral fats to phospholipids varies widely from one specimen to another from donors older than 60 years of age. The ratios of the different phospholipids imply that they are not derived from blood. CONCLUSION: The results are compatible with the concept of hydrophobicity developing with age in Bruch's membrane.
Adolescent↗
Long-term follow-up of diode laser trabeculoplasty for primary open-angle glaucoma and ocular hypertension.
BACKGROUND: Initial studies of laser trabeculoplasty using infrared energy (810 nm) emitted by diode semi-conductor lasers have been encouraging. A 2-year study of diode laser trabeculoplasty (DLT) in the control of primary open-angle glaucoma (POAG) and ocular hypertension has been completed. METHODS: Patients with uncontrolled POAG or ocular hypertension were treated with DLT to one half of the trabecular meshwork using a trabeculoplasty lens. Spot size was 100 microns, exposure time was 0.2 second, and mean power was 1096 mW (+/- 46.5 mW). The desired endpoint was a mild blanching of the meshwork only. RESULTS: Twenty-five eyes of 16 patients were treated. Mean intraocular pressure reduction was 9.24 mmHg (+/- 3.4 mmHg) at 6 weeks, 9.32 mmHg (+/- 3.6 mmHg) at 3 months, 9.34 mmHg (+/- 3.8 mmHg) at 6 months, 8.42 mmHg (+/- 2.62 mmHg) at 12 months, 8.14 mmHg (+/- 3.42 mmHg) at 18 months, and 7.9 mmHg (+/- 3.63 mmHg) at 24 months. No pressure peaks (> 5 mmHg) were recorded after therapy. Inflammation and discomfort were minimal after laser treatment. Of 16 eyes examined at 2 years, there were no peripheral anterior synechiae. During the course of the study, six eyes became uncontrolled, despite one session of DLT. Four eyes regained control with a further session of DLT, but two required trabeculectomy. CONCLUSION: Diode laser trabeculoplasty is an effective form of therapy in POAG and ocular hypertension. Hypotensive effects and success rates are comparable with argon laser trabeculoplasty (ALT). Reduced inflammation after laser treatment may be due to reduced absorption of infrared energy by the melanin of the anterior segment. The portable nature of these lasers may allow for laser delivery in developing countries and remote situations.
Aged↗
Comparison of the anterior chamber inflammatory response to diode and argon laser trabeculoplasty using a laser flare meter.
BACKGROUND: Diode laser trabeculoplasty (DLT) has a similar hypotensive action to argon laser trabeculoplasty (ALT). However, anterior chamber inflammatory response and laser-induced discomfort are less pronounced with DLT. The authors compared the breakdown of the blood-aqueous barrier after DLT and ALT over the time course of 1 week and the hypotensive action over 8 weeks. METHODS: Twenty-five patients with uncontrolled intraocular pressure (IOP) were randomized to either DLT or ALT. If two eyes required treatment, then the second eye would be treated with the alternative laser to the first. Flare values were measured at 1, 3, 24, 48, 72, 96, and 168 hours using a laser flare and cell meter. Intraocular pressures were measured at similar intervals and at 8 weeks. RESULTS: Of 38 eyes treated, 21 underwent ALT and 17 DLT. Both forms of treatment induced a similar hypotensive effect with a significant reduction in IOP at 8 weeks compared with initial IOP (P < 0.01) (paired Student's t test). However, there was a significantly greater disruption of the blood-aqueous barrier with ALT when compared with DLT at 1, 3, and 24 hours (P < 0.001) and at 48, 72, and 96 hours (P < 0.01) (unpaired Student's t test). By 1 week, however, there was no significant difference in flare values between the two groups. Postlaser pain occurred in seven eyes treated with ALT but in no eyes treated with DLT. In addition, peripheral anterior synechiae occurred in four eyes after ALT by 8 weeks but none occurred in the DLT group. CONCLUSION: Argon laser trabeculoplasty produces a greater disruption of the blood-aqueous barrier than DLT. This may be associated with the development of complications in the anterior segment.
Aged↗
The effect of topical corticosteroids on refraction and corneal haze following excimer laser treatment of myopia: an update. A prospective, randomised, double-masked study.
Several thousands of myopic patients have undergone photorefractive keratectomy (PRK) worldwide (prior to the publication of long-term, carefully controlled studies). The evidence in support of the currently widespread use of topical corticosteroids after PRK, however, is limited. We report the results of a prospective, double-masked trial to determine the effect of dexamethasone 0.1% on refraction and anterior stromal haze following -3.00 and -6.00 dioptre PRK procedures (n = 113; minimum follow-up 1 year). Initially the reduction in myopia was significantly greater in the steroid group compared with the placebo group (p < 0.002). However, on discontinuing the corticosteroids at 3 months this difference became statistically insignificant (p > 0.2). There was no statistically significant effect on anterior stromal haze at any stage. We conclude that, in view of their side effects, it would be unacceptable to use topical corticosteroids in the long term to maintain the initial beneficial effect on refraction. We therefore consider that topical corticosteroids should not be used following PRK.
Administration, Topical↗
Assessment during postgraduate training.
The author reviews the traditional process of assessing both undergraduates in medical school and physicians in postgraduate training, contending that this traditional, and still dominant, process is inadequate because of its limitations. It focuses narrowly on end-point evaluation, using predominantly multiple-choice questions, rather than on identifying deficiencies during the training period so that trainees are able to correct these deficiencies before their end-point evaluations. An example of a more valid process of assessment is the set of tests employed by the Royal Australian College of General Practitioners to assess the skills of family practice physicians, by using behavioral ratings. The author concludes that where such tests are used, there may be some difficulty in comparing the performances of undergraduates with those of practicing physicians, in view of the different behaviors being assessed. In any follow-up studies of graduates, attention should be paid to the reliability and validity of the assessment procedures, particularly because differences in the assessment tools may account for some discrepancies between training programs.
Clinical Competence↗
A new trichrome-blue stain for detection of microsporidial species in urine, stool, and nasopharyngeal specimens.
Detection of microsporidia in clinical specimens has relied on electron microscopy, histology, or staining. This article describes further alterations to the modified trichrome staining method which make it easier to identify microsporidial spores. The changes are a decrease in the phosphotungstic acid level and the substitution of a colorfast counterstain, aniline blue, for the fast green of the original stain. The modified stain provides good contrast between microsporidial spores and background material including human and fungal cells. Stool specimens from 139 human immunodeficiency virus-seropositive patients revealed that 5 patients were infected with Enterocytozoon bieneusi and 6 patients had larger spores. Thin-section electron microscopy of the larger spores showed a structure consistent with that of either Encephalitozoon or Septata species. Three of the patients with Encephalitozoon- or Septata-like species had disseminated infection, with spores detected in nasopharyngeal aspirates and urine samples.
AIDS-Related Opportunistic Infections↗
Treatment of band keratopathy by excimer laser phototherapeutic keratectomy: surgical techniques and long term follow up.
A series of 122 eyes with band keratopathy was treated by excimer laser phototherapeutic keratectomy (PTK), with a mean follow up of over 12.3 months (range 3 to 60 months). A single photoablation zone was used to remove the opacity over the visual axis in smooth surfaced band deposition. In eyes with reduced vision, an improvement was reported in 88% and in a series of 66 eyes mean Snellen visual acuity increased significantly (p < 0.05, t = 2.27). A reduction in glare was reported in 88% and in a series of 17 patients, visual contrast sensitivity (p < 0.01) and measurements of disability glare (p < 0.01) improved postoperatively. The mean hyperopic shift in 32 eyes at 6 months was 1.4 D (range 0-4.25 D). Multiple overlapping ablation zones, with mechanical debulking of large calcium plaques, were used to smooth the irregular corneal surface in eyes with rough bands. Ocular discomfort was improved in 95%. Band keratopathy recurred in nine eyes (8%) within 2 to 30 months (mean 12 months) of surgery, with silicone oil responsible in five eyes. Reablation was necessary in three eyes and performed successfully in all cases. Excimer laser PTK is a safe and effective outpatient treatment for band keratopathy.
Adult↗
Absorbed aluminium is found with two cytosolic protein fractions, other than ferritin, in the rat duodenum.
After in vivo perfusion of the upper intestine of the rat with a range of concentrations of aluminium chloride, entry of the metal into the portal system was only detected when the perfusate exceeded 400 mumol/l, suggesting a mucosal block. Using gel filtration of a mucosal cytosol extract, two consistently appearing aluminium peaks were identified which may represent aluminium binding proteins. Both were heat stable at 60 degrees C and had molecular sizes of about 700 (kilo daltons) (kD) and 17 kD respectively. The larger molecule was distinct from ferritin. Neither molecule associated with 59Fe nor 45Ca. It is suggested that the aluminium peaks are relatively specific aluminium binding proteins that have a scavenging role, reducing entry of the metal from the intestinal contents into the portal blood.
Aluminum↗
Case study of a schizophrenic patient during social skills training in a forensic psychiatry ward.
A schizophrenic patient's behaviour was monitored over 12 weeks using the Assessment Schedule and Adult Training Instrument, the patient's self-reports of his symptoms of anxiety, scores on the Social Behaviour Schedule, and the frequency of nocturnal enuresis. Contrary to expectations, the patient's functioning deteriorated generally, with the exception that the trend of deteriorating behaviour, as assessed by hospital staff, appeared to have slowed down during a period involving activities out of the ward.
Activities of Daily Living↗
A survey of aphasia services in the United Kingdom.
A survey of the service available, in speech and language therapy departments, to adults with aphasia revealed that many districts in the United Kingdom are unable to fulfil the professional recommendations for the care of aphasic clients. Although individual 'good practice' criteria could be satisfied, only a minority of districts were able to provide a comprehensive service. Levels of staffing for the adult neurological caseload are variable and even, at best, hinder therapists in delivering the recommended standard of management to aphasic clients and their carers.
Aphasia↗
Lung liquid production by in vitro lungs from fetal guinea pigs: effects of arginine vasopressin and arginine vasotocin.
Lungs from near-term fetal guinea pigs were supported in vitro for 3 h; lung liquid production rates were measured by a dye dilution technique. Seventy preparations were used to study the effects of arginine vasopressin (AVP) placed in the outer saline for the middle hour, at concentrations reported at birth [fetuses 61 +/- 2 days of gestation; 94.7 +/- 16.2 g (SD) body weight]. At 1200 microU/ml, AVP arrested fluid production (rates, successive hours, 3.03 +/- 0.60, 0.50 +/- 0.14 and 0.02 +/- 0.08 ml/kg body weight per h; falls significant, P < 0.01-0.0005). At 600, 300 and 100 microU/ml there were significant but smaller reductions. Reabsorptions were seen in 8 preparations given 600-1200 microU/ml, AVP. Preparations given 10 microU/ml AVP, AVP carrier or control saline showed no significant change. The responses (% reductions during treatment), were linearly related to the log concentration of AVP (r = 0.99); theoretical threshold, 8 microU/ml). Increasing treatment to 2h did not increase final responses. Preparations from 5 fetuses > 120 g body weight showed significantly greater responses (P < 0.025) [fetuses 64 +/- 2 days of gestation; 135.1 +/- 18.6 g (SD) body weight]. 10(-6) M amiloride abolished responses to AVP [fetuses 62 +/- 1 days of gestation; 93.4 +/- 18.5 g (SD) body weight, n = 30; rates, succeeding hours; AVP alone, 1.78 +/- 0.22, 0.48 +/- 0.09, 0.16 +/- 0.99 (P < 0.01-0.0005); AVP with amiloride, 1.15 +/- 0.07, 0.93 +/- 0.10, 0.86 +/- 0.08 (no significant fall) ml/kg body weight per h]. Thirty-six preparations treated with arginine vasotocin (AVT, 10-600 microU/ml) showed closely similar responses to those from AVP. These studies extend results to fetal guinea pigs, and show that AVP, at concentrations reported at delivery, can slow lung liquid production or cause reabsorption by a direct action on the lung. The effect increases close to term, and is due to activation of amiloride-sensitive Na+ channels.
Amiloride↗
Is the risk of coronary heart disease lower in Hispanics than in non-Hispanic whites? The San Luis Valley Diabetes Study.
A less favorable cardiovascular risk factor profile, but paradoxically lower coronary heart disease mortality and prevalence have been reported for Hispanic men compared to non-Hispanic white men. Since mortality and prevalence data are susceptible to bias, the patterns of coronary heart disease incidence, as well as prevalence and mortality, were investigated in a biethnic Hispanic and non-Hispanic white population of the San Luis Valley in Colorado. Little evidence was found for lower incidence, prevalence, or mortality due to coronary heart disease among Colorado Hispanics without diabetes. The risk of coronary heart disease among diabetic Hispanics appeared, however, to be approximately 50% lower than among non-Hispanic whites, especially in men. Adjustment for selected cardiovascular risk factors (age, gender, diabetes, hypertension, cigarette smoking, body mass index, and high-density lipoprotein cholesterol and triglycerides levels) did not change this ethnic pattern. The plausible explanations of a lower coronary heart disease risk among diabetic Hispanics, compared to non-Hispanic whites, include both biologic mechanisms and artifacts due to deficiencies of mortality classification or differential access to health care. The existing evidence is insufficient to conclude that the risk of coronary heart disease in the general population differs between Hispanics and non-Hispanic whites. The ethnic patterns of coronary heart disease incidence should be investigated further through population-based incidence studies.
Adult↗
Diet in the epidemiology of postmenopausal breast cancer in the New York State Cohort.
A number of authors have presented evidence that high dietary fat increases the risk of breast cancer, and a number have presented evidence to the contrary. In this study, dietary histories were obtained in 1980 from 18,586 postmenopausal women in New York State. These women were followed through 1987 to ascertain their incidence of breast cancer and other cancers and deaths from all causes, as registered in the New York State Tumor Registry and Office of Vital Statistics. Survival analysis revealed that the incidence of breast cancer increased with age, was higher among the nulliparous, was higher for those with a late (> 26 years) age at first pregnancy, and increased with increasing socioeconomic status--all risk factors discovered before for breast cancer. No increase in risk was related to the ingested amount of calories, vitamins A, C, or E, dietary fiber, or fat. Although dietary fat has been found to be associated with higher risk of cancer at a number of other sites, e.g., the lung, colon, and rectum, and although some previous writers have suggested an association with risk of breast cancer, the findings in three cohort studies as well as in eight substantial case-control studies are negative and suggest that a relation is far from established.
Age Factors↗
Adjunctive thrombolytic therapy for angioplasty in ischemic rest angina: results of a double-blind randomized pilot study.
OBJECTIVES: A multicenter pilot study was instituted to assess the role of intracoronary thrombolytic therapy during angioplasty for ischemic rest angina. BACKGROUND: Acute thrombotic coronary occlusion is increased during angioplasty for unstable angina, and intracoronary thrombolytic agents have been used to maintain patency. Prophylactic use of intracoronary thrombolytic agents has been advocated in certain high risk subgroups, although no studies have randomized therapy. METHODS: Ninety-three patients with either unstable angina and pain at rest (trial A, 66 patients) or postinfarction pain at rest (trial B, 27 patients) were randomized in double-blind fashion to administration of either intracoronary urokinase, 150,000 U, or saline solution placebo given immediately before angioplasty. Cineangiograms of the culprit lesion were recorded and analyzed in blinded fashion by a core laboratory for definite or possible (haziness) filling defects 15 min after angioplasty or after acute closure. RESULTS: Urokinase decreased filling defects at 15 min after angioplasty in comparison with placebo (14% vs. 29%, respectively, p = 0.08). Four patients in each treatment group developed acute vessel closure. However, although urokinase significantly reduced the incidence of filling defects in trial A (3% vs. 23%, p = 0.03), the drug had no effect at the selected dose in trial B (42% vs. 43%, respectively). Acute vessel closure occurred significantly more frequently in trial B than in trial A, and urokinase at the selected dose also had no effect. Ischemic events after angioplasty appeared to be related more to dissection than to thrombosis, although redilation, which was more frequent after placebo administration, may have reduced their incidence as well as that of acute closure. CONCLUSIONS: These data suggest a possible role for intracoronary urokinase during angioplasty for unstable angina. The lack of effect after infarction may represent a greater thrombus burden or degree of plaque disruption. A trial utilizing higher doses of urokinase in a larger patient group is in progress.
Angina, Unstable↗