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Biomedical subjects

E Chew

Publications and source records attributed to E Chew.

18 recordsLinked to original sources

Radiation exposure: a new risk factor for idiopathic perifoveal telangiectasis.

OBJECTIVE: To examine the association between previous radiation exposure and idiopathic perifoveal telangiectasis (IPT). DESIGN: A multicentered, individually matched, case-control study design was used. PARTICIPANTS/CONTROLS: Sixty-five case subjects were matched with 175 control subjects. Individuals with unequivocal evidence of angiographically confirmed IPT were included as cases. Control subjects were matched for center, age, and gender. MAIN OUTCOME MEASURE: The main exposures of interest were a history of therapeutic head or neck irradiation and environmental radiation exposure. METHODS: A standardized questionnaire was administered to case and control subjects. Data were collected for the main exposures of interest as well as pertinent covariates. Conditional logistic regression was used to evaluate therapeutic and environmental radiation as risks for IPT. RESULTS: On univariate analysis, head or neck irradiation was associated with IPT (odds ratios [OR] = 4.15, 95% confidence interval [CI] = 1.30-13.24). While controlling for diabetes and family history of diabetes, IPT was found to be associated with both head or neck irradiation (OR = 4.06, 95% CI = 1.20-13.76) and with environmental irradiation (OR = 6.73, 95% CI = 1.06-42.74). CONCLUSIONS: This study presents a previously unreported association between prior radiation exposure and IPT.

Aged↗

Risk factors for hemiretinal vein occlusion: comparison with risk factors for central and branch retinal vein occlusion: the eye disease case-control study.

OBJECTIVE: Possible risk factors for hemiretinal vein occlusion were identified and compared with risk factor profiles for central and branch retinal vein occlusion. DESIGN: The design was a multicenter case-control study. METHODS: The authors identified 79 patients with hemiretinal vein occlusion (HRVO), 258 patients with central retinal vein occlusion (CRVO), 270 patients with branch retinal vein occlusion (BRVO), and 1142 control subjects at 5 clinical centers. Risk factor data were obtained through interviews, clinical examinations, and laboratory analyses of blood specimens. RESULTS: Systemic hypertension and history of diabetes mellitus were associated with increased risk of HRVO. Risk of CRVO increased with history of diabetes, systemic hypertension, and higher erythrocyte sedimentation rate (females only); risk of CRVO decreased with increasing amounts of physical activity and increasing amounts of alcohol consumption. Systemic hypertension, higher body mass index, and higher alpha2-globulin levels were associated with increased risk of BRVO, whereas higher high-density lipoprotein levels and increasing levels of alcohol consumption were associated with decreased risk of BRVO. Glaucoma history was associated with all three types of retinal vein occlusion. CONCLUSION: Patients presenting with retinal vein occlusion should be evaluated for cardiovascular disease, diabetes, and glaucoma.

Adult↗

Associations between siblings for esotropia and exotropia.

OBJECTIVE: To quantify familial aggregation of esotropia and exotropia in children examined in a large multicenter study. METHODS: Pregnant women and their children were examined in the Collaborative Perinatal Project of the National Institute of Neurological Disorders and Stroke, Bethesda, Md. Strabismus was evaluated in the children during follow-up examinations up to the age of 7 years. The second-order generalized estimating equations approach to logistic regression was used to estimate familial aggregation of esotropia and exotropia. RESULTS: For any pair of siblings, the odds for one sibling having esotropia more than doubled when the other sibling had esotropia. For exotropia, there were differences in sibling associations based on birth relationships. In particular, there was no statistically significant association between siblings from separate single births. On the other hand, for the pairs of siblings from multiple births (ie, twins, triplets, and quadruplets), the odds for exotropia in one sibling were increased by at least a factor of 17 when the other sibling from that birth also had exotropia. For both esotropia and exotropia, adjustment for previously identified risk factors only somewhat reduced the magnitudes of the observed associations. Limited data on zygosity showed a stronger association between monozygotic twins than between dizygotic twins. CONCLUSIONS: There is a significant familial component in the cause of strabismus. Furthermore, there are important contributions to this familial aggregation beyond those associated with known risk factors for strabismus.

Child↗

Studies on signal transduction mechanisms for adrenaline-driven lipolysis in white and brown adipocytes.

White and brown rat adipocytes have been permeabilised by repeated exposure of the cells in suspension to high voltage electrical discharges. The resulting preparations were permeable to low molecular weight materials, e.g., cyclic AMP, propidium iodide, and were stable in suspension with little evidence of rapid resealing, or of gross damage to the cell membrane. Leakage of lactate dehydrogenase was not markedly enhanced except at voltages in excess of 2 kV cm-1 for brown adipocytes. Exogenously-added cyclic AMP stimulated lipolysis (measured as glycerol release) in the electropermeabilised adipocytes far more effectively than in intact adipocytes. In brown, but not in white, adipocytes this effect was enhanced by addition of millimolar ATP. The EC50 for stimulation of glycerol release by cyclic AMP was 0.2 microM in electropermeabilised brown adipocytes, and 2 microM and 40 microM in electropermeabilised white adipocytes obtained from weanling and adult rats respectively. The effect of cyclic AMP on lipolysis was enhanced by addition of an inhibitor of cyclic AMP phosphodiesterases and was reduced by addition of 5'-AMP, adenosine or inosine (in brown adipocytes). Addition of adenosine deaminase caused a small, but significant, enhancement of cyclic AMP-driven lipolysis. Catecholamine-driven lipolysis was observed in electropermeabilised brown and white adipocytes, especially in the presence of GTP. Adrenaline-, and to a lesser extent cyclic AMP-, driven lipolysis in electropermeabilised white adipocytes was inhibited by insulin. This effect of insulin was not enhanced by addition of GTP or of a metabolically stable GTP analogue. The results obtained establish the electropermeabilised preparation as suitable for analysis of signal transduction pathways in white and brown adipocytes.

Adipose Tissue↗

Risk factors for esotropia and exotropia.

OBJECTIVE: To identify risk factors associated with the two major types of strabismus--esotropia and exotropia--in a cohort of children followed up from gestation to age 7 years. DESIGN: Pregnant women were enrolled in the Collaborative Project of the National Institute of Neurological Disorders and Stroke, Bethesda, Md, from 1959 to 1965 at 12 university centers. This large multidisciplinary study was designed to evaluate the developmental consequences of complications during pregnancy and the perinatal period. Data on maternal, socioeconomic, perinatal, and neonatal characteristics were collected from 39,227 children and their mothers by medical examination and interview. Examinations of the children were performed at birth, 4 months, 8 months, 1 year, and 7 years. OUTCOME MEASURES: The evaluation of the presence of strabismus was performed during follow-up examinations and confirmed at the 7-year follow-up visit. Potential risk factors for strabismus were evaluated from the maternal, socioeconomic, perinatal, and neonatal characteristics. RESULTS: Esotropia developed in 1187 children (3.0%), and exotropia developed in 490 children (1.2%). Esotropia was more common in whites (3.9% in whites vs 2.2% in blacks, P < .0001). The occurrence of exotropia was similar in the two races (1.2% in whites and 1.3% in blacks). Results of multivariable logistic regression models showed that the risk of strabismus increased with low birth weight (P < .0001). For infants weighing 1500 g at birth compared with those weighing 4000 g at birth, the odd ratios were 3.26 (95% confidence interval, 2.50 to 4.25) for esotropia and 4.01 (95% confidence interval, 2.77 to 5.80) for exotropia. Maternal cigarette smoking during pregnancy also increased the risk of each type of strabismus (P < .0001). For offspring of mothers who smoked more than two packs of cigarettes per day compared with those whose mothers did not smoke, the odds ratios were 1.83 (95% confidence interval, 1.51 to 2.22) for esotropia and 2.32 (95% confidence interval, 1.72 to 3.13) for exotropia. Maternal age was also a significant risk factor for esotropia (P = .0005). The risk of esotropia increased with increasing age until age 34 years. In particular, the odds ratio for mothers aged 30 to 34 years relative to that for mothers aged 20 to 24 years was 1.43 (95% confidence interval, 1.19 to 1.70). CONCLUSIONS: Esotropia was more common in whites than in blacks. The occurrence of exotropia was similar in the two races. Maternal cigarette smoking during pregnancy and low birth weight were independent and important risk factors for both esotropia and exotropia. There was an increased risk of esotropia with increasing maternal age.

Adult↗

Screening for von Hippel-Lindau disease by DNA polymorphism analysis.

OBJECTIVE: Von Hippel-Lindau (VHL) disease is a rare, inherited multisystem neoplastic disorder. There is no biochemical test available to distinguish VHL disease gene carriers from their healthy siblings. We evaluated DNA polymorphism analysis as a method for identifying disease gene carriers. DESIGN: Prospective comparison of the results of DNA analysis with a comprehensive clinical screening examination. SETTING: The Clinical Center of the National Institutes of Health. PATIENTS: Blood was collected from 182 members of 16 families with VHL disease. Forty-eight asymptomatic individuals, at risk of developing this hereditary illness (with an affected parent or sibling), were examined for occult disease at the Clinical Center of the National Institutes of Health and tested by DNA polymorphism analysis. RESULTS: DNA polymorphism analysis predicted nine disease gene carriers and 33 individuals with the wild-type (normal) allele among the 48 individuals at risk of developing VHL disease; the test was not informative in six individuals. All nine individuals predicted to carry the VHL gene had evidence of occult disease on clinical examination. There was no clinical evidence of VHL disease in 32 of 33 individuals predicted to carry the wild-type allele. CONCLUSIONS: DNA polymorphism analysis can identify individuals likely to carry the VHL disease gene among asymptomatic members of disease families. This technique serves to focus attention on those individuals who require periodic medical examination and may help to alleviate the morbidity and mortality associated with this disease.

DNA↗

Foscarnet therapy of cytomegalovirus retinitis in AIDS.

Cytomegalovirus (CMV) retinitis is the most common cause of blindness in AIDS. Twenty patients were treated with a 21-day course of foscarnet therapy by continuous infusion. Response to therapy was good in eight (47%) of 17 evaluable patients; partial arrest of progression was observed in eight (47%); and no response was obtained in one (6%). Foscarnet therapy did not lead to suppression of urinary excretion of CMV in four of 12 patients who nonetheless had improvement in retinal lesions. Toxic effects, especially reversible renal failure, were common, with blood creatinine increase in 50% and dialysis in two patients. Renal toxicity occurred primarily during the third week of therapy. Anemia (hemoglobin less than 80 g/L) occurred in 10 patients after a mean of 14.5 +/- 5.1 days of therapy and required transfusion. Review of this study and of data from a previous case series, however, was inconclusive regarding the additional benefit of a third week of therapy. Maintenance therapy was given to seven patients. Four had recurrence of CMV retinitis at a mean interval of 62 +/- 52 days. Only one patient has maintained prolonged remission on maintenance (greater than 24 weeks). Toxicity on the maintenance protocol included anemia (two of seven patients) and increased creatinine blood levels (one of seven patients). Zidovudine therapy in six patients did not contribute to increased toxicity of induction or maintenance therapy. Drug levels during continuous infusion were stable for individual patients but showed wide inter-patient variability. Peak levels of post-maintenance infusion varied both within and between patients.

Acquired Immunodeficiency Syndrome↗

Growth hormone suppression and nonproliferative diabetic retinopathy: a preliminary feasibility study.

We used the long acting somatostatin analogue SMS 201-995 in order to examine the feasibility and effect of medical suppression of growth hormone in nonproliferative diabetic retinopathy. Six insulin-dependent diabetic subjects with nonproliferative retinopathy were studied. After eight weeks of SMS 201-995 administration, 24-hour integrated plasma growth hormone concentrations had declined by 47.0 +/- 9.3% of pretreatment values (p less than 0.01), and insulin requirements fell from 40.7 +/- 6.7 units per day to 32.2 +/- 6.9 units per day (p less than 0.01). Plasma levels of somatomedin-C were low before SMS 201-995 (0.5 +/- 0.1 U/ml) and remained unchanged at eight weeks (0.6 +/- 0.1 U/ml; p = ns). During SMS 201-995 administration, best corrected visual acuity improved in both right eyes (53.8 +/- 2.57 to 59.8 +/- 0.7 letters, p less than 0.05) and left eyes (54.8 +/- 2.8 to 61.7 +/- 1.23 letters, p less than 0.03). Fluorescein angiography and stereo fundus photography demonstrated concurrent improvement in retinopathy level in only two subjects. Following cessation of SMS 201-995 treatment, visual acuity returned to pretreatment levels in both right eyes (54.5 +/- 2.4 letters, p = ns compared to baseline) and left eyes (55.8 +/- 2.6 letters, p = ns compared to baseline). These results demonstrate that growth hormone was only partially suppressed by SMS 201-995 in insulin-dependent diabetic subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Hyperviscosity syndrome in a patient with acquired immunodeficiency syndrome.

The hyperviscosity syndrome is most commonly seen in association with monoclonal gammopathies and has only rarely been described in association with polyclonal hypergammaglobulinemia. We have recently seen a patient with known acquired immunodeficiency syndrome who presented with the hyperviscosity syndrome in the setting of polyclonal hypergammaglobulinemia. To our knowledge, this is the first reported case of a patient with the acquired immunodeficiency syndrome and the hyperviscosity syndrome. The case is presented and the pathogenesis and implications of this diagnosis are discussed.

Acquired Immunodeficiency Syndrome↗

Visual and auditory neurotoxicity in patients receiving subcutaneous deferoxamine infusions.

Of 89 patients receiving nightly subcutaneous deferoxamine for transfusion-dependent thalassemia major or Diamond-Blackfan anemia, 13 presented with visual loss or deafness of acute onset or both. Detailed ophthalmologic, audiologic, and evoked-potential studies uncovered abnormalities caused by neurotoxicity in 27 more. Four patients with visual loss had optic neuropathy, with a marked decrease in acuity, loss of color vision, and delayed visual evoked potentials. Five asymptomatic patients had changes in the pigment of the retinal epithelium. The hearing loss was characterized by a high-frequency sensorineural deficit, which necessitated hearing aids in six patients. When deferoxamine was stopped, recovery of vision was complete in 2 patients and partial in 2, and in 22 patients with abnormal audiograms, reversal of the hearing deficit was complete in 4 and partial in 1. An analysis of the clinical data showed that members of the affected group were younger, had lower serum ferritin values, and were self-administering higher doses of deferoxamine per kilogram of body weight. Significantly lower doses of deferoxamine were being taken by patients without abnormalities than by those with visual symptoms, abnormal audiograms, or prolonged evoked potentials (P less than 0.001, less than 0.006, and less than 0.04, respectively). The data implicate high-dose deferoxamine as a central factor in the pathogenesis of the neurotoxicity. We strongly recommend careful regulation of the deferoxamine dosage and serial audiovisual monitoring in all patients receiving the drug.

Adolescent↗

Yellowish flecks in Leber's congenital amaurosis.

The fundus abnormalities of Leber's congenital amaurosis are extremely variable, from normal to salt-and-pepper changes to typical retinitis pigmentosa. A less commonly seen appearance is that of multiple, irregular shaped, yellowish white flecks deep in the midperipheral retina in a periarteriolar distribution. The nasal fundus as well as the posterior pole are spared. Such a case is presented along with a four-year follow-up together with the fluorescein angiographic findings. The flecks appear to be specific for this entity.

Blindness↗

Glaucoma in children.

Successful management of glaucoma to prevent blindness depends upon early recognition of the condition. This article details the early signs and symptoms of glaucoma so that prompt referral for care and treatment may be facilitated.

Abnormalities, Multiple↗

Amiodarone-induced cornea verticillata.

Among 37 patients treated with amiodarone, an antiarrhythmic drug, a typical keratopathy developed in 35, none of whom had ocular complaints. The keratopathy resembled that seen with Fabry's disease and chloroquine use, as did the membrane-bound lamellar bodies detected by electron microscopy in all layers of the corneal epithelium in the one patient with marked keratopathy in whom a corneal biopsy was performed; membrane-bound bodies, mostly granular, were also noted within this patient's stromal keratocytes. The possible pathogenesis of the keratopathy as a type of drug-induced lipidosis is discussed.

Adult↗

Effect of CT noise on detectability of test objects.

Three types of test objects were superimposed on noisy backgrounds and observed by 58 subjects: large low-contrast disks to simulate tumors, small disks to simulate calcifications, and bars to simulate blood vessels. Three types of noise were used: random noise, CT noise (reconstructed random noise), and an actual water scan taken with an EMI CT1010 scanner. In addition, each image was smoothed by a factor of two, giving six test patterns altogether. Photographic images were presented to 25 lay observers, while 25 other lay observers and eight radiologists used a viewing console. The photographs produced a slightly lower true positive rate than the console, although the degree of confidence in the identifications was slightly greater. The anticorrelation property of CT noise gives it a "lumpy" structure, which aided the identification of bars but impeded the identification of small disks. Smoothing did not significantly affect the detectability with random noise, but with CT noise, smoothing improved the detectability of bars and large disks.

Humans↗

The apical artifact: elevated attenuation values toward the apex of the skull.

An artifact that can cause an elevation in CT numbers of as much as 20 Hounsfield units toward the apex of the head is described. This artifact was first noted on the EMI CT1010 scanner but has been observed in other scanners as well. Phantom studies of this artifact have revealed that its primary origin is the beam hardening distortion due to the large amount of bone present in apical cuts. This has been verified by computer simulations. An intuitive explanation of the origin of the artifact is also presented.

Humans↗

Patient satisfaction.

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Consumer Behavior↗