Successful therapy for trilateral retinoblastoma.
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Biomedical subjects
Publications and source records attributed to D H Abramson.
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Serial ultrasonic measurements performed on 85 melanoma patients who were treated with radioactive plaques and followed for up to 141 months revealed that no 2 patients had identical patterns of change. Eighty-two of the patients were categorized into one of four patterns after treatment: Type D (decrease in height, 57 patients), Type S (same height, 12 patients), Type I (increase in height, 9 patients), and Type RG (tumour regrowth, 4 patients). The calculated mean total change in tumor height for Type D was 1.92 mm at 6 months, 2.58 mm at 12 months, 2.97 mm at 24 months and 3.61 mm at 48 months. For Type I, the mean total change in tumor height was 0.80 mm at 6 months, 0.95 mm at 12 months, 2.28 mm at 24 months, and 3.0 mm at 48 months.
Children diagnosed with retinoblastoma in the first year of life present with differences in laterality, stage, signs, symptoms, and respond differently to treatments when compared to older children. Of those children diagnosed in the first year of life (between 1958 and 1983 inclusive), 280 were bilateral and 121 were unilateral, the most common stage of diagnosis (for unilateral and bilateral) was Group V, and children were affected equally by sex. Children examined in the first three months of life were more often seen because of a positive family history of retinoblastoma, rather than leukocoria. Children who were treated in the first year of life frequently develop second nonocular tumors because they harbor the germinal mutation and receive radiation.
Choroidal melanoma is a rare and deadly cancer. Nursing care for these patients involves ophthalmic clinical expertise as well as psychosocial and emotional support. Although past research has been helpful in determining the prognosis of these patients, it is impossible to predict with certainty which patients have tumors or nevi that will grow, will compromise their vision, or will kill them. Providing emotional support, establishing a good rapport with the patients and their support persons, and encouraging patients to schedule and adhere to their mutually agreed on follow-up appointments are nursing interventions that may save or prolong a patient's life.
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Lactate dehydrogenase (LDH) activity was determined in 24 cases of histologically proved retinoblastoma. The mean level was 1,825 IU/L, with a range of 10 to 15,920 IU/L. Twenty-two patients had elevated aqueous humor LDH levels. In two, the aqueous humor level was within the normal range. Student's t testing showed a highly significant difference between cataract and retinoblastoma aqueous humor. The retinoblastoma aqueous humor to blood ratio ranged from 0.045 to 58,529--not a good index of retinoblastoma. Isozyme patterns of retinoblastoma aqueous humor were statistically similar to those of normal cataract aqueous humor. The serum LDH level of cataract patients was not statistically different from that of retinoblastoma patients. The LDH 5/LDH 1 ratio of retinoblastoma aqueous humor ranged from 0.042 to 27.05--not a good index of retinoblastoma, as 28 of 34 aqueous samples of cataract patients had LDH 5 greater than LDH 1. Ten of 13 retinoblastoma patients had aqueous humor LDH 5 greater than LDH 1. The best index of the presence or absence of retinoblastoma is the total aqueous humor LDH level.
We reviewed the records of 1,323 patients with retinoblastoma treated here between 1922 and 1978. Twenty-eight patients underwent bilateral enucleation alone and received no additional treatment for their advanced tumors. Of the 28 patients, 22 (92%) survived. Four patients were lost to follow-up, and two died of metastatic retinoblastoma. Of 22 survivors of bilateral retinoblastoma treated with enucleation alone, three developed second tumors at a mean of 15.3 years after bilateral enucleation. One patient developed a rhabdomyosarcoma in the left temple region, the second patient developed an osteogenic sarcoma of the right femur, and the third developed a malignant melanoma of the left thigh. All three patients died of their disease. The incidence of second tumors in patients who survived bilateral retinoblastoma treated without radiation was 14% (three patients), which is comparable to a series previously reported in which patients were treated with enucleation and irradiation.
Fifty-eight patients with orbital rhabdomyosarcoma were treated with irradiation alone (25) or irradiation and chemotherapy (33) with follow-ups of 6 months to 14 years (mean 5.2 yr). At present, 74% are alive and 26% have died. Local control of the tumor was accomplished in 91% of cases. When local sinuses were invaded the survival rate was 55%. Chemotherapy appears to be of greatest value when disease is limited to the orbit. Irradiation or irradiation and chemotherapy should now be the treatment of choice for orbital rhabdomyosarcoma.
Transconjunctival cryopexy was applied to a malignant melanoma of the ciliary body and choroid of a 46-year-old white man. The tumor immediately became gray-white and pigment cells were liberated in the anterior chamber. Subsequently, an enucleation was performed which showed a localized area of cryodestruction of the melanoma which was lined by macrophages. The significance of this finding in implications for the use of cryopexy in the management of melanomas are discussed.
Aqueous humor lactate dehydrogenase (LDH) levels were assayed for 23 patients with histologically proved retinoblastoma. The mean aqueous level was 1,886 IU/liter. Twenty-one of these patients had an aqueous humor LDH level above the normal mean. Clinicopathological parameters were determined and evaluated using standard statistical formulas. Only the presence of tumor in the anterior chamber correlated significantly with the aqueous humor LDH level. The following clinical features did not correlate with aqueous humor LDH levels: sex, family history, bilaterality, prior treatment, presentation age, enucleation age, and metastasis. The following pathological features did not correlate with aqueous humor LDH levels: calcification, necrosis, rosettes, inflammation, choroidal or optic nerve invasion, or neovascularization.
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Serum and aqueous humor lactate dehydrogenase (LDH) levels were assayed in 46 patients with senile cataracts at the time of cataract extraction. The mean aqueous level was 37 IU/liter and the mean serum level 152 IU/liter. In each case, the serum LDH level was higher than the corresponding aqueous LDH. The LDH isoenzyme levels were also determined; LDH 4 and LDH 5 were elevated in cataract aqueous samples, while the serum isoenzymes were normal. The LDH studies of normal and diseased globes must be rigorously standardized to avoid artificially high or low levels.
A 59-year-old women with no previous history of diabetes mellitus was admitted in diabetic ketoacidosis. Ultrasonic biometry was used to measure axial vitreous length during therapy. Vitreous length increased from 14.09 mm shortly after admission to 14.26 mm (p less than 0.05) when the episode of diabetic ketoacidosis cleared. An interesting finding was the presence of a horseshoe-shaped retinal tear after the patient was discharged. Severe dehydration with resultant shrinkage of the vitreous compartment may provide sufficient vitreoretinal traction to cause a retinal tear. In addition, an estimate of volume change showed a comparable decrease in vitreous volume seen with therapy using osmotic agents measured in rabbits.
The effect of pilocarpine hydrochloride on the anterior chamber depth and lens thickness was measured in 20 eyes of patients with chronic open-angle glucoma who were receiving long-term glaucoma therapy with pilocarpine. Measurements were made with high-resolution ultrasonic biometry. This study demonstrated that regardless of their age (58.4 years, average) and the fact that they had been regularly using pilocarpine from two to four times a day, 85% of these patients demonstrated narrowing of the anterior chamber (AC) depth (average, 0.19 mm) and thickening of the lens (average, 0.21 mm) with each instillation of pilocarpine. In approximately 15% of eyes, a repeatable deepening of the AC depth and flattening of the lens was noted.
The velocity of sound determinations made in this study are the result of measurements accurate to 0.5 m/sec. The velocity of sound in a series of 50 cataractous lenses was found to be an average of 1,629 m/sec, 11 m/sec slower than that of normal adult lenses. The velocity of sound found in a smaller series of child lenses was 1,659 m/sec, 30 m/sec faster than the cataractous adult lens. It is postulated that this represents a trend of decreasing velocity of sound in the lens with increasing age.
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The aqueous humor of five patients with histologically proven retinoblastoma were analyzed for the main catabolic products of norephinephrine and epinephrine. In each case, there were no detectable levels of normethanephrine, metanephrine, vanilmandelic acid (VMA), and 3-methoxy, 4-hydroxy phenoglycol. The techniques are sensitive to 1 microgram/ml but will not detect the presence of homovanillic acid (HVA).