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Biomedical subjects

M F Goldberg

Publications and source records attributed to M F Goldberg.

At least 163 records · Page 9Linked to original sources

Talc emboli and retinal neovascularization in a drug abuser.

A 38-year-old male drug abuser had multiple emboli in the retinal circulation of the posterior pole of both eyes. He showed widespread peripheral retinal capillary nonperfusion and neovascular proliferation at the junction of perfused and nonperfused retina. The emboli were considered to be talc particles from the intravenous administration of suspensions of oral medications. The presumed mechanism of development of neovascularization in this case was the filtering out of the particles by the retinal vasculature with vaso-occlusion, ischemia and subsequent retinal neovascularization.

Adult↗

The diagnosis and treatment of secondary glaucoma after hyphema in sickle cell patients.

Four patients with sickle cell hemoglobinopathies (one sickle cell hemoglobin C disease (SC); three sickle cell trait (AS)) and hyphemas had a higher percentage of erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure (IOP) was severely increased, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate increase of IOP in sickle cell hemoglobinopathy patients may produced rapid deterioration of visual function, because of a greater than usual effect on vascular perfusion in the central retinal artery and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.

Acetazolamide↗

Vascular anastomoses between the iris and persistent hyperplastic primary vitreous.

Prominent radial iris blood vessels created small but definite pupillary notches in five children with persistent hyperplastic primary vitreous and persistence of the tunica vasculosa lentis. Detection of these abnormal iris vessels suggested the presence of persistent hyperplastic primary vitreous and related syndromes even when the diagnosis was obscured by the presence of an opaque lens. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve. Surgical and amblyopia therapy, therefore, may not provide useful vision, and should be undertaken with caution.

Abnormalities, Multiple↗

Failed intrauterine device contraception and limb reduction deformities: a case-control study.

Three recent case reports have suggested an association between failed intrauterine device (IUD) contraception and limb reduction deformities in the resulting offspring. To clarify further the purported teratogenic role of the IUD in the etiology of these defects, we conducted a case-control study of 96 mothers who had given birth to infants with limb defects. Interview data about IUD exposure at conception among these mothers were compared with interview data from 2 sets of controls: (1) 915 mothers of infants with other major defects and (2) a subset of the first group consisting of 169 mothers with chromosomally defective infants. No significant increase in the incidence of IUD use was found for cases when compared with either control group. Estimates of the relative risk were 1.28 (95% confidence limits [CL]of 0.29 to 5.67) and 1.78 (95% CL of 0.25 to 12.82) when the first and second groups of controls were used, respectively. These risk figures were not significantly different from 1.0. The evidence accumulated thus far militates against a teratogenic role for the IUD.

Clinical Trials as Topic↗

Early detection of retinal involvement in diabetes by vitreous fluorophotometry.

Vitreous fluorophotometry is a new quantitative method for evaluation of the blood-retinal barrier (BRB). The application of this method to a series of diabetic patients with apparently normal fundi revealed the presence of a significant breakdown of the BRB in the early stages of retinal involvement in diabetes. This alteration of the BRB appears to be the earliest clinically detectable change to occur in the retina in diabetes. Higher vitreous fluorophotometry values, indicating a more marked breakdown of the BRB, occurred in patients who were under worse metabolic control. Higher vitreous fluorophotometry values were also associated with the development of visible retinal lesions.

Diabetes Mellitus↗

Neuraminidase deficiency in the original patient with the Goldberg syndrome.

Homogenates of cultured skin fibroblasts from a non-ambulatory, 20-year-old male with cherry-red spots, corneal clouding, seizures, mental retardation, dysostosis multiplex, dwarfism, coarse facies and loss of vision, originally described by Goldberg et al. (1971), have diminished neuraminidase activity and an excess of neuraminic acid-rich compounds. Specifically, these cells have 2-17% normal neuraminidase when measured with 2-(3' methoxyphenyl)-N-acetyl-alpha-neuraminic acid, N-acetyl-neuramin-lactose and fetuin. Activities of 12 other lysosomal enzymes were either at or above the range of normal control fibroblasts. Total neuraminic acid concentration was 44.3 nmol/mg protein versus an average control value of 14.2. It is concluded that the Goldberg syndrome should be considered, along with mucolipidosis I and the cherry-red spot -- myoclonus syndrome, as resulting from a primary neuraminidase deficiency.

Abnormalities, Multiple↗

Management of subluxated and dislocated lenses with the vitrophage.

Thirty-two eyes with subluxated and disclosed lenses were surgically managed by means of the vitrophage. The best results were obtained for those cases in which subluxation or dislocation of lenses into the vitreous was not associated with other complications such as glaucoma, perforating injuries, and retinal detachment. No major intraoperative or postoperative problems were seen in uncomplicated cases during a follow-up period ranging from 5 to 45 months.

Adolescent↗

Cryotherapy of proliferative sickle retinopathy, II: triple freeze-thaw cycle.

Complete closure of retinal neovascularisation due to proliferative sickle cell retinopathy was achieved in 28 'sea fans' (areas of neovascularisation) in 9 eyes of 9 patients by means of a triple freeze-thaw technique. However, 2 eyes developed subsequent rhegmatogenous retinal detachments, presumably related to vitreous traction on necrotic retina. We therefore recommend photocoagulation as the best treatment of proliferative sickle cell retinopathy. If opacities in the media prevent photocoagulation, a single freeze-thaw cycle is preferable.

Adolescent↗

Sickled erythrocytes, hyphema, and secondary glaucoma: II. Injected sickle cell erythrocytes into human, monkey, and guinea pig anterior chambers: the induction of sickling and secondary glaucoma.

Injection of sickle cell erythrocytes (SS, SC, Sthal, and AS) into the anterior chambers of living human, monkey, and guinea pig eyes resulted in conversion of erythrocytes to the sickled configuration. Immune responses did not appear to play an important role in influencing this phenomenon. The number of cells sickled was directly related to the concentration of hemoglobin S. In human and monkey eyes, the severity of elevated IOP appeared to be related to the type of cells injected and their concentration of hemoglobin S. Even the ordinarily benign genotype of sickle cell trait demonstrated sickling in the anterior chamber and elevation of the IOP.

Aged↗

Sickled erythrocytes, hyphema, and secondary glaucoma: VII. The passage of sickled erythrocytes out of the anterior chamber of the human and monkey eye: light and electron microscopic studies.

Sickled erythrocytes leave the anterior chamber of human and monkey eyes by several mechanisms: intracameral and intratrabecular hemolysis and phagocytosis; entry into and phagocytosis within the iris and ciliary body; and passage into Schlemm's canal. Sickled erythrocytes used as biologic tracers allowed definite confirmation, for the first time in the human, that intracameral erythrocytes can enter Schlemm's canal in the orthograde direction. Erythrocytes appeared to traverse the inner wall of Schlemm's canal through the cytoplasm of individual endothelial cells, rather than between adjacent endothelial cells -- a mechanism similar to that employed by red and white blood cells elsewhere in the body. Although some sickled erythrocytes did pass into Schlemm's canal, considerable obstruction appeared to occur at the corneoscleral meshwork, juxtacanalicular connective tissue, and inner wall of Schlemm's canal, caused largely by the elongated, rigid nature of the sickled cells. Total blockage of Schlemm's canal was not observed.

Anemia, Sickle Cell↗

Dominantly inherited cystoid macular edema.

Four patients of Greek ancestry had dominantly inherited cystoid macular edema. Characteristics of this syndrome include the following: an early onset and prolonged course of cystoid changes in the macula, followed by atrophy of the macula in later stages. Some patients also show leakage of fluorescein from the optic disc capillaries, subnormal EOG Lp/Dt ratios, elevated rod dark adaptation thresholds, red-green and blue-yellow color deficiencies, normal ERG findings, hyperopia, peripheral pigmentary retinopathy, and vitreous opacities. Dominantly inherited cystoid macular edema is a distinct genetic trait among the dominantly inherited macular dystrophies.

Adult↗

Sickled erythrocytes, hyphema, and secondary glaucoma: I. The diagnosis and treatment of sickled erythrocytes in human hyphemas.

Four patients with sickle cell hemoglobinopathies (one SC, three AS) and hyphemas were found to have more erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure was severely elevated, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate elevation of IOP in sickle cell hemoglobinopathy patients may produce rapid deterioration of visual function, perhaps because of a greater than usual effect on vascular perfusion in the central retinal artery and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.

Adolescent↗

Sickled erythrocytes, hyphema, and secondary glaucoma: III. Effects of sicle cell and normal human blood samples in rabbit anterior chambers.

When injected into rabbit anterior chambers, human sickle cell erythrocytes cause more intense and prolonged effects than do nonsickling erythrocytes. The consequences of injected sickle cell trait erythrocytes are not less severe than those of injected SC, SS, and Sthal erythrocytes. Regardless of hemoglobin composition, blood with a higher hematocrit value causes more severe complications than blood with a lower hematocrit value.

Anemia, Sickle Cell↗

Sickled erythrocytes, hyphema, and secondary glaucoma: IV. The rate and percentage of sickling of erythrocytes in rabbit aqueous humor, in vitro and in vivo.

The number of erythrocytes adopting the sickled configuration in rabbit aqueous was determined with a masked counting technique in vitro and in vivo. The percentage of cells that became sickled in aqueous humor was greater than that observed in a control salt solution and in the donor blood itself. Aqueous humor, therefore, can be considered a particularly deleterious medium for erythrocytes having a propensity for sickling. Blood samples from six donors with systemically severe hemoglobinopathies (SC, Sthal, and SS) had relatively high levels of hemoglobin S and, in general, showed faster and more frequent sickling than did blood samples from donors with low levels of hemoglobin S (one SS patient, whose chronic blood transfusion regimen had replaced most of his hemoglobin S with normal hemoglobin, and two AS patients). Even erythrocytes from sickle cell trait cases, however, were capable of sickling when immersed in acqueous humor.

Anemia, Sickle Cell↗

Sickled erythrocytes, hyphema, and secondary glaucoma: V. The effect of vitamin C on erythrocyte sickling in aqueous humor.

In vivo incubations of Hgb SC erythrocytes showed significantly more sickling in anterior chambers characterized by high concentrations of ascorbic acid than in anterior chambers with low concentrations of ascorbic acid (normal guinea pigs compared with scorbutic guinea pigs and normal guinea pigs compared with normal rats). Low concentrations of ascorbic acid, however, did not completely eliminate intracameral sickling. Because acetazolamide raises the concentration of aqueous humor ascorbate, it should be used with considerable discretion when treating hyphema and secondary glaucoma caused by sickle cells. Methazolamide may be more desirable.

Acetazolamide↗

Four hundred consecutive pars plana vitrectomies with the vitrophage.

We performed 400 consecutive pars plana vitrectomies, using the vitrophage. Most patients (68%) showed some degree of visual improvement; only 8% had a decline in visual acuity postoperatively. The major operative complications were controllable bleeding from iris vessels and intravitreal stalks (19%) and retinal tears (2%). No retinal dialysis was noted. The major, early postoperative complications were related to corneal decompensation in 38% of the cases; this persisted beyond two weeks in only 7%. Increased intraocular pressure occurred in 19% and postoperative rubeosis iridis developed in 10% of the diabetic patients. Complications related to rebleeding occurred in 18% of the diabetic patients within the first two weeks, and an additional 9% had late rebleeding. Less frequent complications included one case of endophthalmitis, late retinal detachment in 5%, and phthisis bulbi in 2% of all cases. All complications were considerably more frequent and severe in the diabetic population.

Anesthesia, Local↗