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Biomedical subjects

S Brownstein

Publications and source records attributed to S Brownstein.

At least 73 records · Page 4Linked to original sources

Leber's congenital amaurosis with Duchenne's muscular dystrophy.

We studied a family with Leber's congenital amaurosis and Duchenne's muscular dystrophy. One son, the proband, was noted to be blind from shortly after birth and had absent pupillary reflexes, attenuation of retinal blood vessels and pigmentary changes of the retina. He manifested Duchenne's muscular dystrophy at 6 years of age and died of its complication--severe respiratory failure--at 21 years of age. His older brother also was blind since birth, with absent pupillary light reflexes and an extinguished electroretinogram in both eyes. The proband's younger brother with Duchenne's muscular dystrophy died following respiratory failure. Their oldest brother was apparently unaffected. Their mother and sister were proven carriers of Duchenne's muscular dystrophy and a maternal uncle and great-uncle also died from this condition. The parents were consanguineous, third generation cousins.

Adolescent↗

A clinicopathological study of seven globes enucleated after primary radiation therapy for malignant melanoma of the choroid or ciliary body.

Seven patients with malignant melanoma of the choroid or ciliary body were initially managed with radiation therapy, either teletherapy with proton or cobalt 60 external beam, or brachytherapy with the insertion of an episcleral plaque containing iodine 125, 60Co or gold 198. All seven globes subsequently were enucleated because of progressive growth of the tumours, total retinal detachment, persistent vitreous hemorrhage, or neovascular or angle-closure glaucoma. Six of the patients had severe visual loss at the time of enucleation. The effects of radiation therapy on the neoplasms and the mechanisms for the visual loss are discussed.

Adult↗

Sarcoidosis of the eyelid skin.

A 64-year-old white woman presented with nodular lesions on the arms and face, including both medial canthi and the left upper eyelid. Histopathological examination of the biopsied eyelid lesion and other facial lesions disclosed the presence of noncaseating granulomas strongly suggestive of sarcoidosis. No other evidence of ocular sarcoidosis was present. The patient had clinical, radiologic and laboratory findings consistent with a diagnosis of systemic sarcoidosis. Therapy with chloroquine phosphate led to regression of the cutaneous lesions.

Biopsy↗

Transgenic mice carrying the mouse mammary tumor virus ras fusion gene: distinct effects in various tissues.

Transgenic mice carrying the v-Ha-ras oncogene under the control of the mouse mammary tumor virus long terminal repeat were produced. These mice exhibit several phenotypes: mammary tumors, bilateral hyperplasia of the harderian lacrimal gland, primary bronchio-alveolar lung adenocarcinoma, and splenomegaly. High levels of the transgene RNA were detected in mammary, harderian, and lung tumors. Accumulation of cells of the myeloid lineages was found in enlarged spleens. This phenotype may represent an indirect effect of v-Ha-ras expression on myeloid progenitors. Our data illustrate the cell-specific effects of v-Ha-ras.

Animals↗

Ocular complications of a topical methylene blue-vasoconstrictor-anesthetic preparation.

We describe six patients who demonstrated a spectrum of adverse ocular reactions following the use of a nonprescription topical medication composed of methylene blue, naphazoline hydrochloride or nitrate, and amylocaine hydrochloride. This preparation has been commonly sold in Quebec and France for various complaints of ocular discomfort and redness. The ocular complications included asymptomatic blue discoloration of the conjunctiva, follicular and papillary conjunctivitis and varying degrees of cicatrization of the conjunctiva and cornea. The most severe reaction, progressive bilateral ocular pseudopemphigoid, occurred in a 73-year-old woman, presumably due to at least one of the ingredients of this medication.

Adult↗

Fibrous histiocytoma of the corneoscleral limbus and conjunctiva.

An 11-year-old boy and a 28-year-old woman each had a painless, slowly enlarging, elevated, yellow lesion of the inferior corneoscleral limbus and adjacent cornea and conjunctiva of the left eye. Histopathologic examination of both lesions disclosed involvement of the conjunctival substantia propria and the adjacent limbal and peripheral corneal stroma by a nonencapsulated, dense infiltrate composed of elongated fibrocytic cells, plump histiocytic cells occasionally with a vacuolated cytoplasm, some multinucleated giant cells, and scattered lymphocytes. The diagnosis in each case was fibrous histiocytoma. Both patients were otherwise in good health with no dermatologic, systemic, or other ocular abnormalities.

Adult↗

Cryotherapy for conjunctival primary acquired melanosis and malignant melanoma. Experience with 62 cases.

Sixty-two patients were treated by some combination of cryotherapy and surgery with an average follow-up of 3.3 years for one of the following diseases: focal or diffuse flat conjunctival primary acquired melanosis (PAM) with atypia but without a nodule of melanoma (10 cases); unifocal malignant melanoma with or without focal or diffuse PAM (30 cases); and multinodular/multicentric melanoma with and without PAM (22 cases). Of the ten patients who had PAM with atypia, invasive nodules of malignant melanoma did not develop. A second treatment was required to control the disease in four of the ten patients with extensive or diffuse lesions, and one has mild persistent disease. Of the 30 patients with unifocal nodules of malignant melanoma, 27 remained free of recurrence after one treatment, and 2 are asymptomatic after two treatments. One patient with a thick nodule at presentation required a parotidectomy and radical neck dissection for cervical metastases after recurrence in the conjunctival sac. In the group of 22 patients with multinodular malignant melanoma, only two did not have recurrent disease after one treatment. Of those who received multiple therapies, seven remained free of recurrence for at least 2 years after the last treatment; regional or distant metastases developed in nine; four required exenteration; and eight died. Conjunctival adjunctive cryotherapy avoids exenteration in extensive lesions of pure PAM and in unifocal melanoma, but even after multiple therapies, multinodular malignant melanoma had a 45% rate of metastasis. Metastasis was related to the presence of PAM sine pigmento in four patients (microscopically but not clinically detectable PAM); to the location of the nodules (9 of 10 patients who experienced metastases had forniceal, palpebral, and/or caruncular nodules); to the thickness or depth of invasion of the nodules (greater than 2 mm); and to the development of intralymphatic spread ("in-transit" local metastasis) within the conjunctival sac in six patients. No metastases were encountered among patients with strictly limbal nodules and among five patients with invasive nodules composed of spindle cells in part or in toto. Therapeutic success in this spectrum of melanocytic proliferations is closely correlated with the clinical extent of the disease when initiating definitive therapy.

Adult↗

Carbon dioxide laser treatment of the conjunctiva and the cornea.

Carbon dioxide (CO2) laser treatment was applied to the cornea and conjunctiva of 16 eyes from eight young pigs at power levels of 2.5 to 20.0 W, an exposure time of 0.1 seconds and different numbers of overlapping burns. Four animals were euthanatized at 30 minutes, one at 36 hours and three from 18 to 21 days after the treatment. Nitrous oxide (N2O) cryotherapy also was applied in a double freeze-thaw fashion to three of the eyes to compare the effects of the CO2 laser and cryotherapy at each of the time intervals. Epithelial destruction was achieved with CO2 laser power levels of 5.0 W and one or more superimposed burns. At 5.0 W, Bowman's membrane was destroyed while the conjunctival substantia propria protected the underlying sclera. The anterior chamber reaction was minimal and appeared to be less intense than with cryotherapy after 36 hours. At high energy levels (20.0 W), corneal and scleral perforation occurred. Furthermore, we have treated five human patients with the CO2 laser for bulbar conjunctival epithelial proliferative disorders. Minimal scarring occurred and local control of the disease was obtained in four patients. The CO2 laser may offer advantages over other modalities of treatment for bulbar conjunctival epithelial disorders. Its use is not recommended for lesions of the cornea and forniceal or palpebral conjunctiva.

Adult↗

Mucinous eccrine sweat gland adenocarcinoma of the eyelid.

A firm, slow-growing cystic mass of the left upper eyelid developed in a 73-year-old man 5 years after a clinically diagnosed "sebaceous cyst" at the same site had been incised and drained. Histopathological examination disclosed a primary mucinous adenocarcinoma of eccrine sweat gland origin. The patient had no recurrence after 3.5 years of follow-up. Histochemical demonstration of sialomucin differentiates mucinous sweat gland carcinoma from other mucus-secreting adenocarcinomas, such as those arising from lacrimal gland tissue. Enzymatic histochemical analyses or ultrastructural studies are needed to distinguish between mucinous adenocarcinomas of eccrine origin and those of apocrine origin.

Adenocarcinoma, Mucinous↗

Histopathological findings in blepharopigmentation (eyelid tattoo).

We treated three patients with implantation of iron oxide pigment into the margin of the lateral eyelid followed by excision of that region 1 hour later in one patient, 5 days later in the second and 18 months later in the third. Histopathological examination of the specimens obtained 1 hour and 5 days after tattooing revealed persistence of the pigment implant as free granules in the epidermis and dermis. In the specimen resected 18 months after tattooing most of the residual pigment was found within macrophages in the dermis and focally in the endomysial connective tissue of the superficial orbicularis oculi muscle. There were no substantial deleterious effects on the treated tissues.

Aged↗

Inferior oblique myositis presenting as superior oblique muscle palsy.

A 14-year-old girl presented with progressive vertical strabismus simulating superior oblique muscle palsy. However, because of the presence of limitation of infraduction, computed tomography was done; it showed a large mass in the region of the right inferior oblique muscle. A biopsy specimen of the enlarged muscle showed mainly degenerative and some regenerative changes. The diagnosis was orbital myositis, which should be considered in the differential diagnosis of strabismus, especially if the clinical findings are atypical of strabismus.

Adolescent↗

Pyogenic granuloma on a dermis fat graft in acquired anophthalmic orbits.

We treated exposure of the orbital implant with a dermis fat graft in two patients with an acquired anophthalmic orbit. During the first six postoperative months, a large polypoid tumor of the conjunctiva developed over the grafted tissue in each patient. Histologic examination of both lesions disclosed a pyogenic granuloma.

Adipose Tissue↗

An immunohistopathologic study of trilateral retinoblastoma.

We examined retinal and midline pineal tumors from four patients with trilateral retinoblastoma who had antibodies against neural-associated antigens including neuron-specific enolase, photoreceptor cell proteins (S-antigen and rhodopsin), and glial fibrillary acidic protein. Expression of neuron-specific enolase was observed in all four patients. S-antigen immunoreactivity was present in three of four ocular tumors and two of four pineal tumors examined, whereas results of labeling with rhodopsin and glial fibrillary acidic protein were negative in each of the ocular and pineal tumors.

Brain Neoplasms↗

S-antigen immunoreactivity in trilateral retinoblastoma.

Using monoclonal antibody MAbA9-C6, which identifies an antigenic determinant of S-antigen retained in fixed tissue sections, we investigated S-antigen immunoreactivity in the ocular and brain tumors of four cases of trilateral retinoblastoma. In the eye, S-antigen immunoreactivity was present in all retinoblastomas examined, as well as one retinocytoma characterized by benign appearing cells including fleurettes. S-antigen immunoreactivity was focally present in two of the four brain tumors examined. Additionally, two intraocular medulloepitheliomas, one of which contained well-defined rosettes, and 16 primary intracranial tumors, including seven pineal gland tumors and nine other lesions, some of which histopathologically may resemble retinoblastoma, were examined. S-antigen immunoreactivity was observed in two pineal gland tumors but not the remaining nine primary intracranial neoplasms or the two intraocular medulloepitheliomas. Our results further substantiate the immunologic relationship between the retina and the pineal gland, and tumors originating in these tissues.

Antibodies, Monoclonal↗

Lectin binding in retinoblastoma.

Lectins were used as cytochemical probes to examine the relationship between normal retina and retinoblastoma. Cases of retinoblastoma in which there were either Flexner-Wintersteiner rosettes or fleurettes accompanied by nontumorous retina were studied with ten biotinylated lectins. Pineal and ocular tumors from cases of trilateral retinoblastoma were also studied. Con-A, LCA, PNA, WGA and RCA-I labeled tissue of each type to varying degrees, while BS-I, DBA, SBA, S-WGA and UEA-I bound neither tumor nor retina. The high degree of binding homology suggests biochemical as well as structural similarities between these tissues.

Eye Neoplasms↗

Keratoconus with spontaneous perforation of the cornea.

A 24-year-old woman with a 5-year history of bilateral keratoconus who was in her eighth month of pregnancy presented with central corneal edema and mild acute iritis in her right eye. The iritis responded rapidly to therapy with topical corticosteroids, which was stopped after 1 week. The patient returned 1 week later with a markedly edematous central cornea, which had perforated. Management included a therapeutic soft contact lens, patching and, eventually, penetrating keratoplasty. The authors discuss the possible role of the pregnancy and of the topical corticosteroids in the development of the corneal perforation, a rare complication of keratoconus.

Adult↗

The prevalence of macular drusen in postmortem eyes.

In a histologic study of 46 eyes from 23 consecutive autopsy cases (mean age, 67 years; range, 36 to 94 years) with no previously documented ocular disease, drusen were identified in the macula of 87% (40 of 46 eyes), with hard drusen in 83% (38 of 46 eyes) and soft drusen in 39% (18 of 46 eyes). Soft drusen were found without hard drusen in two maculas. In 14 of the 16 eyes that contained both hard and soft drusen, the hard drusen outnumbered the soft drusen. The number of macular drusen in the right eye generally approximated that in the left eye for both hard drusen (r = 0.959; P less than .001) and soft drusen (r = 0.754; P less than .001). There was no correlation (t = 0.538; P greater than .05) between the number of hard drusen and increasing age. However, there was a significant correlation (t = 2.54; P less than .02) between the number of soft drusen and increasing age.

Adult↗