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Biomedical subjects

J H Krachmer

Publications and source records attributed to J H Krachmer.

At least 19 recordsLinked to original sources

Posterior corneal crystalline deposits in benign monoclonal gammopathy: a clinicopathologic case report.

A 74-year-old woman had bilateral, deep stromal, patchy crystalline corneal deposits with the greatest density in the midperiphery. Visual acuity was 6/120 in the right eye and finger counting at 1 m in the left eye. Histological examination of the corneal button showed large, irregular amorphous masses in the posterior stroma. The deposits stained red with Masson's trichrome and were positive for protein with the Danielli stain. Stains for amyloid, copper, and lipid were negative. The immunoperoxidase stain was positive for polyvalent IgG and kappa light chains. Transmission electron microscopy disclosed electron-dense deposits with linear and honeycomb profiles. Laboratory investigations disclosed elevated serum and urinary IgG kappa light chain (Bence Jones protein) levels. Urinary amino acids were normal. The serum copper level was elevated. Antinuclear antibody was positive at a titer of 1:80. A bone marrow aspirate was normal, as were roentgenograms of the skull.

Aged

Evaluation of soft contact lens disinfection in the home environment.

Recent reports of bacterial corneal ulcers in cosmetic soft contact lens wearers prompted an evaluation of one possible source of contamination, inadequate home disinfection of the lenses. Twenty-nine patients were asked to come for an early morning examination. They were told not to wear their lenses so their usual home disinfection procedure could be observed. On arrival, extensive bacterial and fungal cultures were taken of the solution in the previously unopened cases. Ten of the 29 patients (34.5%) had contaminated cases. Some patients did not follow the manufacturer's disinfection guidelines. The current disinfection procedure has repeatedly proven adequate in the laboratory. However, the practical fact is that a substantial percentage (34.5% in this study) of soft lens wearers are inadequately disinfecting their lenses. This serves as a possible source of contamination leading to bacterial corneal ulcers.

Activities of Daily Living

Familial corneal hypesthesia.

A 4-year-old boy with severe, diffuse, asymptomatic, punctate, epithelial corneal erosions had bilateral sharply decreased corneal sensation with normal skin sensation in the distribution of the trigeminal nerves. Subsequent family studies disclosed five family members with similar corneal changes and decreased corneal sensation and no punctate erosions. None had decreased skin sensation in the distribution of the fifth cranial nerve. No environmental factors or evidence of local or systemic disease accounted for these findings.

Adolescent

Bacterial corneal ulcers in cosmetic soft contact lens wearers.

Soft contact lenses provide a safe alternative to spectacles for more than 1 million patients. However, the hazard of bacterial corneal ulcers exists. This report describes five cosmetic soft contact lens wearers who developed bacterial corneal ulcers. In three cases, the resulting visual acuity was 6/120 or less. Possible sources of contamination are discussed, but in no case was it determined.

Adolescent

Traumatic corneal endothelial rings.

Nonpenetrating "blast injuries" to the eye in two patients resulted in multiple, small, corneal epithelial foreign bodies that were associated with characteristic ring-shaped opacities of the corneal endothelium. These endothelial lesions were clinically visible immediately after injury and became more pronounced during the next several hours. They disappeared within days and resulted in no permanent loss of visual acuity. This communication describes and illustrates the appearance of these endothelial lesions both clinically and in an animal model. Light and electron microscopic observations of an experimental model, with the use of monkey and rabbit eyes, revealed that the ring-shaped opacities resulted from swelling of the corneal endothelium, as well as accumulation of fibrin and leukocytes on the injured cells. Except for the epithelial impact site and the concussion injury of the endothelium, the cornea was uninvolved, and the stroma remained clear.

Adolescent

Pellucid marginal corneal degeneration.

Pellucid marginal degeneration of the cornea is a bilateral, clear, inferior, peripheral corneal-thinning disorder. Protrusion of the cornea occurs above a band of thinning, which is located 1 to 2 mm from the limbus and measures 1 to 2 mm in width. American ophthalmologists are generally not familiar with the condition because most of the literature concerning pellucid degeneration is European. Four cases are described. This condition is differentiated from other noninflammatory cornel-thinning disorders such as keratoconus, keratoglobus, keratotorus, and posterior keratoconus. It is also differentiated from peripheral corneal disorders associated with inflammation such as Terrien's peripheral corneal degeneration, Mooren's ulcers, and ulcers from connective tissue disease.

Adult

Posterior keratoconus.

Posterior keratoconus is a rare corneal disorder characterized by a total or localized noninflammatory thinning of the cornea. In front of a conical protrusion of the posterior corneal curvature, there is a thinned stroma and nonprotruding anterior surface. A corneal button from a patient with bilateral posterior keratoconus was studied by light and electron microscopy. Descemet's membrane, particularly in the area of stromal thinning, demonstrated abnormal anterior banding, a multilaminar configuration, and localized posterior excrescences. These alterations suggest an early pathogenetic mechanism, which probably originated prior to the fifth or sixth month of gestation.

Adult

Corneal endothelial dystrophy. A study of 64 families.

A prospective study was undertaken during an 18-month period with 64 families who had endothelial dystrophy. Two hundred twenty-eight relatives were examined. Of those older than the age of 40, 38% were affected. Women were affected more severely and 2.5 times more frequently than men. The disease showed a strong familial tendency: there was one family in which three generations were affected and 16 families in which two generations were affected. There were four families that had members with edema in two generations. There was no association between edema in a parent and edema in a child. The proportion of relatives affected and the severity of involvement increased with age. Fifty-three probands and 18 relatives had endothelial dystrophy with edema (Fuchs' dystrophy). Of these 71, one had glaucoma.

Adolescent

Subepithelial infiltrates: a probable sign of corneal transplant rejection.

A previously undescribed slit-lamp manifestation of a probable corneal transplant rejection reaction was found in 22 patients among 145 who underwent penetrating keratoplasty during a two-year period. The reaction consisted of subepithelial infiltrates that were located only in the donor tissue; were without associated conjunctivitis; and that occurred six weeks to 21 months postoperatively, either alone or in association with epithelial and/or endothelial rejection; and that responded well to topical corticosteroid treatment. In one case, the subepithelial infiltrates preceded a severe endothelial rejection by only a few days. The lesions are a warning that all is not well and that corticosteroid therapy should be instituted or increased.

Administration, Topical

Helminthosporium corneal ulcers.

We studied four cases of Helminthosporium corneal ulcers from four different institutions. One case occurred in an immune-deficient infant, another in a patient three months after removal of a foreign body; the third case occurred in a patient with a corneal dystrophy who was wearing soft contact lenses and using topical corticosteroids; and the fourth occurred in a patient with a history of multiple foreign bodies. Antifungal sensitivities were performed in three cases and showed relatively good sensitivity to available antifungal medications; even though all four patients improved, there was poor correlation between in vitro sensitivities and clinical response.

Adult

Wilms' tumor metastatic to the orbit.

In a 2 1/2-year-old boy, a proptotic right lower lid developed one year after a primary abdominal mass proved to be Wilms' tumor. An orbital abscess or fungal infection was considered because the child was receiving chemotherapy. However, echography demonstrated a firm orbital mass, delineated its dimensions, and showed destruction of the orbital floor. The biopsy specimen showed metastatic tumor cells. Lile neuroblastoma and certain hematologic and reticuloendothelial malignant neoplasms, Wilms' tumor may secondarily invade the ocular adnexa.

Child, Preschool

Fleck corneal dystrophy.

Fourteen members of four families with fleck systrophy of the corneal stroma were examined and studied. Corneal and lens changes were noted in all patients. Corneal sensation was normal. Corneal biopsies were performed on two patients and a corneal button of a patient with fleck dystrophy and keratoconus was studied. Light and electron microscopy and histochemical studies showed this dystrophy to consist of abnormal keratocytes with variable numbers of membrane-limited intracytoplasmic vacuoles containing a granular to fibrogranular material that stains positively for mucopolysaccharide. This appears to be the first reported incidence of a dominantly inherited disorder of mucopolysaccharides affecting the eyes.

Adult

Corneal sensation in Adie's syndrome.

Using the Cochet-Bonnet esthesiometer, we evaluated the corneal sensation of 11 patients with unilateral Adie's tonic pupil. We eliminated six other patients with bilateral Adie's pupil or disease that lowered corneal sensation. An observed unfamiliar with each patient's condition tested 12 clock hour positions in the midperiphery in both eyes of all patients. We studied 30 normal subjects (60 eyes) in a similar fashion. Of those 11 patients with unilateral Adie's syndrome, ten had a regional decrease in corneal sensation. The 30 normal subjects examined did not exhibit any significant decrease in corneal sensation in any areas. Our study supports the concept that the lesion of Adie's tonic pupil is in the ciliary ganglion or short location where the innervation of the iris sphincter and corneal sensation are found together.

Adie Syndrome

Corneal ulcer caused by Pasteurella multocida.

A 44-year-old woman with Graves' disease and exophthalmos developed a severe corneal ulcer caused by Pasteurella multocida after being bumped on her right eye by her pet dog. The organism was transferred from the patient to an abraded rabbit cornea and produced a similiar corneal ulcer and hypopyon.

Adult

Ocular Ehlers-Danlos syndrome with normal lysyl hydroxylase activity.

We report two brothers affected with what has been called either fragilitas oculi or the Ehlers-Danlos syndrome type VI. Previously reported cases of the Ehlers-Danlos syndrome type VI showed a deficiency of lysyl hydroxylase in cultured fibroblasts. Assays of cultured skin fibroblasts from these two boys yielded normal activity of this enzyme, suggesting that there are two variants of this disease.

Adolescent

Familial occurrence of dot (microcystic), map, fingerprint dystrophy of the cornea.

The clinical description of corneal microcysts, maplike changes, and fingerprints has led investigators to the conclusion that these changes may represent a corneal dystrophy. The familial or hereditary evidence which is usually necessary to label a corneal disease a dystrophy has been lacking. This paper describes a familial pattern of disease in two families where three generations were involved and in eight families with corneal changes in at least two generations.

Adult

Ara-A and IDU therapy of human superficial herpetic keratitis.

Ara-A ointment was compared to IDU ointment in patients with dendritic herpes simplex virus infection of the corneal epithelium. Twenty-eight patients were treated with Ara-A ointment and twenty-four with IDU ointment. The lesions healed in 5.1 days with Ara-A and in 6.9 days with IDU. This drug was given in a double-controlled manner, so that neither the patient, nor the investigator knew which drug the patient was receiving. The patient groups were comparable as to length of the dendritic lesion and duration of symptoms. The adverse reactions to each of these drugs were comparable and in no case was there any permanent ocular change from drug use.

Clinical Trials as Topic