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Bilateral corneal dermis-like choristomas; an X chromosome-linked disorder [Case Report]

Topilow HW; Cykiert RC; Goldman K; Palmer E; Henkind P
A male child was born with bilateral corneal opacification. His maternal uncle and a male first cousin of the patient's mother also had been born with identical bilateral corneal opacification that was pathologically proven to result from corneal dermoids. Histopathological examination of tissue removed from the present patient at the time of lamellar keratoplasty revealed thickened, keratinized epithelium, absent Bowman's membrane, and dense, irregular bands of highly vascularized collagenous connective tissue. No adnexal appendages were identified. The remainder of the ocular and general physical examination results was normal. In view of these clinical and histopathological similarities, the diagnosis of corneal dermis-like choristomas was made. To our knowledge, this report is the first to describe a family with X chromosome-linked recessive transmission of this condition
PMID: 7196217
ISSN: 0003-9950
CID: 26004

Orbitofacial mucormycosis with unusual pathological features [Case Report]

Albert DM; Lesser RL; Cykiert RC; Zakov ZN
A 52-year-old man with mild diabetes and acute stem cell leukaemia developed an orbitofacial mucormycosis. Cultures showed the fungus to be Rhizopus oryzae. Vigorous treatment with amphotericin B and other bactericidal and bacteriostatic antibiotics for a concurrent sepsis failed to suppress the infections, and the patient died. On post-mortem examination characteristic haematoxylin-staining, broad, aseptate fungal hyphae were found in the right eye, orbit, and lung. A striking and unusual feature of this case is the presence of brightly birefringent crystals within the severely degenerated eye. These were found by histochemical staining and x-ray diffraction studies to be calcium salts of fatty acids, apparently liberated from necrotic adipose tissue of the orbit
PMID: 508683
ISSN: 0007-1161
CID: 26005

Mycosis fungoides. Intraocular and central nervous system involvement [Case Report]

Keltner JL; Fritsch E; Cykiert RC; Albert DM
A 58-year-old man with mycosis fungoides was treated with chemotherapeutic agents and radiation for the recurrent cutaneous form of this disease. Five years after the onset, he developed blurring of vision, neurologic signs, and then lapsed into coma. Chemotherapy and radiation treatment resulted in improvement of the ocular and systemic picture. Within three months, however, the patient's vision deteriorated and swelling of both optic discs, along with retinal and vitreous infiltrates, was noted. Local radiation of the eyes was followed by improvement of the ocular changes. Five months later the patient died. Results of a histologic examination of the eyes showed extensive involvement of the retina and vitreous by tumor cells. The CNS was remarkably free of tumor cells and it was assumed that the radiation and chemotherapy were responsible for this
PMID: 849188
ISSN: 0003-9950
CID: 26006

Suspected multiple primary tumors of the lacrimal and parotid glands [Case Report]

Cykiert RC; Albert DM; Cornog JL Jr; Bellows AR
A 63-year-old man had apparent primary tumors of the lacrimal and parotid glands. Initially an adenocarcinoma of ectopic lacrimal gland origin was suspected following biopsy of a mass in the left orbit. The patient received radiation therapy to this area and showed no recurrence of the original lesion or development of other tumors for a period of 27 months. At that time, the patient returned with a mass in the left preauricular area. This was surgically resected and found to be an adenocarcinoma of the left parotid gland. Both tumors were histologically similar, mucin-secreting adenocarcinomas. There has been no further evidence of tumor growth in an additional three-year period
PMID: 183638
ISSN: 0003-9950
CID: 26007

Successful therapy of herpes hominis keratitis in rabbits by 5-iodo-5'-amino-2'5'-dideoxyuridine (AIU): a novel analog of thymidine

Albert DM; Lahav M; Bhatt PN; Reid TW; Ward RE; Cykiert RC; Lin TS; Ward DC; Prusoff WH
The efficiency of 5-iodo-5'-amino-2'5'-dideoxyuridine (AIU) in the therapy of experimental herpes keratitis in rabbits has been examined. Virus infections were established bilaterally in 40 animals using herpes simplex, type 1 (NIH strain 11124). Twenty-four hours after infection the rabbits were divided into five matched groups of eight and each group was treated, double-blind, with topical drugs at four-hour intervals for a total of 72 hours. The solutions instilled were: (1) saline; (2) IdUrd, 1 mg. per milliliter; (3) AIU, 1 mg. per milliliter; (4) AIU, 4 mg. per milliter; and (5) AIU, 8 mg. per milliliter. Each eye was examined daily for 12 days and graded independently by two ophthalmologists. Although IdUrd and AIU (8 mg. per milliliter) were effective therapeutically, IdUrd had a greater effect. The AIU at 1 and 4 mg. per milliliter were less active, but showed more rapid healing than the saline control. Viral recovery studies are consistent with the clinical observations. A second independent experiment, similar to that described above, gave essentially identical results. Although less potent than IdUrd, AIU does provide effective therapy for herpes keratitis
PMID: 931691
ISSN: 0020-9988
CID: 26008