Biomedical subjects
Publications and source records attributed to G Messer.
A case of leiomyosarcoma of the scrotum in a 65 year old man is presented. It is, we believe, the sixth case reported in the literature. A hemiscrotectomy was performed with preservation of both testes. Three years after the operation the patient is well and free of metastases. The electron microscopy study is reported and the features are discussed.
A severe, bilateral, posttraumatic recurrent corneal erosion in a 29-year-old woman was treated by bilateral lamellar keratectomy. The surgical procedure was performed first in the right eye. Under local anesthesia the defective corneal region stained by fluorescein was completely removed. It comprised a lamella measuring 2 X 3 mm and included epithelium and underlying superficial stroma, both of which showed abnormal features. Following this procedure, her right eye was asymptomatic for over 6 yr. Surgical procedure in the left eye was performed in the same manner except that only a part of the stained cornea containing superficial layers of epithelium was removed, in order to prevent central scarring. Despite surgery, the recurrent painful attacks in this eye continued.
Cholestatic jaundice developed in four patients after the administration of prajmalium bitartrate. The clinical, histologic, ultrastructural, and immunologic findings were determined. In all patients, the clinical and morphologic features indicated idiosyncrasy. Two antibodies distributed in a granular pattern along the bile canaliculi were detected by immunofluorescence in all patients. In one patient, autoimmune markers were found in the serum, and in two instances, the migration-inhibition factor assay against the offending drug was found to be positive. The data support the concept that immunologic processes may participate in the production of the cholestatic syndrome.
The occurrence of chronic progressive external ophthalmoplegia, pigmentary retinal dystrophy and cardiac disturbances associated with arachnodactily, sternal deformity, high arched palate and severe myopia is reported. A pedigree analysis of this Jewish-Iranian family strongly suggests that the condition is inherited as autosomal recessive trait. The terminology of the condition and the spectrum of it variable phenotypic expression is described.
Pleomorphic membranous cytoplasmic bodies that indicated glycolipid storage were found in the conjunctiva, skin, and gingiva of a Jewish patient with Sandhoff's disease. The clinical symptoms were typical of GM2 gangliosidosis. Both hexosaminidase A and hexosaminidase B activities were deficient in the leukocytes and serum. Glycosaminoglycan levels in cultured fibroblasts were elevated. Membranous cytoplasmic bodies were observed in high concentrations in a large proportion of the vascular endothelial cells, pericytes, and Schwann cells and to a somewhat lesser extent in the fibrocytes of all tissues studied. Ultrastructural analysis of the conjunctiva, skin, and gingiva as an aid for the diagnosis of Sandhoff's disease is suggested.
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We established the organization of the AKR Qa region and determined the sequence of the Q4 and Q5 genes. Restriction mapping and genomic Southern blot analysis revealed that the AKR strain codes for only three H-2K homologous genes in this region. The AKR Q5 gene is not homologous to the Q5 gene of the C57BL strain, but is presumably allelic to the Q5 gene isolated from Balb/c. The organization and structure of the AKR Qa family is virtually identical to the Qa genes of the C3H mouse. The AKR Q5 gene, in contrast to other H-2K homologous Qa region genes, codes for a typical transmembrane region, and upon transfection into BHK cells, a 1.6 kb Q5 transcript is detected.
Amyloidosis of the tonsil is a rare condition and amyloidosis of Waldeyer's ring has not been previously reported. The present study describes a case of amyloidosis of the tonsil, nasopharynx and base of tongue in a 35-year-old patient in otherwise good health and without clinical symptoms. The possibility of systemic amyloidosis was excluded by clinical examination and biopsy of rectal and buccal mucosae. Electron microscopy showed the presence of typical amyloid fibrils. Large masses of the fibrils were closely surrounded by cells which appeared to be phagocytic. The ultrastructure of these cells is described. This case is considered to be a unique type of organlimited amyloidosis of Waldeyer's ring.
Two Caucasian carriers for chronic granulomatous disease (CGD) developed cutaneous lupus erythematosus (LE) with clinically and morphologically characteristic appearance for chronic discoid lupus erythematosus (DLE) and lupus erythematosus tumidus (LET). Direct immunofluorescent examinations and ANA titers were positive in both young women. No systemic involvement due to the ACR criteria was evident. Their sons suffered from X-linked cytochrome-b negative CGD. The diagnosis of CGD was based on measurement of oxidative burst activity by nitroblue tetrazolium (NBT) slide test and by flow cytometry using dihydrorhodamine 123 (DHR). The absence of cytochrome b558 in neutrophilic granulocytes was confirmed photometrically and by flow cytometry using the 7D5 monoclonal antibody against cytochrome b. We report for the first time the association of the photosensitive LE subtype LET and the X-linked CGD carrier state. Tissue damage by UV radiation and a reduced antimicrobial capacity may lead to recurrent immune stimulation and may together with genetic predisposition explain the occurrence of cutaneous LE in female carriers of CGD.