Nephropathic cystinosis: immunohistochemical and histopathologic studies of cornea, conjunctiva and iris.

Specimens of a cornea, conjunctiva and iris were removed at the time of penetrating keratoplasty in a case of nephropathic cystinosis. Immunohistochemical studies revealed the presence of class II antigens on the stromal keratocytes of the cornea and the vascular endothelial cells, pericytes and fibroblasts of the conjunctiva. In addition, there were thinning and focal breaks in Bowman's membrane. Both of these findings may have contributed to the severe photophobia and blepharospasm seen in patients with longstanding cystinosis.

[1]  M. Kaiser-Kupfer,et al.  Long-term ocular manifestations in nephropathic cystinosis. , 1986, Archives of ophthalmology.

[2]  R. Nussenblatt,et al.  T-lymphocyte subsets in experimental autoimmune uveitis. , 1985, Clinical immunology and immunopathology.

[3]  E. Thorsby,et al.  Antigen Presentation by Vascular Endothelial Cells and Epidermal Langerhans Cells: The Role of HLA‐DR , 1982, Immunological reviews.

[4]  C. Foster,et al.  T-cell subsets and Langerhans cells in normal and diseased conjunctiva. , 1982, American journal of ophthalmology.

[5]  M. Rodrigues,et al.  Langerhans cells in the normal conjunctiva and peripheral cornea of selected species. , 1981, Investigative ophthalmology & visual science.

[6]  G. Nabel,et al.  Antigen-specific t lymphocyte clones. II. Purifcation and biological characterization of an antigen-specific suppressive protein synthesized by cloned T cells , 1981, The Journal of experimental medicine.

[7]  G. Nabel,et al.  Antigen-specific T lymphocyte clones. I. Characterization of a T lymphocyte clone expressing antigen-specific suppressive activity , 1981, The Journal of experimental medicine.

[8]  S. Hsu,et al.  Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabeled antibody (PAP) procedures. , 1981, The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society.

[9]  C. Furlong,et al.  Binding assays for amino acids. The utilization of a cystine binding protein from Escherichia coli for the determination of acid-soluble cystine in small physiological samples. , 1974, The Journal of biological chemistry.

[10]  J. Seegmiller,et al.  Biochemical comparisons of the adult and childhood forms of cystinosis. , 1968, The New England journal of medicine.

[11]  Oliver H. Lowry,et al.  Protein measurement with the Folin phenol reagent. , 1951, The Journal of biological chemistry.

[12]  W. Gahl.  Cystinosis coming of age. , 1986, Advances in pediatrics.

[13]  E. Unanue.  The regulatory role of macrophages in antigenic stimulation. Part Two: symbiotic relationship between lymphocytes and macrophages. , 1981, Advances in immunology.