ET Tsilou, D Thompson, AS Lindblad, GF Reed, … - British Journal of …, 2003 - bjo.bmj.com Methods: 20 study subjects were enrolled in the safety study and 16 in the efficacy study. Both
studies were randomised and double blind. The primary outcome for the safety study was the
occurrence of predefined serious ... Cited by 8 - Related articles - BL Direct - All 11 versions
ET Tsilou, BI Rubin, G Reed, RC Caruso, F Iwata, J … - Ophthalmology, 2006 - Elsevier Cystinosis is a rare autosomal recessive lysosomal storage disorder characterized by the
intracellular accumulation of cystine. Treatment involves intracellular cystine depletion with oral
cysteamine. A wide spectrum of ... Cited by 10 - Related articles - All 7 versions
F Iwata, EM Kuehl, GF Reed, LM McCain, WA Gahl, … - Molecular Genetics and …, 1998 - Elsevier In nephropathic cystinosis, corneal cystine crystals cause severe photophobia and corneal erosions.
Topical cysteamine dissolves these crystals, but cannot be marketed because it rapidly oxidizes to
the disulfide form, cystamine, at ... Cited by 17 - Related articles - BL Direct - All 5 versions
F Gebrail, M Knapp, G Perrotta, H … - Archives of Pathology and …, 2002 - arpa.allenpress.com The patient was a 23-year-old white man who underwent a renal transplant at age 12 years for
end-stage renal disease secondary to nephropathic cystinosis. The patient had been taking several
immunosuppressive medications since ... Cited by 2 - Related articles - BL Direct - All 3 versions
A Cantani, O Giardini, CA Ciarnella - American journal of …, 1983 - ncbi.nlm.nih.gov The biochemical hallmark of nephropathic cystinosis is the intralysosomal accumulation of free
cystine in various organs, including the conjunctiva, cornea, bone marrow, leukocytes, lymph nodes,
and internal organs. A patient with ... Cited by 8 - Related articles
P Dureau, M Broyer, JL Dufier - Journal of pediatric ophthalmology and …, 2003 - cat.inist.fr Background: Nephropathic cystinosis is characterized by an accumulation of cystine crystals within
most body tissues. Renal transplantation and oral cysteamine have improved the general prognosis of
the disease, and ocular ... Cited by 9 - Related articles - BL Direct - All 4 versions
- ►asnjournals.org R Kleta, WA Gahl - Journal of the American Society of Nephrology, 2002 - Am Soc Nephrol Nephropathic cystinosis was first described in the early 1900s in a 21-mo-old boy who died of
progressive anorexia; two siblings had previously died in infancy under similar circumstances (1).
By meticulous observations and ... Cited by 5 - Related articles - BL Direct - All 5 versions
B Katz, RB Melles, JA Schneider - Archives of Ophthalmology, 1989 - archopht.highwire.org In therecreational sport "war games" opposingteams fire gelatin pellets con- taining vegetable dye
at each other as they attempt to capture the opponent's flag without being hit. Three articles have
appeared in the ophthalmic liter- ... Cited by 5 - Related articles - All 4 versions
MI Kaiser-Kupfer, MB Datiles, WA Gahl - American journal of …, 1988 - ncbi.nlm.nih.gov 1: Am J Ophthalmol. 1988 Mar 15;105(3):318-9. Clear graft two years after keratoplasty
in nephropathic cystinosis. Kaiser-Kupfer MI, Datiles MB, Gahl WA. Clinical Branch, National
Eye Institute, Bethesda, MD 20892. Mesh Terms: Cornea/pathology; ... Cited by 5 - Related articles
