Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Signs of keratoconus, including Munson's sign, apical thinning, and Vogt's striae, developed in a 41-year-old woman 6 years after successful penetrating keratoplasty. Screening donor corneas for ectatic diseases may prevent such occurrences.
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PMID:Keratoconus after penetrating keratoplasty. 238 77

Keratoconus is characterized by the presence of corneal distortion (secondary to thinning of the apex) and either Fleischer's ring (found in 57% of patients) or Vogt's striae (found in 44% of patients). Often the only presenting symptom is decreased visual acuity. Steep keratometric readings are not usually diagnostic; 18% of patients with keratoconus have readings flatter than 45.00 D. Treatment requires correction of acuity with spectacles or contact lenses. More advanced cases are generally managed with rigid gas-permeable contact lenses. Corneal scarring occurs in approximately 43% of cases; 10% to 20% of patients require penetrating keratoplasty to restore acuity.
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PMID:Keratoconus. 776 20

We report a rare case of keratoconus with granular dystrophy with a follow-up of two decades, documenting the sequential presentation of two diseases confirmed by histology and genetic studies. A 13-year-old boy was diagnosed in 1988 with keratoconus in both eyes (BE) based on slit-lamp biomicroscopy findings of corneal ectasia in BE accompanied by Fleischer's ring, Vogt's striae, a small, old, healed hydrops. The left eye (LE) had central corneal thinning and scar in the central area involving the mid and posterior stroma secondary to healed hydrops. Penetrating keratoplasty (PKP) was advised. The boy was lost to follow-up till 1991 and presented with white, dot-like opacities in the central cornea in the RE only, suggestive of granular corneal dystrophy. Similar findings of white, dot-like opacities were noted in the LE in 1995 and the patient subsequently underwent PKP in BE. Histopathology of corneal buttons confirmed the presence of patchy, crystal-like orange deposits, which stained bright red with Masson's trichrome. Mutational analysis of the TGFBI gene in patient's DNA revealed a heterozygous mutation corresponding to a change in Arg555Trp in the keratoepithelin protein. Granular dystrophy recurred after 8 years in the RE.
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PMID:Late occurrence of granular dystrophy in bilateral keratoconus: penetrating keratoplasty and long-term follow-up. 2183 53

An 18-year-old male presented with complaints of gradually decreasing vision in both eyes for 6 years. He was a diagnosed with juvenile scleroderma at the age of 11 years by a pediatric rheumatologist. Clinical slit lamp examination showed features of ectasia, thinning, Vogt's striae, and apical scarring in both eyes. Bulbar and tarsal conjunctiva was quiet and normal. Corneal tomography revealed mean keratometry 65.8 and 65.4 diopters, thinnest pachymetry of 351 and 224 microns in the right and left eye, respectively. There was no history of itching and eye rubbing in the past. Patients of juvenile scleroderma may have associated keratoconus. The management of advanced keratoconus presents challenges related to handling and insertion of contact lenses in this condition. Keratoplasty is an option in those patients when contact lenses are not acceptable.
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PMID:Advanced keratoconus in a child with juvenile scleroderma. 3217 98