Sayar R B, von Domarus D, Schäfer H J, Beckenkamp G
University Eye Hospital of Hamburg, FRG.
Ophthalmologica. 1988;197(1):1-6. doi: 10.1159/000309909.
The Richner-Hanhart syndrome seen in a middle-aged woman is described. The patient had the typical clinical manifestations of tyrosinemia II with a bilateral keratopathy, palmar and plantar hyperkeratosis. The patient was subjected to a specific diet and to perforating keratoplasty. Postoperative systemic therapy with steroids had a negative influence on the clinical manifestations; dendritic lesions which developed on the corneal graft disappeared after interruption of the systemic steroid therapy. This suggests that cortisone treatment after keratoplasty should be avoided in this special metabolic disorder.
本文描述了一名中年女性所患的Richner-Hanhart综合征。该患者具有典型的酪氨酸血症II型临床表现，包括双侧角膜病变、掌跖角化过度。患者接受了特殊饮食治疗和穿透性角膜移植术。术后使用类固醇进行全身治疗对临床表现产生了负面影响；角膜移植片上出现的树枝状病变在中断全身类固醇治疗后消失。这表明在这种特殊的代谢紊乱疾病中，角膜移植术后应避免使用可的松治疗。
