背景:结膜结膜炎(LC)是I型纤溶酶原缺乏症(T1PD;OMIM#217090)的最常见临床表现,其特征在于在结膜上形成假膜(由于纤维蛋白的沉积),导致进行性视力丧失。在过去的时代,LC患者接受手术治疗,局部抗炎,细胞抑制剂,和效果有限的全身性免疫抑制药物(血液108:3021-3026,2006,眼科129:955-957,2022,SurvOthalmo148:369-388,2003,血液131:1301-1310,2018)。手术还可以触发膜的发育,如在需要眼假体的患者中观察到的(SurvOthalmol48:369-388,2003)。用局部纯化的纤溶酶原治疗用于防止假膜形成(血液108:3021-3026,2006,眼科129:955-957,2022)。
方法:我们介绍了一个16岁女孩患有严重左眼累及的LC的病例。我们报告了使用局部纤溶酶原滴眼液前后患者的临床状况,并描述了治疗方案,从而可以进行假膜减缩手术以及随后使用眼假体进行美学康复。
结论:患者对局部纤溶酶原表现出进行性反应,在12年的随访中完全没有假膜形成,尽管使用了眼假体。
BACKGROUND: Ligneous Conjunctivitis (LC) is the most common clinical manifestation of Type I Plasminogen deficiency (T1PD; OMIM# 217090), and it is characterized by the formation of pseudomembranes (due to deposition of fibrin) on the conjunctivae leading to progressive vision loss. In past times, patients with LC were treated with surgery, topical anti-inflammatory, cytostatic agents, and systemic immunosuppressive drugs with limited results (Blood 108:3021-3026, 2006, Ophthalmology 129:955-957, 2022, Surv Ophthalmol 48:369-388, 2003, Blood 131:1301-1310, 2018). The surgery can also trigger the development of membranes, as observed in patients needing ocular prosthesis (Surv Ophthalmol 48:369-388, 2003). Treatment with topical purified plasminogen is used to prevent pseudomembranes formation (Blood 108:3021-3026, 2006, Ophthalmology 129:955-957, 2022).
METHODS: We present the
case of a sixteen-year-old girl with LC with severe left eye involvement. We reported the clinical conditions of the patient before and after the use of topical plasminogen eye drops and described the treatment schedule allowing the surgical procedure for the pseudomembranes debulking and the subsequent use of ocular prosthesis for aesthetic rehabilitation.
CONCLUSIONS: The patient showed a progressive response to the topical plasminogen, with a complete absence of pseudomembrane formation at a twelve-year follow-up, despite using an ocular prosthesis.