Mohammadpour Mehrdad, Javadi Mohammad-Ali
Ophthalmic Research Center, Labbafinejad Medical Center, Shaheed Beheshti University of Medical Sciences, Tehran, Iran.
Cornea. 2006 Jan;25(1):112-4. doi: 10.1097/01.ico.0000179928.20522.3a.
To report an 8-year-old girl with bilateral progressive visual loss and photophobia secondary to stem cell deficiency as a result of multiple endocrine deficiency.
A case report and review of medical literature.
The patient suffered from severe photophobia and decreased visual acuity since May 2000. Despite multiple outpatient visits, no definite cause was found, and conservative treatments failed. On slit-lamp examination severe meibomian gland dysfunction, loss of eyelashes, decreased tear meniscus, diffuse corneal vascularization, and delayed punctate fluorescein staining of corneal epithelium were detected. She had also episodes of hypotension, oral candidiasis, and seizures. Her systemic work up revealed multiple endocrine deficiency (Addison disease and hypoparathyroidism). Hormone replacement therapy with fludrocortisone and oral calcium accompanied by punctual occlusion led to significant clinical recovery.
In a pediatric patient with diffuse corneal vascularization and no definite cause, systemic workup should be done to rule out multiple endocrine deficiencies. Treatment consists of hormone replacement therapy and management of the dry eye.
报告一名8岁女孩，因多种内分泌缺乏导致干细胞缺乏继发双侧进行性视力丧失和畏光。
病例报告及医学文献复习。
该患者自2000年5月起出现严重畏光和视力下降。尽管多次门诊就诊，但未发现明确病因，保守治疗无效。裂隙灯检查发现严重睑板腺功能障碍、睫毛脱落、泪液弯月面减少、弥漫性角膜血管化以及角膜上皮点状荧光素染色延迟。她还出现过低血压、口腔念珠菌病和癫痫发作。全身检查显示多种内分泌缺乏（艾迪生病和甲状旁腺功能减退）。氟氢可的松激素替代疗法和口服钙剂并结合泪点封闭治疗后，临床症状明显改善。
对于角膜弥漫性血管化且病因不明的儿科患者，应进行全身检查以排除多种内分泌缺乏。治疗包括激素替代疗法和干眼症的处理。
