Fahnehjelm Kristina Teär, Törnquist Alba-Lucia, Winiarski Jacek
Department of Clinical Neuroscience, Karolinska Institut, Stockholm, Sweden.
Acta Ophthalmol. 2008 May;86(3):253-8. doi: 10.1111/j.1600-0420.2007.01120.x. Epub 2007 Dec 18.
To report the prevalence of dry-eye syndrome (DES) in children and young adults treated with allogeneic stem-cell transplantation (SCT) during childhood; to relate DES to conditioning regimes, including total body irradiation (TBI) and chemotherapy, and to immunosuppressive drugs and graft-versus-host disease (GVHD).
This cross-sectional study included 60 children/young adults transplanted because of leukaemia, various haematological disorders and inborn errors of metabolism between 1986 and 2004, with a follow-up time of 7.0 years (median, range 2-18). Clinical assessments, performed at a median age of 15.6 years (range 5.5-23.5), included an inquiry form on dry-eye symptoms, corneal status including fluorescein staining, 'break-up time' (BUT) and Schirmer test.
A total of 37 of 60 patients had DES defined as presence of corneal epithelial lesions with a pathological BUT and/or Schirmer test. Twenty-nine had had staining <1-10% of the corneal surface while eight patients had staining > or =10-25% of the corneal surface. All 37 patients with objective signs of DES, graded and not graded, had significant associations to subjective symptoms of dry eyes including dry eyes, red eyes, ocular irritation, secretion and sensitivity to light. Frequent occasions (above median; n = 7) of high cyclosporine A trough levels above 250 ng/ml were associated significantly with DES (P = 0.002). However, there was no association between DES and conditioning with single-dose (s-TBI) or fractionated TBI (f-TBI), busulfan or other chemotherapy. There were no associations between prolonged corticosteroid treatment or chronic GVHD and DES in the present study. DES was more common in patients with malignant diseases (P = 0.02). Malignant disease increased the risk of DES in girls but not in boys. Increased age at SCT increased the risk for DES in boys but not in girls (P = 0.02). Although severe keratitis occurred in three patients, nobody suffered corneal perforation.
DES with epithelial punctata keratopathy was common in children/young adults treated with SCT and more common if the patients were exposed to repeated high trough levels of cyclosporine A; however, DES was not associated with irradiation, corticosteroids or GVHD in the present study. Patients with objective DES also had subjective symptoms of dry eyes, which facilitate diagnosis. Girls with malignant diseases and boys who underwent SCT at later ages seem to demand higher attention and more frequent check-ups regarding DES. Patients with diagnosed severe DES needed frequent and continuous ophthalmological care to maintain treatment motivation.
报告儿童期接受异基因干细胞移植(SCT)的儿童和青年中干眼症综合征(DES)的患病率;将DES与预处理方案(包括全身照射(TBI)和化疗)、免疫抑制药物及移植物抗宿主病(GVHD)相关联。
这项横断面研究纳入了1986年至2004年间因白血病、各种血液系统疾病和先天性代谢缺陷而接受移植的60名儿童/青年,随访时间为7.0年(中位数,范围2 - 18年)。在中位年龄15.6岁(范围5.5 - 23.5岁)时进行临床评估,包括一份关于干眼症状的询问表、角膜状况(包括荧光素染色)、“泪膜破裂时间”(BUT)和泪液分泌试验。
60例患者中共有37例患有DES,定义为存在角膜上皮病变且BUT和/或泪液分泌试验结果异常。29例患者角膜表面染色<1 - 10%,而8例患者角膜表面染色≥10 - 25%。所有37例有DES客观体征的患者,无论是否分级,均与干眼主观症状显著相关,包括眼干、眼红、眼部刺激感、分泌物增多及畏光。环孢素A谷浓度频繁高于250 ng/ml(高于中位数;n = 7)与DES显著相关(P = 0.002)。然而,DES与单剂量全身照射(s - TBI)或分次全身照射(f - TBI)、白消安或其他化疗预处理之间无关联。在本研究中,长期使用皮质类固醇治疗或慢性GVHD与DES之间无关联。DES在恶性疾病患者中更常见(P = 0.02)。恶性疾病增加了女孩患DES的风险,但对男孩无影响。SCT时年龄增加会增加男孩患DES的风险,但对女孩无影响(P = 0.02)。尽管3例患者发生了严重角膜炎,但无人发生角膜穿孔。
接受SCT治疗的儿童/青年中,点状上皮性角膜炎型DES很常见,如果患者反复暴露于高谷浓度的环孢素A则更常见;然而,在本研究中DES与照射、皮质类固醇或GVHD无关。有DES客观体征的患者也有干眼主观症状,这有助于诊断。患有恶性疾病的女孩和较晚接受SCT的男孩似乎需要更密切关注和更频繁的DES检查。确诊为严重DES的患者需要频繁且持续的眼科护理以维持治疗积极性。