선천저감마글로불린혈증 환아에서 발생한 사이질각막염
Stromal Keratitis in a Patient with Congenital Hypogammaglobulinemia
강현지, 조용운, 유웅선, 정인영, 서성욱, 김성재,
소속 상세정보
강현지 ( Kang Hyun-Ji )
Gyeongsang National University College of Medicine Gyeongsang National University Hospital Department of Ophthalmology
조용운 ( Cho Yong-Wun )
Gyeongsang National University College of Medicine Gyeongsang National University Hospital Department of Ophthalmology
유웅선 ( Yoo Woong-Sun )
Gyeongsang National University College of Medicine Gyeongsang National University Hospital Department of Ophthalmology
정인영 ( Chung In-Young )
Gyeongsang National University College of Medicine Gyeongsang National University Hospital Department of Ophthalmology
서성욱 ( Seo Seong-Wook )
Gyeongsang National University College of Medicine Gyeongsang National University Hospital Department of Ophthalmology
김성재 ( Kim Seong-Jae )
Gyeongsang National University College of Medicine Gyeongsang National University Hospital Department of Ophthalmology
Abstract
목적: 선천저감마글로불린혈증 환아에서 사이질각막염을 경험하여 보고하고자 한다.
증례요약: 15세 남자가 우안 시력저하로 내원하였다. 환아는 생후 16개월에 선천저감마글로불린혈증을 진단받고 정기적으로 면역글로불린을 투여 받고 있었다. 우안 최대교정시력 0.02, 안압 11 mmHg였고 전안부검사상 중심부와 주변부 각막사이질의 침윤과 얇아진 소견이 관찰되었으나 상피결손은 없었다. 급성사이질각막염으로 진단하고 1% Prednisolone acetate, 5% NaCl, Levofloxacin 점안액으로 치료를 시작하였다. 전신검사상에서 혈청 immunoglubulin G (IgG) 수치가 328.9 mg/dL로 3개월 전인 434.8 mg/dL에 비해 감소되어 있었다. 안약 점안 후에도 병변의 호전은 없었으나, 면역글로불린 투여 후 IgG 수치가 394.4 mg/dL로 증가하며 각막침윤이 감소하였다. 이후 IgG 수치는 480-530 mg/dL로 유지되었고 최대교정시력은 0.15, 각막두께는 330 μm로 측정되었으며 각막병변의재발과 악화는 없었다.
결론: 선천저감마글로불린혈증 환자에서 혈청 IgG 수치가 저하되며 발생한 사이질각막염에서 면역글로불린 투여로 호전되는 소견을 관찰하여 이를 보고한다.
Purpose: We report a case of stromal keratitis in a patient with congenital hypogammaglobulinemia.
Case summary: A 15-year-old boy presented with decreased visual acuity in the right eye. He had been diagnosed with congenital hypogammaglobulinemia at about 16 months of age and had received regular doses of intravenous immunoglobulin. The best-corrected visual acuity of the right eye was 0.02 and the intraocular pressure 11 mmHg. On anterior segment examination, thinning combined with stromal infiltration of the paracentral cornea was evident, but no epithelial defect was apparent. We scheduled detailed systemic examinations and laboratory investigations to rule out infectious keratitis. His serum immunoglubulin G (IgG) level was 328.9 mg/dL, thus less than that 3 months priorly (434.8 mg/dL). The lesion did not improve after prescription of topical antibiotics and steroid. The serum IgG level gradually increased to 394.4 mg/dL after immunoglobulin administration, and the corneal infiltration gradually decreased. After 5 months of treatment, the serum IgG levels ranged between 480 and 530 mg/dL; we noted no recurrence or worsening of the corneal lesion.
Conclusions: We report a case of stromal keratitis in a patient with congenital hypogammaglobulinemia; we prescribed intravenous immunoglobulin.
키워드
Congenital hypogammaglobulinemia; Serum Immunoglobulin G; Stromal keratitis
원문 및 링크아웃 정보
등재저널 정보