日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 32(5): 432-436 (2016)
doi:10.9794/jspccs.32.432

症例報告Case Report

TCPC術後の蛋白漏出性胃腸症に伴う低IgG血症に対し,pH 4処理酸性人免疫グロブリン(皮下注射)による在宅補充療法を導入した1例Subcutaneous Immunoglobulin (SCIG) Home Therapy for Treatment of Hypogammaglobulinemia due to Protein-losing Enteropathy (PLE) after Total Cavopulmonary Connection

1大垣市民病院小児循環器新生児科Department of Pediatric Cardiology and Neonatology, Ogaki Municipal Hospital ◇ Gifu, Japan

2愛知県済生会リハビリテーション病院Aichi Saiseikai Rehabilitation Hospital ◇ Aichi, Japan

受付日:2016年4月11日Received: April 11, 2016
受理日:2016年7月14日Accepted: July 14, 2016
発行日:2016年9月1日Published: September 1, 2016
HTMLPDFEPUB3

TCPC(total cavo-pulmonary connection)手術後の蛋白漏出性胃腸症(protein-losing enteropathy: PLE)に伴う低IgG血症に対し,pH 4処理酸性人免疫グロブリン(皮下注射)(Subcutaneous Immunoglobulin: SCIG)による在宅補充療法を導入した1例を経験した.症例は左心低形成症候群の21歳男性.5歳時にTCPC手術を施行,術後5年でPLEを発症した.15歳より感染症等によるPLE症状増悪時に静注免疫グロブリン(intravenous immunoglobulin: IVIG)補充を行い,20歳でSCIG在宅補充を導入した.8 g/週で開始したが血清IgGの上昇を認めず,16 g/週に増量した.SCIG使用前と16 g/週補充後を比較し,血清IgG値は370→484 mg/dLと上昇,血清アルブミンは2.7→2.4 g/dLと低下,入院日数は4.7→1.2日/月と減少した.SCIGにより血清IgGが上昇し,感染症による入院回避に効果があった.PLE患者への維持投与量は,重症度に応じた個別の検討が必要と考えられる.

We report on a patient with protein-losing enteropathy (PLE) after undergoing total cavopulmonary connection (TCPC) operation who was introduced to subcutaneous immunoglobulin (SCIG) home therapy as immunoglobulin G (IgG) replacement therapy. The patient was a 21-year-old man with hypoplastic left heart syndrome who developed edema and ascites secondary to PLE 5 years after the TCPC operation. When he was 15-years-old, intravenous immunoglobulin (IVIG) was administered for the treatment of hypogammaglobulinemia. SCIG home therapy was introduced when he was 20 years old. The serum IgG level did not increase with the initial SCIG dose (8 g/week); therefore, we increased the SCIG dose (16 g/week). After SCIG administration, the serum albumin level was reduced from 2.7 to 2.4 g/dL, IgG level increased from 370 to 484 mg/dL, and the total duration of the hospital stay was shortened from 4.7 to 1.2 d/month. With regard to adverse events, a local reaction was observed at the subcutaneous infusion site, but this improved with time. These findings suggest that SCIG home therapy is effective in increasing and maintaining serum IgG levels and avoiding hospitalization. The appropriate SCIG dose for patients with PLE is unknown. We believe that this should be considered on a case-by-case basis depending on the severity of each case.

Key words: Fontan; protein-losing enteropathy; subcutaneous immunoglobulin

This page was created on 2016-08-22T17:16:10.213+09:00
This page was last modified on 2016-09-28T20:16:05.348+09:00


このサイトは(株)国際文献社によって運用されています。