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

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.