生後間もなく多尿と高ナトリウム血症を呈し先天性中枢性尿崩症と診断された全前脳胞症の児を報告する．当初，水分投与量の調節のみで水・電解質管理をしていたが，生後1か月時に一過性の抗利尿ホルモン不適切分泌症候群（syndrome of inappropriate antidiuretic hormone secretion: SIADH）を合併，以後，SIADHによる血液ナトリウム濃度の乱高下を繰り返した．生後2か月時より経口デスモプレシン製剤により多尿をコントロールしたところ，SIADH発症時の低ナトリウム血症は緩和された．全前脳胞症は中枢性尿崩症の原因として知られているが，視床下部の機能障害により一過性SIADHを合併しうることはあまり認知されておらず，また，そのような病態における治療についての報告は限られる．乳児期早期であってもデスモプレシン製剤を適切に使用することで，低ナトリウム血症のリスクを抑え，安定した全身管理が可能となり在宅医療に移行し得た．

Centrifugal therapeutic plasma exchange (cTPE) enables plasma removal at lower blood flow rates and can be performed using peripheral vascular access (VA), potentially reducing the invasiveness of plasma exchange in pediatric patients. We report four cases of intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) successfully treated with cTPE between illness days 8 and 12. All procedures were performed using peripheral VA under local anesthesia and continuous sedation with propofol. All sessions were performed with red blood cell (RBC) priming. Five-percent albumin was used as the sole replacement fluid, and the exchange volume was set at 1.0–1.5 times the estimated plasma volume. All patients completed 2–4 sessions without major complications or need for intubation. One patient developed transient coronary dilatation that regressed, whereas the remaining 3 had no coronary artery lesions. The median blood flow rate and treatment duration were 2.3 (1.8–2.8) mL/kg/min and 93 (69–118) minutes, respectively, with median reduction rates of immunoglobulin G (IgG) and fibrinogen of 67.3% and 66.8%. No clinically significant hypocalcemia or thrombocytopenia occurred. These findings suggest that cTPE using peripheral VA can be safely and effectively performed in patients with IVIG-resistant KD, offering a less invasive and feasible alternative to membrane TPE, which has been the predominant modality in Japan.