抄録
Chronic granulomatous disease (CGD) is an inhelited illness in which phagocytic cells are unable to kill ingested bacteria. Hepatic abscess is the most commonly reported gastrointestinal complication in these patients. Experience with 4 hepatic abcesses developed in 3 patients with CGD was reviewed. In each case, the diagnosis was confirmed by a negative nitro blue tetrazolium test and by total failure of chemiluminescence after phagocytosis. Operative hepatic debridement and external drainage combined with intravenous antibiotics were curative in every case, but operative morbidity was frequent and severe. Three major postoperative complications included wound disruption with delayed healing, prolonged febrile course and massive hepatic bleeding. One patient received granulocyte transfusions for over 10 days in the pre- and post-operative periods. By the 12th day of therapy, she had become afebrile. For hepatic abscess in CGD patients, drainage must be carried out in an excisional fashion. If the abscess is at periphery of the lobe, partial hepatic lobectomy is the treatment of choice. If placed more centrally within the parenchyma of the liver, operative thepapy should consist of hepatotomy, evacuation of purulent exudate and total granulomatous material and external drainage. Granulocyte transfusion as adjunctive therapy seems to have a significant benefit in CGD patients with serious infection.