Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
Ann Thorac Surg. 2010 Mar;89(3):837-42. doi: 10.1016/j.athoracsur.2009.09.063.
Intestinal inflammation is a component of the pathophysiology of protein-losing enteropathy after the Fontan operation. Oral controlled-release budesonide is 90% metabolized at first pass through the liver, has high enteric anti-inflammatory activity and relatively low systemic effects, and may be an ideal agent for use in treating this disease.
Budesonide was administered to 9 patients (4 male) with protein-losing enteropathy after the Fontan operation. The median interval between the Fontan operation and diagnosis of protein-losing enteropathy was 4 years (range, 0.1 to 13.3). Prior interventional therapy included pulmonary artery stent (1), fenestration (3), pacemaker placement (3) and Fontan revision (2). Prior medical therapy included oral prednisone (5), heparin (4), sildenafil (2), infliximab (1), and octreotide (1), all without persistent success. The starting daily dose of budesonide was 9 mg for patients 4 years old or older, and 6 mg for patients less than 4 years of age.
Mean serum albumin level 3 months before starting budesonide was 1.9 g/dL (range, 1 to 2.4 g/dL). Serum albumin level improved in all patients within 6 months of starting budesonide (mean 2.9 g/dL; range, 2.2 to 3.8 g/dL). Albumin levels of 3 g/dL or more were achieved in 8 of 9 patients within a median of 4.3 months (range, 2 to 25). Side effects included Cushingoid features and osteoporosis (3), infection requiring antibiotic treatment (5), and acne exacerbation (1). Weaning from high initial dose to a lower dose was possible with sustained effect; however, discontinuation of budesonide resulted in recurrence of hypoalbuminemia.
Oral budesonide is an effective therapy for treating protein-losing enteropathy after the Fontan operation. To maintain response, low-dose therapy must be continued.
肠道炎症是法洛四联症根治术后蛋白丢失性肠病的病理生理学组成部分。口服控释布地奈德在首次通过肝脏时 90%被代谢，具有高肠内抗炎活性和相对较低的全身作用，可能是治疗这种疾病的理想药物。
对 9 例法洛四联症根治术后蛋白丢失性肠病患者（男 4 例）给予布地奈德治疗。法洛四联症根治术至蛋白丢失性肠病诊断的中位间隔时间为 4 年（范围，0.1 至 13.3 年）。介入治疗前包括肺动脉支架（1 例）、开窗（3 例）、起搏器植入（3 例）和法洛四联症根治术修正（2 例）。介入治疗前的药物治疗包括口服泼尼松（5 例）、肝素（4 例）、西地那非（2 例）、英夫利昔单抗（1 例）和奥曲肽（1 例），但均未持续成功。年龄 4 岁或以上的患者布地奈德起始剂量为 9mg/天，年龄小于 4 岁的患者为 6mg/天。
开始使用布地奈德前 3 个月的平均血清白蛋白水平为 1.9g/dL（范围，1 至 2.4g/dL）。所有患者在开始使用布地奈德后 6 个月内血清白蛋白水平均有改善（平均 2.9g/dL；范围，2.2 至 3.8g/dL）。9 例患者中有 8 例在中位时间 4.3 个月（范围，2 至 25 个月）内达到 3g/dL 或更高的白蛋白水平。副作用包括库欣样特征和骨质疏松（3 例）、需要抗生素治疗的感染（5 例）和痤疮恶化（1 例）。虽然可以从高初始剂量逐渐减至较低剂量，但仍能保持疗效，但布地奈德停药后白蛋白血症复发。
口服布地奈德是治疗法洛四联症根治术后蛋白丢失性肠病的有效方法。为了保持疗效，必须继续低剂量治疗。
