Gursu Hazım Alper, Erdogan Ilkay, Varan Birgul, Oktay Ayla, Ozcay Figen, Ozkan Murat, Aslamaci Sait
Department of Paediatric Cardiology, Baskent University, Ankara, Turkey.
J Card Surg. 2014 Sep;29(5):712-6. doi: 10.1111/jocs.12355. Epub 2014 Jun 3.
Protein-losing enteropathy is a rare complication of the Fontan palliation surgery. Budesonide is an effective treatment option for protein-losing enteropathy. We reviewed our retrospective experience in four patients who were treated with oral budesonide.
Four patients with refractory protein-losing enteropathy after the Fontan operation were started on oral budesonide 9 mg/daily. After achieving normal serum albumin the dose was tapered to 3 mg. Response to oral budesonide, side effects, and serum albumin levels before the treatment and at first, fourth, and ninth months of the budesonide course were recorded. Efficacy was measured based on serum albumin levels and clinical symptoms.
Mean pretherapy albumin was 2.25 g/dL (range 1.7 to 2.5 g/dL) and nine months after therapy it was 4.15 g/dL (range 3.9 to 4.4 g/dL) (p < 0.05). All patients had at least a transient improvement in serum albumin levels and clinical findings. Systemic side effects included cushingoid features and oral moniliasis. All patients had improvement in side effects after tapering budesonide to 3 mg. The treatment was terminated in one case as soon as serum albumin level exceeded 3 g/dL. One death occurred from respiratory arrest six months after budesonide discontinuation.
Budesonide can be used to treat protein-losing enteropathy in selected patients with cardiac diseases.
蛋白丢失性肠病是Fontan姑息手术罕见的并发症。布地奈德是治疗蛋白丢失性肠病的一种有效选择。我们回顾了4例接受口服布地奈德治疗患者的回顾性经验。
4例Fontan手术后难治性蛋白丢失性肠病患者开始口服布地奈德，剂量为9毫克/天。血清白蛋白恢复正常后，剂量减至3毫克。记录口服布地奈德的反应、副作用以及治疗前、布地奈德疗程第1个月、第4个月和第9个月时的血清白蛋白水平。根据血清白蛋白水平和临床症状评估疗效。
治疗前白蛋白平均水平为2.25克/分升（范围1.7至2.5克/分升），治疗9个月后为4.15克/分升（范围3.9至4.4克/分升）（p<0.05）。所有患者血清白蛋白水平和临床症状至少有短暂改善。全身副作用包括库欣样特征和口腔念珠菌病。布地奈德剂量减至3毫克后，所有患者的副作用均有改善。1例患者血清白蛋白水平超过3克/分升后即终止治疗。1例患者在布地奈德停药6个月后因呼吸骤停死亡。
布地奈德可用于治疗部分患有心脏病的蛋白丢失性肠病患者。
