Oral Budesonide Treatment for Protein-Losing Enteropathy in Fontan-Palliated Patients

Abstract

Protein-losing enteropathy (PLE) is a rare complication of Fontan palliation associated with significant morbidity and mortality. It is characterized by the loss of serum proteins into the intestinal lumen, and its pathophysiology likely involves enteral inflammation. Budesonide, an oral steroid, is an attractive treatment option because of its potent enteral activity and minimal systemic side effects. A single-center, retrospective review of Fontan-palliated PLE patients treated with oral budesonide for 6 months or longer was performed. The patient characteristics reviewed were demographics, anatomic diagnosis, budesonide treatment (dose and duration), other medications and therapeutic interventions, hospitalizations, serum albumin levels, medical complications, and patient status at the time of follow-up assessment. The study enrolled 10 patients representing 228 patient-months of on-therapy follow-up evaluation. Serum albumin levels increased after initiation of budesonide for 90% of the patients, and clinical evidence of fluid overload improved for 60% of them. Symptomatic improvement was reported in 80% of the cases. During the treatment period, 50% of the patients met the primary end point of death or cardiac transplantation. In this series of PLE patients, oral budesonide therapy was associated with significant symptomatic improvement and sustained increases in serum albumin. However, budesonide therapy may not alter the long-term outcome for patients with advanced PLE.

Hemodynamic Phenotype of the Failing Fontan in an Adult Population

“…Although selected medicines may ameliorate symptoms, 16 mortality benefit has not been demonstrated. Furthermore, there is evidence that circulatory failure rather than ventricular failure is most important—Fontan patients with preserved ventricular function have been shown to do worse after heart transplantation compared with patients with decreased ventricular function.…”

Exudative enteropathy in children: causes, diagnosis and therapy

“…Кортикостероиды, в том числе будесонид, применялись у больных с системными заболеваниями соединительной ткани, воспалитель-ными заболеваниями кишечника и у пациентов по-сле операции Фонтена [29,30] болезни Менетрие назначаются антисекреторные препараты [21]. В острых ситуациях для увеличения онкотического давления применяют инфузии альбу-мина, которые эффективны только в краткосрочной перспективе и могут использоваться до назначения терапии ведущей причины.…”

Zespół utraty białek u pacjentów z pojedynczą komorą serca po operacji Fontana

“…Ostatnio pojawiły się doniesienia o korzystnym działaniu budesonidu, steroidu podawanego doustnie, który działa głównie na poziomie śluzówki przewodu pokarmowego, a ponieważ jego wchłanianie do krwi jest niewielkie, ma on mniejsze niż inne steroidy działanie ogólnoustrojowe [43,44].…”