Pediatric Protein-Losing Enteropathy Treatment & Management
- Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD more...
Therapeutic approaches for protein-losing enteropathy depend on the underlying etiology.
In patients with primary intestinal lymphangiectasia, no direct method to address the protein-losing enteropathy is noted. Replacing fat in the diet with medium-chain triglycerides (MCTs) can improve fat malabsorption and the nutritional status of the patient. Supplementing fat-soluble vitamins (ie, A, D, E, K) is also important.
In protein-losing enteropathy associated with lymphatic obstruction, relieving the pressure in the lymphatic system decreases intestinal protein loss. Obstruction of lymphatics has been reported with structural heart disease, constrictive pericarditis, cardiomyopathy, and surgical repair of congenital heart disease. When obstruction of the intra-abdominal lymphatic system is the cause of protein-losing enteropathy, removal of long-chain triglycerides from the diet decreases the pressure in the lacteals and the lymphatic circulation. In addition, because of the increased pressure, there may be rupture of lacteals, which itself results in fat malabsorption. The use of MCT oil in these cases does not relieve any inflammation, but because MCT oil is not absorbed via the lymphatic system, it reduces the pressure of the lacteals.
Protein-losing enteropathy that results after heart surgery (with increased pressure in the right side) is a known postoperative complication of the Fontan procedure that has been a challenge to the surgical procedure's long-term success. Multiple treatments have been used, including corticosteroids, heparin, and additional surgical intervention (baffle fenestration or heart transplantation). Note the following:
As many as 13.4% of patients undergoing a Fontan procedure develop protein-losing enteropathy within 10 years of surgery, and the mortality rate associated with this complication has been reported to be as high as 56% in 5 years.
The use of steroids has produced temporary clinical and pathological resolution of protein-losing enteropathy.
Recently, a single-center retrospective review examined the use of budesonide, an oral steroid with extensive first pass metabolism, for 6 months or longer in Fontan related protein-losing enteropathy patients. This treatment was associated with significant symptomatic improvement and sustained increases in serum albumin but did not markedly change the ultimate outcome and was associated with significant side effects  .
Heparin has also been reported to improve protein-losing enteropathy in children after the Fontan procedure.
Heparin is thought to possibly have a stabilizing effect on the capillary endothelium, reducing protein leakage into the extravascular space and gut lumen, although the precise mechanism of action is unknown.
Although heparin has been successfully used to treat some patients with protein-losing enteropathy that develops after the Fontan procedure, it is by no means the treatment of choice for all the etiologies of protein-losing enteropathy.
Corticosteroids including budesonide, have been used in patients with protein-losing enteropathy associated with collagen vascular diseases, inflammatory bowel disease, heart surgery, and others. Sporadic case reports have documented the successful use of other agents such as cyclosporine for protein-losing enteropathy. Immunosuppressive drugs should not be used in cases of protein-losing enteropathy secondary to infections.
See the list below:
Conner et al reported a case in which localized resection of the involved bowel successfully treated the condition. 
In patients who have undergone a Fontan procedure, fenestration of the baffle that separates the systemic venous pathway from the pulmonary venous atrium has been performed to treat protein-losing enteropathy, and in some cases the symptoms have resolved, presumably because of the decrease in systemic venous pressure.
Cardiac transplantation has also been performed for the management of intractable protein-losing enteropathy related to previous Fontan surgery, with complete resolution of symptoms in most cases and with survival comparable to non-Fontan procedures undergoing transplantation. 
See the list below:
In patients whose protein-losing enteropathy is related to lymphatic pathology, decreasing the lymphatic circulation provides some benefit. This requires dietary limitation of long-chain triglycerides because their absorption from the gut stimulates lymphatic flow. In order to provide adequate energy, medium-chain triglycerides must be added as an alternative source of lipid calories.
As described below, fat soluble vitamins must also be supplemented because their absorption is compromised in these patients.
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