Medical Care

Focus treatment on correcting the underlying process causing the protein-losing gastroenteropathy.^[23]For example, the patient with congestive heart failure may respond to digitalis and diuretics, whereas the patient with intestinal parasites should be treated with the appropriate medication for the infestation.

CHAPLE disease

Pozelimab is indicated for treatment of CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and severe protein-losing enteropathy (CHAPLE) syndrome in patients aged 1 year and older.^[36]Pozelimab is an immunoglobulin (Ig) antibody that is directed against terminal complement protein C5. The role of C5 is to inhibit terminal complement activation by blocking cleavage of C5 into C5a (anaphylatoxin) and C5b. This action blocks formation of the membrane-attack complex (C5b-C9), a structure mediating cell lysis.

FDA approval was based on results from an open-label phase 2/3 trial (N = 10).^[37]All 10 patients achieved normalization of serum albumin and serum IgG concentrations by week 12 and maintained these concentrations through at least 72 weeks of treatment. Five of the 10 patients received a total of 60 albumin transfusions in the 48 weeks preceding treatment. In the 48 weeks after starting treatment, one patient received a single albumin transfusion. Nine of the 10 patients were hospitalized for a total of 268 days in the 48 weeks before treatment, whereas two patients were hospitalized for a total of 7 days in the 48 weeks after starting treatment.^[37]

Surgical Care

Surgery for giant hypertrophic gastropathy (Ménétrier disease) and localized lymphatic obstruction has been suggested.^[10]Surgical lymphovenous anastomosis may also be of benefit in these patients.

Eradicating Helicobacter pylori has also been shown to decrease gastric protein loss in some patients with giant hypertrophic gastropathy.^[10]

Diet

A low-fat diet with supplementation with medium-chain triglycerides is theoretically of benefit in patients with lymphangiectasias. However, in practice, ingesting a diet containing medium-chain triglycerides results in increased blood flow with no reduction in fecal protein loss.^{[38, 39]}

Patients with celiac sprue typically respond to a gluten-free diet. A minority requires corticosteroids.

Medication

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Vyas H, Driscoll DJ, Cetta F, et al. Gastrointestinal bleeding and protein-losing enteropathy after the Fontan operation. Am J Cardiol. 2006 Sep 1. 98(5):666-7. [QxMD MEDLINE Link].
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Protein-Losing Enteropathy Treatment & Management

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