Clinical practice

Abstract

Protein-losing enteropathy (PLE) is a rare complication of a variety of intestinal disorders characterized by an excessive loss of proteins into the gastrointestinal tract due to impaired integrity of the mucosa. The clinical presentation of patients with PLE is highly variable, depending upon the underlying cause, but mainly consists of edema due to hypoproteinemia. While considering PLE, other causes of hypoproteinemia such as malnutrition, impaired synthesis, or protein loss through other organs like the kidney, liver, or skin, have to be excluded. The disorders causing PLE can be divided into those due to protein loss from intestinal lymphatics, like primary intestinal lymphangiectasia or congenital heart disease and those with protein loss due to an inflamed or abnormal mucosal surface. The diagnosis is confirmed by increased fecal concentrations of alpha-1-antitrypsin. After PLE is diagnosed, the underlying cause should be identified by stool cultures, serologic evaluation, cardiac screening, or radiographic imaging. Treatment of PLE consists of nutrition state maintenance by using a high protein diet with supplement of fat-soluble vitamins. In patients with lymphangiectasia, a low fat with medium chain triglycerides (MCT) diet should be prescribed. Besides dietary adjustments, appropriate treatment for the underlying etiology is necessary and supportive care to avoid complications of edema. PLE is a rare complication of various diseases, mostly gastrointestinal or cardiac conditions that result into loss of proteins in the gastrointestinal tract. Prognosis depends upon the severity and treatment options of the underlying disease.

Generalized edema with hypoproteinemia as initial presentation of celiac disease in a 3-year-old boy: case report

“…Celiac disease is considered to be the most common genetically predetermined condition with a prevalence of 1% in many European countries (4)(5)(6)(7)(8). The understanding of this disease has changed from gastrointestinal disease to a multisystem immune disorder (9).…”

Secondary Combined Immunodeficiency in Pediatric Patients after the Fontan Operation: Three Case Reports

“…The reduction of immunoglobulin levels can be explained by the loss of immunoglobulins into the gastrointestinal tract [16]. A predominant IgG decrease can be explained by the extensive loss of proteins [20] with slow turnover, including albumin and IgG [1]. In contrast, proteins with shorter half-lives, such as clotting factors, prealbumin or transferrin, are preserved nearly in the normal range [1,4].…”

“…Together with plastic bronchitis, PLE is a late, life-threatening complication in these patients [8]. PLE is characterized by excessive protein loss through the gastrointestinal mucosa [1]. This condition leads to hypoalbuminemia, hypoproteinemia, elevated alpha-1-antitrypsin clearance, lymphocytopenia and hypocalcemia that are marked clinically by ascites, edema of the limbs, pleural and cardiac effusions and muscle cramps [10,11,12].…”

“…Several rare and complex inborn heart anomalies that are marked by an underdeveloped left side of the heart or a defect of the heart valves can be corrected by a series of palliative surgeries that are finalized by the Fontan procedure (total cavopulmonal connection, TCPC) [1]. The main inborn heart defect that is corrected by TCPC is hypoplastic left heart syndrome (HLHS), with an incidence of 0.16-0.36 per 1,000 live births [2,3,4].…”

Guía colombiana de práctica clínica para el uso de inmonuglobulinas en el tratamiento de reemplazo e inmunomodulación

“…(GRADE A1) 65,70 Enfermedades con pérdida de proteínas R Se ha documentado hipogammaglobulinemia e infecciones secundarias asociadas con diversas patologías, que incrementan la pérdida de proteínas a nivel intestinal (enteropatía perdedora de proteínas, linfangiectasias), renal (síndrome nefrótico, nefritis asociada a enfermedades autoinmunes), torácico (quilotórax) y piel (quemaduras); sin embargo, no existen estudios controlados que evalúen la utilidad de la IgIV o la inmunoglobulina subcutánea en estos pacientes. (GRADE C2) 71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87 Inmunodeficiencias secundarias al uso de anticonvulsivantes R Se ha observado hipogammaglobulinemia en individuos que reciben tratamiento con anticonvulsivantes (carbamacepina, fenitoína, lamotrigina, entre otros). El tratamiento de reemplazo está indicado en pacientes con niveles bajos de IgG y con infecciones graves o recurrentes cuando no pueden emplearse otros anticonvulsivantes o cuando la hipogammaglobulinemia persiste después de suspenderlos.…”