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Clinical practice. Protein-losing enteropathy in children.

Braamskamp MJ, Dolman KM, Tabbers MM - Eur. J. Pediatr. (2010)

Bottom Line: The diagnosis is confirmed by increased fecal concentrations of alpha-1-antitrypsin.Besides dietary adjustments, appropriate treatment for the underlying etiology is necessary and supportive care to avoid complications of edema.Prognosis depends upon the severity and treatment options of the underlying disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Sint Lucas Andreas Hospital, Amsterdam, The Netherlands.

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.

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Work up in children with hypoalbuminemia and edema
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Fig2: Work up in children with hypoalbuminemia and edema

Mentions: After PLE has been diagnosed, a search for the underlying cause should be performed. Bacterial, viral, and parasitic stool cultures can be used to search for infectious diseases. Serologic evaluation will reveal immune disorders and ECG or echocardiography might be necessary to determine the cardiac condition. To localize the intestinal area of involvement, radiographic studies like CT or MRI can be used. Endoscopy can detect characteristic mucosal alterations like giant gastric folds in Ménétrier’s disease. In patients with suspected lymphangiectasia, a high-fat meal before endoscopy is recommended because this increases enteric lymph flow and elevates lymphatic pressures leading to more prominent lymphatics with increased leakage of lymph into the bowel lumen, seen as white spots on the mucosa (Fig. 1). During endoscopy, biopsies are obtained to confirm histological features. Wireless capsule endoscopy identifies abnormalities of the small intestinal intestine in a non-invasive way without using ionizing radiation. It can safely be performed in children >1.5 years old [12]. The approach to the patient with suspected protein-losing gastroenteropathy is outlined in Figure 2.Fig. 1


Clinical practice. Protein-losing enteropathy in children.

Braamskamp MJ, Dolman KM, Tabbers MM - Eur. J. Pediatr. (2010)

Work up in children with hypoalbuminemia and edema
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2926439&req=5

Fig2: Work up in children with hypoalbuminemia and edema
Mentions: After PLE has been diagnosed, a search for the underlying cause should be performed. Bacterial, viral, and parasitic stool cultures can be used to search for infectious diseases. Serologic evaluation will reveal immune disorders and ECG or echocardiography might be necessary to determine the cardiac condition. To localize the intestinal area of involvement, radiographic studies like CT or MRI can be used. Endoscopy can detect characteristic mucosal alterations like giant gastric folds in Ménétrier’s disease. In patients with suspected lymphangiectasia, a high-fat meal before endoscopy is recommended because this increases enteric lymph flow and elevates lymphatic pressures leading to more prominent lymphatics with increased leakage of lymph into the bowel lumen, seen as white spots on the mucosa (Fig. 1). During endoscopy, biopsies are obtained to confirm histological features. Wireless capsule endoscopy identifies abnormalities of the small intestinal intestine in a non-invasive way without using ionizing radiation. It can safely be performed in children >1.5 years old [12]. The approach to the patient with suspected protein-losing gastroenteropathy is outlined in Figure 2.Fig. 1

Bottom Line: The diagnosis is confirmed by increased fecal concentrations of alpha-1-antitrypsin.Besides dietary adjustments, appropriate treatment for the underlying etiology is necessary and supportive care to avoid complications of edema.Prognosis depends upon the severity and treatment options of the underlying disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Sint Lucas Andreas Hospital, Amsterdam, The Netherlands.

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.

Show MeSH
Related in: MedlinePlus