PROTEIN-LOSING ENTEROPATHy IS AN UNCOMPENSATED LOSS OF PLASMA proteins in the intestine, indicated by elevated alpha1-antitrypsin levels in the stool, which leads to panhypoproteinemia in the absence of liver or kidney disease. Protein-losing enteropathy is a syndrome, not a disease; therefore, it is important to identify the underlying cause.1 Many acquired and congenital diseases are manifested as protein-losing enteropathy (Tables 1 and 2). Generally, these disorders either damage the intestinal mucosa or block gastrointestinal lymphatic flow through obstruction and lymphangiectasia. Improved diagnostic approaches, especially genomic testing, have revealed disease entities causing protein-losing enteropathy and have led to the development of effective therapies. The presenting features of protein-losing enteropathy are hypoproteinemia, edema, nutritional deficiencies, infections, and gastrointestinal symptoms, including diarrhea, steatorrhea, abdominal pain, and vomiting. Hypoproteinemia is nonse-lective, with reduced albumin and immunoglobulin levels. Edema (of the face and arms and legs) and effusions (peritoneal, pleural, and pericardial) are caused by reduced oncotic pressure in blood. Infections can result from hypogammaglobulinemia and lymphopenia. In children, malabsorption and malnutrition may be severe, resulting in retarded growth and development.
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