Protein-losing enteropathy

In passive immunotherapy immune globulin (Ig) is an effective replacement in most forms of antibody deficiency (14). In the past, plasma was used instead of immune globulin, but plasma is rarely indicated in the 1990s because of the risk of disease, particularly AIDS, transmission. Because plasma contains many factors in addition to immunoglobulins (Igs), plasma is, however, of particular value in patients with protein-losing enteropathy, complement deficiencies, and refractory diarrhea. 

Su J, Smith MB, Rerknimitr R, Morrow D Small intestine bacterial overgrowth presenting as protein-losing enteropathy. Dig Dis Sci 1998 43 679-681. 

Physiologically, these plasma changes are included here since they reflect impaired numbers or function of B-lymphocytes. Two broad categories are recognizable. First, such immunodeficiency states may exist on congenital basis and often do so with concurrent defects in the T cells (Fig. 1). Alternatively, severe reductions in immunoglobulin levels often develop in the course of chronic lymphocytic leukaemia and myeloma. Not dissimilar impairment of immune competence is found with nephrotic syndrome, protein-losing enteropathy or even malnutrition, and in these instances is equally profound. 

Copper deficiency may present as hematological changes (anemia, leukopenia, and neutropenia) and skeletal demineralization. In severe cases, such as in Menkes syndrome, copper deficiency is further manifested as hypothermia, depigmentation of hair and skin, progressive mental deterioration, and growth retardation. Factors predisposing to copper deficiency include malabsorption states, protein-losing enteropathy, nephrotic syndrome, copper-free parenteral nutrition, and copper-deficient enteral nutrition. 

H35. Hollander, F., and Horowitz, M. L, Serum proteins in gastric mucus and other secretions. Implications in relation to the protein-losing enteropathies. Gastroenterology 43, 75-83 (1962). 

Cardiac failure Protein-losing enteropathy Inflammatory diseases Injury... 

Exudation of large amounts of zinc-protein complexes into the intestinal lumen also may contribute to the decrease in plasma zinc concentrations which occur in patients with inflamamtory disease of the bowel. It seems likely that protein losing enteropathy because of other causes also may impair zinc homeostasis. Another potential cause of negative zinc balance is a massive loss of intestinal secretions. 

Sieving Protein Loss. Because of its small molecular size, AAG is lost into the urine in nephrotic syndrome and into gastrointestinal secretions in protein-losing enteropathy. 

Urinary or Gastrointestinai Loss. Because of its small size, AAT diffuses into the glomerular urine and into the gastrointestinal tract however, AAT is not seen in the excreted urine unless there is damage to the proximal tubular cells or marked overflow proteinuria, as in the nephrotic syndrome. In the latter case, the serum level of AAT may be depressed, especially in the absence of an acute phase reaction. AAT is normally present in the excreted stool, mostly compiexed to pancreatic trypsin and elastase. In protein-losing enteropathies, the loss may be greatly increased. 

Decreased Plasma Levels. Transferrin is a negative APR the most common cause of low levels is inflammation or malignancy. Decreased synthesis is seen with chronic liver disease and malnutrition (see Chapter 47). Protein loss, as in the nephrotic syndrome or protein-losing enteropathies, also results in low levels. In hereditary atrans-ferrinemia, a very low level of Tf is accompanied by iron overload but severe hypochromic anemia resistant to iron therapy. 

Nephrotic syndrome, bums, protein-losing enteropathy... 

This section includes a discussion of celiac disease, disac-charidase deficiency, bacterial overgrowth, bile salt malabsorption, and protein-losing enteropathy and the main laboratory investigations associated with their diagnosis. 

In the healthy bowel, fecal protein is largely derived from enterocytes shed from the mucosal surface and from intestinal secretions. The normal Gl loss of albumin is less than 10% of albumin catabofism, representing a daily loss of less than 1% to 2% of the serum protein pool. " Protein loss may be greatly increased in disease. In studies using Cr-labeled proteins, 0.1% to 0.7% of an injected dose was excreted in feces over 4 days in healthy subjects in protein-losing enteropathies this may increase to 40%, leading to hypoal-buminemia and edema. 

Florent C, L Hirondel C, Desmazures C, Aymes C, Bernier JJ. Intestinal clearance of tti-antitrypsin. A sensitive method for the detection of protein-losing enteropathy. Gastroenterology 1981 81 777-80. 

BGm KE. Protein losing enteropathy. In Feldman M, Friedman LS, Sleisenger MH, eds. Sleisenger and Fordtran s gastrointestinal and liver disease, 7th ed. PhEadelphia WB Saunders, 2002 446-52. 

Estimates of the daily catabolism of IgA vary from 0.6 g (G7) to 2 g (S17), mainly because of different estimates of the normal serum level, as all workers agree on a of about 6 days. It also appears that the degree of I labeling may have a bearing on how much IgA can enter the lamina propria pools (see Section 3.3) and become dimerized for secretion. The absolute total daily synthesis is thus not clear (probably 1.5 g), but the 7S IgA of the serum is distributed similarly to IgG and has an FOR of 40%. Thus even the increased catabolism of protein-losing enteropathy increases the FCR only to 60%, and the serum level rarely if ever falls below the 2 SD lower limit of normal. Subnormal IgA levels are therefore almost always due to impaired synthesis. It is of interest to note here that subjects born without IgA, presumably sensitized by breast-feeding or kissing, often develop antibodies to IgA and thereby show increased catabolism and occasionally reactions to administered IgA (S32). 

The raised IgA (Fig. 10 2) associated with malabsorption states— 60%i of children, (II) 20% of adults, (H31) active Crohn s disease (H39, PIO) and ulcerative colitis, etc.—similarly has no specific value. It seems fair to say persistence or relapse of a high IgA level indicates unmodified disease therefore reassessment of the measures taken, e.g., milk sensitivity, may have been missed (II) or a lymphoma may be developing (AlO). The various immune deficiencies aggravating or resulting from malabsorption can be readily distinguished by their immunoglobulin patterns (H31), e.g., protein-losing enteropathy (see Fig. 10 8), celiac disease (see Fig. 10 9). 

Naturally occurring copper deficiency has not been proven to occur in man, most probably because of minimal requirements and plentiful supply in the diet. The copper deficiency, suspected on the basis of low serum copper levels in infants with various types of protein-losing enteropathy (S48, Z3), is of doubtful pathognomonic significance. 

If left untreated, bacfllary dysentery usually lasts about 1 week (range 1-30 days). Complications are unusual but may include severe dehydration, generalized seizures, septicemia, toxic megacolon, perforated colon, arthritis, protein-losing enteropathy, and HUS. Mortality is rare, but it may be more likely with S. dysenteriae type I. Fess than 3% of persons who are infected with S. flexneri will later develop Reiter s syndrome, characterized by pains in the joints, irritation of the eyes, and painful urination. This can lead to chronic arthritis. ... 

Bowel obstruction, fistulae, radiation enteritis (diarrhea, protein-losing enteropathy, malabsorption)... 

Abnornud e.xcretion ordejirudation. The causes include the nephrotic syndrome, protein-losing enteropathies, burns, haemorrhage and catabolic stales. 

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