Enteropathy syndrome

Owen CJ, Jennings CE, Imrie H, Lachaux A, Bridges NA, Cheetham TD, Pearce SH (2003) Mutational analysis of the FOXP3 gene and evidence for genetic heterogeneity in the immunodysregulation, polyendocrinopathy, enteropathy syndrome. J Clin Endocrinol Metab, 88 6034-6039. 

Gluten intolerance Enteropathy syndrome, also known as celiac disease, caused by the ingestion of gluten present in wheat, barley, rye, triticale, and possibly, oat products it is more common in Caucasians. It may be defined as an inflammatory... 

Extranodal NK/T-cell lymphoma, nasal type Enteropathy-type T-cell lymphoma Hepatosplenic gamma-delta T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides/sezary syndrome... 

Rifaximin Rifamycin Antibiotic Gut bacteria Enteric infection Diarrhea, infectious Hepatic encephalopathy Small intestine bacterial overgrowth Inflammatory bowel disease Colonic diverticular disease Irritable bowel syndrome Constipation Clostridium difficile infection Helicobacter pylori infection Colorectal surgery Bowel decontamination, selective Pancreatitis, acute Bacterial peritonitis, spontaneous Nonsteroidal anti-inflammatory drug enteropathy... 

Transferrin 8-9 Binds iron in plasma and transports iron to bone Iron deficiency, pregnancy, hypoxia, chronic blood loss, estrogens Chronic infection, cirrhosis, burns, enteropathies, nephrotic syndrome, cortisone, testosterone... 

The change in daily output of fecal fat forms the basis for the definitive diagnostic test for gluten induced enteropathy (F16, F21). This diagnosis is justified if the patient presented with the main features of the malabsorption syndrome, if the fecal fat output fell to normal levels on a gluten-free diet, and if subsequent reintroduction of gluten into the diet caused an unequivocal increase in fecal fat... 

M7. Milhaud, G., and Vesin, P., Calcium metabolism in man with calcium-45 malabsorption syndrome and exudative enteropathy. Nature 191, 872-874 (1961). 

Gaudy AA, Reddy ST, Chatila T, Atkinson JP, Verbsky JW CD25 deficiency causes an immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like syndrome, and defective IL-10 expression from CD4 lymphocytes. J Allergy Chn Immunol 2007 119 482-487. 

Wildin RS, Ramsdell F, Peake J, Faravelli F, Casanova JL, Buis TN, et ak X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome is the human equivalent of mouse scurfy. Nat Genet 2001 27 18-20. 

Baud 0, Goulet O, Canioni D, et al Treatment of the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) by allogeneic bone marrow transplantation. N Engl J Med 2001 344 1758-1762. 

Zhan H, Sinclair J, Adams S, et al Immune reconstitution and recovery of FoxP3 (forkhead box P3)-expressing T cells after transplantation for IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-hnked) syndrome. Pediatrics 2008 121 e998-el002. 

Vitamin 8 2 deficiency Vitamin B-12 deficiency due to malabsorption syndrome as seen in pernicious anemia Gl pathology, dysfunction or surgery fish tapeworm infestation malignancy of pancreas or bowel gluten enteropathy sprue small bowel bacterial overgrowth total or partial gastrectomy accompanying folic acid deficiency. Increased vitamin B-12 requirements Increased vitamin B-12 requirements associated... 

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. 

Of significant interest is an attempt to nse Enterosgel for therapy of chronic hard-to-treat diseases of gastro-intestinal tract snch as malabsorption syndrome, gluten enteropathy, exacerbation of chronic enteritis, post-resection syndrome and the syndrome of irritated large bowel. 

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. 

Malabsorption syndromes. Particularly in gluten-sensitive enteropathy and tropical sprue, poor absorption of folic acid from the small intestine often leads to a megaloblastic anaemia. 

An elevation of ChE activity can be detected in fatty liver, obesity, diabetes mellitus, exudative enteropathy, nephrotic syndrome, hyperthyroidism, Meulengracht s icterus, chronic obstructive jaundice, etc. Specificity in liver diseases is 61%, and sensitivity is 49%. In cirrhosis, however, sensitivity is 88% normal ChE therefore widely excludes cirrhosis. In connection with other hepatobiliary enzymes, ChE can be useful in the diagnosis and assessment of the course of liver disease. There is a very good correlation of ChE activity with coagulation factors in liver diseases however, the correlation is less significant with albumin synthesis. 

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. 

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