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Intestinal lymphangiectasia masquerading as coeliac disease.

Intestinal lymphangiectasia (IL) usually presents with either non-specific general or gastro-intestinal symptoms. As IL may mimic other gastro-intestinal disorders, the diagnosis is often delayed. Intestinal lymphangiectasia was diagnosed in three children who were originally treated as cases of coeliac disease. Two were sisters who had been placed on a gluten-free diet, for 3 years in one and 10 years in the other, with no favourable response. The third patient had been tried on various formulae and underwent many investigations for failure to thrive, oedema, abdominal distension and recurrent chest infections. The diagnosis of IL was based on clinical history, physical examination and radiological and histological findings. The three patients were commenced on a medium-chain triglyceride-based diet and vitamins, with satisfactory results.

Adolescent↗

Intestinal permeability to 99mTc-diethylenetriaminopentaacetic acid in inflammatory bowel disease.

Intestinal permeability in inflammatory bowel disease and its relation to periods of disease activity has been investigated by measuring the urinary excretion of DTPA labeled with 99mTc. Urine excretion in 10 control subjects was 2.7 +/- 1% of the test dose. Twelve patients with ulcerative colitis excreted 5.08 +/- 1.6% in remission, 10.61 +/- 2% during periods of mild activity, 19.41 +/- 0.9% during moderate activity, and 15.41 +/- 6.3% with severe activity. Sixteen patients with Crohn's disease excreted 5.7 +/- 1.9% in remission, 8.47 +/- 2.8% during mild activity of the disease, and 14.29 +/- 5.8% during moderate activity. No differences were observed between ulcerative colitis and Crohn's disease, or between ileal and colonic forms of Crohn's disease. Excretion in remission was significantly greater than in control subjects and there was a correlation between excretion and disease activity. In serial determinations done in seven patients we found that urine excretion of the test substance correlated with disease activity. We also studied DTPA excretion in 10 cases with gastric or duodenal ulcer (2.28 +/- 1.4%), six cases of acute gastroenteritis (4.87 +/- 3.1%) and nine cases with other intestinal diseases (3.6 +/- 1.1%). In all these cases, DTPA excretion was lower than in inflammatory bowel disease. Our results show that the urinary excretion of DTPA is a simple test that measures accurately the degree of activity of inflammatory bowel disease. The test is useful in Crohn's disease as well as in ulcerative colitis, and detects intestinal permeability abnormalities even in clinical remission. Significantly lower excretions are found in other intestinal diseases. The test may be recommended as a screening test for use in clinical practice.

Adolescent↗

Stenosis of the small intestine due to nonocclusive ischemic disease.

Intestinal stenosis following nonocclusive disturbances of mesenteric circulation will probably be encountered with increasing frequency as patients with low blood flow states resulting in transiently ischemic but viable bowel are treated early and successfully. The case reported here clearly documents the temporal sequence of dated cardiogenic shock and the subsequent onset of abdominal pain, culminating in intestinal stenosis and obstruction and necessitating later bowel resection. The pertinent literature has been reviewed. It is suggested that the likelihood of this complication be entertained in high-risk patients and supportive therapy be employed promptly and judiciously.

Humans↗

Diaphragm disease: pathology of disease of the small intestine induced by non-steroidal anti-inflammatory drugs.

Operative small bowel resection specimens received over a period of 16 years were reviewed to assess whether any intestinal disease could be directly attributed to the use of non-steroidal anti-inflammatory drugs (NSAID). Seven cases of intestinal disease associated with the use of NSAID were identified, all of which occurred in the final six years of the survey, which may reflect the increasing use of these compounds. A spectrum of patterns was found from multiple pathognomonic ileal mucosal diaphragms to broad strictures similar to those seen as a complication of enteric potassium. It seems likely that the formation of diaphragm lesions requires an additional factor, but what is not known as yet is whether the effects of NSAID are local or systemic.

Adult↗

Systemic immune complex disease following intestinal bypass surgery: bypass disease.

Twenty-one patients with arthritis and dermatitis following intestinal bypass surgery were studied. The arthritis was polyarticular, remittent, and intermittent. Typically, the synovial fluid was inflammatory. The commonest inflammatory skin lesion was a vesiculopustular dermatitis. Nineteen patients and serum immmune complexes using the Raji cell technic. Seventeen patients had serum cryoproteins, primarily consisting of IgG 1, IgG 3, C3, and C4. Three patients had both Escherichia coli antigens and anti-E. coli antibody in their cryoprotein. Five patients had granular and one had linear deposits of immunoglobulin and complement at the dermoepidermal junction. Further evidence that bacterial antigens play a role in tissue injury was provided by detection of granular deposits of E. coli antigen at the dermoepidermal junctions in two patients, and at the glomerular capillary basement membrane in one patient.

Adult↗

Altered patterns of secretion of monomeric IgA and IgA subclass 1 by intestinal mononuclear cells in inflammatory bowel disease.

Intestinal mononuclear cells (MNC) from patients with inflammatory bowel disease (IBD) demonstrated altered patterns of spontaneous secretion of immunoglobulin A (IgA). Control intestinal MNC had markedly high spontaneous secretion of IgA compared to IBD intestinal MNC. Control intestinal MNC secreted predominantly dimeric IgA (only 31% monomeric IgA), whereas IBD intestinal MNC secreted increased amounts (43%-53%) of monomeric IgA. Control intestinal MNC secreted 61% IgA subclass 1 (IgA1), whereas IBD intestinal MNC secreted 71%-74% IgA1. Intestinal MNC from involved portions of resected specimens secreted more of both monomeric IgA and IgA1 than MNC from uninvolved areas of the same bowel. Therefore, intestinal MNC from involved IBD intestinal specimens secrete less total IgA but high percentages of monomeric IgA and IgA1 compared to control intestinal MNC. This could be caused by increased homing of monomeric IgA- and IgA1-producing cells into involved intestine, or the in situ proliferation of monomeric IgA and IgA1 precursor cells resulting from immunoregulatory alterations. These observations may represent the normal mucosal IgA immune response to infectious agents or inducing factors of either a primary or a secondary nature.

Cell Movement↗

Investigation on lymphocyte subpopulations in the intestinal lamina propria in inflammatory bowel diseases.

Intestinal lamina proprial lymphocytes were enumerated in 13 patients with Crohn's disease (CD), 7 with ulcerative colitis (UC) and 9 control subjects with grossly normal bowel. The percentages and absolute numbers of B cells and complement receptor-bearing lymphocytes in the patients with UC did not differ significantly from those of the control group; but in CD, the percentages of these cells were significantly decreased despite that the absolute numbers were normal. The percentages and absolute numbers of T cells identified by E rosettes (regular and stable) were normal in both CD and UC. In both diseases, significantly increased percentages but normal absolute numbers of mature T cells were identified by monoclonal antibody staining with the CD3 reagent. There was an increased proportion of suppressor/cytotoxic T cells and a concomitant significant decrease of the helper to suppressor T-cell ratio (using either percentages or absolute numbers of cells) in tissues from patients with CD, but not with UC as compared with the control group. These results suggest that the imbalance of T-cell subpopulations in the intestinal lamina propria may play a role in the pathogenesis of CD.

Antibodies, Monoclonal↗

Tuberculous colitis mimicking Crohn's disease.

Intestinal tuberculosis is a rare disease in western countries and may mimic a variety of gastrointestinal disorders. Here, we report the case of a 63-yr-old patient who presented with profuse bleeding from a deep rectal ulcer. Similar lesions were found in different parts of the colon. Multilocular colorectal carcinoma was suspected based on the macroscopic appearance. Histology, however, suggested Crohn's disease. Intestinal tuberculosis was initially ruled out by negative staining for acid-fast bacilli, mycobacterial culture, and polymerase chain reaction analysis. A treatment for Crohn's disease was started. Endoscopic reexamination revealed progressive disease with extensive ulcerations of the terminal ileum. Histopathological examination then revealed acid-fast bacilli in the colonic mucosa typical for mycobacterium tuberculosis infection. This case emphasizes the need to include intestinal tuberculosis in the initial differential diagnosis of ulcerative colorectal lesions also in the western population.

Colitis↗

[Scanning electron microscopy (S.E.M.) aspects of intestinal mucosal surface in childhood coeliac disease].

Intestinal mucosal surface in 5 children suffered with Coeliac Disease was observed at Scanning Electron Microscopy. The comparison between two techniques of dehydration demonstrated that the Alcool-Amile Acetate dehydration reduces the technical errors to the minimum. Therefore, a better visualization allows us to recognize the surface coat, the microvillous and some mucosal functional aspects (scale off), which were previously undistinguished. There are no differences between the mucosal aspects in adult coeliac disease and childhood coelia disease.

Celiac Disease↗

[Treatment of chronic diseases of the small intestine].

Multimodality and differentiated treatment of small-intestinal diseases is to combine methods of etiological action with pathogenetic treatment of the main clinical syndromes: chronic diarrhea, malabsorption syndrome, hypercatabolic exudative enteropathy. Each nosological form should be treated specifically. Pathogenetic treatment involves diet therapy, chemotherapeutic correction of metabolic processes (vitamin administration, recovery of normal protein and lipid metabolism, water and electrolyte balance, anemia), management of chronic diarrhea. Treatment regimens are specified for gluten enteropathies, total variable immunodeficiency, Whipple disease, small-intestinal diverticulosis, Crohn's disease, amyloidoses, intestinal lymphoma and retroperitoneal lymph nodes. Clinical experience justifies the above methods as highly effective.

Chronic Disease↗

Diseases of the small intestine.

During the past 10 years considerable progress and increase of knowledge on small intestinal diseases has been achieved. This short review will cover some selected small bowel diseases including coeliac disease, Whipple's disease, rare congenital enteropathies, non-steroidal anti-inflammatory drug enteropathy and autoimmune enteropathy.

Anti-Inflammatory Agents, Non-Steroidal↗

Liver and small bowel transplantation: therapeutic alternatives for the treatment of liver disease and intestinal failure.

Intestinal transplantation is an established life-saving therapy for parenteral nutrition dependent patients suffering from severe complications of parenteral nutrition. Improvements in outcomes over the last decade have occurred with refinements in surgical technique, better immunosuppressive regimens, and clinical experience. The long-term results of intestinal transplantation are not well known and morbidity remains an important obstacle to wider application of this procedure to patients with short bowel syndrome (SBS). This article reviews the indications for intestinal transplantation, the evaluation of potential candidates, therapeutic considerations, postoperative management and common complications experienced by the recipients.

Humans↗

IFN-gamma-induced TNFR2 expression is required for TNF-dependent intestinal epithelial barrier dysfunction.

BACKGROUND & AIMS: Tumor necrosis factor (TNF) plays a critical role in intestinal disease. In intestinal epithelia, TNF causes tight junction disruption and epithelial barrier loss by up-regulating myosin light chain kinase (MLCK) activity and expression. The aim of this study was to determine the signaling pathways by which TNF causes intestinal epithelial barrier loss. METHODS: Caco-2 cells that were either nontransfected or stably transfected with human TNF receptor 1 (TNFR1) or TNFR2 and mouse colonocytes were used for physiologic, morphologic, and biochemical analyses. RESULTS: Colitis induced in vivo by adoptive transfer of CD4(+)CD45RB(hi) T cells was associated with increased epithelial MLCK expression and myosin II regulatory light chain (MLC) phosphorylation as well as morphologic tight junction disruption. In vitro studies showed that TNF caused similar increases in MLCK expression and MLC phosphorylation, as well as barrier dysfunction, in Caco-2 monolayers only after interferon (IFN)-gamma pretreatment. This reductionist model was therefore used to determine the molecular mechanism by which IFN-gamma and TNF synergize to cause intestinal epithelial barrier loss. IFN-gamma priming increased TNFR1 and TNFR2 expression, and blocking antibody studies showed that TNFR2, but not TNFR1, was required for TNF-induced barrier dysfunction. Transgenic TNFR2, but not TNFR1, expression allowed IFN-gamma-independent TNF responses. CONCLUSIONS: IFN-gamma primes intestinal epithelia to respond to TNF by inducing TNFR2 expression, which in turn mediates TNF-induced MLCK-dependent barrier dysfunction. The data further suggest that epithelial TNFR2 blockade may be a novel approach to restore barrier function in intestinal disease.

Animals↗

[Disturbances in volume and electrolytes with intestinal and kidney diseases].

The intestines and kidney are the most important excretion organs. Both organ systems are key players in keeping the homeostatic balance regarding hydration and electrolytes. Disturbances of function can lead to enormous and sometimes life-threatening complications. Intestinal diseases lead often to diarrhoea, which can be associated with fluid loss of up to 20 l per day. The accompanying electrolyte disturbances can be hypo- or hypernatremia in combination with hypokalemia. The therapy is substitution guided by knowledge of the pathophysiology. Kidney diseases lead to excessive volume and electrolyte balances, depending on the underlying molecular or pathological defect, but deficiencies can also be found. In case of kidney impairment with 30-50% total loss of function, calcium and phosphate metabolism is impaired.

Humans↗

Increased intestinal permeability in patients with inflammatory bowel disease.

Intestinal permeability can be measured by the sugar absorption test. This test is based on determining the ratio of the urinary excretion of a large and a small carbohydrate after oral administration. The aim of this study was to determine which combination of carbohydrates used in the test gives the highest correlation with disease activity in inflammatory bowel disease. 26 patients with Crohn's disease, 21 patients with ulcerative colitis and 27 healthy control subjects were included in the study. The patients with inflammatory bowel disease had either minimal or highly active disease or were in remission. Two disaccharides (lactulose: L, and cellobiose: C) and two smaller carbohydrates (rhamnose:R, and mannitol:M) were given orally and the urinary excretion was measured by high pressure liquid chromatography followed by pulsed amperometric electrochemical detection on a gold electrode. The ratios C/R, L/R, C/M and L/M were used as indicators for intestinal permeability. - There were no side effects of oral sugar administration. All patients tolerated the test well. Lactulose, rhamnose and cellobiose concentrations are easily be measured in the urine whereas mannitol measurement requires the use of an anion exchanger. This produced inconsistent results. Patients with Crohn's disease or with ulcerative colitis had increased permeability indices in comparison to healthy controls, even in remission. The L/R ratio gave a better differentiation between the healthy controls and patients with active disease than the other agents. Changes in disease activity are best reflected by use of cellobiose/rhamnose excretion quotient.

Adolescent↗