Stig Jarnum

Diffuse Gastrointestinal Polyposis with Ectodermal Changes: A case with severe malabsorption and enteric loss of plasma proteins and electrolytes

Summary A case of Cronkhite-Canadas syndrome is presented. The syndrome, which is extremely rare, is characterized by diffuse gastrointestinal polyposis, alopecia, nail atrophy, and hyperpigmentation of the skin. Severe malabsorption, hypoproteinemia, and marked depression of serum calcium, magnesium, and potassium were additional features. It could be shown by means of labeled proteins that large amounts of albumin and /G-globulin leaked into the intestinal tract and, in particular, into the colon, whereas gastric protein loss was negligible. Also, although no proper balance studies could be carried out, evidence of an intestinal net loss of electrolytes was present. Severe lesions of the entire gastrointestinal tract were found during laparotomy and at autopsy. The macroscopic picture was different at various sites (polyps in the stomach, villous atrophy in the small intestine, hypertrophic rugae with scattered polyps in the colon). In contrast, the microscopic picture was highly uniform and characterized by cystlike dilations of the glands. The etiology of the syndrome remains obscure. The ectodermal changes seem to be an inherent part of the syndrome and not secondary to malabsorption.

Hypoalbuminemia in gastric carcinoma.

Summary In 9 patients suffering from gastric carcinoma, a pronounced hypoalbuminemia varying from 2 to 3 gm. per 100 ml. was found in 8. In 7 patients an abnormal fecal excretion of I 131 -polyvinylpyrrolidone was found after intravenous injection of the labeled polymer, suggesting a gastrointestinal leakage of high molecular-weight substances. By paper electrophoresis of gastric juice, albumin was detected in 5 of 9 patients, the concentration varying from trace to 0.30 gm. per 100 ml. Albumin production, as measured by means of I 131 -labeled albumin, was normal in 1 patient with a serum albumin of 2.19 gm. per 100 ml. He had small intra- and extravascular albumin pools, but a rapid turn-over, thus resembling albumin metabolism in nephrosis and protein-losing gastro-enteropathy.

Plasma protein turnover (albumin, transferrin, IgG, IgM) in Ménétrier's disease (giant hypertrophic gastritis): Evidence of non-selective protein loss

Ten cases of Ménétriers disease (`giant hypertrophic gastritis) have been studied with radioiodine-labelled albumin (all 10 cases), IgG (eight cases), transferrin (two cases), and IgM (six cases). Abnormal gastric loss of plasma protein was present in all cases as demonstrated by 59Fe-iron dextran (eight cases), 51Cr-albumin (one case), and 131I-polyvinylpyrrolidone (one case). None of the patients had more distal gastrointestinal lesions. The synthetic rate of the proteins studied was normal or slightly elevated. The fractional catabolic rate of the proteins was increased. The increase above the normal mean was similar for albumin, transferrin, and IgG. Since the molecular weight of IgG is more than twice that of albumin and transferrin, it is concluded that the protein loss in Ménétriers disease is nonselective in the sense that it affects a similar fraction of the intravascular masses of all plasma proteins. IgM catabolism was strongly accelerated. Simultaneous studies with 50Fe-iron dextran, radioiodine-labelled albumin or IgG showed that IgM hypercatabolism could only partly be due to abnormal gastric protein loss, since IgM catabolism was significantly more raised than that of albumin and IgG. Faecal radioiodine excretion was normal in most patients after the injection of radioiodine-labelled proteins. It confirms a previous observation that increased gastrointestinal 59Fe clearance after injection of 59Fe-iron dextran associated with normal faecal radioiodine excretion after the injection of radioiodine-labelled proteins permits of a diagnosis of protein loss in the stomach.

IgG and Albumin Turnover in Crohn's Disease

Simultaneous IgG and albumin turnover studies were performed in 10 patients with Crohns disease of the terminal ileum, the colon, or both, by means of radioiodinated proteins. An abnormal intestinal protein loss was demonstrated in everyone with 59Fe-labelled iron dextran. The serum level of IgG was normal in 5 and elevated in 3 cases. The higher the serum level, the higher was the fractional catabolic rate (per cent of intravascular mass catabolized per day). The synthetic rate was markedly increased in most cases. In contrast, serum albumin was negatively correlated to its catabolic rate. Albumin synthesis was normal or moderately elevated. The regression between the catabolic rates of IgG and albumin in 7 cases of intestinal lymphangiectasia and in 10 cases of Crohns disease suggested that, in intestinal lymphangiectasia, the protein loss of albumin was higher than that of IgG, whereas, in Crohns disease, the IgG catabolism for unknown reasons was higher than that of albumin. All patients with Crohn...

Transferrin Metabolism in the Nephrotic Syndrome and in Protein-losing Gastroenteropathy

The metabolism of transferrin was studied by means of radioiodinated transferrin in nine normal subjects, fifteen patients with protein-losing gastroenteropathy, and in eight with the nephrotic syndrome. In normal subjects the mean fractional catabolic rate was 17 per cent of intravascular mass per day and the synthetic rate 16 mg per kg per day. On an average 49 per cent of total transferrin was located intravascularly. Seven of eight patients with the nephrotic syndrome had an increased ‘endogenous’ fractional catabolic rate. The synthetic rate was above normal in seven nephrotic patients. No evidence was found that the glomerular permeability of transferrin and albumin was unequal. In patients with protein-losing gastroenteropathy the synthetic rate was increased in nine out of fifteen. In twenty-one patients, thirteen with protein-losing gastroenteropathy and eight with the nephrotic syndrome, who were studied simultaneously with transferrin and albumin, the serum concentration of albumin was decrease...

Turnover of IgG and IgM in myotonic dystrophy.

IN RECENT YEARS the metabolism of different plasma proteins has been intensively studied in a variety of diseases. In neurological disorders of possible autoimmune etiology, the attention naturally has been focused on the turnover of the immunoglobulins. Patients with myotonic dystrophy have a low serum concentration of IgG due to an accelerated endogenous degradation rate of this pr0tein.l In contrast, the low concentration of IgA in serum from patients with ataxia-telangiectasia is due to a deficient synthesis.2 In the present report, the turnover of radioiodine-labeled IgG and IgM has been investigated in patients with myotonic dystrophy.

IgM turnover in Crohn's disease

Simultaneous turnover studies with radioiodine-labelled IgM and IgG were made in 12 patients with Crohns disease. Intestinal protein loss was estimated by means of 59Fe-labelled iron dextran. The serum levels of IgM, IgG, and IgA were normal in most cases. The catabolic rate of IgM was increased in all but one case. A positive correlation was present between the catabolic rate and serum concentration of IgM, an observation which, so far, has been made only in Crohns disease. The synthetic rate of IgM was raised or high normal in four cases with an intraabdominal abscess. It was normal in the remaining cases. A strong positive correlation was found between the synthetic rates of IgM and IgG. The size of the protein loss was unrelated to the raised catabolic rates of IgM and IgG. Faecal radioiodine excretion from labelled IgM and IgG bore no relation to faecal 59Fe excretion, nor did it indicate the site of the intestinal lesion. However, a close correlation was observed between faecal excretion of the labels from IgM and IgG.

Fecal Radioiodide Excretion Following Intravenous Injection of 131I-Albumin and 125I-Immunoglobulin G in Chronic Inflammatory Bowel Disease.: An aid to topographic diagnosis

Simultaneous studies with 131-I-albumin and 125-I-immunoglobulin G (IgG) were made in 48 cases of chronic inflammatory bowel disease. Twenty-one had ulcerative colitis and 27 had Crohns disease which was confirmed at laparotomy in every case. Intestinal protein loss was measured simultaneously by means of 59-Fe-iron dextran in 44 patients. All patients had abnormal intestinal protein loss. A high correlation was shown between fecal 59-Fe clearance and fractional catabolic rate of albumin, confirming the validity of 59-Fe-iron dextran as a test substance to measure intestinal protein loss. Fecal radioiodide excretion of 131-I from 131-I-albumin (A) and 125-I from 125-I-IgG (G) was significantly different in ulcerative colitis and Crohns disease. The ratio G/A was close to unity (smaller than 1.60) in ulcerative colitis and Crohns disease with exclusive or predominant involvement of the colon, whereas it was high in Crohns disease of the small intestine and highest in cases with jejunal involvement. Thus, the ratio may be valuable in topographic diagnosis of chronic inflammatory bowel disease. A high ratio was found in 2 patients with Crohns disease of the small intestine and normal radiography of the small intestine, and a low ratio was present in 7 cases of ulcerative colitis with normal radiographic findings. In all 9 patients with normal radiography, fecal 59-Fe clearance was elevated as evidence of abnormal intestinal protein loss. No correlation was present between the size of protein loss and the pathoanatomic extent of the lesions on subsequent laparotomy in 25 patients with Crohns disease. Fecal radioiodide excretion (131-I from 131-I-albumin and 125-I from 125-I-IgG) was positively correlated with diarrhea (daily stool mass) in both ulcerative colitis and Crohns disease. Intestinal protein loss was not.

Albumin and Immunoglobulin Turnover in Patients with Chronic Active Liver Disease Treated with Prednisone

Albumin and IgG turnover were studied in 12 patients with chronic active liver disease before, and at a variable time interval during, prolonged prednisone treatment. Before treatment, serum albumin concentration was subnormal in 9 and albumin synthetic rate reduced in 6 and low normal in 4. Serum IgG concentration and IgG synthetic rate were increased in all patients. During treatment 8 patients improved or were unchanged, and 4 deteriorated. In the former group the mean serum albumin concentration and albumin synthetic rate increased significantly, in the latter both values decreased. Serum IgG concentration and IgG synthetic rate decreased in all but one during treatment. The changes were more pronounced in the former than in the latter group. The changes in serum concentrations and synthetic rates of albumin and IgG were significantly negatively correlated, both in the whole material, and especially in patients who improved. The synthetic rates of albumin and IgG were not correlated before or during p...

ALBUMIN METABOLISM AND GASTROINTESTINAL PROTEIN LOSS IN CHILDREN WITH NEPHROTIC SYNDROME

In order to assess the significance of gastrointestinal protein loss as a cause of plasma protein hypercatabolism in patients with nephrotic syndrome, seven nephrotic children were studied simultaneously with radioiodine labelled albumin and a test substance, 51,Fe‐labelled iron dextran, for evaluation of gastrointestinal protein loss.