1/280. Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt. A 21-year-old white woman, born with a univentricular heart, had undergone staged procedures before Fontan correction. She then began to develop edema, protein-losing enteropathy, and ascites refractory to diuretic therapy. Cardiac angiography showed a patent right Blalock-Taussig shunt, with turbulent cavopulmonary circulation. After undergoing an unsuccessful attempt at coil embolization she then underwent shunt ligation, with resolution of symptoms and normalization of protein levels. This report draws attention to the importance of cavopulmonary laminar flow to prevent the development of protein-losing enteropathy. ( info) |
2/280. Spontaneous regression associated with apoptosis in a patient with acute-type adult T-cell leukemia. We describe a 76-year-old man with acute-type adult T-cell leukemia, who demonstrated a spontaneous decrease in leukemic cell number, apparently coincident with apoptotic cell death. On admission the patient's white blood cell count was 38.9 x 10(9)/l with 77% abnormal lymphocytes. He also had hypoproteinemia (4.3 g/dl) from protein losing enteropathy. After admission the leukemic cell count decreased without chemotherapy, reaching 5.9 x 10(9)/l after 2 months. Studies of peripheral lymphocytes demonstrated appearance of the apoptotic cells and dna ladder formation from the beginning of regression. Same truncated proviral dna was recognized in primary ATL cells through the whole clinical course. The hypoproteinemia improved with intravenous nutrition, followed by increase of the leukemic cells. This case is the first report that demonstrates tumor-cell apoptosis induced clinical regression in adult T-cell leukemia. Further, we speculate that the hypoproteinemia may have been involved in the leukemic cell apoptosis. ( info) |
3/280. Reversal of protein losing enteropathy with prednisone in adults with modified fontan operations: long term palliation or bridge to cardiac transplantation? Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation. ( info) |
4/280. Protein-losing enteropathy in congestive heart failure: diagnosis by means of a simple method. Protein-losing enteropathy is frequently overlooked in patients suffering from congestive heart failure. Although textbooks often quote heart failure as one of the causes of protein-losing enteropathy, this association has not been clearly documented in the literature. Furthermore, conventional diagnostic techniques for protein-losing enteropathy are both cumbersome and time-consuming. We report the first case of protein-losing enteropathy of a 79 year-old lady who suffered from dilated cardiomyopathy. The profound hypoalbuminemia could not be explained by urinary loss or impaired hepatic synthesis. Protein-losing enteropathy was confirmed by means of technetium-99m- (99mTc) labelled human serum albumin scintigraphy which showed diffuse activity in the entire colon. The use of this simple and non-invasive diagnostic technique in protein-losing enteropathy is discussed. ( info) |
5/280. Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy. Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management. ( info) |
6/280. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure. Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure. ( info) |
7/280. Fecal clearance of alpha1-antitrypsin with lansoprazole can detect protein-losing gastropathy. Detecting plasma protein loss from the stomach is quite difficult by measuring fecal alpha1-AT clearance because alpha1-AT is rapidly destroyed in the gastric juice at values below pH 3. We examined protein loss from the stomach using fecal alpha1-AT clearance with lansoprazole, a proton pump inhibitor. A 38-year-old Japanese male presented with hypoproteinemia (total serum protein: 4.4 g/dl). Abdominal scintigraphy using technetium-99m-labeled albumin revealed distinct radioactivity accumulation in the small intestine. It strongly suggested excessive protein loss to the gastrointestinal tract. Although, regular fecal alpha1-AT clearance was within the normal range (<13 ml/day), the fecal alpha1-AT clearance with the administration of lansoprazole was 80.5 ml/day. The results indicated that this method using lansoprazole is simple and useful for detecting protein-losing gastropathy. ( info) |
8/280. Protein-losing enteropathy caused by mesenteric venous thrombosis with protein c deficiency. A 64-year-old man presented with leg edema and hypoproteinemia. His alpha-1 antitrypsin clearance rate of 174 mL/day indicated the presence of protein-losing enteropathy (PLE). Computed tomographic scans demonstrated thickened ileal wall and mesenteric edema. angiography revealed occlusion in a peripheral branch of the superior mesenteric vein. Furthermore, both the patient and his son had low protein c levels. The patient was treated successfully with partial resection of the ileum. Histologic examination of the resected ileum revealed multiple erosions and submucosal fibrosis with organized venous thrombi in the mesenteric veins. This is the first case report of PLE caused by mesenteric venous thrombosis, and our findings suggest that serum protein was lost through erosion of the ileum caused by ischemia due to mesenteric venous thrombosis. ( info) |
9/280. Protein-losing enteropathy with an endoscopic feature of 'the watermelon colon'. A watermelon lesion involving the gastrointestinal tract other than the stomach has not been reported previously except for two cases with rectal involvement and another case with caecal involvement. This report describes a case of protein-losing enteropathy with endoscopic features of 'the watermelon lesion' involving nearly the entire colon. A 57-year-old man presented with a six-month history of generalized oedema and diarrhoea. Laboratory findings included elevated erythrocyte sedimentation rate and decreased serum total protein and albumin. Serum fluorescent anti-nuclear antibody, anti-dsDNA antibody and anti-La antibody were positive. Upper gastrointestinal endoscopy was unremarkable. colonoscopy showed seven or eight longitudinal reddish stripes along the entire length of the colon and rectum sparing the caecum. Biopsies from the duodenum and colon revealed mildly ectatic lymphatics in the submucosa. Colonic biopsy also exhibited some haemosiderin-laden macrophages in the muscularis mucosae and submucosa. 99mTc-human serum albumin scintigraphy demonstrated a leak into the intestinal lumen. Oral prednisolone therapy was started and his symptoms and signs gradually improved over months. Steroid could be withdrawn after 7 months of therapy, and the patient has remained free of symptoms for 1 year of follow-up after withdrawal of the steroid. ( info) |
10/280. Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet. We report on a patient with protein-losing enteropathy due to primitive intestinal lymphangiectasia with an early reversal of clinical and biochemical signs and a stable late reversal of pathologic signs after treatment with a hypolipidic diet enriched with medium-chain triacylglycerols. ( info) |