Gastrointestinal bleeding and protein-losing enteropathy after the fontan operation

Gastrointestinal bleeding has not been reported in patients with protein-losing enteropathy. The investigators describe 3 patients with protein-losing enteropathy and gastrointestinal bleeding after Fontan operations. Bleeding occurred at the onset of protein-losing enteropathy, was macroscopic, led to anemia, and required blood transfusion in all patients. Invasive testing failed to demonstrate gastrointestinal pathology as a source of bleeding. In conclusion, unexplained gastrointestinal bleeding may be part of the clinical spectrum of protein-losing enteropathy.     

Application of Technetium-99m-Labeled Human Serum Albumin Scan to Assist Surgical Treatment of Protein-Losing Enteropathy in Cronkhite-Canada Syndrome: Report of A Case

Cronkhite-Canada syndrome is a rare form of nonhereditary gastrointestinal polyposis associated with ectodermal change and protein-losing enteropathy. Here we report a 63-year-old male presenting with diffuse gastrointestinal polyposis, onychodystrophy, cutaneous pigmentation, alopecia, diarrhea, hypoalbuminemia and lower leg edema. Technetium-99m-labeled human serum albumin scan confirmed the patient to have protein-losing enteropathy, which originated from the transverse and descending colon. Subtotal colectomy was performed. Albumin level and ectodermal change were gradually improved during three years of outpatient clinic follow-up. Based on our finding, Technetium-99m-labeled human serum albumin scintigraphy is helpful to localize the protein-losing origins and surgery is an effective treatment for Cronkhite-Canada syndrome with protein-losing enteropathy.     

Medium Chain Triglycerides (MCT) in the Treatment of Protein-losing Enteropathy and Malabsorption Syndromes

Patients with protein-losing enteropathy and other malabsorption syndromes were treated with a low fat diet supplemented with medium chain triglycerides (MCT). A total of 18 patients were studied.

In four out of five cases with protein-losing enteropathy a noticeable improvement of protein loss and steatorrhoea occurred. In the fifth patient, who had no steatorrhoea, improvement was slight.

In nine patients with steatorrhoea secondary to pancreatic insufficiency (three cases), Crohn’s disease (one), intestinal resection (one), intestinal resection + blind loop syndrome (one), postgastrectomy + enteropathy of undefined nature (one), sarcoidosis with mesenteric gland involvement (one), and generalized mastocytosis (one), clinical improvement followed the institution of MCT diet in every case, whereas steatorrhoea ceased in all except two with chronic pancreatitis.

In four patients with chronic intestinal disease without steatorrhoea the beneficial cffect of MCT varied. A low fat diet supplemented with MCT seems to represent an important advance in the medical management of a variety of malabsorption syndromes.     

Scintigraphic diagnosis of protein-losing enteropathy secondary to amyloidosis.

Protein-losing enteropathy is an uncommon syndrome of excessive loss of protein via the gastrointestinal mucosa. 99mTc-dextran scintigraphy was performed on a 42-year-old woman with protein-losing enteropathy. She had secondary amyloidosis due to rheumatoid arthritis. Abnormal leakage of the radiotracer was observed in the mid-abdominal region suggesting the site of protein loss. It is concluded that 99mTc-dextran scintigraphy is useful as a noninvasive and simple test for the imaging and confirmation of diagnosis in protein-losing enteropathy.     

Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia

We study two authentic cases of protein-losing enteropathy, the diagnosis of which was facilitated using Given M2A videocapsule endoscopy. The first case corresponded to a primary intestinal lymphangiectasia confirmed by jejunum biopsies and the second one to a protein-losing enteropathy with lymphatic abnormalities secondary to a chronic constrictive pericarditis. In the first case, the mucosa of jejunum presented with a diffuse oedematous aspect, whitish villi, white curved lines probably related to submucosal dilated lymphatics and lacteal juice. In the second case, capsule endoscopy showed oedematous aspect of jejunum mucosa associated with white curved lines similar to those observed in the first case. Videocapsule endoscopy is useful in cases of protein-losing enteropathy to identify presence of intestinal lymphangiectasia and to specify their localisation after ruling out other disorders liable to induce protein-losing gastrointestinal syndrome.     

Legionella pneumonia and bacteraemia in association with protein-losing enteropathy after Fontan operation.

A 20-year-old young man suffered from severe protein-losing enteropathy 4 years after Fontan operation. His postoperative haemodynamics were unfavourable in terms of poor myocardial contractility and moderate atrioventricular prosthetic paravalvar leakage. A 2-week trial of steroid therapy for protein-losing enteropathy was given without success. The clinical course was complicated by Legionella pneumophila pneumonia and bacteraemia, which respond to the combination therapy with intravenous erythromycin and ciprofloxacin. The vulnerability of the patient to infection was related to an immuno-deficiency state secondary to protein-losing enteropathy and steroid therapy. Steroids should be used with caution in the management of protein-losing enteropathy after Fontan operation.     

Protein-Losing Enteropathy in Systemic Lupus Erythematosus

Although protein-losing enteropathy can be associated with a variety of disorders, only three cases have been described in association with systemic lupus erythematosus. In the case described herein, protein-losing enteropathy was the only clinical manifestation of systemic lupus erythematosus. Small intestinal biopsy revealed edema and mild mononuclear cell infiltration in lamina propria mucosae and no evidence of lymphangiectasia. X-ray studies of the gastrointestinal tract were normal. Protein-losing enteropathy responded to high-dose corticosteroid therapy. Protein-losing enteropathy should be suspected as a possible cause of unexplained hypoalbuminemia in systemic lupus erythematosus.     

PROTEIN-LOSING ENTEROPATHY IN SYSTEMIC LUPUS ERYTHEMATOSUS

Two patients with systemic lupus erythematosus (SLE) presented with anasarca, pleural effusions and severe hypoalbuminema, Both were demonstrated to have protein-losing enteropathy, a rare complication of SLE. Other causes of gastrointestinal protein loss were excluded. There were marked similarities in both cases including circulating ANF with speckled staining, anti-(U1)RNP antibodies and low serum complement levels. Complete remission was achieved in both with prednisolone. Anti-(U1)RNP may be a marker for a subset of SLE in which protein-losing enteropathy is a major manifestation.     

Serum cholesterol in idiopathic and lupus-related protein-losing enteropathy

Abstract The characteristics of protein-losing enteropathy were evaluated in patients with systemic lupus erythematosus. Among the patients with systemic lupus erythematosus (n=380) in a tertiary hospital, we reviewed the records of seven patients with generalized edema, hypoalbuminemia without proteinuria and positive results on 99mTc-labelled human serum albumin scintigrams. Patient characteristics and laboratory findings were compared between these seven patients and patients with lupus enteritis (n=15) or idiopathic protein-losing enteropathy (n=11). Compared with the lupus enteritis patients, the erythrocyte sedimentation rate and serum total cholesterol levels were significantly increased in patients with systemic lupus erythematosus-related protein-losing enteropathy. Compared with idiopathic protein-losing enteropathy patients, the level of serum total cholesterol was significantly increased, but the level of serum albumin was decreased in patients with systemic lupus erythematosus-related protein-losing enteropathy. Among patients with systemic lupus erythematosus-related protein-losing enteropathy, four patients had high serum total cholesterol levels (>or=248 mg/dL) and achieved complete remission after receiving high doses of steroid treatment. However, three patients who had low serum total cholesterol levels (相似文献   

Protein-Losing Gastroenteropathy Detected by Technetiuni-99m-Labeled Human Serum Albumin

Gastric and fecal clearance of alpha 1-antitrypsin were measured in three cases of protein-losing gastroenteropathy and in two control cases. Abdominal scintigraphy using 99mTc-labeled human serum albumin was performed in all five subjects. All three cases of protein-losing gastroenteropathy showed 99mTc activity in the gastrointestinal tract. In the patient with hypertrophic gastropathy, the activity was seen initially in the stomach and duodenum. In the patients with intestinal lymphangiectasia, the activities were initially in the small bowel. We conclude that 99mTc scintigraphy was useful, not only in diagnosing protein-losing enteropathy, but, also, in detecting the responsible region of the gastrointestinal tract.     

Severe esophagitis in a child with Henoch-Sch?nlein purpura presenting as protein-losing enteropathy.

A 4-year-old boy was seen for vomiting, diarrhea and peripheral edema. He had no evidence of nephrosis or liver dysfunction. Upper gastrointestinal endoscopy was performed for investigating the etiology of protein-losing enteropathy. It showed severe esophagitis and multiple ulcers in the descending duodenum. The symptoms and endoscopic mucosal abnormalities subsided after three weeks of hospitalization without specific therapy. Ten days after being discharged he was seen again with characteristic rash of Henoch-Sch?nlein purpura and arthritis without gastrointestinal symptoms. Biopsy of the skin rash revealed leukocytoclastic vasculitis.     

An autopsy case of protein-losing enteropathy due to gastrointestinal amyloidosis, occurring in empyema]

On August 14, 2001, a 76-year-old woman with a history of rheumatoid arthritis was admitted to our hospital with fever, cough, dyspnea and diarrhea. On admission, her chest radiography showed pleural effusion on the right side, and thoracocentesis was used to diagnose empyema. The patient underwent pleural drainage and received antibiotics. Alpha-Streptococcus was detected in both aerobic and anaerobic cultures of the pleural effusion. After 2 weeks of therapy, her empyema had improved; but her diarrhea, which had started 1 week before admission, had worsened, and her hypoproteinemia had progressed. Examination of the fecal clearance of alpha-1-antitrypsin and biopsied rectal material revealed that the diarrhea was caused by protein-losing enteropathy due to gastrointestinal amyloidosis secondary to rheumatoid arthritis. The patient was treated with steroids, but developed an additional infectious disease and died on September 29, 2001. In this case, she suffered from various infectious diseases including empyema and fungus infections. It has been reported that protein-losing enteropathy accompanies abnormalities in the immune system, by the loss of immunoglobulins and lymphocytes from the gut. We therefore suspect that protein-losing enteropathy due to gastrointestinal amyloidosis caused this patient's empyema.     

Protein-losing enteropathy in acquired immunodeficiency syndrome due to intestinal Kaposi's sarcoma

Patients with the acquired immunodeficiency syndrome (AIDS) often have hypoalbuminemia. We report the case of a patient with AIDS in whom marked hypoalbuminemia developed due to a protein-losing enteropathy caused by small intestinal Kaposi's sarcoma--an entity not previously reported in AIDS. The patient presented with ankle edema, pleural effusions, and a decrease in albumin from 3.0 g/dL (30 g/L) to 1.7 g/dL (17 g/L) over one month. Protein-losing enteropathy was confirmed by a marked elevation in fecal alpha-1 antitrypsin, and extensive evaluation of the gastrointestinal tract revealed the source to be small intestinal Kaposi's sarcoma. A protein-losing enteropathy should be considered when hypoalbuminemia is encountered in a patient with AIDS.     

99mTc-HSA-D scintigraphy in the diagnosis of protein-losing gastroenteropathy due to secondary amyloidosis

Secondary amyloidosis frequently involves the gastrointestinal tract and may result in ulceration, hemorrhage, and protein-losing enteropathy. We report a patient with severe hypoalbuminemia in whom endoscopy revealed widespread ulceration of the small intestine. The protein-losing site was detected by^99mTc-diethylene triamine pentaacetic acid human serum albumin (^99mTc HSA-D) scintigraphy. This evidence suggests that the ulcers and mucosal lesions associated with amyloidosis contribute to abnormal protein loss from the gastrointestinal tract.     

Protein-losing enteropathy as the principal manifestation of constrictive pericarditis

Constrictive pericarditis represents a rare cause of protein-losing enteropathy resulting from intestinal lymphangiectasia. We report the case of a patient with an atypical clinical presentation of constrictive pericarditis and protein-losing enteropathy as its principal manifestation; he was successfully treated with pericardiectomy. We conclude that constrictive pericarditis should be considered in the presence of protein-losing enteropathy and also, protein-losing enteropathy should be considered in the differential diagnosis of hypoalbuminemia. The authors have no conflicts of interest, financial or otherwise, to declare.     

Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment

Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.     

A case of systemic lupus erythematosus presenting with protein-losing enteropathy.

We report an unusual case of systemic lupus erythematosus presented with protein-losing enteropathy. A 24-year-old girl was referred to our hospital with generalized edema, thrombocytopenia, hypoalbuminemia, hypercholesterolemia, hypocomplementemia, antinuclear antibody (ANA) (speckled pattern) and anti- SSA/Ro positivities, and elevated CA125 antigen appeared in the blood examination. On the radiological studies, she had mild pleural effusion and moderate ascites which were transudate. A diagnosis of protein-losing enteropathy was made on the basis of increased 99mTc-labelled human immunoglobulin scintigram showing abnormal radioactivity. Endoscopic gastric, duodenal and jejunal biopsies showed chronic inflammation, but vasculitis and immune complex deposition findings were not present. Renal biopsy revealed no definitive findings of lupus nephritis. By the administration of corticosteroids, hypoalbuminemia began to improve, but steroid doses were decreased due to steroid-induced myopathy. Temporary hemiparesis and facial paralysis developed in the patients' follow up. Her cranial magnetic resonance imaging revealed chronic ischemia, and the patient was considered to have neurological involvement due to systemic lupus erythematosus. protein-losing enteropathy and other symptoms then improved dramatically after monthly intravenous cyclophosphamide (three times) combined with oral low-dose corticosteroids. The combination of azathioprine and low-dose steroids was used as maintenance medication. Although about 30 protein-losing enteropathy -associated systemic lupus erythematosus cases have been reported, the patients having initial symptoms as protein-losing enteropathy are rare in the literature. Protein-losing enteropathy -associated systemic lupus erythematosus cases probably represent a subgroup of systemic lupus erythematosus, the characteristics of which are hypocomplementemia, protein-losing enteropathy, ANA positivity showing speckled pattern and anti-ds DNA negativities. In the patients with systemic lupus erythematosus with edema and hypoalbuminemia without renal protein loss, protein-losing enteropathy-associated systemic lupus erythematosus should be kept in mind.     

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.     

Intestinal giardiasis: an unusual cause for hypoproteinemia.

Malabsorption is a well-known complication of infection with Giardia lamblia. However, selective protein-losing enteropathy is rare. We report a child with anasarca due to hypoalbuminemia as a result of gastrointestinal protein loss. Investigations established giardiasis as the etiology. The child returned to normal health after treatment with metronidazole.     

Protein-losing enteropathy revealing an idiopathic constrictive pericarditis

INTRODUCTION: The cause of protein-losing enteropathy is sometimes difficult to establish. It can be rarely due to a constrictive pericarditis. EXEGESIS: We report a patient presenting a protein-losing enteropathy revealing a constrictive pericarditis. CONCLUSION: Chronic pericarditis should be evoked in case of unexplained protein-losing enteropathy. Echocardiography can sometimes be normal. Therefore, chest computed tomography scan or cardiac MRI followed by confirmation right heart catheterization should be performed in case of persistent unexplained protein-losing enteropathy.