1-antitrypsin Clearance Research Articles

Exudative enteropathy in neonates: A case report

The term exudative gastroenteropathy is used to describe an entity characterized by excessive loss of plasma proteins in the gastrointestinal tract. This digestive exudation can be caused either by an obstacle to intestinal lymphatic drainage or by an alteration of the epithelial barrier. The clinical picture is often associated with chronic diarrhea and an edematous syndrome. Lymphedema, infectious complications secondary to lymphopenia or hypogammaglobulinemia, and thromboembolic complications may also be observed. Diagnosis is based on the measurement of fecal alpha-1 antitrypsin clearance. Sometimes primary in the context of Waldmann disease (primary intestinal lymphangiectasis) but more often secondary. It requires an endoscopic and morphological workup to confirm the origin of the protein leak, to evaluate its extent and cause. In addition to etiological treatment, management is based on a hyperprotein and hypolipid diet with medium-chain triglyceride supplementation. We report the case of an exudative enteropathy in a newborn.

S2704 A New Protein Losing Enteropathy: Autoimmune Cryptolytic Enterocolitis

Introduction: Protein losing enteropathy (PLE) can be classified into erosive, non-erosive, and increased interstitial pressure PLE. Presenting signs are often chronic non-bloody diarrhea, anasarca, abdominal pain, and weight loss. We present a complex case of a PLE presentation with a novel diagnosis. Case Description/Methods: A 30-year-old woman with a history of migraines presented with severe abdominal pain and chronic diarrhea. She was on long-term combined estrogen-progestin oral contraceptive pills (OCP) and chronic high dose non-steroidal anti-inflammatory (NSAID) medications. She was exposed to mycoplasma pneumoniae one month prior to presentation, without other infectious exposures. Laboratory tests were notable for hemoglobin 6 g/dL, albumin 0.5 g/dL, stool pathogen PCR negative, and fecal calprotectin >3000µg/g. Extensive infectious work up only revealed Mycoplasma IgM+. Inflammatory markers and alpha-1 anti-trypsin clearance were elevated, and she was found to have warm autoimmune hemolytic anemia. CT angiography excluded ischemia and vasculitis (autoimmune serologies were negative), revealing diffuse enterocolitis, confirmed on magnetic resonance enterography with ileal ulcers. Endoscopic evaluation revealed diffuse superficial ulcerations terminal ileum and colon with diffuse mucosal sloughing. Histopathology revealed diffusely injured crypts with crypt drop-out and minimal inflammation, without any findings to suggest infection, inflammatory bowel disease (IBD) or autoimmune enteritis (Figure). Normal B-cell switching studies excluded common variable immunodeficiency (CVID). Due to profound anasarca and inability to tolerate oral intake, albumin infusions and total parenteral nutrition were started. There was minimal response to systemic or topical steroids. Diarrhea was mildly improved with albumin repletion, and she was empirically started on vedolizumab. Discussion: Given the absence of classic IBD findings, negative infectious and immunological testing, we present the first case of autoimmune cryptolytic enterocolitis, a histopathologic diagnosis of unclear etiology and pathogenesis. The patient has responded to vedolizumab infusions as an empiric treatment for an IBD-like entity. Nevertheless, supporting the autoimmune component of this entity, is a possible concomitant potential pathway of molecular mimicry in the setting of post-mycoplasma infection along with chronic high dose NSAID and OCP exposure.Figure 1.: Terminal Ileum and Sigmoid Colon with Diffuse Crypt Dropout.

Fontan protein-losing enteropathy is associated with advanced liver disease and a proinflammatory intestinal and systemic state.

Protein-losing enteropathy (PLE) after Fontan surgery carries significant morbimortality. Its pathophysiology and association with other Fontan complications are poorly understood. Our aims were to examine whether Fontan-PLE is associated with greater liver damage and to assess the presence of systemic and intestinal inflammation. Fontan patients with PLE and Fontan controls without PLE matched for age and Fontan surgery procedure were included. Data were prospectively compiled on blood and stool tests, liver imaging, elastography, cardiac-MRI and cardiac catheterization. Twenty-nine Fontan patients were enrolled (14 with PLE and 15 controls without PLE). Patients with PLE had more advanced liver disease estimated by non-invasive methods: blunt liver margins on ultrasonography (71.4% vs 26.7%, P=.027), greater median liver stiffness (25.4 vs 14.5kPa, P=.003) and higher FIB-4 (P=.016). Portal hypertension-related signs were more common in patients with PLE including ascites (P=.035), larger spleen size (P=.005), oesophageal varices/splanchnic collateral shunts (P=.03), higher liver stiffness-spleen size-to-platelet ratio risk score (P<.001) and lower platelet count (P=.01). Systemic proinflammatory cytokines (TNF-α, interleukin-6), biomarkers of intestinal permeability (intestinal fatty-acid binding protein) and faecal calprotectin concentrations were also significantly increased in Fontan-PLE (P<.05). Faecal calprotectin directly correlated with alpha-1 antitrypsin clearance and inversely with cardiac index, total serum proteins and body mass index. Fontan-PLE is associated with advanced liver disease and increased markers of systemic inflammation and intestinal permeability. Faecal calprotectin is elevated and correlates with Fontan-PLE severity. Liver assessment is mandatory in all Fontan patients, and especially in those with PLE.

A case of protein-losing gastroenteropathy caused by systemic AA amyloidosis secondary to undifferentiated carcinoma of unknown primary origin.

We report the case of a 61-year-old woman with Kartagener syndrome who presented with a 3-month history of chronic watery diarrhoea and severe hypoalbuminaemia. Histopathological examination of duodenum and large intestine biopsies showed amyloid A (AA) amyloid deposition. Scintigraphy and alpha-1 anti-trypsin clearance evaluations revealed protein-losing gastroenteropathy. Computed tomography with contrast and positron emission tomography showed a pelvic mass with multiple para-aortic lymph node enlargement. We suspected protein-losing gastroenteropathy secondary to AA amyloid produced related to malignant tumours. Following tumour resection, histopathological examination of the lesion revealed undifferentiated carcinoma of unknown origin. Postoperatively, the patient’s nutritional condition improved. There has been no recurrence of protein-losing gastroenteropathy 6 months postoperatively. This is the first report of protein-losing gastroenteropathy and AA amyloidosis secondary to undifferentiated carcinoma. Early recognition and intervention could increase the likelihood of amyloidosis remission.

A Case of Protein Losing Enteropathy as Only Clinical manifestation of Systemic Lupus Erythematosus

Protein losing enteropathy (PLE) due to systemic lupus erythematosus (SLE) is relatively uncommon. PLE may be appeared sequentially after the diagnosis of SLE or concurrently with SLE. In most of concurrent cases, PLE was diagnosed one of various symptoms of SLE. Cases of PLE as the initial and only clinical presentation of SLE have been rarely reported. We described a 30-year old woman with general edema and abdominal distension was diagnosed PLE after stool alpha 1 antitrypsin clearance test. Her symptoms were getting worse even though the treatment with intravenous albumin. She was finally diagnosed PLE associated with SLE by additional laboratory findings (positive antinuclear antibody and anti-dsDNA IgG and low C3, C4 and CH50). She was treated with high dose of steroids and her symptoms were improved.

Gastro-entéropathies exsudatives

Protein-losing enteropathy is a rare syndrome of gastrointestinal protein loss. The primary causes can be classified into lymphatic leakage due to increased interstitial pressure and increased leakage of protein-rich fluids due to erosive or non-erosive gastrointestinal disorders. The diagnosis of protein-losing enteropathy should be considered in patients with chronic diarrhea and peripheral oedema. The diagnosis of protein-losing enteropathy is most commonly based on the determination of fecal alpha-1 antitrypsin clearance. Most protein-losing enteropathy cases are the result of either lymphatic obstruction or a variety of gastrointestinal disorders and cardiac diseases, while primary intestinal lymphangiectasia (Waldmann's disease) is less common. Treatment of protein-losing enteropathy targets the underlying disease but also includes dietary modification, such as high-protein and low-fat diet along with medium-chain triglyceride supplementation.

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal E₁-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response. i 2014 S. Karger AG, Basel

A Rare Cause of Diarrhea and Weight Loss

Purpose: A 70-year-old man presented with a five-month history of abdominal pain, low-grade fever, fatigue, anorexia, diarrhea and a 30-pound weight loss. The initial evaluation included EGD and ileocolonoscopy, with random biopsies showing an increase in colonic intraepithelial lymphocytes and mild plasmacytosis. An abdominal CAT scan showed two separate segments of small bowel circumferential thickening. Infectious etiologies were excluded and a presumptive diagnosis of Crohn's disease was rendered. He was empirically treated with steroids with partial improvement. Several weeks later, he presented with continued symptoms, as well as diffuse edema consistent with anasarca. A repeat evaluation was negative for celiac disease, stool culture, ova and parasite, C. difficile, mycobacterium tuberculosis, HIV, and fecal fat. Laboratory showed a hemoglobin 11.1 g/dL, platelet 131 X 10ˆ9/L, BUN 32.3 mg/dL, creatinine 1.2 mg/dL, albumin 1.9 g/dL, prealbumin 29.9 mg/dL, and increased serum-feces alpha-1 antitrypsin clearance 53 (<27). These findings suggested a protein-losing enteropathy, which prompted a deep enteroscopy. Retrograde deep enteroscopy showed diffuse edema, erythema and shallow ulcerations in the distal ileum up to 80 cm from the ileocecal valve. Terminal ileum histology showed a dense, small, mature-appearing lymphocytic infiltrate expanding the lamina propria, extending into the submucosa with blunted villi without significant intraepithelial lymphocytosis. The immunophenotype was positive for CD3, CD5, CD7, CD56 and T-cell gene rearrangement, and negative for CD4, CD8, and Tdt. Enteropathy-associated T-cell lymphoma (EATL) was diagnosed. The patient was started on cyclophosphamide, etoposide, vincristine and prednisone alternating with pralatrexate. Unfortunately, he developed bacteremia, septic shock and expired six weeks later. EATL is an uncommon gastrointestinal T cell lymphoma and is strongly associated with celiac disease. Patients typically present with abdominal pain, systemic B symptoms, fatigue or anorexia. The small intestine was the most commonly involved site at diagnosis (90%), followed by the large intestine (16%) with frequent involvement of mesenteric (35%) and para-aortic or iliac lymph nodes (11%). Clinical, radiologic and endoscopic manifestations of EATL can mimic Crohn's disease, as demonstrated in this case. The development of a protein-losing enteropathy in this case broadened the differential leading to the final diagnosis. Deep enteroscopy is an important endoscopic tool to obtain tissue for the proper diagnosis of EATL, especially when suggested by other imaging modalities, such as abdominal CT scan.

Systemic lupus erythematosus complicated with protein‐losing enteropathy: A case report and review of the published works

A 45-year-old man was referred to our hospital with a history of multiple erythematous skin lesions of several months' duration. Blood examination revealed extreme hypoproteinemia and hypoalbuminemia, as well as the presence of antinuclear antibodies. A skin biopsy specimen showed liquefaction degeneration at the dermoepidermal junction and dense lymphocyte and neutrophil infiltration around the vessels and appendages in the upper and middle dermis. Chest X-ray and computed tomography showed a pleural effusion and thoracic paracentesis revealed a mononuclear cell-dominant cell infiltration, suggestive of serositis. Technetium-99m ((99m)Tc)-labeled human serum albumin scintigraphy and α(1)-antitrypsin clearance revealed protein leakage along the digestive tracts from the stomach to the jejunum. From the above findings, the patient was diagnosed with systemic lupus erythematosus (SLE) complicated by protein-losing enteropathy (PLE). Treatment with oral prednisolone significantly improved his clinical symptoms and hypoalbuminemia. This case highlighted the utility of (99m)Tc-labeled human serum albumin scintigraphy and α(1)-antitrypsin clearance in the diagnosis of PLE. We also present a published work review on PLE associated with connective tissue disease revealing a relatively higher prevalence in patients of Asian ethnicity, including Japanese.

The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan Operation

Approximately 5% to 15% of patients develop protein-losing enteropathy (PLE) after the Fontan operation. Oral controlled release (CR) budesonide has been used as a treatment strategy, but its use in the older Fontan population has not been described. Seven patients with refractory PLE after the Fontan operation were started on oral CR-budesonide at 9 mg. After 3 to 9 months, the dose was weaned to 3 mg. Response to treatment was assessed by clinical evaluation, serum albumin levels, and fecal α-1 antitrypsin clearance when available. Median age at last evaluation was 20 years (range, 16 to 32 years). Six patients had increases in serum albumin levels but only 4 patients had symptomatic improvement. Systemic side effects included: cushingoid features (5), adrenal insufficiency (4), and new-onset type 2 diabetes mellitus (2). One patient had improvement in cushingoid features after weaning CR-budesonide to 3 mg. Older patients (ages 27 to 32 years) had the worst side effect profiles and were the most refractory to treatment. These patients had sonographic evidence of hepatic cirrhosis but normal serum liver function tests. Two deaths occurred: 1 from sepsis 1 month after CR-budesonide initiation and 1 from respiratory arrest 5 months after CR-budesonide discontinuation. CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well.

Evaluation of Performance of Measurement of Faecal α1-Antitrypsin Clearance and Technetium-99m Human Serum Albumin Scintigraphy in Protein-Losing Enteropathy

Background and Aim: Our study aimed to compare the performance of faecal α₁-antitrypsin clearance (AATC) and radiolabelled human serum albumin (HSA) scintigraphy in protein-losing enteropathy (PLE). Methods: Patients studied by both AATC and technetium-99m (^99mTc)-labelled HSA scintigraphy were recruited and categorized into PLE and non-PLE groups based on clinical and laboratory findings. The performance of AATC and ^99mTc-labelled HSA scintigraphy was evaluated using clinical diagnosis of PLE as a gold standard. Results: 29 patients were recruited and 13 patients were considered to have definite PLE (PLE group). In the PLE group, all patients had a positive HSA scinigraphy and 10 (77%) had demonstrable positive tracing in the early phase. Conversely, only 6 of them (46%) had elevated AATC level (>13 m/day). Results of ^99mTc-labelled HSA scan (but not AATC) showed significant agreement with the clinical diagnosis (ĸ 0.35, p = 0.013). ^99mTc-labelled HSA scintigraphy carried higher sensitivity (100 vs. 46%) and negative predictive value (100 vs. 63%) compared to AATC in diagnosing PLE. The correlation between the results of these two investigations was only modest (ĸ 0.27, p = 0.04). The area under the receiver operating characteristic curve of AATC level showed no optimal diagnostic cut-off for PLE. Conclusion:^99mTc-labelled HSA scintigraphy was superior to AATC in diagnosing PLE.

High Alpha-1 Antitrypsin Clearance Predicts Severity of Gut Graft Versus Host Disease (GVHD) In Children

AbstractAbstract 1242The clinical evaluation and management of gut GVHD is a significant challenge in pediatric hematopoietic stem cell transplantation (HSCT). It is often difficult to obtain pathological evidence to confirm the GVHD diagnosis and/or determine response to treatment. The severity of the disease itself may not just be related to the associated classic symptoms. Our objective herein was to prospectively study the effect of gut GVHD on protein losing enteropathy (PLE) as measured by alpha-1 antitrypsin clearance (AATC) in stools. Thirteen patients who were diagnosed with gut GVHD by clinical criteria were recruited; 5 patients were excluded as 4 had gut GVHD ruled out by biopsy and 1 was unable to complete study due to stool collection issues. Therefore, 8 patients; 6 males and 2 females, were studied. The median age was 9.5 years (range 6–17). Diagnoses included ALL (4), AML (2), Lymphoma (1) and Adrenoleukodystrophy (1). Donor types were; 7 unrelated (BM n=4 and cord n=3) one matched related BM donor. All patients received cyclosporine for GVHD prophylaxis, in addition 4 patients also received methotrexate and 3 others received prednisone as the second agent. All patients had negative stool EM for viruses and cultures for C.difficile on their first collection. Two 24 hour stool collections were taken from each patient at a mean of 8.5d (range 7–13d) apart. Patients' gut GVHD staging is summarized in Table 1.Table 1:Summary of Stage gut GVHD and PLEPatientsAATC Median(range)normal = <22Stool outputml/kg/d Median(range)Stage of gutGVHD (n=)# with PLECollection #1831 (3-339)28 (10-109)Stg 1 (2)4/8Stg 2 (3)Stg 3 (1)Stg 4 (2)Collection #2856 (8-561)19 (0-85)Stg 1 (0)5/8Stg 2 (0)Stg 3 (3)Stg 4 (1)At the time of 1st collection, 6 patients had ≥ stage II gut GVHD and at 2nd collection 4 patients had ≥stage II gut GVHD and 4 collections were of non-diarrheal stool. A total of 7 stool collections were taken at a diagnosis of ≥stage III gut GVHD. Mean alpha-1 antitrypsin clearance (AATC) from all 16 collections was 143 mls/day (range 3–561), levels >22 mls/day indicate the diagnosis of protein-losing enteropathy (PLE). Nine of the collections (56%) showed PLE with a mean AATC of 247mls/day (range 31–561). Six out of the 7 samples from patients with ≥stage III gut GVHD were positive for PLE. Five collections with stool volumes >30 ml/kg/day were positive for PLE. Stool volumes were significantly higher on second collection (Wilcoxon signed ranks test, p = 0.003) consistent with the second stool collections being significantly more likely to have an elevated AATC, therefore more severe PLE over time (p<0.001). We conclude that a highly significant positive correlation exists between the severity of PLE and the stage of gut GVHD, particularly obvious in patients with stage 3–4 GVHD (ANOVA, p=0.015). See Table 2.Table 2:Stage of gut GVHD and AATCStage ofGVHDNumber ofcollectionsMeanAATCStd.Deviation0426.0020.801217.0019.802384.67136.2934171.50176.2143406.33134.32When patients were grouped as stage 0–2 GVHD vs. stage 3–4 GVHD, those with stage 3–4 GVHD had significantly higher AATC values (p =0.02). Despite the small number of patients recruited, this study emphasizes the need to consider PLE as a useful aspect of the clinical picture. We suggest that in order to see a response to therapy and therefore a decrease in AATC, clinicians should not repeat stool collections before 2 weeks from the initiation of therapy. In light of the significant morbidity and mortality associated with grade 3 and 4 gut GVHD, and as an important therapeutic decision for these patients, one may consider escalating GVHD therapy if a patient's AATC is rising. Disclosures:No relevant conflicts of interest to declare.

Protein-Losing Enteropathy: Case Illustrations and Clinical Review

Protein-losing enteropathy (PLE) is a rare syndrome of gastrointestinal protein loss that may complicate a variety of diseases. The primary causes can be divided into erosive gastrointestinal disorders, nonerosive gastrointestinal disorders, and disorders involving increased central venous pressure or mesenteric lymphatic obstruction. The diagnosis of PLE should be considered in patients with hypoproteinemia after other causes, such as malnutrition, proteinuria, and impaired protein synthesis due to cirrhosis, have been excluded. The diagnosis of PLE is most commonly based on the determination of fecal alpha-1 antitrypsin clearance. Treatment of PLE targets the underlying disease but also includes dietary modification, supportive care, and maintenance of nutritional status. In this article, cases illustrating a variety of clinical presentations and etiologies of PLE are presented, and its diagnostic approach and treatment are reviewed.

Œdèmes et entéropathie exsudative compliquant une péricardite chronique constrictive associée à un anévrisme de l’aorte ascendante

Chronic constrictive pericarditis is suspected on clinical and echocardiographic grounds. Its treatment is surgical. We report here the case of a 45-year-old man, admitted for edema characteristic of lymphatic obstruction. Examination revealed ascending aorta ectasia, associated with chronic constrictive pericarditis. Measurement of alpha-1 antitrypsin clearance confirmed protein-losing enteropathy. Total recovery followed pericardectomy and aneurysm resection. The clinical edema in this case was due to several phenomena: protein-losing enteropathy from a functional lymphatic overload, induced by chronic constrictive pericarditis and by compression of the right atrium and vena cava by an aortic aneurysm. The hypoalbuminemia induced by protein loss may also magnify edema. An association between chronic constrictive pericarditis and ascending aortic aneurysm is uncommon. No cause for this association was found.

Intermediate outcomes after serial transverse enteroplasty in children with short bowel syndrome

Background Serial transverse enteroplasty (STEP) is a novel intestinal lengthening procedure introduced in 2003. To date, no human studies exist that report objective assessment of intestinal absorptive capacity. The aim of this study was to report intermediate outcomes in patients who have received the STEP at our institution using clinical and biochemical assessment of intestinal function. Methods All 14 patients who received the STEP since May 2003 were reviewed. Clinical (weight gain, enteral tolerance, stool frequency) and biochemical (citrulline levels, d-xylose absorption, alpha-1 antitrypsin clearance, and fecal fat content) outcomes were performed pre-STEP and post-STEP at 1, 6, and 12 months, respectively. Data are presented as means with standard deviation. Paired t tests were used to compare post-STEP outcomes with pre-STEP values ( P < .05 was significant). Three patients had a STEP as a newborn and are analyzed separately. Main Results There were 14 patients (3 females; mean age, 24.8 months; range, 1 day-14 years). Serial transverse enteroplasty resulted in a mean increase in length of dilated bowel segment of 94% ± 30% and increase in total small bowel length of 49% ± 42% with mean application of 16 ± 9 cartridges and cost of Can$2878.51 ± 1406.22. Patients demonstrated improvement in both clinical parameters, as well as intestinal absorptive function. Complications included 2 patients with staple line leak and 1 patient with gastrointestinal bleeding from staple line ulcers. Three patients died (2 from liver failure and 1 from sepsis and congenital heart disease). Two patients received liver-intestinal transplants at 4 and 5 months post-STEP. Mean follow-up was 23 ± 9 months, with 7 patients followed more than 2 years. Of 8 patients with post-STEP follow-up of more than 1 year, 7 have weaned from parenteral nutrition. Conclusion Clinical and objective biochemical outcomes of intestinal function after the STEP procedure show promise after intermediate follow-up.

Protein-Losing Enteropathy from Eosinophilic Enteritis Solely Diagnosed by Wireless Capsule Endoscopy (WCE) and Double-Balloon Enteroscopy (DBE)

A 47-year-old woman presented with bilateral lower extremities edema for 1 mo. Past history was significant for pernicious anemia and contact dermatitis. Labs were notable for iron-deficiency anemia (hemoglobin 11.3 g/dL and ferritin 5 mcg/L), eosinophilia (20.7%), and hypoalbuminemia (2.8 g/dL). Ultrasound and CT scan of the abdomen were normal. Urinalysis was negative for proteinuria. Stool exams were negative for parasites. 48-hr stool collection confirmed fat malabsorption (16 g/24 hrs) and markedly increased α-1 antitrypsin clearance (424 mL/24 hrs), indicating protein-losing enteropathy. Extended EGD and colonoscopy with extensive biopsies were normal, as similar to small intestinal series. WCE revealed multiple rounded patches of raised erythema with centrally flattened villi throughout the small intestine (A). DBE confirmed the similar finding (B). Pathology of the targeted biopsies of the lesions was diagnostic for eosinophilic enteritis. Following an initial treatment with oral prednisone 30 mg for 2 wks, serum albumin and eosinophils returned to normal with complete resolution of lower extremities edema. Eosinophilic gastroenteritis is a rare disease characterized by tissue eosinophilic infiltration that can involve any layers of the gut wall. Diagnostic criteria include the presence of GI symptoms, demonstration of eosinophilic infiltration on biopsy, absence of eosinophilic involvement of organs outside the GI tract, and absence of parasitic infection. Clinical presentation varies depending on the layer of involvement. Histologic evaluation from endoscopic biopsies represents the best way to confirm the diagnosis. Because the disease tends to be patchy, it is recommended that multiple biopsies should be obtained from different areas throughout the GI tract. In this report, we present a patient with mucosal eosinophilic enteritis. Despite extensive biopsies from conventional endoscopic procedures, the diagnosis cannot be established because the disease localizes in the small intestine. It emphasized the role of WCE followed with DBE as the method of choice in evaluation for mucosal diseases of the small intestine. [figure 1]Figure 1

A Systemic Lupus Erythematosus Patient with Protein Losing Enteropathy

Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and (99m)Tc-human serum albumin scintigraphy.

40-Year-Old Man With Anemia, Chronic Low Back Pain, and Hypoalbuminemia

40-Year-Old Man With Anemia, Chronic Low Back Pain, and Hypoalbuminemia

Atrial pacing: An alternative treatment for protein-losing enteropathy after the Fontan operation

J Thorac Cardiovasc Surg 2001;121:582-3

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.