Protein-losing Gastroenteropathy Research Articles

A case of protein-losing gastroenteropathy due to Sjögren's syndrome detected by foggy vision with refractive error of the intraocular lens.

A woman in her forties visited an ophthalmologist for rapidly progressive foggy vision. Naked visual acuity had decreased to 0.15, and although her eyes showed no abnormalities, internal disease was suspected and albumin 2.6g/dL was found. Protein leakage from the intestinal tract was suspected since there was no urinary protein excretion. 99mTechnetium-labeled albumin D scintigraphy showed protein leakage from the intestinal tract. A stool α1-antitrypsin clearance test showed an increase to 56.3mL/day, leading to a diagnosis of protein-losing gastroenteropathy. Blood biochemistry revealed abnormally high levels of anti-SS-A and anti-SS-B antibodies (≥ 1200U/mL and ≥ 1000U/mL, respectively). A lip salivary gland biopsy revealed lymphocytic infiltrate at least 1 focus per 2mm × 2mm > 50 lymphocytes per conduit). The Schirmer test result was 5mm/5min or less, which led to the diagnosis of Sjögren's syndrome. The serum albumin level increased with intravenous administration of methylprednisolone 50mg (1mg/kg), and the patient is currently on oral prednisolone at a gradually decreasing dose. After administration of prednisolone, visual acuity recovered to 1.2 with recovery of albumin.

Detection of Pancreatitis and Protein-Losing Gastroenteropathy Associated With Systemic Lupus Erythematosus by Protein Leakage Scintigraphy.

Systemic lupus erythematosus (SLE) rarely causes pancreatitis and/or protein-leakage gastroenteropathy as a gastrointestinal (GI) manifestation. Here, we describe the incidental detection of SLE-associated pancreatitis by protein leakage scintigraphy.

A Case of Protein-losing Gastroenteropathy after Superior Mesenteric Artery Occlusion in which Intraoperative Endoscopy was Effective

A Case of Protein-losing Gastroenteropathy after Superior Mesenteric Artery Occlusion in which Intraoperative Endoscopy was Effective

Back Up at the Pump? I Have a Gut Feeling About This: A Review of the Gastrointestinal Manifestations From Congestive Heart Failure.

Heart failure, which is a clinical syndrome characterized by the heart's inability to maintain adequate cardiac output, is known to affect various organ systems in the body due to its ischemic nature and activation of the systemic immune response, but the resultant complications specifically on the gastrointestinal tract and the liver are not well discussed and poorly understood. Gastrointestinal-related phenomena are common symptoms experienced in patients with heart failure and frequently found to increase morbidity and mortality in these populations. The relationship between the gastrointestinal tract and heart failure are strongly linked and influence each other much so that the bidirectional association of the two is oftentimes referred to as cardiointestinal syndrome. Manifestations include gastrointestinal prodrome, bacterial translocation and protein-losing gastroenteropathy by gut wall edema, cardiac cachexia, hepatic insult and injury, and ischemic colitis. More attention is needed from a cardiology perspective to recognize these common presenting gastrointestinal phenomena that affect much of our patient population with heart failure. In this overview, we describe the association between heart failure and the gastrointestinal tract, the pathophysiology, lab findings, clinical manifestations and complications, and the management involved.

A Woman With Chronic Anemia, Hypoalbuminemia, and Ileo-Ileal Fistula

Question: A female patient in her 60s came to our department to determine the cause of her chronic anemia. She was diagnosed as having iron deficiency anemia in her teens, and protein-losing gastroenteropathy at 40 years of age. She had a history of cholecystectomy for gallstones at age 43. She had been treated for chronic thrombotic pulmonary hypertension. No intestinal or skin disease was found in her relatives. She was taking iron tablets for chronic anemia and oral anticoagulants and diuretics for chronic thrombotic pulmonary hypertension.

Management of severe symptomatic abdominal lymphatic malformation complicated by abscess formation, protein-losing gastroenteropathy, and bleeding

BackgroundThe optimal strategy for the management of patients with severe symptomatic abdominal lymphatic malformation (ALM) complicated by abscess formation, protein-losing gastroenteropathy, and bleeding has not yet been established. The present study aimed to determine an appropriate management for patients with severe symptomatic ALM, particularly for those with abdominal complications.Materials and methodsEight infants and young adults who underwent emergency surgery for ALM in our department, between the years 1997 and 2020, were selected for the study. We also evaluated and compared the operative procedures, operative timing, and postoperative surgical outcomes.ResultsEmergency resection was performed in all patients with bleeding. Some patients presented with ALMs that invaded the adjacent organs. Resections that included the involved organs were necessary to achieve full resolution in three patients. After evaluating the diagnostic modalities for symptomatic ALM, we also performed elective surgery for patients without bleeding.ConclusionsClinicians should be aware of severe symptomatic ALM with or without bleeding, as well as its associated complications, in order to select the best surgical management plan.

Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy characterized by dilatation of the intestinal lymphatics and loss of lymph fluid into the gastrointestinal tract, leading to the development of hypoproteinaemia, oedema, lymphocytopenia, hypogammaglobinaemia, and immunologic abnormalities. We report a series of 4 children from Bangalore, India presenting with anasarca, diarrhoea, and hypoproteinaemia. All four patients were confirmed to have of PIL on histopathology. We retrospectively reviewed the case records of children diagnosed with primary intestinal lymphangiectasia presenting to department of pediatric gastroenterology within the time frame of July 2015 to September 2018. Four patients were diagnosed with primary intestinal lymphangiectasia. All of them presented with features of protein losing enteropathy (generalized oedema, ascites and low albumin), chronic diarrhoea, lymphopenia, hypercalcemia, and hypogammaglobulinemia. Endoscopically mucosa was normal in two patients and showed Snowflake appearance in other 2 patients. Intestinal biopsies were characteristic of lymphangiectasia in all four patients.

S3354 Ménétrier’s Disease: A Rare Cause of Edema in Childhood

Introduction: Ménétrier’s disease (MD) is a rare protein-losing gastropathy characterized by enlarged gastric rugal folds. Adult MD is associated with a high degree of morbidity and mortality due to potential malignant transformation and need for gastrectomy. Pediatric MD, in contrast, is typically an acute, self-limited process, frequently associated with cytomegalovirus (CMV) infection of the stomach. Case Description/Methods: A previously healthy 2-year-old boy was admitted for generalized edema, which had been preceded by a week of diarrhea. Growth and development had been normal. Initial laboratory evaluation was notable for serum albumin of 1.3 g/dL. Other labs were largely unremarkable. Cardiac, renal, and liver etiologies were excluded. Esophagogastroduodenoscopy showed a normal gastric antrum, but the remainder of the stomach had enlarged rugae with areas of ulceration and nodularity. The duodenum and esophagus appeared normal. Histology was notable for hyperplastic gastric pits and foveolae. Immunohistochemistry for CMV and Helicobacter pylori were negative, and viral culture of a gastric sample was also negative for CMV. CMV serologies showed elevated IgM and IgG levels. Additionally, a viral enteric pathogens panel was positive for sapovirus. The patient received 2 doses of albumin and antisecretory agents during admission, but his edema and hypoalbuminemia improved without treatment of CMV. Discussion: In a toddler presenting with edema and hypoalbuminemia, it is necessary to consider protein-losing gastroenteropathies on the differential. Endoscopic evaluation with biopsy is essential for diagnosis of MD. Histology may show foveolar hyperplasia with cystic dilation of pits of the gastric body with relative sparing of the gastric antrum. Full-thickness biopsy may be required to detect classic histologic findings. Evaluation for infection should be done as one-third of pediatric cases are associated with CMV. Management of pediatric MD is primarily supportive due to the self-limited nature of the condition. Treatment with ganciclovir can be considered for severe or refractory cases associated with CMV.

Single-Balloon-Assisted Enteroscopy With Endoscopic Mucosal Resection of a Bleeding Jejunal Lymphangioma.

CASE REPORT Lymphangiomas are rare tumors with <1% found in the jejunum or ileum.1 Typically asymptomatic, lymphangiomas can cause gastrointestinal bleeding, intussusception, or protein-losing gastroenteropathy.1–3 We present a case of occult gastrointestinal bleeding caused by a lymphangioma, successfully removed by endoscopic mucosal resection by an upper single-balloon-assisted enteroscopy. A healthy, asymptomatic 75-year-old man had routine tests that revealed a new normocytic anemia: hemoglobin 12.9 g/dL, previously 15 g/dL. Upper endoscopy and colonoscopy were normal. Over 6 months, he developed weakness, worsened microcytic anemia (hemoglobin 8.5 g/dL), and iron deficiency. Celiac disease serology was negative. Video capsule endoscopy captured a white-speckled lesion with fresh blood at 25% of the small bowel transit (Figure 1). An upper single-balloon-assisted enteroscopy localized the polyp in the proximal jejunum (Figure 2). The polyp was lifted with hetastarch-epinephrine-methylene blue, and en bloc endoscopic mucosal resection was performed (Figure 3). Pathology confirmed a lymphangioma (Figure 4). The patient's hemoglobin normalized and symptoms resolved.Figure 1.: A submucosal polypoid lesion with a white and red speckled surface was captured in 1 single image.Figure 2.: An upper single-balloon-assisted enteroscopy localized a 12 mm white-speckled, bleeding, sessile polyp in the proximal jejunum.Figure 3.: The polyp submucosa was injected with a long-lasting lifting agent and resected en bloc using snare cautery endoscopic mucosal resection.Figure 4.: Pathology revealed dilated lymphatic vessels (stars) in the lamina propria consistent with a lymphangioma.Bleeding from lymphangioma is proposed by lymphatic obstruction, increasing lymphatic-venous pressures.4 Surgical resection is the standard for the management of symptomatic jejunal and ileal lymphangiomas.1,2 Similarly, there are only 2 other reported cases of endoscopically treated small bowel lymphangiomas,4,5 demonstrating that endoscopic management is feasible with experienced endoscopists. DISCLOSURES Author contributions: J. Trieu and A. Dua wrote the original manuscript. N. Gupta, RP Venu, and M. Venu critically reviewed the manuscript before submission. Mukund Venu is the article guarantor. Financial disclosure: None to report. Previous presentation: The case was submitted to Digestive Disease Week 2021 but not presented. It received an honorable mention from the ASGE. DDW 2021 was a virtual conference held on May 21-24. Informed consent was obtained for this case report.

Protein-losing gastroenteropathy complicated with asymptomatic primary biliary cholangitis, refractory to immunosuppressant, and improved by Helicobacter pylori eradication: a case report

BackgroundProtein-losing gastroenteropathy (PLGE) is a syndrome with a chief complaint of hypoalbuminemia, which occurs due to plasma protein leakage in the gastrointestinal tract, leading to general edema, ascites, and pleural effusions.Case presentationA 71-year-old woman visited another hospital for evaluation of hypoalbuminemia and systemic edema. She was hospitalized for a close inspection of hypoalbuminemia and was diagnosed with PLGE. Steroid and azathioprine therapy was prescribed; however, hypoalbuminemia did not improve, and the patient’s condition worsened due to anasarca. As hospitalization was prolonged, the patient was transferred to our hospital. She was infected with Helicobacter pylori, and we performed H. pylori eradication. Following H. pylori eradication, her edema improved remarkably.ConclusionWe present the first case wherein H. pylori eradication successfully improved protein leakage in the lower gastrointestinal tract in a patient diagnosed with PLGE complicated with refractory to immunosuppressant treatment. H. pylori eradication should be considered in patients with PLGE complicated with H. pylori infection, without specific endoscopic finding or refractory to immunosuppressants.

A Case of Protein-losing Gastroenteropathy Secondary to Endovascular Treatment of Superior Mesenteric Artery Occlusion

A Case of Protein-losing Gastroenteropathy Secondary to Endovascular Treatment of Superior Mesenteric Artery Occlusion

S3043 A Push to Find the Answer: A Rare Case of Jejunal Lymphangioma

Introduction: Lymphangioma arises from malformations of lymphatic vessels.Approximately 90% of cases present within the first two years of life with 95% occurring in the head, neck, and axilla.It is rarely found in adults, and small bowel lymphangioma represent less than 1% of cases.We present a rare case of a woman with melena and chronic anemia who was found to have a bleeding jejunal lymphangioma. Case Description/Methods: A 60-year-old female with coronary disease, atrial fibrillation, and chronic anemia presented with symptomatic acute on chronic anemia with a hemoglobin of 5.7gm/dL, MCV 67, and ferritin 3 ng/mL.The patient was evaluated for melena one month ago at an outside hospital with a normal esophagogastroduodenoscopy and colonoscopy.At discharge, anticoagulation for atrial fibrillation was discontinued given recurrent gastrointestinal bleeds. At our institution, the gastroenterology service pursued a second look push enteroscopy to evaluate a suspected small bowel source.This revealed three jejunal folds with circumferential, protruding, white plaque-like lymphangiectasia with areas of ulceration and oozing.There was no target to intervene on, so the area was biopsied and tattooed.Pathology was consistent with lymphangioma. Given multiple admissions for gastrointestinal bleeding and requirement of anticoagulation, surgery was consulted for resection.The resection confirmed a 7 cm, full thickness, lymphangioma with arteriovenous malformation of the jejunum which was negative for neoplasm. The patient recovered and was discharged without further bleeding. Discussion: Lymphangiomas are caused by a proliferation of multiple, thin-walled lymphatic space believed to be from a congenital lymphatic malformation, but it is theorized to be associated with inflammation, abdominal surgery, abdominal trauma or obstruction of developed lymphatic vessels.Although small bowel lymphangioma is a benign condition and normally clinically silent, it can present with symptoms such as abdominal pain, nausea, vomiting, and weight loss.Complications associated with lymphangiomas include bleeding, protein-losing gastroenteropathy, volvulus, and intussusception.According to Kida, 15 cases of bleeding small bowel lymphangioma have been reported from 1967 to 2012.While small bowel lymphangiomas are rare, it should be considered in a patient with clinically significant bleeding without an identifiable source.Our case allowed us to demonstrate the unique endoscopic appearance and complications associated with this rare entity.Figure 1.: Image 1: Push enteroscopy revealing three jejunal folds with white-plague lesions consistent with lymphangiomas. Image 2: Lymphangioma with ulceration and active bleeding. Image 3: (A) Lymphangioma of the small intestine. The vascular channels are dilated, lined by inconspicuous endothelium, and contain lymphatic fluid and blood cells. H&E, 50x. (B) Lymphangioma of the small intestine. D2-40 immunostain highlights the lymphatics component. H&E, 20x.

A Rare Case of Nsaid Induced Gastric Body Diaphragmatic-Like Stricture

Protein-losing gastroenteropathy is a rare syndrome of protein loss from the gastrointestinal system. It manifests with hypoproteinemic edema, which may be due to either lymphatic leakage due to increased interstitial pressure or leakage of protein-rich fluids due to intestinal disorders. Our case describes a 65-year-old female with life-threatening protein-losing enteropathy (PLE) requiring multiple transfers to intensive care unit for vasopressor support. In this rare instance, her extensive initial workup did not reveal any etiology for PLE, but she was later found to have underlying Crohn’s colitis. Protein-losing enteropathy is an underdiagnosed complication of inflammatory bowel disease and must be considered while treating patients with colitis.

Case Studies of Acute Cholecystitis Complicating Biliary Metal Stent-in-Stent after ERCP

Protein-losing gastroenteropathy is a rare syndrome of protein loss from the gastrointestinal system. It manifests with hypoproteinemic edema, which may be due to either lymphatic leakage due to increased interstitial pressure or leakage of protein-rich fluids due to intestinal disorders. Our case describes a 65-year-old female with life-threatening protein-losing enteropathy (PLE) requiring multiple transfers to intensive care unit for vasopressor support. In this rare instance, her extensive initial workup did not reveal any etiology for PLE, but she was later found to have underlying Crohn’s colitis. Protein-losing enteropathy is an underdiagnosed complication of inflammatory bowel disease and must be considered while treating patients with colitis.

A Rare Case Report of Life-Threatening Severe Protein-Losing Gastroenteropathy

Protein-losing gastroenteropathy is a rare syndrome of protein loss from the gastrointestinal system. It manifests with hypoproteinemic edema, which may be due to either lymphatic leakage due to increased interstitial pressure or leakage of protein-rich fluids due to intestinal disorders. Our case describes a 65-year-old female with life-threatening protein-losing enteropathy (PLE) requiring multiple transfers to intensive care unit for vasopressor support. In this rare instance, her extensive initial workup did not reveal any etiology for PLE, but she was later found to have underlying Crohn’s colitis. Protein-losing enteropathy is an underdiagnosed complication of inflammatory bowel disease and must be considered while treating patients with colitis.

99mTc-HSA-DTPA Scintigraphy of Protein-Losing Gastroenteropathy Associated with Mixed Connective Tissue Disease Before and After Immunosuppressive Therapy.

We present a female in her sixties with mixed connective tissue disease who underwent 99mTc-human serum albumin diethylenetriaminepentaacetic acid (99mTc-HSA-DTPA) scintigraphy to clarify the cause of generalized edema. Scintigraphy findings directed the diagnosis to protein-losing gastroenteropathy. Various disorders are known to be associated with protein-losing gastroenteropathy; however, mixed connective tissue disease is a rare cause. 99mTc-HSA-DTPA scintigraphy is helpful in the diagnosis and following the response to therapy of protein-losing gastroenteropathy.

S2910 Symptomatic Gastrointestinal Amyloidosis in a Patient With Systemic AL Amyloidosis

INTRODUCTION: Gastrointestinal (GI) amyloidosis is a rare protein deposition disorder that can be limited to the GI tract but is most often a manifestation of systemic amyloidosis. While it can be asymptomatic, typical presentations include dysmotility, malabsorption, bleeding and protein-losing gastroenteropathy. GI amyloidosis is most commonly in the esophagus, duodenum, stomach and rectum. The diagnostic gold standard is tissue biopsy with positive Congo red staining, usually from the rectum (sensitivity, 75-85%) or duodenum. CASE DESCRIPTION/METHODS: A 57-year-old woman with systemic amyloid light-chain (AL) amyloidosis complicated by restrictive cardiomyopathy, heart failure and junctional bradycardia requiring a pacemaker, and treated with bortezomib and dexamethasone with good response, presented to the hospital with recurrent constipation, nausea, vomiting and abdominal discomfort. Abdominal radiographs and CT scan demonstrated dilation of the cecum to 7.9 cm, concerning for ileus, but no obstruction (Figure 1). She was diagnosed with likely colonic pseudoobstruction, treated with neostigmine, and discharged on a regimen of antiemetics and prokinetics. Soon after, she was re-admitted with rectal bleeding; colonoscopy showed normal-appearing mucosa and polyps (Figure 2), with a large hemorrhoid as the likely source. Subsequently, she was re-admitted for a heart failure exacerbation; she was considered for heart transplant as her disease was believed to be in remission but had persistent poor oral intake, nausea and constipation. Endoscopy was notable for a gastric phytobezoar and duodenal ulcers; gastric, duodenal and rectal biopsies with Congo red stain confirmed the diagnosis of GI amyloidosis. She was started on ixazomib and dexamethasone, with improved GI symptoms and only mild dysmotility seen on a gastric emptying study, and is pursuing heart transplantation. DISCUSSION: GI amyloidosis is rare but has important clinical and prognostic implications. Our patient presented with a range of vague GI symptoms and non-specific radiographic and gross endoscopic findings. Maintaining a high index of suspicion and obtaining mucosal biopsies with Congo red staining early in the course avoids delay in diagnosis and symptom progression. Upon diagnosis of GI amyloidosis, this patient was treated with chemotherapy, which targeted the underlying cause of her GI symptoms, resulting in sufficient improvement. In this case, the patient’s diagnosis of GI amyloidosis was key to her consideration for cardiac transplant.Figure 1.: Abdominal radiograph showing increased gaseous distension of the colon compatible with ileus. No evidence of obstruction.Figure 2.: Colonoscopy showing normal appearing mucosa and multiple polyps in the descending colon.

S2795 Not Your Common Diarrhea

INTRODUCTION: Chronic diarrhea is a common encountered problem in the outpatient setting. Differential includes infectious, inflammatory, and malignant etiologies. We present a rare cause of chronic diarrhea, emphasizing need for physician awareness. CASE DESCRIPTION/METHODS: A 68-year-old male presented with chronic diarrhea, up to 12 bowel movements/day with nocturnal stools for the past 12 months. He described diffuse abdominal discomfort, dysgeusia, dysphagia, loss of appetite, & unintentional weight loss of 15 pounds over 1 year. Physical exam was benign. Initial labs including CBC, CMP, ESR, TSH, lipase, and infectious stool studies were unremarkable. An upper endoscopy showed mild antral erythema. Colonoscopy revealed multiple polyps less than 1 cm in size which were removed. Random biopsies were taken from the colon. Biopsies from the stomach, duodenum, and colon demonstrated amyloid deposits with apple-green birefringence on Congo red stain. Patient was referred to a tertiary care center for further evaluation, where a bone marrow biopsy revealed plasma cells expressing monotypic lambda cytoplasmic immunoglobuin light chains with CD38 & CD138, and no CD19 or CD45. This profile was consistent with AL (lambda) type amyloid deposition. He was further found to have amyloid deposits in the kidney, autonomic nerves, and heart. He received treatment with CyBordD (cyclophosphamide, bortezomib, dexamethasone) and an autologous stem cell transplantation. He is now in deep remission of AL amyloidosis. DISCUSSION: Chronic diarrhea is a common symptom, but can have a rare underlying etiology, such as amyloidosis. Prompt workup with endoscopy & biopsy with Congo red stain are diagnostically essential. Additionally, as different types of amyloidosis require different therapies, the diagnosis must be confirmed by biopsy with immunostaining. Gastrointestinal (GI) amyloidosis is a rare disease, clinically apparent in one percent of patients with AL (primary) amyloidosis. It can present as any of 4 syndromes: GI bleeding, malabsorption, protein-losing gastroenteropathy, or chronic GI dysmotility. The pathogenesis involves amyloid deposition in the muscularis mucosae, submucosa, and muscularis propria, which leads to protrusions & thickening of the intestinal wall. The prognosis of AL amyloidosis patients with GI involvement is worse than those without, as it may involve other organs and present with advanced disease. Therefore, early recognition, diagnosis, and management are crucial.

A Case of Protein-Losing Gastroenteropathy due to Constrictive Pericarditis after Cardiac Surgery

A Case of Protein-Losing Gastroenteropathy due to Constrictive Pericarditis after Cardiac Surgery

A Case of Secondary Protein-losing Gastroenteropathy after Open Thrombectomy for Superior Mesenteric Artery Occulusion

A Case of Secondary Protein-losing Gastroenteropathy after Open Thrombectomy for Superior Mesenteric Artery Occulusion