Case Of Protein-losing Enteropathy Research Articles

Protein losing enteropathy: A rare complication of ulcerative colitis

Protein-losing enteropathy (PLE) is a rare gastrointestinal condition that has been related to inflammatory bowel disease (IBD), namely ulcerative colitis (UC) and Crohn’s disease (CD). These pathologies damage the mucosal integrity, thereby leading to an increased transfer of plasma proteins into the bowel lumen. Because of its rarity, incidence and prevalence of this condition are not well known. Depending upon the underlying cause, the clinical course of PLE is highly variable, but mainly consists of edema, ascites, pericardial and pleural effusions. Here, we report a case of PLE caused by UC, in which total colectomy lead to complete improvement of hypoalbuminemia.

Protein Losing Enteropathy in a Post-Cardiac Transplant Adult: A Case Report

Introduction Protein-losing enteropathy (PLE) is a clinical syndrome that can be associated with a number of different pathologies, including congenital heart disease (CHD). However, typically PLE associated with CHD resolves following orthotopic heart transplant (OHT). We report a case of PLE in an adult CHD patient that developed many years following successful OHT. Case report A 32 year old man presented to transplant clinic with anasarca and mild dyspnea on exertion, and was subsequently admitted to hospital. He was born with transposition of the great arteries (TGA) and ultimately underwent cardiac transplantation at age 16. Overall, based on his follow up transplant clinic visits, he had been relatively well since OHT. He had been compliant with his anti-rejection medications. However, over the past 3 years his renal function has gradually declined in spite of careful medication titration. Furthermore, he continues to have a high salt diet, in spite of dietary recommendations. Given his presenting symptoms as well as a significantly elevated NT-pro BNP level, he was initially treated as heart failure due to possible chronic cardiac transplant rejection. He received aggressive intravenous diuresis. However, this only resulted in modest symptomatic improvement, and a rapid decline in his renal function was noted. His echocardiogram at this point showed preserved LV systolic function, and his coronary angiogram was normal. In addition, his cardiac biopsy showed no evidence of rejection. Therefore, other etiologies were sought. While he did have hypoalbuminemia, his renal work up showed no evidence of nephrotic syndrome. In addition, his liver ultrasound as well as other hepatic function tests was unremarkable. Ultimately, a gastroenterology workup for possible PLE showed abnormally elevated active alpha 1 antitrypsin level in his stool, in keeping with PLE. Conclusion This is the first case of PLE post cardiac transplant in an adult CHD patient. The two previous reports in the literature of PLE following cardiac transplant are both in children with hypoplastic heart syndrome and severe tricuspid regurgitation following transplant. As our case illustrates, in spite of the patient's strong cardiac history, renal dysfunction, and a clinical presentation very similar to either heart failure or nephrotic syndrome, PLE can be a rare but important cause of anasarca in this patient population.

Report of 1 case of protein losing enteropathy in elderly patients

Report of 1 case of protein losing enteropathy in elderly patients

Somatic PTPN11 Mutation in a Child With Neuroblastoma and Protein Losing Enteropathy

Neuroblastoma and protein losing enteropathy (PLE) are diagnoses commonly seen by oncologists and gastroenterologists, respectively. The concurrence of these 2 entities is rare, and not well explained. We describe the sixth case of PLE in a child with neuroblastoma, and the first for which genetic information is available. Tumor DNA had a mutation in the PTPN11 gene, which has been described in neuroblastoma, and in Noonan syndrome-a diagnosis in which neuroblastoma and PLE independently have been reported. Constitutional DNA was normal. Genetic studies in future patients will be needed to support the link between neuroblastoma and PLE.

P342 A pediatric case of protein-losing enteropathy induced by cow’s milk

Protein-losing enteropathy (PLE) is an uncommon manifestation of Food-Allergic Disorders characterized by protein leakage into the gastrointestinal tract. Here we report a case of PLE induced by cow's milk.

PROTEIN-LOSING ENTEROPATHY AFTER INFECTIOUS ENTEROCOLITIS IN CHILD – A CASE REPORT AND A REVIEW OF THE LITERATURE

Protein-losing enteropathy (PLE) represents an abnormal protein loss at the level of the digestive tract mucosa, being in most of the cases secondary to other gastrointestinal or extraintestinal pathologies. Nevertheless, there also are primary causes, genetic ones associated with PLE. We present the case of a small female child, 1 year and 1 month-old, with a history of infectious enterocolitis determined by Escherichia coli enteropathogenic, who presented palpebral edemas, especially in the morning for approximately 1 week. The laboratory tests revealed hypoalbuminemia, type A and G hypogammaglobulinemia, leukocytosis with eosinophilia, and anemia. The test for the detection of occult hemorrhages from feces was positive, likewise the fecal calprotectin and level of alpha-1 antitrypsin. We identified also allergy to cow’s milk protein, which we interpreted as being secondary to the intestinal pathology. Thus, we established the diagnosis of protein losing enteropathy after an episode of acute enterocolitis caused by E. coli enteropathogenic, and we administered substitutive treatment with human albumin and steroids, with favorable evolution. The particularity of the case consists in diagnosing a severe case of protein-losing enteropathy in a small child, with palpebral edema, predominantly during the morning, and decreased levels of serum albumin, after an episode of acute enterocolitis caused by E. coli enteropathogenic, which remitted without etiologic treatment.

Abdominal Distension in an Infant

Question: A 3-month-old Caucasian girl presented with a neonatal history of abdominal distension. On physical examination, her abdomen was massively distended, with bulging flanks, and was dull to percussion. The abdominal girth was 50 cm. Bilateral lower limb edema was present. Abdominal ultrasonography showed massive ascites and diffuse edematous bowel wall. Routine laboratory tests showed: white blood cells 2900/mm3 with 27% lymphocytes; serum albumin level, 1.9 g/dL; immunoglobulin (Ig)G, 69 mg/dL; IgA, 32 mg/dL; and IgM, 21 mg/dL. Liver and renal function tests, as well as coagulation tests, were normal. There was no evidence of congenital TORCH infection. Abdominal paracentesis yielded 150 mL of milky fluid with white blood cells of 300/mm3 (93% lymphocytes) and high triglycerides level (169 mg/dL), confirming the diagnosis of chylous ascites. After an unsuccessful 10-day trial with 75% medium-chain triglycerides (MCT)-based formula (Caprilon; Nutricia Advanced Medical Nutrition, The Netherlands), total parenteral nutrition (TPN) was started, followed by rapid improvement of ascites and laboratory test. Upper gastrointestinal endoscopy performed after 2 days of exclusive breast feeding revealed edema of the duodenal mucosa with whitish opaque spots corresponding to swollen villi (“snowflake appearance”; Figure A). What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Histologic evaluation of duodenal biopsies showed dilated mucosal lymphatic vessels with dense lymphoplasmacellular infiltrate of the lamina propria (Figure B). Slow weaning from TPN and introduction of low-fat (90% MCT), high-protein infant formula (Monogen; Nutricia Advanced Medical Nutrition) supplemented with fat-soluble vitamins was started. Full enteral feeding was established over the next 2 weeks without clinical deterioration. At the 6-month follow-up, there was no recurrence of the ascites and growth was normal. Primary intestinal lymphangiectasia (PIL) is a rare disease presenting with diarrhea, ascites, and bilateral lower limb edema. Hypoproteinemia owing to obstruction of the intestinal lymphatic vessels and loss of lymph fluid in the gastrointestinal tract is the main pathophysiologic feature.1Braamskamp M.J. Dolman K.M. Tabbers M.M. Clinical practice Protein-losing enteropathy in children.Eur J Pediatr. 2010; 169: 1179-1185Crossref PubMed Scopus (97) Google Scholar The prevalence of PIL is unknown. PIL primarily affects children (generally diagnosed before 3 years of age), but milder cases may be diagnosed later in adulthood. Neonatal presentation is exceptional.2Salvia G. Cascioli C.F. Ciccimarra F. et al.A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.Pediatrics. 2001; 107: 416-417Crossref PubMed Scopus (35) Google Scholar Diagnosis mainly relies on endoscopic findings with the corresponding histology of intestinal biopsy specimens. A low long-chain triglycerides diet to prevent engorgement and rupture of intestinal lymphatics, supplemented with MCT, which are directly absorbed into the portal venous circulation, is the cornerstone of management of PIL.3Tang Q.Y. Wen J. Wu J. et al.Clinical outcome of nutrition-oriented intervention for primary intestinal lymphangiectasia.World J Pediatr. 2011; 7: 79-82Crossref PubMed Scopus (7) Google Scholar Our patient tolerated 10% long-chain triglycerides, which meets the minimum requirement of essential fatty acids. In resistant or unresponsive cases, somatostatin infusion along with TPN may help to close the lymphatic leakage or relieve the symptoms effectively. A patient-specific approach should be attempted for diagnosing and managing PIL in infancy, and intervention started as soon as possible to relieve symptoms and allow normal growth.

Protein-Loosing Entropathy Induced by Unique Combination of CMV and HP in an Immunocompetent Patient

Protein-losing gastroenteropathies are characterized by an excessive loss of serum proteins into the gastrointestinal tract, resulting in hypoproteinemia (detected as hypoalbuminemia), edema, and, in some cases, pleural and pericardial effusions. Protein-losing gastroenteropathies can be caused by a diverse group of disorders and should be suspected in a patient with hypoproteinemia in whom other causes, such as malnutrition, proteinuria, and impaired liver protein synthesis, have been excluded. In this paper, we present a case of protein-losing enteropathy in a 22-year-old immunocompetent male with a coinfection of CMV and Hp.

Familial Mediteranean fever with protein-losing enteropathy due to constrictive pericarditis

Constrictive pericarditis (CP) represents a rare cause of protein-losing enteropathy (PLE) resulting from intestinal lymphangiectasia (IL). In this report, we describe an 8-year-old Turkish boy with IL and PLE secondary to CP. The boy was introduced to our clinic due to bilateral pretibial edema and swelling of the eyelids caused by hypoproteinemia. Physical examination revealed a distended right jugular vein. Laboratory investigation revealed PLE with fecal concentration of alpha-1 antitripsin of 4.87 mg/g. Histopathologic examination of random biopsies obtained from the duodenum revealed markedly dilated lymphatics compatible with IL. Constrictive pericarditis was diagnosed by tagged cine cardiac magnetic resonance imaging. Pericardiectomy was performed for the patient. Genetic analysis was done and heterozygous mutation E148Q was detected as a disease-causing Mediterranean fever (MEFV) mutation. Colchicine was started after the operation. Six months after the initiation of regular colchicine therapy, echocardiography revealed disappearance of CP. This is the first reported case of PLE with a distended right jugular vein due to CP secondary to familial Mediterranean fever associated with E148Q heterozygosity in the MEFV gene.

A Case of Protein-losing Enteropathy with an Abnormal Cortisol Response to ACTH Stimulation

We hereby report a case of a 62-year-old male patient who was misdiagnosed with adrenal insufficiency during the course of protein-losing enteropathy caused by superior mesenteric arterial thrombosis. The patient was suspected to have adrenal insufficiency due to hyponatremia and severe weakness. The cortisol responses to the initial challenge of 250 μg ACTH were inadequate (maximum serum cortisol level after ACTH challenge was 10.9 μg/dL), while the serum albumin concentration was 1.9 g/dL. Subsequently, intravenous steroid therapy was given to the patient. However, after bowel resection, the serum albumin level increased to 3.4g/dL and the cortisol response to the follow-up rapid ACTH stimulation was completely normal. Accordingly, we discontinued steroid replacement and discharged the patient without any problem. In conclusion, measuring total serum cortisol in a patient with hypo-pro-teinemia may lead to misdiagnosis of adrenal insufficiency. In such cases, caution should be exercised in interpreting the results in terms of total serum cortisol level or measurement of serum free cortisol levels should be considered (J Kor Soc Endocrinol 20:90～95, 2005).

A case of protein-losing enteropathy after liver transplantation (LT)

A case of protein-losing enteropathy after liver transplantation (LT)

A case of protein-losing enteropathy associated with carbamazepine

A case of protein-losing enteropathy associated with carbamazepine

A case of protein-losing enteropathy in idiopathic thrombocytopenic purpura with decreased IgA

A young woman presented with high fever and edema in January, 1984, and was diagnosed as having systemic lupus erythematosus. Prednisolone administration failed to improve her symptoms. In May she was admitted to hospital because of elevated erythrocyte sedimentation rate (ESR), hypoproteinemia, hypogammaglobulinemia, hypocomplementemia, positive antinuclear antibody, elevated immune complex level, and diarrhea. Edema disappeared following administration of diuretics and albumin, although the pathogenesis was still undetermined. In September, she was referred to our institution because of severe watery diarrhea and hypoproteinemia. Endoscopic examination showed a diffuse inflammatory lesion in the duodenum and the colon. Radioisotopic 51Cr-albumin study results were compatible with protein-losing enteropathy. Hypoproteinemia and inflammatory changes of the intestine were improved by antibiotics, suggesting that the inflammatory lesion was caused by bacterial infection. Despite the improvements in clinical symptoms and laboratory findings, the serum IgA level was still low and the thrombocytopenia remained. The morphological characteristics of the megakaryocytes were consistent with idiopathic thrombocytopenic purpura. In May, 1986, the thrombocytopenia deteriorated, causing purpura. Prednisolone was administered again, and this resulted in normalization of the platelet count, although the IgA level remained low. Finally the prednisolone was stopped, and the IgA level gradually recovered, with the improvement of the enterocolitis. The exact pathogenesis of the whole picture in this case is unclear, but an 8-year-long clinical course suggests that the protein-losing was caused by an infectious enterocolitis superimposed on IgA deficiency.

A case of protein-losing enteropathy after Fontan operation for tricuspid atresia

A case of protein-losing enteropathy after Fontan operation for tricuspid atresia

Hypoalbuminemia due to small intestinal lymphangiectasia in a case of amebiasis with amebae in the peritoneal fluid.

A case of protein-losing enteropathy with lymphangiectasia of the small bowel is presented. The patient has been followed for 11 years. Amebiasis of the peritoneum and focal amebic hepatitis were also found at one stage of her disease.