3,064 results match your criteria Protein-Losing Enteropathy

Apoptotic enteropathy, gluten intolerance, and IBD-like inflammation associated with lipotoxicity in DGAT1 deficiency-related diarrhea: a case report of a 17-year-old patient and literature review.

Virchows Arch 2022 Jun 28. Epub 2022 Jun 28.

Department of Pathology, Ghent University Hospital, C. Heymanslaan 10, 9000, Ghent, Belgium.

We present a long-term follow-up in a 17-year-old girl with DGAT1-related diarrhea, an autosomal recessive disorder characterized by impaired triglyceride absorption. Neonatal presentation included severe congenital diarrhea, protein-losing enteropathy, and failure to thrive requiring total parenteral nutrition. Duodenal biopsies revealed apoptotic enteropathy and acute inflammation with the presence of macrophages and Touton giant cells, related to the intake of fat. Read More

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Lymphatic Disorders in Patients With Single Ventricle Heart Disease.

Front Pediatr 2022 10;10:828107. Epub 2022 Jun 10.

Department of Cardiology, Jill and Mark Fishman Center for Lymphatic Disorders, Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Lymphatic abnormalities in patients with single ventricle physiology can lead to early Fontan failure and severe Fontan complications, such as protein-losing enteropathy (PLE), plastic bronchitis (PB), chylothorax, and edema. Recent developments in lymphatic imaging and interventions have shed new light on the lymphatic dysfunction in this patient population and the role of the lymphatic circulation in PLE, PB, and chylothorax. In this study, we reviewed some of the latest developments in this field and discuss new treatment options for these patients. Read More

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A Preliminary Study of Modulen IBD Liquid Diet in Hospitalized Dogs with Protein-Losing Enteropathy.

Animals (Basel) 2022 Jun 20;12(12). Epub 2022 Jun 20.

Department of Clinical Science and Services, Royal Veterinary College, Hertfordshire AL9 7TA, UK.

Modulen IBD is an enteral liquid diet that can induce remission rates similar to glucocorticoids in children with inflammatory bowel disease. The Modulen IBD liquid diet has not been previously investigated in dogs. Our study aimed to describe the use of the Modulen IBD liquid diet in hospitalized dogs with inflammatory protein-losing enteropathy (PLE), including its tolerance and effects on appetite and gastrointestinal signs, and laboratory parameters during hospitalization. Read More

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Protein-losing Enteropathy as a Complication and/or Differential Diagnosis of Common Variable Immunodeficiency.

J Clin Immunol 2022 Jun 23. Epub 2022 Jun 23.

Université de Lille, UFR Médecine, 59000, Lille, France.

As protein-losing enteropathy (PLE) can lead to hypogammaglobulinemia and lymphopenia, and since common variable immunodeficiency (CVID) is associated with digestive complications, we wondered if (1) PLE could occur during CVID and (2) specific features could help determine whether a patient with antibody deficiency has CVID, PLE, or both. Eligible patients were thus classified in 3 groups: CVID + PLE (n = 8), CVID-only (= 19), and PLE-only (n = 13). PLE was diagnosed using fecal clearance of α1-antitrypsin or 111In-labeled albumin. Read More

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Case Report: Protein-Losing Enteropathy in Association With Tuberculosis-Related Constrictive Pericarditis.

Front Pediatr 2022 30;10:875032. Epub 2022 May 30.

Department of Nephrology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

Protein-losing enteropathy (PLE) is a clinical disorder in which an excessive amount of serum protein is lost into the gastrointestinal tract, resulting in hypoproteinemia and malnutrition. PLE is associated with a wide range of gastrointestinal disorders and the rare complication of constrictive pericarditis. We report a case in which pericardiectomy achieved marked improvement of extremely severe hypoalbuminemia caused by PLE associated with tuberculosis-related constrictive pericarditis. Read More

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ALG8-CDG: Molecular and phenotypic expansion suggests clinical management guidelines.

J Inherit Metab Dis 2022 Jun 18. Epub 2022 Jun 18.

Department of Pediatrics, Division of Human Genetics, Section of Metabolism, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Congenital disorders of glycosylation are a continuously expanding group of monogenic disorders of glycoprotein and glycolipid glycan biosynthesis. These disorders mostly manifest with multisystem involvement. Individuals with ALG8-CDG commonly present with hypotonia, protein-losing enteropathy, and hepatic involvement. Read More

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Transcatheter Thoracic Duct Decompression for Multicompartment Lymphatic Failure After Fontan Palliation.

Circ Cardiovasc Interv 2022 Jun 16:101161CIRCINTERVENTIONS121011733. Epub 2022 Jun 16.

Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania.

Background: Lymphatic embolization therapy has proven effective for Fontan failure from plastic bronchitis or protein-losing enteropathy but not when multiple lymphatic compartments are involved; furthermore, embolization does not alter the underlying pathophysiology of lymphatic dysfunction. A technique for transcatheter thoracic duct decompression (TDD), rerouting the thoracic duct to the pulmonary venous atrium to treat multicompartment lymphatic failure is described and early outcomes presented.

Methods: Initially covered stents were used to channel the innominate vein flow inside of the cavopulmonary pathway into the pulmonary venous atrium. Read More

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Lymphatic Phenotype of Noonan Syndrome: Innovative Diagnosis and Possible Implications for Therapy.

J Clin Med 2022 May 31;11(11). Epub 2022 May 31.

Department of Pediatrics, Radboudumc Amalia Children's Hospital, Radboud Institute for Health Sciences, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands.

Dysregulation of the Ras/Mitogen-activated protein kinase (MAPK) signaling pathway is suggested to play a pivotal role in the development of the lymphatic system in patients with Noonan Syndrome (NS). Pathogenic gene variants in the Ras/MAPK pathway can therefore lead to various lymphatic diseases such as lymphedema, chylo-thorax and protein losing enteropathy. Diagnosis and treatment of the lymphatic phenotype in patients with NS remain difficult due to the variability of clinical presentation, severity and, probably, underlying unknown pathophysiologic mechanism. Read More

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Improved Exercise Tolerance in an Adolescent Female After Failed Fontan and Subsequent Biventricular Conversion.

Pediatr Cardiol 2022 Jun 9. Epub 2022 Jun 9.

Department of Pediatrics, Rush University Medical College, Chicago, IL, USA.

We report a case of improved exercise tolerance in a single-ventricle patient following biventricular conversion. An 11 year old with a fenestrated extracardiac failing Fontan was accepted for a biventricular conversion repair at an out-of-town institution. The patient had multiple adverse cardiac events following Fontan surgery including recurrent pleural effusions, arteriovenous malformations, protein-losing enteropathy, and marked exercise intolerance. Read More

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Lymphatic Interventions in the Cancer Patient.

Curr Oncol Rep 2022 May 31. Epub 2022 May 31.

Department of Radiology, Interventional Radiology Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA.

Purpose Of Review: The incidence of lymphatic leakage (iatrogenic and non-iatrogenic) is growing in cancer population due to the increased complexity of the surgical procedures and improved overall survival in cancer patients. The purpose of this article is to review the contemporary approach in the field of percutaneous lymphatic embolization in cancer patients with lymphatic leaks.

Recent Findings: Since the advent of intranodal lymphangiography in 2011 alongside with the MR and CT lymphangiography, the accuracy of diagnosis of the lymphatic diseases has significantly improved significantly. Read More

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Impact of Fontan Fenestration on Long-Term Outcomes: A Propensity Score-Matched Analysis.

J Am Heart Assoc 2022 Jun 27;11(11):e026087. Epub 2022 May 27.

Department of Cardiac Surgery Royal Children's Hospital Melbourne Australia.

Background The long-term impact of fenestration at the time of Fontan operation remains unclear. We aimed to review the early and long-term impact of Fontan fenestration in the Australia and New Zealand cohort. Methods and Results We reviewed 1443 patients (621 fenestrated, 822 nonfenestrated) from the Australia and New Zealand Fontan registry. Read More

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Hypomagnesemia in intestinal lymphangiectasia: a case report and review of the literature.

BMC Gastroenterol 2022 May 15;22(1):246. Epub 2022 May 15.

Department of Gastroenterology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.

Background: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed.

Case Presentation: A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Read More

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Novel mutation causing congenital disorder of glycosylation in a child with recurrent anasarca.

BMJ Case Rep 2022 May 13;15(5). Epub 2022 May 13.

Pediatric Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India.

Protein-losing enteropathy entails an excessive loss of proteins in intestinal tract due to underlying primary or secondary pathologies. It is suspected in patients with chronic diarrhoea and peripheral oedema. Faecal alpha 1 antitrypsin clearance is the gold standard for diagnosis. Read More

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2022 Update of the Consensus on the Rational Use of Antithrombotics and Thrombolytics in Veterinary Critical Care (CURATIVE) Domain 1- Defining populations at risk.

J Vet Emerg Crit Care (San Antonio) 2022 May 2;32(3):289-314. Epub 2022 May 2.

School of Veterinary Medicine, Murdoch University, Murdoch, Australia.

Objectives: To expand the number of conditions and interventions explored for their associations with thrombosis in the veterinary literature and to provide the basis for prescribing recommendations.

Design: A population exposure comparison outcome format was used to represent patient, exposure, comparison, and outcome. Population Exposure Comparison Outcome questions were distributed to worksheet authors who performed comprehensive searches, summarized the evidence, and created guideline recommendations that were reviewed by domain chairs. Read More

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[Disorders of the central lymphatic system].

Ned Tijdschr Geneeskd 2022 03 21;166. Epub 2022 Mar 21.

The central lymphatic system consists of the thoracic duct, cisterna chyli and the retroperitoneal lymphatics through which lymph and chyle flows. Disorders of the central lymphatic system can for instance lead to leakage (chylothorax), accumulation of fluid (lymphedema) and retrograde flow (protein losing enteropathy). Abnormalities in the central lymphatic system were overlooked for years, followed by lack of diagnostic and therapeutic options. Read More

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Case Report: Fulminant Celiac Disease With Combination Immune Checkpoint Therapy.

Front Immunol 2022 14;13:871452. Epub 2022 Apr 14.

Department of Medicine, Massachusetts General Hospital, Boston, MA, United States.

Since the first approval of immune checkpoint inhibitors (ICIs) in 2011, these agents have rapidly become an integral treatment option across tumor types. However, with the increased adoption of ICIs, the incidence of immune-related adverse events (irAEs) continues to rise, and rare toxicity continues to be reported. Here, we present a case of a 70-year-old male patient with widespread metastatic melanoma who developed rapid onset anasarca and transaminitis after initiation of dual anti-PD-1/CTLA-4 inhibition with nivolumab and ipilimumab. Read More

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Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of Mortality.

Korean Circ J 2022 Mar 16. Epub 2022 Mar 16.

Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

Background And Objectives: Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE.

Methods: We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea. Read More

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A Rare Case of Juvenile Polyposis Syndrome Mimicking Ménétrier's Disease.

Cureus 2022 Mar 22;14(3):e23389. Epub 2022 Mar 22.

Gastroenterology, Upstate University Hospital, Syracuse, USA.

There is a wide differential diagnosis within polyposis syndromes. Our case represents an interesting and diagnostically challenging diagnosis involving a 41-year-old male who presented with an incidental gastric mass on imaging and a colonic mass seen on colonoscopy. Following multiple endoscopic evaluations, histological analysis, and genetic testing, the patient was ultimately diagnosed with juvenile polyposis syndrome (JPS)/hereditary hemorrhagic telangiectasia (HHT) despite the initial suspicion for Ménétrier's disease. Read More

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Cytomegalovirus enteritis: a surprising cause of severe diarrhoea and protein-losing gastroenteropathy in an intensive care patient.

BMJ Case Rep 2022 04 22;15(4). Epub 2022 Apr 22.

Department of Emergency and General Internal Medicine, Hitachinaka General Hospital, Hitachinaka, Ibaraki, Japan.

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Protein-losing enteropathy recurrence after pediatric heart transplantation: Multicenter case series.

Pediatr Transplant 2022 Apr 21:e14295. Epub 2022 Apr 21.

Division of Cardiology, Department of Pediatrics, Children's Hospital of Colorado, University of Colorado Denver, Aurora, Colorado, USA.

Background: Protein-losing enteropathy (PLE) is a devastating complication of the Fontan circulation. Although orthotopic heart transplantation (HTx) typically results in resolution of PLE symptoms, isolated cases of PLE relapse have been described after HTx.

Methods: Patients with Fontan-related PLE who had undergone HTx at participating centers and experienced relapse of PLE during follow-up were retrospectively identified. Read More

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To Study Role of Serial Serum Albumin Estimation as Prognostic Marker in Critically Ill Patients.

J Assoc Physicians India 2022 Apr;70(4):11-12

Govt Medical College, Kota.

Albumin is the most abundant plasma protein in humans. It works to maintain the colloid oncotic pressures, acts as a carrier protein, and is involved in metabolism, antioxidant, and various other functions. Patients who are admitted to the Intensive Care Unit (I. Read More

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Surgical and interventional rescue strategies for Fontan failure.

Interact Cardiovasc Thorac Surg 2022 Apr 19. Epub 2022 Apr 19.

Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, Linz, Austria.

Objectives: Fontan patients are at lifelong risk for developing complications which may result in Fontan failure. Survival rates after heart transplantation (HTX) are still unsatisfying in these patients. Long term survival of extracardiac Fontan patients in the modern era was investigated. Read More

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Fontan Circulation Associated Organ Abnormalities Beyond the Heart, Lungs, Liver, and Gut: A Systematic Review.

Front Cardiovasc Med 2022 22;9:826096. Epub 2022 Mar 22.

Division of Pediatric Cardiology, Department of Pediatrics, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, Netherlands.

Introduction: Patients with a Fontan circulation are at risk for sequelae of Fontan physiology during follow-up. Fontan physiology affects all organ systems and an overview of end-organ damage is needed.

Methods: We performed a systematic review of abnormalities in multiple organ systems for patients with a longstanding Fontan circulation. Read More

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Are plasma proteins a valid alternative for assessing nephrotic syndrome in children from low-income countries?

Arch Pediatr 2022 May 2;29(4):263-266. Epub 2022 Apr 2.

Reference Centre for Rare Renal diseases Néphrogones, Hospices Civils de Lyon, Lyon, France; Faculté de Médecine Lyon Est, Université Claude-Bernard Lyon 1, Lyon, France.

Background: A diagnosis of nephrotic syndrome (NS) in children with edema relies on urinary albumin excretion and usually plasma protein (Pprot) and albumin (Palb) concentrations.

Methods: In order to fit laboratory tests to optimal healthcare in low-resource countries, we established correlations between Pprot and Palb in children with NS (217 measurements in 60 patients) and in children with exudative enteropathy and chronic hepatopathy/liver insufficiency (186 measurements in 21 patients); all patients had repeated measurements at various stages of their disease.

Results: There was a good correlation between Pprot and Palb in children with idiopathic NS and genetic NS (ICC=0. Read More

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Polygenic Infantile Juvenile Polyposis Syndrome Managed With Sirolimus and Endoscopic Polypectomy.

Gastroenterology Res 2022 Feb 10;15(1):33-38. Epub 2022 Jan 10.

Division of Pediatric Gastroenterology, University of Iowa Stead Family Children's Hospital, Iowa City, IA 52242, USA.

In the following clinical case of infantile juvenile polyposis syndrome (JPS), administration of a pharmacologic agent sirolimus was associated with reduced disease burden without need for bowel resection. The positive impact included improvement in protein-losing enteropathy, decreased intestinal blood loss, and improved weight gain. In addition, the number of polyps resected per unit time and frequency of upper and lower endoscopic evaluation needed dropped after initiation of sirolimus. Read More

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February 2022

A Preliminary Metabolomic Study of Yorkshire Terrier Enteropathy.

Metabolites 2022 Mar 19;12(3). Epub 2022 Mar 19.

Division of Small Animal Internal Medicine, University of Veterinary Medicine, 1210 Vienna, Austria.

Perturbations of metabolite profiles in human and canine enteropathies have been reported before. However, data in dogs are scarce and inconsistent. Currently, the metabolite profile in Yorkshire Terrier enteropathy (YTE) and the impact of treatment is unknown. Read More

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CD55-deficiency in Jews of Bukharan descent is caused by the Cromer blood type Dr(a-) variant.

Hum Genet 2022 Mar 21. Epub 2022 Mar 21.

The Genetics Institute and Genomics Center, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

The complement system regulator CD55 was initially found to carry the Cromer blood group system antigens, and its complete loss of function was subsequently revealed to cause a severe monogenic gastrointestinal syndrome characterized by protein-losing enteropathy and susceptibility to venous thrombosis. Here we present homozygosity to the CD55 c.596C>T; p. Read More

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Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies.

Can J Cardiol 2022 Mar 18. Epub 2022 Mar 18.

Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA.

Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ∼4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. Read More

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

BMC Gastroenterol 2022 Mar 7;22(1):101. Epub 2022 Mar 7.

Department of Gastroenterology, Nara Medical University, Kashihara, Nara, 634-8522, Japan.

Background: Protein-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 Presentation: A 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. Read More

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Primary intestinal lymphangiectasia: a rare disease as a cause of protein-losing enteropathy.

Rev Esp Enferm Dig 2022 Mar 4. Epub 2022 Mar 4.

Aparato Digestivo, Complejo Asistencial Universitario de León, España.

Primary intestinal lymphangiectasia is a rare disorder associated with protein-losing enteropathy. The main manifestations are those resulting from hypoalbuminemia. Diagnosis requires the typical endoscopic image of intestinal lymphangiectasia and increased 24-hour fecal alpha-1-antitrypsin clearance. Read More

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