2,866 results match your criteria Protein-Losing Enteropathy


Diagnosis of a Lymphoenteric Fistula by Single-Photon Emission Computed Tomography/Computed Tomography Lymphoscintigraphy.

ACG Case Rep J 2020 Jun 11;7(6):e00388. Epub 2020 Jun 11.

Department of Allergy and Clinical Immunology, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, UK.

Protein losing enteropathy can present as an immunodeficiency. This report describes a rare cause of protein losing enteropathy due to a lymphoenteric fistula and how a novel use of a pre-existing combined imaging technique of single-photon emission computed tomography/computed tomography lymphoscintigraphy helped in making the diagnosis. Read More

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http://dx.doi.org/10.14309/crj.0000000000000388DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7304535PMC

Intestinal lymphangiectasia in a 3-month-old girl: A case report of Hennekam syndrome caused by CCBE1 mutation.

Medicine (Baltimore) 2020 Jul;99(27):e20995

Pediatric Clinic, Pietro Barilla Children's Hospital, Department of Medicine and Surgery, University of Parma, Parma, Italy.

Rational: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilatation and rupture of intestinal lymphatic channels leading to protein-losing enteropathy. IL is classified as primary and secondary types.

Patient Concerns: A 3-month-old girl born at term from vaginal delivery with an APGAR score of 10/10 and birth weight of 4. Read More

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http://dx.doi.org/10.1097/MD.0000000000020995DOI Listing

Protein-losing enteropathy as a complication of spontaneous isolated superior mesenteric artery dissection: A case report.

Medicine (Baltimore) 2020 Jun;99(25):e20580

Department of Vascular Surgery, The Clinical Medical School of Yangzhou University, Yangzhou.

Introduction: Protein-losing enteropathy and spontaneous isolated superior mesenteric artery dissection are both rare clinically. Protein-losing enteropathy due to superior mesenteric artery dissection is extremely rare.

Patient Concerns: A 46-year-old male with acute abdominal pain and hematochezia was diagnosed with a complete occlusion of the superior mesenteric artery because of dissection. Read More

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http://dx.doi.org/10.1097/MD.0000000000020580DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310960PMC

RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification.

J Card Surg 2020 Jul 19;35(7):1721-1724. Epub 2020 Jun 19.

Department of Pediatric Cardiac Surgery, Friedrich-AlexanderUniversity Erlangen-Nürnberg, Erlangen, Germany.

Fontan patients with protein-losing enteropathy (PLE) represent poor candidates for cardiac transplantation due to end-organ injury and severely impaired clinical condition. Ventricular assist device (VAD) therapy has evolved as a promising bridge to transplant strategy improving quality of life and survival on the waiting list. However, VAD therapy for the Fontan circulation remains challenging. Read More

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http://dx.doi.org/10.1111/jocs.14658DOI Listing

Tuberous sclerosis complex presenting as primary intestinal lymphangiectasia: A case report.

World J Clin Cases 2020 May;8(10):1995-2000

Department of Gastroenterology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou 510623, Guangdong Province, China.

Background: Primary intestinal lymphangiectasia (PIL) is a rare congenital protein-losing enteropathy caused by dysplasia of the small intestinal lymphatics. The cause of the disease is unknown. Through a literature review, we found that PIL and tuberous sclerosis complex (TSC) have some common symptoms and molecular pathways. Read More

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http://dx.doi.org/10.12998/wjcc.v8.i10.1995DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7262713PMC

Successful treatment of large abdominal lymphatic malformations and chylous ascites with intra-abdominal lymphovenous anastomosis.

J Vasc Surg Venous Lymphat Disord 2020 Jun 4. Epub 2020 Jun 4.

Department of Plastic and Reconstructive Surgery, Mie University Hospital, Mie, Japan.

Large abdominal lymphatic malformations are rare and may occasionally cause life-threatening illness especially when they involve the central lymphatic system, lumbar trunks, cisterna chyli, thoracic duct and their major tributaries forming complex lymphatic anomalies. These LM are often accompanied by chylous leak in various locations and treatment remains challenging. We report a case of large abdominal lymphatic malformation with chylous ascites, protein-losing enteropathy, vaginal chylous leak and lower limb lymphedema successfully treated with microsurgical intra-abdominal lymphovenous anastomosis and discuss the technical details of the procedure. Read More

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http://dx.doi.org/10.1016/j.jvsv.2020.05.017DOI Listing

Comprehensive Targeted Sequencing Identifies Monogenic Disorders in Patients With Early-onset Refractory Diarrhea.

J Pediatr Gastroenterol Nutr 2020 Jun 1. Epub 2020 Jun 1.

Department of Pediatrics, Tohoku University Graduate School of Medicine.

Objectives: Causes of early-onset refractory diarrhea include exudative diarrhea associated with very early-onset inflammatory bowel diseases, osmotic or secretory diarrhea, and protein-losing enteropathy. Monogenic disorders are included in these diseases, yet a comprehensive genetic analysis has not been fully established.

Methods: We established targeted gene panels covering all responsible genes for early-onset diarrhea. Read More

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http://dx.doi.org/10.1097/MPG.0000000000002796DOI Listing

Imaging of complications following Fontan circulation in children - diagnosis and surveillance.

Pediatr Radiol 2020 May 28. Epub 2020 May 28.

Department of Radiology and Clinical Physiology, Queen Silvia Children's Hospital, Rondv.10, S-41516, Gothenburg, Sweden.

The Fontan operation is performed for various cardiac lesions with single-ventricle physiology. The survival rate of Fontan patients is increasing for adolescents and young adults, with an expected 30-year survival of >80%. Medical health care providers including specialists in organ systems and pediatric radiologists need to improve their knowledge about the Fontan circulation and the various organ complications to monitor care. Read More

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http://dx.doi.org/10.1007/s00247-020-04682-5DOI Listing

Intramesenteric dynamic contrast pediatric MR lymphangiography: initial experience and comparison with intranodal and intrahepatic MR lymphangiography.

Eur Radiol 2020 May 27. Epub 2020 May 27.

Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA.

Objectives: To report on our initial experience with intramesenteric (IM) dynamic contrast magnetic resonance lymphangiography (DCMRL) for evaluation of the lymphatics in patients with concern for mesenteric lymphatic flow disorders and to compare IM-DCMRL with intrahepatic (IH) and intranodal (IN) DCMRL.

Methods: This is a retrospective review of imaging findings in 15 consecutive patients who presented with protein losing enteropathy (PLE) and/or ascites undergoing IM-DCMRL, IH-DCMRL, and IN-DCMRL. The IM-DCMRL technique involves the injection of a gadolinium contrast agent into the mesenteric lymphatic ducts or lymph nodes followed by imaging of the abdomen and chest with dynamic time-resolved MR lymphangiography. Read More

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http://dx.doi.org/10.1007/s00330-020-06949-9DOI Listing

Human-Derived α1-Antitrypsin is Still Efficacious in Heavily Pretreated Patients with Steroid-Resistant Gastrointestinal Graft-versus-Host Disease.

Biol Blood Marrow Transplant 2020 May 25. Epub 2020 May 25.

Service d'Hématologie-greffe, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris, Paris, France; Université de Paris, INSERM U976, Paris, France. Electronic address:

Almost one-half of patients developing graft-versus-host disease (GVHD) will not respond to standard first-line steroid treatment. Alpha-1 antitrypsin (AAT) is able to induce tolerance in preclinical models of GVHD. AAT alters the cytokine milieu, promotes a tolerogenic shift of dendritic cells, and skews effector T cells toward regulatory T cells. Read More

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http://dx.doi.org/10.1016/j.bbmt.2020.05.014DOI Listing
May 2020
3.404 Impact Factor

Percutaneous Extra-Anatomic Lymphovenous Bypass Creation: Toward Treatment of Central Conducting Lymphatic Obstructions.

Cardiovasc Intervent Radiol 2020 May 22. Epub 2020 May 22.

Division of Interventional Radiology, University of Washington, 1959 Northeast Pacific Street, Seattle, WA, 98195, USA.

Introduction: Protein-losing enteropathy manifests as a loss of serum proteins through the gastrointestinal tract, resulting in hypoproteinemia, extravascular fluid retention, and edema. Management consists of nutritional maintenance in conjunction with interventions targeted at treating the underlying etiology.

Materials And Methods: This report describes a patient with protein-losing enteropathy from a central conducting lymphatic obstruction who was treated with percutaneous extra-anatomic lymphovenous bypass creation. Read More

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http://dx.doi.org/10.1007/s00270-020-02457-xDOI Listing

Egested Feces Revealed Massive Protein Loss in 99mTc-HSA-D Scintigraphy.

Clin Nucl Med 2020 Jul;45(7):e329-e330

From the Department of Radiology, Keio University School of Medicine, Tokyo, Japan.

A 66-year-old man with severe diarrhea and severe hypoproteinemia was referred for Tc-DTPA-human serum albumin (HSA-D) scintigraphy. The scintigraphy at 24 hours postinjection showed strong accumulation of Tc-HSA-D in not only the colon but also all his diapers containing his egested feces. The ratio of the diapers counts to the total counts was 17. Read More

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http://dx.doi.org/10.1097/RLU.0000000000003080DOI Listing

Bosch-Boonstra-Schaaf optic atrophy syndrome (BBSOAS) initially diagnosed as ALG6-CDG: Functional evidence for benignity of the ALG6 c.391T>C (p.Tyr131His) variant and further expanding the BBSOAS phenotype.

Eur J Med Genet 2020 Jul 11;63(7):103941. Epub 2020 May 11.

Department of Clinical Genomics, Mayo Clinic, Rochester, MN, USA; Center for Individualized Medicine, Mayo Clinic, Rochester, MN, USA.

Bosch-Boonstra-Schaaf optic atrophy syndrome (BBSOAS) is a recently described autosomal dominant syndrome of developmental delay, cortical vision loss with optic nerve atrophy, epilepsy, and autism spectrum disorder. Due to its many overlapping features with congenital disorders of glycosylation (CDG), the differential diagnosis between these disorders may be difficult and relies on molecular genetic testing. We report on a 31-year-old female initially diagnosed with ALG6-CDG based on glycosylation abnormalities on transferrin isoelectrofocusing and targeted genetic testing, and later diagnosed with BBSOAS by whole-exome sequencing (WES). Read More

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http://dx.doi.org/10.1016/j.ejmg.2020.103941DOI Listing

Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia.

Case Rep Med 2020 20;2020:7870154. Epub 2020 Apr 20.

Internal Medicine Department, Hospital de Especialidades Eugenio Espejo, Quito, Ecuador.

Intestinal lymphangiectasia is a pathological dilation of enteric lymphatic vessels resulting in lymph leakage to the intestinal lumen. This chronic lymph leakage leads to a state of immunosuppression secondary to the loss of humoral and cellular components of the immune system and represents a potential risk factor for opportunistic infections. We report a case of protein-losing enteropathy in a seemingly immunocompetent patient. Read More

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http://dx.doi.org/10.1155/2020/7870154DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7191395PMC

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients.

Cardiol Young 2020 May 4;30(5):698-709. Epub 2020 May 4.

Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany.

Objective: Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality. Read More

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http://dx.doi.org/10.1017/S1047951120000864DOI Listing

Lymphatic Management in Single-Ventricle Patients.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020 ;23:41-47

Neours/Dupont Children's Hospital, Wilmington, Delaware.

Lymphatic complications in patients with single ventricle include plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion are a source of significant morbidity and mortality with historically limited therapeutic options. Novel lymphatic imaging techniques such as intranodal lymphangiography, dynamic contrast enhanced magnetic resonance lymphangiography and liver lymphangiography have allowed visualization of the lymphatic system and discovery of the pathophysiological mechanism of these conditions. This mechanism includes the combination of 2 factors: increased lymphatic flow in patients with elevated central venous pressure and presence of the lymphatic anatomical variant that allows the lymph to flow in close proximity to the serous (pleural space in chylothorax) or mucosal (plastic bronchitis and protein losing enteropathy) surfaces. Read More

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http://dx.doi.org/10.1053/j.pcsu.2020.03.001DOI Listing
January 2020

Innominate Vein Turn-down Procedure for Failing Fontan Circulation.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020 ;23:34-40

Division of Pediatric Cardiology, Department of Pediatrics, Herma Heart Institute, Medical College of Wisconsin, Milwaukee, Wisconsin.

After the Fontan, systemic venous hypertension induces pathophysiologic changes in the lymphatic system that can result in complications of pleural effusion, ascites, plastic bronchitis, and protein losing enteropathy. Advances in medical therapy and novel interventional approaches have not substantially improved the poor prognosis of these complications. A more physiological approach has been developed by decompression of the thoracic duct to the lower pressure common atrium with a concomitant increase of preload. Read More

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http://dx.doi.org/10.1053/j.pcsu.2020.01.002DOI Listing
January 2020

Serum amino acid profile in 51 dogs with immunosuppressant-responsive enteropathy (IRE): a pilot study on clinical aspects and outcomes.

BMC Vet Res 2020 Apr 22;16(1):117. Epub 2020 Apr 22.

Professional Association Endovet Rome, Rome, Italy.

Background: Lower levels of tryptophan (TRP) have been identified in people with inflammatory bowel disease and in dogs with protein-losing enteropathy (PLE). No data on serum amino acids (AAs) but some on plasma in canine immunosuppressant-responsive enteropathy (IRE) are available. The aim of this study is to compare serum AAs between healthy and IRE dogs, considering clinicopathological variables and follow-up. Read More

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http://dx.doi.org/10.1186/s12917-020-02334-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7178940PMC

Hypoalbuminemia and generalized edema as an atypical presentation of celiac disease.

J Family Med Prim Care 2020 Feb 28;9(2):1206-1208. Epub 2020 Feb 28.

Department of Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

The clinical presentation of celiac disease has evolved significantly over the last few decades. Previously defined as a malabsorption syndrome in pediatric patients, now it is considered an autoimmune disorder with diverse systemic manifestations among all age groups. We report a case of 56-year-old male presented with pedal edema and gradually progressive abdomen distention for the last 3 months. Read More

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http://dx.doi.org/10.4103/jfmpc.jfmpc_1116_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7114008PMC
February 2020

Improvement in Protein-Losing Enteropathy in a Patient With Fontan Circulation After Cardiac Rehabilitation and Prescriptive Exercise.

World J Pediatr Congenit Heart Surg 2020 May;11(3):364-365

Department of Pediatrics, Section of Pediatric and Adult Congenital Cardiology, Baylor College of Medicine/Texas Children's Hospital, Houston, TX, USA.

Fontan-associated protein-losing enteropathy is difficult to treat and associated with poor prognosis. Cardiac rehabilitation and exercise are thought to have beneficial effects for patients with Fontan circulation. We report the case of a young adult patient palliated to Fontan circulation, with a decade-long history of symptoms related to protein-losing enteropathy. Read More

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http://dx.doi.org/10.1177/2150135119896557DOI Listing

Consensus guideline for the diagnosis and management of mannose phosphate isomerase-congenital disorder of glycosylation.

J Inherit Metab Dis 2020 Jul 21;43(4):671-693. Epub 2020 Apr 21.

Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic.

Mannose phosphate isomerase-congenital disorder of glycosylation (MPI-CDG) deficiency is a rare subtype of congenital disorders of protein N-glycosylation. It is characterised by deficiency of MPI caused by pathogenic variants in MPI gene. The manifestation of MPI-CDG is different from other CDGs as the patients suffer dominantly from gastrointestinal and hepatic involvement whereas they usually do not present intellectual disability or neurological impairment. Read More

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http://dx.doi.org/10.1002/jimd.12241DOI Listing

Anesthetic Considerations for Fontan-Associated Liver Disease and the Failing Fontan Circuit.

J Cardiothorac Vasc Anesth 2020 Aug 29;34(8):2224-2233. Epub 2020 Feb 29.

Department of Anesthesiology, Children's Hospital Colorado, Anschutz Medical Campus, Aurora, CO; School of Medicine, University of Colorado, Aurora, CO.

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http://dx.doi.org/10.1053/j.jvca.2020.02.040DOI Listing

Lymphopenia in adults after the Fontan operation: prevalence and associations.

Cardiol Young 2020 May 6;30(5):641-648. Epub 2020 Apr 6.

Cincinnati Children's Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.

Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Read More

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http://dx.doi.org/10.1017/S1047951120000694DOI Listing

The Usefulness of Scintigraphic Studies in the Assessment of Asymptomatic Bowel Disease in Patients with Primary Antibody Diseases.

J Clin Med 2020 Mar 30;9(4). Epub 2020 Mar 30.

Department of Radiological, Oncological and Pathological Anatomy Sciences, Sapienza University of Rome, 00185 Rome, Italy.

Enteropathy may be the first presentation of immunodeficiency or it may occur during the course of the disease and in association with malabsorption in patients affected by primary antibody diseases. For these patients, immunoglobulin G (IgG) replacement therapy prevents infectious and non-infectious complications. Nonetheless some patients cannot achieve optimal IgG trough levels, even when treated with high Ig doses in absence of protein-losing syndromes. Read More

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http://dx.doi.org/10.3390/jcm9040949DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230964PMC

An overlooked case of a treatable hyperinsulinemic hypoglycemia: congenital glycosylation defect Type Ib.

Turk Pediatri Ars 2020 9;55(1):79-81. Epub 2020 Mar 9.

Department of Pediatric Metabolism, Ankara University Faculty of Medicine, Ankara, Turkey.

Congenital glycosylation defects are autosomal recessive disorders clinically characterized with growth retardation, hypotonia and multisystemic involvement. Congenital glycosylation defect type Ib is due to deficiency in phosphomannose isomerase which converts fructose-6-phosphate into mannose-6-phosphate. Patients usually present with hepatic or gastrointestinal symptoms lacking cranial involvement, making their IQ completely normal. Read More

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http://dx.doi.org/10.5152/TurkPediatriArs.2018.18004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7096565PMC

A Flare-up of Systemic Lupus Erythematosus with Unusual Enteric Predominance.

Cureus 2020 Feb 21;12(2):e7068. Epub 2020 Feb 21.

Internal Medicine, Texas Tech University Health Sciences Center of the Permian Basin, Odessa, USA.

Enteritis associated with systemic lupus erythematosus (SLE) is a rare and unusual manifestation of the gastrointestinal (GI) consequences of SLE itself. Complications of the enteritis component include mesenteric vasculitis, intestinal pseudo-obstruction, and protein-losing enteropathy. Lupus enteritis is very responsive to treatment with pulse steroids in almost 70% of the patients, but it is critical to diagnose it early to prevent devastating organ damage. Read More

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http://dx.doi.org/10.7759/cureus.7068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7089623PMC
February 2020

Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

J Am Heart Assoc 2020 Apr 30;9(7):e015318. Epub 2020 Mar 30.

Division of Cardiology Children's Hospital of Philadelphia PA.

Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein-losing enteropathy. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined. Methods and Results This was a retrospective, single-center study of patients undergoing first-time Fontan completion from 2012 to 2017. Read More

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http://dx.doi.org/10.1161/JAHA.119.015318DOI Listing

Invasive cardiovascular magnetic resonance (iCMR) for diagnostic right and left heart catheterization using an MR-conditional guidewire and passive visualization in congenital heart disease.

J Cardiovasc Magn Reson 2020 03 26;22(1):20. Epub 2020 Mar 26.

Department of Pediatrics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.

Background: Today's standard of care, in the congenital heart disease (CHD) population, involves performing cardiac catheterization under x-ray fluoroscopy and cardiac magnetic resonance (CMR) imaging separately. The unique ability of CMR to provide real-time functional imaging in multiple views without ionizing radiation exposure has the potential to be a powerful tool for diagnostic and interventional procedures. Limiting fluoroscopic radiation exposure remains a challenge for pediatric interventional cardiologists. Read More

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http://dx.doi.org/10.1186/s12968-020-0605-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7098096PMC

An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar.

Front Pediatr 2020 4;8:82. Epub 2020 Mar 4.

Pediatric Section, Department of Health Sciences, Meyer Children's University Hospital, University of Florence, Florence, Italy.

Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g. Read More

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http://dx.doi.org/10.3389/fped.2020.00082DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7065258PMC

Survival Times of Patients With Menetrier's Disease and Risk of Gastric Cancer.

Clin Gastroenterol Hepatol 2020 Mar 14. Epub 2020 Mar 14.

Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota; Faculty of Health and Medicine, University of Newcastle, Callaghan, New South Wales, Australia. Electronic address:

Background & Aims: Menetrier's disease is a rare acquired disorder associated with giant gastric folds along with protein-losing enteropathy, hypochlorhydria or achlorhydria, and histologic features of massive foveolar hyperplasia. Little is known about the etiology, clinical features, or epidemiology of this disorder, including risk of gastric cancer. We investigated the outcomes and characteristics of patients with Menetrier's disease, including development of gastric cancer and survival times. Read More

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http://dx.doi.org/10.1016/j.cgh.2020.03.017DOI Listing

A girl with protein-losing enteropathy during a ketogenic diet: a case report.

BMC Pediatr 2020 Mar 3;20(1):102. Epub 2020 Mar 3.

Department of Neurology, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Chongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory Disorders, Children's Hospital of Chongqing Medical University, No.136, zhongshan 2nd road, yuzhong district, chongqing, 400014, China.

Background: A ketogenic diet (KD) is an effective treatment for intractable epilepsy in children. Protein-losing enteropathy (PLE) is a rarely reported but serious complication of KDs.

Case Presentation: A 3-month-old female patient presented with PLE while following a KD as treatment for intractable epilepsy. Read More

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http://dx.doi.org/10.1186/s12887-020-1991-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7053130PMC

Extended Treatment With Mesenchymal Stromal Cells-Frankfurt am Main (MSC-FFM, Obnitix) in a Pediatric Patient With Steroid-refractory Acute Gastrointestinal Graft-Versus-Host Disease (GVHD): Case Report and Review of the Literature.

J Pediatr Hematol Oncol 2020 Feb 28. Epub 2020 Feb 28.

Department of Pediatrics, Jena University Hospital, Jena, Germany.

In acute graft-versus-host disease (aGVHD) following allogeneic hematopoietic stem cell transplantation, there are various options available after the failure of initial steroid therapy. Since the publication of the first study in 2008, mesenchymal stromal cells (MSCs) have also been used with increasing frequency, including in pediatric patients with steroid-refractory aGVHD, and the manufacturing process has undergone further development. MSC-Frankfurt am Main (MSC-FFM, Obnitix), which is manufactured from pooled mononuclear bone marrow cells from 8 donors using a standardized process, resulted in a response rate of 84% in children with steroid-refractory aGVHD. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001758DOI Listing
February 2020

Protein-losing enteropathy.

Curr Opin Gastroenterol 2020 May;36(3):238-244

Center for Prevention and Diagnosis of Coeliac Disease - Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico.

Purpose Of Review: The present review offers its readers a practical overview of protein-losing enteropathy, particularly with regard to diagnostic and therapeutic approaches. The aim is to support clinicians in their daily practice with a practical tool to deal with protein-losing enteropathy.

Recent Findings: The literature covering protein-losing enteropathy does not appear to be quite recent and also guidelines are scanty. Read More

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http://dx.doi.org/10.1097/MOG.0000000000000629DOI Listing

An emerging role for endothelial barrier support therapy for congenital disorders of glycosylation.

J Inherit Metab Dis 2020 Jul 27;43(4):880-890. Epub 2020 Feb 27.

Division of Genetics and Genomics, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.

Congenital disorders of glycosylation (CDGs) are clinically heterogeneous disorders defined by a decreased ability to modify biomolecules with oligosaccharides. Critical disruptions in protein recognition, interaction, binding, and anchoring lead to broad physiological effects. Patients present with endocrinopathy, immunodeficiency, hepatopathy, coagulopathy, and neurodevelopmental impairment. Read More

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http://dx.doi.org/10.1002/jimd.12225DOI Listing
July 2020
3.365 Impact Factor

Clinical characteristics of dogs with food-responsive protein-losing enteropathy.

J Vet Intern Med 2020 Mar 15;34(2):659-668. Epub 2020 Feb 15.

Laboratory of Veterinary Internal Medicine, Department of Clinical Sciences, Graduate School of Veterinary Medicine, Hokkaido University, Sapporo, Hokkaido, Japan.

Background: In dogs with protein-losing enteropathy (PLE), data on the clinical characteristics of food-responsive PLE (FR-PLE) remain scarce.

Objective: To determine the clinical characteristics of FR-PLE in dogs responsive to ultralow-fat diet (ULFD) management.

Animals: Thirty-three dogs diagnosed with PLE based on standard diagnostic criteria. Read More

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http://dx.doi.org/10.1111/jvim.15720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7096654PMC

A PTPN11 mutation in a woman with Noonan syndrome and protein-losing enteropathy.

BMC Gastroenterol 2020 Feb 13;20(1):34. Epub 2020 Feb 13.

Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1, Shuaifuyuan, Wangfujing St. Beijing, Beijing, 100730, China.

Background: Noonan syndrome is an autosomal dominant, variably expressed multisystem disorder characterized by specific facial and cardiac defects, delayed growth, ectodermal abnormalities, and lymphatic dysplasias. Lymphedema and chylous pleural effusions are common in Noonan syndrome, but protein-losing enteropathy (PLE) has only rarely been described in the condition and little is known about its genetic associations.

Case Presentation: We report the case of a 30-year-old Chinese woman who developed severe recurrent edema and hypoproteinemia. Read More

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http://dx.doi.org/10.1186/s12876-020-01187-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7017519PMC
February 2020
2.365 Impact Factor

Primary Intestinal Lymphangiectasia (Waldmann's Disease) A Rare Case of Protein Losing Enteropathy.

J Assoc Physicians India 2020 Jan;68(1):89

MGM Medical College and MY Hospital, Indore, MP.

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January 2020

Portomesenteric Venous Complications after Pancreatic Surgery with Venous Reconstruction: Imaging and Intervention.

Radiographics 2020 Mar-Apr;40(2):531-544. Epub 2020 Jan 24.

From the Department of Radiology, Division of Vascular and Interventional Radiology (S.M.T., C.J.F., J.C.A.), and Department of Surgery, Division of Hepatobiliary and Pancreas Surgery (L.Y., M.J.T., M.L.K.), Mayo Clinic, 200 1st St SW, Rochester, MN 55905.

Pancreatic surgery with en bloc venous resection and reconstruction is becoming increasingly common in the current era of expanding neoadjuvant oncologic therapies and advanced surgical techniques for patients with more anatomically complex tumors. However, patients who have alterations in their venous outflow are at increased risk for postoperative portomesenteric venous stenosis and/or thrombosis. Cross-sectional imaging for postoperative surveillance, including multiphase CT or MRI, is critical for recognizing portomesenteric venous complications and thus implementing early intervention and preventing complications related to portomesenteric venous hypertension. Read More

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http://dx.doi.org/10.1148/rg.2020190100DOI Listing
January 2020

Are children with protein-losing enteropathy after the Fontan operation at increased risk of cytomegalovirus enteropathy? A report of two cases.

Cardiol Young 2020 Mar 24;30(3):431-432. Epub 2020 Jan 24.

Department for Pediatric Cardiology, Kepler University Hospital GmbH, Johannes Kepler University Linz, Linz, Austria.

Introduction: Aetiology of protein-losing enteropathy in single-ventricle type CHD is multi-factorial.

Report: We describe two Fontan patients with protein-losing enteropathy who presented with cytomegalovirus-associated colitis.

Discussion: Fontan patients display risk factors for cytomegalovirus-induced gastroenteropathy that may affect lymph angiogenesis, disease development, and progression. Read More

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http://dx.doi.org/10.1017/S1047951120000049DOI Listing

Fontan-associated nephropathy: Predictors and outcomes.

Int J Cardiol 2020 05 10;306:73-77. Epub 2020 Jan 10.

Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia; Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia. Electronic address:

Background: Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown.

Methods: The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. Read More

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http://dx.doi.org/10.1016/j.ijcard.2020.01.014DOI Listing

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

Liver Int 2020 Mar 22;40(3):638-645. Epub 2020 Jan 22.

Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, University of Alcala, Madrid, Spain.

Background And Aims: 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. Read More

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http://dx.doi.org/10.1111/liv.14375DOI Listing

Extracardiac Fontan Fenestration Device Closure with Amplatzer Vascular Plug II and Septal Occluder: Procedure Results and Medium-Term Follow-Up.

Pediatr Cardiol 2020 Apr 1;41(4):703-708. Epub 2020 Jan 1.

Division of Pediatric Cardiology, Department of Pediatrics, St. Louis University School of Medicine, Cardinal Glennon Children's Hospital, 1465 S. Grand Blvd, St. Louis, MO, 63104, USA.

The objective of this study was to determine medium-term morbidity and mortality of patients who have undergone device closure of an extracardiac Fontan fenestration with an Amplatzer Vascular Plug II (AVPII) or Septal Occluder (ASO). A secondary objective was to compare medium-term morbidity and mortality between these patients and other fenestrated Fontan patients. A retrospective chart review was performed on patients who underwent an extracardiac fenestrated Fontan procedure between 1992 and 2015 at Cardinal Glennon Children's Medical Center. Read More

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http://dx.doi.org/10.1007/s00246-019-02283-0DOI Listing

Heterotaxy Is Not a Risk Factor for Adverse Long-Term Outcomes After Fontan Completion.

Ann Thorac Surg 2019 Dec 28. Epub 2019 Dec 28.

Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia; Faculty of Health and Medicine, Sydney Medical School, Discipline of Child and Adolescent Health, University of Sydney, Sydney, Australia; Sydney Children's Hospital Network Cardiac Services, Sydney, Australia. Electronic address:

Background: Heterotaxy is considered a risk factor for poor outcomes after the Fontan operation. However, long-term data to support this notion are lacking. The aims of this study were to ascertain the long-term outcomes of patients with heterotaxy after hospital discharge after Fontan completion and to compare these outcomes with those of a contemporary nonheterotaxy cohort. Read More

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http://dx.doi.org/10.1016/j.athoracsur.2019.11.015DOI Listing
December 2019
3.849 Impact Factor

Long-term therapeutic effect of Fontan conversion with an extracardiac conduit.

Eur J Cardiothorac Surg 2020 May;57(5):951-957

Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

Objectives: The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit.

Methods: Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. Read More

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http://dx.doi.org/10.1093/ejcts/ezz355DOI Listing

Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis.

World J Pediatr Congenit Heart Surg 2020 01;11(1):85-91

University of Hawaii Internal Medicine Residency Program, Honolulu, HI, USA.

Background: Recent studies have shown that oral budesonide can be used to improve albumin level in patients with protein-losing enteropathy (PLE) following Fontan procedure. However, there has never been a systematic review and meta-analysis to confirm this finding. We performed a systematic review and meta-analysis to explore the therapeutic effect of budesonide in patients with PLE post-Fontan procedure. Read More

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http://dx.doi.org/10.1177/2150135119872196DOI Listing
January 2020

Protein Losing Enteropathy After Fontan Operation: Glimpses of Clarity Through the Lifting Fog.

World J Pediatr Congenit Heart Surg 2020 01;11(1):92-96

Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Fontan-associated protein losing enteropathy is a challenge to treat and is a major contributor to morbidity and mortality in patients with palliated single ventricle. Numerous strategies for management have been proposed, with confusion as to how best to stratify and implement care among the many treatments available. Medical management can be helpful in some, while novel lymphatic interventions hold potential for remission with good results. Read More

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http://dx.doi.org/10.1177/2150135119890555DOI Listing
January 2020

Bilateral Central Serous Retinal Detachment in Protein-losing Enteropathy.

Korean J Ophthalmol 2019 12;33(6):577-578

Farabi Eye Hospital, Tehran University of Medical Science, Tehran, Iran.

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http://dx.doi.org/10.3341/kjo.2019.0035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6911787PMC
December 2019

An 18-year-old woman with pulmonary nodules found to have cytotoxic T-lymphocyte-associated antigen 4 deficiency.

BMJ Case Rep 2019 Dec 8;12(12). Epub 2019 Dec 8.

The Department of Pediatric Pulmonary Medicine, Johns Hopkins University, Baltimore, Maryland, USA.

An 18-year-old woman was admitted with abdominal pain and hematochezia. She was previously healthy until 15 years of age and was subsequently diagnosed with hypogammaglobulinemia, protein-losing enteropathy, a benign temporal lobe brain lesion/orbital fibroadenoma, autoimmune hepatitis, iron deficiency anaemia and hypothyroidism. She developed respiratory distress and hypoxemia. Read More

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http://dx.doi.org/10.1136/bcr-2019-231600DOI Listing
December 2019

Differential diagnosis of hypoalbuminemia in childhood: protein losing enteropathy associated to systemic lupus erythematosus in a young boy.

Eur J Gastroenterol Hepatol 2020 01;32(1):127-129

Pediatric Rheumatology Unit, Department of Translational Medical Sciences, Federico II University, Via Sergio Pansini n. 5, 80131, Naples, Italy.

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http://dx.doi.org/10.1097/MEG.0000000000001480DOI Listing
January 2020

Ulcerative Jejunoileitis Presenting as Protein-Losing Enteropathy: A Diagnostic and Therapeutic Dilemma.

Am J Ther 2020 May/Jun;27(3):e297-e298

Division of Gastroenterology and Hepatobiliary Diseases, New York Medical College at Westchester Medical Center, Valhalla, NY.

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http://dx.doi.org/10.1097/MJT.0000000000000827DOI Listing
December 2019
1.127 Impact Factor