2,770 results match your criteria Protein-Losing Enteropathy


Successful childbirth in a Fontan with protein losing enteropathy.

Cardiol Young 2019 Apr 10:1-2. Epub 2019 Apr 10.

Congenital Heart Center,420 NE Glen Oak Ave, Suite 301, Peoria, IL 61603,USA.

As survival rates and functional status of the adult single ventricle population have grown, some may become pregnant inadvertently or against our advice. The outcomes are often poor, being worse for the fetus/baby rather than the mother with a Fontan circuit. No reports of a successful delivery of a healthy baby to a Fontan mother with protein losing enteropathy were found in the literature. Read More

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http://dx.doi.org/10.1017/S1047951119000246DOI Listing
April 2019
2 Reads

Food hypersensitivity-induced chronic gastrointestinal inflammation in a non-human primate model of diet-induced obesity.

PLoS One 2019 4;14(4):e0214621. Epub 2019 Apr 4.

Metabolism, Diabetes and Obesity Program, Biomedicine Discovery Institute, Monash University, Victoria, Australia.

Experimental non-human primate models of obesity are induced through the introduction of atypically calorically rich diets. Studies in captive-bred macaques show the development of obesity and diabetes with similar complications to humans including eye and kidney diseases, nerve damage associated with pain and blood vessel damage. Diets differ in outcomes and here we document inflammation of the gastrointestinal tract that can be exacerbated through these dietary interventions. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0214621PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448857PMC
April 2019
5 Reads

Morphology and Function of the Lymphatic Vasculature in Patients With a Fontan Circulation.

Circ Cardiovasc Imaging 2019 Apr;12(4):e008074

Department of Cardiothoracic and Vascular Surgery, Aarhus University Hospital, Denmark (S.M., N.T., B.K., V.H.).

Background: The Fontan procedure has revolutionized the treatment of univentricular hearts. However, it is associated with severe complications such as protein-losing enteropathy, plastic bronchitis, and peripheral edema that may involve the lymphatic circulation. We aimed to assess lymphatic function and morphology in patients with a univentricular circulation. Read More

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http://dx.doi.org/10.1161/CIRCIMAGING.118.008074DOI Listing
April 2019
2 Reads

Imaging the Lymphatic System in Fontan Patients.

Circ Cardiovasc Imaging 2019 Apr;12(4):e008972

Department of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen, The Netherlands (E.T.T.L.T.).

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http://dx.doi.org/10.1161/CIRCIMAGING.119.008972DOI Listing
April 2019
1 Read

High-risk Fontan completion patients achieve low perioperative risk and benefit from cavopulmonary connection 7 years out†.

Eur J Cardiothorac Surg 2019 Apr 1. Epub 2019 Apr 1.

Department of Paediatric Cardiothoracic Surgery, Our Lady's Children's Hospital Crumlin, Dublin, Ireland.

Objectives: Our unit has pursued Fontan completion in all patients except those with immobility or combined poor ventricular function and high pulmonary artery pressures. We assessed retrospectively whether conventional high-risk criteria would predict patients with a poorer outcome.

Methods: One hundred and thirty-three consecutive children who underwent extracardiac Fontan completion (2004-2012) had their outcomes recorded (mean follow-up of 7 years). Read More

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http://dx.doi.org/10.1093/ejcts/ezz069DOI Listing
April 2019
1 Read

Intrahepatic dynamic contrast MR lymphangiography: initial experience with a new technique for the assessment of liver lymphatics.

Eur Radiol 2019 Mar 18. Epub 2019 Mar 18.

Division of Cardiology, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.

Objectives: To describe the technique and report on our initial experience with the use of intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) for evaluation of the lymphatics in patients with hepatic lymphatic flow disorders.

Methods: This is a retrospective review of the imaging and clinical findings in six consecutive patients undergoing IH-DCMRL. The technique involves injection of a gadolinium contrast agent into the intrahepatic lymphatic ducts followed by imaging of the abdomen and chest with both heavily T2-weighted imaging and dynamic time-resolved imaging. Read More

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http://dx.doi.org/10.1007/s00330-019-06112-zDOI Listing
March 2019
1 Read

[Ménétrier's disease in pediatric patients secondary to cytomegalovirus infection: Presentation of two clinical cases in a high complexity center].

Arch Argent Pediatr 2019 Apr;117(2):e158-e162

Hospital Italiano de Buenos Aires.

Ménétrier's disease is a protein losing gastroenteropathy. Defined as a rare entity with an unknown cause, most of the reported cases have been associated with viral infections. In pediatric patients, it is characterized by an acute onset with a benign and self-limiting course. Read More

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http://dx.doi.org/10.5546/aap.2019.e158DOI Listing
April 2019
2 Reads

Reversal of secondary protein-losing enteropathy after surgical revision of a jejunal Roux-en-Y loop in a patient after liver transplantation.

Am J Transplant 2019 Mar 14. Epub 2019 Mar 14.

Department of Pediatric Gastroenterology and Hepatology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Secondary protein-losing enteropathy (PLE) is a rare complication following pediatric liver transplantation (LT), mostly related to venous outflow obstruction of the liver. Here, we discuss a thus far unknown cause of secondary PLE following pediatric LT. A 7-month-old boy underwent LT with biliary anastomosis using a Roux-en-Y jejunal loop. Read More

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http://dx.doi.org/10.1111/ajt.15354DOI Listing
March 2019
1 Read

Phenotype and Genotype of a Cohort of Chinese Children with Early-Onset Protein-Losing Enteropathy.

J Pediatr 2019 Mar 7. Epub 2019 Mar 7.

Department of Gastroenterology, Children's Hospital of Fudan University, Shanghai, China.

Objectives: To examine the phenotypes and perform next-generation sequencing in children with early-onset protein-losing enteropathy.

Study Design: We performed a retrospective review of 27 children with early-onset protein-losing enteropathy. Patients were characterized on clinical, immunologic, and systemic involvements. Read More

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http://dx.doi.org/10.1016/j.jpeds.2018.12.003DOI Listing
March 2019
1 Read

The Bilateral Bidirectional Glenn Operation as a Risk Factor Prior to Fontan Completion in Complex Congenital Heart Disease Patients.

World J Pediatr Congenit Heart Surg 2019 03;10(2):174-181

1 The Edmond J. Safra International Congenital Heart Center, Sheba Medical Center, Ramat Gan, Israel.

Introduction: The Glenn procedure is generally performed as a second-stage palliative procedure toward the completion of Fontan circulation in patients with a functionally univentricular heart. Safe Fontan completion requires normal ventricular function, competent atrioventricular valves, normal pulmonary arteries anatomy, and low pulmonary vascular resistances (PVRs), which is merely an estimation that considers both lungs as a single unit. Clinical observations revealed that patients who previously underwent bilateral bidirectional cavopulmonary anastomosis (b-BCPA) had a stormier postoperative course in respect to other patients with previous unilateral BCPA (u-BCPA) postoperatively after Fontan completion. Read More

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http://dx.doi.org/10.1177/2150135118819997DOI Listing
March 2019
1 Read

Whipple disease after bariatric surgery: from malabsorption to malnutrition status.

Nutr Hosp 2019 03;36(1):238-241

Center of Excellence for the Study and Treatment of the Obesity and Diabetes (CTO).

Introduction: Malabsorptive bariatric techniques are associated with nutritional deficiencies. However, when patients do not respond to supplemental intensive treatments they should be closely followed because they can hide other pathological conditions. We present the case of a 47-year-old man with morbid obesity (body mass index [BMI]: 48 kg/m2) who underwent bariatric surgery. Read More

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http://dx.doi.org/10.20960/nh.02258DOI Listing
March 2019
1 Read

Multisite measurement of regional oxygen saturation in Fontan patients with and without protein-losing enteropathy at rest and during exercise.

Pediatr Res 2019 Feb 22. Epub 2019 Feb 22.

Department of Pediatric Cardiology, Friedrich-Alexander-University of Erlangen-Nuernberg (FAU), Loschgestraße 15, 91054, Erlangen, Germany.

Background: Protein-losing enteropathy (PLE) is a severe complication of Fontan circulation with increased risk of end-organ dysfunction. We evaluated tissue oxygenation via near-infrared spectroscopy (NIRS) at different exercise levels in Fontan patients.

Methods: Assessment of multisite NIRS during cycle ergometer exercise and daily activities in three groups: Fontan patients with PLE; without PLE; patients with dextro-transposition of the great arteries (d-TGA); comparing univentricular with biventricular circulation and Fontan with/without PLE. Read More

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http://dx.doi.org/10.1038/s41390-019-0346-3DOI Listing
February 2019

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation.

Arq Bras Cardiol 2019 Feb;112(2):130-135

Instituto do Coração (InCor) - Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP - Brazil.

Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment.

Objective: To evaluate the results of conversion from FK to TCPC. Read More

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http://dx.doi.org/10.5935/abc.20180256DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6371826PMC
February 2019
2 Reads

Association of chronic enteropathy activity index, blood urea concentration, and risk of death in dogs with protein-losing enteropathy.

J Vet Intern Med 2019 Mar 19;33(2):536-543. Epub 2019 Feb 19.

Bristol Veterinary School, University of Bristol, Bristol, United Kingdom.

Background: Malnutrition is associated with increased risk of premature death in humans with inflammatory bowel disease.

Hypothesis/objective: To determine if historical, clinical, and laboratory markers of malnutrition in dogs at the time of histologic diagnosis of protein-losing enteropathy (PLE) caused by chronic enteropathy (CE) or lymphangiectasia are associated with increased risk of death.

Animals: Seventy-one client-owned dogs diagnosed with PLE. Read More

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http://dx.doi.org/10.1111/jvim.15448DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430906PMC
March 2019
1 Read

Comparative pathophysiology and management of protein-losing enteropathy.

J Vet Intern Med 2019 Mar 14;33(2):383-402. Epub 2019 Feb 14.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota.

Protein-losing enteropathy, or PLE, is not a disease but a syndrome that develops in numerous disease states of differing etiologies and often involving the lymphatic system, such as lymphangiectasia and lymphangitis in dogs. The pathophysiology of lymphatic disease is incompletely understood, and the disease is challenging to manage. Understanding of PLE mechanisms requires knowledge of lymphatic system structure and function, which are reviewed here. Read More

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http://dx.doi.org/10.1111/jvim.15406DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430879PMC
March 2019
1 Read

A rare presentation of hypovolemic shock secondary to Whipple's disease.

Eur J Gastroenterol Hepatol 2019 05;31(5):642-645

Department of Medicine, Division of Gastroenterology, Mount Sinai Hospital, University of Toronto.

Whipple's disease is a rare, multisystem infection caused by the Gram-positive Tropheryma whippelii organism. In addition to neurological and rheumatological manifestations, this disease can result in significant gastrointestinal symptoms such as malabsorption, diarrhea, and weight loss. Given the diagnostic challenge and rare occurrence, a high index of suspicion is critical to prevent morbidity and mortality from this otherwise highly infectious disease transmitted via the fecal-oral route. Read More

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http://dx.doi.org/10.1097/MEG.0000000000001363DOI Listing
May 2019
3 Reads

Severe Protein-Losing Enteropathy Due to an Indolent Splenic Lymphoma: Case Report and Review of the Literature.

Clin Lymphoma Myeloma Leuk 2019 Jan 3. Epub 2019 Jan 3.

Department of Medicine, Division of Hematology and Medical Oncology, Icahn School of Medicine at Mount Sinai, New York, NY.

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http://dx.doi.org/10.1016/j.clml.2018.12.015DOI Listing
January 2019

Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis.

Eur J Heart Fail 2019 Jan 23. Epub 2019 Jan 23.

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.

Aims: The Fontan operation is associated with significant long-term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan palliation. Our aim was to assess haemodynamic profiles for Fontan patients and evaluate their relationships to prognosis. Read More

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http://dx.doi.org/10.1002/ejhf.1365DOI Listing
January 2019
2 Reads
6.526 Impact Factor

Ménétrier's disease presenting as recurrent unprovoked venous thrombosis: a case report.

J Med Case Rep 2019 Jan 17;13(1):14. Epub 2019 Jan 17.

University of Cincinnati College of Medicine, Cincinnati, OH, 45219, USA.

Background: Acquired thrombophilia is a potential sequela of malignancy, chronic inflammation, and conditions characterized by severe protein deficiency (for example, nephrotic syndrome, protein-losing enteropathy). As such, venous thrombosis is often a feature, and occasionally a presenting sign, of systemic disease. Ménétrier's disease is a rare hyperplastic gastropathy that may lead to gastrointestinal protein loss and hypoalbuminemia. Read More

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http://dx.doi.org/10.1186/s13256-018-1952-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335789PMC
January 2019
7 Reads

Determination of the Serum Unbound Fraction of Tadalafil in Children with Protein-Losing Enteropathy and Its Specific Binding to Human Serum Proteins in Vitro.

Biol Pharm Bull 2019 ;42(1):110-115

Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama.

The purpose of this study was to determine the serum protein binding of tadalafil in children with protein-losing enteropathy (PLE) and to evaluate the specific binding of the drug to human serum-derived proteins in vitro. Seventeen serum samples from two PLE patients used after biochemical tests were collected, and the unbound fraction of tadalafil was determined by an ultrafiltration method. The serum albumin concentrations observed in patients #1 and #2 were 2. Read More

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http://dx.doi.org/10.1248/bpb.b18-00652DOI Listing

CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease.

Authors:
Ahmet Ozen

Immunol Rev 2019 Jan;287(1):20-32

Division of Allergy and Immunology, Marmara University School of Medicine, Istanbul, Turkey.

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease was described in 1961 as an important cause of protein-losing enteropathy (PLE). PIL can be the sole finding in rare individuals or occur as part of a multisystemic genetic syndrome. Although genetic etiologies of many lymphatic dysplasia syndromes associated with PIL have been identified, the pathogenesis of isolated PIL (with no associated syndromic features) remains unknown. Read More

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http://dx.doi.org/10.1111/imr.12715DOI Listing
January 2019
3 Reads

Impact of disease process on post-transplant outcomes: Protein-losing enteropathy in Fontan patients.

J Heart Lung Transplant 2019 Jan 15;38(1):3-4. Epub 2018 Nov 15.

Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.

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http://dx.doi.org/10.1016/j.healun.2018.11.004DOI Listing
January 2019
2 Reads

Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation.

Authors:
Christine Peyton

Crit Care Nurse 2018 Dec;38(6):e5-e12

Christine Peyton is a clinical nurse specialist at the Heart Institute at Children's Hospital Colorado, Aurora, Colorado.

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Read More

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http://dx.doi.org/10.4037/ccn2018784DOI Listing
December 2018
3 Reads

Protein-losing enteropathy secondary to nonocclusive mesenteric ischemia: A case report.

Medicine (Baltimore) 2018 Nov;97(48):e13403

Rationale: Nonocclusive mesenteric ischemia (NOMI) is a life-threatening disorder; prompt diagnosis is vital. Surgical treatment is often required, but some cases can be treated conservatively. We herein report an extremely rare case wherein protein-losing enteropathy (PLE) developed after conservative treatment of NOMI. Read More

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http://dx.doi.org/10.1097/MD.0000000000013403DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6283131PMC
November 2018
2 Reads

Eculizumab Is Safe and Effective as a Long-term Treatment for Protein-losing Enteropathy Due to CD55 Deficiency.

J Pediatr Gastroenterol Nutr 2019 Mar;68(3):325-333

The Genetics Institute, Rambam Health Care Campus.

Objectives: Loss of the complement inhibitor CD55 leads to a syndrome of early-onset protein-losing enteropathy (PLE), associated with intestinal lymphangiectasia and susceptibility to large-vein thrombosis. The in vitro and short-term treatment benefits of eculizumab (C5-inhibitor) therapy for CD55-deficiency have been previously demonstrated. Here we present the 18-months treatment outcomes for 3 CD55-deficiency patients with sustained therapeutic response. Read More

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http://Insights.ovid.com/crossref?an=00005176-900000000-9663
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http://dx.doi.org/10.1097/MPG.0000000000002198DOI Listing
March 2019
5 Reads

A Rare Complication of Giardiasis in Children: Protein-losing Enteropathy.

Pediatr Infect Dis J 2018 Dec;37(12):e345-e347

From the Division of Pediatric Gastroenterology, Hepatology and Nutrition.

Protein-losing enteropathy may develop as a complication of a wide spectrum of diseases. Three cases of giardiasis that presented with acute onset of hypoalbuminemia were documented, and resolution of protein loss after treatment was also confirmed. Thus, chronic enteric infections should be considered as an etiology of severe intestinal protein loss, particularly in children. Read More

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http://dx.doi.org/10.1097/INF.0000000000002025DOI Listing
December 2018
16 Reads

Fontan-associated protein-losing enteropathy and post‒heart transplant outcomes: A multicenter study.

J Heart Lung Transplant 2019 Jan 2;38(1):17-25. Epub 2018 Oct 2.

University of California‒San Francisco Benioff Children's Hospital, San Francisco, California, USA.

Background: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality.

Methods: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10532498183168
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http://dx.doi.org/10.1016/j.healun.2018.09.024DOI Listing
January 2019
23 Reads

Myenteric ganglionitis and intestinal leiomyositis in a Jack Russell terrier.

J Small Anim Pract 2018 Nov 2. Epub 2018 Nov 2.

School of Veterinary Science, Massey University, Palmerston North, New Zealand.

A 9-year, 6-month old spayed female Jack Russell terrier presented with a 3-week history of intermittent vomiting, diarrhoea and weight loss. Serum biochemistry demonstrated severe panhypoproteinaemia, mild hypocalcaemia and mild hypocholesterolaemia, consistent with protein-losing enteropathy. Full-thickness biopsies obtained from the stomach and different sections of small intestine demonstrated histological features of both myenteric ganglionitis and early intestinal leiomyositis. Read More

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http://dx.doi.org/10.1111/jsap.12962DOI Listing
November 2018
1 Read

A man with recurrent hypovolemic shock on anti-programmed cell death protein 1 treatment: Immune-related protein-losing enteropathy.

Eur J Cancer 2018 Nov 18;104:104-107. Epub 2018 Oct 18.

Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:

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http://dx.doi.org/10.1016/j.ejca.2018.09.015DOI Listing
November 2018

Two Ventricles Are Not Better Than One in the Fontan Circulation: Equivalent Late Outcomes.

Ann Thorac Surg 2019 Mar 6;107(3):852-859. Epub 2018 Oct 6.

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

Background: A subset of patients who underwent Fontan operations has two adequate-sized ventricles, but an anatomic biventricular circulation cannot be achieved because of complex morphology or for technical reasons. This study sought to determine whether these patients with two-ventricle Fontan circulation had superior outcomes compared with those with a single ventricle.

Methods: A binational Fontan Registry of patients (n = 1,377) was analyzed to identify those patients with two adequate ventricles. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00034975183141
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http://dx.doi.org/10.1016/j.athoracsur.2018.08.024DOI Listing
March 2019
29 Reads
3.850 Impact Factor

Lymphaticovenous bypass of the thoracic duct for the treatment of chylous leak in central conducting lymphatic anomalies.

J Pediatr Surg 2019 Mar 9;54(3):562-568. Epub 2018 Sep 9.

Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115.

Background: Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.

Methods: We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00223468183056
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http://dx.doi.org/10.1016/j.jpedsurg.2018.08.056DOI Listing
March 2019
29 Reads

Successful combined intravenous and subcutaneous immunoglobulin treatment for intractable protein-losing enteropathy in a patient long after Fontan-type operation.

J Cardiol Cases 2018 Feb 16;17(2):52-55. Epub 2017 Oct 16.

Kurume University School of Medicine, Kurume City, Japan.

A 20-year-old patient, who had double outlet right ventricle, mitral atresia, pulmonary atresia, and bilateral superior vena cava and underwent successful lateral tunnel total cavo-pulmonary connection at 6 years old, presented with frequent watery diarrhea, general malaise, and tetany. He was known to have intractable protein-losing enteropathy (PLE) from 7 years of age that was resistant to various treatments. To keep hemodynamics stable, he required intravenous albumin infusion every day. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18785409173009
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http://dx.doi.org/10.1016/j.jccase.2017.09.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6149556PMC
February 2018
20 Reads

Double-balloon enteroscopy for pediatric patients: Application and feasibility evaluation in a medical center in northern Taiwan.

J Formos Med Assoc 2019 Jan 26;118(1 Pt 2):341-346. Epub 2018 Sep 26.

Department of Gastroenterology and Hepatology, Linkou Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Taiwan Association for the Study of Small Intestinal Diseases (TASSID), Taoyuan, Taiwan. Electronic address:

Background/purpose: The diagnostic and therapeutic benefits of double-balloon enteroscopy (DBE) in adults are established, but few data are available on pediatric patients. The aim of this study was to evaluate the clinical efficacy and safety of DBE in pediatric patients in Taiwan.

Methods: From April 2005 to September 2015, DBE procedures performed for diagnosis or therapy of small-bowel disease in children less than 18 years of age at Linkou Chang Gung Memorial Hospital, Taiwan were evaluated. Read More

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http://dx.doi.org/10.1016/j.jfma.2018.05.014DOI Listing
January 2019
5 Reads
1.700 Impact Factor

Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment.

Int J Cardiol 2018 Nov;271:312-316

The Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55906, United States. Electronic address:

Background: Elevation in central venous pressure (CVP) plays a fundamental pathophysiologic role in Fontan circulation. Because there is no sub-pulmonary ventricle in this system, CVP also provides the driving force for pulmonary blood flow. We hypothesized that this would make Fontan patients more susceptible to even low-level elevation in pulmonary vascular resistance index (PVRI), resulting in greater systemic venous congestion and adverse outcomes. Read More

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http://dx.doi.org/10.1016/j.ijcard.2018.05.039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6146405PMC
November 2018
4 Reads
4.040 Impact Factor

Clinical utility of currently available biomarkers in inflammatory enteropathies of dogs.

J Vet Intern Med 2018 Sep 17;32(5):1495-1508. Epub 2018 Sep 17.

Gastrointestinal Laboratory, College of Veterinary Medicine and Biomedical Sciences, Texas A&M University, College Station, TX.

Chronic inflammatory enteropathies (CIE) in dogs are a group of disorders that are characterized by chronic persistent or recurrent signs of gastrointestinal disease and histologic evidence of mucosal inflammation. These CIEs are classified as either food-responsive, antibiotic-responsive, or immunosuppressant-responsive enteropathy. Patients not clinically responding to immunomodulatory treatment are grouped as nonresponsive enteropathy and dogs with intestinal protein loss as protein-losing enteropathy. Read More

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http://doi.wiley.com/10.1111/jvim.15247
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http://dx.doi.org/10.1111/jvim.15247DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6189362PMC
September 2018
16 Reads

Atypical Clinical Presentation of Crohn's Disease with Superior Mesenteric Vein Obstruction and Protein-losing Enteropathy.

Intern Med 2019 Feb 12;58(3):369-374. Epub 2018 Sep 12.

Department of Internal Medicine, National Defense Medical College, Japan.

We herein report a 44-year-old man suffering from systemic edema due to protein-losing enteropathy (PLE) with superior mesenteric vein (SMV) obstruction and development of collateral veins, which subsequently proved to be a chronic result of thrombosis and a complication of Crohn's disease (CD). PLE was supposedly induced by both intestinal erosion and thrombosis-related lymphangiectasia, which was histologically proven in his surgically-resected ileal stenosis. Elemental diet and anti-TNFα agent improved his hypoalbuminemia after surgery. Read More

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http://dx.doi.org/10.2169/internalmedicine.1192-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395114PMC
February 2019
5 Reads

A Case Report of a Young Adult With Progressive Bloody Diarrhea, Protein-Losing Enteropathy, and Extended Polyposis Coli.

Gastroenterology 2019 01 8;156(1):e10-e11. Epub 2018 Sep 8.

Department of Gastroenterology and Hepatology, University Medical Center Groningen, The Netherlands.

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http://dx.doi.org/10.1053/j.gastro.2018.08.054DOI Listing
January 2019
1 Read

Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study.

Int J Cardiol 2019 Feb 5;276:74-80. Epub 2018 Sep 5.

Cardiology, St Luke's International Hospital, Chuo-ku, Tokyo, Japan.

Background: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear.

Purpose: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants.

Method And Results: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01675273183448
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http://dx.doi.org/10.1016/j.ijcard.2018.09.002DOI Listing
February 2019
9 Reads

Development and analytical validation of a radioimmunoassay for the quantification of alpha -proteinase inhibitor in serum and feces from the common marmoset (Callithrix jacchus).

J Med Primatol 2018 Dec 6;47(6):402-411. Epub 2018 Sep 6.

Gastrointestinal Laboratory, Department of Small Animal Clinical Sciences, College of Veterinary and Biomedical Sciences, Texas A&M University, College Station, Texas.

Background: The objective of this study was to develop and analytically validate a radioimmunoassay (RIA) for the measurement of alpha -proteinase inhibitor (α -PI) concentrations in serum and feces from the common marmoset.

Methods: Serum samples (n = 30) and 3-day fecal samples (n = 30) were obtained from healthy marmosets. An RIA was established and validated by determination of sensitivity, working range, dilutional parallelism, spiking recovery, and intra- and interassay variability. Read More

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http://dx.doi.org/10.1111/jmp.12375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234082PMC
December 2018
5 Reads

Variations in Criteria and Practices for Heart Transplantation Listing Among Pediatric Transplant Cardiologists.

Pediatr Cardiol 2019 Jan 18;40(1):101-109. Epub 2018 Aug 18.

University of California San Francisco Benioff Children's Hospitals, San Francisco, CA, USA.

Ethical issues in pediatric heart transplantation (Htx) include resource allocation, benefit, and burden assessment in high-risk recipients, and informed consent. Practice patterns and decision-making was investigated using an internet survey with 47-multiple choice items and vignette-based questions. Of 43 pediatric Htx cardiologists contacted, 28 (65%) responded. Read More

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http://dx.doi.org/10.1007/s00246-018-1965-xDOI Listing
January 2019
2 Reads

Protein-losing enteropathy in an adult with non-ischaemic cardiomyopathy: complete reversal by heart transplantation.

ESC Heart Fail 2018 10 27;5(5):842-845. Epub 2018 Jul 27.

Department of Cardiac Surgery, Institute for Clinical and Experimental Medicine-IKEM, Vídeňská 1958/9, Prague, 140 21, Czech Republic.

Protein-losing enteropathy (PLE) due to leakage of lymph into the gut sometimes occurs in young patients after Fontan palliation but is very rarely reported with other aetiologies of chronic heart failure (HF). PLE leads to severe hypoalbuminemia and immunodeficiency and is associated with poor prognosis. The mechanisms and the predispositions to PLE are poorly understood. Read More

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http://dx.doi.org/10.1002/ehf2.12342DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6165937PMC
October 2018
1 Read

Protein-losing enteropathy and joint contractures caused by a novel homozygous ANTXR2 mutation.

Adv Genomics Genet 2018 27;8:17-21. Epub 2018 Jun 27.

Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Infantile systemic hyalinosis (ISH) is a rare autosomal recessive disorder and an allelic form of hyaline fibromatosis syndrome that is caused by mutations in the gene encoding the transmembrane anthrax toxin receptor 2. Its main features include characteristic skin lesions, joint contractures, persistent diarrhea, and failure to thrive due to accumulation of hyaline material in multiple organs. The resulting severe malnutrition can cause death in early infancy. Read More

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http://dx.doi.org/10.2147/AGG.S159077DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6057141PMC
June 2018
1 Read

Partial small intestinal resection for successful surgical management of refractory protein-losing gastroenteropathy in systemic lupus erythematosus: A case report and literature review.

Medicine (Baltimore) 2018 Jul;97(30):e11357

Department of Pathology, Jichi Medical University, Tochigi, Japan.

Rationale: Although systemic lupus erythematosus (SLE) can be complicated by various gastrointestinal tract diseases, it is rarely associated with lupus enteritis and protein-losing enteropathy (PLE). We report here the successful surgical treatment of lupus enteritis and therapy-resistant and refractory PLE in a patient with SLE. We also provide a review of relevant literature. Read More

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http://dx.doi.org/10.1097/MD.0000000000011357DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078739PMC
July 2018
6 Reads

State of the art of the Fontan strategy for treatment of univentricular heart disease.

F1000Res 2018 27;7. Epub 2018 Jun 27.

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

In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. Read More

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http://dx.doi.org/10.12688/f1000research.13792.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6024235PMC
June 2018
25 Reads

A Vasculitis-Associated Neuromuscular and Vascular Hamartoma Presenting as a Fatal Form of Abdominal Cocoon.

Int J Surg Pathol 2019 Feb 11;27(1):108-115. Epub 2018 Jul 11.

1 All India Institute of Medical Sciences, Bhubaneswar, India.

Neuromuscular and vascular hamartoma (NMVH), also known as neuromesenchymal hamartoma, is a rare hamartomatous condition of the intestine. It usually presents with submucosal humps protruding in the intestinal lumen causing obstructive features. The other clinical manifestations are hematochezia or melena and protein-losing enteropathy. Read More

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http://dx.doi.org/10.1177/1066896918786582DOI Listing
February 2019
3 Reads

Stroke volume ratio derived from magnetic resonance imaging as an indicator of interventricular dyssynchrony predicts future cardiac event in patients with biventricular Fontan circulation.

Heart Vessels 2019 Jan 4;34(1):114-122. Epub 2018 Jul 4.

Department of Pediatric Cardiology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku, Tokyo, 162-8666, Japan.

The prognostic factors in patients with biventricular heart who underwent Fontan surgery remain unclear. This study wanted to assess the hypothesis that interventricular dyssynchrony evaluated by cardiac magnetic resonance imaging (MRI) can predict future cardiac events in patients with biventricular heart who have undergone Fontan surgery. We prospectively enrolled consecutive patients with biventricular Fontan circulation from 2003 to 2016, and performed protocolized cardiac MRI. Read More

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http://dx.doi.org/10.1007/s00380-018-1217-2DOI Listing
January 2019
6 Reads

Successful laparoscopic resection for cap polyposis: case report, literature review.

Surg Case Rep 2018 Jul 3;4(1):69. Epub 2018 Jul 3.

Second Department of Surgery, School of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8510, Japan.

Background: Cap polyposis is a rare gastrointestinal disease with endoscopically and pathologically distinctive features. Its exact etiology has not been fully elucidated. In a few cases, there was recurrence after inadequate treatment. Read More

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http://dx.doi.org/10.1186/s40792-018-0476-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6029993PMC
July 2018
19 Reads

Protein loosing enteropathy after Fontan procedure.

Med J Malaysia 2018 Jun;73(3):175-176

Hospital Tawau, Department of Paediatrics, Sabah, Malaysia.

Fontan and Baudet described the procedure in 1971 and was very useful to improve the quality of life in many complex cyanotic heart diseases. It has gone through various modifications since then to improve the outcome. The mortality was reported as 2. Read More

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June 2018
1 Read

"Will she live a long happy life?" Parents' concerns for their children with Fontan circulation.

Int J Cardiol Heart Vasc 2018 Mar 9;18:65-70. Epub 2018 Mar 9.

Murdoch Children's Research Institute, Melbourne, Victoria, Australia.

Background: Families of children at the worst end of the congenital heart disease endure a significant burden which is often not clearly delineated in the clinical literature. We examined the greatest concerns of parents whose children have a Fontan circulation.

Methods: Parents ( = 107) of children in the Australian and New Zealand Fontan Registry completed online surveys with open-ended and closed questions. Read More

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http://dx.doi.org/10.1016/j.ijcha.2018.02.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5988481PMC
March 2018
13 Reads