2,936 results match your criteria Protein-Losing Enteropathy


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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December 2018
1 Read

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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November 2018
1 Read

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

J Pediatr Gastroenterol Nutr 2018 Nov 8. Epub 2018 Nov 8.

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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November 2018
4 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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December 2018
8 Reads

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

J Heart Lung Transplant 2018 Oct 2. 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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October 2018
9 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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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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November 2018

Two ventricles are not better than one in the Fontan circulation: equivalent late outcomes.

Ann Thorac Surg 2018 Oct 6. 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 Fontan patients has two adequate-sized ventricles, but an anatomic biventricular circulation cannot be achieved due to complex morphology or technical reasons. We sought to determine whether these two-ventricle Fontan patients had superior outcomes compared to those with a single ventricle.

Methods: A bi-national Registry of Fontan patients (n=1377) was analysed to identify Fontan patients with two adequate ventricles. Read More

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October 2018
7 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 2018 Sep 9. 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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September 2018
8 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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February 2018
10 Reads

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

J Formos Med Assoc 2018 Sep 26. 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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September 2018
2 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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November 2018
1 Read
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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September 2018
9 Reads

Sjögren's syndrome concurrent with protein-losing gastroenteropathy with secondary systemic capillary leak syndrome : A case report.

Clin Case Rep 2018 Sep 31;6(9):1829-1833. Epub 2018 Jul 31.

Department of Nephrology Ikeda City Hospital Ikeda Osaka Japan.

Sjögren's syndrome concurrent with protein-losing gastroenteropathy can develop into secondary systemic capillary leak syndrome. Thus, it is important to diagnose the condition as soon as possible and simultaneously administer treatment for Sjögren's syndrome, protein-losing gastroenteropathy, and systemic capillary leak syndrome. Read More

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September 2018
1 Read

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

Intern Med 2018 Sep 12. 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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September 2018
2 Reads

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

Gastroenterology 2018 Sep 8. Epub 2018 Sep 8.

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

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September 2018
1 Read

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

Int J Cardiol 2018 Sep 5. 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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September 2018
5 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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December 2018
1 Read

Immunogenicity and Immune Complex Disease in Preclinical Safety Studies.

Authors:
John L Vahle

Toxicol Pathol 2018 Aug 29:192623318797070. Epub 2018 Aug 29.

1 Lilly Research Laboratories, Indianapolis, Indiana, USA.

This article summarizes a continuing education presentation on immunogenicity that was part of a continuing education course entitled, "Clinical Pathology of Biotherapeutics." Immunogenicity of a biotherapeutic can have diverse impacts including altered systemic exposure and pharmacologic responses and, in a fraction of the cases, safety concerns including cross-reactive neutralization of endogenous proteins or sequela related to immune complex disease (ICD). In most cases, immune complexes are readily cleared from circulation; however, based on physiochemical properties, insoluble complexes form, activate complement, and deposit in tissues. Read More

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August 2018
12 Reads

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

Pediatr Cardiol 2018 Aug 18. 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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August 2018
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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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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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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July 2018
2 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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June 2018
15 Reads

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

Int J Surg Pathol 2018 Jul 1:1066896918786582. Epub 2018 Jul 1.

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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July 2018
2 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 2018 Jul 4. 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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July 2018
5 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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July 2018
9 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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March 2018
12 Reads

Establishing the role of in protein-losing enteropathy: a homozygous missense variant leads to an attenuated phenotype.

J Med Genet 2018 Nov 6;55(11):779-784. Epub 2018 Jun 6.

The Genetics Institute, Rambam Health Care Campus, Haifa, Israel.

Background: Intestinal integrity is essential for proper nutrient absorption and tissue homeostasis, with damage leading to enteric protein loss, that is, protein-losing enteropathy (PLE). Recently, homozygous nonsense variants in the plasmalemma vesicle-associated protein gene () were reported in two patients with severe congenital PLE. PLVAP is the building block of endothelial cell (EC) fenestral diaphragms; its importance in barrier function is supported by mouse models of Plvap deficiency. Read More

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November 2018
3 Reads

Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation.

Int Heart J 2018 Jul 23;59(4):873-876. Epub 2018 May 23.

Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital.

We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Read More

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July 2018
3 Reads

Comparison of Clinical Profiles in Patients with Protein-Losing Enteropathy With and Without Fontan Circulation.

Pediatr Cardiol 2018 Oct 22;39(7):1290-1298. Epub 2018 May 22.

Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.

Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. Read More

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October 2018
1 Read

Small Intestinal Lymphatic Hypoplasia in Three Dogs with Clinical Signs of Protein-losing Enteropathy.

J Comp Pathol 2018 Apr 4;160:39-49. Epub 2018 Apr 4.

Department of Biomedical Sciences, New York Animal Health Diagnostic Center, USA. Electronic address:

Intestinal lymphatic hypoplasia (ILH) is a rare but well-documented cause of protein-losing enteropathy (PLE) in human infants. To our knowledge, this condition has not been reported previously in veterinary medicine. Here we report the clinical and histopathological findings in three dogs that presented with clinical signs of PLE. Read More

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A new surgical strategy for medically uncontrolled protein-losing enteropathy after Fontan surgery.

Interact Cardiovasc Thorac Surg 2018 Oct;27(4):629-631

Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, South Korea.

Managing protein-losing enteropathy after Fontan surgery is challenging. We report a case of protein-losing enteropathy associated with Fontan circulation in a 14-year-old boy. He was treated medically for 2 years, without improvement until Fontan takedown and pulsatile bidirectional cavopulmonary shunting were performed. Read More

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October 2018
2 Reads

Mutations in plasmalemma vesicle-associated protein cause severe syndromic protein-losing enteropathy.

J Med Genet 2018 Sep 16;55(9):637-640. Epub 2018 Apr 16.

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

Background: Protein-losing enteropathy (PLE) is characterised by gastrointestinal protein leakage due to loss of mucosal integrity or lymphatic abnormalities. PLE can manifest as congenital diarrhoea and should be differentiated from other congenital diarrhoeal disorders. Primary PLEs are genetically heterogeneous and the underlying genetic defects are currently emerging. Read More

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September 2018
4 Reads

Molecular diversity of the faecal microbiota of Toy Poodles in Japan.

J Vet Med Sci 2018 May 11;80(5):749-754. Epub 2018 Apr 11.

Research and Education Center for Prevention of Global Infectious Diseases of Animals, Faculty of Agriculture, Tokyo University of Agriculture and Technology, Fuchu-shi, Tokyo 183-8509, Japan.

The intestinal microbiota was revealed with the recent advances in molecular techniques, such as high-throughput sequencing analysis. As a result, the microbial changes are thought to influence the health of humans and animals and such changes are affected by several factors including diet, genetics, age, sex, and diseases. Similar studies are being conducted in dogs, and the knowledge of intestinal microbiota in dogs is expanding. Read More

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May 2018
3 Reads

Abdominal imaging findings in adult patients with Fontan circulation.

Insights Imaging 2018 Jun 5;9(3):357-367. Epub 2018 Apr 5.

Department of Medical Imaging, Toronto General Hospital, 585 University Avenue, Toronto, ON, M5G 2N2, Canada.

The Fontan procedures, designed to treat paediatric patients with functional single ventricles, have markedly improved the patient's survival into adulthood. The physiology of the Fontan circuit inevitably increases systemic venous pressure, which may lead to multi-system organ failure in the long-term follow-up. Fontan-associated liver disease (FALD) can progress to liver cirrhosis with signs of portal hypertension. Read More

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June 2018
13 Reads

Intestinal Failure and Aberrant Lipid Metabolism in Patients With DGAT1 Deficiency.

Gastroenterology 2018 07 29;155(1):130-143.e15. Epub 2018 Mar 29.

Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria; CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria; Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria; St. Anna Kinderspital and Children's Cancer Research Institute, Department of Pediatrics, Medical University of Vienna, Vienna, Austria. Electronic address:

Background & Aims: Congenital diarrheal disorders are rare inherited intestinal disorders characterized by intractable, sometimes life-threatening, diarrhea and nutrient malabsorption; some have been associated with mutations in diacylglycerol-acyltransferase 1 (DGAT1), which catalyzes formation of triacylglycerol from diacylglycerol and acyl-CoA. We investigated the mechanisms by which DGAT1 deficiency contributes to intestinal failure using patient-derived organoids.

Methods: We collected blood samples from 10 patients, from 6 unrelated pedigrees, who presented with early-onset severe diarrhea and/or vomiting, hypoalbuminemia, and/or (fatal) protein-losing enteropathy with intestinal failure; we performed next-generation sequencing analysis of DNA from 8 patients. Read More

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July 2018
26 Reads
1 Citation
16.720 Impact Factor

Alterations in serum amino acid concentrations in dogs with protein-losing enteropathy.

J Vet Intern Med 2018 May 31;32(3):1026-1032. Epub 2018 Mar 31.

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

Background: Certain amino acids are decreased in humans with inflammatory bowel disease (IBD) and supplementation with the same amino acids has shown beneficial effects in animal models of IBD. Currently, the amino acid status of dogs with protein-losing enteropathy (PLE) is unknown.

Hypothesis/objective: To determine if serum amino acid concentrations are abnormal in dogs with PLE and correlated with clinical and laboratory variables and outcome. Read More

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May 2018
3 Reads

A Rare Complıcatıon of Gıardıasıs in Chıldren: Proteın Losıng Enteropathy.

Pediatr Infect Dis J 2018 Mar 19. Epub 2018 Mar 19.

Div. of Pediatric Gastroenterology, Hepatology and Nutrition, Marmara University School of Medicine.

Protein-losing enteropathy (PLE) 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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March 2018
4 Reads

Protein-Amino Acid Metabolism Disarrangements: The Hidden Enemy of Chronic Age-Related Conditions.

Nutrients 2018 Mar 22;10(4). Epub 2018 Mar 22.

Department of Clinical Sciences and Community Health, University of Milan, 20122 Milan, Italy.

Proteins are macro-molecules crucial for cell life, which are made up of amino acids (AAs). In healthy people, protein synthesis and degradation are well balanced. However, in the presence of hypercatabolic stimulation (i. Read More

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March 2018
1 Read

Renal dialysis and long-term treatment of a dog with kidney disease associated with canine leishmaniosis.

Parasit Vectors 2018 03 20;11(1):151. Epub 2018 Mar 20.

Koret School of Veterinary Medicine, The Hebrew University of Jerusalem, Rehovot, Israel.

Background: Renal disease is considered the main cause of natural mortality in dogs with canine leishmaniosis. The pathological mechanisms associated with kidney injury in canine leishmaniosis include immune complex glomerulonephritis, tubulointerstitial nephritis and occasionally renal amyloidosis. Proteinuria is a frequent finding in canine leishmaniosis and its quantification by the urine protein-creatinine ratio (UPC) is an important parameter in the staging of canine lesihmaniosis as presented by the LeishVet group. Read More

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March 2018
7 Reads

Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis.

Heart 2018 Sep 13;104(18):1508-1514. Epub 2018 Mar 13.

Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, USA.

Objective: More patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.

Methods: PubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. Read More

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September 2018
1 Read

Protein-losing enteropathy may be an important characteristic manifestation in Sjögren's syndrome.

Mod Rheumatol 2018 Apr 11:1-3. Epub 2018 Apr 11.

e Section of Clinical Pathology , Saiseikai Kumamoto Hospital , Kumamoto , Japan.

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April 2018
5 Reads

Protein-Losing Enteropathy in the Setting of Severe Iron Deficiency Anemia.

J Investig Med High Impact Case Rep 2018 Jan-Dec;6:2324709618760078. Epub 2018 Feb 26.

Boston Children's Hospital, Boston, MA, USA.

A 20-month-old boy presented with a 2-week history of pallor and progressive abdominal distention. Nutritional history revealed long-standing excessive cow milk intake. He was subsequently found to be profoundly iron deficient and hypoproteinemic, with an elevated fecal α-1-antitrypsin level and occult blood positive stool, consistent with protein-losing enteropathy. Read More

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February 2018
5 Reads

Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD.

Medicine (Baltimore) 2018 Jan;97(3):e9649

Department of Pathology, William Beaumont Hospital.

Rationale: Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients < 3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation. Read More

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January 2018
12 Reads

Beyond the Role of CD55 as a Complement Component.

Immune Netw 2018 Feb 20;18(1):e11. Epub 2018 Feb 20.

Severance Biomedical Science Institute and BK21 PLUS Project to Medical Sciences, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06230, Korea.

The complement is a part of the immune system that plays several roles in removing pathogens. Despite the importance of the complement system, the exact role of each component has been overlooked because the complement system was thought to be a nonspecific humoral immune mechanism that worked against pathogens. Decay-accelerating factor (DAF or CD55) is a known inhibitor of the complement system and has recently attracted substantial attention due to its role in various diseases, such as cancer, protein-losing enteropathy, and malaria. Read More

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February 2018
4 Reads

Concurrent renal amyloidosis and thymoma resulting in a fatal ventricular thrombus in a dog.

J Vet Intern Med 2018 May 27;32(3):1160-1165. Epub 2018 Feb 27.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, Iowa State University, Ames, Iowa.

Thymoma-associated nephropathies have been reported in people but not in dogs. In this report, we describe a dog with thymoma and concurrent renal amyloidosis. A 7-year-old castrated male Weimaraner was presented for progressive anorexia, lethargy, and tachypnea. Read More

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May 2018
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Life After Surviving Fontan Surgery: A Meta-Analysis of the Incidence and Predictors of Late Death.

Authors:
C L Poh Y d'Udekem

Heart Lung Circ 2018 May 20;27(5):552-559. Epub 2017 Dec 20.

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

Aim: We now know that 20-40% of patients with a single ventricle will develop heart failure after the second decade post-Fontan surgery. However, we remain unable to risk-stratify the cohort to identify patients at highest risk of late failure and death. We conducted a systematic review of all reported late outcomes for patients with a Fontan circulation to identify predictors of late death. Read More

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