2,972 results match your criteria Protein-Losing Enteropathy

Lymphedema complicated by protein-losing enteropathy with a 22q13.3 deletion and the potential role of CELSR1: A case report.

Medicine (Baltimore) 2021 Jun;100(24):e26307

Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University.

Introduction: 22q13.3 deletion syndrome is a well-known syndrome characterized by typical clinical findings including neonatal hypotonia, absent or severely delayed speech, intellectual disability, and other various features, and detection of a heterozygous deletion of chromosome 22q13.3 with the involvement of at least part of SHANK3. Read More

View Article and Full-Text PDF

Circulating Endocannabinoids as Diagnostic Markers of Canine Chronic Enteropathies: A Pilot Study.

Front Vet Sci 2021 26;8:655311. Epub 2021 May 26.

Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.

Chronic enteropathies (CEs) in dogs, according to the treatment response to consecutive trials, are classified as food-responsive (FRE), antibiotic-responsive (ARE), and immunosuppressive-responsive (IRE) enteropathy. In addition to this classification, dogs with loss of protein across the gut are grouped as protein-losing enteropathy (PLE). At present, the diagnosis of CEs is time-consuming, costly and sometimes invasive, also because non-invasive biomarkers with high sensitivity and specificity are not yet available. Read More

View Article and Full-Text PDF

Unusual presentation of Crohn's disease.

BMJ Case Rep 2021 Jun 9;14(6). Epub 2021 Jun 9.

Gastroenterology, Nevill Hall Hospital, Abergavenny, UK.

A previously well 37-year-old woman attended the emergency assessment unit with symptoms of lethargy, breathlessness and peripheral oedema, whereby initial basic investigations revealed an iron deficiency anaemia and serum hypoalbuminaemia. The patient subsequently had multiple admissions to secondary care over a 2-year period due to worsening peripheral and central oedema. Investigations ruled out non-gastrointestinal causes of serum hypoalbuminaemia, such as renal, cardiac and hepatic failures. Read More

View Article and Full-Text PDF

Cyclophosphamide-associated enteritis presenting with severe protein-losing enteropathy in granulomatosis with polyangiitis: A case report.

World J Gastroenterol 2021 May;27(20):2657-2663

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai 9808574, Japan.

Background: Although cyclophosphamide (CPA) is the key drug for the treatment of autoimmune diseases including vasculitides, it has some well-known adverse effects, such as myelosuppression, hemorrhagic cystitis, infertility, and infection. However, CPA-associated severe enteritis is a rare adverse effect, and only one case with a lethal clinical course has been reported. Therefore, the appropriate management of patients with CPA-associated severe enteritis is unclear. Read More

View Article and Full-Text PDF

Venous Properties in a Fontan Patient with Successful Remission of Protein-Losing Enteropathy.

Int Heart J 2021 ;62(3):710-714

Department of Pediatrics and Pediatric Cardiology, Kitasato University.

We present the case of a 1-year-old boy who developed protein-losing enteropathy (PLE) within 2 months of a fenestrated Fontan procedure. His fenestration rapidly closed despite bilateral pulmonary stenosis (BPS). Subsequent to PLE onset, both fenestration and the bilateral pulmonary artery were reconstructed, and the patient's PLE had been in remission, with additive use of medications, for more than 2 years. Read More

View Article and Full-Text PDF

The Heart of the Matter: Secondary Hypogammaglobulinemia and Constrictive Pericarditis.

Pediatrics 2021 Jun;147(6)

Divisions of Rheumatology and Immunology

Constrictive pericarditis is the final common result of a number of processes that affect the pericardium. Establishing the diagnosis and determining the underlying etiology of constrictive pericarditis are often a diagnostic rendezvous. Here, we describe a patient who presented to the general practitioner with edema, ascites, and weight gain and was found to have constrictive pericarditis secondary to an inflammatory myofibroblastic tumor of the mediastinum. Read More

View Article and Full-Text PDF

Lymphangiomatosis associated with protein losing enteropathy: A case report.

World J Clin Cases 2021 May;9(15):3758-3764

Department of Gastroenterology, The Affiliated Hospital of Qingdao University, Qingdao 266003, Shandong Province, China.

Background: Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract. Lymphangiomatosis usually has no specific clinical presentation and is easily misdiagnosed. A case report and review of the literature on lymphangiomatosis associated with protein-losing enteropathy will help to improve the overall understanding of this disease. Read More

View Article and Full-Text PDF

Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation.

Pediatr Transplant 2021 May 20:e14023. Epub 2021 May 20.

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

Background: PNP is a malignancy-associated autoimmune mucocutaneous syndrome due to autoantibodies against plakins, desmogleins, and other components of the epidermis and basement membrane of epithelial tissues. PNP-causing malignancies comprise mainly lymphoproliferative and hematologic neoplasms. PNP is extremely rare, especially in children. Read More

View Article and Full-Text PDF

Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review.

Ammar A Khayat

BMC Gastroenterol 2021 May 18;21(1):225. Epub 2021 May 18.

Department of Pediatrics, Gastroenterology Unit, Department of Pediatrics, Faculty of Medicine, Umm AL Qura University, King Abdulaziz University, 24381, Al-Abdiyyah, Makkah, Saudi Arabia.

Background: Primary intestinal lymphangiectasia is an exceedingly rare disorder. Epidemiology is unknown. It usually presents with lower extremity swelling, diarrhea, ascites, and protein-losing enteropathy. Read More

View Article and Full-Text PDF

Pseudo-pseudo Meigs' syndrome (PPMS) in chronic lupus peritonitis: a case report with review of literature.

Mod Rheumatol Case Rep 2021 May 10:1-6. Epub 2021 May 10.

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

Gastrointestinal involvement in systemic lupus erythematosus (SLE) usually occurs in the form of mesenteric vasculitis, protein-losing enteropathy, intestinal pseudo-obstruction, and pancreatitis. We describe a 23-year-old female, a known case of SLE presented with significant ascites and pleural effusion. Further evaluation showed elevated CA-125 levels without evidence of malignancy. Read More

View Article and Full-Text PDF

Failing Fontan.

Indian J Thorac Cardiovasc Surg 2021 May 4;37(3):362-363. Epub 2021 Jan 4.

Children's National Heart Institute, Washington, DC USA.

Dr. O.P. Read More

View Article and Full-Text PDF

Upregulation of signal transducer and activator of transcription 3 in dogs with chronic inflammatory enteropathies.

J Vet Intern Med 2021 May 6;35(3):1288-1296. Epub 2021 May 6.

Internal Medicine, Clinic for Small Animals, Department for Companion Animals and Horses, University of Veterinary Medicine, Vienna, Austria.

Background: In inflammatory bowel disease (IBD) in humans, phosphorylated signal transducer and activator of transcription 3 (pSTAT3) is upregulated in mucosal epithelial cells and correlates with clinical severity.

Hypothesis/objective: To investigate the expression pattern of pSTAT3 in the mucosa of dogs with chronic inflammatory enteropathy (CIE) and explore correlations between its expression and clinical and histopathological severity scoring.

Animals: Twenty-eight canine CIE patients grouped into food-responsive enteropathy (FRE;  9), steroid-responsive enteropathy (SRE;  10), and protein-losing enteropathy (PLE;  9). Read More

View Article and Full-Text PDF

Sizing Up Fontan Failure: Association with Increasing Weight in Adulthood.

Pediatr Cardiol 2021 May 4. Epub 2021 May 4.

Vanderbilt Heart and Vascular Institute, Vanderbilt University Medical Center, Nashville, TN, USA.

Obesity has become increasingly recognized in adults with Fontan palliation, yet the relationship between weight changes in adulthood and Fontan failure is not clearly defined. We hypothesize that increasing weight in adulthood among Fontan patients is associated with the development of Fontan failure. Single-center data from adults with Fontan palliation who were not in Fontan failure at their first clinic visit in adulthood and who received ongoing care were retrospectively collected. Read More

View Article and Full-Text PDF

Tolvaptan treatment in an adult Fontan patient with protein-losing enteropathy: a serial Na-MRI investigation.

Ther Adv Chronic Dis 2021 16;12:20406223211004005. Epub 2021 Apr 16.

Department of Nephrology and Hypertension, Friedrich-Alexander University of Erlangen, Erlangen, Germany.

Background: Protein-losing enteropathy (PLE) is a severe complication of the univentricular Fontan circulation and associated with disturbances in salt and water homeostasis. Fontan patients with PLE have a poor prognosis, with increased morbidity and mortality. Due to limited therapeutic strategies, patients are often treated only symptomatically. Read More

View Article and Full-Text PDF

The effect of assisted enteral feeding on treatment outcome in dogs with inflammatory protein-losing enteropathy.

J Vet Intern Med 2021 May 1;35(3):1297-1305. Epub 2021 May 1.

Department of Clinical Science and Services, Royal Veterinary College, Hatfield, United Kingdom.

Background: The effect of assisted enteral feeding on treatment outcome in dogs with protein-losing enteropathy (PLE) is unknown.

Objectives: To determine if dogs with inflammatory PLE that had an enteral feeding tube placed had better outcome vs dogs with inflammatory PLE without a feeding tube.

Animals: Fifty-seven dogs with inflammatory PLE. Read More

View Article and Full-Text PDF

Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia.

Yonsei Med J 2021 May;62(5):470-473

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Primary intestinal lymphangiectasia (IL) can cause leakage of lymphatic fluids into the gastrointestinal tract, eventually leading to protein-losing enteropathy. A 15-year-old male patient, whose disease began at the age of 8 years, recently felt worsening general weakness. After diagnosing abnormal lymphatic lesions in the duodenum through endoscopy with biopsy and contrast-enhanced magnetic resonance lymphangiography, glue embolization of the leaking duodenal lymphatic channel was successfully performed. Read More

View Article and Full-Text PDF

Impact of bilateral bidirectional Glenn anastomosis on staged Fontan strategy and Fontan circulation.

Eur J Cardiothorac Surg 2021 Apr 25. Epub 2021 Apr 25.

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

Objectives: The aim of this study was to identify the impact of bilateral bidirectional Glenn (BBDG) anastomosis on staged Fontan strategy and late Fontan circulation.

Methods: Of 267 patients who underwent bidirectional Glenn prior to Fontan completion between 1989 and 2013, 62 patients (23%) who underwent BBDG were enrolled in this study. Age at operation was 0. Read More

View Article and Full-Text PDF

Protein-losing enteropathy in an infant with severe atopic dermatitis.

BMJ Case Rep 2021 Apr 22;14(4). Epub 2021 Apr 22.

Department of Pediatrics, Dokkyo Medical University, Shimotsuga, Tochigi, Japan.

Severe atopic dermatitis (AD) may lead to various complications such as hypoproteinaemia. We describe the case of a 7-month-old male infant with severe AD complicated with protein-losing enteropathy (PLE). He was diagnosed with AD at 2 months of age; however, because of familial steroid phobia, topical corticosteroids were not administered. Read More

View Article and Full-Text PDF

Growth in Children with a Fontan Circulation.

J Pediatr 2021 Apr 19. Epub 2021 Apr 19.

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

Objective: To evaluate growth in a population of patients with Fontan circulation.

Study Design: We performed a cross-sectional evaluation of patients followed in our multidisciplinary Fontan clinic from January 2011 through August 2015. We reviewed the historical data, anthropometry, clinical, and laboratory studies and performed bivariate and multivariate analysis of factors associated with height z score. Read More

View Article and Full-Text PDF

Refractory Effusions, Crumbly Bones, Mystifying Cachexia and an Absent Mind: An Unusual Presentation of Whipple's Disease with Review of Literature.

Am J Med Case Rep 2021 1;9(7):348-353. Epub 2021 Apr 1.

Department of Medicine, State University of New York, Downstate Health Sciences University, Brooklyn, NY.

Whipple's disease is a bacterial infection caused by Tropheryma whipplei and is known to cause perplexing clinical presentations, making its diagnosis challenging. The beginning by the involvement of the gastrointestinal tract, Whipple's disease can slowly progress to affect almost any organ system and lead to chronic multi-system inflammatory disease. Hereby, we present a middle age man who initially manifested with shortness of breath and chronic weight loss. Read More

View Article and Full-Text PDF

Long-Term Outcomes After Fenestration Closure in High-Risk Fontan Candidates.

Pediatr Cardiol 2021 Apr 21. Epub 2021 Apr 21.

Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 6-1 Kishibe-Shimmachi, Suita, Osaka, 564-8565, Japan.

The study aimed to assess the long-term outcomes after fenestration closure in patients at risk for Fontan failure. Of 119 patients who underwent Fontan operation between 1995 and 2004, fenestration was not created in 89 patients (NF group) and created in 30 patients with hypoplastic left heart syndrome, heterotaxy syndrome, high pulmonary arterial pressure, high systemic ventricular end-diastolic pressure, low ventricular ejection fraction, or atrioventricular valve regurgitation. All fenestrations were closed spontaneously or by catheter/surgical interventions, excepting two patients, and therefore, they were excluded. Read More

View Article and Full-Text PDF

Unusual presentation of toxoplasmosis with gastro-intestinal involvement in HLA non-identical stem cell transplantation.

Transpl Infect Dis 2021 Apr 19:e13616. Epub 2021 Apr 19.

Department of Hematology, ICANS (Institut for Cancer Strasbourg-Europe), Strasbourg, France.

Toxoplasmosis is a life-threatening infection in allogenic stem cell recipients usually involving the brain or retina and more rarely lungs or bone marrow. Digestive involvement has only been reported in AIDS patient. We report about a 38-year-old man who received a haploidentical allograft for acute myeloid leukemia and developed an unusual digestive presentation with severe protein-losing enteropathy following grade III acute digestive GvHD treated with steroids and ruxolitinib. Read More

View Article and Full-Text PDF

Prospective evaluation of a change in dietary therapy in dogs with steroid-resistant protein-losing enteropathy.

J Small Anim Pract 2021 Apr 13. Epub 2021 Apr 13.

Department of Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, 80523, USA.

Objective: To describe the clinical effect of dietary alteration as a sole change to therapy in dogs with steroid-resistant protein-losing enteropathy.

Materials And Methods: Prospective study. Eligible enrolled dogs received dietary alteration as sole change to their therapeutic plan. Read More

View Article and Full-Text PDF

Successful Treatment of Protein-Losing Enteropathy After Superior Mesenteric Artery Occlusion without Surgery.

Am J Case Rep 2021 Apr 12;22:e931114. Epub 2021 Apr 12.

Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.

BACKGROUND Protein-losing enteropathy as a complication of superior mesenteric artery occlusion is extremely rare and severe, and sometimes requires intestinal resection. However, the ideal treatment strategy has not yet been determined. CASE REPORT A 77-year-old man with underlying hypertension and diabetes was admitted to the Emergency Department with acute abdominal pain after eating. Read More

View Article and Full-Text PDF

Dynamic contrast-enhanced magnetic resonance lymphangiography.

Pediatr Radiol 2021 Apr 8. Epub 2021 Apr 8.

Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, 3401 Civic Center Blvd., Philadelphia, PA, USA.

Lymphatic flow disorders include a broad spectrum of abnormalities that can originate in the lymphatic or the venous system. The development of these disorders is multifactorial and is most commonly associated with congenital heart diseases and palliative surgeries that these patients undergo. Central lymphatic disorders might be secondary to traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema, and they can progress to perfusion anomalies. Read More

View Article and Full-Text PDF

The Consequence of Excessive Consumption of Cow's Milk: Protein-Losing Enteropathy with Anasarca in the Course of Iron Deficiency Anemia-Case Reports and a Literature Review.

Nutrients 2021 Mar 3;13(3). Epub 2021 Mar 3.

Department of Pediatrics, Allergology and Gastroenterology, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 87-100 Toruń, Poland.

Cow's milk is a key component of a child's diet. While the consumption of even trace amounts can result in allergy to its proteins and/or hypolactasia, excessive cow's milk consumption can result in numerous health complications, including iron deficiency, due to the diet being improperly balanced. Although the incidence of iron deficiency has declined, it remains the most widespread nutritional deficiency globally and the most common cause of anemia. Read More

View Article and Full-Text PDF

[CT and MRI Findings of Cortical Venous Thrombosis and Cerebral Venous Sinus Thrombosis].

Naoki Akioka

No Shinkei Geka 2021 Mar;49(2):252-261

Department of Neurosurgery, Faculty of Medicine, University of Toyama.

The author reports the cases of two young patients with cortical venous thrombosis(CVT)and cerebral venous sinus thrombosis(CVST)and demonstrates that CT and MRI investigations are critical for the diagnosis. The first case was an 18-year-old woman who developed symptoms of intracranial hypertension and, 20 days later, suffered from left hemiparesis and generalized seizures. A plain CT scan revealed an increased density of cortical veins("cord sign"). Read More

View Article and Full-Text PDF

Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients.

J Thorac Cardiovasc Surg 2021 Feb 18. Epub 2021 Feb 18.

Harvard Medical School and Boston Children's Hospital, Boston, Mass.

Background: Fontan physiology results in multiorgan dysfunction, most notably affecting the liver and kidney. We evaluated the utility of Model for End-Stage Liver Disease Excluding INR (MELD-XI) score, a score evaluating the function of both liver and kidney to identify Fontan patients at increased risk for morbidity and mortality post-heart transplant.

Methods: The Pediatric Heart Transplant Society database was queried to identify Fontan patients listed for heart transplant between January 2005 and December 2018. Read More

View Article and Full-Text PDF
February 2021

Cytomegalovirus ileitis with protein-losing enteropathy in an immunocompetent adult.

Clin J Gastroenterol 2021 Mar 20. Epub 2021 Mar 20.

Department of Gastroenterology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan.

Symptomatic cytomegalovirus (CMV) infection in immunocompetent hosts has traditionally been considered to have a benign, self-limited course, and those who need intensive therapy are rare. Moreover, there are few reports of CMV infection with protein-losing enteropathy (PLE). We present an immunocompetent 74-year-old woman with CMV ileitis with PLE, which was diagnosed due to severe hypoalbuminemia and edema of the lower extremities. Read More

View Article and Full-Text PDF

The diagnostic value of capsule endoscopy in children with intestinal lymphangiectasia.

Rev Esp Enferm Dig 2021 Mar 18. Epub 2021 Mar 18.

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

Background: Intestinal lymphangiectasia is an unusual cause of protein-losing enteropathy due to either congenital malformation or the obstruction of the intestinal lymphatics. However, few reports have investigated the use of video capsule endoscopy in children with intestinal lymphangiectasia. This study was performed to evaluate the diagnostic value of video capsule endoscopy for paediatric intestinal lymphangiectasia. Read More

View Article and Full-Text PDF