928 results match your criteria Intestinal Lymphangiectasia


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

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Refractory primary intestinal lymphangiectasia effectively managed with subcutaneous octreotide.

BMJ Case Rep 2021 Apr 9;14(4). Epub 2021 Apr 9.

General Surgery, Highgate Private Hospital, London, Highgate, UK.

This case report describes a young man with a history of lymphoedema and long-standing gastrointestinal symptoms since childhood. After undergoing extensive investigations, he was diagnosed with primary intestinal lymphangiectasia (IL). The patient's condition was refractory to conventional medium-chain triglyceride diet but responded well to treatment with subcutaneous octreotide. Read More

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Lymphatic dysfunction in advanced cirrhosis: Contextual perspective and clinical implications.

World J Hepatol 2021 Mar;13(3):300-314

Department of Radiodiagnosis, All India Institute of Medical Sciences, Patna 801507, Bihar, India.

The lymphatic system plays a very important role in body fluid homeostasis, adaptive immunity, and the transportation of lipid and waste products. In patients with liver cirrhosis, capillary filtration markedly increases, primarily due to a rise in hydrostatic pressure, leading to enhanced production of lymph. Initially, lymphatic vasculature expansion helps to prevent fluid from accumulating by returning it back to the systemic circulation. Read More

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[Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome].

Arch Argent Pediatr 2021 04;119(2):e138-e141

Servicio de Anatomía Patológica, Hospital Clínico Universitario Lozano Blesa. Zaragoza, España.

Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with mediumchain triglyceride, our patient presents clinical improvement until today. Read More

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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

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Long-term endoscopic findings in pediatric primary intestinal lymphangiectasia.

Clin Case Rep 2021 Feb 5;9(2):1029-1030. Epub 2020 Dec 5.

Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine Saga University Saga Japan.

This is the first case report comparing endoscopic images of primary intestinal lymphangiectasia (PIL) over 10 years. Regular endoscopic examination is essential in PIL because endoscopic findings do not correlate well with clinical manifestations. Read More

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February 2021

Intestinal Lymphangiectasia Associated With Refractory Ascites in a Cirrhosis Patient.

Cureus 2021 Jan 7;13(1):e12567. Epub 2021 Jan 7.

Radiology, All India Institute of Medical Sciences Patna, Patna, IND.

Lymphatic systems play a very important role in the body fluid homeostasis by interstitial fluid reabsorption. Lymphatic dysfunctions are common in patients with advanced cirrhosis, contributing to ascites and lymphedema. An unusual manifestation of lymphatic dysfunction in patients with cirrhosis is intestinal lymphangiectasia. Read More

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January 2021

Capsule Retention Caused by Cryptogenic Multifocal Ulcerous Stenosing Enteritis.

Rev Esp Enferm Dig 2021 Feb 10. Epub 2021 Feb 10.

Gastroenterology, Beijing Shijitan Hospital. Capital Medical University.

Introduction: Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare illness, and capsule retention (CR) has been reported in a few cases of CMUSE.

Methods: We present four cases of CMUSE with CR. None of the patients showed any symptoms or signs of small bowel obstruction before a capsule endoscopy (CE) and denied a history of any non-steroidal anti-inflammatory drugs intake, radiotherapy treatment, or abdominal surgery. Read More

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February 2021

Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature.

World J Gastroenterol 2020 Dec 8;26(48):7707-7718. Epub 2020 Dec 8.

Department of Internal Medicine, General Hospital Oberndorf, Teaching Hospital of the Paracelsus Medical University Salzburg, Oberndorf 5110, Austria.

Background: Primary intestinal lymphangiectasia (PIL), first described in 1961, is a rare disorder of unknown etiology resulting in protein-losing enteropathy. The disease is characterized by dilatation and leakage of intestinal lymph vessels leading to hypoalbuminemia, hypogammaglobulinemia, and lymphopenia. Since the severity and location of lymph vessels being affected can vary considerably, the range of associated symptoms is wide from mild lower-limb edema to generalized edema, abdominal and/or pleural effusion, and recurrent diarrhea, among others. Read More

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December 2020

Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature.

BMC Pediatr 2021 Jan 7;21(1):21. Epub 2021 Jan 7.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.

Background: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. Read More

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January 2021

A case of "misplaced" Tritrichomonas foetus infection in a dog in Northern Italy.

Vet Parasitol Reg Stud Reports 2020 12 19;22:100451. Epub 2020 Aug 19.

Città di Pavia Veterinary Hospital, 179 Cremona road, 27100 Pavia, Italy. Electronic address:

An 8-year-old, spayed female Rottweiler dog, under immunosuppressant treatment for protein-losing enteropathy (PLE) and intestinal lymphangiectasia, was presented for anorexia, poor general conditions and episodes of diarrhea. A subcutaneous mass between the caudal abdominal mammary glands was found. A fine-needle aspiration cytology was performed and revealed the presence of inflammatory cells mixed with pear- or round-shaped microorganisms with cytomorphological features of flagellated protozoan trophozoites, belonging to Trichomonadida order. Read More

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December 2020

Enhanced Collagen Deposition in the Duodenum of Patients with Hyaline Fibromatosis Syndrome and Protein Losing Enteropathy.

Int J Mol Sci 2020 Nov 2;21(21). Epub 2020 Nov 2.

Pediatric Gastroenterology Unit, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Ramat Gan 5262100, Israel.

Hyaline fibromatosis syndrome (HFS), resulting from mutations, is an ultra-rare disease that causes intestinal lymphangiectasia and protein-losing enteropathy (PLE). The mechanisms leading to the gastrointestinal phenotype in these patients are not well defined. We present two patients with congenital diarrhea, severe PLE and unique clinical features resulting from deleterious mutations. Read More

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November 2020

Dietary and Nutritional Approaches to the Management of Chronic Enteropathy in Dogs and Cats.

Authors:
Aarti Kathrani

Vet Clin North Am Small Anim Pract 2021 Jan 29;51(1):123-136. Epub 2020 Oct 29.

Royal Veterinary College, Hawkshead Lane, Hatfield, Hertfordshire, AL9 7TA, UK. Electronic address:

Nutrition can influence those functions of the gastrointestinal tract that can be adversely affected in chronic enteropathy, such as microbiota, mucosal immune system, intestinal permeability, and motility. Diet serves as a possible risk factor in disease pathogenesis and as a target for treatment in chronic enteropathy. Malnutrition is prevalent in people with inflammatory bowel disease and negatively affects outcome. Read More

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January 2021

Successful Diagnosis and Treatment of Protein-Losing Enteropathy Using Liver Lymphangiography and Embolization of Hepatoduodenal Lymphatic Communications.

J Vasc Interv Radiol 2020 11 7;31(11):1924-1926. Epub 2020 Oct 7.

Interventional Radiology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104.

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November 2020

A pilot study on the effect of fat loading on the gastrointestinal tract of healthy dogs.

J Small Anim Pract 2020 Dec 9;61(12):732-737. Epub 2020 Oct 9.

Idexx Diagnostics, Davis, CA, 95616, USA.

Objective: To assess the effect of a high fat meal (fat loading) on gastrointestinal motility and the appearance of intestinal villi using video capsule endoscopy and ultrasound.

Materials And Methods: Four healthy staff-owned dogs were included in a prospective blinded crossover study. Dogs had initial baseline video capsule endoscopy to measure gastrointestinal transit times and allow for visual assessment of intestinal mucosa. Read More

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December 2020

Primary Intestinal Lymphangiectasia: Diagnostic Accuracy of 99mTc-Labeled Human Serum Albumin Nanocolloid SPECT/CT Before Biopsy.

Clin Nucl Med 2021 Jan;46(1):e34-e35

Department of Gastroenterology and Pancreatology, CHU-Rangueil, University Hospital of Toulouse, Toulouse, France.

Primary intestinal lymphangiectasia is an unusual cause of protein losing enteropathy due to either congenital malformation or obstruction of lymphatics of intestine. The disease can affect all or only a small part of the small intestine. Peripheral lymphedema may be associated. Read More

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January 2021

Overlap of lymphatic dysplasia in Fontan-associated protein-losing enteropathy and Mucosa-Associated Lymphoid Tissue (MALT lymphoma): implications for management of protein-losing enteropathy.

Cardiol Young 2020 Dec 5;30(12):1973-1975. Epub 2020 Oct 5.

Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA48109.

Lymphatic vessel dysplasia is associated with Fontan-associated protein-losing enteropathy. Extra nodal non-Hodgkin lymphomas including mucosa-associated lymphoid tissue (MALT lymphoma) are associated with lymphatic vessel dysplasia. Here, we describe the case of a 7-year-old with Fontan-associated protein-losing enteropathy who developed MALT lymphoma with a clinical course indicative of interaction between these pathologies and improvement in protein-losing enteropathy after MALT lymphoma treatment. Read More

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December 2020

Primary intestinal lymphangiectasia diagnosed by video capsule endoscopy in a patient with immunodeficiency presenting with bacteraemia.

BMJ Case Rep 2020 Sep 13;13(9). Epub 2020 Sep 13.

Division of Gastroenterology and Hepatology, Denver Health and Hospital Authority, Denver, Colorado, USA.

A 24-year-old woman with a medical history of chronic lower extremity oedema, abdominal pain, diarrhoea and recurrent pulmonary infections presented with sepsis from right lower extremity cellulitis. Blood cultures grew Laboratory evaluation revealed lymphopaenia, hypogammaglobulinaemia, a low CD4+ T-cell count and nutritional deficiencies resulting from protein-losing enteropathy (PLE). CT showed small bowel wall thickening in the jejunum and ileum. Read More

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September 2020

A case of Heck's disease with primary intestinal lymphangiectasia treated with imiquimod.

Indian J Dermatol Venereol Leprol 2020 Nov-Dec;86(6):724-725

Department of Dermatology and Venerology, School of Medicine, Akdeniz University, Antalya, Turkey.

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Severe chronic bowel obstruction associated with brown bowel syndrome.

SAGE Open Med Case Rep 2020 22;8:2050313X20945531. Epub 2020 Jul 22.

Department of Pathology, Loma Linda University Medical Center, Loma Linda, CA, USA.

A 61-year-old alcoholic male with history of cholecystectomy presented with a 20-year history of recurrent bowel obstruction and a 30 lb weight loss. After numerous attempts at conservative management, exploratory laparotomy was performed, which showed no mechanical cause. Despite no clear etiology, the obstruction persisted and intensified. Read More

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Protein-Losing Enteropathy in Primary Lymphangiectasia.

J Belg Soc Radiol 2020 Jun 29;104(1):34. Epub 2020 Jun 29.

Cliniques Universitaires Saint-Luc, BE.

Intestinal lymphangectasia should be evoked in the rare context of protein-losing enteropathy with low-attenuation thickening of the bowel wall. Read More

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

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

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

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

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

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

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

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

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

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[Variant analysis of CCBE1 gene in a case of Hennekam lymphangiectasia-lymphedema syndrome type 1].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2020 Jun;37(6):669-672

Cheeloo College of Medicine, Shandong University, Jinan, Shandong 250012, China.

Objective: To explore the genetic etiology of a child with lymphangiectasia and lymphedema.

Methods: DNA sample of the patient was extracted and subjected to whole exome sequencing. Suspected variants were verified by Sanger sequencing. Read More

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Magnetic Resonance Lymphangiography.

Radiol Clin North Am 2020 Jul 6;58(4):693-706. Epub 2020 May 6.

Department of Diagnostic Imaging, The Hospital for Sick Children and Medical Imaging, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada; Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.

Dynamic contrast-enhanced magnetic resonance lymphangiography is a novel technique to image central conducting lymphatics. It is performed by injecting contrast into groin lymph nodes and following passage of contrast through lymphatic system using T1-weighted MR images. Currently, it has been successfully applied to image and plan treatment of thoracic duct pathologies, lymphatic leaks, and other lymphatic abnormalities such as plastic bronchitis. Read More

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Primary intestinal lymphangiectasia in children: A review.

J Paediatr Child Health 2020 Nov 28;56(11):1719-1723. Epub 2020 May 28.

Department of Paediatrics, University of Otago, Christchurch, New Zealand.

Primary intestinal lymphangiectasia is an uncommon condition that usually presents early in childhood. This incurable condition is consequent to underlying lymphatic abnormalities that lead to loss of lymphatic contents into the intestinal lumen. This article outlines an approach to the assessment of children presenting with characteristic features and consideration of other conditions that could lead to enteric protein loss. Read More

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November 2020

Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia.

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

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

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

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Updates on bone health in children with gastrointestinal diseases.

Authors:
Hye Ran Yang

Ann Pediatr Endocrinol Metab 2020 Mar 31;25(1):10-14. Epub 2020 Mar 31.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Chronic gastrointestinal diseases such as inflammatory bowel disease, malabsorption syndromes (e.g., intestinal lymphangiectasia, celiac disease, congenital chloride diarrhea, cystic fibrosis), and postsubtotal gastrectomy state or short-bowel syndrome after extensive bowel resection are related to poor bone health in pediatric patients due to increased risks of low bone mineral density, osteoporosis, and fractures. Read More

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