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Intestinal lymphangiectasia in children

Ziqing Ye, Ying Huang, Yuhuan Wang, Junping Lu, Jie Wu, Zhuowen Yu
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. Targeted gene panel sequencing and whole-exome sequencing were performed in 9 patients. RESULTS: In 27 patients (55.6% male), median age of disease onset was 173 days, and 59...
March 7, 2019: Journal of Pediatrics
Suyun Li, Xiaoqian Liu, Yuan He, Qianyu Li, Linlin Ji, Wenbin Shen, Guansheng Tong
RATIONALE: Intestinal lymphangiectasia (IL) is a rare enteropathy involving the expansion and rupture of intestinal lymphatic channels. Although several reports have studied cases of primary IL (PIL), this condition is very rare, and is even less commonly encountered in infants. This study aimed to investigate the nutritional therapy and effect assessment of chylous reflux disorder caused by PIL in infants. PATIENT CONCERNS: Infantile patients were enrolled in the Affiliated Beijing Shijitan Hospital of the Capital Medical University between January 2012 and March 2014...
December 2017: Medicine (Baltimore)
S Guo, L Song, D X Guan, T L Mei, J Zhou, F H Yu, G L Wang, J Zhang, H Q Shen, X W Xu
Objective: To analyze the clinical manifestations, diagnosis, treatment and prognosis of intestinal lymphangiectasia (IL) in children in order to improve the skills of diagnosis and treatment of IL. Method: Clinical manifestations, laboratory findings, gastroscopic findings, histopathological examinations and lymphatic radionuclide imaging assessments were analyzed retrospectively among 47 IL patients who were hospitalized in the Gastroenterology Department of Beijing Children's Hospital Affiliated to Capital Medical University from June 2007 to December 2015...
December 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Hasan M Isa, Ghadeer G Al-Arayedh, Afaf M Mohamed
Intestinal lymphangiectasia (IL) is a rare disease characterized by dilatation of intestinal lymphatics. It can be classified as primary or secondary according to the underlying etiology. The clinical presentations of IL are pitting edema, chylous ascites, pleural effusion, acute appendicitis, diarrhea, lymphocytopenia, malabsorption, and intestinal obstruction. The diagnosis is made by intestinal endoscopy and biopsies. Dietary modification is the mainstay in the management of IL with a variable response. Here we report 2 patients with IL in Bahrain who showed positive response to dietary modification...
February 2016: Saudi Medical Journal
Margaret E Lawless, Kelly A Lloyd, Paul E Swanson, Melissa P Upton, Matthew M Yeh
OBJECTIVES: Lymphangiomatous lesions involving the gastrointestinal (GI) tract remain incompletely characterized, and their clinical and histopathologic features have not been systematically evaluated. The distinction between a primary lymphatic malformation (lymphangioma) and a dilation of existing lymphatics (lymphangiectasia) is of clinical significance, since lymphangiectasia may occur in the setting of lymphatic obstruction due to an unsampled malignancy. We describe clinical and morphologic features of lymphangiomas of the GI tract in adult and pediatric populations and contrast them with lymphangiectasia...
October 2015: American Journal of Clinical Pathology
D A Morozov, E S Pimenova, V K Tatochenko, M D Bakradze, D D Gadliya, O L Morozova, A G Talalaev
The rare combination of intestinal lymphangiectasia with malrotation of the duodenum in a child of three months of life is described. Basing on the literature review only 3 similar cases were described in the world practice. The boy with protein-losing enteropathy was examined at Moscow Scientific Centre of Children's Health. The child had vomiting, diarrhea, loss in body weight, hypoproteinemia, lymphopenia. The infectious nature of the disease was excluded. It had been suggested the Waldman desease (primary intestinal lymphangiectasia)...
2015: Vestnik Rossiĭskoĭ Akademii Meditsinskikh Nauk
A A Shavrov, A Iu Kharitonova, A A Shavrov junior, N A Kalashnikova, A G Talalaev, S G Khomeriki
BACKGROUND: Aim of the study was to assess the diagnostic yield of second-generation colon capsule in pediatric gastrointestinal diseases. PATIENTS AND METHODS: Five patients with different symptoms of gastrointestinal diseases were included in the study. Among them were: suspicion on diffuse polyposis, intestinal bleeding, lymphangiectasia and inflammatory bowel disease. Image interpretation was made by experienced capsule users, previously trained on small bowel capsule and first generation colon capsule...
2014: Vestnik Rossiĭskoĭ Akademii Meditsinskikh Nauk
Sachin B Ingle, Chitra R Hinge Ingle
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc...
October 16, 2014: World Journal of Clinical Cases
Ladonna J Malone, Laura Z Fenton, Jason P Weinman, Miran R Anagnost, Lorna P Browne
BACKGROUND: Lymphangiectasia is a rarely encountered lymphatic dysplasia characterized by lymphatic dilation without proliferation. Although it can occur anywhere, the most common locations are the central conducting lymphatics and the pulmonary and intestinal lymphatic networks. Recent advances in lymphatic interventions have resulted in an increased reliance on imaging to characterize patterns of disease. OBJECTIVE: To describe the patient populations, underlying conditions, and imaging features of lymphangiectasia encountered at a tertiary pediatric institution over a 10-year period and correlate these with pathology and patient outcomes...
April 2015: Pediatric Radiology
D Valdovinos-Oregón, J Ramírez-Mayans, R Cervantes-Bustamante, E Toro-Monjaraz, M Cázares-Méndez, J Cadena-León, F Zárate-Mondragón, E Montijo-Barrios
BACKGROUND: Primary intestinal lymphangiectasia is a rare congenital disease described by Waldmann in 1961 that is a consequence of obstruction of the lymphatic drainage of the small bowel with secondary lymph vessel dilation. This distorts the architecture of the villi and causes a leakage of lymph into the intestinal lumen, resulting in protein-losing enteropathy and malabsorption. AIM: To describe the clinical, biochemical, radiologic, endoscopic, and histologic characteristics in children with primary intestinal lymphangiectasia...
January 2014: Revista de Gastroenterología de México
N Urganci, S G Gulec, D Kalyoncu, S Karaman
OBJECTIVE: The aim of the study is to evaluate paediatric patients with protein losing enteropathy (PLE). METHODS: Fourteen cases diagnosed as PLE were evaluated in terms ofaetiologies, diagnostic methods, laboratory findings, treatment procedures and long-term prognosis. RESULTS: Four of the cases had coeliac disease, three intestinal lymphangiectasia, three giardia infection, one H pylori infection and three cytomegalovirus (CMV) infection...
March 2013: West Indian Medical Journal
Giselle de Melo Braga, Gabriel Hessel, Ricardo Mendes Pereira
The liver is one of the organs most affected by paracoccidioidomycosis, a systemic mycosis endemic in some Latin American countries. The majority of articles focused on adult populations and failed to describe any detailed experience of liver abnormalities in pediatric patients. Therefore, the aim of this study was to describe the frequency and characteristics of liver involvement in children with paracoccidioidomycosis. This study comprised 102 patients less than 16 years of age (median 104.3 months) diagnosed with paracoccidioidomycosis from 1980 to 2010...
October 2013: Mycopathologia
Siham Al Sinani, Yusria Al Rawahi, Hamed Abdoon
A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL...
November 21, 2012: World Journal of Gastroenterology: WJG
Aglaia Zellos, Diagoras Zarganis, Stelios Ypsiladis, Dimitris Chatzis, Georgia Papaioannou, Christos Bartsocas
Protein-losing enteropathy in children is caused by intestinal metabolic, inflammatory, or infectious processes, or by lymphatic obstruction (intestinal lymphangiectasia). In this report, a 17-month-old child is presented with protein-losing enteropathy due to intestinal malrotation and chronic midgut volvulus causing lymphatic obstruction and spillage of lymph in the intestine and the peritoneum. This report should alert the pediatrician that intestinal malrotation should be added to the wide list of possible causes of protein-losing enteropathy in children...
February 2012: Pediatrics
Jing Guo, Mei Sun
No abstract text is available yet for this article.
May 2011: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Hugh James Freeman, Michael Nimmo
Intestinal lymphangiectasia in the adult may be characterized as a disorder with dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine and resultant hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and reduced number of circulating lymphocytes or lymphopenia. Most often, intestinal lymphangiectasia has been recorded in children, often in neonates, usually with other congenital abnormalities but initial definition in adults including the elderly has become increasingly more common...
February 15, 2011: World Journal of Gastrointestinal Oncology
Qing-Ya Tang, Jie Wen, Jiang Wu, Ying Wang, Wei Cai
BACKGROUND: primary intestinal lymphangiectasia (PIL) is a rare digestive disease and few studies have focused on the therapeutic effect in PIL patients. This study was undertaken to evaluate nutrition-oriented intervention in children with PIL. METHODS: four children with PIL were studied. Their medical records were reviewed. Anthropometric measurements and blood tests were performed during a 8-18 month follow-up. RESULTS: during hospitalization, the 4 patients were subjected to diet intervention...
February 2011: World Journal of Pediatrics: WJP
Marjet J A M Braamskamp, Koert M Dolman, Merit M Tabbers
Protein-losing enteropathy (PLE) is a rare complication of a variety of intestinal disorders characterized by an excessive loss of proteins into the gastrointestinal tract due to impaired integrity of the mucosa. The clinical presentation of patients with PLE is highly variable, depending upon the underlying cause, but mainly consists of edema due to hypoproteinemia. While considering PLE, other causes of hypoproteinemia such as malnutrition, impaired synthesis, or protein loss through other organs like the kidney, liver, or skin, have to be excluded...
October 2010: European Journal of Pediatrics
Sinan Sari, Zeren Baris, Buket Dalgic
OBJECTIVE: Octreotide has been suggested as a medical treatment option in refractory cases of primary intestinal lymphangiectasia (IL). There are few data about the long-term effect and safety of octreotide for IL in the literature. In the present article we analyzed pediatric cases of primary IL with long-term octreotide treatment and discussed its safety profile. METHODS: Between 1999 and 2008, 13 children were diagnosed in our clinic as having IL. Six patients with primary IL were followed up, receiving octreotide therapy...
October 2010: Journal of Pediatric Gastroenterology and Nutrition
Jie Wen, Qingya Tang, Jiang Wu, Ying Wang, Wei Cai
BACKGROUND: Primary intestinal lymphangiectasia (PIL) is a rare digestive disease and most articles on this condition are isolated case reports. AIMS: Our purpose is to investigate the clinical characteristics, therapeutic management, and outcome of PIL through case studies. METHODS: We conducted a retrospective analysis and obtained detailed clinical information for four PIL patients treated at our institution. A MEDLINE database search was also performed using the search term "intestinal lymphangiectasia" and all pertinent literature was carefully reviewed...
December 2010: Digestive Diseases and Sciences
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