Intestinal lymphangiectasia in children

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...

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...

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...

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...

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...

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...

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...

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...

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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...

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...

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...

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...

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...

PURPOSE: The aim of our study was to determine the safety and usefulness of capsule endoscopy (CE) in pediatric patients. METHODS: We prospectively examined children (aged 10-18 years) with suspected small bowel disease and recorded capsule transit times, findings, and complications. RESULTS: We performed 19 CE examinations in 12 patients (median age 11.8 years; range 10-18 years). One of the two patients with obscure gastrointestinal bleeding (OGIB), a 14-year-old girl whose OGIB occurred after cord-blood transplantation due to leukemia, was diagnosed with thrombotic microangiopathy...

OBJECTIVE: The pathological change of small bowel is difficult to examine because it is anatomically unique. The development of wireless capsule endoscopy provides an unique opportunity to visualize the entire small bowel in a minimally invasive manner. The aim of this study was to assess the safety and clinical value of wireless capsule endoscopy in children. METHODS: During the last 4 years (June, 2004-June, 2008), 46 times of wireless capsule endoscopy were performed in 43 patients with suspected small bowel disease, including obscure gastrointestinal bleeding (n = 11), recurrent abdominal pain (n = 20), chronic diarrhea (n = 9), protein losing enteropathy (n = 2), recurrent vomiting (n = 1)...

Primary intestinal lymphangiectasia is a rare disease of children, which is characterized by chronic diarrhea and complicated with malnutrition, including fat-soluble vitamin deficiency. We report a girl aged 4 years and 8 months who was diagnosed with the disease by endoscopic duodenal biopsy at 8 months of age. She presented initially with chronic diarrhea at 4 months of age. Generalized edema with hypoalbuminemia frequently occurred despite regular albumin supplements. Multiple vitamins initially were not supplied regularly...

Angiosarcomas are rare tumors in children, usually occurring in soft tissue and liver. By contrast, angiosarcoma in adults usually occurs in the extremities in conjunction with lymphedema. Mesenteric angiosarcoma has only rarely been reported. When angiosarcomas arise in this location, they usually represent a 2nd malignancy following Hodgkin's lymphoma. We report a child who presented to the emergency room with an acute abdomen and underwent emergency surgery for a mesenteric angiosarcoma with associated lymphangiectasia of the bowel and mesentery...

The main cause of protein losing enteropathy (PLE) in children is intestinal lymphangiectasia. PLE is commonly diagnosed with radiotracer scintigraphy. We report the use of magnetic resonant imaging in diagnosis of a child with primary PLE. MRI clearly revealed abnormality in intestine and mesentery and dilated thoracic duct and mesenteric lymphatic as well as prominent subcutaneous lymphatics in the extremity. We conclude that MRI is a useful tool in diagnose of primary PLE.

Intussusception is defined as telescoping of a segment of gastrointestinal tract into an adjacent one. In small children, it is the commonest cause of intestinal obstruction. More than 90% of childhood intussusceptions are idiopathic. We report a rare case of localized small intestinal lymphangiectasia, presenting as intussusception in a 6-month-old male child. The child presented with features of acute intestinal obstruction for which he was later operated. The gross examination of excised ileocecal mass revealed intussusception...