ABSTRACT
Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia (PIL) in infants.Methods Clinical,laboratory,gastroscopy imaging,lymph radionuclide imagining,and therapeutic intervention data in eight patients admitted with PIL in Beijing Children's Hospital from Jan.2007 to Feb.2012 were reviewed.Results Their ages ranged from 4 to 8 months old.The common complaints were edema(8/8 cases),diarrhea (8/8 cases),infection (8/8 cases),and ascites (7/8 cases).Other symptoms included vomiting (5/8 cases),low body weight (2/8 cases),and convulsions (2/8 cases).Infections involving respiratory tract,blood,and gastrointestinal system were prominent.The causal pathogens for the infections were bacteria,virus,fungi,and ectosarc.The laboratory abnormalities included lymphocytopenia (8/8 cases),hypoalbuminemia (8/8 cases),and hypogammaglobulinemia (8/8 cases).On immunologic evaluation,the CD4 cell counts and serum IgG levels were significantly decreased(7/7 cases) while B cell and NK cell counts were normal.The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots(8/8 cases).Further pathological examinations indicated dilated lymphatic channel in mucosal and submucosal(5/8 cases).Lymph radionuclide imaging discovered abnormalities consistent with PIL in 6 out of 8 patients,including 3 cases that were negative for pathological examination.A multidisciplinary approach was taken to treat each patient.The low-fat and medium chain triglycerides-rich diet was introduced with supplements of albumin and globulin.Infection control and edema alleviation were well managed through medication.However,diarrhea remained a problem.Only 2 out of 8 patients had recovered from hypoalbuminemia and lymphocytopenia.One case had a complete remission after surgical therapy withno relapse.Conclusions PIL in infant has common clinical presentations with an exception of more severe infection.Lymph radionuclide imaging is a sensitive diagnostic method.Medication treatment for infant PIL is not satisfactory.Surgical management is recommended if the locus of pathology is confined.
ABSTRACT
Primary intestinal lymphangiectasia is a rare disease, which is characterized by the obstruction of lymphatic return, dilated lymphatics and distorted structure of villi. Lymph fluid leaks into the small bowel lumen as a result of the obstruction of lymphatics and elevated pressure of lymphatics, and leads to malabsorption and loss of proteins. The causes and pathogenesis of PIL are not clear, hut the abnormal immunologic mechanism has got people's attention. Recently, the endoscopic technique has been improved, and the diagnosis rate of PIL has increased obviously. Antisecosis is the basic and effective treatment, especially for children.
ABSTRACT
Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia, hypoalbuminemia, and lymphopenia. We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. The presence of enteric protein loss was documented with the 24 hour fecal clearance of alpha(1)-antitrypsin and (99m)Tc human serum albumin scintigraphy. A duodenoscopy and biopsy showed scattered white spots and markedly dilated lymphatics in the tips of the villi, respectively. The patient's clinical symptoms improved after placing her on a high protein and low fat diet with medium chain triglyceride supplements.
Subject(s)
Adolescent , Female , Humans , Ascites , Biopsy , Dental Caries , Diarrhea , Diet , Duodenoscopy , Edema , Hypoalbuminemia , Hypoproteinemia , Leg , Lymphopenia , Protein-Losing Enteropathies , Radionuclide Imaging , Serum Albumin , TriglyceridesABSTRACT
Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.
Subject(s)
Child , Humans , Male , Diarrhea , Diet , Edema , Hypoalbuminemia , Intestines , Lymphocytes , Peritoneum , Protein-Losing Enteropathies , TriglyceridesABSTRACT
Primary intestinal lymphangiectasia is a rare congenital disorder characterized by diffuse or localized ectasia of the enteric lymphatics, often in association with lymphatic abnormalities elsewhere in the body. The pathogenesis of these abnormal lymphatic structures is uncertain. Ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to a loss of protein and lymphocytes into the gut or the peritoneal cavity. The mechanism of this lymphatic loss is believed to be due to a rupture of lymphatics across the mucosa with subsequent leakage of the lymph into the bowel lumen. Presentation of primary intestinal lymphangiectasia may occur any time throughout infancy and childhood. Prominent clinical features include protein-losing enteropathy, lymphocytopenia, hypogammaglobulinemia, edema, growth failure, and chronic diarrhea. The mainstay of treatment for intestinal lymphangiectasia is the use of a low-fat, high- protein, medium-chain triglycerides diet. The natural history of this rare disorder is not well known as only a few cases have been reported in the literature. We present herein the follow up of a case with primary intestinal lymphangiectasia who responded to octreotide therapy but did not respond to dietary or antiplasmin therapy.
Subject(s)
Agammaglobulinemia , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Diarrhea , Diet , Dilatation, Pathologic , Edema , Follow-Up Studies , Lymphatic Abnormalities , Lymphocytes , Lymphopenia , Mucous Membrane , Natural History , Octreotide , Peritoneal Cavity , Protein-Losing Enteropathies , Rupture , TriglyceridesABSTRACT
We experienced a case of primary intestinal lymphangiectasia in a 20-month-old boy, which was confirmed gastrofiberscopically and histologically only after a high-fat meal before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenal mucosa, which were proved dilated lymph vessels in the lamina propria on histological examination. Treatment with low-fat diet and medium chain triglycerides supplementation was satisfactory.