title: [Primary intestinal lymphangiectasia: twenty years of experience at a Mexican tertiary care hospital].
abstract: 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.
  
  
  OBJECTIVE
  To describe the clinical, biochemical, radiologic, endoscopic, and histologic characteristics in children with primary intestinal lymphangiectasia.
  
  
  METHODS
  A retrospective observational, descriptive, cross-sectional study was conducted that reviewed the case records of children diagnosed with primary intestinal lymphangiectasia that were seen at the Department of Gastroenterology and Nutrition of the Instituto Nacional de Pediatría within the time frame of January 1, 1992 to September 30, 2012.
  
  
  RESULTS
  Four patients were found that presented with primary intestinal lymphangiectasia. Three of them had been diagnosed before 3 years of age. All the patients presented with chronic diarrhea, edema, lymphopenia, hypocalcemia, and hypogammaglobulinemia, and 3 patients presented with hypocholesterolemia. Bowel transit time, endoscopy, and intestinal biopsies were characteristic of this pathology.
  
  
  CONCLUSIONS
  Intestinal lymphangiectasia should be suspected when there is a clinical picture of chronic diarrhea and protein-losing enteropathy accompanied with edema at any level, as well as hypoalbuminemia, hypocalcemia, lymphopenia, hypogammaglobulinemia, and hypocholesterolemia, which are the main biochemical findings of this pathology. All children presenting with intestinal lymphangiectasia should undergo an upper gastrointestinal series with bowel transit time and endoscopy with biopsies taken at the level of the duodenum. Treatment includes diet and the periodic administration of albumin and gamma globulin.
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México. Electronic address: danelyvaldovinos@hotmail.com.
authors.forename: D
authors.initials: D
authors.lastname: Valdovinos-Oregón
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: J
authors.initials: J
authors.lastname: Ramírez-Mayans
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: R
authors.initials: R
authors.lastname: Cervantes-Bustamante
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: E
authors.initials: E
authors.lastname: Toro-Monjaraz
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: M
authors.initials: M
authors.lastname: Cázares-Méndez
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: J
authors.initials: J
authors.lastname: Cadena-León
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: F
authors.initials: F
authors.lastname: Zárate-Mondragón
authors.affiliation: Servicio de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Secretaría de Salud, México, D.F., México.
authors.forename: E
authors.initials: E
authors.lastname: Montijo-Barrios
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003967:Diarrhea / Q000175:diagnosis / Q000209:etiology; D004032:Diet; D016099:Endoscopy, Gastrointestinal; D005260:Female; D006801:Humans; D007223:Infant; D008201:Lymphangiectasis, Intestinal / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D008800:Mexico; D012189:Retrospective Studies; D062606:Tertiary Care Centers
keywords: Gamma globulin; Linfangiectasia intestinal primaria; Primary intestinal lymphangiectasia; Uso de gammaglobulina