title: [Exudative enteropathy in congenital lymphedema-lymphangiectasia syndrome].
abstract: BACKGROUND
  Congenital peripheral elephantiasiformic alterations are very rare in paediatric patients. In a patient with lymphangiectasia-lymphedema syndrome we demonstrate over a 8-year follow-up that not only cosmetic and social indications for surgical treatments but also internal care become important during the course.
  
  
  METHODS
  We report on a boy with congenital lymphedemas of the extremities and the genital region, which were several times surgically treated. The patient became symptomatic firstly with tetanic cramps caused by malabsorption syndrome due to intestinal lymphangiectasia at the age of 6 years. Synopsis of clinical and laboratory findings and the patient's course are pointing to a mild Hennekam syndrome with still unknown aetiology.
  
  
  RESULTS
  The boy developed adequately with permanent oral substitution of electrolytes and vitamins, protein-rich diet, supplementation of medium-chain fatty acids and compressing bandages. Infusions of human albumin to correct persistent hypalbuminemia as well as cytostatic treatment with cyclophosphamide as a formal trial were ineffective and are not advisable, therefore.
authors.affiliation: Abt. Kinder- und Jugendmedizin, HELIOS Krankenhaus, Leisnig.
authors.forename: M
authors.initials: M
authors.lastname: Heruth
authors.forename: P
authors.initials: P
authors.lastname: Müller
authors.forename: L
authors.initials: L
authors.lastname: Liebscher
authors.forename: G
authors.initials: G
authors.lastname: Kurze
authors.forename: T
authors.initials: T
authors.lastname: Richter
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004044:Dietary Proteins / Q000008:administration & dosage; D004573:Electrolytes / Q000008:administration & dosage; D005500:Follow-Up Studies; D006801:Humans; D007262:Infusions, Intravenous; D008200:Lymphangiectasis / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D008201:Lymphangiectasis, Intestinal / Q000175:diagnosis* / Q000628:therapy; D008209:Lymphedema / Q000151:congenital* / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D011504:Protein-Losing Enteropathies / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D012709:Serum Albumin / Q000008:administration & dosage; D016896:Treatment Outcome; D014815:Vitamins / Q000008:administration & dosage
chemical_list: D004044:Dietary Proteins; D004573:Electrolytes; D012709:Serum Albumin; D014815:Vitamins