title: Protein-losing enteropathy following the Fontan procedure in a child with intestinal lactase deficiency treated with lactose-free diet.
abstract: A 12-year-old girl presented with a protein-losing enteropathy. Symptoms started 4 weeks after undergoing the Fontan procedure at the age of 1.5 years for mitral atresia, ventricular septal defect, and double-outlet right ventricle. Upon referral for 3 weeks of rehabilitation after multiple interventional measures and drug treatments, she appeared in a dystrophic state, with decreased plasma protein and electrolyte levels along with occasional tetanic convulsions. Blood glucose levels after a lactose tolerance test were markedly reduced. The introduction of a lactose-free diet was quickly effective, with plasma protein and electrolyte levels raised to normal levels, and the girl's body weight increased without ascites or oedema. Molecular genetic examination revealed a homozygous C/C13910 polymorphism in the LCT gene.
  
  
  CONCLUSIONS
  Protein-losing enteropathy in the Fontan circulation may be provoked by lactase deficiency and should therefore be ruled out to exclude this rather common condition.
authors: 1 Department of Paediatric Cardiology, Ostseeklinik Boltenhagen, Germany.
authors: Wolfgang
authors: W
authors: Kienast
authors: 1 Department of Paediatric Cardiology, Ostseeklinik Boltenhagen, Germany.
authors: Klaus
authors: K
authors: Haeseler
authors: 2 Children's Hospital St. Marien, Landshut, Germany.
authors: Reinhard
authors: R
authors: Herterich
mesh_terms: D002648:Child; D004310:Double Outlet Right Ventricle / Q000601:surgery; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000601:surgery; D006349:Heart Valve Diseases / Q000151:congenital / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D043322:Lactase / Q000172:deficiency* / Q000235:genetics; D007787:Lactose Intolerance / Q000150:complications / Q000178:diet therapy* / Q000235:genetics; D008943:Mitral Valve / Q000002:abnormalities / Q000601:surgery; D011110:Polymorphism, Genetic; D011183:Postoperative Complications / Q000178:diet therapy*; D011504:Protein-Losing Enteropathies / Q000178:diet therapy* / Q000209:etiology
chemical_list: D043322:Lactase