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Protein-losing enteropathy following the Fontan procedure in a child with intestinal lactase deficiency treated with lactose-free diet

Published online by Cambridge University Press:  27 February 2013

Wolfgang Kienast ,
Klaus Haeseler  and
Reinhard Herterich
Wolfgang Kienast*
Affiliation:
Department of Paediatric Cardiology, Ostseeklinik Boltenhagen, Germany
Klaus Haeseler
Affiliation:
Department of Paediatric Cardiology, Ostseeklinik Boltenhagen, Germany
Reinhard Herterich
Affiliation:
Children's Hospital St. Marien, Landshut, Germany
*
Correspondence to: Dr W. Kienast, MD, PhD, Department of Paediatric Cardiology, Ostseeklinik Boltenhagen, Ostseeallee 108, 23946 Ostseebad Boltenhagen, Germany. Tel: +493814009230; Fax: +493814402589; E-mail: wolfgang.kienast@t-online.de
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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. Conclusion: 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.

Keywords

Protein-losing enteropathyFontan circulationintestinal lactase deficiency
Type
Brief Reports
Information
Cardiology in the Young , Volume 24 , Issue 1 , February 2014 , pp. 175 - 177
Copyright
Copyright © Cambridge University Press 2013 

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