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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: [email protected]
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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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References

1. Robert Koch Institut: Referenzperzentile für anthropometrische Maßzahlen und Blutdruck aus der Studie zur Gesundheit von Kindern und Jugendlichen in Deutschland (KiGGS) 2003–2006. Robert Koch-Institut, Berlin, 2011; pp. 21–31.Google Scholar
2. Mertens, L, Hagler, DJ, Sauer, U, Somerville, J, Gewillig, M. Protein-losing entropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115: 1063–1073.CrossRefGoogle Scholar
3. Braamskamp, MJ, Dolman, KM, Tabbers, MM. Clinical practice. Protein-losing entropathy in children. Eur J Pediatr 2010; 169: 1179–1185.Google Scholar
4. Mottes, M, Belpinati, F, Milani, M, et al. Genetic testing for adult-type hypolactasia in Italian families. Clin Chem Lab Med 2008; 46: 980–984.Google Scholar
5. Tobayo-Torres, CC, Nichols, BL. Molecular differentiation of congenital lactase deficiency from adult-type hypolactasia. Nutr Rev 2007; 65: 95–98.CrossRefGoogle Scholar
6. Heyman, MB, for the American Committee on Nutrition. Lactose intolerance in infants, children, and adolescents. Pediatrics 2006; 118: 1279–1286.Google Scholar