[Congenital intestinal lymphangiectasia: a rare differential diagnosis in hypoproteinemia in infants].
BACKGROUND
Congenital intestinal lymphangiectasia is a rare disease in childhood, which may already cause protein-losing enteropathy in newborns.


METHODS
This is a case report of an infant with generalized edema and protein-losing enteropathy, in whom intestinal lymphangiectasia was diagnosed at the age of two months. Following repetitive intravenous albumin und gamma globulin infusions, the elimination of long-chain fats from the diet and the substitution with medium-chain triglycerides (MCT) led to an improvement of the protein-losing enteropathy.


CONCLUSIONS
In newborns with low level of serum protein and edema protein-losing enteropathy caused by congenital lymphangiectasia might be considered as a differential diagnosis.
Westfälisches Kinderzentrum Dortmund, Klinikum Dortmund gGmbH, Beurhausstrasse 40, 44137 Dortmund. ansgar.moeller@klinikumdo.de
A
A
Möller
H
H
Kalhoff
T
T
Reuter
N
N
Friedrichs
N
N
Wagner
D001706:Biopsy; D003241:Consanguinity; D003937:Diagnosis, Differential; D004041:Dietary Fats / Q000008:administration & dosage / Q000009:adverse effects; D004386:Duodenum / Q000473:pathology; D004487:Edema / Q000209:etiology / Q000473:pathology; D004729:Endothelium, Lymphatic / Q000473:pathology; D006801:Humans; D007019:Hypoproteinemia / Q000151:congenital* / Q000175:diagnosis / Q000178:diet therapy / Q000473:pathology; D007223:Infant; D007413:Intestinal Mucosa / Q000473:pathology; D008201:Lymphangiectasis, Intestinal / Q000151:congenital* / Q000175:diagnosis / Q000178:diet therapy / Q000473:pathology; D008894:Milk Proteins / Q000008:administration & dosage; D011504:Protein-Losing Enteropathies / Q000151:congenital* / Q000175:diagnosis / Q000178:diet therapy / Q000473:pathology
D004041:Dietary Fats; D008894:Milk Proteins; C482039:Monogen