Protein-losing enteropathy after the total cavopulmonary connection: impact of intravenous immunoglobulin.
OBJECTIVE
Despite multiple treatment modalities, protein-losing enteropathy remains a serious complication to Fontan-type operations. Observations suggest inflammation to be involved in the pathogenesis of this condition, and immunomodulating treatment with high-dose intravenous immunoglobulin may modify the condition positively.


METHODS
Four patients with protein-losing enteropathy occurring after the total cavopulmonary connection, presenting with edema, hypoalbuminemia, and hypogammaglobulinemia, received intravenous immunoglobulin replacement therapy.


METHODS
Standard replacement dose (1 g/kg) was used with intervals between infusions adjusted according to albumin and gamma globulin levels. Treatment periods ranged from 1 year to 5.3 years.


RESULTS
Intravenous immunoglobulin treatment was associated with significant increase in plasma albumin and to some extent in immunoglobulin G levels, as well as resolution of edema and the children started to thrive normally. During treatment, no serious infections or serious side effects were seen. Additional follow-up intervals ranged from 2 years to 2.8 years, during which only one episode of clinical relapse was registered and treated.


CONCLUSIONS
We find the increase in albumin level and the resolution of protein-losing enteropathy symptoms after treatment with intravenous immunoglobulin of particular interest considering this serious complication to Fontan-type operations.
Department of Paediatrics, Aarhus University Hospital, Brendstrupgaardsvej 100, Aarhus N, Denmark. louise.zaupper@viborg.rm.dk
Louise B
LB
Zaupper
Bent W
BW
Nielsen
Troels
T
Herlin
D000361:Agammaglobulinemia / Q000097:blood / Q000188:drug therapy / Q000209:etiology; D015415:Biomarkers / Q000097:blood; D002675:Child, Preschool; D003718:Denmark; D004487:Edema / Q000188:drug therapy / Q000209:etiology; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D034141:Hypoalbuminemia / Q000188:drug therapy / Q000209:etiology; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D007223:Infant; D007262:Infusions, Intravenous; D008297:Male; D011504:Protein-Losing Enteropathies / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D012709:Serum Albumin / Q000378:metabolism; D013997:Time Factors; D016896:Treatment Outcome
D015415:Biomarkers; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D012709:Serum Albumin