Functional protein C and anti-cardiolipin antibody in children with portal vein thrombosis.
BACKGROUND
Portal vein thrombosis (PVT) is a common cause of portal hypertension in children from developing countries. Deficiencies of proteins C and S and elevated anticardiolipin antibody (aCL) levels have been shown to predispose to venous thrombosis. We studied these factors in children with idiopathic PVT.


METHODS
19 children with PVT (mean [SD] age 5.7 [2.1] y; 15 boys) were studied; all had had variceal bleeding, and had PVT on ultrasonography. Functional protein C activity was measured using a clotting assay; if it was normal, a clotting assay for functional protein S activity was performed. IgG aCL levels were measured in all sera using an in-house standardized solid-phase ELISA.


RESULTS
Protein C functional activity ranged from 4% to 109%. Eight children had activity below 70%, the lower cut-off of the normal range. Protein S assay, done in 10 of the 11 children with normal protein C activity levels, was normal (above the cut-off level of 65% of the normal range). IgG aCL levels were abnormally elevated (>mean + 2SD of 16 healthy control children) in nine children; of these, three had associated protein C deficiency. Thus, of the 19 children with idiopathic PVT, 14 had abnormality in one or more tests.


CONCLUSIONS
A majority of children with PVT of unknown etiology have functional protein C deficiency or abnormally elevated levels of aCL antibodies.
Yachha
S K
SK
Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow. skyachha@sgpgi.ac.in
Aggarwal
R
R
Sharma
B C
BC
Misra
R N
RN
Aggarwal
A
A
Naik
S R
SR
D017153:Antibodies, Anticardiolipin / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007074:Immunoglobulin G / Q000378:metabolism*; D007223:Infant; D008297:Male; D011169:Portal Vein; D020151:Protein C Deficiency / Q000150:complications*; D018455:Protein S Deficiency / Q000150:complications; D020246:Venous Thrombosis / Q000150:complications* / Q000378:metabolism*
D017153:Antibodies, Anticardiolipin; D007074:Immunoglobulin G