Abstract
In chronic liver disease, the development of a continuous basement membrane along the liver sinusoids (sinusoidal capillarization) results in impaired hepatic circulation and furthur liver damage. Using immunohistochemistry, we have shown laminin, a major component of basement membranes, to be present along the liver sinusioids in diagnostic biopsies from pre-cirrhotic infants (6 biliary atresia [BA] and 5 idiopathic hepatitis of infancy [IHI]) but absent in 4 with no histological abnormality. To determine whether this is reflected in serum, we have measured serum laminin at presentation in 32 patients with BA, 23 with IHI and 9 with α1-antitryps in deficiency [α1ATD] and at follow up in 10 with BA, 9 with IHI and 6 withc α1ATD.
At presentation, all patients had raised serum laminin (3.28±0.97 U/l in BA, 3.46±1.37 in IHI and 2.59±0.47 in αlATD) compared to age-matched controls (1.67±0.41). At 40-60 week follow-up, serum laminin remained elevated in all 10 patients with BA; all had cirrhosis and laminin was similar in the 4 who were jaundiced and the 6 who were jaundice-free. In IHI, serum laminin became normal at follow-up in all patients, although 3 had cirrhosis (1 with jaundice) and 2 others had abnormal LFTS. Similarly, serum laminin became normal at follow up in all patients with αdATD, although 4 had cirrhosis (none with jaundice) and the other 2 had abnormal LFTs.
We conclude that there is no clear cut association either at presentation or follow up between serum laminin and cirrhosis. Persistently elevated laminin in serum in surgically treated BA may reflect on-going sinusoidal capillarization.
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A, W., F, P. BACTERIAL INFECTIONS: THE MAIN CAUSE OF NEONATAL CHOLESTASIS. Pediatr Res 20, 698 (1986). https://doi.org/10.1203/00006450-198607000-00080
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DOI: https://doi.org/10.1203/00006450-198607000-00080