Erythrocyte and Liver Porphobilinogen Deaminase in Cirrhosis and Clinical or Experimental Cholestasis

Authors

  • Dimitrios Manganas
  • George Yannakos
  • George Triantafyllou
  • Peter Danias
  • Konstantinos Kantartzis
  • Sotirios Raptis
  • Athanasios G. Yalouris

DOI:

https://doi.org/10.2015/hc.v2i2.29

Keywords:

Cirrhosis, Cholestasis, ??-aminolevulinic acid synthetase, porphobilinogen deaminase

Abstract

BACKGROUND

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Porphobilinogen deaminase, the third enzyme in the haem synthetic process -mainly expressed in the erythrocytes and liver - has a key role in the pathogenesis of the acute porphyrias.

DESIGN AND RESULTS We studied the effect of cirrhosis or cholestasis on this enzyme activity and found that:

1. Erythrocyte porphobilinogen deaminase was significantly increased (p=0.0003) in 27 patients with non-alcoholic liver cirrhosis (19.89?±6.65 ??moles/h.l) and 24 patients with extrahepatic cholestasis (20.69?±11.17 ??moles/h.l) as compared to 30 controls (12.77?±4.76 ??moles/h.l). Its activity was positively correlated to the prothrombin time in both patient groups and negatively to the alkaline phosphatase in the cholestasis group.

2. Erythrocyte porphobilinogen deaminase of controls significantly increased when their plasma was substituted by that of patients with cholestasis (p lt; 0.001) or cirrhosis (p=0.05), although it remained lower than that of the latter. No change was observed in samples from patients with cirrhosis or cholestasis when their plasma was substituted by that of controls.

3. In 8 rabbits, cholestasis produced by ligation of the common bile duct significantly increased porphobilinogen deaminase both in the erythrocytes (from 30.26?±10.33 to 48.87?±15.82 nmoles/h.l, p=0.002) and the liver (from 13.27?±4.79 to 17.68?±5.42 nmoles/h.g, p=0.035). In 8 sham-operated rabbits erythrocyte porphobilinogen deaminase also increased (from 29.60?±9.85 to 33.32?±12.23 nmoles/h.l, p=0.016), but to a significantly lower degree than that of the ???cholestatic?? group (111.10?±10.95 % versus 167.96?±41.64% p=0.006) while the hepatic enzyme remained unchanged (from 13.40?±3.85 to 13.83?±7.21 nmoles/h.g, p=0.80).

4. In 5 patients with cholestasis the mean hepatic PBG-D activity was higher than in the 5 controls (12.63?±3.24 versus 9.64?±1.17 nmoles/h.g), although not significantly higher (p=0.11).

CONCLUSION PBG-D activity is considerably increased in liver cirrhosis and clinical or experimental cholestasis. It seems probable that plasma factors may play a role in this effect by inducing PBG-D activity.

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Issue

Vol. 2 No. 2 (2007)

Section

ORIGINAL ARTICLES

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