Hasmann R, Grunert D, Reuter N, Stern M
Universitäts-Kinderklinik, Abteilung II, Tübingen.
Klin Padiatr. 1991 Mar-Apr;203(2):97-103. doi: 10.1055/s-2007-1025408.
To investigate early signs of hepatobiliary disease in CF, we measured portal, splenic, superior mesenteric vein and hepatic artery diameters, maximal flow velocity (Vmax) and time average velocity (TAV) in 25 males and 17 females with CF. Hepatic artery resistance, regional blood flow and liver perfusion were calculated. According to liver enzyme data (aminotransferases raised greater than 30 U/l) and sonographic findings (nodular changes), there were 17 CF-patients (mean age 11.4 yrs; range 0.75-31) with and 25 CF-patients (mean age 7.8 yrs; range 0.25-32) without liver involvement (L+/L-). No patient had clinical signs of portal hypertension. 61 healthy children were studied for control. Diameter of portal vein (PVD) and flow data for portal vein showed consistent abnormalities (mean +/- SD): CF-L+ CF-L- Control PVC (mm/m2) 10.7 +/- 3.9*** 10.5 +/- 3.4*** 7.2 +/- 1.3 Vmax (m/sec) 0.23 +/- 0.06*** 0.30 +/- 0.06*** 0.40 +/- 0.14 TAV (m/sec) 0.12 +/- 0.04*** 0.16 +/- 0.04* 0.18 +/- 0.05 Differences were statistically significant (* p less than 0.05, *** p less than 0.001) for CF-patients versus controls. Data for splenic and mesenteric veins and for hepatic artery were moderately alterated, with a significant reduction in TAV and Vmax of splenic vein for CF-L+ versus controls. Liver perfusion and portal vein flow showed no relevant differences in CF-patients versus controls. It is concluded that portal system abnormalities, especially a decrease in Vmax and TAV of portal vein shown by duplex sonography, may be earlier indicators of CF liver disease than biochemical and clinical signs.