Frühnachweis einer hepatobiliären Beteiligung bei Kindern mit Mukoviszidose mit Hilfe der Duplexsonographie des Portalsystems

Abstract

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.

Hypergalactosaemia and portosystemic encephalopathy due to persistence of ductus venosus Arantii

“…At the age of 4 years 2 months, the time average velocity in the ductus venosus of our patient was 15.5 cm/s which compared with that in the portal vein of normal children (18.0 + 5 cm/s) [10]. The estimated diameter of the ductus venosus was 6 mm and thus comparable to that of the portal vein in normal children of this age [10].…”

“…The estimated diameter of the ductus venosus was 6 mm and thus comparable to that of the portal vein in normal children of this age [10]. These observations allowed the assumption of a complete or near complete splanchnic venous bypass of the liver by the venous duct.…”

Prediction of Survival in Extrahepatic Biliary Atresia by Hepatic Duplex Sonography

“…Available data on Doppler sonography in children with chronic liver disorders show lower portal flow velocities and higher hepatic artery RI compared with those in healthy control subjects (13,17,18,20,21). No studies have been published on hepatic vein flow patterns in children, although in our experience, this parameter can be recorded more easily and satisfactorily in a restless infant than can flow velocities of the hepatic artery.…”

“…In control subjects, no blood sampling was performed, and the time‐consuming search for an exact measurement of arterial flow was omitted. An upper limit of normal RI in children (0.8) was taken from the literature (17).…”