R. Kardorff

Liver transplantation in children with chronic end stage liver disease: factors influencing survival after transplantation.

To identify pretransplant factors that are influencing survival after orthotopic liver transplantation a Cox proportional hazards regression model was applied to 118 children with chronic terminal liver failure transplanted at Medical School Hannover during the period of 1978 to 1994. The response variable was survival, as covariates a total of 19 pretransplant variables were entered--i.e. age, diagnosis (biliary cirrhosis, metabolic cirrhosis, postnecrotic cirrhosis, cryptogenetic cirrhosis) sex, laparotomy prior to OLT, height, weight, standard deviation scores for height and weight, date of first OLT, serum alanine aminotransferase, asparagine aminotransferase, albumin, total bilirubin, cholinesterase activity, glomerular filtration rate, and prothrombin time. Significant independent predictors of survival after OLT were bilirubin (P=0.0024), SDS for weight (P=0.034), and albumin (P=0.039). In a subsequent discriminant analysis cut off points for these variables could be identified--i.e., bilirubin >340 micromol/L, SDS for weight <-2.2 and albumin < 33 g/L. Patients with one or more of these risk factors were grouped as urgent indication group (n=76) and those with no risk factor as elective indication group (n=42). Comparing the posttransplantation survival in these groups there is a statistically significant difference at 1 year (57% vs. 90.5%) and 4 years (49% vs. 90.5%) after OLT (P=0.0001, log rank test). It is concluded that the risk of OLT is much higher if liver function is very poor. Optimal nutritional support prior to transplantation is mandatory to optimise the clinical status of the children and to improve the results of OLT.

Prediction of survival in extrahepatic biliary atresia by hepatic duplex sonography

BACKGROUND The clinical course of biliary atresia patients is extremely variable. To optimize conservative treatment and correctly schedule liver transplantation, noninvasive investigations that are predictive of individual survival and that can be performed regularly are needed. In this study, the prognostic value of Doppler sonography was investigated in these patients. METHODS Thirty biliary atresia patients (age range, 1 month to 15.2 years; mean, 4.0 years) and 38 control subjects underwent standardized Doppler sonography of liver and spleen. Biochemical tests of liver function and of fibrogenesis were performed in parallel. Individual clinical outcome was registered 1 and 2 years later. RESULTS In control subjects, maximum portal flow velocity (Vmax) was more than 16 cm/sec, and the hepatic vein flow pattern was triphasic. Among children with biliary atresia, those with diminished portal Vmax, a flattened hepatic vein flow curve, or a hepatic artery resistance index of 0.8 or more had significantly lower indices of hepatic protein synthesis (albumin, cholinesterase), higher bilirubin levels, and higher concentrations of markers of connective tissue turnover (procollagen peptides, laminin P1) than did those with normal Doppler sonography measurements. The rate of survival without transplantation during the following 2 years was significantly lower in children with abnormal Doppler findings. From portal and hepatic vein flow measurements, patient survival 2 years later could be predicted with an accuracy of 93%. CONCLUSIONS In children with extrahepatic biliary atresia, Doppler sonography of the hepatic blood flow is a noninvasive indicator of disease severity. Moreover, it allows a highly accurate prediction of patient survival for the following 2 years.

Auxiliary partial orthotopic liver transplantation for acute liver failure in two children.

Abstract: Acute liver failure in children and adults is associated with a high mortality rate. At present the treatment of choice is orthotopic whole‐liver transplantation. However, allogeneic liver transplantation necessitates lifelong immunosuppressive therapy, which is associated with substantial risks to the patient. Temporary auxiliary partial orthotopic liver transplantation has been developed recently as an alternative, enabling the native liver to regenerate while avoiding the risks of long‐term immunosuppressive treatment. Here we describe two cases of partial orthotopic liver transplantation in children. Auxiliary partial orthotopic liver transplantation was performed in two boys (5 and 6 years old) suffering from acute liver failure of unknown origin. The native left lateral liver lobes (segment II and II) were removed and replaced by left lateral liver grafts from young blood‐group‐compatible adults. In the first child the native liver, which was 80% necrotic at time of transplantation, showed regeneration within two weeks and the partially necrotic graft could be surgically removed on day 15 after auxiliary transplantation. Four years after transplantation, the child is in excellent condition with normal liver function and does not require any treatment. In the second case the native liver (90% necrotic at time of transplantation) regenerated within 6 weeks of transplantation, at which time the transplanted liver was removed. The patient developed aplastic anemia and died 2 months after transplantation from candida sepsis. The conclusion was that auxiliary partial liver transplantation in childhood provides a valuable option to maintain liver function in acute liver failure until functional recovery of the native liver. The main advantage over whole‐liver transplantation is the chance to avoid lifelong immunosuppression. However, there is a higher surgical risk. Therefore, auxiliary transplantation should be considered carefully in every case of acute liver failure in children.

Primäre pulmonale Hypertonie bei einem Kind mit primär sklerosierender Cholangitis : Diagnostik und Verlauf vor und nach Lebertransplantation

ZusammenfassungBei einem an einer primär sklerosierenden Cholangitis erkrankten Mädchen bestand über Jahre ein schwerer portaler Hypertonus. Mit 11 Jahren traten klinische Symptome einer Rechtsherzdekompensation auf. Die orthotope Lebertransplantation, 1 Jahr später durchgeführt, führte zu einer Dekompensation mit Anstieg des rechtsventrikulären Drucks bis 95 mmHg. Die Diagnostik ergab eine primäre pulmonale Hypertonie mit plexiformer pulmonaler Arteriopathie. Unter Sauerstofftherapie und Nifedipin kam es im Verlauf von 10 Monaten zu einer Besserung der rechtsventrikulären Belastung etwa auf den Status vor der Transplantation (rechts-ventrikulärer Druck um 45 mmHg), jedoch nicht zu einer weitergehenden Remission. Diskussion: Eine primäre pulmonale Hypertonie in Assoziation mit einer portalen Hypertension kann auch im Kindesalter auftreten und hat erhebliche Bedeutung für die Prognose und das therapeutische Vorgehen. In der Betreuung chronisch leberkranker Kinder muß bei regelmäßigen Verlaufskontrollen von EKG, Röntgenthorax und Echokardiografie bewußt auf diese oft zunächst asymptomatische Komplikation geachtet werden. Im Verdachtsfall ist eine Herzkatheteruntersuchung zur Diagnosesicherung und zum Austesten des Therapieansprechens erforderlich.SummaryA child who suffered from primary sclerosing cholangitis presented with severe portal hypertension over years. At age eleven, symptoms of right ventricular decompensation were noted. After stabilization, orthotopic liver transplantation was performed and resulted in severe decompensation with an increase of right ventricular pressure up to 95 mmHg. At further investigation, primary pulmonary hypertension with plexiform pulmonary arteriopathy was diagnosed. After ten months under oxygen and nifedipine treatment, the patient reached a stable cardiopulmonary state comparable to the pre-transplant period (right ventricular pressure 45 mmHg) but did not improve further. Discussion: Primary pulmonary hypertension in children can occur in association with severe portal hypertension. As this complication will considerably alter prognosis and management of children with chronic liver disorders, it must be actively searched for and monitored with regular ECG, chest X-ray and echocardiography. To prove the diagnosis and evaluate treatment response, cardiac catheterization is required.