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Biomedical subjects

M Burdelski

Publications and source records attributed to M Burdelski.

At least 91 records · Page 5Linked to original sources
PubMed1991

Treatment of inherited metabolic disorders by liver transplantation.

Among the worldwide accepted indications for liver transplantation, inherited metabolic disorders play an increasing role. In some paediatric centres this indication runs second after extrahepatic biliary atresia. The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient's life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders presenting with cirrhosis, hepatoma, life-threatening progression or failure of other organs with preserved liver function. The timing of liver transplantation has become easier with development of surgical techniques of reduced-size donor livers. These techniques enable the performance of liver transplantation with ABO blood group compatible organs of almost any size if indicated either by deterioration of liver function or impending complications such as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78-95% actuarial 1-year survival rates. However, lifelong immunosuppressive therapy is necessary. This seems to be acceptable even in disorders with only partial liver function defects.

Humans↗
PubMed1991

Assessment of pretransplant prognosis in patients with cirrhosis.

The objective of this prospective study was to assess the prognostic value of dynamic liver function tests and traditional methods of evaluating liver function in potential candidates for hepatic transplantation. Patients who underwent orthotopic liver transplantation within the follow-up period of 120 days were excluded. The study included 107 adult and 57 pediatric patients with cirrhosis. Postnecrotic cirrhosis was present in 107 and biliary cirrhosis in 57 of 164 patients. During the follow-up period, 26 of 164 patients died of their liver disease. At the time of inclusion, we recorded monoethylglycinexylidide (MEGX) formation from lidocaine, indocyanine green (ICG) half-life, bilirubin and albumin serum concentration, activity of cholinesterase and alkaline phosphatase, prothrombin time, the clinical complication of ascites, and--in adults--the Pugh score also. These variables were subjected as covariates to a survival analysis (Cox proportional hazards regression model) using separately the data from adults, pediatric patients, all patients with postnecrotic cirrhosis, and all patients with biliary cirrhosis. In all of these four subgroups there was a significant relationship between MEGX and ICG test results and the 120-day survival. In the stepwise analysis, none of the remaining parameters contributed to a further relevant improvement of our predictive ability when added to the values of ICG and MEGX. Our results suggest that the ICG and the MEGX test are superior to conventional liver function tests and the Pugh score in assessing short-term prognosis in cirrhotics independently from the etiology of the underlying liver disease. These findings may have important implications for determining the optimum timing of transplantation.

Adolescent↗
PubMed1991

Predictors of one-year pretransplant survival in patients with cirrhosis.

The aim of this prospective study was to examine the usefulness of flow-dependent dynamic liver function tests and conventional methods of evaluating liver function as predictors of pretransplant survival in patients with advanced cirrhosis. Patients who underwent orthotopic liver transplantation within the follow-up period of 365 days were excluded. One hundred one patients with histologically confirmed cirrhosis were studied. Fifty-eight patients had post-hepatitic cirrhosis, 13 had cryptogenic cirrhosis and 30 had biliary cirrhosis. During follow-up, 28 patients died of their liver diseases. At entry, we recorded indocyanine green half-life, monoethylglycinexylidide formation from lidocaine, bilirubin and albumin serum concentrations, activities of cholinesterase and alkaline phosphatase, prothrombin time, clinical complications of ascites and encephalopathy and the Pugh score. These variables were subjected as covariates to a stepwise survival analysis by use of the Cox proportional-hazards model. At the final step, Pugh score, monoethylglycinexylidide formation and indocyanine green half-life were found to be the only independent variables significantly related to 1-yr survival. The parallel combination of Pugh score and monoethylglycinexylidide test yielded the highest prognostic sensitivity (82%). The series approach combining either the Pugh score and indocyanine green test or the monoethylglycinexylidide and indocyanine green tests was associated with the highest specificity (96%/97%) and high predictive values of a positive result (81%/82%). These findings suggest that appropriate combinations of the studied flow-dependent dynamic liver function tests and the Pugh score could be useful in improving transplant candidate selection and the timing of transplantation.

Humans↗
PubMed1990

[The Alagille syndrome in an adult].

Severe bleeding from gastric varices occurred in an 18-year-old male known, since he was three years old, to have liver cirrhosis with beginning protal hypertension. The cause of the portal hypertension was chronic cholestasis due to hypoplasia of the interlobular bile ducts. There was also peripheral pulmonary stenosis with pulmonary hypertension (106 mmHg systolic), and a posterior embryotoxon (arcus juvenilis). Skeletal anomalies, particularly of the vertebrae, and a striking facial dysmorphism provided the features of arteriohepatic dysplasia, Alagille's syndrome, an autosomal dominant disease generally becoming manifest during childhood. As the patient's liver functions were only slightly abnormal, liver transplantation was not indicated and a shunt operation performed. A septicaemia developed on the third postoperative day after an at first complication free course, and he died from right-heart failure.

Abnormalities, Multiple↗
PubMed1990

Postoperative recovery of mitochondrial function of the human liver graft procured and preserved with University of Wisconsin (UW) solution.

Changes in arterial blood ketone body ratio (KBR) were investigated in 47 human liver transplantations. Of the 20 grafts preserved with University of Wisconsin (UW) solution, 10 had a cold preservation period of less than 10 h (UWS group) and 10 of more than 10 h (UWL group). In 27 other cases, grafts were preserved with EuroCollins (EC) solution for less than 10 h (EC group). In the EC group, KBR increased over 0.7 within 6 h after reperfusion of the graft in 17 cases (63%) and within 24 h in 7 cases (26%). In the 3 other cases, KBR failed to recover, and these patients underwent retransplantation. In the UW group, KBR recovered within 6 h in 13 cases (65%) and within 24 h in 7 cases (35%). There were no significant differences between the UWS and UWL groups. It is shown that the mitochondrial function of liver grafts preserved with UW solution can be well maintained even after extended preservation periods of more than 10 h.

Adenosine↗
PubMed1990

Lidocaine metabolite formation as a measure of liver function in patients with cirrhosis.

A method for rapid assessment of hepatic function in cirrhotics based on the formation of the lidocaine metabolite, monoethylglycinexylidide (MEGX), was evaluated. The formation kinetics and urinary excretion patterns of MEGX clearly distinguished cirrhotics (n = 12) from healthy volunteers (n = 16). In a prospective study, we compared the prognostic value of the MEGX test with that of traditional parameters in transplant candidates. Patients who underwent transplantation during follow-up were excluded. The study included 58 adult patients with biopsy-proven posthepatitic or biliary cirrhosis. During the follow-up period of 120 days, 10 of 58 patients died of their liver disease. At the time of inclusion, we recorded MEGX formation, indocyanine green (ICG) half-life, caffeine clearance, and the Child-Pugh score. These variables were subjected as covariates to a survival analysis (Cox proportional hazards regression model). The results of the MEGX and the ICG test were significantly related to the 120-day survival. In the stepwise analysis, none of the parameters evaluated contributed to a further significant improvement of our predictive ability when added to the values of ICG (improvement: p less than 0.0005) and MEGX (improvement: p less than 0.0005). These findings suggest that the ICG and MEGX tests were the best short-term prognostic indicators. The easy handling favors the MEGX test over the ICG test as a tool for assessment of hepatic function and short-term prognosis in transplant candidates with cirrhosis.

Adult↗
PubMed1990

Significance of arterial ketone body ratio measurement in human liver transplantation.

Arterial ketone body ratio (KBR), which reflects the NAD+/NADH ratio of hepatic mitochondria, was measured sequentially in 39 liver transplantations. In 22 cases, KBR was increased to above 0.7 within 6 hr after reperfusion (group A). In 11 cases, restoration of KBR was delayed until the first postoperative day (group B) and in 6 cases, KBR failed to recover (group C). The patients in group A survived liver transplantation without complications. By contrast, morbidity and mortality were significantly higher in groups B and C. In 2 cases in group C, the livers were clinically diagnosed as initially nonfunctioning grafts and the patients underwent retransplantation. Another two died of hepatic failure soon after the operation. It is suggested that delayed recovery of KBR is an early indicator of metabolic overload in the liver allograft, and that a delay exceeding 24 hr may imply the need for retransplantation.

Adult↗
PubMed1990

Experience with partial liver transplantation in Hannover.

Within a 7-year period 37 PLTx have been performed in 32 patients (25 children, 7 adults). Age at the time of operation was 5 years in two-thirds of all cases (23 of 37). Indications were elective in 17 patients and urgent in 20. The following types of PLTx were used: a LLL in 24 cases, a LL in 9, and a RL in 4. Three donor livers were split and transplanted into 2 recipients each. The body weight ratio between donors and recipients ranged from 0.9:1 to 12.3:1. Nineteen of 26 adult-donor livers were transplanted into pediatric recipients. Patient and graft survival following elective PLTx at 2 years was 69.7% and 50.5%, respectively, which was not significantly different from the results following urgent transplantation. Overall, 30-day mortality was 18.8% (6 of 32 patients) and was relative to the patients' clinical status at the time of transplantation. Graft loss in 29.7% (11 of 37 transplants) was due to liver-related causes such as chronic rejection (n = 5) and primary nonfunction (n = 4). Postoperative surgical complications were noted in 13 cases but were not different from those seen with WLTx. At present, 19 of 32 recipients (59.4%) are alive between 1 and 43 months following transplantation.

Adolescent↗
PubMed1989

Lignocaine metabolite formation as a measure of pre-transplant liver function.

A method for rapid assessment of hepatic function in liver donors based on the formation of the lignocaine metabolite monoethylglycinexylidide (MEGX), was used in a prospective study of 69 donor-recipient pairs. The probability of graft survival over 120 days was significantly higher for livers from donors with MEGX test values above 90 micrograms/l than for those from donors with MEGX values of 90 micrograms/l or below. Other liver function tests (bilirubin, prothrombin time, activity of aminotransferases, glutamate dehydrogenase, and cholinesterase, indocyanine green clearance, and galactose elimination capacity) were inefficient at predicting early outcome of transplantation. For a 20-day graft survival, the MEGX test showed prognostic sensitivity of 73% and specificity of 78%. These findings suggest that the MEGX formation test could be valuable for selection of donor organs.

Adolescent↗
PubMed1989

[Clinical syndromes with liver and kidney involvement in children and adults].

Multiple pathogenetic mechanisms can lead to dysfunctions or malformations of the liver and kidneys in children and adults. The association of renal and hepatic abnormalities can be found in different congenital malformation syndromes. Hereditary metabolic disorders are capable of alternating liver and kidney function. Immunologic, toxic and septic diseases may damage both organ systems. In patients with liver cirrhosis, both glomerular and tubular dysfunctions can be observed. In the course of liver transplantations, an increased rate of renal dysfunction was observed. This survey summarizes the main clinical syndromes of renal involvement associated with liver disease.

Adult↗