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Biomedical subjects

R Pichlmayr

Publications and source records attributed to R Pichlmayr.

At least 307 records · Page 17Linked 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

Hemostatic studies of ex situ hepatic surgery.

Ex situ hepatic surgery, in which a diseased liver is resected from outside the body, was first reported in 1988. This study investigates the hemostatic changes occurring during such surgery in two cases. During the anhepatic period of more than 5 hours, veno-venous bypass without heparin was performed. The tests included platelet count, prothrombin activity (PT), partial thromboplastin time (PTT), fibrinogen (Fbg), factor II (F.II), factor V (F.V), and thromboelastography (TEG). Three to 4 hours after entering the anhepatic phase, marked fibrinolysis and a fall in the values of PT, Fbg and F.V. were observed. Every parameter temporarily deteriorated immediately after revascularization of the graft, however, all returned to almost normal values within 1-2 hours after hepatic reperfusion except for F.V and platelets. In conclusion, the coagulopathy during ex situ hepatic surgery is caused by the marked fibrinolysis and depletion of hemostatic factors which develop 3-4 hours after the onset of the anhepatic phase.

Adult↗
PubMed1991

[Fatty emulsions in parenteral feeding following liver transplantation. A study of the neurotropic effect of MCT/LCT emulsions using EEG].

In the early postoperative period after liver transplantation a possible neurotropic side effect of middle-chain triglycerides used for total parenteral nutrition (TPN) was evaluated by EEG and Glasgow Coma Scale. Group I: no fat for TPN; Group II: 0.7 g/kg body weight (BW) and day, of MCT/LCT emulsion; Group III: 1.5 g/kg BW and day, of MCT/LCT emulsion. Only on postoperative day 5/6 significant differences in distribution of background activity in the EEG were seen between Group I and II. Patients in Group I showed more pathological EEG patterns (Mann-Whitney-U-test p less than 0.05). Per discriminant analysis an influence of applicated fatty acids on the registered background activity could be excluded. Instead an underlying rejection of the graft in 7 of 10 patients could be made responsible for deterioration of the EEG pattern. Following the presented data a negative neurotropic effect of MCT/LCT emulsions in the described dosages can be negated.

Adult↗
PubMed1991

Coagulation disorder during liver transplantation.

Detailed haemostatic changes were investigated during eight liver transplantations. The patients were divided into two groups; group 1 had minor operative bleeding (four cases) and group 2 had major bleeding (four cases). Group 2 had lower levels of platelets, fibrinogen, factor V (FV), and alpha 2-antiplasmin than group 1, and the thromboelastography showed fibrinolysis. In both groups, plasma tissue-plasminogen activator levels rose slightly. After revascularization of the graft liver, reductions in the values of PT, fibrinogen, FV and FVII were noted, along with a prolongation of the PTT and an increase in thrombin-antithrombin III complex levels. Plasma levels of protein C, protein S, antithrombin III, and plasminogen remained relatively stable throughout the operation. These results show that the preceding fibrinolysis and subsequent superimposed activation of the clotting system are the main causes of coagulopathy during liver transplantation, which correlate with the amount of operative haemorrhage and the abnormalities found in haemostatic tests.

Adult↗
PubMed1991

Differentiation of liver graft dysfunction by transplant aspiration cytology.

Episodes of graft dysfunction are frequently observed after liver transplantation and can be due to different causes requiring specific therapy. In this study the usefulness and reliability of liver transplant aspiration cytology (TAC) for differential diagnosis of liver graft dysfunction is assessed. Out of more than 1500 TACs performed, 292 TACs, taken during episodes of liver dysfunction due to retrospectively defined causes, were analyzed. Immune activation and parenchymal damage in the aspirates were determined cytologically. In 63 episodes of acute rejection, marked immune activation was present in aspirate but not in blood, with varying degrees of hepatocyte damage and cholestasis. No or only minimal immune activation was observed in 86 cases of toxic, ischemic, or septic liver damage, but considerable parenchymal damage and cholestasis were observed. In 3 cases of hepatitis slight-to-moderate immune activation with large granular lymphocytes was found in the aspirate, while 17 cases of viral infection presented with slight-to-moderate immune activation in aspirate and blood. After successful treatment the cytologic patterns normalized, except when the cause of liver dysfunction persisted. Moreover, typical patterns of parenchymal changes were found for preservation damage of the liver (n = 108), fatty degeneration (n = 3), obstructive cholestasis (n = 5), and acute arterial ischemia (n = 2). One case of moderate subcapsular hematoma was the only complication observed (less than 0.1%). Thus, liver TAC is an easy, safe, and clinically useful method for differential diagnosis of liver graft dysfunction. In particular, differentiation between acute rejection and nonimmunologic causes of dysfunction is very reliable, but hepatitis and viral infections also present distinctive patterns in liver TAC.

Biopsy, Needle↗
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

Does orthotopic liver transplantation heal Wilson's disease? Clinical follow-up of two liver-transplanted patients.

Two patients with Wilson's disease (WD) underwent orthotopic liver transplantation, one for subacute liver failure and the other for severe oesophageal haemorrhage. After transplantation both patients fully recovered within five months, and copper metabolism returned to normal. Follow-up examinations were continued for 4 and 6 years. Clinical as well as electrophysiological testing in these two patients yielded better results than in most of 12 WD-patients being conventionally treated for a similar period or even longer.

Adult↗
PubMed1991

[Pregnancy following organ transplantation].

Pregnancies following organ transplantation are high-risk pregnancies for mother and fetus. There are many reports concerning pregnancies following renal transplantation whereas only few reports are published about pregnancies in recipients of liver, heart and bone marrow transplants. During the pregnancy after kidney transplantation pregnancy-induced hypertension can develop or the renal function can decrease. Risks for the fetus are prematurity, growth retardation and prenatal infections. A higher incidence of complications for mother and fetus is observed in patients with renal insufficiency or severe hypertension prior to conception. The risks can be reduced by an intradisciplinary cooperation of gynecologist, transplant specialist and paediatrician. The paediatrician. The experiences with pregnancies in women after organ transplantations in the Medical School of Hannover are analyzed and compared with the reports in the literature. The management of these patients is described on current state of knowledge.

Female↗
PubMed1991

[The diagnostic value of quantitative Doppler ultrasound signal analysis in diagnosis of postoperative organ dysfunction following orthotopic liver transplantation].

As hepatic transplantation becomes more successful, there is increasing demand on noninvasive methods for studying graft dysfunction during the postoperative period. Duplexsonography now is a routine investigation with the possibility of quantitative description of changes in hepatic blood flow during graft dysfunction. Serial quantitative doppler measurements of hepatic artery, portal vein and hepatic veins were obtained in 20 adult patients during hospitalisation after transplantation and retrospective correlated to clinical datas. The resistive index of hepatic artery was maximal elevated in the initial period after graft reperfusion. There was no correlation with acute rejection. The angle independent damping index (minimum frequency shift/maximum frequency shift) calculated for portal- and hepatic vein showed significant changes during acute rejection, chronic rejection and cholangitis.

Graft Rejection↗
PubMed1991

[Preventive operations in transplantation surgery].

Historically, there are three phases of development: initially, prophylactic operations in transplant candidates were performed frequently, particularly to minimize complications of infection and stress ulceration. As a consequence of advanced experience and reduced immuno-suppression, the need for those operations declined. Today, by extending transplant indications to elderly patients and to more complicated situations, prophylactic surgery-particularly for concomitant cardiovascular disease-plays an increasing role. Indications for prophylactic surgery are also provided in endocrinal, oncologic and immunologic conditions.

Humans↗
PubMed1991

[Liver transplantation. The Hannover experience].

Since 1972, 610 liver transplantations have been performed in 520 patients in Hannover, including 98 children from the age of 9 months. In 146 cases, the indications were nonresectable malignant tumors, in 274 cases benign diseases, mainly terminal cirrhoses of various etiologies. The results have been considerably improved, with the recipient's general condition being the key factor of the prognosis: in elective transplantations, the survival rate after one year is 90%, while in complicated or emergent cases, it is only 40-60%. Thus early surgery plays an essential part. The quality of life following liver transplantation is quite satisfactory, and the patients are very happy as a rule. Recurrence of the initial disease reduces the quality of life. Recurrence of malignant tumors, in particular, is frequent. Considerable progress was made in the surgical technique, including transplantation of a reduced liver in children, division of a donor liver in 2 parts, hepatectomy and maintenance of the liver-deprived condition during several hours to achieve the stabilization of hemodynamics, transplantation of auxiliary livers and finally, liver surgery off-site. Thus the accumulation of many elements of experience in liver transplantation has allowed developing new surgical techniques. Like for any organ transplantation, the ethical and economical issues must be constantly kept in mind.

Germany↗