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Ein 21-jähriger Patient entwickelte ein septisches Krankheitsbild mit multiplen intraabdominellen Abzessen und einem Multiorganversagen. Als Grunderkrankung bestand eine septische Granulomatose, ein angeborener Defekt der Granulozytenfunktion mit intrazellulär verminderter Produktion von Sauerstoffradikalen. Trotz chirurgischer Sanierung und einer kalkulierten antibiotischen und antimykotischen Therapie verstirbt der Patient nach einigen Tagen im septischen Schock. Dieser Fallbericht schildert typische Probleme eines Patienten mit angeborenem Immundefekt, der aus der Betreuung durch hochspezialisierte, pädiatrisch-immunologische Abteilungen herausgewachsen ist. A 21-year-old male patient developed an acute septic clinical picture with intra-abdominal abscesses and multiorgan failure. The underlying disease was chronic granulomatous disease, an inherited disorder of granulocyte function caused by failure of intracellular superoxide production. In spite of surgical procedures and a calculated antibiotic and antimycotic therapy, the patient died within a few days from septic shock. This case report describes the typical problems of patients with a congenital immunodeficiency who grow out of the care of highly specialised pediatric-immunological departments.

Previous studies reported on both visible and invisible particles in University of Wisconsin (UW) solutions. Those particles originated from components of the bags. In recent clinical observations we noticed macroscopically visible, indissoluble particles in UW bags reaching subzero temperatures during transportation of organs and preservation solutions. In an experimental model we examined whether those particles could be detected following perfusion of abdominal organs with established perfusion solutions. UW-, HTK- or physiological saline solutions reached - 3 ± 0.5 °C under conditions frequently applied during transportation. UW solutions demonstrated the accumulation of visible, indissoluble crystals and were subsequently used for the perfusion of abdominal organs in LEW rats. After perfusion with UW solutions stored at freezing temperatures, crystals were detected in all abdominal organs localized in and around vessels, bile ducts, glomeruli and in the interstitium of harvested livers, kidneys and pancreas. By spectroscopy, we were able to characterize crystals as adenosine. A 40-μm pore-size filter eliminated crystals from UW solutions. Crystals were absent in organs perfused with HTK- or saline solutions kept at subzero conditions. UW solutions can reach subzero temperatures under commonly used transportation conditions. Under these conditions, visible crystals accumulate and can be detected in abdominal organs of an experimental system.

Carcinogenesis and Surgical Therapy of Hepatocellular Carcinoma in Viral Hepatitis and Liver Cirrhosis Chronic viral hepatitis B or C is one of the main risk factors of hepatocarcinogenesis. The exact mechanisms have not been unravelled yet, but the process is thought to be based on virusspecific factors in combination with genomic instability. The latter is based on hepatocyte necrosis/apoptosis with consecutive inflammatory response and increased proliferation during chronic hepatits. Specific viral factors that are thought to be involved are the hepatitis B x (HBx) protein in case of HBV and the hepatitis C (HCV) core protein. Under certain circumstances, both can increase hepatocyte proliferation and have anti-apoptotic effects. In addition, both can inhibit the function of tumor suppressor genes likep53. In the treatment of hepatocellular carcinoma one has to distinguish patients with cirrhosis of the liver from patients without. Patients without cirrhosis should, as far as possible, undergo liver resection. In contrast, in patients with cirrhosis, liver function is the limiting factor for resections. The result is a relatively high postoperative mortality rate, which in most studies in case of viral cirrhosis is increased compared to nonviral cirrhosis. In addition, patients with posthepatitic cirrhosis show a relatively high percentage of multicentric hepatocellular carcinomas. Because of the increased postoperative mortality and high recurrence rates the survival rates of patients with viral cirrhosis are impaired in comparison with nonviral cirrhotic patients. But in several studies it has been shown that, with careful preoperative evaluation, an acceptable postoperative mortality rate and 5-year survival rates of approximately 50% can be achieved. The results after liver transplantation in selected patients with posthepatitic cirrhosis and HCC are slightly better; in recent studies 5-year survival rates of 60–80% were reported. Therefore, liver transplantation of selected HCC patients with viral cirrhosis should be performed if there are no contraindications. Local ablative methods should at present only be performed if surgery (resection or transplantation) is contraindicated; in the future, long-term results of local therapy remain to be awaited.

Surgical Therapy of Precancerous Lesions and Early Carcinomas of the Liver The only potentially curative treatment for liver malignancy is still limited to surgical procedures, i.e. liver resection or transplantation. Since the tumors are often discovered in advanced stages, identification of risk groups with precancerous lesions is of crucial importance. A careful screening allows an early diagnosis of malignancy with an improved long-term prognosis. Dysplastic lesions in liver cirrhosis and other precancerous conditions like hereditary hemochromatosis and liver adenoma increase the risk for the development of hepatocellular carcinoma (HCC). The prevalence of cholangiocellular carcinoma (CCC) is significantly higher in patients with primary sclerosing cholangitis (PSC) and polycystic liver disease. New imaging techniques allow a highly specific discrimination of benign lesions; histopathological evidence of liver cell dysplasia may also be helpful. Due to standardized patient selection, the results of liver resection for HCC have improved especially in non-cirrhotic patients and in patients with tumors ≤3 cm. Thus, 5-year survival rates over 50% can be achieved. Liver resection for adenoma and unilobar Caroli’s disease has an excellent prognosis, whereas only 20% of patients with CCC are still alive after 5 years. Liver transplantation is a treatment option for small HCCs in cirrhosis, since beside the curative intention the underlying disease is also cured. For solitary tumors <5 cm or up to three lesions <3 cm, 5-year survival rates are between 58 and 80%. PSC, bilobar Caroli’s disease and hereditary metabolic diseases as facultative precancerous disorders have even better results. Survival rates of CCC after transplantation have also increased due to new neoadjuvant treatment options and limitation to early stages. The scarce donor organ resources, however, limit the availability of liver transplantation as a treatment option for many patients, and tumor progress of those on the waiting list remains a major problem. Living-donor liver transplantation may prove to be very beneficial in some of these patients.

Zusammenfassung.: Einleitung: Bei Patienten mit einer terminalen Leber- und Nierenerkrankung ist die kombinierte Transplantation dieser Organe die einzig mgliche Therapie. Hierbei ist eine Differenzierung zwischen chronischer und akuter Niereninsuffizienz wichtig, denn akute Nierenversagen sind nach erfolgreicher Lebertransplantation hufig reversibel. In dieser Studie untersuchten wir die Indikationen und Ergebnisse dieses Verfahrens. Patienten und Methoden : Wir berichten retrospektiv ber 27 kombinierte Leber- und Nierentransplantationen. Die zugrundeliegenden Erkrankungen waren Virushepatitiden (n = 12), polycystische Leber- und Nierenerkrankungen (n = 9), primre Hyperoxalurie (n = 4) und kryptogene Lebercirrhose (n = 2) mit terminaler Niereninsuffizienz bei Glomerulonephritis, diabetischer Nephropathie oder chronischer Cyclosporintoxizitt nach Lebertransplantation und Transplantatinsuffizienz. Neun Patienten hatten vor Transplantation positive lymphocytotoxische Antikrper und 5 Patienten wurden bei einem positiven Crossmatch transplantiert. Ergebnisse: Ein Patient verstarb an rezidivierenden intraabdominellen Blutungen, 2 Patienten verloren das Nierentransplantat frh-postoperativ aufgrund einer initialen Nichtfunktion und technischer Probleme. Keiner der Patienten mit einem positiven Crossmatch oder lymphocytotoxischen Antikrpern entwickelte eine hyperakute Abstoung des Nierentransplantats. Die Incidenz akuter und steroidresistenter Abstoungen des Lebertransplantats war 60 % und 20 % bei Patienten mit einem positiven und 32 % und 14 % bei Patienten mit einem negativen Crossmatch. Nur 2 Patienten hatten eine akute Rejektion des Nierentransplantats (3,7 %). Das Langzeitpatienten- und Transplantatberleben war bei Patienten mit einem positiven Crossmatch nicht beeintrchtigt. Das aktuelle 1- und 5-Jahresberleben der kombiniert transplantierten Patienten betrug 97 % und 93 % verglichen mit 91 % und 83 % bei nur lebertransplantierten Patienten. Schlussfolgerungen: Die kombinierte Leber- und Nierentransplantation ist ein sicheres Verfahren in der Therapie terminaler Leber- und Niereninsuffizienzen. Ein positives Crossmatch ist keine Kontraindikation fr das kombinierte Vorgehen.

Abstract: From September 1988 through April 1998, 1000 liver transplantations were performed on 911 patients. During the postoperative control examinations of 837 patients, we found 23 (2.74 %) with hepatic artery thromboses, 27 stenoses of the hepatic artery (3.22 %), and 6 aneurysms of the graft artery. Seventeen patients underwent retransplantation because of arterial complications. Depending on the clinical symptoms, we treated both the local situation as well as the resulting complications of inadequate arterial graft flow. The aneurysms were primarily treated surgically. The first choice of treatment of stenoses was balloon angioplasty. Early postoperative artery thromboses were also treated surgically by thrombectomy in selected cases. For the resulting biliary and local septic complications we preferred endoscopic and drainage procedures. Our clinical experiences have led us to find pretransplantation angiography recommendable, especially in the case of splanchnic artery stenoses, for bypassing from the aorta for arterial perfusion of the graft.

Abstract: Introduction: With the good prognosis associated with differentiated carcinoma, the morbidity and mortality of different surgical approaches are of crucial importance. Methods: At the Department of Surgery (Virchow Klinikum Berlin), 139 patients who underwent surgery for differentiated thyroid carcinoma between 1979 and 1994 were reviewed, focussing on postoperative complications. In 113 and 18 patients, respectively, primary and completion thyroidectomy was performed. In five patients, less than total thyroidectomy and in three patients only palliative surgery was carried out. We performed thyroidectomy without systematic lymphadenectomy (LAD) in 70 patients (51.1%). In 15 patients (10.8%), lymphadenectomy of the lateral compartment and, in 53 patients (38.1%), central LAD was performed. LAD did not significantly influence survival time in either follicular (n = 42) or papillary carcinoma (n = 97). Results: No patient died because of postoperative complications. Permanent laryngeal nerve palsy occurred in no patients after thyroidectomy without LAD, in one patient after central LAD (1.9%) and in one patient after lateral LAD (6.7%). Transient laryngeal nerve palsy was seen in ten patients [six (8.6%) after thyroidectomy only, two (3.7%) after central LAD and two (13.3%) after lateral LAD] (P = 0.19). Hypocalcemia was distributed equally within the LAD groups: total transient hypocalcemia could be recorded in 54 patients (38.8%), but permanent hypocalcemia occurred only in one patient (0.7%). Postoperative recovery was delayed in patients when a more radical approach was used (P = 0.03). Conclusion: The magnitude of the benefit of LAD in therapy for differentiated thyroid carcinoma is still controversial. This more radical approach is not necessarily accompanied, however, by higher morbidity and mortality.

Hepatic associated metabolic disorders represent 5% of the indications for orthotopic liver transplantation (OLTX) according to the European Liver Transplant Registry. We studied the outcome of this group at our institution after OLTX and combined liver/kidney transplantation. Between September 1988 and January 1997, 837 OLTXS were performed in 735 patients. Patient survival and graft function at 1 yr were 91.3 and 86%, respectively. Thirty‐nine OLTXs were performed in 38 patients (15 female/23 male, median age ± SD: 35 14 yr, range 4‐60 yr) due to liver associated metabolic disorders (4.7%). Indications included Wilson's disease (n = 14), α‐1‐anti‐trypsin‐deficiency (n = 7), hemochromatosis (n = 4), erythropoetic protoporphyria (n = 4), cystic fibrosis (n = 2), Crigler‐Najjar syndrome type I (n = 1), glycogenosis type I (n = 1), ornithine‐transcarbomylase‐deficiency (n = 1). In addition 4 patients suffering from primary hyperoxaluria type I received combined liver/kidney grafts. Survival rate the 1 yr after OLTX and combined OLTX/NTX was 91.8%. Twenty patients received cyclosporin A (55%) and 17 patients tacrolimus (45%) as primary immunosuppression. The mean follow‐up was 28.6 months (range 4‐73 months). Two patients with hemochromatosis died 1 and 3 months after OLTX, respectively, from Aspergillus sepsis followed by multiorgan‐failure. One patient died of malignant lymphoma 5 months after transplantation. One patient required retransplantation 2 months after OLTX following arterial thrombosis and ischemic type biliary lesion. One year after OLTX, all patients demonstrated good graft function, liver grafts (ALT 17.9 ± 13.6 IU/L, bilirubin 0.8±0.3.mg/dl, thromboplastin time 94±15%), and combined liver/kidney grafts (creatinine 2.4±1.4 mg/dl). OLTX, respectively combined OLTX/NTX, represent a successful therapy for hepatic associated metabolic disorders. Survival rates and graft function are similar to those in liver graft recipients for established indications at our institution. OLTX seems to be an excellent treatment for hepatic based therapy resistant neurological disorders.