Long-term outcome of liver transplantation in HCV/HIV coinfected haemophilia patients. A single centre study of 10 patients.

UNLABELLED: The outcome and clinical features during long term follow-up of 10 haemophilia patients (haemophilia A n = 9, haemophilia B n = 1), who underwent successful orthotopic liver transplantation (OLT) due to hepatitis associated liver disease, are summarised. PATIENTS: Eight patients were HIV/HCV co-infected. Despite severe postoperative complications, which were not bleeding-associated, all patients survived OLT. RESULTS: Long-term survival was 70% after in mean 8 years follow-up. Twelve years after OLT one patient developed a cyclosporine-induced nephropathy requiring haemodialysis. HIV-HAART was initiated in all patients after OLT, and allowed a successful HCV treatment in 6 patients. Factor VIII production was sufficient in mean 72 h after OLT and remained stable at subnormal to normal FVIII levels of in median 30% (range 14-96%) also during long-term follow-up. Post-OLT spontaneous bleeding events were rare compared to pre-OLT, therefore, the performance status improved in all patients. DISCUSSION: OLT substitutes the hepatic FVIII but has no effect on the extra-hepatic endothelial FVIII production, suggesting that in case of severe tissue injury enhanced bleeding might occur. Additionally, after OLT there is no acute phase reaction of the FVIII protein. Therefore, our OLT patients received in case of a reduced FVIII activity a peri-interventional prophylactic short-term FVIII substitution in surgical and diagnostic interventions with high bleeding risk. CONCLUSION: Bleeding and wound healing disturbances were not seen.

[Primary neuroendocrine carcinoma of the liver. From carcinoid tumor to small-cell hepatic carcinoma: case reports and review of the literature]. / Primäre neuroendokrine Karzinome der Leber: Vom Karzinoidtumor bis zum kleinzelligen Leberkarzinom: Fallberichte und Literaturübersicht.

Primary hepatic neuroendocrine tumors are rare neoplasms. While primary hepatic carcinoid tumors (PHCT) are well-differentiated tumors, primary hepatic small-cell carcinomas (PHSCC) represent the poorly differentiated end of the spectrum of neuroendocrine carcinomas. The first patient, suffering from PHCT, has had a follow-up for 32 years and is still alive. Within this time, the tumor relapsed 4 times with unchanged histology and immunohistochemistry features. The second patient suffered from small-cell carcinoma of the liver. There were no risk factors for a hepatocellular carcinoma. An extensive preoperative and postoperative diagnostic investigation could rule out an extrahepatic primary site. Immunohistochemically the tumor was negative for Hepar-1, AFP, TTF1 and CDX2 but reacted positively with CD56 and sporadically with the keratins 8, 18 and 20. A neuroendocrine PHSCC was diagnosed. After neoadjuvant cytostatic treatment the carcinoma was completely extirpated and 18 months after treatment the patient is healthy.PHCT and PHSCC have to be clearly separated from hepatocellular and cholangiocellular carcinomas. Exclusion of an extrahepatic primary site requires an accurate and synoptic analysis of clinical, radiologic and pathologic findings. Surgical resection is the treatment of choice.

Biliary metal stents and air embolism: a note of caution.

Metal stents are a valuable treatment modality for patients with biliary obstruction. However, we present here two patients whose cases may serve as a warning about an unusual complication associated with these stents. We encountered this complication after endoscopic retrograde cholangiography for obstructed metal biliary stents. The first patient, an 87-year-old man with a benign biliary stricture, failed to regain consciousness after clearing of his stent using a Dormia basket and balloon catheter. Cerebral air embolism was diagnosed on cerebral computed tomography, and transesophageal echocardiography revealed a patent foramen ovale as a precipitating factor for paradoxical air embolism. He survived and was discharged with a residual hemiparesis. In the second patient, a 54-year-old man who had a history of a Billroth II operation and chronic pancreatitis and who had a portal cavernoma with biliary obstruction due to collateral veins, electromechanical dissociation complicated the balloon-catheter stent revision. Echocardiography performed during cardiopulmonary resuscitation showed major air embolism to the right heart. The patient died. These cases demonstrate that air may gain access to the venous system during therapeutic endoscopic procedures of this type. It is likely that the large diameter of metal stents and the potential for these stents to lacerate venous structures facilitate the entry of air into the venous circulation, an event which may have life-threatening consequences.

[Budd-Chiari syndrome in a patient with paroxysmal nocturnal haemoglobinuria]. / Budd-Chiari-Syndrom bei einem Patienten mit paroxysmaler nächtlicher Hämoglobinurie.

HISTORY AND CLINICAL FINDINGS: A 61-year-old man with dyspnea and diffuse abdominal pain due to increasing ascites caused by liver cirrhosis of unknown etiology was admitted for consideration of transjugular intrahepatic portosystemic stent-shunting (TIPSS). The patient's medical history included paroxysmal nocturnal hemoglobinuria (PNH), presenting as slight hemolysis diagnosed 24 years previously. One year before the patient underwent radical retropubic prostatectomy for a localized prostate cancer. Shortly after this intervention he developed ascites. INVESTIGATIONS: Color Doppler ultrasonography revealed an abnormal flow in the major hepatic veins. Transjugular liver biopsy indicated hepatic a circulatory disorder. Hepatic venography revealed the so-called "spider web" pattern characteristic for the Budd-Chiari syndrome. The hypercoagulable state due to paroxysmal nocturnal hemoglobinuria was accentuated by manipulation on the prostate during prostatectomy and presumably resulted in a thrombotic obstruction of the hepatic veins. TREATMENT AND CLINICAL COURSE: After exclusion of contraindications a transjugular intrahepatic portosystemic stent shunt (TIPSS) was performed, which led to a decrease of portal pressure. Signs of portal hypertension such as esophageal varices and ascites resolved completely. The patient has been free of complaints for one year. CONCLUSION: We assume that a hypercoagulopathy due to asymptomatic paroxysmal nocturnal hemoglobinuria resulted in Budd-Chiari syndrome when boosted by postoperative release of procoagulation factors in the thrombokinase-rich prostate. TIPSS is a therapeutic option in these patients.