Orthotopic liver transplantation in the management of end stage liver disease: the University of Vienna experience.

Two hundred and eight orthotopic liver transplantations (OLT) were performed in 191 patients at the I Department of Surgery, University of Vienna from 1982-1990. The most frequent indications were hepatocellular carcinoma, alcoholic cirrhosis, posthepatic cirrhosis, primary biliary cirrhosis, and fulminant hepatic failure. Patients with malignancy constituted 33.8% of cases. The overall results showed a 64% one-year and 58% two-year survival; best results were seen in patients with primary biliary cirrhosis and the poorest long-term results were in malignancy. There were 23 postoperative deaths (11%). Primary non-function was seen in 14 (7%) cases; acute rejection episodes were seen in 62% of patients. The presence of a well organised cadaver organ procurement system in eastern Austria with upto 41 donors per million population per year ensures that the 57% growth rate in OLT achieved in 1990 will be maintained with even better results.     

Evolving trends in mechanical circulatory support: Clinical development of a fully magnetically levitated durable ventricular assist device

Early generation left ventricular assist devices (LVAD) for treatment of refractory heart failure attempted to mimic the pulsatility of a native heart and were bulky and less durable due to valves within the inflow and outflow pathways. The next disruption came with the introduction of continuous flow pumps characterized by a low-pulse pressure circulation. Since the mechanism of action did not involve displacement of blood, these pumps were much smaller and less noisy in part due to fewer moving parts. Such devices include the HeartMate II axial-flow pathway pump which is implanted thoraco-abdominally and the HVAD, a centrifugal-flow pathway pump which is smaller and is implanted entirely within the thoracic cavity and uses a combination of hydrodynamic and magnetic levitation of the internal rotor. These pumps improved survival compared with the early generation LVADs and in a trial, the HVAD demonstrated non-inferiority compared with the HeartMate II but with an increase in ischemic and hemorrhagic strokes. The HeartMate 3 LVAD is an intrapericardial centrifugal-flow pathway pump with a full magnetically levitated, frictionless rotor with a fixed intrinsic pulse. In a randomized trial, the HeartMate 3 “hybrid” pump was associated with near-elimination of pump thrombosis, a reduction in strokes of any type or severity and lower mucosal bleeding rates. Despite improvements in hemocompatibility, right heart failure and infections contribute to significant morbidity, and devices designed to be internally contained with a wireless power source and physiological flow characteristics are still required despite great strides in the field.     

Extracorporeal bypass using a centrifugal pump during resection of malignant liver tumors.

BACKGROUND/AIMS: Total hepatic vascular exclusion (THVE) during extracorporeal bypass is used for hepatic resection in patients with malignant liver tumors. The aim of this study was to determine the efficacy of hepatectomy during total hepatic vascular exclusion using a centrifugal pump (Bio-pump). METHODOLOGY: Fourteen patients with malignant liver tumors who underwent hepatectomy during total hepatic vascular exclusion using the Bio-pump were studied retrospectively. RESULTS: In 3 of 14 patients, insufficient hepatic vascular exclusion was achieved. Six patients underwent tumor resection during total hepatic vascular exclusion, without extracorporeal bypass. In the remaining 5 patients, flow exclusion averaging 1500 ml was achieved with the Bio-pump, and hepatectomy was performed during the procedure. In these 5 patients, the mean operative time and blood loss were 11 hours 38 minutes and 6850 +/- 2451 ml. The Bio-pump bypass time, the excluded blood flow and the mean blood pressure were 82 minutes, 1650 ml and 108/53 mmHg, respectively. The arterial ketone body ratio (AKBR) decreased from a pre-operative value of 1.85-0.32 during total hepatic vascular exclusion. CONCLUSIONS: Total hepatic vascular exclusion was useful for hepatectomy in patients with tumor invasion into the hepatic vein and inferior vena cava, or tumor thrombus in the inferior vena cava and right atrium. However, this technique did not decrease blood loss or improve outcome in patients undergoing hepatectomy.     

Electron-beam therapy for mycosis fungoides: the Stanford University experience.

The use of high-dose electron-beam therapy for mycosis fungoides at Stanford University is reviewed. Since 1966, 140 patients have been treated in this fashion. Their clinical characteristics including initial extent of disease are reviewed. The results of routine staging studies are examined. Eighty-four percent of the patients achieved an initial complete remission; this was inversely related to the initial extent of skin involvement. The overall survival rate was 46% at 10 years with the major prognostic factor being the initial extent of skin involvement. Other factors which had an influence on the patient's progrnosis included the presence of palpable adenopathy, the patient's age, the achieving of an initial complete remission, the initial dose of electron-beam therapy, and the treatment with adjuvant topical mechlorethamine. The development of a rationale for the treatment of patients with all stages of mycosis fungoides based upon these clincal observations is discussed.     

Hyperferritinemia in the Chinese and Asian community: a retrospective review of the University of British Columbia experience.

BACKGROUND AND METHODS: Elevated serum ferritin is a common clinical finding. The etiology of hyperferritinemia in the Asia-Pacific population is less clear due to a low prevalence of known HFE mutations such as C282Y and H63D, as well as an increased prevalence of viral hepatitis and hereditary anemia. A retrospective case review of 80 patients of Asian ethnicity referred to three subspecialists in tertiary care teaching hospitals between January 1997 and March 2005 for assessment of hyperferritinemia was performed. RESULTS: Only four patients (5%) had iron overload on liver biopsy or quantitative phlebotomy. Forty-nine patients (61%) had secondary causes for their hyperferritinemia, of which 26 had liver disease; 16 of those patients also had viral hepatitis. Thirteen patients fulfilled criteria for the insulin resistance syndrome. Other causes included hematological disorders (n=10), malignancy (n=2) and inflammatory arthritis (n=2). Twenty-seven cases (34%) of unexplained hyperferritinemia were found. Of a total of 22 patients who underwent liver biopsy, significant iron deposition was found in one patient. Fifteen patients underwent C282Y and H63D genotyping, with two cases of H63D heterozygosity. Fourteen patients had first-degree relatives with hyperferritinemia. Three families were identified with more than two members affected, which is suggestive of a possible hereditary hyperferritinemia syndrome. CONCLUSION: Secondary causes of elevated ferritin in the Asian population, particularly liver disease, are common, but primary iron overload syndromes appear to be rare. In a significant proportion of patients, the etiology remains unexplained. The genetic basis for hyperferritinemia in Asians is poorly defined and requires further study.     

Therapeutic leukapheresis: 9-year experience in a University Hospital

Background

Hyperleucocytosis is associated with higher morbidity and mortality related to possible development of leucostasis, tumour lysis syndrome and/or disseminated intravascular coagulation. There is insufficient evidence of the need for leukocytapheresis during early treatment of hyperleucocytosis, and its efficiency remains controversial, although leucoreduction is a measure that can prevent adverse events and death. The aim of this study was to analyse the safety and effectiveness of therapeutic leukocytapheresis and its influence on early mortality in our case series, adjusted to independent mortality risk factors described in the literature.

Materials and methods

This was a retrospective review (June 2003–June 2012) of procedures carried out for the treatment of hyperleucocytosis at the Haematology and Haemotherapy Service of Miguel Servet University Hospital. The patients’ data and technical information were prospectively registered for each leukocytapheresis session.

Results

Thirteen patients underwent a total of 27 leukocytapheresis procedures. After an average of two sessions, a statistically significant drop in the initial leucocyte counts was observed (p<0.01), as well as a relevant drop in lactate dehydrogenase levels. The only analytical value statistically related to early mortality in univariate analysis was initial creatinine level greater than 1.2 mg/dL (p=0.012, OR=2.5).

Discussion

Despite the small size and limited homogeneity of our case series, we can conclude that leukocytapheresis is a safe and effective therapeutic measure for leucoreduction in haematological pathologies of any lineage, particularly in patients without acute myeloid leukaemia. Patients with acute myeloid leukaemia had worse outcomes within 6 months of having finished leukocytapheresis sessions, as well as in terms of mean global survival and mean time of mortality. However, global mortality rates were similar in patients with or without acute myeloid leukaemia.     

Group G streptococcal bacteremia. The clinical experience at Boston University Medical Center and a critical review of the literature

Twenty-six patients with bacteremia caused by group G streptococci were seen during a 29-month period. Our findings suggest that the epidemiology of these infections has not changed appreciably during the last 15 years. The disease occurs most commonly as an acute community-acquired infection in older persons with underlying conditions predisposing to skin breakdown. Patients with these bacteremias fall into three categories: those with associated cutaneous infection only; those with serious focal infection; and those with probable infection. Underlying neoplastic conditions were relatively uncommon (25%) in our series compared with others (65%). In contrast to recent reports emphasizing this complication, endocarditis was uncommon in our patients. A prompt response to therapy with beta-lactam antibiotics occurred in most of our patients.