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Splenomegaly in 2,505 patients at a large university medical center from 1913 to 1995. 1963 to 1995: 449 patients.

Splenomegaly was studied retrospectively at the University of California, San Francisco (UCSF), School of Medicine in 301 patients from 1963 to 1995 and compared with the UCSF service of the San Francisco General Hospital Medical Center (SFGH) in 148 patients from 1979 to 1994. The combined 449 patients were classified into several diagnostic groups and were studied by means of several clinical and laboratory associations. Hepatic disease in the percentage of patients at UCSF (with those at SFGH given in parentheses) was associated with splenomegaly in 29% (41%), hematologic disease, 32% (16%); infectious diseases, 16% (36%); congestive or inflammatory disease, 10% (4%); primary splenic disease, 6% (1%); other, 5% (1%); and cause unknown, 2% (1%). Massive splenomegaly occurred in 27% of the patients of the combined series, particularly in patients with hematologic diseases. The acquired immunodeficiency syndrome (AIDS) occurred in more than half of the patients with infectious diseases at SFGH and was four times frequent than in the patients at UCSF. The commonest diseases associated with splenomegaly were hematologic (lymphoma), hepatic (chronic liver disease), infectious diseases (AIDS and endocarditis), congestive (congestive heart failure), primary splenic (splenic vein thrombosis), and other (malignancy not metastatic to the spleen). In 11 patients with AIDS and massive splenomegaly, Mycobacterium avium complex occurred in 8 (73%). Splenectomy was performed in 117 patients (26%), primarily for hematologic amelioration. I conclude that for splenomegaly of unknown origin, the invasive procedure of choice for patients with hematologic associations may be a bone marrow biopsy; for hepatic association, a liver biopsy; and for infectious disease associations, a lymph node biopsy, before any consideration of a diagnostic splenectomy.

Acquired Immunodeficiency Syndrome↗

Gastrointestinal emergencies with marathon-type running: omental infarction with pancreatitis and liver failure with portal vein thrombosis.

This paper reports two patients with catastrophic complications after marathon-type running not hitherto documented. The first, who collapsed with acute abdominal pains, was found at surgery to have infarction of the omentum and later, after a second laparotomy, acute oedematous pancreatitis. The second patient, who collapsed semi-comatose with hyperthermia, developed disseminated intravascular coagulation, rhabdomyolysis, renal shutdown and progressive hepatic failure. With regular dialysis, his condition stabilised but liver function continued to decline, associated with thrombosis of the portal vein. The spectrum of potentially life threatening disorders includes upper gastrointestinal bleeding, haemorrhagic colitis and rarely infarction of the bowel. The present two cases provide further support for ischaemia being a major contributor to the gastrointestinal catastrophes of marathon-type running.

Acute Disease↗

Phase II study of Spherex (degradable starch microspheres) injected into the hepatic artery in conjunction with doxorubicin and cisplatin in the treatment of advanced-stage hepatocellular carcinoma: interim analysis.

Patients with advanced-stage unresectable hepatocellular carcinoma (HCC) were treated with intrahepatic arterial doxorubicin 30 mg/m2 plus escalating doses of cisplatin up to 100 mg/m2 in conjunction with rapid bolus injection of Spherex (degradable starch microspheres; Kabi Pharmacia, Lund, Sweden) into the hepatic artery, until slowing or reversal of blood flow. Treatments were repeated every 4 to 6 weeks until progression, or were continued indefinitely if there was disease stability or response. Thirty-five evaluable patients have so far been accrued to the study. Objective tumor responses have occurred in 22 patients (63%), of whom 20 had partial responses and two had complete responses. Four of the patients had reversal of tumor-induced portal vein thrombus. Toxicities included death, one patient (and a death of uncertain cause in an additional patient); hepatitis, two patients; pancreatitis, one patient; dyspnea/hypotension, two patients; and hepatic artery nontransient thrombosis in four patients. Six patients have survived 2 years and an additional 10 patients have survived 1 year. The addition of Spherex to intrahepatic arterial chemotherapy for advanced-stage HCC appears to be relatively safe and is well tolerated even in patients with portal vein thrombosis, which represent the majority of patients with advanced-stage HCC.

Antineoplastic Combined Chemotherapy Protocols↗

Aneurysm of the inferior vena cava: case report and review of the literature.

Aneurysms of the inferior vena cava (IVC) include a diverse group of anomalies with distinct anatomic and clinical characteristics. We report a diverticular aneurysm of the suprarenal IVC in a 45-year-old man who presented with extensive IVC and unilateral lower extremity thrombosis. A CT scan revealed an 8 cm smooth-walled mass containing thrombus to the right of the IVC and behind the second portion of the duodenum. At laparotomy a connection between the mass and the suprarenal IVC was established. Biopsy of the wall of the mass revealed vascular smooth muscle, thus establishing the diagnosis of a diverticular IVC aneurysm. MR angiography further disclosed interruption of the infrahepatic vena cava and return of renal vein flow via the azygous and hemiazygous veins. Twelve additional cases of IVC aneurysm are reviewed. A proposed classification of these aneurysms into four types consistent with their anatomic and embryologic characteristics is presented.

Aneurysm↗

The middle colic vein: an alternative source of portal inflow in orthotopic liver transplantation complicated by portal vein thrombosis.

BACKGROUND: Portal vein thrombosis (PVT) was previously considered a contraindication to orthotopic liver transplantation (OLT) since adequate portal blood supply is mandatory for graft function and patient survival. Improvements in surgical technique, however, have meant that this problem now can be circumvented in most instances. Nevertheless portal vein thrombosis remains an obstacle in OLT and is associated with increased incidence of primary non-function and long-term liver failure. METHODS: A 55-yr-old patient underwent OLT for secondary biliary cirrhosis associated with hepatitis C infection and complicated by long standing PVT. Involvement of the portal, mesenteric, and splenic veins prevented standard portal venous reconstruction. Portal inflow was accomplished by a side-to-end anastomosis between the middle colic vein and the donor portal vein. RESULTS: Hepatic reperfusion and subsequent liver function were excellent. Portal blood flow, as measured by color-enhanced Doppler ultrasound, was normal following surgery until discharge. The post-operative course was complicated by abdominal wound dehiscence and recurrent cytomegalovirus (CMV) infection. The patient was discharged in good clinical condition, with excellent liver function and patent portal vein 89 d after OLT. CONCLUSIONS: The middle colic vein is a novel, not previously described, source of portal venous inflow for OLT complicated by extensive splanchnic venous inflow thrombosis.

Anastomosis, Surgical↗

Long-term follow-up study of adult patients with non-cirrhotic obstruction of the portal system: comparison with cirrhotic patients.

Thirty-two patients with non-cirrhotic portal system obstruction and oesophageal varices of non-malignant etiology were recruited over 13 years. Diagnosis was based on the presence of oesophageal varices at endoscopy, minor alterations in liver function tests and liver histology, a low hepatic venous pressure gradient, and pertinent angiographic patterns. Twenty-three had portal vein thrombosis, nine had splenic vein thrombosis. Twenty-one had idiopathic portal vein obstruction, 11 had secondary obstruction. The outcome was compared with a group of 32 patients with cirrhosis and portal hypertension, matched for age, Child-Pugh class, previous history of gastrointestinal bleeding, and size of oesophageal varices. Patients with non-cirrhotic obstruction of the portal system were followed for up to 171 months (mean 94 months). During follow-up ten patients had gastrointestinal bleeding, and eight died (five of gastrointestinal bleeding). After 6 years of follow-up, the cumulative risk of gastrointestinal bleeding was 24%, the cumulative risk of death was 17%, and the cumulative risk of death from gastrointestinal bleeding was 14%. Cumulative probability of death by any cause and the probability of gastrointestinal bleeding were significantly lower in patients with non-cirrhotic obstruction of the portal system than in patients with cirrhosis comparable for liver function and portal hypertension (p = 0.04 for both). The cumulative probability of death by gastrointestinal bleeding was not significantly different. In conclusion, the prognosis for non-cirrhotic obstruction of the portal system is significantly better than for patients with cirrhosis with comparable levels of liver function impairment and severity of portal hypertension.

Adult↗

[Criteria and modalities of assessment of work fitness for jobs in food industry].

Current legislation establishes the general methods and standards of judgement concerning fitness of workers to perform particular jobs. The object of this study was to verify particular criteria and methods that, in observance of the law, allow the physician to maintain in productive activity workers with chronic disease or permanent consequences of industrial accidents in Apulia, Southern Italy. In accordance with the law, 156 workers and their work environment are regularly under surveillance. In the last few years it was possible to accurately diagnose all diseases that may be involved in particular work fitness checks. 3 workers suffered the permanent consequences of very serious labour accidents amputation of II, III, IV fingers of the right hand; a slight post-traumatic shortening (shrinkage) of the right femur; tympanic membrane perforation). 7 workers suffered from non-work related disease (substitution of aortic valve, allergic asthma, virus B chronic hepatitis, chronic glomerulonephritis, replacement of right knee, rheumatoid arthritis, thrombosis of the retinal central vein without a vision reduction). The criteria for an "fitness judgement" are: an accurate diagnosis and evaluation of residual functional ability, analysis of the original job, with an investigation in the field, and break down into the single operations in order to assess all work-related risks (concerning health, safety and physical resources), and evaluation of work organization per job. It was thus possible to define particular adjustments to the original job, so as to be compatible with the current physical conditions the workers. Workers, factory directors and union officers accepted these particular criteria and standard of "work fitness judgement, which made it possible to apply them in practice with consequent reinstatement of workers in productive activity.

Accidents, Occupational↗

[Cardiovascular manifestations of Behçet's disease].

Vascular involvement in Behçet's disease, recognized since 1946, is peculiar since it occurs in young subjects with no vascular risk factor. Recurrent phlebitis, sometimes associated with fever and biological signs of inflammation, commonly involve the large vessels (superior and inferior vena cava, hepatic veins) and cerebral veins. Arterial involvement was more recently identified and is expressed by thrombosis, stenosis and/or aneurysms diversely associated. Aneurysms, true "arterial aphthae", may be multifocal and can involve all arterial territories with a clear preference for the abdominal aorta and the pulmonary arteries (Hughes-Stovin syndrome). Mortality is significant due to rupture and the risk of recurrence. Cardiac involvement includes coronary artery disease which merits attention since it affects young subjects and is often expressed by myocardial infarction or angina; all three tunics can be involved; mortality is high: 20% in the months or years following diagnosis. A few cases of recurrent pericarditis have been reported. Myocardiopathy can be of inflammatory nature or secondary to coronary artery disease. Endocardiac involvement may be limited to valve disease or spread to the ventricular wall. Endomyocardial fibrosis is exceptional and usually associated with intracavitary thrombus formation. Coagulation disorders have been reported but they cannot explain the different thrombotic manifestations which are probably the consequence of an abnormal response of the vascular endothelial cells.

Behcet Syndrome↗

Fibrolamellar hepatocellular carcinoma: a case report.

A 6-year-old Malay boy presented with fever and abdominal pain for 2 months. Computerised tomography showed a nodular mass in the left lobe of the liver. There was also portal vein thrombosis on the left side. Serum alpha-fetoprotein was not elevated and Hepatitis B antigen was negative. Biopsy of the liver mass led to a histological diagnosis of fibrolamellar hepatocellular carcinoma. In view of extensive tumour involvement, he could not be operated on but was treated with chemotherapy. However, the tumour did not respond. While this is expected for fibrolamellar hepatocellular carcinoma, the possibility of the tumour having a component of ordinary hepatocellular carcinoma could not be excluded as the tumour was not resected. Fibrolamellar hepatocellular carcinoma is a rare histological subtype of hepatocellular carcinoma, associated with a better prognosis. It affects the younger age group and has no association with cirrhosis, hepatitis B virus infection or exposure to oral contraceptives, all of which are implicated in ordinary hepatocellular carcinoma. Serum alpha-fetoprotein level is usually within normal limits and other laboratory values are not contributory to the diagnosis. The diagnosis is usually suggested by radiographic studies viz. CT scan of the abdomen, which would show an irregular non-homogenous mass in the liver, and confirmed by histological examination. The most characteristic microscopical feature is fibrosis arranged in a lamellar fashion around polygonal and deeply eosinophilic neoplastic hepatocytes.

Carcinoma, Hepatocellular↗

[Analysis of the characteristics of responders to chemotherapy of advanced hepatocellular carcinoma].

To evaluate characteristics of the responders to chemotherapy for advanced hepatocellular carcinoma, clinicopathological findings of the responders (9 cases) and those of the non-responders (58 cases) have been compared. As for clinical characteristics, no difference was found in age and sex between the two groups. The responders were, however, restricted to the good cases in respect to performance status (PS) and hepatic reserve based on Child's classification. Concerning characteristics of tumors, no significant difference between the two groups was observed in size and number of tumors, or in the frequency of portal vein thrombosis and that of remote metastasis. There was no difference between them in chemotherapy regimen, either. It was concluded that PS and hepatic reserve might be the most important factors determining the efficacy of chemotherapy for advanced hepatocellular carcinoma.

Adult↗

[Doppler pulsed and color echography of the inferior vena cava].

B mode real time echotomography associated with duplex and colour doppler makes possible a complete investigation of the lower vena cava. The ilio-cava fossa is difficult to explore because of gas and digestive superpositions. Diagramatically, it is possible to distinguish five levels of investigation. 1) Iliac veins and ilio-cava convergence. 2) Sub-renal LVC. 3) Inter-renal LVC and communications with renal veins. 4) Retro-hepatic LVC: communications with liver and supra-hepatic veins. 5) Thoracic LVC and termination in the right atrium. Sector-based probes provide a 60 to 110 degrees sector thanks to which it is possible to obtain a sufficient exploration field for a very limited acoustic fenestra. Duplex and colour doppler assess the venous flow and its variations during the respiratory cycle and during the operations of compression or Valsalva's experiment. Colour doppler detects more precisely slow, collateral or repermeation flows. The recent complete thrombosis leads no duplex and colour doppler signal but an increase of the vein diameter associated with a collateralitis syndrome. In case of partial thrombosis, the vein is partially compressible as colour doppler fits thrombus closely round and visualizes the remaining lumen. In the ilio-cava fossa, compressions (either tumorous, ganglial or aneurysmal) which are often associated are diagnosed in the meanwhile. It is important to know the change of diameter and the abnormalities of the LVC position when a cava blocking is advised. A LVC whose diameters exceed 28 mm (mega-cava) contra indicated filter because of the risk of migration. Colour doppler makes easier the supervision of the blocking.

Collateral Circulation↗

Percutaneous mechanical fragmentation and stent placement for the treatment of early posttransplantation portal vein thrombosis.

BACKGROUND: Early portal vein thrombosis is a rare but severe complication of liver transplantation requiring retransplantation or at least surgical thrombectomy, both hampered by high morbidity and mortality. METHODS: We describe of a case of successful long-term recanalization of early posttransplantation portal vein thrombosis by a minimally invasive percutaneous transhepatic angiographic approach using both mechanical fragmentation and pharmacological lysis of the thrombus followed by stent placement. RESULTS: Mechanical fragmentation and contemporaneous local urokinase administration resulted in complete removal of the clot; the use of a vascular stent after balloon dilatation allowed restoration of normal blood flow to the liver after 9 months of follow-up. CONCLUSIONS: This case report confirms the possibility of successful recanalization of the portal vein after early posttransplantation thrombosis by a minimally invasive angiographic approach. Balloon dilatation and placement of a vascular stent could help to decrease the risk of recurrent thrombosis.

Anastomosis, Surgical↗

[Inferior vena cava thrombosis responsible for chronic Budd-Chiari syndrome during hepatic and digestive amyloidosis].

The case of a 39 year old woman with amyloidosis of the liver and the digestive tract presenting with obstruction of the inferior vena cava is reported. Computed tomography after bolus injection of contrast material revealed a typical patchy fan-shaped image, of hepatic vein obstruction. The association of Budd-Chiari's syndrome with amyloidosis may be related to the increased risk of thrombosis observed in the latter disease.

Adult↗

Treatment of vascular complications following liver transplantation: multidisciplinary approach.

BACKGROUND/AIMS: Complications affecting the vascularization of the graft following orthotopic liver transplantation still represent a significant cause of graft loss and patient mortality. Strategies have recently been developed for the early detection and treatment of these complications before irreversible graft failure takes place. METHODOLOGY: A series of 429 consecutive liver transplants performed on 384 patients between April 1986 and December 1998 was retrospectively reviewed to assess the incidence of all the vascular complications and the results of their treatment with either surgery or interventional radiology. RESULTS: The incidence of vascular complications was 6.06% for the hepatic artery, 2.56% for the inferior vena cava and 1.16% for the portal vein. As regards anastomotic stenosis and thrombosis, the requirement of retransplantation decreased progressively with the advent of systematic postoperative screening with duplex Doppler ultrasonography and the introduction of graft-salvage procedures, falling from 50% for those cases diagnosed before 1996 to 19% for those diagnosed from 1996 on. Mortality following 18 graft-salvage procedures was 11.1% versus 41.6% following retransplantation. Graft-salvage procedures were successful in 14 out of 18 cases. CONCLUSIONS: Close surveillance of the vascular anastomoses and multidisciplinary approach to the treatment of vascular complication after liver transplantation considerably reduces graft loss and patient mortality.

Anastomosis, Surgical↗

[Acute portal thrombosis revealing hereditary hemochromatosis: report of a case].

The authors report the case of a 49-year old man in whom an inaugural portal vein thrombosis led to the diagnosis of hereditary hemochromatosis. In this case, the increase in ferritinemia and the T2-weighted MRI hepatic segmental hyposignal were considered as consequences of tissular necrosis while they did probe a real iron overload. Genetic testing, revealing C282Y/H63D compound heterozygoty, provided evidence for a diagnosis of hereditary hemochromatosis. Weekly venesections induced a calculated iron depletion of 3.5 g without occurrence of anemia, further supporting the diagnosis. We suggest that hemochromatosis should be considered in the differential diagnosis of idiopathic portal vein thrombosis when signs of abnormal iron accumulation exist.

Acute Disease↗