Study of hemostasis in pediatric patients with portal vein thrombosis

We describe the behavior of hemostatic variables in children with portal vein thrombosis (PVT) and in a control pediatric population. Hereditary protein C (PC) or protein S (PS) deficiency was not a etiologic factor for PVT in children. Minor signs of consumption of coagulation factors II, V, fibrinogen and hyperfibrinolysis were detected. One child had lupus anticoagulant (LA).     

Improved results of liver transplantation in patients with portal vein thrombosis

OBJECTIVE: To analyze the impact of preexisting portal vein thrombosis (PVT) on the operative management and outcome of liver transplantation. DESIGN: Retrospective review of 1423 patients who received transplants over 11 years. SETTING: Tertiary referral center. PATIENTS OR OTHER PARTICIPANTS: Seventy patients who underwent liver transplantation who had preexisting PVT. INTERVENTIONS: Portal vein thromboendovenectomy, vein grafting, or use of portal collateral veins for inflow during liver transplantation. MAIN OUTCOME MEASURES: Postoperative PVT, intraoperative transfusion, retransplantation rate, 30-day and 1-year actuarial survival rates. RESULTS: Operative management consisted of thromboendovenectomy in 61 cases, vein graft to the superior mesenteric vein in 6 cases, and vein graft to other mesenteric veins in 3 cases. The incidence of posttransplant PVT was 3% (n = 2). The mean +/- SD transfusion requirement was 23 +/- 18 U. The 1-year actuarial survival rate was 74% but improved from 66% in the first 35 cases to 82% in the latter 35 cases. CONCLUSIONS: Thromboendovenectomy is the procedure of choice for PVT. Results of liver transplantation in patients with PVT improve significantly with experience gained and are equivalent to results in patients without PVT.     

Thickening of the wall of the bile duct due to intramural collaterals in three patients with portal vein thrombosis

OBJECTIVE: We report three cases of a rare form of cavernoma developed within the wall of the common bile duct. CONCLUSION: To our knowledge, this kind of portal cavernoma has not been described in the literature. Because the cavernoma may be easily confused with other causes of bile duct wall thickening, color Doppler sonography is mandatory for a correct diagnosis.     

Acute thrombosis of the portal system. Treatment with alteplase and heparin or with heparin alone in 10 patients

OBJECTIVES: We report the efficacy of alteplase (a recombinant tissue plasminogen activator) with heparin or heparin alone in the treatment of acute thrombosis of the portal venous system. METHODS: Ten consecutive patients with acute portal venous system thrombosis were studied. Five patients were treated with alteplase and heparin, and the remaining 5 patients, who were asymptomatic or had a contraindication to alteplase, were treated with heparin alone. RESULTS: In 3 of the 5 patients treated with alteplase, ultrasonography showed total resolution of the thrombus; the remaining 2 had partial resolution of the thrombus. In 4 of the 5 patients treated with heparin alone, ultrasonography showed total resolution of the thrombus, and no change in one. No bleeding occurred. CONCLUSION: Treatment with heparin can result in complete recanalisation of acute portal venous system thrombosis. These data suggest that combined therapy with systemic alteplase does not increase the efficacy of heparin.     

Measurement of secondary colony formation after 5 weeks in long-term cultures in patients with myelodysplastic syndrome

Pancytopenia is a frequent manifestation of myelodysplastic syndromes (MDS). In the presence of an empty bone marrow, clinical distinction from aplastic anemia may be difficult. The hypoplastic marrow morphology seen in some cases of MDS raises questions about etiologic and pathophysiologic relationships between aplastic anemia and MDS. The goal of our study was to compare the degree of the hematopoietic failure in these diseases at the level of the most immature progenitor and stem cells that can be measured in vitro. In a systemic, prospective fashion, we have studied bone marrow (n = 45) and peripheral blood (n = 33) of patients with MDS for the number of long-term culture initiating cells (LTC-IC) in comparison to 17 normal controls and patients with new, untreated aplastic anemia (46 marrow; 62 blood samples). Due to the low numbers of cells available for the analysis, formal limiting dilution analysis could not be performed, instead secondary colony-forming cells (CFC) after 5 weeks of LTBMC were measured. As the number of these cells is proportional to the input number of LTC-IC, the number of secondary CFC per 10(6) mononuclear cells (MNC) initiating the LTBMC can be used as a measure of the content of immature stem cells in bone marrow and peripheral blood. The MDS group consisted of 34 RA, three RARS, eight RAEB and two RAEB-T patients with mean absolute neutrophil values of 1992, 1413, 1441, and 380 per mm3, respectively. The diagnosis was established based on bone marrow morphology and results of cytogenetic studies. In comparison to controls (147 +/- 38/10(6) MNC), significantly decreased numbers of bone marrow secondary CFC were found in MDS: in patients with RA and RARS, 21 +/- 7 secondary CFC per 10(6) bone marrow MNC (P < 0.001); patients with RAEB and RAEB-T: 39 +/- 12 CFC per 10(6) marrow MNC (P < 0.001). In all groups tested, the decrease in peripheral blood secondary CFC numbers was consistently less pronounced. In MDS patients with hypocellular bone marrow, secondary CFC were lower but not significantly different in comparison to MDS with hypercellular marrow (18 +/- 6 vs 35 +/- 11; NS; hypoplastic bone marrow also was not associated with significantly lower neutrophil counts). However, in 24% of patients with MDS, bone marrow secondary CFC were within the normal range, while in the aplastic anemia group only one of the patients showed secondary CFC number within normal range. Bone marrow and blood secondary CFC numbers in hypoplastic RA were significantly higher than those in severe aplastic anemia 919 +/- 5 in bone marrow, P < 0.01; 7 +/- 2 in blood, P < 0.05). This trend was even more pronounced in hypoplastic RA with chromosomal abnormalities. However, no significant differences were found between the secondary CFC numbers in hypoplastic RA and moderate aplastic anemia. We concluded that, although the deficiency in the stem cell compartment is less severe in MDS than in aplastic anemia, depletion of early hematopoietic cells is an essential part of the pathophysiology in both diseases.     

A case of deep vein thrombosis in idiopathic nephrotic syndrome with resistance to activated protein C

The article presents data of a complex clinical/radiation examination of flying personnel with diagnosed diffuse pathologies of pancreas. Consistent patterns of the fibrotic process in pancreatic parenchyma were related to professional specifics. Preponderance of ultrasonic investigations, mathematical analyses, and X-ray computer tomography for the enhanced diagnostic algorithm for the medical certification of flying personnel were demonstrated.     

Frequency of deep vein thrombosis in patients with patent foramen ovale and ischemic stroke or transient ischemic attack

To evaluate the additional value of transesophageal (TEE) compared with transthoracic (TTE) echocardiography and the role of patent foramen ovale (PFO) and deep vein thrombosis in the work-up of embolic events, patients with presumed cardiac embolic stroke or transient ischemic attack (neurovascular etiology was excluded) were prospectively studied by transthoracic and transesophageal contrast echocardiography. If PFO was detected echocardiographically, PFO size was assessed semiquantitatively and phlebography of both legs was performed. Two hundred forty-two consecutive patients (153 men, 60 +/- 15 years) were studied. In 197 patients, neuroimaging showed evidence of embolic infarction. TEE identified 138 potential cardiac sources of embolism in 111 patients, compared with 69 by TTE (p <0.01) in 59 patients. TEE detected potential cardiac sources in 52 patients with negative TTE examination and was significantly superior compared with TTE for identifying left atrial thrombi, spontaneous echo contrast, PFO, atrial septal aneurysm, and atheroma of the ascending aorta. In patients with a positive TTE, additional diagnostic information by TEE was found in only 6 patients and did not change therapy. Phlebography was performed in 53 patients with PFO and revealed deep vein thrombosis in 5 patients (9.5%); all had medium or large PFOs. Thus, in patients with cerebral ischemia of suspected cardiogenic origin and a normal TTE examination, TEE detects potential causes of embolism in 31% of patients and is therefore of diagnostic relevance. Conversely, in the presence of a diagnostic TTE an additional TEE confers only marginal diagnostic benefit. Deep venous thrombosis was detected in nearly 10% of patients with PFO as the sole identifiable cardiac risk factor. Given that in 4 of 5 patients deep vein thrombosis was clinically silent, phlebography should be performed in patients with medium or large interatrial shunts if paradoxical embolism is suspected.     

Distribution of thrombosis in patients with symptomatic deep vein thrombosis. Implications for simplifying the diagnostic process with compression ultrasound

BACKGROUND: Two different diagnostic strategies are used to perform compression (real-time) ultrasound for the diagnosis of clinically suspected deep-vein thrombosis. One is to examine the entire proximal venous system from common femoral to distal popliteal vein; the other is a limited examination of only the common femoral and the entire popliteal vein. The latter strategy, which is less time-consuming and requires less expensive equipment, is based on a strong impression from prospective studies using limited compression ultrasound that proximal vein thrombi always involve the common femoral or popliteal vein. This impression, which is supported by the demonstrated safety at long-term follow-up of not treating patients whose limited compression ultrasound is normal at presentation and then repeated within the next week, has not been tested in a formal study. Therefore, we reviewed a large series of venograms performed in consecutive patients with clinically suspected venous thrombosis to determine the distribution of venous thrombosis in symptomatic patients. METHODS: Venograms were performed using 150 mL of radiographic contrast material. Before the study, a panel of experts agreed on the standardized criteria for the assessment of venograms. Venograms were adjudicated blindly for the presence of deep vein thrombosis and to determine the distribution of proximal vein thrombosis and isolated calf-vein thrombosis, the size of proximal thrombi, and whether they were occlusive or nonocclusive. Subsequently, the duration of symptoms was related to the venographic findings. RESULTS: Five hundred sixty-two venograms from consecutive patients with a first episode of clinically suspected deep vein thrombosis were adjudicated. Of these, 20 (3.6%) were inadequate for interpretation. In the remaining 542, venous thrombosis was demonstrated in 189 instances (prevalence, 35%; 95% confidence interval, 31% to 39%) and were located in the proximal veins in 166 (88%; 95% confidence interval, 82% to 92%) venograms. Isolated calf-vein thrombosis was present in the remaining 23 (12%; 95% confidence interval, 8% to 18%) venograms. Proximal with concurrent calf thrombosis was detected in 164 (99%) of the 166 patients. Proximal thrombi involved only the popliteal vein in 16 (10%); the popliteal and superficial femoral veins in 70 (42%); and the popliteal, superficial, and common femoral vein in eight (5%); whereas thrombi involving the entire proximal deep venous system were detected in 58 (35%) venograms. Isolated thrombosis of the superficial femoral, common femoral, and iliac vein was not observed. Proximal venous thrombi were occlusive in 146 (88%) patients. No relation between the duration of symptoms and the extent or the occlusiveness of venous thrombi could be demonstrated. CONCLUSIONS: Most symptomatic patients have extensive occlusive proximal vein thrombosis at the time of presentation. Thrombi isolated to the superficial femoral or iliac vein were not observed in this large sample of consecutive patients. Our data support the use of the relatively simple, inexpensive, and rapid compression ultrasound method that limits the examination of the proximal veins to the common femoral and popliteal veins.     

Endoscopic sclerotization of the oesophagus wall or transhepatic thrombosis of the coronary vein: Alternative or complementary therapy of bleeding oesophageal varices in portal hypertension (author's transl)

Endoscopic sclerotization of esophageal wall has been performed in 48 patients with bleeding of esophageal varices. The mortality rate in the acute bleeding was 29,2%. There was no mortality, if sclerotherapy was done in the non hemorrhagic interval. Of the 48 patients 25 are still alive, 8 more than 2 years without bleeding. In 12 cases we performed transhepatic obliteration of the V. coronaria ventriculi. Only in 3 of them no further hemorrhage occurred within 6 months. Fibrosclerotization seems to be superior to the transhepatic obliteration, because the primary mortality and complications are lower and long term results better.     

Graduated compression stockings in the prevention of deep vein thrombosis in patients with acute myocardial infarction

Venous volume (venous capacity) of the calf is low in patients with acute myocardial infarction, who also have a high risk of deep vein thrombosis (DVT). The effect of graduated compression stockings on the venous volume and on the incidence of DVT was therefore studied in 80 patients aged 70 years and above with acute myocardial infarction. Graduated compression stockings were randomly fitted to one leg, the other serving as a control, after which the venous volume was measured by strain gauge plethysmography. The incidence of DVT was measured by the 125I fibrinogen uptake test. Venous volume was significantly higher in legs treated with graduated compression stockings compared to control legs. DVT developed in eight control legs but not in any leg treated with graduated compression stockings (P = 0.003). DVT was also significantly more frequent in women compared to men and the majority of DVT developed in legs with very low venous volume values.     

Fibrinolytic treatment with ultra-high streptokinase infusion via the dorsalis pedis vein offers no advantage over systemic infusion via the brachial vein in patients with deep vein thrombosis of the leg

BACKGROUND: Diagnosis of intraparenchymal brain lesions has usually required invasive diagnostic procedures, because too few cells are shed into cerebrospinal fluid to permit cytologic diagnosis. Polymerase chain reaction technology makes it possible to identify cell populations that are present at a much lower frequency than traditional techniques. CASE REPORT: A young woman presented with multiple brain lesions raised the question of primary central nervous system lymphoma. Polymerase chain reaction analysis of cerebrospinal fluid showed evidence of a monoclonal B-cell population heightening suspicion of lymphoma. Brain biopsy showed acute demyelination most consistent with multiple sclerosis. CONCLUSION: Although T-cell restriction has been demonstrated in multiple sclerosis lesions, the finding of a monoclonal B-cell population was unexpected and to our knowledge has not been previously reported. This case emphasizes that monoclonality is not always indicative of a neoplastic process, particularly in the central nervous system.     

A prospective controlled study of the efficacy of short-term anticoagulation therapy in patients with deep vein thrombosis of the lower extremity

This study was undertaken to investigate changes in aortic geometry and compliance after long-term blockade of angiotensin receptors type 1 (AT1) and AT2 receptors under basal conditions and after myocardial infarction (MI). Sham-operated (sham) or MI rats received either no treatment, AT1 antagonist GR138950C (GR; 2 mg/kg/day i.v.), or AT2 antagonist PD123319 (PD; 3 mg/kg/day s.c.). After 3 weeks, mean arterial blood pressure (MAP) was measured. Thoracic aorta diastolic diameter (D[dia]), compliance coefficient (CC), and distensibility coefficient (DC) were determined noninvasively in anesthetized rats by using ultrasound and wall tracking. After the rats were killed, histologic measurements were made on aortic cross sections. In sham rats, MAP was reduced by GR treatment (76 +/- 6 vs. 106 +/- 5 mm Hg), but not by PD. D(dia) was reduced in both GR-treated (1.74 +/- 0.08 vs. 2.09 +/- 0.05 mm) and PD-treated (1.83 +/- 0.05 vs. 2.09 +/- 0.05 mm) sham rats. CC and DC were not modified by either treatment. Although media cross-sectional area was not affected by either GR or PD treatment in sham rats, media thickness and media/lumen ratio were increased in both cases. Induction of MI had no effect on aortic structure, geometry, or mechanics; however, treatment with either GR or PD improved DC versus untreated MI rats. We conclude that AT1 and AT2 receptors are involved in angiotensin II-mediated effects on aortic geometry and mechanics under both basal conditions and after MI. Whereas blockade of AT1 receptors most likely influences vascular properties through a depressor mechanism, AT2 receptors induce pressure-independent remodeling.     

Use of an ultrasonic scalpel as an alternative to electrocautery in patients with pacemakers

We report the safe and effective use of an ultrasonically activated scalpel as an alternative to unipolar electrocautery during surgical procedures in 4 patients with pacemakers. This scalpel provided adequate hemostasis without the adverse consequences of electromagnetic interference. As the scalpel was able to easily cut through the silicone connector sleeve of one generator, it is not recommended for routine generator replacement because it may damage existing pacing leads. However, its use during nonpacemaker operations in patients with pacemakers should be considered.     

Response to symptom-limited exercise in patients with the hepatopulmonary syndrome

OBJECTIVE: To study the response to symptom-limited exercise in patients with the hepatopulmonary syndrome (HPS). DESIGN: The response to maximal cardiopulmonary exercise (CPX) was studied in 5 patients with HPS and compared with 10 case control (normoxemic, NC) cirrhotics (matched for age, gender, etiology and severity of liver disease, tobacco use, and beta-blocker therapy) and 9 hypoxemic control cirrhotics (HC) without clinical evidence of HPS. SETTING: Cardiopulmonary exercise physiology laboratory in a tertiary care referral center. PATIENTS: Cirrhotics referred for CPX as part of their preliver transplantation evaluation. MEASUREMENTS: Standard pulmonary function tests and echocardiography were performed to assess resting pulmonary and cardiac function. Peak oxygen consumption (VO2), minute ventilation, arterial blood gases, and dead space (VD/VT) were determined during symptom-limited maximal CPX. RESULTS: Resting spirometry and lung volumes were similar between HPS and NC subjects, while HC subjects had restrictive physiology. Differences existed in diffusing capacity corrected for hemoglobin and alveolar volume percent predicted (HPS, 45+/-2 vs NC, 68+/-3, p<0.05; vs HC, 70+/-4, p<0.05), PaO2 (HPS, 70+/-5 mm Hg; HC, 79+/-3 mm Hg, vs NC, 102+/-3 mm Hg, p<0.05) and alveolar-arterial (A-a) O2 gradient (HPS, 42+/-8 mm Hg vs HC, 27+/-2 mm Hg, p<0.05; vs NC, 6+/-2 mm Hg, p<0.05). During CPX, HPS patients achieved a lower peak VO2 percent predicted (HPS, 55+/-6 vs NC, 73+/-3, p<0.05; vs HC, 71+/-5, p<0.05) and VO2 at the ventilatory threshold as percent predicted peak VO2 (HPS, 36+/-2 vs NC, 55+/-4, p<0.05; vs HC 55+/-5, p<0.05). While no differences existed in heart rate and breathing reserve, HPS patients had significantly lower PaO2 (HPS, 50+/-5 mm Hg vs NC, 97+/-4 mm Hg, p<0.05; vs HC, 87+/-6 mm Hg, p<0.05), wider A-a O2 gradient (HPS, 73+/-5 mm Hg vs NC, 13+/-3 mm Hg, p<0.05; vs HC, 31+/-5 mm Hg, p<0.05) and higher VD/VT (HPS, 0.36+/-.03 vs NC, 0.18+/-.02, p<0.05; vs HC, 0.28+/-.02, p<0.05) at peak exercise. For HPS patients, VO2 was negatively correlated with VD/VT (r2=0.9) and positively correlated with PaO2 (r2=0.41) at peak exercise. Conclusions: Patients with HPS demonstrate a severe reduction in aerobic capacity, beyond that found in cirrhotics without syndrome. The significant hypoxemia and elevated VD/VT at peak exercise suggest that an abnormal pulmonary circulation contributes to further exercise limitation in patients with HPS.     

Alteration of the LIS1 gene in Japanese patients with isolated lissencephaly sequence or Miller-Dieker syndrome

Maffucci's syndrome is a rare, congenital disease due to neuro-ectodermal dysplasia and is characterized by enchondromatosis and multiple soft tissue haemangiomata. A case of Maffucci's syndrome with haemangiomata along the upper airways requiring laser surgery is described. The literature on Maffucci's syndrome is reviewed.     

Impaired recognition memory in patients with lesions limited to the hippocampal formation.

A recent literature survey of results from a widely used recognition memory test raised questions about the extent to which recognition memory impairment ordinarily occurs in human amnesia and, in particular, whether recognition memory is impaired at all after damage limited to the hippocampal region (J. P. Aggleton & C. Shaw, 1996). Experiment 1 examined the performance of 6 amnesic patients on 11 to 25 different recognition memory tests. Three patients had bilateral lesions limited primarily to the hippocampus (G.D.) or the hippocampal formation (W.H. and L.M.), as determined by postmortem, neurohistological analysis (N. Rempel-Clower, S. M. Zola, L. R. Squire, & D. G. Amaral, 1996). All 6 patients exhibited unequivocally impaired recognition memory. In Experiment 2, the 3 patients still available for study were each markedly impaired on a test of object recognition similar to the kind used to test recognition memory in nonhuman primates. Recognition memory impairment is a robust feature of human amnesia, even when damage is limited primarily to the hippocampus. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

The safety and efficacy of transcatheter arterial chemoembolization in the treatment of patients with hepatocellular carcinoma and main portal vein obstruction. A prospective controlled study

BACKGROUND: Transcatheter arterial chemoembolization (TACE) has been contra-indicated for the treatment of patients with hepatocellular carcinoma (HCC) and main portal vein (MPV) obstruction because of the potential risk of hepatic insufficiency resulting from ischemia after TACE. The current controlled study was undertaken to assess the safety, efficacy, and predictive factors of favorable response to TACE in patients with HCC and MPV obstruction with good hepatic function and adequate collateral circulation around the MPV. METHODS: Of a total of 47 patients, 31 were treated with TACE, and 16 who declined the procedures were untreated controls. Thirty-six patients (77%) had diffuse-type HCC and 11 (23%) had nodular-type HCC. During the first week after TACE immediate postprocedural complications were evaluated, and the development of hepatic insufficiency as a late complication was assessed at the end of the fourth week. The cumulative survival rate was estimated by the Kaplan-Meier method, and predictors of better prognosis were obtained by univariate and multivariate analyses. RESULTS: Although no patients showed clinical evidence of hepatic insufficiency as an immediate complication, transient fever and abdominal pain were common. Progressive hepatic insufficiency developed at the fourth week; however, there was no difference between the treated and untreated groups. The survival time of treated patients was statistically no longer than that of untreated patients. In the univariate analysis, tumor type and size, the pattern of iodized oil uptake in the tumor, and the presence of iodized oil uptake in the tumor thrombi at the MPV significantly influenced the prognosis. Tumor type, whether treated or not, was the most important prognostic factor patients with nodular-type HCC had significantly longer survival time (median, 11 months) than those with diffuse-type HCC (median, 4 months). Regarding the efficacy of TACE, there was no statistical difference in survival between treated and untreated diffuse-type HCC patients. In comparison, with nodular-type HCC it seemed that survival time was longer for TACE-treated patients (median, 30 months) than for untreated patients (median, 7 months). CONCLUSIONS: TACE may be a safe modality for the treatment of patients with HCC and MPV obstruction, provided that the patients have good hepatic function and collateral circulation around the MPV. However, TACE was not efficacious in the treatment of diffuse-type HCC. The authors recommend TACE for treating nodular-type HCC because of the potential benefit of prolonged survival.