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Selected AbstractsDelayed embryonic development and impaired cell growth and survival in Actg1 null mice,CYTOSKELETON, Issue 9 2010Tina M. Bunnell Abstract Actins are among the most highly expressed proteins in eukaryotes and play a central role in nearly all aspects of cell biology. While the intricate process of development undoubtedly requires a properly regulated actin cytoskeleton, little is known about the contributions of different actin isoforms during embryogenesis. Of the six actin isoforms, only the two cytoplasmic actins, ,cyto - and ,cyto -actin, are ubiquitously expressed. We found that ,cyto -actin null (Actg1,/,) mice were fully viable during embryonic development, but most died within 48 h of birth due to respiratory failure and cannibalization by the parents. While no morphogenetic defects were identified, Actg1,/, mice exhibited stunted growth during embryonic and postnatal development as well as delayed cardiac outflow tract formation that resolved by birth. Using primary mouse embryonic fibroblasts, we confirm that ,cyto -actin is not required for cell migration. The Actg1,/, cells, however, exhibited growth impairment and reduced cell viability, defects which perhaps contribute to the stunted growth and developmental delays observed in Actg1,/, embryos. Since the total amount of actin protein was maintained in Actg1,/, cells, our data suggests a distinct requirement for ,cyto -actin in cell growth and survival. © 2010 Wiley-Liss, Inc. [source] Survival outcome of patients with nasopharyngeal carcinoma with first local failure: A study by the Hong Kong Nasopharyngeal Carcinoma Study GroupHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2005Kwok Hung Yu FRCR Abstract Background. The purpose of this article is to report the overall survival (OS) outcome of patients with nasopharyngeal carcinoma (NPC) with local failure who received salvage treatment and to identify prognostic factors for OS. Methods. Between January 1996 and December 2000, 2915 patients received primary radiotherapy (RT) with or without chemotherapy for nonmetastatic NPC. At a median follow-up of 3.1 years, 319 patients had developed local failure as the first failure, with or without synchronous regional/distant failure. OS was calculated from the start of primary RT. Univariate and multivariate analyses were performed to identify prognostic factors for OS in patients with isolated local failure. Results. The T classification distribution of the local failure (rT classification) was as follows: 68 (21%) rT1 to T2a, 92 (29%) rT2b, 82 (26%) rT3, and 77 (24%) rT4. The rT classification was the same as the initial T classification in 82% of patients. Two hundred seventy-five patients (86%) had isolated local failure, and 232 (84%) of them did not have any distant metastasis or regional failure develop during follow-up. Salvage treatment was given to 200 patients (73%) with isolated local failure. One hundred fifty-nine patients (80%) received reirradiation (108 external beam RT [EBRT], 44 brachytherapy, and seven EBRT plus brachytherapy), 22 patients (11%) underwent nasopharyngectomy with or without postoperative RT, and 19 patients (9%) were treated with chemotherapy alone. Four patients died of RT complications, and one died of chemotherapy toxicity in the absence of active NPC. The 3-year actuarial OS for patients with isolated local failure was 74%. On multivariate analysis, advanced initial T classification (hazard ratio [HR], 1.44; p = .0006) and the use of salvage treatment (HR, 0.54; p = .0038) were independent prognostic factors. For the subgroups of patients who had the same recurrent and initial T classification, salvage treatment was associated with improved OS only in the subgroup with T1 to T2 local failure (n = 127; p = 0.0446), but not in the subgroups with T3 (n = 48) or T4 (n = 54) disease. Conclusions. Most patients with first local failure have localized disease. Salvage treatment is feasible in most of the patients with clinically isolated local failure. Patients who had early initial T classification have a more favorable prognosis. Subgroup analysis suggests that salvage treatment only prolongs survival in patients with T1 to T2 recurrent disease. © 2005 Wiley Periodicals, Inc. Head Neck27: XXX,XXX, 2005 [source] Adjuvant fractionated high-dose-rate intracavitary brachytherapy after external beam radiotherapy in Tl and T2 nasopharyngeal carcinomaHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2004Jiade J. Lu MD Abstract Background. The value of high-dose-rate intracavitary brachytherapy (HDRIB) for persistent or recurrent nasopharyngeal carcinoma has been well described; however, the benefit of routine adjuvant fractionated HDRIB following external beam radiation therapy (EBRT) has not been completely determined. The objective of this analysis was to evaluate the outcome of two fractions of adjuvant HDRIB treatment in Tl and T2 nasopharyngeal carcinoma. Methods. Thirty-three consecutive and nonselected patients who had Tl and T2 non-disseminated nasopharyngeal carcinoma were treated according to an IRB approved institutional research protocol between March 1999 and July 2001. By the 1997 AJCC cancer staging classification, 22 patients (67%) had Tl disease and 11 patients (33%) had T2 disease. Seventeen of these patients who had stage I or stage II disease (i.e., NO or Nl) were treated with EBRT followed by two fractions of adjuvant HDRIB (group 1); 16 patients who had stage III or stage IV disease (i.e., N2 or N3) were treated with concurrent cisplatin, EBRT and adjuvant HDRIB and subsequent adjuvant cisplatin and fluorouracil (5-FU) chemotherapy (group 2). EBRT was delivered by daily conventional fractionation to a total dose of 66 Gy to the primary tumor. Nodal disease received 66 Gy if it was less than 3cm in maximum diameter and 70 Gy if larger or there was palpable residual disease after 66 Gy. A total of 10 Gy of HDRIB in 2 equal fractions of 5 Gy spaced 1 week apart was delivered starting 1 week after the completion of EBRT. All patients were assessed for treatment response, local control, survival, and toxicity. Results. The median follow up for all 29 surviving patients is 29 months (range: 17,38 months). One patient died 7 months and one died 18 months after radiation therapy from the effects of distant metastases; two died of unrelated causes. At the time of this analysis, one patient (3%) had persistent local disease and one patient (3%) developed pathologically confirmed local recurrence in the nasopharynx. In addition, one patient (3%) developed recurrence only in a neck node followed by distant metastasis, and two patients (6%) developed distant metastasis without locoregional relapse. The 2-year local control rate at the primary site was 93.6%, and the overall survival and disease-free survival rates were 82% and 74% respectively. All patients experienced some degree of acute and/or late toxicity related to radiation therapy. Ten patients (30%) experienced grade 3 acute and/or late toxicity and six patients (18%) developed grade 4 acute and/or late toxicity. No grade 5 toxicity occurred. No unexpected damage of structures within the HDRIB fields was detected. Conclusions. EBRT supplemented by two fractions of adjuvant HDRIB produced a 93.6% local control rate for Tl and T2 nasopharyngeal cancer at 2 years of follow up, with acceptable rates of acute and late toxicity. Brief adjuvant HDRIB appears to permit dose escalation safely, even in patients who receive chemotherapy concurrently with conventional radiation therapy. This strategy needs to be optimized and then tested in a prospective randomized phase III trial to learn if it can improve outcome. © 2004 Wiley Periodicals, Inc. Head Neck26: 389,395, 2004. [source] Bastard Feudalism, Overmighty Subjects and Idols of the Multitude during the Wars of the RosesHISTORY, Issue 279 2000Michael Hicks A handful of overmighty subjects exercised a disproportionate influence on the events of the Wars of the Roses. This article considers how and why. Circumstances were certainly propitious. Not only did the greatest noblemen command exceptional resources of their own, albeit always less than the king, but they deployed the principal military commands against the crown and sought to enlist the populace on their side. Successful overmighty subjects were also idols of the multitude. Generally they failed and almost all died violently. Their misfortunes, a recovery of royal power and the disappearance of the desire to disturb the realm all help to explain the demise of their type. [source] Long-term survival of HIV-infected patients treated with highly active antiretroviral therapy in Serbia and MontenegroHIV MEDICINE, Issue 2 2007DO Jevtovi Background Highly active antiretroviral therapy (HAART) has dramatically changed the prognosis of HIV disease, even in terminally ill patients. Although these patients may survive many years after the diagnosis of AIDS if treated with HAART, some still die during treatment. Methods A retrospective study in a cohort of 481 HIV-infected patients treated with HAART between January 1998 and December 2005 was conducted to compare subgroups of long-term survivors (LTSs) and patients who died during treatment. Results A total of 48 patients survived for more than 72 months (mean 83.8±standard deviation 5.6 months). Thirty patients died during treatment (mean 35.3±25.0 months), of whom nine died from non-AIDS-related causes, 18 died from AIDS-related causes, and three died as a result of HAART toxicity. Although LTSs were significantly (P=0.015) younger at HAART initiation, age below 40 years was not a predictor of long-term survival. The subgroups did not differ in the proportion of clinical AIDS cases at HAART initiation, in the prevalence of hepatitic C virus (HCV) coinfection, or in pretreatment and end-of-follow-up CD4 cell counts. In contrast, the viral load achieved during treatment was lower in the survivors (P=0.03), as was the prevalence of hepatitis B virus (HBV) coinfection (P=0.03). Usage of either protease inhibitor (PI)-containing regimens [odds ratio (OR) 9.0, 95% confidence interval (CI) 2.2,35.98, P<0.001] or all three drug classes simultaneously (OR 7.4, 95% CI 2.2,25.1, P<0.001) was associated with long-term survival. Drug holidays incorporated in structured treatment interruption (STI) were also associated with a good prognosis (OR 14.9, 95% CI 2.9,75.6, P<0.001). Conclusions Long-term survival was associated with PI-based HAART regimens and lower viraemia, but not with the immunological status either at baseline or at the end of follow up. STI when CD4 counts reach 350 cells/,L, along with undetectable viraemia, was a strong predictor of long-term survival. [source] Good outcome in HIV-infected refugees after resettlement in New Zealand: population studyINTERNAL MEDICINE JOURNAL, Issue 5 2007S. M. Nisbet Abstract Background: The aims of this study were to determine the clinical characteristics on arrival and the subsequent clinical outcome of HIV-infected UN quota refugees who settled in New Zealand during the last 11 years and to estimate their rate of HIV transmission. Methods: A population study was conducted. Data were provided by the Mangere Refugee Resettlement Centre, the infectious disease physicians caring for the subjects, the New Zealand AIDS Epidemiology Group and laboratories carrying out HIV viral load assays. Results: One hundred of 7732 (1.3%) UN quota refugees were HIV positive; mean age 30 years, 56% were men, median initial CD4 count was 320 (range 20,1358). HIV infection was most commonly acquired by heterosexual intercourse (74%). The median follow up was 5.0 years (range 1 month to 9.7 years). Five died and 15 subjects had 16 AIDS-defining illnesses, most commonly tuberculosis (n = 10). Sixty subjects commenced highly active antiretroviral therapy of whom 36/59 (61%) had an undetectable HIV viral load after 1 year of treatment. None of the six children born to HIV-infected women in New Zealand were infected. There were two known cases of horizontal transmission of HIV infection. Conclusion: Although HIV-infected quota refugees often have to overcome severe social, cultural and financial handicaps, their clinical outcome is generally very good, with response rates to highly active antiretroviral therapy that are similar to other patient groups. Furthermore, they have not been a significant source of transmission of HIV infection after resettlement in New Zealand. [source] Analysis of chimerism during the early period after allogeneic peripheral stem cell transplantationINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 6 2001B. Gleissner As there are few reports on early evaluation of chimerism, we assessed fluorescence short tandem repeats (STR) by polymerase chain reaction (PCR) assays to analyse donor and recipient characteristics at early time points after peripheral stem cell transplantation (PBSCT). Peripheral blood of 13 patients was analysed in 1- to 2-day intervals starting from the day of PBSCT. Donor and recipient allelic patterns were determined by a commercially available multiplex STR assay that simultaneously evaluates four or five gene loci. Mixed chimerism appeared in all patients during days 1,9 after transplantation and preceded haematologic engraftment for 3,12 days. Even patients without myeloablative conditioning therapy (n=4) revealed donor allelic patterns within 1,5 days. Nine patients changed during the following days to a complete donor allelic pattern and had an uncomplicated post-transplant disease course. Four patients did not consistently retain complete donor chimerism; two of them relapsed within the next 3 months, one died from septicemia within 7 days, and the fourth, transplanted for aplastic anaemia, is still in complete remission. Overall, STR analysis using a simple and comparatively cheap multiplex system permits the detection of chimerism very early after transplantation and may provide relevant information that correlates with the clinical follow-up. [source] Application of cardiopulmonary bypass for resection of renal cell carcinoma and adrenocortical carcinoma extending into the right atriumINTERNATIONAL JOURNAL OF UROLOGY, Issue 3 2006TATSUMASA OCHI Aim:, The application of cardiopulmonary bypass to atrial involvement represents an important advance that has improved the safety and technical efficacy of a difficult surgical undertaking. Our experiences of the management of extended thrombi into the right atrium in patients with retroperitoneal malignancy using a cardiopulmonary bypass were discussed. Methods:, Data were reviewed for five patients (two men and three women; mean age, 60.4 years; range, 49,79 years) with retroperitoneal tumors displaying intracardiac tumor extension. Tumors originated in the right kidney in four patients, and in left adrenal gland in one patient. Cardiopulmonary bypass was used in all cases. Results:, Mean total blood loss was 6059 mL. Mean operative time was 14.7 h. No intra- or postoperative complications due to surgical technique were encountered, and no significant bleeding occurred during incision of the inferior vena cava or after removal of tumor thrombus. The follow-up period ranged from 3 to 20 months with a mean of 12.6 months. Of the five patients, three died of metastatic diseases, one died of liver dysfunction and one remains disease free as of 18 months postoperatively. Conclusions:, Our experience indicates that this procedure can be safely used for atrial involvement. Although superior long-term survival cannot be shown yet, favorable early results and a lack of perioperative complications were identified. [source] Retrograde endoscopic laser therapy and ureteroscopic surveillance for transitional cell carcinoma of the upper urinary tractINTERNATIONAL JOURNAL OF UROLOGY, Issue 1 2006SOICHI MUGIYA Objective:, To investigate the efficacy of endoscopic laser therapy and ureteroscopic surveillance for transitional cell carcinoma (TCC) of the upper urinary tract. Methods:, Tumors of the upper urinary tract were detected at ureteroscopy. After TCC was diagnosed by biopsy, retrograde endoscopic laser therapy was performed. Recurrent tumors were treated endoscopically and the patients were followed by ureteroscopic surveillance at 3- to 6-month intervals. Results:, Seven patients underwent ureteroscopic treatment. The tumor was grade 1 in five patients and grade 2 in two patients. The average tumor size was 1.3 cm. One patient with large, multifocal tumors died of metastatic disease, and one died of an unrelated cause. One patient requested nephroureterectomy after endoscopic treatment. The remaining four patients were followed up for a mean of 32 months after initial treatment. Each patient received an average of 5.3 ureteroscopic surveillance procedures while 3.3 recurrences on average were detected. Recurrence occurred in all the patients who showed normal radiographic findings. Urine cytology was also of little value in predicting tumor recurrence, except in one patient with carcinoma in situ. The recurrent tumors detected by ureteroscopy were successfully treated by repeated endoscopic procedures. After the follow up, three patients remained alive with no signs indicative of disease, but one patient with an initial grade 2 tumor died of recurrence after 30 months. Conclusions:, Given that ureteroscopic evaluation is essential for surveillance after endoscopic treatment of upper urinary tract TCC because of residual concern about recurrence, patients treated endoscopically should be recommended to undergo long-term endoscopic follow up. [source] The role of zoos in biosurveillanceINTERNATIONAL ZOO YEARBOOK, Issue 1 2007T. McNAMARA Zoos are ideally placed to act as epidemiological monitoring stations because for decades, many have been building up detailed collections of serum banks, tissue banks and medical record-keeping systems that could be mined for information that would be beneficial to public health. For example, in 1999 wild Crows Corvus brachyrhynchos in the United States of America started to die of unknown causes but it was not until some died in the grounds of a zoological institution that West Nile virus, which is a threat to both human and animal health, was identified. There is a serious disparity in the type and amount of biosurveillance provided for humans, agricultural livestock and wildlife agencies, often driven by economic factors. There is an argument for public-health entities to contribute funds to the cost of managing serum-banks and testing stations within zoos to enhance biosurveillance in urban settings, in a cost-effective and mutually beneficial manner. The key to sustainable and integrated biosurveillance lies in public-health professionals working with zoo professionals, who care for wild animals on a day-to-day basis, to create electronic surveillance networks. This could be of utmost benefit to everyone. [source] Non-invasive ventilation in do-not-intubate patients: five-year follow-up on a two-year prospective, consecutive cohort studyACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 9 2009H.-H. BÜLOW Background: End-of-life decisions are common in intensive care units (ICUs), and increasingly, non-invasive ventilation (NIV) is used as a ceiling of ventilatory care. However, little is known about the outcome following that decision. Methods: An observational, single-center, retrospective, follow-up study with no interventions, on ICU patients treated with NIV and a do-not-intubate (DNI) order. The patients were followed until a 5-year survival rate could be calculated. Results: One hundred and fifty-seven patients were treated with NIV during 2002 and 2003, and among 38 a DNI order was in effect. Of the 38 DNI patients, 11 died in the ICU, 16 died on the ward and 11 survived the hospital stay. Five of these 11 survivors died within 6 months, two died after 2.7 and 3.3 years, respectively, but four were still alive after 5 years. The long-term (>6 months) survivors have, surprisingly only been admitted to the hospital 0,2 times a year , and seldom with the need for ICU treatment. Conclusions: According to this study, and previous ones, it seems worthwhile treating DNI patients with NIV. Twenty-five to 35% leave the hospital alive, every 6th patient lives for at least 1 year, and this paper shows that 10% may survive for 5 years or more. However, only chronic obstructive pulmonary disease and chronic heart failure patients (both with a concomitant low APACHE score) seem to have a reasonable outcome, and patients should be informed about this. So far, no study has investigated the quality of life of these survivors. [source] Substrate and Procedural Predictors of Outcomes After Catheter Ablation for Atrial Fibrillation in Patients with Hypertrophic CardiomyopathyJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 10 2008T. JARED BUNCH M.D. Background: Hypertrophic cardiomyopathy (HCM) is often accompanied by atrial fibrillation (AF) due to diastolic dysfunction, elevated left atrial pressure, and enlargement. Although catheter ablation for drug-refractory AF is an effective treatment, the efficacy in HCM remains to be established. Methods: Thirty-three consecutive patients (25 male, age 51 ± 11 years) with HCM underwent pulmonary vein (PV) isolation (n = 8) or wide area circumferential ablation with additional linear ablation (n = 25) for drug-refractory AF. Twelve-lead and 24-hour ambulating ECGs, echocardiograms, event monitor strips, and SF 36 quality of life (QOL) surveys were obtained before ablation and for routine follow-up. Results: Twenty-one (64%) patients had paroxysmal AF and 12 (36%) had persistent/permanent AF for 6.2 ± 5.2 years. The average ejection fraction was 0.63 ± 0.12. The average left atrial volume index was 70 ± 24 mL/m2. Over a follow-up of 1.5 ± 1.2 years, 1-year survival with AF elimination was 62%(Confidence Interval [CI]: 66-84) and with AF control was 75%(CI: 66-84). AF control was less likely in patients with a persistent/chronic AF, larger left atrial volumes, and more advanced diastolic disease. Additional linear ablation may improve outcomes in patient with severe left atrial enlargement and more advanced diastolic dysfunction. Two patients had a periprocedureal TIA, one PV stenosis, and one died after mitral valve replacement from prosthetic valve thrombosis. QOL scores improved from baseline at 3 and 12 months. Conclusion: Outcomes after AF ablation in patients with HCM are favorable. Diastolic dysfunction, left atrial enlargement, and AF subtype influence outcomes. Future studies of rhythm management approaches in HCM patients are required to clarify the optimal clinical approach. [source] Prenatal diagnosis of congenital heart disease: Trends in pregnancy termination rate, and perinatal and 1-year infant mortalities in Korea between 1994 and 2005JOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (ELECTRONIC), Issue 3 2010Ji Eun Lee Abstract Aim:, To determine the pregnancy termination rate, and perinatal and 1-year infant mortality rates following prenatally-detected congenital heart disease (CHD) and trends over an 11-year period. Methods:, Between 1994 and 2005, 1603 gravidas underwent fetal echocardiography in our institution, in which 378 fetuses were diagnosed with CHD. The study period was divided into the following three groups for time-trend analysis: 1994,1997, 1998,2001, and 2002,2005. Data regarding gestational age at diagnosis and delivery, the presence of extracardiac or chromosomal abnormalities, pregnancy termination rate, and perinatal and 1-year mortalities were collected by review of medical records and telephone interviews. Results:, Among 378 fetuses with a prenatally-detected CHD, complete perinatal and infant outcomes were available for 336 fetuses (88.9%). There was a gradual increase in prenatally-detected CHD by fetal echocardiography during the study period (1994,1997, 10.3%; 1998,2001, 17.3%; and 2002,2005, 24.3%). The mean gestational ages at diagnosis and delivery were 27.2 ± 5.6 and 37.8 ± 2.9 weeks, respectively. Overall, the pregnancy termination rate in this study population was 20.2% and the perinatal and 1-year infant mortality rates were 6.3% and 9.7%, respectively. Among the fetuses who underwent cardiac surgery, surgical mortality occurred in two (3.8%); both died more than 1 month after surgery. Although the pregnancy termination rates remained unchanged, there was a significant decrease in perinatal and 1-year infant mortality rates over the study period. Conclusion:, Although the perinatal and 1-year infant mortalities following prenatally-detected CHD have continued to decrease significantly during the past 11 years, pregnancy termination rates have remained unchanged. [source] Fate of the unligated vertical vein after repair of supracardiac anomalous pulmonary venous connectionJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 7 2005YF Cheung Objective: To determine the fate of the unligated vertical vein after repair of isolated supracardiac total anomalous pulmonary venous connection (TAPVC). Methods: We reviewed the outcome of 28 patients who were diagnosed to have isolated supracardiac TAPVC and determined the fate of the unligated vertical vein. Results: Of the 28 patients, four died before surgery. The remaining 24 patients underwent surgical correction of TAPVC with (n = 5) or without (n = 19) ligation of vertical vein at a median age of 20 days (range: 1,574 days). There were no significant differences in age, weight, presence of pulmonary venous obstruction, need for preoperative inotropic and ventilatory support, cardiopulmonary bypass duration, postoperative pulmonary hypertensive crisis and requirement of peritoneal dialysis between patients with and those without vertical vein ligation. The in-hospital surgical mortality was 50% (12/24), with 83% (10/12) of deaths occurring before 1990. Patients who died after surgery were significantly younger (median age: 5.5 days vs 37 days, P = 0.005), lighter (3.3 ± 0.5 kg vs 3.9 ± 0.6 kg, P = 0.016), more likely to have pulmonary venous obstruction preoperatively (75% vs 12%, P = 0.039) and have undergone surgery before 1990 (83% vs 33%, P = 0.036). The 12 survivors were followed up for a median of 4.7 years (range: 2.3,18.1 years), 10 of whom had their vertical vein unligated. The vertical vein remained patent in five (50%) patients, while stenosis of pulmonary venous anastomosis was only present in one patient. Of these five patients, three had subsequently undergone surgical ligation of the vertical vein to eliminate a large left-to-right shunt. Conclusions: Patency of the unligated vertical vein is common after the repair of supracardiac TAPVC, even in the absence of pulmonary venous obstruction. The degree of left-to-right shunt through the patent vertical vein may be so significant as to warrant surgical ligation. [source] Detection of disseminated tumour cells in bone marrow of patients with isolated liver metastases from colorectal cancerJOURNAL OF SURGICAL ONCOLOGY, Issue 4 2003Kristin Bjørnland MD Abstract Background and Objectives In colorectal cancer (CRC) patients, tumour recurrence is common following potentially curative surgery for liver metastases. This may be caused by occult tumour cells present at the time of surgery. Dissemination of micrometastatic cells may occur early in patients with solid cancer, and micrometastases may signify a poor prognosis. The aim of the present study was to evaluate the frequency of micrometastatic cells in the bone marrow of patients with potentially resectable liver metastases. Methods Twenty millilitres of bone marrow was aspirated from both anterior iliac crests from 48 patients. Mononuclear cells were isolated and incubated with superparamagnetic Dynabeads® coated with an anti-epithelial monoclonal antibody (MOC31). Magnetically selected cells were identified by light microscopy as cells with bead rosettes (>5 beads/cell). Results Micrometastatic tumour cells were identified in four of 48 (8%) patients who all had their liver metastases surgically removed. Two of the four died after 17 and 18 months, respectively, whereas two are alive after 10 and 12 months. None of the 19 inoperable patients had micrometastases. Conclusions The frequency of bone marrow micrometastases in patients with clinically isolated liver metastases from CRC was low. This is biologically interesting, but bone marrow status should not affect current treatment protocols. J. Surg. Oncol. 2003;82:224,227. © 2003 Wiley-Liss, Inc. [source] Application of a Quality of Life Measure, the Life Situation Survey (LSS), to Alcohol-Dependent Subjects in Relapse and RemissionALCOHOLISM, Issue 11 2000J. H. Foster Background: Recent studies have shown that quality of life (QOL) is improved significantly when subjects do not relapse to heavy drinking, and QOL deteriorates significantly on prolonged relapse. This article further investigates these relationships using a QOL index, the Life Situation Survey (LSS). Methods: Eighty-two DSM-IV alcohol-dependent subjects admitted for alcohol detoxification were studied at baseline and 12 week follow-up. Sociodemographic data were collected, and severity of alcohol dependence (SADQ) and General Health Questionnaire (GHQ-12) were baseline indices only. The main outcome measure, the LSS, was administered at both time points. Results: Two subjects were lost to follow-up and one died during the study period. Thus, the relapse/nonrelapse analysis related to 79 subjects. Fifty subjects (63%) had relapsed to heavy drinking at 3 months follow-up. There was a significant correlation between LSS and GHQ-12 scores. Significant changes occurred in total LSS scores as a result of relapse and nonrelapse. The improvement in LSS scores associated with nonrelapse was larger than the deterioration that accompanied relapse. In those subjects who did not relapse to heavy drinking, the mean follow-up score remained in the poor/borderline LSS range. Remission from heavy drinking was accompanied by significant improvements in appetite, sleep, and self-esteem. Relapse to heavy drinking coincided with a significant deterioration in mood/affect, public support, and work/life role scores. Conclusion: QOL as assessed by the LSS in recently detoxified alcoholics is impaired significantly. In the nonrelapse group, there was a significant improvement in LSS scores after 3 months. Relapse was accompanied by a smaller deterioration in LSS scores. The LSS can play an important role in monitoring the clinical care and progress of alcohol-dependent subjects. [source] Hepatitis A acute liver failure: follow-up of paediatric patients in southern BrazilJOURNAL OF VIRAL HEPATITIS, Issue 2008C. T. Ferreira Summary., We retrospectively analysed 33 children and adolescents who had been hospitalized in a liver transplant unit within the previous 10 years for acute liver failure (ALF). The patients' age varied between 2 months and 15 years of age (median 6.2 ± 5.3), and 21 (63%) were male. Thirteen patients (39%) were immunoglobulin-M anti-hepatitis A virus (HAV) sero-positive. Eleven cases (33%) had an undetermined aetiology. The 13 children with HAV ALF were between 17 months and 15.6 years of age (median 5.8 ± 4.6) and eight were male (61.5%). All were on a list for urgent liver transplant. Of these, five (38%) died while waiting for a liver. Only one patient recovered spontaneously. Seven patients received a liver transplant; three died in the immediate postoperative period and one died 45 days after transplant. Three children are alive 1, 2 and 5 years after transplant. We conclude that HAV was the most frequent cause of ALF, which had high mortality even when a liver transplant was possible. The results support universal HAV vaccination in this area. [source] The model for end-stage liver disease score is the best prognostic factor in human immunodeficiency virus 1,infected patients with end-stage liver disease: A prospective cohort study,,LIVER TRANSPLANTATION, Issue 9 2009Javier Murillas End-stage liver disease (ESLD) has become the main cause of mortality in patients coinfected by human immunodeficiency virus (HIV) and hepatitis B virus or hepatitis C virus in developed countries. The aim of this study was to describe the natural history of and prognostic factors for ESLD, with particular attention paid to features affecting liver transplantation. This was a prospective cohort study in 2 Spanish community-based hospitals performed between 1999 and 2004. One hundred four consecutive patients with cirrhosis and a first clinical decompensation of their chronic liver disease or hepatocellular carcinoma were included in the study. During a median follow-up of 10 months (endpoint: death, liver transplantation, or the last checkup date), 61 patients (59%) died. The probability of mortality (Kaplan-Meier method) at 1, 2, and 3 years was 43% [95% confidence interval (CI), 34%,60%], 59% (95% CI, 48%,70%), and 70% (95% Cl, 59%,81%), respectively. In a multivariate analysis, the Model for End-Stage Liver Disease (MELD) score and the inability to reach an undetectable plasma HIV-1 RNA viral load at any time during follow-up were the only variables independently associated with the risk of death (P < 0.001). Fifteen (14%) of the 104 patients were accepted for liver transplantation, although only 5 underwent the procedure, and 10 died while on the waiting list. The waiting list mortality rate in patients with a MELD score < 20 and in patients with a MELD score >20 was 58% and 100%, respectively (median follow-up, 5 months). In conclusion, HIV-1,infected patients with ESLD, especially those with poorly controlled HIV and a high MELD score, have a poor short-term outcome. The MELD score may be useful in deciding whether to indicate liver transplantation in these patients. However, because only a small proportion of the patients in this study were considered candidates for liver transplantation and most died while on the waiting list, few received a transplant. Liver Transpl 15:1133,1141, 2009. © 2009 AASLD. [source] The role and limitation of living donor liver transplantation for hepatocellular carcinomaLIVER TRANSPLANTATION, Issue 3 2004Chung-Mau Lo Liver transplantation for hepatocellular carcinoma (HCC) is restricted by the scarcity of cadaver grafts. Living donor liver transplantation (LDLT) may potentially increase the applicability but its role and limitation are not clear. We studied the outcome of a cohort of 51 patients with unresectable HCC who were accepted on list for both options of deceased donor liver transplantation (DDLT) and LDLT. Twenty-five of 51 (49%) patients had voluntary living donors (group 1) and 26 did not (group 2). Patients in group 1 were younger, and more often had a MELD score more than 20 or blood group other than O. Twenty-one patients of group 1 underwent LDLT after a median waiting time of 24 days (range, 2,126 days), but 4 did not because the donors were not suitable (HBsAg-positive, 2; ABO-incompatible, 1; liver dysfunction, 1). Of the 30 patients who remained on list, only 6 underwent DDLT after a median waiting time of 344 days (range, 22,1359 days, P < .005). Nineteen died before transplantation and 2 were alive but taken off the list because of disease progression (drop-out rate, 70%). One patient was alive on list and 2 had undergone transplantation outside Hong Kong. The 1-, 2-, 3-, and 4-year intention-to-treat survival rates were 88%, 76%, 66%, and 66%, respectively, for group 1 and 72%, 46%, 38%, and 31%, respectively, for group 2 (relative risk of death for group 1, 0.35; 95% CI, 0.14 to 0.90; P = .029). In conclusion, although complicated factors such as donor voluntarism and selection criteria limit the role of LDLT for HCC, LDLT allows more patients to undergo early transplantation and results in a better outcome. (Liver Transpl 2004;10:440,447.) [source] Review: Neuropathology of acute phase encephalitis lethargica: a review of cases from the epidemic periodNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 5 2009L. L. Anderson Introduction: Encephalitis lethargica (EL), an epidemic disease of the early 20th century, has continued to be diagnosed sporadically since that time, including a report of 20 new cases in 2004. Many of the recent case reports state that the primary neuropathology of acute EL consists of inflammatory changes and lesions within the midbrain, basal ganglia and substantia nigra. However, the neuropathology of acute EL cases from the epidemic period was actually much more widespread. Methods: In order to characterize the neuropathology of acute phase EL, we developed a database of EL pathology based on 112 cases from the years 1915 to 1940, of which most died within 2 weeks of EL onset. Results: Our analysis revealed that cortical damage was prevalent in 75% of the 112 cases; damage to the meninges and brainstem occurred in approximately half of the cases; and the substantia nigra was damaged in only 13% of these acute cases. We also found that after 1921, damage to cranial nerve nuclei was not reported. An analysis of the neuropathology and clinical symptoms revealed little correlation. Conclusions: Based on these findings, putative modern cases of acute EL with MRI/CT indicated lesions confined solely to the midbrain, brainstem, and/or basal ganglia should not be considered, consistent with that reported during epidemic period. [source] Acute and Chronic Effects of Extensive Radiofrequency Lesions in the Canine Caval Veins: Implications for Ablation of Atrial ArrhythmiasPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 12 2006GUILHERME FENELON M.D. Background: Although radiofrequency (RF) ablation within the caval veins has been increasingly used to treat a variety of atrial tachyarrhythmias, the consequences of RF ablation in the caval veins are unknown. We explored the acute and chronic angiographic and pathological effects of extensive RF ablation in the caval veins. Methods: Under fluoroscopy guidance, conventional (4 mm tip, 60°C, 60 seconds) RF applications (n = 6,7) were delivered in each vena cava (from ±2 cm into the vein to the veno-atrial junction) of 15 dogs (10 ± 3 kg). Animals were killed 1 hour and 5 weeks after ablation for histological analysis. Angiography was performed before ablation (acute dogs only) and at sacrifice to assess the degree of vascular stenosis. Results: In acute dogs (n = 5), luminal narrowing was noted in 10/10 (100%) targeted veins (mild in two; moderate in three and severe in five, including two total occlusions). In the six chronic animals that completed the protocol (four died during follow-up), stenosis was also observed in 12/12 (100%) ablated veins (mild in six; moderate in four and severe in two). Of these, one superior vena cava was suboccluded with development of extensive collateral circulation. Histologically, acute lesions displayed typical transmural coagulative necrosis, whereas chronic lesions revealed intimal proliferation, necrotic muscle replaced with collagen, endovascular contraction, and disruption and thickening of the internal elastic lamina. Conclusion: In this model, extensive RF ablation in the caval veins may result in significant vascular stenosis. These findings may have implications for catheter ablation of arrhythmias originating within the caval veins. [source] Day 15 bone marrow driven double induction in young adult patients with acute myeloid leukemia: Feasibility, toxicity, and therapeutic results,AMERICAN JOURNAL OF HEMATOLOGY, Issue 9 2010Felicetto Ferrara The strategy named double induction (DI) in acute myeloid leukemia (AML) consists of two courses of chemotherapy irrespective of the degree of cytoreduction in the bone marrow (BM) after the first course, unless severe complications prohibit its application. We describe treatment results from a series of 33 patients in whom DI was adopted only after demonstration of persistence of more than 10% blast cells at day 15 (D15) examination of BM. All patients received as induction idarubicin, cytarabine, and etoposide. As second induction, we administered the combination of fludarabine, intermediate dose cytarabine, and Granulocyte colony stimulating factor (G-CSF). The median blast count at D15 was 30 (15,90). Overall, 30 of 33 patients were judged as eligible to receive DI, reasons for exclusion being in all cases active infection in the context of severe pancytopenia. Nineteen patients (63%) had unfavorable karyotype and 11 (37%) normal karyotype; seven of these had Fms-like tyrosine kinase gene internal tandem duplication (FLT3/ITD) mutation. Overall, complete remission (CR) was achieved in 20/30 patients (67%), while eight patients (27%) were refractory and two died of infectious complications. All refractory patients had unfavorable cytogenetics. All patients achieving CR were programmed to receive allogeneic stem cell transplantation (allo-SCT), which was actually performed in 11 patients. Our study suggest that D15 driven DI represents a feasible and effective therapeutic strategy in young adult AML patients, improving therapeutic results and not compromising feasibility of allo-SCT. When compared with conventional DI, it offers the potential to avoid unnecessary toxicity in a consistent proportion of patients. Am. J. Hematol., 2010. © 2010 Wiley-Liss, Inc. [source] Pacemaker Lead Infection in Young PatientsPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 7p1 2003DIDIER KLUG Transvenous pacing has become widespread in the pediatric population, but related pacemaker lead infection in young patients has rarely been reported. To determine prevalence and optimal management of pacemaker lead infection in children and young adults, the authors reviewed their pacing database including 4,476 patients who previously had pacemaker implantations from 1975 to 2001. A pacemaker was implanted in 304 patients under the age of 40. Of these patients 217 of them had congenital heart disease: 108 with structural defect, 109 without (mainly complete AV blocks). Among patients with congenital heart disease, 12 developed a pacemaker lead infection (5.5%, 6 patients with structural defect, 6 without). This incidence was significantly higher than in patients < 40 years at first implantation without congenital heart disease (2.3%) and in > 40-year-old patients(1.2%, P < 0.001). However, the number of reinterventions at the pulse generator site was higher in patients having had their first implantation before the age of 40. In patients with structural cardiac defect: two died after surgical lead extraction and one died before the scheduled lead extraction. The three remaining patients had successful surgical (n = 1) or percutaneous (n = 2) lead extractions. In patients without structural cardiac defect successful percutaneous extraction (5/6) or surgical extraction (1/6 with vegetation > 25 mm) was performed. One patient with percutaneous extraction developed chronic cor pulmonale during follow-up. One infection recurred in one patient with structural cardiac defect although complete removal of the pacing material had been performed. The prevalence of pacemaker lead infection is higher in younger patients, perhaps in part due to a higher number of procedures at the pacemaker site than in the general population of patients with a pacemaker. Patients with structural cardiac defect who underwent surgical lead removal were at high risk for death. Patients with percutaneous lead extraction may develop cor pulmonale. (PACE 2003; 26[Pt. I]:1489,1493) [source] Home care for chronic respiratory failure in children: 15 years experiencePEDIATRIC ANESTHESIA, Issue 4 2002L. APPIERTO MD Background:,Advances in paediatric intensive care have reduced mortality but, unfortunately, one of the consequences is an increase in the number of patients with chronic diseases. It is generally agreed that home care of children requiring ventilatory support improves their outcomes and results in cost saving for the National Health Service. Methods:,Since 1985, the Children's Hospital Bambino Gesù of Rome has developed a program of paediatric home care. The program is performed by a committed Home Health Care Team (HHCT) which selects the eligible patients for home care and trains the families to treat their child. During the period January 1985 to January 2001, 53 children with chronic respiratory failure were included in the home care program. Of these, seven patients were successively excluded and six died in our intensive care unit (ICU), while one still lives in our ICU since 1997. The results obtained in the remaining 46 children are reported. Results:,The pathologies consisted of disorders of respiratory control related to brain damage (26%), upper airways obstructive disease (26%), spinal muscular atrophy (22%), myopathies and muscular dystrophies (6.5%), bronchopulmonary dysplasia (6.5%), tracheomalacia (6.5%), central hypoventilation syndrome (4.3%) and progressive congenital scoliosis (2.2%). Of these 46 patients, 34 children are mechanically ventilated and the median of their ICU stay was 109.5 days (range 54,214 days), while the remaining 12 children were breathing spontaneously and the median of their ICU stay was 90.5 days (range 61,134 days). We temporarily readmitted six patients to our ICU to perform scheduled otolaryngological surgery, eight patients for acute respiratory infections and two patients for deterioration of their neurological status due to high pressure hydrocephalus for placement of a ventriculoperitoneal shunt; these 16 patients were discharged back home again. Two other patients were readmitted for deterioration of their chronic disease and died in our ICU, while seven patients died at home. Conclusions:,Thirty-seven children are still alive at home and four of them improved their respiratory condition so that it was possible to remove the tracheostomy tube. Our oldest patient has now achieved 15 years of mechanical ventilation at home. [source] Hepatitic pattern of graft versus host disease in childrenPEDIATRIC BLOOD & CANCER, Issue 5 2007Héctor Melín-Aldana MD Abstract Background Liver involvement by graft-versus-host disease (GVHD) is characterized histologically by bile duct damage, which may be severe. A different pattern, "hepatitic GVHD," has been described in adult patients. This pattern also shows marked lobular hepatitis and hepatocellular damage. We report the development of hepatitic GVHD in six pediatric patients. Procedure Clinical information and histologic features of liver biopsy samples were retrospectively reviewed. Results Patients' ages ranged from 3 to 11 years. Underlying diagnosis, pre-transplant conditioning and GVHD prophylaxis varied. Peripheral blood stem cells were the source of the allograft in four patients, matched sibling in one, and matched-unrelated donor in one. Hepatic GVHD was detected between 149 and 310 days post-transplant. Prior acute GVHD had developed in two patients, and involved the skin and/or gastrointestinal tract. No patients had significant ductopenia. Only one patient had significant lymphocytic infiltration of bile ducts (ductitis). Bile duct epithelial damage and significant portal/periportal inflammation were present in all patients. Lobular necro-inflammation was present in five patients. Five patients improved with immunosuppression and one died with progressive GVHD. Conclusions This series focuses on hepatitic GVHD in pediatric patients. Clinical and histologic patterns are similar to what has been described in adults. Specific etiology and pathogenesis of this entity remain unclear. Pediatr Blood Cancer 2007;49:727,730. © 2006 Wiley-Liss, Inc. [source] Desmoplastic small round cell tumor in childhood: The St. Jude Children's Research Hospital experiencePEDIATRIC BLOOD & CANCER, Issue 3 2007Raya Saab MD Abstract Background Desmoplastic small round cell tumor (DSRCT) is a rare, primarily intra-abdominal tumor that has a poor outcome with current therapies. Procedure We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of 11 pediatric patients with DSRCT at our institution. Results The cohort included 1 female and 10 male patients. Median age at diagnosis was 14 years (range 5,21 years). In eight (73%) patients, the primary tumor was abdominal or pelvic, and in one patient each, it was submental, mediastinal, and paratesticular. Nine (82%) patients had metastatic disease. All tumors showed polyphenotypic differentiation by immunohistochemistry. The EWS-WT1 transcript was detected in six of seven tumors tested. One tumor showed rhabdomyoblastic differentiation after therapy. All patients received chemotherapy; eight underwent surgical resection, seven received primary site radiation, and four received myeloablative chemotherapy with stem-cell support. Three (27%) patients are alive 23 months, 8 years, and 10 years from diagnosis. Two died of treatment-related toxicity, six died of disease. None of the patients in whom surgery and initial chemotherapy failed to induce complete remission survived. Conclusions DSRCT is an aggressive malignancy that does not respond well to contemporary treatments, and patients who do not enter complete remission after initial chemotherapy and surgery appear to have a particularly dismal outcome. Patients with localized extra-abdominal disease have a better prognosis, most likely due to increased feasibility of resection. Better understanding of molecular and genetic mechanisms of tumorigenesis and treatment-related changes may contribute to development of more effective therapy for DSRCT. Pediatr Blood Cancer 2007;49:274,279. © 2006 Wiley-Liss, Inc. [source] Neonates with severe infantile hepatic hemangioendothelioma: Limitations of liver transplantationPEDIATRIC TRANSPLANTATION, Issue 5 2009Enke Grabhorn Abstract:, IHHE as the most common vascular tumor of the liver in infancy can present with acute postnatal liver and congestive heart failure. LTx may be a lifesaving option, but can be complicated by extrahepatic involvement and bleeding complications, especially in neonates. Here we discuss the benefit of LTx in cases of acute postnatal deterioration and massive extent of the hepatic tumor. Three infants with IHHE were transplanted at our institution between 2005 and 2007. Two were neonates with acute postnatal decompensation and progressive liver and heart failure within days. Treatment with steroids and chemotherapy was ineffective; resection surgery and interventional treatment were not considered appropriate. LTx was performed at the age of 7 and 24 days, respectively. An additional infant with a bilobar tumor that evolved more slowly was transplanted on day-of-life 56. Patients 1 and 2 had to be resuscitated during the LTx procedure because of massive bleeding and both died during the procedure. Patient 3 had a complicated post-operative course but is doing well one-yr post-LTx. Neonates with extended hepatic and extrahepatic involvement of IHHE should be evaluated carefully prior to LTx. Whenever possible, alternative interventional treatment options should be considered. [source] Pediatric cardiac transplant: Results using a steroid-free maintenance regimenPEDIATRIC TRANSPLANTATION, Issue 1 2003H. Leonard Abstract: We report on survival, rejection, lymphoma and renal function following cardiac transplant using a steroid-free maintenance immunosuppressive regimen. We have performed 73 cardiac transplants in 71 children under 16 yr of age in the last 12 yr. There were eight perioperative and four late deaths giving actuarial survival of 88, 88, 85 and 70% at 1, 2, 5 and 10 yr, respectively. A total of 11 (15.3%) children had one episode of rejection (grade 3) in the first 6 months; one died and one was re-transplanted because of rejection. There was only one episode of late rejection (8 yr post-transplant) because of low drug levels in a patient with lymphoma and sepsis. This patient did not survive. Three other children (5.6%) also developed lymphoma and recovered but one died subsequently of graft failure. Four children have developed severe renal failure (glomerular filtration rate GFR <30 mL/min/m2). Two have not survived and one is expected to commence dialysis soon. The remainder have mild to moderate renal impairment. We report excellent survival and low rejection rates without use of long-term steroids. However the doses of cyclosporin used have had a significant effect on renal function in many cases. [source] CD5-Negative, CD10-Negative small B-cell leukemia: Variant of chronic lymphocytic leukemia or a distinct entity?,AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2002Salwa S. Sheikh Abstract CD5- and CD10-negative chronic lymphocytic leukemias are quite uncommon as compared to the CD5-positive CLL. We reviewed 250 sequential cases of peripheral blood lymphocytosis to characterize cases of small B-cell lymphoproliferative disorders, submitted with a clinical diagnosis of chronic lymphocytic leukemia exhibiting a non-classic immunophenotypic profile. Six cases of CD5-, CD10-negative chronic lymphocytic leukemias and no tissue involvement were identified that revealed high-density surface-membrane immunoglobulin and CD20 expression, with variable expression of CD11c, CD23, and CD25. Most had a profound leukocytosis (mean WBC 180 × 109/L) with proliferation of mature-appearing lymphocytes. Subsequent bone marrow biopsies showed diffuse infiltration by neoplastic cells in all evaluated patients. The clinical course appeared indolent, with follow-up revealing three patients alive (survival time 38,68 months), while two died of unrelated causes and one was lost to follow-up soon after diagnosis. These cases may represent somewhat unusual chronic lymphoproliferative disorders, with morphologic features and immunophenotypic profile not readily classifiable, but which are certainly atypical for classic chronic lymphocytic leukemia. Some of these features are reminiscent of those seen in marginal-zone lymphoma. However, it is most unusual for this known to be tissue-based disease to present primarily as leukemia rather than lymphoma. Am. J. Hematol. 71:306,310, 2002. © 2002 Wiley-Liss, Inc. [source] Is Sanfilippo type B in your mind when you see adults with mental retardation and behavioral problems?,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 3 2007Ute Moog Abstract Sanfilippo type B is an autosomal recessive mucopolysaccharidosis (MPS IIIB) caused by deficiency of N -acetyl-,- D -glucosaminidase, a lysosomal enzyme involved in the degradation of heparan sulfate. It is characterized by neurologic degeneration, behavioral problems, and mental decline. Somatic features are relatively mild and patients with this disorder can reach late adulthood. It is the most common subtype of MPS in the Netherlands and probably underdiagnosed in adult persons with mental retardation (MR). In order to increase knowledge on the adult phenotype and natural history in Sanfilippo type B, we present the clinical data of 20 patients with this disorder. Sixteen of them were followed for one to three decades. Six died between 28 and 69 years of age, mainly from pneumonia and cachexia; the surviving patients were 18,63 years old. Apart from the youngest, they had lost mobility at 36,68 years. Most had developed physical problems, in particular in the 4th,6th decade of life: cardiac disease (cardiomyopathy, atrial fibrillations), arthritis, skin blistering, swallowing difficulties requiring feeding by a gastrostomy tube, and seizures. The course of the disease was dominated in most of them by challenging behavioral problems with restlessness, extreme screaming and hitting, difficult to prevent or to treat pharmaceutically. Even in absence of knowledge of the history of an elderly patient with MR, the presence of behavioral problems should prompt metabolic investigation for MPS. © 2007 Wiley-Liss, Inc. [source] | ||||||||||||||||||||||||||||||||||||||||