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Showing papers by "Rambam Health Care Campus published in 2007"


Journal ArticleDOI
TL;DR: 18F-FDG PET/CT is a reliable noninvasive imaging modality for the diagnosis of vascular graft–related infection and enables accurate differentiation between graft and soft-tissue infection.
Abstract: Graft infection after prosthetic vascular reconstruction is an uncommon but severe complication. The clinical presentation is often subtle and nonspecific and may occur long after surgery. Although defining a prosthetic vascular graft infection can be difficult, early diagnosis and treatment are important because of the relatively high rates of amputation and death. The present study assessed the role of PET/CT using 18F-FDG for the diagnosis of vascular graft infections. Methods: Thirty-nine patients (35 men and 4 women; age range, 44–82 y) with suspected vascular graft infection underwent 18F-FDG PET/CT. The performance of PET/CT for the diagnosis of an infectious process and its localization to the graft or soft tissues was assessed. The final diagnosis was based on histopathologic findings and microbiologic assays obtained at surgery or on clinical and imaging follow-up. Results: PET/CT detected foci of increased 18F-FDG uptake suspected as infection in 27 patients and localized these findings to the graft in 16 patients. Vascular graft infection was confirmed in 14 of these patients (88%). PET/CT excluded graft involvement in 11 patients, and in 10 (91%) of these 11, long-term follow-up further confirmed that the infectious process was limited to surrounding soft tissues only. No abnormal 18F-FDG uptake was found in any of the 12 patients with no further evidence of infection. PET/CT had a sensitivity of 93%, specificity of 91%, positive predictive value of 88%, and negative predictive value of 96% for the diagnosis of vascular graft infection. Conclusion:18F-FDG PET/CT is a reliable noninvasive imaging modality for the diagnosis of vascular graft–related infection. The precise anatomic localization of increased 18F-FDG uptake provided by PET/CT enables accurate differentiation between graft and soft-tissue infection.

189 citations


Journal ArticleDOI
TL;DR: In this small study, Synbiotic 2000 had no effect on postoperative recurrence of patients with CD, and larger studies in patients with the inflammatory type of CD undergoing surgery, using higher doses of probiotics cocktail might prove effective.
Abstract: Complications of Crohn's disease (CD) lead to surgery in about 70-90% of patients. The majority of patients suffer from relapse of the disease. Colonic bacteria are essential to the development of CD. Therefore, a rationale exists in trying to prevent relapse by manipulation of gut microflora. This is feasible by treatment with probiotics or antibiotics. Synbiotic 2000 is a cocktail containing 4 probiotic species and 4 prebiotics. It is rational to pursue that it could be effective in preventing postoperative disease. We sought to check weather treatment with Synbiotic 2000 could prevent postoperative recurrence in patients with CD. This was a prospective multicenter, randomized study. Patients were randomized to active treatment or placebo in a 2:1 ratio. Follow-up consisted of endoscopic, clinical, and laboratory parameters. Thirty patients were enrolled. No differences were found between the 2 treatment groups regarding gender, age at diagnosis, age at surgery, weight, smoking status, type of disease, length of the resected segment, or medical treatment prior to surgery. No difference in either endoscopic or clinical relapse rate was found between patients treated with once daily dose of Synbiotic 2000 or placebo. In our small study, Synbiotic 2000 had no effect on postoperative recurrence of patients with CD. Larger studies in patients with the inflammatory type of CD undergoing surgery, using higher doses of probiotics cocktail might prove effective.

173 citations


Journal ArticleDOI
TL;DR: Marked skin hyperkeratosis due to impaired degradation of the stratum corneum corneodesmosomes was observed in the affected individuals, which suggests that matriptase plays a significant role in epidermal desquamation.
Abstract: In this article, we describe a novel autosomal recessive ichthyosis with hypotrichosis syndrome, characterized by congenital ichthyosis associated with abnormal hair. Using homozygosity mapping, we mapped the disease locus to 11q24.3-q25. We screened the ST14 gene, which encodes matriptase, since transplantation of skin from matriptase−/−-knockout mice onto adult athymic nude mice has been shown elsewhere to result in an ichthyosislike phenotype associated with almost complete absence of erupted pelage hairs. Mutation analysis revealed a missense mutation, G827R, in the highly conserved peptidase S1–S6 domain. Marked skin hyperkeratosis due to impaired degradation of the stratum corneum corneodesmosomes was observed in the affected individuals, which suggests that matriptase plays a significant role in epidermal desquamation.

172 citations


Journal ArticleDOI
TL;DR: Two new LAD cases are reported in which lymphocytes, neutrophils, and platelets share severe defects in β1, β2, and β3 integrin activation, and CalDAG-GEFI is identified as a critical regulator of inside-out integrinactivation in human T lymphocytes.
Abstract: Leukocyte and platelet integrins rapidly alter their affinity and adhesiveness in response to various activation (inside-out) signals. A rare leukocyte adhesion deficiency (LAD), LAD-III, is associated with severe defects in leukocyte and platelet integrin activation. We report two new LAD cases in which lymphocytes, neutrophils, and platelets share severe defects in β1, β2, and β3 integrin activation. Patients were both homozygous for a splice junction mutation in their CalDAG-GEFI gene, which is a key Rap-1/2 guanine exchange factor (GEF). Both mRNA and protein levels of the GEF were diminished in LAD lymphocytes, neutrophils, and platelets. Consequently, LAD-III platelets failed to aggregate because of an impaired αIIbβ3 activation by key agonists. β2 integrins on LAD-III neutrophils were unable to mediate leukocyte arrest on TNFα-stimulated endothelium, despite normal selectin-mediated rolling. In situ subsecond activation of neutrophil β2 integrin adhesiveness by surface-bound chemoattractants and of primary T lymphocyte LFA-1 by the CXCL12 chemokine was abolished. Chemokine inside-out signals also failed to stimulate lymphocyte LFA-1 extension and high affinity epitopes. Chemokine-triggered VLA-4 adhesiveness in T lymphocytes was partially defective as well. These studies identify CalDAG-GEFI as a critical regulator of inside-out integrin activation in human T lymphocytes, neutrophils, and platelets.

154 citations


Journal ArticleDOI
TL;DR: This database is very informative with respect to mtDNA phylogeny and mutational dynamics, and its size allows us to develop a nearest neighbor–based methodology for mtDNA haplogroup prediction based on HVS-I motifs that is superior to classic rule-based approaches.
Abstract: The Genographic Project is studying the genetic signatures of ancient human migrations and creating an open-source research database. It allows members of the public to participate in a real-time anthropological genetics study by submitting personal samples for analysis and donating the genetic results to the database. We report our experience from the first 18 months of public participation in the Genographic Project, during which we have created the largest standardized human mitochondrial DNA (mtDNA) database ever collected, comprising 78,590 genotypes. Here, we detail our genotyping and quality assurance protocols including direct sequencing of the mtDNA HVS-I, genotyping of 22 coding-region SNPs, and a series of computational quality checks based on phylogenetic principles. This database is very informative with respect to mtDNA phylogeny and mutational dynamics, and its size allows us to develop a nearest neighbor-based methodology for mtDNA haplogroup prediction based on HVS-I motifs that is superior to classic rule-based approaches. We make available to the scientific community and general public two new resources: a periodically updated database comprising all data donated by participants, and the nearest neighbor haplogroup prediction tool.

146 citations


Journal ArticleDOI
TL;DR: The pattern of mentalizing impairments in schizophrenia resembled those seen in patients with lesions of the frontal lobe, particularly with VM damage, providing support for the notion of a disturbance of the fronto-limbic circuits in schizophrenia.

120 citations


Journal ArticleDOI
TL;DR: Only a small proportion of eligible patients reach the intensive care unit, and early admission is imperative for their survival advantage, which was most pronounced among patients who acutely deteriorated while on hospital wards rather than among newly admitted patients.
Abstract: Objective: A lack of intensive care units beds in Israel results in critically ill patients being treated outside of the intensive care unit. The survival of such patients is largely unknown. The present study's objective was to screen entire hospitals for newly deteriorated patients and compare their survival in and out of the intensive care unit. Design: A priori developed intensive care unit admission criteria were used to screen, during 2 wks, the patient population for eligible incident patients. A screening team visited every hospital ward of five acute care hospitals daily. Eligible patients were identified among new admissions in the emergency department and among hospitalized patients who acutely deteriorated. Patients were followed for 30 days for mortality regardless of discharge. Setting: Five acute care hospitals. Patients: A total of 749 newly deteriorated patients. Interventions: None. Measurements and Main Results: Crude survival of patients in and out of the intensive care unit was compared by Kaplan-Meier curves, and Cox models were constructed to adjust the survival comparisons for residual case-mix differences. A total of 749 newly deteriorated patients were identified among 44,000 patients screened (1.7%). Of these, 13% were admitted to intensive care unit, 32% to special care units, and 55% to regular departments. Intensive care unit patients had better early survival (0–3 days) relative to regular departments (p = .0001) in a Cox multivariate model. Early advantage of intensive care was most pronounced among patients who acutely deteriorated while on hospital wards rather than among newly admitted patients. Conclusions: Only a small proportion of eligible patients reach the intensive care unit, and early admission is imperative for their survival advantage. As intensive care unit benefit was most pronounced among those deteriorating on hospital wards, intensive care unit triage decisions should be targeted at maximizing intensive care unit benefit by early admitting patients deteriorating on hospital wards.

108 citations


Journal ArticleDOI
TL;DR: The potential role of FDG‐PET/CT in the diagnosis of local recurrence and distant metastases of pediatric sarcomas was investigated.
Abstract: Background Combined positron emission tomography with 18fluoro-deoxyglucose and computed tomography (FDG-PET/CT) has been used in the diagnosis and staging of various malignancies, but their use in the management of pediatric sarcomas is less well defined. The potential role of FDG-PET/CT in the diagnosis of local recurrence and distant metastases of pediatric sarcomas was investigated. Procedure Nineteen children (aged 2–21) with sarcoma (9 Ewing sarcoma, 3 osteogenic sarcoma, 7 rhabdomyosarcoma) were evaluated between January 2000 and December 2005 by FDG-PET/CT for suspected local relapse or distant metastases. The results of 21 FDG-PET studies, 16 CT scans, 9 magnetic resonance imaging (MRI) studies, and 7 bone scans (BSs) were compared with surgical pathology or clinical follow-up for at least 3 months. Results FDG-PET detected local relapse in all seven patients and distant metastases in 10/13 (77%). FDG-PET/CT and CT/MRI/BS results were discordant in eight patients. FDG-PET/CT was the only modality that detected distant metastases in two patients. PET/CT was true negative and excluded disease in three patients with abnormal CT/BSs and was false negative in three patients with distant metastases. Conclusion FDG-PET/CT may be useful and complementary to other imaging modalities for the detection of recurrent pediatric sarcomas, especially at the primary site. Its potential advantages and limitations compared with conventional imaging modalities need to be further investigated in larger homogenous patient groups. Pediatr Blood Cancer 2007;49:901–905. © 2007 Wiley-Liss, Inc.

106 citations


Journal ArticleDOI
TL;DR: Lower HRQoL was found in adolescents with severe heart disease, and psychosocial factors have a significant effect on the psychological state of adolescents, and they should be addressed and treated.
Abstract: Objective: To assess health-related quality of life (HRQoL), depressed mood, and self-esteem in adolescents with heart disease and compare them with age-matched healthy adolescents (control group). Methods: Ninety adolescents (aged 12 to 18 years with congenital or acquired heart disease) and 87 controls completed the HRQoL (TAAQOL-CHD), Center for Epidemiologic Studies Depression scale, and Rosenberg self-esteem questionnaires. Relevant medical details were collected. The patients and their parents were asked to rate their perceived severity of heart disease. Results: Adolescents with severe heart disease reported higher levels of depressed mood and lower self-esteem than did adolescents with moderate and mild heart disease and age-matched healthy controls. Adolescents with severe heart disease also reported worse HRQoL than those with moderate and mild disease. According to the multiple regression analysis, 44% of variance of HRQoL was explained by the study variables. Disease severity alone explained 11% of the variance, but when entered with the other study variables, depressed mood, self-esteem, and adolescents’ perceived severity of disease were the only significant contributors to the explained variance of HRQoL. An exploratory mediation analysis, using the Sobel test, was therefore applied, and it showed that depressed mood and perceived disease severity, but not self-esteem, mediated the relationship between disease severity and HRQoL. Conclusions: Lower HRQoL was found in adolescents with severe heart disease. Psychosocial factors have a significant effect on the psychological state of adolescents, and they should be addressed and treated.

97 citations


Journal ArticleDOI
TL;DR: Implant placement in augmented areas presents high survival and radiologic success rates with minimal bone loss, and intraoral bone block graft surgery is a predictable operation for the use of dental implants.
Abstract: Background: The purpose of this study was to evaluate the survival and success of dental implants placed in alveolar bone following augmentation using intraoral block bone grafts. Methods: A consecutive retrospective study was conducted on patients who had onlay bone grafts for vertical or horizontal augmentations followed by dental implantation from 1999 to 2001. Files of 50 healthy patients who received 129 implants in augmented sites were reviewed. Implant survival, radiologic implant success (marginal bone loss), and complications were recorded. Results: Follow-up from time of implantation ranged from 6 to 67 months (mean: 24.3 ± 11.2 months). Ranges of implant widths and lengths were 3.25 to 4.7 mm and 10 to 16 mm, respectively. The overall survival rate was 96.9% (four implants were removed). Marginal bone loss around implants ranged from 0 to 3.3 mm (average: 0.22 ± 0.45 mm). Only 5% of the implants presented marginal bone loss ≥1.5 mm over the follow-up time. Conclusions: Intraoral bone block graft surgery is a predictable operation for the use of dental implants. Implant placement in augmented areas presents high survival and radiologic success rates with minimal bone loss.

95 citations


Journal ArticleDOI
TL;DR: Un-fractionated as well as low molecular weight heparin (enoxaparin), which exhibit a strong inhibitory activity towards heparanase, have proven efficacious in ulcerative colitis and Crohn's disease patients, suggesting that heparANase is actively involved in these pathologies and thus may be considered as a target for the development of anti-inflammatory therapies.

Journal ArticleDOI
TL;DR: The results demonstrated that the Truview blade is a useful option for tracheal intubation in patients with normal and anticipated difficult airways, and no significant difference was found in the anaesthetist's estimation ofintubation effort, tooth damage or postoperative stridor and hoarseness.
Abstract: Summary The Truview® blade facilitates a view of the vocal cords by indirect laryngoscopy. We prospectively compared the view obtained at laryngoscopy and intubating conditions of Truview (Group 1) or Macintosh (Group 2) blades in 170 patients who were scheduled to undergo general anaesthesia. We studied pre-operative airway evaluation, laryngoscopic view, duration of intubation, maximal force applied during intubation, anaesthetist's estimation of intubation effort on a 1–3 scale, bleeding, teeth and soft tissue damage, and postoperative stridor and hoarseness. The results demonstrated that, whilst the Truview produced a better laryngoscopic view and less maximal force applied during intubation, the duration of intubation was longer. No significant difference was found in the anaesthetist's estimation of intubation effort, tooth damage or postoperative stridor and hoarseness. Significantly fewer patients suffered bleeding and soft tissue damage following intubation with the Truview than with the Macintosh blade. The Truview blade is a useful option for tracheal intubation in patients with normal and anticipated difficult airways.

Journal Article
TL;DR: PET/CT provides fused images that demonstrate the complementary roles of functional and anatomic assessments in the diagnosis of cancer recurrence through the precise localization of suspected 18F-FDG foci and their characterization as malignant or benign.
Abstract: Early detection of recurrence is clinically important and can improve the prognosis and survival of patients with cancer. CT, considered the primary method of investigation because of its low cost and widespread availability, provides high-resolution anatomic details but may underestimate the actual tumor burden by overlooking small tumor clusters in areas of distorted anatomy after treatment. 18F-FDG PET is an effective whole-body imaging technique that detects metabolic changes preceding structural findings. However, the specificity of PET is impaired by false-positive or equivocal results attributable to the lack of precise anatomic landmarks and to sites of increased 18F-FDG uptake of nonmalignant etiology. PET/CT provides fused images that demonstrate the complementary roles of functional and anatomic assessments in the diagnosis of cancer recurrence through the precise localization of suspected 18F-FDG foci and their characterization as malignant or benign. In addition to the accurate diagnosis and definition of the whole extent of recurrent cancer, PET/CT has an impact on patient management because it can assist in defining potential candidates for surgery for cure, planning the appropriate surgical or radiotherapy approach, and referring patients with unresectable disease to other therapeutic options.

Journal ArticleDOI
TL;DR: PET/CT is an imaging modality with high diagnostic performance in the assessment of head and neck cancer, and induced a change in further clinical management in more than half of the study population.
Abstract: Background To assess the value of positron emission tomography/computed tomography (PET/CT) with 18 F-Fluorodeoxyglucose (FDG) in patients with head and neck carcinoma as compared with PET and conventional imaging alone, and to assess the impact of PET/CT on further clinical management. Study Design Prospective nonrandomized study. Setting Ninety patients with head and neck tumors had 107 PET/CT examinations. Results The study analysis showed that PET/CT had a sensitivity of 89%, specificity 95%, PPV 94%, NPV 90%, and accuracy of 92%. PET/CT altered management in 51 patients (56%). PET/CT eliminated the need for previously planned diagnostic procedures in 24 patients, induced a change in the planned therapeutic approach in 21 patients and guided biopsy in 6 patients. Conclusions PET/CT is an imaging modality with high diagnostic performance in the assessment of head and neck cancer, and induced a change in further clinical management in more than half of the study population.

Journal ArticleDOI
TL;DR: The results support the reliability and validity of the SSC-GIT 1.0 as a measure of SSc GIT involvement and further research is needed to examine the ability to detect change over time and define minimally important differences.
Abstract: Objective Gastrointestinal tract (GIT) involvement occurs in ∼90% of patients with systemic sclerosis (SSc) and has a major impact on health-related quality of life (HRQOL). We developed an HRQOL instrument for persons with SSc. Methods The Scleroderma Gastrointestinal Tract 1.0 (SSC-GIT 1.0) survey was developed after an extensive literature search, solicitation and consideration of experts' opinions, and 2 focus groups of 16 subjects with SSc and GIT involvement. A 75-item, self-reported measure assessing bowel involvement, emotional well-being, and social functioning was administered to subjects with SSc and GIT involvement. Also, subjects completed the Short Form 36 and rated the severity of their GIT symptoms (very mild to very severe), and items were transformed linearly to a scale with a possible range of 0 (worse health) to 100 (better health). Evaluation of psychometric properties included internal consistency reliability, test–retest reliability (1.3-week median time interval), multitrait scaling analysis, and exploratory factor analysis. Results Study participants (n = 88) were primarily female (95.5%), white (79.3%), and had a mean age of 52.4 years. Self-rated severity of GIT involvement ranged from very mild or mild (36.0%) to moderate (44.0%) to severe or very severe (20.0%). Of 75 items, 23 had low item-total correlations (≤0.39) and were excluded, leaving a 52-item instrument. Analyses supported 6 multi-item HRQOL scales: reflux/indigestion, diarrhea, constipation, pain, emotional well-being, and social functioning. Test–retest reliability estimates ranged 0.69–0.90 and Cronbach's alpha ranged 0.69–0.93. Participants who rated their GIT disease as mild had the highest scores (better health) on all 6 scales; participants who rated their GIT as severe had the lowest scores (poor health). Conclusion The results support the reliability and validity of the SSC-GIT 1.0 as a measure of SSc GIT involvement. Further research is needed to examine the ability to detect change over time and define minimally important differences.

Journal ArticleDOI
01 Jul 2007-Blood
TL;DR: The graft-versus-leukemia effect appeared effective, even in patients with unfavorable cytogenetics, however, treatment-related mortality was high and matched unrelated donor HSCT should be considered for all patients with favorable cytogenetic risk who lack a suitable HLA-matched sibling donor.

Journal ArticleDOI
TL;DR: The presence and size of ED and LE at multidetector CT is closely related to follow-up segment myocardial dysfunction and MFR, and if present, were significantly smaller than in segments remaining abnormal (P<05).
Abstract: Purpose: To prospectively evaluate the sensitivity of myocardial early perfusion defects (EDs) and late enhancement (LE) at multidetector computed tomography (CT) following acute myocardial infarction (AMI) to predict segment myocardial dysfunction and myocardial functional recovery (MFR), by using echocardiography as the reference standard. Materials and Methods: Institutional review board approval and informed consent were obtained. Twenty-six patients (25 men, one woman; mean age, 53 years ± 9 [standard deviation]), underwent baseline multidetector CT, coronary angiography, and echocardiography within a week of AMI and a follow-up echocardiography at 3 months. ED, LE, and late hypoattenuation were compared with regional left ventricular function and MFR. A logistic regression model and generalized estimating equation analysis were applied to estimate the predictive effect of ED and LE. Differences between groups were evaluated by using nonpaired Student t tests. Results: All EDs and LE corresponded wit...

Journal ArticleDOI
TL;DR: The results demonstrate for the first time a novel relationship between PON1 and PON3 expression and several inflammatory gastrointestinal disorders and it may be suggested that Pon1/2/3 may have extracellular functions as part of the host response in IBD and celiac disease.

Journal Article
TL;DR: Cardiac 18F-FDG uptake was lower in patients receiving bezafibrate and levothyroxine and higher in Patients receiving benzodiazepines, and manipulation of these drugs may represent tools for optimized PET/CT imaging.
Abstract: The effect of multiple patient-related factors on the degree of cardiac 18F-FDG uptake was assessed. Methods: Five hundred four consecutive patients undergoing routine 18F-FDG PET/CT studies completed a clinical questionnaire. 18F-FDG uptake was measured as the mean standardized uptake value within the heart delineated on the CT component of the study. Univariate and multivariate analyses assessed the influence of 51 clinical factors on cardiac 18F-FDG uptake. Results: On both multivariate and univariate analyses, cardiac 18F-FDG uptake was significantly lower in diabetics and in patients receiving bezafibrate or levothyroxine. Cardiac 18F-FDG uptake was significantly higher in men, patients younger than 30 y old, fasting duration of

Journal ArticleDOI
01 Jan 2007-Leukemia
TL;DR: RIC transplants show encouraging results in this older patient population with de novo AML, and in patients in 1st (CR), fewer relapses were counterbalanced by significantly increased NRM, so there was no survival advantage in this subgroup.
Abstract: We conducted a retrospective registry-based analysis to compare the outcome of 361 allogeneic human leukocyte antigen (HLA)-identical peripheral blood stem cell transplants (PBSCT) with reduced intensity conditioning (RIC) to that of 1369 autologous (auto) PBSCT in patients aged 50 years or older with de novo acute myeloid leukemia (AML), performed from 1997 until 2003 and reported to the European Group for Blood and Marrow Transplantation. Median age was 58 and 57 years in the RIC and auto groups, respectively. RIC patients had more advanced disease at the time of transplant. At a median follow-up of 24 months for RIC and 16 months for auto, multivariate analysis showed a lower risk for relapse (RR 0.77, P=0.013) without increased non-relapse mortality (NRM) in RIC patients (RR 1.26, P=0.28). Moreover, leukemia-free survival (RR 1.22, P=0.02) and overall survival (OS) (RR 1.32, P=0.005) were superior in the RIC group. In patients in 1st (CR), fewer relapses were counterbalanced by significantly increased NRM. Therefore, there was no survival advantage in this subgroup. In patients in 2nd or subsequent CR, LFS and OS were superior in the RIC group. RIC transplants show encouraging results in this older patient population with de novo AML.

Journal ArticleDOI
TL;DR: It is suggested that an early first full-term birth does not confer protection against breast cancer in BRCA mutation carriers and opting for a prophylactic oophorectomy as a breast and/or ovarian cancer risk-reducing strategy should complete childbearing prior to age 40 when this prevention modality is most effective.
Abstract: An early age at first full-term birth is associated with a reduction in the subsequent development of breast cancer among women in the general population. A similar effect has not yet been reported among women who carry an inherited BRCA1 or BRCA2 mutation. We conducted a matched case-control study on 1816 pairs of women with a BRCA1 (n = 1405) or BRCA2 (n = 411) mutation in an attempt to elucidate the relationship between age at first full-term pregnancy and the risk of developing breast cancer. Information about the age at first childbirth and other pregnancy-related variables was derived from a questionnaire administered to women during the course of genetic counselling. There was no difference in the mean age at first full-term birth in the cases and controls (24.9 years vs. 24.8 years; P = 0.81, respectively). Compared to women whose first child was born at or before 18 years of age, a later age at first full-term birth did not influence the risk of developing breast cancer (OR = 1.00 per year; 95% CI 0.98-1.03; P-trend = 0.67). Stratification by mutation status did not affect the results. These findings suggest that an early first full-term birth does not confer protection against breast cancer in BRCA mutation carriers. Nonetheless, BRCA mutation carriers opting for a prophylactic oophorectomy as a breast and/or ovarian cancer risk-reducing strategy should complete childbearing prior to age 40 when this prevention modality is most effective.

Journal ArticleDOI
TL;DR: A novel mutation in KRT5 is reported in a patient with clinical and histopathological features typical of Galli–Galli disease, a rare autosomal dominant disorder characterized by reticulate flexural hyperpigmentation associated with hyperkeratotic papules, pitted perioral scars and comedo-like lesions or cysts.
Abstract: Dowling–Degos disease (DDD; MIM 179850) is a rare autosomal dominant disorder characterized by reticulate flexural hyperpigmentation associated with hyperkeratotic papules, pitted perioral scars and comedo-like lesions or cysts. Characteristic histopathological features include filiform epithelial downgrowths of the rete ridges, typically involving the follicular infundibulum, basilar hyperpigmentation and dermal melanosis. Follicular retention cysts and perivascular mononuclear infiltrates can also be present. Galli–Galli disease is a term that has been used to describe patients displaying prominent acantholytic changes on histology, in addition to clinical and pathological features resembling those of DDD. Two mutations in KRT5, encoding one of the two major basal epidermal keratin intermediate filaments, were recently reported to underlie DDD in a number of European cases. In the present study, we report a novel mutation in KRT5 in a patient with clinical and histopathological features typical of Galli–Galli disease.

Journal ArticleDOI
TL;DR: It is shown that the effects of smoking/nicotine on CD and UC depend on the site of inflammation and not on the type of disease, and this assumption is based on animal and human studies.
Abstract: Smoking is an important environmental factor in inflammatory bowel disease (IBD) with differing effects in ulcerative colitis (UC) and Crohn's disease (CD). Never smoking and formerly smoking increase the risk of UC, whereas smoking exacerbates the course of CD. The potential mechanisms involved in this dual relationship are yet unknown. A reasonable assumption is that smoking has different effects on the small and large intestine. This assumption is based on animal and human studies that show that the effects of smoking/nicotine on CD and UC depend on the site of inflammation and not on the type of disease.

Journal ArticleDOI
TL;DR: In cancer of the nasopharynx, the diagnostic performance of PET/CT is better than that of stand-alone PET or CI and had a major impact on further clinical management in 57% of patients.
Abstract: Purpose: To assess the value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in patients with nasopharyngeal carcinoma as compared with PET and conventional imaging (CI) alone, and to assess the impact of PET/CT on further clinical management. Methods and Materials: Thirty-three patients with nasopharyngeal carcinoma had 45 PET/CT examinations. The study was a retrospective analysis. Changes in patient care resulting from the PET/CT studies were recorded. Results: Positron emission tomography/computed tomography had sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of 92%, 90%, 90%, 90%, and 91%, respectively, as compared with 92%, 65%, 76%, 86%, and 80% for PET and 92%, 15%, 60%, 60%, and 60% for CI. Imaging with PET/CT altered further management of 19 patients (57%). Imaging with PET/CT eliminated the need for previously planned diagnostic procedures in 11 patients, induced a change in the planned therapeutic approach in 5 patients, and guided biopsy to a specific metabolically active area inside an edematous region in 3 patients, thus decreasing the chances for tissue sampling errors and avoiding damage to nonmalignant tissue. Conclusions: In cancer of the nasopharynx, the diagnostic performance of PET/CT is better than that of stand-alone PET or CI. Positron emission tomography/computed tomography had a major impact on further clinical management in 57% of patients.

Journal ArticleDOI
TL;DR: It was concluded that palliative sedation may be the only efficacious and safe treatment for the alleviation of suffering in these children at the end of life, despite differing indications.
Abstract: Despite progress in the treatment of pediatric cancer, approximately 25% of these children will die of the disease. The last period of life is characterized by profound physical and psychological suffering, both of the children and their loved ones. Adequate alleviation of this suffering becomes the priority in the management of these patients. The authors retrospectively evaluated the indications, incidence, and characteristics of palliative sedation (PS) in 19 children with brain tumors (BT) and 18 with sarcomas (S) at the end of life. Twelve of the 18 S patients received PS, as did 13 of the 19 BT patients. Indications for initiation of PS for those with BT were seizures and/or pain, for those with S were pain and/or respiratory insufficiency. It was concluded that PS may be the only efficacious and safe treatment for the alleviation of suffering in these children at the end of life, despite differing indications.

Journal ArticleDOI
TL;DR: The relatively frequent presence of 1p/19q codeletion in both glial and sarcomatous components supports the notion that the sarcom atous component represents a metaplastic change occurring in the glial element, the same mechanism active in classic astrocytic gliosarcomas.
Abstract: Gliosarcomas are morphologically biphasic tumors composed of glial and sarcomatous elements Only rare examples of gliosarcoma with oligodendroglial components have been reported Seven patients with oligodendroglial tumors and a sarcomatous component were identified Fluorescence in situ hybridization for 1p/19q was sought in glial and sarcomatous regions in all cases Their mean age at diagnosis of gliosarcoma was 48 years (range 36 to 68) (F:M ratio=5:2) At first resection, the tumors included grade II oligodendroglioma (n=3), grade III oligodendroglioma (n=1), grade II oligoastrocytoma (n=1), and grade III oligoastrocytoma (n=2) The sarcomatous component developed in recurrent/progressive tumors in 6 cases but was a focal finding at first tumor resection in 1 and included fibrosarcoma (n=5), leiomyosarcoma (n=1), or pleomorphic myogenic sarcoma (n=1) Rhabdoid change was a focal finding in the sarcomatous component of 1 tumor The glial component expressed both glial fibrillary acidic protein and S-100 in all cases, whereas the sarcomatous component at least focally showed smooth muscle actin (n=6), CD34 (n=4), S-100 protein (n=3), and epithelial membrane antigen (n=2) reactivity Fluorescence in situ hybridization studies demonstrated 1p/19q codeletion in 5 cases, showed no evidence of deletion in 1 case, and technically failed in 1 case Three of the 5 cases demonstrated 1p/19q codeletion in the sarcomatous component as well Gliosarcomas with oligodendroglial elements are rare The relatively frequent presence of 1p/19q codeletion in both glial and sarcomatous components supports the notion that the sarcomatous component represents a metaplastic change occurring in the glial element, the same mechanism active in classic astrocytic gliosarcomas

Journal Article
TL;DR: Within the limits of this study, immediate restoration of dental implants in periodontally susceptible patients had a variable success rate.
Abstract: Purpose: Immediate restoration of dental implants in patients with a history of periodontal disease was examined. The influence of insertion torque and implant stability quotient (ISQ) on the survival rate was compared in immediately restored, nonrestored, and submerged implants. Materials and Methods: Patients received periodontal treatment after which “all in one” implant surgery was performed: hopeless teeth were extracted, debridement around remaining adjacent teeth was performed, implants were inserted and, in some cases, a prefabricated screw-retained provisional restoration was immediately delivered. Insertion torque and ISQ were recorded at baseline and 6 and 12 months postsurgery. Results: Nineteen patients were treated, and 74 implants were placed. Twelve implants, 10 of which were maxillary, failed in 4 patients. Survival rates were 100% in partial-arch restorations, 94% in the mandible, and 78% in the maxilla. The survival rate of restored implants was 65% in extraction sites versus 94% in healed, nonextraction sites. Implants exhibited a decrease in ISQ at 6 months followed by an increase at 12 months. There were no statistically significant differences in insertion torque or ISQ between failed and successful implants, restored and nonrestored implants, or extraction-site and nonextraction-site implants. Mandibular implants demonstrated higher insertion torque and higher ISQ at baseline and 6 and 12 months. Conclusions: Within the limits of this study, immediate restoration of dental implants in periodontally susceptible patients had a variable success rate. Several factors were shown to affect these results. (Comparative Cohort) INT J ORAL MAXILLOFAC IMPLANTS 2007;22:423–429

Journal ArticleDOI
TL;DR: Visual, but not auditory, triggers appear to be needed to prompt paradoxical kinesia in Parkinson's disease patients whose residential area was suddenly a war zone, under a life threatening missile attack, necessitating immediate evacuation.
Abstract: Paradoxical kinesia is the sudden transient ability of a patient with Parkinson's disease to perform a task he was previously unable to perform, usually when facing an immediate threat. The sensory cues governing this behavior and the prevalence in real life situations are unknown. The objective of this study was to determine the occurrence of paradoxical kinesia in Parkinson's disease (PD) patients whose residential area was suddenly a war zone, under a life threatening missile attack, necessitating immediate evacuation. Fifty PD patients were interviewed during and immediately following the war. Only two patients experienced paradoxical kinesia, one war related and the other historical, both in response to visual cues. In contrast, an auditory stimulus in the form of a frightening loud siren, warning patients of an imminent missile attack, did not induce paradoxical kinesia. When questioned about their general function during wartime, patients reported significant increases in OFF time (P < 0.01), dyskinesia (P < 0.009), anxiety (P < 0.002), and depression (P < 0.01) as compared with their performance before the war. Paradoxical kinesia is uncommon, even in the face of danger. Visual, but not auditory, triggers appear to be needed to prompt its occurrence.

Journal ArticleDOI
TL;DR: An artifact reduction algorithm (ARA) was developed to reduce metal artifacts in CT-based AC-PET and may improve detectability of small lesions located near metal hip implants.
Abstract: CT beam hardening artifacts near metal hip implants may erroneously enhance or diminish radiotracer uptake following CT attenuation correction (AC) of PET images. An artifact reduction algorithm (ARA) was developed to reduce metal artifacts in CT-based AC-PET. The algorithm employed a Bayes classifier to identify beam-hardening artifacts, followed by a partial correction of the attenuation map. ARA was implemented on phantom and patient 18F-FDG studies using a clinical PET/CT scanner. In phantom studies ARA successfully removed two artifacts of erroneously elevated uptake near a stainless steel hip prosthesis which were depicted in the standard CT-AC PET. ARA has also identified two targets absent on the scanner PET images. Target-to-background ratios were 1.5–3 times higher for ARA-PET than scanner images. In a patient study, metal artifacts were of lower intensity in ARA-PET as compared to standard images. Potentially, ARA may improve detectability of small lesions located near metal hip implants.

Journal ArticleDOI
TL;DR: In this experimental model, the use of bovine pericardium as a staple-line buttress in LSG was easy and safe; however, it did not decrease the occurrence of complications.
Abstract: We studied the usefulness of Bovine Pericardial strips (BPS) as a buttress in the prevention of complications from the gastric staple-line in laparoscopic sleeve gastrectomy (LSG). LSG was carried out in 18 pigs. Resection of the stomach was performed with 4.8-mm/ 30-mm linear stapler either without (Control Group – n = 9) or with BPS (Buttress Group – n = 9). Intra- and postoperative blood losses were assessed. Leaks were evaluated with methylene blue test intra-operatively and then clinically. The animals were sacrificed 2 weeks after surgery and the abdominal cavity was evaluated for fluid collections and adhesions, and the burst pressure of the stomach was measured and histopathological study of the staple-line was performed. Student t-test was used for statistical analysis. No leaks were detected except for one small subclinical leak in the buttress group. Internal ulcers at the staple-line were seen more frequently in the Buttress group (6 vs. 3, not significant). There was no significant difference between the two groups with regards to operative time (65.3 ± 14.2 min, 69.7 ± 12.8 min), intra-operative bleeding (9.6 ± 2.2 ml, 8.2 ± 1.5 ml), postoperative hemoglobin levels (11.3 ± 1.9 g%, 11.8 ± 2.2 g%), and burst pressure (152.6 ± 23.5 mmHg, 161.2 ± 15.8 mmHg) for the Control and Buttress groups respectively. More intense adhesions and inflammatory response were observed in the Buttress Group. In this experimental model, the use of bovine pericardium as a staple-line buttress in LSG was easy and safe; however, it did not decrease the occurrence of complications.