Eating disorder professionals' perceptions of oral health knowledge.

OBJECTIVE: The aim of this study was to assess the oral health knowledge among professionals who specialize in treating eating disorders, and identify to what extent their education, and training addresses oral health care delivery, and recommendations for individuals with eating disorders. METHOD: Participants for this study were licensed behavioural and medical providers specializing in eating disorder treatment (n = 107), and recruited through professional eating disorder organizations. Participants completed an anonymous, online questionnaire (33 items) assessing level of oral health-related education, knowledge and treatment recommendations within the participant's respective eating disorder discipline. RESULTS: The majority of respondents (85%) were formally trained in eating disorders, and of those trained, 64.4% were not satisfied with the level of oral health education during formal education, and 19.5% report no oral health education. Respondents consider their knowledge of risk of oral disease for their clients/patients as average or above (84%), and ranked tooth erosion as the greatest reason for oral care (63%) while dry mouth led in the rankings for least significant reason for oral care (33%). Referral for oral care was found to be more common after reports of complication (55%). DISCUSSION: According to these findings, eating disorder professionals regard oral health care for their clients as significant, and may be unaware of associated oral risk factors, current oral care standards and long-term oral effects of disordered eating apart from enamel erosion.

Liver transplantation in the United States, 1999-2008.

Changes in organ allocation policy in 2002 reduced the number of adult patients on the liver transplant waiting list, changed the characteristics of transplant recipients and increased the number of patients receiving simultaneous liver-kidney transplantation (SLK). The number of liver transplants peaked in 2006 and declined marginally in 2007 and 2008. During this period, there was an increase in donor age, the Donor Risk Index, the number of candidates receiving MELD exception scores and the number of recipients with hepatocellular carcinoma. In contrast, there was a decrease in retransplantation rates, and the number of patients receiving grafts from either a living donor or from donation after cardiac death. The proportion of patients with severe obesity, diabetes and renal insufficiency increased during this period. Despite increases in donor and recipient risk factors, there was a trend towards better 1-year graft and patient survival between 1998 and 2007. Of major concern, however, were considerable regional variations in waiting time and posttransplant survival. The current status of liver transplantation in the United States between 1999 and 2008 was analyzed using SRTR data. In addition to a general summary, we have included a more detailed analysis of liver transplantation for hepatitis C, retransplantation and SLK transplantation.

Disruption of transforming growth factor-beta signaling through beta-spectrin ELF leads to hepatocellular cancer through cyclin D1 activation.

Transforming growth factor-beta (TGF-beta) signaling members, TGF-beta receptor type II (TBRII), Smad2, Smad4 and Smad adaptor, embryonic liver fodrin (ELF), are prominent tumor suppressors in gastrointestinal cancers. Here, we show that 40% of elf(+/-) mice spontaneously develop hepatocellular cancer (HCC) with markedly increased cyclin D1, cyclin-dependent kinase 4 (Cdk4), c-Myc and MDM2 expression. Reduced ELF but not TBRII, or Smad4 was observed in 8 of 9 human HCCs (P<0.017). ELF and TBRII are also markedly decreased in human HCC cell lines SNU-398 and SNU-475. Restoration of ELF and TBRII in SNU-398 cells markedly decreases cyclin D1 as well as hyperphosphorylated-retinoblastoma (hyperphosphorylated-pRb). Thus, we show that TGF-beta signaling and Smad adaptor ELF suppress human hepatocarcinogenesis, potentially through cyclin D1 deregulation. Loss of ELF could serve as a primary event in progression toward a fully transformed phenotype and could hold promise for new therapeutic approaches in human HCCs.

Effect of time after transplantation on microbiology of urinary tract infections among renal transplant recipients.

We evaluated the microbiology of renal transplant recipients hospitalized with urinary tract infections over a 10-year period. While gram-negative organisms were seen most frequently (73%), the median time to infection post transplantation was shorter in patients infected with gram-positive organisms (9.0 vs. 44.7 months).

Enterococcus spp. in a single blood culture: bacteremia or contamination?

We retrospectively evaluated adult cases with Enterococcus spp. in 1 blood culture (BC) (1/1/2010-12/31/2015; n=294) and stratified them into bacteremia or contamination. Contamination frequency was similar in community versus hospital-onset, E. faecalis versus E. faecium, and number of BC drawn per day. Contamination predictors were vancomycin-resistance, ampicillin-resistance, commensal organism copresence, and nonurinary/abdominal sources.

Prospects for understanding avirulence gene function.

Avirulence genes are originally defined by their negative impact on the ability of a pathogen to infect their host plant. Many avirulence genes are now known to represent a subset of virulence factors involved in the mediation of the host-pathogen interaction. Characterization of avirulence genes has revealed that they encode an amazing assortment of proteins and belong to several gene families. Although the biochemical functions of the avirulence gene products are unknown, studies are beginning to reveal the features and interesting relationships between the avirulence and virulence activities of the proteins. Identification of critical virulence factors and elucidation of their functions promises to provide insight into plant defense mechanisms, and new and improved strategies for the control of plant disease.

Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002.

Mitten deformities of the hands and feet occur in nearly every patient with the most severe subtype (Hallopeau-Siemens) of recessive dystrophic epidermolysis bullosa, and in at least 40-50% of all other recessive dystrophic epidermolysis bullosa patients. Smaller numbers of patients with dominant dystrophic, junctional, and simplex types of epidermolysis bullosa are also at risk of this complication. Surgical intervention is commonly performed to correct these deformities, but recurrence and the need for repeated surgery are common. Higher numbers of epidermolysis bullosa patients also develop musculoskeletal contractures in other anatomic sites, further impairing overall function. Lifetable analyses not only better project the cumulative risk of mitten deformities and other contractures but also emphasize the need for early surveillance and intervention, since both of these musculoskeletal complications may occur within the first year of life.

Blood culture series benefit may be limited to selected clinical conditions: time to reassess.

Blood cultures are often submitted as series (two to three sets per 24 hours) to maximize sample recovery. We assessed the actual benefit of additional sets. Blood cultures submitted from adults (≥ 18 years old) over 1 year (1 February 2012 to 31 January 2013) were examined. The medical records of patients with positive cultures were reviewed. Cultures with commensal organisms were considered contamination in the absence of a source and clinical findings. The impact of additional sets on antibiotic therapy was estimated. We evaluated 15,394 blood cultures. They were submitted as two to five sets per 24 hours in 12,236 (79.5%) instances. Pathogens were detected in 1227 sets, representing 741 bacteremias, of which 618 (83.4%) were detected in the first set and 123 (16.6%) in the additional sets. Pathogens missed in the first set were recovered from patients receiving antibiotics (n = 72; 58.5%) and after undergoing a procedure (n = 54; 43.9%). The additional sets' results could have influenced antibiotic therapy in 76/6235 (1.2%) instances, including 40 (0.6%) antibiotic switches and 36 (0.6%) possible extensions of therapy. The potential impact of the detection of missed pathogens on antibiotic therapy was not apparent in patients who had an endovascular infection (26/27, 96.3%) and those who lacked an obvious source of pathogens (10/10, 100%). These findings suggest that one blood culture is probably adequate in patients with an obvious source of pathogens. Blood culture series are beneficial in patients without an obvious source of pathogens and in those with endovascular infections. It is time to reassess the benefit of blood culture series, perhaps limiting them to selected conditions.

AvrXa10 contains an acidic transcriptional activation domain in the functionally conserved C terminus.

The avrXa10 gene of Xanthomonas oryzae pv. oryzae, the causal agent of bacterial blight of rice, is a member of the avrBs3 avirulence gene family and directs the elicitation of resistance in a gene-for-gene manner on rice lines carrying the resistance gene Xa10. The carboxyl (C) terminus of AvrXa10 has a previously undescribed domain that is structurally similar to the acidic activation domain of many eukaryotic transcription factors in addition to three nuclear localization signal (NLS) sequences. Removal of the C-terminal 38 codons containing the putative activation domain, but retaining the NLS sequences, was concomitant with the loss of avirulence activity. The C-terminal coding regions of avrBs3 and avrXa7 can be replaced by the corresponding region of avrXa10, and the genes retained specificity for the resistance genes Bs3 in pepper and Xa7 in rice, respectively. The avrBs3 and avrXa7 avirulence activities of the hybrid genes were also lost upon removal of the terminal 38 codons. When fused to the coding sequence of the Gal4 DNA binding domain, AvrXa10 activated transcription in yeast and Arabidopsis thaliana. Removal of the carboxyl region severely reduced transcriptional activation. AvrXa10 would have to be localized to the host cell nucleus to function autonomously in transcriptional activation. Consistent with this requirement, mutations in all three NLS sequences of avrXa10 caused a loss in avirulence activity. The findings demonstrate the requirement of the C terminus for AvrXa10 function and the potential for the members of this family of avirulence gene products to enter the host nucleus and alter host transcription.

The National Epidermolysis Bullosa Registry.

Since its inception in 1986, the NEBR has proved to be an excellent example of how a relatively small allocation of federal research funds for the development of a registry of cases of a single rare disease can have a major impact on the rapid expansion in the depth of knowledge of not only the disease itself but of a number of associated biologic principles, including keratinization and epithelial cell-extracellular matrix interactions. At present, the NEBR is generating extensive clinical, laboratory, and demographic data, both from cross-sectional and longitudinal perspectives, as well as establishing a centralized cell and tissue bank that will serve the scientific community at large as a valuable resource for future basic research on this oftentimes devastating genetic disease.

Intracytoplasmic retention of type VII collagen and dominant dystrophic epidermolysis bullosa: reversal of defect following cessation of or marked improvement in disease activity.

It has been recently shown that the presence of perinuclear "stellate bodies" within the epidermis in patients with a form of dominant dystrophic epidermolysis bullosa named "transient bullous dermolysis of the newborn" corresponds to collections of type VII collagen. To determine the temporal relationship of this unique immunohistochemical defect with course of clinical disease activity, we have longitudinally studied the expression of two epitopes of type VII collagen (LH 7:2; L3d) in nine patients in four such kindreds by immunofluorescence and immunoelectron microscopic technique. In every infant so studied at the time of active blistering, type VII collagen was detectable primarily within basilar and, to a lesser extent, suprabasilar keratinocytes. In contrast, type VII collagen was detectable solely in linear array along the dermoepidermal junction in skin from each patient following complete cessation or at least marked diminution of visible clinical disease activity. These findings support the hypothesis that the temporary mechanical fragility and blistering of the skin in infants with this rare subset of dominant dystrophic epidermolysis bullosa reflect the presence of reduced amounts of type VII collagen along the dermoepidermal junction, and that this diminution may be the result of either a delay in transport and integration of type VII collagen from basilar keratinocytes into the skin basement membrane or excessive phagocytosis of type VII collagen.

Laparoscopic donor nephrectomy increases the supply of living donor kidneys: a center-specific microeconomic analysis.

BACKGROUND: A tenet of microeconomics is that new technology will shift the supply curve to the right. Laparoscopic donor nephrectomy (LDN) is a new technique for removal of living donor kidneys. Centers performing this procedure have noted an increased number of patients presenting for donor evaluation. This has not been previously studied. METHODS: The records of all LDN performed from May 1998 to February 1999 were reviewed. The following variables were examined: sex, age, related vs. unrelated donation, estimated blood loss, i.v. analgesia, length of stay, and time out of work. Donors undergoing traditional open donor nephrectomy during January 1997 to May 1998 served as the control group. A composite cost index was constructed. RESULTS: LDN significantly decreased length of stay, pain, and time out of work; the supply function shifted to the right. Telephone interviews revealed that 47% donated solely because of the LDN procedure. CONCLUSIONS: LDN increases the supply of living donor kidneys.

Graft function and outcome of older (> or = 60 years) donor livers.

Livers from donors > or = 60 years of age are often considered inadequate for transplantation by many centers. With waiting times exceeding 1 year in our region, we have aggressively used livers from this donor age group. Between 1990 and 1994, 209 patients received 223 liver grafts at our institution. Of these, 29 (13%) were from donors > or = 60 years of age (group A) and 194 (87%) were from donors < 60 years of age (group B). The two groups were matched for recipient diagnosis and severity of disease. Group A and B donors had similar liver, renal, and hematologic studies prior to donation. Weight, sex, race and vasopressor requirement were also similar. Postoperative alanine aminotransferase, aspartate aminotransferase,and prothrombin time were not significantly different over the first 10 postoperative days. Group A grafts were significantly more cholestatic than group B grafts on postoperative days 6-10. The retransplantation rate for primary graft nonfunction was not significantly different from group A (6.7%) and group B (3.4%; P=0.04). Patient and graft survival rates at 1 year were 58.6 % and 44.8% for group A and 79.2% and 74.5% for group B (P<0.001 for both). Four of 12 deaths in the first year in group A were completely unrelated to graft function. If these are excluded, patient and graft survival rates were 68% and 52%, which are better but still significantly less than in group B. Initial graft function of older donor livers are similar to that of the matched younger group. However, patient and graft survival rates were significantly worse for the older donors, even when corrected for unrelated deaths. Livers should not be discarded based on age alone without inspection and/or biopsy to rule out significant steatosis. Prompt retransplantation for primary graft nonfunction of older donors are generally more cholestatic than those from the younger donor age group; however, many of them function quite well. At the present time, given the inability to identify donor variables associated with decreased recipient survival, we recommend cautious use of older liver grafts in healthier recipients.

Simultaneous pancreas/kidney transplantation--a comparison of enteric and bladder drainage of exocrine pancreatic secretions.

Simultaneous pancreas/kidney transplantation (SPK) has evolved to become a therapeutic option for patients with renal failure resulting from type 1 diabetes mellitus. However, the appropriate route for drainage of the exocrine secretions of the pancreas allograft remains unclear. While bladder drainage (BD) is the current state of the art, it is associated with a high frequency of urologic complications, including urinary tract infections, hematuria, metabolic acidosis, dehydration, and reflux pancreatitis. Although enteric drainage (ED) is the more physiologic route, it has been associated in the past with decreased graft survival and increased infectious complications. In addition, BD offered a technique for detection of rejection through measurement of urinary amylase. However, with the advent of improved immunosuppression and antibiotic therapy, percutaneous pancreas biopsy, improved radiologic imaging, and greater understanding of pancreas transplantation, we hypothesized that ED could be performed without increased morbidity or cost. A group of 23 consecutive SPK was performed with ED during the period from July 1995 to November 1995. Another 23 age- and sex-matched recipients of SPK with BD performed from November 1994 to June 1995 served as a historical control group. Because of the differing lengths of follow-up, data were analyzed with respect to the first six months posttransplant. ED and BD were associated with equivalent actuarial one-year patient and graft survival rates: 100% and 88% for ED, and 96% and 91% for BD, respectively. Hospital charges, length of stay, readmissions, rejection, sepsis-related procedures were also equivalent in ED and BD. However, ED was associated with significantly fewer urinary tract infections and urologic complications. In addition, no grafts were lost as the result of sepsis. In the setting of SPK, ED represents a viable alternative to BD for primary drainage of pancreas exocrine secretions. Further studies with extended lengths of follow-up are necessary to confirm our observations.

Outcomes of laparoscopic donor nephrectomy in obese patients.

The applicability of laparoscopic donor nephrectomy (LDN) has not been assessed in the obese donor. We hypothesized that obesity is not a technical contraindication to LDN. From May 1998 to February 1999, 40 patients underwent LDN at the Georgetown Transplant Institute with the transperitoneal technique. Prophylaxis against deep venous thrombosis consisted of venous compression stockings, low-molecular weight heparin in obese patients, and early ambulation. The following variables were examined: donor sex, age, weight, height, related versus nonrelated donation, body mass index (BMI; wt/ht2), operating room time, estimated blood loss, length of stay, time out of work, and complications. BMI>31 indicates morbid obesity, BMI>27 indicates >20% over ideal body weight, and normal BMI is 25. The patients were divided into nonobese (BMI< or =31) and obese groups (BMI>31). The two groups do not differ in outcome after LDN. Our data indicate that obesity is not associated with increased morbidity or mortality after LDN.

Safe pancreas transplantation in patients with coronary artery disease.

BACKGROUND: This study was conducted to determine the risk of clinically significant posttransplant cardiac events (PCEs) in a cohort of diabetic patients referred for pancreas transplantation. METHODS: Between April 1991 and December 1995, 316 insulin-dependent diabetics were evaluated for pancreas transplantation. Patients were assessed for risk factors for coronary artery disease (CAD), and underwent screening for significant CAD by a standardized algorithm that included selective coronary angiography. For the 3-year period following transplantation, PCEs were identified, and related to pretransplant cardiac risk factors. RESULTS: Only four patients (1.3%) were turned down for cardiac contraindications. Coronary angiography was done in 74 patients (27% of the active transplant candidates) during the evaluation period because of the patient's history or a positive stress test. Significant coronary artery stenoses were found in 54% of the patients catheterized. Twenty-five of these 40 patients (63%) underwent revascularization with percutaneous transluminal coronary angioplasty and/or coronary artery bypass grafting. A total of 359 organs were subsequently transplanted into 194 of these patients. No deaths occurred within 30 days of any of the transplants; four percent of transplant recipients died of cardiac causes within the follow-up period (median 23 months). Those with no pretransplant evidence of CAD had significantly lower rates of PCE (2% and 8% at 1 and 3 years, respectively) than those with pretransplant evidence of CAD (11% and 29% at 1 and 3 years, P<0.01; relative risk, 4.3). CONCLUSIONS: Routine cardiac screening of pancreas recipients with selective angiography and revascularization allows patients with significant CAD to safely undergo pancreas transplantation. Patients should rarely be excluded from pancreas transplantation for cardiac causes.

Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension.

The association of pulmonary hypertension with portal hypertension, also called portopulmonary hypertension (PPHTN), is a known complication of chronic liver disease. Previously, the presence of PPHTN was considered to be a contraindication to orthotopic liver transplantation (OLT). Although there are selected case reports of successful OLT in the setting of PPHTN, an excessive mortality rate is associated with OLT and PPHTN. Heretofore, therapy for chronic management of PPHTN was lacking. Recently, continuous intravenous infusion of epoprostenol has been demonstrated to improve symptomatology and survival in the general population of patients with primary pulmonary hypertension. We now report the use of epoprostenol in the more specific instance of PPHTN. Over a period of 6-14 months, epoprostenol (10-28 ng/kg/min) therapy was associated with a 29-46% decrease in mean pulmonary artery pressure, a 22-71% decrease in pulmonary vascular resistance, and a 25-75% increase in cardiac output in a group of four patients. These results suggest that effective chronic therapy for PPHTN is available. In conjunction with inhaled nitric oxide as acute intraoperative therapy, epoprostenol infusion represents an additional therapeutic option for treatment of PPHTN in the liver transplant candidate.

Successful emergency transplantation of a liver allograft from a donor maintained on extracorporeal membrane oxygenation.

The critical shortage of cadaveric donors for organ transplantation has led many transplant centers to accept life-saving organs from donors who would have previously been refused for transplantation. We report a novel case of the use of a liver allograft from a donor whose oxygen delivery was maintained by extracorporeal membrane oxygenation (ECMO) for 29 days before suffering an anoxic brain injury from ECMO dysfunction. Liver transplantation was successfully performed in a patient with fulminant hepatic failure. Immediate graft function was obtained in the recipient, with full neurologic recovery and return to gainful employment 4 months after transplantation. ECMO may provide an intriguing option for the maintenance of organ function in the critically unstable brain-dead organ donor to salvage organs for transplantation. Further studies are currently underway.

Prevention of autoimmune islet allograft destruction by engraftment of donor T cells.

The results of clinical islet transplantation have remained poor when compared with the consistent success of pancreas transplantation. Autoimmunity has usually been discounted as a cause of islet transplant failure. Previously, we demonstrated that pancreas transplants from the diabetes resistant BB rat (BB-DR) function indefinitely in autoimmune diabetic hosts, but islets from the same donor are vulnerable to recurrent autoimmunity. Addition of 100 million pancreatic lymph node cells (PLNC) to BB-DR islets restores resistance to autoimmunity and leads to repletion of a T cell subset (RT6.1) in the recipients. Autoimmune (BB-Ac) and streptozocin (BB-Sz) diabetic BB rats were recipients of Wistar Furth (WF) intraportal islet or islets plus PLNC transplants with cyclosporine 5 mg/kg/day recipient treatment. One cohort of Brown Norway (BN) islet transplants to BB-Ac with CsA was performed. At the termination of the experiment, recipient peripheral blood lymphocytes (PBL) were characterized by flow cytometry (FACS) for class I, CD4, CD8, RT6.1, and RT6.2, a T cell maturation marker found in WF but not BB rats. All (14/14) WF and 75% (6/8) BN islet transplants to BB-Ac recipients failed after a mean of 42 and 36 days, respectively, despite CsA immunosuppression. WF islets were successful in 6/8 (75%) transplants to BB-Sz recipients (P<0.001 vs. BB-Ac recipients), confirming that autoimmunity is the major cause of islet failure in BB-Ac rats. Addition of PLNC to WF islets increased the survival in BB-Ac to 82% (9/11) (P<0.0001 vs. WF islets alone). Recipients of islet+PLNC express 19.7% RT6.2 compared with 4.6% and 4.0% for WF islets alone in BB-Ac (P<0.01) and BB-Sz (P<0.01), respectively. Autoimmunity is an important factor leading to islet transplant failure in autoimmune diabetic BB rats. Addition of donor PLNC prevent islet allograft failure and leads to recipient chimerism for a donor T cell subset (RT6.2) associated with resistance to autoimmunity.

Outcome after splenic vein thrombosis in the pancreas allograft.

The outcome and management of isolated splenic vein thrombosis in the pancreas transplant is unknown. We retrospectively reviewed the records of 76 simultaneous pancreas-kidney transplantations (SPK) and 56 solitary pancreas transplantations (SPT) performed at the University of Maryland from January 1995 to December 1996. A total of 24 patients were identified (9 SPK and 15 SPT recipients). All were systemically anticoagulated for a period of 6-8 weeks after diagnosis. In the SPK thrombosis group, anticoagulation resulted in 1-year graft survival that was equivalent to that of SPK controls (86.1% vs. 95.3%). In contrast, in SPT, thrombosis and subsequent anticoagulation were associated with decreased graft survival compared with SPT controls (26.8% vs. 78.3%; P<0.01). Although the outcome of splenic vein thrombosis in the absence of anticoagulation is unknown, these data suggest that (1) in SPK, anticoagulation for splenic vein thrombosis maintains graft survival, and (2) in SPT, anticoagulation does not alter the ultimate progression of splenic vein thrombosis to complete graft thrombosis.